Category: Case Studies

Spontaneous Bilateral Cavernosal Abscess

October 30, 2012/by admin Z.9

Cavernosal abscesses are rarely reported with the majority of reported cases having an identifiable underlying causative factor. We present the case of a 50-year-old male with poorly controlled diabetes mellitus and large abscesses of the bilateral cavernosal bodies. He was successfully treated with surgical debridement and healing of the wound by secondary intention.

Authors: Bradley, Nathan; Slobodov, Gennady; Culkin, Dan; Palmer, Blake

Corresponding Author: Bradley, Nathan

Introduction
We present the case of a 50 year-old male with bilateral cavernosal abscesses. These infections are a rare entity and have previously been reported in association with intracavernosal injection therapy (1,2), cavernosography (3) priapism (4,5), foreign body (6) and trauma (7). A literature search revealed five previously reported cases of spontaneous cavernosal abscesses, which are defined as abscesses without a definite underlying etiology (8,9,12,13,16). We present a case of spontaneous, bilateral cavernosal abscess in a patient with uncontrolled diabetes mellitus and balanitis xerotica obliterans (BXO).

Case Report
A 50 year-old male with poorly controlled diabetes mellitus presented to our institution with a three week history of worsening perineal swelling, pain, dysuria and weakening of his urinary stream. He had been evaluated at an outside facility a week prior and was treated with doxycycline. His medical history was significant only for poorly controlled diabetes mellitus, currently managed with oral hypoglycemics. His glycosylated hemoglobin level was found to 12.6% (normal 4.4-6.4%). He had no history of prior genitourinary surgery, instrumentation, urinary tract infections, STD or cavernosal injection therapy. On physical examination there was firmness and fluctuance of the perineum posterior to the scrotum, surrounding the urethra. Examination of his glans penis demonstrated white macules, suspicious for balanitis xerotica obliterans, with no apparent involvement of the meatus. Laboratory examination revealed a white blood cell count of 10,100 per mm3, serum glucose of 378 mg/dL. Microscopic urinalysis revealed 2 to 5 red blood cells per high power field and 2 to 5 white blood cells per high power field. A CT scan of the pelvis with contrast revealed a peripherally enhancing fluid collection involving the bilateral cavernosal bodies (see Fig. 1). Flexible cystourethroscopy was attempted in the operating room revealing external compression of the bulbar urethra by the cavernosal bodies. Operative debridement was then carried out through a midline perineal incision. The cavernosal bodies were found to be quite enlarged and tense. (see Fig. 2). Aspiration revealed purulent drainage and both cavernosal bodies were opened and irrigated (see Fig. 3). There was also abscess found in the corpus spongiosum requiring debridement to the level of the bulbar urethra. A Foley catheter was then placed and the debrided bulbar urethra closed over the catheter with an absorbable suture. After all necrotic tissue was debrided, a suprapubic catheter was placed and the perineal wound was packed with saline soaked gauze. Post-operatively blood glucose levels were tightly controlled and the patient was discharged home performing twice-daily wet-to-dry dressing changes. The perineal wound was allowed to heal by secondary intention with weekly wound checks in an outpatient clinic. Wound cultures for aerobes, anaerobes and fungal organisms grew only Lactobacillus spp. Blood and urine culture yielded no growth. After six weeks, a retrograde urethrogram demonstrated no significant flow-limiting urethral lesions or fistulae. At three month follow-up, healing of all wounds was complete. The patient reported no erectile dysfunction prior to development of the described process, however, objective measures of pre-operative erectile function were not available. Post-operatively he reported a decrease in the quality of his erections but was able to achieve erection sufficient for penetration with oral phosphodiesterase inhibitors.

Discussion
Spontaneous cavernosal abscess has previously been described as an abscess without an identifiable underlying cause (8). In previous reports of spontaneous cavernosal abscesses, attempts have been made to identify an underlying cause with proposed etiologies including infection from overlying skin (8), external trauma (9), and hematogenous spread with subsequent seeding of the cavernosa (8). Pearle and Wendel reported on a patient in whom oral pathogens were isolated from culture of the cavernosa with concurrent periodontal abscesses. (10). In our patient, the overlying skin was clearly uninvolved in the infection and there was no evidence to suggest an occult traumatic event leading to secondary infection. The patient had no dental abscesses, however hematogenous spread from dental caries with seeding of the cavernosal bodies in the setting of uncontrolled diabetes mellitus, remains a potential cause. The isolation of Lactobacillus spp. from cavernosal cultures at least partially supports this theory. This organism is known to inhabit the mouth, gastrointestinal tract and female genital tract (11). It is infrequently associated with clinically significant infections and the significance of its presence in our cultures is unknown. The patient was previously treated with a course of antibiotics and this organism may be a contaminant in the cultures. However, in a review of 241 cases of Lactobacillus infections with 39 cases being localized infection, Cannon et. al., identified eight cases of abscesses in which Lactobacillus spp. was isolated as the causative organism. The most common underlying factor in the 39 localized infections was diabetes, which was present in seven of their patients (11).
Various open surgical approaches have been described for management of cavernosal abscesses in the distal cavernosal bodies including percutaneous aspiration for small, uncomplicated abscesses. Post-operative drainage has been obtained with open packing (10), Penrose drains (12,13), as well as closed suction drains (4). Regardless of the operative approach chosen, the importance of aggressive initial debridement and close follow-up to monitor wound healing is illustrated by the case report from Ehara, et. al. This case describes a 54-year-old patient that underwent open surgical drainage of a cavernosal abscess with a Penrose drain left post-operatively. Three weeks after his initial operation he developed a recurrent abscess with methicillin-resistant Staphylococcus aureus. The infection failed to respond to medical treatment and the patient eventually went on to require total penectomy three months after his original surgery (12).
To our knowledge our case is the first report of isolated cavernosal abscess involving the proximal cavernosal bodies. Due to the location and extent of the abscess on pre-operative imaging, we followed principles of surgical management similar to those set forth for the management of Fournier’s gangrene (14). Our goals in the management of this patient were to extensively debride all necrotic and infected tissue, and to minimize the functional impairment after wound healing. The perineal incision provided sufficient exposure of both cavernosal bodies as well as the bulbar urethra. This allowed for extensive debridement of the involved tissue as well as repair of the involved urethra. Due to the extent of debridement, attempting primary closure of the wound would most likely have been unsuccessful and would have put the patient at risk for recurrent abscesses or secondary infections.
Cavernosal abscesses are a rare entity with potential for serious morbidity. Open surgical drainage has proven to be an effective treatment for this disease. Pre-operative patient counseling is crucial with regard to post-operative function and appearance of the genitalia. Treatment should be individualized to each patient based on their medical comorbidities and the unique characteristics of their infection.

References
1. Nalesnik JG, Jones L, Kraus S, Group B streptococcal septicemia following intracavernous injection therapy for erectile dysfunction in diabetes. J Urol, 2004:172: 151.
2. Kropman RF, De La Fuente RB, Venema PL, Van Imhoff WL, Treatment of corpus cavernosum abscess by aspiration and intravenous antibiotics. J Urol, 150: 1502, 1993.
3. Velcek D, Evans J, Cavernosography. Radiology, 1982:144: 781.
4. Schwarzer JU, Hofman R, Purulent corporeal cavernositis secondary to papaverine-induced priapism. J Urol, 1991:146: 845.
5. Sood R, Wadhwa SN, Jain V, Neonatal priapism associated with spontaneous bilateral pyocavernositis. Ann Acad Med Singapore, 2006:35: 425.
6. Peppas DS, Moul JW, Mcleod DG, Candida albicans corporal abscess following penile prosthesis placement. J Urol, 1988:140: 1540.
7. Niedrach WL, Lerner RM, Linke CA, Penile abscess involving the corpus cavernosum: a case report. J Urol, 1989:141: 374.
8. Sagar J, Sagar B, Shah DK, Spontaneous penile (cavernosal) abscess: case report with discussion of aetiology, diagnosis, and management with review of literature. The Scientific World Journal, 2004:5: 39.
9. Kumar S, Sharma SK, Goswami AK, Spontaneous bacterial abscess of corpus cavernosum. Indian J Uro, 2004:20: 66.

Fig. 1 Contrast-Enhanced CT Scan: Arrow – Fluid collection involving corpora cavernosa

Fig. 2 Contrast-Enhanced CT Scan: Arrows – Fluid collections in bilateral corpora cavernosa

Fig. 3 Intraoperative Photograph: Circle – Left corpora cavernosa, Large arrow – Purulent drainage from corpora cavernosa, Small arrow – Urethra

Date added to bjui.org: 30/10/2012

DOI: 10.1002/BJUIw-2012-023-web

The Position and Suction Power of the Pelvic Drain: A key point after Surgical Repair for Urinary Tract Injury Complicating Gynaecological Surgery

October 27, 2012/by admin Z.9

We report two cases with persistent urinary ascites in the bulbs of pelvic drains post surgical repair for urological complications, which subsided after adjusting the position and suction power of pelvic drains.

Authors: Chen, Kuo-Hu; Chen, Li-Ru; Yang, Bing-Shiang

Corresponding Author: Chen, Kuo-Hu

Kuo-Hu Chen1,2,, MD, PhD, Li-Ru Chen3,4, MD, MSc, Bing-Shiang Yang4, PhD, PE.

1 Department of Obstetrics and Gynecology, Buddhist Tzu-Chi General Hospital, Taipei Branch, Taipei, Taiwan.
2 School of Medicine, Buddhist Tzu-Chi University, Hualien, Taiwan.
3 Mackay Memorial Hospital, Main Branch, Taipei, Taiwan.
4 Department of Mechanical Engineering, National Chiao-Tung University, Taiwan.

Abstract
Pelvic drains and Foley catheters are widely used in the patients that undergo surgical repair for urological complications. However, persistent discharge from the drain tube, an implication of leakage at the site of repair due to the presence of urinary ascites, is a common reason why physicians do not remove the drain tube and Foley catheter. Herein, we report on two cases, where patients presented with urinary ascites after secondary surgery. It is supposed that a close position of the drain tube to the site of repair, as well as excessive suction power of the bulb, may be the primary cause of the persistent discharge, as these would produce a higher negative pressure, and thereby create a “fistula” effect. An attempt of moving the drain tube away from the site of repair and decreasing its suction power by reducing the compression of the bulb was made and appeared to be helpful. We suggest that this cause should be considered and managed in the patients who fail to have a drain removed because of persistent discharge, except the condition of true leakage from the site of repair.

Introduction
Iatrogenic injuries are the most common causes of lower urinary tract trauma. The close proximity of all pelvic organs makes the lower urinary tract susceptible to injury during various types of pelvic surgery [1]. Pelvic drains are often inserted into the body following surgical repairs to adequately remove discharges or bloody ascites produced during these operations, as well as to monitor whether there is any leakage that may have gone unnoticed. However, the pelvic drains themselves may become a primary source of complications that delay recovery.

Case report
Case 1
A 49-year-old, parous female was diagnosed with adenomyosis and chronic pelvic inflammation disease. She underwent a laparoscopic hysterectomy, during which severe pelvic adhesion was noted. In the postoperative period, her condition remained stable and she was afebrile. However, abdominal pain, distension, and oliguria developed after surgery. A physical examination found pelvic tenderness, and an abdominal ultrasound revealed pelvic ascites. An intravenous urogram was performed and revealed the findings of ileus, right ureteric obstruction, and hydronephrosis (Figure 1A), which may have resulted from a ureteric injury. Ureteroscopy followed by a laparotomy (including ureteroureterostomy and enterolysis) with an indwelling JJ catheter were arranged and performed meticulously by an experienced urologist one week after the first operation. A Jackson-Pratt (JP) drain was placed into the right pelvis at the end of the operation, and a Foley catheter was inserted into the bladder to ensure adequate decompression of the bladder.
After the second operation, the ileus subsided and the patient’s daily activity increased day by day. She remained afebrile and was able to independently maintain normal daily intake of food and water. Her condition improved, with the exception of the persistent discharge from the JP drain even three weeks after the second surgery, which is suggestive of urinary leakage from the site of repair. The daily amount of discharge from the JP drain decreased in the first two weeks after surgery, but increased in the third week, fluctuating between 30 and 100 mL. The discharge consisted of urinary ascites, as determined via biochemical testing, which revealed the presence of urine with a creatinine level of 22.1 mg/dL. Abdominal computed tomography and a plain abdominal radiograph of the kidney, ureter, and bladder (KUB film) were arranged and disclosed a close position of JP tube to the site of re-anastomosis (Figure 1B). We supposed that the close position of the JP drain tube and excessive suction power of the bulb may be the primary cause of the persisted discharge. Consequently, the tube was pulled out by 5-10 cm and the compression of the suction bulb was decreased by 50-75%. Similar to the method used in Whitson’s study, the suction power of the JP bulb we used (Jackson-Pratt, 100 mL) is gauged with a Mikro-tip catheter pressure transducer system (Millar Instruments, Inc., Houston, TX) by connecting the bulb to the reservoir of water in the pressure system after clamping the end of the drain tube. The negative pressure of the JP bulb was measured with a maximum of –130 mmHg. The “50-75%” reduction in negative pressure was confirmed by connecting the bulb again to the pressure transducer system after adjusting the JP bulb. This attempt at adjusting the position and suction power of the JP drain appeared to be helpful, as the daily amount of drainage decreased, and two weeks after, there was no drainage at all. The patient was then discharged after the removal of both the JP drain and Foley catheter, and she did not present with any sequelae at the follow-up appointment.

Case 2
A 34-year-old woman was diagnosed with poorly controlled diabetes mellitus in 2009. Her medical history included two caesarean sections, a right tubo-ovarian abscess, post-right salpingo-oophorectomy and pelvic adhesiolysis. She had suffered from persistent low abdominal pain, and was hospitalized because of a left tubo-ovarian abscess with sepsis. Prior to admission, her WBC count and CRP levels were 19,200/mL and 25.5 mg/dL, respectively.
The patient underwent an emergency laparoscopic left partial salpingo-oophorectomy and drainage of abdominal abscess. A bladder rupture with a laceration of 2 cm in diameter was noted during this operation, and it was laparoscopically repaired by cystorrhaphy with 2-0 vicryl. A culture of the pus in the abscess yielded mixed bacterial and yeast growth, including Candida albicans. After surgery, her diabetes mellitus was controlled with oral hypoglycemic agents, and her infection was treated by administration of intravenous antibiotics. A JP drain was placed into the lower pelvis at the end of the surgery, and a large French Foley catheter was inserted to ensure decompression of the bladder.
The discharge from the JP drain decreased after the surgery. When the daily amount of discharge was less than 5 mL (near zero) for five more days, an intravenous urogram was performed and showed an intact urinary tract (Figure 2A). She began to undergo Foley clamping, and was prepared for the removal of the JP drain and Foley catheter. Unfortunately, the discharge from the JP drain increased to 300 mL per day, and it was confirmed that there was the presence of urinary ascites, a sign of poor healing at the repair site of the bladder. A cystogram revealed contrast medium leakage in the left anterior aspect of the urinary bladder (near the bladder dome), and that the JP tube was close to the site of repair (Figure 2B). Based on our previous successful experience, the JP tube was pulled out by 5-10 cm and the compression of the suction bulb was decreased by 50-75%. Her condition improved, and the daily amount of discharge from the JP drain began to decrease until there was less than 5 mL being discharged for more than 10 days. Another cystogram was performed and revealed an intact urinary tract without any leakage from the site of repair. Subsequently, the patient had the Foley catheter removed, and was able to void from her bladder. Following the removal of the Foley catheter, there was no discharge from the JP drain, which was then removed on the next day. The patient was in a stable condition and discharged. She did not present with any sequelae on follow-up.

Discussion
Many types of drains, including Penrose, Surgidyne, Jackson-Pratt, and Hemovac, are used in a variety of surgeries for different purposes [2]. Of these, the JP bulb has demonstrated medium suction power and volume collection [3]. The drains are inserted into the body following major surgery for different purposes, such as to adequately remove discharges or bloody ascites produced during these operations, or to monitor whether any injury occurs and may have gone unnoticed. Nevertheless, the drains themselves can become a primary source of complications that delay recovery.
Gynaecological operations are the major causes of iatrogenic injuries in the urinary tract [4]. Some patients may experience symptoms of incontinence [5], which are due to the development of ureterovaginal or vesicovaginal fistulas, or may present with massive urinary ascites [6,7], if the injury is not found and managed. Current evidence suggests that repair by laparoscopic surgery [8-10] and repair at an earlier stage [11] are feasible, if surgical intervention is considered. Pelvic drains are commonly used after surgical repair of iatrogenic injuries. However, persistent discharge observed at bedside, which may imply leakage from the site of re-anastomosis, is often confusing to clinicians, and consequently, they hesitate to remove the pelvic drains. We suppose that the high negative pressure resulting from the close position of the tube to the repair site and the strong suction power of the bulb creates a “fistula” effect between the drain and the site of repair, thereby preventing the tissues from healing appropriately. Thus, the negative pressure that is usually beneficial in the reconstruction of tissues at other times may inversely bring about negative consequences by breaking down the healing tissues.
To our best knowledge, this is the only report to demonstrate the effects of the position of drain tubes and suction power of the bulbs on the healing of the repair site. A literature review via PubMed reveals only one article that reports on a relatively similar concept. Specifically, it was reported that intermittent clamping of suction drains following a total hip replacement reduces postoperative blood loss [12]. However, there are no specific explanations for the motivation and for the observations made in the present study. Nonetheless, our experience provides evidence that persistent urinary leakage from the site of re-anastomosis, despite meticulous repair, can be improved by adjusting the position of the drain tubes and suction power of the bulbs. By doing so, the potential “fistula effect” can be eliminated, thereby improving healing by providing a dry environment, other than the consideration of preventing infection and controlling the underlying medical disorder in general wound care.

Conclusion
The presence of persistent urinary ascites in the bulbs of pelvic drains following surgical repair of urological complications can be reduced by adjusting the position and suction power of the pelvic drain.

References
1. Wagner JR, Russo P. Urologic complications of major pelvic surgery. Semin Surg Oncol. 2000;18(3):216-28.
2. Durai R, Ng PC. Surgical vacuum drains: types, uses, and complications. AORN J. 2010;91(2):266-71.
3. Whitson BA, Richardson E, Iaizzo PA, Hess DJ. Not every bulb is a rose: a functional comparison of bulb suction devices. J Surg Res. 2009;156(2):270-3.
4. Brandes S, Coburn M, Armenakas N, McAninch J. Diagnosis and management of ureteric injury: an evidence-based analysis. BJU Int. 2004;94(3):277-89.
5. Mandal AK, Sharma SK, Vaidyanathan S, Goswami AK. Ureterovaginal fistula: summary of 18 years’ experience. Br J Urol. 1990;65(5):453-6.
6. Hassan I, López C, Gee H, Toozs-Hobson P. Urinary ascites following caesarean section: an unusual presentation of bladder injury. Eur J Obstet Gynecol Reprod Biol. 2009;147(2):237-8.
7. White V, Hardwick RH, Rees JR, Slack M. Massive urinary ascites after removal of a supra-pubic catheter: case report and review of the literature. Int Urogynecol J Pelvic Floor Dysfunct. 2007;18(7):831-3.
8. Asimakopoulos AD, D’Orazio A, Pereira CF, et al. Surgery illustrated–focus on details: laparoscopic repair of obstructing retrocaval ureter. BJU Int. 2011;107(8):1330-4.
9. Taskin O, Wheeler JM. Laparoscopic repair of bladder injury and laceration. J Am Assoc Gynecol Laparosc. 1995;2(2):227-9.
10. Dalessandri KM, Bhoyrul S, Mulvihill SJ. Laparoscopic hernia repair and bladder injury. JSLS. 2001;5(2):175-7.
11. Badenoch DF, Tiptaft RC, Thakar DR, Fowler CG, Blandy JP. Early repair of accidental injury to the ureter or bladder following gynaecological surgery. Br J Urol. 1987;59(6):516-8.
12. Brueggemann PM, Tucker JK, Wilson P. Intermittent clamping of suction drains in total hip replacement reduces postoperative blood loss: a randomized, controlled trial. J Arthroplasty. 1999;14(4):470-2.

Figures

Figure 1 A

Figure 1 B

Figure 2 A

Figure 2 B

Figure Legends

Figure 1: (A) Intravenous urogram revealing ileus, right ureteral obstruction, and hydronephrosis. (B) Plain film of kidney, ureter, and bladder (KUB) revealing the close position of the JP tube to the site of re-anastomosis (arrow), which may be a primary cause of persistent discharge by producing a higher negative pressure, and thereby creating a “fistula” effect.
Figure 2: (A) Intravenous urogram showing an intact urinary tract. (B) A cystogram revealing contrast medium leakage in the left anterior aspect of the urinary bladder (near the bladder dome), and a JP tube close to the site of repair (arrow), which may be a primary cause of persistent discharge by producing a higher negative pressure, and thereby creating a “fistula” effect.

Date added to bjui.org: 27/10/2012

DOI: 10.1002/BJUIw-2012-021-web

Delayed renocolic fistula formation following percutaneous radiofreqency ablation of renal mass: case report and review of the published cases

October 24, 2012/by admin Z.9

We report a case of a 71-year-old man who developed a renocolic fistula 10 months after radiofrequency ablation. At the time of fistula formation there was no evidence of tumor recurrence.

Authors: Douglas, Sean; Ogles, Mitchell; Vick, Kenneth; Pound, Charles R.

Corresponding Author: Douglas, Sean

Introduction
Image-guided radiofrequency ablation (RFA) is an established minimally invasive treatment for renal cell carcinoma (RCC) in patients with significant comorbidities or limited renal function (1). Reported complication rates of image-guided RFA are lower than for extirpative surgery, ranging from 0% to 11% for RFA and 14% to 26% for surgical excision (2). Renocolic fistula is a rarely described complication. We present a case of delayed renocolic fistula following radiofrequency ablation of a small renal cell carcinoma and a review of the three previously reported cases.

Case Report
The patient is a 71-year-old white male who was found to have an anterior right lower pole, 2.1 centimeter exophytic renal mass after abdominal computed tomography (CT) for vague abdominal pain. The patient elected to undergo CT guided radiofrequency ablation. Intra-procedural and post-procedural imaging showed no evidence of injury to adjacent organs. At four month follow up the patient had no complaints, and there was no evidence of tumour recurrence on CT scanning. At an unscheduled ten month appointment he reported pneumaturia and had developed a urinary tract infection. CT demonstrated air within the collecting system. The ascending colon was now immediately adjacent to the area of ablation concerning for a raising concern that the patient may have a renocolic fistula. A barium enema demonstrated filling of the right renal pelvis confirming the fistula.

Right partial nephrectomy and right hemicolectomy was performed without intraoperative complication. The collecting system defect was closed with interrupted absorbable sutures and the renal defect was closed with absorbable sutures bolstered with pledgets and Surgiseal. Pathological evaluation of the specimen revealed no residual tumor. His post operative course was complicated by colonic anastomotic leak and intra-abdominal abscesses requiring the placement of percutaneous drains, open debridement, and eventual placement of an end ileostomy over a nine month period. His ileostomy has been reversed. He is doing well with no tumor recurrence 29 months post ablation.

Discussion
We have identified 2 cases of renocolic fistula directly attributable to treatment of a renal tumour, one percutaneous and one surgical assisted, and one further case which was confounded by extensive local tumor recurrence.
In a cohort of 24 patients, Weizer et al reported on one fistula occurring in a 56 year old male with a 1.8 cm left sided anterior upper pole lesion and a solitary kidney who underwent percutaneous RFA. The fistula manifested 7 days post ablation. The renal unit was successfully salvaged with nephrostomy tube decompression and total parenteral nutrition. (3)
Medina et al reported a case of renocolic fistula in a patient with a left solitary kidney who had undergone two previous partial nephrectomies intraoperative RFA (approach not reported). In this case the renocolic fistula manifested 7 days postoperatively as watery diarrhea. The patient was treated with double J stent placement, colonic resection and closure of the renal fistula tract. Four days after repair the patient developed a renoperitoneal fistula and ultimately had to undergo a radical nephrectomy. Pathology demonstrated Fuhrman grade 1/4 clear cell carcinoma. (4)
Uribe reported on a 78 year old man who underwent laparoscopic RFA of a 3 cm left sided lesion that was to be followed by laparoscopic partial nephrectomy (ablate and resect arm of a prospective study). The resection was abandoned due to an intraoperative cardiac arrhythmia and subsequent myocardial infarction. Eight months later the patient’s renal mass had increased in size to 18 cm with invasion through the wall of the colon and fistula formation. Eventual resection revealed a Fuhrman grade 4/4 clear cell carcinoma. In this case of aggressive local recurrence, it is difficult to directly attribute the fistula to the RFA procedure, although it may have played some role. (5)
Of the above two cases without tumor recurrence, renocolic fistula was diagnosed within 7 days of treatment. Our patient was completely asymptomatic until several months post ablation. Post RFA renocolic fistulas seem to manifest with typical signs and symptoms: urinary tract infections, pneumaturia, and watery diarrhea. Standard urological imaging techniques provide confirmation of the suspected diagnosis.
The location of the renal lesion is relevant in preventing bowel injury during RFA. Anterior tumors are more likely to be in contact with intra-abdominal contents and thus increase the risk of bowel injury (1,6). A 5 mm layer of fat has been described as sufficient to insulate the bowel from thermal injury (1,8). Multiple maneuvers have been described to decrease the chance of bowel injury, including patient positioning, levering the tumor with the RFA probe, and injection of sterile fluid to hydrodissect the tumor away from the bowel (1,6). Of the above cases without tumor recurrence, in all, the lesion was located anteriorly, increasing the risk of bowel injury and fistula formation. Some authors (8) have proposed a laparoscopic approach to anterior tumors to decrease the chance of bowel injury and while this may decrease the risk of injury, it is not entirely protective as evidenced by the above cases.

Conclusion
Renocolic fistula formation following radiofrequency ablation is an extremely rare complication. The manifestation of the fistula 10 months following treatment represents a delayed presentation of a previously described adverse event. Surgically assisted RFA may decrease the possibility of fistula formation for anterior tumors but is not entirely protective. Percutaneous RFA is an acceptable minimally invasive treatment option for small renal masses but is not without complications.

Axial CT status post radiofrequency ablation demonstrated no evidence of residual tumor with ample separation between the ablated mass and ascending colon. There is a small perinephric hematoma at the site of ablation.

Axial CT at 10 months post ablation displaying the area of ablation closely contiguous to the ascending colon, as well as air within the collecting system of the right kidney suggesting a fistula.

Barium Enema showing uptake into the right collecting system confirming communication between the ascending colon and the right renal collecting system.

Coronal CT showing fistula formation.

References:
1. Park S, et al. Radiofrequency ablation treatment for renal cell carcinoma: Early clinical experience. Korean J Radiol 2008; 9:340-47.
2. Boss A, Clasen S, et al. Image-guided radiofrequency ablation of renal cell carcinoma. Eur Radiol 2007; 17: 735-33.
3. Weizer AZ, Raj GV, O’Connell M, Robertson CN, Nelson RC, Polascik TJ. Complications after percutaneous radiofrequency ablation of renal tumors. Urology. 2005 Dec;66(6):1176-80.
4. Sáenz Medina J, Redondo González E, Hernández-Atance JM, Crespo Martínez L, Llanes González L, Rendón Sánchez D, Páez Borda A. Renocolic fistula as a complication of radiofrequency in the treatment of renal cell carcinoma. Arch Esp Urol. 2010 Jan-Feb;63(1):74-7.
5. Uribe PS, Costabile RA, Peterson AC. Progression of renal tumors after laparoscopic radiofrequency ablation. Urology. 2006 Nov;68(5):968-71. Epub 2006 Nov 7.
6. Gervais D, Arellano R, Mueller P. Percutaneous radiofrequency ablation of renal cell carcinoma. Eur Radiol 2005; 15: 960-67.
7. Park B, Chan K. Complication of image-guided radiofrequency ablation of renal cell carcinoma: causes, imaging features and prevention methods. Eur Radiol 2009; 19:2180-90.
8. Rhim H, Dodd G 3rd, Chintapalli K, Wood B, Dupuy D, Hvizda J, Sewell P, Goldberg S. Radiofrequency thermal ablation of abdominal tumors: lessons learned from complications. Radiographics 2004; 24:41-52

Date added to bjui.org: 24/10/2012

DOI: 10.1002/BJUIw-2012-020-web

Scrotum Ulcer Developed After Intraperitoneal Hyperthermic Chemotherapy with Mitomycin-C

October 21, 2012/by admin Z.9

We report a pacient with a scrotum ulcer developed after intraperitoneal hyperthermic chemotherapy with mitomycin-C. There is only one article in the literature describing two similar cases. Our group suggests that an image investigation should be considered on male patients prior to intraperitoneal chemotherapy, since a procedure complication might be dramatic to the patient.

Authors: Silva, Fabiana; Avancini, Joao; Criado, Paulo; Pfiffer, Tulio; Lobo, Alice; Sanches, Jose

Corresponding Author: Silva, Fabiana

Case Report
A 65 year‐old Caucasian male patient with metastatic colorectal cancer was referred to the Dermatology Service with a scrotal ulcer in August 2011. The patient was diagnosed with rectal adenocarcinoma in 2008 and underwent rectosigmoidectomy with colostomy, followed by adjuvant radiotherapy and chemotherapy until May 2009. In February 2011 the patient was diagnosed with peritoneal tumor recurrence and in May 2011 he underwent a salvage surgical procedure with peritonectomy, hepatic metastasis excision and intraperitoneal hyperthermic chemotherapy with mitomycin‐C.

Nine days after this last procedure, the patient complained of scrotal pain and pruritus and slowly developed local erythema and edema that further evolved to a local ulceration. At first it was interpreted as local infection and the patient received systemic antibiotics including Cefalexin, and later Ciprofloxacin and Clindamycin without improvement.

In August 2011 the patient was referred to our Dermatology Department, presenting with scrotal induration and ulcer with dark eschars on the anterior wall of the scrotum, without evidence of scrotal abscess or Fournier’s gangrene (Figure 1).

A testicular ultrasound scan was performed, demonstrating signs of an inflammatory process of the scrotum, without testicular involvement. A cutaneous punch biopsy was performed and the histopathology was suggestive of ischemic necrosis, without positive findings on special histological stains for fungal and mycobacterial agents.

Tests for acquired and congenital thrombophilia were performed and no abnormalities were found. On September 2011 the patient underwent excision of the affected scrotal tissue that left the testicles exposed. The intraoperative findings were extensive deep necrosis with tunica albuginea involvement and thrombosis of local vasculature. Suture of the tunica vaginalis was performed protecting the testicles and vacuum assisted wound closure was used. The patient’s pain was relieved, and after one week he underwent another wound debridement and the scrotum was primarily repaired. There was suture dehiscence and a local dressing with Papain 8% was used with good response.

Discussion
Akhavan A. et al (1) described two similar cases, and to our knowledge, our case is the third clinical report of a scrotal ulcer developing after intraperitoneal hyperthermic chemotherapy with mitomycin‐C. Two possible causes of the scrotal ulcers were proposed: (i) a patent processus vaginalis, allowing mitomycin‐C to become sequestered in the scrotum, inducing an inflammatory reaction, resulting in scrotal wall inflammation and subsequent ulceration (Figure 2). This was proposed since previous studies have shown that intradermal administration of mitomycin‐C inhibits wound healing and induces skin necrosis (2); (ii) local spillage of mitomycin‐C onto the scrotal skin, with resulting inflammation and ulceration.

Previous studies with patients on continuous ambulatory peritoneal dialysis (CAPD) have shown that 10% of CAPD patients developed genital swelling (3). A possible cause of the scrotal swelling is a patent processus vaginalis causing a communicating hydrocele, that can be found in 15–37% of adult men and may not be clinically evident until CAPD has begun (3, 4, 5, 6). Our group suggests that an image investigation, such as computed tomographic peritoneography (7), should be considered on male patients prior to intraperitoneal chemotherapy, since this complication is potentially serious for the patient.

Figure 1

Figure 2

References
1. Akhavan A, Yin M, Benoit R. Scrotal Ulcer After Intraperitoneal Hyperthermic Chemotherapy. UROLOGY. 2007; 69: p. 778.e9–778.e10.
2. Porter GT, Gadre SA, Calhoun KH. The effects of intradermal and topical mitomycin C on wound healing. Otolaryngol Head Neck Surg. 2006; 135: p. 56 – 60.
3.Kopecky RT, Funk MM, Kreitzer PR. Localised genital edema in patients undergoing continuous ambulatory peritoneal dialysis. 1985; 134: p. 880‐4.
4. Cochran ST, Do HM, Ronaghi A, Nissenson AR, Kadell BM. Complications of peritoneal dialysis: Evaluation with CT peritoneography. Radiographics. 1997; 17: p. 869‐78.
5. Litherland J, Gibson M, Sambrook P, Lupton E, Beaman M, Ackrill P. Investigation and treatment of poor drains of dialysate fluid associated with anterior abdominal wall leaks in patients on chronic ambulatory peritoneal dialysis. Nephrol Dial Transplant. 1992; 7: p. 1030‐4.
6. Hollett MD, Marn CS, Ellis JH, Francis IR, Swartz RD. Complications of continuous ambulatory peritoneal dialysis: Evaluation with CT peritoneography. AJR. 1992; 159: p. 983‐9.
7.Xie JY, Chen N, Shi T, Xu ZP, Lu Y, Ren H. Computed tomographic peritoneography in investigation of patent processus vaginalis in CAPD patients. Clin Nephrol. 2010; 73(2): p. 153‐6.

Date added to bjui.org: 21/10/2012

DOI: 10.1002/BJUIw-2012-019-web

A musculoskeletal approach for patients with pudendal neuralgia: a cohort study

October 18, 2012/by admin Z.9

Selected patients with pudendal neuralgia, dupuytren’s contracture, low back pain responded favourably to education, postural advice, and mobilisation and exercises directed to the lumbopelvic region.

Authors: Dornan, Peter R.1; Coppieters, Michel W.2

Corresponding Author: Dornan, Peter R.

Affiliations
(1): Peter Dornan Physiotherapy Centre
(2): Centre of Clinical Research Excellence in Spinal Pain, Injury and Health, School of Health and Rehabilitation Sciences, The University of Queensland

Corresponding author
Peter R. Dornan
Peter Dornan Physiotherapy Centre
13 Morley Street
Toowong (Brisbane)
QLD 4066
Australia
Tel: +61 7 3371 9155
Fax: +61 7 3871 0301
Email: [email protected]

Abstract
Objective: To investigate the effect of musculoskeletal management of the lumbopelvic region in patients with pudendal neuralgia and musculoskeletal dysfunctions in the lumbopelvic region.
Patients: Twenty-five male patients with pudendal neuralgia without a urological cause for the symptoms and with lumbopelvic musculoskeletal disorders participated.
Methods: The intervention consisted of explanation and postural advice, specific manual mobilisation techniques and motor control exercises for the lumbopelvic region. A modified Pelvic Pain Symptom Survey was used to evaluate changes in pain and sexual dysfunction at the end of treatment and at three months follow-up. A repeated-measure analysis of variance was used to analyse the data.
Results: At the end of the treatment period and at follow-up, pain and sexual dysfunction had improved significantly. Although 39% of patients had experienced limited recurrence of symptoms during the follow-up period, patients stated that a home exercise program was effective at reducing the symptoms and no additional treatment was sought.
Conclusion: This cohort study provides Level 2b evidence that a musculoskeletal treatment approach has a positive influence on pain and sexual dysfunction in a specific subgroup of patients presenting with pudendal neuralgia.

Introduction
Urologists and gynaecologists regularly see patients presenting with signs of pudendal nerve entrapment. Pudendal nerve entrapment is a recognised cause of chronic pelvic pain in the regions served by the pudendal nerve, typically presenting as pain in the penis, scrotum, labia, perineum or anorectal region [1, 2]. Other symptoms may include dysuria, urge incontinence, and pain during or after ejaculation[3]. There may be corresponding sexual and erectile dysfunction problems[4], including impotence[5].

These presentations demand a complete urological investigation by a medical specialist. For the majority of these patients there is a definite urological or gynaecolocial diagnosis, such as prostadynia (prostatitis), orchialgia (testicular pain) or vulvodynia (vulva pain)[6]. However, a sub-group of these patients does not have a clear diagnosis. Once prostate, gynaecological, urinary or rectal pathology has been excluded, patients are often referred for physiotherapy.

Many patients with signs and symptoms characteristic for pudendal neuralgia also present with low back pain, with or without groin, buttock and leg symptoms[7]. Furthermore, patients frequently indicate that sitting will either bring on or make the symptoms worse [3, 8]. One explanation for some of the symptoms is that they may be caused by a dysfunction in the thoracolumbar junction as described by Maigne [9, 10]. Maigne stated that referred pain from the spinal nerves T12 and L1 can manifest itself as low back pain, which mimics pain of lumbosacral or sacroiliac origin. Although less common, referred pain from the spinal nerves originating in the thoracolumbar junction can also be felt in the lower abdomen, the medial aspect of the upper thigh and the groin, labia majora or scrotum [9, 10]. Recommended treatment includes spinal manipulation of the thoracolumbar region and infiltration of the painful zygapophysial joint with a corticosteroid [9, 10].

It has also been suggested that a range of musculoskeletal disorders of the lumbopelvic region, such as altered intra-pelvic motion and pelvic muscle control, may cause entrapment, excessive strain or irritation of the pudendal nerve[4].

The pudendal nerve emerges from the sacral plexus (primarily S2-S4, with possible contributions from S1 and S5)[11]. The pudendal nerve enters the pelvic cavity approximately 3 cm. below the sacro-iliac joint. The nerve journeys posterior to the coccygeus muscle and descends ventral to the sacrotuberous ligament. According to Robert et al[1], the nerve passes under the sacrospinous ligament medial to the ischial spine and re-enters the pelvic cavity through the lesser sciatic foramen. Ramsden et al[3] have shown that the pudendal nerve re-enters immediately adjacent to the inferior border of the sacrospinous ligament. A perineal branch of the nerve, the inferior pudendal (long scrotal nerve) curves below and in front of the ischial tuberosity, pierces the fascia lata and runs to the skin of the scrotum in the male and the external labia in the female [12].

Ramsden et al [3] suggest that entrapment can occur between the sacrospinous and sacrotuberous ligaments. Entrapment may also occur in the pudendal canal [3] (also called Alcock’s canal), which is formed by a split of the fascia of the obturator internus muscle. Clinically, we have observed that pudendal neuralgia is often associated with articular dysfunctions in the lumbopelvic region.

There are no randomised clinical trials or case series available which have investigated whether musculoskeletal management of the lumbopelvic region could reduce pudendal neuralgia. Moreover, no treatment protocols have been suggested in this domain in the peer reviewed literature. The aim of this paper is therefore to propose a musculoskeletal management approach focussed on the lumbopelvic region for patients with pudendal neuralgia without urological pathology, and to evaluate its efficacy in a prospective cohort design.

Methods
Study design
A cohort of 25 consecutive male patients with pudendal neuralgia referred from urologists was included in this study. Outcome measures at the end of the treatment period and at three months follow-up were compared to baseline data. The study was approved by the Institutional ethics committee and all participants signed an informed consent form.

Participants
Patients were included in the study if their signs, symptoms and history fulfilled the definition of pudendal neuralgia [1, 2]. Patients were included if they presented with: (i) pain or other symptoms in one or more regions including the penis, scrotum, perineum, lower abdomen or anorectal region; (ii) pain during or after ejaculation; and/or (iii) dysuria, urge incontinence, sexual and erectile dysfunction problems or impotence.

To be eligible, patients were also required to have signs of a pelvic girdle dysfunction. Four pain provocation tests and one mobility test were performed to identify a sacroiliac joint dysfunction (see Appendix 1). All four provocation tests have been reported to have good inter-examiner reliability[13]. Following the recommendation of their use[14], two of the four tests had to be positive to reflect a diagnosis of sacroiliac joint dysfunction. In addition to the pain provocation tests, Gillet’s test for sacroiliac joint mobility[15] had to be positive. Herzog et al[16] demonstrated substantial reliability for this test.

Patients were excluded if they presented with a urological diagnosis, such as prostadynia.

The mean (±SD) age, weight and height of the 25 patients who participated in the study were 44 (±12) years, 84 (±13) kg and 180 (±6) cm, respectively. The mean (±SD) duration of symptoms was 47 (±70) months. The mean number of days pain was experienced in the month preceding the start of treatment was 18.7 (± 10.2) days.

Treatment
At the start of each treatment session, mild heat was applied to the patient’s lower back for 10 minutes (Physiotherm short wave diathermy). After the application of heat, a series of exercises was performed (see Appendix 2). The exercises were designed to mobilise the thoracolumbar spine and sacroiliac joint, and to retrain the muscles of the lumbopelvic region. After the exercises, manual techniques were performed. A mobilising technique for the sacroiliac joint was used which has been devised to mobilise a restricted anterior rotation of the innominate bone [15] (see Appendix 3). The clinical assumption was that this mobilisation technique could address a possible nutation of the sacrum or posterior rotation of the ilium which may have increased the stress on the pudendal nerve as it runs between the sacrospinous and sacrotuberous ligament.

In addition, explanation and advice was provided, including postural advice. Patients were encouraged to try to adopt a more physiological spinal curvature throughout the day, especially during sitting.

The rationale for this management approach was that via exercises and mobilisation techniques directed to the thoraco-lumbo-pelvic region, the mechanical provocation and irritation of the pudendal nerve might be reduced. Improvement of postural awareness might result in a healthier loading of the neuromusculoskeletal structures of the lumbopelvic area resulting in less provocation.

No predetermined number of treatment sessions was provided. The treatment was ended when patient and clinician believed no further improvements could be expected with the intervention under investigation.

Baseline and Outcome measures
The prime outcome measure in this study was the modified Pelvic Pain Symptom Survey (Appendix 4), which was a shortened version of the survey developed at the University of Washington[17] and Stanford University School of Medicine[7]. The survey included an 8 item pain domain, and a 5 item sexual dysfunction domain. For each item, the degree by which the symptoms troubled the patient was scored on a scale ranging from 0 (not at all) to 4 (extremely). The survey also included an 11-point numeric rating scale to rate the intensity of the pain and a body chart to indicate the location of the symptoms.

The baseline and outcome measures were recorded before the start of treatment, at the end of the treatment period and at 3 month follow-up.

Statistical analysis
Data were analysed with a repeated-measures analysis of variance (ANOVA). The level of significance was set at p < 0.05. Of the 25 patients who participated, two participants could not be contacted at the three month follow up. For these two patients, baseline data were used in the analysis.

Results
At baseline, the mean (± SD) overall pain intensity was 5.0 (± 2.1) on the numeric pain rating scale. The modified Pelvic Pain Symptom Survey revealed that all patients reported pain, predominantly in the testicles (80%), lower back (80%), and the abdomen or pubic area (68%). Forty eight percent of patients also reported some form of sexual dysfunction. Lack of interest (36%) was the most frequently cited dysfunction. Table 1 illustrates the mean severity for all items of the Pelvic Pain Symptom Survey.

The mean number of treatment sessions was 4.4, with a maximum of 16 sessions. The maximum treatment duration was three months.

At the end of the treatment period, the mean (± SD) overall pain intensity had decreased to 0.6 (± 2.0) (p < 0.001). The modified Pelvic Pain Symptom Survey demonstrated that the number of patients reporting pain had reduced substantially. Pain in the low back (8%) and penis (8%) were the most frequently cited locations, at much lower frequencies compared to baseline. Eight percent of patients reported some form of sexual dysfunction. Apart from the item ‘Difficulty ejaculation’ (p = 0.20), the analysis of variance demonstrated that all items of the Pelvic Pain Symptom Survey had improved significantly at the end of treatment when compared to baseline (p £ 0.021). Table 1 illustrates the improvement for the individual items of the modified Pelvic Pain Symptom Survey.

At three month follow-up, the mean (± SD) overall pain intensity was 0.8 (± 2.1) on the numeric pain rating scale. This was significantly lower than at baseline (p < 0.001) and not different compared to the end of treatment (p = 0.62). Compared to end of treatment, the number of patients reporting pain had risen to 20%. Low back pain (16%), pain in the testicles (12%) and penis (12%) were the most commonly listed areas. Sixteen percent of patients reported sexual dysfunction. All items of the Pelvic Pain Symptom Survey which had improved at the end of treatment were still improved at three month follow-up (p £ 0.042). None of the items at three month follow-up was significantly different compared to end of treatment (p ≥ 0.56), demonstrating that improvements were maintained.

Table 1 – Pain and Sexual Dysfunction Scores

Although symptoms had reduced significantly by the end of the treatment period and at the time of follow-up, nine of the 23 patients (39%) had experienced limited recurrence of symptoms during the three month follow-up period. These patients stated that the prescribed home exercise program was effective in relieving the symptoms. No one reported to have sought further treatment.

With respect to the two participants who did not complete the program and could not be contacted, one had previously reported perineal pain and penile pain for seven months. He could not be contacted after receiving two treatments having recorded no perceivable difference in symptoms. The other participant reported urinary, faecal and seminal fluid loss and pain in the anal ring, perineum, shaft of penis and scrotum for three months prior to treatment. He had an underlying complication of a neuritis for seven months prior to enrolling into the trial. He could not be contacted after 4 treatments over 2 weeks.

Discussion
According to the levels of evidence published by the Centre for Evidence Based Medicine[18], this cohort study provides Level 2b evidence that specific manual mobilisation techniques and motor control exercises for the lumbopelvic region, together with postural advice and education, have a positive influence on pain and sexual dysfunction in a specific subgroup of patients presenting with pudendal neuralgia. In addition to pudendal neuralgia, patients were only included in this study if they also had signs of a lumbopelvic dysfunction and no evidence of urological pathology. Pain and sexual dysfunction improved significantly at the end of the treatment period and the improvement was maintained at three months follow-up.

The improvement in sexual function was more varied than the reduction in pain. Besides urologicial[19] and pain of musculoskeletal origin[20], there are many causes of sexual dysfunction. These include smoking[21], alcohol and substance use [22], diabetes[23] and cardiovascular disease[24], often associated with psychological and behavioural overtones [20]. It has been suggested that therapeutic approaches to sexual dysfunction in patients with chronic pain might best be focused on improving psychological factors, particularly depression and coping skills [20]. It is reasonable to assume that a more multidisciplinary approach would have yielded more consistent improvements in sexual function.

Although statistically significant and clinically meaningful improvements in pain intensity and sexual function were observed at the end of the treatment period and at three month follow-up, a substantial proportion (39%) of patients had experienced limited recurrence of symptoms during the three month follow-up period. Importantly, recurrence of these symptoms could be managed with the prescribed home exercise program, suggesting that the exercises provided an effective self-management strategy.

Various musculoskeletal impairments have been associated with pudendal neuralgia, such as pelvic floor dysfunction, connective tissue restrictions, myofascial trigger points, muscle hypertonicity, altered neurodynamics, and structural and biomechanical abnormalities, such as lumbopelvic dysfunctions [25, 26]. Although clinical trials are not available, Prendergast and Rummer [26] suggested that each of the above mentioned impairments can be addressed resulting in less pain in the distribution of the pudendal nerve. For sacroiliac joint dysfunction, they advise treatment involving manual therapy techniques to correct joint dysfunction and a home exercise program to strengthen and re-educate the muscles to maintain proper joint position and stability [26].

Although it has been stated that the pudendal nerve can be significantly affected by abnormalities of the sacroiliac joint because innominate rotations may result in increased tension in the ligaments through which the pudendal nerve travels, [26, 27], there are many mechanisms that may have been influenced during our multimodal intervention. Altered recruitment patterns of the trunk muscles have been demonstrated in people with sacroiliac joint dysfunction.[28] Altered muscle activation is reversible following specific exercises[29] and sacroiliac joint manipulation [30]. Postural advice, especially directed to sitting postures, may also have influenced symptoms as different sitting postures are associated with different resting activity levels of the pelvic floor muscles [31].

To the authors’ knowledge, the natural history of pudendal neuralgia is unknown. As a consequence, it can not be ruled out that the observed improvements could be due to natural recovery. However, as the mean symptom duration was approximately 4 years and the treatment period was relatively short, we believe it is unlikely that a substantial portion of the improvements can be attributed to natural recovery.

A more important limitation of the present study is that non-specific treatment effects cannot be discarded. Furthermore, as all outcome measures were self-reported, report bias cannot be ruled out. Having demonstrated positive effects in a cohort design, future clinical studies will have to adopt a randomised clinical trial design, and include a combination of self-reported and clinician-rated outcome measures. Physiological outcome measures should also be considered.

Although this study has several limitations and only provides level 2b evidence, we believe this cohort study has merit. The findings of this study suggest that a subgroup of patients with pudendal neuralgia can have a significant reduction in symptoms following musculoskeletal management of the lumbopelvic region. Considering the substantial and relatively fast reduction in symptoms, we recommend that patients with pudendal neuralgia without contributing urological pathology should be referred for musculoskeletal screening of the lumbopelvic region, and musculoskeletal management of this region if dysfunctions can be substantiated. The low monetary cost, non-invasive nature, and potential for self-management following the treatment period are additional benefits. If sexual dysfunctions are foremost, a multidisciplinary approach might be needed.

Appendix 1
SIJ Pain
Four provocation tests were selected (Figures 1, 2, 3 and 4).

1. Thigh Thrust – The patient lies supine with the hip and knee flexed where the thigh is at 90° to the table and slightly adducted. One of the examiners hands cups the sacrum and the other arm and hand wraps around the flexed knee. The pressure applied is directed dorsally along the line of the vertically oriented femur. The procedure is carried out on both sides. The presumed action is posterior shearing force to the SIJ of that side [14]

Thigh Thrust SIJ Provocation Test – Figure 1

2. Distraction Test – The patient lies supine and the examiner applies a posteriorly directed force to both anterior-superior iliac spines. The presumed effect is a distraction of the anterior aspects of the SIJ [14]

Distraction Test SIJ Provocation Test – Figure 2

3. Compression – The patient lies on the side with hips and knees flexed to about a right angle. The examiner kneels on the table and applies a force vertically downward on the uppermost iliac crest. The presumed action is a compression force to both SIJ’s [14]

Compression provocation Test – Figure 3

4. Sacral Thrust – The patient lies face down. The examiner applies a force vertically downward to the centre of the sacrum. The presumed action is an anterior shearing force of the sacrum on both ilia [14]

Sacral Thrust Provocation Test – Figure 4

SIJ Mobility
With the patient standing with his weight evenly distributed through both lower limbs, the therapist palpates with one thumb the PSIS while the other palpates the sacral base directly parallel. The patient is directed to flex the ipsilateral hip joint and the relative movement is noted of the thumbs. A reduction or total absence of relative motion between the innominate and the sacrum is reflective of SIJ hypomobility/dysfunction.

Appendix 2

(a)

Figure 5 – Hip Rolls
Patient lying supine, legs hip width apart, lift the buttocks to form a long diagonal line between the shoulders, hips and knees. Roll or glide the pelvis to one side, back to the middle, then the other. Return to the middle and lower buttocks to the floor. 10 repetitions.

(b)

Figure 6 – Low Back Stretch

Patient lying supine, use two hands to draw one thigh and knee to the chin, keeping the other leg flat and extended. Hold this thigh on strong overpressure for 5 seconds and release. Repeat 5 repetitions each leg.

(c)

Figure 7 – Full Spine Strengthening
Patient kneeling on all fours, hands underneath shoulders, knees under hips. Flex and stretch one knee towards the chin, then extend head and leg. Repeat 10 times each leg.

(d)

Figure 8 – SIJ Stretch
Patient kneeling on all fours on strong table. One leg (knee) is positioned over the edge of the table and hooked over the other leg for support. One leg is stretched fully below the table edge and held in this position for 5 seconds, then lifted fully higher than the table edge and held here for 5 seconds. This is repeated each side 10 times.

(e)

Figure 9 – Abdominal (core) Exercises

Patient in supine, knees flexed, feet flat on floor, hands behind head and supporting neck. Draw the naval to the floor to flatten abdominals, and then raise head and shoulders, (as in ‘crunches’). Repeat 6 times. Then, as the patient sits up rotate the trunk towards one knee, repeat 6 times, then rotate to the other knee 6 times.

Home Exercises – The patient was instructed to perform the above described exercises twice daily (morning and evening). These exercises were also prescribed as a long term management program on discharge.

Appendix 3
With the patient lying prone close to the edge of the table, the therapist supported the anterior aspect of the distal thigh with one hand and lifted the hip into extension, while the posterior superior iliac spine of the innominate was palpated with the heel of other hand. The limit of anterior rotation of the innominate was reached by passively extending the hip with one hand and applying an anterior rotation force to the innominate with the other hand. This pressure was held for 15 seconds, and repeated 3 times per session.

Fig 10 – Mobilisation Technique.

Appendix 1: Clinical tests sacro-iliac joint

Figure 1 – Thigh Thrust SIJ Provocation Test

Appendix 2: Home exercise program

Figure 5 – Hip Rolls
Patient in supine, legs hip-width apart. Lift buttocks, then roll or glide the pelvis to either side, then lower buttocks. 10 repetitions.

Figure 6 – Low Back Stretch
Patient in supine, draw one knee to the chin. Hold for 5 seconds and release. Repeat five times, each leg.

Figure 7 – Full Spine Strengthening
Patient kneeling, palms on floor. Flex one knee towards the chin, then extend head and leg. 10 repetitions each leg.

Figure 8 – SIJ Stretch
Patient kneeling, palms on strong table. One knee is positioned over the edge of the table and stretched fully below the table edge, holding here for five seconds, then lifted higher than the table edge and held here for five seconds. 10 repetitions each leg.

Figure 9 – Abdominal (core) Exercises
Patient supine, knees flexed, feet flat on floor, hands behind head and supporting neck. Flatten the abdomen, and raise head and shoulders. 10 repetitions.

Appendix 3

Figure 10 – Mobilisation technique

Appendix 4:
Pelvic Pain Symptom Survey for men (PPSS) [17]

Over the past month, including today, how much were you bothered by the following:
Not at all
A little bit
Moderate
Quite a bit
Extreme

Pain in lower back 0 1 2 3 4
Pain in the lower abdomen or pubic area 0 1 2 3 4
Pain during urination 0 1 2 3 4
Pain with bowel movement 0 1 2 3 4
Pain in the rectum 0 1 2 3 4
Pain in the prostate gland 0 1 2 3 4
Pain in the testicles 0 1 2 3 4
Pain in the penis 0 1 2 3 4

Total pain score =

Not at all
A little bit
Moderately
Quite a bit
Extremely

Lack of interest in sexual activity 0 1 2 3 4
Difficulty getting an erection 0 1 2 3 4
Difficulty maintaining an erection 0 1 2 3 4
Difficulty reaching an ejaculation 0 1 2 3 4
Pain with ejaculation 0 1 2 3 4

Total sexuality score =
Areas of symptoms:
Please draw the location of your symptoms

Appendix 4:
Name: …………..……………………………..……………

How long have you had the symptoms for? ..…………….

Number of days pain experienced in the last month 0 6 15 24 30

How bad is the pain on average? 0 10
Put an X on the line from 0 to 10 No pain Worst pain ever

References
1. Robert, R., et al., Anatomic basis of chronic perineal pain: role of the pudendal nerve. Surg Radiol Anat, 1998. 20(2): p. 93-8.
2. Shafik, A., et al., Surgical anatomy of the pudendal nerve and its clinical implications. Clin Anat, 1995. 8(2): p. 110-5.
3. Ramsden, C.E., et al., Pudendal nerve entrapment as source of intractable perineal pain. Am J Phys Med Rehabil, 2003. 82(6): p. 479-84.
4. Prendergast, S.A. and J.M. Weiss, Screening for musculoskeletal causes of pelvic pain. Clin Obstet Gynecol, 2003. 46(4): p. 773-82.
5. Andersen, K.V. and G. Bovim, Impotence and nerve entrapment in long distance amateur cyclists. Acta Neurol Scand, 1997. 95(4): p. 233-40.
6. Gushing, B.L. and M. Francis. Epidemiological data on the prevalent diagnostic and treatment procedures for chronic prostatitis in the ambulatory care setting. in Annual Workshop of the International Prostatitis Collaborative Network. 2000. Arlington,VA.
7. Anderson, R.U., et al., Integration of myofascial trigger point release and paradoxical relaxation training treatment of chronic pelvic pain in men. J Urol, 2005. 174(1): p. 155-60.
8. Beco, J. Pudendal neuropathy. One of the main “defects” in perineology. in 31st Annual Meeting of the International Urogynecological Association. Athens.
9. Maigne, R., Low back pain of thoracolumbar origin. Arch Phys Med Rehabil, 1980. 61(9): p. 389-95.
10. Maigne, R., Le syndrome de la charniere dorso-lombaire. Lombalgus basses, douleurs pseudo-viscerales, pseudo-douleurs de hanche, pseudo-tendinite des adducteurs. Sem Hop, 1981. 57(11-12): p. 545-554.
11. Schraffordt, S.E., et al., Anatomy of the pudendal nerve and its terminal branches: a cadaver study. ANZ J Surg, 2004. 74(1-2): p. 23-6.
12. Gray, H., et al., Gray’s anatomy : the anatomical basis of clinical practice. 39th ed. 2005, Edinburgh ; New York: Elsevier Churchill Livingstone. xx, 1627 p.
13. Kokmeyer, D.J., et al., The reliability of multitest regimens with sacroiliac pain provocation tests. J Manipulative Physiol Ther, 2002. 25(1): p. 42-8.
14. Laslett, M., et al., Diagnosis of sacroiliac joint pain: validity of individual provocation tests and composites of tests. Man Ther, 2005. 10(3): p. 207-18.
15. Lee, D.G. and M.C. Walsh, A workbook of manual therapy techniques for the vertebral column and pelvic girdle. 2nd ed. 1996, [Vancouver: Nascent Pub. 261 p.
16. Herzog, W., et al., Reliability of motion palpation procedures to detect sacroiliac joint fixations. J Manipulative Physiol Ther, 1989. 12(2): p. 86-92.
17. Krieger, J.N., et al., Chronic pelvic pains represent the most prominent urogenital symptoms of “chronic prostatitis”. Urology, 1996. 48(5): p. 715-21; discussion 721-2.
18. Centre for Evidence Based Medicine, U.o.O. Levels of Evidence (https://www.cebm.net/index.aspx?o=1025). 2009.
19. Gontero, P., et al., Male and female sexual dysfunction (SD) after radical pelvic urological surgery. ScientificWorldJournal, 2006. 6: p. 2302-14.
20. Kwan, K.S., L.J. Roberts, and D.M. Swalm, Sexual dysfunction and chronic pain: the role of psychological variables and impact on quality of life. Eur J Pain, 2005. 9(6): p. 643-52.
21. Lam, T.H., et al., Smoking and sexual dysfunction in Chinese males: findings from men’s health survey. Int J Impot Res, 2006. 18(4): p. 364-9.
22. Johnson, S.D., D.L. Phelps, and L.B. Cottler, The association of sexual dysfunction and substance use among a community epidemiological sample. Arch Sex Behav, 2004. 33(1): p. 55-63.
23. Jackson, G., Sexual dysfunction and diabetes. Int J Clin Pract, 2004. 58(4): p. 358-62.
24. Billups, K.L., Sexual dysfunction and cardiovascular disease: integrative concepts and strategies. Am J Cardiol, 2005. 96(12B): p. 57M-61M.
25. Baker, P.K., Musculoskeletal origins of chronic pelvic pain. Diagnosis and treatment. Obstet Gynecol Clin North Am, 1993. 20(4): p. 719-42.
26. Prendergast, S.A. and E.H. Rummer. The Role of Physical Therapy in the Treatment of Pudendal Neuralgia. in 31st Annual Meeting of the International Urogynecological Association. 2006. Athens, Greece.
27. Rummer, E.H. and S.P. Prendergast, The Role of Physical Therapy in Treating Myofascial Pelvic Pain Syndrome, in IASP SIG on Pain of Urogenital Origin – Newsletter, vol 1, no 2. 2009.
28. Hungerford, B., W. Gilleard, and P. Hodges, Evidence of altered lumbopelvic muscle recruitment in the presence of sacroiliac joint pain. Spine (Phila Pa 1976), 2003. 28(14): p. 1593-600.
29. Tsao, H., et al., Motor Training of the Lumbar Paraspinal Muscles Induces Immediate Changes in Motor Coordination in Patients With Recurrent Low Back Pain. J Pain, 2010.
30. Marshall, P. and B. Murphy, The effect of sacroiliac joint manipulation on feed-forward activation times of the deep abdominal musculature. J Manipulative Physiol Ther, 2006. 29(3): p. 196-202.
31. Sapsford, R.R., et al., Pelvic floor muscle activity in different sitting postures in continent and incontinent women. Arch Phys Med Rehabil, 2008. 89(9): p. 1741-7.

Date added to bjui.org: 18/10/2012

DOI: 10.1002/BJUIw-2012-054-web

Metastatic Papillary Thyroid Carcinoma to the Prostatic Urethra Presenting as Gross Hematuria

October 15, 2012/by admin Z.9

Papillary thyroid cancer (PTC) is the most common welldifferentiated cancer of the thyroid. While regional metastatic disease to lymph nodes is most common, there have been rare cases of distant metastases to the kidney. We report a case of papillary thyroid carcinoma (PTC) with metastases to the prostatic urethra presenting as gross hematuria.

Authors: Vora, Anup; Krishnan, Jayshree; Verghese, Mohan

Corresponding Author: Vora, Anup

Case Report
A 31-year-old Indian male with a known history of metastatic papillary thyroid cancer was referred for investigation of intermittent gross haematuria. The patient was first diagnosed with PTC at age 4 and was treated with subtotal thyroidectomy, followed by radioactive iodine (I-31) therapy. The patient underwent a second course of radioactive I-31 therapy several years later for metastatic disease to the lungs and eventually underwent a total thyroidectomy in 2007. He received a third course of I-31 infusion in 2009 followed by trial of sunitinib for disease unresponsive to radioactive Iodine therapy. Radiographic imaging revealed a 1.9 cm enhancing mass in the interpolar region of the right kidney .Microscopic examination of the kidney biopsy showed focal infiltration by cohesive malignant cells including a papillary cluster, with the cells showing nuclear grooves. Immunohistochemical stains showed that the tumour cells were positive for thyroglobulin and thyroid transcription factor-1 (Figure 1).
With regards to haematuria, the patient denied any previous significant genitourinary history. Haematuria was intermittent and painless with passage of small clots. The patient was taken to the operating room and underwent diagnostic cystoscopic evaluation of his lower urinary tract. Cystoscopy revealed friable frond-like tissue in the prostatic urethra, with mucosal sloughing and bleeding. Bladder urothelium was grossly normal, with bilateral efflux of clear urine from the ureteral orifices. Microscopic examination of the tissue showed sheets of tumour cells with nuclear crowding, nuclear grooves and intranuclear inclusions. Immunostains showed that the tumour cells are positive for thyroid transcription factor 1 and thyroglobulin (Figure 2). Urine cytology and cell block also showed similar tumour cells.
Histomorphological features and the immunohistochemical staining features in both the renal and prostatic urethral tumor tissue supported a diagnosis of metastatic thyroid carcinoma.

Discussion
Although the prognosis of PTC is generally good, up to 10% of the patients eventually die of the disease and greater proportion would face the morbidity of recurrences. PTC is known to metastasize preferentially to lymph nodes, and distant metastases may be present in upto 10% of patients during the course of follow-up. [1] The most common metastatic locations include the lungs, bones and brain.However, rare cases of metastatic PTC have been reported in the scalp [2], pancreas [3] and maxilla [4]. A small number of metastastic renal lesions have been reported [5]. These cases typically occured after a long latency period followingprimary therapy. There have been no known reported cases of metastatic deposits in the prostatic urethra [1,6]. Other malignancies that have reported metastases to the urethra include rectal adenocarcinoma [7] and malignant melanoma [8].
In the management of patients who have distant PTC metastases, lesions in areas where continued growth into surrounding structures would result in serious morbidity should be addressed with surgical or other palliating treatment. Lesions in the brain, spinal column, weight-bearing bones, or upper mediastinum could progress and result in acute spinal cord compression, superior vena cava syndrome or an acute central nervous system event from brain metastases [6].
We present a rare case of metastatic PTC to the prostatic urethra resulting in intermittent, painless gross haematuria. Fulguration of the lesion resulted in a short term control of bleeding only. Our patient is currently considering external beam radiotherapy to the prostatic urethra to palliate his gross haematuria and further systemic therapy for diffuse metastatic disease.

Figure 1: Microscopic examination of the kidney biopsy showed focal infiltration by cohesive malignant cells including a papillary cluster, with the cells showing nuclear grooves (A/B haematoxylin& eosin stain with magnification x 25). Immunohistochemical stains showed that the tumour cells were positive for thyroglobulin and thyroid transcription factor-1(C) . D. Immunostain for thyroglobulin, magnification x 40.

Figure 2. Microscopic examination of this prostatic urethral biopsy tissue showed sheets of tumour cells with nuclear crowding, nuclear grooves and intranuclear inclusions (A. magnification x 25, B. magnification x 40. Immunostains showed that the tumour cells are positive for thyroid transcription factor 1 (C. magnification x 25) and thyroglobulin (D. magnification x 40).

References
1. Pakdaman MN, Rochon L, Gologan O et al. Incidence and histopathological behavior of papillary microcarcinomas: Study of 429 cases. Otolaryngol Head Neck Surg2008;139:718–22.

2. Aghasi MR, Valizadeh N, Soltani S. A 64 year-old female with scalp metastasis of papillary thyroid cancer.Indian J EndocrinolMetab. 2011 Jul;15:136-7.

3. Borschitz T, Eichhorn W, Fottner C et al. Diagnosis and treatment of pancreatic metastases of a papillarythyroid carcinoma. Thyroid. 2010 Jan;20(1):93-8.

4. Nikitakis NG, Polymeri A, Polymeris A, Sklavounou A. MetastaticPapillaryThyroid Carcinoma to the Maxilla: Case Report and Literature Review. Head Neck Pathol.2011 Dec 30.epub

5. Borde C, Basu S, Kand P, Arya S, Shet T. Bilateral renal metastases from papillarythyroid carcinoma on post 131I treatment scan: flip-flop sign, radioiodine SPET, 18F-FDG PET, CECT and histopathological correlation. Hell J Nucl Med. 2011 Jan-Apr;14(1):72-3.

6. Woolner LB, Lemmon ML, Beahrs OH, Black BM, Keating FR Jr. Occult papillary carcinoma of the thyroid gland: A study of 140 cases observed in a 30-year period. J ClinEndocrinolMetab 1960;20:89-105

7. Wakahara T, Yamamoto S, Fujita S, Akasu T, Onouchi S, Moriya Y. A case of advanced rectaladenocarcinoid tumor with long-term survival.Jpn J ClinOncol. 2010 Jul;40(7):690-3.

8. MartíMestre J, OlivaEncina J, Sánchez Martín F et al. Melanomametastatic to the female urethra. Report of a case.ActasUrol Esp. 2002 Mar;26(3):231-4.

Date added to bjui.org: 15/10/2012

DOI: 10.1002/BJUIw-2012-001-web

Obstructing Calculi within the Male Urethra

October 12, 2012/by admin Z.9

Calculi within urethral diverticula are a rare manifestation of urinary stone disease. We report a case of a 63-year-old male presenting with lower urinary tract symptoms, who was subsequently found to have two large calculi located within corresponding urethral diverticula. The patient underwent open urethrotomy, stone extraction, and primary repair of associated urethral diverticula. The patient’s recovery was uneventful with resolution of urinary symptoms six weeks after surgery.

Authors: Kim, Dae Y.; Le, Brian V.; Dupree, James M.; Newman,Jessica; Dreyfuss, Justin C.; Helfand, Brian T.; Gonzalez, Chris M.

Corresponding Author: Gonzalez, Chris M.

Corresponding Author
Chris M. Gonzalez, MD, MBA
Northwestern University Feinberg School of Medicine
Department of Urology
303 E. Chicago Ave., Tarry 16-703
Chicago, IL 60611
P: 312-908-8145
F: 312-908-7275
E: [email protected]

Abstract
Calculi within urethral diverticula are a rare manifestation of urinary stone disease. We report a case of a 63-year-old male presenting with lower urinary tract symptoms, who was subsequently found to have two large calculi located within corresponding urethral diverticula. The patient underwent open urethrotomy, stone extraction, and primary repair of associated urethral diverticula. The patient’s recovery was uneventful with resolution of urinary symptoms six weeks after surgery.

Introduction
Incidence
In the Western Hemisphere, a urethral calculus within a diverticulum is a rare clinical entity as compared to developing nations where male urethral calculi are more common due to the increased incidence of bladder calculi(1). Urethral stones account for less than 1% of all urinary stone disease in the Western Hemisphere(2).

Location
The most common site of urethral calculi is the posterior urethra, but calculi have been reported along the entire urethra(3). The majority of studies have demonstrated a higher prevalence rate of posterior urethra stones with the rate ranging from 50-88% of all urethral calculi(1, 4, 5).

Presentation
The diagnosis can be complicated as some patients are asymptomatic while others present with severe symptoms such as pain in the ano-genital area, dysuria, urinary retention, frequency, dribbling, weak urinary stream, hematuria, urethral discharge, and dyspareunia. Posterior urethral stones can present with pain referred to the perineum or rectum, while anterior urethral stones tend to present with pain at the site of impaction. Rarely, patients present with a palpable mass in the penis or at the peno-scrotal junction. Careful physical examination can assist in determining the location of the calculi as a digital rectal examination can demonstrate a firm mass with posterior urethral stones.

The Case
A 63-year-old Asian male with no significant past medical history presented to the emergency department with a four month history of urinary frequency, urgency, nocturia, and a weak stream. These symptoms increased in severity during the previous 7 days, culminating in fever, chills, and scrotal discomfort. The patient denied any previous episodes of urinary tract infectionor stone disease. Physical examination revealed scrotal induration and tenderness with a palpable mass at the peno-scrotal junction. Urine culture was positive for polymicrobial growth with greater than 3 species demonstrated. Doppler ultrasound of the scrotum revealed increased flow to the epididymides and testes consistent with bilateral epididymo-orchitis. Abdominal x ray (Figure 1) and a CT scan demonstrated two masses with high attenuation, measuring 4.3 x 1.8 cm and 2.6 x 2.8 cm within the anterior urethra (Figure 1).
Following a 4-week regimen of antibiotic treatment, the patient underwent cystoscopy with retrograde urethrogram confirming two large calculi within corresponding diverticula at the proximal penile and distal bulb of the urethra (Figure 2). Retrograde manipulation of the calculi into the bladder was unsuccessful due to impaction of the calculi. After unsuccessful cystourethroscopy, open urethrotomy and stone extraction were performed.
Surgical exploration through a scrotal incision revealed two diverticuli with an enclosed branched calculus. The stones were extracted without difficulty from the surrounding urothelium (Figure 3). Each diverticulum was then subsequently excised and tailored to 26 Fr in circumference utilizing a 5-0 Vicryl suture for primary closure of the urethra. The length of the excision to extract both stones measured 8 cm (Figure 3) with the stone burden approximated at 7 x 3 cm in size (total mass 33 g) (Figure 4). An 18 Fr. Foley catheter was left in place for two weeks, and upon postoperative follow-up at six weeks, the patient had resolution of their urinary symptoms with an International Prostate Symptom Score of 2. Stone composition was determined as calcium carbonate apatite with pathological evaluation of the excised urethral diverticulum identified as benign urethral and periurethral tissue.

Discussion
Etiology
The etiology of urethral calculi can be classified as either originating within the urethra itself (de novo) as a result of an anatomical abnormality, or from distal stone migration from the bladder or upper urinary tracts(3). The normal adult urethra has a diameter of 30 F and therefore should technically allow passage of stones <10 mm; however, the presence of strictures, previous manipulation, or congenital abnormalities decrease urethral diameter and allow trapping of stones(5). Urethral calculi originating de novo are most commonly associated with urethral diverticuli, strictures, schistosomiasis, or neurogenic bladder, the latter of which predisposes patients to urinary stasis and infection(6). The frequency of urethral abnormalities associated with these calculi has been estimated to be as high as 56% of patients(1, 5, 7).
Kamal et al. examined 51 patients with urethral stones and found 86% of patients with calcium oxalate stones, 6% with struvite stones, and 2% with uric acid stones(1). As in the present case, the calcium carbonate calculi likely originated within a pre-existing diverticulum at the peno-scrotal junction.

Diagnostic Evaluation
Diagnostic evaluation of urethral calculi include imaging, such as ultrasound and CT-scan, along with direct visualization modalities via cystourethroscopy. Ultrasonography is a non-invasive diagnostic tool that may be useful for the screening of non-opaque posterior urethral calculi(4).Sonography can be used to demonstrate an echogenic area with acoustic shadowing representing the calculi. Plain X-rays of the abdomen, pelvis, and entire course of the urethra can be used to identify and measure radio-opaque stones.

Treatment and Management
Management of urethral stones depends on their size, location, and elucidation of relevant anatomic abnormality which predisposed their formation. Occasionally non-invasive measures are attempted first in patients without urethral obstruction or spiked calculi, including spontaneous expulsion and milking. Instillation of 2% lidocaine jelly in the urethra has been reported to aid spontaneous passage of stones, and has been associated with success rates ranging from 14.8% to 77.7%(8, 9).
Posterior urethral calculi have been managed with retrograde manipulation of the stone into the bladder either by external physical manipulation, Foley catheterization, or endoscopic guidance. Subsequently, litholopaxy is achieved using transurethral fragmentation with mechanical, ultrasonic, hydraulic, or laser energy. There is high success rate using this combination of retrograde stone manipulation into the bladder and holmium lithotripsy(10).
Stones within the anterior urethra naturally tend to be treated in a different fashion based on anatomy. For example, a meatotomy can be used for impacted stones within the fossa navicularis. For impacted urethral calculi, as in the present case, successful extraction of each large urethral calculus using open urethrotomy, diverticulectomy, and tailoring of the urethra resulted in successful removal of the stones and ultimate resolution of urinary symptoms. This procedure should strongly be considered in the unusual circumstance of large palpable calculi within the anterior urethra.

Conclusion
In conclusion, this case is unusual in that a patient presented with large urethral calculi that manifested as a palpable mass associated with symptoms of urinary obstruction and infection. Although rare, urethral calculi should be included in the differential of any patient presenting with acute urinary retention. Treatment options are based on stone size, shape, location, and associated urethral anatomical pathology. After surgical removal and correction, our patient had a favorable outcome on follow-up with correction of voiding symptoms and without recurrence of calculi to date.

References
[1] Kamal BA, Anikwe RM, Darawani H, Hashish M, Taha SA. Urethral calculi: presentation and management. BJU Int. 2004; 93: 549-52.
[2] Larkin GL, Weber JE. Giant urethral calculus: a rare cause of acute urinary retention. J Emerg Med. 1996; 14: 707-9.
[3] Kaplan M, Atakan IH, Kaya E, Aktoz T, Inci O. Giant prostatic urethral calculus associated with urethrocutaneous fistula. Int J Urol. 2006; 13: 643-4.
[4] Koga S, Arakaki Y, Matsuoka M, Ohyama C. Urethral calculi. Br J Urol. 1990; 65: 288-9.
[5] Selli C, Barbagli G, Carini M, Lenzi R, Masini G. Treatment of male urethral calculi. J Urol. 1984; 132: 37-9.
[6] Hayashi Y, Yasui T, Kojima Y, Maruyama T, Tozawa K, Kohri K. Management of urethral calculi associated with hairballs after urethroplasty for severe hypospadias. Int J Urol. 2007; 14: 161-3.
[7] Sharfi AR. Presentation and management of urethral calculi. Br J Urol. 1991; 68: 271-2.
[8] el-Sherif AE, Prasad K. Treatment of urethral stones by retrograde manipulation and extracorporeal shock wave lithotripsy. Br J Urol. 1995; 76: 761-4.
[9] Takasaki E, Suzuki T, Honda M, Imai T, Maeda S, Hosoya Y. Chemical compositions of 300 lower urinary tract calculi and associated disorders in the urinary tract. Urol Int. 1995; 54: 89-94.
[10] Maheshwari PN, Shah HN. In-situ holmium laser lithotripsy for impacted urethral calculi. J Endourol. 2005; 19: 1009-11.

Date added to bjui.org: 12/10/2012

DOI: 10.1002/BJUIw-2012-068-web

Papillary Cell Carcinoma in Post transplant Dialysis dependent patient – presenting as retroperitoneal hematoma

October 9, 2012/by admin Z.9

We present the case of a middle age male with ESRD with post transplant graft rejection, who presented with fever and left flank pain. Histopathology revealed a papillary cell carcinoma. Papillary cell carcinoma presenting as haemorrhage has not been previously reported in literature.

Authors: Nadeem, Mehwash; Ather, M Hammad; Sulaiman, M Nasir

Corresponding Author: Nadeem, Mehwash

For Correspondence and reprint requests

Dr Mehwash Nadeem
Dept of Surgery
Aga Khan University
P O Box 3500, Stadium Road
Karachi 74800, Pakistan
E mail [email protected]

Abstract
Renal cell carcinoma (RCC) is one of the known complications of end stage renal disease (ESRD) but these patients have favourable outcome in comparison to the general population. Spontaneous bleeding in renal tumours is a rare presentation and reported only anecdotally. We present the case of a middle age male with ESRD with post transplant graft rejection, who presented with fever and left flank pain. On investigation he was found to have a left perinephric collection. Initially he was managed conservatively, but subsequently underwent surgical exploration due to persistent flank pain. At operation, he was found to have a retroperitoneal hematoma. Histopathology revealed a papillary cell carcinoma. Papillary cell carcinoma presenting as haemorrhage has not been previously reported in literature.

Key Messages
Patients with ESRD on haemodialysis (HD) have a 100 times greater risk of developing RCC so these patients should be screened annually. There should be a high index of suspicion to diagnose these tumours and more specifically identifying these tumours as a cause of haemorrhage.

Introduction
Renal cell carcinoma (RCC) in patients with dialysis dependant end stage renal disease (ESRD) is about 100 times more common than in the general population. The histopathological type and features are distinct with a clear relation to the duration that a patient has remained haemodialysis (HD) dependent. Clear cell carcinoma is the most common renal tumour in the in general population as well as in patients with ESRD, papillary cell carcinoma represents a small fraction of cases and has not previously been reported in literature to present as haemorrhage.

Case History
The patient is a 48 year old man with co morbidities of hypertension, Hepatitis C, Crohn’s disease and ESRD (on thrice weekly haemodialysis). He underwent renal transplant in 2003 after remaining on haemodialysis (HD) for one year. Four years later he had graft rejection and again become dialysis dependent. He presented complaining of a high grade intermittent fever responding to antipyretics and vague left flank pain which was non-radiating and aggravated on movement. On examination he had a blood pressure of 100/60 mmHg, pulse of 68 beats/minute and he was afebrile. On abdominal examination, there was a scar in the right iliac fossa, the graft was palpable but not tender and there was mild tenderness in the left flank. His haemoglobin was 12gm/dl with a total leukocyte count of 12.3. A non contrast enhanced CT scan (CT KUB) showed extensive fat stranding around the left kidney with an area of high density representing a collection, the corticomedullary differentiation was lost in all three kidneys and there were small calculi in the renal graft (Figure 1 and 2). The patient was admitted with a probable diagnosis of acute pyelonephritis. The possibility of drain placement was discussed, but due to multi-focality and the small size of the collection, the decision was made for conservative management. He was started on Imipenim to which his fever responded but his haemoglobin dropped to 4gm/dl without any obvious source of bleeding. His CT scan was discussed with a radiologist and they raised suspicion of small areas of haemorrhage in the left kidney rather than a collection, along with a mixed density mass in the upper pole of the right kidney which could represent a complex cyst. A contrast enhanced CT scan of his abdomen was performed which demonstrated similar findings without any evidence of ongoing bleeding. His haemoglobin stabilized at 8gm/dl after the transfusion of 2 units of packed cells but he continued to have left flank pain despite epidural analgesia. Due to persistent pain, the drop in haemoglobin and his contralateral kidney showing a solid mass highly suspicious of a cancer an elective surgical exploration was planned. Per-operatively, the patient was found to have a huge retroperitoneal hematoma on left side crossing the mid line, from which approximately 2 litres of blood clots were evacuated. The graft kidney was small and shrunken with a stone in the renal pelvis. The right kidney contained an upper pole mass. Bilateral native and graft nephrectomy was done. The patient’s post operative course was uneventful. He was discharged home on the 6th post operative day with advice on alternate day HD. Histopathology showed a papillary renal cell carcinoma in right kidney, with foci of similar tumour seen in the left kidney as well. Extensive infarct in the left kidney was seen, the margins of the graft kidney as well as the ureteric margins of both kidneys were tumour free. Since the disease was organ confined, no further treatment was given to the patient. The patient was last seen three months ago and was well.

Discussion
End stage renal disease (defined in the Framingham Study as a GFR that is < 60% of the normal level) developed in about 9% of the subjects in the Framingham Study cohort during an 18-year follow-up period(1). The data of National Health and Nutrition Examination Surveys of 1988 to 1994 and 1999 to 2004 suggest that the prevalence of chronic kidney disease increased from 10 to 13% over the 10-year period from 1994 to 2004(2). The rate of renal replacement therapy over the 5-year observation period in a study was 1.1%, 1.3%, and 19.9%, respectively (3).
Patients with end-stage renal disease (ESRD) on dialysis have more than a 100 times greater risk of RCC than age-matched healthy controls, (4-5) and the risk is considered to be progressively higher in patients with a longer duration of dialysis (6-7). A multicenter retrospective study of more than 1200 patients by Neuzillet et al has reported that RCC occurring in patients with ESRD has many favourable clinical, pathologic, and outcome features compared with those diagnosed in patients from the general population. In the ESRD group, RCC occurred in younger patients (55+/-12 yrs vs. 62+/-12 yrs; p < 0.0001) and more often in male patients (3.2 men vs.1.6 women; p < 0.0001). Tumours were more frequently discovered incidentally in the ESRD group (87% vs.44%; p < 0.0001) (8). In this series, the incidental RCC diagnosis rate was in 87% compared with 44% in the general population. Such a high rate (75-100%) of incidental diagnosis had already been reported (9-10). This may be due to either to the constitutional low aggressiveness of ESRD tumours, related in part to their favourable histological phenotype, or to early diagnosis of renal tumours in the ESRD setting (due to screening) (8).
The spectrum of histological types of RCC arising in ESRD is distinct from that of sporadic RCC. Tumours arising in the setting of ESRD show microscopic features that are either similar to sporadic cases (clear cell, papillary and chromophobe RCC) or unique to ESRD, including acquired cystic disease (ACD)-associated RCC, and clear cell-papillary RCC (11). Clear cell carcinomas are less frequent (59% vs. 89%) and papillary carcinomas are more frequent (37%) than in the general population (7%) but with clear cell still being the most common (8).
A spontaneous retroperitoneal haemorrhage (SRH) is defined as a retroperitoneal haemorrhage that occurs without proceeding trauma or any underlying pathology. The majority of the patients respond to conservative treatment, with stopping of the offending agent (anticoagulants), judicious resuscitation with fluids and blood products and other supportive care. Surgery or radiological intervention is performed if there is evidence of continued bleeding, but these two modalities have some disadvantages. Spontaneous rupture of a renal neoplasm is a rare entity. Most commonly reported are angiomyolipomas. Other renal tumours reported in literature presenting as retroperitoneal hematoma are leiomyosarcoma (12-13) and metastatic gestational trophoblastic tumour (14). Papillary cell carcinoma of renal origin has not yet been reported in the literature presenting as retroperitoneal hematoma.
In our patient triple nephrectomy was dictated by three different indications. The left kidney was removed because it was the source of pain, bleeding and relative hemodynamic instability, the right kidney was removed as it was harbouring a solid mass suspicious of malignancy and the graft was removed as it had been rejected and also contained a calculus.

Conclusion
Annual screening of patients with ESRD should be done for RCC and ACKD. Although spontaneous rupture of renal neoplasm is a rare entity, there should be a high index of suspicion for bleeding when patients with ESRD present with flank pain in order to avoid a fatal outcome. Presentation of papillary cell carcinoma as a ruptured renal neoplasm has not been reported in literature as yet.

Figure 1: white arrow showing mixed density mass and red arrow showing extensive perinephric stranding

Figure 2: Image of right upper pole mixed density mass. Arrow showing area of collection around left kidneyReferences:

References
1. Fox CS, Larson MG, Leip EP, Culleton B, Wilson PW, Levy D. Predictors of new-onset kidney disease in a community-based population. JAMA. 2004 Feb 18;291(7):844-50.
2. Coresh J, Selvin E, Stevens LA, Manzi J, Kusek JW, Eggers P, et al. Prevalence of chronic kidney disease in the United States. JAMA. 2007 Nov 7;298(17):2038-47.
3. Keith DS, Nichols GA, Gullion CM, Brown JB, Smith DH. Longitudinal follow-up and outcomes among a population with chronic kidney disease in a large managed care organization. Arch Intern Med. 2004 Mar 22;164(6):659-63.
4. Choyke PL. Acquired cystic kidney disease. Eur Radiol. 2000;10(11):1716-21.
5. Hora M, Hes O, Reischig T, Urge T, Klecka J, Ferda J, et al. Tumours in end-stage kidney. Transplant Proc. 2008 Dec;40(10):3354-8.
6. Vamvakas S, Bahner U, Heidland A. Cancer in end-stage renal disease: potential factors involved -editorial. Am J Nephrol. 1998;18(2):89-95.
7. Peces R. Malignancy and chronic renal failure. Saudi J Kidney Dis Transpl. 2003 Jan-Mar;14(1):5-14.
8. Neuzillet Y, Tillou X, Mathieu R, Long JA, Gigante M, Paparel P, et al. Renal cell carcinoma (RCC) in patients with end-stage renal disease exhibits many favourable clinical, pathologic, and outcome features compared with RCC in the general population. Eur Urol. 2011 Aug;60(2):366-73.
9. Kojima Y, Takahara S, Miyake O, Nonomura N, Morimoto A, Mori H. Renal cell carcinoma in dialysis patients: a single center experience. Int J Urol. 2006 Aug;13(8):1045-8.
10. Neuzillet Y, Lay F, Luccioni A, Daniel L, Berland Y, Coulange C, et al. De novo renal cell carcinoma of native kidney in renal transplant recipients. Cancer. 2005 Jan 15;103(2):251-7.
11. Tickoo SK, dePeralta-Venturina MN, Harik LR, Worcester HD, Salama ME, Young AN, et al. Spectrum of epithelial neoplasms in end-stage renal disease: an experience from 66 tumor-bearing kidneys with emphasis on histologic patterns distinct from those in sporadic adult renal neoplasia. Am J Surg Pathol. 2006 Feb;30(2):141-53.
12. Grasso M, Blanco S, Fortuna F, Crippa S, Di Bella C. Spontaneous rupture of renal leiomyosarcoma in a 45-year-old woman. Arch Esp Urol. 2004 Oct;57(8):870-2.
13. Moazzam M, Ather MH, Hussainy AS. Leiomyosarcoma presenting as a spontaneously ruptured renal tumor-case report. BMC Urol. 2002 Nov 19;2:13.
14. Vijay RK, Kaduthodil MJ, Bottomley JR, Abdi S. Metastatic gestational trophoblastic tumour presenting as spontaneous subcapsular renal haematoma. Br J Radiol. 2008 Sep;81(969):e234-7

Date added to bjui.org: 09/10/2012

DOI: 10.1002/BJUIw-2012-050-web

Paratesticular mass caused by Dirofilaria repens

September 19, 2012/by admin Z.9

This report presents a case of a paratesticular lesion caused by Dirofilaria repens, a relatively rare human infection, treated by organ sparing surgery.

Authors:

Freifeld Y MD^1*, Stein A MD^1*, Nashashibi M MD², Baneth G PhD³, Dekel Y MD¹

1- Department of Uology, Carmel Medical Center and Bruce Rappaport Faculty of Medicine, Technion, Israel

2- Department of Pathology, Carmel Medical Center and Bruce Rappaport Faculty of Medicine, Technion, Israel

3- School of Veterinary Medicine, Hebrew University Israel, Rehovot

* Equally contributed

Corresponding Author: Freifeld Yuval MD, Urology department, Carmel Medical Center, Haifa, Israel. Michal 7 street, 34362 Tel. +972-52-3153511 Fax: +972-48250122 E-Mail: [email protected]

Abstract
Dirofilariasis is a relatively rare human infection, most commonly manifested as a subcutaneous nodule. A paratesticular lesion caused by Dirofilaria is extremely rare, such a lesion might be suspected to be malignant until pathological examination is performed. Since micro-filaremia is not expected surgical excision of the lesion is the treatment of choice. We present a case of a paratesticular lesion caused by Dirofilariarepens treated by organ sparing surgery.

Introduction
Human filarial infections are classified as lymphatic and non lymphatic. Among the lymphatic filarial infections, Wuchereriabancrofti and Brugiamalayiare the most common pathogens, endemic in the tropics. Non lymphatic filaria include Loa loa, Onchocercavolvulus, and Mansonellastreptocerca. Human dirofilariasis is a relatively rare infection and Dirofilariarepens infection is the most widespread form of human dirofilariasis in the world3. Dirofilariasis is apparently prevalent in Italy where over 180 cases have been reported1, however, reports of human dirofilariasis have been documented worldwide and bias regarding prevalence might arise due to underreporting. Clinically, human infection presents most often as a subcutaneous nodule. Other sites documented include conjunctiva, lungs and breast3. To date, five case reports of human dirofilariasis have been documented in Israel, one of them involving the spermatic cord.2 Worldwide, 8 cases of spermatic cord or paratesticular dirofilariasis have been reported resulting in scrotal exploration or orchiectomy3.

Case report
A 35 year old Caucasian male, who was otherwise healthy, presented with right testicular pain which started one day prior to admission. He had no urinary symptomsor pyrexia. The patient is a native Israeli and works as a mailman. He had no recent history of travel. On physical examination there were no signs of inflammation over the scrotum.
On palpation, both testes were of normal size and consistency. A firm, non tender and irregular mass, 2 cm in diameter, was palpated independent of the upper pole of the right testis. The right spermatic cord was thickened. Laboratory evaluation (complete blood count, blood chemistry and urinalysis, testicular tumor markers – αfeto protein, β HCG and LDH) was within normal limits. Ultrasongraphy (Fig. 1) revealed a hyperechoic oval mass next to the upper pole of the right testis, adherent to a markedly thickened spermatic cord. The testicle itself was uninvolved.
Inguinal exploration revealed a firm mass, adherent to the spermatic cord involving the cord’s blood vessels. The mass was carefully dissected from the spermatic cord. Frozen section showed an inflammatory process with no evidence of malignancy.
Gross pathological examination of the specimen showed a 2.5X1.6X1.7 cm grayish fibrotic mass covered with a thin serosal layer.
Histopathology showed extensive areas of necrotizing inflammation and large numbers of eosinophils. Longitudinal and transverse sections of a helminth could be seen in some of the levels of the sectioned mass (figures 2, 3 and 4). The cuticle’s external surface showed longitudinal ridges. The muscle bundles were of the coelomyarian type and filled the pseudocoelomic space. The muscle mass was divided into dorsal and ventral components by prominent lateral cords. There was a single large reproductive tube but no spermatozoa were found. A much smaller digestive tube was located nearby. Polymerase chain reaction (PCR) was performed, a DNA fragment consisting of 203 base pairs was found with a 98% match to Dirofilariarepens.
Based on these morphological characteristics and PCR results, the worm was identified as Dirofilariarepens.
Follow up ultrasound showed marked improvement with reduction of the spermatic cord swelling, no mass was identified, right testicular perfusion seemed appropriate. No specific anti-filarial treatment was given.
Discussion
Dirofilariasis is a zoonotic infection, caused by nematodes of the genus Dirofilaria, affecting primarily dogs and other mammals4. Several mosquito species have been identified as vectors of filarial infections5. A filarial larva (stage 2) is taken up by the mosquito from an infected host, the larva then further develops to become infective (stage 3) and passed on to its new host on the next blood meal. After maturation female worms again produce first stage larvae causing micro-filaremia. However in infected humans the worms die causing host response and tissue reaction.4
It takes several months for filarial larvae to reach sexual maturity in their definitive host4, as mentioned, dirofilaria rarely reaches maturity in humans. Usually no host response is observed in humans after the initial infection. It is assumed that only after the death of the worm a host response is induced and symptoms arise. Most commonly only one worm is found in an infected human and micro-filaremia is extremely rare4. Surgical excision is considered a definitive treatment and usually no systemic treatment is indicated.
A paratesticular mass or spermatic cord lesion caused by Dirofilaria spp. is a rare occurrence and may be suspected to be malignant until proven otherwise. The clinical diagnosis of filarial infection in humans is difficult since symptoms are variable and non-specific6, to our knowledge eight cases of paratesticular dirofilariasis have been reported, in these cases some patients had symptoms such as swelling and groin pain concomitantly with the mass.

Conclusions
Although relatively rare, filarial infections should be considered in the differential diagnosis of any paratesticular mass. We believe that every paratesticular mass should be explored trans-inguinally. On frozen section, a severe inflammatory reaction to filaria may be detected leading to organ sparing surgery. Filarial infections have unique and obvious features on pathological examination. We believe that confirmation of filarial subtype by PCR is not absolutely necessary since surgical excision of the mass is the treatment of choice with no need for further medical treatment regardless of filarial subtype.

Conflicts of interest
No conflicts interest to be declared

References
1. Pampiglione S, Rivasi F.Human dirofilariasis due to dirofilariarepens: an update of world literature from 1995 to 2000. Parassitologia42, 231-254
2. Munichor M, Gold D, Lengy J, Linn R, Merzbach D.An unusual case of dirofilaria conjunctivae infection suspected to be a malignancy of the spermatic cord. IMAJ3, 860-861
3. .Pampiglione S, Fioravanti ML, Piccolotti D, Pizzicannella G, Reale D.Human dirofilariasis in italy: a new case in the spermatic cord. parassitologia44, 93-96
4. Orihel TC, Eberhard ML. Zoonotic Filariasis. ClinMicrobiol Rev.11(2), 366–381
5 Anyanwu IN, Agbede RI, Ajanusi OJ, Umoh JU, Ibrahim ND.The incrimination of Aedes (Stegomyia) aegypti as the vector of Dirofilariarepens in Nigeria.Journal Veterinary Parasitology92(4), 319-327
6. Sabu L, Devada K, Subramanian H. Dirofilariosis in dogs & humans in Kerala. Indian J Med Res. 121, 691-693

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Date added to bjui.org: 19/09/2012

DOI: 10.1002/BJUIw-2012-071-web

Pheochromocytomas of the vesical and paravesical region

August 8, 2012/by admin Z.9

We report our experience with four cases of vesical and paravesical pheochromocytomas treated in our hospital over the past 15 years.

Authors: Nitin Kapoor¹,M.S Seshadri ¹,Nihal Thomas¹, Antony Devasia²

John Banerjee², Ramani Manoj Kumar³,Simon Rajaratnam¹

1. Department of Endocrinology; 2 Department of Urology; 3 Department of Pathology, Christian Medical College, Vellore, India

Corresponding Author: Dr Simon Rajaratnam, Department of Endocrinology, Diabetes & Metabolism, Christian Medical College, Vellore, India. 632004. Email: [email protected] ; Phone: +91-416-2282528

Abstract

Purpose

Pheochromocytomas of the urinary bladder are extremely rare. They account for less than 0.06% of all the tumors arising from the urinary bladder. Even though 10% of pheochromocytomas occur at extra adrenal sites, barely 1% arise from the urinary bladder. Embryonic rests of chromaffin tissue within the sympathetic plexus in the bladder wall are the source of these tumors. Due to varied symptomatology and nonspecific physical findings, a high index of suspicion is required for accurate diagnosis. Therapeutic strategies are not well-defined owing to the rarity of these tumors.

Material and Methods

We report a series of four cases of vesical and para vesical pheochromocytoma treated in our hospital over the past 15 years. Here, the authors have described the clinical presentation, physical findings, laboratory investigations and treatment provided in all the 4 cases. We have also included radiological images and histopathology slides with input from both radiologists and pathologists. Surgical management and post-operative follow up are discussed, as are details of previous published data.

Results and Conclusion

Bladder pheochromocytomas are rare but unique tumors. We share our experience with four cases treated in our hospital.

Introduction

Pheochromocytomas of the urinary bladder are extremely rare. They account for less than 0.06% of all tumors arising from the urinary bladder. [1, 2] Even though 10% of pheochromocytomas occur in extra adrenal sites, barely 1% arise from the urinary bladder.[3] Embryonic rests of chromaffin tissue arising from the sympathetic plexus within the bladder wall are the source of these tumors .[4] Due to varied symptomatology and nonspecific physical findings, a high index of suspicion is required for accurate diagnosis. Therapeutic strategies are not well-defined owing to the rare occurrence of these tumors.

We report our experience with four cases of vesical and paravesical pheochromocytomas treated in our hospital over the past 15 years.

Case Report 1

A 28 year old woman presented in 1996 with a history of recurrent episodes of chest pain with profuse sweating and palpitation for 4 years, 5 these episodes usually occurred during micturition or soon after micturition. One month prior to admission she also had an episode of hematuria. Prior to the onset of her symptoms she went through two uneventful vaginal deliveries. Her family history was unremarkable.

On examination, her pulse rate was 90/min and blood pressure 140/90 mmHg. After micturition her pulse rate increased to120/min and blood pressure increased to 160/110 mmHg. Cardiovascular, respiratory system, abdominal and CNS examination were all normal. No bruits were heard over the abdomen. Fundus examination revealed grade 2 hypertensive changes.

Her full blood count and urea and electrolytes were normal. Urine microscopy was normal; there was no proteinuria or glycosuria. Twenty four hour urinary Vanillyl Mandelic Acid (VMA) levels were elevated at 8 mg and 11mg (normal <7 mg/24 hours). ECG revealed sinus tachycardia with no evidence of LVH. Chest X ray was normal. Ultrasound revealed an intravesical mass extending into the perivesical space. MIBG (Meta-Iodo-Benzyl-Guanidine) scan showed tracer uptake over the tumor.

After adequate control of blood pressure she underwent partial cystectomy. The post operative period was uneventful and all anti-hypertensive agents were successfully withdrawn. She has remained asymptomatic on follow up.

Case Report 2

A 50 year old man who was being treated for diabetes and hypertension presented in 1999, with a one year history of fatigue, weight loss, palpitations and breathlessness. Routine ultrasound examination revealed a paravesical mass. CT scan revealed a large 10 x 10 cm heterogeneous mass at the superior aspect of the prostate and base of the bladder, extending up to the symphysis pubis. He had no bowel or micturition related symptoms. He underwent laparotomy at his local hospital. Since the tumor could not be excised he was referred to us for further management. A biopsy was reported as showing a paraganglioma. MIBG scan showed uptake over the tumor. He underwent cysto-prostatectomy with Mainz II pouch diversion. The immediate post-operative period was uneventful, however he later developed sepsis and succumbed on the 10th postoperative day.

Case Report 3

A 28 year old woman, presented in 2010 with a history of intermittent severe headache for 15 years. Two years following the onset of these symptoms she had an episode of severe headache and loss of consciousness. She was hospitalized and was found to have sustained a right hemiparesis secondary to an intracerebral bleed. She gradually regained motor power on the affected side, but continued to experience frequent headaches.

One year before she started complaining of blurring of vision and was found to have severe hypertension. Her documented blood pressure readings were as high as 210/140 mmHg. She complained of severe headache, palpitations and sweating following micturition.

On examination her blood pressure was 150/110 mmHg. Following micturition her blood pressure rose to 190/130 mmHg. There were no bruits over her abdomen. She had grade 1 hypertensive changes in her fundi. The rest of the systemic examination was normal.

On investigation she had mild anemia. Her serum calcium, phosphorus, electrolytes and creatinine were normal. Urine examination showed no abnormality. Her chest X-ray, ECG and echocardiogram showed left ventricular hypertrophy. Her 24 hour urinary metanephrine level was 88 μg/1450 ml (normal < 350 μg/24 hour) and urinary

nor-metanephrine level was 1848 μg/1450 ml (normal <600 μg/24 hour).

CT angiogram revealed a 4.3 x 4 cm hypervascular mass in the inferolateral wall of the urinary bladder bulging into the lumen. Her MIBG scan however showed no uptake over the lesion.

After adequate control of blood pressure she proceeded to surgery. Cystoscopy revealed a normal external urethral meatus, normal urethra and ureteric orifices. The bladder mucosa appeared normal. At laparotomy, there was a 5 x 5 cm mass involving the anterior and left lateral wall of the bladder. At cystotomy, the mucosa appeared normal but the tumor could be felt extending up to the bladder neck, adjacent to the ureteric orifice. A partial cystectomy was performed and the tumor was excised with a margin beyond its palpable edge. The postoperative period was uneventful and her anti-hypertensive medication was discontinued. Histopathological examination revealed features of pheochromocytoma, with typical ‘zell-ballen’ arrangement of tumour cells, separated by a delicate network of capillary sized blood vessels. There was mild nuclear pleomorphism, but no evidence of malignancy. The tumour cells were positive for Chromogranin A, synaptophysin, and negative for cytokeratin (Fig 1 & 2).

Figure 1.

Figure 2.

At subsequent follow up her urinary nor- metanephrine level had normalized (180 µg/24 hours), she however needed to be restarted on antihypertensive medication. Follow up cystogram was normal.

Case Report 4

A 24 year old man presented in 2011 with intermittent painless hematuria for 12 years. There was no history of headache, blurred vision, palpitations or syncope. He was not on any medication. He had been evaluated elsewhere and found to have a bladder tumor for which he underwent 4 transurethral resections over a period of 9 years. An open resection was also attempted but surgery was abandoned as the patient developed extremely high intraoperative blood pressure.

The patient was evaluated at our centre and found to have elevated urinary nor-metanephrine levels (3019 µg/24 hours) and normal urinary metanephrine levels (141 µg/24 hours). His MIBG scan showed a functioning neuroendocrine tumor in the superolateral aspect of the bladder. CT scan revealed a well defined enhancing mass lesion arising from the right lateral wall of the urinary bladder.

Figure 3.

Cystoscopy under general anesthesia revealed a 3 x 3 cm extra mucosal mass arising from the right lateral bladder wall, above the right ureteric orifice. There was a 1X1cm ulcerated area at the center of the lesion. The left ureteric orifice and the rest of the bladder mucosa were found to be normal. We proceeded with excision of the mass with partial cystectomy. The mass was found to be adherent to the lateral pelvic wall probably due to previous attempts at excision. There was no fluctuation of blood pressure during surgery. Histopathology of the tumour came back as showing malignant pheochromocytoma arising from the bladder wall. On follow up his urinary metanephrine levels have normalized and he has remained asymptomatic.

Figure 4.

Figure 5.

Discussion

Pheochromocytoma of the urinary bladder is a rare tumor. Such tumors arise from the chromaffin tissues associated with sympathetic nerves located within the bladder wall. They are hormonally active and cause symptoms including palpitations, sweating, headache and hypertension (paroxysmal/sustained). These symptoms are usually precipitated by micturition.[6] A rise in blood pressure can be demonstrated immediately following micturition. The other precipitating factors include abdominal palpation, defecation and sexual intercourse. However, not all patients demonstrate these signs and symptoms due to receptor down regulation following prolonged catecholamine exposure. Signs and symptoms of urethral obstruction may also occur. [7] Painless hematuria occurs in 50-60% of patients. The diagnosis of this condition warrants a high index of clinical suspicion.

The most useful laboratory test is to determine blood and urinary catecholamine levels and measure their metabolic byproducts. Urinary VMA levels >9.0 mg/24 hours, nor epinephrine > 80 μg/24 hours and epinephrine > 20 μg/24 hours have been found in most patients. While CT/MR aid in the anatomical localization of these tumors, iodine-131– metaiodobenzylguanidine (MIBG) and indium-111 pentoctreotide scintigraphy serve as complementary functional diagnostic tools as they have 85-100% sensitivity in localizing these tumors.[8,9] One of our patients, (case 3) however had a negative MIBG scan. Positron emission tomography (PET) using 6 – [18F] fluorodopamine is a more recently developed tool for imaging these tumors.

As preoperative preparation patient require alpha adrenoreceptor blockers for at least 2 weeks, beta blockers are subsequently added to control heart rate. They also need adequate hydration to increase intravascular volume prior to surgery.

Most vesical pheochromocytomas are intramural as the sympathetic plexus is scattered between all the layers of the bladder wall. At cystoscopy these tumors may appear granulated and lobulated with or without ulceration.[2] Transurethral resection is not recommended as it will not remove the entire tumor. Open surgery is required to completely resect these tumors. [2,7,11] Laparoscopic tumor resection has also been attempted.[10]

As part of the intraoperative strategy, the use of cystoscopic examination may help delineate the exact location of these tumors and identify the depth of invasion and the involvement of the ureters. However, cystoscopic examination in case 3 was non-contributory probably due to the intramural nature of the tumor. In this case excision with a margin was guided by palpation. A thorough examination of surrounding structures and regional lymph nodes should be done as the malignant potential of these tumors is predicted not by histology but by clinical evaluation. In addition, these patients should also be monitored for post-operative complications such as stricture or obstruction at the urethral reimplantation site.

Overall, pheochromocytomas of the urinary bladder have a slightly better prognosis as compared to other extra adrenal pheochromocytomas. As these tumors are known for recurrence and metastases, these patients will require lifelong follow up. Though the diagnosis of pheochromocytoma is confirmed biochemically and the tumor located by using imaging techniques, biochemical evidence of excess catecholamine production usually antedates clinical symptoms, and therefore annual determination of urinary catecholamine / metabolites is recommended.[13,14] Prognosis will depend on the presence of familial endocrinopathy or the presence of metastases .[11]

In summary, due to varied symptomatology and nonspecific physical findings, a high index of suspicion is often required for accurate diagnosis and management of these patients.[15]

References

1. Onishi T, Sakata Y, Yonemura S, et al. Pheochromocytoma of the urinary bladder without typical symptoms. Int J Urol 2003; 10:398-400.

2. Doran F, Varinli S, Bayazit Y, et al. Pheochromocytoma of the urinary bladder. APMIS 2002; 110:733-6.

3. Salanitri J, Smith P, Schlicht S. Multifocal malignant extra-adrenal paragangliomas of the Organ of Zuckerkandl and urinary bladder. Australas Radiol 2001; 45:229-32.

4. Tan TL, Young BW (1962) Pheochromocytoma of the bladder: Case report. J Urol 87:63–67

5. S Rajaratnam, MS Seshadri, G Gopalakrishnan, SM Chandy(1999) Pheochromocytoma of the urinary bladder. J Assoc Physicians India Vol 47: 246-247.

6. Bowne RB, Beltaos E (1967) Pheo of the bladder: Case report and summary of literature. J Urol 98:361

7. Bonacrzu Kazzi G. Asymptomatic bladder pheochromocytoma in a 7-year-old boy. J. Paediatr Child Health 2001; 37:600-2.

8. Berglund AS, Hulthen UL, Manhem P, et al. Metaiodobenzylguanidine (MIBG) scintigraphy and computed tomography (CT) in clinical practice. Primary and secondary evaluation for localization pheochromocytomas. J Int Med 2001; 249:247-51.

9. Nakatani T, Hayama T, Uchida J, et al. Diagnostic localization of extra-adrenal pheochromocytoma: Comparison of (123)I-MIBG imaging and (131)I-MIBG imaging. Oncol Reports 2002; 9:1225-7.

10. Kozlowski PM, Mihm F, Winfield HN. Laparoscopic management of bladder pheochromocytoma. Urology 2001; 57:365.

11. Hwang JJ, Shoaf G, Uchio EM, et al. Laparoscopic management of extra-adrenal pheochromocytoma. J Urol 2004; 171:72-6.

12. Snavely MD, Mohan LC, O’Conor DT, Insel PA (1983) Selective down regulation of adrenergic receptor subtype in tissues from rats with pheochromocytoma. Endocrinology 113:354–361

13. Whalen RK, Althausen AF, Gilbert HD (1992) Extra-adrenal pheochromocytoma. J Urol 147:1–10

14. Ahmed S. Safwat, Nabil K. Bissada, Raouf M. Seyam, Saif Al Sobhi, Kamal A. Hanash. The clinical spectrum of pheochromocytoma: analysis of 115 patients. BJUI 2008; 101: 1561-1564

15. Safwat S. Ahmed; Bissada K. Nabil. Pheochromocytoma of the urinary bladder. Can J Urol 2007; 14: 3757 – 3760

Date added to bjui.org: 08/08/2012

DOI: 10.1002/BJUIw-2011-144-web