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Safety first? Necrotizing cystitis after autoerotic manipulation

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To the best of our knowledge, this is the first reported case of  necrotising cystitis caused by the insertion of a foreign body.

 

Authors: S. Knipper, B. Feyerabend, A.J. Gross, T. Bach

Department of Urology, Asklepios Klinik Barmbek, Hamburg, Germany.

 
Corresponding Author: Sophie Knipper MD, Asklepios Klinik Barmbek, Department of Urology, Rübenkamp 220 , 22291 Hamburg/ Germany.   E-mail: [email protected]

 

Abstract
 
We present the case of a 72-year old man with necrotising cystitis. The patient was admitted after having been diagnosed with gross haematuria and an acute abdomen complicated by systemic inflammatory response syndrome (SIRS) and severe hyperglycemia in a peripheral hospital. Work-up included a CT scan that revealed a gas-forming infection of the lower abdomen, most likely caused by a foreign body which appeared to be within the urethra. The patient died within hours of multi-organ failure.
Emphysematous cystitis, in this case complicated by necrosis, is a rare clinical entity, most frequently seen in diabetic women. It can rapidly progress to severe sepsis in the absence of early therapeutic intervention. Conservative management leads to a full recovery in most cases, in some however, further treatment such as partial cystectomy, cystoprostatectomy or surgical debridement is required.

 

Introduction
 
Gas-forming (emphysematous) infection of the urinary tract is a potentially life-threatening condition (1). Emphysematous cystitis is a rare complication of  lower urinary tract infection, and is characterised by air within the bladder wall and lumen (2). Most commonly, it is found in middle-aged diabetic women. Risk factors for complicated urinary tract infections include the presence of an indwelling urethral catheter, urinary tract outlet obstruction, neurogenic bladder  or recurrent urinary tract infection (3). Pre-existing conditions such as diabetes, alcoholism and malnutrition are also described as possible risk factors for emphysematous cystitis (4).
To the best of our knowledge, this is the first reported case of  necrotising cystitis caused by the insertion of a foreign body..

 

Case report
 
A 72-year old mentally confused man was found in the street and was admitted to a peripheral hospital. He  presented with gross haematuria and an acute abdomen complicated by SIRS and severe hyperglycaemia. Initially, the patient was conscious and responsive. The complete blood cell count indicated a mild leucocytosis of 12.5/nl and a serum glucose level of 970 mg/dl. The patient underwent a CT scan for further evaluation. The scan showed a gas-forming infection in the lower abdomen with a necrotic bladder and partial necrosis of the prostate. A 6 cm long metal object appeared to be located within the urethra (Fig. 1 and 2).

 

Figure 1. CT-scan with the signs of an gas-forming infection.

 

 

Figure 2. CT-scan with a metal object in the urethra.

 

 

Approximately three hours after the primary admission he was transferred to our hospital, , in a deteriorating clinical condition.
Upon admission to our department, the patient was no longer responsive, and was hypotensive and tachycardic. The complete blood cell count now indicated a leucocytosis of 29.1/nl, his blood chemistry showed a creatinine of 4.7 mg/dl,  CRP of 305.3 mg/l and an persistent severe hyperglycaemia with a glucose level of 502 mg/dl.
In this terminal condition, the patient was no longer suitable for anaesthesia and we decided against an exploratory laparotomy. The patient died within hours.
Autopsy revealed extensive ulceration of the bladder mucosa with diffuse haemorrhagic changes in all layers and invasion into the prostate. In addition it showed the formation of air-filled cavities, within the bladder. Microbiology suggested an infection with a gram-positive organism (Enterococcus faecalis).
The cause of the necrotising cystitis was two safety pins, found in the bladder and urethra, which were most likely inserted for autoerotic purpose (Fig. 3 , 4 and 5).
 

Figure 3. Macroscopic image of  the urogenital system showing the kidneys and haemorrhagic bladder. The safety pin can be seen within the bladder.

 


 
Figure 4. Histological image of the emphysematous infection. Air-filled cavities can be seen.

 

 
 
Figure 5. Histological image of the bladder wall. Extensive ulceration of the bladder mucosa with diffuse haemorrhagic changes in all layers can be seen.

 


 
 
Discussion
 
Necrotising cystitis is a very rare entity in clinical practice. It is characterized as a emphysematous cystitis which can be complicated by complete necrosis of the bladder ( ).
In most cases, the course of disease is favourable. In the literature, it is stated that only about 10% of patients need medical and surgical treatment, with an overall mortality rate of 7% (2).
The pathogenesis of gas-forming infections of the urinary tract is not fully understood at present. It is thought to be due to a combination of the presence of gas-producing organisms, impaired tissue perfusion, and a high tissue glucose concentration; all are prerequisites that favour the development of emphysematous infections ( ). In most cases, the bacteria causing the infections are identified as E.coli, but Klebsiella pneumoniae, Clostridium species and Enterobacter species are also often found. In this case however, the basic cause of the infection was two foreign bodies which were inserted into urethra and bladder. This presumably happened for autoerotic purposes. However, since a detailed medical history could not be taken, , a traumatic cause can not be excluded.
The symptoms of emphysematous cystitis vary from a patient being almost asymptomatic, to presenting with an acute abdomen and severe sepsis. The most common clinical presentation is with lower abdominal pain, followed by dysuria, haematuria and sometimes pneumaturia (3). The infections occur more commonly in middle-aged woman, the main comorbidity is diabetes. Other risk factors include malnutrition or alcoholism.
Emphysematous or necrotising cystitis can be diagnosed radiologically by a simple plain film of the abdomen or on CT scanning, which can also differentiate vesicocolic fistula, intra-abdominal abscess, neoplastic diseases or the complication of an emphysematous pyelonephritis (2). In our case a foreign body could be seen in the CT scan.
Concerning the outcome, the most important issue is an early diagnostic and therapeutic approach. Depending on the course of the infection,  medical treatment with an intravenous antibiotic should be commenced. In some cases, additional treatment has to be considered. Severe infections might require partial cystectomy or even radical cystoprostatectomy.
In our case the patient deteriorated very rapidly. Since he presented in multi-organ failure caused by urosepsis and septic shock, no radical operation could have been considered life-prolonging. This underlines the need for  early diagnosis and treatment.
The authors declare that they have no competing interests.

 

References
 
1. Grayson DE, Abbott RM, Levy AD, Sherman PM. Emphysematous infections of the abdomen and pelvis: a pictorial review. Radiographics. 2002; 22:543-561.
2. Thomas AA, Lane BR, Thomas AZ, Remer EM, Campbell SC, Shoskes DA. Emphysematous cystitis: a review of 135 cases. BJU Int. 2007; 100:17-20.
3. Pérez Fentes D, Blanco Parra M, Lema Grille J, Toucedo Caamaño V, Novás Castro S, Lamas Cedrón P, Villar Núñez M. Emphysematous cystitis: case report. Arch Esp Urol. 2009; 62:392-395.
4. Perlemoine C, Neau D, Ragnaud JM, Gin H, Sahnoun A, Pariente JL, Rigalleau V. Emphysematous cystitis. Diabetes Metab. 2004;30:377-379.
5. Rindom AB, Gudnason HM, Thind PO. Emphysematous cystitis with total necrotization of the bladder. Ugeskr Laeger. 2008;170:3876.
6.  Huang JJ, Chen KW, Ruaan MK. Mixed acid fermentation of glucose as a mechanism of emphysematous urinary tract infection. J Urol. 1991;146:148-151.

 

Date added to bjui.org: 10/01/2012 


DOI: 10.1002/BJUIw-2011-114-web

 

Zero Ischemia Robotic Laser Partial Nephrectomy: Use of the Thulium laser in Renal Cell Carcinoma

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We present a technique for zero-ischemia robotic laser partial nephrectomy, which addresses current concerns focusing on renal ischemia during surgery.

 

Authors: Janet Colli, Gregory Mitchell, Benjamin R. Lee

Department of Urology, Tulane University, New Orleans, LA, USA
 
Corresponding Author: Benjamin R. Lee,  Department of Urology, Tulane University, New Orleans, LA, USA. E-mail: [email protected]
 
ABSTRACT

Objective

To describe use of a robotic-assisted laparoscopic partial nephrectomy technique utilizing the thulium laser for tissue welding without clamping the hilar vessels.

Materials and Methods

A Thulium laser at 30W and a 400 micron fiber placed through a robot arm to excise and achieve hemostasis during resection of renal masses. The masses were localized intraoperatively with a 10mm laparoscopic renal ultrasound probe.  At no point during the procedure were the renal hilar vessels clamped. There was minimal blood loss during the operation. Following resection, the base of the tumor resection bed was oversewn with a running 2-0 Vicryl suture and secured with LapraTy clips. The renal capsule was then closed with 0 Vicryl over a Surgicel bolster together with Floseal for an additional degree of hemostasis.

Results

The robotic partial nephrectomies were completed without complication, with complete tumor excision confirmed by negative pathologic surgical margins.  Final pathology demonstrated clear cell cell carcinoma. Patients were discharged on postoperative day 2, and have had no long term complications.

Conclusions

Partial nephrectomy continues to grow in application for treatment of T1 renal cell carcinoma.  We present a technique for zero-ischemia robotic laser partial nephrectomy, which addresses current concerns focusing on renal ischemia during surgery.

INTRODUCTION

Widespread application of laparoscopic and/or robotic assisted partial nephrectomy has been limited by concerns regarding warm and cold ischemia limits during resection as well as technical performance. Currently, there is a lack of reliable means to achieve cold ischemia laparoscopically or robotically , and controversy persists as to the limits of warm ischemia before irreversible nephron death occurs. The ultimate goal is to develop a laparoscopic partial nephrectomy technique which allows bleeding to be controlled during the operation without the need to clamp hilar vessels, hence resulting in zero ischemia time and maximizing nephron preservation.

Over the past decade, several researchers have demonstrated laser partial nephrectomy without the need to clamp the hilar vessels in the animal model [1-3].  In 1998, the Nd:YAG laser was the first to be tested on a series of seven patients with small renal masses during laparoscopic partial nephrectomy without hilar clamping [4].  Since that time, reports have been published detailing operative experience from a series of five patients who underwent laser-supported partial nephrectomies without hilar clamping using open surgical techniques [5]. We describe a robotic assisted laparoscopic partial nephrectomy technique with laser tissue welding using a Thulium laser and robotic arm adaptor to induce parenchymal resection and minimize hemorrhage whereby the vessels are not clamped during resection.  This technique was utilized in the care of two patients at our institution after approval by Tulane’s Institutional Internal Board Review committee.

CASE REPORTS

An incidental solid renal mass was discovered in a 55 year old male during an evaluation for vague abdominal symptoms. CT scan revealed a 2.5 centimeter peripherally located solid, enhancing, left renal mass. In similar circumstances, a left lateral 2cm renal mass was incidentally found in a 62 year old male. Both patients underwent robotic assisted left partial nephrectomies and we share our experience with laser tissue welding and present the salient points of the procedure.

A standard modified flank position with transperitoneal approach was used.  Two 8 mm robotic trocars were placed and two assistant working ports were placed (one 5mm and one 12mm port). Location of the robotic trocars and working ports vary depending on the location of the renal mass (see Figure 1 for illustration of port placements).

Figure 1. Trocar Placement for Robotic Left Partial Nephrectomy

After docking the robot, a 30 degree robotic lens, focused down, was placed in the camera port, the Monopolar scissors were placed in the right robot port and the Prograsp forceps were placed in the left robot port.  The white line of Toldt was incised to expose the retroperitoneum, and the ispilateral colon was reflected medially. The ureter was identified and mobilized from the surrounding tissues, and care taken to not devascularize the ureter. The renal mass was identified and localized with  laparoscopic renal ultrasound.  The capsule around the renal tumor was scored with the bipolar forceps.

Using a Thulium laser at 30 Watts, we resected the renal tumor with a 400 micron fiber placed through a robotic arm.  The wavelength of the Thulium laser system, which is almost identical to the well accepted Holmium laser, is known for its suitability for resection and fragmentation, its safe application in an aqueous medium, and excellent hemostasis. However, in contrast to ruptured cutting edges affected by the “pulsed operation mode of Holmium lasers, the Thulium laser operates in a continuous wave mode and evaporates tissue continuously without generating pressure waves” [6]. The Thulium laser wavelength penetrates tissue to a depth of 0.5mm and provides strong absorption in any kind of tissue and gives access to a wide range of flexible fiber delivery systems [6]. The laser fiber traversed the kidney parenchyma while sealing the tissue and achieving hemostasis. Irrigation was provided through both the robotic arm and the assistant port using an irrigator-aspirator. Smoke was evacuated with the suction device through a 5mm assistant port. No bulldog clamps were placed on the renal hilum during the entire procedure.

There was minimal bleeding during the resection. The collecting system was not entered during the surgery. The base of the tumor resection bed was oversewn with a running 2-0 Vicryl (Ethicon Inc., Somerville, NJ) suture and secured with LapraTy.  The renal capsule was closed with 0 Vicryl over a Surgicel (Ethicon Inc., Somerville, NJ) bolster together with Floseal (Baxter, Deerfield, IL) for an additional degree of hemostasis. A Jackson-Pratt drain was left over the resection bed. The patient was admitted to the hospital wards for routine postoperative care.

The robotic partial nephrectomies were performed without complication. The operative times were 191 and 155 minutes, with a blood loss of 200 mL and 150mL respectively (see Table 1 for patient demographic information).

Table 1. Demographic and laboratory information from patients undergoing unclamped robot-assisted laparoscopic partial nephrectomy

DG JP
Date 10/19/2010 1/13/2011
Tumor Size 1.0 cm 1.1 cm
Tumor Location Mid Pole of Left Kidney Mid Pole of Left Kidney
Pathology Type Clear Cell Polypoid Renal Cell Carcinoma Papillary Renal Cell Carcinoma
Preop Creatinine 0.9 1.3
Preop GFR 66 59
Postop Creatinine Day 1& 2 1.3,     1.1 1.2,     1.0
Postop GFR Day 1 60 64
Operative Time 3h11m (dock time: 2h20m) 2h33m
Length of Hospital Stay 2 days (~52 hours) 2 days (~51 hours)

 
In both cases, postoperative drain output was minimal and abdominal examination demonstrated no clinical evidence of urinoma or delayed hemorrhage. The patients’ hospital courses were uneventful, and they were discharged on postoperative day two.  Follow-up care has been conducted on an outpatient basis.

Histopathologic analysis showed preservation of the renal parenchyma immediately beneath the laser scoring, with final pathology revealing Clear Cell Renal Carcinoma, Fuhrman nuclear grade 1-2/4 with negative margins, stage T1/ N0/M0 and Papillary Renal Cell Carcinoma in the second patient. Tumor excision was complete on all sides and margins were free of cancer (see Figure 2a and 2b).

Figure 2a and 2b. Pathological Photographs

Serum creatinine on postoperative day two= 1.1 (eGFR=73), and 1.0 (respectively) unchanged from that measured preoperatively. Serum hemoglobin and hematocrit remained unaltered during or after the surgery.

DISCUSSION

Recent American Urologic Association guidelines recommend partial nephrectomy for surgical treatment of small, T1 renal masses in order to preserve renal function and minimize cardiovascular comorbidities [7]. Partial nephrectomies are performed with increasing application as the technique has evolved to address previously daunting technical issues such as risk of hemorrhage and development of fistulae postoperatively. Laparoscopic partial nephrectomy (LPN) has been developed to reduce morbidities that occur with the open technique, but LPN requires advanced training to accomplish tumor resection and renal reconstruction while minimizing warm ischemia times. Robotic assistance is able to potentially decrease the learning curve of LPN, largely due to the reconstruction and needle placement afforded provided by the articulation of the robotic arms, the ability to move 180 degrees, and the 3-D vision provided by the binocular laparoscope lens.   Compared to laparoscopic partial nephrectomy, the use of the Davinci robot has been shown to decrease warm ischemia time due to its ability to facilitate the sutured parenchymal reconstruction [8].  However, widespread application of laparoscopic and/or robotic assisted partial nephrectomy has been limited by the lack of a reliable means of instilling ice to attain cold ischemia coupled with limits of warm ischemia during hilar clamping.

A recent study reported  approximately one third of patients who undergo a partial nephrectomy with renal hilar clamping in addition to definable high risk surgical factors and preexisting renal insufficiency go on to develop chronic kidney disease (stage III) [9]. The cause of chronic renal insufficiency in these patients may be related to warm ischemia. Warm ischemia decreases the threshold for ischemic/perfusion injury, which subsequently results in decreased glomerular filtration rate and may be permanent in cases with prolonged warm ischemia time. Ultimately, minimizing warm ischemia time is the most important modifiable factor in limiting kidney injury following partial nephrectomy.

It is not surprising, therefore, that techniques that eliminate the clamping of the renal vessels during partial nephrectomy are continuously sought.  One such recently described technique employs induced hypotension with nadir systemic blood pressure occurring during excision of the deepest portion of a tumor (along with continuous monitoring of a variety of physiological parameters) in order to minimize blood loss during partial nephrectomy with unclamped renal vessels [10].  In contrast, our technique makes use of the unique tissue-welding properties of the Thulium laser to provide hemostasis.  Over the past decade, various lasers have been evaluated as potential hemostatic energy sources during partial nephrectomy [1-3]. The first laser to be tested during an open partial nephrectomy was a CO2 laser used on a canine model in 1972 [11]. This laser was found to have poor hemostatic qualities and its use in kidney surgery was discontinued. The YAG laser was then investigated by Malloy during open partial nephrectomy performed under cold ischemia in a series of six patients with solitary kidneys in 1986 [12].   The Ho: YAG laser was used in the canine model during open partial nephrectomies in 1992, and was found to result in a two-fold reduction in the depth of necrosis when compared to the Nd: YAG laser [13].  Even so, the overall conclusion at that time was that laser dissection offered no advantage over cold ischemia routinely used in the open surgical technique.

In 1998, Janetscheck was the first to use laser tissue welding (Nd:YAG) during laparoscopic partial nephrectomy in a series of seven patients [4]. Over the next ten years, several investigators tested a multitude of lasers and evaluated their ability to coagulate renal tissue during partial nephrectomies in the animal model [14, 15]. In 2001, Lotan demonstrated a laser laparoscopic partial nephrectomy in an unclamped porcine model [14].  Moinzadeh performed laparoscopic partial nephrectomies in a series of six calves in 2005, using a 532nm wavelength laser (KTP Greenlight) [15].  In separate studies, Hindley and Liu further investigated the characteristics and settings of the KTP laser. They found the KTP laser had excellent hemostatic properties; however saline irrigation and smoke evacuation were required to overcome the problem of char build-up and smoke production [16, 17]. In 2007, Anderson examined the KTP laser in the porcine model, and found the setting of 80W was best for tissue cutting and 30W was best for coagulation delivered via a 365 um fiber [18]. The Cleveland clinic examined the Greenlight laser system use in conjunction with a robotic assisted laparoscopic partial nephrectomy. They found hemostasis was problematic throughout the case and also charring of the renal parenchyma obscured the intrarenal dissection and contributed to the focally positive margin [15]. Furthermore, high affinity of the 532 nm wavelength light for hemoglobin resulted in excessive absorption of the laser light with resultant decrease in the cutting efficiency of the laser beam [14].

By 2007, the Thulium laser was approved for use and Bui investigated the 30 Watt laser system delivered via a 365 μm fiber in a porcine model [19]. In 2008, Gruschwitz performed open partial nephrectomies in five patients without hilar clamping using the Thulium laser system [5]. The authors found the thulium laser could coagulate vessels up to 1.5 mm and contributed to short operative times, minimal blood loss and decreased the need for cold ischemia. This technique has an even greater need in laparoscopic and robotic cases in which cold ischemia is difficult to achieve.

We previously reported the technique of laser partial nephrectomy via a transgastric route in the animal model in 2010 [20].  With a controlled gastrotomy through the fundus of the stomach, an endoscope was passed to visualize the upper pole of the left kidney.  The thulium laser was used to resect the upper pole with adequate hemostasis, and specimen extraction with a wire snare.  Closure of the stomach was performed with metal clips.

Recent experimental studies have demonstrated other potential uses of the Thulium laser. Blackmon[21]evaluated the Thulium laser fiber’s capability of vaporizing urinary stones, and found vaporization rates for the Thulium laser averaged 5-10 times higher than for the holmium laser at 70 mJ pulse energies.[21]   Other researchers studied the ability of the Thulium laser to resect prostate tissue.[22]  Xia[22] performed a randomized prospective trial comparing Thulium laser prostatectomy with transurethral prostatectomy, and found equal efficacy of the two techniques and decreased catheterization time, blood loss and hospital stay, in addition to shorter learning curve in favor of the Thulium laser.

One heretofore unmentioned technical challenge we encountered during this study was maintaining optimal visualization during the delivery of irrigation to the laser fiber intraoperatively. The 400 micron laser fiber requires continuous irrigation to diminish debris and char formation on the fiber.  Controversy exists as to the limits of renal injury caused by warm ischemia. Some researchers have shown that limited warm ischemia < 20-30 minutes is transient and irreversible [23], while recent studies have demonstrated that any amount of ischemia has adverse effects on the kidney over time [24]; and efforts to minimize (or eliminate) warm ischemia should be performed. While performing a partial nephrectomy with an unclamped hilum leads to zero ischemia, it can often produce significant bleeding, leading to poor visualization and rendering precise renal surgery difficult. Application of laser techniques to control bleeding was able to allow resection and completion of the partial nephrectomy with zero ischemia during our case series.

CONCLUSIONS

We present a technique of laser tissue welding during robotic laparoscopic partial nephrectomy which provided reliable hemostasis without the need to clamp hilar vessels. Performance of the surgery without having to clamp the renal vessels resulted in zero ischemia time and therefore optimal preservation of renal function. In addition to successfully controlling bleeding (without hilar clamping), this technique protected the underlying parenchyma from the deleterious effect of coagulation. Furthermore, pathologic evaluation showed preservation of the renal parenchyma immediately below the laser incision with preserved ability to identify staging, grading and margin status. This technique demonstrates that the Thulium laser probe can be used for both cutting renal parenchyma while providing adequate hemostasis.

This new approach provides an exciting option that may be applied in selected cases of laparoscopic or robotic partial nephrectomies. Further studies need to be conducted to identify if this technique can be applied to larger and endophytic renal tumors.

REFERENCES

  1. Gettman MT, Lotan Y, Lindberg G, Napper CA, Hoopman J, Pearle MS, et al. Laparoscopic interstitial laser coagulation of renal tissue with and without hilar occlusion in the porcine model. J Endourol. 2002 Oct;16(8):565-70.
  2. Ogan K, Jacomides L, Saboorian H, Koeneman K, Li Y, Napper C, et al. Sutureless laparoscopic heminephrectomy using laser tissue soldering. J Endourol. 2003 Jun;17(5):295-300.
  3. Lotfi MA, McCue P, Gomella LG. Laparoscopic interstitial contact laser ablation of renal lesions: an experimental model. J Endourol. 1994 Apr;8(2):153-6.
  4. Janetschek G, Daffner P, Peschel R, Bartsch G. Laparoscopic nephron sparing surgery for small renal cell carcinoma. J Urol. 1998 Apr;159(4):1152-5.
  5. Gruschwitz T, Stein R, Schubert J, Wunderlich H. Laser-supported partial nephrectomy for renal cell carcinoma. Urology. 2008 Feb;71(2):334-6.
  6. Dasgupta P FJ, Fitzpatrick JM, Kirby R, Gill I. Lasers in Laparoscopic Surgery.  New Technologies in Urology. London: Springer; 2010. p. 68.
  7. Campbell SC, Novick AC, Belldegrun A, Blute ML, Chow GK, Derweesh IH, et al. Guideline for management of the clinical T1 renal mass. J Urol. 2009 Oct;182(4):1271-9.
  8. Benway BM, Wang AJ, Cabello JM, Bhayani SB. Robotic partial nephrectomy with sliding-clip renorrhaphy: technique and outcomes. Eur Urol. 2009 Mar;55(3):592-9.
  9. Clark MA, Shikanov S, Raman JD, Smith B, Kaag M, Russo P, et al. Chronic kidney disease before and after partial nephrectomy. J Urol. 2011 Jan;185(1):43-8.
  10. Gill IS, Eisenberg MS, Aron M, Berger A, Ukimura O, Patil MB, et al. “Zero ischemia” partial nephrectomy: novel laparoscopic and robotic technique. Eur Urol. 2011 Jan;59(1):128-34.
  11. Hughes BF, Scott WW. Preliminary report on the use of a CO 2 laser surgical unit in animals. Invest Urol. 1972 Jan;9(4):353-7.
  12. Malloy TR, Schultz RE, Wein AJ, Carpiniello VL. Renal preservation utilizing neodymium:YAG laser. Urology. 1986 Feb;27(2):99-103.
  13. Johnson DE, Cromeens DM, Price RE. Use of the holmium:YAG laser in urology. Lasers Surg Med. 1992;12(4):353-63.
  14. Lotan Y, Gettman MT, Ogan K, Baker LA, Cadeddu JA. Clinical use of the holmium: YAG laser in laparoscopic partial nephrectomy. J Endourol. 2002 Jun;16(5):289-92.
  15. Moinzadeh A, Gill IS, Rubenstein M, Ukimura O, Aron M, Spaliviero M, et al. Potassium-titanyl-phosphate laser laparoscopic partial nephrectomy without hilar clamping in the survival calf model. J Urol. 2005 Sep;174(3):1110-4.
  16. Hindley RG, Barber NJ, Walsh K, Petersen A, Poulsen J, Muir GH. Laparoscopic partial nephrectomy using the potassium titanyl phosphate laser in a porcine model. Urology. 2006 May;67(5):1079-83.
  17. Liu M, Rajbabu K, Zhu G, Petersen A, Muir GH, Poulson J. Laparoscopic partial nephrectomy with saline-irrigated KTP laser in a porcine model. J Endourol. 2006 Dec;20(12):1096-100.
  18. Anderson JK, Baker MR, Lindberg G, Cadeddu JA. Large-volume laparoscopic partial nephrectomy using the potassium-titanyl-phosphate (KTP) laser in a survival porcine model. Eur Urol. 2007 Mar;51(3):749-54.
  19. Bui MH, Breda A, Gui D, Said J, Schulam P. Less smoke and minimal tissue carbonization using a thulium laser for laparoscopic partial nephrectomy without hilar clamping in a porcine model. J Endourol. 2007 Sep;21(9):1107-11.
  20. Boylu U, Oommen M, Joshi V, Thomas R, and Lee BR.  Natural Orifice Transluminal Endoscopic Surgery (NOTES) Partial Nephrectomy in the Porcine Model.  Surg Endosc. 2010 Feb;24(2):485-9
  21. Blackmon RL, Irby PB, Fried NM. Holmium:YAG (lambda = 2,120 nm) versus thulium fiber (lambda = 1,908 nm) laser lithotripsy. Lasers Surg Med. 2010 Mar;42(3):232-6
  22. Xia SJ, Zhuo J, Sun XW, Thulium laser versus standard transurethral resection of the prostate: A randomized prospective trial. Eur Urol 2008;53:382-90
  23. Novick AC. Renal hypothermia: in vivo and ex vivo. Urol Clin NorthAm 1983;10:637–44.
  24. Thompson RH, Lane BR, Lohse CM, et al. Every minute counts when the renal hilum is clamped during partial nephrectomy. Eur Urol 2010;58:340–5.

Acknowledgement:  Photographs in Image 1 were supplied by Tulane Department of Pathology ( Dr. Sharon Hirsh)

Date added to bjui.org: 05/01/2012

DOI: 10.1002/BJUIw-2011-064-web

 

Bilateral massive renal infarction as manifestation of endocarditis

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Here we present a young female patient who presented with intense lumbar pain, which investigation revealed to be due to bilateral renal infarction. 

 

Authors: Torricelli, Fabio; Guglielmetti, Giuliano; Abe, Daniel; Mesquita, Jose Luis; Srougi, Miguel   University of Sao Paulo Medical School, Urology, Av Dr Eneas Carvalho de Aguiar, 255, 7th floor, Central Institute, Sao Paulo, Brazi

Corresponding Author: Fabio Torricelli, University of Sao Paulo Medical School, Urology, Av Dr Eneas Carvalho de Aguiar, 255, 7th floor, Central Institute, Sao Paulo, Brazil. Email: [email protected]

Introduction
 
Lumbar pain is usually a benign condition related to posture or physical strain, and it generally resolves spontaneously. However, more serious diseases may present as low-back pain and misdiagnosis may change patient evolution and survival.
Acute renal infarction is rarely detected in clinical practice, because it is uncommon and has no specific clinical presentation. There are neither risk factors nor laboratory examinations that have a clinical specificity for acute renal infarction, although most patients have a medical history associated with a high risk of thromboembolism and elevated serum levels of lactate dehydrogenase and/or haematuria within 24 hours after the onset of pain[1]. Computed tomography is mandatory for early diagnosis.
Here we present a young female patient who presented with intense lumbar pain, which investigation revealed to be due to bilateral renal infarction. Further investigation showed endocarditis as the  cause of thomboembolism and renal damage.

 

Case report
 
A 24-year-old female presented at the emergency room (ER) with sudden onset of intense right lumbar pain, suggestive of renal colic. She had a history of diffuse abdominal pain for one month, associated with asthenia and weight loss (9 kg). She had no history of fever, haematuria, dysuria, frequency or urgency. In childhood she had suffered from rheumatic fever and was taking benzathine penicillin once every 21 days, but had stopped taking her medication within the previous 3 months. Microscopic haematuria and severe proteinuria were identified on urinalysis. An abdominal ultrasound was normal, except for a mild increase in renal echogenicity. CT of the abdomen was therefore performed and revealed a massive right renal infarct, a small left renal infarct, and a small splenic infarct (figure 1).

 

Figure 1 – Abdominal CT showing a massive right, and a small left, renal infarct

 

 

Three sets of blood cultures all  isolated Streptococcus oralis,  and echocardiography demonstrated endocarditis with vegetations on her aortic valve (figure 2).

 

Figure 2 – Echocardiography revealing vegetations on the aortic valve AE – left atrium, VE – left ventricle, AO – Aorta, VEG – vegetation 

 

 

The patient also suffered an epileptic fit, and therefore  underwent a CT of her head. This revealed a small cerebral abscess, 1.3 cm in diameter, which was treated with intravenous antibiotics with no further complication.
The patient received intravenous gentamicin and penicillin for 6 weeks with good effect. She did not develop any intracerebral complications or impairment in her renal function. She did not receive any anticoagulants. After an odontologic evaluation, three of her teeth were removed because of infection and the others were treated with a professional dental whitening system. A follow up abdominal CT was performed, and showed an atrophic right kidney with residual function and a small scar on her left kidney.

 

Discussion
 
Massive renal infarction associated with endocarditis is a rarely diagnosed condition. Risk factors for  thromboembolism and subsequent acute renal infarction, include atrial fibrillation, previous embolism, and valvular or ischemic heart disease. However, it has also been associated with other conditions, including trauma, hereditary and acquired clotting disorders, cocaine use, blood vessel anomalies, hereditary diseases such as Marfan’s syndrome, renal transplantation, undergoing endovascular catheterization, and malignant disease1. Contrast-enhanced CT is the noninvasive standard of reference for imaging acute renal infarction [1].
Dursun et al [2] reported a case of a previously healthy 37 year-old man with bilateral renal infarction due to fibromuscular dysplasia that was successful treated by placement of a  stent into the stenotic right renal artery. Ronco F et al [3] reported an unusual case of paradoxical embolism through a patent foramen ovale as a cause of renal infarction. Nakayama et al [4] reported a 46 year-old woman with the sudden onset of severe right sided pain due to right renal infarction. Investigation with cardiac ultrasonography revealed a vegetation on the posterior cusp of her mitral valve, and the renal infarction was associated with renal artery embolism from infective endocarditis. This patient was surgically treated by mitral annuloplasty, because the vegetation was still present after antimicrobial therapy. In our case, antibiotic therapy was enough to control the endocarditis, however massive renal infarction was already established at the time of diagnosis.
Patients with the sudden onset of lumbar pain, absence of hydronephrosis, and with risk factors for thromboembolism must be suspected of having a renal infarct. A contrast enhanced CT is mandatory in this situation. Furthermore, these patients also commonly present with haematuria and elevated serum lactate dehydrogenase on testing.

 

References
 
1. de la Iglesia F, Asensio P, Díaz A, Darriba M, Nicolás R, Diz-Lois F. Acute renal infarction as a cause of low-back pain. South Med J. 2003;96(5):497-9.
2. Dursun B, Yagci B, Batmazoglu M, Demiray G. Bilateral renal infarctions complicating fibromuscular dysplasia of renal arteries in a young male. Scand J Urol Nephrol. 2011 Jun 1.
3. Ronco F, Rigatelli G, Dell’Avvocata F, Giordan M, Ronco C, Cardaioli P. Embolic renal infarct, patent foramen ovale and coronary artery dissection: a strange case of cardio-renal connection. Cardiovasc Revasc Med. 2011;12(1):67.e5-7.
4. Eknoyan G, Lister BJ, Kim HS, Greenberg SD. Renal complications of bacterial endocarditis. Am J Nephrol. 1985;5(6):457-69.

Date added to bjui.org: 12/12/2011


DOI: 10.1002/BJUIw-2011-068-web

Right sided Bochdalek hernia causing ureteric obstruction

/by

We report a case in a elderly female who presented with symptomatic BH causing obstruction of the right renal pelvis and ureter.

 

Authors: Vikram Balakrishnan, MBBS, BMedSc, Dip. Surg Anatomy; Gregory Neerhut MBBS, FRACS.

Urology Unit, Geelong Hospital, Victoria, Australia

 
Corresponding Author: Vikram Balakrishnan, Urology Unit, Geelong Hospital, Victoria, Australia.  T: +613 421 709 541  Email: [email protected]

 

Introduction
 
Bochdalek hernia (BH) is a congenital hernia of the diaphragm that occurs in 1 in every 2200 to 12,500 births with an overall prevalence in adults of between 0.17% and 10.5% [1-4]. It results from developmental failure of the posterolateral diaphragmatic foramina to fuse properly and most commonly presents during the neonatal period [5]. Adult patients presenting with symptomatic BH, especially on the right side, are exceedingly rare, and there have been less than 20 cases reported in the literature [6,7]. We report a case in a elderly female who presented with symptomatic BH causing obstruction of the right renal pelvis and ureter.

 

Case Report
An 83 year old female presented to the emergency department with severe right flank pain on a background of a 12 month history of intermittent mild flank pain. Her past history included chronic renal impairment and right renal colic. Clinical examination revealed a frail elderly lady with normal vital signs. There was some mild flank tenderness on the right and respiratory examination was normal. Creatinine was chronically elevated at 192umol/L, eGFR was 23mL/min, white cell count was 12.0 10^9g/L (reference rage 4-11.0 x10^9 g/L) and the urine was sterile. Plain radiographs of the abdomen demonstrated degenerative changes of the lumbar spine consistent with osteoarthritis and there was no evidence of kidney stones. The patient was discharged with a presumptive diagnosis of chronic back pain.
At outpatient follow-up, the patient had ongoing flank pain and ultrasound demonstrated normally sized and positioned kidneys with hydronephrosis of the right collecting system and a renal pelvis measuring 6cm in diameter. A computerised tomography (CT) scan demonstrated a small BH of the posterior aspect of the right diaphragm. The dilated renal pelvis was drawn superiorly through the hernia into the thorax with the pelviureteric junction located superior to the remainder of the collecting system. The ureter then passed inferiorly through the neck of the Bochdalek hernia back into the abdomen. There was no dilatation of the ureter below the hernia (Figure 1 and 2).
 

Figure 1. Axial CT scan demonstrates dilated right renal pelvis drawn superiorly through the diaphragmatic hernia

 

 

Figure 2. Coronal CT scan demonstrates distended ureter and renal pelvis above the neck of the Bochdalek hernia and normal calibre ureter below

 

 

Rigid cystoscopy and a right retrograde pyelogram demonstrated superior and medial deviation of the proximal ureter with kinking of the ureter and proximal hydronephrosis (Figure 3).
 

Figure 3. Retrograde pyelogram pre and post double J stent placement

 

 

A guide-wire was passed to the renal pelvis without difficulty and a 6 French double J stent was placed. The patient’s pain resolved immediately, she made an uneventful recovery and was discharged home the following day. The stent is changed electively every six months and 12 months following presentation she remains pain free.

 

Discussion
Congenital diaphragmatic hernia was first described by Vincent Alexander Bochdalek in 1848 and results from incomplete closure between the pars lumbaris and pars costalis parts of the diaphragm during fetal development [5,8]. The majority of patients present during neonatal life with concomitant congenital pulmonary abnormalities and consequently have a poor prognosis [1]. Adult presentation with symptomatic BH is rare with approximately 100 cases reported in the literature. Whilst asymptomatic BH tend to occur equally on the right and left hand sides of the diaphragm [3,4], symptomatic BH tend to occur on the left with a ratio of 9:1 [1,2]. The contents of BH depend on the side of the hernia and have included liver, omentum, bowel, spleen, kidneys and stomach [1,10]. The presence of renal pelvis or ureter within a BH is exceedingly rare with only three cases reported in the literature [11-13].
The clinical presentation of BH in adults ranges from an incidental finding on imaging to strangulation of intra-abdominal contents with significant morbidity and mortality. Symptoms are often non-specific and include abdominal pain, dyspnea, chest pain and nausea and vomiting [10]. Of the three cases reported in the literature on BH containing ureter, one was asymptomatic and was discovered incidentally [11], one presented with right upper quadrant pain [12] and the other presented almost identically to our case with a 12 month history of intermittent flank pain [13]. Precipitating factors are sometimes identified and include any mechanism that increases intra-abdominal pressure, however, the patient in our study did not have any history of chronic cough or constipation.
Diagnosis is ultimately via imaging and CT provides the most accurate and reliable method [2,14]. Discontinuity of the posterior diaphragm is usually clearly identified and protrusion of intra-abdominal contents including ureter is clear [2,5,14]. Importantly, CT can identify associated complications of ureteric herniation including hydronephrosis and hydroureter, which is evidenced by dilatation above the hernia neck and a normal calibre ureter below. Of the three reported cases on BH containing ureter, two were associated with obstructive uropathy with similar CT findings to our case [12,13]. Ultrasound, whilst able to identify hydronephrosis in our case, was not able to identify the hernia or the site of obstruction.
The optimal treatment of BH causing obstructive uropathy is unclear. Despite kinking of the ureter we were able to successfully pass a double J stent to the renal pelvis which provided immediate relief of the patient’s symptoms. Similarly, Song et al [14] were able to successfully pass a retrograde stent with follow-up CT confirming resolution of hydronephrosis. Definitive surgical correction of BH is recommended because of the risk of herniation and strangulation of abdominal viscera and excellent outcomes have been described with no evidence of hernia recurrence [10]. In particular, laparoscopic repair with or without mesh appears to be reliable and confers all the benefits of minimally invasive surgery [15]. However, diaphragmatic surgery is not without complications and elderly patients in particular gain only a minimal benefit as the risk of further herniation over their remaining lifetime is low.
Our elderly frail patient was managed with a retrograde ureteric stent. Patients managed this way need to have their stent changed every 6 to 12 months because of the risk of stent encrustation causing obstruction. In addition, these patients should have regular imaging such as chest radiography because, as discussed, clinical symptoms and signs may not correlate with severity of disease. In a younger fitter patient we would recommend surgical correction of the hernia.

 

Conclusion
 
We described the case of an 83 year old female presenting with a right sided BH containing the right renal pelvis and ureter causing pain, hydronephrosis and hydroureter. Symptomatic BH in adult patients is rare and there have been only three cases of BH containing ureter in the literature. Ureteric stent offers a safer alternative to surgical repair of the hernia and may be a more appropriate treatment in elderly patients.

 

References
1. Gedik E, Tuncer MC, Onat S, Avci A, Tacyildiz I, Bac B. A review of Morgagni and Bochdalek hernias in adults. Folia Morphol (Warsz). 2011 Feb;70(1):5-12.
2. Wilbur AC, Gorodetsky A, Hibbeln JF. Imaging findings of adult Bochdalek hernias. Clin Imaging. 1994 Jul-Sep;18(3):224-9.
3. Mullins ME, Stein J, Saini SS, Mueller PR. Prevalence of incidental Bochdalek’s hernia in a large adult population. AJR Am J Roentgenol. 2001 Aug;177(2):363-6.
4. Temizöz O, Gençhellaç H, Yekeler E, Umit H, Unlü E, Ozdemir H, Demir MK. Prevalence and MDCT characteristics of asymptomatic Bochdalek hernia in adult population. Diagn Interv Radiol. 2010 Mar;16(1):52-5. Epub 2009 Dec 28.
5. Sandstrom CK, Stern EJ. Diaphragmatic hernias: a spectrum of radiographic appearances. Curr Probl Diagn Radiol. 2011 May-Jun;40(3):95-115.
6. Rout S, Foo FJ, Hayden JD, Guthrie A, Smith AM. Right-sided Bochdalek hernia obstructing in an adult: case report and review of the literature. Hernia. 2007 Aug;11(4):359-62. Epub 2007 Mar 7.
7. Agrafiotis AC, Kotzampassakis N, Boudaka W. Complicated right-sided Bochdalek hernia in an adult. Acta Chir Belg. 2011 May-Jun;111(3):171-3.
8. Haller JA Jr. Professor Bochdalek and his hernia: then and now. Prog Pediatr Surg. 1986;20:252-5.
9. Bujanda L, Larrucea I, Ramos F, Muñoz C, Sánchez A, Fernández I. Bochdalek’s hernia in adults. J Clin Gastroenterol. 2001 Feb;32(2):155-7.
10. Brown SR, Horton JD, Trivette E, Hofmann LJ, Johnson JM. Bochdalek hernia in the adult: demographics, presentation, and surgical management. Hernia. 2011 Feb;15(1):23-30. Epub 2010 Jul 8.
11. Chawla K, Mond DJ. Progressive Bochdalek hernia with unusual ureteral herniation. Comput Med Imaging Graph. 1994 Jan-Feb;18(1):53-8.
12. Song YS, Hassani C, Nardi PM. Bochdalek hernia with obstructive uropathy. Urology. 2011 Jun;77(6):1338. Epub 2010 Jul 1.
13. Paterson IS, Lupton EW. Pelviureteric junction obstruction secondary to renal pelvic incarceration in a congenital diaphragmatic hernia. Br J Urol. 1989 Nov;64(5):548-9.
14. Shin MS, Mulligan SA, Baxley WA, Ho KJ. Bochdalek hernia of diaphragm in the adult. Diagnosis by computed tomography. Chest. 1987 Dec;92(6):1098-101.
15. Palanivelu C, Rangarajan M, Rajapandian S, Amar V, Parthasarathi R. Laparoscopic repair of adult diaphragmatic hernias and eventration with primary sutured closure and prosthetic reinforcement: a retrospective study. Surg Endosc. 2009 May;23(5):978-85. Epub 2009 Mar 14.

 

Date added to bjui.org: 03/12/2011


DOI: 10.1002/BJUIw-2011-104-web
 

Blind-ending complete ureteric duplication with reflux and ectopia

/by

We report a rare case of complete blind-ending refluxing ectopic ureteric duplication.

 

Authors: Dr. Abdelmoniem  Hassan  Koko FRCS(Ed), Dr. Khaled Ezzeddin  MD,PhD, Dr.Hussien Al Ghahtani FACHARTS, Dr. Khaled Al Gammal MD

Armed Forces Hospital, King Abduaziz Air Base ,Dhahran
P.O.BOX 4192, Al Khobar, 31952
Saudi Arabia

Corresponding Author: Dr. Abdelmoniem  Hassan  Koko FRCS(Ed), Armed Forces Hospital, King Abduaziz Air Base, Dhahran P.O.BOX 4192, Al Khobar, 31952 Saudi Arabia.  E-mail: [email protected]

 

Introduction
Blind-ending ureteric duplication is a rare developmental anomaly of the ureter. Embryologically, it was postulated that the affected ureteric bud is abortive and fails to make contact with the mesonephros. Histologically, it contains all the ureteric layers; it tends to have bulbous dilatation (1). Sometimes they are short without reflux and are minimally symptomatic. However, patients with long blind-ending duplications can present with severe infection and reflux, requiring extensive surgery (2). Here we report a rare case of complete blind-ending refluxing ectopic ureteric duplication.

 

Case report
A 20 year old female was admitted to the hospital with right sided abdominal pain, lower urinary tract symptoms and fever. She gave a history of recurrent febrile urinary tract infections since childhood, with multiple hospital admissions.  On examination she was febrile, and there was tenderness and guarding on the right side of the abdomen. Urine culture grew E coli and renal profile was normal. Ultrasound showed mild dilatation of the right pelvicalyceal system, with a cystic area above and medial to the upper pole of the right kidney, and a cystic area on the right side of the pelvis, representing  the dilated right ureter (Fig 1).

 

Fig 1. Ultrasound showed mild clayceal dilatation and cystic mass at the medial aspect of the upper pole of the right kidney.

 

 

Intravenous pyelography showed right sided calyectasia; the renal pelvis was not dilated and there was lateral displacement of the right kidney. The left kidney and urinary bladder were normal (Fig 2).

 

Fig 2. IVU showing lateral displacement of the right kidney, calyceal dilatation, renal pelvis and ureter not dilated.

 

 

After controlling the infection, she was scheduled for micturating cystourethrogram (MCUG). She developed severe intolerable pain when the Foley’s catheter was inserted. The radiologist found the catheter in the right ureter so it was removed immediately and the procedure was aborted. Indirect radioisotope MCUG demonstrated right sided vesicoureteric reflux (Fig 3).

 

Fig 3. Indirect micturating cystourethrogram with radioactive tracer in the dilated distal right ureter indicating vesicoureteric reflux.

 

 

She was scheduled for cystography and cystoscopy under anaesthesia. The Foley’s catheter was found again in the right ureter, which was dilated, tortuous and blind-ending (Fig 4).

 

Fig 4A. Preferential passage of the urethral catheter into the right ureter.

 

 

Fig 4B. Retrograde study showing blind ending tortuous dilated ureter as well as the collecting system on the right side.

 

 

Fig 4C, D. CT scan with  contrast material through ureteric catheter showing the blind ending duplication, and it’s relation to the right kidney.

 

 

Urethroscopy showed the opening of the ectopic right ureter just below the bladder neck (Fig 5).

 

Fig 5. Urethroscopy with guide wire in the ectopic ureteric orifice in the urethra.

 

 

Surgical exploration and excision of the blind ending right ureter was done (Figs 6A, B, C).

 

Fig 6. Duplication of the ureters on the right side.

 

 

Fig 6B. Upper pole right kidney after complete excision of the blind ending ureter.

 

 

Fig 6C. Surgical specimen.

 

 

The post operative recovery and subsequent follow up for four years was uneventful.

 

Discussion
Blind ending ureteric duplication is a rare anomaly of the ureter. Most of the cases reported were involving one limb of a bifid system; it is more common in females than in males, and present twice as often on the right side. The majority of the identified cases are diagnosed in the third or fourth decade of life. Embryologically, it was postulated that the affected ureteric bud is abortive and fails to make contact with the mesonephros. Histologically, it contains all the ureteral layers; it shares a common sheath and blood supply with the normal ureter (1). Our patient had recurrent severe febrile urinary tract infections, which led to frequent hospitalizations.
The only reported similar case found in the English literature described ureteric ectopia with preferential passage of the Foley’s catheter into an ectopic blind- ending ureter in the prostatic urethra, in a 67 year old man (3).
Surgical dissection of the lower segment of the blind ending ureter may compromise the vascularisation of the remaining ureter on that side.  This can be prevented by conserving the common ureteric sheath or re-implanting the normal ureter.  Since the ensheathment is less dense at the proximal end, the dissection should start there and care is taken not to denude or injure the normal ureter (1, 4, and 5). Careful and systematic steps must be adhered to in diagnosis and management of children presenting with febrile urinary tract infections. If the correct diagnosis was made initially, our patient could have been saved from unnecessary morbidity.

 

Conclusions
Blind-ending complete ureteral duplication is a rare developmental anomaly of the ureter. Careful and systematic steps must be followed in diagnosis and management of children presenting with febrile urinary tract infections.

 

References
 
1. Schulussel RN, Retik AB. Ectopic ureter, ureterocele, and other anomalies of the ureter. Chapter 58. Walsh PC (ed). Campbell’s Urology, 8th Edition. Philadelphia: Saunders; 2002; 2007 – 52.
2. Marshall FF, McLoughlin MG. Long blind ending ureteral duplications. J Urol. 1978; 120(5): 626-630.
3. Chai TC, Ohl DA. Difficult urethral catherterization due to ectopic ureter: an unusual presentation of ureteral ectopia in a man. J Urol. 1995; 153(6): 1899-900.
4. V.DE BOE, J. BRAECKMAN and F. KEUPPENS, A rare duplication anomaly of the upper urinary tract: a blind-ending uretic duplication with ectopic and refluxing ureterocele  BJU International (1999). 84(1): 173 – 174.
5. Rege VM, Deshumukh SS, Borwankar SS, Gandhi RK. Blind ending bifid ureter (a case report).
J Postgrad Med 1983; 32(4): 233-5.

 

Date added to bjui.org: 27/11/2011


DOI: 10.1002/BJUIw-2010-083-web

 

Intravesical herniation of small bowel: a very rare complication of bladder perforation

/by

We describe a case of bladder injury following abdominal hysterectomy.

 

Authors: Twemlow MRP1, Narava S2, Ali T1, Graham JY1, Hilton P2

1. Department of Radiology, Royal Victoria Infirmary, Newcastle upon Tyne, UK
2. Directorate of Women’s Services, Royal Victoria Infirmary, Newcastle upon Tyne, UK

 
Corresponding Author: Twemlow MRP, Department of Radiology, Royal Victoria Infirmary, Newcastle upon Tyne, UK. E-mail: [email protected]

 

Abstract
Internal hernias are rare with only a handful of cases of intravesical herniation of small bowel described in the literature. We describe a case of bladder injury following abdominal hysterectomy. Presentation was delayed as small bowel had herniated into the bladder sealing the defect and preventing any urine leak. This rare complication of pelvic surgery was suspected by an excretory phase CT scan with the findings confirmed at laparotomy,after the patient re-presented with ureteric obstruction. This complication could present with signs of small bowel obstruction, small bowel ischaemia, ureteric obstruction, haematuria or urine retention.

 

Introduction
Internal herniation occurs when a viscus, usually small bowel, herniates through a congenital or acquired defect within the peritoneal cavity. Internal hernias are a rare cause of small bowel obstruction, with a reported incidence of less than 0.9%. [1] Viscus to viscus herniation is an unusual form of internal hernia. Less than a handful of cases of small bowel herniation through silent defects in the wall of the urinary bladder have been reported in the literature, making it one of the rarest forms of internal hernia. We report a case of a female presenting with some features of small bowel obstruction and left ureteric obstruction secondary to intravesical herniation of small bowel following recent gynaecological pelvic surgery.

 

Case report
A 49 year old woman with a fibroid uterus underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy for symptoms of pelvic pain and pressure. A preoperative ultrasound confirmed the presence of multiple fibroids in the uterus. Intraoperatively a 16-week size uterus with relatively normal ovaries and endometriosis in the pouch of Douglas was encountered. The anterior vaginal vault was vascular, and required oversewing with cauterisation to venous bleeders. She was discharged from hospital on the third post-operative day.
She was re-admitted on the fifth postoperative day with persistent nausea, dehydration and dysuria. She was treated with intravenous antibiotics for presumed urinary tract infection. Ultrasound of her abdomen demonstrated mild left hydronephrosis and a small volume ascites in the pelvis with several loops of associated aperistaltic small bowel (Figure 1).

 

Figure 1. Abdomino-pelvic ultrasound images showing aperistalic small bowel loops in the pelvis with surrounding fluid (markers).

 

 

A subsequent CT scan of the abdomen and pelvis was performed following intravenous administration of iodinated contrast (Figure 2).

 

Figure 2. Axial CT scan of the abdomen and pelvis in the portal venous phase following intravenous administration of iodinated contrast demonstrating a thick-walled oedematous loop of small bowel in the pelvis with surrounding free fluid.

 

 

This demonstrated mild left sided hydronephrosis and hydroureter down to the pelvis. There was a thick-walled oedematous loop of small bowel in the pelvis with some surrounding fluid. The afferent and efferent loops of bowel and associated mesenteric vessels appeared to be confluent posterior to the abnormal loop of bowel raising the possibility of an internal hernia. The bladder could not be identified separately.
A delayed excretory phase CT scan was subsequently performed (Figure 3).  This demonstrated that small bowel had herniated into the bladder forming an acute angle at the site of the bladder defect. There was no demonstrable associated urine leak. Contrast had not opacified the distal left ureter.

 

Figure 3. CT scan of the pelvis in the excretory (delayed) phase (a) scout image demonstrating a large intravesical filling defect; (b) axial image demonstrating intravesical herniation of small bowel with no apparent extra-vasation of contrast; this was confirmed on coronal reformats (c).

 

(a)
(b)

 


 

(c)

 
Consequently the patient was scheduled for emergency cystoscopy and laparotomy.

 

Cystoscopy demonstrated a large defect in the posterior aspect of the bladder with necrotic edges; left ureteric obstruction was confirmed 2cm proximal to the ureterovesical junction. (Figure 4)

 

Figure 4. Intraoperative images (a) cystoscopy – demonstrating normal bladder wall on the left of the image and herniated small bowel loop on the right; (b) bladder anteriorly (under the retractor) with Babcock tissue forceps on the necrotic edge, just above the herniated bowel segment.

 

(a)
(b)

 

 

At laparotomy the loop of small bowel which herniated through the bladder was confirmed to be viable and no bowel resection was necessary; the left ureter was re-implanted, and the bladder repaired after excision of the necrotic edges.  Bilateral ureteric stents were left in place postoperatively, and were brought through the anterior abdominal wall across the bladder.
The patient had an uncomplicated recovery following the procedure.  Retrograde pyelogram via the stents ten days after the procedure showed a non-dilated left ureter and renal pelvis with no evidence of leak around the left ureteric re-implantation site. At two months follow-up she was free of symptoms and had a normal isotope renogram with good concentration and drainage bilaterally, with a functional split of left 47%: right 53%; she was discharged from the clinic at that stage.

 

Discussion
Intraperitoneal bladder injury is a known complication of pelvic and gynaecological surgery.[2]  Traumatic and spontaneous bladder ruptures are also recognised entities. In intoxicated patients, bladder injuries can result from minor abdominal trauma with no associated bony injury.[3]  The resulting aperture in the bladder wall presents a potential risk for internal herniation of bowel. The probability of occurrence is clearly very low, with only 5 cases reported in the international literature.[4,5,6] This is most likely attributed to the usually florid presentation of bladder rupture with signs of peritonitis and haematuria.  In the rare occasion of a loop of bowel completely or partially sealing the defect minimizing urine leak, this may result in reducing or delaying of the signs of peritonitis.  If the bladder defect is small, narrow neck herniation can result with the added risk of small bowel obstruction and strangulation.
In our case, the bladder rupture was thought to be iatrogenic following recent abdominal hysterectomy. In previous reported cases, the causations for bladder injury also included alcohol-associated bladder injury and spontaneous rupture following radiotherapy. [4,5,6]
In the limited number of reported cases, patients have presented with symptoms of haematuria or inability to void. Abdominal pain was also a shared feature. Our case and the case described by Yalla et al shared some features of strangulation and small bowel obstruction.[6] We described in addition coexistence of symptoms of flank pain related to left sided distal ureteric obstruction and associated hydronephrosis. In the immediate acute phase features of internal bleeding and hypovolemia have also been reported.[5]
The diagnosis of intravesical herniation could be made on a number of modalities.[4,5,6] We advocate CT as the most useful tool; and in today’s practice it is more likely to be the first line examination in the symptomatic patient.  The likely findings are those of internal herniation, with a cluster of bowel loops or a U-shaped solitary loop. This can be associated with prominent, engorged mesenteric vessels which along with the efferent and afferent ends of the herniated loop converge to the point of herniation. If the bowel lumen was sufficiently compressed, features of small bowel obstruction could also be present.[7] If the blood supply drops below the critical threshold, radiological signs of bowel ischemia could manifest.
The diagnosis is made by delineating the relationship between the herniated lower abdominal loop of bowel and the urinary bladder. The bladder might be underfilled as in our case which adds to the diagnostic difficulties. Coexistence of urinary retention or unexplained ureteric obstruction should raise the suspicion, especially where a normal-shaped bladder can not be fully identified.
From our experience we recommend the use of delayed excretory phase CT images through the pelvis with clamping of the urinary catheter if present. This will opacify the bladder and would help identification of the acute angle between the herniated bowel and the bladder wall which is in keeping with an intravesical lesion rather than external compression. This will also help confirm the site of ureteric obstruction – if present – to be the vesicoureteric junction. Extravasation of opacified urine can also be demonstrated.
Ultrasound may also have a role in diagnosis. The interpreter should be aware of the existence of this rare complication to aid diagnosis. Our patient had an ultrasound one day prior to her CT which correctly identified the adynamic loops of small bowel surrounded by fluid. The operator then failed to identify this fluid to be intravesical.
Cystography has also successfully shown the herniated bowel as intravesical filling defects, but usually requires the aid of CT or cystoscopy to delineate the true nature of the mass lesion.[5]
All cases of diagnosed intravesical herniation will require surgical reduction and repair to eliminate the risk of bowel obstruction, strangulation and the ongoing potential risk of urine leakage with urinary peritonitis and possible fistula formation.

 

Conclusion
Intravesical herniation of bowel is a rare but recognised complication of bladder perforation. Presentation may be delayed when a small defect in the bladder is sealed by small bowel, preventing a urine leak.  Clinical suspicion should be raised in patients with haematuria, urine retention or ureteric obstruction. Excretory phase pelvic CT has been shown to be a very useful diagnostic tool.

 

References
1. Passas V, Karavias D, Grilias D, Birbas A. Computed tomography of left paraduodenal hernia. J Comput Assist Tomogr 1986 10:542–543.
2. Mathevet P, Valencia P, Cousin C, Mellier G, Dargent D. Operative injuries during vaginal hysterectomy. Euro J Obs & Gynae 2001 97(1): 71-75.
3. Festini G, Greqorutti S, Reina G, Bellis G. Isolated intraperitoneal bladder rupture in patients with alcohol intoxication and minor abdominal trauma. Ann Emerg Med 1991 20(12) 1371-1372.
4. Liegeois F, Thoumas D, Lemercier E, et al. [Spontaneous rupture of the bladder. Apropos of 2 cases with an unusual presentation]. [French] Rupture spontanee de vessie. A propos de deux cas de presentation inhabituelle Journal de Radiologie 1998 79(11):1404-6.
5. Oesterling JE; Goldman SM; Lowe FC. Intravesical herniation of small bowel after bladder perforation.  Journal of Urology 1987 138(5): 1236-8.
6. Yalla SV; Slavick H; Burros HM. Intravesical strangulation of the small bowel. An unusual complication of rupture of urinary bladder. Urology 1973 2(5): 572-3.
7. Balthazar E. CT of small-bowel obstruction. Am J Roentgenol 1994 162: 255-261.

 

Date added to bjui.org: 21/11/2011 


DOI: 10.1002/BJUIw-2011-090-web

 

Non-Hodgkin’s lymphoma presenting as macroscopic haematuria and acute urinary retention in pregnancy

/by

We present the case of a 30 year old primigravida in her third trimester with visible haematuria secondary to a genital-tract lymphoma. 

 

Authors: Mark Sayles1, Sarah E Gull2, James DD Allan1

1. Department of Urology
West Suffolk Hospital
Hardwick Lane
Bury St Edmunds
Suffolk
IP33 2QZ
2. Department of Obstetrics and Gynaecology
West Suffolk Hospital
Hardwick Lane
Bury St Edmunds
Suffolk
IP33 2QZ

 
Corresponding Author: Mark Sayles, Department of Urology, West Suffolk Hospital, Hardwick Lane, Bury St Edmunds, Suffolk. E-mail: [email protected]

 

Abbreviations
MRI  Magnetic Resonance Imaging
R-CHOP  Rituximab-(Cyclophosphamide, Doxorubicin, Vincristine, Prednisolone)
PCR  Polymerase Chain Reaction
FISH   Flourescent In-Situ Hybridisation
DLBCL   Diffuse Large B-Cell Lymphoma

 

Introduction
 
Non-Hodgkin’s lymphoma of the female genital tract during pregnancy is rare. We present the case of a 30 year old primigravida in her third trimester with visible haematuria secondary to a genital-tract lymphoma. We describe the management of this unusual occurrence and review the relevant literature.

 

Case Report
 
A 30 year old primigravida presented at 29 weeks gestation with macroscopic haematuria and intermittent acute urinary retention associated with blood clots. She had no significant past medical history, and the early pregnancy had been largely unremarkable. Three months earlier she had been treated with oral antibiotics for a presumed urinary tract infection, having presented to her general practitioner with haematuria. She was taking iron supplements because of anaemia noted at a routine antenatal check (haemoglobin 7g/dL).
Ultrasound examination revealed bilateral hydronephrosis and a complex solid mass on the posterior bladder wall. She underwent pelvic MRI, and urgent flexible cystoscopy, vaginal examination, and biopsy under general anaesthesia. MRI showed a 10cm x 8cm irregular soft-tissue mass involving the anterior vaginal wall and fornix, the proximal urethra, and the posterior bladder wall (Figure 1).

 

Figure 1. T2-weighted magnetic resonance images of the abdomen and pelvis in coronal (A) and sagittal (B) section. Note the large mass invading into the bladder, and the proximity of tumour to the gravid uterus.

 

 

The tumour extended into the bladder, and the bilateral hydronephrosis seen on ultrasound imaging was due to involvement of both distal ureters by tumour.
Cystoscopy and examination revealed a partially fixed mass, between the anterior vaginal wall and the bladder, which was invading the trigone. Biopsies were taken from the mass in the bladder and from the portion in the vaginal wall. These were submitted for detailed immunohistochemical analysis.
On microscopy the pathological specimens showed a diffuse infiltrate of large atypical lymphoid cells. Immunostaining demonstrated expression of CD20, BCL2, CD30 and CD5, with a proportion of cells expressing MUM-1. PCR analysis detected clonal IgH and IgΚ rearrangements, while FISH showed no evidence of IgH, BCL2, BCL6, or MYC translocation but detected gain of extra copies of each of these gene loci. In summary, the immunohistochemical analyses were consistent with a diagnosis of diffuse large B cell lymphoma (DLBCL).
After multidisciplinary discussion, it was decided to commence R-CHOP chemotherapy. The fetus was delivered at 35 weeks gestation by classical Caesarian section, between the first and second cycles of chemotherapy. Both mother and fetus tolerated the first cycle of chemotherapy well, and a 2.6 kg boy was born at 35 weeks gestation as planned. The boy spent one week in a special care baby unit because of prematurity, but required no specific isolation measures. The patient’s tumour responded well to chemotherapy, and she remains under long-term follow up.

 

Discussion
 
Cancer complicates approximately 1:1000 pregnancies [1]. Lymphoma is the fourth most commonly diagnosed malignancy in pregnancy, complicating approximately 1:6000 deliveries [2]. The majority of lymphomas in women of childbearing age are Hodgkin’s disease, with Non-Hodgkin’s lymphoma (NHL) being much rarer in this age group [2, 3]. When NHL does occur in pregnancy, it tends to be of an aggressive subtype such as DLBCL [4]. In general, primary extranodal disease occurs in 20-30% of lymphoma cases, most frequently involving the gastrointestinal tract or skin [5]. Only 0.5% of extranodal lymphomas originate in the female genital tract [6].
DLBCL is associated with an aggressive natural history; median survival in untreated patients is less than one year [7]. Treatment with a combination chemotherapy regime (Cyclophosphamide, Doxorubicin, Vincristine, and Prednisolone; CHOP) has been standard practice for several decades [8]. The addition of rituximab (a chimeric IgG1κ anti-CD20 monoclonal antibody) to this regime (R-CHOP) significantly reduces the risk of relapse and improves overall survival [9, 10].
Because the diagnosis of cancer during pregnancy is a relatively rare event, experience with the use of chemotherapeutic agents and monoclonal antibodies in pregnancy is limited [1, 2, 11]. Almost all chemotherapeutic agents are known to be teratogenic in animals, and to lead to major malformations or fetal death if administered during the first trimester [12, 13]. Exposure during the second and third trimester is not associated with fetal malformations, but increases the risk of fetal and neonatal death, intrauterine growth retardation, preterm delivery and low birth weight [14]. Clearly, the decision to undergo chemotherapy during pregnancy is complex, and has to balance the risks and benefits to both mother and fetus.
Importantly, there are no preclinical reproductive toxicity data available for rituximab. There are only seven previously documented cases of rituximab being used for lymphoma in pregnancy [15-21]. In the majority of these cases rituximab was administered during the second trimester for an aggressive NHL [15-20]. In one case the patient was taking a maintenance dose of rituximab for relapsing follicular lymphoma during which she conceived unintentionally [21]. The rituximab was stopped and the pregnancy continued to term. Of the seven children exposed to rituximab in utero, three were found to have severely decreased CD19+ B cells as neonates. However, none experienced any significant postnatal infection, and in each case the level of B cells returned to normal within three to six months.

 

Conclusion
 
Rare cancers in pregnancy present diagnostic and therapeutic challenges. The successful outcome in this case required a multidisciplinary approach involving obstetrics and gynaecology, urology, and oncology specialist input. This is the eighth documented case of a fetus being exposed to rituximab, without apparent short term clinically significant effects. However, the long term effects of this exposure are unknown.

 

References
[1] Pentheroudakis G, Pavlidis N. Cancer and pregnancy: poena magna, not anymore. Eur J Cancer. 2006 Jan: 42:126-40
[2] Pavlidis NA. Coexistence of pregnancy and malignancy. Oncologist. 2002: 7:279-87
[3] Ward FT, Weiss RB. Lymphoma and pregnancy. Semin Oncol. 1989 Oct: 16:397-409
[4] Lishner M, Zemlickis D, Sutcliffe SB, Koren G. Non-Hodgkin’s lymphoma and pregnancy. Leuk Lymphoma. 1994 Aug: 14:411-3
[5] Krol AD, le Cessie S, Snijder S, Kluin-Nelemans JC, Kluin PM, Noordijk EM. Primary extranodal non-Hodgkin’s lymphoma (NHL): the impact of alternative definitions tested in the Comprehensive Cancer Centre West population-based NHL registry. Ann Oncol. 2003 Jan: 14:131-9
[6] Lagoo AS, Robboy SJ. Lymphoma of the female genital tract: current status. Int J Gynecol Pathol. 2006 Jan: 25:1-21
[7] Fisher RI, Miller TP, O’Connor OA. Diffuse aggressive lymphoma. Hematology Am Soc Hematol Educ Program. 2004:221-36
[8] Flowers CR, Sinha R, Vose JM. Improving outcomes for patients with diffuse large B-cell lymphoma. CA Cancer J Clin. 2010 Nov-Dec: 60:393-408
[9] Coiffier B, Lepage E, Briere J, et al. CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma. N Engl J Med. 2002 Jan 24: 346:235-42
[10] Pfreundschuh M, Trumper L, Osterborg A, et al. CHOP-like chemotherapy plus rituximab versus CHOP-like chemotherapy alone in young patients with good-prognosis diffuse large-B-cell lymphoma: a randomised controlled trial by the MabThera International Trial (MInT) Group. Lancet Oncol. 2006 May: 7:379-91
[11] Azim HA, Jr., Azim H, Peccatori FA. Treatment of cancer during pregnancy with monoclonal antibodies: a real challenge. Expert Rev Clin Immunol. 2010 Nov: 6:821-6
[12] Cardonick E, Iacobucci A. Use of chemotherapy during human pregnancy. Lancet Oncol. 2004 May: 5:283-91
[13] Leslie KK, Koil C, Rayburn WF. Chemotherapeutic drugs in pregnancy. Obstet Gynecol Clin North Am. 2005 Dec: 32:627-40
[14] Weisz B, Meirow D, Schiff E, Lishner M. Impact and treatment of cancer during pregnancy. Expert Rev Anticancer Ther. 2004 Oct: 4:889-902
[15] Cordeiro A, Machado AI, Borges A, Alves MJ, Frade MJ. Burkitt’s lymphoma related to Epstein-Barr virus infection during pregnancy. Arch Gynecol Obstet. 2009 Aug: 280:297-300
[16] Rey J, Coso D, Roger V, et al. Rituximab combined with chemotherapy for lymphoma during pregnancy. Leuk Res. 2009 Mar: 33:e8-9
[17] Decker M, Rothermundt C, Hollander G, Tichelli A, Rochlitz C. Rituximab plus CHOP for treatment of diffuse large B-cell lymphoma during second trimester of pregnancy. Lancet Oncol. 2006 Aug: 7:693-4
[18] Magloire LK, Pettker CM, Buhimschi CS, Funai EF. Burkitt’s lymphoma of the ovary in pregnancy. Obstet Gynecol. 2006 Sep: 108:743-5
[19] Friedrichs B, Tiemann M, Salwender H, Verpoort K, Wenger MK, Schmitz N. The effects of rituximab treatment during pregnancy on a neonate. Haematologica. 2006 Oct: 91:1426-7
[20] Herold M, Schnohr S, Bittrich H. Efficacy and safety of a combined rituximab chemotherapy during pregnancy. J Clin Oncol. 2001 Jul 15: 19:3439
[21] Kimby E, Sverrisdottir A, Elinder G. Safety of rituximab therapy during the first trimester of pregnancy: a case history. Eur J Haematol. 2004 Apr: 72:292-5

 

Date added to bjui.org: 15/11/2011 


DOI: 10.1002/BJUIw-2011-061-web

 

Inflammatory polyneuropathy presenting as acute urinary retention

/by

We present the case of a 16 year old boy in acute urinary retention secondary to an inflammatory polyneuropathy; likely the Miller Fisher variant of GBS. 

 

Authors: Mark Sayles1, Daniella Maleknasr1, Francesca A. Crawley2, James D.D. Allan1

1.  Department of Urology
West Suffolk Hospital
Hardwick Lane
Bury St Edmunds
Suffolk
IP33 2QZ
2.  Department of Neurology
West Suffolk Hospital
Hardwick Lane
Bury St Edmunds
Suffolk
IP33 2QZ

 
Corresponding Author: Mark Sayles, Department of Urology, West Suffolk Hospital.  Email: [email protected]

Abbreviations
AUR, Acute Urinary Retention
MRI, Magnetic Resonance Imaging
PCR, Polymerase chain reaction
CSF, Cerebro-Spinal Fluid
GBS, Guillain-Barré syndrome
MFS, Miller Fisher syndrome

 

Introduction
 
Guillain-Barré syndrome (GBS) is a common cause of acute neuromuscular paralysis. We present the case of a 16 year old boy in acute urinary retention secondary to an inflammatory polyneuropathy; likely the Miller Fisher variant of GBS. We discuss the management of this unusual urological presentation, and review the relevant literature.

 

Case Report
 
A previously healthy 16 year old boy presented to the emergency department with acute urinary retention. He had a three-day history of difficulty passing urine, and had recently developed intense suprapubic pain associated with a complete inability to spontaneously void. Following initial assessment, a urethral catheter was inserted; with drainage of  a residual urine volume of 1L.
The patient took no regular medications, denied illicit drug use, and was sexually active with a single female partner. There was no history of recent trauma, and no significant family history. He had experienced neither dysuria, nor any urethral symptoms to suggest a sexually-transmitted infection. Two weeks earlier he had been generally unwell with an intermittent fever, nausea and vomiting. Five days after the onset of this illness he developed facial paraesthesiae and fluctuating changes in his voice pitch. In addition, he had experienced paroxysmal spasms involving his left arm several times per day for one week preceding his presentation.
On initial assessment, apart from a palpable urinary bladder, no abnormality was found on examination. Importantly, he had normal anal tone and intact perianal sensation, and cardiorespiratory function was not compromised. Neurological examination revealed no focal signs. Reflexes were all present. However, the history and presentation were considered highly suspicious for demyelination, requiring specialist neurological investigation.
Routine haematology and serum biochemistry were normal, as were urinary microscopy and culture. Magnetic resonance imaging (MRI) of his brain and spinal cord was requested and was normal, with no mass lesion and no demyelinating plaques in the white matter. His cerebro-spinal fluid (CSF) was acellular, with normal biochemistry, microscopy, gram stain and culture. His CSF protein level was within normal limits at 0.32g/L [0.15-0.45]. There were no oligoclonal bands in his CSF or serum, and CSF PCR was negative for herpes simplex, varicella zoster, and enterovirus. Serology was subsequently negative for coeliac, paraneoplastic, and anti-GQ1b (associated with Miller Fisher syndrome) antibodies.
After initial catheterisation, a trial without catheter was unsuccessful. The patient did not accept the idea of a long term catheter. He was taught intermittent self catheterisation and discharged home after 3 days as an inpatient. One week after discharge he was readmitted under the care of the neurologists with limb ataxia, a complex bilateral ophthalmoplegia, and bilateral ptosis. He was now areflexic. Repeat CSF analysis was again normal. Neurophysiology found no evidence of a neuropathy or neuromuscular junction disorder. He was given general supportive care and his symptoms gradually improved.
At follow up two weeks later his symptoms had largely resolved, apart from some residual ophthalmoplegia and a continued inability to spontaneously pass urine. He is currently using ISC three to four times per day and once at night. He remains under regular urological follow up until his bladder emptying returns to normal.

 

Discussion
 
Acute urinary retention (AUR) is the sudden inability to void despite having a full bladder. The majority of causes can be classified as obstructive, inflammatory, pharmacologic, or neurologic [1].
Normal bladder function relies on complex signalling pathways in a neural network involving the central nervous system, and both autonomic and somatic nerves innervating the bladder and urethra. Disruption of these pathways at any point may result in a neurogenic bladder, manifest as either urinary incontinence or urinary retention [2]. The aetiology of neurogenic bladder is therefore diverse both in terms of anatomical site and underlying pathology.
Our patient presented with AUR associated with neurological features which had evolved over the course of several days following an acute infectious illness. The characteristic temporal pattern of his presenting features, preceded by an acute gastro-intestinal tract illness, and the absence of other causes for his bladder symptoms detected on routine blood analyses and MRI, pointed towards an acute post-infectious demyelinating process such as Guillain-Barré syndrome.
Guillain-Barré syndrome is typically a post-infectious disorder, with an estimated incidence of 1.2-2.3 per 100,000 population per year [3]. Miller Fisher syndrome (MFS) is a rare variant of GBS, with an annual incidence of 0.09 per 100,000 [4]. In contrast to the generalised limb weakness and sensory symptoms often seen in GBS, the clinical manifestations of MFS are restricted to ophthalmoplegia, limb ataxia, and loss of deep tendon reflexes [3, 5-7]. In addition to MFS, there are other variants and subtypes of GBS, such as acute motor (and sensory) axonal neuropathy, acute inflammatory demyelinating polyneuropathy, acute panautonomic neuropathy, and Bickerstaff brainstem encephalitis. Increasingly these syndromes are understood as representing discrete parts of a continuous spectrum of disease characterised by immune-complex mediated demyelination at sites throughout the nervous system [3, 8, 9]. Accordingly there is significant overlap between these syndromes with patients presenting with features of both GBS and MFS.
GBS typically presents with a rapidly progressing (over several days) weakness, often associated with pain, sensory impairment, and paraesthesiae affecting the limbs in a relatively symmetrical pattern. Involvement of respiratory muscles can lead to respiratory failure and death. Careful monitoring of vital capacity is therefore required to detect the 25% of severely affected patients who will need artificial ventilation [3].
Unusual patterns of muscle involvement can occur. There are several reports in the literature of patients presenting with AUR as the initial symptom of GBS [10-16]. Urinary dysfunction occurs in approximately 25% of patients with GBS, with AUR in 10% [13, 14, 17]. This is thought to be due to a combination of hypo- and hyper-activity in both the parasympathetic and sympathetic innervation of the bladder and urethra [12-14]. The prevalence of bladder dysfunction in MFS is unknown [17]. Approximately two-thirds of GBS patients have symptoms of autonomic dysfunction. Symptoms include cardiac arrhythmias, paralytic ileus, sustained hypertension, and sudden asystole [3, 11, 18, 19].
The underlying pathology in GBS (and variants) is thought to be an immune-complex mediated damage to peripheral nerves. Antibodies to various gangliosides (e.g., GM1, GM1b, GD1a, GalNAc-GD1a, GD3, GT1a, and GQ1b) on the surface of neurons have been found in the serum of patients with GBS [3]. These bind to the cell-surface antigen, activate the complement cascade, and thereby damage the myelin sheath, the axolemma, or voltage-gated cation channels. The resulting impaired nerve conduction leads to the observed clinical features. Because of differential expression of gangliosides across neuronal types, different patterns of antibody production are associated with distinct syndromes. Acute motor (and sensory) axonal neuropathy is associated with anti-GM1, -GM1b, -GD1a, and -GalNAc-GD1a antibodies, whereas MFS and GBS with ophthalmoplegia is associated with anti-GD3, -GT1a, and -GQ1b antibodies [3, 7, 20].
Approximately 85% of patients with MFS have detectable anti-GQ1b antibodies during the first week of the illness [21]. The high sensitivity and specificity of this serological finding for MFS makes it a useful diagnostic test. The classical CSF finding in GBS (and variants) is an acellular sample with an elevated protein concentration. In MFS only 25% of patients have an elevated CSF protein level during the first week, increasing to 85% by the third week. This is in contrast to GBS, where 45% have an elevated protein in week one, and 75% by week three [21]. Our patient exhibited neither elevated CSF protein concentration, nor anti-GQ1b antibodies. This may be related to the timing of the tests.
In support of the diagnosis of a post-infectious neuropathy, our patient experienced a febrile illness characterised by gastrointestinal disturbance several days before the onset of his neurological symptoms. Approximately two-thirds of GBS patients have a history of an antecedent infective illness. The most commonly implicated organism is Campylobacter jejuni [22]. Antibodies are generated to lipooligosaccharides in the cell wall of C. jejuni. These lipooligosaccharides mimic the carbohydrates of gangliosides. Therefore the antibodies cross react with gangliosides on the surface of peripheral neurons causing neurological disturbance. The specific gene variants expressed by the causative microorganism are thought to result in specific patterns of antibody production which lead to the distinct clinical syndromes by virtue of the pattern of expression of the target epitopes [3].
The GQ1b ganglioside is expressed by human oculomotor, abducens, and trochlear nerve fibres but not by other cranial nerves [23]. Oculomotor muscle fibres also express large amounts of GQ1b on their surface [24]. These two targets for anti-GQ1b antibodies (nerve and muscle) are thought to result in the ophthalmoplegia characteristic of MFS.
Regarding autonomic dysfunction, a recent in vitro study has shown that antibodies from GBS patients increase the release of noradrenaline from sympathetic neurons [25]. One previously documented case of AUR in a woman with GBS showed that the retention was due to a non-relaxing internal sphincter, presumably secondary to overactivity of the sympathetic innervation [14]. In a case series, the same authors concluded that the urodynamic abnormalities found in GBS patients were a combination of overactive and underactive detrusor muscle, and an overactive internal sphincter [13]. Wosnitzer et al. [15] reported the case of a 20-year old woman with persistent urinary retention 18 months after the onset of GBS. She was successfully treated with an implantable sacral neuromodulator.
Treatment for GBS involves frequent observation to detect autonomic dysfunction, monitoring of vital capacity, and swallowing, as well as meticulous attention to venous thrombo-embolism prophylaxis in immobilised patients. Immunotherapy with intravenous immunoglobulin or plasma exchange has been shown to significantly improve overall outcome in terms of morbidity and mortality [26]. There are no randomised-controlled trials of immunotherapy for MFS. Typically MFS patients have a good natural recovery, with between 60% and 100% having complete clinical recovery within six months. Immunotherapy is therefore not currently recommended for MFS due to a lack of evidence to support its use [27].

 

Conclusions
 
A young patient in acute urinary retention was found to have associated neurological features consistent with a clinical diagnosis of an inflammatory demyelinating polyneuropathy. This was likely Miller Fisher syndrome, although diagnostic testing was inconclusive. This is a rare but important differential diagnosis to consider in patients with bladder dysfunction of neurologic origin, not least because patients with autonomic involvement in an acute peripheral neuropathy may rapidly become unstable, requiring cardio-respiratory support.

 

References
 
[1] Selius BA, Subedi R. Urinary retention in adults: diagnosis and initial management. Am Fam Physician. 2008 Mar 1: 77:643-50
[2] Fowler CJ, O’Malley KJ. Investigation and management of neurogenic bladder dysfunction. J Neurol Neurosurg Psychiatry. 2003 Dec: 74 Suppl 4:iv27-iv31
[3] van Doorn PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and treatment of Guillain-Barre syndrome. Lancet Neurol. 2008 Oct: 7:939-50
[4] Casmiro M, Guarino M, D’Alessandro R. Guillain-Barre syndrome variants in Emilia-Romagna, Italy, 1992-3: incidence, clinical features, and prognosis. Emilia-Romagna Study Group on Clinical and Epidemiological Problems in Neurology. J Neurol Neurosurg Psychiatry. 1998 Aug: 65:218-24
[5] Fisher M. An unusual variant of acute idiopathic polyneuritis: syndrome of ophthalmoplegia, ataxia and areflexia. N Engl J Med. 1956: 255:57-65
[6] Willison HJ, O’Hanlon GM. The immunopathogenesis of Miller Fisher syndrome. J Neuroimmunol. 1999 Dec: 100:3-12
[7] Willison HJ. The immunobiology of Guillain-Barre syndromes. J Peripher Nerv Syst. 2005 Jun: 10:94-112
[8] Nagashima T, Koga M, Odaka M, Hirata K, Yuki N. Continuous spectrum of pharyngeal-cervical-brachial variant of Guillain-Barre syndrome. Arch Neurol. 2007 Oct: 64:1519-23
[9] Yuki N. Fisher syndrome and Bickerstaff brainstem encephalitis (Fisher-Bickerstaff syndrome). J Neuroimmunol. 2009 Oct 30: 215:1-9
[10] Kogan BA, Solomon MH, Diokno AC. Urinary retention secondary to Landry-Guillain-Barre syndrome. J Urol. 1981 Nov: 126:643-4
[11] Labovitz AE, Mangurten HH. Guillain-Barre syndrome presenting with urinary retention and hypertension. Clin Pediatr (Phila). 2004 Sep: 43:659-61
[12] Sakakibara R, Hattori T, Kuwabara S, Yamanishi T, Yasuda K. Micturitional disturbance in patients with Guillain-Barre syndrome. J Neurol Neurosurg Psychiatry. 1997 Nov: 63:649-53
[13] Sakakibara R, Uchiyama T, Kuwabara S, et al. Prevalence and mechanism of bladder dysfunction in Guillain-Barre Syndrome. Neurourol Urodyn. 2009: 28:432-7
[14] Sakakibara R, Uchiyama T, Tamura N, Kuwabara S, Asahina M, Hattori T. Urinary retention and sympathetic sphincter obstruction in axonal Guillain-Barre syndrome. Muscle Nerve. 2007 Jan: 35:111-5
[15] Wosnitzer MS, Walsh R, Rutman MP. The use of sacral neuromodulation for the treatment of non-obstructive urinary retention secondary to Guillain-Barre syndrome. Int Urogynecol J Pelvic Floor Dysfunct. 2009 Sep: 20:1145-7
[16] Wu SH, Lynn JJ, Chan YL, Chiu TF, Chen JC, Chang YC. Acute urinary retention as the initial manifestation of Guillain-Barre syndrome. Am J Emerg Med. 2011 Aug 2:
[17] Kuwabara S. Guillain-Barre syndrome: epidemiology, pathophysiology and management. Drugs. 2004: 64:597-610
[18] Nowe T, Huttemann K, Engelhorn T, Schellinger PD, Kohrmann M. Paralytic ileus as a presenting symptom of Guillain-Barre syndrome. J Neurol. 2008 May: 255:756-7
[19] Patel MB, Goyal SK, Punnam SR, Pandya K, Khetarpal V, Thakur RK. Guillain-Barre Syndrome with asystole requiring permanent pacemaker: a case report. J Med Case Reports. 2009: 3:5
[20] Chiba A, Kusunoki S, Shimizu T, Kanazawa I. Serum IgG antibody to ganglioside GQ1b is a possible marker of Miller Fisher syndrome. Ann Neurol. 1992 Jun: 31:677-9
[21] Nishimoto Y, Odaka M, Hirata K, Yuki N. Usefulness of anti-GQ1b IgG antibody testing in Fisher syndrome compared with cerebrospinal fluid examination. J Neuroimmunol. 2004 Mar: 148:200-5
[22] Hadden RD, Karch H, Hartung HP, et al. Preceding infections, immune factors, and outcome in Guillain-Barre syndrome. Neurology. 2001 Mar 27: 56:758-65
[23] Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I. Ganglioside composition of the human cranial nerves, with special reference to pathophysiology of Miller Fisher syndrome. Brain Res. 1997 Jan 16: 745:32-6
[24] Liu JX, Willison HJ, Pedrosa-Domellof F. Immunolocalization of GQ1b and related gangliosides in human extraocular neuromuscular junctions and muscle spindles. Invest Ophthalmol Vis Sci. 2009 Jul: 50:3226-32
[25] Lehmann HC, Jangouk P, Kierysch EK, Meyer zu Horste G, Hartung HP, Kieseier BC. Autoantibody-mediated dysfunction of sympathetic neurons in Guillain-Barre syndrome. Arch Neurol. 2010 Feb: 67:203-10
[26] Hughes RA, Swan AV, Raphael JC, Annane D, van Koningsveld R, van Doorn PA. Immunotherapy for Guillain-Barre syndrome: a systematic review. Brain. 2007 Sep: 130:2245-57
[27] Overell JR, Hsieh ST, Odaka M, Yuki N, Willison HJ. Treatment for Fisher syndrome, Bickerstaff’s brainstem encephalitis and related disorders. Cochrane Database Syst Rev. 2007:CD004761

 

Date added to bjui.org: 09/11/2011 


DOI: 10.1002/BJUIw-2011-075-web

 

Fat-containing renal mass with small areas of calcification: surgical excision reveals renal cell carcinoma with osseous metaplasia

/by

We report a case of a  fat containing renal mass, which was suggestive of angiomyolipoma and percutaneous ablation was initially considered. 

 

Authors: A.Pai, S. Kumar, N. Onwu, A. Jones, Royal Berkshire Hospital
Corresponding Author: A. Pai, Department of Urology, Royal Berkshire Hospital, Reading. E-mail: [email protected]

Abstract 

 

We report a case of a  fat containing renal mass, which was suggestive of angiomyolipoma and percutaneous ablation was initially considered.  The presence of tiny calcifications made us reconsider our differential diagnosis; a robotic partial nephrectomy revealed a clear cell renal cell carcinoma.

 

Introduction

 

It is widely accepted that evidence of macroscopic fat within a renal mass on CT is suggestive of angiomyolipoma (1).  We describe a case where a 5cm fat-containing renal tumour was consistent with an angiomyolipoma and percutaneous ablation was initially recommended. Tiny areas of intratumoral calcification made us reconsider our management.  A robotic partial nephrectomy was carried out, which revealed a clear cell renal cell carcinoma with osseous metaplasia. Despite the current consensus that angiomyolipomas over 4cm should be considered for percutaneous ablation (2) , this case shows that when there is diagnostic uncertainty, surgical excision should be considered as first line management.

 

Case Report

 

53 year old mechanical engineer presented as a general surgical patient with left sided abdominal pain.  He underwent an ultrasound scan, with the incidental finding of a well defined heterogeneous solid mass in the upper pole of the right kidney.

 

A contrast CT scan confirmed a 5cm solid mass arising from the upper pole of the right kidney, separate from the adrenal gland.  The lesion contained multiple hypoattenuating foci suggestive of macroscopic fat and a small amount of intratumoral calcification was shown (Figure 1).

 

Figure 1 Axial (Figure 1a), coronal (Figure 1b) CT images with IV contrast enhancement, showing a right renal mass with multiple foci of fat.  Intratumoral microcalcifications are present.

 

 

Figure 1b. 

 

 

The images were highly suggestive of an angiomyolipoma (AML) and since the lesion was bigger than 4cm, percutaneous ablation was initially recommended.  However, the presence of a tiny amount of calcification visible within the tumour raised the possibility of a renal cell carcinoma (RCC).
A robotic right partial nephrectomy was carried out.  Pathological examination revealed solid architecture with areas of metaplastic bone and adipose tissue associated with haemorrhage and degenerative changes with the tumour (Figure 2).

 

Figure 2. Photomicrograph of surgical specimen (haematoxylin eosin stain; original magnification: x100). Clear cell renal cell carcinoma with foci of metaplastic bone and adipose tissue. 

 

 

The features were consistent with a clear cell renal cell carcinoma, Fuhrman Grade 3, with clear resection margins.

 

Discussion

 

Although it is clear that radiologically detectable fat is strongly associated with angiomyolipoma, there have been exceptions.  In particular, there have been reports of fat-containing RCC’s (3-5).   The engulfing of perinephric fat and cholesterol necrosis misinterpreted as macroscopic fat are the two most common mechanisms of fat deposition within RCC (4).  The third, and rarest mechanism, is osseous metaplasia of the nonepithelial stromal portion of the tumour, with  growth of fatty marrow elements and trabeculae (1,4).  However this usually happens in cases of osseous metaplasia without calcification. Since this case contained calcification, this is unlikely to be the cause of the fat seen in this tumour.
In the reported cases of RCC with osseous metaplasia, the majority have shown some evidence of macroscopic calcification (3,5).  In the present case, there was a tiny amount of calcification.  Since AML only rarely shows evidence of calcification (4), it is clear that RCC should be considered in any fat-containing mass with calcification.
The advancement of percutaneous ablation for AML means it is imperative that the correct provisional diagnosis is made based on imaging.  We suggest that in fat-containing lesions larger than 4 cm where there is uncertainty over the diagnosis of AML,  diagnostic renal mass biopsy or surgical excision, should be considered as first line, invasive management.

 

References 

 

1. Hélénon O, Merran S, Paraf F, Melki P, Correas JM, Chrétien Y, et al. Unusual fat-containing tumors of the kidney: a diagnostic dilemma. Radiographics. 1997 Feb;17(1):129-144.
2. Kothary N, Soulen MC, Clark TWI, Wein AJ, Shlansky-Goldberg RD, Crino PB, et al. Renal angiomyolipoma: long-term results after arterial embolization. J Vasc Interv Radiol. 2005 Jan;16(1):45-50.
3. Hélénon O, Chrétien Y, Paraf F, Melki P, Denys A, Moreau JF. Renal cell carcinoma containing fat: demonstration with CT. Radiology. 1993 Aug;188(2):429-430.
4. Richmond L, Atri M, Sherman C, Sharir S. Renal cell carcinoma containing macroscopic fat on CT mimics an angiomyolipoma due to bone metaplasia without macroscopic calcification. Br J Radiol. 2010 Aug;83(992):e179-181.
5. Roy C, Tuchmann C, Lindner V, Guth S, Vasilescu C, Saussine C, et al. Renal cell carcinoma with a fatty component mimicking angiomyolipoma on CT. Br J Radiol. 1998 Sep;71(849):977-979.

 

Date added to bjui.org: 02/11/2011 


DOI: 10.1002/BJUIw-2011-066-web

 

Granulomatous Cholangitis as a Complication of Intravesical BCG Administration for Bladder Cancer

/by

We describe a patient who presented with findings suggestive of acute cholecystitis and cholangitis. 

 

Authors: Hani M. Babiker, MD1, Robyn E. Stiefeld1, MD, and Holenarasipur R. Vikram2, MD

1Department of Hospital Internal Medicine, and
2 Division of Infectious Diseases, Mayo Clinic, Phoenix, Arizona

Corresponding Author: Holenarasipur R. Vikram, MD, Division of Infectious Diseases, 5777 E. Mayo Blvd., Phoenix, AZ 85054. Phone: (480) 342-0115  E-mail: [email protected]

 

Abstract
 
Intravesical instillation of Bacillus Calmette-Guerin (BCG) remains a first-line treatment for superficial transitional cell carcinoma of the bladder. Although uncommon, clinicians should be aware of major adverse effects and complications resulting from intravesical BCG therapy in order to promptly arrive at the diagnosis and initiate therapeutic measures.
Herein, we describe a patient who presented with findings suggestive of acute cholecystitis and cholangitis. He was started on antibiotics and underwent Endoscopic Retrograde Cholangiopancreatography (ERCP) with sphincterectomy and stent placement. However, his liver function tests remained abnormal. Further inquiry delineated a history of bladder cancer treated with intravesical BCG instillation. A liver biopsy obtained during laparoscopic cholecystectomy confirmed granulomatous cholangitis. The patient received anti-tuberculous therapy and a tapering course of corticosteroids with a successful outcome.

 

Introduction
  Bacillus Calmette-Guerin (BCG) is a live attenuated vaccine containing Mycobacterium bovis that is administered in many countries to prevent childhood tuberculous meningitis and military tuberculosis. BCG is not utilized in the United States because of the low risk of infection with M. tuberculosis and questionable efficacy. Intravesical BCG administration has been found to be very effective in the treatment in superficial bladder cancer. However, treatment with BCG can be associated with local or systemic complications. Herein, we present a case of granulomatous cholangitis and hepatitis following intravesical BCG therapy. Timely diagnosis and prompt initiation of therapy is essential for ensuring a favorable outcome.

 

List of Abbreviations
ALT Alanine Aminotransferase
ALK Alkaline Phosphatase
AST Aspartate Aminotransferase
BCG Bacillus Calmette-Guerin
ERCP Endoscopic Retrograde Cholangiopancreatography
LFTs Liver Function Tests
Tbili Total Bilirubin
Dbili Direct Bilirubin

 

Case Report
A 65 year-old-male with an underlying history of coronary artery disease, hypertension, obstructive airway disease, and bladder cancer presented to our Emergency Department with abdominal discomfort. Computed tomography (CT) of the abdomen was within normal limits. He was discharged once his symptoms abated.
Two weeks later, he presented with fever, jaundice, hypotension, abdominal pain, and jaundice. Abdominal examination revealed normal bowel sounds and mild right upper quadrant tenderness. There was no hepatosplenomegaly, guarding, rebound tenderness, or rigidity. His labs revealed a white blood cell count of 7.8 x 109/L, aspartate aminotransferase (AST) of 278 U/L, alanine aminotransferase (ALT) 305 U/L, alkaline phosphatase (ALK) 227 U/L, total bilirubin (Tbili) of 6.3 mg/dl, direct bilirubin (Dbili) of 3.6 mg/dl, lipase of 30 U/L, and ammonia of 36 mcg/dl. His Hepatitis A, B, and C serologies were negative. Abdominal ultrasound revealed gallbladder wall thickening, biliary sludge, and a normal calibre common bile duct.
He was fluid resuscitated and commenced on ciprofloxacin and metronidazole for a tentative diagnosis of cholangitis and cholecystitis. The next day, his Tbili increased to 7.3 mg/dl and his liver enzymes remained elevated. Repeat abdominal CT scan revealed mild thickening of the gallbladder wall, cholelithiasis, and heterogenous enhancement of the liver parenchyma. ERCP was performed with removal of biliary sludge, stent placement, and sphincterectomy. However, the next day, his Tbili and Dbili increased to 10.2 mg/dl and 8.5 mg/dl, respectively; liver enzymes remained abnormal. An intrahepatic process versus biliary stent stenosis was considered, and repeat ERCP and cholangiogram were planned along with cholecystectomy. Prior to surgery, his liver function    remained abnormal with a TBili of 16.1 mg/dl, DBili 12.8 mg/dl, ALK 507 U/L, AST 135 U/L, and ALT 159 U/L. Further inquiry into his past medical history revealed that he had received a total of three courses of intravesical BCG for superficial bladder cancer within the past 7 months (the last administration was 2 months prior to his current hospitalisation).
A liver biopsy performed at the time of cholecystectomy revealed portal infiltration with non-caseating granulomas centered around the bile ducts. There was focal bile duct proliferation with neutrophilic infiltration consistent with bile duct outflow impairment. Histology of the gall bladder showed acute inflammation with Gram-negative and Gram-positive cocci; granulomas were not evident.

 

Figure 1. Liver Histopathology

 

Liver histology demonstrates a granuloma centered around a bile duct ( arrow) and a hepatocyte (arrowhead). Concurrent neutrophilic and eosionphilic infiltration, and bile ductular proliferation resulted in biliary obstruction. Mild fibrosis and marked cholestasis in the surrounding liver was also noted. (Hematoxylin-eosin; ~x400.)

 

 

Bacterial, fungal, and mycobacterial smears and cultures were all negative from the liver biopsy specimen. A diagnosis of Mycobacterium bovis granulomatous cholangitis secondary to intravesical BCG administration was entertained. He was commenced on a regimen of isoniazid, rifampin, ethambutol, and vitamin B6 for six months and a 3-week tapering course of corticosteroids. His bilirubin and liver enzymes pursued a downward trend. Isoniazid was discontinued after the first month for transient liver enzyme elevation. His liver enzymes and bilirubin completely normalized within 2 months, and he remained asymptomatic. He completed a 6-month course of rifampin and ethambutol; LFTs remained normal at follow-up 2 months after discontinuing antituberculous medications.

 

Discussion
Bacillus Calmette-Guerin (BCG) vaccine was introduced in 1910 for protection against tuberculosis (TB). It is a live, attenuated vaccine containing M. bovis. It is utilized in many countries with a high prevalence of TB to prevent childhood tuberculous meningitis and military TB. BCG is not recommended in the United States because of the low risk of infection with M. tuberculosis, its variable and questionable efficacy against adult pulmonary TB, and its interference with tuberculin skin test reactivity. In 1976 Morales and colleagues described a novel utility for BCG – anticancer immunotherapy by intravesical instillation for superficial bladder cancer.  Several studies have subsequently demonstrated its efficacy in treating superficial bladder cancer.   This is now the adjuvant treatment of choice for high grade and recurrent superficial bladder cancer.    Its mechanism of action is incompletely understood; it triggers a local cellular immune response with induction of cytokines that have antiangiogenic activity.
Complications of intravesical BCG treatment can be either local (cystitis) or disseminated, with an early or late presentation. Early manifestations occurs 8 to 12 weeks after BCG instillation, while late manifestations can occur more than a year after BCG therapy.8 Cystitis following BCG administration presents with dysuria, urinary frequency, low-grade fever, and malaise. These symptoms occur in 70% of patients approximately 2 to 4 hours after BCG instillation and resolve within 48 hours. Symptomatic therapy is successful in most instances. For persistent or severe symptoms that last beyond 48 hours, isoniazid should be administered.  Rarely, a sepsis-like syndrome can occur soon after BCG instillation; patients develop fever, hypotension, and respiratory failure. This is thought to represent a hypersensitivity reaction to BCG as opposed to true dissemination.9 Other local complications (<1% each) include hematuria, epididymitis, prostatitis, and ureteral obstruction. Disseminated infection (‘BCGosis’) may result in granulomatous hepatitis, pneumonitis, osteomyelitis, endophthalmitis and prostatitis and other organ involvement.2,5,9 These complications can occur weeks to months after BCG administration in approximately 1% of patients. The most serious complication following BCG therapy is sepsis with hypotension and multiorgan failure in 0.4% of cases and carries a high mortality.5 Other published complications of intravesical BCG therapy include testicular masses, peritonitis, psoas abscess, tuberculous spondylitis, chest wall mass, acute renal failure, rhabdomyolysis, pancytopenia secondary to bone marrow infiltration and ruptured mycotic abdominal aortic aneurysm. 10 In a large study involving 2602 patients who received intravesical BCG, granulomatous hepatitis occurred in 0.7% of patients.9
BCGosis is diagnosed by growth of M. bovis from tissue specimens, or by DNA hybridization. 11 However disseminated M. bovis infection is often paucibacillary, and it is difficult to isolate the pathogen in vitro. Many of the systemic manifestations of BCGosis can be attributed to a hypersensitivity reaction to mycobacterial antigens. Thus, a tentative diagnosis is often made based on the temporal relationship to intravesical BCG administration, clinical manifestations, histopathologic findings, and response to empiric antituberculous therapy. M. bovis is susceptible to first-line anti-tuberculous agents (except pyrazinamide). A 6 to 12 month course of therapy with isoniazid, rifampin, and ethambutol along with vitamin B6 supplementation has been widely utilised in published reports with an excellent response. A tapering dose of corticosteroids is usually included to combat the associated hypersensitivity reaction to mycobacterial antigens.2

 

Conclusion
We report a case of bacterial cholecystitis wherein liver enzyme and bilirubin levels remained abnormal despite cholecystectomy and antibiotic therapy. Concurrent liver biopsy demonstrated granulomatous cholangitis. LFT abnormalities completely resolved following a tapering course of prednisone and 6 months of antituberculous therapy aimed at M. bovis. This case highlights the importance of considering BCG-induced granulomatous hepatitis and cholangitis as a possible etiology in patients with LFT abnormalities following intravesical BCG administration. To the best of our knowledge, this is the first reported case of BCG-induced granulomatous cholangitis. Awareness of this entity, collection of tissue specimens for histopathologic and microbiologic examination, and prompt initiation of appropriate therapy led to a favorable outcome in our patient.

 

References
1. Morales A, Eidinger D, Bruce AW. Intracavitary bacillus Calmette-Guerin in the treatment of superficial bladder tumors. J Urol. 1976 Aug: 116:180-183
2. Lamm DL. Complications of bacillus Calmette-Guerin immunotherapy. Urol Clin North Am. 1992 Aug: 19:565-72.
3. Witjes JA, vd Meijden AP, Debruyne FM. Use of intravesical Bacillus Calmette-Guerin in the treatment of superficial transitional cell carcinoma of the bladder: an overview. Urol Int. 1990: 45(3):129-136.
4. Kamat AM, Lamm DL. Immunotherapy for bladder cancer. Curr Urol Rep 2001 Feb: 2(1):62-9.
5. Lamm DL. Efficacy and safety of bacillus Calmette-Guerin immunotherapy in superficial bladder cancer. Clin Infect Dis. 2000 Sep: 31(suppl 3):S86-90.
6. Shelley MD, Wilt TJ, Court J, Coles B, Kynaston H, Madson MD. Intravesical bacillus Calmette-Guerin is superior to mitomycin C in reducing tumor recurrence in high-risk superficial bladder cancer: a meta-analysis of randomized trials. BJU Int. 2004 Mar :93(4):485-90.
7. Bohle A. BCG’s mechanism of action–increasing our understanding. Eur Urol. 2000 :37:Suppl 1:1-8.
8. Gonzales OY, Musher DM, Brar I, et al. Spectrum of Bacille Calmette-Guerin (BCG) Infection after Intravesical BCG Immunotherapy. Clin Infect Dis. 2003 Jan: 36(2):140-8.
9.  Lamm DL, van der Meijden PM, Morales A, et al. Incidence and treatment of complications of bacillus Calmette-Guerin intravesical therapy in superficial bladder cancer. J Urol. 1992 Mar:147(3):596-600.
10.  Safdar N, Abad CL, KauL DR, Jarrard D, Saint S. Clinical problem-solving. An unintended consequence–a 79-year-old man with a 5-month history of fatigue and 20-lb (9-kg) weight loss presented to his local physician. N Engl J Med. 2008 Apr: 358(14):1496-1501.
11. Leebeek FW, Ouwendijk RJ, Kolk AH, et al. Granulomatous hepatitis caused by Bacillus Calmette-Guerin (BCG) infection after BCG bladder instillation. Gut. 1996 Apr: 38(4):616-618.

 

Date added to bjui.org: 26/10/2011


DOI: 10.1002/BJUIw-2011-093-web