Long-term nephrostomy in an adult male spinal cord injury patient who had normal upper urinary tracts but developed bilateral hydronephrosis following penile sheath drainage: pyeloplasty and balloon dilatation of ureteropelvic junction proved futile: a case report

Introduction The consequences of spinal cord injury upon urinary bladder are readily recognised by patients and health care professionals, since neuropathic bladder manifests itself as urinary incontinence, or retention of urine. But health care professionals and persons with spinal cord injury may not be conversant with neuropathic dysmotility affecting the ureter and renal pelvis. We report an adult male patient with spinal cord injury, who developed bilateral hydronephrosis after he started managing neuropathic bladder by penile sheath drainage. Case presentation A male patient, born in 1971, sustained spinal cord injury following a motorbike accident in September 1988. In November 1988, intravenous urography showed normal upper tracts. He was advised spontaneous voiding with 2-3 catheterisations a day. In February 1995, this patient developed fever, chills and vomiting. Blood urea: 23.7 mmol/L; creatinine: 334 umol/L. Ultrasound revealed marked hydronephrosis of right kidney and mild hydronephrosis of left kidney. Bilateral nephrostomy was performed in March 1995. Right pyeloplasty was performed in May 1998. In July 2005, this patient developed urine infection and was admitted to a local hospital with fever and rigors. He developed septicaemia and required ventilation. Ultrasound examination of abdomen revealed bilateral hydronephrosis and multiple stones in left kidney. Percutaneous nephrostomy was performed on both sides. Subsequently, extracorporeal shock wave lithotripsy of left renal calculi was carried out. Right nephrostomy tube slipped out in January 2006; percutaneous nephrostomy was performed again. In June 2006, left ureteric antegrade stenting was performed and nephrostomy tube was removed. Currently, right kidney is drained by percutaneous nephrostomy and left kidney is drained by ureteric stent. This patient has indwelling urethral catheter. Conclusion It is possible that regular intermittent catheterisations along with anticholinergic medication right from the time of rehabilitation after this patient sustained paraplegia might have prevented the series of urological complications. Key components to successful management of external drainage of kidney in this patient are: [1] use of size 14 French pigtail catheter for long-term nephrostomy, [2] anchoring the catheter to skin to with Percufix catheter cuff to prevent accidental tug [3], replacing the nephrostomy dressing once a week by the same team in order to provide continuity of care, and [4] changing nephrostomy catheter every six months by a senior radiologist.


Introduction
Pyeloureteral tract receives its innervation mainly by unmyelinated fibres, which originate from the renal, ovarian/spermatic, and sympathetic plexuses. The lower part of the ureter may receive additional pelvic innervation. The sympathetic supply to the ureter arises from T11-L1 spinal segments. At least part of these fibres synapses in the distal pole of the inferior mesenteric ganglion [1]. The consequences of spinal cord injury upon urinary bladder are readily recognised by patients and health care professionals, as clinical presentation of neuropathic bladder is very obvious in terms of urinary incontinence, or retention of urine. But health care professionals and spinal cord injury patients may not be conversant with neuropathic dysmotility affecting the ureter and renal pelvis. We report an adult male patient, who developed bilateral hydronephrosis after he started managing neuropathic bladder by penile sheath drainage. Impaired drainage of urine from renal pelvis due to neuropathic dysmotility contributed to development of bilateral hydronephrosis, which manifested clinically as severe urinary sepsis. Initially, percutaneous nephrostomy was performed as an emergency procedure. Later we attempted to improve drainage from renal pelvis by performing pyeloplasty. As pyeloplasty was unsuccessful, balloon dilatation of pelviureteric junction was performed, which was also futile. In hindsight, we recognised the futility of carrying out these procedures, as pyeloplasty and balloon dilatation of pelviureteric junction were aimed solely to improve mechanical aspects of urinary drainage. These procedures did not correct the underlying pathology, which was neuropathic dysmotility of renal pelvis and ureter due to spinal cord injury.  On 10 January 2006, right nephrostomy did not drain urine. Nephrostogram revealed the tube to be lying outside pelvicalyceal system. The contrast entered perinephric tissue; therefore, the right nephrostomy tube was removed. Left nephrostogram showed that the contrast did not flow freely down left ureter. Intravenous urography, performed on 26 January 2006, showed right hydronephrosis due to pelvi-ureteric junction obstruction. There was dilatation of left pelvicalyceal system. On 31 January 2006, percutaneous right nephrostomy was performed. Since then right nephrostomy was anchored to skin with Percufix catheter cuff (Boston Scientific Corporation, One Boston Scientific Place, Natick, MA 01760-1537, USA).

Case presentation
MAG-3 renogram, performed on 06 February 2006, showed relative function of left kidney to be 71% and the right kidney 29%. There was normal uptake on the left and reduced uptake on the right at two minutes. Excretion was slow and sluggish from left kidney; however, excretion was diminished and poor from right kidney with the radioisotope activity gradually increasing with time. There was functionally significant obstruction within right kidney. The left kidney showed evidence of partial obstruction at the level of pelviureteric junction with preserved function.  http://www.casesjournal.com/content/2/1/9335 of tubing, which was of the same calibre as the left sided JJ stent. A number of calcific densities were seen in the region of the lower pole left kidney. No other urinary tract calcification was seen on the control film. The collecting systems of the left kidney were Duplex in nature and the left kidney was enlarged compared with the right. There was bilateral contrast excretion but again this was more marked on the left than the right. There was blunting of the minor calyces throughout the left kidney and prominence of the left renal pelvis suggesting previous pelviureteric junction obstruction (Figure 4). Very little anatomical detail was visible in the right kidney. The right ureter was not visualised but overall appearances did not suggest any obstruction. No useful contrast enhancement was seen within the bladder.
On 29 February 2008, cystoscopy was performed; left ureteric stent was removed. A 12-month stent was inserted in left ureter. Flexible ureteroscopy was performed on 28 March 2008. It was not possible to retrieve fragment of ureteric stent, which had been lying within right kidney. On 23 May 2008, right nephrostomy track was dilated to size 24 French. Flexible cystoscope was inserted. The fragment of ureteric stent was grasped and retrieved. ever, was seen within the right nephrostomy, left ureter and bladder. The left kidney was contributing 49% and the right 51% of total renal function.
Cytology of urine from right kidney taken on 13 May 2009 showed large numbers of acute inflammatory cells, suggesting current acute urine tract infection. Benign epithelial cells were also present, many of which were squamous, suggesting the presence of squamous metaplasia ( Figure 6). Occasional groups of cytologically bland urothelial cells were also present, but these could be explained by the presence of nephrostomy tube. No anucleate squames were present to suggest keratinising squamous metaplasia. There was no evidence of high-grade malignancy.
Microbiology of a swab taken from right nephrostomy site showed a heavy growth of coliforms on 28 April 2009. Currently, right kidney is drained by percutaneous nephrostomy and left kidney is drained by ureteric stent. This patient has indwelling urethral catheter drainage. He wears two leg bags and works full time.

Discussion
Instead of external drainage of kidney by means of percutaneous nephrostomy, nephrovesical subcutaneous ureteric bypass has been performed in patients with ureteric obstruction due to inoperable malignancy [2,3]. Nephrovesical subcutaneous ureteric bypass consists of two subcutaneously connected 12 French polyurethane tubes, placed as a nephrostomy and cystostomy. This nephrovesical ureteric bypass is a simple, minimally invasive, and highly effective treatment for patients with hydronephrosis resulting from advanced oncologic disease. Patients gain a better quality of life due to increased independence and mobility during their final stages of life. Subcutaneous urinary diversion with a nephrovesical stent provides effective urinary drainage and may improve the quality of life of patients with malignant metastatic ureteral obstruction. The Detour extra-anatomic stent (Mentor-Porges, UK) has also been used for permanent bypass of complete upper urinary tract obstruction [4]. This self-retaining expanded polytetrafluoroethylene-silicone tube is placed in the kidney using a percutaneous route, tunnelled under the skin, and sutured into the bladder to establish extra-anatomical urinary drainage. Preliminary data suggested that the Detour extra-anatomic stent offered a permanent and minimally invasive method to establish internalisation of urinary drainage to bypass complete ureteric obstructions for which conventional stenting had failed, open surgery had been tried and failed or was not considered feasible, and long-term nephrostomy drainage was not favoured.

Intravenous urography (18 February 2008) -30 minutes film showed dilated left renal pelvis and clubbing of calyces
When pyeloplasty is unsuccessful, a repeat open pyeloplasty is an option in neurologically intact individuals. Thomas and associates from Vanderbilt Children's Hospital, Nashville, Tennessee, USA [5], reviewed their experience with open dismembered pyeloplasty, with specific focus on the presentation and management of failed pyeloplasty in the pediatric population. Failure of pyeloplasty was most likely secondary to technical issues, including missed crossing vessels and dependency of the anastomosis. In this series, failed pyeloplasties did not respond well to balloon dilation, likely due to scar formation. These authors' current practice was to manage failures by open surgery, although endoscopic management by an incision might be an option. Braga and associates [6] compared retrograde endopyelotomy to redo pyeloplasty for the treatment of failed pyeloplasty in children. Retrograde endopyelotomy had a significantly lower suc-cess rate than redo pyeloplasty for correction of recurrent ureteropelvic junction obstruction after failed pyeloplasty in children.
Our patient with spinal cord injury and paraplegia developed bilateral hydronephrosis after he started managing his bladder by reflex voiding. In this patient, spinal cord injury resulted in neuropathic urinary bladder and neurogenic dysmotility of ureter and renal pelvis. Initially we performed right pyeloplasty and then balloon dilatation of right pelviureteric junction. Both procedures were unsuccessful in establishing satisfactory drainage of urine from right kidney. In hindsight, we recognised futility of these procedures, as neither of these procedures addressed the underlying pathology of neurogenic dysmotility of renal pelvis and ureter. In retrospect, we admitted our folly of performing these surgical procedures for treatment of hydronephrosis due to neurogenic dysmotility of pyeloureteral tract. Then, we adopted a pragmatic approach to the problem and relied upon percutaneous nephrostomy for drainage of right kidney and ureteric stent for drainage of left renal pelvis. At present, this patient has a size 14 Fr. pigtail catheter for nephrostomy. The nephrostomy is securely anchored to skin. The dressing is changed every Tuesday afternoon. The nephrostomy catheter is changed every six months. The patient has been coping with external drainage of kidney very well.

Conclusion
We learn from this case the importance of preventing urological complications in patients with spinal cord injury. It is possible that regular intermittent catheterisations along with anticholinergic medication right from the time of rehabilitation might have prevented the series of urological complications, which occurred in this patient. Key components to successful management of external drainage of kidney in this patient are: [1] use of size 14 French pigtail catheter for long-term nephrostomy, [2] anchoring the catheter to skin to prevent accidental tug, [3] replacing the nephrostomy dressing once a week by the same team in order to provide continuity of care, and [4] changing nephrostomy catheter every six months by a senior Radiologist. This patient has been doing well and he is in full time employment as an expert web-designer.

Patient's perspective
I have lived with nephrostomy drainage since July 2005, when I was taken into hospital with blockages in both kidneys. This was due to a large stone in my left kidney and restriction to my right ureter. This along with a chest infection, left me quite unwell so that I had to be sedated and ventilated for a few weeks. When I was taken off sedation, I discovered nephrostomy drainage to both kidneys and I had also been given a tracheostomy.
Cytology of urine from nephrostomy, shows three benign squamous cells (large cells with abundant, pink-orange cyto-plasm), with numerous inflammatory cells and inflammatory debris in the background Figure 6 Cytology of urine from nephrostomy, shows three benign squamous cells (large cells with abundant, pink-orange cytoplasm), with numerous inflammatory cells and inflammatory debris in the background.