Abdominoperineal Pull-through with Simultaneous Extravesical Detrussoraphy: An Alternative Surgical Technique for Congenital Pouch Colon with High-grade Vesicoureteric Reflux.

Congenital pouch colon (CPC) is frequently associated with vesicoureteric reflux (VUR). These patients require long-term antibiotic prophylaxis and/or an additional surgical intervention for the management of the refluxing system. We propose a single-stage alternative approach in these patients. Two patients diagnosed to have CPC underwent pouch excision and an end colostomy at birth. Further evaluation revealed high-grade reflux in both the patients. At 6 months of age, definitive abdominoperineal pull-through (APPT) surgery along with extravesical detrusorrhaphy was performed. In the follow-up at 1 year, they are thriving well with no urinary complaints. Micturating cystourethrogram revealed complete resolution of VUR. This approach takes the advantage of the anesthesia for APPT and offers a relatively simple and quick solution for the refluxing system, thus, enabling the stoppage of antibiotic prophylaxis and obviating the need for a future endoscopy/surgery.

INTRODUCTION

Congenital pouch colon (CPC) syndrome is a rare variant of high anorectal malformation in which there is a complete absence of the rectum and variable length of the colon terminating in a dilated pouch that most commonly opens directly into the bladder through a wide fistula.[1] Based on the length of the colon, there are four types. About 37.46% of patients of CPC have associated genitourinary anomalies, among which vesicoureteric reflux (VUR) accounts for almost 17%.[2]

The presence of high-grade VUR in a patient of pouch colon necessitates the institution of long-term antibiotic prophylaxis even after definitive repair. In case of nonresolution of reflux, there remains a possible need for a third procedure in the form of endoscopic bulking agent injection or reimplantation surgery.

We report two cases of CPC with high-grade bilateral VUR managed by extravesical detrusorraphy done during the definitive reconstruction for CPC.

CASE REPORTS

Case 1

A 2-day-old male child presented with absent anal opening. The erect abdominal radiograph and cross table prone lateral (CTPL) view were suggestive of pouch colon [Figure 1]. Intraoperatively, there was Type 4 pouch colon, with dilated bilateral ureters. The pouch was excised and end stoma at the level of descending colon was made.

Figure 1

Preoperative radiographs showing pouch colon in the patients. (a) AXR of case 1, (b) CTPL of case 1, (c) AXR of case 2, (b) CTPL of case 2

The patient was discharged on full feeds and oral amoxicillin (15 mg/kg/day) uroprophylaxis. Sonography revealed bilateral hydroureteronephrosis, and a subsequent micturating cystourethrogram (MCU) showed bilateral Grade V VUR [Figure 2]. Renal function tests were within the normal range. Nuclear scan revealed a split function of 55% and 45% on the right and left sides respectively, with bilateral scarring and impaired cortical function.

Figure 2

Pre- and postoperative cystograms of the patients showing complete resolution of vesicoureteric reflux. (a) Preoperative MCU of case 1, (b) Postoperative MCU of case 1, (c) Preoperative MCU of case 2, (d) Postoperative MCU of case 2

Case 2

A male child was admitted on day 1 of life with absent anal opening. Abdominal and CTPL radiographs were suggestive of pouch colon [Figure 1]. Intraoperatively, Type 1 pouch colon was found along with bilateral dilated ureters. Pouch excision and end ileostomy were done. The patient had an uneventful recovery and was discharged on amoxicillin prophylaxis. MCU showed bilateral high-grade VUR [Figure 2]. Nuclear scan showed bilateral good cortical function without any evidence of scar.

At the age of 6 months, both patients underwent definitive abdominoperineal pull-through (APPT) surgery. End stoma was mobilized and pulled down in between the sphincter complex. Bilateral ureters were reimplanted by extravesical approach with detrusorraphy as described by Lich and Gregoir.[34] Postoperatively, an indwelling catheter was placed for 5 days. The recovery was uneventful, and they were discharged on full feeds without any tubes on the 6th postoperative day.

Ultrasound done after 1 month of surgery showed mild bilateral hydronephrosis with no postvoid residual urine in both the patients. Urine cultures continued to be sterile on uroprophylaxis. Serum creatinine was well within the normal range. MCU done 3 months postoperatively did not show any residual VUR in both the cases [Figure 2]. Hence, uroprophylaxis was stopped. The follow-up dimercaptosuccinic acid [DMSA] done after 3 months of surgery in Case 1 revealed no scarring in the left kidney, while the right kidney had persistent old scars without any new scar formation and deterioration in its function. Similarly, Case 2 did not show any deterioration or new scars on DMSA.

Urodynamics was performed after 1 year of surgery. Since both the children are not yet toilet trained, only cystometry could be done. It showed good bladder capacity with normal pressures and no detrusor instability in both the patients.

DISCUSSION

The association of pouch colon anomaly with VUR has previously been defined in various studies,[12] but the exact incidence is still not known. Associated genitourinary system anomalies were seen in 20/56 patients (35.71%), of which VUR was seen in all six patients who underwent cystourethrogram; in rest of the 50 patients, cystourethrogram was not done. In another study, associated genitourinary system anomalies were seen in 212/566 (37.4%), of which VUR is seen in 36/566 (6.36%) cases of CPC.[5]

None of the studies has described the surgical approach for VUR associated with pouch colon. In VUR, the primary goal of therapy is to prevent renal cortical damage, because repeated upper urinary tract infections (UTIs) can lead to hypertension, reduced somatic growth, renal insufficiency, renal scarring, and end-stage renal disease. If a child already has reduced renal function, then additional treatment may be necessary to preserve existing renal function as the child grows.

It is well proven that lower grades of reflux undergo spontaneous resolution over time, and the results are better in younger patients. For the nonsurgical therapy to be effective, it is mandatory that the urine remains sterile. This spontaneous resolution is presumed to be due to bladder growth and functional maturation with time, but it is still unproved if the intramural tunnel length of ureter increases with time.[5] Antibiotic prophylaxis is usually recommended till 4–5 years of age. Goldraich and Goldraich found that no new scar occurred in children over 4 years, so they recommended discontinuing prophylaxis after 5 years of age.[6]

Many questions have been raised against it especially with respect to their efficacy, concerns about the development of resistance,[7] hypersensitivity reaction with penicillin and sulfa drugs, and gastrointestinal disturbances with penicillin and nitrofurantoin. According to Hensle et al.,[8] only 17% of patients were adherent to antibiotic prophylaxis and the mean compliance was 41.4%.

If a child on antibiotic prophylaxis is having frequent breakthrough UTIs, intolerance or noncompliance of antibiotics, and new scar formation on follow-up nuclear scans, then, the only alternative is surgical intervention.

Although endoscopic injection for VUR is less invasive than open ureteric reimplantation, the success rate is less with single injection (60%–80%) as compared to open surgery (almost 100%) in high-grade VUR (IV and V).[9] Moreover cost, availability of deflux, endourology setup, technical expertise, and the need for more than one sitting to completely abolish the high-grade VUR are points of great concern.

Intravesical surgery is considered to be more invasive than the extravesical approach. The bladder has to be opened and gross hematuria sometimes requiring prolonged hospitalization and catheterization may result. Moreover, in intravesical reimplantation, the ureterovesical junction is dissected dividing the ureter's trigonal attachment. This is avoided using the Lich–Gregoir technique.[10]

The Lich–Gregoir ureteral reimplantation procedure is simple, fast, and does not require placement of stents in the ureters, hence requiring a shorter hospital stay. Moreover, extravesical approach avoids contamination of urinary system while simultaneously handling the gastrointestinal system.

Although postoperative urinary retention is a well-known complication of extravesical ureteric reimplantation, we did not encounter this problem in either of our patients.

The advantage of a single-stage procedure (pull-through and extravesical reimplantation) is in the fact that it provides a permanent renal protection in a single sitting using a single incision that is cost-effective and affords early stoppage of antibiotic prophylaxis without a significant increase in the operative time and length of hospital stay.

CONCLUSIONS

Simultaneous APPT and extravesical detrussoraphy have the advantage of managing both the genitourinary and gastrointestinal system in a single sitting without any complications. The outcomes in these two cases are promising and warrant a further study comparing the outcomes of cases operated as a single stage versus those managed by the standard protocol.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.