Reconstruction of umbilicus during functional closure of bladder exstrophy

In babies born with bladder exstrophy, the umbilicus is caudally displaced, and attached to the upper margin of the exstrophied bladder. Reconstructive surgery often removes the navel. A surgical technique for preservation and repositioning of the umbilicus is presented.     

Advancement flap umbilicoplasty after abdominal wall closure: postoperative results compared with normal umbilical anatomy

BACKGROUND/PURPOSE: Umbilical reconstruction frequently is an adjunct used after repair of congenital abdominal wall defects. The authors describe a new technique of umbilicoplasty and compare their results with normal neonatal umbilical anatomy. METHODS: After fascial closure, 6 newborn infants with either gastroschisis (n = 3) or omphalocele (n = 3) underwent umbilicoplasty performed by advancing bilateral skin flaps including creation of an umbilical skin collar. The umbilical collar height, circumference, and distance from the xiphoid with respect to the pubis (ratio of xiphoid-umbilicus distance to xiphoid-pubis distance or XU:XP) were recorded and referenced to normal umbilical anatomy in additional 28 infants (24 to 40 weeks gestation [mean, 33.2 weeks] and age 2 to 131 days [mean, 26.5 days]). RESULTS: After umbilicoplasty, all infants had a near-normal-appearing umbilicus (collar height, 0.75 +/- 0.25 cm and circumference, 5.24 +/- 1.20 cm) compared with normal umbilical anatomy (collar height, 4.36 +/- 1.32 cm and circumference, 1.03 +/- 0.38 cm). However, after umbilicoplasty, the neoumbilicus was positioned more cephalad (XU:XP = 0.53 +/-.05) compared with normal (mean XU:XP, 0.67 +/- 0.07). The only complication encountered was a small dehiscence of the superior aspect of the abdominal wound. CONCLUSIONS: Closure of abdominal wall defects with simultaneous umbilicoplasty provides a cosmetically pleasing result. When performing umbilicoplasty, attempt should be made to position the neoumbilicus as inferior as possible, optimally at two thirds the distance from the xiphoid to the pubis.     

Long-term followup and outcome of continent catheterizable vesicocstomy using the Rink modification

PURPOSE: Multiple techniques have been described to create a Mitrofanoff channel in the pediatric population. A small subset of patients only requires creation of a catheterizable channel without bladder augmentation. These patients are ideal candidates for a procedure that avoids the use of intestine, especially in the absence of a suitable appendix. We used a modification of the Casale vesicostomy, as described by Rink, to create a continent vesicostomy in these children. We report our long-term experience with this technique. MATERIALS AND METHODS: We retrospectively reviewed the medical records of all patients who underwent continent vesicostomy at our institution between 1992 and 2000. Patient diagnosis, stomal site, associated bladder procedures, stomal continence, followup and complications associated with continent vesicostomy were documented. RESULTS: Of the 31 patients who underwent continent vesicostomy, as described by Rink, 14 were female and 17 were male. Average age was 9 years (range 2.5 to 22). Primary diagnosis included neuropathic bladder in 15 cases, the prune-belly syndrome in 6, cloacal exstrophy/anomaly in 5 and other in 5. The stoma was placed in the lower abdomen in 17 patients, in the umbilicus in 7 and in a neoumbilicus in 7. Simultaneous procedures included ureteral reimplantation in 8 cases, bladder augmentation in 5, bladder neck surgery in 4 and reduction cystoplasty in 2. Mean followup was 41 months. All patients achieved excellent stomal continence. Complications included stomal stenosis requiring revision in 14 cases (45%). Stenosis developed in 60% of the patients with neuropathic bladder and in 86% with an umbilical stoma. Eventually 6 patients underwent conversion to an alternative catheterizable channel. CONCLUSIONS: Continent vesicostomy can be performed successfully when there is any underlying bladder pathology with 100% stomal continence. Despite the higher rate of stomal problems with this type of Mitrofanoff channel we think that continent vesicostomy is a reasonable alternative in patients with a large bladder requiring only catheterizable channel creation. Because of the excellent results reported with the Monti-Yang technique, we would currently recommend this procedure over continent vesicostomy when bowel is used for bladder reconstruction.     

Continent urinary diversion and bladder augmentation in children: the Mainz pouch procedure

The formation of a bowel reservoir of large capacity at low pressure by using small and large bowel (ileocaecal segment) has proved reliable for achieving continent urinary diversion (n=80), for bladder augmentation (n=42) as well as for total bladder replacement (n=24). Encouraged by the results we obtained in our adult patients, we have used this technique during the last 3.5 years in 29 children. Indications for urinary diversions in children have been: neurogenic bladder with diplegia (n=8), bladder exstrophy (n=2), traumatic loss of the bladder (n=1), urogenital sinus (n=1) and rhabdomyosarcoma of the prostate or bladder (n=2). Bladder augmentation was indicated in 6 children with iatrogenic bladder loss, in 5 children with neurogenic bladder without diplegia and in 4 boys with incontinent epispadias or exstrophy. In children with bladder exstrophy or incontinent epispadias, continence was achieved using a modified Young-Dees technique with formation of a long intra-abdominal muscular tube made out of the bladder plate or the low-capacity bladder. The capacity of the urinary reservoir was guaranteed by bladder augmentation or bladder replacement with an ileocaecal pouch. During a mean follow-up period of 26 months (bladder augmentation) and 21 months (continent diversion) there was only one postoperative complication (intussusception ileus) which required operative revision. Two children had to undergo reoperation because of nipple problems. Follow-up, with monitoring of biochemical and metabolic parameters, is necessary to show whether this technique will provide a long-term successful solution for these problems.     

Surgical management of proboscoid herniae

The management of a large proboscoid umbilical hernia presents challenging problems to the surgeon. As children and parents are concerned with the appearance of the actual hernia they are also intensely interested in the postsurgical result. Presented is a simple one-stage umbilicoplasty that allows repair of the fascial defect and the management of the redundant skin in such a way as to produce a "scarless" and natural-appearing umbilicus.     

Influence of enterocystoplasty on linear growth in children with exstrophy

PURPOSE: Many children with bladder exstrophy undergo reconstruction incorporating bowel into the lower urinary tract, which may result in metabolic changes affecting height. Linear growth in children with exstrophy who underwent enterocystoplasty was studied. MATERIALS AND METHODS: We retrospectively reviewed the charts of children with classic bladder exstrophy who underwent bladder augmentation with small or large bowel, or creation of a bowel neobladder before age 11 years. Mean followup plus or minus standard error was 9.4 +/- 0.9 years. Most patients were assessed yearly with measurement of height and serum electrolytes. Metabolic acidosis was treated with oral bicarbonate. Preoperative height percentiles at enterocystoplasty were compared to height percentiles at the most recent visit. In addition, height percentiles in a control group of individuals with exstrophy who did not undergo enterocystoplasty were compared to postoperative height percentiles in the enterocystoplasty group. The t test was used for statistical analysis. RESULTS: Data on 18 children who underwent bladder reconstruction with bowel were compared with those on a control group of 18 with exstrophy. The mean preoperative height percentile at a mean age of 5.2 years was 35.6 +/- 4.5 and the postoperative height percentile at a mean age of 14.6 years was 20.3 +/- 5.7 (p <0.01). The mean height percentile in the control group at a mean age of 15.2 years was 30.6 +/- 7.8 (p <0.01 versus the postoperative height percentile in the study group. In the enterocystoplasty group 2 patients were receiving oral bicarbonate for metabolic acidosis. Five patients who underwent enterocystoplasty and 6 controls were below the third percentile for height. CONCLUSIONS: In children with bladder exstrophy bladder augmentation or neobladder creation may have an adverse effect on linear growth. The height of children with bladder exstrophy is less than average compared with standard growth charts.     

FAVORABLE EXPERIENCE WITH A SIMPLE TECHNIQUE TO CREATE A CONCEALED UMBILICAL STOMA

Purpose

After using different sites for continent urinary diversion stomas the umbilicus became the preferred site. Experience with a simple technique to place the continent diversion stoma at the umbilical site is reported.

Materials and Methods

Cutaneous continent urinary diversion was performed in 95 patients with placement of the stoma at the umbilical site. A triangular skin flap is elevated to the right of the umbilicus a 1 cm. piece of the umbilical scar is excised and the apex of the skin flap is anastomosed to the spatulated appendix, tapered ileum or gastric tube.

Results

All patients had satisfactory cosmetic results, 3 required simple revision and 4 needed dilation. These 7 patients as well as all others easily catheterized the urinary pouch.

Conclusions

This simple technique results in good cosmesis and it is associated with a minimal need for stomal revision.     

女性膀胱外翻诊治2例报告并文献复习

目的 报道2例女性膀胱外翻的诊治过程,提高对本病的认识.方法 病例1为先天性成人膀胱外翻,采用膀胱壁瓣重建尿道;病例2为外伤导致下腹部广泛皮肤及皮下组织缺如,破裂的膀胱裸露并外翻于腹壁,关闭膀胱后,采用大腿内侧带蒂股薄肌肌皮瓣覆盖下腹部创面.结果 病例1术后通畅排尿,且在术后6个月妊娠、生子,随访28个月,产后排尿、控尿均良好;病例2随访12个月,下腹部转移皮瓣成活,排尿状况良好.结论 成年女性膀胱外翻患者,采用膀胱壁瓣重建尿道是一种可行的方法,可获得良好的控尿效果;带蒂股薄肌肌皮瓣可用于下腹部皮肤缺损的覆盖,创伤小、效果好.     

Use of forearm free‐flap phalloplasty in bladder exstrophy adults

OBJECTIVE

To assess the long‐term outcome and cosmetic results of forearm free‐flap phalloplasty for correcting micropenis associated with bladder exstrophy in men.

PATIENTS AND METHODS

From 1992 to 2000, six men with bladder exstrophy and unsatisfactory penile appearance underwent phalloplasty at our institution. The mean (range) follow‐up was 113 (62–153) months. The surgical procedure consisted of a modified radial free‐flap phalloplasty and prosthesis implantation. One‐stage urethroplasty was electively performed according to the patient’s voiding pattern. Early and late complications were recorded and quality of life after phalloplasty was assessed.

RESULTS

The mean (range) age was 21.2 (17–26) years and flap survival was 100%. Two incontinent patients underwent immediate perineal urethrostomy. One sepsis occurred, requiring the removal of the penile prosthesis and a cutaneous continent urinary diversion; this patient was the only one to complain about the outcome. Two anastomotic urethral strictures were conservatively treated by endoscopic urethrotomy and only one patient underwent subsequent urethral self‐dilatations. Five patients were very satisfied with the cosmesis of the neophallus and three patients reported having regular sexual intercourse.

CONCLUSIONS

Although short, this series indicates the low incidence of early complications and the excellent long‐term satisfaction with forearm free‐flap phalloplasty in bladder exstrophy. Phalloplasty has potential indications when adult exstrophy patients are dissatisfied with the cosmetic appearance of their external genitalia or have unsatisfactory sexual intercourse.     

A simple and reliable continent bladder stoma constructed from bladder wall

Patients presenting with recurrent bladder neck strictures or severe incontinence refractory to all forms of infravesical treatment usually require chronic catheterization or supravesical urinary diversion. As an alternative, we use a simple continent bladder stoma made from the bladder wall. After extraperitoneal bladder mobilization, a Lapides-like 4×8 cm U-shaped bladder flap is excised and sutured to a tube. Depending on the location of the stoma, the base of the flap can be chosen and/or the length of the flap can be adapted. After bladder neck closure the tube is reflected and, after incision and dissection of the detrusor in the middle of the base of the flap, embedded in Lich-Gregoir's technique. The distal end of the tube is then sutured to the stoma site. Five of our patients now have a minimum follow-up of 11 months (mean 17 months) . All are continent with a bladder capacity between 250 and 560 ml. In two, stomal stenosis at the skin level required epifascial stomal revision.     

Treatment of incontinence in children with bladder exstrophy after rectal urinary diversion: the anal sling procedure

PURPOSE: An original technique for reinforcement of the anal sphincter using a sling prosthesis with a flexible fixing point that prevents incontinence and rectal prolapse is presented. MATERIALS AND METHODS: Of 43 patients with exstrophy who have undergone urinary rectal diversion between 1988 and 1997 incontinence was observed in 9 boys and 5 girls. From January 1993 to December 1998 these children were treated with an original method, the anal sling procedure. RESULTS: There were no postoperative complications. Investigations revealed no urinary or fecal incontinence after a mean followup of 3.2 years (range 6 months to 61/2 years). CONCLUSIONS: A short and simple operation eliminates incontinence after rectal urinary diversion in patients with bladder exstrophy. It can be used as an alternative to the formation of an isolated urinary reservoir.     

TRANSVERSE RETUBULARIZED SIGMOIDOVESICOSTOMY CONTINENT URINARY DIVERSION TO THE UMBILICUS

PURPOSE: The most widely used conduit when creating continent urinary diversion based on the Mitrofanoff principle has been appendicovesicostomy. However, appendix is not always available and it is increasingly used for the antegrade continence enema in situ appendix procedure. In 1993 the technique of transverse retubularization of the ileum to create a continent catheterizable conduit for an ileal reservoir was described and in 1997 this technique was studied in an animal model. Larger patients may need 2 ileal segments in series to bridge the distance between the umbilicus and bladder. To avoid using 2 segments we used transverse retubularized sigmoid colon to create a catheterizable sigmoidovesicostomy to the umbilicus. METHODS AND METHODS: In 5 children 6 to 19 years old (mean age 15) with neurogenic bladder secondary to spina bifida a transverse retubularized sigmoidovesicostomy to the umbilicus was performed. In all patients an antegrade continence enema procedure was done for refractory constipation and overflow fecal incontinence secondary to neurogenic bowel. Laparoscopy was performed to mobilize the appendix in patients who requested a low Pfannenstiel incision for better cosmesis. Additional procedures included the pubovaginal sling placement and sigmoid colocystoplasty. RESULTS: Mean length of the sigmoidovesicostomy, which was equal to the circumference of the sigmoid before retubularization, was 13 cm. (range 10 to 15). This sigmoid conduit reached the umbilicus easily in all cases. All sigmoidovesicostomies were easily catheterized and all were continent. One patient with morbid obesity (body mass index 40.4) had a mucocutaneous anastomotic breakdown 3 months postoperatively. Minimum followup was 1 year (mean 1.5). CONCLUSIONS: Transverse retubularized sigmoidovesicostomy is effective for creating a continent urinary diversion to the umbilicus in patients with neurogenic bladder secondary to spina bifida. Dilating the sigmoid colon from neurogenic bowel creates a long conduit based on the Yang-Monti principle and 2 ileal segments with an anastomosis are not required. Additional benefits are that the sigmoid colon is readily accessible via a low Pfannenstiel incision and may also be used for augmentation in select cases.     

Isolated vesicorectal internal urinary diversion: a 37-year review of the Boyce-Vest procedure

The challenges that management of exstrophy of the bladder pose the pediatric urological surgeon have resulted in a multitude of ingenious operations and a voluminous literature on exstrophy. Despite this intense interest in an uncommon anomaly, no consistently satisfactory approach to achieve urinary continence in children with exstrophy has evolved. We summarize a 37-year clinical experience with a form of urinary diversion that provides sphincteric urinary continence, and unexcelled long-term preservation of renal function and upper urinary anatomy. From our review it appears that the infant with exstrophy is served best by neonatal closure of the exstrophic bladder with attempts to establish urinary continence later in childhood. When other methods to establish urinary continence are unsuccessful and satisfactory anal continence has been demonstrated, a diverting colostomy combined with anastomosis of the isolated bladder to the isolated rectal stump offers an acceptable long-term alternate form of urinary diversion to produce sphincteric urinary continence with relative freedom from upper urinary deterioration.     

Continent cutaneous urinary diversion in children: experience with Charleston pouch I

PURPOSE: Complete continent urinary diversion not incorporating the bladder is not commonly used in children. We evaluated the short and long-term outcome of a form of continent cutaneous urinary diversion (Charleston pouch I) in children. MATERIALS AND METHODS: A total of 17 children underwent Charleston pouch I continent cutaneous urinary diversion between 1988 and 2005. Patient records were reviewed for age, sex, indications for diversion, preoperative and postoperative laboratory and radiological studies, continence, patient and family acceptance, complications and long-term functional status. RESULTS: Patient age ranged from 6 to 16 years. The main indication for diversion was bladder exstrophy in 8 patients (47%), neurogenic bladder in 6 (35%) and cloacal abnormalities in 3 (18%). Mean followup was 87.5 months. One patient was lost to followup. With moderate fluid intake the other patients were dry with a mean catheterizing time of 3.4 hours (range 2 to 6). Catheterization intervals were adjusted for individual patients. Generally, the patients became damp or leaked if they did not catheterize at recommended intervals. Continence was achieved at variable postoperative intervals, with some patients attaining continence soon after and others at 3 to 12 months before pouch maturation. Patients irrigated the pouch a mean of 4 times weekly (range 0 to 14). Three patients (18%) had bladder stones. Ultrasound and/or other upper tract studies revealed no deterioration of the upper urinary system. No patient experienced clinical pyelonephritis or acidosis. Family and patient acceptance was satisfactory. CONCLUSIONS: Continent cutaneous urinary diversion with Charleston pouch I was satisfactory in this group of children. It provided preservation of the upper urinary tract, and achieved acceptable continence rates while allowing leakage when catheterization was not performed at recommended intervals. In addition, patient and parent acceptance was good, and complication rates were acceptable.     

Continent cutaneous diversion for bladder exstrophy in adults

ObjectivesBladder exstrophy is a rare and devastating congenital anomaly. These patients are usually seen immediately after birth where correction can be attempted. Presentation in adulthood where bony fusion has occurred and bladder capacity is very low presents a major surgical challenge. Such patients require urinary diversion. This is a review of our initial experience with continent cutaneous urinary diversion in patient with classic bladder exstrophy who presented in adulthood.Patients and methodsWe reviewed the social and clinical characteristics, operative reports, and postoperative course of two female and five male patients who have been living with bladder exstrophy since birth. They underwent augmentation cystoplasty or simple cystectomy, bladder neck transection, repair of epispadias followed by a continent cutaneous diversion. The continent catheterizable outlet was constructed using the serous lined extramural tunnel technique as a continence mechanism. Postoperative continence, renal function, and changes in social life were documented.ResultsFollow-up has ranged from 17 months to 5 years in these initial patients. Thus far, all these patients have achieved complete daytime and night time continence, Complications consisted of pouch cutaneous fistula which was successfully repaired with interposition of a rectus abdominis flap and one case of urosepsis. All upper urinary tract imaging studies have shown stable function. All the patients are doing well with improved social interaction.ConclusionBladder neck closure in conjunction with continent cutaneous diversion is a reliable and safe method for achieving continence in adults presenting with bladder exstrophy. Total continence can be achieved without resorting to multiple complex and expensive surgeries.     

Laparoscopic excision of the urachus

Embryologically, the urachus represents the obliterated alantois in the form of a fibrous cord extending from the dome of the bladder to the umbilicus. Incomplete obliteration can result in the formation of an umbilical sinus, fistula, cyst, or abscess in the infraumbilical area. The traditional treatment of a residual urachus is surgical resection of the entire tract from the dome of the bladder to the umbilicus through a lower midline incision. We report the case of a 14-year-old girl who presented with an abscess below the umbilicus. After initial drainage, she developed a sinus that communicated with the navel. Surgical resection of the underlying urachus was carried out laparoscopically. The peritoneum was incised at the umbilicus, and the whole tract along with the skin sinus was excised using diathermy scissors. The lower end was ligated and the specimen retrieved. The patient was discharged the following day. One week later, her wounds had healed. Histology confirmed an epithelial-lined urachus. We believe that laparoscopic excision of a patent urachus is feasible and safe. This technique gives the surgeon good access to the area and has a cosmetically better result.     

Continent Gastric Tube: New Techniques and Long-Term Followup

Purpose

The successful use of stomach for bladder augmentation and substitution is well documented. Gastric tissue has been used more recently to create continent catheterizable tubes. We describe 2 new techniques of gastric tube construction, and report our long-term followup of catheterizable gastric tubes in children and adults undergoing complex urinary tract reconstruction.

Materials and Methods

A retrospective chart review of 6 male and 4 female patients 5 to 43 years old was done. Primary diagnoses included bladder exstrophy, cloacal exstrophy, rhabdomyosarcoma and neurogenic bladder. Five patients underwent gastrocystoplasty with simultaneous creation of a continent gastric tube from the anterior gastric flap. In 2 patients who had undergone previous gastrocystoplasty a continent gastric tube was created from an anterior flap raised from the existing gastric bladder. Isolated gastric tubes were constructed in 3 patients.

Results

Followup ranged from 2 to 9 years (median 3.5). All patients demonstrated easy reliable catheterization. One patient required revision of the proximal end of the tube for incontinence. At followup all tubes were continent. Complications occurred only in flush or protuberant stomas, and resolved after stomal revision with recessed skin flaps.

Conclusions

Several techniques can be used to create a continent gastric tube. Long-term followup reveals reliable catheterization and good continence rates. Recession of the gastric tube stoma with a skin flap prevents peristomal complications.     

Laparoscopic-assisted continent urinary diversion in obese patients

BACKGROUND AND PURPOSE: The Pfannenstiel incision provides good access to the bladder and bladder neck for major reconstructive surgery in the thin patient, whereas a midline incision is often necessary to get adequate exposure in the obese patient. We describe our experience using laparoscopic-assisted continent urinary diversion in conjunction with other bladder and bladder neck surgery in obese patients. PATIENTS AND METHODS: Three female patients (mean age 18; mean weight 175 pounds) with neurogenic bladder underwent Mitrofanoff appendicovesicostomy continent urinary diversion to the umbilicus and pubovaginal sling. An umbilical port for the telescope and two lateral ports were used. Once the appendix and right hemicolon had been completely mobilized up to the hepatic flexure, reconstruction was completed through a low Pfannenstiel incision. RESULTS: There were no laparoscopic complications. None of the laparoscopic port sites was visible postoperatively, as one was in the base of the umbilicus, and the other two had been incorporated into the Pfannenstiel incision. With a mean follow-up of 1 year, all patients were continent and catheterizing their umbilicus easily. Pfannenstiel incisions were well healed, and the patients were quite satisfied with their cosmesis. CONCLUSION: Laparoscopic-assisted Mitrofanoff appendicovesicostomy continent urinary diversion to the umbilicus can be performed in conjunction with a Pfannenstiel incision to complete major bladder and bladder neck surgery in the obese patient with good postoperative cosmesis. This procedure represents a nice compromise between a very lengthy bladder reconstructive procedure done purely laparoscopically and a midline incision with good exposure but suboptimal cosmesis.     

Nonrefluxing colon conduit for temporary or permanent urinary diversion in children.

Colon conduit, with nonrefluxing ureterosigmoid anastomoses, was performed upon 21 children in the past 3 1/2 yr for the following indications: (1) previously unoperated exstrophy of the bladder; (2) exstrophy of the bladder with prior ileal conduit; (3) previously unoperated neurogenic bladder; (4) neurogenic bladder with prior ileal conduit; and (5) with anterior pelvic exenteration for sarcoma of the prostate. In some of these patients the conduit will be a permanent diversion; in others it will be anastomosed later to the colon, providing a staged method of ureterosigmoid urinary diversion. Nine patients have undergone the second stage of anstomosis of the conduit to the colon with satisfactory outcome. Colon conduit diversion is a more time-consuming procedure than ileal loop diversion. It can require 6-8 hr in a patients with a prior ileal diversion, particualarly if the ureters require tapering. Our experience with this procedure leads us to the following conclusions: (1) this is a better method than ileal conduit for permanent diversion; (2) it offers an alternative way for treating patients with bladder exstrophy; (3) it is useful in patients with pelvic cancer; and (4) it should be considered for those patients with ileal conduits who are not doing well, i.e., who have infection and renal deterioration.