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PubMed2005

Anterior perineal reconstruction in exstrophy-epispadias complex.

OBJECTIVES: To assess the role of correct anatomical reconfiguration of the anterior perineal musculature in exstrophy-epispadias (E-E) patients. To stress the use of a bipolar stimulator to detect the perineal muscular complex intraoperatively, and to increase the functional results of reconstruction in E-E patients. METHODS: A total of 22 patients with E-E complex were treated in a 7-year period: 17 patients presenting classic bladder extrophy (aged 3 days to 6 years) and 5 incontinent male epispadias (aged 9 months to 16 years). An electric bipolar stimulator was used to identify and reapproximate at the midline the muscular fibers that constitute the periurethral muscular complex, as a part of the anterior perineal membrane. Outcome was evaluated at 24 months from surgery, considering bladder capacity, dry intervals, urinary infections (UTI's), upper tract deterioration and surgical complications (fistula, obstruction, dehiscence). Results were compared with a matched group of 19 E-E patients treated in the previous 5-year period, without the presented technique (control group). Student T-test was used for statistical analysis, considering p<or=0.05 as significant. RESULTS: No bladder neck or urethral dehiscence was observed. Mean bladder capacity at 2-year follow-up was 80 cc in the exstrophic patients and 120 cc in the male epispadias. Mean dry interval increased to 75 minutes in the exstrophy patients and to 130 minutes in the male epispadias. Full daytime continence was achieved in 3 exstrophic and in all the epispadic patients. The difference with the control group of patients was significant (p<0.05). CONCLUSIONS: Proper identification of the anterior perineal muscular complex, using a bipolar stimulator, and its reapproximation at the posterior urethra on the midline was demonstrated to be effective in increasing bladder cycling and in developing adequate bladder volume, anticipating coordinated micturition.

Adolescent↗
PubMed2004

Use of the silastic sheath in bladder neck reconstruction.

OBJECTIVE: The study compared two populations of patients undergoing bladder neck reconstruction using the silastic sheath in two major pediatric centers. The success with this technique was markedly different in the two centers. The purpose of the study was to determine factors that might explain the divergent results. PATIENTS AND METHODS: Fifteen patients treated in Indianapolis were compared with 94 patients treated in London with the silastic sheath technique of bladder neck reconstruction. Eighty-seven percent of the Indianapolis patients had myelomeningocele whereas 86% of the London group had exstrophy/epispadias. Median age of the Indianapolis patients was 11 years whereas it was 8.4 years in London. Seventy-three percent of patients in Indianapolis were female and 79% in London were male. Patients were followed for a minimum of eight years in Indianapolis and a mean of seven years in London. Similar surgical technique was employed in the two centers but, over time, the London approach included use of a non-reinforced silastic wrapped loosely around the bladder neck with the interposition of omentum. RESULTS: Both groups achieved continence rates exceeding 90%. Of the Indianapolis patients, two-thirds experienced erosion of the silastic at a mean of 48 months. With modifications in the London technique, the erosion rate of silastic was lowered from 100% to 7%. CONCLUSION: Direct, snug wrap of silastic without omentum around the Young-Dees tube as well as simultaneous bladder augmentation placed patients at increased risk for erosion. The silastic sheath technique may be less applicable to myelomeningocele patients. It seems most applicable to older male patients with exstrophy or epispadias undergoing Young-Dees bladder neck reconstruction who have the ability to void.

Child↗
PubMed1997

Current urologic management of cloacal exstrophy: experience with 11 patients.

PURPOSE: Since 1980 the authors have treated 12 infants with cloacal exstrophy (10 classical and 2 variants). Eleven patients had repair, and are all surviving. The initial phases of management that led to improved survival have previously been reported. Quality of life is now a major focus for the cloacal exstrophy patient. During the past 10 years, nine of the 11 patients had lower urinary tract reconstructive procedures. This review evaluates experience with reconstructive efforts to achieve bowel and bladder control and to improve the quality of life in this complex group of patients. METHODS: Through review of patient charts and by patient interviews, data were collected to evaluate the ability to provide urinary and bowel control. A continence score was applied to provide a measure of success: voluntary control, 3; control with an enema program or intermittent catheterization, 2; incontinence with a well-functioning stoma, 1; and incontinence without a stoma, 0. The best continence score is 6 (genitourinary and gastrointestinal). Surgical complications, urodynamic and metabolic sequelae of continent urinary diversion were reviewed. RESULTS: At the time of the authors' previous report, eight of 11 patients had a continence score of 2 or less. Currently, eight of 11 patients have a score of 3 or better (five with enteric stoma and continent urinary diversion, two with enema program and continent urinary diversion, and one with enema program and continent bladder). Urinary-diversion procedures have included two gastric augmentations and five gastric reservoirs, two of which have required subsequent bowel augmentation. Gastric augmentations carry a definite risk of metabolic problems with three of our patients demonstrating significant episodes of metabolic alkalosis. In addition, results of urodynamic monitoring suggests that gastric reservoirs may be less compliant than reservoirs formed using other bowel segments. CONCLUSIONS: Modern principles of continent urinary diversion have been successfully applied to the cloacal exstrophy patient further improving their quality of life. Use of gastric flaps with preservation of intestinal length has been central to urologic reconstructive efforts. Use of stomach alone for formation of urinary reservoirs may produce suboptimal compliance, and composite ileogastric construction should be considered if the gastric flap is of marginal size.

Bladder Exstrophy↗
PubMed1992

Cloacal exstrophy: prenatal diagnosis before rupture of the cloacal membrane.

Embryologically, cloacal exstrophy is thought to result from persistence and subsequent rupture of the infraumbilical cloacal membrane during the fifth embryonic week. We report a case of cloacal exstrophy in which a prenatal diagnosis was made prior to rupture of the cloacal membrane. A routine ultrasound at 17 weeks' gestation demonstrated monoamniotic twins. One twin was normal, but the other was found to have a sacral myelomeningocele, "rocker-bottom" feet, splaying of the pubic rami, and a large cystic mass protruding from the infraumbilical anterior abdominal wall. A repeat ultrasound was performed at 22 weeks, with the same findings. At 26 weeks, further examination showed disappearance of the abdominal cyst, a small omphalocele, no demonstrable bladder, and the suggestion of prolapsed bowel inferior to the umbilical cord insertion. After delivery at 34 weeks, the abnormal twin was found to have the typical findings of cloacal exstrophy, myelomeningocele, bilateral lower limb anomalies, and extremely foreshortened small bowel. Rupture of the presumed cloacal membrane after 22 weeks in this case is inconsistent with our current understanding of the embryology of this anomaly, and should stimulate a reexamination of the current concepts. If the characteristic features are recognized, cloacal exstrophy can be diagnosed by prenatal ultrasound, permitting prenatal counseling and appropriate perinatal management.

Abnormalities, Multiple↗
PubMed1990

Success of the artificial urinary sphincter after failed surgery for incontinence.

We implanted the artificial urinary sphincter in 15 incontinent patients for whom multiple urethral and bladder neck operations, including sphincter placement, had been unsuccessful. The 5 male and 10 female patients ranged from 3 to 26 years old (mean age 11 years). The etiology of incontinence was neurogenic bladder in 10 patients, epispadias in 2, exstrophy in 1, ectopic ureters in 1 and traumatic urethral disruption in 1. Of the 15 patients 13 required augmentation enterocystoplasty and clean intermittent catheterization. The initial anti-incontinence procedures were Young-Dees-Leadbetter bladder neck reconstruction in 10 patients, artificial urinary sphincter placement in 4 and bladder neck suspension in 1. Causes of failure of the primary treatment were erosion (artificial urinary sphincter), and incontinence and/or difficult catheterization (Young-Dees-Leadbetter). Followup from the last salvage operation averaged 21 months (range 6 to 37 months). A total of 58 operations was performed. Among the 4 patients in whom the artificial urinary sphincter eroded the bladder neck repeated attempts to place the cuff at the same site were unsuccessful and erosion occurred in all 4 within 1 year. Sphincter placement was more successful among the 11 patients who initially underwent Young-Dees-Leadbetter bladder neck reconstruction or bladder neck suspension; acceptable continence was attained in 8 patients (73%). We conclude that placement of the sphincter cuff around a previously eroded bladder neck probably will result in erosion. Sphincter implantation should be attempted in patients in whom bladder neck reconstruction has failed. Persistence in the treatment of these patients is essential because multiple operations often are necessary to achieve continence.

Adolescent↗
PubMed1988

Use of the rectus abdominis muscle and fascia flap in reconstruction of epispadias/exstrophy.

Inferiorly based rectus abdominis muscle flaps and fascial flaps have been used to construct a firm abdominal wall without hernias and to provide coverage of the bladder, bladder neck, and proximal urethra in the secondary reconstruction of patients with epispadias/exstrophy complex. They have also been used to produce an elevation of the mons area, which is lacking in the typical exstrophy patient. Rectus fascial flaps have been the mainstay of abdominal closure when wide diastasis of the rectal muscles is present and when the lower abdomen lacks fascial support. We are pleased with the results of utilizing either the rectus muscle or rectus fascia in this complex condition. We have been using fascial flaps for over 10 years in our epispadias/exstrophy closures and abdominal wall strengthening procedures. We have been using bone grafts and the rectus muscle for the construction of a mons for the past 5 years. Although the rectus muscle procedure to cover the bladder and the bladder neck has been in use for only 2 years, we have seen patients with incontinence restored to a continent state; these patients have not been plagued with fistula problems when a neourethra has to be constructed to elongate the phallus. Previous attempts at urethral repair in this area have been successful in the main, but an occasional fistula at the junction between the penile skin and the abdominopubic skin has resulted. Since using the rectus muscle to cover the bladder and bladder neck area, we have not had this problem. Patients with epispadias/exstrophy remain such difficult problems that every advance in technique should be used to improve results.(ABSTRACT TRUNCATED AT 250 WORDS)

Bladder Exstrophy↗
PubMed1989

Neuroanatomy of the pelvis in an infant with cloacal exstrophy: a detailed microdissection with histology.

The internal anatomy of the pelvis in the patient with cloacal exstrophy has not been described in detail previously. We present in detail the pelvic anatomical relationships of a patient with cloacal exstrophy who died 72 days after birth and whose body subsequently was perfused with a formalin-based anatomical fluid. Microdissection of the pelvis with histological confirmation of the identity of structures dissected was performed. The vascular supply to the urinary hemibladders arises from the internal iliac arteries, which pass along the lateral portion of the pelvis and enter each hemibladder. The autonomic innervation to the bladder and corporeal bodies arises from a pelvic plexus that lies on the anterior surface of the sacrum. The innervation to the hemibladders then travels in the midline along the posteroinferior surface of the pelvis before extending laterally to communicate with each hemibladder. Autonomic innervation to the duplicated corporeal bodies arises from the sacral pelvic plexus and travels in the midline to pierce the inferior portion of the pelvic floor posterior and medial to the hemibladders. These nerves then enter the crural region of the corpora. Sensory innervation to the corporeal bodies arises from the sacral trunk and passes posterior to the pelvic floor muscles, traveling just medial to the widely separated ischial spines and then laterally along the corporeal bodies. Illustrations of the anatomy and implications for management of the exstrophy patient are presented.

Abnormalities, Multiple↗
PubMed1987

Pediatric urinary diversion and undiversion.

Many things have changed in our management of the urinary tract of children and young adults. Important contributions from many sources have resulted in the refinement of therapy and the progression from diversion to undiversion and reconstruction. Primary urinary diversion is now uncommon. Patients with exstrophy, for example, are treated with primary bladder closure as newborns, and newborns with valves often are treated with primary valve ablation. Children with neurogenic bladder dysfunction resulting from myelodysplasia are rarely diverted, but are started at an early age with ICC. It is hoped that in future the contents of this article on diversion techniques and undiversion will be of historical interest, however. Note: The editors also have found the use of the Mitrofanoff procedure, utilizing a nonrefluxing tunnel and a catheterizable stoma made out of the ureter or appendix, to be a very valuable addition to the reconstructive surgeon's armamentarium. We also feel that use of detubularized bowel for either bladder augmentation or replacement provides more efficient storage capability. Occasional reports of extremely significant diarrhea after removal of the ileocecal segment from the intestinal tract have been reported in children with myelodysplasia.

Adolescent↗
PubMed2001

Complete diphallia associated with features of covered exstrophy.

This report describes a 1-day-old boy with complete true diphallia associated with features of covered or pseudo-exstrophy and an anorectal malformation. The urinary bladder and rectum were single. A divided sigmoid colostomy was constructed at birth for the anorectal anomaly. Complete diphallia and its likely embryogenesis are discussed.

Anal Canal↗
PubMed1978

Embryology of the lower genitourinary tract.

The urinary and genital systems are closely related in their development so that dividing them into separate divisions is often artificial. The lower urinary system begins as a cloaca. The urorectal septum then divides the cloaca into a ventral urogenital sinus and a dorsal hindgut. The ureteral bud forms off the mesonephric duct. The male and female genital systems are the same until the gonad assumes characteristics of the testis or ovary in the eighth week of gestation. It is not until the eleventh or twelfth week that the external genitalia assume specific sexual characteristics. Anomalies of the urethra and bladder can vary from a severe epispadias-exstrophy complex to a minimal Type I urethral valve. Ureteral anomalies include duplications, ectopia, and ureteroceles. An undescended testis is probably the most common anomaly in the male genital tract. Female genital anomalies can include a variety of uterine duplication anomalies. Gonadal abnormalities are often created by a chromosomal defect and are ultimately reflected by abnormal sexual differentiation.

Cloaca↗
PubMed2001

Neoplasia in adult exstrophy patients.

OBJECTIVE: To document the incidence of neoplasia in a cohort of 103 patients born with classical exstrophy. PATIENTS AND METHODS: The notes of patients born before 1964 with exstrophy were reviewed retrospectively. The patients were divided into two groups; 42 were thought to be at high risk of developing neoplasia because they had (at some time) had mixing of urine and faeces in a colorectal reservoir, whereas 61 had never been exposed to such a mixture and were thought to have a low risk of neoplasia. RESULTS: At a minimum of 35 years of follow-up, complete data were available for 61 patients; 42 were lost to follow-up, of whom 14 were at high risk and 28 at low risk of neoplasia. In the high-risk group, there were three with colonic carcinoma (two of whom presented before 1980 and died), one with carcinoma in situ of the colon, 10 with benign colonic neoplasms and three with bladder cancer (two of whom died). In the low-risk group, there was one patient with bladder cancer (who died) and one with a clear cell carcinoma of the kidney. Three of the four patients with bladder cancer had undergone cystectomy before 5 years of age. Assuming that all the lost patients are alive and free of neoplasia, the risk of neoplasia in adults born with exstrophy is 17.5%. The main risk is in those who have been exposed to mixing of urine and faeces in a colorectal reservoir (38%). Even in low-risk patients the risk of malignant neoplasia is 3.3% at a median (range) age of 42 (40-44) years, which is 27 times higher than that of the age-matched general population. CONCLUSIONS: Annual colonoscopy of patients deemed at high risk of colorectal neoplasia appears to be an effective screen for colorectal carcinoma, by identifying a premalignant stage, as there were no deaths after this was introduced. Despite bladder closure or diversion surgery within the first few years of life, patients with exstrophy have an almost 700-fold greater incidence of carcinoma of the bladder than the age-matched general population. Early cystectomy is not protective.

Adult↗
PubMed1988

Gastrocystoplasty: an alternative solution to the problem of urological reconstruction in the severely compromised patient.

An isolated segment of stomach was used for bladder augmentation in 10 patients or construction of a continent urinary reservoir in 3. Diagnosis in these 13 patients included cloacal exstrophy (5), myelodysplasia (4), posterior urethral valves (2), radiation cystitis (1) and neurogenic bladder secondary to a rectal pull-through procedure (1). Indications for the use of stomach in bladder reconstruction were decreased renal function and acidosis (6 patients), insufficient large and small bowel (6) and decreased mucus production (1). Postoperative followup averaged 13 months (range 6 to 23 months). All patients have stable upper tracts radiographically and stable or improved renal function. Of 13 patients 10 require intermittent clean catheterization to empty and 11 are completely continent. Nine patients have remained free of infection, while 4 had asymptomatic bacteriuria. Mucus production is reduced relative to other intestinal segments and 10 patients require no bladder irrigations. Postoperative urodynamic evaluation is similar to that of ileocystoplasty or colocystoplasty. Use of stomach has protected these patients from the development of new or worsened hyperchloremic acidosis. Serum chloride values have decreased and serum total carbon dioxide values have increased after bladder reconstruction, particularly in patients with impaired renal function. Stomach should be considered when lower urinary tract reconstruction is necessary in such compromised patients.

Adolescent↗
PubMed2000

A case of female epispadias without exstrophy treated with a novel vulvoplasty method.

Epispadias surgery has mainly two components: bladder and genitourethral reconstruction. Herein, a novel vulvoplasty method, an alternative for the latter component, carried out in a rare case of female epispadias was presented. In a 6-year-old girl admitted with complaint of total incontinence, epispadias without evident extrophy associated with adequate bladder capacity was determined. Firstly the vulvoplasty in which a circumferencial skin flap around the urethral meatus was rolled backwards, and secondly bladder neck reconstruction was performed. The vulvoplasty resulted in sufficient cosmetic outcome and continence up to half an hour, and after cervicoplasty total day and night continence at rest or on exertion were obtained. It was concluded that this vulvoplasty with sufficient cosmetic outcome could be considered as a first stage operation in female epispadias, because it could facilitate bladder neck reconstruction by increasing urethral resistance, and contribute to continence.

Child↗
PubMed1998

Cloaca, the most severe degree of imperforate anus: experience with 195 cases.

OBJECTIVE: To provide a follow-up of 195 patients with cloacal malformations seen by the author from 1959 to 1998. SUMMARY BACKGROUND DATA: Cloaca, which occurs in approximately 1 of 50,000 births, is the most complex type of imperforate anus with confluence of the rectum, vagina, and bladder in a urogenital sinus. Functional results for the bowel, the genital tract, and the urinary tract were formerly poor. Cloacal exstrophy, which is an even more complex spectrum of malformations, was uniformly fatal until 1960. In addition to imperforate anus, these babies have an omphalocele, two exstrophic bladders, between which there is an open cecum, and a blindly ending colon hanging down in the pelvis from the cecum. Although both of these diagnoses contain the word "cloaca," which is Latin for sewer, they are really two separate entities in terms of surgical management. Cloaca and cloacal exstrophy in most cases are very different anatomic problems. However, there are variants that are like a hybrid, which is the rationale for reporting together an experience with both entities. METHODS: Records were reviewed of 154 patients with cloaca and 41 patients with cloacal exstrophy to assess anorectal function, urinary continence, and sexual function where available. RESULTS: Follow-up was available in 141 cloaca patients: 82 have spontaneous bowel movements and satisfactory control, 38 use enemas to evacuate, 9 have a colostomy, 7 have fecal soiling, and 5 are too recently operated to evaluate. Regarding urinary control, 83 void spontaneously, 40 catheterize to empty, 4 have urinary diversion, 1 has a continent diversion, 5 patients are wet, and 8 are too recently operated to judge. Twenty-four patients are now adults, 17 of who have experienced coitus and 7 have not. Seven have had babies, all except one by cesarean section. Results of surgery for cloacal exstrophy are not as good, but are encouraging nonetheless for an anomaly that was uniformly fatal before 1960. Of the 41 cloacal exstrophy patients being followed, 7 have not undergone surgery. Fifteen have a colostomy; 19 had pull-through of the colon, but 3 were subsequently reversed for fecal incontinence. Most depend on enemas to evacuate. Urinary dryness was attained in 30 patients, usually by intermittent catheterization of the bladder, which was augmented with small bowel or stomach or both. Only three void voluntarily. Fifteen of the completed long-term patients wear no bag. Only three of the completed patients wear two bags. The rest have one bag. CONCLUSIONS: Imperforate anus and associated malformations in cloaca and cloacal exstrophy are not hopeless problems. A reasonable lifestyle can be achieved for most of these children with comprehensive surgical planning.

Anus, Imperforate↗
PubMed2003

Pregnancy after lower urinary tract reconstruction for congenital abnormalities.

OBJECTIVE: To evaluate the effect of pregnancy on renal function, and the effect of congenital urinary tract abnormality and reconstruction on pregnancy and delivery. PATIENTS AND METHODS: The case notes were reviewed of 20 women (median age 32.5 years) who had had 29 live babies. Data collected included patient demographics, congenital urological abnormality, urological reconstructive procedure(s) and any subsequent urological complications. Pregnancy details, including urological and obstetric complications, presentation and mode of delivery, were obtained via a postal questionnaire from the relevant obstetrician. RESULTS: Seven patients had exstrophy-epispadias, seven spinal dysraphism, two sacral agenesis, and one each cerebral palsy, epispadias, imperforate anus and small bladder with vesico-ureteric reflux and congenital incontinence. They had had a mean (range) of 5.7 (1-12) urological reconstructive procedures each. Patients with exstrophy-epispadias had significantly more operations (mean 7.8) than those with spinal dysraphism (mean 4.14) or other diagnoses (mean 2.6) (P < 0.01). At the last follow-up 13 patients had an enterocystoplasty, six a neobladder and one an ileal conduit. Pregnancy-related urological complications were urinary tract infection in 15, upper tract obstruction requiring nephrostomy and stent in three, Mitrofanoff difficulties in two and pyelonephritis in one. There was no significant deterioration in glomerular filtration rate or serum creatinine after pregnancy. Only 10 of the births were normal or assisted vaginal deliveries. Seven patients had emergency and 12 had elective Caesarean sections for obstetric indications, including four breech births in the seven patients with vesical exstrophy. CONCLUSIONS: Pregnancy has no long-term effect on renal function and does not compromise reconstruction. Although there is a substantial complication rate and an increased need for Caesarean section, pregnancy in women with lower urinary tract reconstruction for congenital urological abnormalities is ultimately safe for both mother and baby. Interdisciplinary co-operation is desirable for a successful outcome.

Adult↗
PubMed1990

Cloacal exstrophy reconstruction for urinary continence.

From 1978 to 1989 we treated 16 patients with cloacal exstrophy, including 8 who have been treated since birth. To achieve urinary continence bladder reconstruction was required in 12 patients, including 3 ileocystoplasties, 4 gastrocystoplasties and 5 gastric continent urinary reservoirs. All 12 patients underwent simultaneous bladder neck/urethral continence procedures. Of the 12 patients with completed reconstruction 10 were evaluable for continence. All 10 patients are dry for periods of 3 or more hours, and 5 are totally dry day and night. All but 1 patient are totally dependent on clean intermittent catheterization. Followup ranges from 2 to 66 months, and all except 1 patient have stable upper tract function. Patients with cloacal exstrophy can undergo reconstruction in a fashion to maintain urinary continence, and stomach is the ideal tissue for such reconstruction.

Adolescent↗
PubMed1995

Cloacal exstrophy sequence: an exceptional ultrasound diagnosis.

OBJECTIVE: To establish sonographic criteria for an early prenatal diagnosis of cloacal exstrophy sequence, an extremely rare congenital malformation. METHODS: We assessed all cases of anterior midline abdominal wall defects occurring between November 1, 1986, and May 31, 1993. The charts of those with cloacal exstrophy sequence were reviewed thoroughly. The sonographic findings in each case of cloacal exstrophy sequence were evaluated and compared with the ultrasonic manifestations in other types of anterior abdominal wall defects. RESULTS: During the study period, six cases of cloacal exstrophy were diagnosed by ultrasound and confirmed by postnatal autopsy examination. Two of the cases were in different twin gestations. There were 58,288 deliveries during the study period; 665 (1.14%) major malformations were diagnosed ultrasonographically. Identical specific sonographic signs appeared in all six cases of cloacal exstrophy. These included a large infraumbilical, anterior midline defect, a protruding large omphalocele, an absent bladder, a narrowed thorax, a distorted spine, a large sacral meningomyelocele, and bilateral clubfeet. In all cases, the fetal bowel was floating in a large amount of ascites within the omphalocele sac. Polyhydramnios was present in four of the six cases. CONCLUSION: Based on our sonographic criteria, we believe that an accurate prenatal diagnosis of cloacal exstrophy sequence is feasible, even in the first trimester of pregnancy. These signs distinguish cloacal exstrophy from other types of midline anterior abdominal wall defects, ie, gastroschisis, amniotic band syndrome, and limb-body wall complex (body-stalk syndrome).

Adult↗
PubMed2000

Cloacal exstrophy: a unified management plan.

BACKGROUND/PURPOSE: The belief that patients with cloacal exstrophy have a short and therefore useless colon is all too common. Frequently, the colon is used for urinary or vaginal reconstruction, and the possibility of a pull-through is lost. In the authors' experience, the use of a unified management plan allowed most patients to undergo pull-through and avoid a permanent stoma. METHODS: Twenty-five patients were treated for cloacal exstrophy in the authors' institution from 1985 through 1999. In all patients, bladder closure, omphalocele repair, and creation of a colostomy were performed at birth. All available colon, no matter how small, was incorporated into the fecal stream. After at least 1 year, patients were assessed for the ability to form solid stool through their stoma. Normal colonic length, capacity to form solid stool, or success with a bowel management regimen through the stoma were considered indications for pull-through. Genitourinary reconstruction was contingent on the colorectal plan. RESULTS: Colonic length ranged from normal in 12 patients, 40 to 70 cm in 3 patients, 10 to 30 cm in 4 patients, and less than 10 cm in 2 patients. All 25 patients underwent pull-through. Three are totally continent, 4 are continent with occasional soiling, 11 remain clean with a bowel management regimen, and 4 are too young to assess. One patient was clean, but now refuses bowel management. Two early patients, both with less than 10 cm of colon, now have ileostomies. CONCLUSIONS: During neonatal repair, a colostomy should be formed incorporating all pieces of colon, no matter how small. With time, most patients will be able to form solid stool, and a pull-through should be undertaken if that ability exists. Decisions regarding genitourinary reconstruction should be made only after the gastrointestinal plan is established to achieve the optimal use of available bowel.

Adolescent↗