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PubMed1986

[Results of bladder and urethra reconstruction for exstrophy].

Sixty-one patients with classical bladder exstrophy were seen at our institution over the last twenty-two-years. Five patients underwent primary urinary diversion, in six reconstruction according Couvelaire--Arap method was attempted without success. For fifty patients a staged approach to functional closure was elected. A review of the success, failures and complications provides the bases for treatment now being used on new patients. The technical aspects of this treatment that provide the best chance for continence without renal damage and satisfactory external genitalia are discussed. An excellent surgical result, defined as achievement of a day time dry interval for 3 hours with normal social life and without renal damage was achieved in 74% of children (100% of girls).

Bladder Exstrophy↗

PubMed1996

Use of the expanded thoracoepigastric myocutaneous flap in the closure of cloacal exstrophy.

We describe the first reported use of an expanded thoracoepigastric myocutaneous flap in the closure of cloacal exstrophy. This approach offers several distinct advantages. The expander increases the available cutaneous surface area of the thoracoepigastric region, improves vascularity, induces a fibrous capsule that augments the abdominal wall, permits primary closure, and avoids prosthetic adjuncts that increase scarring and hinder delayed urinary tract reconstruction. Osteotomy and spica casting may be obviated by using this flap, but mesh may be required eventually. We anticipate its use in all future cases in which the abdomen cannot be closed safely at the primary procedure at this institution. This technique also should be considered for classic bladder exstrophy or any other large congenital or acquired defect of the lower abdomen.

Abdominal Muscles↗

PubMed1995

Severe case of Al Awadi/Raas-Rothschild syndrome or new, possibly autosomal recessive facio-skeleto-genital syndrome.

A Sicilian girl whose parents were first cousins had a severe tetramelic limb deficiency (amelia of lower limbs, peromelia of upper limbs) and other defects including cleft lip and palate, facial anomalies, athelia, low umbilicus, bladder exstrophy, no external genitalia, and anteriorly displaced anus. This probably represents a particularly severe case of Al Awadi/Raas-Rothschild syndrome (limb/pelvis-hypoplasia/aplasia syndrome, LPHAS), but the possibility of a new autosomal recessive facio-skeleto-genital syndrome cannot be excluded.

Abnormalities, Multiple↗

PubMed1991

Superior vesical fistula: a case report.

Variants of bladder exstrophy are rare developmental abnormalities. Only a few cases have been reported in the literature to date. A case of superior vesical fistula with split symphysis and dorsal chordee is presented. If associated anomalies are absent, diagnosis and management of these lesions are easy and have a good prognosis. The patient is usually continent after surgical correction.

Child, Preschool↗

PubMed1993

Appendicovesicostomy (and variations) in bladder reconstruction.

Bladder reconstruction using bowel segments was advanced when intermittent catheterization proved so acceptable. Access to the reservoir by way of the urethra is often not possible in children, especially boys. Implantation of the isolated appendix into the bladder remnant or colon segment similar to a ureteral reimplant provides a continence mechanism with ready catheter access (the Mitrofanoff principle). Since 1982 we have applied this principle in 41 pediatric cases of bladder reconstruction (64% boys). Primary diagnoses included bladder exstrophy (46%) and myelomeningocele (34%). Extending the concept of a flap valve continence channel with a narrow tube into the reservoir, segments of tapered ileum and ureters were also used (appendix 61%, ileum 12%, ureter 27%). Results of continence (100%) and uncomplicated catheterization (93%) have been satisfactory. Unfortunately, the longer the experience (average 3.2 years of followup), the more stone formation we experienced (32%), which is due to mucus production and bacteriuria as the stone nidus. There were 2 deaths, including 1 from a renal stone obstructing the ureter with sepsis and 1 a likely suicide. Reoperation was required for 3 stomal revisions, 2 bowel obstructions and 7 stone removals in 3 of the 6 cases in which the bladder neck was closed. Unique aspects of these reconstructions are presented, such as our current preference to bury the stoma in the umbilicus (7 cases), placement of the ureteral segment into the perineum as a neourethra and an unusual conjoined twin reconstruction.

Adolescent↗

PubMed1995

[Uretero-colonic implantation in children. Late results].

An uretereo-colic anastomosis in children is performed in three occasions: 1) The trans-intestinal cutaneous ureterostomy (Bricker) has a low rate of complications concerning ureterocolic anastomosis. Nevertheless, occurrence of pyelonephrites may oblige to perform an anti-reflux procedure. 2) Implantation of ureters in the intestinal segment of an augmentation colocystoplastys in unusual; when necessary, this type of implantation is safe because colon is appropriate for an antireflux procedure. 3) Ureterocolic diversion (Coffey) is quite comfortable for patients. Risks of renal failure and of adenocarcinoma are minimal thanks to an annual control. Concerning exstrophy of bladder, it seems now that risk of infertility is lower for patients with an ureterocolic diversion than those with a genito-urinary reconstruction.

Acid-Base Imbalance↗

PubMed1997

Helicobacter pylori infection in a child with gastric augmentation.

The existence of Helicobacter pylori (HP) infection in those children who had previous bladder augmentation with gastric patch has not been described before. In this report, a girl with bladder exstrophy, who previously underwent gastric augmentation is presented. She had multiple admissions to our unit with persisting urinary symptoms (perineal pain, dysuria, hematuria) and gastric symptoms as well. Enzyme-linked immunosorbent assay (ELISA) test results for HP infection were positive, and plasma gastrin level was high. The histopathologic examination of the biopsy specimen showed HP infection in the gastric part of the reconstructed neobladder. This report indicates that the pediatric surgeon should think about HP infection in gastrocystoplasty patients to eliminate the potential risks of HP colonization.

Female↗

PubMed1985

Epispadias: contending with continence.

Forty-seven patients with epispadias not associated with bladder exstrophy were treated at our institution over the last half decade. There were 8 female and 39 male patients, 37 of whom had retrosymphyseal epispadias. Of the 20 male patients with subsymphyseal epispadias and incontinence 13 had a fair to excellent result. Of the 7 females with retrosymphyseal epispadias and incontinence, all but 1 came to urinary diversion. An analysis of our data suggests that premature operative intervention precludes obtaining maximal functional and cosmetic results. In addition, the discrepancy in our overall success rate utilizing a one-stage technique versus a staged procedure favored by others may support the latter.

Adolescent↗

PubMed2006

Dynamic magnetic resonance imaging of the pelvic floor in children and adolescents with vesical and anorectal malformations.

BACKGROUND: Magnetic resonance imaging (MRI) of the pelvic floor allows better assessment of pelvic pathology and has a potential as an adjunct for therapy planning. In complex congenital malformations of the pelvic floor and continence organs, it plays a major role in assessing urinary and fecal incontinence or constipation, especially when performed as a dynamic investigation such as MRI defecography. PATIENTS AND METHODS: Twenty-three patients (3-21 years old) with urinary and/or fecal incontinence or constipation attributable to congenital malformations of the pelvic region presented at our institution. The diagnoses were anorectal malformations (18), bladder exstrophy (2), and cloacal exstrophy (3). All patients underwent static and dynamic MRI of the pelvic floor with rest, squeeze, and evacuation in supine position. RESULTS: Morphology and function of the pelvic floor and pelvic organs could be demonstrated in each case. The reason for urinary incontinence, fecal incontinence, or constipation could be defined, and an individual therapeutic management concept was made based upon the data obtained by the investigation. CONCLUSIONS: The advantages of this method, in comparison to others, are direct visualization of the pelvic floor muscles and continence organs and their anatomical relationship during different functional actions. Pelvic floor dysfunction is often the reason for fecal and urinary incontinence and can be detected by MRI. Especially in children, minimizing radiation exposure is of major concern. Disadvantages are the costs and long investigation time.

Adolescent↗

PubMed1996

Colonic conduit in children: protection of the upper urinary tract 16 years later?

PURPOSE: Complication rates of up to 86.6% have been reported after creation of an ileal conduit. In contrast to others, we construct an antirefluxing isoperistaltic colonic for incontinent diversion in children. MATERIALS AND METHODS: Between 1968 and 1989 a colonic conduit was created in 105 patients up to age 20 years for neurogenic bladder (76), bladder exstrophy (16) and other reasons (13). Long-term followup (minimum 5 years, mean 16.3 years, range 5 to 26) was possible in 84 patients (159 renal units). RESULTS: Early and late stenosis at the ureterocolic anastomosis, and stomal stenosis occurred in 7.6 and 15.5% of the cases, respectively. Renal calculi developed in 8.2% of the renal units. Eight kidneys without function after recurrent pyelonephritis, calculi or stenosis at the ureterocolic anastomosis were removed during followup. Compared to preoperative status 3 of the remaining 151 renal units had increased dilatation of the upper urinary tract at the last followup. A total of 31 patients had complications during followup and 18 underwent conversion to continent urinary diversion. CONCLUSIONS: When incontinent urinary diversion is necessary in children, a colonic conduit with the option of continent conversion should be created before deterioration of the upper urinary tract.

Adolescent↗

PubMed1996

Outcome of sling cystourethropexy in the pediatric population: a critical review.

PURPOSE: We performed a critical retrospective analysis of the records of all consecutive children who underwent sling cystourethropexy at our institutions. We particularly assessed the incidence and outcome in children who underwent enterocystoplasty in addition to a sling procedure. MATERIALS AND METHODS: Between May 1992 and August 1994, 24 girls and 15 boys 4 to 17 years old (mean age 9) consecutively underwent 1 of 4 techniques of sling cystourethropexy for stress urinary incontinence using rectus fascia via an abdominal approach. Diagnoses included spinal dysraphism in 34 patients, spinal trauma in 2, cecoureterocele in 1, epispadias in 1 and classic bladder exstrophy in 1. All children underwent preoperative video urodynamics. In 4 patients only a sling procedure was performed (group 1), while in 9 others a sling procedure was performed after enterocystoplasty (group 2). In 26 patients a sling procedure and concomitant enterocystoplasty were performed (group 3). RESULTS: Patients who underwent concomitant enterocystoplasty had more hyperactive preoperative urodynamics than those who did not. Postoperative continence was subcategorized in terms of patient age, sex, diagnosis (neurogenic versus nonneurogenic), preoperative video-urodynamics, surgeon technique, group (that is with or without cystoplasty) and type of enterocystoplasty (that is stomach versus ileum). Of these factors only concomitant enterocystoplasty was predictive of postoperative dry continence (73% of group 3, 33% of group 2 and 25% of group 1). In most patients who were not dry postoperative video urodynamics suggested suboptimal outlet resistance overcome by a hyperactive detrusor. No patient had postoperative upper tract deterioration. CONCLUSIONS: These results suggest that concomitant enterocystoplasty should be critically considered for pediatric patients undergoing sling cystourethropexy.

Adolescent↗

PubMed2004

Mainz Pouch II technique: 10 years' experience.

OBJECTIVE: To report the long-term results with the Mainz Pouch II procedure. PATIENTS AND METHODS: Between 1990 and 2000 a Mainz Pouch II ureterosigmoidostomy was used in 123 patients (49 females and 74 males, mean age 43.6 years, range: 1-73). The indications for urinary diversion were cystectomy for bladder cancer in 92 patients, bladder exstrophy and/or incontinent epispadias in 26, irreparable traumatic loss of the sphincteric urethra in four and cloacal malformation (sinus urogenitalis) in one. In all, 102 patients with a follow-up of >/= 12 months were evaluated (mean 46.2 months). RESULTS: Day- and night-time continence rates were 97% and 95%, respectively. The remaining patients occasionally lose some drops of urine during coughing or straining, or reported minimal soiling of undergarments during the night. The mean voiding frequency was six during the day and once at night. There were 14 ureteric implantation stenoses (7.2% of 194 evaluated reno-ureteric units) and they were treated successfully by open repair (13) or antegrade balloon dilation (one). For metabolic disturbances, 69% of the patients had a capillary base excess of <-2.5 mmol/L and use oral alkalinizing drugs to prevent hyperchloraemic acidosis. There was no clinically evident metabolic acidosis. CONCLUSION: Applying the principles of detubularization and spherical reconfiguration to create a low-pressure reservoir and stratifying ureteric implantation between submucosal and serous-lined extramural tunnel techniques succeeded in giving better continence rates and long-term preservation of the upper urinary tract than a classical ureterosigmoidostomy. The Mainz Pouch II ureterosigmoidostomy is simple and reliable as a viable alternative for continent urinary diversion in selected patients.

Adolescent↗

PubMed1994

Continent urinary diversion using the Mitrofanoff principle.

OBJECTIVE: To review the clinical and symptomatic outcome of patients undergoing cystectomy and continent urinary diversion using the Mitrofanoff principle. PATIENTS AND METHODS: There were seven men and five women (median age 62 +/- 21 years: range 21-79). The underlying diagnoses were: bladder carcinoma (8), neuropathic bladder disease (1), bladder exstrophy (1), tuberculosis (1) and interstitial cystitis (1). An ileo-caecal segment was used for construction of the reservoir. The appendix was used as a catheterizable conduit in 11 patients and a narrowed ileal tube in one patient. Symptomatic outcome was assessed by using a modified Visick grading. The median follow-up period was 15 +/- 10 months (range 6-39). RESULTS: The median hospital stay was 22 days. There were no post-operative deaths, but major post-operative complications developed in three patients and included intra-abdominal lymphocele (1), intra-abdominal abscess formation (1) and prolonged ileus (1). In the late post-operative period (> 30 days), four patients developed stenosis of the mucocutaneous junction and required dilatation. The overall symptomatic outcome was satisfactory in 11 patients. There were no deaths in the peri-operative period and none of the patients has since required revision. Urinary continence was achieved in all 12 patients during the day. One patient reported minimal, occasional leakage at nights. The median catheterization interval was 4 +/- 1 hours during the day (range 3-5) and 7 +/- 1 hours at night (range 6-9). CONCLUSION: Our experience with the Mitrofanoff procedure has been limited to a small number of patients. Despite this, the clinical outcome of the procedure was satisfactory. The procedure potentially offers an effective continence mechanism with acceptable post-operative morbidity.

Adult↗

PubMed1997

The spectrum of congenital anomalies of the VATER association: an international study.

The spectrum of the VATER association has been debated ever since its description more than two decades ago. To assess the spectrum of congenital anomalies associated with VATER while minimizing the distortions due to small samples and referral patterns typical of clinical series, we studied infants with VATER association reported to the combined registry of infants with multiple congenital anomalies from 17 birth defects registries worldwide that are part of the International Clearinghouse for Birth Defects Monitoring Systems (ICB-DMS). Among approximately 10 million infants born from 1983 through 1991, the ICB-DMS registered 2,295 infants with 3 or more of 25 unrelated major congenital anomalies of unknown cause. Of these infants, 286 had the VATER association, defined as at least three of the five VATER anomalies (vertebral defects, anal atresia, esophageal atresia, renal defects, and radial-ray limb deficiency), when we expected 219 (P<0.001). Of these 286 infants, 51 had at least four VATER anomalies, and 8 had all five anomalies. We found that preaxial but not other limb anomalies were significantly associated with any combination of the four nonlimb VATER anomalies (P<0.001). Of the 286 infants with VATER association, 214 (74.8%) had additional defects. Genital defects, cardiovascular anomalies, and small intestinal atresias were positively associated with VATER association (P<0.001). Infants with VATER association that included both renal anomalies and anorectal atresia were significantly more likely to have genital defects. Finally, a subset of infants with VATER association also had defects described in other associations, including diaphragmatic defects, oral clefts, bladder exstrophy, omphalocele, and neural tube defects. These results offer evidence for the specificity of the VATER association, suggest the existence of distinct subsets within the association, and raise the question of a common pathway for patterns of VATER and other types of defects in at least a subset of infants with multiple congenital anomalies.

Abnormalities, Multiple↗

PubMed1998

Congenital anomalies in the teratological collection of Museum Vrolik in Amsterdam, The Netherlands. III: primary field defects, sequences, and other complex anomalies.

The Museum Vrolik collection of the Department of Anatomy and Embryology of the Academic Medical Center, University of Amsterdam, founded by Gerardus Vrolik (1775-1859) and his son Willem Vrolik (1801-1863), consists of more than 5,000 specimens of human and animal anatomy, embryology, pathology, and congenital anomalies. Recently, the collection of congenital anomalies, comprising 360 specimens, was recatalogued and redescribed according to contemporary morphogenetic views. The original descriptions, if preserved, were compared with the clinical, radiographical, and magnetic resonance imaging findings. In 120 specimens the following midline anomalies, primary field defects, and sequences were diagnosed: holoprosencephaly sequence; aprosencephaly/atelencephaly; cleft lip; Pierre Robin sequence; omphalocele; diaphragmatic hernia; ectopic heart; bladder exstrophy; cloacal exstrophy; caudal dysgenesis; prune belly sequence; sirenomelia; fetal akinesia sequence; and disruption sequences. Polytopic field defects and associations were diagnosed in 25 specimens. We discuss the opinions of Gerardus and Willem Vrolik, who tried to find reasonable explanations for the dysmorphogenetic entities they investigated, rather than accepting the traditional superstitions.

Abnormalities, Multiple↗

PubMed1979

Ureterosigmoidostomy followed by carcinoma of the colon.

Adenocarcinoma of the colon developing as a late complication or ureterosignoidostomy has been reported with increasing frequency. A patient is presented who developed adenocarcinoma of the colon 28 years after ureterosigmoidostomy for bladder exstrophy and 13 years after conversion of the ureterosigmoidostomy to an ileal conduit. The colonic tumor was documented at postmortem examination to be at the ureterosigmoidostomy site. Because of the potential late development of adenocarcinoma of the colon, careful follow-up of patients with ureterosigmoidostomies, particularly those performed in children many years ago, is indicated.

Adenocarcinoma↗

PubMed1986

Colorectal adenocarcinoma in young Lebanese adults. The American University of Beirut-Medical Center experience with 32 patients.

Colorectal adenocarcinoma is uncommon in Lebanon. The low frequency and the low average age at the time of diagnosis, 53.7 years, is similar to that observed in other developing countries. Over a period of 40 years (1945-1985), 32 patients (5.8%) developed colorectal adenocarcinoma before age 30 years. Seventeen and 15 patients were males and females, respectively (age range, 14-29 years). The most common presenting symptoms were blood per rectum (27 patients) and abdominal pain (23 patients). The average interval from the first symptom to histologic diagnosis was 5.7 months. The only significant predisposing factors were the presence of a positive family history for colorectal carcinoma in one patient and bladder exstrophy with ureteral diversion in another. Twenty-four patients had surgery with curative intent. Colloid and signet ring adenocarcinoma were present in 22 patients (68.7%). Classification by Duke's staging system demonstrated Stage C in 15 and Stage D in 5 patients. These findings show a definite increase in carcinoma with high histologic grade and advanced stage at presentation in young Lebanese patients.

Academic Medical Centers↗

PubMed2003

First-year mortality rates for selected birth defects, Hawaii, 1986-1999.

Birth defects have been the leading cause of infant death in the United States for over the last decade. However, there is little population-based data on the first-year mortality rates for many specific birth defects and the factors that may affect these mortality rates. This investigation examined the first-year mortality rates for 54 selected birth defects of various organ systems in Hawaii during 1986-1999 using data from a population-based birth defects registry and evaluated the impact of the presence of chromosomal abnormalities and other structural birth defects and the year of delivery on the mortality rates. Mortality rates varied widely by defect, being highest for anencephaly (100%), trisomy 13 (82%), and trisomy 18 (74%), while no first-year deaths were reported for glaucoma, bladder exstrophy, and persistent cloaca. The majority (36 of 54 or 67%) of the birth defects had a mortality rate of less than 25%. Among the 51 structural birth defects, 38 (75%) had higher first-year mortality rate for cases with chromosomal abnormalities and 42 (82%) had higher first-year mortality rates for cases with other major structural birth defects. The mortality rate among 1986-1992 deliveries was higher than the mortality rate among 1993-1999 deliveries for 37 (69%) of the 54 birth defects. This study indicates that first-year mortality rates vary widely by type of birth defect, although the mortality rate for the majority of birth defects is relatively low. The presence of a chromosomal abnormality or other structural birth defect increases the mortality rate, and mortality rates for the majority of birth defects have declined in Hawaii during the study period.

Congenital Abnormalities↗