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PubMed2005

Incisional pubic hernia: treatment of a case with congenital malformation of the pelvis.

Parapubic hernia is a rare entity, the aetiology of which is usually associated with pelvic surgery, traumatisms and bone malformations. Treatment is difficult because the defect is usually limited by altered fibrotic anatomical planes. We present a case of incisional pubic hernia in a patient with congenital bladder exstrophy and multiple surgeries for pelvic reconstruction, who was treated using a plasty combined with a double intra-abdominal/supra-aponeurotic mesh. The therapeutic possibilities are described for the laparoscopic and open approaches in the management of these complex hernias.

Adult↗
PubMed1998

[Colon carcinoma after ureterosigmoidostomy--a case report].

Ureterosigmoidostomy was the method of choice for continent urinary diversion in cases of bladder exstrophy. The development of colonic cancer at the ureterocolic junction is one of the major complications in the late course of this procedure. The risk of large bowel carcinoma is increased 150-500-fold compared to the general population. Therefore regular examinations are mandatory. Based on a case report the problems associated with ureterosigmoidostomy are presented.

Adenocarcinoma↗
PubMed1998

Construction of continent catheterizable urinary conduit from an isolated segment of colon.

BACKGROUND: Continent urinary diversion may be necessary in a range of urological abnormalities. In circumstances where the standard techniques are not possible, alternative innovative techniques may be used. METHODS: In a female patient with bladder exstrophy, a continent diversion was recommended. The appendix was not available, the ureters were not suitable and a continent stoma was fashioned from an isolated segment of colon. RESULTS: The stoma proved to be continent, although it was somewhat stenotic. However, clean intermittent catheterization maintained its patency. CONCLUSIONS: A continent catheterizable stoma may be constructed from a segment of colon. The technique may be considered when other well recognized methods are not feasible.

Bladder Exstrophy↗
PubMed1999

Transcolonic retrograde ureteric catheterization assisted by 3-D computed tomography.

A 42-year-old woman had bladder exstrophy at birth, treated by ureterocolic anastomosis of her single kidney. She suffered recurrent hyperammonaemia, leading to comas, but refused an ileal conduit. During her most recent coma, it was decided to divert her urine to test whether this would reduce hyperammonaemia: this was accomplished by transcolonic retrograde catheterization of the ureter. This was only possible after computed tomography ureterography to show the ureterocolic anastomosis.

Adult↗
PubMed1979

The trigonointestinal implantations.

The author reports the indications, operative technique and long-term results of trigonosigmoidostomy in 26 cases of bladder exstrophy. The long-term results were considered as excellent in 20 cases, satisfactory in 4 and bad in 2. Even after a long period of up to 20 years, the function of the ureterotrigonal junction remains intact and ascending pyelonephritis does not occur. Social life is normal and sexual function is possible. In cases when internal diversion is counterindicated, the trigone can be successfully implanted into an isolated loop of the ileum or colon.

Adolescent↗
PubMed2001

Implications of prenatal ultrasound screening in the incidence of major genitourinary malformations.

PURPOSE: During the last 3 decades infant mortality has decreased nearly 50%. While neonatal intensive care deserves much of the credit, the recent increase in prenatal ultrasound from 33% of pregnancies in 1980 to 78% in 1987 has improved early detection. We evaluate the impact of prenatal ultrasound on major genitourinary malformations. MATERIALS AND METHODS: Data obtained from the Malformations Surveillance Program at Brigham and Women's Hospital between 1974 and 1994 tracked 163,431 pregnancies and termination rates of fetuses with spina bifida, bladder exstrophy, the prune belly syndrome and posterior urethral valves. RESULTS: Hospital data revealed that pregnancy was electively terminated due to spina bifida in 65% of cases, posterior urethral valves in 46%, the prune belly syndrome in 31% and exstrophy in 25%. CONCLUSIONS: Clearly, surveillance programs and improved accuracy of prenatal ultrasound have allowed early diagnosis of major genitourinary malformations. Many factors influence decision making in these affected fetal cases, including the financial and emotional impact of these major anomalies during lifetime. Future societal decisions and reduction in these anomalies may influence our training programs, manpower needs, medical facility requirements and character of our practices. These findings may have significant implications in the field of pediatric urology.

Abortion, Eugenic↗
PubMed1991

Complications of the Heitz-Boyer urinary diversion: case report of late development of malignancy.

We report on 3 patients with bladder exstrophy followed for more than 15 years after having undergone Heitz-Boyer urinary diversion. One patient had adenocarcinoma at the ureterocolonic anastomoses 16 years after diversion. To our knowledge this is the first reported case of a malignancy developing after a Heitz-Boyer procedure. The other 2 patients had significant problems with urinary incontinence and recurrent urinary tract infections. Initial success with this form of urinary diversion can be followed by significant long-term complications.

Adenocarcinoma↗
PubMed1982

Nonrefluxing colonic conduit: efficiency and complications of ureterocolic anastomosis.

The efficiency of and complications in 65 cases of colonic conduit have been studied (64 cases of bladder exstrophy and 1 case of cloacal exstrophy). For the ureterocolic reimplantation, we employed the Leadbetter technique and a modified kelalis technique. The results after 1-12 years in 128 renoureteral units showed 28 complications (21 obstruction and 7 refluxing units). Other types of complications have been observed and reported in this work. The high incidence of obstruction was probably due to the long submucous tunnel in the ureterocolic reimplantation. We believe that the sigmoidal conduit is an adequate method for temporary or permanent diversion.

Bladder Exstrophy↗
PubMed1996

Complete penile disassembly for epispadias repair: the Mitchell technique.

PURPOSE: We report a new technique for epispadias repair, which relies on the unique blood supply of the corpus cavernosum and glans. The epispadiac phallus is completely disassembled into 3 discrete components: the urethral plate, and the right and left hemicorporeal glandular bodies. MATERIALS AND METHODS: Ten boys 10 months to 17 years old presented for repair of epispadias between 1990 and 1994 (6 primary and 4 secondary repair). One patient underwent bladder exstrophy closure at the time of epispadias surgery. Two patients underwent prior para-exstrophy flap procedures and neither had fistulas. RESULTS: Followup (8 to 57 months) revealed a conical glans in 8 patients, straight shaft upward in 1 and ventral orthotopic meatus in 7. In 2 patients who underwent secondary repair 3 fistulas formed that were repaired on an outpatient basis. All patients are potent. CONCLUSIONS: Complete disassembly allows tubularization and ventralization of the entire distal urethra; makes glans and urethral repair independent; separates the 2 corporeal glandular bodies, permitting easier and more complete release of the rotation contributing to dorsal chordee, and improves exposure for corporotomy or dermal grafts.

Adolescent↗
PubMed1984

Pregnancy associated with exstrophy of the bladder and obstructive renal failure.

I have reported a case of pregnancy in a patient with exstrophy of the bladder. Early establishment of renal function and status is most important, and detailed counseling of the patient is preferred ideally before conception. The patient should be reassured that her defect has not been proven to have a genetic link. Patients with ureterosigmoidostomy diversion should be observed closely for renal obstructive symptoms.

Adult↗
PubMed1994

Renal transplantation using external continent urinary diversion.

A 29-year-old man born with bladder exstrophy presented with end stage renal failure many years after ileal conduit diversion. Bilateral nephrectomy and continent external urinary diversion were performed, and 1.5 months later a cadaveric kidney was grafted into the right iliac fossa. The patient was well at 18 months with a serum creatinine level of 1.2 mg./dl. and he was completely dry with 4 or 5 daily catheterizations. Although followup is still short, renal transplantation with drainage into an external continent urinary diversion permits excellent quality of life and good renal function. Therefore, this alternative is worth consideration whenever other reconstructive alternatives are not possible in candidates for renal transplantation.

Adult↗
PubMed1991

Ureterosigmoidostomy: a long-term follow-up of 15 patients with urinary diversion.

We reviewed 15 patients who underwent 16 ureterosigmoidostomies from 1975 to 1989. The underlying disease was bladder exstrophy in 13 patients. Urinary diversions have been performed according to Mathisen in 15 patients, one reoperation has been performed according to Coffey. Standard post-operative controls consist of physical examinations, capillary blood gas samples, intravenous pyelograms, inulin clearance measurements, 123I-hippuran scintigraphies and colonoscopies. Results concerning continence are judged very satisfactory in 80% of patients. 34% of patients presented with one or more episodes of unilateral or bilateral pyelonephritis. Intravenous pyelograms showed improved or unchanged urinary tract dilatation in 45% of kidneys and moderate and severe dilatation in 55%. Inulin clearance remained in normal range in 4 out of 6 patients followed. Renal function assessed by means of renal scintigraphy remained unchanged in 61% of kidneys, slightly decreased in 22% and severely in 17%. All patients are kept on a sodium-citrate therapy. Only one patient shows growth disturbances. Out of 9 patients followed with colonoscopy, none showed signs of malignancy.

Adolescent↗
PubMed1994

Comparative epidemiology of selected midline congenital abnormalities.

We present comparative epidemiologic characteristics of five congenital abnormalities that have been suggested to result from midline abnormal developmental disturbances: esophageal atresia with or without tracheoesophageal fistula (EA/TEF), imperforate anus with or without fistula (IA/F), omphalocele (OM), bladder exstrophy (BE), and diaphragmatic hernia (DH). The purpose was to assess the extent of epidemiologic similarities among these five defects. Data were collected as part of a population-based case-control study of infants with these defects born to mothers residing in Maryland, Washington, D.C., or Northern Virginia from 1980 through 1987. The estimated annual birth prevalences (per 10,000 live births) and 95% confidence intervals (CI) of these five defects were 0.40 (0.26-0.61) for BE, 1.34 (1.08-1.67) for OM, 1.59 (1.29-1.95) for DH, 2.11 (1.76-2.53) for EA/TEF, and 2.97 (2.55-3.46) for IA/F. The birth prevalence of IA/F and DH increased between 1980 and 1987. In contrast to the other four defects, DH showed a significant male preponderance (rate ratio 1.57, 95% CI 1.03-2.47), a significant white excess (rate ratio white:other, 1.56, 95% CI 1.00-2.48), and a lower proportion of multiple associated defects (30% vs. 46-61%). We concluded from this study that the descriptive epidemiology of diaphragmatic hernia is different from that of the other four defects. This finding may imply differences in etiologic and pathogenetic mechanisms underlying DH.

Abnormalities, Multiple↗
PubMed1998

A population-based study of abdominal wall defects in South Australia and Western Australia.

The purpose of this study was to determine the prevalence, clinical characteristics, prenatal diagnosis and occurrence of other birth defects with abdominal wall defects in births and terminations of pregnancy in South Australia (SA) and Western Australia (WA) over the period 1980-90. Cases of gastroschisis, exomphalos, bladder exstrophy, cloacal exstrophy and body stalk anomaly were ascertained from the WA Birth Defects Registry (1980-90) and the SA Birth Defects Register (1986-90). The registers are comparable population-based data collections with information on livebirths and stillbirths of at least 400 g birthweight or 20 weeks' gestation, and terminations of pregnancy for fetal abnormality. The prevalence of gastroschisis was 1.65/10,000 births (59 cases) and of exomphalos 2.90/10,000 births (104 cases). There was no significant difference in prevalence of exomphalos or gastroschisis between SA and WA for the years 1986-90. However, if data from WA for the years 1980-85 were included, SA had a significantly higher prevalence of exomphalos (prevalence ratio 1.71, confidence interval [CI] 1.16-2.55), although not of gastroschisis (prevalence ratio 1.35, CI 0.79-2.32). Exomphalos was significantly more common in mothers < 20 years (odds ratio [OR] 2.45, CI 1.22-4.86) and in mothers of 40 years or older (OR 5.65, CI 1.69-16.77). Gastroschisis was more common in younger mothers (OR 8.76, CI 4.02-19.32). Both exomphalos and gastroschisis were associated with low birthweight, prematurity, intrauterine growth retardation and caesarean section. The reason for the higher prevalence of exomphalos in SA than WA was not clear, but may be related to differences in prenatal diagnosis. The association between maternal age < 20 years and exomphalos raises the possibility of common factors in the aetiology of gastroschisis and exomphalos.

Abdominal Muscles↗
PubMed2000

The effect of intestinal urinary reservoirs on renal function: a 10-year follow-up.

OBJECTIVE: To study the effect of the storage of urine in intestinal reservoirs on long-term renal function and the possible causes of deterioration. PATIENTS AND METHODS: Eighty-seven patients (aged 4-35 years) with bladder exstrophy who underwent reconstruction of the lower urinary tract using a bowel segment were enrolled in a prospective protocol. The glomerular filtration rate (GFR) was measured before and after surgery at 1, 2, 5 and 10 years using 51Cr-ethylenediamine tetra-acetic acid. Patients with a decline in GFR of > 5% were investigated to identify the cause. RESULTS: Of 58 patients with a follow-up of > or = 10 years, 53 were evaluable, four having been lost to follow-up and one refusing to accept the protocol. In these 53 patients, the mean (SD) GFR decreased from 97. 9 (20.4) to 92.9 (23.6) mL/min/1.73 m2 (P = 0.24). However, this decrease was accounted for by 10 patients (19%) whose GFR fell by > or = 20% over the 10 years. The causes of renal deterioration in these 10 patients were; chronic retention and/or infection caused by inadequate catheterization in poorly compliant patients (five), uretero-ileal stenosis (one), a high-pressure reservoir (one) and uncertain causes (three). CONCLUSIONS: For 80% of the patients, the storage of urine in intestinal reservoirs did not change renal function for at least 10 years. However, approximately 20% of patients had some deterioration in renal function during the 10-year follow-up, usually from identifiable and remediable causes. The storage of urine in bowel does not appear to be inherently damaging to kidney function. Patients with an enterocystoplasty need regular monitoring of renal function; when deterioration is detected the urinary tract must be functionally assessed.

Adolescent↗