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PubMed2003

[Anesthetic management for a radical operation in an infant with cloacal exstrophy].

We report the anesthetic management for a radical operation in an infant with cloacal exstrophy. Diagnosis of cloacal exstrophy with meningocele was made at the 26th week of gestation. Cesarian section was performed under general anesthesia in order to keep the infant in a state of apnea to prevent aerophagia, the so-called "sleeping baby", at the 38th week of gestation. As soon as the infant was born, she was intubated orotracheally and a radical operation (abdominal closure, removal of meningocele, and vesico-intestinal-pelvioplasty) was performed. Some complications occurred during the operation including hypotension, oliguria, hypothermia, hypoproteinemia, hyponatremia and hyperpotassemia. The operation time was 10 hours and 30 minutes. Mechanical ventilation was continued for 4 days in the NICU and the infant was discharged 37 days after the operation. Early prenatal diagnosis and simulation of the operation enabled an effective surgical procedure allowing us to minimize these intraoperative complications.

Abnormalities, Multiple↗
PubMed2001

OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): a review of 14 cases.

OEIS complex refers to a combination of defects consisting of omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects. Possible embryologic mechanisms proposed for these findings have included: a single defect of early blastogenesis or a defect of mesodermal migration during the primitive streak period. Fourteen cases with OEIS complex and related malformations were reviewed for demographic features, prenatal and family histories, and clinical, radiological and pathological findings including the frequency and types of associated anomalies. The pathogenetic mechanisms causing OEIS complex and related malformations, such as anorectal and spinal defects, are discussed. The findings in these cases illustrate the spectrum of defects that can occur in the embryologic development of the cloaca and the urorectal septum. Differences in the timing and extent of mesenchymal ingrowth as well as cloacal membrane rupture may account for these variable findings. A developmental field defect involving the intraembryonic mesoderm suggests a possible etiologic role for homeobox genes, such as HLXB9 with mutations, resulting in anorectal and spine abnormalities, or retinoic acid receptors. OEIS complex with its mostly sporadic occurrence suggests etiologic heterogeneity with a possible role for environmental and genetic causes.

Abnormalities, Multiple↗
PubMed1986

The management of erectile deformity in adults with exstrophy and epispadias.

The management of 20 patients with erectile deformities owing to exstrophy and epispadias is described. The type of deformity was defined by cavernosography and intraoperative artificial erections. Of the 20 patients 15 had dorsal chordee, 2 had lateral deviation (1 also had dorsal chordee) and 3 had bilateral rudimentary corpora producing small and inadequate erections. The latter 3 patients were considered inoperable. Of the 15 patients with dorsal chordee 12 were treated by insertion of lyophilized human dura in the concave side of the corpora. The results were good after 1 attempt in 8 and after 2 attempts in 1, while 1 had recurrent chordee distal to the first site and further dural phalloplasty was successful, 1 was improved and 1 was a failure. Of the remaining 3 patients in this group 2 underwent excision of scar tissue (1 also had a ventral Nesbit procedure) and the condition was not bad enough to require an operation in 1. Of the 2 patients with lateral deviation 1 was treated by division of a rudimentary corpus and dural phalloplasty of the normal corpus, which produced a straight but rather unstable erection, and 1 underwent bilateral dural phalloplasty with a good result.

Adolescent↗
PubMed2004

Discordant sexual identity in some genetic males with cloacal exstrophy assigned to female sex at birth.

BACKGROUND: Cloacal exstrophy is a rare, complex defect of the entire pelvis and its contents that occurs during embryogenesis and is associated with severe phallic inadequacy or phallic absence in genetic males. For about 25 years, neonatal assignment to female sex has been advocated for affected males to overcome the issue of phallic inadequacy, but data on outcome remain sparse. METHODS: We assessed all 16 genetic males in our cloacal-exstrophy clinic at the ages of 5 to 16 years. Fourteen underwent neonatal assignment to female sex socially, legally, and surgically; the parents of the remaining two refused to do so. Detailed questionnaires extensively evaluated the development of sexual role and identity, as defined by the subjects' persistent declarations of their sex. RESULTS: Eight of the 14 subjects assigned to female sex declared themselves male during the course of this study, whereas the 2 raised as males remained male. Subjects could be grouped according to their stated sexual identity. Five subjects were living as females; three were living with unclear sexual identity, although two of the three had declared themselves male; and eight were living as males, six of whom had reassigned themselves to male sex. All 16 subjects had moderate-to-marked interests and attitudes that were considered typical of males. Follow-up ranged from 34 to 98 months. CONCLUSIONS: Routine neonatal assignment of genetic males to female sex because of severe phallic inadequacy can result in unpredictable sexual identification. Clinical interventions in such children should be reexamined in the light of these findings.

Abnormalities, Multiple↗
PubMed2005

Simplified mons plasty: a new technique to improve cosmesis in females with the exstrophy-epispadias complex.

PURPOSE: Long-term data suggest that a significant number of women with the exstrophy-epispadias complex (EEC) report dissatisfaction with the cosmetic appearance of the genitalia. We developed a new technique of mons plasty in an attempt to improve the overall cosmetic appearance of the external genitalia in females with EEC. MATERIALS AND METHODS: Nine consecutive females with a mean age of 6.4 years (range 2 days to 12.2 years) previously diagnosed with EEC underwent simplified mons plasty concomitantly with various other urological reconstructive procedures. Briefly, a simplified mons plasty was performed by approximating the bifid hemiclitoris and subsequently incising the lateral sulci between the labia majora and minora, which allowed the superomedial rotation of the labia majora and underlying peripubic adipose tissue, thus, recreating the mons. RESULTS: There were no perioperative complications. With a mean followup of 6 months (range 4 to 12) all patients had improved cosmesis with respect to a more prominent mons, more appropriately situated labia and adequately recessed introitus. CONCLUSIONS: Simplified mons plasty improves cosmesis in females with external genital abnormalities secondary to EEC. Although our followup is admittedly short, we believe that this technique should be considered in the armamentarium of the reconstructive surgeon during the operative treatment of this complex group of patients.

Abdominal Wall↗
PubMed2004

The exstrophy-epispadias complex: is aesthetic appearance important?

OBJECTIVE: To show the relevance of cosmetic appearance in the adequate treatment of patients with exstrophy-epispadias complex (EEC), and to indicate that surgery by experienced teams can improve the long-term treatment forecast and the patient's body image. PATIENTS AND METHODS: From 1978 to 2002, 71 patients diagnosed with EEC were treated in the authors' institution; 24 (aged 2-23 years) were selected to undergo different surgical procedures. The criterion for surgery considered interviews conducted by the psychology team with the parents and children. The plastic surgery and paediatric urology teams carried out the procedures jointly; the follow-up was 0.33-7 years. RESULTS: Five female patients and six male had abdominoplasty to treat multiple scars; eight had intermittent catheterization conduits repositioned from the right iliac fossa to the umbilicus. Six female patients had plastic surgery of the external genitalia and three had a broad mobilization of the urogenital sinus. Thirteen male patients had a small penis and had the corpora cavernosa fully mobilized and the penis reconstructed. Five female patients and one male had anterior osteotomy. One patient with no left testis had it replaced and one patient with uterine prolapse had the uterus fixed to the posterior abdominal wall. Six patients had a second procedure, in two because the outcome of the initial operation was poor and in the others to complement the initial treatment. In all but one patient there was an improvement in the objective criteria, e.g. school absences, difficulty in establishing long-lasting social relationships and refusal to participate in sports activities. However, none of the patients would attempt sexual intercourse. CONCLUSIONS: Body image, self-esteem, sexuality, sexual function and fertility are deemed crucial by adolescents; in patients with EEC customised surgical procedures can give a satisfactory aesthetic outcome, and be a further reason for adequately following occasional urinary complications and renal function, to avoid loss to follow-up.

Adolescent↗
PubMed2002

Diploid/tetraploid/t(1;6) mosaicism in a 17-year-old female with hypomelanosis of Ito, multiple congenital anomalies, and body asymmetry.

Many types of chromosome mosaicism have been identified in cases of hypomelanosis of Ito, often in association with chromosome instability; however, there have been very few cases with diploid-tetraploid mosaicism described in the literature. We present a patient with a tetraploid mosaicism: a 17-year-old girl who has hypomelanosis of Ito in association with diploid/tetraploid/t(1;6) mosaicism. She had multiple congenital anomalies of omphalocele, exstrophy of bladder, duodenal web, and imperforate anus. These features have not been described previously in diploid-tetraploid mosaicism.

Abnormalities, Multiple↗
PubMed1988

Urology.

Explore the source record for details and available documents.

Bladder Exstrophy↗
PubMed1983

Carcinoma at the site of ureterosigmoidostomy.

A 31-year-old woman who had undergone bilateral ureterosigmoidostomy at age five had a moderately differentiated adenocarcinoma at the anastomotic site that was not detected by preoperative studies or intraoperative palpation. The authors review other reports of anastomotic carcinomas occurring after ureterosigmoidostomy for benign disease and recommend a diagnostic protocol. Whenever the urine is diverted away from the sigmoidostomy (rediversion), the anastomotic site should be resected. Ureterosigmoidostomy should be undertaken only with the understanding that meticulous long-term follow-up is mandatory.

Adenocarcinoma↗
PubMed1999

Continent urinary undiversion to modified ureterosigmoidostomy in bladder extrophy patients.

The objectives of urinary diversion are expanding from merely preserving kidney functions to enabling the patient to have a good quality of life while maintaining an acceptable certain body image. During the new era of continent urinary diversion the psychological drawbacks of noncontinent skin stoma in young adolescents cannot be overlooked. Ureterosigmoidostomy has been the technique of choice for continent urinary diversion in bladder extrophy patients when bladder reconstruction is not feasible or has failed. Although it provides a good daytime continence it is associated with a high rate of nighttime incontinence and delayed complications of pyelonephritis and hyperchloremic acidosis. We managed five male bladder extrophy patients with noncontinent skin stoma (sigmoid colon conduit in three and ileal conduit in two) including two patients who had previously had complicated classic ureterosigmoidostomy. They underwent urinary undiversion to the valved and augmented rectum (three patients) and the valved S-shaped rectosigmoid pouch (two patients) with some modifications. The conduit was used in the construction, and the ureters were implanted behind an isolated intussusception ileal nipple valve. Tube cecostomy and total parenteral nutrition was used for 7 to 10 days instead of a temporary defunctioning transverse colostomy. With a mean follow-up of 19.8 months (range 9-36 months) all the patients are fully continent during the day and night, with an emptying intervals of 3 to 6 hours. Follow-up intravenous pyelography and renal scans revealed improvement or stabilization of the function and configuration of the upper tracts in all renal units. No prophylactic alkali therapy was given. No clinical evidence of acidosis or symptomatic urinary tract infection was observed. Modified ureterosigmoidostomy is a good alternative for continent urinary undiversion even in those who have previously had complicated classic ureterosigmoidostomy. Our modification of using tube cecostomy and parenteral nutrition instead of a temporary transverse colostomy warrants attention; it made the technique simpler and more attractive.

Acidosis↗
PubMed2001

Unenhanced helical CT in the evaluation of the urinary tract in children and young adults following urinary tract reconstruction: comparison with sonography.

PURPOSE: To compare the accuracy of unenhanced, helical CT with sonography for the detection of complications of urinary tract reconstruction. MATERIALS AND METHODS: Forty-six kidneys in 24 patients were examined with CT and sonography. All scans were assessed for ease of renal visualization, presence of renal, ureteral, and bladder calculi, renal scars, hydronephrosis, and abdominal wall hernia. The results of both imaging modalities were independently reported. RESULTS: CT provided excellent visualization of all 46 kidneys, while sonography provided poor visualization of 8 kidneys (17%) (P < 0.001). CT detected calculi in 10 kidneys, 1 ureter, and 7 bladders. Sonography detected calculi in only 2 kidneys, and 2 bladders. Overall, CT detected significantly more calculi than US (18 vs 4, P = 0.01). CT detected scarring in 15 kidneys, while sonography detected scarring in 10. Hydronephrosis was detected in 6 kidneys by CT and in 8 kidneys by sonography. Three abdominal wall hernias were seen at CT that were not seen at sonography. CONCLUSION: CT is superior to sonography for the detection of urinary tract calculi and renal scarring. CT will demonstrate abdominal wall hernias that are unsuspected.

Adolescent↗
PubMedDate not supplied

Neoomphaloplasty: An old and new technique.

The authors review several proposed approaches to neoomphaloplasty, with emphasis on the use of three flaps anchored to the fascia, allowing the exposed central area to heal unaided. The procedure is straightforward and can be performed singly or during abdominoplasty. Results are both cosmetic and natural-looking.

Bladder Exstrophy↗