Complete covered cloacal exstrophy

Oshita, Masaaki; Okazaki, Tadaharu; Lee, Kyeong Deok; Lane, Geoffrey J.; Yamataka, Atsuyuki

doi:10.1007/s00383-007-1991-x

Cited by 11 publications

(3 citation statements)

References 6 publications

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“…[ 17 18 19 ] Apart from a long, narrow cloacal channel, this anatomy is strikingly similar to that described here. There is also a similarity with the anatomy in cases of cloacal exstrophy in girls[ 20 ] and a marked similarity with that in patients of “completely covered cloacal exstrophy.”[ 21 22 ]…”

Section: Discussionmentioning

confidence: 99%

Congenital pouch colon in girls: Genitourinary abnormalities and their management

Chadha

Khan

Shah

et al. 2015

J Indian Assoc Pediatr Surg

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Aims:To discuss the assessment and management of genitourinary (GU) tract abnormalities in 21 girls with Types I-III congenital pouch colon (CPC), studied over a period of 10 years.Materials and Methods:Assessment included clinical and radiological assessment, examination under anesthesia (EUA), endoscopy of the lower GU tract, and evaluation of the surgical findings, operative procedures for the GU anomalies, and the results of management.Results:Initial examination of the external genitalia showed a “clover-leaf” appearance (n = 6) and a single perineal opening (n = 6). In 9 patients, the openings of the urethra and double vagina were seen, of which a vestibular fistula was seen in 5 and an anterior perineal fistula in 1. Seventeen patients (81%) had urinary incontinence (UI) - partial in 10, and complete in 7. Renal function tests, X-ray sacrum, and abdominal US were normal in all patients. Micturating cystourethrogram (n = 9) showed a wide, bladder neck incompetence (BNI) with reduced bladder capacity in seven patients. EUA and endoscopy revealed a septate vagina in all patients and the urethral opening at a “high” position (n = 14) or at a relatively normal or “low” position (n = 7). In 8 patients, the intervaginal septum was thick and fleshy. Endoscopy showed a short, wide urethra, an open incompetent bladder neck, poorly developed trigone, and reduced bladder capacity in the patients with UI. The fistula from the colonic pouch opened in the proximal urethra (n = 4), high in the vestibule (n = 3), low in the vestibule (n = 8), perineum just posterior to the vestibule (n = 1), and undetermined (n = 5). Vaginoscopy (n = 8) showed normal cervices in all and cervical mucus in 4 patients. The subtypes of CPC were Type I CPC (n = 4), Type II CPC (n = 16), and Type III CPC (n = 1). All 21 patients had uterus didelphys. In four patients with UI, during tubular colorraphy, a segment of the colonic pouch was preserved for later bladder augmentation if required. A Young-Dees bladder-neck repair (BNR) was performed in four older girls for treatment of UI, with marked improvement in urinary continence in two girls, some improvement in one girl with complete urinary incontinence, and minimal improvement in one child. Division of the intervaginal septum was performed in three girls.Conclusions:GU abnormalities in girls with CPC need to be assessed and managed by a tailored protocol. UI is frequent, and its correction may require BNR. A segment of the colonic pouch can be preserved for possible future bladder augmentation. All girls have a septate vagina, often widely separated, and uterus didelphys. Gynecologic assessment and monitoring is required throughout adult life. Considering the wide opening of the vestibule, surgical management of the urogenital component by division of the intervaginal septum and if required, the vagino-fistula septum on each side results in a normal or a hypospadiac urethral opening and an adequate vaginal inlet.

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Section: Discussionmentioning

confidence: 99%

Congenital pouch colon in girls: Genitourinary abnormalities and their management

Chadha

Khan

Shah

et al. 2015

J Indian Assoc Pediatr Surg

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“…Patients with a completely covered cloacal exstrophy variant usually have an intact abdominal wall and normally positioned umbilicus combined with internal derangements including cloacal formation, vesicointestinal fistulas, vesicovaginal fistulas, and intestinal atresias [8]. These infants typically have the mildest form of cloacal exstrophy and the best functional outcomes.…”

Section: Discussionmentioning

confidence: 98%

Gastrointestinal ramifications of the cloacal exstrophy complex: a 44-year experience

Sawaya

Goldstein

Seetharamaiah

et al. 2010

Journal of Pediatric Surgery

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“…60% of cases with BEEC are presented by classic bladder exstrophy, 30% by epispadias cases and only 10% account for uncommon variants including pseudoexstrophy and covered cloacal exstrophy [2,3]. Covered cloacal exstrophy has been described in several postnatal cases [4,5,6,7], but, to the best of the authors' knowledge, only 1 prenatal case [8]. Diagnosis of covered cloacal exstrophy can be made prenatally by sonographic signs of classic cloacal exstrophy such as a low-set umbilicus, an omphalocele, imperforate anus, diastasis of the pubic rami and a split vulva yet presence of bladder filling, an intact lower abdominal wall without any visceral extroversion and the missing characteristic ‘elephant trunk' deformity of the usually prolapsing terminal ileum [4].…”

Section: Discussionmentioning

confidence: 99%

Prenatal Diagnosis of Covered Cloacal Exstrophy

Mallmann¹,

Reutter²,

Müller³

et al. 2014

Fetal Diagn Ther

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The authors present the case of a prenatally diagnosed covered cloacal exstrophy in a female fetus and potential differential diagnoses within the exstrophy-epispadias complex. While on prenatal ultrasound, low-set umbilicus, omphalocele, diastasis of the pubic rami, split vulva and left-sided renal agenesis were suggestive of classic cloacal exstrophy, the presence of bladder filling and the missing characteristic elephant trunk appearance of the usually prolapsing terminal ileum suggested the diagnosis of covered cloacal exstrophy. Besides urogenital anomalies, the fetus presented with an additional small muscular ventricular septal defect. Postnatal presentation showed an omphalocele with a low-set umbilicus and a directly inferior positioned large everted (exstrophic) bladder plate with low positioned ureteric orifices. The external genitalia impressed with rudimentary vulvae and hemiclitorises. An imperforate anus was present, yet bowel movements were passed shortly after birth through a small vesicointestinal fistula positioned at the lower vesical trigone. We conclude that prenatal diagnosis of covered cloacal exstrophy can be made if bladder filling is present during prenatal ultrasound, although the bladder might become exstrophic during later gestation.

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Complete covered cloacal exstrophy

Abstract: Complete covered cloacal exstrophy (CCCE) is extremely rare. The anatomy of CCCE is complex and often unique, and each case must be treated individually. We present the case of a 5-year-old girl with CCCE whom we treated successfully with great improvement in her quality of life.

Cited by 11 publications

References 6 publications

Congenital pouch colon in girls: Genitourinary abnormalities and their management

Congenital pouch colon in girls: Genitourinary abnormalities and their management

Gastrointestinal ramifications of the cloacal exstrophy complex: a 44-year experience

Prenatal Diagnosis of Covered Cloacal Exstrophy

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