Completely covered cloacal exstrophy: recognition of a new clinical sub-entity

Komura, Makoto; Tsuchida, Yoshiaki; Honna, Toshiro; Kamii, Yoshiyuki; Kitahara, Shuichiro; Ishizone, Satoshi

doi:10.1007/bf00184227

Cited by 7 publications

(5 citation statements)

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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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“…There are only five cases of CCCE reported in the English literature (two boys and three girls), with varying outcomes [1,2,5]. Both boys had colostomies and diversion of the colovesical fistula; two of the girls only had ileostomies with no attempt at separating the urinary, digestive and reproductive tracts, and the last girl had a colon pullthrough after an ileostomy with division of the cecum from the CC.…”

Section: Discussionmentioning

confidence: 94%

“…Unlike cloacal exstrophy, where the internal organs are grossly exposed and deformed, CCCE presents with an intact abdominal wall and a normally positioned umbilicus [1,2], although the internal derangement, including cloaca, intestinal atresia, vesicointestinal fistula, and vesicovaginal fistula are similar. The anatomy of CCCE is complex and often unique, and each case must be treated individually; the most major challenge being reconstruction of the urogenital tract to achieve urinary continence.…”

Section: Introductionmentioning

confidence: 93%

Complete covered cloacal exstrophy

et al. 2007

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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%

“…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]. Nevertheless, cloacal exstrophy with a cystic structure in the lower abdomen can be present if diagnosed in early pregnancy when the cloacal membrane has not ruptured yet [9,10].…”

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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Completely covered cloacal exstrophy: recognition of a new clinical sub-entity

Cited by 7 publications

References 10 publications

Congenital pouch colon in girls: Genitourinary abnormalities and their management

Congenital pouch colon in girls: Genitourinary abnormalities and their management

Complete covered cloacal exstrophy

Prenatal Diagnosis of Covered Cloacal Exstrophy

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