Prenatal sonographic features of complete covered cloacal exstrophy

Ono, Kyoko; Kikuchi, Akira; Takagi, K.; Takahashi, Diana; Yoshizawa, Kazuki; Nishizawa, Shuji

doi:10.1002/uog.7346

Cited by 8 publications

(4 citation statements)

References 7 publications

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“…The presence of bladder filling and absence of the characteristic elephant trunk appearance of the prolapsing terminal ileum may suggest the diagnosis of a covered cloacal exstrophy prenatally. 3 4 In addition, these patients often commonly have severe spinal abnormalities, such as a tethered cord or spinal dysraphisms. 5 …”

Section: Discussionmentioning

confidence: 99%

Image of the Month: Clinical Features in a Newborn with Covered Cloacal Exstrophy

Vilanova-Sánchez

Ching

Gasior

et al. 2017

European J Pediatr Surg Rep

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Cloacal exstrophy is the most severe type of anorectal malformations that belongs to the bladder–exstrophy–epispadias complex of genitourinary malformations. Interestingly, its variant, the covered cloacal exstrophy, is often missed. The clinical findings of this variant may include an imperforate anus, low lying umbilicus, thick pubic bone, and pubic diastasis but with an intact abdominal wall. We present an interesting case of covered cloacal exstrophy with a side-by-side duplicated bladder and discuss important considerations for the time of colostomy creation in the newborn period.

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Section: Discussionmentioning

confidence: 99%

Image of the Month: Clinical Features in a Newborn with Covered Cloacal Exstrophy

Vilanova-Sánchez

Ching

Gasior

et al. 2017

European J Pediatr Surg Rep

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“…5c), so it is easy to understand how it would be difficult to be differentiated from a classic cloaca as the difference relies in the location of the most distal portion of the urogenital sinus. Covered cloacal exstrophy [12, 21, 30] represents a spectrum of the cloacal exstrophy with the majority of cases represented by an intact abdominal wall or a small omphalocele, and low implantation of the umbilical cord. Yet, the intra-abdominal findings are consistent with those seen in cases of cloacal exstrophy (separated pubic bones, single perineal orifice, absent bladder neck, normal colon, short colon or almost absent colon, and duplicated appendix, etc).…”

Section: Discussionmentioning

confidence: 99%

“…The first radiologic criteria for the prenatal diagnosis of cloacal exstrophy was proposed in 1995 [7] and was subsequently refined by others [8–12]. …”

Section: Introductionmentioning

confidence: 99%

Prenatal counseling for cloaca and cloacal exstrophy—challenges faced by pediatric surgeons

et al. 2012

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IntroductionWith the advance of prenatal imaging, more often pediatric surgeons are called for prenatal counseling in suspected cases of cloaca or cloacal exstrophy. This presents new challenges for pediatric surgeons since no specific guidelines have been established so far. The purpose of this review is to analyze our experience in prenatally diagnosed cloaca or cloacal exstrophy and to provide some guidelines for prenatal counseling of these complex congenital anomalies.MethodsA retrospective review of the medical charts of patients with prenatally diagnosed cloaca and cloacal exstrophy who received postnatal care in our institution between July 2005 and March 2012 was performed. Representative images of prenatal studies were selected from 13 cases to illustrate different scenarios and the recommendations given. In addition, a review of the literature was performed to support our advice to parents.ResultsEleven patients were female and two patients were male. The postnatal diagnoses were cloacal exstrophy (6), cloaca (5), posterior cloaca variant (1), and covered cloacal exstrophy (1). The selected abnormal prenatal imaging findings in these 13 patients included hydronephrosis (12), neural tube defect (8), omphalocele (7), lack of meconium at expected rectal location (7), vertebral anomaly (7), non-visualize bladder (5), distended bladder (5), hydrocolpos (4), dilated or echogenic bowel (3), umbilical cord cyst (3), separated pubic bones (2), and the “elephant trunk” sign (2). The prenatal diagnosis was correct in 10 cases, partially correct in two cases, and it was missed in one case. All parents received prenatal counseling depending on the specific diagnosis.ConclusionThe continuous technologic innovations in prenatal imaging make it possible to prenatally diagnose more complex anomalies including cloaca and cloacal exstrophy with increased levels of confidence and enhance the benefit of prenatal counseling. Together, these allow the parents to be better prepared for the condition and the care team to provide the best possible initial management in order to improve the outcomes of these challenging patients.

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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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Prenatal sonographic features of complete covered cloacal exstrophy

Cited by 8 publications

References 7 publications

Image of the Month: Clinical Features in a Newborn with Covered Cloacal Exstrophy

Image of the Month: Clinical Features in a Newborn with Covered Cloacal Exstrophy

Prenatal counseling for cloaca and cloacal exstrophy—challenges faced by pediatric surgeons

Prenatal Diagnosis of Covered Cloacal Exstrophy

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