Prenatal Diagnosis of Covered Cloacal Exstrophy

Abstract

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.

Vesical Extrophia and Pregnancy. Case Report

“…There were obstruction in the high part of the urinary tract that nedeed intervention (10%) and preeclampsia (10%). A third of the term pregnancies in this group of patients, developed as a complication a cord prolapse near to term after the rupture of membranes (1). The pregnancy control after 20 week´s should be every two week´s until 28 week´s, with ultrasound evaluation of urinary tract modifications, urinary tests and cultures.…”

“…The main complication is to open urinary bladder to external environment. (1)(2) The reported prevalence is from 1 in 30.000 to 1 in 50.000 live newborns (3).…”

Covered cloacal exstrophy with pulmonary hypoplasia due to urethral obstruction

“…Although CCE has been described in several postnatal cases, the prenatal course and findings of CCE are not fully understood . To the best of our knowledge, all previously reported cases of CCE were not associated with lethal pulmonary hypoplasia due to urethral obstruction.…”

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

“…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 …”