Prenatal ultrasonographic features of cloacal exstrophy and literature review / 中国医学影像技术

Objective: To investigate prenatal ultrasonographic features of fetal cloacal exstrophy. Methods: Prenatal ultrasound and birth/autopsy results of 12 fetuses with cloacal exstrophy were analyzed retrospectively. The ultrasonic features of cloacal exstrophy were observed and compared with pathologic findings. Results: Prenatal ultrasonography showed omphalocele in 11 cases, bladder non-visualization in 8 cases, anal atresia in 8 cases, neural tube defect (acrococcygeal mass or absence of vertebrae) in 7 cases. The mainly accompanied abnormalities included lower limb deformity in 2 cases, urinary malformation in 5 cases, ambiguous genitalia in 9 cases, intrauterine growth restriction in 1 case, heart malformation in 2 cases, digestive tract abnormalities in 2 cases, single umbilical artery in 1 case, nuchal translucency thickening with hydrocephalus in 1 case. Conclusion: When prenatal ultrasound found features such as omphalocele, bladder non-visualization, anal atresia and ambiguous genitalia etc., cloacal exstrophy should be considered.

Bladder exstrophy.

Bladder exstrophy, more properly, the exstrophy-epispadias complex is a rare congenital anomaly occurring once every 10,000-50,000 live births with a 2.3:1 as male & female ratio. The diagnosis involves a spectrum of anomalies of the lower abdominal wall, bladder, anterior bony pelvis and external genitalia. It occurs due to failure of the abdominal wall to close during fetal development and results in protrusion of the posterior bladder wall through the lower abdominal wall. We report a case of bladder exstrophy managed by us.

Cloacal exstrophy: a rare and complex anomaly.

Cloacal exstrophy is a very rare and complicated birth defect. It occurs once in every 200,000 births. It is a complex anomaly of the urogenital tract and intestinal tract resulting in exstrophy of both bowel and bladder; the most severe form of anomaly is in the exstrophy–epispadias complex. Its features include omphalocele, imperforate anus and exstrophy of two hemibladders, between which lies the everted cecum. A small colon ends blindly in the pelvis, and the terminal ileum often prolapses out of the exposed cecum. We are reporting a case of cloacal exstrophy with lumbosacral meningomyelocoele and deformity of left foot which was managed successfully.

Cloacal exstrophy: A continuing management challenge

A cloacal exstrophy is a multisystem anomaly involving the gastrointestinal, nervous musculoskeletal and genito-urinary system and it is managed by a multidisciplinary approach. Despite improvements in care and approaches in reconstruction, surgical correction remains a challenge to the urologist. Recently, a newborn with cloacal exstrophy was admitted at the Department of Urology, East Avenue Medical Center. Department specialties in pediatrics, pediatric-surgery, pediatric orthopedics, and urology planned for the treatment of this patient. Surgery included excision of omphalocoele, separation of the cecal plates from the bladder halves, joining of the bladder halves, creation of the end tail gut colostomy and approximation of the pubic bones. (Author)

A Cloacal Exstrophy Variant Associated with Hindgut Duplication

Diphallus is a rare congenital anomaly and is frequently associated with duplication of the urinary tract and rectosigmoid, and commonly associated with vertebral anomalies. Remzi reported less than 100 cases of duplication of all or a portion of the penis, but about 10 cases of complete diphallus with exstrophy of cloaca was reported, and a case of complete diphallus associated with hingut duplication was reported, and complete diphallus with displacement of bladder associated hindgut duplication and imperforate anus was not reported in Korea. We experienced a case of the complete diphallus associated with displacement of bladder, hindgut duplication, and imperforate anus as a variant of cloacal exstrophy. A review of published cases suggests that this may be the first example of a complete dip hall us with displacement of bladder coexisting with the hindgut duplication and imperforate anus.