cloacal exstrophy


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exstrophy

 [ek´stro-fe]
the turning inside out of an organ, such as the urinary bladder.
exstrophy of the bladder congenital absence of a portion of the abdominal wall and bladder wall, the bladder appearing to be turned inside out, with the internal surface of its posterior wall showing through the opening in the anterior wall.
exstrophy of cloaca (cloacal exstrophy) a developmental anomaly in which two segments of bladder (hemibladders) are separated by an area of intestine with a mucosal surface, resembling a large red tumor in the midline of the lower abdomen.

clo·acal exstrophy

congenital anomaly with two exstrophied bladder units separated by an exstrophied segment of intestine, which is usually cecum, receiving ileum superiorly and continuing distally to blind ending microcolon. A number of variants of anatomic disarray can occur.
Synonym(s): ectopia cloacae
References in periodicals archive ?
Psychosexual development in genetic males assigned female: The cloacal exstrophy experience.
Other Syndromes/Conditions with Genital Abnormalities Cloacal exstrophy Abnormal Features include of the bladder embryogenesis wide pubic causing multiorgan symphyseal pelvic defect diastasis, resulting in severe omphalocele, inadequacy of imperforate anus, absence of exstrophy of two genitalia.
Omphalocele: Some abdominal organs, covered by a thin membrane, protrude through abdominal muscles Exstrophy of the cloaca: Bladder is open in half; rectum communicates with the bladder Imperforate anus: Anus has not been formed or perforated and colon communicates with bladder Spinal defects: Babies with cloacal exstrophy may have varying degrees of a spinal defect called spina bifida
Presenting Defects of Twin A and Twin B Twin A Twin B Heart Functional single ventricle Double outlet [R] ventricle Tricuspid stenosis PDA D transposition of the great Superior, inferior ventricles arteries Coarctation of the aorta Shared pericardium VSD, PDA Shared pericardial sac Liver Midline - shared Midline - shared Own hepatic veins Own hepatic veins Neuro No defect Thoracic- lumbar myelomeningocele Arnold Chiari defect Caudal regression syndrome Renal 2 kidneys Cloacal exstrophy of bladder 1 kidney GI Gastroschisis Gastroschisis Imperforate anus
7) Schinzel et al (1979) estimated that 10% of cloacal exstrophy occur in same sex twins and suggested that multiple pregnancies and early malformations like exstrophy are related (3).
A cloacal malformation or a cloacal exstrophy could be excluded, as well as other accompanying spinal abnormalities.