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Cloacal exstrophy
  1. R A Reddy,
  2. B Bharti,
  3. S C Singhi
  1. Dept of Pediatrics, Advanced Pediatrics Centre, PGIMER, Chandigarh, V., India; drsinghi{at}

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This newborn baby was referred to us for malformed lower abdomen since birth. On examination the umbilical cord was displaced downward and the lower abdominal wall was replaced by a large red coloured mass. The mass had a thin, red, protruding tubular structure in the middle resembling an elephant trunk, and five visible orifices. External genitalia could not be identified (fig). A diagnosis of cloacal exstrophy was made.

Cloacal exstrophy is the rarest and most extreme form of the exstrophy–epispadias complex that occurs once in 200 000 to 400 000 births.1 Embryologically four separate folds—cephalic, caudal, and right and left lateral—each of which has a splanchnic and somatic aspect, form the anterior abdominal wall. Failure of the caudal fold to close results in cloacal exstrophy.2 Classically it consists of an exstrophic central bowel field flanked by two hemi-bladders. Omphalocoele is present in 90% of cases. There are 3–4 orifices in the central bowel field, which is an ileocaecal region. The proximal orifice leads to the terminal ileum, which often prolapses, producing an elephant trunk deformity. The distal orifice leads to a short, blind ending colon segment. One or more appendiceal orificesmay be present. The anus is imperforate, and external genitalia have a wide range of anomalies, including absent penis/clitoris, absent scrotum, epispadias, hemiscrota, etc.1