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SIRENOMELIA A RARE PRESENTATION

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Author(s): K Ramesh Reddy | S Srinivas | Shiva Kumar | Surweshwar Reddy | Hariprasad | G M Irfan

Journal: Journal of Neonatal Surgery
ISSN 2226-0439

Volume: 1;
Issue: 1;
Start page: 7;
Date: 2012;
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Keywords: sirenomelia | mermaid syndrome | caudal regression syndrome

ABSTRACT
We are presenting two cases of Sirenomelia (Mermaid Syndrome), which is an extreme example of the caudal regression syndrome. It invariably presents with lower limb fusion, sacral and pelvic bony anomalies, absent external genitalia, imperforate anus, and renal agenesis or dysgenesis. There are approximately 300 cases reported in the literature, 15% of which are associated with twinning, most often monozygotic. The syndrome of caudal regression is thought to be the result of injury to the caudal mesoderm early in gestation. One of our cases survived for 12 days after birth. This new born had an unusually high anorectal anomaly in which the colon was ending at the level of mid transverse colon, fused lower limbs and genital anomalies. Ultrasound of the abdomen revealed horseshoe kidney. Colostomy was performed on day 2 of life. The second case encountered was a stillborn baby on whom an autopsy was performed.
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