Mermaid syndrome. medi
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mermaid syndrome, sirenomelia, congenital anomaly, obstetrics, rare case
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Mermaid syndrome. medi
- 1. MERMAID SYNDROME MEDIANA SUTOPO LIEDAPRAJA DEPARTEMEN OBSTETRI DAN GINEKOLOGI FAKULTAS KEDOKTERAN UNIVERSITAS INDONESIA NOVEMBER 2015
- 2. • Mermaid syndrome known as sirenomelia • Sever form of caudal regression • Very rare congenital deformity ( 1: 100.000) legs are fused together • Looks like mermaid’s tail • Fatal because complication abnormal kidney and bladder development and function • Failure of vascular supply from lower aorta in utero maternal diabetes
- 3. ETIOLOGY There are 2 theory: • In mice Causes by lacking Cyp26a1 ( enzyme that degrades retinoic acid) reduced bone morphogenetic protein (Bmp) In Human excess RA signaling and deficit BMP signaling in caudal body. • Primary defect in the generation of the mesoderm
- 4. STOCKER AND HEIFETZ CLASSIFIED SIRENOMELIA INTO TYPE 1 TO TYPE VII. ( ACCORDING TO SKELETAL ELEMENTS THIGH AND LEG) • Type I All bones in two fused lime are present, fusion only affects superficial tissue • Type VII Most severe, only single bone is present with no indication of legs and feet.
- 9. THANK YOU