MERMAID SYNDROME
MEDIANA SUTOPO LIEDAPRAJA
DEPARTEMEN OBSTETRI DAN GINEKOLOGI
FAKULTAS KEDOKTERAN UNIVERSITAS INDONESIA
NOVEMBER 2015
• 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
ETIOLOGYThere 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
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.
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