Sirenomelia

Description

Sirenomelia is a rare lethal malformation characterised by severe anomalies of the caudal part of the fetus that include a single lower limb, with various degrees of involvement ranging from single to separate femurs in the same skin shaft, presence of two feet (sympode mermaid) or one foot (monopode mermaid), to absence of both feet (ectromelic mermaid). Urogenital anomalies are also present and include bilateral renal agenesis, absence of outflow tract and absence of external genitalia. Imperforate anus and sacro-coccygeal agenesis have also been reported. Together these malformations comprise the extreme form of the caudal regression sequence.

Index terms

Sirenomelia
Sirenomelia syndrome

Exclusions from above levels

http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

LD2F.10 – Prune belly syndrome
LD2F.11 – VATER association
LD2F.13 – Meckel-Gruber syndrome
LD2F.14 – MURCS association
LD2F.15 – Noonan syndrome
LD2F.16 – Otomandibular dysplasia
LD2F.1Y – Other specified syndromes with multiple structural anomalies, not of environmental origin
LD2F.1Z – Syndromes with multiple structural anomalies, not of environmental origin, unspecified

Foundation entity

Sirenomelia – http://id.who.int/icd/entity/473306797

Part of: LD2F.1: Syndromes with multiple structural anomalies, not of environmental origin › LD2F: Syndromes with multiple structural anomalies, without predominant body system involvement › 20: Developmental anomalies