Holoprosencephaly-caudal dysgenesis syndrome

Orpha code: 2165OMIM code:

Definition

Holoprosencephaly-caudal dysgenesis syndrome is a central nervous system malformation syndrome characterized by holoprosencephaly with microcephaly, abnormal eye morphology (hypotelorism, cyclopia, exophthalmos), nasal anomalies (single nostril or absent nose), and cleft lip/palate, combined with signs of caudal regression (sacral agenesis, sirenomelia with absent external genitalia).

Disease data
Classification

Malformation syndrome

ORPHA code
2165
OMIM code
-
ICD10 code
Q04.2
ICD11 code
-

No additional description.

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