Megalencephaly-capillary malformation-polymicrogyria syndrome

Orpha code: 60040OMIM code: 602501

Definition

A rare developmental defect during embryogenesis that is characterized by growth dysregulation with overgrowth of the brain and multiple somatic tissues, with capillary skin malformations, megalencephaly (MEG) or hemimegalencephaly (HMEG), cortical brain abnormalities (in particular polymicrogyria), typical facial dysmorphisms, abnormalities of somatic growth with asymmetry of the body and brain, developmental delay and digital anomalies.

Disease data
Classification

Malformation syndrome

Synonyms
MCAP
Makrocefalia - skóra marmurkowata telangiektazja wrodzona
MCAP
MCM
MCMTC
Mózg olbrzymi - - skóra marmurkowata telangiektazja wrodzona
Zespół makrocefalia - malformacje kapilarne
Zespół wielkogłowie - malformacje kapilarne
MCM
MCMTC
Macrocephaly-capillary malformation syndrome
Macrocephaly-cutis marmorata telangiectatica congenita syndrome
Megalencephaly-capillary malformation syndrome
Megalencephaly-cutis marmorata telangiectatica congenita syndrome
ORPHA code
60040
OMIM code
602501
ICD10 code
Q87.3
ICD11 code
-

No additional description.

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