Delayed membranous cranial ossification

Orpha code: 3034OMIM code: 155980

Definition

Delayed membranous cranial ossification is a rare, genetic primary bone dysplasia characterized by absent ossification of calvarial bones at birth and characteristic facial dysmorphisms (frontal bossing, hypertelorism, downward-slanting palpebral fissures, proptosis, flat nasal bridge, low-set ears, midface retrusion). Patients present a soft skull at birth which, over time, progressively ossifies and in adulthood typically results in a deformed skull (with brachycephaly and prominent occiput). No other skeletal abnormalities are associated and patients have normal cognitive and motor development.

Disease data
Classification

Malformation syndrome

Synonyms
Gonzales-del Angel syndrome
Zespół Gonzales del Angela
ORPHA code
3034
OMIM code
155980
ICD10 code
Q75.8
ICD11 code
LB70.Y

No additional description.

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