X-linked mandibulofacial dysostosis

Orpha code: 1131OMIM code: 301950

Definition

X-linked mandibulofacial dysostosis is an extremely rare multiple congenital abnormality syndrome that is characterized by microcephaly, malar hypoplasia with downslanting palpebral fissures, highly arched palate, apparently low-set and protruding ears, micrognathia, short stature, bilateral hearing loss, and learning disability. Occasionally, additional features have been observed such as bilateral cryptorchidism, cardiac valvular lesions, body asymmetry, and pectus excavatum.

Disease data
Classification

Malformation syndrome

Synonyms
Mandibulofacial dysostosis, Toriello type
Dyzostoza żuchwowo-twarzowa sprzężona z chromosomem X z anomaliami kończyn
Dyzostoza żuchwowo-twarzowa, typu Toriello
Zespół łuku skrzelowego sprzężonego z chromosomem X
X-linked branchial arch syndrome
X-linked mandibulofacial dysostosis with limb anomalies
ORPHA code
1131
OMIM code
301950
ICD10 code
Q75.4
ICD11 code
LD25.2

No additional description.

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