Isolated congenital syngnathia

Orpha code: 141214OMIM code:

Definition

Isolated congenital syngnathia is a very rare developmental defect during embryogenesis disorder characterized by varying degrees of congenital fusion (ranging from simple mucosal adhesions to extensive bony fusion) of mandible to maxilla that is not associated with any other malformations. Patients present with mouth opening limitation (which could range from severe to minimal restriction) that typically results in feeding, swallowing and/or respiratory difficulties which may lead to failure to thrive, malnutrition and/or temporomandibular joint ankylosis.

Disease data
Classification

Malformation syndrome

Synonyms
Isolated congenital maxillomandibular fusion
Wrodzony zrost szczękowo-żuchwowy
ORPHA code
141214
OMIM code
-
ICD10 code
Q67.4
ICD11 code
-

No additional description.

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