OBSOLETE: Congenital bowing of long bones-short stature-dolichomacrocephaly-ocular hypertelorism syndrome

Orpha code: 2292OMIM code: 264050

Definition

A rare primary bone dysplasia characterized by congenital symmetric or asymmetric shortness and bowing of long bones, resulting in shortness of limbs and limited extension at the knees and elbows. Additional reported features are ''beaten metal'' appearance of the skull, dolichomacrocephaly, ocular hypertelorism, and anterior beaking and bone-within-bone appearance of vertebrae. There have been no further descriptions in the literature since 1993.

Disease data
Classification

Malformation syndrome

Synonyms
Wrodzony zespół wygięcia kości długich, niskiego wzrostu, dolichomakrocefalii i hiperteloryzmu ocznego
ORPHA code
2292
OMIM code
264050
ICD10 code
Q68.5
ICD11 code
LB96.1

No additional description.

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