Humeral agenesis/hypoplasia

Orpha code: 294973OMIM code:

Definition

Humeral agenesis/hypoplasia is a rare, non-syndromic limb reduction defect characterized by the unilateral or bilateral presence of a short arm with completely absent or underdeveloped humerus, frequently associated with ulnar and/or radial malformations. Patients may present with the appearance of the forearm directly attached to the shoulder, no articulation at the shoulder joint, impossible passive extension of the arm beyond the mid-axillary line, no elbow joints, bowing of the radius, a short ulna and/or ulnar/radial deviation of the hand at the wrist.

Disease data
Classification

Morphological anomaly

Synonyms
Congenital absence of humerus
Congenital hypoplasia of humerus
Humeral intercalary meromelia
Congenital absence of humerus
Congenital hypoplasia of humerus
Humeral intercalary meromelia
ORPHA code
294973
OMIM code
-
ICD10 code
Q71.8
ICD11 code
LB99.1

No additional description.

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