Unilateral longitudinal radial ray deficiency of the hand and metacarpal 4-5 synostosis.
We report 3 non related patients with severe hypoplasia/aplasia of the thumb with an ipsilateral synostosis of the fourth and fifth metacarpals. A review of few reports on this unusual association is presented and the possible pathogenetic mechanism is discussed.
De Smet
L
L
Center for Human Genetics, U.Z. Gasthuisberg, Leuven, Belgium.
Keymolen
K
K
Fryns
J P
JP
D000293:Adolescent; D000328:Adult; D005260:Female; D006228:Hand Deformities, Congenital; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008663:Metacarpus / Q000002:abnormalities*; D013580:Synostosis; D013933:Thumb / Q000002:abnormalities*