[A new case of macrodactyly of the hand reported in France. An attempt at a statistical study (author's transl)].
A boy of 4 years presented with hypertrophy of the last three fingers of the right hand associated with syndactyly of the middle and ring fingers. The fingers were of adult length and wider. Arteriography did not show any evidence of an aneurism but demonstrated the existence of an extensive collateral circulation. Thermography confirmed the hypervascularization with the presence of hot points. The authors found reports on 118 cases in the literature published during the last century which could be analyzed statistically. There is a slight predominance in boys (55%) and on the right side. Two fingers are usually involved, the index and middle finger being those affected. Discussions as to aetiology (GOURNET) usually vary between a neurological cause with the possibility of a fibrolipoma of the middle finger being present, and it being due to a phacomatosis. Effective therapy in adults is amputation. In the young there are several possibilities; ablation of the neurovascular masses at the base of the fingers (even though the metacarpals are often involved), destruction of proliferating cartilage, etc.
D000792:Angiography; D002675:Child, Preschool; D003097:Collateral Circulation; D005385:Fingers / Q000002:abnormalities* / Q000098:blood supply / Q000000981:diagnostic imaging / Q000294:innervation; D006227:Hand Deformities, Acquired / Q000209:etiology; D006801:Humans; D008297:Male; D013817:Thermography
Un nouveau cas français de macrodactylie de la main. Essai d'étude statistique de l'affection.