Clinical manifestations of congenital insensitivity of the hand and classification of syndromes.
Lesions of the upper extremity, particularly of the hand, are common in congenital insensitivity syndromes. Five cases are described with findings including fractures, infections, stiffness of fingers, self-mutilation, and traumatic amputations. The best form of treatment is preventative, since reconstructive surgery has little to offer. If conservative efforts are of no avail, amputation of the part is, unfortunately, the best way to obtain satisfactory results.
D000293:Adolescent; D000673:Amputation, Traumatic / Q000209:etiology; D002675:Child, Preschool; D004402:Dysautonomia, Familial / Q000145:classification; D005260:Female; D050723:Fractures, Bone / Q000209:etiology; D006227:Hand Deformities, Acquired / Q000209:etiology*; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000145:classification / Q000150:complications*; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D000699:Pain Insensitivity, Congenital / Q000145:classification / Q000150:complications*; D012652:Self Mutilation / Q000209:etiology*; D013577:Syndrome