[Hereditary neuropathy with liability to pressure palsies (HNPP) in hand surgery: reminds and warn against a usually unrecognised disease].
Tomacula is a rare hereditary disease due to a deletion on chromosome 17. Clinical presentation varies but patients usually complain of recurrent paraesthesiaes and palsies related to compression or trauma of a peripheral nerve. Diagnosis is based on electrophysiological studies, nerve biopsies and genetic tests. Implications for the patient and family members are a genetic counselling and some simple preventive measures. Although there is no curative treatment for this neuropathy, surgery can be useful for decompression of nerves and neurolysis. However, the surgical act increases the risk of nerve damage. Knowing about the diagnosis can help the patient and the surgical team avoid causing lesions.
Lazar
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CC
Unité de chirurgie plastique, esthétique et reconstructrice, service de chirurgie de la main du Professeur-Milliez, centre hospitalo-universitaire Charles-Nicolle, 1, rue de Germont, 76031 Rouen cedex, France. lazarcalin@yahoo.fr
Auquit-Auckbur
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I
Milliez
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D002872:Chromosome Deletion; D002886:Chromosomes, Human, Pair 17 / Q000235:genetics; D006225:Hand / Q000503:physiopathology* / Q000601:surgery*; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000235:genetics*; D006801:Humans; D007431:Intraoperative Complications / Q000517:prevention & control*; D008297:Male; D008875:Middle Aged; D009408:Nerve Compression Syndromes / Q000209:etiology / Q000503:physiopathology / Q000517:prevention & control