Difficulty in the management of congenital insensitivity to pain

We report a case of hereditary sensitive neuropathy associated with insensitivity to pain in an infant Case report. - The girl was born after a normal full term pregnancy. She was hospitalized in the neonatal period because of hypotonia and recurrent cyanotic episodes due to false passage. The diagnosis of insensitivity to pain was suspected at nine months of age, as parallel with dentition, multiple mutilations of the tongue and the fingers were observed. The diagnosis was confirmed by biopsy taken from the sural nerve which showed a rarefaction of small myelinated fibres whereas unmyelinated fibres remained unaltered. At the age of six years, the general condition was good and the neurologic developpement was satisfactory, neurotrophic and urologic complications currently being the main problem. Conclusion. - A multidisciplinary and specialized care associated with parental compliance was necessary to minimize the complications of this potentially severe disorder (C) 2002 Editions scientifiques et medicales Elsevier SAS.