Revista Cubana de Anestesiología y Reanimación
versão On-line ISSN 1726-6718
LOPEZ RIVERO, Jaime et al. Anesthesia in case of type 1 neurofibromatosis. Rev cuba anestesiol reanim [online]. 2011, vol.10, n.2, pp. 165-172. ISSN 1726-6718.
Introduction: The type 1 neurofibromatosis or Von Recklinghausen's neurofibromatosis is the more frequent neurocutaneous disorder, present in approximately 1 of each 3000 persons. It is a genetic syndrome with an autosomal dominant way of transmission. Criteria for the clinical diagnosis were made in 1988, from two or more spots of white coffee color larger than 5 millimeters diameter in postpubertal subjects, two or more neurofibroma of any type or a plexiform neurofibroma, axillary freckles or in the inguinal region, optical glioma; two or more Lisch's nodules, bone lesions such as sphenoid dysplasia or a thin cortex of large bones with or without pseudoarthrosis, as well as a first grade relative blood relation diagnosed with type 1 neurofibromatosis. Objective: To present the operative behavior of patients presenting with type 1 neurofibromatosis. Clinical case: IRA white male patient aged 71 with a pathologic history of type 1 neurofibromatosis diagnosed from 15 years old with a left heart insufficiency, high blood pressure and chronic auricular fibrillation with a normal ventricular response. Diagnosis of lumbar neurofibroma was made carrying out a total exeresis of the lesion. Conclusions: The peri-anesthetic conduction of patient diagnosed with Von Recklinghausen's disease is a challenge for the anesthesiologist.
Palavras-chave : Type 1 neurofibromatosis; anesthesia; perioperative complications.