Background and Goal of Study: Neurofibromatosis type 1 (NF1) is a common inherited autosomal-dominant disorder with an incidence of 1:3000. With variable severity, lesions may involve all the physiologic systems (airway, respiratory, cardiovascular, musculocutaneous, peripheral and central nervous system). Anaesthetic management should consider the multiple clinical presentation of the disease. The aim of this study was to describe a five-year experience of regional anaesthesia in NF1 patients in a tertiary hospital.
Materials and Methods: Retrospective analysis of electronic records of patients with NF1 who underwent surgery, between January/2004 and January/2009. Demographic data, physical status (ASA), NF1-related disorders, preoperative CNS imaging, type of surgery, type of regional anaesthetic technique and perioperative complications were collected.
Results and Discussion: Twenty six patients with NF1 were scheduled for a total of 58 surgical procedures. Neuraxial anaesthesia was performed in 4 patients (6%) - 2 lumbar epidural blocks (LEB); 2 spinal blocks (SB) - and combined technique (balanced with lumbar epidural) in 1 patient. Non-elective surgery was done in 3% of cases. All patients submitted to regional anaesthesia had clinical and radiologic exclusion of intracranial and spinal lesions. Case 1: Male, 61y, ASA II, with scoliosis, BMI:21 kg/m2, submitted to bilateral inguinal hernia repair, under SB with 0,5% hyperbaric bupivacaine (15 mg) - unsuccessful blockade, requiring conversion to general anaesthesia and systemic analgesia. Case 2: Male, 73y, ASA hypertension and left branch block, BMI: 28 kg/m2, submitted to bilateral inguinal hernia repair, under SB with 0,5% hyperbaric bupivacaíne (10 mg) and fentanyl (0,02 mg) - uneventful. Case 3: Female, 27y, ASA II, nulliparous, BMI:29 kg/m2, labour analgesia with LEB with boluses of 0,2% ropivacaíne (total dose: 40 mg) - uneventful. Case 4: Female, 23y, ASA II, nulliparous, BMI: 24 kg/m2, labour analgesia with LEB with boluses of 0,25% laevobupivacaíne (total dose: 37,5 mg) - uneventful. Case 5: Male, 73y, ASA II, history of epilepsy, submitted to radical prostatectomy under combined technique with balanced general anaesthesia and LEB with 0,75% ropivacaine continuous infusion (total dose: 112,5 mg) and epidural morphine (3 mg) - uneventful.
Conclusion(s): Despite technical difficulties due to related disorders, regional anaesthesia can be safely performed in NF-1 patients, even in labour analgesia, if imagiological exclusion of central nervous system lesions is assured.