Spinal Anesthesia in a Patient with Congenital Insensitivity to Pain and Anhidrosis
Compartilhe!
5 curtiram
Autores: Carlos R Degrandi Oliveira, Gilberto W N Arenas*, Celso S Nogueira, Marcos H L Costa, Emerson J Mainardes
CET em Anestesiologia da Santa Casa de Santos – Santos – SP Brazil
Background – Congenital insensitivity to pain and anhidrosis (CIPA) is a rare, hereditary, autonomic recessive disorder resulting from the mutation of the neurotrophic tyrosine receptor kinase 1 gene (NTRK1). NTRK1 mutations cause defects in nerve growth factor signaling, leading to death of various nerve growth factordependent neurons (nociceptive sensory and autonomic sympathetic neurons) during the embryonal period. There are only a few reports concerning the anesthetic management of patients with CIPA. We report a 19-yr-old woman with CIPA receiving bilateral arthrodesis of the ankle with blade plate fixation under uneventful spinal anesthesia.
Case Report – A 19-yr-old woman, weighing 65 kg, with a history of CIPA, was admitted to our hospital due to joint deformities of both ankles. CIPA was not diagnosed until she was 8-yr old. Spinal anesthesia was placed through a 27G Whitacre needle at the L3–4 interspace, with the patient in the sitting position, using 4 ml of 0.5% isobaric bupivacaine. Sensory block could not be evaluated. The patient was placed in the supine position immediately after injection and 15 min later was positioned prone. Intraoperative hypotension was treated with a total of 15 mg of ephedrine administered IV in incremental boluses of 5 mg each. Her systolic blood pressure was maintained between 100 and 120 mm Hg and heart rate between 60 and 80 bpm. No analgesic was necessary, because of her insensitivity to pain. There were no anesthetic complications.
Discussion – Patients with CIPA lack pain sensation, but may have tactile hyperesthesia, which may produce unpleasant sensations during surgical manipulation. Mental retardation in CIPA can be absent or it may vary from mild to severe. In our case, spinal anesthesia was conducted with light sedation and a good clinical outcome. Had our patient been severely mentally retarded, general anesthesia would have been preferred.
References – 1. Miranda M, Di Virgilio M, Selleri S, et al. Novel pathogenic mechanisms of congenital insensitivity to pain with anhidrosis genetic disorder unveiled by functional analysis of neurotrophic tyrosine receptor kinase type 1/nerve growth factor receptor mutations. J Biol Chem 2002;277:6455– 62. 2. Rosemberg S, Marie SK, Kliemann S. Congenital insensitivity to pain with anhidrosis (hereditary sensory and autonomic neuropathy type IV). Pediatr Neurol 1994;11:50–6. 3. Tomioka T, Awaya Y, Nihei K, et al. Anesthesia for patients with congenital insensitivity to pain with anhidrosis: a questionnaire study in Japan. Anesth Analg 2002;94:271-4.
Contato: drdegrandi@uol.com.br
CET em Anestesiologia da Santa Casa de Santos – Santos – SP Brazil
Background – Congenital insensitivity to pain and anhidrosis (CIPA) is a rare, hereditary, autonomic recessive disorder resulting from the mutation of the neurotrophic tyrosine receptor kinase 1 gene (NTRK1). NTRK1 mutations cause defects in nerve growth factor signaling, leading to death of various nerve growth factordependent neurons (nociceptive sensory and autonomic sympathetic neurons) during the embryonal period. There are only a few reports concerning the anesthetic management of patients with CIPA. We report a 19-yr-old woman with CIPA receiving bilateral arthrodesis of the ankle with blade plate fixation under uneventful spinal anesthesia.
Case Report – A 19-yr-old woman, weighing 65 kg, with a history of CIPA, was admitted to our hospital due to joint deformities of both ankles. CIPA was not diagnosed until she was 8-yr old. Spinal anesthesia was placed through a 27G Whitacre needle at the L3–4 interspace, with the patient in the sitting position, using 4 ml of 0.5% isobaric bupivacaine. Sensory block could not be evaluated. The patient was placed in the supine position immediately after injection and 15 min later was positioned prone. Intraoperative hypotension was treated with a total of 15 mg of ephedrine administered IV in incremental boluses of 5 mg each. Her systolic blood pressure was maintained between 100 and 120 mm Hg and heart rate between 60 and 80 bpm. No analgesic was necessary, because of her insensitivity to pain. There were no anesthetic complications.
Discussion – Patients with CIPA lack pain sensation, but may have tactile hyperesthesia, which may produce unpleasant sensations during surgical manipulation. Mental retardation in CIPA can be absent or it may vary from mild to severe. In our case, spinal anesthesia was conducted with light sedation and a good clinical outcome. Had our patient been severely mentally retarded, general anesthesia would have been preferred.
References – 1. Miranda M, Di Virgilio M, Selleri S, et al. Novel pathogenic mechanisms of congenital insensitivity to pain with anhidrosis genetic disorder unveiled by functional analysis of neurotrophic tyrosine receptor kinase type 1/nerve growth factor receptor mutations. J Biol Chem 2002;277:6455– 62. 2. Rosemberg S, Marie SK, Kliemann S. Congenital insensitivity to pain with anhidrosis (hereditary sensory and autonomic neuropathy type IV). Pediatr Neurol 1994;11:50–6. 3. Tomioka T, Awaya Y, Nihei K, et al. Anesthesia for patients with congenital insensitivity to pain with anhidrosis: a questionnaire study in Japan. Anesth Analg 2002;94:271-4.
Contato: drdegrandi@uol.com.br