Anestesia em paciente com insensibilidade congênita a dor e anidrose

Carlos Rogério Degrandi Oliveira; Valter César Paris; Renato Augusto Pereira; Felipe Souza Thyrso de Lara

doi:10.1590/S0034-70942009000500009

Clinical Information

Anestesia em paciente com insensibilidade congênita a dor e anidrose

Anesthesia in a patient with congenital insensitivity to pain and anhidrosis

Carlos Rogério Degrandi Oliveira; Valter César Paris; Renato Augusto Pereira; Felipe Souza Thyrso de Lara

http://dx.doi.org/10.1590/S0034-70942009000500009 Braz J Anesthesiol, vol.59, n5, p.602-609, 2009

PDF

Downloads: 0

Resumo

JUSTIFICATIVA E OBJETIVOS: A insensibilidade congênita a dor e anidrose (ICDA) ou neuropatia hereditária sensorial e autonômica tipo IV (NHSA tipo IV) é neuropatia autossômica recessiva rara do grupo das neuropatias hereditárias sensoriais e autonômicas (NHSA), caracterizada por insensibilidade ao estímulo doloroso, anidrose e retardo mental. Existem poucos relatos sobre a conduta anestésica em pacientes com ICDA devido sua extrema raridade. O objetivo deste relato foi apresentar a conduta anestésica em paciente com ICDA submetida à artrodese de tornozelo esquerdo com colocação de haste e discutir as características de interesse para a anestesia nestes pacientes. RELATO DO CASO: Paciente com história de ICDA foi admitida para artrodese de tornozelo esquerdo devido à artropatia de Charcot. Na sala de operação foi monitorizada com eletrocardiógrafo, índice bispectral, SEF 95%, pressão arterial não invasiva e saturação periférica da hemoglobina, medicada com midazolam como pré-anestésico e submetida à anestesia venosa com propofol e cisatracúrio. Não houve a necessidade de administração de analgésicos. Após intubação traqueal, foi acrescentada monitorização da pressão expiratória final do gás carbônico e da temperatura esofágica. Não apresentou complicações no período perioperatório. Teve alta hospitalar no segundo dia de pós-operatório. CONCLUSÕES: Embora apresentem insensibilidade à dor, alguns pacientes apresentam hiperestesia tátil, o que poderia causar sensações desagradáveis durante a manipulação cirúrgica. Apesar de relatos na literatura de pacientes submetidos a bloqueios no neuroeixo e até mesmo a procedimentos sem anestesia, neste caso utilizou-se a anestesia venosa que proporcionou condições adequadas para o procedimento anestésico-cirúrgico.

Palavras-chave

ANESTESIA, Geral, DOENÇAS, Congênita, DOENÇAS, Congênita

Abstract

ACKGROUND AND OBJECTIVES: Congenital insensitivity to pain and Anhidrosis (CIPA) or hereditary sensory and autonomic neuropathy type IV (HSAN IV) is a rare autosomal recessive neuropathy of the group of hereditary sensory and autonomic neuropathies (HSAN) characterized by insensitivity to pain, anhidrosis, and mental retardation. Since it is a rare condition, reports on the anesthetic conduct in patients with CIPA are not easily found in the literature. The objective of this report was to present the anesthetic conduct in a patient with CIPA undergoing left ankle arthrodesis with placement of an implant, and to discuss the characteristics of this disorder that concern anesthesiologists the most. CASE REPORT: A female patient with a history of CIPA was admitted for left ankle arthrodesis due to Charcot arthropathy. In the operating room, the patient was monitored with an electrocardiograph, bispectral index, 95% SEF, non-invasive blood pressure, and peripheral hemoglobin saturation; she was pre-medicated with midazolam and underwent intravenous anesthesia with propofol and cisatracurium. The administration of analgesics was not necessary. After tracheal intubation, monitoring of end-expiratory pressure of carbon dioxide and esophageal temperature were added. The patient did not develop postoperative complications. She was discharged from the hospital on the second postoperative day. CONCLUSIONS: Although there is insensitivity to pain, some patients present tactile hyperesthesia that can cause unpleasant feelings during surgical manipulation. Despite reports in the literature of patients undergoing neuroaxis blocks, and even procedures without anesthesia, intravenous anesthesia, which provided adequate conditions for the anesthetic-surgical procedure was used in this case.

Keywords

ANESHTESIA, General, DISEASES, Congenital, DISEASES, Congenital

Referencias

Swanson AG. Congenital insensitivity to pain with anhidrosis: A unique syndrome in two male siblings. Arch Neurol. 1963;8:299-306.

Indo Y, Tsuruta M, Hayashida Y. Mutations in the TRKA/NGF receptor gene in patients with congenital insensitivity to pain with anhidrosis. Nat Genet. 1998;13:485-488.

Hefti FF, Rosenthal A, Walicke PA. Novel class of pain drugs based on antagonism of NGF. Trends Pharmacol Sci. 2006;27:85-91.

Rosemberg S, Marie SKN, Kliemann S. Congenital insensitivity to pain with anhidrosis (Hereditary sensory and autonomic neuropathy type IV). Pediatr Neurol. 1994;11:50-56.

Tomioka T, Awaya Y, Nihei K. Anesthesia for patients with congenital insensitivity to pain with anhidrosis: a questionnaire study in Japan. Anesth Analg. 2002;94:271-274.

Shatzky S, Moses S, Levy J. Congenital insensitivity to pain with anhidrosis (CIPA) in Israeli-Bedouins: genetic heterogeneity, novel mutations in the TRKA/NGF receptor gene, clinical findings, and results of nerve conduction studies. Am J Med Genet. 2000;92:353-360.

Karkashan EM, Joharji HS, Al-Harbi NN. Congenital insensitivity to pain in four related Saudi families. Pediatr Dermatol. 2002;19:333-335.

Oliveira CRD, Santos FA, Nogueira CS. Spinal anesthesia in a patient with congenital insensitivity to pain with anhidrosis. Anesth Analg. 2007;104:1561-1562.

Kao SC, Ting CK, Cheng KW. Desflurane used in a patient with congenital insensitivity to pain with anhidrosis during septic shock. J Chin Med Assoc. 2004;67:305-307.

Okuda K, Arai T, Miwa T. Anesthetic management of children with congenital insensitivity to pain with anhidrosis. Pediatr Anaesth. 2000;10:545-548.

Rozentsveig V, Katz A, Weksler N. The anaesthetic management of patients with congenital insensitivity to pain with anhidrosis. Paediatr Anaesth. 2004;14:344-348.

Layman PR. Anaesthesia for congenital analgesia: A case report. Anaesthesia. 1986;41:395-397.

Rodriguez Pérez MV, Fernandes Daza PL, Cruz-Villaseñor JA. Anestesia epidural a un niño con fractura de fémur e insensibilidad congénita al dolor. Rev Esp Anestesiol Reanim. 2002;49:555-557.

Challands JF, Facer EK. Epidural anaesthesia and familial dysautonomia (the Riley Day syndrome): Three case reports. Paediatr Anaesth. 1998;8:83-88.

Nagasako EM, Oaklander AL, Dworkin RH. Congenital insensitivity to pain: an update. Pain. 2003;101:213-219.

Mitaka C, Tsunoda Y, Hikawa Y. Anesthetic management of congenital insensitivity to pain with anhidrosis. Anesthesiology. 1985;63:328-329.

Haworth AE, Thomas NH, Cook LJ. Hereditary sensory and autonomic neuropathy with anhidrosis (type IV). J R Soc Med. 1998;91:84-86.

Thakur LC, Chandran V, Anand KS. Congenital sensory neuropathy with anhidrosis. Indian Pediatr. 1992;29:1046-1048.

Yoshitake S, Matsumoto K, Miyagawa A. Anesthetic consideration of a patient with congenital insensitivity to pain with anhidrosis. Masui. 1993;42:1233-1236.

Trope GE, Jay JL, Dudgeon J. Self-inflicted corneal injuries in children with congenital corneal anaesthesia. Br J Ophthalmol. 1985;69:551-554.

Amano S, Fukuoka S, Usui T. Ocular manifestations of congenital insensitivity to pain with anhidrosis. Am J Ophthalmol. 2006;141:472-477.

Anestesia em paciente com insensibilidade congênita a dor e anidrose

Anesthesia in a patient with congenital insensitivity to pain and anhidrosis

Carlos Rogério Degrandi Oliveira; Valter César Paris; Renato Augusto Pereira; Felipe Souza Thyrso de Lara

Resumo

Palavras-chave

Abstract

Keywords

Referencias

Links

compartir

Braz J Anesthesiol