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

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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

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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

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