Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1590/S0034-70942003000100006
Brazilian Journal of Anesthesiology
Clinical Information

Anestesia em paciente com Xeroderma Pigmentoso: relato de caso

Anesthesia in patient with Xeroderma Pigmentosum: case report

Carlos Rogério Degrandi Oliveira; Luciana Elias; Ana Cláudia de Melo Barros; Diogo Brüggemann da Conceição

http://dx.doi.org/10.1590/S0034-70942003000100006 Braz J Anesthesiol, vol.53, n1, p.46-51, 2003
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Resumo

JUSTIFICATIVA E OBJETIVOS: O Xeroderma Pigmentoso é uma doença autossômica recessiva rara, caracterizada pelo desenvolvimento prematuro de neoplasias devido à extrema sensibilidade à radiação ultravioleta. Estas manifestações ocorrem por falha no mecanismo de excisão e reparo do DNA. Se comparados a indivíduos normais, estes pacientes apresentam risco 1000 vezes maior de desenvolver neoplasias em áreas expostas ao sol. O objetivo deste relato é apresentar a conduta anestésica em uma paciente portadora de Xeroderma Pigmentoso submetida à cirurgia oftalmológica. RELATO DO CASO: Paciente do sexo feminino, 7 anos, portadora de Xeroderma Pigmentoso com comprometimento facial extenso, admitida para exérese de lesão papilar no olho direito. Foi prescrito midazolam (10 mg) por via oral, como medicação pré-anestésica. A monitorização inicial consistiu de cardioscópio, oxímetro de pulso, estetoscópio precordial e pressão arterial não invasiva. Foi realizada pré-oxigenação com oxigênio a 100% por 3 minutos e indução inalatória sob máscara com oxigênio a 100% e sevoflurano em concentrações crescentes até 7%. Após acesso venoso periférico com cateter 22G, foram injetados propofol (50 mg) e succinilcolina (20 mg) e realizada intubação traqueal com tubo 5,5 mm sem balonete. Um guia de metal foi utilizado para facilitar a introdução do tubo traqueal. A manutenção da anestesia foi feita com sevoflurano a 3,5% e oxigênio a 100%, com sistema de Bain. A criança foi extubada na sala cirúrgica e encaminhada à sala de recuperação pós-anestésica em boas condições. CONCLUSÕES: As alterações faciais e orofaríngeas decorrentes desta doença determinaram dificuldades na adaptação da máscara facial e intubação traqueal. A educação constante do paciente e de seus familiares constitui o objetivo mais importante no manuseio desta doença.

Palavras-chave

ANESTESIA, DOENÇAS

Abstract

BACKGROUND AND OBJECTIVES: Xeroderma Pigmentosum is a rare, autosomal recessive disease characterized by the premature development of neoplasias due to an exacerbated hypersensitivity to UV radiation. These manifestations are due to DNA excision and repair mechanism damage. As compared to normal individuals, these patients have a 1000-fold increased risk for developing neoplasias on sun-exposed areas. This report aimed at describing the anesthetic management of a patient with Xeroderma Pigmentosum submitted to ophthalmologic surgery. CASE REPORT: Female patient, 7 years of age, with Xeroderma Pigmentosum and extensive facial involvement, submitted to right eye papillomatous lesion excision. Patient was premedicated with 10 mg oral midazolam. Initial monitoring consisted of cardioscope, pulse oximetry, precordial stethoscope and noninvasive blood pressure. Patient was preoxygenated with 100% oxygen for 3 minutes and inhalational anesthesia was induced with sevoflurane under mask in incremental concentrations up to 7%. Peripheral venous access was achieved with a 22G catheter followed by intravenous 50 mg propofol and 20 mg succinylcholine and tracheal intubation with a 5.5 mm uncuffed tracheal tube. A guide wire was used to help tracheal tube introduction. Anesthesia was maintained with 3,5% sevoflurane and 100% oxygen with Bain's Circuit. Patient was extubated in the operating room and was sent to the post-anesthetic care unit in good conditions. CONCLUSIONS: Facial and oropharyngeal changes caused by this pathology have imposed many difficulties for facial mask adaptation and tracheal intubation. Patient and relatives continuous education are the most important Xeroderma Pigmentosum management objective.

Keywords

ANESTHESIA, DISEASES

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