Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1590/S0034-70942004000600008
Brazilian Journal of Anesthesiology
Clinical Information

Anestesia em paciente portadora de síndrome de Mckusick-Kaufman: relato de caso

Anesthesia in Mckusick-Kaufman syndrome patient: case report

Adriano Bechara de Souza Hobaika; Ziltomar Donizetti Borges; Vera Coelho Teixeira

http://dx.doi.org/10.1590/S0034-70942004000600008 Braz J Anesthesiol, vol.54, n6, p.799-801, 2004
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Resumo

JUSTIFICATIVA E OBJETIVOS: A síndrome de Mckusick-Kaufman é uma doença rara, caracterizada tipicamente por hidrometrocolpos, polidactilia e defeitos cardíacos congênitos. Pacientes portadores desta doença podem ser submetidos a diversos procedimentos cirúrgicos durante a sua vida e o anestesiologista deve estar preparado para possíveis alterações. O objetivo deste artigo é relatar a conduta anestésica adotada em uma paciente portadora desta síndrome. RELATO DO CASO: Paciente do sexo feminino de 11 anos, 37 kg, portadora da síndrome de Mckusick-Kaufman, insuficiência renal crônica, encefalopatia hipertensiva e asma grave submetida à retirada de cateter peritoneal infectado e confecção de fístula arteriovenosa. História pregressa de intubação prolongada. A anestesia foi induzida com alfentanil (1 mg), propofol (50 mg) e atracúrio (25 mg) e mantida com sevoflurano (2% a 4%) e doses fracionadas de alfentanil. A traquéia foi intubada sem complicações e a extubação foi realizada na sala de cirurgia após o retorno satisfatório da função neuromuscular. CONCLUSÕES: Apesar de a síndrome de Mckusick-Kaufman tratar-se de uma associação variável de defeitos congênitos, alguns cuidados anestésicos comuns podem ser definidos. Este caso apresentou fatores complicadores da anestesia e a indução com propofol e alfentanil e a manutenção com sevoflurano proporcionaram à paciente uma anestesia com mínimas repercussões ventilatórias e hemodinâmicas.

Palavras-chave

ANESTESIA, ANESTESIA

Abstract

BACKGROUND AND OBJECTIVES: Mckusick-Kaufman syndrome is an uncommon disease, typically characterized by hydrometrocolpos, polydactyly and congenital heart defects. These patients are often submitted to different surgical procedures throughout their lives and the anesthesiologist must be prepared to deal with possible complications. This article aimed at reporting the anesthetic management adopted for a patient with this syndrome. CASE REPORT: A 11-year-old, 37 kg, female with Mckusick-Kaufman syndrome, chronic renal failure, hypertensive encephalopathy and severe asthma was scheduled for surgical arterial-venous fistula preparation and removal of infected peritoneal dialysis catheter. Previous prolonged tracheal intubation was reported. Anesthesia was induced with alfentanil (1 mg), propofol (50 mg) and atracurium (25 mg) and was maintained with inhalational sevoflurane (2% to 4%) and intermittent IV alfentanil doses. Trachea was intubated without intercurrences and extubation was performed in the operating room after satisfactory neuromuscular function recovery. CONCLUSIONS: Although Mckusick-Kaufman syndrome is a variable association of congenital defects, some standard anesthetic cares may be defined. This specific case presented complicating factors for anesthesia, and induction with propofol and alfentanil and maintenance with sevoflurane have provided the patient a perioperative period with minimal ventilatory and hemodynamic repercussions.

Keywords

ANESTHESIA, DISEASES

References

David A, Bitoun P, Lacombe D. Hydrometrocolpos and polydactyly: a common neonatal presentation of Bardet-Biedl and McKusick-Kaufman syndromes. J Med Genet. 1999;36:599-603.

Pul N, Pul M, Gedik Y. McKusick-Kaufman syndrome associated with esophageal atresia and distal tracheoesophageal fistula: a case report and review of the literature. Am J Med Genet. 1994;49:341-343.

el Hammar F, Dubreuil M. Benoit I et al - A difficult intubation of an infant with McKusick-Kaufman syndrome. Failure of the laryngeal mask-fibroscope sequence. Ann Fr Anesth Reanim. 1998;17:240-242.

Cuyler JP, Giovanetto DR. Acute respiratory failure secondary to a complete tracheal ring. J Otolaryngol. 1992;21:54-55.

Brouard J, Voirin J, Laloum D. McKusick-Kaufman syndrome and fatal congenital tracheal stenosis. Arch Fr Pediatr. 1988;45:373.

Ortiz JR, Garcia J, Archilla J. Latex allergy in anesthesiology. Rev Esp Anestesiol Reanim. 1995;42:169-174.

Esteban RMP, Losa JMO, Celis FP. The McKusick- Kaufman syndrome: a report of five new cases, including a male. An Esp Pediatr. 1996;44:493-495.

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