Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1590/S0034-70942007000400007
Brazilian Journal of Anesthesiology
Clinical Information

Anestesia em paciente com síndrome de Marshall-Smith: relato de caso

Anesthesia in a patient with Marshall-Smith syndrome: case report

Beatriz Lemos da Silva Mandim; Neuber Martins Fonseca; Roberto Araújo Ruzi; Paulo Cezar Silva Temer

http://dx.doi.org/10.1590/S0034-70942007000400007 Braz J Anesthesiol, vol.57, n4, p.401-405, 2007
PDF Português
PDF English
Downloads: 0
Views: 1720

Resumo

JUSTIFICATIVA E OBJETIVOS: A síndrome Marshall-Smith é uma doença rara, caracterizada por dismorfismo facial, acelerada maturação óssea, atraso no desenvolvimento neuropsicomotor e anormalidade das vias aéreas. Os pacientes com essa síndrome apresentam grande probabilidade de complicações anestésicas, sobretudo com relação ao manejo das vias aéreas. Há poucos dados na literatura anestésica a respeito desta síndrome. O objetivo deste relato foi apresentar as dificuldades e a conduta anestésica em uma criança de 28 dias, portadora dessa síndrome, submetida à cirurgia para correção de atresia de coanas sob anestesia geral. RELATO DO CASO: Criança do sexo masculino, 28 dias de vida, 2,8 kg, submetida à anestesia geral para correção cirúrgica de atresia de coanas. Apresentava as características típicas da síndrome Marshall-Smith como tórax estreito, pectus escavatum, mãos e pés grandes, pescoço comprido, dismorfismo facial, palato alto e estreito e acelerada maturação óssea. A indução anestésica foi inalatória sob máscara com O2 a 100% associado ao sevoflurano. Devido à possibilidade de intubação difícil, foi programada intubação traqueal com fibrobroncoscópio. Após intubação traqueal e ventilação assistida manual, foi administrado 1,5 mg de rocurônio e, passados dez minutos, o paciente apresentou bradicardia (80 bpm) e hipóxia acentuada (30% de saturação de O2) e impossibilidade de ventilação manual através do tubo traqueal, sendo necessária a realização de traqueostomia de urgência, quando se optou por suspender o procedimento cirúrgico. CONCLUSÃO: Em casos de emergência anestésico-cirúrgica, nos quais a criança não ventila e não é possível a intubação traqueal, ocorre dessaturação com bradicardia associada e a tomada de decisão deve ser rápida e apropriada para garantir uma ventilação pulmonar adequada. Esses pacientes necessitam avaliação cuidadosa das vias aéreas para identificação de obstrução alta e baixa. Durante a anestesia, a ventilação espontânea deve ser mantida durante a indução até que seja possível o controle das vias aéreas evitando a utilização de bloqueadores neuromusculares.

Palavras-chave

ANESTESIA, DOENÇAS

Abstract

BACKGROUND AND OBJECTIVES: The Marshall-Smith Syndrome is a rare disease characterized by facial dysmorphism, accelerated osseous maturation, retarded neuropsychomotor development, and abnormalities of the airways. Patients with this syndrome have a high risk of developing anesthetic complications, especially concerning the maintenance of the airways. There are very few data in the anesthetic literature regarding this syndrome. The objective of this report was to show the difficulties and anesthetic management in a 28-day old child with this syndrome, who underwent surgery for correction of choanal atresia under general anesthesia. CASE REPORT: A male child, 28 days old, weighing 2.8 kg, undergoing general anesthesia for surgical correction of choanal atresia. The child presented the typical manifestations of the Marshall-Smith syndrome, with a narrow thorax, pectus excavatum, large hands and feet, long neck, facial dysmorphism, high and arched palate, and accelerated osseous maturation. Anesthetic induction was done with a mask with 100% O2 associated with sevoflurane. Due to the possibility of a difficult intubation, tracheal intubation with a fibrobronchoscope was scheduled. After tracheal intubation and assisted manual ventilation, 1.5 mg of rocuronium were administered and, after ten minutes, the patient developed bradycardia (80 bpm), severe hypoxemia (O2 saturation of 30%), and manual ventilation through the tracheal tube became impossible. An urgent tracheostomy was done and the surgical procedure was cancelled. CONCLUSION: In cases of anesthetic-surgical emergencies, in which the child does not ventilate and tracheal intubation is not possible, there is desaturation and bradycardia, requiring fast and appropriate decision making to guarantee adequate pulmonary ventilation. These patients need careful evaluation of the airways to identify upper and lower airways obstruction. During anesthesia, spontaneous ventilation should be maintained during induction until control of the airways is possible, avoiding the use of neuromuscular blockers.

Keywords

ANESTHESIA, DISEASES

References

Dernedde G, Pendeville P, Veyckemans F. Anaesthetic management of a child with Marshall-Smith syndrome. Can J Anaesth. 1998;45:660-663.

Roodhooft AM, Van Acker KJ, Van Thienen MN. Marshall-Smith syndrome: new aspects. Neuropediatrics. 1988;19:179-182.

Jonson JP, Carey JC, Glassy FJ. Marshall-Smith syndrome: two case reports and a review of pulmonary manifestations. Pediatrics. 1983;71:219-223.

Marshall RE, Graham CB, Scott CR. Syndrome of accelerated skeletal maturation and relative failure to thrive: a newly recognized clinical growth disorder. J Pediatr. 1971;78:95-101.

Charon A, Gillerot Y, Van Maldergem L. The Marshall-Smith syndrome. Eur J Pediatr. 1990;150:54-55.

Antila H, Laitio H, Aantaa R. Difficult airway in a patient with Marshall-Smith syndrome. Paediatr Anaesth. 1998;8:429-432.

Adam MP, Hennekam RC, Keppen LD. Marshall-Smith syndrome: natural history and evidence of an osteochondrodysplasia with connective tissue abnormalities. Am J Med Gen. 2005;137A:117-124.

Cullen A, Clarke TA, O'Dwyer TP. The Marshall-Smith syndrome: a review of the laryngeal complications. Eur J Pediatr. 1997;156:463-464.

Sperli D, Concolino D, Barbato C. Long survival of a patient with Marshall-Smith syndrome without respiratory complications. J Med Genet. 1993;30:877-879.

Washington K, Rourk MH, McDonagh D. Inflammatory cloacogenic polyp in a child: part of the spectrum of solitary rectal ulcer syndrome. Pediatr Pathol. 1993;13:409-414.

Frankville DD. Congenital malformations: the usual and unusual. ASA Refresher Courses Anesthesiol. 2001;29:123-133.

Butler MG, Hayes BG, Hathaway MM. Specific genetic diseases at risk for sedation/anesthesia complications. Anesth Analg. 2000;91:837-855.

5dd824eb0e8825fa1013f288 rba Articles
Links & Downloads
2352-2291 (Electronic) 0104-0014 (Printed)
Braz J Anesthesiol ©2024 All rights reserved.

Braz J Anesthesiol

Share this page
Page Sections