Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1016/j.bjane.2020.02.006
Brazilian Journal of Anesthesiology
Case Report

Anesthesia for a cesarean section on a pregnant patient with Cockayne syndrome: case report

Anestesia para cesárea em gestante com síndrome de cockayne: relato de caso

Viviane Barrada Ribeiro, Leonardo Teixeira Ribeiro Alonso de Faria, Roberta de Lima Machado, Bruno Mendonça Barcellos, Marco Antonio Cardoso de Resende, Rogério Luiz da Rocha Videira

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Abstract

Cockayne Syndrome is an autosomal recessive multi-systemic disorder due to DNA repair failure. It was originally described in 1936 in children of small stature, retinal atrophy and deafness, characterized by dwarfism, cachexia, photosensitivity, premature aging and neurologic deficits. The most typical feature is described as birdlike facies: protruding maxilla, facial lipoatrophy, sunken eyes, large ears and thin nose. Difficult airway management with subglottic stenosis and risk of gastric content aspiration has been described. Although the clinical characteristics of Cockayne Syndrome have been well described in pediatric publications, there is only one report in the literature on anesthesia for an obstetric patient. We report the case of a pregnant patient diagnosed with Cockayne Syndrome, submitted successfully to spinal anesthesia for a cesarean section due to cephalopelvic disproportion. In view of the difficult decision between inducing general anesthesia in a patient with a likely difficult airway, or neuraxial anesthesia in a patient with cardiovascular, respiratory and neurocognitive limitations, we suggest tailored management to reach the best results for the mother and newborn.

Keywords

Cockayne syndrome;  Difficult airway;  Obsteric anesthesia;  Pregnancy;  Progeria

Resumo

A síndrome de Cockayne é doença multissistêmica autossômica recessiva devido à falha no reparo do DNA. Originalmente descrita em 1936 em crianças de baixa estatura, atrofia retiniana e surdez, é caracterizada por nanismo, caquexia, fotossensibilidade, envelhecimento acelerado e déficits neurológicos. O mais típico é a fácies descrita como similar à de um pássaro: maxila proeminente, atrofia do coxim adiposo bucal, olhos profundos, orelhas grandes e nariz fino. Tem sido descrita dificuldade no manejo da via aérea com estreitamento subglótico e risco de aspiração gástrica. Embora as características clínicas da síndrome de Cockayne sejam bem relatadas em publicações pediátricas, há apenas um relato de anestesia em paciente obstétrica na literatura. Relatamos o caso de gestante com diagnóstico de síndrome de Cockayne, submetida com sucesso a raquianestesia para parto cesariano por desproporção cefalopélvica. Diante da difícil decisão entre induzir anestesia geral em paciente com provável via aérea difícil ou anestesia neuroaxial, em meio a limitações cardiovasculares, respiratórias e neurocognitivas da paciente, conduta individualizada é sugerida para alcançar os melhores resultados para a gestante e o neonato.

Palavras-chave

Síndrome de Cockayne;  Via aérea difícil;  Anestesia Obstétrica;  Gravidez;  Progeria

References

1. Pasquier L, Laugel V, Lazaro L, et al. Wide clinical variability among 13 new Cockayne syndrome cases confirmed by biochemical assays. Arch Dis Child. 2006;91:178-82.

2. Rawlinson SC, Webster VJ. Spinal anaesthesia for caesarean section in a patient with Cockayne syndrome. Int J Obstet Anesth. 2003;12:297-9.

3. Natale V. A Comprehensive Description of the Severity Groups in Cockayne Syndrome. Am J Med Genet Part A. 2011;155:1081-95.

4. Gaddam D, Thakur MS, Krothapalli N, et al. Case report dental management of a 14- year-old with Cockayne syndrome under general anesthesia. Case Rep Dent. 2014;2014:1-3.

5. Wilson BT, Stark Z, Sutton RE, et al. The Cockayne Syndrome Natural History (CoSyNH) study: clinical findings in 102 individuals and recommendations for care. Genet Med. 2016;18:483-93.

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Rev. Bras. Anestesiol.

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