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DOI 10.1016/j.bjan.2019.10.002

Медицинская библиотека · Brazilian journal of anesthesiology (Elsevier) · 2020

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

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

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Аннотация

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.

Для цитирования: Ribeiro Viviane Barrada, Faria Leonardo Teixeira Ribeiro Alonso de, Machado Roberta de Lima, Barcellos Bruno Mendonça, Resende Marco Antonio Cardoso de, Videira Rogério Luiz da Rocha. [Anesthesia for a cesarean section on a pregnant patient with Cockayne syndrome: case report].. Brazilian journal of anesthesiology (Elsevier). 2020. DOI: 10.1016/j.bjan.2019.10.002.

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