ABSTRACT
Abstract Mucopolysaccharidosis (MPS) are a group of rare genetic inherited diseases with a progressive course due to the accumulation of glycosaminoglycans resulting in anatomic abnormalities and organ dysfunction, including the respiratory, cardiovascular, skeletal, and neurological systems that can increase the risk of anesthesia complications. Clinical manifestations are variable, multisystemic, and include severe morphological changes. The anesthetic management of these patients is complex, particularly airway management, which can be planned to include a fiberoptic airway investigation prior to surgery. We present two cases of patients with MPS type VI and VII who underwent fiberoptic airway mapping under conscious sedation, with no complications. Since MPS is a rare but challenging disease concerning the airway management, we propose a safe and effective anesthetic technique that could be used for fiberoptic bronchoscopy and allow fiberoptic-assisted tracheal intubation at the time of surgery.
Subject(s)
Humans , Wakefulness , Mucopolysaccharidoses/complications , Bronchoscopy/methods , Airway Management/methods , Intubation, Intratracheal/methodsABSTRACT
Abstract The Ex Utero Intrapartum Treatment (EXIT) is a surgical procedure performed in cases of expected postpartum fetal airway obstruction, allowing the establishment of patent airway while maintaining placental circulation. Anesthesia for EXIT procedure has several specific features such as adequate uterine relaxation, maintenance of maternal blood pressure fetal anesthesia and fetal airway establishment. The anesthesiologist should be aware of these particularities in order to contribute to a favorable outcome. This is a case report of an EXIT procedure performed on a fetus with a cervical lymphangioma with prenatal evidence of partial obstruction of the trachea and risk of post-delivery airway compromise.
Resumo O procedimento Intraparto Extra-Uterino (EXIT) é procedimento cirúrgico realizado em casos de previsão de obstrução de via aérea fetal no pós-parto, que permite estabelecer via aérea patente enquanto a circulação placentária é mantida. A anestesia para o procedimento EXIT apresenta várias características específicas, tais como relaxamento uterino adequado, manutenção da pressão arterial materna, anestesia fetal e estabelecimento da via aérea fetal. O anestesiologista deve estar ciente dessas especificidades para contribuir para desfecho favorável. Trata-se de relato de caso de procedimento EXIT realizado em feto com linfangioma cervical e evidência pré-natal de obstrução parcial de traqueia e risco de comprometimento de via aérea pós-parto.