Abstract

LUCIO-MEDINA, IM et al. Morquio syndrome in cesarean section under general anesthesia: a case report. Anest. Méx. [online]. 2019, vol.31, n.1, pp.56-61. ISSN 2448-8771.

The mucopolysaccharidoses (MPS) are an heterogeneous group of metabolic disorders, usually inherited in an autosomal recessive manner, characterised by the lack of enzymes useful for decomposition of glycosaminoglycans (GAGs) (1). Resulting from abnormalities of the chondrogenesis and osteogenesis rear (Dysostosis multiplex) are largely responsible for the typical phenotype with visible elements such as short stature, kyphoscoliosis, chest prominent campaign/barrel-shaped, frontal bossing, genu valgum (unconscious knees) and laxity in the joints (6). We present the case of a patient for resolution of pregnancy carrying a Mucopolysaccharidosis type IV. The lack of evidence literature about this type of obstetric patients, is the reason why we share our experience in the management of this disease.

Keywords : Morquio syndrome; Mucopolysaccharidosis and pregnancy; general anesthesia; difficult airway.

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