RESUMO
Management of an anticipated difficult airway in Hurler syndrome. Hurler syndrome is a subtype of Mucopolysaccharidosis (MPS) type 1. Mucopolysaccharidosis (lysosomal storage diseases) are a group of inherited disorders caused by deficiency of specific lysosomal enzyme required for a normal degradation of glycosaminoglycons (GAGs). Administration of general anaesthesia in patients who have congenital syndromes such as Hurler's is often a challenge because of progressive airway, craniofacial and skeletal abnormalities that may make both the ventilation and intubation difficult. We encountered difficult mask ventilation and endotracheal intubation was not possible and finally ventilated with laryngeal mask airway in a known case of Hurler syndrome posted for umbilical hernia repair.
RESUMO
Eisenmenger's syndrome is a cyanotic congenital heart disease that includes pulmonary hypertension with reversed or bidirectional shunt associated with septal defects or patent ductus arteriosus. The decreased systemic vascular resistance associated with pregnancy increases the degree of right to left shunting, thereby carrying substantial risk to both the mother and the foetus. The maternal mortality rate of pregnancy in the presence of Eisenmenger's syndrome is reported to be as high as 30-70%. We present a case of a 22-year-old primigravida with Eisenmenger's syndrome who gave birth at 37 weeks of gestation via caesarean section to a live female baby under general anaesthesia. On the third post-operative day, the patient developed tachycardia, tachypnoea, hypotension and decrease in oxygen saturation despite supplemental oxygen, clinically suspected pulmonary thromboembolism. We describe the anaesthetic management for caesarean section and its complications in a patient with Eisenmenger's syndrome. Although pregnancy should be discouraged in women with Eisenmenger's syndrome, it can be successful.