ANAESTHETIC MANAGEMENT OF A 6-YEAR-OLD WITH MAROTEAUX-LAMY SYNDROME (MPS -VI) UNDERGOING ADENOTONSILLECTOMY AND INGUINAL HERNIA REPAIR

Dr. PRATIBHA MUDGAL; Dr. AKSHAYA KUMAR DAS; HIMANSHU GAUR

Volume : X, Issue : VIII, August - 2020

ANAESTHETIC MANAGEMENT OF A 6-YEAR-OLD WITH MAROTEAUX-LAMY SYNDROME (MPS -VI) UNDERGOING ADENOTONSILLECTOMY AND INGUINAL HERNIA REPAIR

Dr. Pratibha Mudgal, Dr. Akshaya Kumar Das, Himanshu Gaur

Abstract :

Mucopolysaccharidosis VI or MPS–VI is a subgroup of mucopolysaccharidosis which is inherited through autosomal recessive manner. The disease is due to mutation in the enzyme Arylsulfatase B (also called N–acetylgalactosamine–4–sulfatase). It leads to defect in metabolism of Glycosaminoglycans (GAGs) and accumulation of dermatan sulfate in different body tissue. Patient suffering from MPS–VI has some common features as like other types of mucopolysaccharidosis are short stature, typical course faces, spinal deformity, corneal clouding and hepatosplenomegaly. The other features which are specific to MPS–VI are valvular heart disease and normal cognitive function with no mental retardation. We are presenting the perioperative management of a child with MPS–VI for congenital inguinal hernia repair and adeno–tonsillectomy procedure. Difficult airway anatomy and presence of cardiac abnormalities are few challenges the anesthesiologist while managing these groups of patients.