A Child With Moyamoya Disease-A Case Study.

Abstract

Pediatric palliative care is a narrow subspeciality dealing with specialized comprehensive care approach for children living with chronic life-limiting illness and their families. Moyamoya disease with extracranial involvement is one such rare disease, where the most appropriate curative treatment is not yet determined. It is a vaso-occlusive disorder affecting intracranial vessels commonly, and rarely extracranial vessels. It is considered as one of the infrequent causes of acute stroke in children. We report a severe case of Moyamoya disease in a child, affecting both cerebral and extra-cranial vessels. The child had renal vessel involvement, which led to renovascular hypertension and end-stage renal disease. Considering the intricacies of complicated transplant outcomes, parents opted for pediatric palliative care. Bearing in mind the poor outcome of the extensive disease and need for regular follow-up, periodic counselling is imperative in successful management of children with Moyamoya disease. Given the progressive and relentless nature of the child's disease, a coordinated, multifaceted management is required to optimize the physical, psychological and social functioning of these children and their families.