Anaesthetic challenges in a child with a rare triad of kyphoscoliosis, neurofibromatosis and sickle cell trait

Abstract

Scoliosis is a complex deformity of spine and may be of varied etiology. It often poses a challenge in its anaesthetic management. We present anaesthetic management of a child who underwent scoliosis reconstruction with a rare triad of kyphoscoliosis, Neurofibromatosis type 1 (NF1, von Recklinghausen’s disease) and sickle cell trait. Anaesthetic management of this case is challenging as it focuses on systemic changes particularly, cardiovascular, respiratory and nervous systems; related to deformity, pharmacological alteration due to Neurofibromatosis, precautions related to sickle cell trait as under unusual circumstances serious morbidity or mortality can result from complications related to polymerization of deoxy-hemoglobin S and the younger age group. Anaesthetic management in these patients with this triad was focussed primarily on optimisation of cardio-respiratory functions. Comprehensive pre-operative strategy and perioperative management of all coexisting diseases enables a successful outcome after the surgery.