PERIOPERATIVE ANAESTHESIA CARE : IN POLAND SYNDROME - A RARE CASE REPORT.

Abstract

Poland syndrome (PS) is a rare musculoskeletal congenital anomaly with variable presentations . Incidence is 1/30,000 live births, more often seen in males than females (3:1). Several theories have been proposed regarding the genesis of PS as vascular disruption theory “ The subclavian artery disruption sequence”. Clinical features include cardiovascular (dextrocardia, atrial septal defects) musculoskeletal (club foot, toe syndactly, hemi vertebrae and scoliosis) genitourinary (renal aplasia and hypospadiasis) hemopoietic (spherocytosis and myelogenous leukemia, lymphoma) abnormalities. Absence of sternal head of pectoralis major muscle is an essential feature of PS and most often it manifests as ipsilateral thoracic cage and upper limb anomalies. Clinical features are phenotypically variable and manifestations are often subtle in infancy and childhood delaying their diagnosis and plan for intervention. PS patients does not always have functional defects but they present for cosmetic surgical repair where the safe anaesthetic intervention need to be planned .