Poland syndrome: A case of difficult weaning of ventilation after surgery

Abstract

Poland syndrome is a rare congenital anomaly that occurs in 1 in 30,000 children. It is characterised by unilateral underdevelopment or lack of the chest wall muscle that is apparent at birth. Rarely is it discovered to involve a bilateral chest muscular deficit. Poland syndrome presents in a wide variety of ways, which can often make diagnosis challenging.We would like to highlight the rarity of Poland syndrome, and the challenges of post-operative recovery, primarily the ventilation.We presented a case of a 44-year-old female with non-investigated chest wall deformity, whom required emergency surgical intervention under general anaesthesia. Post-operatively, she required prolonged ventilatory support, and was then diagnosed with Poland syndrome.The patient had undergone an emergency laparotomy and left lateral hepatectomy for left hepatolithiasis with liver abscess. Post-operation, she required prolonged ventilation, with gradual weaning of oxygen. Imaging studies showed absence of left pectoralis major, thus a diagnosis of Poland syndrome. Gradually she was weaned off and had a successful post operative recovery.Poland syndrome is a rare congenital abnormality. In patients undergoing general anaesthesia, the normal mechanics of breathing while ventilated are absent. Their recovery is longer and requires prolonged ICU admission.