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

Abstract

IntroductionPoland 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.AimWe would like to highlight the rarity of Poland syndrome, and the challenges of post-operative recovery, primarily the ventilation.Case studyWe 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.Results and discussionThe 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.ConclusionsPoland 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.