Anaesthetic challenges and difficult airway in a child with Poland’s syndrome

: Poland’s syndrome is an uncommon clinical disorder with a typical musculoskeletal deformity of thorax and ipsilateral upper limb with a variable spectrum of associated anomalies. Although patients of this musculoskeletal disorder have several features with potential anaesthetic risks including malignant hyperthermia, previous reports about anaesthetic management of these patients are limited. : We report the successful anaesthetic management of a child of Poland’s syndrome posted for surgical correction of club foot who had an anticipated difficult airway alongwith unilateral chest wall defect with paradoxical breathing and ipsilateral syndactyly and hence, posing challenges in the insertion of endotracheal tube, ventilatory management, regional anaesthesia and prevention of any risk of development of malignant hyperthermia. Paramount in the anaesthetic care of such patients is a thorough preoperative evaluation and a cautious intraoperative anaesthetic management. Avoidance of halogenated inhalational agents and depolarising neuromuscular blocking drugs, and use of controlled ventilation and regional anaesthetic techniques are recommended. The possibility of a compromised airway should always be kept in mind while anaesthetising these patients.

Poland’s syndrome mimicking traumatic chest injury

Poland’s syndrome (PS) is a rare developmental anomaly that can manifest mild (pectoralis muscles involvement) to severe deformities (rib hypoplasia and hand deformities). We report a case of 69-year-old man who presented to the emergency department with a traumatic chest injury after a fall. It was initially thought to have a significant chest injury as the trauma survey revealed a palpable defect and tenderness in the right anterior chest wall. There was also a symbrachydactyly deformity in the right hand. CT of the chest showed lack of right pectoralis muscles, which were consistent with PS. This case highlights the importance of gathering detail history in adult trauma patients such as congenital disorder especially in the presence of bony deformity. With possibilities of several traumatic conditions in trauma patients eliminated, one can expand the non-traumatic differential, keeping in mind the possibility of a congenital disorder that can mimic traumatic chest injury.

Reinforcing the vascular disruption theory of the genesis of Poland’s syndrome: a rare association of diaphragmatic eventration in a preterm infant with severe musculoskeletal defects

A preterm female infant was admitted at birth with respiratory distress. On examination, she had an asymmetric right chest wall and ipsilateral small hand. Air entry was reduced over the right chest. A clinical diagnosis of Poland’s syndrome was made based on the hypoplasia of the right pectoral muscles, absent nipple, deformed ribs and symbrachydactyly of the ipsilateral hand. Chest X-ray suggested and ultrasound confirmed eventration of the right hemidiaphragm. ‘Subclavian artery supply disruption sequence’ (SASDS) theory by Bavnick and Weaver remains the most accepted pathogenic mechanism in Poland’s syndrome. This case reinforces SASDS theory associated with the genesis of Poland’s syndrome that relates to the pathogenicity of vascular disruption of subclavian artery, characteristics of which are unilateral pectoral defects, symbrachydactyly and eventration of the diaphragm. At 2 months, she underwent diaphragm plication. She is under review by our multidisciplinary surgical team for reconstruction of the chest deformity.