Anesthetic experience during reconstruction surgery of a chest wall defect in a patient with Poland syndrome - A case report -

Abstract

Poland syndrome, which is characterized by an absence of pectoralis muscles and their innervated nerves, is marked by variable chest wall defects due to deficits of 2nd, 3rd, 4th, and 5th ribs and costal cartilage. Additionally, there are other reported combined anomalies of the heart, lung, kidney, ipsilateral hand and foot associated with the syndrome. The lung on the side with the thoracic deformity is more hypoplastic or smaller than the lung on the unaffected side, which can cause herniation of the lung or paradoxical ventilatory movement in severe cases, thereby increasing the risk of respiratory depression and hypoxemia. Patients may have profound lung complications due to depressed respiratory muscle function and exacerbation of the underlying chronic pulmonary disease during the perioperative period. These patients also show increased risk of malignant hyperthermia and therefore require careful attention during general anesthesia. We report here an anesthetic experience during reconstruction surgery of a chest wall defect in a male patient with Poland syndrome.

Consensus based recommendations for diagnosis and medical management of Poland syndrome (sequence)

“…5 Clinical Genetics Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy. 6 Medical Genetic Unit, University Hospital of Modena, Modena, Italy. 7 Division of Medical Genetics, Fondazione IRCCS-Casa Sollievo della Sofferenza, San Giovanni Rotondo, Foggia, Italy.…”

“…By contrast, severely affected individuals with multiple muscle and bony abnormalities of the chest, upper limb or both are recognized at birth. In a minority of the cases, PS combines with extra-musculoskeletal congenital defects such as lung or kidney malformations and dextrocardia [6]. Rarely, chest and/or upper limb abnormalities of the PS type occur bilaterally [7].…”