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Multiple Exostosesof the Ribs: Video-assisted Thoracoscopy Excision

February 19, 2016

Contributors: Luca Labianca, MD; Daniele Mazza, MD; Cosma Calderaro, MD; Carlo Iorio, MD; Antonello Montanaro, MD; Francesco Turturro, MD; Daniele Fabbri, MD; Andrea Ferretti, MD

Multiple hereditary exostoses (MHE) is an autosomal dominant disorder characterized by formation of ectopic, cartilage-capped, growth plate-like exostoses. The aim of this video is to report an exceptional case of multiple exostoses of the ribs in a young patient affected by MHE. A 16 years old patient with familiar history of MHE came to our observation for a left-sided chest atraumatic pain. The chest x-ray and CT revealed exostoses, which were located at the left third, fourth, and sixth ribs with the bone cortex intact. Under general anesthesia, a double-lumen endotracheal tube was used for unilateral ventilation. A skin incision was made in the most superior aspect of the axilla posterior to the pectoralis major muscle and anterior to the latissimus dorsi muscle. The fifth intercostal space immediately beneath the skin incision was and the intercostal muscles were separated from the superior aspect of the rib to avoid nerve injury. Three significant exostoses were identified within the left side of the chest by thoracoscopy. They scratched the pericardium during cardiac pulsations under unilateral ventilation. Each exostosis has been resected using a chisel through an additional 4-cm long mini-thoracotomy incision, to prevent organ injury and recurrent exostosis. The lung is re-expanded to evaluate correct ventilation. Pathological examination showed costal exostoses, measuring: 2 cm x 1 cm in the third rib, 2 cm x 0.5 cm in the fourth one, and 2.5 cm x 1.5 cm in the sixth one. Rarely ribs, spine, metatarsals, metacarpals, phalanges are involved by MHE, but axial sites are sites of increased risk of malignant transformation. Osteochondromas grow in size and gradually ossify during skeletal development and stop growing with skeletal maturity, after which no new osteochondromas develop. Exostoses require no therapy in the absence of clinical problems, but should be recommended in selected patients, in whom a potential risk of lethal thoracic organ injury or risk of hemotorax due to traumas or vascular wound directly caused by the tip of the exostosis has been suspected. In conclusion video-assisted thoracoscopic surgery is a minimally invasive surgical technique recently used to assist the surgeon to successfully treat symptomatic costal exostoses, that reduces thoracotomy morbidity.

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