Critical Care: Global Case Report Poster - Miscellaneous |

Pneumonectomy in an 8-Year-Old Male With Ewing Sarcoma of the Rib FREE TO VIEW

Ana Noelle Dominguez, MD
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Philippine Heart Center, Quezon City, Philippines

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):269A. doi:10.1016/j.chest.2016.08.282
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SESSION TITLE: Global Case Report Poster - Miscellaneous

SESSION TYPE: Global Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Ewing sarcoma accounts for 3% of all pediatric malignancies with rib involvement accounting for 12%. Extension into the thoracic cavity can cause cardiopulmonary compromise. Pneumonectomy is rarely performed in children except in cases of malignancy.

CASE PRESENTATION: This is an 8-year-old male with a one-year history of dull pain over the right anterior chest, progressing to cough, dyspnea and weight loss. Work-up for pulmonary tuberculosis was negative. On physical examination, he was cachectic and in respiratory distress. Breath sounds were absent over the right hemithorax and the cardiac apex was displaced to the left anterior axillary line over the 4th intercostal space. Chest CT scan showed a heterogenous, calcified mass occupying almost the entire right hemithorax and mixed lytic and sclerotic changes in the anterior right 5th rib, which was confirmed on bone scan. Due to its mass effect on the mediastinal structures, especially compression of the right atrium, and encroachment onto the right lung, the mass was excised, along with the right lung and segments of the right 4th, 5th, and 6th ribs. The biopsy revealed a Ewing sarcoma of the rib. Typical postpneumonectomy changes were demonstrated on serial chest xrays. Chemotherapy with vincristine, cyclophosphamide, carboplatin and etoposide was started four weeks postoperatively. His post-operative lung function showed a restrictive ventilatory defect. He was discharged improved.

DISCUSSION: A review by Moser showed that 34 out of 328 cases of histologically proven and radiologically correlated Ewing sarcoma originated from the ribs. Thirty-two of these had an accompanying soft-tissue mass that was disproportionately large compared to the intraosseous involvement. This directly extends into the thoracic cavity, causing compression of the mediastinal structures. This patient had similar manifestations. Neoadjuvant therapy would have prevented significant morbidity, however, the mass effect caused cardiopulmonary compromise, prompting urgent surgical resection and a right pneumonectomy. Pneumonectomy is seldom performed in children. A bronchiectatic lung secondary to tuberculosis was the most common indication in the pre-antibiotic era. Malignancy is now the most common indication. Postpneumonectomy changes include elevation of the ipsilateral hemidiaphragm, hyperinflation of the remaining lung, shifting of the mediastinum towards the post-pneumonectomy space (PPS), and progressive resorption of air in the PPS and replacement with fluid, all of which were present in this patient. Fluid accumulates in the postpneumonectomy space and its careful management is important to maintain hemodynamic and respiratory stability. In this patient, fluid was intermittently drained from the postpneumonectomy space. There has only been 4 published cases of pneumonectomy done in children for pulmonary neoplasms and all survived the procedure. Postoperatively, the one-lung ventilation strategies of low tidal volume, high PEEP and a low FiO2 were employed. These aim to protect the lung from barotrauma and atelectasis. A restrictive ventilatory defect, which the patient demonstrated, was common in pneumonectomized patients. Postoperative complications of pneumonectomy are bronchopleural fistula, empyema, herniation of the remaining lung to the opposite side, scoliosis, and reduction in lung function (FVC and FEV1).

CONCLUSIONS: We report a case of an 8-year-old male who underwent a right pneumonectomy for a Ewing sarcoma of the rib with intrathoracic extension. Children with chest pain and suspicious bone growths along the ribs should be thoroughly investigated. Fluid accumulation in the postpneumonectomy space should be carefully managed to maintain hemodynamic and respiratory stability.

Reference #1: Esiashvili N et al. Changes in incidence and survival of Ewing sarcoma patients over the past 3 decades: Surveillance epidemiology and end results data. J Pediatr Hematol Oncol 2008;30:425-430

Reference #2: Moser R et al. Primary Ewing sarcoma of the rib. Radiographics 1990; 10:899-914.

Reference #3: Wolf A et al. Managing the pneumonectomy space after extrapleural pneumonectomy: postoperative intrathoracic pressure monitoring. European Journal of Cardiothoracic Surgery 37 (2010) 770-775

DISCLOSURE: The following authors have nothing to disclose: Ana Noelle Dominguez

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