Total pleural coverage followed by lung transplantation in patient with lymphangioleiomyomatosis
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CASE REPORT
Total pleural coverage followed by lung transplantation in patient with lymphangioleiomyomatosis Do Hyung Kim1 · Hyo Yeong Ahn2 · Bong Soo Son1 · Joohyung Son1 Received: 30 May 2019 / Accepted: 21 September 2019 © The Author(s) 2019
Abstract Tuberous sclerosis complex lymphangioleiomyomatosis (TSC-LAM) is a rare disease, which may develop an intractable pneumothorax. Chemical or mechanical pleurodesis is a general management to prevent recurrence of pneumothorax, rendering it difficult to later dissect the pleura and control intraoperative bleeding. Since total pleural coverage (TPC) alternative to pleurodesis has been firstly reported by Kurihara et al. (Jpn J Thorac Cardiovasc Surg 54:274, 2006), TPC was performed in case of a 46-year-old female with a secondary spontaneous pneumothorax caused by TSC-LAM and followed by lung transplantation. Final pathological report showed the reinforced visceral pleura in the absence of dense adhesions. Keywords Pneumothorax · Lymphangioleiomyomatosis · Lung transplantation
Introduction
Case report
Tuberous sclerosis complex lymphangioleiomyomatosis (TSC-LAM) is a rare disease, which may trigger an intractable pneumothorax. Chemical or mechanical pleurodesis is recommended to prevent recurrence, rendering it difficult to later dissect the pleura and control intraoperative bleeding. We performed total pleural coverage (TPC) as an alternative to pleurodesis, based on the recommendations of recent reports. Pathological findings are important when evaluating the efficacy of TPC. Herein, we report the case of a 46-year-old female with a secondary spontaneous pneumothorax caused by TSC-LAM who underwent TPC prior to lung transplantation.
A 46-year-old female was admitted to the emergency room with dyspnea, and right pneumothorax was diagnosed. Chest computed tomography (CT) revealed the features of lymphangioleiomyomatosis (LAM), including facial angiofibroma, hypomelanotic macules, and renal angiomyolipoma; we thus diagnosed tuberous sclerosis complex (TSC)-LAM. As the air leakage had developed 10 days prior, airleak control was performed by video-assisted thoracoscopic surgery (VATS), which showed multiple lung cysts with ruptured bullae surrounded by a dense adhesion in the right upper lobe. After releasing the adhesion, the bullae was ligated, and covered a huge fragile cyst at risk of imminent rupture with an absorbable polyglycolic acid sheet (Neoveil; Gunze Ltd., Kyoto, Japan) and fibrin sealant (Tisseel; Baxter Healthcare Corp., Deerfield, IL, USA). To prevent recurrence, we performed total pleural coverage (TPC) of the entire lung surface using 12 sheets of oxidized regenerated cellulose (ORC) mesh (Ethicon SURGICEL ® absorbable Hemostat gauze, Johnson & Johnson, Brunswick, NJ, USA) (Fig. 1). The patient was discharged on postoperative day (POD) 9. One month later, she was readmitted to treat a contralateral recurrent pneumothorax. She again underwent TPC after ligation of the ruptured bullae and was discharged on POD 22. Home oxygen therapy and sirolimu
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