MINIMALLY INVASIVE THORACOSCOPIC SURGERY AS A DIAGNOSTIC AND THERAPEUTIC APPROACH IN BILATERAL PNEUMOTHORAX IN PREGNANCY CAUSED BY LYMPHANGIOLEIOMYOMATOSIS – A CASE REPORT

  • Željko Garabinović
  • Nikola Čolić
  • Jelena Vasić Madžarević
  • Milan Savić
Keywords: pneumothorax, pregnancy, lymphangioleiomyomatosis

Abstract


Introduction: Lymphangioleiomyomatosis (LAM) is a rare disease which mainly occurs in women in the generative period, as well as during pregnancy, while only a few individual cases have been described in men. It occurs in sporadic form or is associated with tuberous sclerosis complex. The diagnosis can be made on
the basis of high-resolution computed tomography (HRCT) findings, or histopathological analysis is required. Clinical manifestations of the disease include the following: progressive dyspnea on exertion, recurrent pneumothorax, chylothorax, angiomyolipomas and lymphangiomyomas.

Case report: A 32-year-old female patient was admitted to our clinic, in her third trimester of pregnancy, after a left-sided pneumothorax was verified on chest X-ray. Initial treatment included needle aspiration, followed by thoracic drainage of the left pleural cavity. Due to the prolonged air leak through the thoracic drain and the advanced stage of the pregnancy, a caesarean section was performed. After delivery, chest X-ray revealed complete right-sided pneumothorax, which required thoracic drainage, as well as an insufficiently reexpanded left lung. HRCT was performed and cystic bullous changes in the lungs were noted; LAM was histopathologically verified through a minimally invasive thoraco-surgical approach, first on the left and then on the right side, while bilateral pneumothorax was surgically treated.

Conclusion: Pneumothorax is a common complication of LAM. Due to the high recurrence rate, definitive early surgical intervention should be performed. Current guidelines recommend chemical pleurodesis and surgery for the first pneumothorax. When treating pneumothorax in pregnancy, the appropriate therapeutic procedure should be applied, taking into account the safety of the pregnancy and of the delivery.

References


  1. Cohen MM, Pollock-BarZiv S, Johnson SR. Emerging clinical picture of lymphangioleiomyomatosis. Thorax2005;60:875–879. 

  2. Harknett EC, Chang WY, Byrnes S, Johnson J, Lazor R, Cohen MM, et al. Use of variability in national and regional data to estimate the prevalence of lymphangioleiomyomatosis. 2011;104(11):971-979.

  3. Ferrans VJ, Yu ZX, Nelson WK, Valencia JC, Tatsuguchi A, Avila NA, et al. Lymphangioleiomyomatosis (LAM): a review of clinical and morphological features.J Nippon Med Sch. 2000;67(5):311-329.

  4. Aubry MC, Myers JL, Ryu JH, Henske EP, Logginidou H, Jalal SM, et al..Pulmonary lymphangioleiomyomatosis in a man.Am J Respir Crit Care Med.2000;162:749–752.

  5. Brunelli A, Catalini G, Fianchini A. Pregnancy exacerbating unsuspected mediastinal lymphangioleiomyomatosis and chylothorax. Int J Gynaecol Obstet 1996;52(3):289-2

  6. Johnson SR, Tattersfield AE. Clinical experience of lymphangioleiomyomatosis in the UK. Thorax 2000;55:1052–1057.

  7. Mitra S, Ghosal AG, Bhattacharya P. Pregnancy unmasking lymphangioleiomyomatosis. J Assoc Phys India 2004;52: 828-8

  8. Taylor JR, Ryee J, Colby TV, Raffin TA. Lymphangioleiomyomatosis : clinical course in 32 patients. N Engl J Med 1990;323:1254-1260.

  9. Northrup H, Krueger DA. International Tuberous Sclerosis Complex Consensus Group. Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 Iinternational Tuberous Sclerosis Complex Consensus Conference. Pediatr Neurol. 2013;49(4):243-254.

  10. Ryu JH, Moss J, Beck GJ, Lee JC, Brown KK, Chapman JT, et al.: The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Respir Crit Care Med. 2006;173(1): 105–111.

  11. Gonano C, Pasquier J, Daccord C, et al. . Air travel and incidence of pneumothorax in lymphangioleiomyomatosis. Orphanet J Rare Dis2018;13:222.    

  12. Johnson SR, Whale CI, Hubbard RB, et al. . Survival and disease progression in UK patients with lymphangioleiomyomatosis. Thorax2004;59:800–803.     

  13. Taylor JR, Ryu J, Colby TV, et al. . Lymphangioleiomyomatosis. N Engl J Med1990;323: 1254–1260.            

  14. Urban T, Lazor R, Lacronique J, et al. . Pulmonary lymphangioleiomyomatosis. A study of 69 patients. Groupe d'Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM“O”P). Medicine (Baltimore)1999;78:321–337.          

  15. Cohen MM, Freyer AM, Johnson SR: Pregnancy experiences among women with lymphangioleiomyomatosis. Respir Med. 2009;103(5):766–772.

  16. Shen L, Xu W, Gao J, Wang J, Huang J, Wang Yet, al. Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM). Orphanet J Rare Dis. 2021;16(1):133.

  17. Crawford TC, Grimm JC, Magruder JT, Stephens RS, Sciortino CM, Vaught AJ, et al. A curious case of acute respiratory distress syndrome. J Surg Case Rep.; 2015(11) pii: rjv140.

  18. Grzegorek I, Lenze D, Chabowski M, Janczak D, Szolkowska M, Langfort R, et al. Immunohistochemical evaluation of pulmonary lymphangioleiomyomatosis. Anticancer Res. 2015;35(6):3353-3360.

  19. Hayashida M, Seyama K, Inoue Y, Fujimoto K, Kubo K.The epidemiology of lymphangioleiomyomatosis in Japan: a nationwide cross-sectional study of presenting features and prognostic factors. Respirology 2007;12:523–530.  

  20. Gupta N, Lee HS, Ryu JH, Taveira-DaSilva AM, Beck GJ, Lee JC, et al. . The NHLBI LAM registry: prognostic physiologic and radiologic biomarkers emerge from a 15-year prospective longitudinal analysis. Chest2019;155:288–296.             

  21. Oprescu N, McCormack FX, Byrnes S, Kinder BW. Clinical predictors of mortality and cause of death in lymphangioleiomyomatosis: a population-based registry. Lung2013;191: 35–42.        

  22. Johnson SR, Cordier JF, Lazor R, et al.: European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 2010;35(1): 14–26.

  23. MacDuff A, Arnold A, Harvey J. BTS Pleural Disease Guideline Group. Management of spontaneous pneumothorax: British Thoracic Society Pleural Disease Guideline 2010. Thorax. 2010;65(2):ii18-31. 

  24. McCormack FX, Gupta N, Finlay GR, et al, for the TS/JRS Committee on Lymphangioleiomyomatosis. Official American Thoracic Society/Japanese Respiratory Society clinical practice guidelines: lymphangioleiomyomatosis diagnosis and management. Am J Respir Crit Care Med. 2016;194(6):748-61. 

  25. Tsai CF, Hsiao CH, Lee JM, Chen KC, Shieh MJ, Lai HS, Chen JS. Video-assisted thoracoscopic surgery for recurrent pneumothorax in pulmonary lymphangioleimyomatosis with tuberous sclerosis complex. J Cardiothorac Surg. 2013;8:101.

  26. Meraj R, Wikenheiser-Brokamp KA, Young LR, Byrnes S, McCormack FX. Utility of transbronchial biopsy in the diagnosis of lymphangioleiomyomatosis.Front Med. 2012;6:395–405.        

  27. Gupta N, Finlay GA, Kotloff RM, Strange C, Wilson KC, Young LR, et al. Lymphangioleiomyomatosis diagnosis and management: high-resolution chest computed tomography, transbronchial lung biopsy, and pleural disease management. An official American Thoracic Society/Japanese Respiratory Society clinical practice guideline. Am J Respir Crit Care Med2017;196:1337–1348.                

  28. Almoosa KF, Ryu JH, Mendez J, Huggins JT, Young LR, Sullivan EJ, et al.: Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications. Chest. 2006;129(5):1274–1281.

  29. Weill D, Benden C, Corris PA, Dark JH, Davis RD, Keshavjee S, et al. A consensus document for the selection of lung transplant candidates: 2014 – an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant2015;34:1–15.     


 


 

Published
2022/06/29
Section
Case reviews