Lenfanjiyoleiomyomatozise bağlı iki taraflı nüks spontan pnömotoraks
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Dosyalar
Tarih
2013
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info:eu-repo/semantics/openAccess
Özet
Pulmoner lenfanjiyoleiomyomatozis oldukça nadir görülen bir interstisyel akciğer hastalığıdır. Klinik tablo genellikle nüks spontan pnömotoraks ve progresif dispne şeklindedir. Kesin bir tedavi seçeneği olmamakla birlikte, yıllar içerisinde akciğer parankiminde oluşan multikistik destrüksiyonla solunum yetmezliğine neden olmaktadır. Otuz dokuz yaşında kadın hasta ani başlayan nefes darlığı ve yaygın göğüs ağrısı yakınmaları ile kliniğimize başvurdu. Oskültasyonda her iki akciğerde solunum seslerinde azalma tespit edildi. Akciğer grafisinde iki taraflı pnömotoraks gözlenmesi üzerine iki taraflı tüp torakostomi uygulandı. Toraks tomografisinde sağda daha fazla olmak üzere her iki akciğerde yerleşmiş birkaç ince duvarlı kistik yapı tespit edildi. Hastaya sağ posterolateral torakotomi ile büllektomi, apikal kama rezeksiyonu, apikal parietal plörektomi ve geri kalan plevral alanlara mekanik plevral abrazyon yapıldı. Hastada bir ay sonra oluşan sol nüks pnömotoraks nedeni ile sol posterolateral torakotomi ile büllektomi, bül ligasyonu, apikal kama rezeksiyonu, apikal parietal plörektomi ve geri kalan plevral alanlara mekanik plevral abrazyon uygulandı. Histopatolojik inceleme ile hastaya lenfanjiyoleiomyomatozis tanısı konuldu. İki yıllık takip süresinin sonunda pnömotoraks nüksü gözlenmedi. İki taraflı nüks spontan pnömotoraks gelişen premenopozal kadınlarda lenfanjiyoleiomyomatozis mutlaka akılda tutulmalıdır. Bu hastalarda konservatif yöntemlerle pnömotoraksın sıklık ile nüks etmesi nedeni ile erken dönemde cerrahi tedavi yapılmalıdır.
Pulmonary lymphangioleiomyomatosis is an extremely rare interstitial lung disease. The clinical presentation is generally recurrent spontaneous pneumothorax and progressive dyspnea. No definitive treatment option is available and it leads to respiratory failure due to multicystic destruction of the lung parenchyma in the following years. A 39-year- old female patient was admitted to our clinic with the complaints of sudden onset dyspnea and diffuse chest pain. Auscultation revealed decreased respiratory sounds in both lungs. Chest X-ray showed bilateral pneumothorax and bilaterally tube thoracostomy was performed. Thoracic tomography demonstrated a few thin-wall cystic structures in both lungs with a higher number on the right side. We performed bullectomy, apical wedge resection, apical parietal pleurectomy and mechanic pleural abrasion on residual pleural spaces through right posterolateral thoracotomy. One month later, we performed bullectomy, bulla ligation, apical wedge resection, apical parietal pleurectomy and mechanic pleural abrasion on residual pleural spaces through left posterolateral thoracotomy due to the left recurrent pneumothorax. The patient was diagnosed with lymphangioleiomyomatosis based on the histopathological examination. No recurrent pneumothorax was observed at the end of the two-year follow- up period. Lymphangioleiomyomatosis should be kept in mind in premenopausal women who have bilaterally recurrent spontaneous pneumothorax. Early surgical treatment should be planned in these patients due to multiple pneumothorax recurrences with conservative procedures.
Pulmonary lymphangioleiomyomatosis is an extremely rare interstitial lung disease. The clinical presentation is generally recurrent spontaneous pneumothorax and progressive dyspnea. No definitive treatment option is available and it leads to respiratory failure due to multicystic destruction of the lung parenchyma in the following years. A 39-year- old female patient was admitted to our clinic with the complaints of sudden onset dyspnea and diffuse chest pain. Auscultation revealed decreased respiratory sounds in both lungs. Chest X-ray showed bilateral pneumothorax and bilaterally tube thoracostomy was performed. Thoracic tomography demonstrated a few thin-wall cystic structures in both lungs with a higher number on the right side. We performed bullectomy, apical wedge resection, apical parietal pleurectomy and mechanic pleural abrasion on residual pleural spaces through right posterolateral thoracotomy. One month later, we performed bullectomy, bulla ligation, apical wedge resection, apical parietal pleurectomy and mechanic pleural abrasion on residual pleural spaces through left posterolateral thoracotomy due to the left recurrent pneumothorax. The patient was diagnosed with lymphangioleiomyomatosis based on the histopathological examination. No recurrent pneumothorax was observed at the end of the two-year follow- up period. Lymphangioleiomyomatosis should be kept in mind in premenopausal women who have bilaterally recurrent spontaneous pneumothorax. Early surgical treatment should be planned in these patients due to multiple pneumothorax recurrences with conservative procedures.
Açıklama
Anahtar Kelimeler
Cerrahi, Kalp ve Kalp Damar Sistemi
Kaynak
Türk Göğüs Kalp Damar Cerrahisi Dergisi
WoS Q Değeri
Scopus Q Değeri
Cilt
21
Sayı
4
