Article
A seldom indication for lung transplantation: pulmonary alveolar microlithiasis – the Vienna experience
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Published: | October 14, 2013 |
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Objective: Objective: Pulmonary alveolar microlithiasis (PAM) is a very rare lung disease caused by calcifications within the alveolar spaces. To date, the only known effective treatment is lung transplantation (LuTX). Only 11 cases of LuTX in PAM which were performed at 10 different centers have been reported in the literature. We intend to report about 5 consecutive cases of PAM undergoing bilateral sequential LuTX at our institution.
Methods: We retrospectively reviewed our database of 1280 patients that underwent LuTX between 1989 and 2013 at our institution.
Results: There were 4 females and 1 male with a mean age of 46.3 years at the time of transplantation. Extracorporal membrane oxygenation (ECMO) support was required intraoperatively in 4 cases and postoperatively in 1 case. Mean postoperative intubation time was 3.3 (range, 2–5) days and mean intensive care unit (ICU) stay was 8.3 (range, 4–12) days. No intraoperative complications were observed. Postoperative complications occurred in 2 (of 5) patients. One early patient (1995) suffered from primary graft dysfunction (PGD) and underwent acute re-transplantation on the second postoperative day (POD). This patient deceased from sepsis on the eleventh POD. In one patient reperfusion edema was observed which caused a prolonged weaning process. Four of 5 patients were still alive at the time of this investigation with normal follow-up parameters. No incidence of recurrence was observed.
Conclusion: Lung transplantation is a feasible therapy option in patients with PAM showing good postoperative results comparable to other indications.