Successful bilateral lung transplantation in pulmonary alveolar microlithiasis: A case report and review of literature

Abstract

Background: Pulmonary alveolar microlithiasis (PAM) is a rare autosomal recessive genetic disorder with approximately 1000 known cases worldwide, in which calcium phosphate microliths deposit in the alveolar air spaces. As of writing this report, no definitive conventional therapy exists, and many PAM cases may progress to severe respiratory failure and potential death. Bilateral lung transplantation (BLx) seems to be the most optimal solution; however, this procedure is challenging along with limited reports regarding the outcome in PAM. We report a case of PAM successfully treated with BLx for the first time in Iran.

Method: We present the case of a 42-year-old female with a longstanding history of cough, not responding to conventional antitussive medication, who was diagnosed as a case of PAM following a hospitalization due to coughing, dyspnea on exertion, and hemoptysis. Despite treatment with corticosteroid and medical treatment, no improvement was achieved and she subsequently developed respiratory and right ventricular failure, with oxygen ventilation dependence. Eventually, she was scheduled for BLx. The operation was successful and during her 2-year follow-up, no recurrence or significant postoperative complications has been reported.

Conclusion: This case presentation and literature review confirm the effectiveness of BLx as a promising treatment for PAM-diagnosed patients, improving both life expectancy and quality of life.

Keywords: cough; dyspnea; lung transplantation; pulmonary alveolar microlithiasis.

FIGURE 1

Chest X‐ray showing bilateral diffuse micronodular opacity involving all zones.

FIGURE 2

High‐resolution computed tomography of a patient with pulmonary alveolar microlithiasis. (A₁, A₂, A₃) multiple bilateral calcified micronodules diffusely scattered over the lung parenchyma through three different sections in pulmonary window setting (B₁, B₂, B₃) three different sections through the lung parenchyma showing combination of septal thickening and ground glass opacity illustrating “crazy paving sign” in the mediastinal window setting.

FIGURE 3

High‐resolution computed tomography of the patient with pulmonary alveolar microlithiasis over 2 years (A₁, A₂) a section of the lung in a pulmonary window setting, demonstrating expansion of fibrosis over the years (B₁, B₂) a mediastinal section of the lung, showing an increased number and size of calcified foci in B₂.

FIGURE 4

Gross view of the left lung of the donor for a lung transplant.

FIGURE 5

Pathologic findings in macroscopic view of the explanted lung in pulmonary alveolar microlithiasis. Cut sections of both lungs showing gritty‐granular surfaces.

FIGURE 6

Gram‐stained histological section of lung tissue. Arrows show diffuse alveolar air space filled by lamellated calcifications and numerous introalveolar microliths. (A) and (B) magnification at 10×, and (C) magnification at 40×.

FIGURE 7

High‐resolution computed tomography of the patient with pulmonary alveolar microlithiasis, conducted at the first year postoperative follow‐up without any abnormality.