Anaesthetic challenges in pulmonary alveolar microlithiasis – a rare disease treated with bilateral lung transplantation, first case from India

Pulmonary alveolar microlithiasis (PAM) is an uncommon hereditary lung disease characterized by widespread deposition of calcium phosphate microliths within the alveolar spaces. It is considered an autosomal recessive disease with a mutation in a gene encoding a sodium phosphate cotransporter. The imaging findings in the early phase of disease can be mistaken for miliary tuberculosis or sarcoidosis. However, the classic radiologic findings in the later phases of disease show numerous opacities causing a “snowstorm” appearance to the lungs that corresponds with widespread deposition of microliths throughout the lung parenchyma. Although the disease often progresses over a slow time course, there are no effective therapies, and bilateral lung transplantation is recommended when there are increasing oxygen requirements or evidence of pulmonary hypertension.

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Bilateral lung transplantation (BLTx) in a 9-year-old girl with bronchopulmonary dysplasia with pulmonary hypertension

10.22541/au.161608542.24683352/v1 ◽

2021 ◽

Author(s):

Chiharu Ota ◽

Ryoko Saito ◽

Junya Tominaga ◽

Shinya Iwasawa ◽

Hirama Takashi ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Lung Transplantation ◽

Bronchopulmonary Dysplasia ◽

Respiratory Infections ◽

Treatment Options ◽

Chronic Respiratory Disease ◽

Bilateral Lung Transplantation ◽

Pulmonary Vasodilators ◽

First Case ◽

Bilateral Lung

Background Bronchopulmonary dysplasia (BPD) is a chronic respiratory disease that occurs in premature infants and the prognosis is variable depending on the comorbidities including fibrosis, emphysema, or pulmonary hypertension (PH). We present a case of a 9-year-old girl who developed PH associated with severe BPD (BPD-PH) and underwent bilateral lung transplantation (BLTx). Case description A 9-year-old girl admitted to our department to undergo BLTx. She was born at 23 weeks and 4 days gestation with a weight of 507 grams. She received ventilation for the first 2 months and required further respiratory care due to repetitive, severe respiratory infections. She was diagnosed with BPD-PH at 6 months of age and oral administration of pulmonary vasodilators were initiated. She was registered as a lung transplant candidate at 4 years of age after the life-threatening exacerbation. Chest computed tomography (CT) revealed severe lung conditions with ground-glass opacities and emphysematous low-density areas in the upper and lower lobes. BLTx from a brain-dead male donor was performed. The pathological findings of her resected lung revealed saccular, hypoplastic lung with alveolar repair/regeneration and medial hypertrophy and muscularization of peripheral arteries. The postoperative course was mostly uneventful. She was free from oxygen administration and showed no signs of PH after 6 months of the surgery. Conclusion This is the first case report of BLTx in a pediatric, irreversible BPD-PH patient with detailed pathohistological findings and clinical examination. Lung transplantation is one of the treatment options for severe BPD-PH.

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Lung transplantation in an intensive care patient with pulmonary alveolar microlithiasis - a case report

F1000Research ◽

10.12688/f1000research.4035.1 ◽

2014 ◽

Vol 3 ◽

pp. 118 ◽

Cited By ~ 3

Author(s):

Bülent Güçyetmez ◽

Aylin Ogan ◽

Aylin Çimet Ayyıldız ◽

Berrin Yalçın Güder ◽

Walter Klepetko

Keyword(s):

Heart Failure ◽

Intensive Care ◽

Case Report ◽

Life Expectancy ◽

Lung Transplantation ◽

Right Heart Failure ◽

Right Heart ◽

Bilateral Lung Transplantation ◽

Bilateral Lung ◽

Alveolar Microlithiasis

Introduction: Pulmonary alveolar microlithiasis (PAM) is an autosomal recessive disease characterized by the deposition of phosphate and calcium in the alveoli. The disease progresses asymptomatically until later stages. When it becomes symptomatic, lung transplantations performed before the onset of right heart failure may improve life expectancy and quality. Here we present a case report concerning the very first Turkish PAM patient to have undergone lung transplantation surgery.Patient information: A 52 year-old female, Caucasian patient, already diagnosed with PAM in infancy, was admitted to the intensive care unit, diagnosed with pneumonia and hospitalized for 20 days. We decided to refer the patient to a specialized center for lung transplantation. Bilateral lung transplantation was performed in Vienna 14 months later and no recurrence was observed during the first postoperative year.Conclusion: Bilateral lung transplantation may improve both the life expectancy and the quality of life of PAM diagnosed patients with severe respiratory failure who do not suffer from right heart failure. The risk of recurrence should not be considered as a justifying reason to avoid transplantation as a treatment method.

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