3D Imaging in Unilateral Primary Pulmonary Hypoplasia in an Adult: A Case Report

Abstract

Unilateral primary pulmonary hypoplasia is rare in adulthood (UPHA); it is characterized by a decreased number of bronchial segmentation and decreased/absent alveolar air space. Classical chest X-ray may be confusing, and the biological tests are unspecific. We present a case of UPHA in a 60-year-old female, smoker, with 3 term normal deliveries, who presented with late recurrent pneumonias and bronchiectasis-type symptomathology, arterial hypertension, and obesity. Chest X-rays revealed opacity in the left lower pulmonary zone, an apparent hypoaerated upper left lobe and left deviation of the mediastinum. Preoperatory multidetector computer tomography (MDCT) presented a small retrocardiac left lung with 5-6 bronchial segmentation range and cystic appearance. After pneumonectomy the gross specimen showed a small lung with multiple bronchiectasis and small cysts, lined by hyperplasic epithelium, surrounded by stromal fibrosclerosis. We concluded that this UPHA occurred in the 4–7 embryonic weeks, and the 3D MDCT reconstructions offered the best noninvasive diagnosis.

Hypoplasia of lung a rare entity mimicking as a case of tuberculosis

“…The most favourable survival has been observed in cases of left lung hypoplasia because of the satisfactory compensatory hypertrophy of the larger right lung [13,14], as also appreciated in our case.…”

Unilateral Primary Lung Hypoplasia Diagnosed in Adulthood

“…The most favorable survival has been observed in cases of left lung hypoplasia because of the satisfactory compensatory hypertrophy of the larger right lung. 2,8 With regard to the present case, we speculate that lung hypoplasia should have occurred in the first 7-10 weeks of gestational age despite the fact that the individual had no symptoms referable to the chest for 28 consecutive years. It is quite likely that right lung compensatory expansion prevented the presence of chest symptomatology.…”

“…A multidetector CT scan provides a noninvasive modality of confirming the diagnosis by clearly delineating the deformed and stunted pulmonary vasculature and bronchial tree. 8,9 Bronchoscopy, ventilation-perfusion scanning, or CT-angiography can equally identify the absence of bronchovascular structures.…”

Unilateral lung hypoplasia presenting in seventh decade of life: a rare case report

“…Left lung hypoplasia shows best survival because of the good compensatory hyperinflation of the larger right lung. 5 Symptomatic treatment is the mode of management for adults and older children in form of expectorants, bronchodilators, empirical antibiotics, control of recurrent infections, and management of other complications. Prophylaxis with pneumococcal and influenza vaccines are recommended.…”

“…1 Although rare even in adults several case reports are available. [2][3][4][5] The developmental anomalies of the lung at the 4 th and 24 th weeks of gestation may cause bronchopulmonary foregut abnormalities. According to Boyden, there are three degrees of maldevelopment: 6 • Agenesis-complete absence of lung tissue (absence of airways, parenchyma and vessels) • Aplasia-where rudimentary bronchus is present without any lung tissue, and • Hypoplasia -in which all the normal pulmonary tissues are present but are under-developed.…”