Primary Unilateral Pulmonary Hypoplasia: Neonate through Early Childhood — Case Report, Radiographic Diagnosis and Review of the Literature

Abstract

Unilateral pulmonary hypoplasia is a rare cause of respiratory distress in the neonate. It is usually secondary to other causes such as diaphragmatic hernia. We present a case of a newborn with primary hypoplasia of the right upper lobe who was later found to also have tracheobronchomalacia. We describe the clinical course through early childhood.

Usefulness of ultrasonography and MRI for diagnosis of fetal pulmonary agenesis: case report and review of the literature

“…The poor prognostic factors are class II/III pulmonary agenesis, bronchial stenosis, tracheoesophageal fistulation, tracheomalacia, and scoliosis. In the case of class II/III pulmonary agenesis, it has been suggested that rudimentary bronchus and lung provide a hotbed for bacterial infection and aggravate respiratory disorder [22]. In our case, close observation was necessary given that class II pulmonary agenesis and scoliosis were present.…”

Death Due to Late-Presenting Congenital Diaphragmatic Hernia in a 2-Year-Old Child

“…[2][3][4][5] Those newborns that do present with respiratory distress demonstrate pulmonary hypoplasia at the expense of abdominal contents, which have herniated into the thoracic cavity through the diaphragmatic defect. 6 In contrast, asymptomatic patients may or may not have herniated abdominal contents into the thoracic cavity. 7,8 Some authors make a distinction between "acquired CDH" and "late-presenting CDH," the difference being that an acquired CDH is not present on previous chest radiograph, thus leading to the conclusion that the patient acquired the herniation at some time other than birth, whereas a late-presenting CDH is one that has existed since birth but has been asymptomatic.…”

“…6,14 Furthermore, the right lung may have enlarged in a compensatory fashion. It may be argued that herniation with mediastinal shift would also impinge on contralateral lung growth and development.…”

Imaging of primary unilateral pulmonary hypoplasia: a case report

“…It may be caused by an embryologic defect of the lung or vascular tissues, as in congenital acinar dysplasia [6,7] or by an in utero vascular accident. Only a few cases of primary unilateral pulmonary hypoplasia have been described in the literature with varying degrees of severity ranging from complete hypoplasia of one lung to simple hypoplasia of an isolated lobe [1,5,6,8,9], including in familial series [3,4].…”

“…Unilateral pulmonary hypoplasia or agenesis is a rare cause of respiratory distress in the neonate [1,8,9]. Most cases are secondary to conditions that limit fetal lung growth such as diaphragmatic hernia or bronchial stenosis.…”