Right‐sided pulmonary aplasia: Longitudinal lung function studies in two cases and comparison to results from term healthy neonates

Prenatal Diagnosis of Isolated Right Pulmonary Agenesis Using Sonography Alone

“…Figure 2 summarizes the process of literature identification and selection. Of the 749 potential citations, 66 were selected on the basis of the title and abstract, and 16 primary articles were included in the systematic review, 2 – 4 , 19 – 23 , 26 – 33 reporting 36 cases of prenatal diagnosis. Table 1 shows the characteristics of the included cases, the outcomes, and postnatal confirmation.…”

“…The suspicion of pulmonary agenesis may be raised because of the high frequency of other associated abnormalities; thus, the diagnosis is more difficult when an isolated lesion exists. Moreover, fetal MRI or postnatal CT is often required to reach or confirm the diagnosis 3 , 4 , 19 , 23 . These imaging methods are expensive and not always readily available.…”

“…Conversely, there are only a few reported cases diagnosed prenatally, most which are in association with other coexistent structural anomalies. However, even in these circumstances, a substantial proportion report uncertainty of diagnosis prenatally with verification required by fetal magnetic resonance imaging (MRI) or postnatal computed tomography (CT) 3 , 4 , 19 – 23 . We report the prenatal diagnosis of isolated right lung agenesis diagnosed with sonography alone at a relatively early gestational age and an accompanying systematic review of the literature relating to the prenatal diagnosis of pulmonary agenesis.…”

Lung Aplasia: Anatomy, History, Diagnosis and Surgical Management

“…Some authors have proposed that hypoplastic pulmonary parenchyma may coexist with lung aplasia [14]. Associated anomalies can include esophageal atresia with tracheoesophageal fistula [3 -6,10,15, 29, 31], tracheal stenosis [6,20,21], diaphragmatic hernia [25], isolated left subclavian artery [7], interrupted aortic arch, venticular septum defect and aortic valve stenosis [22], pulmonary artery sling [19], total anomalous pulmonary venous drainage [11], rightsided aortic arch [12], hypertelorism syndrome [10], velocardiofacial syndrome [8,12], cleft lip and palate, maxillary and mandibular hypoplasia, microtia, horseshoe kidney [9], and extrathoracic hepatic ectopia with amelia [27]. Lung aplasia and agenesis are often associated with VACTERL syndrome [16,24].…”

“…Takahashi et al [32] found that olygohydramnion during the early canalicular stage of lung development (16th -20th week of gestation) might cause defects in development. In the literature, lung agenesis and lung aplasia are often viewed as one anomaly, and respiratory distress syndrome is caused by inflammation in a single lung [3,14,21,24,26,28]. However, the "small" anatomical differences between cases with unilateral lung aplasia and agenesis may produce significant differences in the pathogenesis of respiratory distress and thus in the clinical course and prognosis [18].…”