A spectrum of bronchopulmonary anomalies associated with tracheoesophageal malformations

Abstract

The combination of tracheoesophageal and pulmonary malformations is unusual and reportedly carries a high mortality. We have observed six patients with esophageal atresia and tracheoesophageal fistula and one with a bronchoesophageal fistula who had associated bronchopulmonary anomalies ranging from lobar hypoplasia and agenesis to unilateral pulmonary hypoplasia or agenesis. All of the pulmonary malformations were right-sided. Vertebral or rib anomalies were present in five patients, congenital heart disease in two, imperforate anus in one and one patient had radial aplasia and Pierre Robin syndrome. The bronchopulmonary anomalies complicated the surgical care of tracheoesophageal malformations and required radiologic differentiation from aspiration pneumonia and atelectasis. Six of the seven patients survived. Mortality and morbidity were related to complications and associated cardiac anomalies as well as severity of the lung anomaly.

Bilateral pulmonary agenesis: prenatal sonographic appearance simulates congenital diaphragmatic hernia

“…Abnormalities of lung lobulation can be isolated but often occur in association with anomalies of other organ systems that also originate from the primitive foregut. 7,10,21,22 In our case, the BPA was associated with a tracheoesophageal fistula. Several investigations have stressed the importance of examination of the fetus or neonate for findings that may suggest an underlying syndrome or association.…”

Identical twins concordant for pulmonary sequestration communicating with the esophagus and discordant for the VACTERL association

“…Lesions have been described in all lobes of the lungs with various blood supplies and in conjunction with several other congenital anomalies [1,4,[5][6][7][8][9][10][11][12][13][14][15]. In addition, there is a spectrum of laryngotracheal abnormalities that may be associated with tracheoesophageal malformations as well as bronchopulmonary aberrations [16,17].…”

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

“…Cases of isolated lobar defects with or without tracheobronchial abnormalities, has only been rarely reported. [8][9][10][11][12][13] With unilateral lobar pulmonary hypoplasia, gross pathology of the affected lobes shows a decrease in size and weight and, histologically, a decrease in the number of alveoli. The alveoli are often immature but otherwise demonstrate no distinct abnormalities.…”

“…A number of studies have described associated anomalies including heart defects, skeletal and vertebral anomalies, abdominal defects, facial abnormalities, kidney defects, as well as numerous lung and tracheo-bronchial tree defects. 6,8,[19][20][21][22][23][24] There have been a number of case reports of pulmonary hypoplasia and tracheoesophageal fistula and a number of reports of an association of tracheal stenosis with pulmonary agenesis. 8,19,21,22,24 -27 Similar to patients with more severe degrees of pulmonary hypoplasia, neonates with isolated lobar hypoplasia may demonstrate other congenital malformations.…”