Diagnosis and treatment of communicating bronchopulmonary foregut malformation

Abstract

Rationale:Communicating bronchopulmonary foregut malformation (CBPFM) is a rare congenital malformation involving both the digestive and respiratory systems. To our best knowledge, most cases of CBPFM reported in the literature were in infancy or adulthood and CBPFM in infantile is even rarer with a high case-fatality rate partly due to misdiagnosis.Patient concerns:We presented 2 cases of neonatal CBPFM. A 11-hour male newborn was admitted because of moaning for 7 hours, and a 1-day male newborn was referred to us with profuse foams, choking on breast-milk feeding and facial cyanosis.Diagnoses:With the assistance of upper gastrointestinal tract imaging and contrast-enhanced chest computed tomography (CT), the diagnosis was established according to the most recent diagnostic criteria.Interventions:The case one recieved a lower left pneumonectomy and surgical repair of esophageal fistula. The case two was performed with a surgical repair of esophageal atresia and esophageal tracheal fistula firstly, and then also received a repair of communicating bronchopulmonary foregut malformation two weeks after the first operation.Outcomes:The case one was cured and discharged 2 weeks after admission. Unfortunately the case two died from respiratory failure.Lessons:Pediatric surgeons should therefore be aware that type I CBPMF is rare and preoperative diagnosis is usually difficult. Maldiagnosis is uncommon because clinicians often focus their attention on esophageal atresia and neglect pulmonary abnormalities. Other than upper gastrointestinal tract radiography and CT scan, bronchoscopy should be considered in pediatric patients with esophageal atresia complicated with pulmonary abnormalities, knowing that bronchoscopy may help confirm the diagnosis and select surgical strategies.

Communicating Bronchopulmonary Foregut Malformation Type IB: Diagnostic and Surgical Challenges

“…A CT scan and bronchoscopy aided the diagnosis in other cases. 1,2 In our case, it was an upper GI contrast series undertaken in light of bilious aspirates after initial surgery for EA þ TOF that revealed the CBPFM and DA/AP. The second contrast study done after surgical repair of the duodenal atresia added to the dilemma after it showed another connection between the esophagus and the right lung, raising the suspicion of a recurrent TOF.…”

“…Hemoptysis, nocturnal cough, and epigastric pain have also been described as presenting features. 1,2 CBPFMs are also known to be associated with other congenital malformations including EA þ TOF, cardiovascular anomalies, VACTERL associations, skeletal malformations, anorectal malformations, and diaphragmatic hernias. 1,2 Our patient had DA/AP and intestinal malrotation that have never been reported before with CBPFM.…”

“…The neonatal diagnosis is difficult. 2 The symptoms in group I patients will be due to EA + TOF and present with drooling of saliva, failure to pass a nasogastric tube or choking on feeds. The children in other groups can have persistent cough, repeated aspirations or choking with feeds, recurrent respiratory tract infections, and failure to thrive.…”

“…Delayed recognition can lead to considerable complications. 2 Srikanth et al divided CBPFM into four major groups after a review of 57 cases. 3 CBPFM group I consists of anomalies associated with esophageal atresia and tracheoesophageal fistula with two subdivisions—IA, total sequestered lung in communication with the foregut, and IB, a sequestered segment or lobe of lung in communication with the foregut.…”

Communicating Bronchopulmonary Foregut Malformation Type III with Pulmonary Sequestration Diagnosed in a Newborn: A Case Report

“…. Clinical presentations are recurrent aspiration epis odes or respiratory tract infection in most cases; thus, these are often diagnosed at the age of ≥1 month after birth 3,5,7,10,11) . To .…”