A 15-month-old boy with communicating bronchopulmonary foregut malformation (CBPFM) type II and VACTERL association: A case report

Author:

Sadr Saeed1,pour Ahmad Safa1,tabari Ahmad khalegh nejad2,Sarafi Mehdi3,kiani Masoud4,Khalili Mitra5,Qomi Zahra6,Derikvandi Shima7

Affiliation:

1. Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences

2. Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences (SBMU)

3. research institute for children’s health, shahid Beheshti university of medical sciences

4. Babol University of Medical Sciences

5. Mofid Children Hospital, Shahid Beheshti University of Medical Science

6. Shahid Beheshti University of Medical Sciences

7. Student University of Tehran

Abstract

Abstract

Background Communicating bronchopulmonary foregut malformation (CBPFM) is a rare congenital anomaly involving abnormal connections between respiratory tissue and the esophagus or stomach. This case report details a 15-month-old boy with CBPFM type II, presenting with cardiac dextroposition, an esophageal lung, and associated VACTERL (vertebral defects, anal atresia, cardiac defects, tracheo-oesophageal fistula, renal anomalies, and limb abnormalities) association. Case presentation: The patient's clinical history, prenatal screenings, and three hospitalizations were documented. Diagnostic tools included chest X-rays, upper GI contrast series, CT scans, echocardiography, bronchoscopy and esophagoscopy. Surgical intervention involved thoracotomy, lung resection, and fistula repair. Pathological examination and postoperative care were conducted, with outcome monitoring. The patient initially presented with recurrent respiratory distress and pneumonia. Imaging revealed severe right lung hypoplasia, cardiac dextroposition, and an esophageal bronchus. Surgical intervention identified a fistula between the esophagus and lung. Unfortunately, postoperative complications, including Acinetobacter sepsis, led to the patient's demise four days after surgery. Conclusions CBPFM, a complex congenital anomaly, poses diagnostic challenges, often leading to delayed recognition. Early diagnosis is crucial, especially considering the potential for associated malformations. This case emphasizes the importance of a multidisciplinary approach, including imaging modalities like CT scans and bronchoscopy, in achieving accurate diagnoses. Surgical intervention, although currently the primary treatment, may carry risks, and postoperative complications can impact outcomes.

Publisher

Research Square Platform LLC

Reference17 articles.

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2. Congenital bronchopulmonary foregut malformation initially diagnosed as esophageal atresia type C: challenging diagnosis and treatment;Boersma D;J Pediatr Surg,2012

3. Congenital bronchopulmonaryforegut malformation. Pulmonary sequestration communicating with the gastrointestinal tract;Gerle RD;N Engl J Med,1968

4. Communicating bronchopulmonary foregut malformations: classification and embryogenesis;Srikanth MS;J Pediatr Surg,1992

5. Esophageal atresia and anal atresia in a newborn with heterotaxia combined with other congenital defects;Smigiel R;J Pediatr Genet,2012

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