Congenital communicating bronchopulmonary foregut malformation including ectopic pancreatic tissue in an infant

Background Several reports have documented that the pulmonary sequestration is in communication with the gastrointestinal tract and the concept of bronchopulmonary foregut malformation (BPFM) has become more widespread. However, there are few reports of the sequestration associated with the pancreas derived from the foregut. We describe the history and pathophysiology of BPFM including pancreatic tissue in a male infant with respiratory distress. Case presentation A male patient was born at 38 weeks of gestation and weighed 2752 g at birth. He developed pneumonia and was hospitalized at 3 months of age. Chest radiographs and CT scans led to the diagnosis of a lung abscess in the left lower intralobar pulmonary sequestration with aberrant arteries from the abdominal cavity. At 4 months of age, when the abscess had resolved, left lower lobectomy and the resection of the intralobar sequestration were performed. The pulmonary sequestration was conjoined with the esophagus. A fistula was found between the lower esophageal wall and the pulmonary sequestration. An additional small segment of the esophageal wall was excised. Histologically, the mediastinal surface of the sequestration tissue contained pancreatic tissue. Furthermore, esophageal and gastric tissue, cartilage tissue, and ciliated epithelium were confirmed. A definitive diagnosis of BPFM was made. Conclusions We postulated the rare case of a communicating BPFM with intrapulmonary sequestration on one end and the esophagus on the other forming a mass lesion, which included ectopic pancreatic tissue in a male infant.

Esophagus-Like Bronchus: A Noncommunicating Bronchopulmonary Foregut Malformation

We describe a previously unreported bronchopulmonary foregut malformation wherein a segment of a bronchus of the lower lobe of the left lung in a 4-year-old girl was entirely esophageal in structure. No communication was identified between the tracheobronchial tree and the esophagus by radiologic examination or at surgery. The esophagus-like bronchus was associated with an adjacent atretic bronchus and a downstream cavity in the lower lobe of the left lung. The child sought clinical attention because of recurrent pulmonary infections localized to the lower lobe of the lung. We posit that this esophagus-like bronchus is a novel noncommunicating bronchopulmonary foregut malformation.

Thoracoscopic treatment of a rare bilateral extralobar lung sequestration in a 3-years old girl

Majority of sequestrations fall into two categories: Intra-Lobar (ILS) and Extra-Lobar (ELS). Rarely the abnormal lung could be attached to the gastrointestinal tract, Bronchopulmonary Foregut Malformation (BPFM). We described a case of a girl of 3-years-old with antenatal diagnosis of left intrathoracic mass of the inferior lobe. Postnatal Computed-Tomography (CT) revealed a bilateral ELS with an isthmic bridge crossing the vertebral spine. She follows a MRI follow-up at 18months/30months confirming the lesion. Before surgery, a three-dimensional-CT-angiography was performed to study the mass, its blood supply and to plan surgery. She underwent to thoracoscopic resection. Two aberrant blood vessels were dissected from the thoracic aorta and ligated. The postoperative course was uneventful. She was discharged after 3 days. The rarity of our case is due to the bilateral extension. An appropriate preoperatory imaging study is necessary for the success of surgery while thoracoscopy is particularly appropriate in surgical treatment.

Congenital Bronchopulmonary Foregut Malformation: Systematic review of the literature

Background : Congenital bronchopulmonary foregut malformation (CBPFM) is a rare congenital malformation involving both the digestive and respiratory system. Early diagnosis is difficult, and delayed recognition may result in considerable complications. The aim of the study was to identify and analyze the clinical characteristics and radiological features of CBPFMs. Methods : A systematic review was conducted in accordance with PRISMA guidelines. PubMed, Ovid database, EMBASE were searched for relevant publications to identify all published case-reports of CBPFM since 1992. Data about the demography, clinical presentation, pathology, imaging features, treatment and prognosis were collected. Results : Sixty-one cases were included in our study. Cases were aged from 1 day to 59 years with the majority aged 3 years or younger. The most common type was group III (37.7%), followed by group II (29.5%)，group I (27.9%) and group IV (4.9%). The presentations included respiratory distress (32.8%), cough/choking following food intake and other presentations associated respiratory infection. Thirty-eight cases (62.3%) were diagnosed by upper gastrointestinal series (UGI). Misdiagnosis was common. Eight cases (13.1%) of the included cases died. Conclusions : Early recognition and extensive delineation of the anatomy of CBPFM are important to correct these anomalies successfully. UGI is the first choice to confirm the abnormal bronchus communicating with the esophagus. Resection of abnormal pulmonary tissue, lobe or even unilateral lung is preferred. Reconstruction procedures are feasible in selected patients.