One hundred and seventy-two papers were identified by searching the databases, one additional article was identified by manual searching. No reports were repetitive. Eighty-three reports were eligible for full-text screening. Of them, 38 papers matched our criteria and were included 2, 4-40. The selection study process is showed in the PRISMA ﬂowchart in Figure 2. They included 60 cases of CBPFM. The 134 papers excluded were review articles, animal cases, published in a language other than English or malformation not meeting the diagnostic criteria depicted by Srikanth et al. Therefore, 61 cases were reviewed including our case (Table 1). The table in the appendix shows the details of the included cases.
The gender of five cases was not reported, there were 26 males and 30 females, and male to female ratio was 0.87:1. The sex distribution among each CBPFM group was similar. The age of cases ranged from 1 day to 59 years. A large proportion of these patients were aged 1 year or younger at presentation (n = 46) and 38 presented immediately after birth. Seven adults have been described.
Classification and affected side
Among the included 61 cases, there were 13 cases (21.3%) in group IA, 4 cases (6.6%) in group IB, 18 cases (29.5%) in group II, 23 cases (37.7%) in group III and 3 (4.9%) cases in group IV. Most of the malformations located in the right side (72.1% vs 23.0%). Two cases (3.3%) had bilateral malformations. The right to left ratio was 3.1:1. The blood supply of abnormal lung tissue was reported in 39 cases, with supply from the pulmonary artery in 17 cases and the systemic artery in 22 cases.
Gestation and family history
Polyhydramnios was reported in 10 cases (3 cases in Group I, 3 cases in Group II, 3 cases in Group III and 1 case in Group IV). Fetal MRI detected the tubular structure directing to the gastroesophageal junction during the second-trimester in 5 cases. These 5 cases belonged to Group III. A mass was detected in the fetal chest cavity in 2 cases.
There were four cases from three sets of twins. Two cases were monochorionic, monoamniotic twins. One twin of was classified in group IA with VACTERL association with the twin sibling in group II without associated malformations. The other two cases were twins whose siblings were not affected.
Twenty patients presented with respiratory distress after birth. Patients with group I malformations presented with drooling, feeding intolerance and failure to pass nasogastric tube because of esophageal atresia. Recurrent respiratory infection, presenting with cough and/or fever, were the main symptoms in older children and adults (10/22, 45.5%). Other symptoms included cough/choking following food intake (n=5), hemoptysis (n=2), nocturnal cough (n=1), epigastric pain (n=1).
Since CBPFM group I is associated with esophageal atresia and tracheoesophageal fistula (EA/TEF), EA/TEF was not included as an associated malformation in our review. Cardiovascular anomalies were the most common associated malformation (n= 11, 18.0%), followed by VACTERL association (n=6, 9.8%), skeletal malformation (n=6, 9.8%), anorectal malformation (n=2, 3.3%), diaphragmatic hernia (n=2, 3.3%) and other less frequently malformations.
For group I, chest radiographs were obtained in 70.6%. The hazy hemithorax and mediastinal shift with dextrocardia were revealed in 52.9%, hypoaerated or normal lung in 17.6%. Only one case of CBPFM was suggested by CT scan initially. One case without chest X-ray was demonstrated during surgery for EA/TEF. Fourteen cases (82.4%) with group I CBPFM were misdiagnosed and were initially operated on for EA/TEF (ligation of tracheoesophageal fistula, primarily esophageal anastomosis or gastrostomy). Definitive diagnoses were confirmed by further evaluations that were prompted by persistent atelectasis of one lung, refractory respiratory distress or routine postoperative UGI.
Opacification of ipsilateral lung and mediastinal shift were also the uniform presentations in chest radiograph of group II (100%). Plain X-ray in group III and group IV were reported in 42.3%. The presentations were various, including mediastinal or pulmonary mass (19.2%), consolidation of part of the lung (23.1%).
The diagnoses of CBPFM were confirmed by UGI in 38 case (62.3%), CT in 7 cases (11.5%), bronchoscopy in 1 case (1.6%) and intraoperative finding in 8 cases (13.1%). There were five cases (8.2%) diagnosed prenatally by ultrasonography and fetal MRI.
Fifty-five cases underwent surgeries. Unilateral pneumonectomies were performed in six cases with group I, 11 cases with group II and one case with group IV. Three cases with Group IA and four cases with group II received reimplantation of the esophageal bronchus. Lobectomies or resection of aberrant lung tissue and bronchus were performed in 24 cases with group IB, group III or group IV. The procedures of the other 6 cases were unknown.
Eight patients died in this series with 4 in group I, 2 in group IB, 1 in group II and group III respectively. However, the outcomes of 15 cases (24.6%) were unknown, so the mortality was 17.4% in 45 cases with outcome reported. In survivors, 44.3% were reported as uneventful follow-up. Eight cases (13.1%) had respiratory morbidities such as air way stenosis, tracheomalacia, recurrent respiratory infection or difficulty in weaning from ventilator. Postpneumonectomy syndrome occurred in one case.
In these series, seven cases received bronchial reconstruction procedures. The longest follow-up was 7 years. Two cases died (one during operation, the other on postoperative day 5). Three cases received more more than one operation for tracheal stenosis, bronchial stenosis, feeding difficulty, bronchomalacia, accompanied laryngotracheoesophageal cleft. The other complications included severe gastroesophageal reflux, anastomosis stricture, recurrent respiratory infection. Only two cases were reported as free of symptoms with normal growth in long-term follow-up.