Miyano Go, Watanabe Yukio, Hayashi Takuo, Lane Geoffrey J, Suzuki Kenji, Yamataka Atsuyuki
Department of Pediatric General & Urogenital Surgery, Juntendo University School of Medicine, Japan.
Department of Thoracic Surgery, Juntendo University School of Medicine, Japan.
Int J Surg Case Rep. 2020;77:235-242. doi: 10.1016/j.ijscr.2020.10.132. Epub 2020 Nov 2.
Communicating bronchopulmonary foregut malformation (CBPFM) is a rare congenital anomaly comprising a spectrum of airway anomalies connected to the oesophagus or stomach. Our management of a case of CBPFM is presented to improve knowledge of its treatment.
A 17-month-old Japanese girl presented with fever (39.1 °C) and persistent cough. She was noted to be poorly developed (7.5 kg: -2SD). Chest X-ray radiography was suggestive of pneumonia involving the left lung; WBC was 41600/μL and CRP was 12.9 mg/dL. Computed tomography, upper gastrointestinal series, and bronchoscopy identified a fistula between the oesophagus and the left lung and severe left pulmonary artery hypoplasia, typical of type II CBPFM. Her left thoracic cavity was small with sclerotic lung tissue. We customised management by commencing a trial of intensive duodenal tube feeding without oral ingestion. She gained weight and her pneumonia improved enough to enable thoracoscopic left pneumonectomy through an unconventional dorsal approach, necessary because of the location of her CBPFM. Surgery was successful and tolerated well with unremarkable postoperative recovery. Currently she is asymptomatic and growing well.
We used minimally invasive surgery (MIS) to resect an anomalous bronchus and perform a left pneumonectomy after two weeks of intensive conservative management, including nil-by-mouth to optimise her condition for surgery.
Our case provides further evidence that CBPFM can be treated successfully by MIS (interval thoracoscopic pneumonectomy), especially after a period of intensive conservative management. Interval surgery should be considered actively prior to major surgery in smaller children if indicated.
交通性支气管肺前肠畸形（CBPFM）是一种罕见的先天性异常，包括一系列与食管或胃相连的气道异常。本文介绍了我们对一例CBPFM病例的治疗情况，以提高对其治疗的认识。
一名17个月大的日本女孩出现发热（39.1°C）和持续咳嗽。她发育不良（体重7.5kg：低于标准差2）。胸部X光片提示左肺肺炎；白细胞计数为41600/μL，C反应蛋白为12.9mg/dL。计算机断层扫描、上消化道造影和支气管镜检查发现食管与左肺之间存在瘘管以及严重的左肺动脉发育不全，这是II型CBPFM的典型表现。她的左胸腔较小，肺组织硬化。我们通过开始强化十二指肠管饲试验且不进行口服摄入来定制治疗方案。她体重增加，肺炎有所改善，足以通过非常规的背部入路进行胸腔镜下左肺切除术，这是由于她的CBPFM位置所必需的。手术成功，术后恢复顺利，耐受性良好。目前她无症状，生长良好。
我们采用微创手术（MIS）切除异常支气管，并在两周的强化保守治疗后进行左肺切除术，包括禁食以优化她的手术条件。
我们的病例进一步证明，CBPFM可以通过MIS（间隔胸腔镜肺切除术）成功治疗，尤其是在经过一段时间的强化保守治疗之后。如果有指征，对于较小儿童在进行大手术之前应积极考虑间隔手术。
