Oyachi Noboru, Numano Fuminori, Koizumi Keiichi, Shinohara Tamao, Matsubara Hirochika
Department of Pediatric Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Kofu, Yamanashi, 409-8506, Japan.
Department of Pediatrics, Yamanashi Prefectural Central Hospital, Kofu, Japan.
Surg Case Rep. 2021 May 24;7(1):128. doi: 10.1186/s40792-021-01211-w.
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.
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.
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.
多项报告记载肺隔离症与胃肠道相通,支气管肺前肠畸形(BPFM)的概念已更为广泛。然而,源于前肠的隔离症与胰腺相关的报道较少。我们描述了一名患有呼吸窘迫的男婴中包含胰腺组织的BPFM的病史和病理生理学。
一名男性患者孕38周出生,出生体重2752克。他在3个月大时患肺炎并住院。胸部X光片和CT扫描诊断为左下叶肺内隔离症伴腹腔异常动脉形成肺脓肿。4个月大时,脓肿消退后,进行了左下叶切除术和肺内隔离症切除术。肺隔离症与食管相连。在食管下段壁和肺隔离症之间发现了一个瘘管。另外切除了一小段食管壁。组织学检查显示,隔离组织的纵隔面含有胰腺组织。此外,还证实存在食管和胃组织、软骨组织及纤毛上皮。最终确诊为BPFM。
我们推测了一种罕见的BPFM病例,一端为肺内隔离症,另一端为食管,形成一个肿块病变,该男婴的病变中包含异位胰腺组织。