Late presentation of esophageal bronchus in a patient with contralateral pulmonary hypoplasia: a diagnostic and therapeutic challenge.

I Bada Bosch, M A García Casillas, J Ordóñez, J Cerdá, L Pérez Egido, M D Blanco Verdú, E Sanjuan, J C de Agustín
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Abstract

Introduction: Congenital bronchopulmonary foregut malformations are infrequent, with less than 60 cases reported. Group 3 or esophageal bronchus involves an abnormal origin of a lobar bronchus in the esophagus.

Clinical case: Patient with a history of heart disease requiring surgery and with left pulmonary hypoplasia. At 9 years of age, a chest CT-scan was carried out as a result of coughing following liquid intake. It revealed an esophageal bronchus of the right upper lobe. Given the history of pulmonary hypoplasia, a ventilation-perfusion scintigraphy was requested. It demonstrated lack of functionality of the right upper lobe. A right upper lobectomy was scheduled, with resection of the esophageal bronchus. 3D reconstruction was requested as part of preoperative planning. Following surgery, clinical signs disappeared, with an adequate respiratory function.

Discussion: Esophageal bronchus typically manifests in early childhood in the form of persistent atelectasis and repeated infections. However, in cases of stenotic bronchi, clinical signs may be unspecific, which requires multiple complementary tests and involves a high diagnostic suspicion. Late diagnosis usually leads to pulmonary resection. However, the latter is well tolerated even in patients with respiratory and cardiac pathologies. Patients with complex malformations benefit from preoperative planning with 3D reconstructions.

对侧肺发育不全患者食管支气管的晚期表现:诊断和治疗的挑战。
前言:先天性支气管肺前肠畸形是一种罕见的畸形,据报道不足60例。第3组,即食管支气管,包括食道大叶支气管的异常起源。临床病例:有心脏病史,需手术治疗,左肺发育不全。9岁时,由于摄入液体后咳嗽,进行了胸部ct扫描。显示右上叶有食管支气管。考虑到肺发育不全的病史,需要进行通气灌注显像检查。显示右上叶功能缺失。计划行右上肺叶切除术,同时切除食管支气管。三维重建被要求作为术前计划的一部分。术后临床症状消失,呼吸功能正常。讨论:食管支气管在儿童早期典型表现为持续性肺不张和反复感染。然而,在支气管狭窄的病例中,临床症状可能不明确,这需要多次补充检查,并涉及高度的诊断怀疑。晚期诊断通常导致肺切除术。然而,后者即使在患有呼吸和心脏疾病的患者中也具有良好的耐受性。复杂畸形患者受益于术前计划的三维重建。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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