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阻塞性支气管内纤维组织细胞瘤:肺挽救的可能性。

Obstructing endobronchial fibrous histiocytoma: potential for lung salvage.

作者信息

Tagge E, Yunis E, Chopyk J, Wiener E

机构信息

University of Pittsburgh School of Medicine, PA.

出版信息

J Pediatr Surg. 1991 Sep;26(9):1067-9. doi: 10.1016/0022-3468(91)90675-j.

Abstract

In the past 10 years two children, ages 3 1/2 and 7 years, have been seen with central endobronchial fibrous histiocytoma causing complete atelectasis. Each child had a 4- to 6-month history of cough, dyspnea and fever. After antibiotic failure, x-rays demonstrated complete unilateral lung collapse. Bronchoscopy in case 1 demonstrated total occlusion of the left mainstem bronchus with 80% stenosis of the right. Left pneumonectomy was ultimately performed with removal of tumor frm the right bronchus. Ten years later the patient is well. Case 2 had an extrinsic etiology ruled out by computed tomography scan. Ventilation/perfusion scan showed no ventilation and only faint perfusion of the involved lung. Pulmonary function testing showed moderate to severe restrictive changes with air trapping. Total obliteration of the left mainstem with tumor projecting into the carina was observed on bronchoscopy. Endoscopic resection was not felt to be safe. Thoracotomy showed a totally atelectatic, edematous lung filled with pus. The left main bronchus was opened, the tumor removed, and the distal bronchus lavaged and selectively ventilated. Partial lung expansion was obtained. The child is well 1 1/2 years later with a normal chest radiograph, pulmonary function tests, and bronchoscopy. Fibrous histiocytomas of the lung, if in a central endobronchial location, can cause complete lung collapse. A combination of tumor resection, lavage, and selective ventilation should be attempted as an alternative to pneumonectomy, even if the lung appears to be nonsalvageable.

摘要

在过去10年里,我们诊治了两名儿童,年龄分别为3岁半和7岁,他们患有中央型支气管内纤维组织细胞瘤,导致完全性肺不张。每个孩子都有4至6个月的咳嗽、呼吸困难和发热病史。抗生素治疗无效后,X线显示单侧肺完全萎陷。病例1的支气管镜检查显示左主支气管完全阻塞,右主支气管狭窄80%。最终实施了左肺切除术,并从右支气管切除了肿瘤。10年后,该患者情况良好。病例2通过计算机断层扫描排除了外部病因。通气/灌注扫描显示患侧肺无通气,仅有微弱灌注。肺功能测试显示有中度至重度限制性改变并伴有气体潴留。支气管镜检查发现左主支气管完全闭塞,肿瘤突入隆突。内镜切除被认为不安全。开胸手术显示肺完全萎陷、水肿,充满脓液。打开左主支气管,切除肿瘤,对远端支气管进行灌洗并选择性通气。肺得到部分复张。1年半后,患儿情况良好,胸部X线片、肺功能测试和支气管镜检查均正常。肺纤维组织细胞瘤,如果位于中央支气管内,可导致肺完全塌陷。即使肺看起来无法挽救,也应尝试联合肿瘤切除、灌洗和选择性通气作为肺切除术的替代方法。

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