Lam Christopher Z, Bhamare Tanmay Anant, Gazzaz Tamadhir, Manson David, Humpl Tilman, Seed Mike
Department of Diagnostic Imaging, The Hospital for Sick Children, Department of Medical Imaging, University of Toronto, 555 University Ave., M644, Toronto, ON, M5G 1X8, Canada.
Department of Pediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
Pediatr Radiol. 2017 Oct;47(11):1441-1451. doi: 10.1007/s00247-017-3892-z. Epub 2017 Jun 19.
Secondary pulmonary lymphangiectasia is a complication of congenital heart disease that results from chronic pulmonary venous obstruction.
We aimed to evaluate the performance of chest ultrasound (US) in diagnosing secondary pulmonary lymphangiectasia and to review the clinical course of children with secondary pulmonary lymphangiectasia.
Chest US was performed on 26 children with hypoplastic left heart syndrome, total anomalous pulmonary venous connection or cor triatriatum in a prospective observational study. Thirteen children had pulmonary venous obstruction (62% male; median age: 17 days old, range: 1-430 days old) and 13 children did not have obstruction (62% male; median age: 72 days old, range: 4-333 days old). US features of secondary pulmonary lymphangiectasia were documented and diagnostic performance was determined. Clinical course of patients with secondary pulmonary lymphangiectasia was reviewed.
Eleven of 13 (84.6%) patients in the obstructed group had a clinical and/or biopsy diagnosis of secondary pulmonary lymphangiectasia. Statistically significant chest US criteria for diagnosis were presence of irregular lung surface (likelihood ratio [LR] 6.8, 95% confidence interval [CI] 1.9-25.1), subpleural cystic appearing structures (LR 3.6, 95% CI 1.2-10.7), and combination of subpleural cystic appearing structures and surface irregularity together (LR 10.9, 95% CI 1.6-75.0). Seven of 11 (63.6%) patients with secondary pulmonary lymphangiectasia died during follow-up, the majority due to cardiopulmonary failure or complications.
Chest US is an accurate and reproducible bedside method for diagnosing secondary pulmonary lymphangiectasia in patients with pulmonary venous obstruction. These patients may have worse prognoses.
继发性肺淋巴管扩张症是先天性心脏病的一种并发症，由慢性肺静脉梗阻引起。
我们旨在评估胸部超声（US）在诊断继发性肺淋巴管扩张症中的表现，并回顾继发性肺淋巴管扩张症患儿的临床病程。
在一项前瞻性观察研究中，对26例患有左心发育不全综合征、完全性肺静脉异位连接或三房心的儿童进行了胸部超声检查。13例儿童存在肺静脉梗阻（男性占62%；中位年龄：17天，范围：1 - 430天），13例儿童无梗阻（男性占62%；中位年龄：72天，范围：4 - 333天）。记录继发性肺淋巴管扩张症的超声特征并确定诊断性能。回顾继发性肺淋巴管扩张症患者的临床病程。
梗阻组13例患者中有11例（84.6%）经临床和/或活检诊断为继发性肺淋巴管扩张症。具有统计学意义的胸部超声诊断标准为存在肺表面不规则（似然比[LR] 6.8，95%置信区间[CI] 1.9 - 25.1）、胸膜下囊性结构（LR 3.6，95% CI 1.2 - 10.7）以及胸膜下囊性结构与表面不规则同时存在（LR 10.9，95% CI 1.6 - 75.0）。11例继发性肺淋巴管扩张症患者中有7例（63.6%）在随访期间死亡，大多数死于心肺衰竭或并发症。
胸部超声是诊断肺静脉梗阻患者继发性肺淋巴管扩张症的一种准确且可重复的床边检查方法。这些患者的预后可能较差。
