Department of Pathology, Kasturba Medical College, Karnataka 575001, India.
J Pediatr Surg. 2010 Feb;45(2):e25-8. doi: 10.1016/j.jpedsurg.2009.11.022.
Congenital cystic adenomatoid malformation, a rare developmental, hamartomatous abnormality of the lung, usually is unilateral, is localized, and presents in early infancy. Delayed occurrence in older children and multilobar involvement are rare. We describe a case of congenital cystic adenomatoid malformation type 1 with multilobar involvement, associated emphysema, and coexistent tracheobronchopathia osteochondroplastica in an adolescent girl for whom the correct diagnosis was achieved only on histologic examination. The importance of an accurate diagnosis of this entity enables proper subtyping, management to minimize the risk of infections and malignancy, and exclusion of associated malformations.
先天性囊性腺瘤样畸形是一种罕见的肺发育性错构瘤性异常，通常为单侧、局限性的，并且在婴儿早期出现。在年龄较大的儿童中发生延迟和多叶受累是罕见的。我们描述了一例 1 型先天性囊性腺瘤样畸形合并多叶受累、肺气肿和同时存在的支气管软骨骨肥厚症的病例，该病例仅通过组织学检查才做出正确诊断。准确诊断该疾病对于正确分型、管理以最大程度降低感染和恶性肿瘤风险以及排除相关畸形非常重要。
