Instituto Nacional de Salud del Niño-Breña.
Unidad de Investigación para la Generación y Síntesis de Evidencias en Salud, Vicerrectorado de Investigación, Universidad San Ignacio de Loyola. Lima, Peru.
Bol Med Hosp Infant Mex. 2023;80(4):235-241. doi: 10.24875/BMHIM.23000055.
Congenital pulmonary malformations (CPMs) are rare in children. This study aimed to describe the clinical, imaging characteristics, and treatment of patients with this pathology.
We conducted a descriptive and retrospective study with data from patients with CPMs diagnosed at Instituto Nacional de Salud del Niño-Breña (Lima-Peru), from January 2010 to December 2020. We described CPM clinical and imaging characteristics, type and treatment.
The sample was formed of 70 patients. The median age was 29 months (range 15 days-14 years) and the male/female ratio was 1.4. The chest tomography found parenchymal involvement in 50 (71.4%) cases and mixed involvement (parenchymal and vascular) in 18 (25.7%) cases. Congenital malformation of the pulmonary airway was present in 39 (55.7%) cases, followed by bronchogenic cyst in 10 (14.3%), intralobar pulmonary sequestration in 9 (12.9%), and extralobar pulmonary sequestration in 7 (10%). Lobectomy was performed in 61 (87.1%) cases, cystectomy in 5 (7.1%), segmentectomy in 2 (2.9%), and embolization in 2 (2.9%). The most frequent post-operative complication was pneumonia, found in 9 (12.9%) cases. The median hospital stay was 26 days. No patient died during hospitalization.
In our institution, the most frequent CPM was congenital malformation of the pulmonary airway, and lobectomy was the most frequently performed surgical procedure. CPMs represent a diverse group of disorders of lung development with varied imaging patterns and clinical manifestations.
先天性肺畸形（CPM）在儿童中较为罕见。本研究旨在描述患有这种疾病的患者的临床、影像学特征和治疗方法。
我们进行了一项描述性和回顾性研究，纳入了 2010 年 1 月至 2020 年 12 月在秘鲁利马-布雷尼亚国家儿童研究所诊断为 CPM 的患者的数据。我们描述了 CPM 的临床和影像学特征、类型和治疗方法。
该样本由 70 例患者组成。中位年龄为 29 个月（范围 15 天-14 岁），男女比例为 1.4。胸部 CT 发现实质受累 50 例（71.4%），混合受累（实质和血管）18 例（25.7%）。肺气道先天性畸形 39 例（55.7%），支气管囊肿 10 例（14.3%），肺隔离症 9 例（12.9%），肺隔离症 7 例（10%）。61 例（87.1%）患者行肺叶切除术，5 例（7.1%）行囊肿切除术，2 例（2.9%）行节段切除术，2 例（2.9%）行栓塞术。最常见的术后并发症是肺炎，9 例（12.9%）。中位住院时间为 26 天。住院期间无患者死亡。
在我们的机构中，最常见的 CPM 是肺气道先天性畸形，肺叶切除术是最常进行的手术。CPM 是一组表现为不同影像学模式和临床表现的肺部发育障碍的疾病。
