Walvir Nazia Manzoor, Makhdoomi Rumana, Abeer Inara, Ganaie Farooq, Maqsood Shadab
Department of Pathology, Sher-i-Kashmir Institute of Medical Sciences, Srinagar, J and K, India.
Department of CVTS, Sher-i-Kashmir Institute of Medical Sciences, Srinagar, J and K, India.
Lung India. 2023 Sep-Oct;40(5):423-428. doi: 10.4103/lungindia.lungindia_17_23.
Congenital Cystic Adenomatoid Malformations (CCAM) are rare congenital anomalies of the lungs characterised by bronchopulmonary foregut malformations due to a sudden arrest in the development of the bronchial tree in the first trimester of the gestational period.
: The present study was aimed to describe the clinical and histopathological profiles of the patients and study patient outcomes after 1 year of surgical resection.
All patients diagnosed with CCAM by histological examination of tissue obtained on surgical resection during the study period were included in the study. Data, such as patient demographics and clinical, radiological and histopathological findings, were recorded, and follow-up information was taken on OPD follow-up till 1 year after surgery regarding respiratory infections, haemoptysis or mortality.
: Out of 21 patients, 11 were female and included in the study between the ages of 1 month and 32 years, with >50% younger than 2 years. Most patients in the study had recurrent pneumonia, with difficulty in breathing being the second most common presenting complaint. All patients had undergone computed tomography (CT) of the lungs, which was able to diagnose cystic lesions accurately in >80% of cases. Histologically, all cases were classified based on recent Strocker's classification, and Type 1 was the most commonly observed with 13 cases, followed by Type 2 in five and Type 3 in three patients. There was no evidence of malignant transformation in any of the cases. There was 100% survival at the end of 1 year, with six patients having respiratory infections and none of the patients getting hospitalised over 1 year after surgery.
CCAM is a rare congenital anomaly associated with significant morbidity and may present at any age. It can be histologically classified into three subtypes, with Type 1 being the most common. Early surgical management is mandatory to prevent complications such as recurrent infections, respiratory distress, pneumothorax, lung abscess and malignant transformation. All patients included in the study had undergone surgical resection, and there was 100% survival at 1-year follow-up.
先天性囊性腺瘤样畸形（CCAM）是一种罕见的肺部先天性异常，其特征为支气管肺前肠畸形，这是由于孕期头三个月支气管树发育突然停滞所致。
本研究旨在描述患者的临床和组织病理学特征，并研究手术切除1年后的患者预后。
本研究纳入了在研究期间通过手术切除获得的组织的组织学检查诊断为CCAM的所有患者。记录患者人口统计学以及临床、放射学和组织病理学检查结果等数据，并在门诊随访直至术后1年，了解呼吸道感染、咯血或死亡率等随访信息。
21例患者中，11例为女性，年龄在1个月至32岁之间，超过50%的患者年龄小于2岁。研究中的大多数患者有反复肺炎，呼吸困难是第二常见的主诉。所有患者均接受了肺部计算机断层扫描（CT），在超过80%的病例中能够准确诊断囊性病变。组织学上，所有病例均根据最近的斯特罗克分类法进行分类，1型最为常见，有13例，其次是2型5例，3型3例。所有病例均无恶变证据。1年末生存率为100%，6例患者有呼吸道感染，术后1年无患者住院。
CCAM是一种罕见的先天性异常，伴有明显的发病率，可在任何年龄出现。它可在组织学上分为三个亚型，1型最为常见。早期手术治疗对于预防反复感染、呼吸窘迫、气胸、肺脓肿和恶变等并发症至关重要。本研究纳入的所有患者均接受了手术切除，1年随访时生存率为100%。
