背景:先天性肺气道畸形(CPAM)是由子宫内异常气道发育引起的一系列囊性和非囊性异常,发病率为25,000至35,000人中的1人。CPAM可在产前或产后出现呼吸窘迫,反复感染,或者偶尔作为偶然发现。这个病例系列旨在突出临床,放射学,通过三例儿科病例,CPAM的组织病理学特征,其中包括类型1、2和3CPAM。
方法:案例1:一名4个月大的男性出现咳嗽,冷,和呼吸窘迫。影像学显示左上叶衰减减弱和过度膨胀伴纵隔移位。左上叶切除术证实CPAM2型。病例2:1个月大的女性反复出现呼吸窘迫和感染。影像学显示右侧中叶有较大的囊性病变。右中叶切除术证实CPAM3型。病例3:2个月大的男性出现呼吸困难。影像学显示右胸部有一个大的大疱,纵隔移位。右上叶切除术证实CPAM1型。
■CPAM是一种罕见的先天性肺畸形,其特征是支气管发育异常和局部腺体过度生长。管理包括手术切除,手术的时机取决于症状学。早期识别和干预对于预防肺发育不全和反复感染等并发症至关重要。切除后的组织病理学检查对于CPAM的准确分类和管理至关重要。
结论:本病例系列提供了有关临床,放射学,和CPAM的组织病理学特征,包括在每种情况下遇到的类型。它强调了早期诊断和及时手术干预的重要性,倡导常规产前和产后筛查,以有效识别和管理CPAM。
BACKGROUND: Congenital pulmonary airway malformations (CPAM) are a spectrum of cystic and non-cystic anomalies arising from abnormal airway development in utero, with an incidence of 1 in 25,000 to 35,000 births. CPAM can present prenatally or postnatally with respiratory distress, recurrent infections, or occasionally as an incidental finding. This case series aims to highlight the clinical, radiological, and histopathological characteristics of CPAM through three pediatric cases, which include types 1, 2, and 3 CPAM.
METHODS: Case 1: A four-month-old male presented with cough, cold, and respiratory distress. Imaging revealed hypoattenuation and overinflation of the left upper lobe with mediastinal shift. Left upper lobectomy confirmed CPAM type 2. Case 2: A one-month-old female presented with recurrent respiratory distress and infections. Imaging showed a large cystic lesion in the right middle lobe. Right middle lobectomy confirmed CPAM type 3. Case 3: A two-month-old male presented with dyspnea. Imaging showed a large bullae in the right chest with mediastinal shift. Right upper lobectomy confirmed CPAM type 1.
UNASSIGNED: CPAM is a rare congenital lung malformation characterized by abnormal bronchial development and localized glandular overgrowth. Management involves surgical resection, with the timing of surgery dependent on symptomatology. Early identification and intervention are crucial for preventing complications such as pulmonary hypoplasia and recurrent infections. Histopathological examination post-resection is essential for accurate classification and management of CPAM.
CONCLUSIONS: This case series provides valuable insights into the clinical, radiological, and histopathological features of CPAM, including the types encountered in each case. It underscores the importance of early diagnosis and timely surgical intervention, advocating for routine prenatal and postnatal screenings to effectively identify and manage CPAM.