Department of Cardiothoracic Surgery, Shenzhen Children's Hospital, No.7019, Yitian Road, Shenzhen, 518038, Guangdong, China.
J Cardiothorac Surg. 2022 Jun 29;17(1):168. doi: 10.1186/s13019-022-01921-8.
Extralobar pulmonary sequestration is an uncommon congenital pulmonary malformation. Clinically, pedicle torsion of extralobar pulmonary sequestration is extremely rare. Due to inadequate awareness of its atypical presentation and imaging characteristics, clinical diagnosis is very difficult, and it is extremely easy to misdiagnose.
There were 6 children (3 males and 3 females), aged 3-12 years old. The main clinical symptoms of the children were abdominal and chest pain (3 cases), abdominal pain (1 case), chest pain (1 case), and vomiting and abdominal distension (1 case). Two cases were accompanied by fever. Preoperative ultrasound revealed a well-bordered mass with soft-tissue density, accompanied by pleural effusion. On contrast-enhanced computed tomography scans, the mass showed no obvious enhancement. A blood supply was only present in 1 case, and there was no feeding artery shown in the other 5 cases. Extralobar pulmonary sequestration with haemorrhagic infarction was pathologically confirmed. On postoperative days 2-6, the children were discharged uneventfully. There were no complications during the median follow-up of 4 months.
Torsed extralobar pulmonary sequestration usually occurs in childhood or adolescence, with abdominal and/or chest pain as the primary symptoms. Imaging examination shows a well-defined soft-tissue mass without enhancement. The feeding vessel is not clearly displayed in the mass, and extralobar pulmonary sequestration is accompanied by varying amounts of pleural effusion. Video-assisted thoracoscopic surgical resection is associated with excellent prognosis.
肺隔离症是一种罕见的先天性肺畸形。临床上,肺隔离症的蒂扭转极为罕见。由于对其非典型表现和影像学特征认识不足,临床诊断非常困难,极易误诊。
共 6 例患儿(男 3 例,女 3 例),年龄 312 岁。患儿主要临床表现为腹痛和胸痛(3 例)、腹痛(1 例)、胸痛(1 例)和呕吐、腹胀(1 例)。2 例伴有发热。术前超声显示边界清楚的软组织密度肿块,伴有胸腔积液。增强 CT 扫描显示肿块无明显强化,仅 1 例存在供血动脉,其余 5 例均未见供血动脉。术后病理证实为肺隔离症伴出血性梗死。术后第 26 天,患儿顺利出院。中位随访 4 个月无并发症。
扭转性肺隔离症通常发生在儿童或青少年期,以腹痛和/或胸痛为主要症状。影像学检查显示边界清楚的软组织肿块,无强化。肿块内未见明确的供血血管,肺隔离症伴有不同程度的胸腔积液。胸腔镜辅助手术切除具有良好的预后。