Clinical analysis of extralobar pulmonary sequestration with torsion in children: report of 6 cases

Abstract

Background: 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.

Case presentation: 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.

Conclusions: 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.

Keywords: Children; Extralobar; Pedicle torsion; Pulmonary sequestration.

Fig. 1

Axial Contrast-enhanced CT image of the chest A indicates that a well-bordered, soft-tissue density mass without enhancement in the right thoracic cavity, coronal CT image B shows a well-defined soft tissue mass between the diaphragm and the spine, video-assisted thoracoscopy C reveals a purplish-red mass with congestion and necrosis in the pleural cavity, and D shows a twisted vascular pedicle