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.

Torsed extralobar pulmonary sequestration exhibiting characteristic MRI findings

“…Blood supply from a systemic artery is an important finding in pulmonary sequestration, and there has been a report describing such findings on MRI even in torsed ELS 3. However, a mass at the base of the thoracic cavity with poor blood flow on CE-CT or MRI, as in this case, is an important finding suggestive of torsed ELS 2. There are few studies on MRI findings of torsed ELS, and we were unable to find any reports of DWI in the literature.…”

“…3 However, a mass at the base of the thoracic cavity with poor blood flow on CE-CT or MRI, as in this case, is an important finding suggestive of torsed ELS. 2 There are few studies on MRI findings of torsed ELS, and we were unable to find any reports of DWI in the literature. If there is haemorrhagic infarction in ELS, the signal intensity may vary depending on the time of imaging.…”

“…ELS is usually asymptomatic and torsed ELS is extremely rare. Children with torsed ELS sometimes present with symptoms mimicking intrathoracic infections 2. Due to its resemblance to intrathoracic or mediastinal tumours, torsed ELS is difficult to diagnose preoperatively without awareness of its imaging features 2.…”

“…Children with torsed ELS sometimes present with symptoms mimicking intrathoracic infections 2. Due to its resemblance to intrathoracic or mediastinal tumours, torsed ELS is difficult to diagnose preoperatively without awareness of its imaging features 2. Blood supply from a systemic artery is an important finding in pulmonary sequestration, and there has been a report describing such findings on MRI even in torsed ELS 3.…”

Torsed extralobar pulmonary sequestration associated with congenital lobar emphysema: a rare entity

“…3 Torsion of ELS was more common in the left thorax. 1,3,4,[7][8][9][10][11][12]14,15 Contrast-enhanced CT scan was the most commonly used examination for the diagnosis of torsed ELS. Generally, the demonstration of a dominant feeding vessel suggests the diagnosis of ELS.…”