Torsion of Extralobar Pulmonary Sequestration Presenting as an Acute Abdomen: When the True Culprit is in the Chest

A 4-year-old boy with sudden, severe abdominal pain without the occurrence of fever, suggesting a surgical abdominal aetiology, was referred to our center. On admission, laboratory tests showed a biological inflammatory syndrome (C-reactive protein 200 mg/L, procalcitonin [PCT] negative) associated with neutrophil hyperleukocytosis (white blood cell count 15 G/L, including 8.8 G/L neutrophils). The patient had not undergone a pre-incident imaging examination. The clinical course was marked by the secondary onset of acute respiratory distress. A thoracic computed tomography (CT) scan and pleural ultrasound revealed a non-vascularised left lower pleural tissue mass within a large effusion (Figure 1). Exploratory thoracoscopy confirmed the diagnosis of torsion and infarction of extralobar pulmonary sequestration (ELS) associated with haemothorax. Surgical management consisted of clipping of the vascular pedicle followed by resection of the infarcted mass, leading to a rapid improvement of symptoms. ELS is a rare congenital lung malformation. It is characterized by abnormal lung parenchyma not connected to the tracheobronchial tree, with its own visceral pleura and vascularized by a systemic artery. Torsion is an exceptional complication of the rare congenital ELS malformation, for which a pre-operative diagnosis can be made based on clinical and radiological assessments. To date, only 29 pediatric patients with torsion of ELS have been reported. Symptoms can be misleading and may initially suggest an abdominal etiology. Moreover, torsion can cause the supply artery not appear on imaging, which causes difficulty in diagnosing ELS with torsion. A young patient presenting with sudden abdominal pain, with a homogeneous, well-defined mass of soft tissue density in the thorax at CT scan, should prompt an assessment for ELS with torsion. Pierre Gourdan accepts full responsibility for the work, had access to the data, and controlled the decision to publish. He wrote the article and performed the litterature search.Olivier Haas Ferrua wrote the article and performed the litterature search. Jean-Marc Gilardoni, Sibylle Blanc, Souad Ghattas, Valérie Triolo, Myriam Guesmi, Aurélie Gerstner, Sébastien Barthelemy, Audrey Dupont, Emmanuelle Gondon, and Jean-François Lecompte had done the final approval.Lisa Giovannini-Chami had the idea for the article, revising critically and done the final approval. The authors received no specific funding for this work. The authors have nothing to report. The authors have nothing to report. The authors declare no conflicts of interest. The authors have nothing to report.