Intra-diaphragmatic Extralobar Pulmonary Sequestration: Surgical Approaches and Outcome

Background/aims: Intra-diaphragmatic extralobar pulmonary sequestration (IDEPS) is an extremely rare congenital lung malformation characterized by non-functional lung tissue within the diaphragm. Although surgical resection is frequently performed, management strategies vary significantly between centers and remain controversial due to difficulties in accurately locating the lesion intraoperatively and the absence of large case series. This retrospective multicenter study analyzes the experience in the treatment of IDEPS and compares the findings with those reported in the existing literature. Materials and methods: All pediatric patients treated for IDEPS between 2010 and 2024 were included in the study. Data were collected from hospital records. Demographic characteristics, surgical approach, operative time, complications, and postoperative recovery were analyzed. A literature review was also conducted to identify relevant studies on IDEPS in pediatric patients. Results: Twenty-three patients (24 IDEPS) were identified, with a male-to-female ratio of 1.8:1. The median age at surgery was 8.2 months. Prenatal diagnosis was confirmed in all cases. Surgical approaches included thoracoscopy (n = 11), laparoscopy (n = 8), and open surgery (n = 5). The conversion rate was 1/11 for thoracoscopy and 0/8 for laparoscopy. A double-cavity approach was required in 3 cases. The median operative time was 157 minutes, and the median hospital stay was 4.5 days. Postoperative complications occurred in 4.3% of patients. Histological examination revealed hybrid lesions in 39% of cases. Conclusions: IDEPS is a rare condition that presents diagnostic and surgical challenges. Surgical resection remains the treatment of choice, with minimally invasive techniques proving effective when the lesion is well localized. Accurate preoperative imaging and timely intervention are essential for achieving successful outcomes, particularly given the frequent occurrence of hybrid lesions. Further studies are warranted to establish standardized management guidelines. Keywords: Congenital Pulmonary Airway Malformation (CPAM); Intra-diaphragmatic Extralobar Pulmonary Sequestration (IDEPS); Minimally-Invasive Surgery (MIS).