Prenatal Diagnosis and Surgical Innovations in Congenital Diaphragmatic Hernia: Evaluation of Pre- And Post-Operative Management

Abstract

Introduction. Congenital diaphragmatic hernia (CDH) is still today considered a challenge from surgeons. Considerable progress in prenatal diagnosis, intensive care unit of neonates and surgical techniques, with the possibility to perform minimally access surgery, widely increased survival rates. The aim of this study is to analyze our series about long and short-term outcomes, also considering the progress made by minimally invasive techniques. Methods. The study was performed at Pediatric Surgery of Siena. It is a retrospective study that analyzed all patients with CDH (Bochdalek) treated in the last 14 years, from 2000 to 2013. Sex, side of the defect, presence of prenatal diagnosis, age of onset and symptoms, associate malformation, herniated organs, surgical technique and site of surgery, complications, recurrences, survival and followup were analyzed. Results. We included 23 patients. Five of them, were ruled out because of affected by diaphragmatic eventration or acquired diaphragmatic hernia. Forteen patients (77%) presented left CDH and 4 patients (23%) a right one. The male female ratio were 14:4. Prenatal diagnosis was performed in 5 patients (27,5%) at a mean gestational age of 29 weeks. Forteen patients (77%) had an early onset of symptoms (first day of life). Most common symptoms were respiratory distress and cyanosis; 4 patients (23%) had a late onset of symptoms, at a mean age of 9 months, and most common symptoms were failure to thrive and vomiting. Seven patients (39%) had associated malformation: common mesenterium (5 pts-71%). The colon was the most commonly herniated organ, present in 15 patients (83%), followed by small intestine in 13 patients (72%), stomach in 11 patients (61%), spleen in 9 patients (50%) and liver in 4 patients (23%). Seventeen patients (94,5%) underwent open surgery: 10 of them (59%) underwent a subcostal laparotomy approach, 7 of them (41%) underwent a supraumbelical laparotomy approach; 1 patient (5%) underwent minimally access surgery with thoracoscopy access. We performed performed surgery in the intensive care unit in 3 patients (16,5%). Six patients (33%) developed minor postoperatory complications. No patient had recurrence. Four patients died so we report a mortality rate of 23%. A follow-up investigation, with an average duration of 87 months, it is still going on in 5 patients (27,5%). Conclusions. The our survival rate was 77% and it reflects the encouraging reported data in the recent literature. These results are due to the reliability of the new resuscitation strategies, such as high-frequency oscillatory ventilation and the use of NO, the ability to perform surgery in the neonatal intensive care unit and, especially, to successfully perform minimally invasive surgery in newborn. The improvement of the survival showed the increasing of long-term morbidity end the requirement of a multidisciplinary followup. For these reasons, a multidisciplinary pathway for the management of young patients has been created, to follow them in a standardized way as early as the prenatal diagnosis.