2015 Volume 51 Issue 5 Pages 868-872
Purpose: We assessed the validity of aggressive surgical intervention in the management of trisomy 13 and trisomy 18.
Methods: The clinical records of 21 patients with trisomy 13 or 18 admitted to our neonatal intensive care unit between 2008 and 2014 were retrospectively analyzed.
Results: All 21 patients were diagnosed as having some abnormalities, and 9 patients (42.9%) were diagnosed as having chromosomal anomalies prenatally. Eleven patients (52.4%) were diagnosed as having pediatric surgical diseases postnatally: intestinal malrotation, esophageal atresia, gastroesophageal reflux disease, diaphragmatic hernia, hepatoblastoma, anorectal malformation, omphalocele, and inguinal hernia. Two patients with intestinal malrotation and 1 patient with esophageal atresia had radical operation and have survived for 2–5 years at home. A patient with hepatoblastoma as well as congenital heart disease made the transition to palliative care. Three patients with poor prognosis underwent palliative procedure, and 2 patients died without undergoing surgical treatment. Tracheostomy for long-term endotracheal intubation was carried out in 4 patients. Cardiovascular surgery including intracardiac repair was performed in 3 patients. Heart disease in 20 patients (95.2%) was the major prognostic factor, and neonatal intensive care affected short-term mortality. Procedures by pediatric surgeons did not seem to have a direct effect on the patients’ prognosis and did not disturb the time shared with their families.
Conclusions: Recent reports focus on long-term survivors with trisomy 13 and trisomy 18 suggesting the necessity of appropriate treatment in accordance with individual condition. It is ethically reasonable that pediatric surgeons provide aggressive surgical intervention on the basis of objective medical evaluation.