Baerg, Kanthimathinathan, Moores. Late Presenting Congenital Diaphragmatic Hernia: A 21 year Single Center Review Poster and oral presentation (3 min) at the International Meeting of the CDH Study Group Rome, Italy February 2011 ( 2/2011 )
LATE PRESENTING CONGENITAL DIAPHRAGMATIC HERNIA: A 21-YEAR SINGLE CENTER REVIEW J Baerg MD, V Kanthimathinathan MD, D Moores MD Loma Linda University Children’s Hospital, Loma Linda, CA Aim: To describe the outcome of children with late presenting congenital diaphragmatic hernia (CDH) and compare the most recent decade (2000-2009) to the previous decade (1989-1999) for changes in the clinical practice. Methods: After IRB approval, patients who presented with CDH between 30 days and 18 years, between January 1989 and December 2009, were reviewed retrospectively. Outcome data were collected by telephone survey in 2010. Data were compared by Fisher exact test and t-test. P<0.05 was significant. Results: During the study period, 31 children (22/31(71%) males) presented between 45 days and 13 years (median: 9 months). Morgagni hernia was present in 14/31(45%) and Bochdalek in 17/31(55%). There were 20/31(64%) left-sided, 8/31(26%) right-sided and 3/31(10%) bilateral. Acute symptoms were present in 24/31(77%), chronic symptoms in 5/31(17%), and 2/31(6%) had an incidental diagnosis of CDH. There were 10/31(32%) misdiagnosed as pneumonia, one patient having undergone a prior thoracotomy for empyema at another center. There was 1/31(3%) misdiagnosed as a chest mass, who required extensive intestinal resection after strangulation in a left Bochdalek hernia. Between 1989-1999, 14/31(45%) open repairs were performed, 2/14(14%) with patch. Between 2000-2009, 17/31(55%) repairs were performed, 7/17(41%) laparoscopic. Blood loss (p=0.04), post-op ventilation (p=0.004) and hospital stay (p= 0.04) were significantly higher between 1989-1999. Operative times were significantly longer (p=0.005) between 2000-2009. Telephone follow-up was complete for 27/31(87%) and ranged from 1-20 years (median: 7 years). All survived, none had surgery for recurrence, malrotation or bowel obstruction. The child with bowel strangulation is alive at 18 years with short bowel on TPN. Conclusion: Patients with repaired, late presenting CDH survive without sequellae, unless misdiagnosis occurs. Clinical practice is improving. The most recent decade demonstrates significant improvements in length of stay, operative blood loss and use of postoperative ventilation. The increased operative times reflect the learning curve of laparoscopic repairs.