SPONTANEOUS LOCALIZED NON-NECROTIZING ENTEROCOLITIS PERFORATIONS IN EXTREMELY LOW BIRTH WEIGHT INFANTS By J. E. Uceda, C.A. Laos, H.W. Kolni, and A.M. Klein Dallas, Texas Background/ Purpose: Localized non-NEC intestinal perforations have been commonly observed in premature infants weighing less than 1000 grams during the last fifteen years. Conservative management with peritoneal lavage and drainage has been advocated but mortality figures are still high. We wish to present our experience with 56 consecutive cases treated at a single institution. Methods: The authors reviewed the outcome of extremely low birth weight infants (ELBWI) undergoing surgery for non-NEC intestinal perforations. Results: From 1986 to 2001, 56 ELBWI with spontaneous, localized, non-NEC intestinal perforations were treated. Their birth weight and gestational age averaged 705 grams and 24.8 weeks, respectively. A one inch long laparotomy incision was followed by saline lavage and enterostomy. After recovery, reanastomosis was done. Our survival was 91.1%. 88% of 51 survivors were followed for an average of 77 months and 34 children had excellent recovery. 5 patients died, 3 with Candida sepsis at 1, 3 and 8 weeks post operatively, one with severe broncopulmonary dysplasia at 6 weeks and one with cardiopulmonary failure at 15 months of age. Conclusions: Early enterostomy with subsequent reanastomosis is a satisfactory management of spontaneous, localized, non-NEC intestinal perforations in ELBWI. 2 INDEX WORDS: Extremely low birth weight infants, intestinal perforations, nonnecrotizing enterocolitis. Spontaneous, localized, non-NEC intestinal perforations have been commonly observed in extremely low birth weight infants during the past fifteen years. These patients typically weigh less than 1000 grams and are 23 to 28 weeks of gestational age. Several reports have proposed peritoneal lavage and drainage as the initial and even only treatment for this condition but overall survival has been low or the series have been small. We wish to report our experience with 56 consecutive infants treated with an initial exteriorization of the perforation followed by a subsequent reanastomosis. MATERIAL AND METHODS During the eleven year period between February 1991 and November 2001, 55 consecutive <1000 gram infants were treated by one surgeon at a single institution. All patients presented with a typical non-NEC, spontaneous, localized intestinal perforation. Another patient had been seen in 1986 and was included in the study. Bell’s diagnostic criteria were used to exclude necrotizing enterocolitis cases managed during the study period. Post operative examinations by us or the referring pediatricians or telephone interviews with relatives provided follow up information. RESULTS There were 35 males and 21 females. Birth weights and gestational ages averaged 704 grams (range, 460-1000) and 24.5 weeks (range, 23-28), respectively. Perinatal medications included indomethacin (34 patients), dexamethasone (48 patients) and inotropics (54 patients). Most infants were on conventional or high frequency 3 ventilation at the time their perforations were diagnosed which typically occurred within fifteen days of life. Bluish abdominal discoloration was seen in 42 infants. Marked leukocytosis (>20,000 in 45 patients), rare thrombocytopenia (<50,000 in 6 patients) and positive cultures to S. Epidermidis (26 patients) were observed. Left lateral decubitus abdominal X rays detected pneumoperitoneum in 40 instances. None of our patients had pneumatosis intestinalis or portal venous gas on their abdominal roentgenograms. Nine infants underwent patent ductus closure, 4 before and 5 after their perforation. All but two patients with duodenal perforations had an initial enterostomy. After bile stained peritoneal fluid was cultured, saline lavage was done and a typically single, 5-10 mm perforation became visible. Most of them were in the distal ileum. Excessive handling of the bowel was purposely avoided, the perforation resected and the resulting bowel ends carefully attached to the right corner of the incision with 5-0 polypropilene sutures to the peritoneal edge. Wound closure was completed with a few interrupted 3-0 vicryl to fascia and peritoneum. Skin edges were not reapproximated. Reanastomosis was initially done when infants reached 2000 grams, or earlier in the few instances when stomas began to prolapse. Anastomoses were done in two layers and we had no leaks. With added experience, reanastomoses were accomplished earlier than before, the smallest infant weighing 765 grams. 51 (91.1%) of our 56 patients survived. Three infants died at 1, 3 and 8 weeks postoperatively due to severe bronchopulmonary dysplasia (BPD) and candida sepsis. The other two deaths occurred 6 weeks and 15 months after surgery, due to severe BPD and cardiopulmonary failure, respectively. 45 (88%) of the survivors were followed up an average of 77 months. 38 patients had good to excellent results. Bilateral blindness occurred in 2, unilateral blindness in 2, fundoplication and lysis of adhesions were 4 needed in one each and one last patient developed diabetes insipida which was medically controlled. DISCUSSION Ten years ago we published our early experience with laparotomy and enterostomy on non-NEC intestinal perforations in extremely low weight (<1000 grams) premature infants. We compared 17 such patients with 16 under-1000 grams NEC patients operated upon at our institution. Bell’s criteria were followed to differentiate both groups. 15 patients in the non-NEC group survived (88.2%).1 Our non-NEC patients were strikingly similar to those reported in the neonatal literature at that time.2-6 Peritoneal drainage (PD) for necrotizing enterocolitis perforation was first reported in 19757 and a 13-year experience by the same group was published in 1990. Of 37 patients drained, 9 died soon thereafter, 16 had to be operated upon and 7 of these died, but 12 (32%) recovered without other treatment.8 By the end of the 1990’s three studies of perforated non-NEC infants reported survivals of 67%, 100% and 90%, but these were small series (9, 8 and 10 patients, respectively).9-11 Two more recent series of 25 and 19 patients, reported 66% and 53% survivals12,13 There is no question than many ELBWI with non-NEC perforations improve initially with PD and some even recover without further operations9-13 but our experience with 56 infants and their 91.1% long term survival supports our earlier judgement that primary laparotomy remains a safe and consistent approach to treat infants with non-NEC perforation. Two additional recent reports have expressed similar conclusions.14,15 REFERENCES 5 1. Uceda JE, Laos CA, Kolni HW, et al: Intestinal perforations in infants with a very low birth weight: A disease of increasing survival? J Pediatr Surg 30:13141316, 1995 2. Weinberg G, Kleihaus S, Boley SJ: Idiopathic intestinal perforations in the newborn: An increasingly common entity. J Pediatr Surg 24:1007-1008, 1989 3. Novack CM, Waffarn F, Sills JH, et al: Focal intestinal perforation in the extremely low birth weight infant. J Perinatol 14:450-453, !994 4. Meyer CL, Payne NR, Roback SA: Spontaneous isolated intestinal perforations in neonates with birth weight <1000g. J Pediatr Surg 26:714-717, 1991 5. Mintz AC, Applebaum H: Focal gastrointestinal perforations not associated with necrotizing enterocolitis in very low birth weight neonates. J Pediatr Surg 28:857-860, 1993 6. Wolf WM, Snover DC, Leonard AS: Localized intestinal perforation following intravenous indomethacin in premature infants. J Pediatr Surg 24:409-410, 1989 7. Marshall DG: Peritoneal drainage under local anesthesia for necrotizing enterocolitis perforation. Presented at the meeting of the Canadian Association of Pediatric Surgeons, Winnipeg, Manitoba, January 1975 8. Ein SH, Shandling B, Wesson D, et al: A 13-year experience with peritoneal drainage under local anesthesia for necrotizing enterocolitis perforation. J Pediatr Surg 25:1034-1037, 1990 9. Lessin MS, Luks FI, Wesselhoeft CW, et al: Peritoneal drainage as definitive ttreament for intestinal perforation in infants with extremely low birth weight (<750g). J Pediatr Surg 33:370-372, 1998 6 10. Rovin JD, Rodgers BM, Burns RC: The role of peritoneal drainage for intestinal perforation in infants with and without necrotizing enterocolitis. J Pediatr Surg 34:143-147, 1999 11. Cass DL, Brandt ML, Patel DL, et al : Peritoneal drainage as definitive treatment for neonates with isolated intestinal perforation. J Pediatr Surg 35:1531-1536, 2000 12. Gollin G, Abarbanell A, Baerg J: Peritoneal drainage as definitive management on intestinal perforation in extremely low birth weight infants. J Pediatr Surg 38:1814-1817, 2003 13. Sharma R, Tepas III JJ, Mollitt DL, et al : Surgical management of bowel perforations and outcome in very low birth weight infants (<1200g). J Pediatr Surg 39:190-194, 2004 14. Camberos A, Patel K, Applebaum H: Laparotomy in very small premature infants with necrotizing enterocolitis or focal intestinal perforation: Postoperative outcome. J Pediatr Surg 37:1692-1695, 2002 15. Hwang H, Murphy JJ, Gow KW, et al: Are localized intestinal perforations distinct from necrotizing enterocolitis? J Pediatr Surg 38:763-767, 2003