Victoria H. Koa,b; Abigail Walshb, and Christopher A. Gitzelmannb
aDepartment of General Surgery, Rutgers-New Jersey Medical School, 185 S. Orange Ave, Newark, NJ 07103, USA
bDivision of Pediatric Surgery, Saint Barnabas Medical Center, 94 Old Short Hills Road, Livingston, NJ 07039, USA
Neonates with multiple, complex congenital lesions necessitating surgical intervention require medical and surgical expertise for successful outcomes.We report the case of a neonate born with a rare combination of ruptured omphalocele andType IV intestinal atresia who was medically and surgically managed at a New Jersey hospital.This case demonstrates the effective and successful care of a surgically complex neonate at a local New Jersey acute care hospital that provides tertiary pediatric surgical services.
Keywords: omphalocele, ruptured omphalocele, multiple atresia, apple-peel atresia,
Neonates prenatally diagnosed with multiple, complex congenital lesions requiring surgical intervention are commonly managed at a tertiary referral center [1,2].The case presented here illustrates the successful medical and surgical management of a neonate with two complex congenital lesions, ruptured omphalocele and a Type IV atresia, at a tertiary pediatric surgical program at a New Jersey hospital with excellent outcome and minimal morbidity.
A newborn male with a prenatal diagnosis of small omphalocele was born at 36 weeks gestation via planned Cesarean section after premature labor. No other prenatal abnormalities were identified. Immediate evaluation of the newborn on Day of Life (DOL) 0 demonstrated obvious ruptured omphalocele (Figure 1), given its midline location through the umbilical ring and prenatal diagnosis on ultrasound with previously intact sac, and he was urgently brought to the operative room for exploratory laparotomy. Intraoperative evaluation showed multiple intestinal atresias in addition to ruptured omphalocele which involved the liver.An abdominal woundVAC was placed followed by return to NICU for further resuscitation and work up. A renal ultrasound additionally revealed bilateral cryptorchidism. On DOL 3, he was brought back for a second-look laparotomy where he was found to have a Type IV intestinal atresia which consisted of a Type IIIb “apple-peel” atresia and multiple Type II atresias.The most proximal atresia was 30 cm distal to the pylorus. A 17 cm dilated portion of jejunum (Figure 1, label A) proximal to the first atresia was resected and brought up as an end jejunostomy. An illustration of the abnormal anatomy is shown in the right panel of Figure 1. On DOL 23, he underwent excision of five additional intestinal atresias.The hypoplastic segments of small intestine were thread onto a 5F feeding tube and atresias were repaired over the catheter via end-to-end anastomoses (Figure 2). In total, sixty-five centimeters of small intestine remained from the pylorus to the ileocecal valve. A mucous fistula was created at the proximal end of the repaired hypoplastic intestine and a wound VAC was placed (Figure 3)
Figure 1. Neonate on DOL 0 with ruptured omphalocele shown in the panel on the left. A dilated segment of jejunal bowel which was ultimately resected is labeled (A). An illustrative schematic of the encountered anatomy is shown in the panel on the right.
Figure 2. Intraoperative evaluation of multipleType II atresias on DOL 23. A 5F feeding tube was passed through multiple hypoplastic segments of small intestine.The atresias were then repaired over the feeding tube via end-to-end anastomoses.
Figure 3. Following the completion of multiple intestinal atresia repairs, a mucous fistula was created and a wound VAC was placed.
Proximal enteral feeds were started on DOL 26 and refeeding through the mucous fistula started on DOL 32, after a contrast study confirmed no anastomotic leaks, to encourage intestinal growth and prevent mucosal atrophy. Serial contrast radiographs were performed to follow the intestinal development of the baby. As enteral feeds and refeeds were increased, concurrent intestinal growth was seen (Figure 4). On DOL 193, when adequate intestinal growth was documented and the patient’s nutritional status was sustained on enteral feeds alone, the patient underwent takedown of his end jejunostomy and mucous fistula with creation of an end-to-end anastomosis with primary repair (Figure 5). Post-operatively the patient resumed feeds on post-operative day 4 and remained hospitalized for 4 weeks to improve his PO intake. He did require excision of a surgical granuloma 3 weeks after his final surgery. He was discharged home on DOL 220 on full oral feeds with adequate weight gain. At follow up appointments, he exhibits excellent growth and is meeting his developmental milestones.
Figure 4. Serial contrast studies were performed through the mucous fistula. On post-operative day (POD) 8 after repair of multiple intestinal atresias, no intestinal leak was identified and refeeding through the mucous fistula was begun (A). Subsequent studies demonstrate progressive intestinal development with continued refeeding on POD 40 (B) and POD 161 (C).
Figure 5. Side-by-side intraoperative comparison of small intestine on DOL 23 (A) prior to intestinal atresia repair and on DOL 193 (B) at the time of end jejunostomy (ej) and mucous fistula (mf) take down with primary anastomosis.
Omphaloceles are among the most common congenital anterior abdominal wall defects with an incidence of 1 to 2 per 10,000 live births per year [3-5]. Clinically, omphaloceles are described as midline defects at the umbilical ring through which the midgut and abdominal organs may herniate.The herniated contents are typically covered by a sac consisting of an outer amniotic layer, middleWharton’s jelly layer, and an inner peritoneal layer . Omphaloceles can be classified by their size, contents, location, and integrity of the sac . Ruptured omphaloceles are defined as those in which when the sac is no longer intact, and evisceration of abdominal organs occur.These present a unique surgical challenge as early diagnosis and management prevent associated morbidity and mortality.
Omphaloceles are more commonly associated with other congenital anomalies and have higher mortality as compared to infants with gastroschisis. However, intestinal atresias are not commonly associated with omphalocele. Patients with gastroschisis historically have higher association with intestinal atresias which confer a poorer outcome when associated with complex gastroschisis[12,13]. Intestinal atresias are categorized by type and location of the atresia with an incidence of 1.6-3.4 per 10,000 births. In type I atresias, bowel remain connected by a thin septum or membrane with dilation of the proximal segment of bowel. In type II atresias, the bowel ends are in discontinuity, but the mesentery is intact.Type IIIa are like type II atresias with the exception that a “V-shaped” deformity is seen in the mesentery.Type IIIb atresias are known as “apple peel” or “Christmas tree” deformities.These atresias are described as spiralized or twisted bowel surviving on a single, short mesenteric artery and are the rarest variant, accounting for 5% of all intestinal atresias . Its rarity makes clinical prognosis challenging, however, duration of parenteral feeds, remaining bowel length, and other associated anomalies does affect long-term outcome of all atresias[14-16]. Finally, type IV atresias are reserved for identification of multiple atresias of varying types, as in our patient presented here.
While risks of morbidity and mortality were relatively high in the immediate hospitalized period for this child, his long-term outcomes are favorable [10,14].This child will have to undergo reconstructive surgery of his abdominal muscles in the future as well as corrective surgery for his bilateral cryptorchidism, but an intra-abdominal reoperation is not anticipated.
We present a surgically complex neonate with two complicated congenital malformations that was unexpected at the time of delivery. Historically, in New Jersey, an expected mother, whose fetus is diagnosed with a congenital malformation, is referred to regional centers out of state for neonatal surgical management.This often requires mothers to transfer their obstetrical care as well depart their local support systems of family and community and in many instances, is an unnecessary transfer. New Jersey is home to highly skilled pediatric surgeons staffed in hospitals with level 3 NICUs who can care for these complex neonates, addressing their surgical needs.
Authors VKH, AW, and CG equally contributed to the research development, writing assistance, and proofreading of this manuscript. No additional funding was awarded towards the development of this manuscript.
- Kitchanan S, Patole SK, Muller R,Whitehall JS (2000) Neonatal outcome of gastroschisis and exomphalos: a 10-year review. J Paediatr Child Health 36 (5):428-430. doi:10.1046/j.1440-1754.2000.00551.x
- McCormick MC, Shapiro S, Starfield BH (1985) The regionalization of perinatal services. Summary of the evaluation of a national demonstration program. JAMA 253 (6):799-804
- Benjamin B,Wilson GN (2014) Anomalies associated with gastroschisis and omphalocele: analysis of 2825 cases from the Texas Birth Defects Registry. J Pediatr Surg 49 (4):514-519. doi:10.1016/j.jpedsurg.2013.11.052
- Marshall J, Salemi JL,Tanner JP, Ramakrishnan R, Feldkamp ML, Marengo LK, Meyer RE, Druschel CM, Rickard R, Kirby RS, National Birth Defects Prevention N (2015) Prevalence, Correlates, and Outcomes of Omphalocele in the United States, 1995-2005. Obstet Gynecol 126 (2):284-293. doi:10.1097/AOG.0000000000000920
- Stallings EB, Isenburg JL, Short TD, Heinke D, Kirby RS, Romitti PA, Canfield MA, O’Leary LA, Liberman RF, Forestieri NE, Nembhard WN, Sandidge T, Nestoridi E, Salemi JL, Nance AE, Duckett K, Ramirez GM, Shan X, Shi
J, Lupo PJ (2019) Population-based birth defects data in the United States, 2012-2016: A focus on abdominal wall defects. Birth Defects Res 111 (18):1436-1447. doi:10.1002/ bdr2.1607
- Klein M (2012) Congenital defects of the abdominal wall. In: Coran A, Adzick NS (ed) Pediatric Surgery. 7th edn. Elsevier Saunders, Philadelphia, PA, pp 973-985
- Khan FA, Hashmi A, Islam S (2019) Insights into embryology and development of omphalocele. Semin Pediatr Surg 28
- Verla MA, Style CC, Olutoye OO (2019) Prenatal diagnosis and management of omphalocele. Semin Pediatr Surg 28 (2):84-88. doi:10.1053/j.sempedsurg.2019.04.007
- Gonzalez KW, Chandler NM (2019) Ruptured omphalocele: Diagnosis and management. Semin Pediatr Surg 28 (2):101- 105. doi:10.1053/j.sempedsurg.2019.04.009
- Saxena AK, Raicevic M (2018) Predictors of mortality in neonates with giant omphaloceles. Minerva Pediatr 70 (3):289-295. doi:10.23736/S0026-4946.17.05109-X
- Corey KM, Hornik CP, Laughon MM, McHutchison K, Clark RH, Smith PB (2014) Frequency of anomalies and hospital outcomes in infants with gastroschisis and omphalocele. Early Hum Dev 90 (8):421-424. doi:10.1016/j. earlhumdev.2014.05.006
- Eggink BH, Richardson CJ, Malloy MH, Angel CA (2006) Outcome of gastroschisis: a 20-year case review of infants with gastroschisis born in Galveston,Texas. J Pediatr Surg 41 (6):1103-1108. doi:10.1016/j.jpedsurg.2006.02.008
- Baerg J, Kaban G,Tonita J, Pahwa P, Reid D (2003) Gastroschisis: A sixteen-year review. J Pediatr Surg 38 (5):771-774. doi:10.1016/jpsu.2003.50164
- Zhu H, Gao R, Alganabi M, Dong K, Ganji N, Xiao X, Zheng S, Shen C (2019) Long-term surgical outcomes of apple-peel atresia. J Pediatr Surg 54 (12):2503-2508. doi:10.1016/j. jpedsurg.2019.08.045
- DallaVecchia LK, Grosfeld JL,West KW, Rescorla FJ, Scherer LR, Engum SA (1998) Intestinal atresia and stenosis: a 25- year experience with 277 cases. Arch Surg 133 (5):490-496; discussion 496-497. doi:10.1001/archsurg.133.5.490
- Burjonrappa SC, Crete E, Bouchard S (2009) Prognostic factors in jejuno-ileal atresia. Pediatr Surg Int 25 (9):795-798. doi:10.1007/s00383-009-2422-y