Staged repair of giant omphalocele in the neonatal period

doi:10.1016/j.jpedsurg.2005.01.042

Journal of Pediatric Surgery

Volume 40, Issue 5, May 2005, Pages 785-788

https://doi.org/10.1016/j.jpedsurg.2005.01.042 Get rights and content

Abstract

Background/Purpose

The aim of this study was to analyze the outcome of giant omphalocele repaired in the neonatal period.

Methods

Twelve consecutive (1997-2004) neonates with giant omphalocele (defect >6 cm with liver herniation) were reviewed. A silo of Prolene mesh (Ethicon) was attached to the fascia and the defect was closed without opening the amniotic sac after sequential reduction. In 2 neonates with ruptured omphalocele a plastic sheet was inserted below the mesh. Data are reported as median and range.

Results

Gestational age was 38 weeks (range, 32-40 weeks) and birth weight was 2.9 kg (range, 1.0-3.1 kg). The final closure was achieved at 26 days (range, 16-62 days). Three neonates (25%) died before final closure (causes: ruptured omphalocele, lung hypoplasia, cardiac anomalies, and intestinal failure). In the 9 surviving neonates, mechanical ventilation was required for 8 days (range, 2-20 days), hospital stay was 42 days (range, 23-73 days), and full enteral feeding was achieved on day 12 (range, 4-53 days). Complications included wound infection in 5 neonates and midgut volvulus in 1. Prophylactic Ladd's procedure was performed laparoscopically at a later stage in 4 children. At laparoscopy, intraperitoneal adhesions were minimal and the central liver did not preclude the operation. The 9 survivors are all well after 46 months (range, 12-67 months).

Conclusions

Giant omphalocele can be safely repaired in the neonatal period without opening the amniotic sac. Intestinal malrotation should be excluded and Ladd's procedure can be performed laparoscopically at a later stage.

Section snippets

Materials and methods

A review of 12 neonates with giant omphalocele consecutively treated at Great Ormond Street Hospital for Children, London, between January 1997 and November 2004 was performed. The study was approved by the Institute of Child Health and Great Ormond Street Hospital for Children Research Ethics Committee. Giant omphalocele was defined as abdominal wall defect larger than 6 cm and containing a major portion of the liver. Patients' characteristics, management, and outcome were reviewed from

Results

There were 12 neonates with giant omphalocele (5 males and 7 females). Maternal age was 30 years (range, 27-36 years), gestational age at birth was 38 weeks (range, 32-40 weeks), and birth weight was 2.9 kg (range, 1.0-3.1 kg). Two patients were born by emergency cesarean delivery at 32 and 34 weeks. The remaining patients were born by elective cesarean delivery at term. The diameter of the anterior abdominal wall defect was 7 cm (range, 6-15 cm). Congenital associated anomalies included

Discussion

The survival rate of neonates with omphalocele depends on the size of the defect, the degree of viscero-abdominal disproportion, the presence of associated anomalies, and the presence of respiratory distress at birth [18], [19]. The surgical management of giant omphalocele is still controversial. The anterior abdominal defect can be closed in the neonatal period or later in life. Both approaches require a staged procedure because attempts to reduce the herniated organs in the abdominal cavity

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Omphalocele is the result of the abnormal interaction between biological/molecular mechanisms and a genetic predisposition during the first twelve weeks of development, where the return of the intestinal loops to the abdominal cavity is disturbed. Muscle relaxation with botulinum toxin A (TxBtA) has been used as an adjuvant postnatal therapy for the repair of complex abdominal wall defects.
A female fetus was diagnosed with a giant omphalocele at 14 weeks of gestation. At 34 weeks and 5 days of gestation, 35 units of TxBtA (diluted at 100 units per 10 ml) were injected under ultrasound guidance into each muscle of the left and right sides of the abdominal wall with a 25G spinal needle. The fetus tolerated the procedure well. Two weeks after the injection, the first ultrasound examination was done and revealed a noteworthy change in the shape of the abdominal circumference, with a 50 % decrease in the omphalocele size. The newborn was delivered at 38 weeks and 3 days of gestation. The omphalocele had a maximum diameter of 7 cm. The patient required mechanical ventilation for 24 hours. The reconstruction of the anterior abdominal wall was completed on the fifth day of life using a component separation technique. Feedings were initiated one day after the closure and the patient was discharged home on the 12th day of life. The patient has not experienced any complications at 2 months of follow up.
Our case suggests that prenatal muscle relaxation of the abdominal wall with TxBtA could reduce the need for mechanical ventilation, the time on parenteral nutrition, and the overall hospital stay in newborns with giant omphalocele.
Fistula between a patent omphalomesenteric duct and a ruptured omphalocele sac in a neonate
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This case report serves to document a rare case of a neonate who presented to the NICU with a patent omphalomesenteric duct (OMD) connected to the top of a ruptured omphalocele sac. Initial evaluation of the neonate in-utero led to the diagnosis of gastroschisis due to the presence of free-floating intestines on ultrasound. However, a ruptured omphalocele sac was discovered grossly upon delivery. Additionally, intestines were found in the midline of the abdomen, a common characteristic of omphaloceles. Therefore, the diagnosis was modified from gastroschisis to ruptured omphalocele. Further gross examination revealed a patent omphalomesenteric duct forming a fistula to the top of the ruptured omphalocele sac. After the appropriate workup and necessary procedures, the neonate had only one bowel movement over 2.5 weeks and was intolerant of food, raising suspicion for an intestinal atresia. Evaluation revealed a type 1 intestinal atresia distal to the patent OMD and omphalocele sac. The atresia was initially masked by bowel inflammation in the neonate at birth. Resection of the atretic bowel led to appropriate bowel movements and increased food intake. The neonate was eventually discharged in good health.
The incidence of abdominal surgical site infections after abdominal birth defects surgery in infants: A systematic review with meta-analysis
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Citation Excerpt :
In 2178 patients a total of 100 wound dehiscences were seen. The pooled proportion of total wound dehiscence was 0.04 (95%-CI: 0.03–0.07; I2 = 73%, p ≤ 0.01) (Fig. 3) [12,18,21,22,28-30,32,41,45,58,67,69,73-75,77-79,87,89,90,92,93,103,107,118,125,133,134,136,138,141,149,155,159,162]. Separate pooled proportions were calculated for the following conditions: Anorectal malformations 0.04 (95% CI: 0.02–0.07; n = 52/1140; I2 = 74%; p ≤ 0.01); Gastroschisis 0.06 (95% CI: 0.04–0.08; n = 22/389; I2 = 0%; p = 0.11); Duodenal obstruction 0.01 (95% CI: 0.00–0.03; n = 3/315; I2 = 0%; p = 1.00); Hirschsprung's disease 0.06 (95% CI: 0.03–0.11; n = 8/137; I2 = 0%; p = 0.49); Biliary atresia 0.04 (95% CI: 0.01–0.10; n = 4/105; I2 = −0%; p = 0.19).
: Surgical site infections (SSI) are a frequent and significant problem understudied in infants operated for abdominal birth defects. Different forms of SSIs exist, namely wound infection, wound dehiscence, anastomotic leakage, post-operative peritonitis and fistula development. These complications can extend hospital stay, surge medical costs and increase mortality. If the incidence was known, it would provide context for clinical decision making and aid future research. Therefore, this review aims to aggregate the available literature on the incidence of different SSIs forms in infants who needed surgery for abdominal birth defects.
: The electronic databases Pubmed, EMBASE, and Cochrane library were searched in February 2020. Studies describing infectious complications in infants (under three years of age) were considered eligible. Primary outcome was the incidence of SSIs in infants. SSIs were categorized in wound infection, wound dehiscence, anastomotic leakage, postoperative peritonitis, and fistula development. Secondary outcome was the incidence of different forms of SSIs depending on the type of birth defect. Meta-analysis was performed pooling reported incidences in total and per birth defect separately.
: 154 studies, representing 11,786 patients were included. The overall pooled percentage of wound infections after abdominal birth defect surgery was 6% (95%-CI:0.05–0.07) ranging from 1% (95% CI:0.00–0.05) for choledochal cyst surgery to 10% (95%-CI:0.06–0.15) after gastroschisis surgery. Wound dehiscence occurred in 4% (95%-CI:0.03–0.07) of the infants, ranging from 1% (95%-CI:0.00–0.03) after surgery for duodenal obstruction to 6% (95%-CI:0.04–0.08) after surgery for gastroschisis. Anastomotic leakage had an overall pooled percentage of 3% (95%-CI:0.02–0.05), ranging from 1% (95%-CI:0.00–0.04) after surgery for duodenal obstruction to 14% (95% CI:0.06–0.27) after colon atresia surgery. Postoperative peritonitis and fistula development could not be specified per birth defect and had an overall pooled percentage of 3% (95%-CI:0.01–0.09) and 2% (95%-CI:0.01–0.04).
: This review has systematically shown that SSIs are common after correction for abdominal birth defects and that the distribution of SSI differs between birth defects.
Modified sequential sac ligation and staged closure technique for the management of giant omphalocele
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This study aimed to describe sac ligation and sequential closure for the management of giant omphalocele (GO) and analyze its outcomes.
The medical records of 13 neonates with GO treated at a tertiary general hospital between July 2012 and April 2020 were reviewed. Sac ligation and progressive external compression were performed on most cases immediately after birth. Staged closure with or without a prosthetic patch was conducted after a period of sac suspension.
Sac ligation-traction-compression was performed on 12 cases, of which 10 underwent staged closure, one with delayed closure. One patient with coexistent esophageal atresia was deemed ineligible for surgery. Among those who had undergone staged closure, 9 survived; however, one neonate who complicated with bilateral diaphragmatic eventration and severe ventilator-associated pneumonia died from multiple-organ failure. Pentalogy of Cantrell was excluded. One patient in whom primary closure was performed after birth died aged 29 h. Pneumonia was the most common infection among patients (5/13), with three having ventilator-associated pneumonia. The median durations of mechanical ventilation and hospital stay were 22.2 days (range, 1–151) and 44.2 days (range, 2–152), respectively, and 25.6 days and 46.4 days, respectively, among patients with staged closure. Among five infants who required oxygen support for more than 28 days, four had pulmonary hypoplasia.
Aside from abdominal wall defects, other major comorbidities and pulmonary hypoplasia influence the prognosis of GO. Sac ligation and staged closure is a effective choice for GO.
Type of Study: Retrospective Study
Level of Evidence: Level IV
Systematic review of mortality associated with neonatal primary staged closure of giant omphalocele
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Surgical management of giant omphalocele has evolved at a slow pace, but evidence on the survival of patients who underwent primary staged closure is scattered and atomized.
To analyze the studies about of mortality associated with neonatal primary staged closure of giant omphalocele.
Systematic review in three databases using ex-ante search protocol and selection of studies following the phases suggested by PRISMA and MOOSE criteria. Reproducibility and evaluation of methodological quality were guaranteed by using CARE and STROBE.
Seven studies of clinical cases with nine patients, and six cross-sectional studies with 85 individuals were analyzed. These were conducted in the USA mainly, between 1985 and 2018. In the case studies, the death was 11.1% owing to hepatic necrosis and portal system angiomatosis. On the cross-sectional studies, mortality was registered in 18.8% of patients owing to coarctation of the aorta, heart, kidney, intestinal, respiratory or multiple organ failure, an anomaly of venous return, prematurity, ruptured omphalocele, pulmonary hypoplasia, trisomy 13, ARDS, sepsis, and septic shock. The main complication was wound infection with subsequent confection of the silo, found in 5.4% of patients.
Only a few studies on staged closure of giant omphalocele were found on a low number of patients. The high survival rate and the low percentage of complications on the 94 analyzed patients suggest the effectiveness and safety of the procedure.
According to the Journal of Pediatric Surgery this research corresponds to type of study level II for retrospective studies, and level IV for case series with no comparison group.
Giant omphalocele closure utilizing botulinum toxin
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An omphalocele is a rare congenital abdominal wall defect of the umbilical ring. A giant omphalocele is typically defined as 5 cm or larger. Here we present a case of utilizing botulinum toxin to aid in closure of a giant omphalocele. Lack of abdominal domain prevented visceral reduction and initial treatment involved and alloderm silo and wound vac dressing allowing granulation tissue epithelialization. Patient underwent two rounds of botox injections and fascial closure was obtained. Chemical component separation with botox injection of the abdominal wall musculature is safe and effective for augmenting closure of giant omphalocele.

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: Presented at the 36th Annual Meeting of the Canadian Association of Pediatric Surgeons, Winnipeg, Manitoba, Canada, September 30 October 3, 2004.

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