Choice of topical substances in the conservative management of Exomphalos - A systematic review.

AIM: Exomphalos is a congenital anomaly found in 1/4500 newborns. Choice of non-operative management of exomphalos major unamenable to primary repair is controversial. This study aims at reviewing conservative management modalities and compare outcomes and complications. METHODS: A systematic review was performed according to PRISMA guidelines of all English publications in MEDLINE and EMBASE databases. Search words were exomphalos OR omphalocoele AND conservative OR non-operative AND management. Studies were scrutinised for patient demographics, co-morbidities, mode of treatment, time to full feeds, time to full epithelialisation, length of stay, complications and mortality. Studies not specifically describing mode of management and/or describing primary or staged surgical repairs were excluded. RESULTS: Initial search resulted in 1243 studies. Forty-two studies were deemed suitable offering 822 patients for analysis after excluding duplicates and non-eligible studies. Management methods varied including painting with Alcohol, Mercurochrome, silver products, Povidone Iodine, honey and other materials. Mortality was mostly due to associated anomalies. There was mixed reporting of alcohol, silver, Povidone Iodine and mercury toxicity as well as infection during the course of treatment. CONCLUSION: This report has recognised the variations in topical substances employed for conservative management with no clear consensus. Reports on safety of different methods remain unclear.

Outcomes of patients with exomphalos and associated congenital heart diseases.

INTRODUCTION: Exomphalos is an anterior abdominal wall defect resulting in herniation of contents into the umbilical cord. Severe associated chromosomal anomalies and congenital heart disease (CHD) are known to influence mortality, but it is not clear which cardiac anomalies have the greatest impact on survival. METHODS: We performed a retrospective review of the treatment and outcome of patients with exomphalos over a 30-year period (1990-2020), with a focus on those with the combination of exomphalos major and major CHD (EMCHD). RESULTS: There were 123 patients with exomphalos identified, 59 (48%) had exomphalos major (ExoMaj) (defect > 5 cm or containing liver), and 64 (52%) exomphalos minor (ExoMin). In the ExoMaj group; 17% had major CHD (10/59), M:F 28:31, 29% premature (< 37 weeks, 17/59) and 14% had low birth-weight (< 2.5 kg, 8/59). In the ExoMin group; 9% had major CHD (6/64), M:F 42:22, 18% premature and 10% had low birth-weight. The 5-year survival was 20% in the EMCHD group versus 90% in the ExoMaj with minor or no CHD [p < 0.0001]. Deaths in the EMCHD had mainly right heart anomalies and all of them required mechanical ventilation (MV) for pulmonary hypoplasia prior to cardiac intervention. In contrast, survivors did not require mechanical ventilation prior to cardiac intervention. CONCLUSION: EMCHD is associated with high mortality. The most significant finding was high mortality in those with right heart anomalies in combination with pulmonary hypoplasia, especially if pre-intervention mechanical ventilation is required.

A case series of successfully managing exomphalos major with awake graduated compression dressing and early enteral feeding.

INTRODUCTION: Exomphalos anomaly is defined as the herniation of abdominal viscera into the base of the umbilical cord, with only a membranous sac covering these contents. It has an incidence of approximately 1 in 4000-6000 births. Management of exomphalos major (EM) remains controversial and limited, with very few studies to guide decision-making. METHOD: This is a case series of four neonates with EM treated at a tertiary paediatric referral centre between 2018 and 2021 with a gradual compression dressing technique. RESULTS: Four neonates were diagnosed with EM. The average gestational age was 38 + 5 (range 38 + 2 - 39 + 2), and the average birth weight was 3.1 kg (range 2.56 - 3.49 kg). The defect size ranged between 5 and 7 cm. All patients were commenced on gradual compression dressing between days 1 and 3 of life. Dressings were applied at the bedside in the general neonatal ward. The average time taken to reach full feeds was 1 week; only one patient required parenteral nutrition. Three underwent surgical repair at two and 16 weeks of age; one had delayed repair at the age of 1 year because of the COVID-19 pandemic. None required patch repair. None required prolonged ventilation after repair. CONCLUSION: This case series describes a successful compression dressing technique that reduces sac content without the need for general anaesthetic or respiratory compromise, whereby simultaneous enteral feeding is tolerated.

Exomphalos major: Conservative management using Manuka honey dressings and an outreach surgical nursing team.

INTRODUCTION: Controversy exists over the best dressing for conservative management of exomphalos major. Here we describe our experience of using Manuka Honey. METHODS: Our regimen involved covering the sac with Manuka honey (Advancis Medical™) wrapped with gauze and crepe bandage. Initially, dressings were changed 3 times a week and then twice weekly until full epithelialisation. Babies went home after reaching full feeds, with our outreach nurses continuing dressings in clinic until the parents were trained to do them alone. Only patients needing management of co-morbidities were transferred to our unit. Patients would be reviewed by video consultation. Data was prospectively collected. RESULTS: From 2011-2019, 24 consecutive patients (11:13 M:F; median gestation 37 weeks, birth weight 3.1 kg) with exomphalos major were managed with honey dressings. Fourteen babies had significant associated anomalies of which 10 died of problems unrelated to the exomphalos. Time to full feeds 6 (2-58) days; time to discharge 21(7-66) days if no associated anomalies; time to epithelialisation 73 (27-199) days. Dressings were well tolerated. Definitive closure occurred at 17(11-38) months and was uneventful. No patient required fundoplication and all patients were orally fed. Only one patient developed a clinically significant infection. CONCLUSION: This is the largest report of using Manuka honey for the management of exomphalos major. Benefits include early feeding, early discharge and a 'normalisation' of the neonatal period. Key to our success was the surgical outreach service supporting parents doing the dressings, first at the local hospital and then at home.

Initial conservative management of exomphalos major with gentian violet.

AIM: The purpose of the study was to assess the results of topical use of gentian violet (GV), among the babies with exomphalos major in our institute. METHODS: The study was carried out retrospectively in a tertiary care hospital during the period from 2005 to 2010 inclusive. Exomphalos patients were classified as major if diameter was >5 cm and/or had liver in the sac as content. These patients were initially preferentially treated conservatively with topical 1% GV over the sac resultig a ventral hernia to be repaired later. RESULTS: A total of 84 exomphalos patients were admitted during the study period. Among them, 37 neonates (26 males and 11 females) had exomphalos major (EM). Ten of them were prenatally diagnosed. The mean gestational age at delivery was 35 weeks, and mean birth weight was 2.1 Kg. Mean age at presentation was 3.7 days. Thirty (81%) had other associated anomalies, mostly cardiac (66.6%) and pulmonary (46.6%). Ten patients with EM needed early operation because of ruptured sac, and other anomalies. There were 2 pre-operative and 8 postoperative deaths in this subgroup. Twenty seven patients were treated conservatively, among these 4 died of overwhelming sepsis. Remaining 23 patients left the hospital with a ventral hernia planned to be repaired at 1 year of age. Overall mortality in our series was 37.83%. CONCLUSION: Initial conservative treatment of the sac with GV results in satisfactory outcome for infants with EM who cannot undergo immediate closure.

Is progressive pneumoperitoneum useful in delayed repair of large omphaloceles?

Repair of exomphalos major in the neonatal period is fraught with risks and complications. Progressive pneumoperitoneum was found to be safe and cost-effective in six patients with exomphalos major who underwent repair at an older age. The technique is particularly suitable for hospitals that do not have facilities for intensive care, ventilation and total parenteral nutrition.