Predictors of long-term respiratory insufficiency of exomphalos major

Matcovici, Melania; Stoica, Ionica; Burhamah, Waleed; Alshafei, Abdulrahman; Murphy, Rebecca; Sue, Tea; Muntean, Ancuta; Awadalla, Sami

doi:10.1016/j.jpedsurg.2020.12.017

Cited by 7 publications

(5 citation statements)

References 23 publications

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“…Initial search resulted in 1243 studies; after excluding duplicates and non‐eligible studies, 42 studies were deemed suitable and offered 822 patients for analysis 3–45 . Gestational age was reported in 16 studies with a median of 36 (29–39) weeks.…”

Section: Resultsmentioning

confidence: 99%

“…Initial search resulted in 1243 studies; after excluding duplicates and non-eligible studies, 42 studies were deemed suitable and offered 822 patients for analysis. Gestational age was reported in 16 studies with a median of 36 (29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39) weeks. Birth weight was reported in 21 studies with a median of 2.6 (1.3-3.1) kg.…”

Section: Re Sultsmentioning

confidence: 99%

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

Goneidy,

Saxena

2023

Acta Paediatrica

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AimExomphalos 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.MethodsA 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.ResultsInitial 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.ConclusionThis report has recognised the variations in topical substances employed for conservative management with no clear consensus. Reports on safety of different methods remain unclear.

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Section: Resultsmentioning

confidence: 99%

Section: Re Sultsmentioning

confidence: 99%

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

Goneidy,

Saxena

2023

Acta Paediatrica

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“…We found particularly poor outcomes in those patients with EMCHD who needed MV/PMV prior to cardiac intervention. Several explanations for pulmonary insu ciency in patients with ExoMaj have been proposed such as a) impaired diaphragmatic development [27], b) the marked difference in chest con guration in patients with ExoMaj compared to other abdominal wall defects [28], and c) increased abdominal pressure and diaphragmatic elevation following early surgical repair [29]. It has also been suggested that infants with anterior abdominal wall defects may have impaired antenatal lung growth [30].…”

Section: Discussionmentioning

confidence: 99%

Outcomes of patients with exomphalos and associated congenital heart diseases

et al. 2022

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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 in uence 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 , with a focus on those with the combination of exomphalos major and major CHD (EMCHD).Results: There were 123 patients with exomphalos identi ed, 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). 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 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 signi cant nding was high mortality in those with right heart anomalies in combination with pulmonary hypoplasia, especially if pre-intervention mechanical ventilation is required.

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“…About a third of neonates with a defect greater than 5 cm in diameter will need immediate invasive respiratory support, and a substantial proportion of these children require continuing respiratory support for more than 6 months. 49 Some centres advocate an initial conservative approach, allowing the defect to become epithelialised and performing a delayed repair around the age of 1 year. Dressings include silver sulfadiazine, iodine, hypertonic saline, mercury, gentian violet and Manuka honey.…”

Section: Special Considerations: Omphalocelementioning

confidence: 99%

Gastrointestinal congenital anomalies requiring surgery: diagnosis, counselling, and management

Mires,

Raychoudhury,

Overton

et al. 2023

The Obstetric & Gynaecologis

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Congenital anomalies affect more than 2% of fetuses in the UK, with 70% noncardiac in origin. Around 10% are gastrointestinal anomalies such as abdominal wall defects and intestinal atresias. Most gastrointestinal anomalies require postnatal surgical management. Obstetricians must understand the key features of diagnosis and management of common gastrointestinal anomalies. Clinically relevant and evidence-based information helps facilitate parental reproductive autonomy through timely and informed counselling, planning for delivery and optimising perinatal outcomes. Management of pregnancies complicated by congenital gastrointestinal anomalies requires a multidisciplinary approach with specialist input. Learning objectivesTo be familiar with the antenatal diagnosis of gastrointestinal anomalies, associated conditions and potential obstetric complications To understand the antenatal and postnatal management of gastrointestinal anomalies including the approach to surgery To appreciate the role of multidisciplinary counselling in fetal medicine Ethical issuesAntenatal counselling in the context of diagnostic and outcome uncertainties Consideration of termination of pregnancy for fetal anomaly, including grounds for, and timing of, decision-making

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Predictors of long-term respiratory insufficiency of exomphalos major

Cited by 7 publications

References 23 publications

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

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

Outcomes of patients with exomphalos and associated congenital heart diseases

Gastrointestinal congenital anomalies requiring surgery: diagnosis, counselling, and management

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