Outcomes of exomphalos: an institutional experience

Vachharajani, Akshaya; Rao, Rakesh; Keswani, Sundeep G.; Mathur, Amit M.

doi:10.1007/s00383-008-2301-y

Cited by 37 publications

(29 citation statements)

References 9 publications

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“…In infants with giant omphalocele, respiratory failure has been shown to be the major cause of mortality [5,17]. These infants have been shown to have a statistically longer length of stay and number of days on mechanical ventilation [6]. A higher incidence of respiratory failure, prolonged ventilatory support, abnormal pulmonary function testing in survivors, and need for tracheostomy have been described in many reports [5,9,14,23,24].…”

Section: Discussionmentioning

confidence: 99%

“…Poor long-term outcomes for infants with omphalocele are more likely in the presence of associated structural or chromosomal abnormalities that occur in approximately 50-77% of these infants [2,3,4]. Other factors associated with poor outcomes include early respiratory distress [5], presence of liver within the omphalocele sac [6], high in utero omphalocele diameter to abdominal circumference ratio [7], low birth weight, low gestational age, and antenatal rupture of the sac [8]. …”

Section: Introductionmentioning

confidence: 99%

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High Prevalence of Pulmonary Hypertension Complicates the Care of Infants with Omphalocele

Hutson

Baerg²,

Deming³

et al. 2017

Neonatology

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Background: Omphalocele is one of the most common abdominal wall defects. Many newborn infants born with omphalocele present with significant respiratory distress at birth, requiring mechanical ventilatory support, and have clinical evidence of pulmonary hypertension. Little information exists on the prevalence of and risk factors associated with pulmonary hypertension in this cohort of infants. Objectives: To describe the prevalence of and risk factors associated with pulmonary hypertension among infants with omphalocele. Methods: This is a multicenter retrospective chart review of demographic data and clinical characteristics of infants with omphalocele admitted to the neonatal intensive care units of Loma Linda University Children's Hospital and Children's Mercy Hospital between 1994 and 2011. Echocardiogram images were reviewed for pulmonary hypertension, and statistical analyses were performed to identify risk factors associated with the presence of pulmonary hypertension. Results: Pulmonary hypertension was diagnosed in 32/56 (57%) infants with omphalocele. Compared to infants without pulmonary hypertension, infants with pulmonary hypertension were more likely to have a liver-containing defect (16/32 [50%] vs. 5/24 [21%], p = 0.03), require intubation at birth (18/32 [56%] vs. 6/24 [17%], p = 0.03), and die during initial hospitalization (12/32 [38%] vs. 2/24 [8%], p = 0.01). Conclusion: The majority of infants with omphalocele have evidence of pulmonary hypertension which is associated with increased mortality. Echocardiograms to screen for pulmonary hypertension should be obtained at ≥2 days of life in infants with omphalocele, especially in those with liver within the omphalocele sac and/or in those infants who require intubation at birth to screen for pulmonary hypertension.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

High Prevalence of Pulmonary Hypertension Complicates the Care of Infants with Omphalocele

Hutson

Baerg²,

Deming³

et al. 2017

Neonatology

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“…Some studies have associated giant (defined as the presence of liver herniation) or ruptured omphalocele with respiratory insufficiency due to pulmonary hypoplasia [11,21], and recently, a decreased lung/thorax ratio and an increased chest/trunk length ratio were also associated with pulmonary hypoplasia and need for prolonged mechanical ventilation in fetuses with giant omphalocele [22]. In fact, 3-dimensional ultrasound has shown that lung volume in fetuses with abdominal wall defects is smaller compared to normal fetuses [23].…”

Section: Discussionmentioning

confidence: 99%

Should We Measure Fetal Omphalocele Diameter for Prediction of Perinatal Outcome?

Kiyohara

Brizot²,

Liao³

et al. 2013

Fetal Diagn Ther

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Objective: To evaluate the fetal omphalocele diameter/abdominal circumference ratio (OD/AC) as a predictor of adverse perinatal outcome. Methods: Analysis involving 47 singleton pregnancies with fetal omphalocele, normal karyotype and absence of other major abnormalities. The OD/AC ratio was determined antenatally by ultrasound and the best cutoff for the prediction of neonatal death was determined by receiver operating characteristic curve analysis. Additional secondary outcomes included need for oral intubation in the first 24 h of life, two-step surgery or use of synthetic mesh, reoperation, parenteral feeding and need for respiratory assistance >21 days, time to first oral feed, and time to hospital discharge. Results: Fetal OD/AC did not change significantly with gestational age. Postnatal death occurred in 10 (21.3%) cases and the best cutoff for prediction was an OD/AC ratio ≥0.26. In pregnancies with the first ultrasound evaluation performed before 31 weeks' gestation and an OD/AC ≥0.26, the likelihood ratio for needing intubation in the first 24 h of life was 2.6 (95% CI: 1.2-5.7), needing two-step surgery or use of mesh was 4.9 (95% CI: 1.9-14.4), and postnatal death was 4 (95% CI: 1.9-7.5). Conclusion: A fetal ultrasound OD/AC ratio ≥0.26 is associated with increased postnatal morbidity and mortality.

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“…In a 10-year retrospective study of outcome of exomphalos in 52 infants (24 minor, 27 major), Vachharajani et al found a mortality rate of 33% in the major group and 8% in the minor group. 9 Exomphalos has chromosomal and syndromic associations, which may impact on the total LOS. The median LOS in the study by Vachharajani et al was 10 days for minor and 47 days for major exomphalos.…”

Section: Exomphalosmentioning

confidence: 99%

Length of stay and cost analysis of neonates undergoing surgery at a tertiary neonatal unit in England

Shetty

Kennea

Desai

et al. 2016

annals

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INTRODUCTION There is a lack of knowledge on the average length of stay (LOS) in neonatal units after surgical repair of common congenital anomalies. There are few if any publications reporting the activity performed by units undertaking neonatal surgery. Such activity is important for contracting arrangements, commissioning specialist services and counselling parents. The aim of this study was to describe postnatal LOS for infants admitted to a single tertiary referral neonatal unit with congenital malformations requiring surgery. METHODS Data on nine conditions were collected prospectively for babies on the neonatal unit over a five-year period (2006)(2007)(2008)(2009)(2010)(2011). For those transferred back to their local unit following surgery, the local unit was contacted to determine the total LOS. Only those babies who had surgery during their first admission to our unit and who survived to discharge were included in the study. Cost estimates were based on the tariffs agreed for neonatal care between our trust and the London specialised commissioning group in 2011-2012. RESULTS The median LOS for the conditions studied was: gastroschisis 35 days (range: 19-154 days), oesophageal atresia 33 days (range: 9-133 days), congenital diaphragmatic hernia 28 days (range: 7-99 days), intestinal atresia 24 days (range: 6-168 days), Hirschsprung's disease 21 days (range: 15-36 days), sacrococcygeal teratoma 17 days (range: 12-55 days), myelomeningocoele 15.5 days (range: 8-24 days), anorectal malformation 15 days (range: 6-90 days) and exomphalos 12 days (range: 3-228 days). The total neonatal bed day costs for the median LOS ranged from £8,701 (myelomeningocoele) to £23,874 (gastroschisis). The cost of surgery was not included. CONCLUSIONS There is wide variation in LOS for the same conditions in a single neonatal unit. This can be explained by different types and severity within the same congenital anomalies, different surgeons and other clinical confounders (eg sepsis, surgical complications, associated anomalies). These data will enable us to give more detailed information to families following prenatal or postnatal diagnosis. They also allow more detailed planning of resource allocation for neonatal admissions.

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Outcomes of exomphalos: an institutional experience

Abstract: Neonates with exomphalos minor have better survival, decreased LOS, time to full enteral feeds and shorter duration of mechanical ventilation. Syndromic associations were more common in exomphalos minor. Respiratory failure was the major cause of mortality in infants with exomphalos major.

Cited by 37 publications

References 9 publications

High Prevalence of Pulmonary Hypertension Complicates the Care of Infants with Omphalocele

High Prevalence of Pulmonary Hypertension Complicates the Care of Infants with Omphalocele

Should We Measure Fetal Omphalocele Diameter for Prediction of Perinatal Outcome?

Length of stay and cost analysis of neonates undergoing surgery at a tertiary neonatal unit in England

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