Omphalocele and alveolar capillary dysplasia: A new association

Gerrits, Luella C.; Mol, Arjen; Bulten, Johan; Staak, F H van der; Heijst, Arno van

doi:10.1097/pcc.0b013e3181b80a75

Cited by 14 publications

(13 citation statements)

References 12 publications

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“…Among survivors, median length of hospitalization was shorter for infants with omphalocele than for those with gastroschisis (17 days [9, 36] vs. 33 days [24, 52], p<0.001). Infants with omphalocele who survived to hospital discharge were started on their first feeds at an earlier postnatal age (median 6 days [3, 10] vs. 15 days [11, 21], p<0.001) and had a shorter duration of mechanical ventilation (median 2 days [0, 5] vs. 4 days [2, 8], p<0.001) compared with infants with gastroschisis.…”

Section: Resultsmentioning

confidence: 99%

Frequency of anomalies and hospital outcomes in infants with gastroschisis and omphalocele

Corey

Hornik

Laughon

et al. 2014

Early Human Development

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Background Gastroschisis and omphalocele are the most common anterior abdominal wall defects affecting infants. There are few large cohort studies describing the frequency of associated anomalies in infants with these 2 conditions. We describe associated anomalies and outcomes in infants with these defects using a large, multi-center clinical database. Methods We identified all infants with gastroschisis or omphalocele from a prospectively collected database of infants discharged from 348 neonatal intensive care units in North America from 1997–2012. Maternal and patient demographic data, associated anomalies, and outcome data were compared between infants with gastroschisis and omphalocele. Results A total of 4687 infants with gastroschisis and 1448 infants with omphalocele were identified. Infants with omphalocele were more likely to be diagnosed with at least 1 other anomaly compared with infants with gastroschisis (35% vs. 8%, p<0.001). Infants with omphalocele were more likely to develop pulmonary hypertension compared with those with gastroschisis (odds ratio [OR] 7.78; 95% confidence interval 5.81, 10.41) and had higher overall mortality (OR 6.81 [5.33, 8.71]). Conclusion Infants with omphalocele were more likely to have other anomalies, be diagnosed with pulmonary hypertension, and have higher mortality than infants with gastroschisis.

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Section: Resultsmentioning

confidence: 99%

Frequency of anomalies and hospital outcomes in infants with gastroschisis and omphalocele

Corey

Hornik

Laughon

et al. 2014

Early Human Development

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“…Described therapies have included the use of sildenafil, inhaled nitric oxide, and hyperventilation [14,16,17,31]. In a severe case of PH associated with alveolar capillary dysplasia and omphalocele, the use of extracorporeal membrane oxygenation was attempted but was unsuccessful [32]. We have successfully used inhaled nitric oxide, sildenafil, and bosentan to treat PH in these infants.…”

Section: Discussionmentioning

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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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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“…More than 90% of affected infants are born at term (17,21), and in greater than 60% of cases, onset of cyanosis and respiratory failure occurs within 48 hours of birth (9,23). In a recent review of 21 cases of ACD/MPV from England, Melly and colleagues (24) proposed that the severity of disruption in capillary density and lack of contact with the alveolar epithelium may be two major factors that determine clinical course and prognosis, and they ascribed the potential for survival beyond the neonatal period in a minority of patients to higher capillary density and apposition.…”

Section: Epidemiologymentioning

confidence: 99%

Alveolar Capillary Dysplasia

Bishop

Stankiewicz

Steinhorn

2011

Am J Respir Crit Care Med

206

220

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Omphalocele and alveolar capillary dysplasia: A new association

Abstract: This is the first description of an association between omphalocele and alveolar capillary dysplasia. In newborns with omphalocele who have severe respiratory insufficiency and pulmonary hypertension, alveolar capillary dysplasia should be considered.

Cited by 14 publications

References 12 publications

Frequency of anomalies and hospital outcomes in infants with gastroschisis and omphalocele

Frequency of anomalies and hospital outcomes in infants with gastroschisis and omphalocele

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

Alveolar Capillary Dysplasia

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