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Published on: August 7, 2017
Predictors of long-term respiratory insufficiency of exomphalos major
Melania Matcovici1, Ionica Stoica1, Waleed Burhamah1
1Department of Paediatric Surgery, Temple Street Children University Hospital, Dublin, Ireland.
Insights
Exomphalos major is linked to significant health issues, especially long-term respiratory problems. Pulmonary hypertension and resuscitation at birth are key predictors of persistent respiratory insufficiency in these infants.
Area of Science:
- Pediatric Surgery
- Neonatology
- Pulmonology
Background:
- Exomphalos major (EM) is a congenital anomaly associated with considerable morbidity and mortality.
- A significant risk of long-term pulmonary disease exists in infants with EM.
Purpose of the Study:
- To evaluate the outcomes of exomphalos in a tertiary pediatric center.
- To identify prognostic factors for respiratory insufficiency requiring prolonged ventilatory support at six months.
Main Methods:
- Retrospective review of infants with exomphalos from 2005-2015.
- EM defined by defect size (>= 5 cm) and/or liver content.
- Analysis of demographic, prenatal, birth, and outcome data, focusing on respiratory insufficiency.
Main Results:
- 30 of 46 infants (65%) had EM; 35% experienced respiratory complications.
- 8 infants (50%) required long-term ventilation (>= 6 months), 5 needed tracheostomy.
- Pulmonary hypertension and resuscitation at birth were strong predictors of ventilation support at six months.
Conclusions:
- Pulmonary hypertension and resuscitation at birth are critical prognostic factors for long-term respiratory insufficiency in EM.
- Identifying these factors aids in improved parental counseling regarding respiratory outcomes.
Introduction:
Exomphalos major (EM) is associated with significant morbidity and even mortality with an important risk of long-term pulmonary disease.
Aim:
To assess the outcomes of exomphalos in a single tertiary pediatric unit and to identify prognostic factors for patients with respiratory insufficiency who still require ventilatory assistance at six months.
Material And Methods:
All infants admitted to our institution over a 10-year period (2005 to 2015) with exomphalos were retrospectively reviewed. EM was defined when the abdominal wall defect measured >= 5 cm and/or contained liver within the sac. Data were collected on patient demographics, prenatal course and imaging, birth information, immediate and long-term outcomes. Those with long-term respiratory insufficiency were identified as the primary outcome and reviewed to assess prognostic factors. A p value of ≤0.05 was regarded as significant. Data are quoted as median(range).
Results:
A total of 46 infants were diagnosed with exomphalos during the study period, with most (n = 30, 65%) defined as exomphalos major. Respiratory complications occurred in 16 (35%) with 8 (50%) of these requiring long-term (≥6 months) mechanical ventilation and 5 (31%) required a tracheostomy. On univariate analysis, resuscitation at birth (p = 0.0004), birth weight <3000 g (p = 0.008), use of nitric oxide (p = 0.004), high frequency oscillatory ventilation (HFOV) (p = 0.001), pulmonary hypoplasia (p<0.0001) and pulmonary hypertension (PHTN) (p = 0.02) were significantly associated with respiratory insufficiency. The strongest predictive model for ventilation support at six months was resuscitation at birth in combination with PH (OR = 1.57). Five infants (11%) died at 5(1-122) days.
Conclusions:
In patients with EM, the presence of pulmonary hypertension along with resuscitation at birth are the most important prognostic factors for long-term respiratory insufficiency. Acknowledgement of these factors allows for better parental counselling regarding respiratory outcomes.
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