Trends, correlates, and survival of infants with congenital diaphragmatic hernia and its subtypes

Ramakrishnan, Rema; Salemi, Jason L.; Stuart, Amy L.; Chen, Henian; O’Rourke, Kathleen; Običan, Sarah; Kirby, Russell S.

doi:10.1002/bdr2.1357

Cited by 20 publications

(25 citation statements)

References 29 publications

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“…Our five‐year survival rates for specific isolated anomalies were also similar to contemporary studies assessing infants with cardiac anomalies born in Taiwan and for infants with cardiac, orofacial clefts and Down syndrome born in Western Australia . While others have found higher five‐year survival rates for infants with isolated diaphragmatic hernia from Florida, USA (80%) and with Down syndrome from Norway (96%) .…”

Section: Discussionsupporting

confidence: 82%

“…Other recent studies have explored survival of infants with CA, separating those with anomalies in isolation to those with multiple anomalies but have been limited to groups or specific CA such as cardiac , orofacial clefts , diaphragmatic hernia or Down syndrome . Additionally, given that gestational age is a key determinant of survival of infants diagnosed with CA or without CA, it is important to take into account its effect across different CA groups.…”

Section: Introductionmentioning

confidence: 99%

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Five‐year survival of infants with major congenital anomalies: a registry based study

et al. 2019

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Aim: To determine survival of infants with major congenital anomalies (CA) and assess the effect of co-existing anomalies and gestational age.Methods: All liveborn infants with major CA born in New South Wales (NSW), Australia, 2004Australia, -2009 were identified from the NSW Register of Congenital Conditions. Deaths were identified via record linkage to death registrations and five-year survival was estimated using Kaplan-Meier methods.Results: There were 8521 liveborn infants with CA of whom 617 (7.2%) died within the first five years of life. Half of deaths occurred in the first week of life. The overall five-year survival rate was 92.8% (95%CI: 92.2-93.3) and 83.2% (95%CI: 79.0-87.4) for syndromes, 83.4% (95%CI: 80.9-85.9) for multiple, 85.1% (95%CI: 82.6-87.5) for chromosomal, 95.3% (95%CI: 94.8-95.8) for isolated and 96.2% (95%CI: 94.3-98.1) for non-Q chapter anomalies. Five-year survival for chromosomal, syndromes and subgroups was higher for isolated compared with multiple anomalies ranging from 77.5% to 98.9% and 68.6% to 89.5%, respectively. Survival was lower for preterm (79.4%; 95%CI: 77.5-81.4) than for term infants (95.8%; 95%CI: 95.3-96.3). Conclusion:Nine in ten infants with major CA survive up to five years, although there is variability in survival across CA groups. Survival of infants with major congenital anomalies has improved in recent years.Abbreviations CA, Congenital anomalies; CI, Confidence interval; EUROCAT, European surveillance of congenital anomalies; NSW, New South Wales; RoCC, Register of congenital conditions. Key notesThis study suggests that nine in ten infants with major congenital anomalies survive past five years of age.Five-year survival ranged from 83% for infants with syndromes to more than 95% for infants with isolated and non-Q chapter anomalies (e.g. congenital hypothyroidism, cystic fibrosis) Five-year survival was higher for children with isolated than those with multiple anomalies, while survival rates were lower for preterm than term infants.2008

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Section: Discussionsupporting

confidence: 82%

Section: Introductionmentioning

confidence: 99%

Five‐year survival of infants with major congenital anomalies: a registry based study

et al. 2019

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“…The advance of neonatal intensive care, including the gentle ventilation strategy with high frequently oscillation ventilator (HFOV) and the advent of ECMO, has improved the survival rate of patients with CDH, especially those who are in severe conditions [4][5][6]. At the same time, increasing number of children who have survived arduous neonatal period is giving rise to new issues related to long-time morbidity and transition of follow-up care [7][8][9].…”

Section: Discussionmentioning

confidence: 99%

Long-term feeding issue and its impact on the daily life of congenital diaphragmatic hernia survivors: results of the first patient-led survey

Power¹,

Shibuya

Lane³

et al. 2019

Pediatr Surg Int

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Background CDH UK is a registered charity governed by a volunteer committee and providing informal support to patients, families and healthcare workers affected directly or indirectly with congenital diaphragmatic hernia (CDH) internationally. This is the first patient-led survey undertaken by CDH UK aiming for highlighting the feeding problems and their impact on the daily life of CDH survivors. Methods Answers from CDH survivors were collected through an online questionnaire (SurveyMonkey ® ) undertaken by CDH UK. The questionnaire contained questions about their feeding problems and support they were receiving for it. Main results Overall, 151 patients answered some parts of the survey and 102 patients completed the questionnaire. Overall, 116 (76.8%) responders reported suffering from any type of feeding issue. Gastric acid reflux (GER) and growth retardation were the commonest symptoms experienced by 97 (91.5%) and 72 (62.2%) responders, respectively. Only 18 (17.0%) responders have received any written information on feeding or details of patient/parent support. Eighty (75.5%) responders are satisfied with the level of support they are receiving, but 78 (76.4%) answered that the whole experience associated with the disease has been very or extremely stressful. Conclusions CDH survivors frequently have various issues with feeding, which may not be adequately supported or discussed clinically. It is desirable to assist the patients to reliable resources of long-term support, including multidisciplinary team (MDT) approach.Publisher's Note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

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“…Congenital diaphragmatic hernia (CDH), characterized by a range of developmental defects in the thoracic diaphragm, the muscular tendinous partition between the thorax and abdomen, affects 1 in 3,000 pregnancies. [1][2][3][4] Bochdalek, Morgagni, anterior, and central defects are recognized anatomical variants with distinct embryological origins. Alternatively, CDH can be classi-fied as being "isolated" or "complex" associated with other structural anomalies, chromosomal aberrations or syndromes.…”

Section: Introductionmentioning

confidence: 99%

Does the Presence of a Hernia Sac Improve Survival in Newborns with Congenital Diaphragmatic Hernia? A United Kingdom Single-Center Experience

2020

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Introduction A developing body of literature suggests that the presence of a hernia sac in fetuses with congenital diaphragmatic hernia (CDH) may indicate improved prognosis. By examining a large cohort of CDH newborns admitted to a single United Kingdom specialist center, we aimed to establish if presence of hernia sac is a robust predictor of improved survival. Materials and Methods All CDH patients admitted to a single center were recruited. Postneonatal presentations and Morgagni hernias were excluded. Demographics, defect type, laterality, survival, and hernia recurrence were recorded. Results In this study, 192 CDH newborns were managed from 1997 to 2017; 39 were excluded (10 Morgagni and 29 postneonatal); 22 (14%) neonates had a hernia sac. Survival in patients with a hernia sac was 21/22 (95%) versus 107/124 (86%) in cases without hernia sac (p = 0.2). There was no difference in hernia sac proportion by gender (male:female 15 vs. 13.2%, p = 0.8). Conclusion In contrast to studies showing a survival advantage, albeit with smaller patient numbers, we report a statistical nonsignificant benefit of hernia sac. Better survival outcomes at this specialist center with CDH patients without a hernia sac than reported in other published studies are likely responsible for the lack of statistical significance observed, despite a larger cohort. National and international CDH registries yielding “big data” may provide further answers on the utility of a CDH hernia sac as a new prognostic scoring tool.

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Trends, correlates, and survival of infants with congenital diaphragmatic hernia and its subtypes

Cited by 20 publications

References 29 publications

Five‐year survival of infants with major congenital anomalies: a registry based study

Five‐year survival of infants with major congenital anomalies: a registry based study

Long-term feeding issue and its impact on the daily life of congenital diaphragmatic hernia survivors: results of the first patient-led survey

Does the Presence of a Hernia Sac Improve Survival in Newborns with Congenital Diaphragmatic Hernia? A United Kingdom Single-Center Experience

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