Meta-Analysis

. 2023 Jul;108(7):550-555.

doi: 10.1136/archdischild-2022-324716. Epub 2023 May 9.

Survival, hospitalisation and surgery in children born with Pierre Robin sequence: a European population-based cohort study

Michele Santoro¹, Ester Garne², Alessio Coi³, Joachim Tan⁴, Maria Loane⁵, Elisa Ballardini⁶, Clara Cavero-Carbonell⁷, Hermien Ek de Walle⁸, Miriam Gatt⁹, Mika Gissler^{10

11}, Sue Jordan¹², Kari Klungsøyr^{13

14}, Natalie Lelong¹⁵, Stine Kjaer Urhoj², Diana G Wellesley¹⁶, Joan K Morris⁴

Affiliations

¹ Unit of Epidemiology of rare Diseases and Congenital Anomalies, Institute of Clinical Physiology, National Research Council, Pisa, Italy msantoro@ifc.cnr.it.
² Department of Paediatrics and Adolescent Medicine, Lillebaelt Hospital, University Hospital of Southern Denmark, Kolding, Denmark.
³ Unit of Epidemiology of rare Diseases and Congenital Anomalies, Institute of Clinical Physiology, National Research Council, Pisa, Italy.
⁴ Population Health Research Institute, St George's, University of London, London, UK.
⁵ Faculty of Life and Health Sciences, Ulster University, Newtowanbbey, UK.
⁶ Neonatal Intensive Care Unit, Paediatric Section, IMER Registry (Emilia Romagna Registry of Birth Defects), Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
⁷ Rare Diseases Research Unit, Foundation for the Promotion of Health and Biomedical Research in the Valencian Region FISABIO, Valencia, Spain.
⁸ Genetics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
⁹ Directorate for Health Information and Research, Malta Congenital Anomalies Registry, G'mangia, Malta.
¹⁰ Department of Knowledge Brokers, THL Finnish Institute for Health and Welfare, Helsinki, Finland.
¹¹ Region Stockholm, Academic Primary Health Care Centre, Stockholm, Sweden.
¹² Faculty of Medicine, Health and Life Science, Swansea University, Swansea, UK.
¹³ Department of Global Public Health and Primary Care, University of Bergen, Bergen, Norway.
¹⁴ Division of Mental and Physical Health, Norwegian Institute of Public Health, Bergen, Norway.
¹⁵ Université de Paris, CRESS-Epopé, INSERM, INRA, Paris, France.
¹⁶ Faculty of Medicine, University of Southampton and Wessex Clinical Genetics Service, Princess Anne Hospital, Southampton, UK.

PMID: 37160334
PMCID: PMC10314079
DOI: 10.1136/archdischild-2022-324716

Meta-Analysis

Survival, hospitalisation and surgery in children born with Pierre Robin sequence: a European population-based cohort study

Michele Santoro et al. Arch Dis Child. 2023 Jul.

. 2023 Jul;108(7):550-555.

doi: 10.1136/archdischild-2022-324716. Epub 2023 May 9.

Authors

Affiliations

¹ Unit of Epidemiology of rare Diseases and Congenital Anomalies, Institute of Clinical Physiology, National Research Council, Pisa, Italy msantoro@ifc.cnr.it.
² Department of Paediatrics and Adolescent Medicine, Lillebaelt Hospital, University Hospital of Southern Denmark, Kolding, Denmark.
³ Unit of Epidemiology of rare Diseases and Congenital Anomalies, Institute of Clinical Physiology, National Research Council, Pisa, Italy.
⁴ Population Health Research Institute, St George's, University of London, London, UK.
⁵ Faculty of Life and Health Sciences, Ulster University, Newtowanbbey, UK.
⁶ Neonatal Intensive Care Unit, Paediatric Section, IMER Registry (Emilia Romagna Registry of Birth Defects), Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
⁷ Rare Diseases Research Unit, Foundation for the Promotion of Health and Biomedical Research in the Valencian Region FISABIO, Valencia, Spain.
⁸ Genetics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
⁹ Directorate for Health Information and Research, Malta Congenital Anomalies Registry, G'mangia, Malta.
¹⁰ Department of Knowledge Brokers, THL Finnish Institute for Health and Welfare, Helsinki, Finland.
¹¹ Region Stockholm, Academic Primary Health Care Centre, Stockholm, Sweden.
¹² Faculty of Medicine, Health and Life Science, Swansea University, Swansea, UK.
¹³ Department of Global Public Health and Primary Care, University of Bergen, Bergen, Norway.
¹⁴ Division of Mental and Physical Health, Norwegian Institute of Public Health, Bergen, Norway.
¹⁵ Université de Paris, CRESS-Epopé, INSERM, INRA, Paris, France.
¹⁶ Faculty of Medicine, University of Southampton and Wessex Clinical Genetics Service, Princess Anne Hospital, Southampton, UK.

PMID: 37160334
PMCID: PMC10314079
DOI: 10.1136/archdischild-2022-324716

Abstract

Objective: To evaluate survival, hospitalisations and surgical procedures for children born with Pierre Robin sequence (PRS) across Europe.

Design: Multicentre population-based cohort study.

Setting: Data on 463 live births with PRS from a population of 4 984 793 from 12 EUROCAT congenital anomaly registries.

Methods: Data on children with PRS born 1995-2014 were linked electronically to data on mortality, hospitalisations and surgical procedures up to 10 years of age. Each registry applied a common data model to standardise the linked data and ran common syntax scripts to produce aggregate tables. Results from each registry were pooled using random-effect meta-analyses.

Main outcome measures: Probability of survival, proportion of children hospitalised and undergoing surgery, and median length of hospital stay.

Results: The majority of deaths occurred in the first year of life with a survival rate of 96.0% (95% CI 93.5% to 98.5%); 95.1% (95% CI 92.7% to 97.7%) survived to age 10. In the first year of life, 99.2% (95% CI 95.0% to 99.9%) of children were hospitalised with a median stay of 21.4 days (95% CI 15.6 to 27.2), and 67.6% (95% CI 46.6% to 81.8%) underwent surgery. In the first 5 years of life, 99.2% of children underwent a median of two surgical procedures. Between ages 5 and 9, 58.3% (95% CI 44.7% to 69.7%) were hospitalised with a median annual stay of 0.3 days.

Conclusions: Children with PRS had high mortality and morbidity with long hospital stays in the first year of life, and almost all had surgery before 5 years of age. Survival improved after infancy with fewer hospitalisations after age 5. This study provides reliable estimates of the survival and morbidity of children with PRS for families and healthcare providers.

Keywords: Child Health; Epidemiology; Mortality.

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Conflict of interest statement

Competing interests: None declared.

Figures

**Figure 1**
Percentage hospitalised for children with PRS, children with any CA and children without a CA (‘reference’), by age, 1995–2014. CA, congenital anomaly; PRS, Pierre Robin sequence.

See this image and copyright information in PMC

References

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Survival, hospitalisation and surgery in children born with Pierre Robin sequence: a European population-based cohort study

Affiliations

Survival, hospitalisation and surgery in children born with Pierre Robin sequence: a European population-based cohort study

Authors

Affiliations

Abstract

Conflict of interest statement

Figures

References

Publication types

MeSH terms

LinkOut - more resources

Full Text Sources

Miscellaneous