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

Santoro, M; Garne, E; Coi, A; Tan, J; Loane, M; Ballardini, E; Cavero-Carbonell, C; de Walle, HE; Gatt, M; Gissler, M; et al. Santoro, M; Garne, E; Coi, A; Tan, J; Loane, M; Ballardini, E; Cavero-Carbonell, C; de Walle, HE; Gatt, M; Gissler, M; Jordan, S; Klungsøyr, K; Lelong, N; Urhoj, SK; Wellesley, DG; Morris, JK (2023) Survival, hospitalisation and surgery in children born with Pierre Robin sequence: a European population-based cohort study. Arch Dis Child, 108 (7). pp. 550-555. ISSN 1468-2044 https://doi.org/10.1136/archdischild-2022-324716
SGUL Authors: Tan, Joachim Wei Li Morris, Joan Katherine

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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.

Item Type: Article
Additional Information: © Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. http://creativecommons.org/licenses/by-nc/4.0/ This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.
Keywords: Child Health, Epidemiology, Mortality, Child Health, Epidemiology, Mortality, 1103 Clinical Sciences, 1114 Paediatrics and Reproductive Medicine, 1117 Public Health and Health Services, Pediatrics
SGUL Research Institute / Research Centre: Academic Structure > Population Health Research Institute (INPH)
Journal or Publication Title: Arch Dis Child
ISSN: 1468-2044
Language: eng
Dates:
DateEvent
19 June 2023Published
9 May 2023Published Online
17 February 2023Accepted
Publisher License: Creative Commons: Attribution-Noncommercial 4.0
Projects:
Project IDFunderFunder ID
733001European Commission Directorate-General for Research and InnovationUNSPECIFIED
PubMed ID: 37160334
Web of Science ID: WOS:000990643300001
Go to PubMed abstract
URI: https://openaccess.sgul.ac.uk/id/eprint/115179
Publisher's version: https://doi.org/10.1136/archdischild-2022-324716

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