Survival, hospitalisation and surgery in children born with Pierre Robin sequence: a European population-based cohort study
Pierre Robin sequence is a rare congenital anomaly with a prevalence of 12.0 per 100,000 births. Respiratory disorders and feeding problems represent the main burden of the condition. This EUROlinkCAT study aimed to investigate survival, hospitalisation andsurgical procedures undergone by children born with Pierre Robin sequence during their first 10 years of life. Data on 463 livebirths with Pierre Robin sequence from 12 EUROCAT congenital anomaly registries in 9 European countries were linked to healthcare databases.
Ninety-five percent of children survived to age 10 years and survival improved after the first year of life. In the first year of life nearly all children went into hospital with a median length of stay in hospital of 21 days and 68% of children underwent surgery. Between ages 5-9, 58% of children were hospitalised and the hospital stay decreased to less than half a day per year. In the first 5 years children underwent a median of two surgical procedures. During the first five years of life about one in five of the children had at least one admission to intensive care, one in ten needed mechanical ventilation at least once, and one in twenty needed surgery for a permanent feeding tube.
Efforts must be done to support families throughout these children’s childhood. The health outcome indicators calculated in this study provide useful information to support the healthcare system and the clinical management of children born with Pierre Robin sequence.