Quality of life in children with Robin Sequence.
BACKGROUND
Parents may play an important role during the management of children with Robin Sequence (RS). However, so far only one study has been done on both parent-reported health-related quality of life (HRQoL) and obstructive sleep apnea (OSA) symptoms in children with RS.


METHODS
Overall, 63 children with RS, aged 1 and 18, were included in this cross-sectional study. Fifty-three parents of children with RS with a median age of 8.9 [IQR 5.1-12.7] completed questionnaires on HRQoL (OSA-18) and symptoms of OSA (the Brouillette score) in their child with RS. Ten children between 12 and 18 years filled out the self-reported HRQoL questionnaire OSA-12.


RESULTS
At cross-section, 10 children still had respiratory problems. Overall, parents of children with RS reported a lower HRQoL in their child compared with parents in the general population. Parents of children with RS who still had respiratory problems, i.e. OSA or airway support, reported significantly worse OSA-18 scores compared with parents of RS children without OSA. Children with RS themselves (n = 10) reported less physical suffering and less emotional distress on the OSA-12 compared with children in the norm population. Parental perceptions of OSA-related symptoms were accurate, except for the incidence of snoring. In assessing snoring, the multidimensional OSA-18 sleep domain was more informative.


CONCLUSIONS
Parents of children with RS reported a lower HRQoL in their child compared with parents in the general population. Parental perceptions of health and HRQoL in children with RS might have an additional value to recognize and treat respiratory problems.
Department of Pediatrics, Intensive Care Unit, Erasmus Medical Center, Sophia Children's Hospital, P.O. Box 2060, 3000 CB Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry/Psychology, Erasmus MC - Sophia Children's Hospital, P.O. Box 2060, 3000 CB Rotterdam, The Netherlands. Electronic address: k.dulfer@erasmusmc.nl.
Karolijn
K
Dulfer
Department of Oral and Maxillofacial Surgery, Special Dental Care and Orthodontics, Erasmus MC - Sophia Children's Hospital, P.O. Box 2060, 3000 CB Rotterdam, The Netherlands.
Manouk J S
MJ
van Lieshout
Department of Otorhinolaryngology, Erasmus MC - Sophia Children's Hospital, P.O. Box 2060, 3000 CB Rotterdam, The Netherlands.
Marc P
MP
van der Schroeff
Department of Oral and Maxillofacial Surgery, Special Dental Care and Orthodontics, Erasmus MC - Sophia Children's Hospital, P.O. Box 2060, 3000 CB Rotterdam, The Netherlands.
Maarten J
MJ
Koudstaal
Department of Plastic and Reconstructive Surgery, Erasmus MC - Sophia Children's Hospital, P.O. Box 2060, 3000 CB Rotterdam, The Netherlands.
Irene M J
IM
Mathijssen
Department of Oral and Maxillofacial Surgery, Special Dental Care and Orthodontics, Erasmus MC - Sophia Children's Hospital, P.O. Box 2060, 3000 CB Rotterdam, The Netherlands.
Eppo B
EB
Wolvius
Department of Pediatrics, Intensive Care Unit, Erasmus Medical Center, Sophia Children's Hospital, P.O. Box 2060, 3000 CB Rotterdam, The Netherlands.
Koen F M
KF
Joosten
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D006304:Health Status; D006801:Humans; D015994:Incidence; D008297:Male; D010290:Parents / Q000523:psychology; D010465:Perception; D010855:Pierre Robin Syndrome / Q000150:complications* / Q000523:psychology; D011788:Quality of Life; D020181:Sleep Apnea, Obstructive / Q000175:diagnosis / Q000209:etiology / Q000523:psychology; D012913:Snoring / Q000453:epidemiology; D011795:Surveys and Questionnaires
Child; Infant; Quality of life; Robin Sequence