Managing infants with craniofacial malformations – Where to go next?

Published:September 16, 2021DOI:https://doi.org/10.1016/j.siny.2021.101289

      Abstract

      Treatment of infants with craniofacial malformations, e.g. Robin sequence, is characterized by considerable heterogeneity and a lack of randomized trials to identify an optimal approach. We propose to establish an international register using a common minimal dataset that will better allow for a comparison between key determinants and outcomes in these patients. In infants, this should include an assessment of mandibular micrognathia, glossoptosis, upper airway obstruction, weight gain and mode of feeding. Later on, neurocognition, speech development, hearing and quality of life should also be included. Together, these data will help better to advice parents on which treatment to choose for their baby with a craniofacial malformation.
      To read this article in full you will need to make a payment

      Subscribe:

      Subscribe to Seminars in Fetal and Neonatal Medicine
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Buchenau W.
        • Urschitz M.S.
        • Sautermeister J.
        • et al.
        A randomized clinical trial of a new orthodontic appliance to improve upper airway obstruction in infants with Pierre Robin sequence.
        J Pediatr. 2007; 151: 145-149
        • Breugem C.C.
        • Evans K.N.
        • Poets C.F.
        • et al.
        Best practices for the diagnosis and evaluation of infants with Robin sequence: a clinical consensus report.
        JAMA Pediatr. 2016; 170: 894-902
        • Wiechers C.
        • Buchenau W.
        • Arand J.
        • et al.
        Mandibular growth in infants with Robin sequence treated with the Tubingen palatal plate.
        Head Face Med. 2019; 15: 17
        • Manica D.
        • Schweiger C.
        • Sekine L.
        • et al.
        Association of polysomnographic parameters with clinical symptoms severity grading in Robin sequence patients: a cohort nested cross-sectional study.
        Sleep Med. 2018; 43: 96-99
        • Schweiger C.
        • Manica D.
        • Kuhl G.
        Glossoptosis. Semin Pediatr Surg. 2016; 25: 123-127
        • de Sousa T.V.
        • Marques I.L.
        • Carneiro A.F.
        • Bettiol H.
        • Freitas J.A.
        Nasopharyngoscopy in Robin sequence: clinical and predictive value.
        Cleft Palate Craniofac J. 2003; 40: 618-623
        • Cole A.
        • Lynch P.
        • Slator R.
        A new grading of Pierre Robin sequence.
        Cleft Palate Craniofac J. 2008; 45: 603-606
        • Morice A.
        • Soupre V.
        • Mitanchez D.
        • et al.
        Severity of retrognathia and glossoptosis does not predict respiratory and feeding disorders in pierre Robin sequence.
        Front Pediatr. 2018; 6: 351
        • MacLean J.E.
        • Fitzsimons D.
        • Fitzgerald D.A.
        • Waters K.A.
        The spectrum of sleep-disordered breathing symptoms and respiratory events in infants with cleft lip and/or palate.
        Arch Dis Child. 2012; 97: 1058-1063
        • Poets C.F.
        • Wiechers C.
        • Koos B.
        • Muzaffar A.R.
        • Gozal D.
        Pierre Robin and breathing: what to do and when?.
        Pediatr Pulmonol. 2021; (In press)https://doi.org/10.1002/ppul.25317
        • Kaditis A.G.
        • Alvarez M.L.A.
        • Boudewyns A.
        • et al.
        ERS statement on obstructive sleep disordered breathing in 1-to 23-month-old children.
        Eur Respir J. 2017; 50: 1591-1598
        • Logjes R.J.H.
        • MacLean J.E.
        • de Cort N.W.
        • et al.
        Objective measurements for upper airway obstruction in infants with Robin sequence: what are we measuring? A systematic review.
        J Clin Sleep Med. 2021; (In press)https://doi.org/10.5664/jcsm.9394
        • Brockmann P.E.
        • Schaefer C.
        • Poets A.
        • Poets C.F.
        • Urschitz M.S.
        Diagnosis of obstructive sleep apnea in children: a systematic review.
        Sleep Med Rev. 2013; 17: 331-340
        • Kirk V.
        • Baughn J.
        • D'Andrea L.
        • et al.
        American Academy of sleep medicine position paper for the use of a home sleep apnea test for the diagnosis of OSA in children.
        J Clin Sleep Med. 2017; 13: 1199-1203
        • Brockmann P.E.
        • Poets A.
        • Poets C.F.
        Reference values for respiratory events in overnight polygraphy from infants aged 1 and 3 months.
        Sleep Med. 2013; 14: 1323-1327
        • Marques I.L.
        • Bettiol H.
        • de Souza L.
        • Barbieri M.A.
        • Bachega M.I.
        Longitudinal study of the growth of infants with isolated Robin sequence considered being severe cases.
        Acta Paediatr. 2008; 97: 371-375
        • Cozzi F.
        • Totonelli G.
        • Frediani S.
        • Zani A.
        • Spagnol L.
        • Cozzi D.A.
        The effect of glossopexy on weight velocity in infants with Pierre Robin syndrome.
        J Pediatr Surg. 2008; 43: 296-298
        • Hsu C.T.
        • Chen C.H.
        • Lin M.C.
        • Wang T.M.
        • Hsu Y.C.
        Post-discharge body weight and neurodevelopmental outcomes among very low birth weight infants in Taiwan: a nationwide cohort study.
        PloS One. 2018; 13
        • Maas C.
        • Poets C.F.
        Initial treatment and early weight gain of children with Robin Sequence in Germany: a prospective epidemiological study.
        Arch Dis Child Fetal Neonatal. 2014; 99: F491-F494
        • Indrayan A.
        Demystifying LMS and BCPE methods of centile estimation for growth and other health parameters.
        Indian Pediatr. 2014; 51: 37-43
        • Dulfer K.
        • van Lieshout M.J.
        • van der Schroeff M.P.
        • et al.
        Quality of life in children with Robin sequence.
        Int J Pediatr Otorhinolaryngol. 2016; 86: 98-103
        • Basart H.
        • van Oers H.A.
        • Paes E.C.
        • et al.
        Health-related quality of life in children with Robin sequence.
        Am J Med Genet. 2017; 173: 54-61
        • Drescher F.D.
        • Jotzo M.
        • Goelz R.
        • Meyer T.D.
        • Bacher M.
        • Poets C.F.
        Cognitive and psychosocial development of children with Pierre Robin sequence.
        Acta Paediatr. 2008; 97: 653-656
        • Caillot A.
        • Ambroise B.
        • Benateau H.
        • Veyssiere A.
        Impact of early intravelar veloplasty at six months on mandibular growth in patients with Pierre Robin Sequence.
        J Cranio-Maxillo-Fac Surg. 2018; 46: 1059-1064
        • Wiechers C.
        • Gerdes R.
        • Ciuffolotti M.
        • et al.
        Retrospective study on growth in infants with isolated Robin sequence treated with the tuebingen palate plate.
        Orphanet J Rare Dis. 2021; 16 (in press): 338https://doi.org/10.1186/s13023-021-01959-2
        • Gomez O.J.
        • Baron O.I.
        • Penarredonda M.L.
        Pierre Robin sequence: an evidence-based treatment proposal.
        J Craniofac Surg. 2018; 29: 332-338
        • Hicks K.E.
        • Billings K.R.
        • Purnell C.A.
        • et al.
        Algorithm for airway management in patients with pierre Robin sequence.
        J Craniofac Surg. 2018; 29: 1187-1192
        • Almajed A.
        • Viezel-Mathieu A.
        • Gilardino M.S.
        • Flores R.L.
        • Tholpady S.S.
        • Cote A.
        Outcome following surgical interventions for micrognathia in infants with pierre Robin sequence: a systematic review of the literature.
        Cleft Palate Craniofac J. 2017; 54: 32-42
        • Resnick C.M.
        • Dentino K.
        • Katz E.
        • Mulliken J.B.
        • Padwa B.L.
        Effectiveness of tongue-lip adhesion for obstructive sleep apnea in infants with Robin sequence measured by polysomnography.
        Cleft Palate Craniofac J. 2016; 53: 584-588