Mortality in Robin sequence: identification of risk factors

European Journal of Pediatrics
Robrecht J H LogjesCorstiaan C Breugem

Abstract

Although Robin sequence (RS) is a well-known phenomenon, it is still associated with considerable morbidity and even mortality. The purposes of this study were to gain greater insight into the mortality rate and identify risk factors associated with mortality in RS. We retrospectively reviewed all RS infants followed at the Wilhelmina Children's Hospital from 1995 to 2016. Outcome measurements were death and causes of death. The authors identified 103 consecutive RS infants with a median follow-up of 8.6 years (range 0.1-21.9 years). Ten of the 103 infants (10%) died at a median age of 0.8 years (range 0.1-5.9 years). Nine of these ten infants (90%) were diagnosed with an associated syndrome. Of these, seven infants died of respiratory insufficiency due to various causes (two related to upper airway obstruction). The other two syndromic RS infants died of arrhythmia due to hypernatremia and of West syndrome with status epilepticus. One isolated RS infant died of brain ischemia after MDO surgery. Cardiac anomalies were observed in 41% and neurological anomalies in 36%. The presence of a neurological anomaly was associated with a mortality rate of 40% versus 7% in infants with no neurological anomaly (p = 0.016), with an odds rat...Continue Reading

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Citations

Apr 14, 2020·The Journal of Craniofacial Surgery·Marco FontanariPiero Cascone
Sep 11, 2020·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·Lauren K SalineroAlbert K Oh
Aug 11, 2020·Acta Medica (Hradec Králové)·Girish Gulab MeshramKanwaljeet Singh Hura
Aug 18, 2020·Chest·Christopher M CieloIgnacio E Tapia
May 8, 2021·Journal of Clinical Sleep Medicine : JCSM : Official Publication of the American Academy of Sleep Medicine·Robrecht J H LogjesCorstiaan C Breugem
Jun 17, 2021·Paediatric and Perinatal Epidemiology·Michele SantoroEster Garne
Jul 23, 2021·American Journal of Medical Genetics. Part a·Tara L WengerKelly N Evans
Jul 28, 2021·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·Fabian BlancGuillaume Captier
Jul 29, 2021·Plastic and Reconstructive Surgery·Robrecht J H LogjesCorstiaan C Breugem

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