Treatment of the tongue-based respiratory obstruction minimizes the risk of hypoxic cerebral injury and repeated (aspiration) pneumonia. Upper airway management plays a central role in the treatment of RS. Reported mortality rates in RS vary from 2 to 26%. Clinicians mainly focus on the morbidities of RS, which include respiratory complications due to upper airway obstruction, feeding problems, a related failure to thrive, and the associated cleft palate problems, when present. RS infants represent a heterogeneous patient population because RS might be an isolated condition or be part of a syndrome (in about 26 to 83% of cases). Cleft palate is frequently encountered, but is not considered a prerequisite for the diagnosis. ![]() ![]() Recently, an international consensus was achieved regarding the three distinguishing characteristics (micrognathia, glossoptosis, and upper airway obstruction) that should be included in the diagnosis of RS in newborns. RS is a congenital condition occurring in approximately 1 in 5600–8000 live births. Robin sequence (RS) was first described by the French stomatologist Pierre Robin in 1923 and is characterized by the triad of micrognathia, subsequently leading to glossoptosis and varying degrees of upper airway obstruction. A multidisciplinary approach in all infants born with RS, including genetic testing and examination of neurological anomalies in a standardized way, is crucial to identify infants with underlying syndromes potentially associated with increased mortality. ![]() The present study reports a mortality rate of 10% significantly associated with syndromic RS and the presence of neurological anomalies. Mortality was not significantly associated with the presence of a cardiac anomaly, surgical treatment for severe respiratory distress in the neonatal period, or prematurity.Ĭonclusion: RS represents a heterogeneous patient population and is associated with a high level of underlying syndromes. Mortality was 15% in infants with syndromic RS versus 2% in infants with isolated RS ( p = 0.044). 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 ratio of 8.3 (95% CI 1.4–49.0) for neurological anomaly versus no neurological anomaly. Cardiac anomalies were observed in 41% and neurological anomalies in 36%. One isolated RS infant died of brain ischemia after MDO surgery. The other two syndromic RS infants died of arrhythmia due to hypernatremia and of West syndrome with status epilepticus. Of these, seven infants died of respiratory insufficiency due to various causes (two related to upper airway obstruction). Nine of these ten infants (90%) were diagnosed with an associated syndrome. Ten of the 103 infants (10%) died at a median age of 0.8 years (range 0.1–5.9 years). The authors identified 103 consecutive RS infants with a median follow-up of 8.6 years (range 0.1–21.9 years). ![]() Outcome measurements were death and causes of death. We retrospectively reviewed all RS infants followed at the Wilhelmina Children’s Hospital from 1995 to 2016. The purposes of this study were to gain greater insight into the mortality rate and identify risk factors associated with mortality in RS. Although Robin sequence (RS) is a well-known phenomenon, it is still associated with considerable morbidity and even mortality.
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |