Late Problems in the Management of the Pierre Robin Syndrome

Hoffman, Saul; Kahn, Sidney; Seitchik, Murray W.

doi:10.1097/00006534-196505000-00007

Cited by 37 publications

(10 citation statements)

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“…They are at risk for hypoxic brain damage, impaired mental development, cor pulmo nale, aspiration pneumonia, and failure to thrive. 8,13,14 Even with modern intensive care, mortality risk is still recognized as a significant problem, with rates ranging with 0% to 13.6%. 1,[15][16][17][18] The causes of morbidity and mortality have been attributed to delayed identification of RS in infants, delay in securing the airway to prevent chronic hypoxia, other disorders in syndromic RS, and long-term sequelae of the disease.…”

Section: Discussionmentioning

confidence: 99%

“Natural” Decannulation in Patients with Robin Sequence and Severe Airway Obstruction

Han¹,

Seruya²,

Oh³

et al. 2012

Ann Otol Rhinol Laryngol

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Section: Discussionmentioning

confidence: 99%

“Natural” Decannulation in Patients with Robin Sequence and Severe Airway Obstruction

Han¹,

Seruya²,

Oh³

et al. 2012

Ann Otol Rhinol Laryngol

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“…Upper airway management plays a central role in the treatment of RS. Treatment of the tongue-based respiratory obstruction minimizes the risk of hypoxic cerebral injury and repeated (aspiration) pneumonia [ 13 , 18 , 25 ]. Nonsurgical interventions include positional change, the nasopharyngeal airway, continuous positive airway pressure, and the palatal plate [ 1 , 2 , 16 , 22 ].…”

Section: Introductionmentioning

confidence: 99%

Mortality in Robin sequence: identification of risk factors

et al. 2018

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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 ratio of 8.3 (95% CI 1.4–49.0) for neurological anomaly versus no neurological anomaly. Mortality was 15% in infants with syndromic RS versus 2% in infants with isolated RS (p = 0.044). Mortality was not significantly associated with the presence of a cardiac anomaly, surgical treatment for severe respiratory distress in the neonatal period, or prematurity.Conclusion: RS represents a heterogeneous patient population and is associated with a high level of underlying syndromes. The present study reports a mortality rate of 10% significantly associated with syndromic RS and the presence of neurological anomalies. 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. What is Known: • Reported mortality rates in Robin sequence vary from 2% to 26%.• Clinicians mainly focus on the morbidity of Robin sequence that includes respiratory complications due to upper airway obstruction in the period after birth.• Robin sequence represents a heterogeneous patient population and is associated with a high level of underlying syndromes. What is New: • The present study reports a mortality rate of 10% significantly associated with syndromic Robin sequence and the presence of neurological anomalies.• A multidisciplinary approach in all infants born with Robin sequence, including genetic evaluation and standardized workup for neurological anomalies, is crucial to identify infants with underlying syndromes potentially associated with increased mortality.

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“…The estimated prevalence of PRS ranges between 1 in 8000 to 1 in 14000 live births (Robin, 1994; Printzlau & Andersen 2004; Maas & Poets, 2014). The sequelae of untreated airway obstruction and feeding difficulty can result in severe complications; however, the optimal workup and management strategy is not universally accepted (Hoffman et al, 1965; Parsons & Smith, 1982; Greathouse et al, 2016). Treatment targets the obstructive tongue base and depends on disease severity.…”

Section: Introductionmentioning

confidence: 99%

Impact of Hospital Characteristics on Mandibular Distraction Osteogenesis Outcomes Among Patients With Pierre Robin Sequence Utilizing a National Inpatient Database

Marston

Patel

Pecha

et al. 2021

The Cleft Palate Craniofacial Journal

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Objectives: This study collected national inpatient data to investigate the impact of hospital specialty and size on patient outcomes following mandibular distraction osteogenesis (MDO). Design: Kids’ Inpatient Database was used to identify patients less than 12 months of age with Pierre Robin sequence (PRS) who underwent MDO in one of the following years: 2006, 2009, and 2012. Setting: Inpatient database from the United States. Participants: Two hundred seventy-six patients with PRS underwent MDO with 134 (48.6%) identified as nonsyndromic and 142 (51.4%) as syndromic. Interventions: Mandibular distraction osteogenesis. Main Outcome Measures: Length of hospital stay, adjunct airway and nutritional interventions and disposition. Results: The average length of stay was 24 and 30 days for patients with nonsyndromic and syndromic PRS, respectively ( P = .066). Patients with a syndromic as compared to nonsyndromic diagnosis had a higher incidence of gastrostomy tube placement (21.8 vs 12.7%, P = .045). Univariate analysis showed that a lower proportion of patients at children’s hospitals as compared to non-children’s hospitals necessitated 1 or more airway or nutrition-related intervention (19/148 [12.8%] vs 31/127 [24.4%]; P = .012) and had a lower incidence of a nonroutine discharge (transfer or patient death; 7.4% vs 40.0% nonroutine; P < .001). Multivariable analysis additionally revealed that patients at children’s hospitals were less likely to discharge nonroutine (OR = 0.07, 95% CI: 0.02-0.32). Conclusions: Results from this national cohort demonstrated that at children-specific hospitals patients with PRS were less likely to require additional airway and nutritional procedures and more likely to discharge to home.

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Late Problems in the Management of the Pierre Robin Syndrome

Cited by 37 publications

References 0 publications

“Natural” Decannulation in Patients with Robin Sequence and Severe Airway Obstruction

“Natural” Decannulation in Patients with Robin Sequence and Severe Airway Obstruction

Mortality in Robin sequence: identification of risk factors

Impact of Hospital Characteristics on Mandibular Distraction Osteogenesis Outcomes Among Patients With Pierre Robin Sequence Utilizing a National Inpatient Database

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