Treatment Options for Severe Upper Airway Obstruction in Pierre-Robin Sequence

Elluru, Ravindhra G.

doi:10.1016/j.jpeds.2005.04.012

Cited by 10 publications

(10 citation statements)

References 23 publications

(14 reference statements)

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“…Before establishing a clear role of mandibular distraction in the treatment algorithm for severe PRS, carefully designed prospective studies on resolution of obstructive apnoea, the natural history of dysphagia, mandibular growth, height, and weight gain, are much needed [14]. The present findings strongly suggest that TLA does not cause dysphagia and failure to thrive.…”

Section: Discussionmentioning

confidence: 68%

The effect of glossopexy on weight velocity in infants with Pierre Robin syndrome

Cozzi¹,

Totonelli²,

Frediani³

et al. 2008

Journal of Pediatric Surgery

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Section: Discussionmentioning

confidence: 68%

The effect of glossopexy on weight velocity in infants with Pierre Robin syndrome

Cozzi¹,

Totonelli²,

Frediani³

et al. 2008

Journal of Pediatric Surgery

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“…The severity of the micrognathia, respiratory and feeding problems vary widely amongst RS patients. There are several management strategies such as prone positioning [Elluru, ; Butow et al, ; Evans et al, ; Glynn et al, ], nasopharyngeal airway [Meyer et al, ; Glynn et al, ; Abel et al, ], tongue lip adhesion [Denny et al, ; Bijnen et al, ; Cheng et al, ; Scott and Mader, ], mandibular distraction osteogenesis [Burstein and Williams, ; Shen et al, ; Cicchetti et al, ; Paes et al, ; Flores et al, ; Scott and Mader, ], and tracheostomy [Butow et al, ; Mobin et al, ; Scott and Mader, ]. However, there is no consensus which strategy should be followed and which parameters in the patients should dictate the choice of management [Mackay, ].…”

Section: Introductionmentioning

confidence: 99%

Etiology and pathogenesis of robin sequence in a large Dutch cohort

Basart

Paes

Maas

et al. 2015

American J of Med Genetics Pt A

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Robin sequence (RS) can be defined as the combination of micrognathia and upper airway obstruction/glossoptosis causing neonatal respiratory problems, with or without a cleft palate and either isolated or non-isolated. Pathogenesis varies widely. We hypothesize that optimal treatment depends on pathogenesis and therefore patients should be stratified according to diagnosis. Here, we evaluate diagnoses and (presumed) pathogeneses in an RS cohort. Medical records of all RS patients presenting between 1995-2013 in three academic hospitals were evaluated. Four clinical geneticists re-evaluated all information, including initial diagnosis. Diagnoses were either confirmed, considered uncertain, or rejected. If uncertain or rejected, patients were re-evaluated. Subsequent results were re-discussed and a final conclusion was drawn. We included 191 RS patients. After re-evaluation and changing initial diagnoses in 48 of the 191 patients (25.1%), 37.7% of the cohort had isolated RS, 8.9% a chromosome anomaly, 29.3% a Mendelian disorder, and 24.1% no detectable cause. Twenty-two different Mendelian disorders were diagnosed, of which Stickler syndrome was most frequent. Stratification of diagnoses according to (presumed) pathogenic mechanism in 73 non-isolated patients with reliable diagnoses showed 43.9% to have a connective tissue dysplasia, 5.5% a neuromuscular disorder, 47.9% a multisystem disorder, and 2.7% an unknown mechanism. We diagnosed more non-isolated RS patients compared to other studies. Re-evaluation changed initial diagnosis in a quarter of patients. We suggest standardized re-evaluation of all RS patients. Despite the relatively high diagnostic yield pathogenesis could be determined in only 59.7% (71/119), due to limited insight in pathogenesis in diagnosed entities. Further studies into pathogenesis of entities causing RS are indicated.

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“…Reasons that guidelines are lacking may include the highly variable phenotype of PRS, the persistent discrepancy in the diagnosis of PRS among providers, and difficulty in predicting which children will resolve their obstruction with conservative measures. Also, there is a lack of compelling comparative studies due to the age of the patient population and scarcity of this disease entity (Elluru, 2005). In the present era without PRS guidelines to direct more consistent training, patient care, and outcome measures, it is helpful to know current practice tendencies.…”

Section: Discussionmentioning

confidence: 99%

“…It is even unclear if there is a “one surgery fits all” type of approach that is superior to a more adaptable and patient-dependent approach (Mackay, 2011). Studies have identified patient factors that may help guide management decisions (Kirschner et al, 2003; Schaefer et al, 2004; Dauria and Marsh, 2008), but reliable, high-evidence studies are difficult considering the low incidence of pathology and the age of the patient population (Elluru, 2005). Each procedure has significant potential complications that must be considered.…”

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confidence: 99%

Airway Management in Pierre Robin Sequence: Patterns of Practice

Collins

Powitzky

Robledo

et al. 2014

The Cleft Palate Craniofacial Journal

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Objectives : To report survey results from American Cleft Palate-Craniofacial Association members on the practice patterns of airway obstruction management in patients with Pierre Robin sequence. Design : A 10-question online survey was sent and the data were reviewed. Setting : Online survey of members of the American Cleft Palate-Craniofacial Association. Patients : Surveys assessed management patterns of patients with Pierre Robin sequence whom a surgeon member of the American Cleft Palate-Craniofacial Association treated for airway obstruction. Interventions : The survey comprised data on management strategies for airway obstruction in Pierre Robin sequence, including tracheostomy, tongue-lip adhesion, mandibular distraction, and treatments that falls in the "other" category. Results : A total of 87 American Cleft Palate-Craniofacial Association members completed the survey. Respondents' results were analyzed as a whole and by individual subspecialty: plastic surgery (n = 33), oromaxillofacial surgery (n = 21), and otolaryngology (n = 29). Although most of the surgeons were trained to manage airway obstruction in Pierre Robin sequence patients using tracheostomy (47%, n = 39) and tongue-lip adhesion (31%, n = 26), 48% reported a current preference for mandibular distraction (n = 40). Of surgeons who preferred to manage Pierre Robin sequence with tongue-lip adhesion (n = 23), 65% were trained to do so (n = 15). Surgeons preferring mandibular distraction (n = 40) and tracheostomy (n = 14) more often reported they were trained to manage Pierre Robin sequence with tracheostomy. Conclusions : Currently there are various practice patterns for the management of airway obstruction in Pierre Robin sequence. Training habits and subspecialty category may influence a surgeon's preference in patients who fail conservative therapy. Treatment guidelines are lacking and may require significant collaboration among centers and subspecialties to develop a more standardized approach to a challenging clinical entity.

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Treatment Options for Severe Upper Airway Obstruction in Pierre-Robin Sequence

Cited by 10 publications

References 23 publications

The effect of glossopexy on weight velocity in infants with Pierre Robin syndrome

The effect of glossopexy on weight velocity in infants with Pierre Robin syndrome

Etiology and pathogenesis of robin sequence in a large Dutch cohort

Airway Management in Pierre Robin Sequence: Patterns of Practice

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