Regional variations in the presentation and surgical management of Pierre Robin sequence

Scott, Andrew R.; Mader, Nicholas S.

doi:10.1002/lary.24782

Cited by 57 publications

(84 citation statements)

References 24 publications

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“…A yet higher incidence of 1 case per 3130 live births has been described in the USA, from a study which utilised a hospital admissions database (Kids’ Inpatient Database; KID) for case ascertainment . RS was assumed in all cases where ICD‐9 codes for micrognathia and cleft palate coexisted.…”

Section: Discussionmentioning

confidence: 99%

“…1 However, significantly higher rates of surgical intervention (including distraction osteogenesis and tongue-lip adhesion) and tracheostomy of 19-77% have been reported in several studies which are largely from North American centers. 5,[24][25][26][27] This wide variation in practice with an apparent geographical pattern suggests that physician or treatment center preferences, rather than clinical necessity, may direct RS management.…”

Section: Discussionmentioning

confidence: 99%

“…The most recent study of RS epidemiology in the UK, published over 30 years ago, similarly reported RS incidence as 1 case per 8500 live births . However, more recently data from USA and The Netherlands suggest that RS may be more common than previously suggested, with an estimated RS birth prevalence of 1 case per 3130‐5600 live births reported …”

Section: Introductionmentioning

confidence: 97%

“…3 However, more recently data from USA and The Netherlands suggest that RS may be more common than previously suggested, with an estimated RS birth prevalence of 1 case per 3130-5600 live births reported. 4,5 We hypothesized that the incidence of RS in the UK had increased since last reported. The study was designed to address this research question, and reports RS incidence in the East of Scotland (EoS) from 2004 to 2013.…”

Section: Introductionmentioning

confidence: 99%

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Epidemiology of Robin sequence with cleft palate in the East of Scotland between 2004 and 2013

Wright

Mehendale

Urquhart

2018

Pediatric Pulmonology

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 97%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Epidemiology of Robin sequence with cleft palate in the East of Scotland between 2004 and 2013

Wright

Mehendale

Urquhart

2018

Pediatric Pulmonology

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“…Studies about RS primarily focus on cause, interventions, and medical outcomes of the treatment strategies [Evans et al, ; Scott and Mader, ]. Despite their importance for optimal care and evaluating treatment success, patient‐reported outcomes (PROs) such as self‐esteem, psychosocial functioning, and health‐related quality of life (HRQOL) have been studied only infrequently.…”

Section: Introductionmentioning

confidence: 99%

Health‐related quality of life in children with Robin sequence

Basart

Oers

Paes

et al. 2016

American J of Med Genetics Pt A

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This patient-reported outcome (PRO) study reports on 102 children with Robin sequence (RS) and their parents. There has been differentiated between those with isolated RS and those with RS as part of a syndrome, and take various treatments into account. All RS families from an earlier described cohort were invited to participate. Parents and RS children completed online questionnaires regarding health-related quality of life (HRQoL), satisfaction with appearance, parental distress, and RS specific topics. Results were compared with the Dutch norm population if available. There was no major difference in HRQoL in RS children and the Dutch norm population, nor between children with isolated RS and those with RS as part of a syndrome. The latter is likely due to the large percentage of children with Stickler syndrome, and small number of RS children with intellectual disability. Parental distress was higher in RS children with syndromes compared to parents of isolated RS children. When comparing various treatments, the subgroup treated by mandibular distraction showed a tendency of lower HRQoL scores, less satisfaction with appearance, and more parental distress. Also in the NPA group parents showed a tendency of more parental distress. Subgroups for each treatment were very small, however, and firm conclusions cannot be drawn. In this study, HRQoL in RS children is demonstrated comparable to the norm population, despite variations in treatment, possibly with less favorable outcome for children who received mandibular distraction. Markedly larger studies are needed to allow more reliable comparison of PROs in various treatments, and to incorporate PROs in management guidelines to obtain optimal patient care. © 2016 Wiley Periodicals, Inc.

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Use of External Distractors and the Role of Imaging Prior to Mandibular Distraction in Infants With Isolated Pierre Robin Sequence and Stickler Syndrome

Mingo

Sidman

Sampson

et al. 2016

JAMA Facial Plast Surg

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IMPORTANCE Computed tomographic (CT) scans are often obtained before mandibular distraction osteogenesis in patients with isolated Pierre Robin sequence. There is concern regarding the risk of radiation exposure from CT in children. OBJECTIVE To evaluate whether preoperative CT is necessary for adequate airway, feeding, and aesthetic outcomes following mandibular distraction with external distraction devices in infants with isolated Pierre Robin sequence. DESIGN, SETTING, AND PARTICIPANTS In a retrospective review of medical records, infants who underwent mandibular distraction between January 1, 1998, and September 30, 2014, at 2 tertiary children's hospitals were identified using procedure codes. Data analysis was conducted December 1, 2014, to March 31, 2015. Fifty-two patients fit the inclusion criteria of isolated Pierre Robin sequence or Stickler syndrome, of being younger than 9 months at the time of distraction, and of use of external distractors. Forty-two of these infants did not receive preoperative CT imaging. EXPOSURE Mandibular distraction osteogenesis for isolated Pierre Robin sequence or Stickler syndrome. MAIN OUTCOME MEASURES Number of infants who were able to avoid tracheostomy or achieve decannulation, who were able to avoid placement or achieve removal of a gastrostomy tube, and in whom there were no intraoperative complications, no open-bite deformity, no malocclusion, no asymmetry, and no postoperative complications. RESULTS In comparison with the 10 infants who underwent preoperative CT, all 42 of the infants (100%) who did not receive preoperative CT imaging successfully avoided tracheostomy or achieved decannulation (P = .04) and 40 patients (95%) did not require placement of a gastrostomy tube or were able to undergo gastrostomy tube removal postoperatively (P < .001). There were no significant differences between the CT and non-CT groups in the other 5 outcome measures. Two patients (5%) required postoperative gastrostomy tube placement, 2 patients (5%) had minor intraoperative complications that might have been anticipated with CT, 2 patients (5%) demonstrated malocclusion, and 1 infant (2%) had asymmetry at the end of the distraction phase. CONCLUSIONS AND RELEVANCE This series suggests that the absence of preoperative CT does not compromise functional or aesthetic outcomes in mandibular distraction with external distraction devices in infants with isolated Pierre Robin sequence or Stickler syndrome. This finding has implications for cost containment and reduction of radiation exposure to a vulnerable population. LEVEL OF EVIDENCE 4.

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Regional variations in the presentation and surgical management of Pierre Robin sequence

Abstract: 2c.

Cited by 57 publications

References 24 publications

Epidemiology of Robin sequence with cleft palate in the East of Scotland between 2004 and 2013

Epidemiology of Robin sequence with cleft palate in the East of Scotland between 2004 and 2013

Health‐related quality of life in children with Robin sequence

Use of External Distractors and the Role of Imaging Prior to Mandibular Distraction in Infants With Isolated Pierre Robin Sequence and Stickler Syndrome

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