Mandibular Catch-Up Growth in Pierre Robin Sequence: A Systematic Review

2018 ◽  
Vol 56 (2) ◽  
pp. 168-176 ◽  
Author(s):  
Chad A. Purnell ◽  
Lindsay E. Janes ◽  
Julian L. Klosowiak ◽  
Arun K. Gosain
Keyword(s):  
Systematic Review ◽  
Growth Rates ◽  
English Literature ◽  
Pierre Robin Sequence ◽  
Cross Sectional ◽  
Mandibular Growth ◽  
Gonial Angle ◽  
Robin Sequence ◽  
Catch Up ◽  
Pierre Robin

Objective: The concept of mandibular catch-up growth is often quoted in the literature regarding Pierre Robin sequence (PRS). We endeavored to perform a systematic review of whether the literature supports this concept. Design: Systematic review. Interventions: A PubMed-based systematic review of the English literature was performed of articles objectively measuring mandibular growth or position after nonoperative management of PRS. Main Outcome Measures: Rate and end point of mandibular length, ramus length, gonial angle, and maxillomandibular discrepancy. Results: The initial search delivered 607 English-language abstracts. Of these, 16 met inclusion criteria. Eight articles evaluating 143 patients followed longitudinal patient data and therefore allowed comparison of growth rates to controls. Ten articles evaluating 228 patients presented cross-sectional data and therefore could only evaluate a single time point. Two of the 8 longitudinal studies reported faster than normal growth of mandibular length in a significant portion of their cohort. Five of 8 reported equal growth rates. One of 16 studies reported that mandibular length of patients with PRS normalized compared to controls. Two of 16 studies reported no difference in maxillomandibular discrepancy between PRS and controls, whereas 10 reported a posteriorly displaced mandible relative to the maxilla in PRS. Significant differences in control groups, patients, and age existed between studies. Conclusions: While the concept of catch-up growth in PRS is often quoted, a minority of objective studies suggest increased mandibular growth rates in isolated PRS. Even fewer studies suggest that the maxillomandibular discrepancy in PRS completely resolves.

The mandibular catch-up growth controversy in Pierre Robin sequence

2001 ◽  
Vol 120 (3) ◽  
pp. 280-285 ◽  
Author(s):  
John Daskalogiannakis ◽  
R.Bruce Ross ◽  
Bryan D. Tompson
Keyword(s):  
Pierre Robin Sequence ◽  
Robin Sequence ◽  
Catch Up ◽  
Pierre Robin

Experimental Aspects of the Pathogenesis of Robin Sequence

2005 ◽  
Vol 42 (4) ◽  
pp. 372-376 ◽  
Author(s):  
Johannes Schubert ◽  
Heiko Jahn ◽  
Markus Berginski
Keyword(s):  
Nmri Mouse ◽  
Primary Role ◽  
Pierre Robin Sequence ◽  
Mandibular Growth ◽  
Robin Sequence ◽  
Secondary Palate ◽  
Tongue Position ◽  
Mouse Fetuses ◽  
Mandibular Retrognathia ◽  
Pierre Robin

Objective The Pierre Robin Sequence (PRS) is a good example of disturbed embryonic development of the secondary palate involving insufficient mandibular growth, failed forward tongue movement, and, in the case of a cleft, impeded fusion of the secondary palate. Discussion continues regarding which of the involved pathogenetic factors is the primary cause of the induced cascade of signs: insufficient mandibular growth or failed descent of the tongue. Design Forty-five randomly selected, 18-day-old formalin-fixed A/WySn mouse fetuses were investigated. The strain is known to have a basic genetic defect and as much as 44% clefts in the offspring. Twenty-four fetuses in the group had a cleft palate. Mandible position was measured relative to the head and to the presence or absence of a cleft. Cleft width and tongue position were also determined. Thirty-eight NMRI mouse fetuses of the same age served as controls. Results All A/WySn fetuses showed marked mandibular retrognathia, which was more severe in the cleft group (p < .05), but there was no correlation between the degree of retrognathia and cleft width. The median cleft width was 3.4 mm (1.6 through 6.3 mm). The tongue was in the cleft in all 12 fetuses with wide clefts (>3.4 mm wide), and free in the oral cavity in the other 12. Tongue position did not influence the degree of retrognathia (p < .05). Moreover, the tongue was free in all fetuses with severe retrognathia. Conclusion The results support the primary role of retroposition of the mandible in the development of cardinal symptoms of Pierre Robin Sequence.

scholarly journals 1173 Airway and Breathing Problems in Pierre Robin Sequence: A Systematic Review Protocol

2021 ◽  
Vol 108 (Supplement_6) ◽  
Author(s):  
A Ramjeeawon ◽  
P van der Plas ◽  
L van de Lande ◽  
J Ong ◽  
M Wyatt ◽  
...  
Keyword(s):  
Systematic Review ◽  
Grey Literature ◽  
Upper Airway ◽  
Sleep Apnoea ◽  
Cochrane Central Register ◽  
Pierre Robin Sequence ◽  
Expert Opinions ◽  
Robin Sequence ◽  
Assessment And Monitoring ◽  
Pierre Robin

Abstract Aim The primary aim is to systematically review the specific airway and/or breathing problems which have been reported in Pierre Robin Sequence (PRS). Secondary aims are to understand the prevalence and severity of these airway and breathing problems, and options for screening, assessment, and monitoring. Method A search strategy will be designed to search the following databases: MEDLINE, Embase, Web of Science, and the Cochrane Central Register of Controlled Trials(CENTRAL) including a grey literature search, to identify publications on airway and/or breathing problems in Pierre Robin Sequence patients. Airway or breathing problems to be included are defined upper airway conditions (e.g., sleep apnoea) or clinically or objectively defined airway/breathing problems (e.g., increased respiratory rate, polysomnography). Screening will exclude non-English articles, abstracts, letters, editorials, expert opinions and breathing problems not anatomically related to craniofacial underdevelopment. Results This systematic review has been successfully registered on the PROSPERO International Prospective Register of Systematic Reviews (CRD42020210572) Conclusions PRS is a rare craniofacial condition which presents at birth, consisting of micrognathia, glossoptosis and airway problems, and is usually, but not always, associated with a cleft palate. There is no consensus on the type of airway problems that affect patients with PRS. The findings of this systematic review will help to develop a consensus on the airway and breathing problems in PRS and options for assessment and monitoring of the airway and breathing problem. This data may also contribute to development of a standardized guideline for management of airway and breathing problems in PRS.

An Anthropometric Assessment of Mandibular Growth in Pierre Robin Sequence

2011 ◽  
Vol 128 ◽  
pp. 21
Author(s):  
Andrew S. Newman ◽  
Christopher Derderian ◽  
Michael N. Mirzabeigi ◽  
River M. Elliott ◽  
Oksana Jackson ◽  
...  
Keyword(s):  
Pierre Robin Sequence ◽  
Mandibular Growth ◽  
Robin Sequence ◽  
Pierre Robin ◽  
Anthropometric Assessment

scholarly journals Quality of life and phonatory and morphological outcomes in cognitively unimpaired adolescents with Pierre Robin sequence: a cross-sectional study of 72 patients

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Béatrice Thouvenin ◽  
Véronique Soupre ◽  
Marie-Anne Caillaud ◽  
Charlotte Henry-Mestelan ◽  
Christel Chalouhi ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Upper Airway ◽  
Well Being ◽  
Pierre Robin Sequence ◽  
Craniofacial Malformations ◽  
Cross Sectional ◽  
Robin Sequence ◽  
Pierre Robin ◽  
Better Than

Abstract Background Pierre Robin sequence (PRS) is a heterogeneous condition involving retro(micro)gnathia, glossoptosis and upper airway obstruction, very often with posterior cleft palate. Patients with PRS, either isolated or associated with Stickler syndrome have good intellectual prognosis. Nevertheless, the quality of life in adolescence and the phonatory and morphological outcomes are rarely analysed. We assessed the phonatory and morphological outcomes of 72 cognitively unimpaired adolescents with PRS, studied their oral (COHIP-SF19), vocal (VHI-9i) and generic quality of life (QoL; KIDSCREEN-52), and searched for determinants of these outcomes. Results Two-thirds of our adolescents retained low or moderate phonation difficulties, but risk factors were not identified. For 14%, morphological results were considered disharmonious, with no link to neonatal retrognathia severity. Only one vs two-stage surgery seemed to affect final aesthetic results. The oral QoL of these adolescents was comparable to that of control patients and was significantly better than that of children with other craniofacial malformations (COHIP-SF19 = 17.5, 15.4 and 25.7, respectively). The oral QoL of the adolescents with non-isolated PRS was significantly worse (COHIP-SF19 = 24.2) than that of control patients and close to that of children with other craniofacial malformations. The vocal QoL of the adolescents (mean [SD] VHI-9i = 7.5 [5.4]) was better than that of patients with other voice pathologies and better when phonation was good. The generic QoL of the adolescents was satisfactory but slightly lower than that of controls, especially in dimensions concerning physical well-being, relationships and autonomy. QoL results were lower for adolescents with non-isolated than isolated PRS. Only non-isolated PRS and low oral QoL affected generic QoL. Conclusion Morphological or phonatory impairments remain non-rare in adolescents with PRS but do not seem to be directly responsible for altered QoL. These adolescents, especially those with non-isolated PRS, show self-confidence and social-relation fragility. We must focus on long-term functional and psychological results for PRS patients and improve therapy protocols and follow-up, notably those affecting the oral aspects of the disease.

Airway Morphological Changes in Pierre Robin Sequence: A Retrospective Study

2020 ◽  
Vol 57 (7) ◽  
pp. 828-839
Author(s):  
Laura S. Humphries ◽  
Tulsi Roy ◽  
Anne Huang ◽  
John Collins ◽  
Fuad M. Baroody ◽  
...  
Keyword(s):  
Surface Area ◽  
Distraction Osteogenesis ◽  
Cross Sectional Area ◽  
Pierre Robin Sequence ◽  
Sectional Area ◽  
Cross Sectional ◽  
Mandibular Distraction ◽  
Robin Sequence ◽  
Mandibular Distraction Osteogenesis ◽  
Pierre Robin

Objective: To investigate airway morphology changes in patients with Pierre Robin sequence (PRS) pre–/post–mandibular distraction osteogenesis (MDO) and to compare morphologic changes to age-matched controls. Design: Retrospective case–control study. Setting: Urban, academic, tertiary medical center. Patients, Participants: Fifteen patients with PRS after MDO to relieve upper airway obstruction (UAO) (2008-2018); age-matched controls for post-MDO patients. Interventions: Mandibular distraction osteogenesis, curvilinear internal mandibular distractors. Main Outcome Measures: (1) Physiologic improvement after MDO (apnea–hypopnea index; minimum oxygen saturation); (2) airway size (volume, surface area, length, mean/minimum cross-sectional area), shape (lateral:anterior–posterior ratio, cross-sectional area ratios, uniformity, sphericity), and changes with MDO; and (3) post-MDO airway size, shape versus age-matched controls. Results: Airway size increased after MDO (volume, P = .01; surface area, P = .02; length, P = .01), as did cross-sectional area (mean, P = .02; minimum, P = .02; minimum retropalatal, P = .05, mid-retroglossal, P = .02). Post-MDO PRS airways were larger than controls (volume, P < .01; surface area, P < .01; length, P < .01, cross-sectional area, P = .03). Airway shape remained nonuniform and flat post-MDO; control airways were round. Two syndromic patients required repeat MDO and had subphysiologic post-MDO airway cross-sectional area. Post-MDO PRS patients with supraphysiologic cross-sectional area along the entire airway had no UAO recurrence. Conclusions: In this small, heterogenous patient sample, MDO increases airway size, may preferentially affect the retropalatal airway, and often results in supraphysiologic airway dimensions. These retropalatal changes may be important in relieving severe UAO in patients with PRS. Generalizability of our results is limited by small cohort size and patient heterogeneity.

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