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Pierre Robin sequence: Subdivision, data, theories, and treatment – Part 3: Prevailing controversial theories related to Pierre Robin sequence

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ABSTRACT

Context:: The disorder currently accepted as Pierre Robin syndrome/anomaly/sequence (PRS) has been plagued by controversy ever since initially being described. Controversy exists not only about the appropriate terminology and etiopathogenesis of the disorder but also about its management. Therefore, clinical findings and treatment outcomes of a large database of 266 PRS cases were compared with the current state of knowledge in the scientific literature related to history, clinical description, diagnostic criteria, epidemiology, theories of oligohydramnios, mandibular catch-up growth, midfacial hyperplasia, and the early management.

Aim:: The aims of Part 3 debate the controversial biological theories relating to PRS.

Materials and methods:: Oligo-/poly-hydramnios, mandibular catch-up growth, and midfacial hyperplasia, the three in the literature most prevailing theories related to PRS, have been compared and discussed with the findings provided by this large database of 266 Siebold-Robin sequence (SRS) and Fairbairn-Robin triad (FRT) cases.

Results:: History and clinical findings evaluated in this database refute the first two theories. Although manifold midfacial appearances were demonstrated in FRT cases, a third of all SRS cases presented with mid-facial hyperplasia.

Conclusion:: The three main biological theories regarding PRS could not be verified after thorough analysis of the database.

No MeSH data available.


(a and b) Mandibular catch-up growth in Fairbairn-Robin triad
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Figure 2: (a and b) Mandibular catch-up growth in Fairbairn-Robin triad

Mentions: Mandibular catch-up [Figure 2a and b] and noncatch-up growth [Figure 3a and b] were evaluated in 59.8% (159 of 266) of the patients in this database. Whereas none of the 13 evaluated SRS cases revealed catch-up growth, 34.9% (51 of 146) of FRT patients presented with catch-up growth [Figure 4],[5]82.4% (42 of 51) of which were nonsyndromic cases.


Pierre Robin sequence: Subdivision, data, theories, and treatment – Part 3: Prevailing controversial theories related to Pierre Robin sequence
(a and b) Mandibular catch-up growth in Fairbairn-Robin triad
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4979340&req=5

Figure 2: (a and b) Mandibular catch-up growth in Fairbairn-Robin triad
Mentions: Mandibular catch-up [Figure 2a and b] and noncatch-up growth [Figure 3a and b] were evaluated in 59.8% (159 of 266) of the patients in this database. Whereas none of the 13 evaluated SRS cases revealed catch-up growth, 34.9% (51 of 146) of FRT patients presented with catch-up growth [Figure 4],[5]82.4% (42 of 51) of which were nonsyndromic cases.

View Article: PubMed Central - PubMed

ABSTRACT

Context:: The disorder currently accepted as Pierre Robin syndrome/anomaly/sequence (PRS) has been plagued by controversy ever since initially being described. Controversy exists not only about the appropriate terminology and etiopathogenesis of the disorder but also about its management. Therefore, clinical findings and treatment outcomes of a large database of 266 PRS cases were compared with the current state of knowledge in the scientific literature related to history, clinical description, diagnostic criteria, epidemiology, theories of oligohydramnios, mandibular catch-up growth, midfacial hyperplasia, and the early management.

Aim:: The aims of Part 3 debate the controversial biological theories relating to PRS.

Materials and methods:: Oligo-/poly-hydramnios, mandibular catch-up growth, and midfacial hyperplasia, the three in the literature most prevailing theories related to PRS, have been compared and discussed with the findings provided by this large database of 266 Siebold-Robin sequence (SRS) and Fairbairn-Robin triad (FRT) cases.

Results:: History and clinical findings evaluated in this database refute the first two theories. Although manifold midfacial appearances were demonstrated in FRT cases, a third of all SRS cases presented with mid-facial hyperplasia.

Conclusion:: The three main biological theories regarding PRS could not be verified after thorough analysis of the database.

No MeSH data available.