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Pierre Robin sequence: Subdivision, data, theories, and treatment - Part 4: Recommended management and treatment of Pierre Robin sequence and its application

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 early management.

Aims of part 4:: To provide a systematic treatment protocol for Fairbairn–Robin triad (FRT) and Siebold Robin sequence (SRS) patients based on clinical findings and experience with 266 PRS cases.

Subjects and methods:: A plethora of treatment modalities and their outcome in literature have been compared to those applied in this database and their outcomes.

Results:: The management of SRS/FRT depends on various factors including compromised airways, feeding difficulties, as well as the sequence of the reconstructive ladder.

Conclusion:: Based on the novel PRS subdivisions, a stepwise sequential treatment approach is outlined, addressing the particular needs of each disorder systematically.

No MeSH data available.


Related in: MedlinePlus

Hard palate width before soft palate repair
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Figure 3: Hard palate width before soft palate repair

Mentions: As highlighted in Table 4, three different sequences were used for cleft repair in the here presented patients. With the two-stage approach where the sP cleft was repaired first, postoperative compromised airways occurred in only 2.7%. A two-stage approach with an initial sP repair, therefore keeping the hP cleft, facilitates postoperative breathing in FRT patients.[2] The tongue's position slowly adapts to an anteroinferior positioning of the palatal shelves which favors respiration and feeding. The hP cleft, which shrinks in width over a period of 11 months [Figures 3 and 4] can then be closed at 18 months of age.[28]


Pierre Robin sequence: Subdivision, data, theories, and treatment - Part 4: Recommended management and treatment of Pierre Robin sequence and its application
Hard palate width before soft palate repair
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4979342&req=5

Figure 3: Hard palate width before soft palate repair
Mentions: As highlighted in Table 4, three different sequences were used for cleft repair in the here presented patients. With the two-stage approach where the sP cleft was repaired first, postoperative compromised airways occurred in only 2.7%. A two-stage approach with an initial sP repair, therefore keeping the hP cleft, facilitates postoperative breathing in FRT patients.[2] The tongue's position slowly adapts to an anteroinferior positioning of the palatal shelves which favors respiration and feeding. The hP cleft, which shrinks in width over a period of 11 months [Figures 3 and 4] can then be closed at 18 months of age.[28]

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 early management.

Aims of part 4:: To provide a systematic treatment protocol for Fairbairn–Robin triad (FRT) and Siebold Robin sequence (SRS) patients based on clinical findings and experience with 266 PRS cases.

Subjects and methods:: A plethora of treatment modalities and their outcome in literature have been compared to those applied in this database and their outcomes.

Results:: The management of SRS/FRT depends on various factors including compromised airways, feeding difficulties, as well as the sequence of the reconstructive ladder.

Conclusion:: Based on the novel PRS subdivisions, a stepwise sequential treatment approach is outlined, addressing the particular needs of each disorder systematically.

No MeSH data available.


Related in: MedlinePlus