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Pierre Robin syndrome: mandibular growth during the first year of life
F Vegter1, J J Hage, J W Mulder
1Department of Plastic and Reconstructive Surgery, Academisch Ziekenhuis Vrije Universiteit, Amsterdam, The Netherlands.
Insights
Mandibular growth in Pierre Robin syndrome patients does not show significant "catch-up" during the first year. A study using the jaw index found similar proportional mandibular growth compared to healthy infants.
Area of Science:
- Craniofacial development
- Pediatric genetics
- Medical diagnostics
Background:
- Pierre Robin syndrome is characterized by micrognathia and glossoptosis, leading to severe respiratory and feeding issues in newborns.
- It is commonly believed that the mandible undergoes significant compensatory growth during the first year of life in these patients.
Purpose of the Study:
- To investigate and quantify the alleged "catch-up" mandibular growth in infants with Pierre Robin syndrome.
- To assess the utility of the jaw index as a noninvasive method for evaluating mandibular growth.
Main Methods:
- The jaw index, calculated as (alveolar overjet x maxillary arch width) / mandibular arch width, was used.
- Measurements included alveolar overjet and arch widths (maxillary and mandibular) using specific anatomical landmarks.
- Mandibular growth in 7 Pierre Robin patients was compared to healthy control groups at 0, 6, and 12 months of age.
Main Results:
- The study could not objectively confirm "catch-up" mandibular growth in Pierre Robin syndrome patients within the first year.
- The proportional mandibular growth observed in Pierre Robin syndrome patients was similar to that of healthy control subjects.
- The jaw index measurement proved to be a noninvasive and harmless method for assessing jaw development.
Conclusions:
- The hypothesis of significant "catch-up" mandibular growth in Pierre Robin syndrome during the first year is not supported by this study's findings.
- Mandibular growth patterns in infants with Pierre Robin syndrome appear proportionately similar to those of healthy infants.
- Further research may be needed to explore alternative or later-stage growth dynamics and management strategies for Pierre Robin syndrome.
Abstract:
Micrognathia and glossoptosis may cause severe respiratory and feeding difficulties in newborns with Pierre Robin syndrome. The growth of the mandible is alleged to more or less "catch up" during the first year of life. We could not objectify such a catch-up growth using the noninvasive and harmless measurement of the jaw index. The jaw index is defined as alveolar overjet x maxillary arch/mandibular arch. The alveolar overjet is the frontodorsal distance between the most anterior points of the upper and lower alveolar arches, whereas the maxillary arch is measured from the left tragus to the right tragus via the subnasal point, and the mandibular arch is measured from the left to the right tragus via the pogonion point. The growth of the mandible in 7 Pierre Robin patients was found to be proportionately similar to that observed in healthy control subjects age 0 months (N = 100), 6 months (N = 42), and 1 year (N = 32).
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