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A comparative clinical study of Pierre Robin syndrome and isolated cleft palate
1Division of Oral Surgery, Faculty of Dental Sciences, University of Peradeniya, Sri Lanka.
Insights
This study compared infants with Pierre Robin syndrome and isolated cleft palate, finding shared traits like female preponderance and certain malformations. However, the findings do not definitively support theories on the cause of Pierre Robin syndrome.
Area of Science:
- Craniofacial anomalies
- Pediatric genetics
- Developmental biology
Background:
- Pierre Robin syndrome is a condition characterized by a specific set of facial abnormalities.
- Isolated cleft palate is a common congenital condition affecting the palate.
- Understanding the relationship between these conditions is crucial for diagnosis and treatment.
Purpose of the Study:
- To compare the clinical features of infants with Pierre Robin syndrome and those with isolated cleft palate.
- To investigate potential associations and etiological factors shared between Pierre Robin syndrome and isolated cleft palate.
- To evaluate the prevalence of specific malformations and dental anomalies in both groups.
Main Methods:
- Retrospective comparison of 37 infants with Pierre Robin syndrome and a cohort with isolated cleft palate.
- Analysis of craniofacial skeletal relationships, specifically jaw relation (Skeletal I vs. Skeletal II).
- Assessment of associated malformations (e.g., hypertelorism, strabismus) and hypodontia frequency.
Main Results:
- A female preponderance was observed in both Pierre Robin syndrome and isolated cleft palate groups.
- Skeletal II jaw relation was present in 32% of isolated cleft palate cases.
- Associated malformations and hypodontia were more frequent in Pierre Robin syndrome and in isolated cleft palate with Skeletal II jaw relation compared to isolated cleft palate with Skeletal I jaw relation.
Conclusions:
- While an association between Pierre Robin syndrome and isolated cleft palate is suggested by shared features, the findings are insufficient to support existing etiological hypotheses for Pierre Robin syndrome.
- Further research is needed to elucidate the complex etiology of Pierre Robin syndrome.
- The study highlights overlapping clinical presentations that warrant careful differential diagnosis.
Abstract:
Thirty seven infants with Pierre Robin syndrome were compared with children who had isolated cleft palate. A female preponderance was seen in both groups. Skeletal II jaw relation was observed in 32% of the children with isolated cleft palate. Associated malformations such as hypertelorism and strabismus were more common in infants with Pierre Robin syndrome and also in those with isolated cleft palate combined with a skeletal II jaw relationship when compared with infants who had cleft palate with a skeletal I jaw relationship. The frequency of hypodontia also was greater in the former two groups than in the latter. Furthermore, no difference was found in the frequencies of U- and V-shaped clefts. The frequencies of near relatives with clefts were also not different between groups. Whilst it may be possible to observe from these findings an association between Pierre Robin syndrome and isolated cleft palate, it is difficult to conclude that they support any of the postulates regarding the aetiology of Pierre Robin syndrome.
