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Midface Hypoplasia and Cranial Base Morphology in Syndromic Craniosynostosis: A Comparative Analysis Study Using a Predictive Regression Model
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Craniofacial characteristics evidenced in Bartsocas-Papas syndrome from birth to five years. Case report

Anthony L Maganzini1, Andrea Rios, Alan Shanske

  • 1Montefiore Medical Center, University Hospital for the Albert Einstein College of Medicine, Bronx, USA.

The New York State Dental Journal
|August 24, 2006
PubMed

Insights

Bartsocas-Papas Syndrome (BPS) is a rare congenital disorder often leading to early death. This case highlights often-overlooked oral, facial, and dental anomalies in a BPS patient and their impact on facial growth.

Area of Science:

  • Genetics and Developmental Biology
  • Pediatric Medicine
  • Craniofacial Anomalies

Background:

  • Bartsocas-Papas Syndrome (BPS) is a rare congenital disorder characterized by severe physical anomalies, with a high rate of intrauterine or neonatal death.
  • Only 26 cases of BPS have been reported since its initial description in 1972, making it exceptionally rare.
  • Commonly reported BPS features include limb webbing (pterygia) and syndactyly, but craniofacial and dental aspects are less frequently documented.

Observation:

  • This case report details the oral, facial, cranial, and dental anomalies in a 5-year-old patient diagnosed with Bartsocas-Papas Syndrome.
  • The study focuses on anomalies that are often overlooked in the broader description of BPS.
  • The patient's specific craniofacial and dental presentation is examined in relation to overall facial development.

Findings:

  • The case highlights significant oral, facial, cranial, and dental anomalies associated with Bartsocas-Papas Syndrome.
  • These anomalies have a notable effect on the facial growth patterns of affected individuals.
  • The findings underscore the importance of evaluating craniofacial structures in BPS patients.

Implications:

  • Recognizing and addressing craniofacial and dental anomalies is crucial for comprehensive BPS patient care.
  • Further research into the specific mechanisms causing craniofacial abnormalities in BPS is warranted.
  • This case contributes to a deeper understanding of the phenotypic variability and long-term consequences of Bartsocas-Papas Syndrome.

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