Unique Dental and Craniofacial Manifestations of Hypoplastic Amelogenesis Imperfecta in a Patient With Prune Belly Syndrome: A Rare Case Report.

Introduction

Prune belly syndrome (PBS) is a rare congenital disease characterized by hypoplastic abdominal wall muscles, urological abnormalities, and bilateral cryptorchidism. This report describes a rare case of aberrant hypoplastic amelogenesis imperfecta in a patient with PBS. Case Presentation: A 10-year-old boy with PBS presented with difficulties in speech and mastication. Oral and radiological examinations revealed aberrant hypoplastic enamel defects, mandibular hypoplasia and retrusion, maxillary constriction, and anterior open bite.

Conclusion

Although dental manifestations are not frequent in PBS, rare cases may present with aberrant hypoplastic amelogenesis imperfecta.