423 - Gingival Fibromatosis in a Pediatric Patient with Zimmermann-Laband Syndrome

Introduction: Zimmermann-Laband Syndrome is a rare genetic disorder. Research suggests that it is an autosomal dominant trait but some evidence shows autosomal recessive inheritance. The syndrome is characterized by craniofacial and hands and feet abnormalities. Facial abnormalities include narrow facial appearance with increases size of tongue, lips, nose, and/or ears. Children with Zimmermann-Laband Syndrome also commonly present with overgrowth of gingival tissue. This gingival hyperplasia may completely cover the teeth. This can cause malocclusion, chewing problems, excessive salivation, and/or difficulty swallowing. Treatment includes gingivectomy to uncover dentition with a likely future recurrence due to Zimmermann-Laband Syndrome.

 


Case Abstract:  A 5-year-old female presented to the dental clinic at Riley Hospital for Children for initial examination, routine recall examinations, and any necessary treatment. Her health history includes Zimmermann-Laband Syndrome and history of seizures. The patient is non-verbal with limited use of her extremities and in a wheelchair. She is taking oxcarbazepine for seizures and is allergic to amoxicillin and clindamycin. The patient had an initial exam in October 2019 and was monitored until her recall in April 2021. In April 2021, the patient’s mother reported difficulty eating with probable pain and only eating a soft diet due to gingival overgrowth in the upper right. The patient’s mother agreed to gingivectomy treatment under general anesthesia. Treatment was completed in the operating room at Riley Hospital for Children in December 2021. This report will include discussion of Zimmermann-Laband Syndrome along with treatment for hereditary gingival fibromatosis including differential diagnoses, radiographic and histological findings.