Kufta Kenneth, Kang Steve, Alawi Faizan, Moran Anna, Panchal Neeraj
a DMD -- Resident, Department of Oral and Maxillofacial Surgery , University of Pennsylvania Health System.
b BS - Dental Student , University of Pennsylvania School of Dental Medicine.
Fetal Pediatr Pathol. 2017 Oct;36(5):416-422. doi: 10.1080/15513815.2017.1324547. Epub 2017 May 30.
Pierre Robin sequence (PRS) is a rare disorder classically observed as a triad of features including micrognathia, glossoptosis, and upper airway obstruction. It is associated with a syndrome in about 60% of cases. While odontogenic tumors are common findings in patients with familial adenomatous polyposis and nevoid basal cell carcinoma syndromes, PRS has not been found to be consistently associated with any tumors of the jaw.
The current report aims to describe a patient with PRS who presented with an extensive ameloblastic fibro-odontoma (AFO) of the maxilla.
Continued observation for odontogenic tumors in PRS is necessary.
皮埃尔·罗宾序列征（PRS）是一种罕见的病症，典型表现为包括小颌畸形、舌后坠和上呼吸道梗阻在内的三联征。约60%的病例与一种综合征相关。虽然牙源性肿瘤在家族性腺瘤性息肉病和痣样基底细胞癌综合征患者中是常见表现，但尚未发现PRS与任何颌骨肿瘤存在持续关联。
本报告旨在描述一名患有PRS的患者，该患者上颌出现广泛的成釉细胞纤维牙瘤（AFO）。
对PRS患者的牙源性肿瘤进行持续观察很有必要。
