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International Poster Journal of Dentistry and Oral Medicine
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Int Poster J Dent Oral Med 19 (2017), No. 3     15. Sep. 2017
Int Poster J Dent Oral Med 19 (2017), No. 3  (15.09.2017)

Poster 1139, Language: German/English


Prosthetic Challenges in a 17-Year-old Female Patient With Treated Embryonic Rhabdomyosarcoma of the Fossa Pterygopalatina
Benz, Korbinian / Kozmacs, Carla / Jackowski, Jochen
Introduction: Rhabdomyosarcoma (RMS) develops as a malignant soft tissue tumour from the cells of the cross-striated muscle. For the under 15-year-olds, the annual incidence is estimated at 1: 224,000. The median age at diagnosis is 5 years. Rhabdomyosarcoma can occur anywhere in the body, even in areas where there is no cross-strained muscle. The head and neck area is affected in 40% of cases, with the orbitae and the paranasal sinus being the most common areas. Cause and origin are still largely unknown. The sequence and the execution of the individual types of therapy depend on the tumour- and patient-induced risk factors.
Case report: In a 17-year-old female patient at the age of 3 years, a rhabdomyosarcoma in the right pterygopalatine fossa was diagnosed with intracranial spreading tendency, which was successfully treated by excision with subsequent radiotherapy and polychemotherapy. As a result, there was complete anesthesia in the supply area of the right trigeminal nerve and visual loss of the right eye. The growth of the skull developed on the irradiated side less than on the unaffected side; the maxilla and mandibula are micrognathic. From the point of view of the positional relationship of both jaws, an Angle Class III was found. The patient also complained about the intraoral situation, which had existed for years, since all teeth had a degree of relaxation of II-III, resulting in severe ingestion. In addition, there was pain in the area of the temporomandibular joints and the masticatory muscles. Radiographically, the panoramic view showed multiple ageneses of the permanent teeth and rudimentary apices. The capites mandibulae and coronoid processes appeared degenerated and flattened. In addition to producing an acceptable functionality, the patient also wishes to improve the aesthetic appearance.
Method: After orthodontic and oral-maxillofacial surgery, a reconstruction osteotomy in combination with implant-mediated rehabilitation was considered not indicated because of the unpredictable healing tendency. Thereupon, a bite elevation was initiated in the patient with removable bite splints, in order to potentially achieve intercuspidation and to evaluate the resilience of the temporomandibular joints, the muscles, and the teeth. After 6 months of easy wearing time, a removable interim denture based on a deep drawing splint was inserted into the upper and lower jaw. The teeth still present were used to guide the patient during incorporation and to achieve a slight friction. After 3 months of wearing this in combination with logopedic support, the patient reported a significant improvement, especially in the social environment due to improved aesthetics. However, step-by-step corrections in the areas of ingestion and language have been announced for the coming months. As a definitive supply variant, we are striving for a supply by means of galvanic telescopes.
Summary: Since rhabdomyosarcomas are located in sensitive body regions (e.g. head and neck area), the treatment is sophisticated and multidisciplinary. The aim of the therapy is the oral rehabilitation of the young patient in the sense of personalized medicine.

Keywords: embryonic rhabdomyosarcoma, oral rehabilitation

Conference/Exhibition:
66. Jahrestagung der Deutschen Gesellschaft für Prothetische Zahnmedizin und Biomaterialien e. V. (DGPro)
19.-20. May 2017
Gießen, Germany