A Case of Odontogenic Keratocyst with Nevoid Basal Cell Carcinoma that Was Treated by Endoscopic Endonasal Surgery

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  • Morishita Hiroyuki
    Department of Otorhinolaryngology-Head and Neck Surgery, Mie University Graduate School of Medicine
  • Kubo Hisami
    Department of Otorhinolaryngology-Head and Neck Surgery, Mie University Graduate School of Medicine
  • Kato Chiaki
    Department of Oral and Maxillofacial Surgery, Department of Clinical Sciences, Medical Life Science, Mie University, Graduate School of Medicine
  • Kurohara Kazuto
    Department of Oral and Maxillofacial Surgery, Department of Clinical Sciences, Medical Life Science, Mie University, Graduate School of Medicine
  • Takeuchi Kazuhiko
    Department of Otorhinolaryngology-Head and Neck Surgery, Mie University Graduate School of Medicine

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  • 基底細胞母斑症候群に伴う上顎骨歯原性角化嚢胞に対し鼻内内視鏡下に嚢胞を開窓した例

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<p>Nevoid basal cell carcinoma syndrome (NBCCS, Gorlin Syndrome) is a rare disease that was first reported by Gorlin in 1960. It is a syndrome characterized by abnormalities, such as palmar/plantar pits, calcification of the falx cerebri, and congenital malformations, as well as susceptibility to tumorigenesis in multiple basal cell carcinomas and odontogenic keratocysts of the jaw. The patient was a 14-year-old boy who had been aware of swelling of the left cheek one week before the first visit. He visited a nearby otolaryngology clinic and was referred to our hospital for further examination. He had a history of surgery for cleft lip and palate, and he exhibited swelling of the upper left maxilla, palmar pits, and hypertelorism of the orbit, which are characteristic of NBCCS. CT and MRI showed multiple cystic lesions in the upper and lower jawbones, and calcification of the falx cerebri. Based on the above findings, a definitive diagnosis of NBCCS was made, and surgery was performed on the keratocyst of the symptomatic upper left maxilla. The surgery was performed with endonasal endoscope in consideration of the preservation of postoperative function. First, the keratocyst was opened from the middle nasal meatus through the maxillary sinus, and a second hole was then created via the inferior nasal meatus. After the operation, the swelling was alleviated, the pressure symptoms disappeared, and the fenestration to the keratocyst was maintained. While NBCCS is a rare disease, it is important to understand the symptoms and diagnostic criteria of the disease for early diagnosis. In addition, for odontogenic keratocysts that occur in the maxilla, endoscopic endonasal surgery can improve the symptoms while preventing a decrease in the quality of life.</p>

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