新生儿颅基畸胎瘤的外科治疗及舌瓣腭部重建:17年随访1例

Sylvio Luiz Costa De-Moraes, R. D. Fares, Alexandre Maurity de Paula Afonso, R. Mattos, Roberto Gomes dos Santos, Rayssa Lima Pereira, J. Ribeiro da Silva
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摘要

表ignathus或Tessier裂隙0-14是一种罕见的畸胎瘤,由三个胚胎胚层组成。临床表现为覆盖皮肤和粘膜的肿块,起源于多能干细胞。根据世界卫生组织的最新分类,畸胎瘤是鼻窦区主要的良性生殖细胞肿瘤,其组织学特征与性腺和其他生殖腺外部位的畸胎瘤相似。本研究的目的是提出一个病例的表腺肌起源于颅底的白人女性患者腭裂后的尖锐孔,不影响鼻底。体检时,经口内路径,在颅底以下区域,发现一广泛的实性肿块,被黏膜覆盖,并有毛发。计算机断层扫描证实了病变,位于患者颅底相应的区域。第一次手术切除发生在患者生命的第40天,在全身麻醉下,随后初级关闭腭缺损。第二次手术是在10个月后进行的,当时在上颚使用组织扩张器来增加粘膜表面。5岁时,患者接受了舌背带前蒂舌瓣手术。患者保持镇静和松弛10天,以保持皮瓣的自主性。术后进行舌背皮瓣的切除和舌背重建。患者病情发展良好,继续接受门诊治疗。儿科医生和儿科牙医应该意识到产前检查和出生后不久的重要性,以识别新生儿患者可能的畸形,这有利于诊断。术后17年的随访,手术切除畸胎瘤并重建腭裂,结合使用腭裂组织扩张器和舌背皮瓣,证明是改善患者生活质量的可行资源。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Surgical Treatment of the Cranial Basis Teratoma in a Newborn and Palatal Reconstruction With Tongue Flap a 17-Years Follow-Up: A Case Report
Epignathus or Tessier fissure 0-14 is a rare teratoma composed of the three embryonic germ layers. Clinically, it appears as a mass covered with skin and mucosa, originating from pluripotential stem cells. According to the latest classification by the World Health Organization teratoma represents the main benign Germ cell tumors of the sinus region and presents similar histological aspects with its counterparts in the gonads and other extragonadal sites. The purpose of this study is to present a case of epignathus originating from the base of the skull in a Caucasian female patient with a cleft palate after the incisive foramen, not affecting the nasal floor. During physical examination, via intraoral route, in the region below the base of the skull, an extensive solid mass, covered by mucosa and with the presence of hair. Computed tomography confirmed the lesion, in the region corresponding to the base of the patient’s skull. The first surgical approach of resection occurred at the 40th day of the patient’s life, under general anesthesia and subsequent primary closure of the palatal defect. The second surgery was performed 10 months later, when tissue expanders were used on the palate to increase the mucosal surface. At the age of 5, the patient underwent surgery with a tongue flap, from the lingual dorsum, with an anterior pedicle. The patient was kept under sedation and relaxation for a period of 10 days for flap autonomy. After this period, the section of the flap and the reconstruction of the tongue dorsum were performed. The patient had a good evolution and was maintained under outpatient treatment. Pediatricians and pediatric dentists should be aware of the importance of prenatal exams and soon after birth, to identify possible deformities in neonatal patients, which favors the diagnosis. The 17-year postoperative follow-up of complete surgical removal of the teratoma and reconstruction of the cleft palate, combining the use of a tissue expander on the palate and a flap on the dorsum of the tongue, proved to be a viable resource for improving the patient’s quality of life.
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