背景:畸胎瘤是最常见的先天性肿瘤,但是轨道位置很少见。它由外胚层组织组成,中胚层,和内胚层。
方法:先天性眼眶畸胎瘤通常表现为单侧眼球突出,随着快速增长,导致暴露性角膜病变。
方法:产前超声可以检测眼眶肿块,计算机断层扫描(CT)扫描,磁共振(MR)成像更好地显示多房性囊性和实性肿块,没有骨侵蚀。实验室检查应包括甲胎蛋白(AFP)和B-人绒毛膜促性腺激素(B-HCG),和组织病理学,它包含所有三个生殖细胞层组件。治疗方法是手术切除病灶,成熟的畸胎瘤有良性行为,未成熟者预后较差。我们描述了一个罕见的先天性眼眶畸胎瘤,病变颅内扩展,其中采用眼眶切除术治疗。手术后,AFP水平下降,中间工作面位移有所改善,发展里程碑适当。
BACKGROUND: Teratoma is the most common congenital tumor, but the orbital location is rare. It is composed of tissues from ectoderm, mesoderm, and endoderm.
METHODS: Congenital orbital teratoma commonly presents as unilateral proptosis, with rapid growth, leading to exposure keratopathy.
METHODS: Prenatal ultrasound may detect the orbital mass, computed tomography (CT) scans, and magnetic resonance (MR) imaging are better in demonstrating multilocular cystic and solid mass, without bone erosion. Laboratory tests should include alfa-fetoprotein (AFP) and B-human chorionic gonadotropin (B-HCG), and histopathologically, it contains all three germ cell layers components. The management is surgical removal of the lesion, the mature teratoma has a benign behavior, and the immature has a poor prognostic. We describe a rare
case of congenital orbital teratoma with intracranial extension of the lesion, in which was treated with orbital exenteration. After surgery, AFP levels decreased, the middle face displacement has improved and development milestones were appropriate.