Abstract：
BACKGROUND: Congenital orbital teratomas are extremely rare, usually benign neoplasms, comprised of cells originating from all three germ cell layers. Clinically the tumor appears solid, most of the times is intraconal and presents as a rapidly growing mass leading to a massive unilateral axial proptosis, chemosis, exposure keratopathy, markedly distended eyelids and often, loss of vision. To prevent these complications, tumor excision usually involves enucleation or even orbital exenteration.
METHODS: We report a case of a 1-day old infant who presented with dramatic proptosis at birth due to a true congenital orbital teratoma. We describe the clinical findings, the preoperative neuroimaging, the surgical management which included complete tumor resection with preservation of the globe to allow for optimal orbital growth, the histopathological evaluation, and the clinical course during 18 years of follow up.
CONCLUSIONS: Every effort to salvage the globe should be made to achieve the best possible orbito-facial development. Furthermore, the value of prompt surgical management with a less invasive transconjunctival globe sparing procedure can be appreciated in our case.
METHODS: We report a case of a 1-day old infant who presented with dramatic proptosis at birth due to a true congenital orbital teratoma. We describe the clinical findings, the preoperative neuroimaging, the surgical management which included complete tumor resection with preservation of the globe to allow for optimal orbital growth, the histopathological evaluation, and the clinical course during 18 years of follow up.
CONCLUSIONS: Every effort to salvage the globe should be made to achieve the best possible orbito-facial development. Furthermore, the value of prompt surgical management with a less invasive transconjunctival globe sparing procedure can be appreciated in our case.
摘要：
背景：先天性眼眶畸胎瘤极为罕见，通常是良性肿瘤，由来自所有三个生殖细胞层的细胞组成。临床上肿瘤呈实性,大部分时间是内的，并表现为快速增长的质量，导致大量的单侧轴向突出，化疗，暴露性角膜病变，眼睑明显扩张，经常，视力丧失。为了防止这些并发症，肿瘤切除通常包括眼球摘除甚至眼眶切除术。
方法：我们报告了一例1天大的婴儿，由于真正的先天性眼眶畸胎瘤，在出生时出现了剧烈的眼球突出。我们描述了临床发现，术前神经成像，手术治疗包括完整的肿瘤切除并保留眼球以实现最佳的眼眶生长，组织病理学评估，以及18年随访期间的临床过程。
结论：应尽一切努力挽救地球，以实现最佳的面部发育。此外，在我们的案例中，可以理解及时手术治疗和侵入性较小的经结膜眼球保留程序的价值。
方法：我们报告了一例1天大的婴儿，由于真正的先天性眼眶畸胎瘤，在出生时出现了剧烈的眼球突出。我们描述了临床发现，术前神经成像，手术治疗包括完整的肿瘤切除并保留眼球以实现最佳的眼眶生长，组织病理学评估，以及18年随访期间的临床过程。
结论：应尽一切努力挽救地球，以实现最佳的面部发育。此外，在我们的案例中，可以理解及时手术治疗和侵入性较小的经结膜眼球保留程序的价值。
