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<p>Congenital orbital teratomas are extremely rare germ cell tumors composed of tissues derived from all three embryonic germ layers (1–6). They typically present at birth or early infancy with rapidly progressive unilateral proptosis and can result in severe visual morbidity. We report a case of a 14-month-old male child presenting with progressive axial non-pulsatile proptosis of the right eye since birth. Neuroimaging revealed a large mixed solid-cystic retro-orbital lesion compressing the optic nerve. The child underwent right fronto-orbital craniotomy with gross total excision of the lesion. Histopathological examination confirmed a mature orbital teratoma. Postoperative recovery was uneventful. This case highlights the diagnostic challenges of congenital orbital teratomas, emphasizes the role of early surgical intervention, and reviews current literature regarding clinical presentation, imaging characteristics, histopathology, and management strategies.</p>

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Congenital orbital teratoma presenting as progressive proptosis in an infant: a case report and review of literature

  • 期刊论文

  • 发表日期：

    2026年4月26日

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  • Sarvesh Goyal,
  • Shubhi K.,
  • Hitesh Kumar Gurjar,
  • Amita Deshpande,
  • Swati Mahajan

摘要

Congenital orbital teratomas are extremely rare germ cell tumors composed of tissues derived from all three embryonic germ layers (1–6). They typically present at birth or early infancy with rapidly progressive unilateral proptosis and can result in severe visual morbidity. We report a case of a 14-month-old male child presenting with progressive axial non-pulsatile proptosis of the right eye since birth. Neuroimaging revealed a large mixed solid-cystic retro-orbital lesion compressing the optic nerve. The child underwent right fronto-orbital craniotomy with gross total excision of the lesion. Histopathological examination confirmed a mature orbital teratoma. Postoperative recovery was uneventful. This case highlights the diagnostic challenges of congenital orbital teratomas, emphasizes the role of early surgical intervention, and reviews current literature regarding clinical presentation, imaging characteristics, histopathology, and management strategies.