[Orbital tumor emergencies in childhood].
Emergencies in childhood orbital tumorals are rare. The absolute emergency involves malignant primary orbital tumors, such as rhabdomyosarcoma or secondary malignant tumors (metastatic neuroblastoma, leukemia), involving a vital prognosis requiring prompt diagnosis. Delayed emergencies are usually vascular lesions. Among these lesions, immature orbital hemangioma, with a good prognosis, must be distinguished from orbital adnexal lymphangiomas, which are less frequent but can lead to dramatic cosmetic and functional disorders. In rare cases, they can be responsible for sudden, painful proptosis, due to orbital hemorrhage, with a risk of optic nerve compression, requiring emergency surgical treatment. Neurogenous lesions, either isolated, such as in gliomas, or associated with a systemic disease, such as Recklinghausen neurofibromatosis, threaten the functional prognosis. Diagnosis of pediatric orbital tumors is based on a good clinical examination, precise imaging investigations, and evaluation of the locoregional extension of the tumor. Biopsy is required in emergency situations, when rhabdomyosarcoma is suspected, in order to start the chemotherapy. However, the biopsy can be superfluous, and even useless or dangerous, when clinical and imaging investigations are sufficient to provide a diagnosis of capillary hemangioma, lymphangioma, or metastatic tumor from an abdominal malignancy. Treatment is closely related to the etiopathogenesis of the tumor. The outcomes are vital, functional and cosmetic. They may require orbital surgery (biopsy, tumoral resection, orbital decompression in case of a compressive hemorrhage), systemic corticotherapy (as in immature adnexal hemangioma), radiation, and chemotherapy (rhabdomyosarcoma, secondary malignant tumor). These diseases require a pediatric ophthalmological medical center specializing in orbital surgery, with close collaboration of multiple specialists such as onco-pediatricians and neurosurgeons.
Morax
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Service d'ophtalmologie et de chirurgie plastique et reconstructive ophtalmologique, Fondation Ophtalmologique A. de Rothschild, Paris, France. vdauvin@fo-rothschild.fr
Desjardins
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L
D016888:Angiodysplasia / Q000175:diagnosis / Q000628:therapy; D002648:Child; D004630:Emergencies; D006801:Humans; D008202:Lymphangioma / Q000175:diagnosis / Q000628:therapy; D009918:Orbital Neoplasms / Q000175:diagnosis / Q000628:therapy; D012208:Rhabdomyosarcoma / Q000175:diagnosis / Q000628:therapy