Secondary chronic open-angle glaucoma after intravitreal triamcinolone acetonide.

American cooperative group in 1972, the Intergroup Rhabdomyosarcoma Study Group has undertaken a series of clinical trials that have employed chemotherapy, surgery, and irradiation for local control. The addition of systemic chemotherapy has markedly improved long-term survival in affected children. These children now have an excellent prognosis with current multimodality therapy. A recent review by Oberlin and associates demonstrates the 10year overall survival rate to be 87% regardless of initial approach to therapy (chemotherapy alone or chemotherapy with local irradiation). In response to the superior survival rates for patients with isolated orbital rhabdomyosarcoma, achieved during the first 2 generations of North American cooperative studies, subsequent efforts have focused on reducing the shortand long-term morbidity associated with therapy while maintaining a very high cure rate. Klippel-Trénaunay syndrome is a congenital vascular malformation characterized by 3 features: (1) a cutaneous vascular nevus (capillary malformation), (2) soft tissue or bony hypertrophy in the involved region, and (3) varicose veins or venous malformations. It is frequently associated with deep venous anomalies and lymphatic malformations. Common complications associated with KTS include pain in the affected extremity, thrombophlebitis, and cellulitis. Surgical therapy is ineffective, but pulseddye laser, hydrotherapy, and compression treatments can be helpful. The cause of KTS is unknown. Numerous vascular lesions have been associated with KTS, but malignancies are extremely rare. Lymphatic and venous malformations can be found either ipsilateral or contralateral to the affected site. Orbital lymphatic malformation would not be unexpected in such a setting. In this case, the presence of a URTI at the time of the initial examination gave further support for this diagnosis. Head and neck lymphatic malformations, including orbital lesions, typically enlarge rapidly during respiratory tract infections, presumably owing to infectious stimulation of the aberrant lymphatic tissue. Results of radiographic studies were equally misleading: the lesion molded to the globe, there was no bony erosion, and there were areas of heterogeneity (but no frank cystic or hemorrhagic areas). Corticosteroids are effective in reducing the size of inflamed lymphatic malformations in cases of spontaneous (nonhemorrhagic) enlargement or postoperative swelling and were, therefore, chosen as the initial therapy in this case. Medical treatment with early clinical reevaluation was chosen to avoid potentially unnecessary surgery. Four recognized experts in their respective fields (orbital surgery, vascular dermatopathology, pediatric otolaryngology, and head and neck radiology) were misled by these signs and symptoms. In the setting of an expected, benign vascular tumor, one must exclude all potential serious and malignant diagnoses. As demonstrated by our case, rhabdomyosarcoma of the orbit can exhibit clinical and radiographic features that may be misconstrued as a benign, lymphatic malformation.