Primary orbital retinoblastoma has been managed in the past with orbital exenteration, chemotherapy, or external beam radiotherapy exclusively or in sequential combination, with variable results [18–23]. It is well known that local treatments have a limited effect on the course of orbital retinoblastoma. Orbital exenteration alone is unlikely to achieve complete surgical clearance and preclude secondary relapses and systemic metastasis; external beam radiotherapy will not affect systemic micrometastasis; and chemotherapy alone may not eradicate residual orbital disease [21, 22]. Therefore, multimodal therapy with a judicious, customized, and sequential combination of neoadjuvant and adjuvant chemotherapy, surgery, and EBRT is considered to be more effective. In a case series of five children, Goble and associates demonstrated long-term survival with local surgical excision, orbital radiotherapy, and systemic chemotherapy [21].

We have developed a treatment protocol (Table 17.1) consisting of triple-drug (vincristine, etoposide, and carboplatin) high-dose neoadjuvant chemotherapy (3–6 cycles) followed by surgery (enucleation, extended enucleation, or orbital exenteration as appropriate after determining the extent of residual orbital tumor by CT scan), orbital radiotherapy, and adjuvant chemotherapy (Table 17.2) [24, 25]. In all, 12 cycles of chemotherapy are administered.

Our treatment protocol outlined for primary orbital retinoblastoma currently under evaluation for secondary orbital retinoblastoma and early results have been very encouraging. Surgical intervention in such cases may be limited to excision of the residual orbital mass or an orbital exenteration depending on the extent of the residual tumor after the initial 3–6 cycles of high-dose neoadjuvant chemotherapy. Surgery is not necessary if the orbital tumor completely resolves following neoadjuvant chemotherapy. Treatment is completed with orbital EBRT and chemotherapy for a total of 12 cycles.

The surgeon should be careful not to accidentally perforate the eye during enucleation for retinoblastoma. Many surgeons prefer to avoid traction sutures applied at the insertion of extraocular muscles to minimize the risk of accidental perforation. Instead, hemostat applied to medial or lateral rectus muscle stump or cryoprobe applied at the limbus provides adequate traction. Eyes manifesting tumor necrosis with aseptic orbital cellulitis pose specific risk for accidental perforation. Surgery in such eyes is best performed when the inflammation is resolved. A brief course of preoperative oral and topical steroids helps control inflammation. If inadvertent perforation does occur during enucleation, further steps of surgery should be performed carefully, with minimal manipulation, under good illumination and magnification, and preferably by a senior surgeon. If the perforation is small, orbital contamination can be limited by sealing the perforation site with a patch of Tenons glued into position with cyanoacrylate glue. Larger perforations can be handled by isolating the area with dry absorbent cotton, suturing the perforation if possible and sealing the suture site with a glued-on Tenon’s patch. Extensive perforations can be managed by isolating the area with dry absorbent cotton and suction evacuation of tumor tissue prolapsing through the wound using a powered suction, followed by wound suturing and glued-on Tenon’s patch. In all these situations, enucleation is completed as planned with minimal manipulation. Integrated orbital implants are best avoided, and a polymethyl methacrylate or silicone implant is preferred in such cases, since there would be an impending need for adjuvant radiotherapy.