Pars plana vitrectomy and endoresection for retinoblastoma recalcitrant to focal and systemic therapy in an only remaining eye
Author Disclosure Block: K.A. Roelofs: None. S. Soliman: None. B. Gallie: None. J. Zhao: None. O. Li: None. S. Wu: None. L. Jin: None. K. Kimani: None.
Abstract Body:
Purpose: To describe a case of recalcitrant retinoblastoma in an only remaining eye managed with careful pars plana vitrectomy (PPV), endoresection, and silicone oil tamponade.
Study Design: Case report
Methods: The clinical course of child with retinoblastoma from Kenya was reviewed.
Results: In July 2013, a 4-month old female in Nairobi, Kenya, was diagnosed with bilateral retinoblastoma. Her right eye underwent prompt enucleation. Pathological examination identified massive choroidal invasion with optic nerve involvement past the lamina cribosa but not extending to the transected portion of the nerve (pT3a). The child received 6 cycles of systemic chemotherapy following the vincristine - etoposide - carboplatin (VEC) protocol and the remaining left eye was concurrently treated with laser 8 times between August 2013 and December 2014. In June 2015, florid intraocular tumour recurrence was noted and the child was re-started on systemic chemotherapy. She subsequently received 6 additional cycles of VEC between June and September 2015. There was a poor response to chemotherapy, and enucleation of the remaining left eye was the only option left. Because endoresection by PPV was available in China, the child was referred to QuanZhou Children’s Hospital for a second opinion, and in January 2016, the team proceeded with PPV, endolaser to reinforce the barrier laser and endoresection of the tumor base. Intraoperatively, melphalan 5μg/ml was used in the irrigation fluid. Silicone oil was injected. Incision ports were closed with 6-0 absorbable sutures and 0.2 ml melphalan (5 μg, 25 μg/ml) was injected subconjunctivally at the three port sites. In April, 2016 the silicone oil was removed and at most recent follow up in November 2017, the eye is stable, and the child is systemically well with 6/6 vision in her only remaining eye.
Conclusions: Retinoblastoma treatment prioritizes first and foremost, saving the child’s life, second salvaging the eye and finally, optimizing vision. Following the success and safety of intravitreal chemotherapy, PPV and endoresection for selected eyes with tumor recalcitrant to any conventional therapy, may be useful in only remaining eyes.
