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Exploding retinoblastoma- Management of spontaneous scleral rupture in an infant with bilateral retinoblastoma
Suganeswari Ganesan1, Henna V Valakkadavil1, Charanya C1
1Shri Bhagwan Mahavir Vitreoretinal services, Sankara Nethralaya,Chennai, Tamil Nadu, India.
Insights
Spontaneous globe rupture is a rare retinoblastoma complication. Management involved enucleation, chemotherapy, and radiotherapy, with no recurrence after two years.
Area of Science:
- Ophthalmology
- Pediatric Oncology
Background:
- Retinoblastoma (RB) is the most common primary intraocular malignancy in children.
- Spontaneous globe rupture is an extremely rare but severe complication of RB.
Purpose of the Study:
- To report a case of spontaneous globe rupture in an infant with bilateral retinoblastoma.
- To discuss the management challenges and outcomes of this rare complication.
Main Methods:
- A case of an infant with bilateral retinoblastoma undergoing neoadjuvant chemotherapy (VEC regimen) who developed spontaneous globe rupture.
- Management included urgent enucleation, followed by adjuvant chemotherapy and radiotherapy.
- Metastatic workup was performed post-enucleation.
Main Results:
- The infant presented with spontaneous globe rupture in the more advanced eye after conjunctivitis during chemotherapy.
- Post-enucleation metastatic workup was negative.
- The remaining eye showed good regression of lesions with treatment, maintaining a Cardiff acuity of 6/24.
- No recurrence was observed during a 2-year follow-up period.
Conclusions:
- Spontaneous globe rupture is an exceptionally rare complication of retinoblastoma, with only two cases reported globally.
- Potential contributing factors include advanced disease, high intraocular pressure, chemotherapy-induced immunosuppression, and superimposed infection.
- This complication poses a significant risk of life-threatening metastasis.
Purpose:
To describe a case of spontaneous globe rupture in an infant with bilateral retinoblastoma (RB) and the challenges faced during its management.
Methods:
An infant with bilateral retinoblastoma on treatment, presented with spontaneous globe rupture in the eye with advanced disease, after an episode of conjunctivitis while on neoadjuvant chemotherapy with VEC (vincristine, etoposide and carboplatin) regimen, and was managed by urgent enucleation followed by chemotherapy and radiotherapy.
Results:
Metastatic workup, post enucleation was negative, and she had good regression of lesions in the other eye with chemotherapy and maintains a Cardiff acuity of 6/24. Follow up of 2 years showed no recurrences.
Conclusion:
Spontaneous globe rupture is an exceptionally rare complication of retinoblastoma, this being the second reported case worldwide. Advanced disease stage, high intraocular pressure, chemotherapy induced immunosuppression, and superadded infection could all be the possible etiologies in this case. This condition carries a significant risk of life-threatening metastasis.
