Pars Plana Vitrectomy and Evisceration Resulting in Death Due to Misdiagnosis of Retinoblastoma in Children

Abstract

Retinoblastoma is a curable intraocular malignancy in children. However, in clinical practice, retinoblastoma can sometimes be misdiagnosed and mismanaged, leading to extraocular extension and even death. In this report, a series of 3 cases are related that emphasize the conditions and consequences resulting from misdiagnosis and mismanagement of retinoblastoma. The clinical features, imaging findings, histopatholigical examination, and management in 3 case reports of children with misdiagnosed retinoblastoma are presented. Two of the cases received pars plana vitrectomy after being misdiagnosed with Coats disease or ocular blunt trauma, whereas the third case received evisceration after being misdiagnosed with suppurative endophthalmitis. When the diagnosis of retinoblastoma had been confirmed after a second surgery was performed in our hospital, only 2 of the cases received adjuvant orbital radiotherapy. All 3 cases died of systemic tumor metastases. Intraocular surgical procedures should be avoided in any equivocal case until the possibility of latent retinoblastoma is eliminated.We strongly recommend that early enucleation be executed as soon as possible followed by postoperative adjuvant therapy under conditions wherein an intraocular surgery was inadvertently performed in an eye with retinoblastoma.

Long-Term Clinical Results and Management following Vitrectomy in Undetected Retinoblastoma Eyes

“…One child was suffering from metastatic disease on referral, the others were well and free of metastases with a mean follow-up of 7 years. A similar study was presented in 2015 by Shen et al [10] where they published a review of 3 cases of pars plana vitrectomy and evisceration in misdiagnosed retinoblastoma eyes. Again, the children were older than expected for unilateral retinoblastoma with 4.3 years on average.…”

“…It is well known that a delay in treatment, with the risk of tumour cells invading the CNS, regional lymph nodes, bones or bone marrow, or mismanagement of retinoblastoma eyes worsens the prognosis of the young patients considerably [10]. In the presence of metastatic disease or tumour cell spread to the CNS, prognosis is poor, and most patients die of disease progression despite aggressive treatment [17].…”

“…It is widely accepted that a vitrectomy or biopsy in eyes with uncertain diagnosis in children should be avoided until the possibility of an underlying retinoblastoma has been excluded, as it can cause extraocular spread along the needle tract or vitrectomy sites [8-10].…”

<p>Head and Eye Trauma Before Retinoblastoma Diagnosis</p>

“…In recent years, researchers have reported a decrease in highly advanced tumors and tumors with a poor prognosis that has been attributed to early recognition and less mistreatment of these conditions 4. However, a clear-cut history of trauma may also mask the diagnosis of RB in patients with various ocular complications, such as anterior chamber hemorrhage,5 secondary glaucoma,6 panophthalmitis,7 and traumatic cataract,8 and mislead the clinician, causing them to take inappropriate management measures, including cataract surgery,8 vitrectomy,9,10 and trabeculectomy 9. Unfortunately, a traumatic history not only obscures the diagnosis of RB but may also lead to cases not diagnosed based on the tumor presentation being a diagnosis in the advanced stages of the diseases.…”

MR Imaging Features to Differentiate Retinoblastoma from Coats’ Disease and Persistent Fetal Vasculature

“…Although differentiation between retinoblastoma and pseudoretinoblastoma can be challenging [3][4][5][6][7][8][9][10][11][12], it is vital for initiating adequate treatment. A missed retinoblastoma diagnosis can cause life-threatening treatment delay, while a false-positive retinoblastoma diagnosis can induce mistreatment including chemotherapy [7][8][9][10][11][12]. Additionally, correct classification can reduce unnecessary enucleations, as 5-12% of suspected retinoblastoma appear to be pseudoretinoblastoma upon histopathologic assessment [13,14].…”