Retinoblastoma in patients with regressed retinopathy of ...
3
November - December 2011 501 Retinoblastoma in patients with regressed retinopathy of prematurity Usha Vasu, Suneetha Nithyanandam, Sharon D’Souza, Sripathi Kamath Retinopathy of prematurity (ROP) is a well-known clinical entity in premature babies. We report two patients (1 and 2) with regressed ROP who later presented with retinoblastoma (RB). To the best of our knowledge, there is only one such report in the literature so far. Two unrelated patients 1 and 2, born at 32 weeks gestation were screened for ROP at 34 weeks gestation. This showed Zone II Stage II ROP which regressed by 38 weeks of gestation on follow-up. Both patients were lost to follow-up by 40 weeks of gestation. They presented at four years of age with white reflex in the eye. Patient 1 was found to have unilateral and patient 2 bilateral RB. The occurrence of RB in these patients with regressed ROP is probably coincidental. Key words: Retinoblastoma, retinopathy of prematurity, risk factor Cite this article as: Vasu U, Nithyanandam S, D'Souza S, Kamath S. Retinoblastoma in patients with regressed retinopathy of prematurity. Indian J Ophthalmol 2011;59:501-2. Access this article online Quick Response Code: Website: www.ijo.in DOI: 10.4103/0301-4738.86322 PMID: *** Retinopathy of prematurity (ROP) and retinoblastoma (RB) are both well documented in the pediatric population. While the incidence and severity of ROP is inversely related to the weight and gestational age at birth, the occurrence of RB has no such prerequisites. RB is a rare tumor but is highly malignant and if undetected can cause significant visual morbidity and mortality. The occurrence of RB in eyes with previous ROP has scant mention in the available literature. Whether some factor known to cause ROP can also predispose the eye to RB is not yet known. We report two cases of RB in eyes with previous ROP. We wish to highlight that regular follow-up of ROP patients at least on a yearly basis, will help in early diagnosis and treatment of other vision-threatening problems in these patients. Case Reports Case 1 Department of Ophthalmology, St. John’s Medical College Hospital, Bangalore, India Correspondence to: Dr. Sharon D’Souza, Department of Ophthalmology, St. John’s Medical College Hospital, Sarjapur Road, Bangalore – 560 034, India. E-mail: [email protected] Manuscript received: 19.12.10; Revision accepted: 12.06.11 A male neonate born at 31 weeks of post-conceptional age and birth weight 980 g was admied to the neonatal ICU for care. There was no history of prolonged supplemental oxygen administration. First ROP screening at 33 weeks post conceptional age showed Stage I Zone II ROP, which later progressed to Stage II Zone II by 35 weeks. The ROP remained stable thereaſter till 38 weeks, then regressed without treatment. The last visit at 40 weeks of post-conceptional age showed bilateral regressed Zone II ROP. The parents noticed a white reflex in the child’s leſt eye, when the child was four and a half years old [Fig. 1]. On examination, the leſt eye showed a Group D tumor by the International Classification of Intraocular Retinoblastoma [Fig. 2] and this eye was enucleated. The right eye on examination under general anesthesia, with intraoperative 360 0 scleral indentation, was normal. The enucleated leſt eye on histopathological examination showed scleral infiltration with malignant cells. Hence this patient underwent chemotherapy (CT) and external beam radio therapy (EBRT) to the left anophthalmic socket. At four years follow-up the child is doing well with no tumor recurrence in the leſt socket and normal vision in the right eye. Case 2 A neonate was referred from the neonatal ICU (NICU) for ROP screening at 34 weeks post-conceptional age, whose gestational age was 32 weeks and birth weight was 1.4 kg. The child was treated in the NICU for respiratory infection and needed supplemental oxygen for two weeks. On examination, the child was found to have Zone II Stage II ROP, both eyes. The ROP started regressing aſter the 36 th week and the child was discharged. A visit at 38 weeks showed regressing ROP. The patient was subsequently lost to follow-up. After four years the child’s parents reported to the Ophthalmology OPD with a history of bilateral white reflex in the child’s eyes. On examination under general anesthesia, the right eye had Group B tumor by International Classification of Retinoblastoma. There were four tumors in the superotemporal quadrant and two in the inferonasal quadrant, with two of them being 6-10 mm in diameter. Another small tumor, 1-2 mm in diameter, was located 2 mm nasal to the fovea. The left eye showed Group D tumor by International Classification of Retinoblastoma with multiple tumors in the superotemporal, nasal and inferonasal quadrants, two of which were greater than 10 mm in diameter with a height reaching more than half the vitreous cavity. One of them showed vitreous seeding close to the tumor. As the leſt eye showed more advanced malignancy, the patient underwent leſt eye enucleation, and the enucleated eye on histopathological examination showed involvement of the cut end of the optic nerve with malignant cells. Hence antimetastatic chemotherapy and EBRT to the leſt socket was given. The right eye underwent chemoreduction and subsequent cryotherapy in three sessions [Fig. 3]. At four years follow-up the child is doing well with no tumor recurrence in the leſt socket and right eye, with normal vision in the right eye. Brief Communications
Transcript of Retinoblastoma in patients with regressed retinopathy of ...
November - December 2011 501
Retinoblastoma in patients with regressed retinopathy of prematurity
Usha Vasu, Suneetha Nithyanandam, Sharon D’Souza, Sripathi Kamath
Retinopathy of prematurity (ROP) is a well-known clinical entity in premature babies. We report two patients (1 and 2) with regressed ROP who later presented with retinoblastoma (RB). To the best of our knowledge, there is only one such report in the literature so far. Two unrelated patients 1 and 2, born at 32 weeks gestation were screened for ROP at 34 weeks gestation. This showed Zone II Stage II ROP which regressed by 38 weeks of gestation on follow-up. Both patients were lost to follow-up by 40 weeks of gestation. They presented at four years of age with white refl ex in the eye. Patient 1 was found to have unilateral and patient 2 bilateral RB. The occurrence of RB in these patients with regressed ROP is probably coincidental.
Key words: Retinoblastoma, retinopathy of prematurity, risk factor
Cite this article as: Vasu U, Nithyanandam S, D'Souza S, Kamath S. Retinoblastoma in patients with regressed retinopathy of prematurity. Indian J Ophthalmol 2011;59:501-2.
Access this article onlineQuick Response Code: Website:
www.ijo.in
DOI:10.4103/0301-4738.86322
PMID: ***
Retinopathy of prematurity (ROP) and retinoblastoma (RB) are both well documented in the pediatric population. While the incidence and severity of ROP is inversely related to the weight and gestational age at birth, the occurrence of RB has no such prerequisites. RB is a rare tumor but is highly malignant and if undetected can cause signifi cant visual morbidity and mortality. The occurrence of RB in eyes with previous ROP has scant mention in the available literature. Whether some factor known to cause ROP can also predispose the eye to RB is not yet known. We report two cases of RB in eyes with previous ROP. We wish to highlight that regular follow-up of ROP patients at least on a yearly basis, will help in early diagnosis and treatment of other vision-threatening problems in these patients.
Case ReportsCase 1
Department of Ophthalmology, St. John’s Medical College Hospital, Bangalore, India
Correspondence to: Dr. Sharon D’Souza, Department of Ophthalmology, St. John’s Medical College Hospital, Sarjapur Road, Bangalore – 560 034, India. E-mail: [email protected]
Manuscript received: 19.12.10; Revision accepted: 12.06.11
A male neonate born at 31 weeks of post-conceptional age and birth weight 980 g was admitt ed to the neonatal ICU for care. There was no history of prolonged supplemental oxygen administration. First ROP screening at 33 weeks post conceptional age showed Stage I Zone II ROP, which later progressed to Stage II Zone II by 35 weeks. The ROP remained stable thereaft er till 38 weeks, then regressed without treatment. The last visit at 40 weeks of post-conceptional age showed bilateral regressed Zone II ROP.
The parents noticed a white refl ex in the child’s left eye, when the child was four and a half years old [Fig. 1]. On examination, the left eye showed a Group D tumor by the International Classifi cation of Intraocular Retinoblastoma [Fig. 2] and this eye was enucleated. The right eye on examination under general anesthesia, with intraoperative 3600 scleral indentation, was normal. The enucleated left eye on histopathological examination showed scleral infi ltration with malignant cells. Hence this patient underwent chemotherapy (CT) and external beam radio therapy (EBRT) to the left anophthalmic socket. At four years follow-up the child is doing well with no tumor recurrence in the left socket and normal vision in the right eye.
Case 2A neonate was referred from the neonatal ICU (NICU) for ROP screening at 34 weeks post-conceptional age, whose gestational age was 32 weeks and birth weight was 1.4 kg. The child was treated in the NICU for respiratory infection and needed supplemental oxygen for two weeks. On examination, the child was found to have Zone II Stage II ROP, both eyes. The ROP started regressing aft er the 36th week and the child was discharged. A visit at 38 weeks showed regressing ROP. The patient was subsequently lost to follow-up.
After four years the child’s parents reported to the Ophthalmology OPD with a history of bilateral white refl ex in the child’s eyes. On examination under general anesthesia, the right eye had Group B tumor by International Classifi cation of Retinoblastoma. There were four tumors in the superotemporal quadrant and two in the inferonasal quadrant, with two of them being 6-10 mm in diameter. Another small tumor, 1-2 mm in diameter, was located 2 mm nasal to the fovea.
The left eye showed Group D tumor by International Classifi cation of Retinoblastoma with multiple tumors in the superotemporal, nasal and inferonasal quadrants, two of which were greater than 10 mm in diameter with a height reaching more than half the vitreous cavity. One of them showed vitreous seeding close to the tumor.
As the left eye showed more advanced malignancy, the patient underwent left eye enucleation, and the enucleated eye on histopathological examination showed involvement of the cut end of the optic nerve with malignant cells. Hence antimetastatic chemotherapy and EBRT to the left socket was given. The right eye underwent chemoreduction and subsequent cryotherapy in three sessions [Fig. 3]. At four years follow-up the child is doing well with no tumor recurrence in the left socket and right eye, with normal vision in the right eye.
Brief Communications
AzharS
Rectangle
502 Indian Journal of Ophthalmology Vol. 59 No. 6
DiscussionBoth the patients in this report had a similar stage of
ROP that regressed spontaneously and both presented approximately four years later with advanced RB. The occurrence of RB in eyes that had previously developed ROP could be just a coincidence as stated in the case report by Benz et al.[1] The report by Benz et al, is the only other report in the literature of these two serious retinal conditions occurring in the same eyes. The timing of occurrence of the two conditions are, however, a litt le diff erent. In the report by Benz et al., ROP and RB were detected simultaneously. This is in contrast to the patients in our report where ROP and RB were not related temporally, with RB lesions occurring many years aft er ROP
in both patients. The increased survival of preterm babies with bett er neonatal care has resulted in ophthalmologists being able to examine these babies as they grow older.[2,3] The awareness among the parents of the possible occurrence of ocular abnormalities in premature babies, facilitated by regular screening and patient education for ROP, led the parents to report to the treating ophthalmologist early when the white refl ex was noticed subsequently.
The occurrence of ROP and RB in the same eyes although rare and possibly coincidental, highlights the fact that these premature infants are not totally out of danger for developing other eye problems. In conclusion, we would like to emphasize that children with ROP should be followed up at least yearly to detect and treat other well-known ocular associations in premature infants including refractive errors, strabismus and rarely, glaucoma.[4,5]
References 1. Benz MS, Escalona EM, Murray TG. Simultaneous presentation of
retinopathy of prematurity and bilateral familial retinoblastoma in a premature infant. J Pediatr Ophthalmol Strabismus 2003;40:98-100.
2. Shenoi A, Prasad VSV. Research issues in low birth weight feeding. J Neonatol 2009;23:291-3.
3. Field DJ, Dorling JS, Manktelow BN, Draper ES. Survival of extremely premature babies in geographically defi ned population: Prospective cohort study of 1994-9 compared with 2000-5. BMJ 2008;336:1221.
4. Schalij -Delfosa NE, de Graafa ME, Treff ersa WF, Engelb J, Catsc BP. Long term follow up of premature infants: Detection of strabismus, amblyopia, and refractive errors. Br J Ophthalmol 2000;84:963-7.
5. Gallo JE, Holmstrom G, Kugelberg U, Hedquist B, Lennerstrand G. Regressed retinopathy of prematurity and its sequelae in children aged 5–10 years. Br J Ophthalmol 1991;75:527-31.
Figure 1: Clinical photograph of Case 1 showing leucocoria in the left eye
Figure 2: Fundus photograph of the left eye of Case 1 prior to enucleation showing large inferiorly located retinoblastoma
Figure 3: Fundus photograph of right eye of Case 2, showing regressed retinoblastoma with areas of retinal scarring surrounding the mass following cryotherapy
Copyright of Indian Journal of Ophthalmology is the property of Medknow Publications & Media Pvt. Ltd. and
its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's
express written permission. However, users may print, download, or email articles for individual use.
![M36 LED Regressed [L36J/L36JR1/L36JR2] selux](https://static.fdocuments.us/doc/165x107/61e088d582e45244d411b027/m36-led-regressed-l36jl36jr1l36jr2-selux.jpg)
![M100 LED Regressed [L100JR] selux](https://static.fdocuments.us/doc/165x107/61e088d582e45244d411b029/m100-led-regressed-l100jr-selux.jpg)
![The Guide - Diabetic Retinopathy - Vision Lossvisionloss.org.au/wp-content/uploads/2016/05/The... · the guide [diabetic retinopathy] What is Diabetic Retinopathy? Diabetic Retinopathy](https://static.fdocuments.us/doc/165x107/5e3ed00bf9c32e41ea6578a8/the-guide-diabetic-retinopathy-vision-the-guide-diabetic-retinopathy-what.jpg)
