|Year : 1968 | Volume
| Issue : 2 | Page : 76-78
Retinoblastoma in phthisis bulbi
IS Jain, Kapalmit Singh
Department of Ophthalmology, lnstitute of Post Graduate Medical Education and Research, Chandigarh, India
I S Jain
Department of Ophthalmology, lnstitute of Post Graduate Medical Education and Research, Chandigarh
|How to cite this article:|
Jain I S, Singh K. Retinoblastoma in phthisis bulbi. Indian J Ophthalmol 1968;16:76-8
Phthisis bulbi is not uncommonly encountered in children, one of the most important causes being retino blastoma. The case under study guides us, as to the approach to such a case. It also tells us that it is incumbant upon every ophthalmologist to observe the other eve of the patient.
Since Kneiper's report (1911) of regression of retinoblastoma, many cases have been added to the list. Majority of the cases described in literature are only clinical impressions. There are only a few cases on record that have been histologically proved.
It is because of the rarity of proven cases and the important lesson that such type of cases deliver, the present study is being put on record.
| Case report|| |
A nine months old female child was brought by parents with a shrunken left eyeball. For two months there were recurrent attacks of redness in the left eye, following which the eye got shrunk. Examination revealed that the left eye of the child was phthisical while the right eye was perfectly normal. Fundus examination did not reveal any abnormality in the right eye. The cause of phthisis of left eve was considered to be post inflamatory by majority of the surgeons. After being discharged, the patient was brought back after six months with a cat's eye reflex in the right eye. At this time the left eye was removed, and submitted to histopathology. The parents of the child were normal and there was no family history of retinoblastoma.
| Pathology|| |
Macroscopically the enucleated eye ball was very much shrunken. The cornea was 3.5 mm. both vertically and horizontally.
Microscopically, there was atrophy of all the structures of the eye ball. In the interior of the eye ball, internal to the choroid - there were groups of calcified retinoblastoma cells and fibrous tissue. Calcium deposition was very much marked. The individual cell necrosis could be seen. The tumour cells showed the characteristic picture of retinoblastoma cells. [Figure - 1],[Figure - 2].
At one place of the optic nerve near the lamina cribrosa, a small area of calcified retinoblastoma cells could be seen.
| Discussion|| |
Reviewing the literature one finds that regression of retinoblastoma is not very common, but as STEWARD  SMITH AND ARNOLD rightly observed that in some non-familial unilateral regressed retinoblastoma cases with phthisical eyes it might have been overlooked, otherwise if all phthisical eyes be examined then the incidence might be even more significant. All the same the end result of the regression is not always a phthisical eye. The following modes of regression are observed in literature:
(i) Phthisical eye following pain and redness (? inflammaton) in the eye - DeKLEIJN,  KNEIPER  DAS  , STEWARD ET AL  CARBAJAL  .
(ii) The tumour gets calcified -
(iii) The previous tumour site shows choroidal atrophy with a small grey, green or pink area showing calcification - HINE  .
The diagnosis of regression has been made either (1) on clinical grounds and follow up - KNEIPER  STALLARD  , HINE  CARBAJAL  or (2) it has been made from family history e.g. if either of the parents have a phthisical eye with suggestive regression and if the children develop retinoblastoma, then the diagnosis of regression in parents becomes certain - SOVIK  , STEWARD ET AL  , or (3) it is proved histologically wherein calcification, even bone formation is observed (DEKLEIJN  , STEWARD et al  , SHERMAN  , Das  .
Whether all the eyes where retinoblastoma presents as inflammation lead to phthisis- is. not definitely known. But one thing, definitely comes out in literature that those eyes that become phthisical do become irritable- at one stage or the other. On the other hand the visible tumours that undergo calcification under observation do not lead to phthisis of the eye. These are the tumours that apparently suffocate themselves to death while others make a-hue and cry before death. The latter category produce some inflammation probably toxic in nature. Two cases of gliomatous panophthalmitis reported by KHOSLA ANGRA & AGARWAL  do not answer the question whether all gliomatous inflammations lead to phthisis, because in their report one eye was eviscerated because of diagnosis of panophahnitis and the other was lost in follow up.
We do not agree with the cointention of DANIELSON  that retinoblastoma can only occur in a normal. sized eye with normal anterior chamber. SHERMAN  had stressed the fact that retinoblastoma can occur in a small sized eye and even in a phthisical eye. The case under discussion also supports this concept.
Two types of hypothesis have been put forward to explain the aetiology of regression. Either the tumour outgrows its blood supply leading to necrosis and production of toxic products, which further lead to necrosis and a vicious circle is set up, or, as suggested by STEWARD  one eye develops a sort of `resistance' following a tumour in the other eye.
Due to this 'resistance' according to him the tumour regresses.
The latter hypothesis might be applicable to types of cases where one eye is treated for retinoblastoma and the other eye shows regression later as is seen in large majority of cases reported in literature. But in our case one eye showed regression and then later the second eye developed the tumour. The former hypothesis seems to be more plausible, but it still remains an open question why some tumours outgrow their blood supply while others do not.
We agree with Reese and Sharman that any phthisical eye in a child must be removed. It also is incumbant upon the attending surgeon to keep under observation the other eye regularly for any appearance of retinoblastoma later.
| Summary|| |
A nine months old baby, had phthisis of left eye, with a history suggestive of some inflammation, preceding the shrinkage of the eyeball. A year later the right eye which was normal when the baby was first seen, showed a retinoblastoma. The left eye was removed and when examined histolojically it showed a regressed retinoblastoma. It is argued that in the absence of history in parents, phthisis bulbi in a child should always be suspected of malignancy and also that the other eye must be periodically observed.
The literature has been reviewed and the various theories relating to the regression are discussed.
| References|| |
|1.||Carbajal V. M.: Metastasis in Ritino-blastoma, Amer. J. Ophth. 45, 39, (1938). |
|2.||DAS S. P.: Some Observations on Retinoblastoma, J. All-India Ophth. Soc. 12, 128 (1964). |
|3.||DE KLEIJN, A. (1911) V. Graef Arch. Ophth. 80, 371. |
|4.||DANIELSON R. W.: The Differential Diagnosis of Retinoblastoma Arch of Ophth. (Chicago) 58, 15 (1957). |
|5.||HINE M. L. (1937) Trans. Ophth. Soc. U.K. 57 Part I, 178. |
|6.||KHOSLA P. K., ANGRA S. K. AND AGARWAL L. P.: Gliomatous Panophthalmitis. Orient. Arch. Ophth. 2, 228(1964). |
|7.||KNEIPER, C. (1911) V. Graef Arch. Ophth. 86, 141. |
|8.||STALLARD H. B.: Glioma Retinae Treated by Radon Seeds. B. M. J. ii., 962 (1936). |
|9.||SOVIK W. E.: Bilateral Retinal Blastoma in Six Siblings. Amer. J. Ophth. 35, 1611 (1952). |
|10.||STEWARD J. K., SMITH J. L. S. AND ARNOLD E. L.: Spontaneous Regression of Retinoblastoma Brit. J. 0phth. 40, 449 (1956). |
|11.||SHERMAN N. S.: Significance of Phthisis Bulbi in Retinoblastoma. Amer. J. Ophth. 57, 403 (1959). |
|12.||VEHOEFF F. H.: Retinoblastoma Successfully Treated with X-Rays: Normal Vision Retained after Thirty-four years. Arch. of Ophth. (Chicago), 48, 720 (1952). |
[Figure - 1], [Figure - 2]