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ARTICLE
Year : 1968  |  Volume : 16  |  Issue : 2  |  Page : 76-78

Retinoblastoma in phthisis bulbi


Department of Ophthalmology, lnstitute of Post Graduate Medical Education and Research, Chandigarh, India

Date of Web Publication22-Dec-2007

Correspondence Address:
I S Jain
Department of Ophthalmology, lnstitute of Post Graduate Medical Education and Research, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


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How to cite this article:
Jain I S, Singh K. Retinoblastoma in phthisis bulbi. Indian J Ophthalmol 1968;16:76-8

How to cite this URL:
Jain I S, Singh K. Retinoblastoma in phthisis bulbi. Indian J Ophthalmol [serial online] 1968 [cited 2020 May 30];16:76-8. Available from: http://www.ijo.in/text.asp?1968/16/2/76/37498

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 Top


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 Top


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 Top


Reviewing the literature one finds that regression of retinoblastoma is not very common, but as STEWARD [10] 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, [3] KNEIPER [2] DAS [7] , STEWARD ET AL [10] CARBAJAL [1] .

(ii) The tumour gets calcified -

(iii) The previous tumour site shows choroidal atrophy with a small grey, green or pink area showing calcification - HINE [5] .

The diagnosis of regression has been made either (1) on clinical grounds and follow up - KNEIPER [5] STALLARD [8] , HINE [5] CARBAJAL [1] 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 [9] , STE­WARD ET AL [10] , or (3) it is proved histologically wherein calcification, even bone formation is observed (DEKLEIJN [3] , STEWARD et al [10] , SHER­MAN [11] , Das [2] .

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 [6] 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 [4] that re­tinoblastoma can only occur in a nor­mal. sized eye with normal anterior chamber. SHERMAN [11] 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 [10] 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 Top


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.[12]

 
  References Top

1.
Carbajal V. M.: Metastasis in Ritino-blastoma, Amer. J. Ophth. 45, 39, (1938).  Back to cited text no. 1
    
2.
DAS S. P.: Some Observations on Retinoblastoma, J. All-India Ophth. Soc. 12, 128 (1964).  Back to cited text no. 2
    
3.
DE KLEIJN, A. (1911) V. Graef Arch. Ophth. 80, 371.  Back to cited text no. 3
    
4.
DANIELSON R. W.: The Differential Diagnosis of Retinoblastoma Arch of Ophth. (Chicago) 58, 15 (1957).  Back to cited text no. 4
    
5.
HINE M. L. (1937) Trans. Ophth. Soc. U.K. 57 Part I, 178.  Back to cited text no. 5
    
6.
KHOSLA P. K., ANGRA S. K. AND AGARWAL L. P.: Gliomatous Pano­phthalmitis. Orient. Arch. Ophth. 2, 228(1964).  Back to cited text no. 6
    
7.
KNEIPER, C. (1911) V. Graef Arch. Ophth. 86, 141.  Back to cited text no. 7
    
8.
STALLARD H. B.: Glioma Retinae Treated by Radon Seeds. B. M. J. ii., 962 (1936).  Back to cited text no. 8
    
9.
SOVIK W. E.: Bilateral Retinal Blas­toma in Six Siblings. Amer. J. Ophth. 35, 1611 (1952).  Back to cited text no. 9
    
10.
STEWARD J. K., SMITH J. L. S. AND ARNOLD E. L.: Spontaneous Regression of Retinoblastoma Brit. J. 0phth. 40, 449 (1956).  Back to cited text no. 10
    
11.
SHERMAN N. S.: Significance of Phthisis Bulbi in Retinoblastoma. Amer. J. Ophth. 57, 403 (1959).  Back to cited text no. 11
    
12.
VEHOEFF F. H.: Retinoblastoma Suc­cessfully Treated with X-Rays: Nor­mal Vision Retained after Thirty-four years. Arch. of Ophth. (Chicago), 48, 720 (1952).  Back to cited text no. 12
    


    Figures

  [Figure - 1], [Figure - 2]



 

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