|Year : 1960 | Volume
| Issue : 4 | Page : 94-96
MS Nirankari, GC Gulati, MR Chaddah
Department of Opthalomology, Medical College, Amritsar, India
|Date of Web Publication||5-May-2008|
M S Nirankari
Department of Opthalomology, Medical College, Amritsar
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Nirankari M S, Gulati G C, Chaddah M R. Diktyoma. Indian J Ophthalmol 1960;8:94-6
In 1908 Fuchs described four cases of turnouts which he named "diktyoma" (from the Greek word diktyosnet). It is due to the peculiar arrangement of cells which gives a net like appearance. Malignant tumours of the ciliary body especially those arising from par ciliaris retinae are quite rare. But if the histopathological examination of all enucleated eyes is done probably more cases might be recorded. All types of intra-ocular tumours are believed to be quite rare. According to Berrisford (1916), the incidence of intra-ocular growth is 001%,. In our hospital, the incidence is 0.1% of all eye diseases i.e. about 10 times the figure given by Berrisford. If every enucleated eye is sent to the centrally situated pathological laboratory, it might reveal quite a different picture. Sometimes patients report for examination late when an endopathalmitis like picture has set in and at that stage underlying picture is completely missed if histopathological examination is not carried out. The purpose of writing this paper is to stress the importance of histopathological examination of all enucleated eyes in arriving at a correct diagnosis.
| Case Report|| |
R. S., a male child aged 3 ½ years was admitted in Rain Lal Eye and E. N. T. Hospital, Amritsar. His parents had noticed the following:
i. Redness of the eyes since 20 days.
ii. Deterioration of vision in the right eye for the last 20 days.
iii. White reflex in right eye since 12 days.
The history dated back to 20 clays when the patient got redness of both eyes more so on the right side. Penicillin and Streptomycin drops were used locally and intra-muscular penicillin was given by the village doctor.
The redness subsided to some extent but the vision started deteriorating in the right eye. The parents noticed a white reflex in the pupillary area of the right eye 8 days later.
Before this episode, he developed as a healthy child. There was no history of trauma to the affected eye. He suffered from fever of short duration (5 days), one month before he developed the eve trouble.
General physical examination showed that lie was a poorly built and undernourished boy obviously looking ill. Examination of circulatory system, chest abdomen and nervous system revealed no abnormality.
Local examination of the right eye revealed enlargement of the eye-ball. Bulbar conjunctiva was congested. There was circuin-corneal congestion. A raised black area, 4mm x 4mm was present 3-4mm from the lirnbus at 6 O'Clock. position. Anterior chamber was normal. Iris pattern was disturbed and crypts not clearly visible. Reflex through pupil was whitish, pupil irregular and did not react to light. Only perception of light was present. Tension was 12mm Hg (Schiotz). Fundus reflex was not visible. Left eye revealed no abnormality.
Results of blood examination were as follows:-
Total Leucocyte count 12,000 per c.mm.; Differential Leucocyte count 48/52/-/-; Serological tests for Syphilis Negative; Erythrocyte Sedimentation rate; 2omm (first hour Westergreen);
Urine examination showed nothine abnormal.
Skiagrams of orbits and optic foramina did not show any abnormality.
The possibility of pseudoglioma was favoured due to history of fever, redness of the eye, muddy iris and low tension. The patient was treated ors anti-tubercular lines but without any beneficial effect. This made us think that it may be an unusual type of retinoblastoma producing iridocyclitis and low tension. The eye was enucleated and sent for histopathological examination.
The pathologist reported it as a case of diktyoma. The detailed report of the pathologist is as follows:
"Microscopic examination shows rows of cells with distinct cell borders. The cells appeared elongated. Nuclei are compact and ovoid, arranged in irregular rows situated mostly near the base of the cells. These rows are forming folds with double row of nuclei, one (inner) surface of these folds is free while the other (outer) is bordered on connective tissue stroma [Figure - 1]. Diagnosis-Diktyoma.
| Discussion|| |
There are three types of tumours which are known to develop from pars ciliaris retinae (Walter & James 1959).
i. Fuch's epithelioma.
ii. Embryonic type of medulloepithelioma (diktyoma) and
iii. Adult type of medullo-epithelioma.
Fuch's epithelioma occurs in senile type of eyes or cases of chronic inflammation in which hyperlasia of epithelium is seen. Some believe that medullo-epithelioma of ciliary body develops after chronic irritation of such hyperplastic epithelium.
Diktyoma arises from internal nonpigmented epithelium of ciliary body. It occurs in young children between the age of 3 to 5 years, the oldest case reported by Soudakoff was of 28 years. Growth is malignant as shown by mitotic figures on histopathological examination, and its invasive nature, but no instance of metastasis has ever been reported. It usually originates as a white flat lesion from the ciliary body and penetrates in the surrounding area showing its destructive nature. A characteristic of these tumours is that they have no melanin pigment, one of the features which differentiates it from adult type of medullo-epithelioma although there are reports of diktyoma having pigment which are considered as transitions between two types. The histopathological structure may resemble embryonic retina, membranes resembling pars ciliaris retinae, areas resembling retinoblastoma, areas of cartilage and areas of neuroglia but mostly the structure resembles embryonal retina. Though the tumour is locally malignant, yet the situation of the tumour demands enucleation. Secondly, the growth may progress and lead to secondary rise of tension and other complications in which enucleation becomes inevitable.
Adult type comprises of heterogenotrs group of tumours. The age group of the patients is 10 to 63 years. Usually it originates from both pigmentary and non-pigmentary epithelial layers of ciliary body but it may arise purely from pigment epithelium, rarely it is said to have grown - from non pigment epithelium only. Eyes which are usually affected have already got severe endogenous inflammation and show the residual inflammatory process as organised membrane or the eyes have gone to phthisis bulbi since early childhood, but there are cases on record where tumours develop spontaneously in apparently healthy eyes. Sometimes the tumours may be preceded by hyperplasia of ciliary epithelium provoked by old pathologic process in the eye. This hyperplasia may arise as a senile manifestation or as a result of increase in intra-ocular pressure over a long period or in old detachment of retina. Usual clinical appearance is that of a small dark non-translucent nodule pushing the iris forward. In later stages it may appear in the pupillary area. In the initial stages the tumour is localised and tension is normal but in late stages the tension rises and eye gets disorganised.
| Summary|| |
- A case of diktyorna is reported.
- Importance of histopathological examination of serial sections in all enucleated eyes is stressed.
- The types of tumours which are known to arise from pars ciliaris retinae are discussed.
| References|| |
Berrisford:-(1916). R. L. O. H. Rep.. 20, 296.
Fuchs, E. (1908): Arch. F. Ophth. 68 ,
:435,--Quoted by Dc Buen, S. and Gonzalez-Angulo, A. (1960). Am. J. Ophth., 49 ,
Soudakoff, P. S.(1936), Arch. Ophth. 15,
Wolter, J, R, and James, B. R. (1959), Am. J. Ophth. 46,
[Figure - 1], [Figure - 2]