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ARTICLE
Year : 1963  |  Volume : 11  |  Issue : 3  |  Page : 68-72

Exudative type of retinal detachment- "Harada disease"


India

Date of Web Publication28-Jan-2008

Correspondence Address:
R K Mishra
India

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Source of Support: None, Conflict of Interest: None


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How to cite this article:
Mishra R K, Dhanda R P. Exudative type of retinal detachment- "Harada disease". Indian J Ophthalmol 1963;11:68-72

How to cite this URL:
Mishra R K, Dhanda R P. Exudative type of retinal detachment- "Harada disease". Indian J Ophthalmol [serial online] 1963 [cited 2019 Dec 7];11:68-72. Available from: http://www.ijo.in/text.asp?1963/11/3/68/38885

Harada's disease is a well known syndrome of rare occurrence, and unknown etiology, affecting chiefly the coloured races, and involving the uvea, retina and optic nerve. To our knowledge, no case has so far been reported from India. The following case is reported for the severity of the disease, for the unsatisfactory visual recovery despite complete reattachment of the retina and the presence of pigmented strips in the retinae in both eyes after reattachment.


  Case History Top


D.L. a 26-year-old Hindu male noticed redness arid vague pain in the right eye on 1-3-60. The vision gradually dropped to perception of hand movements only, within 17 days (18-3-60). He consulted his physician, who found no systemic disease and treated him with multivitamins arid atropin ointment with no relief. The left eye remained normal till 3-4-60, when he first noticed blurring of vision, which continued deteriorating despite treatment by the physician. A month later on 4-5-60, the patient could see nothing with either eye. He consulted us at the Medical College Hospital Indore on 6-5-60. He was admitted in the eye-ward as a case of bilateral exudative detachment of the retina.

Ocular findings: The patient had a vacant staring look. The lids were normal. Mild circum-corneal congestion was present on both sides. The cornea looked bright and transparent but its endothelium was dusted with numerous K.P.'s., visible with a corneal loupe. The pupils were widely dilated and reaction to light, direct and consensual was extremely feeble. Posterior synechia, though light, were numerous and a large number of cellular and pigmentary deposits were seen on the lens capsule. Pin point subcapsular lenticular opacities were seen scattered all over the lens in both eyes. Behind the lens, the detached retina which was of a dull grey colour could be seen. Under magnification, the blood vessels and folds of retina were distinctly visible. Intra ocular tension recorded 10 mm. Hg. Schiotz. Ophthalmoscopy was difficult due to vitreous haze. The reflex in general was poor and the retina appeared greyish yellow. The entire retina was lifted enmass in large lobulations. No tear or holes were detected. The blood vessels were tortuous and dark. Optic discs could not be seen on either side being covered by folds of the detached retina. Vision was reduced to perception of light.

Family history was non-contributory. A routine screening of chest, urine examination and blood cytology were normal. Kahn's test was negative. Erythrocyte sedimentation rate was 28 mm/hour (Wintrobe) and the tuberculine test was negative to 1 :1000 dilution. E.R.G. showed extinguished E wave.

Initial therapy:

Local:- Atropin drops 1% twice a day for 3 days - once a day for 10 days subsequently. Hydrocortison eye drops 4 hourly, dry fomentations twice a day for 10 minutes each time.

General:- Daily 30 mgs. of Deltacortrill and sodasalysylats 30 mg. orally with vitamin B1, 100 mg. intramuscularly.

On 18-5-60 the patient showed a little improvement. Right eye could perceive hand movements at 1 foot and left eye could count fingers at 3 m. Circum-corneal congestion had disappeared and K.Y.'s. had taken a brownish tinge. Vitreous chamber showed remarkable clearing and the retina had retracted considerably. No holes were found. Disc could not be visualized properly but looked a little hyperemic and did not suggest papilloedema.

On 14-0-60 the patient's right eye could count fingers from 1 m, and left eye from 4 m. Signs of Irridocyclitis were much less and retina was reattached. No haemorrhages were seen. On the contrary multiple strips of pigment were visible. They lay deep to the blood vessels and they gave an impression of raising the retina over them. Of these strips a prominent one was seen above the disc on both sides in identical positions. The retina as a whole appeared pale. The discs showed a return to normal colour. E.R.G. still showed an extinguished 'E' wave. Tension was recorded as 10 mm. Hg.

On 1-7-60 the patient was discharged at his request, his vision being : right eye counting fingers from 2 m. and left eye from 4 m. Thereafter the patient has been regularly followed up monthly at the medical college Jabalpur. The progress is very slow but yet steady. Seen last (Nov. 1961) he had visual acuity of 6/18 on left side and 6/6 on right side. Aqueous flare was still visible by slit lamp microscope on the right side. The retina had completely reattached. Multiple patches and strips of depigmentation were seen. All these months he has been treated with corticosteroids atropin and Vit. B complex.


  Comments Top


This rare disease described first in 1926 by Harada continues to be reported sporadically but nevertheless the number reported is about 30. This disease is known to have many components e. g. bilateral spontaneous exudative detachment with tendency for reattachment, malaise, headache, pyrexia and occasionally also meningeal irritation. In addition it may be associated with dysacusia, vitiligo and poliosis whence it resembles certain unusual forms of sympathetic ophthalmia and Vogt-Koyanagi syndrome. A bilateral granulomatous uveitis is characteristic of all the three conditions. In Harada disease, it is mostly a posterior uveitis leading to early detachment of retina with mild signs of irridocyclitis appearing late whereas in the other two syndromes, detachment is uncommon but the irridocyclitis is an early and prominent feature. Many people consider them to be different variants of essentially the same disease. Etiology of this disease remains unknown, it has been attributed to virus infection, tuberculosis, hyper-sensitivity to uveal pigmentation etc.

In the case reported, the onset and the course with its response to the steroid therapy points to the usual form of Harada's disease. However this case had two interesting features. Firstly, inspite of the complete reattachment of retina, vision did not improve proportionately and secondly the presence of pigmented strips. The failure of visual return may be attributed to the extreme severity of the


  Discussion Top


The rarity of this disease, particularly in the eye arid orbit have already been stated. The possibility of Hydatid cyst was not thought of in this case because of the extreme rarity of the disease.

Clinical Picture, Hydatid cyst tends to occur in the young as children handle dogs more intimately than adults. More than half the cases are seen between the ages of 10 and 30.

In the majority of cases the disease remains symptom less and latent for many years and its presence is only detected at autopsy. The presence of the cyst may be suspected when, (1) it produces pressure effects on the surrounding tissues (Chatterjee 1952), (2) it suppurates, and (3) when there is trauma which either activates the growth of the cyst causing the clinical triad of symptoms proptosis, tumour and pain or produces an anaphylactic type of reaction, sometimes even fatal, due to liberation of the cyst fluid. Our case was a child in whom the triad of symptoms followed a definite history of trauma.


  Diagnosis Top


1) Clinical.-In the triad of symptoms sometimes pain may be entirely absent, which strongly suggests a rapidly growing sarcoma.

2) Laboratory Tests.-

a) Casoni's reaction - (Allergic test) - Intradermal injection of 0.2 c.c. of sterile Hydatid fluid (obtained from animal sources) produces a wheal of 5 cms. in diameter within hour of injection in all positive cases.

b) Precipitation reaction of Fleig and Lisbonne-The aspirated fluid precipitates with the blood serum.

c) Complement fixation test of Weiberg-Parvu.- This is positive in 85% of cases.

Unfortunately one or all these may be negative (Duke-Elder, 1952).

3)Puncture.- Puncture of any suspicious swelling and examination of any aspirated fluid should be made in all cases of painful findings. Puncturing a Hydatid cyst with a hollow needle is a very risky and undesirable method of diagnosis, though an accurate diagnosis is established by withdrawing a few c.c. of Hydatid fluid and. examining it tinder the microscope for scolices. Many a times there may be no scolices, the fluid being sterile. Great caution should be exercised while aspirating as the fluid is under high tension and leakage of fluid into the tissues may cause (a) ) Anaphylactic shock. (b) Secondary Echinoccosis as a result of the liberated scolices entering the general blood circulation of the host. c) Bacterial infection of the cyst leading to suppuration. (Chatterjee, 1952).

Preparation to excise the whole cyst radically should be at hand, along with all the measure; to deal with any possible anaphylaxis.

In our case, the mistaken clinical diagnosis of a rapidly spreading orbital sarcoma precluded undertaking of these tests.

Treatment. Excission of the cyst is the only treatment available. There is no satisfactory chemotherapy (Sorsby, 1958). Before evacuating the cyst contents it is advisable to inject Formalin 10%) to kilt the scolices. The cyst wall is exposed by incising the adventitia. The cyst is then separated from the adventitia and removed. The cavity should he swabbed again with 10 Formalin. After this the cavity is obliterated by suturing without leaving any drainage.


  Summary Top


A case of Hydatid cyst of the Right Orbit in a girl aged 8 years has been described. On clinical grounds the case was mistaken for a Orbital Sarcoma. No investigations were done. The diagnosis was made at the operation



disease in the right eye which led to total detachment of retina but the pigment strips are a little more difficult to explain. A somewhat similar case was reported by P. Shivasubramaniam as a unique feature of this disease. The condition thereof was referred to as angiod streak. In our case we will choose the somewhat similar picture to be labeled as strips rather than streaks. These strips were black in colour and were certainly deep to the blood vessels. In our case however, at their ends the strips did not show any sign of pigment stippling as described by Shivasubramaniam. To us the causation of the pigmentary strips appears to be merely depigmentation at the side of stress consequent to the extreme exudative separation of the retina.


  Summary Top


A case of Harada's disease is reported with two interesting features, namely proportionately poor visual return inspite of complete retinal reattachment in one eye and presence of pigmented strips in retina deep to blood vessels. Prognosis of Harada's disease must be regarded as variable depending on the severity of the uveal inflammation. Etiology of our case remained undermined.[5]

 
  References Top

1.
Cordes, F, C., (1954), Trans. A.M. Soc. Ophth. 52, 78-104.  Back to cited text no. 1
    
2.
Mitsuit and Solanes (personal communication to R. David Sudarsky), A.M.A. Archives Ophthalmology, July, 2959,  Back to cited text no. 2
    
3.
Bullington, S, J. and Walksman, B. H., (1958), A.M.A. Archives of Ophthalmology, 59, 435-445  Back to cited text no. 3
    
4.
Sudarshy, R. D. (1959), A.M.A. Archives Oph. 62.  Back to cited text no. 4
    
5.
Siva Subramaniam, P., (1958), Brit. J. of Ophth. 42, 759.  Back to cited text no. 5
    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7]



 

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