SCLEROMALACIA PERFORANS TREATED \VITI CORTISONE
A CLINICAL RlSPORT WITH SOME REMARKS ABOUT ULCERATIVE S C L E R I T I S
By Humld G. A. Gjessiny,') Drammcn, Xonvay.
It is a common doctrine generally taught the students that a scleritis never ulcerates. This is, however, a truth with modi- fications, even if the exceptions are rather rare ones.
In leprosy we can have yellow-brown, transparent nodules without vascularisation localized to the different layers of the sclera. Often they are very much alike a neoplasm, ( E . Fuchs: Lehrb. der Augenh. XVI ed. 1939 p. 158 and 303) . They are never seen in the macula anestetic cases, only in the tuberous ones, (Lyder Borthen and H. P. Lie: Lepra des Auges 1899 p. 84 and 182). They are apt to destruate the sclera and thus the bulbus will be completely destroyed.
Lues sclerae occurs usually like a gumina as a circumscript nodule or nodules, which have a tendency to ulcerate. As a rule there is a t the same time a specific uveitis, (Biirki: Krankheiten der Lederhaut - Schweizsiches Lehrbuch der Augenheilk. Kar- ger 1954 1). 548) .
Still there is the fortunately very rare brawny or gelatinous scleritis, described by F. H. Verhoeff 1913 in Ophthalmoscope I1 1). 21. It is a diffuse and virulent scleritis with a very bad prognosis. Duke-Elder describes it in his excellent textbook T. I1 sec. ed. p. 2057 thus:
>>We have a diffus inflammation with swelling of the epi- scleral tissues of a gelatinous and succulent appearance and pitting on pressure, encircling the cornea. (Because of that the
*) Received July 4th 1954.
Germans also call it: nialigne sulzige Skleroperikeratitis) . It is more common in women and usually bilateral. As the disease proceeds the affected area extends backwards merging with the normal tissues, but never passes the equator. The infil- trated tendons of the muscles often stand out like nodules. An- teriorely the swelling ends abruptly a t the limbus, which itself is not affected. Eventually the cornea, however, becomes infil- trated by a sclerosing keratitis. A more or less severe uveitis is a constant accompaniment.
The disease is extremely chronic and runs a slow progressing cows with intermissions and exacerbations. It may occasion- ally be painless. But as a rule pain is a prominent feature. - - Occasionally the appearing is atypical. The brawny red colour may be lacking. The etiology is obscure.>An area in the sclera first undergoes a simple necrosis. This area is surrended by epitheloid cells. Pus cells
slowly penetrate the wall and surround the necrotic area, which become more or less disintegrated by eosinophilic grains. Ultimately the sequestrum thus formed becomes completely necrotic. That the process of the abscess formation is extremely slow is evidenced by the fact, that no pus cells are to be found outside the wall.<
The holes in the sclera may be covered by conjunctiva. But this is not invariable and the uvea may be laid bare. Even if this occurs no herniation may appear. But if they open into the anterior chamber a cystic formation may be evident and a fistula may develop. (Chiefly cited from Duke-Elders text- book I1 p. 2066-6i and Eber Am. Journ. of Ophth. 1934 1). 329) . Till recently the disease seemed to drag on indefinitely without doing much damage or exciting many symptoms. But if a fistulization occured a flap operation was indicated. In a somewhat similar case, which, however, appeared to show inflammatory signs initially, Oust (Arch. of Ophth. 1935 1). 696) produced a beneficial reaction by local ultraviolet rays. However, after a report by E . B. Reese from the Presbyterian Hospital (The year Book of the Eye, Ear, Nose and Throat 1953 -54 p. 210-11) in three cases B-ta and Gamma-rays, which he used for removing of tumours from the external layers of the sclera, developed a circumscript necrosis of the sclera. But now we seem in cortisone - cortone - have a quite specific remedy against scleromalacia perforans.
As I understand it the scleromalacia perforans seems to be a very rare ailment. In the literature a t my disposal I have not recorded more than up to some 50 cases. None of them are published from the Scandinavian papers. Not even is the dise- ase mentioned in the great text book of pathology by Robert Allan Moore, 1948. I thus think I may be permitted to refer quite shortly a case, showing by the excellent photos, which I owe to my friend the Clinical Assistent at the Municipal Ho- spital of Drammen, Dr. Per Kolstad. They illustrate rather nicely the symetrical affections of both eyes. The case shows also the excellent effect of cortone. The patient was demon- strated by me at The medical Society of Drammen May 11th 1954.
A coachman, formerly always healthy, was operated by me for a cataract on his left eye April 1942 with excellent result. Visus a t the leaving the hospital 20 days after the operation was full 7/7 with cor- rection: 90" + 2,5 + 11.0.
I did not see him again before 15 - I11 - 53. He was then not quite 82 years of age. He had in the meantime got a severe arthritis of both hands and feet, so he could scarcely walk and was rather decrepit, though his brain was clear and he still was quite fit inspite of his old age.
F i g . !.
He came to mc because both of his eyes had trouble him by water- ing and a slight pain for some months. Since January there had also been some photophobia. I found:
Some slight mucous discharge from the conjuncti\a 0. u. - else thc eyes were pale.
On both eyes - vidc the figures - outside of tlie limbus the sclerae. covered by the conjunctiva, showccl a peculiar appearance:
Symetrically - abt. 4-5 mm. from the limbus - the sclerae seemed to be quite absent. The black bluish uxea was laying - a little bulging though - subconjunctivally. The defect of the sclerae was sharply defined. - Uvea was not perforated and there was n o sign of any uveitis, thus no precipitates on the membrane of Dascrmel . The scleral atrophy messed abt. 12 mm. in liiglit and 5-G mm. on its broadest size forming a crescent with the concavity to the limbus.
Visus on the operated eye was sunk to < 7110 due to a thin second- ary cataract membrane. There was no sign of any senile macular alteration. - The vision of the other eye was only finger counting in abt. 1,5 m. due to a mature senile cataract. The tension of both eyes was normal: 5/5,5 0. d. and Gl5,5 0. s., Schiclt;' old tonometer.
A s I had never seen a similar case before I was a t the first yisit quite in the dark as to the riglit diagnosis. But thinking that, as sclera was affected, cortone might be of use I prescribed sol. cortone 2.00 in lot. Ringer 8.00 5 times a day for a month and during the same time also a zinc solution 0,25-10,OO three times a day. He was ordered to come back abt. a month.
Fig. 2. Riglit eye.
Fig. 3. Left eye.
20 - IV - 53 I saw liim again. I t was status idem. Ordre: coiit. 1 mont.
I examined liim again June Gtli. His corijuiictivitis liatl now quite subsided. Tlicre were some fine grey traces - just like a fine film - crossing the scleral defects. The zinc solution was seponated but Iic had to continue with tlie cortone drops 5 times daily for a montli.
July 4th: The cicatrication was more pronounced - just like a fine web crossing the scleral defects, which were not bulging. He liad 110 pain nor discharge or photophobia.
August 29tli I saw him last time before I clemonstrated him before The Medical Association of Drammen Mai 11th - 1954 - I found: The fine, grey streaks crossing the uveal patches, which still per- sisted, were now mucli more increased. Else the eyes externally seemed normal.
I must emphasize that the case is not so well examined as it ouglit to be. Because of his great age and as lie for economical reasons could not afford to stay in the hospital n blood examination especially of tlie amount of calcium and phosphor was not made. This is a pity.
The etiology of the scleronialacia perforans is still sub judice.
Van der Hoeve thought, when he first described the con- dition, that it was a result from a dyslipoidosis in which the cholesterol metabolism was deranged. Later very thoroughly examinated cases have not confirmed this supposition. Syphilis is not recorded in any of the published cases. A few cases - reported by Roset and Algan (Arch. of Ophth. 1947 p. 698) seem to have showed a dysfunction of the pituary body. But as they also were afflicted with polyarticular rheumatism their findings were not convincing. Another case - published by Paufique (vide Hobbs, Transact. of The United Kingd. vol.
Acta Ophthalmol., Vol. 33. 111 17
LXXII, 1953 p. 48) was associated by a typical tuberculous arthritis of the sacro-iliac joint.
In 1938 Verhoeff and King - cit. by Hobbs - pointed out the histological resemblance between the fibrinoid necrosis of scleromalacia perforans and that of rheumatic subcutaneous nodules, both affections also giving alike response to treatment with cortisone. It must be emphasized, though, that we not yet know the real, deepest origin of the rheumatic diseases neither of the eye nor of the other parts of the body. We only know that we have a fibrinoid degeneration of the collagenous tissue just as we see it in scleromalacia perforans. Duke-Elder there- fore suggests to call it: >>rheumatic nodules of the sc1era.e
Not being a gynecologist I feel myself scating on thin ice when I venture to make a comparison between two to such a degree apparently dissimilar diseases as the above mentioned and osteomalacia in pregnant women !
It is well known that there are different opinions as to the etiology of this last mentioned ailment. I think the now most accepted theory is, that it is due to a hyperfunction of the glandulae parathyroideae. This leads to a deficit in the Ca- and P- as well as vitamin D-metbolism. These three substanses are found in an excessiv amount in the feces in the pregnancy. And just during this period the body needs them highly. We can now treat the osteomalacia effectfully by giving the women these stuffs. I refer to the work of Karl F. Paschki and others in in Clinical Endocrinology 1954 p. 688 and to the above men- tioned work of Hoebs. And this is still more probable as we know that the socalled senile scleral spots, due to degenerative alterations of the sclerotic, most likely is due to local malnu- tritions - Biirki (Schw. Lehrb. 1954 p. 550). They appear as a rule symetrically mostly on the medial side of the limbus, not so often on the temporal one. Biirki thinks that they in some cases may be a prestadium of the perforating sclero- malacia.
I therefore mean that in cases of named disease the Ca- and P-metabolism as well as the vitamin metabolic processes is to be examined thoroughly and the patients is to be treated not only with cortisone but also be given those mentioned remedies in sufficient doses together with vitamin D.
In a man, operated 69 years of age for cataract on his left eye and at that time quite healthy, came abt. 82 years old suf- fering from a polyarticular arthritis and a peculiar eye disease under the picture of a scleromalacia perforans on both eyes. He was treated with apparently good result with cortisone.
As the etiology of scleromalacia perforans - a very rare disease - is still sub judice, the author is sorry that the case is not sufficiently examined because of unavoidable circum- stances especially in vicw of the Ca-, P- and vitamin D-meta- bolism. He thinks there may be an analogon with the above mentioned ailment and the osteomalacia in pregnant women.
He therefore suggests that in addition to the cortisone thera- py always a sufficient amount of calcium and phosphorus as well as vitamin D is to be given in treatment of scleromalacia perforans.