Scleromalacia perforane is a condition characterized clinically by progressive melting of the sclera with secondary exposure and ectasia of the underlying uveal tissue. This condition is often associated with rheumatoid arthritis, and has its greatest incidence in post me p opa u sa I females.' The lesions may develop from pre-existing scierai nodules whose microscopic appearance resembles that of the rheumatoid nodule. The prognosis of this condition continues to be poor despite various medical and surgical modalities of treatment.1'18 The purpose of this communication is to discuss the role of periosteum as a graft material and to report a case of scleromalacia perforane in an eye with central retinal artery occlusion and Mooren's corneal degeneration successfully treated with a periosteal graft.
This 60-year-old white male was first seen by us on September 2, 1975. He was initially seen by his ophthalmologist in August 1973 with a history of sudden loss of vision in his left eye and was found to have a central retinal artery occlusion. The vision in his rig ht eye had al ways been poor following an injury sustained during his youth.
In October 1973 he was noted to have a 3+ injection of conjunctiva and sclera of his left eye and was given Maxitrol drops locally only with limited success. He sustained an episode of elevated intraocular pressure in both eyes in December 1973 which was recorded at 42 mm Hg in the right eye and 70 mm Hg in the left eye. This was controlled to 1 6 mm in each eye by oral carbonic anhydrase inhibitors. The sclera! injection persisted and in April 1 974 he was found to have iritis of his right eye which progressed with posterior synechiae and seclusion of the pupil, in spite of treatment with topical Atropine and 1% methyl prednisolone. In the first week of August 1 975 he developed increased irritation and drainage of both eyes. Examination at this time revealed 4+ conjunctiva) injection with vascular invasion with a deep corneal ulcer from 7 to 11 o'clock position, extending to 2/3 the depth of the corneal strema. A tentative diagnosis of Moore n 's ulcer was made. The patient was placed on topical steroids and referred to us for further treatment.
FIGURE 1: Left cornea showing peripheral corneal degeneration with marked vascular invasion 9/20/75.
FIGURE 2: Cornea showing improvement with the use of hydrophilic bandage lens 6/12/76.
FIGURE 3: Superior limbal area showing gross degree of sclera/ thinning with the uvea! tissue shining through. Note the engorged vessels in the area 6/12/76.
FIGURE 4: Ninth postoperative day showing the periosteum and buccal mucous membrane grafts in situ over the area of scierai defect.
Our examination revealed a visual acuity of finger counting in his right eye and 20/400 in his left eye. He showed a 3+ conjunctiva! and scierai injection in his right eye with few small K.P. on the inferior half of the corneal endothelium. There was complete seclusion of the pupil. The left eye showed 3+ conjunctiva! and scierai injection along with several peripheral cornea! ulcérations extending from 7 to 1 1 o'clock position and another from about 12 to 3 o'clock position. The ulcération extended to 2/3 of the strema. The intraocular pressures were 1 4 and 1 5 mm in right and left eyes respectively on applanation. The fundus could not be visualized in the right eye and the left showed evidence of old central retinal artery occlusion. The patient was fitted with a hydrophilic bandage lens in his left eye and was started on initial doses of Maxitrol drops locally QID and Prednisolone 40 mg orally per day. He did well on this regimen until May 1 8, 1 976, when he was found to have evidence of scierai thinning from 11 to 2 o'clock close to the limbus and a diagnosis of scleromalacia was made. Despite elevated doses of systemic and topical steroids, the lesion progressed to the point of threatening the integrity of the globe.
FIGURE 5: Same area as in Figure 4 three months postoperatively. The graft is richly vascularized and blending well with the surrounding tissue.
On July 1 5, 1 976, under general anesthesia, the area of scierai necrosis was cleaned and the conjunctiva surrounding it was all undermined. A periostea! graft taken from the anterior surface of the tibia was placed over the area of sclera! defect and was sutured in place using 10-0 Perlon on the limbal side and 9-0 on the side of the fornix. The graft was covered by buccal mucous membrane obtained f rom the lower lip of the patient. Postoperative Iy the patient was started on 1 % Pred Forte drops every hour. Atropine 1% drops 4 times a day and Prednisolone 80 mg per day orally. The patient did very well on this regimen and the above drugs were slowly tapered to a point that he was getting Pred Forte drops 4 times a day. Atropine 1% drops twice a day and Prednisolone 1 0 mg orally every other day at the time of his last visit on November 12, 1976. The eye was showing trace injection, minimal photophobia and the graft was in place with minimal reaction.
FIGURE 6: Postoperative appearance of Cardona "through-and-through" keratoprosthesis in which periosteum and buccal mucous membrane were utilized to cover the flange. Fifteen months postoperative with no tendency for extrusion.
All the laboratory investigations done to find out any possible connective tissue disorders as to etiology of the problem turned out to be negative.
Many connective tissue disorders of the body present with a variety of ocular manifestations involving different structures of the eye. The sclera, composed of a connective tissue stroma, participates in manyofthesediseaseprocesses. Scleromalacia perfora n s is one manifestation of scierai involvement in systemic connective tissue disorders such as rheumatoid arthritis. Van Der Hoeve2 in 1931 coined the term "scleromalacia perforans" to describe a condition whose principal feature was the appearance of holes in the sclera, which could coalesce so that the sclera showed large gaps in which the uvea was either covered by only conjunctiva or laid bare. He also dealt with the possibility of two different types of scleromalacia, one associated with rheumatoid arthritis and the other without this association. There have been various reports in the literature belonging to either of these two categories. Rheumatoid arthritis as an association has been reported in the majority of reports2'10 and a good number of reports indicated its association with other systemic diseases like Wegener's granulomatosis" and Crohn's Disease.12 Duke-Elder described association of this problem with a great variety of connective tissue disorders such as systemic lupus erythematous and periarteritis nodosa. The other associations have been with metabolic diseases (porphyria) and viral infections (herpes simplex). Ashton10 described the pathological picture of a scierai nodule, which later on can develop into an area of scleromalacia, and found that the picture resembles the histological picture of a rheumatoid nodule. These nodules consist of masses of hyalinized and completely degenerate scierai fibers surrounded by a pale, clearly defined zone of palisading fibroblasts which in turn is surrounded with a zone of chronic inflammatory cells, in which plasma cells and lymphocytes predominate while eosinophils and polymorphs are noticeably few in numbers.
Later, several authors including Welter and Bentley confirmed these findings. All these observations give us an impression that scleromalacia perforane is but one clinical manifestation of a generalized connective tissue disorder. However, there have been various reports in literature13'14 where there was no clinical or laboratory evidence of a systemic disorder in patients presented with scleroma lacia perforans. In our patient, we could not find any evidence of systemic connective tissue disorder by our clinical and laboratory investigations, which included a total blood count, a differential count, E. S. R., LE. cell preparation. Latex fixation test, urinalysis and x-ray of chest.
Duke-Elder describes various forms of marginal corneal infiltrates and vascularized peripheral corneal lesions in the various collagen disorders. Limbal guttering in both eyes has been described12 along with scleromalacia perforans. Most of the descriptions give a picture of an associated anterior uveitis. Our patient had a picture of scleritis in both eyes and later went on to develop peripheral Mooren's type of ulcération in the left eye before he developed scleromalacia. He did very well on a regimen of soft lenses and topical and systemic steroids. The ulcers became quieter and showed considerable healing.
Wolter and Boldt,13 on histopathotogy of an enucleated eye with scleromalacia perforans, found a cotton wool patch in the retina. This particular patient had no evidence of any collagen disorder. Our patient had an episode of central retinal artery occlusion preceding the development of all the anterior segment problems in the eye which later developed scleromalacia. This clinical feature was not described in association with scleromalacia in literature as far as we know.
Various modalities of treatment both medical and surgical have been advocated in the treatment of scleromalacia perforans. The most popular method of medical treatment is the usage of corticosteroids by both local and systemic routes. Ashton10 found remarkable alteration in the progress of scleromalacia with the use of local and systemic steroids. All the areas of perforation were covered by firm new scierai tissue. However, Anderson and Margolis7 did not have much success with the use of systemic steroids and the sclera continued to melt away. Even a trial of ACTH did not help. Various other reports indicated variable response to steroids. Evans and Eustace12 used EDTA locally on the basis that it acts as a collagenase inhibitor and thereby reduces the future progress of scierai melting. Ze r et al. '* reported a dramatic response to the oral administration of d-penicillamine. This was encouraged by reports of success in cases of rheumatoid vasculitis. In our patient, we did not have any encouraging response to treatment with local and systemic steroids. The scierai melting continued to progress on steroid therapy.
The surgical methods of treatment are in the form of reinforcing the areas of scierai defect by using different types of tissue as grafts. Those tried were sclera, fascia lata, aortic tissue, buccal mucous membrane and auricular cartilage. There have been claims that each one was better than the other. Van Der Hoeve found success with buccal mucous membrane. Some authors5 felt that autogenousfascia lata grafting should be the treatment of choice since it is autogenous, readily available, durable and easy to work. Armstrong and McGovern15 found h istologica Uy that fascia lata was firmly adherent to the adjacent sclera and episclera. Several felt that scierai onlay graft was better as it becomes incorporated into the surrounding sclera and prevents the stretching reaction in the scierai fibers of the defective area thereby reducing pain. Merz9 used aortic tissue as a graft and the advantages of this are its availability, strength, eta sticityand malleability.
Our experience with periosteum to cover the Cardona keratoprosthesis has been favorable so that the use of this autologous tissue in scleromalacia is a natural choice. The other possible advantages of autogenous periostea! graft are its greater strength, greater chances of getting vascularized and so blending with surrounding tissue, easy availability and less chances of immune reaction because of its autogenous nature. Periostea! graft also decreases the chances of necrosis of both conjunctiva and periosteum and it reinforces the areas of scierai thinning." We are encouraged by the response that we obtained in our patient. The graft is well vascularized and blended with the surrounding tissue. Subjectively the patient has no pain and very little irritation or photophobia. We feel that this form of surgical treatmentof scleromalacia perforane is consistent with a good subjective and objective result. Though our experience with periosteum is too limited to draw any conclusions, it certainly focuses attention on the usefulness of periosteum as graft material in these desperate cases. Rare occurrence of this condition does not give enough scope for more experience with any one graft material to any one surgeon. Indeed most of the reports in literature related to experience with various types of graft material in these cases have been based on one or two cases. If sufficient conjunctiva is not available, mucous membra ne has proven to be a satisfactory selection to cover the periosteal graft.
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11. Frayer WC: The histopathology of perilimbal ulcération in Wegener's granulomatosis. Arch Ophthaímol 64:58, 1960.
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16. Sevel D: Necrogranulomatous scleritis. Amer J Ophthalmol 64: 11 25, 1964.
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