F R O M T H E E Y E D E P A R T M E N T OF T H E S T E N G A R D H O S P I T A L P H Y S I C I A N - I N - C H I E F : J . G . L I N D B E R G

ECTROPION OPERATIONS IN ICHTYOSIS CONGENITA

By Henrik Forsius.')

Ichtyosis is a comparatively uncommon disease. In severe cases the patient's appearance becomes almost repulsive, and for that reason physicians, in particular dermatologists, have taken a great interest in ichtyosis. Also ophthalmologists have contributed to the extensive literature on the subject. Zngman is the principal author in Finland; his dissertation on ichtyosis was published in 1924.

The principal changes in ichtyosis congenita are : thicken- ing of the stratum corneum in the skin, with marked hypopla- sia of the hair follicles and sebaceous glands, but the sweat glands are generally normal. We distinguish between ichtyosis vulgaris, a disesase of the newborn, leading to death, and ichtyosis congenita, which arises gradually during the first years of life and is fully developed a t about the age of ten.

In the eye, ichtyosis congenita attacks principally the cilia, the skin of the eyelids, the conjunctiva and the cornea. In all cases of ichtyosis the skin of the eyelid is contracted which, in very severe cases, causes eversion of all four eyelids. The se- vere, chronic catarrhal conjunctivitis, which is always present, may be caused by the violent ectropion. The lachrymal openings cannot be found as a rule and, naturally enough, they a re not working. Corneal injuries are occasionally observed. In typical cases they consist of opacitates, of the size of 1 sq. mm., in the superficial layers of the cornea-parenchyma. They do not cause vascularization. There is simultaneously conjunctival

* ) Rewived May 20th 1949.

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papillary hypertrophy. The disease develops in stages. A change in the cornea, resembling pannus, has been described. Hypertrophy of the cornea, zonular cataract and microphthal- mia, possibly also strabismus should be mentioned among other rare complications of this disease of the eye.

The patient to be discussed is a motor mechanic, living in Helsingfors. His only sister and one brother are quite healthy. A second brother is, however, afflicted with the same disease as the patient, and in about the same degree. No other cases of ichtyosis have occurred in the family. The patient became ill at the age of one year and since the age of ten his condition has not changed. - - - In 1944 he reported to the outpa- tients’ department for ulcus cornea simplex which healed per primam intentionem. On March 5 1948 a straw pierced his left eye. Three days later the pain was intense. He was admitted to the department and typical ulcus serpens corneae cum hypopo was diagnosed. The lachrymal openings were occluded. He was given drops of atropine in 1 per cent solution three times a day, penicillin in drops every hour, and penicillin injections, 40000 I. U. X 8 i. m. The wound healed rapidly and on March 15 he was discharged at his own request. On July 31 1948 some mortar got into his right eye. He went to the outpatients’ de- partment on the same day, his eye was cleaned and one drop of atropine dropped into his eye. On the following day there was a serpiginous ulcer in the site of the erosion and a small hypopon. Upon intensive treatment with sulfadiazine, by mouth and locally, penicillin, in drops and intramuscularly, as well as atropine, the wound was epithelialized in fice days.

For the purpose of protecting the eye from further injury, an ectropion operation was suggested, to which the patient readily agreed. - According to the literature Elschnig, in 1912, performed homotransplantation of skin in a similar case. Tissue was taken from the patient’s sister who was his senior by some years, and all four eyelids were operated upon. The transplants healed per primam intentionem. The skin gra- dually became scaly and hard and assumed the appearance of the surrounding tissue. Eleven years later, when the case was published, the result was still good. Lagophthalmus, which had been present before, was now cured.

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In 1945 a similar case was published by Shimkin, in Palestine, according to Zndex Medicus. Unfortunately details regarding the late results cannot be given as the article is not obtainable, neither in Finland nor in Sweden. - In the avail- able literature I have found no mention of operations in which the patient’s own skin has been used for grafting in ichtyosis.

In the case discussed in this paper homotransplantation was first considered, and the patient‘s only healthy brother placed himself at disposal. However, due to a cure consisting of baths and ointment treatment with 10 per cent of salicylic vaseline the patient’s skin improved surprisingly and a trial with his own skin was decided upon. The skin was quite free from scales (Fig. 1) in several parts, also the eyelids, except the ciliary border where there was still a rim of scales. The lower edge of the eyelid was drawn down to about 6 mm. below the border formed by the conjunctiva which was everted to 180° around the tarsus. The cilia were contracted and pointed in all directions. On the upper eyelid there was partial ectropion. The normal eyelid fold was completely lacking. The skin was shrunken and the eyelids too short, hence lag- ophthalmos of 3-4 mm. when the patient closed his eyes. The border of the cilium was drawn upwards and the position of the tarsus skew. The conjunctiva was cornified and inflamed and there was constant secretion from the eye. The lachrymal openings were occluded. On the cornea there were large ma- culae oc. amb. Visus oc. dx: 6120. Visus oc. sin: 6160.

On November 8 1948 local anaesthesia, novocaine in 2 per cent, was given and an incision, 5 cm. in length, was made 1 cm. below the margin of the left cilium and a patch of tissue, 1% X 6 cm. in size, was taken from the area behind the ear and fixed with six sutures. This operation and the subsequent ones were all done by Dr. J. G. Lindberg. Great difficulties were experienced in forcing the needles through the skin a s it was thick, like leather, and non-elastic, resembling the thick tissue of the scar left by a burn. There was no distinct de- marcation between the various layers of skin. Bleeding from the wound was normal. The transplant healed very slowly. The sutures were removed on the 6th day. The medial end of the patch was still loose and secretion oozed from the wound. This

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state persisted for a further five days, whereupon a coarse secondary suture was made downwards and a finer one up- wards, without anaesthesia. The sutures were removed eight days later and by that time the transplant had taken well to the surrounding skin, notwithstanding the wound not being quite dry. It was curious to see a great bulge in the transplant a few days after the operation. There seemed to be strong pressure from the surrounding skin on the grafted tissue, almost causing ejection of the patch. Therefore still coarser skin was to be used for the next operation. Tissue of the scrotum and the prepuce might have been used as in these parts the patient's skin was almost normal. The brother's skin was not considered as his soft tissue would not have been able to withstand the intense pressure of the surrounding skin. The late results of the first operation were satisfactory. The ciliary edge was pressed upwards about 4 mm. and was now about 2-3 mm. below its proper place. In the lateral angle of the eye the etropion was still disturbing.

The next operation was done on December 14 1948 on the lower eyelid of the right eye. The transplant was taken from the chest where the skin was more scaly, but also coarser and firmer. Similarly as in the first operation the patch of tissue, about 6 X l'/z cm. in size, was taken in longitudinal direction of the skin fold. An incision of 6 cm. was made 2 mm. from the margin of the cilium and the surrounding skin was under- mined on all sides. The transplant was carefully fixed with 10 fine sutures. In the first operation the skin was found to be strong enough for suture and this time a greater number of stitches was used. New needles, small and thin, were employed, which greatly facilitated suturation. The wound was dusted over with sulfadiazine. The patient complained of more severe pain during the first week following the interference than he had done after the first operation. The edge of the cilium was pushed upwards into normal position and the late results were much better than in the case of the left eye. A crust of secretion covered the transplant for a long time. In spite of penicillin treatment two weeks passed before the lower edge of the bor- der took to the surrounding tissue. Also in this operation the strange protrusion of the transplant was striking. It was less

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than upon the first operation, however, probably due to the graft being much thicker. The sutures were removed on the 6th day.

On December 29 1948 the last operation was done on the upper eyelid of the left eye. An incision was made on the upper edge of the tarsus, 8 mm. from the margin of the cilium, for the purpose of preparing the place for the normal eyelid fold, which the patient lacked. The transplant, 5 X 1 cm. in size, was again taken from the chest and the skin was attached to the eyelid with eight sutures. Collodiurn gauze was laid on the wound. This was not good as the whole area became swollen and the inflammatory oedema spread right up to the forehead. PeniciIlin was given and the infection disappeared rapidly. The upper part of the area operated upon was open on the 4th day. The swelling was evidently an infected haematoma. The transplant was compressed to a band, 4 mm. in breadth, and healed within a fortnight after the operation. There was hardly any protrusion of the transplant this time, probably due to the graft being very narrow. On January 14 1949 a new photograph was taken (Fig. 2 ) . The eye-opening was reduced by 3-4 mm. and the lids closed well.

The final results of the operation should be considered good. The disturbing lagophthalmos had disappeared almost completely and the patient’s eyelids were lengthened. In sum- mer 1949 the patient’s brother was operated upon in the same manner, and the results were quite as good.

REFERENCES Elschnig, A.: Lidplastik bei Ichtyosis congenita. Klin. Mbl. f. Augenh.

2: 4.55: 1 9 3 . Ingman. 0. A.: Studien uber Ichtyosis congenita s. foetalis. Acta derm.

Lener. 5: 125: 1924. Shimkin, N.: Ectropium due to Iclityosis of both Upper and Lower

Lids, corrected by Homoplastic Grafting of Skin from Child’s Mother. Harefuah, Journal of the Palestine Medical Associa- tion. Tel-Aviv. 29: 455: 1945.