CLINICAL CASE OF TREATMENT OF CONGENITAL SEVERE COMBINED PATHOLOGY OF KERATOGLOBUS IN COMBINATION WITH MEGALOCORNEA ON THE BACKGROUND OF UNDIFFERENTIATED CONNECTIVE TISSUE DYSPLASIA.

To evaluate the effectiveness of treatment of a patient with keratoglobus against the background of megalocornea and to describe a clinical case of staged corneal keratoplasty in a patient with a very rare combined pathology: Megalocornea and Keratoglobus.

S. Fyodorov Eye Microsurgery Federal State Institution, Moscow, Russian Federation.

A 36-year-old woman with a history of undifferentiated connective tissue dysplasia went to the clinic with complaints of low visual acuity in the right eye, the impossibility of selecting a spectacle correction. Diagnosed : megalocornea, advanced keratoglobus in both eyes. With best-corrected visual acuity of 0,05 in the right eye and 0,6 in the left eye. The corneal diameter is 14.2 mm. Keratometry 37.75 D – 84°, 42.25 D – 174°. The minimum thickness of the cornea along the periphery is 109 µm. Complicated cataract. Due to the risk of perforation and low visual acuity, surgical treatment was decided. Initially, penetrating keratoplasty of the right eye was performed in February 2019. A 9 mm trepanation hole was made. Further, after assessing the condition of the residual cornea (sharp thinning along the periphery) , it was excised from limb to limb. Due to the abnormally large capsular bag structure, we had to suture the IOL to the iris after implantation, then the entire corneal-scleral complex was transplanted. Although the likelihood of developing secondary glaucoma was high, the early and late postoperative periods were proceeded without incident. 6 months after surgery, vision = 0.2. However, 18 months after surgery, the patient developed graft disease. Based on objective research data, DSAEK was recommended. In June 2021, the operation was performed. Its course coincided with the traditional one. Although it was complicated by the presence of anomalies in the structure of the eyeball and the patient's somatic condition (the absence of one kidney and other undifferentiated connective tissue diseases). Complete adhesion of the graft was achieved immediately after the operation. Visual acuity after 2 months = 0.3.

Keratoplasty on the eyes with such a sharp combined pathology as megalocornea and keratoglobus always has high risks and unpredictable results. The technique of performing the operation differs from the standard one due to anomalies in the structure of the eyeball. However, this method of performing staged keratoplasty is the only possible way to reduce injuries and increase visual acuity in this situation. The patient has a similar picture in the paired eye. Surgical intervention is planned in the future.