[Long-term results after perforating corneo-scleroplasty in a case of acute unilateral superior pellucid marginal corneal degeneration].

Purpose: This case report shows the long-term results after perforating corneo-scleroplasty in a rare case of superior pellucid marginal corneal degeneration with acute hydrops due to rupture of Descemet's membrane.

Patient: In the left cornea of a 20-year-old patient with peripheral stromal thinning from 9 to 3 o'clock a rupture in Descemet's membrane occurred followed by lamellar splitting of the mid-stromal region. Due to the decrease in visual acuity and pain from corneal edema a surgical treatment was performed consisting of a perforating/lamellar corneo-scleroplasty protecting the anterior chamber angle.

Results: The status has remained stable for 17 years after surgery with nearly clear graft, best corrected visual acuity of 0.8 and no signs of recurrence or progression of the disease. Central astigmatism is regular, the endothelial cell count is 1250/mm2 in the central cornea, the central corneal thickness is 540 microm and only a mild vascularised superficial pannus and slight opacities in the predescemetal layer of the graft are found. There are no anterior synechia. On the right eye visual acuity is 0.8 due to slight amblyopia. There are no corneal changes which would indicate bilaterality of the disease.

Conclusions: Our findings must be interpreted as an atypically localised superior pellucid marginal corneal degeneration with rupture of Descemet's membrane followed by acute corneal hydrops. When reduction of visual acuity or pain occurs a surgical treatment by perforating/lamellar corneo-scleroplasty can be performed stopping the progression of the disease and achieving a stable optical rehabilitation and absence of pain even after decades.