Terrien's Marginal Degeneration Research Articles

Terrien marginal degeneration presenting with corneal perforation

PurposeTerrien marginal degeneration is a slow‐progressing, bilateral but asymmetric degeneration of the peripheral cornea. The spontaneous corneal perforation is rare. To report a imminent corneal perforation as a presenting sign Terrien marginal degeneration, treated with amniotic membrane implant.MethodsA 70‐year‐old man presented with complaints of pain, redness,and decreased visión in the left eye (LE). Examination revealed peripheral corneal thinning 360º with superficial vascularization and secondary lipid keratopathy. There was an area of more thinning at the 5 o′clok position. His best‐corrected visual acuities (BCVAs) were 90°−4 + 4: 0.8 on the RE and 80°−3.75 + 4: 0.6 on the LE. Corneal topography with Orbscan of the LE revealed high oblique astigmatism with Sim K values of 48.75D at 140° and 43.25D at 50°. AS‐OCT showed corneal thinning and cavity formation with intact epithelial layer and affected endothelium in the thinned área.ResultsDue to the imminet corneal perforation was selected to make an amniotic membrane implant. Several circular layers of amniotic membrane rolled up on themselves and placed in the thinned área, fixing them with nylon 10‐0 suturing it to perilesional healthy cornea. The post‐operative AS‐ OCT reveals good adhesion of the amniotic membrane and corneal thickness recovery.ConclusionsTerrien marginal degeneration is an unusual cause for a spontaneous corneal perforation. Patients with this disorder should be warned about this possibility. Surgery (lamellar graft, amniotic membrabe implant) can preserve corneal integrity and is indicated when conventional options fail to maintain vision or if perforation is imminent.

Unusual Terrien's marginal degeneration, unilateral presentation in a young female

A 26-year-old female presenting with unilateral loss of vision, was incidentally detected to have peripheral corneal thinning, clinically diagnosed to be Terrien's marginal degeneration in the right eye. Unusual presentation of a rare disorder, which is usually more common in males and bilateral, although asymmetrical.

Terrien Marginal Degeneration: Clinical Characteristics and Outcomes.

To describe outcomes of patients with Terrien marginal degeneration. Retrospective case series. Database review of 25 patients (43 eyes) seen over 10 years (2004-2013) at Toronto Western Hospital cornea clinic. Outcome measures included demographics, location of disease, topographic astigmatism, visual acuity, coexisting ocular disease, and surgical management. Mean age at presentation was 44 years (range, 20-82 years) and 54% were male. Eighteen patients (72%) had bilateral disease. Mean follow-up was 30.3months. Mean topographic astigmatism was4.02 diopters (D) at 5 degrees. Mean change in astigmatism 1 year from baseline was 0.75 D; at 2 years was 1.22 D; and at 3 years was 1.68 D. Mean best spectacle-corrected visual acuity (BSCVA) at presentation was 20/46 and 20/48 at last follow-up. Eyes requiring surgery (23.3%) had mean BSCVA of 20/81 at presentation and 20/106 after surgery. Five eyes perforated: 4 spontaneously, and 1 from trauma. Three eyes (6.9%) presented with pseudopterygium. Two eyes (4.7%) had intracorneal cysts. Fourteen patients (56%) presented with ocular surface inflammation. Terrien marginal degeneration is a slow-progressing, bilateral but asymmetric degeneration of the peripheral cornea. Men over 40 are more commonly affected. Stromal thinning, vascularization, lipid deposition, and against-the-rule astigmatism are classic signs. Though typically noninflammatory, a variant form characterized by prominent inflammation exists. Surgery (lamellar graft) can preserve corneal integrity and is indicated when conventional options fail to maintain vision or if perforation is imminent. Perforations are rare but can result in significant vision loss.

Peripheral deep anterior lamellar keratoplasty using a cryopreserved donor cornea for Terrien’s marginal degeneration

To evaluate the clinical efficacy of peripheral deep anterior lamellar keratoplasty (DALK) using a cryopreserved donor cornea for Terrien's marginal degeneration (TMD). Thirty-one eyes of 27 patients with TMD underwent peripheral DALK using cryopreserved donor corneas. According to the distance between the inner edge of the lesion and the limbus, a ring-shaped or D-shaped DALK was performed. All grafts were stored at -20 °C. Cryopreserved corneoscleral rims were prepared for ring-shaped grafts and cryopreserved whole eyeballs were prepared for D-shaped grafts. The general conditions, intraoperative performance, postoperative corneal reconstruction, astigmatism, best corrected visual acuity (BCVA), and various complications were analyzed. Ring-shaped DALK was performed in 28 eyes and D-shaped DALK was performed in 3 eyes. Postoperative follow-up time was (28.4±24.8) months. There was evidence of inflammation before surgery in 12 eyes (38.7%) and intraoperative perforation occurred in 13 eyes (41.9%). The corneal structures of all eyes were reconstructed. Postoperative astigmatism and BCVA showed improvement (both P=0.00) except for cases that underwent D-shaped DALK. Ten eyes (32.2%) developed transient ocular hypertension and one eye (3.2%) developed secondary glaucoma. No primary disease recurrence or corneal allograft rejection was observed. Peripheral DALK for TMD using cryopreserved donor tissue is an effective technique that eliminates rejection and extends the use of donor eyes. Inflammatory history or intraoperative perforation has no adverse effect on graft recovery. However, D-shaped DALK did not achieve good visual outcomes.

Terrien's marginal degeneration: Nordic collaborative study

AbstractPurpose The objective of our study is to describe the clinical and imaging features of Terrien's marginal degeneration (TMD) and subsequently to define the clinical diagnostic criteria for TMD. Lastly we aim to detect the natural course of this disease.Methods Our study design is cross‐sectional and includes TMD patients diagnosed in Helsinki University Eye Hospital, Aarhus University Hospital, Sahlgrenska University Hospital and Oslo University Hospital by corneal specialists. Analysis include gender distribution, age, symptoms, as well as clinical, topographical and imaging findings. The analysis is based on descriptive variables due the limited sample size.Results We present here clinical findings of Finnish TMD patients. All patients were asymptomatic and did not suffer from any systemic autoimmune diseases. Topographical analysis showed high astigmatism due to severe corneal thinning as evidenced by optical coherence tomography imaging.Conclusion TMD is a bilateral corneal disease. Patients are commonly asymptomatic and visit an ophthalmologist because of deteriorated vision due to increasing astigmatism. TMD is thus diagnosed by coincidence. TMD does not seem to be associated with any systemic autoimmune diseases and it is commonly non‐progressing.

Age distribution of various corneal diseases in China by histopathological examination of 3112 surgical specimens.

To analyze the age distribution of corneal disease in China by histopathological examination. A retrospective histopathological analysis of the prevalence and age distribution of corneal disease was performed on corneal specimens from 3112 patients who underwent corneal surgery between 2001 and 2012 in the Henan Eye Institute. Infectious keratitis (2212 specimens, 71.08%) was the most common corneal disease. The distribution of age groups was significantly higher after age 30 (16.29%), reaching a peak by age 40 (22.04%) and remaining high until age 59 (21.05%, P < 0.01). The highest age-specific percentage of corneal infection was between the ages of 50 and 59 (83.21%, P < 0.01). Noninfectious corneal diseases occurred mainly in the age range of 10 to 19 years (42.47%) and 20 to 29 years (40.78%). Between the ages of 10 and 19, the leading pathologic diagnosis was keratoconus, accounting for 21.29% of cases. In patients aged 80 and older, the most common noninfectious corneal diseases were Terrien-marginal degeneration (8.33%) and Mooren's ulcer (4.17%). The highest incidence of corneal tumor was seen between the ages of 0 and 9 years (65.83%) and age 80 and older (45.86%; P < 0.01). The age distribution of corneal diseases in the Chinese population is distinct from other countries. The corneal diseases tend to increase in prevalence in middle age, especially for infectious corneal disease. The prevalence of corneal tumor is high in early and late life. Corneal degenerative disease occurs predominately in seniors.

Partial lamellar keratoplasty for peripheral corneal disease using a graft from the glycerin-preserved corneoscleral rim.

To evaluate the feasibility of partial lamellar keratoplasty (LK) for treatment of peripheral corneal disease (PCD) using a graft from the corneoscleral rim preserved in glycerin. Patients who underwent LK for PCD at Shandong Eye Hospital from January 2006 to December 2012 were included. Corneoscleral rims of donor grafts, out of which the corneal buttons had been punched and used for penetrating keratoplasty, were preserved in glycerin and used for LK procedures. A trephine of 7.5-8.0mm was used to mark the central cornea. An excision was made according to the size of the lesions, after which a partial ring-shaped corneoscleral graft was transplanted to repair the peripheral cornea. Visual acuity, refractive astigmatism, and complications were monitored. There were 26 patients (28 eyes), including 15 patients with Terrien's marginal degeneration, eight with Mooren's ulcer, two with Wegener's granulomatosis, and one with peripheral corneal ulcer. The mean follow-up was 15months (range, 6-36months). Uncorrected visual acuity was improved by a mean of 3.17 Snellen chart lines (P = 0.010), and best spectacle-corrected visual acuity was improved by a mean of 0.85 Snellen chart lines (P = 0.045) at 6months after surgery. The refractive astigmatism decreased by 5.1 ± 8.6 diopters (P = 0.003). Two patients suffering graft ulcers (recurrence) were successfully treated by a second partial LK. No graft rejection occurred. Partial LK using a graft from the corneoscleral rim appears to be effective in the treatment of patients with PCD. This approach saves the donor graft, which is important in the countries or regions with scarcity of donor tissue.

Terrien’s Marginal Degeneration Accompanied by Latticed Stromal Opacities

ABSTRACTPurposeWe report a case of Terrien’s marginal degeneration (TMD) with a unilaterally typical narrow band of peripheral corneal stroma thinning, accompanied by the presence of an unusual network of opacities diffusing throughout the anterior stroma layers.Case ReportA 43-year-old woman presented with superior nasal peripheral corneal thinning and an unusual network of polygonal stromal opacities in the anterior corneal stroma of the right eye. Latticed corneal changes were unusually extensive and distributed diffusely in the stroma. No abnormalities were found in the corneal epithelium and in the basal epithelial cells. No noticeable changes were found in the left eye. Because of a progressively worse ocular irritation of the right eye, a diagnosis of TMD was made for this patient.ConclusionsThis case of TMD accompanied by keratopathy was unusual. The branching stromal lattice pattern of the corneal opacities was difficult to distinguish from lattice corneal dystrophy. In this case, the polygonal stromal opacities were located in the anterior corneal stroma and therefore were distinguished from a similar manifestation in posterior crocodile shagreen.

Fuchs’ Superficial Marginal Keratitis Managed with Circumferential Marginal Corneoscleral Lamellar Patch Graft

To report a case of Fuchs' superficial marginal keratitis managed with circumferential marginal corneoscleral lamellar patch graft. Interventional case report. A 34-year-old man presented with several years' history of ill-defined symptoms of binocular ocular irritation associated with vision loss, mostly in the left eye. A superior marginal corneal thinning was found at biomicroscopy of the left eye, with 2 finely vascularized descemetoceles, and a gray epithelial demarcation line without lipid infiltrates. The right eye was clinically normal. Anterior segment optical coherence tomography demonstrated an important corneal thinning from the 7 o'clock to 3 o'clock positions, without scleral involvement. A circumferential marginal corneoscleral lamellar patch graft was done involving 3 mm of sclera and 3 mm of cornea. Fuchs' superficial marginal keratitis is a rare entity, mostly affecting young adults. It should be considered part of a spectrum of corneal thinning disorders, together with Terrien's marginal degeneration. Definitive treatment with a marginal corneoscleral lamellar patch graft with or without conjunctival autograft is suggested.

Depiction of Cavity Formation in Terrien Marginal Degeneration by Anterior Segment Optical Coherence Tomography

To evaluate detailed morphologic changes of Terrien marginal degeneration (TMD) using anterior segment optical coherence tomography (AS-OCT). Two patients with TMD were examined by 1310-nm wavelength swept source optical coherence tomography, corneal topography, and slit-lamp microscopy. AS-OCT depicted corneal thinning with intact epithelial layer in the peripheral cornea of both patients. Moreover, AS-OCT images of both patients clearly showed cavity formation surrounded and enclosed by intact epithelial and endothelial layers in the thinned area. We demonstrate here the existence of cavity formation in the peripheral cornea with TMD. This observation suggests a possibility that corneal thinning in TMD is associated with cavity formation in the corneal stroma. AS-OCT is useful for the observation of detailed morphologic changes of diseased cornea.

Central corneal epithelium self-healing after ring-shaped glycerin-cryopreserved lamellar keratoplasty in Terrien marginal degeneration.

Foundation items: National Natural Science Foundation of China (No. 30973247); Shanghai Excellent University Teacher Foundation, China (No. 1500144019)

Ring-shaped corneoscleral lamellar keratoplasty guided by high-definition optical coherence tomography and Scheimpflug imaging for severe Terrien’s marginal corneal degeneration

To evaluate the clinical efficacy of ring-shaped corneoscleral lamellar keratoplasty under guidance of high-definition optical coherence tomography (HD-OCT) and Scheimpflug imaging for severe Terrien's marginal corneal degeneration. Twenty-five patients (25 eyes) with Terrien's marginal corneal degeneration and ≥ 6 diopters of astigmatism were treated at the Shandong Eye Institute from January 2008 through December 2010. In these patients, the diseased area was > 1 quarter of the cornea, and the thickness at the thinnest corneal area was < 150 μm with residual corneal stroma. Ring-shaped corneoscleral lamellar keratoplasty was performed, and the depth and width of dissection was set according to the HD-OCT and Pentacam examinations. Data of complications, surgical effects, visual acuity, and astigmatism were collected. No intraoperative corneal perforation occurred. No patients had liquid accumulation between the graft and the recipient. At 1 year after surgery, the average best-corrected visual acuity improved to 6/9 from preoperative 6/36, and the average astigmatism reduced to 4.37 (2.04) diopters from preoperative 13.39 (7.52) diopters. There was no recurrence of the disease or corneal graft rejection in the follow-up period. Ring-shaped corneoscleral lamellar keratoplasty guided by HD-OCT and Pentacam imaging appears to be effective in the treatment of severe Terrien's marginal corneal degeneration. Surgical perforation can be significantly reduced.

Peripheral Ulcerative Keratitis Associated With Vasculitis Manifesting Asymmetrically as Fuchs Superficial Marginal Keratitis and Terrien Marginal Degeneration

To report a case of bilateral peripheral corneal thinning with an appearance of Fuchs superficial marginal keratitis in the right eye and Terrien marginal degeneration in the left eye. Interventional case report. A 28-year-old woman presented with bilateral corneal thinning and a pseudopterygium in the right eye and peripheral corneal thinning in the left eye. Laboratory examinations were negative. Over the next several years, the patient experienced periodic episodes of bilateral ocular redness and discomfort associated with gradual corneal thinning. However, other signs of progression were different in each eye. Pseudopterygium growth was observed in the right eye, consistent with Fuchs superficial marginal keratitis. Progressive lipid deposition at the leading edge of corneal thinning was observed in the left eye, consistent with Terrien marginal degeneration. Chronic topical corticosteroid therapy was initiated with little, if any, subsequent disease progression. A perinuclear antineutrophil cytoplasmic antibody (p-ANCA)-positive vasculitis was subsequently diagnosed. Fuchs superficial marginal keratitis and Terrien marginal degeneration may represent different clinical features of the same disease process. Laboratory investigation for underlying systemic disease may be warranted in patients with the appearance of either disorder. Chronic antiinflammatory therapy may be effective in limiting the progression of corneal thinning in these diseases.

In Vivo Confocal Microscopy in Terrien Marginal Corneal Degeneration: A Case Report

To report an advanced case of Terrien marginal corneal degeneration (TMD) analyzed by means of confocal microscopy. A 62-year-old woman with TMD underwent a routine ophthalmological examination and was evaluated for the disease by using in vivo laser scanning confocal microscopy with the Heidelberg Retina Tomograph 2 Rostock Cornea Module. The slit-lamp examination of the right eye showed an upper peripheral cornea thinning associated with a deposition of refractile yellow-white materials in the anterior stroma. Superficial radial vessels in the upper peripheral cornea were the only pathological findings of the left eye. Confocal microscopy of the right eye lesion showed deposition of hyperreflective material, and the stroma below presented a honeycomb-like pattern. Inflammatory cell infiltration, abnormal subbasal nerve plexus, epithelial basal membrane defects, irregular Bowman membrane, and the presence of activated keratocytes were observed in the cornea of both eyes. In vivo confocal microscopy can detect subtle corneal changes in an advanced case of TMD, which may be signs of subclinical inflammation.

Scanning slit topography: Diagnostic boon in presumed unilateral Terrien's marginal degeneration

To describe the scanning slit topography in two children with presumed unilateral Terrien's marginal degeneration. Two girls aged 9 and 12 years presented with redness and reduced vision in their right eyes. The younger patient had scarring adjacent to the superior limbus, with vessels coursing across, and lipid deposition. Visual acuity improved to 20/20 from 20/400 with +1.0 -3.5×90°. The older patient had similar features inferiorly. Her visual acuity improved to 20/20 from 20/40 with -3.5×65°. Their left eyes were normal. A diagnosis of unilateral Terrien's Marginal Degeneration (TMD) was made. Scanning slit topography (Orbscan II™, Orbscan Inc., Salt Lake City, UT) of the right eyes revealed high against-the-rule astigmatism. The left eyes showed elevations of anterior and posterior corneal surface above the best-fit-sphere, skewing of principal meridians and increased astigmatism from 3.0 to 5.0mm zones. Based on these findings bilateral asymmetric TMD was diagnosed. Though the diagnosis of TMD is mainly clinical, scanning slit topography findings in TMD have not been described earlier and are useful in establishing the diagnosis of bilateral asymmetric TMD.

Laser Scanning In Vivo Confocal Microscopy in a Case of Terrien Marginal Degeneration

To describe corneal microstructural modifications in an early case of Terrien marginal degeneration by means of laser scanning in vivo corneal confocal microscopy. A 20-year-old man affected by Terrien marginal degeneration in his right eye was examined with the Heidelberg Retina Tomograph 2 with a Rostock Cornea Module. The patient presented a corneal lesion with marked superior thinning and no clinical signs of inflammation. The left eye showed no clinical signs of degeneration. The right eye showed irregular Bowman's membrane, amorphous-appearing substance co-localizing with lipid exudation, and needle-like hyperreflective material in the affected peripheral corneal region. Abnormally scarce and branched sub-basal plexus nerves as well as numerous activated keratocytes, sometimes organized in hyperreflective nests, were observed in the central cornea of the same eye. Although the left eye was clinically unaffected, activated keratocytes were detected in its anterior central stroma. In vivo confocal microscopy supports the hypothesis of a mild inflammatory state with an atypical sub-basal nerve pattern in a patient affected by Terrien marginal degeneration. This new technique shows promise in studying this still elusive pathology.

Keratoplasty in the management of Terrien's marginal degeneration

To evaluate the efficacy and safety of keratoplasty combined with corneal foci resection in the treatment of Terrien's marginal degeneration (TMD). In this nonrandomized retrospective case series, the records of 48 eyes from 40 patients with TMD who received keratoplasty from January 1995 to December 2005 in Zhongshan Ophthalmic Center were reviewed retrospectively. Orbscan topography examination was undertaken in 8 eyes of 8 patients and the refractive error of 9 eyes from 9 patients was tested before and after the operation. The mean age of the patients was (30 +/- 6) years old. The mean follow up period was (7 +/- 6) years. It took (3 +/- 1) months postoperatively to obtain a stable visual acuity. Before operation, the naked eye and best corrected visual acuity (VA) (Q25,Q75) were (0.05,0.4), and (0.1,0.5), respectively, while improved to (0.2,0.6) and (0.4,0.7) after operation, respectively (Z = 4.63, 3.85, both P<0.01). VA was improved in 39 eyes (81.3%), remained at the same level in 4 eyes (8.3%), decreased 1-2 lines in 3 eyes (6.3%), and decreased more than 2 lines in 2 eyes (4.1%) after the operation. The median spherical diopter and cylinder diopter were (-2.00 D, -8.50 D) and (2.50 D,12.00 D) before operation, while decreased to ( -1.25 D, -4.75 D) and (0.75 D, 4.25 D) after operation (Z= 2. 49, 2.54, P = 0.01, 0.01). The improvement in Sim K's astigmatism, astigmatism in 3 mm zone and mean power in 3 mm and 5 mm zone were reduced statistically significant after the operation (P <0.05); with the exception of astigmatism in the 5 mm zone, which was not reduced significantly after the operation (Z = 1.86, P = 0.06) . The operative complications included corneal perforation during operation in 5 eyes (10.4%), hydrops between graft and recipient interface in 8 eyes (16.7%), epithelial in-growth in 4 eyes (8.3%), choroidal detachment in 1 eye (2.1%), graft rejection in 7 eyes (14.6%), and recurrence in 3 eyes (6.3%). Secondary surgery was required in 5 eyes (10.4%) for interface hydrops, epithelial in-growth and recurrence of TMD. Keratoplasty combined with foci resection is effective and safe in the treatment of TMD. This procedure can preserve and improve the visual activity.

Lamellar keratoplasty for treatment of peripheral corneal perforation

To explore the indications, efficacy, and complications of different types of lamellar keratoplasty (LKP) for peripheral corneal perforation. In our nonrandomized retrospective case series, 40 cases (42 eyes) with peripheral corneal perforation at Zhongshan Ophthalmic Center between January 2003 and June 2006 underwent semi lunar LKP (14 eyes), crescent LKP (11 eyes), biconvex LKP (7 eyes), ring-shaped LKP (5 eyes), and total LKP (5 eyes). The selection of various procedures was based on the size and shape of corneal ulceration and perforation. Postoperative visual acuity (VA), corneal clarity, astigmatism, and complications were studied during a follow-up of 3 to 21 months. The initial causes and recurrence for peripheral corneal perforation were analyzed. The initial causes of corneal perforation were mostly Terrien's marginal degeneration (35.7%), Mooren's ulcer (31.0%), herpes simplex keratitis (11.9%), chemical/thermal burns (9.5%), and corneal ecstatic diseases (7. 1%). The lamellar graft attached to the host well and was transparent in 34 eyes (81.0%) following LKP. VA less than 0.1 were presented in 13 eyes (31.0%) preoperatively and in 3 eyes (7.1%) postoperatively. VA in 27 eyes (64.3%) improved after LKP, and best-corrected VA in nine eyes (21.4%) was 0.5 or better. The mean corneal astigmatism was (7.23 +/- 1.22) D three months postoperatively, and this decreased to (3.72 +/-1.76) D in six to nine months postoperatively when sutures were removed. The postoperative complications included graft-host interface effusion (8 eyes, 19.1%), secondary glaucoma (8 eyes, 19.1%) and reoccurrence of primary diseases, including Mooren's ulcer (3 eyes, 23. 1%) and herpes simplex keratitis (1 eye, 20.0%). LKP is effective to restore the integrity of the eyes with peripheral corneal perforation. Choosing the appropriate type of LKP based on different shape of corneal perforations can remove the corneal lesion and spare the normal tissue to the maximum. Moreover, the well-matched graft configuration can reduce the postoperative astigmatism, helping restore or improve the vision in most cases.

Therapeutic Keratoplasty for Corneal Perforation

To report the clinical results, postoperative progress, and complications after therapeutic keratoplasty for corneal perforation. Twenty consecutive eyes (20 patients) that underwent therapeutic keratoplasty between December 2003 and May 2006 were included. The eyes were evaluated retrospectively for the cause of the corneal perforation, the type of surgical technique and intraoperative complications, anatomic cure rates, graft clarity, visual prognosis, and postoperative complications. The causes of corneal perforation were herpetic keratitis (n = 5), bacterial ulcer (n = 1), fungal ulcer (n = 1), neurotrophic ulcer (n = 3), rheumatoid arthritis (n = 2), Mooren ulcer (n = 2), Terrien marginal corneal degeneration (n = 1), keratoconus (n = 1), and Wegener granulomatosis (n = 1). In 3 cases, the etiology was unknown. Six cases had a previous history of corneal transplantation. Anatomic cures were obtained in 16 (80%) of 20 eyes after the first transplantation procedure. Visual acuity (VA) equal to or better than the preoperative level was achieved in 17 (85%) of 20 eyes. The graft transparency rate was 67% in 15 eyes that underwent central penetrating keratoplasty with fresh donor tissue. Major postoperative complications included cataract (n = 6), glaucoma (n = 4), and recurrent disease (n = 3). Keratoplasty is valuable for maintaining the ocular integrity and VA. In cases with severe preoperative inflammation of the anterior segment, it is difficult to achieve transparency after the first graft.

Terrien Marginal Degeneration Presenting With Spontaneous Corneal Perforation

To report spontaneous corneal perforation as a presenting sign of unilateral Terrien marginal degeneration in a young adult. A 19-year-old woman presented with sudden loss of vision in her left eye of 1-day duration. Examination revealed superior corneal thinning with superficial vascularization and secondary lipid keratopathy. There was an area of corneal perforation at the 11 o'clock position, leading to a shallow anterior chamber, ocular hypotony, and a hypotonic maculopathy. The right eye was normal on biomicroscopy. The area of perforation was confirmed by a positive Seidel test. A 22-mm-diameter bandage contact lens was fitted, which led to closure of the perforation and reformation of the anterior chamber. Orbscan corneal topography confirmed superior corneal thinning, 2.3 D of against-the-rule astigmatism, and 3.4 D of irregular astigmatism in the central 3-mm zone. Topography of the right showed no apparent abnormality. Terrien marginal degeneration is an unusual cause for a spontaneous corneal perforation. Patients with this disorder should be warned about this possibility.