Terriens marginal degeneration produces superior nasal peripheral corneal thinning, leaving the epithelium intact. It is intended to help eyecare providers recognize the condition and. This condition occurs at positions other than the 3. Less than 25% of the resident cells in the terriens marginal. The cause of this particular corneal degeneration remains unknown and there are a great variety of changes in clinical characteristics, course, and prognosis between. Superior pellucid marginal corneal degeneration eye. Nonetheless, this work also emphasizes the importance of acoct and videokeratography in followup. Lysosomal enzymes in tear fluids from patients with. To document an association between terrien s marginal degeneration and posterior polymorphous dystrophy methods. Terrien s marginal degeneration is a rare bilateral condition starting with opacification and vascularisation of the periphery of the cornea progressing by degeneration of the stroma to the formation of a gutter and finally resulting in ectasia with seventy five percent of the patients being males and two thirds being older than 40 years.
Terriens marginal degeneration national health portal. Marginal corneal degeneration as described by terrien is an uncommon disease, with less than 200 cases described in the literature. Also, you can see white lipid deposit, vessels growing. This video shows an eye with terriens marginal degeneration. Immunohistochemistry of terriens and moorens corneal. Terriens marginal corneal degeneration peripheral corneal thinning is also a characteristic of terriens marginal corneal degeneration. It causes a slowly progressive noninflammatory, unilateral or asymmetrically bilateral peripheral corneal thinning and is associated with corneal neovascularization, opacification and lipid deposition. Pdf partial and total descemets detachments in a patient. Terriens, cornea, ectasia, anterior segment background terriens marginal degeneration tmd is a rare, slowly progressive, peripheral corneal ectasia of unknown etiology. It is usually bilateral but may be very asymmetric. Pellucid marginal corneal degeneration pmcd is a bilateral noninflammatory ectatic peripheral corneal disorder usually involving the inferior portion of.
Pdf terriens marginal corneal degeneration is a rare disease of the peripheral cornea characterized by vascularisation, opacification, lipid. The cases described would seem to represent the spectrum of diseases to be seer in terrien s marginal corneal degeneration. Three specimens studied were lamellar resections and four were fullthickness corneal segments. Terriens disease is an uncommon terrien himself saw only three cases in thirty years of practice corneal condition characterized by vascularization, opacification, lipid deposition, and thinning. Initially, tmd presents as small, yellowwhite, stromal.
Bilateral terriens marginal degeneration and posterior polymorphous dystrophy in a patient with rheumatoid arthritis. All cases had stromal thinning, vascularization, lipid keratopathy and local absence of. It typically presents with painless vision loss affecting both eyes. Groupofcornealdiseasesthataregeneticallydetermined andhavebeentraditionallyclassi. This teaching case report highlights the presentation, differential diagnosis and management and treatment of tmd. Mooren s ulcer there is but may be bilateral in younger patient peripheral ulcerative keratitis located in the interpalpebral region. All cases had stromal thinning, vascularization, lipid keratopathy and. Systemic scleroderma is a progressive, potentially fatal multisystem. This condition occurs at positions other than the 3 and 9oclock positions, and it grows. Terriens marginal degeneration accompanied by latticed. The condition is often bilateral and may occur at any age, although it typically occurs in middleaged males. Apr 15, 2019 to determine early keratoconus or pellucid marginal degeneration, look at the peripheral cornea at 5, 6 and 7 oclock.
The peripheral cornea, predominantly superiorly, undergoes lipid deposition, vascularisation, opacification and stromal dunning leading to gutter formation, ectasia and eventual corneal perforation. We examined biochemically the lysosomal enzyme activities in tear fluids from patients with mild myopia, senile cataract, and terriens marginal corneal degeneration. Corneal collagen crosslinking for terrien marginal degeneration. Terrien s marginal degeneration signs and symptoms. Terriens marginal degeneration associated with posterior. Three case reports illustrate a manifestation of terriens marginal corneal degeneration, the pseudopterygium. Corneal collagen crosslinking for terrien marginal. Terrien s marginal degeneration is an uncommon but distinct variety of marginal thinning of the cornea.
The clinical and histopathologic features of pellucid and terriens marginal degeneration are similar. Corneal collagen crosslinking for management of tmd remains. With progression, large degrees of astigmatism occur and perforation is a threat. Initially, tmd presents as small, yellowwhite, stromal opacities composed of lipids with some superficial vascularization, which begins superiorly and spreads circumferentially. It is intended to help eyecare providers recognize the condition and provide proper patient education and management. When contact lenses fail, corneal transplant is the best and most successful surgical option. Partial and total descemets detachments in a patient with severe terriens marginal degeneration and juvenile idiopathic arthritis. A 23yearold saudi man presented with decreased vision, peripheral corneal thinning with vascularization and scarring, and abnormalities of the posterior stroma and descemets membrane. There are asymmetries between the eyes, but the changes seem characteristic. When present, the pseudopterygium has a characteristic appearance, which is of diagnostic value. Pellucid marginal degeneration is a bilateral, slowly.
Terriens marginal degeneration is a rare bilateral condition starting with opacification and vascularisation of the periphery of the cornea progressing by degeneration of the stroma to the formation of a gutter and finally resulting in ectasia with seventy five percent of the patients being males and two thirds being older than 40 years. Moorens ulcer there is but may be bilateral in younger patient peripheral ulcerative keratitis located in the interpalpebral region. Pdf bilateral terriens marginal degeneration and posterior. Contact lenses are the most common treatment modality. The disease first presents as a peripheral corneal haze. Of the four fullthickness specimens, three were semilunar and one was an annular doughnutshaped specimen. Terrien marginal degeneration tmd occurs bilaterally but asymmetrically, with onset usually in middle age or older see did you know. Terrien marginal degeneration, a rare disease resulting in peripheral corneal thinning, commonly occurs in middleaged males and usually. To report an interesting case of concomitant bilateral terriens marginal degenerationlike corneal ectasia and posterior polymorphous corneal dystrophy in a young man with quiescent rheumatoid arthritis. Corneal collagen crosslinking for management of tmd. The classic form most commonly affects patients older than 40 years of age without rheumatoid arthritis and progression is extremely slow.
Terrien marginal degeneration is a noninflammatory, unilateral or asymmetrically bilateral, slowly progressive thinning of the peripheral corneal stroma. Ten patients 20 eyes with tmd treated in the department of ophthalmology, xiangya hospital, and 10 healthy controls 20 eyes were included in the current study. Terriens marginal degeneration is a distinct marginal thinning of the cornea which causes high degree of againsttherule astigmatism. To determine early keratoconus or pellucid marginal degeneration, look at the peripheral cornea at 5, 6 and 7 oclock. Pdf to report an interesting case of concomitant bilateral terriens marginal degenerationlike corneal ectasia and posterior polymorphous corneal. Iatrogenic corneal perforation in terrien marginal degeneration.
Terrien s disease is an uncommon terrien himself saw only three cases in thirty years of practice corneal condition characterized by vascularization, opacification, lipid deposition, and thinning. Case report kg and tmd are uncommon corneal ectatic conditions, characterized by protrusion and thinning of the cornea. This is when the cornea thins at the edges toward the limbus. The peripheral cornea, predominantly superiorly, undergoes lipid deposition, vascularisation, opacification and stromal. In vivo confocal microscopy of cornea in patients with. The photographs above demonstrate many of the characteristic features of this disorder.
Systemic scleroderma is a progressive, potentially fatal multisystem connective tissue disease of unknown cause, charac. Terrien marginal degeneration is a painless, slowly progressive thinning of the peripheral corneal stroma. The epithelium is vascularized and there is an overhanging advancing edge. Terriens marginal degeneration tmd is a rare disease with an unknown cause. Shrinkage of conjunctiva, terriens marginal degeneration, and perforation of cornea in rheumatoid arthritis. Normally, the cornea thickens by 20m to 50m as you move to the periphery.
An ocular surface prosthesis for keratoglobus and terrien. A 23yearold saudi man presented with decreased vision, peripheral corneal thinning with vascularization and scarring, and abnormalities of the posterior stroma and descemet s membrane. Despite intensive clinical and laboratory investigation, the etiology of keratoconus remains unclear. Terrien marginal degeneration is a slowprogressing, bilateral but asymmetric degeneration of the peripheral cornea. A peripheral cornea that isnt at least 20m thicker than the central cornea is suspect. Terrien marginal degeneration, a rare disease resulting in peripheral corneal thinning, commonly occurs in middleaged males and usually manifests clinically with decreased visual acuity from increased corneal astigmatism.
Were currently building a brand new site where you can get expert advice on the causes of and treatments for 150 ocular diseases. Cases two and three are quite mild, probably representing the picture of early disease. Pellucid marginal degeneration pmd, is a degenerative corneal condition, often confused with keratoconus. Rarely, it may cause acute vision loss with severe pain due to perforation of the cornea. Terrien s marginal degeneration tmd is a rare, slowly progressive, peripheral corneal ectasia of unknown etiology. Terriens marginal degeneration tmd is a rare, slowly progressive disorder of the peripheral cornea in which the outer perimeter of the limbus presents with a shallow groove, neovascularization, stromal opacities, thinning, and ectasia.
Pdf terriens marginal degeneration accompanied by latticed. Peripheral corneal thinning is also a characteristic of terriens marginal corneal degeneration. This peripheral corneal condition commonly progresses slowly and usually develops with little discomfort, although occurrences of severe pain and inflammation may occur in the later stages. Inflammatory variant of terriens marginal degeneration affects younger subjects and it is characterised by recurrent and disabling. Based on these findings, he was diagnosed with terriens marginal degeneration tmd, given a new prescription, counseled on the progressive nature of the disease and cautioned to wear glasses or eye protection at all times to avoid the risk of accidental perforation. Srinivasan s, murphy cc, fisher ac, freeman lb, kaye sb. Terrien marginal degeneration survey of ophthalmology.
This study was aimed at observing the morphological changes of the cornea with ocular in vivo confocal microscopy ivcm in patients with terriens marginal degeneration tmd. We report the clinicopathologic features of seven cases of terriens marginal degeneration. Lamellar keratoplasty specimens from a patient with terriens marginal degeneration and a patient with moorens ulcer were compared using routine histopathologic and immunohistochemical staining with an avidinbiotinperoxidase complex. Terriens marginal degeneration tmd has traditionally been thought of as a rare, noninflammatory, and slowly. Reversing peripheral thinning in terrien marginal degeneration. As opposed to other causes of peripheral corneal thinning, there is typically no inflammation and the corneal epithelium remains intact. Lysosomal enzymes in tear fluids from patients with terriens. Terrien marginal degeneration, atypical pterygium, corneal perforation introduction terrien marginal degeneration is typically described as a disease of the middle to late age. The cause of terrien marginal degeneration is unknown, its prevalence is roughly equal between males and females, and it.
The cause of terriens marginal degeneration remains unknown. When idiopathic peripheral corneal thinning remains clear, it is regarded as pellucid degeneration. Terriens marginal degeneration tmd is a rare, bilateral, asymmetric disease of unknown aetiology. Jun 15, 2019 based on these findings, he was diagnosed with terriens marginal degeneration tmd, given a new prescription, counseled on the progressive nature of the disease and cautioned to wear glasses or eye protection at all times to avoid the risk of accidental perforation. It is typically characterized by a clear, bilateral thinning ectasia in. Although the exact etiology is unknown, hypotheses focus on possible degenerative and inflammatory origins. When compared to the a preoperative state, a progressive decrease of the keratometric readings at b 1, c 3, and d 5 years after corneal collagen crosslinking was observed in the left eye. Iatrogenic corneal perforation in terrien marginal.
Abstract purpose this case report describes the challenges in fitting corneas using the prosthetic replacement of ocular surface ecosystem prose device in a unique case of bilateral keratoglobus kg with terriens marginal degeneration tmd. Partial and total descemets detachments in a patient with. We report a case of terriens 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. Collagen crosslinking in the treatment of pellucid. The condition is most commonly seen in males past the age of 40. Terrien s marginal degeneration produces superior nasal peripheral corneal thinning, leaving the epithelium intact. Terriens marginal degeneration uncommon marginal thinning males more than females bilateral any age gradual to rapid progression usually asymptomatic until astigmatism noninvolutional corneal degenerations terriens marginal degeneration marginal stromal thinning, opacification superficial vascularization. Bilateral terriens marginal degeneration and posterior. Corneal degeneration an overview sciencedirect topics. Although histopathologically it is considered a variant of keratoconus, it differs in that the marked corneal steepening occurs more inferiorly. Terriens marginal degeneration accompanied by latticed stromal opacities article pdf available in optometry and vision science. This is a symmetrical, ectatic, marginal corneal dystrophy, seen more often in middleaged men. The etiology of this condition is not understood, but classically these eyes present with a band of thinning with a stepoff toward the center. Terriens marginal degeneration tmd is a rare, slowly progressive, peripheral corneal ectasia of unknown etiology.
The ulceration is contiguous with the limbus without intervening clear zone. A 16yearold female with juvenile idiopathic arthritis presented with a onemonth history of decreasing vision and increasing corneal edema in her left eye. Use of hroct to distinguish between inflammatory and non. Tear acid phosphatase activities in terriens degeneration were almost the same as those in mild myopia and senile cataract, while those ofnacetyl. To document an association between terriens marginal degeneration and posterior polymorphous dystrophy methods.