I. Buerkleur syndrome type III alias.
①Corneal lattice-like dystrophy syndrome;
② corneal lattice-like dystrophy;
⑧ corneal lattice-like dystrophy;
④retinal corneal degeneration;
⑤ reticular corneal dystrophy;
(6) Lattice degeneration;
(7) Lattice keratitis;
⑧Familial hereditary corneal dystrophy (lattice type);
⑨Dimmer (Dimmer) corneal dystrophy;
⑩Haab-Dimmer syndrome; ⑪Biber-Haab-Dimmer syndrome.
Summary: The main feature is vitreous degeneration of the parenchymal layer of the cornea, which is seen in children as early as 2 years old; symptoms usually appear at the age of 20-30 years, and blindness can occur at the age of 40 years, and it can occur in both sexes, usually in one eye.
Etiology: the cause is unclear, autosomal dominant inheritance. Pathology can be seen in the parenchymal layer of the cornea with vitreous degeneration, no mucopolysaccharide accumulation, amyloid material, produced by the corneal cells.
IV. Ocular features.
1, vision loss is progressive, the corneal epithelium is not flat, the corneal parenchyma layer appears numerous microscopic latticework, cross-shaped, cobweb-like gray-white cloudy lines, the clouding is largely confined to the central and peripheral parts of the cornea between the boundaries are clear.
2, the lesion can gradually expand to the periphery, but does not yet reach the corneal edge. There are grayish-white dot clouding between the lines and each other, and the cornea between the clouding points is transparent. Late stage of the lesion is thickened due to corneal edema and the lattice lesion is blurred.
3, pain due to recurrent erosions.
V. Systemic features: no special manifestations.
VI. Differentiation.
Because of the atypical symptoms of this disease, if not carefully examined, it is easy to misdiagnose as keratitis or corneal degeneration, but the corneal clouding of keratitis is not characteristic like the corneal clouding of this disease, even if it is atypical dendritic clouding, there is no appearance of finely dendritic refractive contours. Facial corneal degeneration is the degeneration of corneal tissue and its diminished function, and corneal dystrophy are two clinically distinct corneal diseases.
Corneal degeneration also has similar complaints to this disease, but examination will reveal that corneal degeneration is mostly secondary to ocular or systemic disease, without characteristic corneal manifestations and no family history of corneal degeneration; whereas this disease has a genetic history and no other ocular or systemic disease is found. The main point of differentiation with other corneal dystrophy diseases is also from the typical clouding of the cornea, which is not difficult to distinguish. Timely and accurate diagnosis and effective treatment should be carried out to actively control the development of the disease, which can
Avoid unnecessary use of medication due to misdiagnosis. Once misdiagnosis can lead to prolonged and increased medication, which on the one hand destroys the immune defense system of the ocular surface and dysregulates the normal flora of the conjunctival capsule; on the other hand, it can induce new corneal lesions due to drug toxicity, aggravating the disease and increasing the patient’s pain. Therefore, it is necessary for ophthalmologists to properly understand this disease.
Seven, treatment: perform corneal transplantation.