The most common Stromal dystrophy seen in Fellowship
The most likely stromal dystrophy to recur after keratoplasty.
Inheritance Pattern
AD
Visual Acuity
LCD-1: progressive visual impairment, which starts in the first decade of life.
LCD-2: Visual impairment usually occurs within the third or fourth decade of life.
FACE AND FACIAL SYMMETRY
Systemic Amyloidosis
Macroglossia & Periorbital papules
Facial Paralysis
Mask-like facial expression.
EYELIDS AND ADNEXA
Droopy eyelid – LCD 2
Incomplete eyelid Closure – LCD2
CORNEA
Slit-lamp exam reveals the presence of subepithelial spots originating from the center, exhibiting a widespread haziness, and resembling a ground-glass texture.
The stroma has refractile, branching lines(sometimes with dots). The lines may have double contours in advanced cases.
When using retro-illumination, lattice-like lines can be observed within the stromal layer, extending outward from the central cornea towards the periphery.The periphery of the cornea is spared.
Type I, classic type, type AA amyloid, no systemic amyloid deposition
Type II, lattice dystrophy with coexistent systemic amyloidosis, the lines are less numerous and located more peripherally.
Type III, mid-stromal and larger than those with type I, the deposits are of type AP.
Check for Associated dry eye and contributors - due to neuropathy assorted with LCD 2 – Diminished blink reflex and limited contraction of the orbicularis muscle, both controlled by the trigeminal and facial nerves respectively.
Check Corneal sensitivity – reduced in LCD 1
Fluorescein dye test - Epithelial erosions are more common in LCD 1 than in LCD2.
SYSTEMIC CONDITIONS
Systemic Amyloidosis – LCD 2 – severe skin laxity, neuropathy, facial paralysis.
QUESTIONS:
What is the Triad of Symptoms for LCD 2?
The Triad of symptoms includes presence of lattice corneal dystrophy, loose skin, and progressive cranial and peripheral neuropathy.
What are the presenting symptoms of Lattice Dystrophy?
LCD-1: Individuals diagnosed with LCD typically experience ocular discomfort and gradual decline in vision, beginning in childhood with LCD Type 1. These symptoms typically affect both eyes, although they may appear unevenly, and often include reduced corneal sensation.
LCD-2: Conversely, LCD Type 2 is characterized as a systemic disorder with ocular manifestations, where visual decline often emerges as one of the initial signs, usually manifesting in the third or fourth decade of life. Alongside ocular symptoms, individuals with LCD Type 2 might also encounter skin and neurological complications, alongside other organ dysfunctions..[1]
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