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Mystery Case 1

Mystery Case 1. Optometry 8570 Fall 2007. 31 y.o. AAF presents to U.E.C. for a comprehensive exam. Cc: “I have a headache” “Like a band around the head”; mild to severe; 1 month duration; “sunny days worse, darkness helps” No complaints about vision clarity

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Mystery Case 1

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  1. Mystery Case 1 Optometry 8570 Fall 2007

  2. 31 y.o. AAF presents to U.E.C. for a comprehensive exam • Cc: “I have a headache” • “Like a band around the head”; mild to severe; 1 month duration; “sunny days worse, darkness helps” • No complaints about vision clarity • MHx: Mild M.I. ’99 (no cause found, but mom has heart disease); depression; sickle cell trait; non-infectious pneumonia ’95 and ’98 of unknown etiology; “joint pain when younger, never found cause of that either” • OHx: Told 2 years ago that “I have holes in my cornea”; ocular allergy (Patanol and Allegra prn, none previous 2-3 weeks); reports that she “rubs eyes a lot” • Meds: Celebrex, Wellbutrin • Orientation: yes x 3; Mood/Affect: normal

  3. Objective Findings • W: OD -6.25-3.50x014 20/30+2 OS -7.50-4.00x158 20/30 • Pupils, EOMs, CF, CT unremarkable • R: OD -4.75-3.00x031 20/30+1 OS -6.50-4.25x162 20/25-2 (Refract after T1/P2.5 unchanged)

  4. Objective Findings, cont. • SLEx: • Lids: Thickened lids, collarettes OU • Conj: Clear OU • Cornea: “Central circular stromal infiltrative scars” OU (Note: Small corneal drawing of little use for ongoing documentation) • Iris: Unremarkable OU • A/C: Deep and quiet OU • Lens: Clear OU

  5. Objective Findings, cont. • NCT: 11 OD, 13 OS @ 2:59 • DFE: • ONH: C/D = 0.55/0.50 OD, 0.60//0.55 OS “Margins clear and distinct” OU; Color? • Vessels, macula, & periphery unremarkable

  6. Assessment 1. “Corneal deposits (stromal infiltrative) OU” • “Blepharitis OU” • “CMA OU, decrease in myopia possible cause (contributing to) HA” • “Large CD OS>>OD”

  7. Plan • “Consult with EHMS, photodocument, possible steroid Tx for infiltrates (?)” • “Educate patient on lid scrubs” • “RTC to EHMS, trial frame, issue Rx” • “Monitor annually for change”

  8. 1 Week Later… • No change in history and symptoms • Large corneal drawing labeled as follows: • Mixed hazy/discrete round, nodular, subepithelial lesions in annular pattern OU • Deep stromal fluffy, white, round opacities within upper third of cornea OU • Grade 2-3 gutatta, with possible additional endothelial deposits OU

  9. OD OS

  10. OD

  11. OS

  12. OD

  13. OD

  14. OS

  15. You make the call… • What’s your diagnosis? • What general class of etiology is responsible for the corneal findings? • What are the white lesions/opacities? • Infiltrates? Infiltrative scars? Deposits? Other? • Is this condition inflammatory? • Should we be worried about systemic manifestations of this disorder? • How do we manage this patient?

  16. Macular Corneal Dystrophy • Autosomal recessive • Most severe and least common of the stromal dystrophies • Progressive vision loss, with severe impairment by age 20-40 • Recurrent bouts of photophobia and irritation • Recurrent erosions also seen, but less frequently than in lattice dystrophy

  17. MCD • Corneal changes usually noted by age 9, with diffuse, central, superficial stromal clouding • Clouding extends peripherally and into the deeper stroma with time • By the teen years, the opacification usually involves the entire thickness of the cornea and may extend to the limbus • The cornea becomes increasingly cloudy as the condition progresses

  18. MCD • Within the diffuse, cloudy cornea are denser opacities with indistinct borders • These denser opacities can protrude anteriorly to result in epithelial surface irregularity • They can protrude posteriorly to cause irregularity, grayness, and a guttata-like Descemet’s appearance • They can enlarge with time and coalesce • The stroma may be up to one third thinner than normal

  19. Epithelial thickening Endothelial involvement Corneal Opacities

  20. MCD - Pathology • Characterized by accumulation of acid mucopolysaccharide (abnormal, incompletely synthesized keratin sulfate) between the stromal lamellae, underneath the epithelium, within stromal keratocytes, and within endothelial cells (stains with colloidal iron) • Bowman’s may be thinned or absent in some areas

  21. Histology- colloidal iron stain

  22. MCD • Several subgroups of MCD exist, based on immunohistological identification (I, IA, II) • Different subgroups have slightly different characteristics based on subtle mutation variations in the keratin sulfate gene on chromosome 16 • Evidence strongly suggests that the defect in keratin sulfate synthesis in MCD is not restricted to corneal cells • Therefore, it’s believed that the defect represents the underlying mechanism of a systemic disorder of keratin sulfate (a.k.a. mucopolysaccharidosis)

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