1/12. A sighting of orbital pseudotumor.A 39-year-old woman developed bilateral proptosis, photophobia, and pain with extraocular movements over the course of 5 days. Her findings initially were ocular pain and photophobia which progressed to periorbital edema and nasal discharge ultimately resulting in proptosis with vertical globe displacement and decreased visual acuity. She was diagnosed with corneal abrasion and sinusitis respectively during two initial emergency department visits. On her third visit to the emergency department within 4 days, she developed acute visual deficits. The patient was subsequently diagnosed with orbital pseudotumor after computed tomography scan revealed inflammation of orbital structures bilaterally.- - - - - - - - - - ranking = 1keywords = ocular (Clic here for more details about this article) |
2/12. Phototherapeutic keratectomy for Schnyder's crystalline corneal dystrophy.PURPOSE: To describe the treatment of Schnyder's crystalline dystrophy (SCD) with excimer laser phototherapeutic keratectomy (PTK). methods: Chart review of three patients (four eyes) with SCD treated with PTK between March 1992 and December 1998. Pre- and posttreatment visual acuity, subjective glare, photophobia, manifest refraction, ultrasound pachymetry, hyperopic shift, and biomicroscopic findings were studied. Brightness acuity testing (BAT) was evaluated in two eyes. RESULTS: Four eyes were treated during the study period. The average best corrected visual acuity (BCVA) improved from 20/175 to 20/40 under bright conditions. All patients reported subjective improvement in glare and photophobia. No visually significant recurrence was observed during the follow-up period which ranged from seven months to three years. The mean spherical equivalent refractive shift was 3.28 diopter (D). One patient developed irregular astigmatism from an eccentric ablation. No vision threatening complications were observed. CONCLUSION: PTK can be effective at improving visual symptoms in patients with SCD. It can be a useful therapeutic alternative to lamellar or penetrating keratoplasty in these patients.- - - - - - - - - - ranking = 2.5185601505033keywords = dystrophy (Clic here for more details about this article) |
3/12. photophobia as the presenting visual symptom of chiasmal compression.Five patients with a chief visual complaint of photophobia were subsequently found to have compressive lesions of the optic chiasm. visual acuity and visual field deficits were often subtle. magnetic resonance imaging scanning revealed large suprasellar masses, including three pituitary adenomas, a craniopharyngioma, and a clivus chordoma. photophobia resolved in all patients following treatment of the tumors. A compressive lesion of the optic chiasm should be considered in patients who experience persistent photophobia unexplained by ocular abnormalities.- - - - - - - - - - ranking = 0.5keywords = ocular (Clic here for more details about this article) |
4/12. ichthyosis follicularis, alopecia, and photophobia (IFAP) syndrome: report of a new family with additional features and review.Two brothers with ichthyosis follicularis, noncicatricial universal alopecia, photophobia, hyerkeratotic psoriasis-like lesions, nails dystrophy, inguineal herniae, cryptorchidism, short stature, seizures, and psychomotor developmental delay are described. These features correspond to the ichthyosis follicularis, alopecia, photophobia (IFAP) syndrome. The youngest brother had in addition a bilateral absence of 4th fingers and camptodactyly, features never reported in patients with IFAP syndromes.- - - - - - - - - - ranking = 0.50371203010066keywords = dystrophy (Clic here for more details about this article) |
5/12. Keratopathy of multiple myeloma masquerading as corneal crystals of ocular cystinosis.We describe a 49-year-old woman in whom ocular cystinosis was diagnosed on the basis of a routine eye examination 12 years previously. Conjunctival biopsy was reported to support the diagnosis. The patient described photophobia for the past 5 years and reported a 2-fold increase in her serum IgG level for the past 12 years. On ophthalmic examination, corneal crystals were evident in the epithelium and superficial stromal layers, rather than throughout the corneal epithelium and the entire stroma as in ocular cystinosis. The patient's serum protein level was elevated at 8.7 g/dL; protein electrophoresis showed an elevated gamma-globulin peak, and the IgG level was twice that of normal at 2820 mg/dL. bone marrow biopsy confirmed the diagnosis of multiple myeloma. This case illustrates that multiple myeloma can mimic corneal findings of cystinosis.- - - - - - - - - - ranking = 3keywords = ocular (Clic here for more details about this article) |
6/12. Ocular findings in ichthyosis follicularis-alopecia-photophobia (IFAP) syndrome.PURPOSE: To report the ocular findings in two siblings with IFAP and their mother and to review the natural course of the keratopathy of this disease. methods: Clinical ophthalmological examination of all patients and fundus photography of the carrier mother were performed. RESULTS: Both affected male children had severe photophobia, total superficial and deep corneal vascularization, and reduction of vision to counting fingers.The mother had tortuous retinal vessels. CONCLUSIONS: Males with IFAP have an inexorable progression of corneal vascularization and loss of vision. Retinal vascular tortuosity may be another clinical sign of carrier status in females.- - - - - - - - - - ranking = 0.5keywords = ocular (Clic here for more details about this article) |
7/12. Progressive cone dystrophy and sensorineural hearing loss.A 39-year old man presented 13 years ago with a history of progressive loss of vision and photophobia. A full ophthalmological and ENT work-up during several years of follow-up, including psychophysical as well as electrophysiological tests, revealed a progressive cone dystrophy in combination with sensorineural hearing loss. His younger sister presented with very similar features and underwent the same work-up. A novel syndrome of progressive cone dystrophy and sensorineural hearing loss is described in both siblings. Both also suffered from non-ocular disease possibly related to ciliary dysfunction. The condition is likely to be inherited as an autosomal recessive trait.- - - - - - - - - - ranking = 3.5222721806039keywords = dystrophy, ocular (Clic here for more details about this article) |
8/12. X-linked reticulate pigmentary disorder with systemic manifestations: report of a third family and literature review.Three siblings, two boys and one girl, presented with pigmentary abnormalities. The brothers, ages 11 and 6 years, had diffuse reticulate macular hyperpigmentation with onset in early childhood. In addition, these boys had hypohydrosis, coarse hair with an upswept frontal hairline, failure to thrive, and chronic pulmonary disease. The older boy also had corneal dystrophy and marked photophobia. A punch biopsy specimen from the 11-year-old showed melanophages and necrotic keratinocytes. Stains for amyloid were negative. The sister, age 2 years, had congenital linear hyperpigmented patches involving the intertrigenous areas, but was otherwise normal. The clinical findings of these children were consistent with X-linked reticulate pigmentary disorder with systemic manifestations. We present a summary of the clinical manifestations of this rare disorder and discuss efforts to identify the causative gene.- - - - - - - - - - ranking = 0.50371203010066keywords = dystrophy (Clic here for more details about this article) |
9/12. ichthyosis follicularis, alopecia and photophobia (IFAP) syndrome treated with acitretin.We describe a 3-year-old male patient with the ichthyosis follicularis, alopecia and photophobia (IFAP) syndrome, who developed cutaneous and ocular involvement in infancy. In addition, he had growth retardation and borderline intelligence; no other systemic involvement was found on detailed investigation. A moderate response to acitretin therapy (1 mg/kg) administered for 6 months was observed, with improvement in cutaneous features and corneal erosions and no change in alopecia or photophobia.- - - - - - - - - - ranking = 0.5keywords = ocular (Clic here for more details about this article) |
10/12. ichthyosis follicularis with congenital atrichia, nail dystrophy and palmoplantar keratoderma. Variant of IFAP syndrome or a new entity?A 23-year-old man was seen for dry, rough skin and alopecia present since birth. There was no history of impaired sweating, photophobia, or lacrimation. Examination revealed generalized cutaneous thorn-like projections with nonscarring alopecia, twenty-nail dystrophy, and palmoplantar keratoderma.- - - - - - - - - - ranking = 2.5185601505033keywords = dystrophy (Clic here for more details about this article) |
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