Henry S O’Halloran

Microangiopathy of the brain, retina and cochlea (Susac syndrome). A Report of five cases and a review of the literature

OBJECTIVE This study reports five new cases of microangiopathy of the brain, retina, and cochlea (Susac syndrome) and reviews the worlds literature. DESIGN Five cases were systematically studied by the authors. The cases in the literature were identified through Medline searches for Susac syndrome; microangiopathy of the brain, retina, or ear; and cross-referencing the indexes of each retrieved article. PARTICIPANTS The number of new patients studied in this report was five. An additional 41 patients were culled from the literature. INTERVENTION Patients were treated with corticosteroids, antineoplastic agents, and other methods in a noncontrolled, nonrandomized fashion. MAIN OUTCOME MEASURES With respect to therapeutic intervention, the main clinical outcome measures were return of vision, improvement of neurologic and psychiatric manifestations, and recovery of auditory function. Alterations of abnormalities observed by cranial magnetic resonance imaging also were monitored. RESULTS Of 46 identified patients, 39 were women. The mean age of the patients was 30 years. Forty-one patients (89%) had arterial occlusions, which were bilateral in 60%. Thirty-one patients (67%) reported hearing loss. Twenty patients (44%) had a global encephalopathy, but other neurologic manifestations were common. The mean duration of the illness was 46.7 months. CONCLUSION This rare syndrome is more common than previously thought, has a strong female preponderance, and often can be identified at an early stage with a careful history and physical examination. Magnetic resonance imaging of the brain often shows lesions suggestive of multiple sclerosis. Fluorescein angiography may show arteriolar wall hyperfluorescence. Early treatment with corticosteroids often is, although not invariably, associated with a good prognosis. The disease appears to be self-limited in most patients.

Acute multifocal placoid pigment epitheliopathy and central nervous system involvement: Nine new cases and a review of the literature

OBJECTIVE The authors describe nine new cases of acute multifocal placoid pigment epitheliopathy (AMPPE) with associated central nervous system (CNS) involvement and permanent visual sequelae. The study includes a review of the literature and discussion of evaluation, management, and treatment options. DESIGN Retrospective, noncomparative case series. PARTICIPANTS Nine patients were identified with AMPPE and CNS involvement in addition to 22 patients reviewed in the literature. MAIN OUTCOME MEASURES A review of nine patients with AMPPE and CNS involvement was performed. Charts were reviewed for age, gender, preceding viral prodromes, visual acuity, ophthalmologic examination findings, CNS findings, and treatment. RESULTS Thirty-one patients (nine new patients) were diagnosed with AMPPE and various degrees of CNS involvement. Ages ranged from 8 to 54 years, with an average of 27 years. Twenty-one males (68%) and 10 females (32%) were identified. Eleven patients (35%) had antecedent viral illnesses. Visual acuity was variable and ranged from 20/20 to count fingers. The spectrum of CNS findings ranged from headaches to sagittal sinus thrombosis. CONCLUSIONS Acute multifocal placoid pigment epitheliopathy can be associated with CNS abnormalities and permanent visual deficits. Neuroimaging, lumbar puncture, and cerebral angiography analysis provide useful diagnostic tools when CNS involvement is suspected. Intravenous corticosteroids and collaboration with neurovascular colleagues should be considered in these situations. In cases complicated by CNS arteritis, immunosuppressive agents can be a beneficial adjunct to corticosteroids.

Airbags and bilateral eye injury: five case reports and a review of the literature.

We report five cases of bilateral eye injuries from airbag deployment in motor vehicle crashes and review the worlds literature on ocular injuries associated with airbags. The cases in the literature were identified by cross-referencing Medline searches from airbags and ocular injuries. Additional cases were identified after review of references from each article in the search. An additional 89 cases from the literature were identified and are included for discussion. Patients were treated individually in a noncontrolled, nonrandomized fashion according to the nature of each injury with regular follow-up examinations in clinic. Of the 94 cases studied, 24 (27%) were bilateral eye injuries, and 15 (16%) patients were wearing spectacles at the time of the accident. The most common injuries included corneal abrasions, eyelid trauma, and hyphemas. Outcomes ranged from complete resolution of symptoms and return of normal visual acuity to primary enucleation. This report describes the wide spectrum of eye injuries that may occur after airbag deployment. We suggest a management plan for the evaluation and treatment of the ocular complications of airbag-related trauma.

Phakic anterior chamber intraocular lens (Verisyse™) implantation in children for treatment of severe ansiometropia myopia and amblyopia: Six-month pilot clincial trial and review of literature

Purpose: The current study aims to evaluate both safety and efficacy of Verisyse™ (AMO, Irvine, CA) phakic anterior chamber intraocular lens (IOL) in the reduction of clinically significant (>−8 D) myopic anisometropia in children who are noncompliant to traditional medical treatment including spectacle correction or contact lenses. Design: Retrospective interventional case series. Methods: Six anisometropic myopic pediatric patients in one practice were identified through chart-review. None of the patients were compliant with specatacle correction or contact lens wear and as a result had dense amblyopia of less than 20/400 by Snellen or Allen visual acuity (mean <20/400). All patients underwent Verisyse™ phakic IOL implantation in the more myopic eye by one surgeon (AP). Pre- and post-operative visual acuity, anterior/posterior segment examination, stereoacuity, axial biometry measurements, cycloplegic refraction, and endothelial cell counts were performed in all patients whenever feasible. Results: The age of patients ranged from 5–11 years. The mean post-operative follow-up time was six months from the time of IOL insertion. Improvement in visual acuity >6 lines was achieved in four patients (mean visual acuity of 20/70 at six months). Improvement in stereoacuity was noted in all six patients (from total mean zero seconds-arc to six-months post-operative mean of 500 seconds-arc by randot stereoacuity testing). Improvement of >2 lines of visual acuity lines was achieved in the other two patients. No patient lost any lines of visual acuity. Enhanced physical activity, coordination, and improved social interaction were noted in patients and were reported by the parents. No intra/post-operative complications were noted. Discussion: Irreversible or intractable amblyopia secondary to severe anisometropic myopia is a serious medical concern in the pediatric population. Failure of compliance with contact lens therapy consistently leads to visual loss. Anterior chamber phakic IOLs may provide a safe alternative in treatment of noncompliant anisometropic myopic patients who do not accept spectacle wear or contact lens therapy. Conclusion: To reduce or eliminate highly significant anisometropic myopia in children who are noncompliant with traditional medical treatment, phakic anterior chamber IOL implantation may be considered as an alternative modality of treatment.

A report of canine tooth syndrome.

The authors describe the case of a 5-year-old girl traumatized from a dog bite to the superior aspect of the orbit in the right eye. The dog’s canine tooth penetrated deep into the posterior orbit and severed the attachment of the superior oblique muscle from the globe posterior to the trochlea. The management and clinical course of the patient are described and photographs documenting the initial ocular damage and postoperative course are provided. In addition, the entity known as ‘canine tooth syndrome’ is reviewed.

Early-Onset Scleral and Corneal Ectasias following Low-Dose Mitomycin-C-Augmented Trabeculectomy in a Uveitic Glaucoma Patient

A case of early-onset sclerolimbal ectasia following low-dose topical mitomycin C application during uveitic glaucoma surgery is reported. Intraoperative and postoperative clinical courses were consistent with sclerolimbal ectasia. Adjunctive utilization of intraoperative low-dose 0.02% mitomycin C for the management of chronic uveitic glaucoma patients who are under concomitant systemic immunosuppressive regimen may enhance the risk of subacute postoperative sclerolimbal ectasia. Mitomycin C concentrations <0.02%, decreased scleral exposure time, and a strict informed consent policy are strongly recommended in this subset of uveitic patients.