1/36. Neurologic, ophthalmic, and neuropsychiatric manifestations of pediatric systemic lupus erythematosus. BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune, multisystem disorder with diverse manifestations. There are limited reports on the neuro-ophthalmic and neuropsychiatric findings in childhood SLE. CASE REPORT: An 8-year-old patient with suspected SLE with a history of skin rash, joint pain, transient hematologic abnormality, headaches, seizures, and psychosis, presented with papilledema secondary to acquired, obstructive hydrocephalus. DISCUSSION: SLE-related ocular and neuropsychiatric findings are reviewed. Proposed mechanisms for ocular and cerebral lupus include immune complex deposition, vascular thrombosis, and postinflammatory lesions. CONCLUSION: The prevalent neurologic and neuropsychiatric complications of pediatric SLE require close follow-up and interdisciplinary management. ( info) |
| Free submandibular salivary gland transfer was investigated as a surgical method for the treatment of severe keratoconjunctivitis sicca. In an animal model, we examined the tolerance of warm ischemia of the submandibular gland. After temporary interruption of the blood supply (1 to 6 hours), the morphologic changes in the submandibular gland were analyzed histologically and immunohistochemically in 41 rabbits. From 1.5 hours ischemia onward, an increasing structural damage of the parenchyma with emphasis on the secretory cells was seen. Six hours of ischemia caused total necrosis of the salivary gland. Our clinical experience includes 24 highly selected patients suffering from keratoconjunctivitis sicca, in whom we transferred 31 autologous submandibular glands to the temple for permanent autologous tear substitution within the past 4 years. The glands were implanted into a pocket prepared in the temporalis muscle, and the nourishing vessels were anastomosed to the superficial temporal artery and vein. The submandibular duct was implanted into the upper lateral conjunctival fornix. The transferred glands were left denervated. In addition to the clinical examination, scintigraphy with Tc 99m pertechnetate was used to document the graft's viability after the transfer. Viable incorporation with longstanding secretory function occurred in 26 of the 30 transplanted denervated salivary glands. The resulting lubrication of the treated eyes was irregular for up to 3 months in almost even case. One year after surgery, all patients with a viable transplant developed at least occasional epiphora, which was surgically managed by reducing the size of the graft in 10 patients. No severe side effects were seen in this series. The ophthalmologic evaluation of the method included the assessment of dry eye symptoms and of the volume and quality of ocular lubrication (Schirmer test, fluorescein break-up time), the pathology of the ocular surface (rose bengal staining), and the need for pharmaceutical tear substitutes. One year after surgery, 18 of 27 cases assessed were judged as significantly improved by these tests. ( info) |
| sjogren's syndrome is a chronic inflammatory systemic autoimmune disease mainly affecting the exocrine and, particularly, the salivary and lacrimal glands. The condition usually occurs in adults. In 1994, the criteria for this syndrome were redefined in a multicenter European study. In children, sjogren's syndrome is a rare and probably underdiagnosed disease. To date, sjogren's syndrome in children has only been described in case reports and in the comparative presentation of various study results. So far, no study of a comparative classification into primary and secondary sjogren's syndrome has been carried out in a patient population of any size. sjogren's syndrome should be considered in the differential diagnosis of children with recurrent parotitis, keratoconjunctivitis sicca, or pronounced and early tooth decay associated with xerostomia. In this study of 23 children and adolescents under the age of 16 with the clinical symptoms and laboratory findings of sjogren's syndrome, we differentiate between primary and secondary sjogren's syndrome. The value of the individual methods of assessing the oral and the ophthalmological components and the manifestation of the underlying rheumatic condition are discussed on the basis of the EULAR criteria. The EULAR diagnostic criteria are of limited applicability in children because reliable anamnestic data are frequently lacking. Another problem in diagnosing sjogren's syndrome is the short-term detection of serological alterations and clinical symptoms. Even if young patients do not completely fulfill the required criteria, sjogren's syndrome can be assumed or confirmed in the presence of positive testing for oral and ocular manifestations and recurrent salivary gland enlargement. ( info) |
| OBJECTIVE: androgens have been reported to influence lipid production of sebaceous glands and even many ocular tissues. The effect of topical androgen therapy on a 54-year-old patient with keratoconjunctivitis sicca (KCS) and decreased lipid phase of the tear film is reported. methods: For assessment of the lipid phase of the tear film, break up time (BUT) and lipid layer thickness (LLT) were monitored during 6 months before treatment as well as 3 months while using a daily topical androgen therapy. RESULTS: During the topical androgen therapy the pathological lipid phase of the tear film was completely restored indicated by the normalisation of the values of BUT and LLT. CONCLUSION: These findings are consistent with animal experiments indicating that topical administered androgen can restore the decreased lipid phase of the tear film. This may open up new therapeutic strategies for KCS. ( info) |
| PURPOSE: To describe a case of severe dry eye syndrome in a child. methods: Observational case report. The authors describe a 10-year-old male with severe dry eyes who was profoundly disabled by pain and photophobia despite aggressive conventional therapy. Lacrimal gland histology was consistent with the primary Sjogren syndrome, and serologic and immunohistologic evidence supported the hypothesis of Epstein-Barr virus causality. RESULTS: Treatment with systemic acyclovir and cyclosporin A resulted in dramatic and rapid reversal of the profound sicca syndrome and enabled the patient to resume his normal activities. CONCLUSION: Epstein-Barr virus dacryoadenitis should be considered in the differential diagnosis of keratoconjunctivitis sicca in children. Epstein-Barr virus can cause keratoconjunctivitis sicca, which can be treated successfully with acyclovir therapy in addition to suppression of the inflammatory response. ( info) |
| PURPOSE: To describe a case of inferior corneal steepening with a keratoconus-like pattern in a patient with nocturnal lagophthalmos and aqueous tear deficiency (ATD). methods: Axial curvature mapping was performed with the Tomey TMS-2N videokeratoscopy instrument. keratoconus-like topography patterns were identified with the Klyce software package of this instrument. RESULTS: Axial videokeratoscopy showed asymmetric inferior corneal steepening in the right eye. The surface regularity index (SRI), surface asymmetry index (SAI), and the simulated keratometric cylinder change (CYL) were 1.05, 5.05, and 0.75, respectively. The keratoconus screening indices were as follows for the right eye: Klyce/Maeda index of 95% similarity (clinical keratoconus interpreted) and Smolek/Klyce index of 23.09% severity (keratoconus suspect interpreted). Ultrasound pachymetric mapping showed a normal central corneal thickness. We found a similar topographic pattern of keratoconus in seven eyes of 74 dry eye patients who were previously evaluated with the Tomey TMS-2N. CONCLUSION: Chronic ocular desiccation and aqueous tear deficiency can produce inferior corneal steepening and high astigmatism resembling keratoconus. ( info) |
7/36. Management of filamentary keratitis associated with aqueous-deficient dry eye. PURPOSE: To review the incidence, underlying pathophysiology, and clinical features of filamentary keratitis and to identify evidence-based best-practice strategies for managing filamentary keratitis. methods: A comprehensive review of published literature was undertaken. Recommendations for best-practice management strategies were based on the available evidence. Three cases are presented to illustrate the clinical findings and management of patients with chronic filamentary keratitis. RESULTS: Although the evidence base is limited by the absence of well-designed studies, current evidence indicates the following: (1) Aqueous-deficient dry eye (keratoconjunctivitis sicca) is the most common ocular condition associated with filamentary keratitis. (2) Current best-practice management of filamentary keratitis involves treating the underlying dry eye and specific treatments for the corneal filaments. Proposed treatments include nonpreserved lubricants, topical steroidal and nonsteroidal anti-inflammatory agents, and punctal plugs for aqueous-deficient dry eye as well as mechanical removal of filaments, hypertonic saline, mucolytic agents, and bandage contact lenses for the filaments. (3) Filamentary keratitis can be induced or exacerbated by contact lens wear and ocular surgical procedures such as cataract surgery and corneal graft surgery. Pre- and postoperative ocular surface management strategies should be considered in the surgical planning of patients with, or who are susceptible to, filamentary keratitis. Filamentary keratitis can also be induced and/or exacerbated by chronic use of ocular and/or systemic medications, and alternate medications or additional measures to manage the tear film and ocular surface may be required in these cases. CONCLUSIONS: Filamentary keratitis can be a chronic, recurrent, and debilitating condition. With a systemic approach to diagnosis and management, the condition can be effectively controlled and the incidence and severity of recurrences minimized. ( info) |
| A 31-year-old female physician with bilateral myopia had unilateral myopic laser in situ keratomileusis in the right eye. The patient was overcorrected with the initial treatment and had 2 subsequent hyperopic treatments, resulting in central corneal irregularity. During the course of treatment, the patient was diagnosed with sjogren's syndrome with marked keratoconjunctivitis sicca. The patient had anisometropia, aniseikonia, and monocular diplopia and was unable to tolerate contact lenses. Oral pilocarpine (Salagen) was prescribed, which improved salivation, lacrimation, and keratoconjunctivitis sicca. The patient was able to tolerate rigid gas-permeable contact lenses to achieve 20/20 visual acuity in both eyes and regain binocular vision. ( info) |
9/36. Remission of Behcet's disease and keratoconjunctivitis sicca in an hiv-infected patient treated with HAART. A 34-year-old woman presented with a 10-year history of recurrent oral and genital ulcerations and recurrent episodes of bilateral conjunctivitis associated with hiv infection. A diagnosis of Behcet's disease (BD) in association with keratoconjunctivitis sicca (KCS) was made after exclusion of other viral and autoimmune diseases according to the international criteria for BD. This is the first reported case of a combination of BD and KCS in a patient with hiv infection in which a complete resolution was observed as a result of successful highly active antiretroviral therapy. The likelihood that a direct viral effect or hiv-induced autoimmune mechanisms act in the pathogenesis of both BD and KCS in hiv-infected patients is discussed. ( info) |
10/36. Bilateral dacryocystitis after punctal occlusion with thermal cautery. A 61-year-old woman developed acute bilateral dacryocystitis secondary to staphylococcus aureus 3 weeks after undergoing punctal occlusion with thermal cautery for keratoconjunctivitis sicca. The dacryocystitis resolved with intravenous antibiotics, aspiration of the lacrimal sacs, injection of sulfacetamide into the lacrimal sacs, and bilateral dacryocystorhinostomy. Preexisting bilateral nasolacrimal duct obstruction was postulated as the underlying cause. In these cases, irrigation of the lacrimal system is recommended before proceeding with punctal occlusion. ( info) |