Corneal xanthogranuloma in association with multiple endocrine neoplasia 1: a clinicopathologic case report and review of the literature

Juvenile xanthogranuloma [JXG] is a benign inflammatory condition of uncertain pathogenesis. It is characterized by skin and ocular involvement - typically in the iris - in children. It has been reported in older age groups and has been also observed to involve other ocular structures such as the cornea and conjunctiva. In this case report, we are presenting an extensive right eye corneal lesion in a 43-year old male which showed the typical histopathological feature of JXG and in association with multiple endocrine neoplasia [type 1]. Similar cases in the Englishlanguage literature have been also reviewed

Ocular mycosis at a referral center in Saudi Arabia: a 20-year study

To review the clinical experience of fungal keratitis cases at King Khaled Eye Specialist Hospital [KKESH] in Riyadh, Saudi Arabia. Retrospective observational review and analysis of 124 patient charts with confirmed diagnosis of fungal keratitis between 1984 and 2004. One hundred and twenty four eyes of 124 patients had proven fungal infection; 101 eyes had fungal keratitis and 23 eyes had fungal endophthalmitis complicating keratitis. Estimated proportion of fungal keratitis and endophthalmitis was 10.3%. Mean age was 55 years with male predominance [79.0%]. Commonly associated factors were previous intraocular surgery [38.7%] and trauma [20.9%]. Major risk factor for progressing to endophthalmitis was previous intraocular surgery [65.2%], p < 0.001. Initial laboratory results were fungal positive only in 30.6% [p < 0.001]. Commonest organisms isolated were Aspergillus spp. [29.8%] followed by Trichophyton sp. [16.1%], then Candida and Fusarium sp. Comparison of both phases of the study showed improvement in the rate of successfully treated cases from 34.6% to 58.3%, and a decline in cases progressing to endophthalmitis from 25.0% to 13.9%. Therapeutic penetrating keratoplasty increased from 26.9% to 73.6% [p < 0.001]. Thirteen eyes required enucleation or evisceration. In contrast to other studies on fungal keratitis, Aspergillus spp. and Trichophyton sp. were the most commonly isolated fungal pathogens; the former carries the worst prognosis. Risk factors included previous intraocular surgery and trauma. Poor outcome was associated with Aspergillus spp., delayed presentation, previous intraocular surgery and late surgical intervention. This study recommends early surgical intervention to improve the outcome

Amyloid corneal deposition in corneal buttons of congenital hereditary endothelial dystrophy [CHED] - a clinical and histopathological case series

To determine the frequency, pathology and clinical relevance of amyloid deposited in corneas of CHED. Clinical and histopathological case series. Amyloid subepithelial deposition was found in 5 [6.6%] corneal buttons of 75 patients with histopathologically confirmed CHED diagnosis. Clinical findings included history of parental consanguinity, poor vision [ranging from counting fingers from one foot to 3/200], corneal edema, and central whitish subepithelial corneal nodules in all the five cases and positive family history in 4 of 5 cases. The patients underwent PKP at a mean age of 15 years [range 3-22 years]. Histological findings included attenuated endothelium [6/6] thickened Descemet's membrane [6/6], stromal edema [2/6], and subepithelial amyloid deposits [6/6]. All patients improved from vision point of view. To date, no recurrence of the amyloid has been seen in the grafts. Considering the consanguinity, family history, early onset, and bilaterality, this study supports our hypothesis that the amyloid is primary in nature in our patients and indicates a new subtype of autosomal recessive CHED that require further chemical and genetic analysis. This subtype has the same prognosis for PKP as all CHED patients, if not better

Tuberculous aortitis

Tuberculous aortitis [TA] is a rare entity that is invariably indicative of disseminated tuberculosis. TA is associated with aneurysm formation in about half of cases. Another possible complication is perforation of adjacent structures [1-7]. Both abdominal and thoracic aorta are involved with equal frequency.[7-9] Fatal outcomes are frequently reported even after antituberculosis chemotherapy and surgical intervention. We present a case of tuberculous aortic aneurysm [TBAA] that underwent surgical resection and graft replacement in the bed of the infected aorta. Following an apparently successful chemotherapy, the patient died suddenly. We postulate that reactivation of the un-eradicated bacilli precipitated graft failure. Similar cases in the literature are reviewed. We propose lifelong suppressive therapy with antituberculosis agents to prevent such a catastrophic event

Retinal pigment epithelial cell culture and cooperation of L-carnitine in reducing stress induced cellular damage

To show that L-carnitine [LC] is capable of reducing non-oxidative stress in the retinal pigment epithelial cells [RPE] of the human eye. The RPE cells were cultured from donor eyes, obtained immediately after post-mortem. The interaction between bovine serum albumin [BSA] and non-oxidative [sodium hydroxide and methyl methane sulphonate] stressinducers was observed by recording the change in the absorption profiles of the interacting molecules after incubation in light for 5 hours and after treatment with LC. The isolated and cultured RPE cells from human eyes were treated with sodium hydroxide or methyl methane sulphonate and/or LC for 5 hours under light, and the qualitative effect on cell morphology after treatment was analyzed by staining the cells with Giemsa and visualization by light microscopy. The cell morphology was also qualitatively analyzed by scanning electron microscopy [SEM]. L-carnitine and stress-inducers interact with BSA and bring about changes in the spectral profile of the interacted molecules. Light microscopy as well as SEM show that the changes in the cellular morphology, induced by 100 micro M concentrations of non-oxidative stress-inducers, are considerably reduced in the presence of 100 micro M LC. However, L-carnitine alone does not cause any qualitative damage to the cell morphology during incubation under similar conditions. The results give a preliminary indication that LC has the ability to reduce the changes brought about by the non-oxidative stress-inducers in the RPE cells in culture

Ocular complications of junctional epidermolysis bullosa in a 4-year-old

To report the ocular findings in a proven case of Junctional epidermolysis bullosa. A 4-year-old boy known to have epidermolysis bullosa presented with left corneal epithelial defect. After treatment with fusidic acid suspension, lubricants and cycloplegia, he was observed for 3 months. Skin biopsy was submitted for both light and electron microscopy to confirm the diagnosis. Epithelial defect healed after 5 days leaving faint subepithelial opacity at the level of Bowman's layer. Three months later, both palpebral conjunctiva showed marked scarring in addition to the subepithelial haze in the left cornea. Light and electron microscopy of a skin biopsy showed subepidermal cleft involving lamina lucida as a diagnostic feature of Junctional epidermolysis bullosa. Junctional epidermolysis bullosa can be associated with various ocular complications such as superficial corneal scarring. Skin biopsy is needed to confirm the diagnosis

Fungal keratitis: a review

Keratomycosis is a vision-threatening fungal corneal infection. The dramatic increase in the number of cases over the past three decades is attributable not only to better diagnostic recognition, improved laboratory techniques and greater awareness by the ophthalmic society as a whole, but is also due to a true increase in the incidence of keratitis related to the indiscriminate use of topical broad-spectrum antibiotics, corticosteroids and immunosuppressive drugs, as well as surgical trauma. Corneal trauma has remained the main predisposing factor over the years, though in recent years HIV-positive cases and AIDS are taking the lead in certain areas. Aspergillus, Fusarium and Candida species remains the commonest 'organisms' isolated worldwide. Although the approach to this form of keratitis is similar to other types of microbial keratitis, it remains the most difficult in terms of diagnosis and management. Early recognition, prevention, prompt treatment and timely keratoplasty are crucial for a better outcome

Mobile Pigmented Posterior Vitreous Cyst

Purpose: To describe a 30-year-old man who presented with a mobile pigmented vitreous cyst in the left eye without any other ocular abnormalities. Clinical evaluation along with photography and video recording to document the appearance and mobility of the cyst. The patient was observed and followed up over a 10-year period. The patient was able to tolerate the trivial symptoms produced by the cyst, which did not increase in size. Observation with regular follow up is advised as the initial plan for this condition if the cyst is benign

Changing patterns of visual loss in the eastern province, Kingdom of Saudi Arabia

Considerable attention has been given to the problems of prevention of blindness and trachoma in the Eastern Province of Saudi Arabia over the past 40 years. It was reported in the 1960s that trachoma prevalence in Eastern Province preschool children was in excess of 90% in villages and 70% in towns, leading to a high incidence of blindness. Unpublished data from the Kig Khaled Eye Specialist Hospital 1984 G/ 1404 Hnational Survey of Eye Disease and Visual Loss demonstrated that, according to World Health Organization criteria, the prevalence of blindness in the Eastern Province was 3.3% and the prevalence of active trachoma was 11.3%. Recent socioeconomic improvement, including the health services, prompted a new survey in order to assess the current situation. From a stratified geographic cluster sample, 4819 persons were registered and 4340 were examined. Results from this 1990 survey are compared with the 1984 data. Blindness was reduced from 3.3% to 1.5%, and cataract remains the major cause of blindness [37.5%]. Trachoma is responsible for 9.4% of the causes of blindness in 1990, in comparison to 27.3% in 1984. Active trachoma has decreased to a level where it can no longer be considered a major health hazard [1%]; however, total elimination of the disease, and protection of families at risk is recommended

Causes of blindness in students attending schools for the blind in Saudi Arabia

Purpose: To determine causes of visual loss in blind school students in Saudi Arabia. All students enrolled in 17 schools for the blind were interviewed and had complete ophthalmic examination between 8 February and 30 May 1998. Medical files were accessed to obtain previously documented findings. Enrollment numbered 913, 68% males and 32% females. Causes of visual loss: retinal dystrophies [40%], congenital glaucoma [15%], optic nerve disease [12%], congenital cataract, corneal diseases, and others [each 11%]. Congenital [63%] and hereditary [25%] causes were more common than acquired [12%], p = 0.05. Consanguinity was 43% in congenital and 55% in hereditary versus 34% in acquired cases [p<0.0005]. Blindness occurred in 65% of students' relatives. Conclusions: Visual loss was caused predominantly by congenital disorders and retinal dystrophies were the most frequently encountered. Consanguinity is an issue that should be addressed

Human immunodeficiency virus in Saudi Arabia

The human immunodeficiency virus HIV type 1 has evolved as one of the most important global infectious pathogens. Although the virus had initially emerged among certain high risk groups in developed countries, it quickly gained momentum in developing countries threatening most population groups. The first case of acquired immunodeficiency syndrome from the Kingdom of Saudi Arabia was diagnosed in 1984. Twenty years later, by the end of 2003, 1509 patients have been reported to have acquired HIV 1. The majority of the early infected patients have acquired HIV 1 from blood product transfusion. Subsequently, the most prevalent mode of transmission became heterosexual. In this review, the distribution of HIV infected persons, prevalence data, and future outlook are presented. Communities considered conservative are not immune from a sexually transmissible virus that has infected 60,000,000 people globally

Extrapulmonary tuberculosis, clinical presentation and outcome

To identify patterns, features, and outcome of extrapulmonary tuberculosis in a tertiary care setting. A retrospective case-series was carried out of all cases diagnosed and treated as extrapulmonary tuberculosis during 1991 through to 2000 at King Faisal Specialist Hospital and Research Centre [KFSH and RC], Riyadh, Kingdom of Saudi Arabia. Demographic, clinical, laboratory, and outcome data were abstracted from medical records. Over a 10-year period, 394 cases of extrapulmonary tuberculosis were diagnosed and treated at KFSH and RC. Isolated extrapulmonary tuberculosis was identified in 339 [86%] patients, 55 cases [14%] had both pulmonary and extrapulmonary tuberculosis. Mean age was 45-years, and 188 patients [47%] had co-morbidities, most commonly diabetes mellitus in 14.2% of patients. Laboratory confirmation of extrapulmonary tuberculosis was available on 386 patients. The most frequent site involvement was lymphadenopathy in 41% of the time. Chest x-ray was normal in 75% of patients. Among 298 patients with follow up data, 10 [3.4%] had documented relapse and 50 [16%] died. Death was related to tuberculosis in 24 [48%] patients. A high level of clinical suspicion is essential for early diagnosis and treatment of extrapulmonary tuberculosis to reduce the significant morbidity and mortality

Mycobacterium tuberculosis susceptibility in Saudi Arabia

To present the available susceptibility data of Mycobacterium tuberculosis [M. tuberculosis] isolates from the Kingdom of Saudi Arabia [KSA] published in peer-reviewed journals. In a meta-analysis, studies published between 1966 and 2001 were included. Publication sites include Medline-indexed and non-indexed. Numbers of grown and resistant isolates were tabulated for first-line anti-tuberculosis agents. Twelve studies met the pre-set criteria. Data on 6,316 isolates between 1979 and 2000 were available. Resistance to at least one agent of the first-line anti-tuberculosis agents was 18.4%. Monoresistance to a single first-line agent was found in 10.9%, while polyresistance was noted in 7.6%. Multidrug-resistant M. tuberculosis was noted in 5.7% of all isolates. Resistance to isoniazid was most common noted in 11% of isolates. Resistance rates to other agents were: rifampin 9.7%, streptomycin 9.1%, pyrazinamide 3.1%, and ethambutol 2.5%. The overall resistance rate to at least one agent was not statistically different in isolates grown between 1979-1991 [18.5%] and 1989-2000 [18.3%]. There were large regional variations and higher resistance rates in the Western and Southern regions. Mycobacterium tuberculosis resistance rates to first-line antituberculosis agents and multidrug-resistant M. tuberculosis are high in KSA. A survey and monitoring program for drug-resistant tuberculosis will determine resistance rates at the community level

Urinary tract infection in spinal cord injury patients

Development and industrialization, military conflicts and various sports have dramatically increased the number of spinal cord injury patients. the skills of caring for these victim have also improved significantly. A common problem encountered by these patients, and their care providers, remains without significant recent advances. Urinary tract infections in spinal cord injury patients are frequent, difficult to evaluate, treat and prevent. At this stage, no antimicrobial prophylaxis is advocated routinely. The best available preventative measure remains simple hygienic techniques, intermittent catheterization and close monitoring. This review will summarize the pathogenesis, epidemiology, diagnosis, treatment and prevention of urinary tract infections in spinal cord injury patients