1/21. Orbital drainage from cerebral arteriovenous malformations.OBJECTIVE: To describe the neuro-ophthalmic findings in patients with orbital drainage from cerebral arteriovenous malformations (AVMs). methods: We reviewed the records of 100 consecutive adult patients with cerebral AVMs who presented to our institution during a 4-year period. All patients with orbital drainage were identified, and their neuro-ophthalmic evaluations were reviewed. RESULTS: Three patients (3%) were identified with orbital drainage from a cerebral AVM. The first patient presented with typical chiasmal syndrome (reduced visual acuity, bitemporal hemianopia, and optic atrophy). magnetic resonance imaging demonstrated a large left temporal and parietal lobe AVM with compression of the chiasm between a large pituitary gland and a markedly enlarged carotid artery. The second patient presented with headaches and postural monocular transient visual obscurations. Examination revealed normal visual function with minimal orbital congestion and asymmetrical disc edema, which was worse in the left eye. magnetic resonance imaging revealed a large right parietal and occipital lobe AVM without mass effect or hemorrhage and an enlarged left superior ophthalmic vein. The third patient had no visual symptoms and a normal neuro-ophthalmic examination; a right parietal lobe AVM was discovered during an examination for the cause of headaches. CONCLUSION: Orbital drainage from cerebral AVMs is rare. Manifestations may include anterior visual pathway compression, dilated conjunctival veins, orbital congestion, and asymmetrical disc swelling.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/21. Conjunctival biopsy in the diagnosis of ocular sarcoidosis.sarcoidosis is a multisystem granulomatous disease of unknown etiology that can affect almost every organ in the body, particularly the lungs, skin, eyes, and thoracic lymph nodes. A definitive diagnosis of sarcoidosis requires that a biopsy be performed. A specimen can be obtained from any affected ocular structure, including conjunctiva, lacrimal gland, eyelid skin, and orbit. Among them, conjunctival biopsy has been suggested as a sensible and safe procedure for confirming suspected sarcoidosis. We describe three patients in whom ocular symptoms were the sole initial manifestations of sarcoidosis and who were diagnosed based on the results of a conjunctival biopsy. We also discuss the efficacy of and indications for conjunctival biopsy.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
3/21. Costs and gains of complex procedures to rehabilitate end stage ocular surface disease.BACKGROUND: The management of patients with end stage ocular surface disease sometimes requires extensive surgical treatment that can only be provided by specialised ophthalmology. The authors discuss the costs and gains of such complex techniques based on a case of bilateral corneal blindness secondary to stevens-johnson syndrome. methods and results: Despite multiple lid surgery for trichiasis and repeated corneal grafting for perforations a white female patient became bilaterally blind at the age of 29 years. She also suffered from disabling discomfort in the right dry eye. At the age of 52 a two stage osteo-odonto-keratoprosthesis achieved visual rehabilitation in the left eye and microvascular transplantation of an autologous, submandibular gland resulted in sufficient lubrication to alleviate her severe discomfort in the right eye. As a result of these procedures she was able to take up a regular job again. The total costs of rehabilitation were pound 13 661 which compare with annual gains and regains for society of pound 13 497. An additional pound 4625 was saved annually in guide dog costs. CONCLUSION: This estimate shows that despite the expense of these complex techniques gains are made well within the second year after rehabilitation. In view of the benefit in quality of life for the patient and monetary savings for society these procedures should be funded by national health services at specialist centres.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
4/21. keratitis, ichthyosis, and deafness (KID) syndrome.An 8-year-old boy with keratitis, ichthyosis, and deafness (KID) syndrome is reported. The patient has ichthyosis and deafness. Additional clinical features include hair and tooth abnormalities, as well as absence of the mammary glands. Although keratitis is an important element in the triad of KID syndrome, this patient has another ophthalmologic defect, a developmental anomaly of the lacrimal puncta, characterized by their complete absence. The case appears to be unique in the literature in that, to my knowledge, this particular ocular anomaly has not been described previously.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
5/21. ichthyosis follicularis: a case report and review of the literature.ichthyosis follicularis (IF) is a very rare neurocutaneous, X-linked recessive condition affecting the skin, hair, eyes, and central nervous system (CNS). This report describes a child with facial dysmorphism, mental retardation, psychomotor delay, congenital alopecia of the scalp, eyebrows, and eyelashes, and extensive spiny follicular papules. A skin biopsy specimen showed the characteristic absence of sebaceous glands. We also reviewed the literature on this very rare entity. Additional findings observed in our patient, including hepatosplenomegaly, undescended testicles, and ptosis, have not been reported before.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
6/21. Cushing's syndrome caused by unsupervised use of ocular glucocorticoids.OBJECTIVE: To report a case of Cushing's syndrome in an adult patient caused by prolonged and unsupervised use of glucocorticoid ocular drops. methods: We present the clinical and laboratory findings and describe the clinical course of our patient. RESULTS: A 33-year-old man with extensive ophthalmologic complaints and procedures, who had used glucocorticoid ocular drops for an extended period, was referred to the endocrinologist for new onset of type 2 diabetes mellitus. Clinically, the patient appeared cushingoid with a buffalo hump, thin skin, bruises, and purple striae, and his blood pressure was 130/90 mm Hg. Laboratory evaluation revealed a very low serum cortisol level with a concomitantly low-normal level of adrenocorticotropic hormone (corticotropin). In addition, the response to a corticotropin stimulation test was abnormal, an indication of suppression of the adrenal glands. After the patient discontinued the use of the glucocorticoid eyedrops, his cushingoid features gradually faded, and his blood pressure and serum glucose levels normalized. CONCLUSION: Prolonged unsupervised use of glucocorticoid ocular drops may result in Cushing's syndrome and its numerous associated complications. physicians and pharmacists should be aware of this possibility and must educate patients about the potentially serious adverse effects of such use.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
7/21. Ocular symptoms and signs in patients with ectodermal dysplasia syndromes.PURPOSE: The ectodermal dysplasia syndromes are underestimated although precise inclusion criteria have been formulated. The purpose is to establish easily detectable ophthalmologic symptoms and signs as reliable criteria for ectodermal dysplasia syndromes. methods: Thirty-six patients with confirmed ectodermal dysplasia syndromes were included in an observational case series: hypohidrotic ectodermal dysplasia (30), EEC syndrome (3), AEC syndrome (2), Gorlin-Goltz syndrome (1). Each patient was examined ophthalmologically. The principal outcome measures were ocular symptoms and signs in patients with different ectodermal dysplasia syndromes of varying severity. methods: Some 94.4% of the patients suffered from dry eye symptoms. Reduction of eyebrows was seen in 94.4%; the lashes were altered in 91.6%. Changes of the meibomian glands were detected in 95.45%. Corneal changes such as pannus occurred later in life. CONCLUSIONS: Alterations of the meibomian glands, which were detected by meibomianoscopy, are the most reliable ocular sign of ectodermal dysplasia syndromes.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
8/21. Familial Bell's palsy in females: a phenotype with a predilection for eyelids and lacrimal gland.The authors report a family with familial Bell's palsy affecting seven individuals, six of whom are females. This is a distinct subtype of Bell's palsy with a predilection for juvenile females, previously reported only very rarely. In conjunction with a review of the literature, this case suggests that this phenotype carries with it a greater risk of serious complications affecting the eyelids and lacrimal gland. These carry significant functional and cosmetic implications owing to aberrant regeneration of the seventh, sixth and possibly third cranial nerves, chronicity and relapses. Clinical features include synkinesis of the eyelids with the orbicularis oris causing synkinetic ptosis, recurrent paralytic ectropion, paralysis of facial muscles of expression with dry eye, hyperlacrimation (crocodile tears), and transient strabismus. Clinically, the decision to offer surgery in place of conservative treatment should consider the natural history of chronicity and relapses often seen with this subtype of familial Bell's palsy. Botulinum toxin injections are especially versatile in managing the complications associated with this phenotype.- - - - - - - - - - ranking = 5keywords = gland (Clic here for more details about this article) |
9/21. multiple endocrine neoplasia type III.The multiple endocrine neoplasia (MEN) syndromes are autosomally dominant inherited disorders in which hyperplastic or neoplastic changes occur in a wide variety of tissues. The specific syndromes are classified according to the endocrine glands affected. MEN type I consists of an aggregation of tumors of parathyroid, pancreatic, and pituitary glands. The association of medullary carcinoma of the thyroid (MCT) and pheochromocytoma is called MEN type II or type IIA, and if combined with mucosal neuromas, intestinal ganglioneuromatosis, and prominent corneal nerves, is named MEN type III or type IIB. Individuals afflicted with MEN type III are characterized by a marfanoid habitus, mucosal neuromas involving oral and ocular tissues, and a number of ophthalmologic findings including prominent corneal nerves, thickened eyelids, and subconjunctival neuromas. These features are easily recognized during the ocular exam, allowing the ophthalmologist to make an early diagnosis of this syndrome prior to the onset of life-threatening manifestations like medullary thyroid carcinoma and pheochromocytoma.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
10/21. Seroprevalence of antibodies to HTLV-I in patients with ocular disorders.Human T-lymphotropic virus type 1 (HTLV-I) has been shown to spread worldwide and to be responsible for distinct systemic diseases, namely adult T-cell leukaemia and HTLV-I-associated myelopathy. Immune-mediated, inflammatory lesions in the lungs, joints, and lacrimal glands (sjogren's syndrome) are also suggested to be associated with the retrovirus. We studied seroprevalence of antibodies to HTLV-I in patients with various ocular disorders who are residents of south-west japan, one of the endemic areas of HTLV-I. Of 310 patients with ocular disease 72 (23.2%) were seropositive. This seroprevalence did not differ significantly from that of the general population of the area. As regards individual ocular diseases, aetiologically undefined nonspecific uveitis showed a significantly high seropositivity for HTLV-I. Of 44 patients 18 (40.9%) were seropositive. Their clinical features were acute or subacute, transient and sometimes recurrent, and granulomatous changes in the anterior uvea. patients with isolated cotton-wool spot of the retina, non-familial retinitis pigmentosa, or keratoconjunctivitis sicca did not show any significantly high prevalence of HTLV-I infection.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
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