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PubMed1996

Ophthalmologic manifestations of acquired immune deficiency syndrome-associated progressive multifocal leukoencephalopathy.

PURPOSE: Progressive multifocal leukoencephalopathy (PML) is increasingly described as a late complication of the acquired immune deficiency syndrome (AIDS). The purpose of this study is to evaluate retrospectively the ophthalmologic, clinical, and investigational aspects of AIDS-associated PML. METHODS: The authors evaluated ten patients in whom ophthalmologic manifestations developed in the course of AIDS-associated PML. Findings at clinical examination and their progression over time, neuroimaging correlates, the results of pathologic investigation, and visual outcomes were reviewed. RESULTS: Progressive multifocal leukoencephalopathy was the AIDS-defining illness in six of ten patients. Homonymous visual field defects were the presenting symptom in three patients and detected in six patients overall. Occipital blindness developed in one patient. Cerebellar signs and brain stem nuclear and supranuclear palsies also were common. Confluent white matter lesions with increased intensity on T2-weighted magnetic resonance imaging were supratentorial in seven patients and infratentorial in three patients. With incomplete data, the median survival time was 3 months from PML onset. Histopathologic confirmation of PML diagnosis was available for nine of the ten patients. CONCLUSIONS: The development of progressive retrochiasmal visual field defects, supranuclear and nuclear cranial nerve palsies, or nystagmus ataxia in the relatively young patient should alert the ophthalmologist to the possibility of PML, particularly in the presence of long-tract central nervous system signs or dementia. Progressive multifocal leukoencephalopathy will often be human immunodeficiency virus associated. Human immunodeficiency virus encephalopathy, cerebral toxoplasmosis, lymphoma, and infarction need to be discriminated. Effective therapy is required urgently for this devastating disease.

AIDS Dementia Complex↗

PubMed1996

Ophthalmologic findings in Leber hereditary optic neuropathy, with special reference to mtDNA mutations.

BACKGROUND: Leber hereditary optic neuropathy (LHON) is associated with primary and secondary mutations in mitochondrial DNA. Clinical studies suggest that there is a wide spectrum of clinical expression. METHODS: Fifty-three affected and 131 unaffected maternal relatives from 21 pedigrees with LHON were studied neuro-ophthalmologically and followed over a period of 14 years. Mitochondrial DNA analysis was performed on their blood specimens. RESULTS: Thirty-two affected (60%) individuals from ten families harbored the 11778 mutation and ten individuals (19%) from three families harbored the 3460 mutation. No confirmed primary mutation was detected in 11 (21%) affected individuals from eight families. The visual outcome was better in families with the 3460 mutation than in those with the 11778 mutation. Secondary mutations did not affect the penetrance or the visual outcome. Fifteen patients had a favorable outcome; seven of whom had subclinical disease, two had slowly progressive LHON with a favorable visual outcome, and six had classic LHON with spontaneous recovery. In seven patients, the onset of the disease had been in childhood. These patients had a more favorable prognosis than the adults. Results of eye examinations of asymptomatic maternal relatives showed subclinically affected individuals. CONCLUSIONS: In addition to classic LHON, the disease can manifest itself in three different atypical forms: subclinical disease, slowly progressive LHON with a favorable visual outcome, and LHON with the classic acute stage but spontaneous visual recovery. The current study suggests that the ophthalmologic findings and outcome in LHON are independent of secondary mutations.

Adolescent↗

PubMed1999

PHACE: a neurocutaneous syndrome with important ophthalmologic implications: case report and literature review.

OBJECTIVES: To introduce PHACE syndrome (Posterior fossa malformations, Hemangiomas, Arterial anomalies, Coarctation of the aorta and other cardiac defects, and Eye abnormalities) to the ophthalmologic literature; to report the first case of PHACE syndrome associated with congenital glaucoma; and to review the ocular and systemic findings that may occur in this entity. DESIGN: Case report and literature review. METHODS: The authors report a child with PHACE syndrome and congenital glaucoma and review the ophthalmologic and systemic manifestations of this syndrome. RESULTS: A 9-month-old girl with PHACE syndrome was treated. She had a large right facial hemangioma, central nervous system (CNS) abnormalities, and cardiac anomalies. Glaucoma was detected in her left eye, and she underwent glaucoma surgery. She did well following two glaucoma procedures, and the facial hemangioma is responding to medical treatment. CONCLUSIONS: Ophthalmologists who examine children with large facial hemangiomas should consider PHACE syndrome in the differential diagnosis and should obtain appropriate CNS imaging studies and cardiac evaluation when the diagnosis is suspected. Congenital glaucoma should be added to the list of PHACE-associated ocular anomalies.

Abnormalities, Multiple↗

PubMed1998

Ophthalmological findings in patients with Takayasu disease.

We examined 65 (61 female and 4 male) Takayasu patients. Patient age ranged from 17 to 78 years old (mean 50.2); age of onset was from 11 to 60 years old (mean 32.8); and duration from onset to referral ranged from 1 month to 43 years (mean 16.8 years). Routine ophthalmological examinations were performed. Fluorescein angiography, kinetic perimetry by Goldmann perimetry, static perimetry by Octopus 1-2-3, electroretinography (ERG), and measurements of central retinal arterial pressure were also performed, as appropriate. Major causes of impaired visual acuity (less than 16/20) were cataract. A few patients had low visual acuity caused by Takayasu disease itself. On the other hand, although not many complained of visual disturbance, about 35% of patients had subnormal visual functions. Because the visual deterioration may be based on ocular hypoperfusion, which may subsequently lead to more serious changes, regular ophthalmological examination for every Takayasu disease patient is recommended.

Adolescent↗

PubMed1981

Ophthalmologic considerations in maxillofacial trauma. Anatomy and diagnostic evaluation.

The high incidence of orbital involvement in facial injuries necessitates a thorough ophthalmologic evaluation in virtually all cases. This article will provide the oral and maxillofacial surgeon with a review of the anatomy of the eye and ocular adnexa and present a systematic method of conducting and interpreting an ophthalmologic examination.

Conjunctiva↗

PubMed2002

Segmentation techniques for tissue differentiation in MRI of ophthalmology using fuzzy clustering algorithms.

This paper presents MRI segmentation techniques to differentiate abnormal and normal tissues in Ophthalmology using fuzzy clustering algorithms. Applying the best-known fuzzy c-means (FCM) clustering algorithm, a newly proposed algorithm, called an alternative fuzzy c-mean (AFCM), was used for MRI segmentation in Ophthalmology. These unsupervised segmentation algorithms can help Ophthalmologists to reduce the medical imaging noise effects originating from low resolution sensors and/or the structures that move during the data acquisition. They may be particularly helpful in the clinical oncological field as an aid to the diagnosis of Retinoblastoma, an inborn oncological disease in which symptoms usually show in early childhood. For the purpose of early treatment with radiotherapy and surgery, the newly proposed AFCM is preferred to provide more information for medical images used by Ophthalmologists. Comparisons between FCM and AFCM segmentations are made. Both fuzzy clustering segmentation techniques provide useful information and good results. However, the AFCM method has better detection of abnormal tissues than FCM according to a window selection. Overall, the newly proposed AFCM segmentation technique is recommended in MRI segmentation.

Algorithms↗

PubMed2003

Generalized Kohonen's competitive learning algorithms for ophthalmological MR image segmentation.

Kohonen's self-organizing map is a two-layer feedforward competitive learning network. It has been used as a competitive learning clustering algorithm. In this paper, we generalize Kohonen's competitive learning (KCL) algorithm with fuzzy and fuzzy-soft types called fuzzy KCL (FKCL) and fuzzy-soft KCL (FSKCL). These generalized KCL algorithms fuse the competitive learning with soft competition and fuzzy c-means (FCM) membership functions. We then apply these generalized KCLs to MRI and MRA ophthalmological segmentations. These KCL-based MRI segmentation techniques are useful in reducing medical image noise effects using a learning mechanism. They may be particularly helpful in clinical diagnosis. Two real cases with MR image data recommended by an ophthalmologist are examined. First case is a patient with Retinoblastoma in her left eye, an inborn malignant neoplasm of the retina frequently metastasis beyond the lacrimal cribrosa. The second case is a patient with complete left side oculomotor palsy immediately after a motor vehicle accident. Her brain MRI with MRA, skull routine, orbital CT, and cerebral angiography did not reveal brainstem lesions, skull fractures, or vascular anomalies. These generalized KCL algorithms were used in segmenting the ophthalmological MRIs. KCL, FKCL and FSKCL comparisons are made. Overall, the FSKCL algorithm is recommended for use in MR image segmentation as an aid to small lesion diagnosis.

Algorithms↗

PubMed2000

Ophthalmologic emergencies in the patient with diabetes.

Diabetes is associated with many emergent ophthalmologic conditions. The management of patients with diabetes requires careful monitoring for visual symptoms and frequent physical examination for signs of retinopathy. Randomized studies have documented a significant reduction in the development of new retinopathy and the progression of existing retinopathy with tight control of diabetes. Photocoagulation laser therapy is helpful in preserving vision in severe nonproliferative retinopathy, for proliferative retinopathy, and for clinically significant macular edema. Vascular events include arterial and venous occlusions and cranial nerve palsies; important diagnostic clues are visual symptoms and the findings of ocular and neurologic examinations. Life-threatening infections associated with diabetes include endophthalmitis and mucormycosis, which require prompt diagnosis to prevent blindness or systemic infection. Herpes zoster infection, which is common in older patients and in patients with immunosuppression, may affect the trigeminal nerve and cause anterior uveitis and keratitis. Patients with zoster and skin vesicles on the face need emergent ophthalmologic evaluation and treatment because untreated ocular infection and inflammation may lead to scarring and synechiae formation in the anterior chamber, resulting in vision loss.

Blindness↗

PubMed1999

Ophthalmologic outcome of transvenous embolization of spontaneous carotid-cavernous fistulas: a preliminary report.

We give a preliminary report of the ophthalmologic outcome of four patients with transvenous embolization studied for their spontaneous carotid-cavernous fistulas. One of them is a direct type while three of them are indirect dural shunts. In indirect dural shunts, traditional transarterial embolization rarely achieves a complete clinical cure in a short period of time. All cases had an ophthalmologic disturbance justified for endovascular intervention. We performed catheterization, and subsequently embolization with Guglielmi Detachable coils, to the cavernous sinus via the femoral vein and inferior petrosal sinus/superior ophthalmic vein. All four patients achieved clinical and angiographic improvement with a follow-up period range from two to sixteen months except for one patient who had residual bilateral sixth nerve palsy. The transvenous approach offers an effective and safe alternative for the management of spontaneous carotid-cavernous fistula.

Abducens Nerve Diseases↗

PubMed2001

Cerebrotendinous xanthomatosis: heterogeneity of clinical phenotype with evidence of previously undescribed ophthalmological findings.

Cerebrontendinous xanthomatosis (CTX) is a rare autosomal recessive neurometabolic disease involving lipid metabolism. The classical phenotype is characterized by neurological dysfunction, tendon xanthomas and juvenile cataracts. Other ophthalmological findings have occasionally been reported. To gain more insight into the type and frequency of ophthalmological alterations in this multisystem metabolic disorder, we examined 13 CTX patients. Besides cataracts, found in all cases, the second most frequent ocular abnormality was paleness of the optic disk, which was found in 6 patients and was probably previously underestimated. Signs of premature retinal senescence were also observed. We discuss the possible relation between these ocular manifestations and the metabolic defect.

Adult↗

PubMed2000

Visually impaired Swedish children. The 1980 cohort study--a 19-year ophthalmological follow-up.

PURPOSE: To assess the ophthalmological outcome in a cohort of visually impaired children in a geographically defined area. METHODS: A 19-year follow-up of medical records, interview and, if needed, re-examination of the initial 128 patients, born 1962-1976. RESULTS AND CONCLUSION: Follow-up was possible in 123 patients (96%); 76 (59%) were still visually impaired, 17 (13%) were deceased, while 30 (23%) had acquired a visual function > or = 0.3. The chances of gaining a visual function outside the WHO limits for visual impairment was significantly higher for patients without additional impairments (p=0.0023). The initial ophthalmologic diagnosis remained unchanged in 88%. The diagnoses with improved visual development included albinism and congenital nystagmus, while retinal diseases showed poorer results. An increase in visual function could be seen even in the initially older age-groups, indicating a maturation of visual function beyond what is usually considered the limit of plasticity of the visual system.

Adolescent↗

PubMed2001

Results of ophthalmologic screening examinations of German Pinschers in Finland--a retrospective study.

OBJECTIVE: To retrospectively review ophthalmologic findings in German Pinschers in Finland. ANIMALS STUDIED: One hundred and twenty-two German Pinschers that had ophthalmologic examination performed according to the Finnish Kennel Club's Eye Scheme before June 15, 1999. PROCEDURES: A total of 154 eye examination reports of 122 dogs were analysed and all described findings were reported. RESULTS: Persistent hyperplastic tunica vasculosa lentis (PHTVL) was diagnosed in 8.4% of all cases, and hereditary cataract (HC) in 6.5%. Even higher numbers of similar changes were reported by ophthalmologists; some of the dogs, however, were officially diagnosed as "free of symptoms" of inherited ocular diseases. A relatively high number (4.5%) of dogs had reported changes in Y sutures. A pedigree analysis suggests recessive inheritance for both diseases. Because of missing information about many dogs in the pedigree, an autosomal incomplete inheritance pattern cannot be ruled out in either case. CONCLUSIONS: Both HC and PHTVL are inherited diseases in German Pinschers. Further studies are needed to determine the importance of the changes found in Y sutures. Discrepancies between the official diagnosis and described changes are probably partially due to the lack of familiarity with the published literature concerning this breed. Further studies are needed to ascertain the inheritance pattern for both diseases. So far breeding with affected animals should be avoided.

Animals↗

PubMed1999

Viscoelastic materials in veterinary ophthalmology.

Viscoelastic materials have applications in both human and veterinary ophthalmology and orthopedics and for humans in otorhinolaryngology, urinary incontinence, cosmetic surgery and cosmetics. In ophthalmology, viscoelasties are most commonly used in facilitating cataract and other anterior segment surgeries, but also have applications in posterior segment surgery and in the topical management of keratoconjunctivitis sicca. Viscoelastics are routinely used during anterior segment surgery in filling and maintaining the anterior chamber, repositioning the iris, and to coat and protect the corneal endothelium, and expand the capsular bag prior to intraocular lens implantation. Viscoelastics can also be essential in the management of intraoperative complications such as miosis, hemorrhage, posterior capsular tears, and vitreous presentation. The most common products available for ophthalmic use include various concentrations of sodium hyaluronate, chondroitin sulfate, and hydroxypropylmethylcellulose. The physical properties of each viscoelastic material are dependent on chain length, and intra-and interchain interactions. The rheologic properties of viscosity, pseudoplasticity, viscoelasticity, and surface tension dictate the usefulness of each material for a specific purpose.

Journal Article↗

PubMed2001

[Gene therapy in ophthalmology. Review of options and trends in corneal diseases].

BACKGROUND: Gene therapy has gained increasing attention and a number of ongoing clinical trials have been initiated. This article provides current perspectives and limitations on gene therapy in ophthalmology. Since a number of comprehensive studies on gene therapy for retinal diseases already exist, we focus attention to the treatment of anterior segment disorders of the eye. MATERIAL AND METHODS: We undertook a reference search (DIMDI, PubMed) of articles published between (1989-2000) using the key words cornea, conjunctiva, eye, gene therapy, and keratoplasty. The search was restricted to publications in English, French and German. In addition, we incorporated some results of our recent experiments on cytokine gene transfer to the cornea. RESULTS: Attention to gene therapy in ophthalmology is currently focused on retina and choroidea (40 articles) however, an increasing number of publications includes the cornea (12 articles). The majority of these contributions deals with improvements in the design of gene therapy vectors in particular for targeted application. CONCLUSIONS: Gene therapy to the cornea may offer interesting new venues. Currently, insufficient gene transfer technologies and safety concerns prevent the broad application in humans. However, a broad spectrum of applications can be supposed.

Corneal Diseases↗

PubMed2001

[Nonorganic disorders in ophthalmology: overview of diagnosis and therapy].

BACKGROUND: Nonorganic diseases may be psychogenic (somatoform diseases) or due to malingering. Both occur frequently in the ophthalmologic practice. METHODS: This article reviews the different diagnostic and therapeutic procedures in nonorganic ocular diseases. RESULTS: After presenting the general symptoms, the origins of psychogenic diseases are discussed. Then, the specific tests used in ophthalmology to diagnose nonorganic diseases and the organic eye diseases most frequently confused with nonorganic diseases will be reported. At the end, we will discuss the treatment and the prognosis of psychogenic diseases. CONCLUSION: An early diagnosis of nonorganic diseases is important, since a delayed diagnosis may worsen their prognosis.

Diagnosis, Differential↗

PubMed2003

[Prevalence of optic atrophy and associated ocular and systemic diseases in a department of paediatric ophthalmology].

BACKGROUND: Optic atrophy is one of the most common causes of severe visual impairment in children. So far an analysis of ocular and systemic findings comparing patients with and without optic atrophy has not performed. PATIENTS AND METHODS: Ocular and systemic findings of a total of 1042 patients (examination of all patients in May 1995 [N = 485] and May 2001 [N = 557]) of the department of paediatric ophthalmology in Homburg/Saar were retrospectively evaluated and diagnoses of patients with and without optic atrophy were compared. Optic atrophy was diagnosed ophthalmoscopically and in 1/3rd of the patients by VEP as well. RESULTS: 18 % of all patients (N = 188; 87.2 % [N = 164] were children) had optic atrophy. Nearly half of these children were prematurely born (46.7 %). 53.2 % of patients with optic atrophy (N = 88) showed nystagmus (without atrophy: 10.7 %), especially sensory defect nystagmus. Median of visual acuity level was 0.2 with optic atrophy and 0.8 without. Albinism and buphthalmia were common findings. 69.5 % of all patients with optic atrophy suffered from systemic diseases (without atrophy: 25.2 %), especially mental retardation, neurologic findings and oculocutaneous albinism. In 10.4 % more than two systemic diagnoses could be found. 55.3 % of all the patients with optic atrophy were disabled, 31.9 % multiply disabled. CONCLUSIONS: Sequelae of prematurity, peripartal asphyxia and congenital brain damages are the main findings in patients with optic nerve atrophy. Such children are worst-case patients of any paediatric ophthalmology department with a high prevalence of severe visual impairment, mental retardation and multiple disability. Treatment in a specialised center is therefore necessary for an efficient therapy. In addition, the statistical survey shows that in children who survived the critical phases of prematurity secondary damages can lead to persistent impairment and to alterations of visual and general development.

Adolescent↗

PubMed1982

[Current scanning and biometry equipment in ophthalmologic ultrasound diagnosis].

The development of ophthalmological sonographic equipment started as early as 1958 with the ultrasonic "slit lamp" of Baum and Greenwood. This was an immersion-type device with a closed membrane. Subsequent developments took place mainly after the introduction of the contact B-scanner by Bronson and Turner approximately 10 years ago. The instrument designed by them still serves as a source of inspiration for the creation of many "small-part" scanners. Among the special display modes featured in ophthalmological equipment, we have the deflection-modulated B-image (AB-mode) and the so-called "continuous-vector" mode for the A-trace selection from a real time B-mode image. Other examples are the specially designed A-mode equipment for measurement of the axial length of the human eye, and the A-mode equipment with a more or less standardized-gain characteristic curve of the overall amplification.

Anthropometry↗

PubMed1996

[Ophthalmologic spectrum of neurofibromatosis type 2 in childhood].

BACKGROUND: Neurofibromatosis type 2 (NF2) is a disorder with autosomal dominant inheritance which leads to tumor growth in the central and peripheral nervous system. In affected adult patients there is a typical association with ocular abnormalities like juvenile cataract. METHODS: Ophthalmologic investigation was carried out in ten children aged one to fourteen years with suspected NF2. The diagnosis was confirmed by further clinical examination and-in one patient-by segregation analysis. RESULTS: Nine of these ten children showed ocular abnormalities such as juvenile subcapsular cataracts, retinal hamartomas, optic nerve sheath tumors, fibrotic maculopathies as well as one case of a perineural calcification of the optic nerve and one case of a cerebral hamartoma on the ground of the third ventricle. DISCUSSION: In six children ophthalmological symptoms were the presenting symptom of the disease. The knowledge of these symptoms allows for the diagnosis of NF2 in children who present with isolated ocular deficits or with other typical criteria of the disease. The early diagnosis of the disease may lead to an improved prognosis with regard to preservation of hearing by surgery of bilateral vestibular schwannoma which occur in more than 90% of the NF2-patients.

Adolescent↗