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Biomedical subjects

Klara Landau

Publications and source records attributed to Klara Landau.

15 recordsLinked to original sources

Optic nerve sheath meningiomas in patients with neurofibromatosis type 2.

OBJECTIVE: To determine the prevalence of optic nerve sheath meningiomas (ONSMs) in patients with neurofibromatosis type 2 (NF2). METHODS: An observational retrospective case series of 30 consecutive patients with NF2 referred to an academic ophthalmology unit from November 1, 1991, through August 31, 2003. Twenty-six patients were followed up for a mean of 93 months (range, 3-150 months). One individual was lost to follow-up, and 3 had been referred recently. Diagnosis of ONSM was made based on typical neuroradiologic and clinical features in 7 patients and on histologic criteria in 1. RESULTS: Eight of 30 patients harbored unilateral (n = 6) or bilateral (n = 2) ONSMs. Six ONSMs were diagnosed at initial examination, and 4 during follow-up. CONCLUSIONS: There is a strong association between ONSMs and NF2 that parallels the well-known association of optic nerve gliomas with NF1. Physicians should be aware of the possibility that patients with ONSMs may also have NF2.

Adolescent↗

Ophthalmologic findings and long-term course in patients with neurofibromatosis type 2.

PURPOSE: To evaluate ophthalmologic findings and long-term course in patients with neurofibromatosis type 2 (NF2). DESIGN: Retrospective observational case series. METHODS SETTING: Single-center institutional practice. STUDY POPULATION: Thirty referred patients with NF2 were enrolled from 1991 to 2003 and underwent at least one thorough neuroophthalmologic examination. Follow-up of 23 patients ranged from 4.3 to 12.5 years. MAIN OUTCOME MEASURES: Visual function, structural ocular abnormalities, onset and type of presenting NF2-related symptoms, and number of central nervous system tumors. RESULTS: Initial symptoms for patients with early-onset NF2 mostly comprised ophthalmologic symptoms (n = 7) and lower motor neuron extremity weakness (n = 6), as opposed to eighth nerve impairment (n = 11) in late disease onset. NF2-specific ocular findings were noted in 83% of all patients (94% childhood onset; 67% adult onset): 67% had cataracts, 40% epiretinal membranes, 3% hamartoma, 13% disk gliomas, and 27% optic nerve sheath meningiomas. Only 14% in the childhood-onset group-as opposed to 78% in the adult-onset group-sustained visual acuity of 1.0 in both eyes at final examination. Significantly more patients with early onset of symptoms developed multiple central nervous system tumors (P = .004) and showed a higher amount of NF2-specific findings (P = .015). CONCLUSIONS: Initial manifestations of NF2 differ between children and adults. NF2-specific ophthalmologic findings can help establish the diagnosis. Symptom onset at a young age is clearly a risk factor for marked disease progression. These patients should be carefully followed because survival rates have increased, and vision becomes increasingly important as the disease progresses.

Adolescent↗

Sirolimus-eluting stenting of the external carotid artery for the treatment of ocular ischemia.

PURPOSE: To present a patient in whom severe external carotid artery (ECA) stenosis causing ocular ischemia was treated with a drug-eluting stent. CASE REPORT: A 55-year-old woman with severe, diffuse atherosclerosis presented with impaired left ocular perfusion and amaurosis fugax. Duplex ultrasonography and angiography documented bilateral occlusion at the origin of the internal carotid arteries, bilateral subtotal ECA stenoses, and subtotal distal left common carotid artery (CCA) restenosis following endarterectomy. Percutaneous revascularization of the left ECA and CCA stenoses was performed using a short coronary balloon-expandable sirolimus-eluting stent and a self-expanding nitinol stent, respectively. The procedure was uneventful, and the ocular symptoms resolved. At 6 months, the patient remained asymptomatic, with angiographically patent stents. CONCLUSION: Drug-eluting stenting may be a novel option to treat symptomatic ECA stenosis.

Alloys↗

Comparison of Harms tangent screen and search coil recordings in patients with trochlear nerve palsy.

Harms tangent screen, a subjective measurement method of three-dimensional binocular alignment, was compared with search coil recording. Twenty-three patients with unilateral trochlear nerve palsy were measured in nine gaze positions. The two methods correlated best for the horizontal gaze deviation, the vertical gaze deviation, and the vertical incomitance, but there was no correlation for the results of torsional incomitance. Using Harms tangent screen, torsional deviation underestimated the torsional incomitance measured by the search coils. Therefore, central torsional fusional mechanisms or alignment error in the Harms tangent screen are assumed.

Adolescent↗

The visual impact of fractionated stereotactic conformal radiotherapy on seven eyes with optic nerve sheath meningiomas.

BACKGROUND: Treatment of primary optic nerve sheath meningiomas (ONSMs) remains controversial. Although recent studies have suggested a favorable outcome of radiotherapy, controlled data on the efficacy of fractionated stereotactic conformal radiotherapy (SCRT) in primary ONSMs are still lacking. METHODS: Seven eyes treated with SCRT (total dose: 54 Gy) were compared with six eyes that were not treated because of patient or physician preference. The indication for intervention was deterioration of visual function with or without imaging evidence of tumor progression. Patients with secondary ONSMs and those with neurofibromatosis type 2 were excluded. The mean follow-up period was 57 months for the treated eyes and 61 months for the untreated eyes. RESULTS: Among the seven treated eyes, visual acuity improved in six, five of which sustained improvement of three or more Snellen lines. One eye deteriorated by two lines. Visual field improved in four eyes, remained stable in two, and deteriorated in one. Four untreated eyes showed worsening of visual acuity and two remained stable. Visual field deteriorated in three eyes and was stable in three. None of the untreated eyes experienced improvement in visual acuity or visual field. No complications of treatment were documented. CONCLUSIONS: In agreement with previous reports, these results indicate that SCRT is superior to observation in its impact on visual function in eyes with primary ONSMs.

Adolescent↗

Atypical midface tumor complicating nevus of ota.

In the differential diagnosis of midface masses, the nevus of Ota (also called oculodermal melanocytosis) is a rare entity. We present a case of a young white man, who lost his left eye function by progression of a melanocytotic lesion involving the ophthalmic (VI) and maxillary (VII) divisions of the trigeminal nerve. The time course, distribution along the trigeminal nerve, and characteristic MR signal intensities of the lesion, in correlation with the clinical, ophthalmological, and dermatological findings, point to the correct diagnosis.

Adolescent↗

Early improvements in vision after fractionated stereotactic radiotherapy for primary optic nerve sheath meningioma.

BACKGROUND AND PURPOSE: To report on the efficacy and follow-up of 23 patients with primary optic nerve sheath meningioma (ONSM) with fractionated stereotactic conformal radiotherapy (SCRT). PATIENTS AND METHODS: Between 1996 and 2003, 23 patients ( = 23 eyes) with ONSM were treated. Indications for primary stereotactic radiotherapy were tumour progression documented by imaging or symptoms (loss of vision, pain). All patients received SCRT with a median dose of 50.4Gy in 6 weeks. RESULTS: After a median follow-up of 20 months (1-68 months) a 95% (21 of 22) visual control was seen: vision improved in 16 patients and remained stable in 5. For 13/16 patients improvement was documented already within 1-3 months after SCRT. Vision became worse in one patient. An improvement of pain was observed after radiotherapy in 6 patients as well as of proptosis in 1 patient. For 1 patient pain was persistent after SCRT. In one patient 4 years after SCRT a radiation retinitis and vitreous haemorrhage was seen. CONCLUSIONS: Fractionated stereotactic radiotherapy improves vision, often shortly after treatment, and is thus a viable treatment option for this tumour entity.

Adolescent↗

Ocular rotation axes during dynamic Bielschowsky head-tilt testing in unilateral trochlear nerve palsy.

PURPOSE: To explain the positive Bielschowsky head-tilt (BHT) sign in unilateral trochlear nerve palsy (uTNP) by the kinematics of three-dimensional eye rotations. METHODS: Twelve patients with uTNP monocularly fixed on targets on a Hess screen were oscillated (+/- 35 degrees, 0.3 Hz) about the roll axis on a motorized turntable (dynamic BHT). Three-dimensional eye movements were recorded with dual search coils. Normal data were collected from 11 healthy subjects. RESULTS: The rotation axis of the viewing paretic or unaffected eye was nearly parallel to the line of sight. The rotation axis of the covered fellow eye, however, was tilted inward relative to the other axis. This convergence of axes increased with gaze toward the unaffected side. Over entire cycles of head roll, the rotation axis of either eye remained relatively stable in both the viewing and covered conditions. CONCLUSIONS: In patients with uTNP, circular gaze trajectories of the covered paretic or unaffected eye during dynamic BHT are a direct consequence of the nasal deviation of the rotation axis from the line of sight. This, in turn, is a geometrical result of decreased force by the superior oblique muscle (SO) of the covered paretic eye or, according to Hering's law, increased force parallel to the paretic SO in the covered unaffected eye. The horizontal incomitance of rotation axes along horizontal eye positions can be explained by the same mechanism.

Adolescent↗

Heroin and strabismus.

QUESTIONS UNDER STUDY: We report on a series of patients with acute strabismus related to heroin and methadone intake or withdrawal, discuss possible mechanisms involved in this clinical picture and make recommendations for work-up and referral. METHODS: Retrospective study. RESULTS: Five patients presented with acute esotropia within days of heroin withdrawal, and two patients developed exotropia related to heroin or methadone intake. Neurological work-up and neuroimaging was non-contributory in all four patients who were examined. CONCLUSION: Acute esotropia with double vision is a disturbing side effect of heroin withdrawal, and, similarly, acute exotropia may be related to heroin intake. In the absence of focal neurological signs, further work-up is not mandatory. Referral to a specialised orthoptic service is beneficial in offering the patient symptomatic treatment and reassurance, thus supporting successful withdrawal therapy.

Adult↗

Ocular complication of PhotoDerm VL therapy for facial port-wine stain.

BACKGROUND: A case of focal damage to the iris with distortion of the pupil secondary to PhotoDerm therapy in a 2-year-old boy is reported. OBJECTIVE: To study ocular complication of photoDerm VL therapy for facial port-wine stain. METHODS. : Observatory case report. RESULTS: PhotoDerm VL therapy may damage ocular tissues. CONCLUSION: Appropriate protection during the procedure is essential.

Child, Preschool↗

Primary position and listing's law in acquired and congenital trochlear nerve palsy.

PURPOSE: In ocular kinematics, the primary position (PP) of the eye is defined by the position from which movements do not induce ocular rotations around the line of sight (Helmholtz). PP is mathematically linked to the orientation of Listing's plane. This study was conducted to determine whether PP is affected differently in patients with clinically diagnosed congenital (conTNP) and acquired (acqTNP) trochlear nerve palsy. METHODS: Patients with unilateral conTNP (n = 25) and acqTNP (n = 9) performed a modified Hess screen test. Three-dimensional eye positions were recorded with dual search coils. RESULTS: PP in eyes with acqTNP was significantly more temporal (mean: 21.2 degrees ) than in eyes with conTNP (6.8 degrees ) or healthy eyes (7.2 degrees ). In the pooled data of all patients, the horizontal location of PP significantly correlated with vertical noncomitance with the paretic eye in adduction (R = 0.59). Using a computer model, PP in acqTNP could be reproduced by a neural lesion of the superior oblique (SO) muscle. An additional simulated overaction of the inferior oblique (IO) muscle moved PP back to normal, as in conTNP. Lengthening the SO and shortening the IO muscles could also simulate PP in conTNP. CONCLUSIONS: The temporal displacement of PP in acqTNP is a direct consequence of the reduced force of the SO muscle. The reversal of this temporal displacement of PP, which occurs in some patients with conTNP, can be explained by a secondary overaction of the IO muscle. Alternatively, length changes in the SO and IO muscles, or other anatomic anomalies within the orbit, without a neural lesion, may also explain the difference in location of PP between conTNP and acqTNP.

Adaptation, Ocular↗

Incomitance of ocular rotation axes in trochlear nerve palsy.

Strabismus due to palsy of a single muscle in one eye is always incomitant, which is a consequence of Hering's law of equal innervation. We asked whether this law had similar consequences on the orientation of ocular rotation axes. Patients with unilateral trochlear nerve palsy were oscillated about the nasooccipital (= roll) axis (+/-35 degrees, 0.3 Hz), and monocularly fixed on targets on a head-fixed Hess screen. Both the covered and uncovered eyes were measured with dual search coils. The rotation axis of the covered eye (paretic or healthy) tilted more nasally from the line of sight when gaze was directed toward the side of the healthy eye. The rotation axis of the viewing eye (paretic or healthy), however, remained roughly aligned with the line of sight. We conclude that incomitance due to eye muscle palsy extends to ocular rotation axes during vestibular stimulation.

Adolescent↗

Paraganglioma of the cervical sympathetic trunk.

We report the case of a 17-year-old man who presented with left-sided Horner's syndrome. Magnetic resonance imaging revealed a spindle-shaped cervical tumor in the left paravertebral space. During operation, a tumor originating from the left sympathetic trunk was found. The histopathologic analysis showed a sympathetic paraganglioma. The sympathetic trunk is an extremely rare location for a cervical paraganglioma; only a few cases have been reported in the literature.

Adolescent↗