Optical coherence tomography confirms shunt malfunction and recurrence of raised intracranial pressure in optic atrophy.

BACKGROUND: Investigating potential cerebrospinal fluid (CSF) shunt malfunction can be a challenge. Optical coherence tomography (OCT), a non-invasive imaging technique, is used to monitor changes at the optic nerve head in papilloedema. Conventional teaching suggests that in the presence of optic atrophy the optic nerve head may not re-swell in response to a relapse in raised intracranial pressure (ICP). METHODS: A retrospective case series of three patients who had prior CSF diversion surgery for idiopathic intracranial cranial hypertension (IIH) is presented demonstrating the benefit of non-invasive OCT imaging confirming raised ICP. RESULTS: Recurrence of raised ICP, due to malfunctioning CSF shunt, was diagnosed in three patients requiring further surgical intervention. All re-presented acutely with headache and visual disturbances. All had a prior diagnosis of optic atrophy. In all patients, OCT peripapillary retinal nerve fibre layer qualitative image analysis and quantified progression analysis permitted easy detection of the recurrence of papilloedema. CONCLUSION: OCT imaging supports clinical decision making in shunt malfunction, even in the presence of established optic atrophy secondary to IIH.

Six-month results of suprachoroidal adipose tissue-derived mesenchymal stem cell implantation in patients with optic atrophy: a phase 1/2 study.

PURPOSE: This prospective clinical case series aimed to investigate the safety and efficacy of suprachoroidal adipose tissue-derived mesenchymal stem cell (ADMSC) implantation in patients with optic nerve diseases. METHODS: This prospective, single-center, phase 1/2 study enrolled 4 eyes of 4 patients with optic atrophy of various reasons who underwent suprachoroidal implantation of ADMSCs. The best-corrected visual acuity (BCVA) in the study was HM at 1 m. The worse eye of the patient was operated. Patients were evaluated on the first day, first week, first month, third and sixth months postoperatively. BCVA, anterior segment and fundus examination, color photography, optical coherence tomography (OCT) and visual field examination were carried out at each visit. Fundus fluorescein angiography and multifocal electroretinography (mfERG) recordings were performed at the end of the first, third and sixth months and anytime if necessary during the follow-up. RESULTS: All 4 patients completed the six-month follow-up. None of them had any systemic or ocular complications. All of the patients experienced visual acuity improvement, visual field improvement and improvement in the mfERG recordings. We found choroidal thickening in OCT of the 4 patients. CONCLUSION: Even though the sample size is small, the improvements were still encouraging. Stem cell treatment with suprachoroidal implantation of ADMSCs seems to be safe and effective in the treatment for optic nerve diseases that currently have no curative treatment options.

Long-Term Follow-Up with Video of a Patient with Deafness-Dystonia Syndrome Treated with DBS-GPi.

BACKGROUND: The prevalence of deafness-dystonia syndrome (DDS) is relatively low. To our knowledge, only 2 cases of this syndrome treated with deep brain stimulation (DBS) have been reported. OBJECTIVES: We present a patient with DDS of unknown cause, refractory to medical treatment, who has been successfully treated with DBS of the internal globus pallidus (DBS-GPi) and followed up for 4 years. METHODS: A 21-year-old male, with progressive bilateral sensorineural hearing loss since the age of 3, developed dystonic movements at the age of 12. The patient presented with progressive segmental craniocervical dystonia with jaw-opening, tongue protrusion, retrocollis and gradual overflow including upper limb dystonia. Pharmacological therapy was ineffective. At the age of 17, the patient's condition deteriorated with the risk of developing a dystonic state. RESULTS: DBS-GPi implantation resulted in a striking improvement. The Burke-Marsden-Fahn Dystonia Rating Scale (BMFDRS) score improved from 75 points before the surgery to 10 points at 3 months after DBS-GPi implantation. Neurological examination at the age of 21 showed mild dystonic movements, mainly oromandibular dystonia (BMFDRS: 15 points). The clinical phenotype of our patient was consistent with Mohr-Tranebjaerg syndrome (MTS). We performed genetic analysis of the TIMM8A gene (the only gene in which mutations are known to cause MTS), but the result was negative; however, other potentially new mutations have to be considered. CONCLUSIONS: Based on our case with the longest reported follow-up of 4 years and 2 earlier reports, we advise to consider DBS-GPi in patients with DDS with unsatisfactory effect of pharmacological treatment.

Visual disturbance following shunt malfunction in a patient with congenital hydrocephalus.

A 25-year-old woman presented with complaints of nausea and headache. She had been treated with a ventriculoperitoneal shunt for hydrocephalus when she was 7 months old. Her bilateral optic discs showed moderate atrophy. Right visual acuity allowed only perception of hand movement and left visual acuity was 0.02 (1.2). Computed tomography (CT) showed mild ventricular dilation but no periventricular lucency. Intracranial pressure (ICP) was not high when the shunt valve was punctured. Her visual acuity deteriorated 5 days after the consultation. She was referred again 8 days after the first consultation. The bilateral optic discs were completely pale. Both pupils were dilated on admission, and the bilateral direct light reflexes were absent. The patient could slightly detect only green light stimulus. CT showed moderate enlargement of the ventricle. ICP was 47 cmH(2)O when the shunt valve was punctured. Shuntgraphy showed obstruction of the shunt at the distal end of peritoneal catheter. Emergent total shunt revision was performed. She could detect dark stimulus and the still-dilated left pupil had recovered direct light reflex on the next day. The visual acuity was 0.01 (0.7) on the left 6 months after the operation, although she was blind in the right eye and the bilateral optic discs were completely pale. Visual loss associated with shunt failure remains a major morbidity in shunted congenital hydrocephalus. Early diagnosis and shunt revision may allow visual recovery.

[A topodiagnostically characteristic visual field defect]. / Ein topodiagnostisch wegweisender Gesichtsfeldausfall.

A 69-year-old female patient presented with unilateral optic nerve atrophy and visual loss. The visual field examination showed not only a subtotal visual field loss in the affected eye but also a visual field defect in the other eye which was classified as an anterior junction syndrome. Cranial MRI revealed a tuberculum sellae meningioma which was treated surgically.

[Endoscopic transnasal approach for optic nerve decompression]. / Przeznosowy dostep endoskopowy celem odbarczenia nerwu wzrokowego.

UNLABELLED: Optic nerve decompression is nowadays increasingly performed using endoscopic technique. THE AIM of this report is to present a clinical series of 8 patients operated on with this technique due to posttraumatic loss of vision and other visual disturbances. Technical details of this procedure and surgical anatomy will also be discussed. MATERIAL AND METHODS: Endoscopic optic nerve decompression was performed in eight patients using transnasal transethmoidal approach. The loss of vision was caused by posttraumatic optic neuropathy in 6 patients, optic neuritis in 1 patient and by cystic lesion of the orbital apex and sphenoid sinus in 1 patient. RESULTS: Vision acuity improvement was achieved in 4 patients (50%). Complications included intraoperative cerebrospinal fluid leak occurred in 2 patients. In one of them the leak had to be sealed using intracranial approach. CONCLUSIONS: Endoscopic optic nerve decompression can be used in appropriately selected patients with optic nerve lesions to improve the visual functions. High technical skill, proper endowment and thorough knowledge of surgical anatomy are prerequisites of success. Good interdisciplinary cooperation between the laryngologist and neurosurgeon helps in minimizing the risk of serious complications, especially in posttraumatic cases.

[Biomechanical properties of the cribriform lamina in glaucoma-induced atrophy of the optic nerve and after decompression operations on the scleral canal].

The authors made a mathematical and clinical analysis of the cribriform lamina (CL) of the optic nerve and nerve fiber bundles passing through its foramens in glaucoma-induced optic neuropathy. It was shown that in elevated intraocular pressure, there was a significant CL deformity leading to the compression and deformity of nerve fibers. The important biomechanical characteristic of CL deformity is the tangent of the angle between the longitudinal axis of the scleral canal and the perpendicular to the CL surface. An increase in the inner radius of the scleral canal of the optic nerve due to its dissection at decompression surgery is one of the ways of diminishing CL flexure and nerve fiber deformity. A small increase in the scleral canal radius (by approximately 10%) was demonstrated to eliminate significant CL flexure.

Intracranial Rosai-Dorfman disease in a child mimicking bilateral giant petroclival meningiomas: a case report and review of literature.

UNLABELLED: OBJECTIVES AND IMPORTANCE: Rosai-Dorfman disease (RDD) is a rare but distinctive entity of unknown etiology; isolated intracranial RDD is uncommon. Of 37 reported intracranial RDD cases, only three were reported in children. CLINICAL PRESENTATION: We report an unusual case of a 15-year-old boy presenting with 4 months history of raised intracranial pressure with visual deterioration. Computed tomography and magnetic resonance imaging revealed bilateral petroclival enhancing lesions with cavernous sinus extension mimicking meningioma. However, histological examination was diagnostic of RDD. INTERVENTION: The patient underwent extended right-sided middle fossa approach and near-total tumor removal from petroclival region and cavernous sinus on both sides in two stages 6 weeks apart. CONCLUSION: Ours is the first case of pediatric isolated intracranial RDD presenting with giant bilateral petroclival masses successfully managed with bilateral extended middle fossa approach in two stages. An optimal treatment for RDD is not established, but complete surgical resection alone seems effective.

Case report of optic atrophy in pansynostosis: an unusual presentation of scalp edema from hair braiding.

Pansynostosis (fusion of all cranial sutures) and optic atrophy were found as incidental CT scan and ophthalmological findings in an 8-year-old who presented to the emergency room with scalp edema from tight 'cornrow' hair braiding. Cranial vault expansion was successfully performed. Ophthalmological problems have stabilized but have not reversed. Late presentation of craniosynostosis and the pathophysiology of secondary optic atrophy are discussed.

Visualization of residual perfluorocarbon liquid using intravitreal triamcinolone acetonide.

The visualization of transparent perfluorocarbon liquid (PFCL) using triamcinolone acetonide is described. Intravitreal injection of triamcinolone acetonide enabled visualization of residual PFCL intraoperatively. In addition, it was shown that triamcinolone acetonide could visualize PFCL in an in vitro preparation of balanced salt solution. This in vitro experiment confirmed that triamcinolone acetonide also could be adsorbed by PFCL outside the vitreous. Triamcinolone acetonide was helpful to visualize transparent PFCL both in vivo and in vitro, and may be useful at the end of vitrectomy to completely remove residual PFCL from the eye.

Tuberculum sellae meningioma associated with lymphomatoid papulosis.

BACKGROUND: Meningiomas involving the tuberculum sellae and planum sphenoidale typically cause visual loss by affecting the optic nerve and anterior chiasm. We present a case of a tuberculum sellae meningioma concurrent with lymphomatoid papulosis, a T-cell lymphomatoid skin disorder. CASE REPORT: A 50-year-old black woman came to the eye clinic with a symptom of decreased vision in the right eye for one week, especially when reading. Best-corrected vision was counting fingers at two feet O.D. and 20/40 O.S. She was seen seven months earlier, with 20/25 O.D. and O.S. Dilated fundus examination revealed bilateral optic atrophy, O.D. greater than O.S. MRI revealed a meningioma arising from the tuberculum sellae and planum sphenoidale region. The meningioma was partially resected nine days later Visual field performed 18 days postoperatively demonstrated an overall depression in the right eye and no defect in the left eye. Best-corrected vision improved to 20/150 O.D. and 20/30 O.S. CONCLUSION: Meningiomas are generally benign tumors that can cause symptoms if vital structures are compromised. Altered visual function and optic atrophy may be the only presentation of intracranial and orbital tumors. Intracranial meningiomas may be associated with systemic conditions that can infiltrate the optic nerve, including lymphocytic disorders. Lymphomatoid papulosis (LyP) is a rare cutaneous disorder involving infiltrating clonal T-cells that has been associated with disseminated lymphomatic skin tumors. It is unclear if there is a direct association between LyP and meningiomas.

[The study on the value of the decompression to the optic nerve channel for the optic atrophy patients].

OBJECTIVE: To evaluate the effective treatment method for the optic nerve atrophy. METHOD: Sixteen patients were diagnosed as optic nerve atrophy by examining the sight, eye base and VEP. There were no occupied lesion in the eye, nose sinus and the intracranial through the CT examination. The visual ability were become worse before the operation. This group of the patients were underwent the eye-nose-ethmoid -sphenoid approach to undergo the decompress of the optic nerve channel. During the operation, the ethmoid was removed and the sphenoid sinus was opened. When the optic nerve channel was found, more than half of it was removed. Then the whole nerve channel was opened. The sheath and the tendon of the optic nerve were opened in longitude direction. The antibiotic, hormone vessel dilation drugs were given. The changes of the visual ability, eye base and VEP between preoperation and postoperation were compared. RESULT: The visual ability and the VEP of 16 cases of the of the patients were improved. A week after the operation and three months latter examination of the eye base showed that ten of the patients got improved and six of them no change. There were no serious postoperative complication. CONCLUSION: The decompression of the optic nerve channel is the ideal method to deal with optic nerve atrophy.

[Effectiveness of sub-Tenon's capsule implantation of collage sponge in the treatment of glaucomatous atrophy of the optic nerve]. / Effektivnost' substenonovoi implantatsii kollagenovoi gubki pri lechenii glaukomatoznoi atrofii zritel'nogo nerva.

The surgery of "subtenon implantation of collagen sponge" is offered; its essence is in implanting a fragment of hemostatic collagen sponge for the purpose of revasculizing and improving the trophism of the retina and optic nerve. A total of 96 patients (105 eyes) with glaucomatous atrophy of the optic nerve were operated on. The reliable results of visometry as well as of kinetic and static perimetry and of rheography were obtained. The efficiency of the surgery (without any additional conservative therapy) ranged from 63.4 to 78.6% according to various examination methods. The visual functions improved within 6 to 12 months after surgery.

[Optic nerve bilateral atrophy in a suprachiasmatic astrocytoma]. / Atrofie optica bilaterala la copil prin astrocitom pilocitic.

The paper presents a clinical case of a 5 years old child with bilateral optic atrophy and the specific diagnosis and treatment problems of this case. The image investigations have a major part in establishing the positive diagnosis: computerized tomography, magnetic resonance imaging and the elective treatment belongs to the field of neurosurgery.

Neurosurgical interventions in children with Maroteaux-Lamy syndrome. Case report and review of the literature.

This paper reports the case of a 14-year-old child with Maroteaux-Lamy syndrome (mucopolysaccharidosis type 6) who was treated consecutively for compressive damage of the optic nerves, hydrocephalus communicans and progressive spastic tetraparesis within 2 years. The clinical course of the patient is presented and the pathophysiologic mechanisms of disease progression in patients with Maroteaux-Lamy syndrome are discussed and reviewed.

[Changes to visual acuity and the visual field in a case of meningioma operated at the anterior level]. / Modificari ale acuitatii vizuale si ale câmpului vizual într-un caz de meningiom de etaj anterior operat.

It's presented a midline meningioma of anterior level case which preoperative has an importance affectation of the visual acuity and visual field, and postoperative in dynamic is founded the important improvement of visual acuity and visual field. It's discussed visual field topography and atrophy optic pathophysiology. It's a neuro-ophthalmology case example, where the cooperation neurosurgery-ophthalmology permit medical solution and restored in social life of the patient.

[Mechanisms of sensory function recovery in partial optic nerve atrophy before and after treatment with ampli-pulse phoresis]. / O mekhanizmakh vosstanovleniia sensornykh funktsii pri chastichnoi atrofii zritel'nogo nerva do i posle lecheniia amplipul's-forezom.

Eighty-three patients (119 eyes) with partial optic nerve atrophy of different origin were treated by amplipulse phoresis and observed for 1 year. Based on the data of modern methods of examination of visual functions (changes in visual evoked potentials and achromatic static campimetry), the authors suggest that several mechanisms of visual function improvement underlie the therapeutic effect of amplipulse phoresis. These mechanisms are as follows: activation of the intact part of nerve fibers with restoration of myelin membranes, restoration of viability of nerve fibers in a state of parabiosis; a probable mechanism of visual acuity improvement is modification of the conduction of visual route on-channels, but this hypothesis needs further investigations.