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Cureus Apr 2024Background Treatment of patients with a giant pituitary neuroendocrine tumor (GPitNET) is challenging. Here, we present the methods used for the clinical management of...
Background Treatment of patients with a giant pituitary neuroendocrine tumor (GPitNET) is challenging. Here, we present the methods used for the clinical management of patients who underwent GPitNET resection mainly via endoscopic endonasal surgery along with multimodal support to avoid surgical complications, which can affect the outcomes. Methodology The medical records of 25 patients with a GPitNET who underwent endonasal endoscopic surgery were retrospectively reviewed. Complications were analyzed and factors affecting the extent of resection were evaluated. Results Gross total resection was achieved in six (24%), near-total resection (>90%) in nine (36%), and partial resection in 10 (40%) patients. Multivariate analyses revealed that tumors invading the middle fossa had negative effects on the extent of resection (odds ratio = 0.092, p = 0.047). Postoperative vision improved or normalized in 16 (64%), remained stable in eight (32%), and worsened in one (4%), while a new hormonal deficit was noted in seven (28%) patients. Complications included permanent oculomotor nerve palsy in one (4%) and transient oculomotor palsy in one (4%), apoplexy of the residual tumor resulting in ischemic stroke in one (4%), postoperative cerebrospinal fluid leakage in one (4%), and permanent diabetes insipidus in six (24%) patients. Conclusions For GPitNETs that extend into the middle fossa, our study underscored the difficulties in surgical extraction and the necessity for tailored treatment approaches. To ensure the safest and most complete removal possible, the surgical strategy must be specifically adapted to each case. Additionally, employing a comprehensive support approach is essential to reduce the chance of complications in patients impacted by this condition.
PubMed: 38707178
DOI: 10.7759/cureus.57498 -
European Journal of Ophthalmology May 2024Isolated acquired superior rectus palsy (ASRP) is extremely rare. The goal of this report is to describe the clinical manifestations and surgical protocols for treatment...
PURPOSE
Isolated acquired superior rectus palsy (ASRP) is extremely rare. The goal of this report is to describe the clinical manifestations and surgical protocols for treatment of isolated traumatic ASRP.
DESIGN
Retrospective observational case series.
METHOD
Records of patients presenting with traumatic isolated ASRP from 2011 to 2020 were retrospectively reviewed. Variables analyzed included nature of the damage, ocular alignment, ocular motility, binocular vision, fundus photography, imaging and surgical treatment.
RESULT
Of the 23,498 strabismus surgeries performed, 16 (0.07%) were identified as being attributable to traumatic isolated ASRP. Iatrogenic injury during orbital/brain tumorectomy was the most common cause for ASRP, followed by injury resulting from a sharp object, blunt instrument, car accident, dog bite, accidental fall and explosion. The main clinical features were hypotropia and deficient supraduction of the affected eye, accompanied by exotropia, extorsion, decreased vision and ptosis. The imaging examination showed the superior orbital wall fracture in 3 cases and superior rectus rupture in 7 cases. Inferior rectus recession and/or superior rectus resection comprised the major surgical procedures employed for these cases. Vertical deviations were reduced from 45.60 ± 17.52 PD preoperatively to 12.20 ± 12.97 PD postoperatively after an average of 22.70 months at follow-up, with a success rate of 50%.
CONCLUSION
Traumatic isolated ASRP comprised only 0.07% of cases receiving strabismus surgery at our hospital. Orbital imaging is essential to identify whether muscle rupture, orbital fractures and/or other possible disorders are present. Superior rectus resection and inferior rectus recession represented the most frequently used surgical procedures.
PubMed: 38706252
DOI: 10.1177/11206721241252332 -
Frontiers in Neuroanatomy 2024The cavernous sinus (CS) is a demanding surgical territory, given its deep location and the involvement of multiple neurovascular structures. Subjected to recurrent...
BACKGROUND
The cavernous sinus (CS) is a demanding surgical territory, given its deep location and the involvement of multiple neurovascular structures. Subjected to recurrent discussion on the optimal surgical access, the endoscopic transorbital approach has been recently proposed as a feasible route for selected lesions in the lateral CS. Still, for this technique to safely evolve and consolidate, a comprehensive anatomical description of involved cranial nerves, dural ligaments, and arterial relations is needed.
OBJECTIVE
Detailed anatomical description of the CS, the course of III, IV, VI, and V cranial nerves, and C3-C7 segments of the carotid artery, all described from the ventrolateral endoscopic transorbital perspective.
METHODS
Five embalmed human cadaveric heads (10 sides) were dissected. An endoscopic transorbital approach with lateral orbital rim removal, anterior clinoidectomy, and petrosectomy was performed. The course of the upper cranial nerves was followed from their apparent origin in the brainstem, through the middle fossa or cavernous sinus, and up to their entrance to the orbit. Neuronavigation was used to follow the course of the nerves and to measure their length of surgical exposure.
RESULTS
The transorbital approach allowed us to visualize the lateral wall of the CS, with cranial nerves III, IV, V1-3, and VI. Anterior clinoidectomy and opening of the frontal dura and the oculomotor triangle revealed the complete course of the III nerve, an average of 37 (±2) mm in length. Opening the trigeminal pore and cutting the tentorium permitted to follow the IV nerve from its course around the cerebral peduncle up to the orbit, an average of 54 (±4) mm. Opening the infratrochlear triangle revealed the VI nerve intracavernously and under Gruber's ligament, and the extended petrosectomy allowed us to see its cisternal portion (27 ± 6 mm). The trigeminal root was completely visible and so were its three branches (46 ± 2, 34 ± 3, and 31 ± 1 mm, respectively).
CONCLUSION
Comprehensive anatomic knowledge and extensive surgical expertise are required when addressing the CS. The transorbital corridor exposes most of the cisternal and the complete cavernous course of involved cranial nerves. This anatomical article helps understanding relations of neural, vascular, and dural structures involved in the CS approach, essential to culminating the learning process of transorbital surgery.
PubMed: 38693948
DOI: 10.3389/fnana.2024.1367533 -
Journal of Medical Case Reports May 2024Orbital metastasis is a possible complication of small cell lung cancer and a pattern of bilateral invasion of the extraocular muscles has rarely been reported in...
BACKGROUND
Orbital metastasis is a possible complication of small cell lung cancer and a pattern of bilateral invasion of the extraocular muscles has rarely been reported in literature.
CASE PRESENTATION
A 46-year-old white male with a past medical history of smoking and stage IV small cell lung carcinoma presented with loss of vision and pain in the left eye. Examination revealed bilateral proptosis and left afferent pupillary defect, and visual acuity was hand motion on the left eye and 4/10 on the right eye. An orbital computed tomography scan showed a compression of the left optic nerve between the extraocular muscles at the apex, and a lateral canthotomy was performed for a new-onset compressive optic neuropathy, with residual visual improvement. There was also significant enlargement of the extraocular muscles in the right orbit. The patient was maintained in palliative treatment with both chemotherapy and local medical and surgical (amniotic membrane cover for exposure keratopathy) ophthalmological treatments until he eventually died 5 months after.
CONCLUSION
Bilateral metastasis to the extraocular muscles is a very rare manifestation of small cell lung cancer and the palliative treatment in these cases is challenging.
Topics: Humans; Male; Middle Aged; Fatal Outcome; Lung Neoplasms; Oculomotor Muscles; Orbital Neoplasms; Palliative Care; Small Cell Lung Carcinoma; Tomography, X-Ray Computed
PubMed: 38693545
DOI: 10.1186/s13256-024-04525-z -
Optometry and Vision Science : Official... Apr 2024A wearable optical apparatus that compensates for eye misalignment (strabismus) to correct for double vision (diplopia) is proposed. In contrast to prism lenses,...
SIGNIFICANCE
A wearable optical apparatus that compensates for eye misalignment (strabismus) to correct for double vision (diplopia) is proposed. In contrast to prism lenses, commonly used to compensate for horizontal and/or vertical misalignment, the proposed approach is able to compensate for any combination of horizontal, vertical, and torsional misalignment.
PURPOSE
If the action of the extraocular muscles is compromised (e.g., by nerve damage), a patient may lose their ability to maintain visual alignment, negatively affecting their binocular fusion and stereo depth perception capability. Torsional misalignment cannot be mitigated by standard Fresnel prism lenses. Surgical procedures intended to correct torsional misalignment may be unpredictable. A wearable device able to rectify visual alignment and restore stereo depth perception without surgical intervention could potentially be of great value to people with strabismus.
METHODS
We propose a novel lightweight wearable optical device for visual alignment correction. The device comprises two mirrors and a Fresnel prism, arranged in such a way that together they rotationally shift the view seen by the affected eye horizontally, vertically, and torsionally. The extent of the alignment correction on each axis can be arbitrarily adjusted according to the patient's particular misalignment characteristics.
RESULTS
The proposed approach was tested by computer simulation, and a prototype device was manufactured. The prototype device was tested by a strabismus patient exhibiting horizontal and torsional misalignment. In these tests, the device was found to function as intended, allowing the patient to enjoy binocular fusion and stereo depth perception while wearing the device for daily activities over a period of several months.
CONCLUSIONS
The proposed device is effective in correcting arbitrary horizontal, vertical, and torsional misalignment of the eyes. The results of the initial testing performed are highly encouraging. Future study is warranted to formally assess the effectiveness of the device on multiple test patients.
Topics: Humans; Strabismus; Vision, Binocular; Wearable Electronic Devices; Oculomotor Muscles; Equipment Design; Depth Perception; Eyeglasses; Eye Movements
PubMed: 38684063
DOI: 10.1097/OPX.0000000000002122 -
Neurology May 2024We developed repetitive ocular vestibular-evoked myogenic potentials (roVEMP) as an electrophysiologic test that allows us to elicit the characteristic decrement of...
BACKGROUND AND OBJECTIVES
We developed repetitive ocular vestibular-evoked myogenic potentials (roVEMP) as an electrophysiologic test that allows us to elicit the characteristic decrement of extraocular muscles in patients with ocular myasthenia gravis (OMG). Case-control studies demonstrated that roVEMP reliably differentiates patients with OMG from healthy controls. We now aimed to evaluate the diagnostic accuracy of roVEMP for OMG diagnosis in patients with ptosis and/or diplopia.
METHODS
In this blinded prospective diagnostic accuracy trial, we compared roVEMP in 89 consecutive patients presenting with ptosis and/or diplopia suspicious of OMG with a multimodal diagnostic approach, including clinical examination, antibodies, edrophonium testing, repetitive nerve stimulation of accessory and facial nerves, and single-fiber EMG (SFEMG). We calculated the roVEMP decrement as the ratio between the mean of the first 2 responses compared with the mean of the sixth-ninth responses in the train and used cutoff of >9% (unilateral decrement) in a 30 Hz stimulation paradigm.
RESULTS
Following a complete diagnostic work-up, 39 patients (44%) were diagnosed with ocular MG, while 50 patients (56%) had various other neuro-ophthalmologic conditions, but not MG (non-MG). roVEMP yielded 88.2% sensitivity, 30.2% specificity, 50% positive predictive value (PPV), and 76.5% negative predictive value (NPV). For comparison, SFEMG resulted in 75% sensitivity, 56% specificity, 55.1% PPV, and 75.7% NPV. All other diagnostic tests (except for the ice pack test) also yielded significantly higher positive results in patients with MG compared with non-MG.
DISCUSSION
The study revealed a high sensitivity of 88.2% for roVEMP in OMG, but specificity and PPV were too low to allow for the OMG diagnosis as a single test. Thus, differentiating ocular MG from other neuro-ophthalmologic conditions remains challenging, and the highest diagnostic accuracy is still obtained by a multimodal approach. In this study, roVEMP can complement the diagnostic armamentarium for the diagnosis of MG.
CLASSIFICATION OF EVIDENCE
This study provides Class I evidence that in patients with diplopia and ptosis, roVEMP alone does not accurately distinguish MG from non-MG disorders.
TRIAL REGISTRATION INFORMATION
ClinicalTrials.gov: NCT03049956.
Topics: Humans; Myasthenia Gravis; Male; Female; Diplopia; Middle Aged; Vestibular Evoked Myogenic Potentials; Adult; Blepharoptosis; Aged; Prospective Studies; Electromyography; Sensitivity and Specificity; Oculomotor Muscles; Young Adult
PubMed: 38669629
DOI: 10.1212/WNL.0000000000209395 -
Medicine Apr 2024Joubert syndrome (JS) is a rare genetic disorder that presents with various neurological symptoms, primarily involving central nervous system dysfunction. Considering...
RATIONALE
Joubert syndrome (JS) is a rare genetic disorder that presents with various neurological symptoms, primarily involving central nervous system dysfunction. Considering the etiology of JS, peripheral nervous system abnormalities cannot be excluded; however, cases of JS accompanied by peripheral nervous system abnormalities have not yet been reported. Distinct radiological findings on brain magnetic resonance imaging were considered essential for the diagnosis of JS. However, recently, cases of JS with normal or nearly normal brain morphology have been reported. To date, there is no consensus on the most appropriate diagnostic method for JS when imaging-based diagnostic approach is challenging. This report describes the case of an adult patient who exhibited bilateral peroneal neuropathies and was finally diagnosed with JS through genetic testing.
PATIENT CONCERNS AND DIAGNOSIS
A 27-year-old man visited our outpatient clinic due to a gait disturbance that started at a very young age. The patient exhibited difficulty maintaining balance, especially when walking slowly. Oculomotor apraxia was observed on ophthalmic evaluation. During diagnostic workups, including brain imaging and direct DNA sequencing, no conclusive findings were detected. Only nerve conduction studies revealed profound bilateral peroneal neuropathies. We performed whole genome sequencing to obtain a proper diagnosis and identify the gene mutation responsible for JS.
LESSONS
This case represents the first instance of peripheral nerve dysfunction in JS. Further research is needed to explore the association between JS and peripheral nervous system abnormalities. Detailed genetic testing may serve as a valuable tool for diagnosing JS when no prominent abnormalities are detected in brain imaging studies.
Topics: Humans; Male; Adult; Kidney Diseases, Cystic; Cerebellum; Eye Abnormalities; Peroneal Neuropathies; Abnormalities, Multiple; Retina; Magnetic Resonance Imaging
PubMed: 38669389
DOI: 10.1097/MD.0000000000037987 -
Cureus Mar 2024Epstein-Barr virus (EBV) can cause follicular conjunctivitis, keratitis, oculoglandular syndrome, meningitis, and encephalitis. We report a 54-year-old Hispanic male who...
Epstein-Barr virus (EBV) can cause follicular conjunctivitis, keratitis, oculoglandular syndrome, meningitis, and encephalitis. We report a 54-year-old Hispanic male who presented with right pupil-involved complete ophthalmoplegia, orbital and masticatory muscle inflammation, trigeminal enhancement, and new corneal infiltrate highly suggestive of EBV. Labwork was negative except for positive EBV polymerase chain reaction (PCR) in serum. Magnetic resonance imaging (MRI) of his brain and orbits with contrast showed enhancement of the right ganglion of the trigeminal nerve, oculomotor nerve, all extraocular muscles in the right orbit, and right masticatory and temporalis muscles and a right subacute lacunar infarct. The patient was diagnosed with encephalitis and orbital-face inflammation secondary to EBV infection. The patient improved with systemic steroids.
PubMed: 38659504
DOI: 10.7759/cureus.56888 -
Indian Journal of Ophthalmology May 2024
Topics: Humans; Retinal Ganglion Cells; Tomography, Optical Coherence; Macula Lutea; Oculomotor Muscles; Male; Female; Nerve Fibers; Follow-Up Studies; Ophthalmologic Surgical Procedures; Adult; Strabismus
PubMed: 38648439
DOI: 10.4103/IJO.IJO_2353_23