We report a 39-year-old male who had generalized tonic-clonic seizure with loss of awareness. Investigations led to a diagnosis of a left temporal lobe tumor. He underwent resection of the mass with consequent loss of brain tissue in the temporal lobe and was found to have a complete right homonymous hemianopia in the immediate postoperative period. Macular ganglion cell analysis on optical coherence tomography (OCT) showed homonymous thinning affecting the inferonasal sector in the right eye and inferotemporal sector in the left eye. This case demonstrates transsynaptic retrograde degeneration through the interruption of the inferior optic radiation, and its corresponding effect on the structure and function of the affected retinal field. Temporal lobe lesions may cause not only a homonymous visual f ield defect contralateral to the side of the lesion but also result to homonymous sectoral thinning of the macular ganglion cell complexes in both eyes located ipsilateral to the side of the lesion.

Objective: This study described the clinical profile and outcomes of patients with ocular dermoid cysts at a subspecialty Ophthalmology clinic of the Philippine General Hospital (PGH). Methods: Medical records of 38 patients seen at the External Disease & Cornea Clinic from January 2012 to June 2023 were reviewed. Demographics, best-corrected visual acuity (BCVA), refraction, other associated malformations, and dermoid characteristics were collected. For those that underwent surgery, the procedures performed and histopathology reports were reviewed. Most recent refraction, BCVA, presence of amblyopia, recurrence, and complications were recorded. Results: Mean age at presentation was 8.33 years old with equal male:female distribution (52% vs 48%). Thirtyfour (34) participants (89.5%) had unilateral dermoids and 4 participants (10.5%) had bilateral dermoids, for a total of 42 eyes. Thirty-five (35) or 83.3% of the dermoids were located at the inferotemporal quadrant of the cornea. There were 42.1% participants who had associated congenital anomalies, with dermolipoma and preauricular tags being the most common ocular and craniofacial findings, respectively. Twenty-seven (27) eyes (64.2%) underwent surgery and the most common procedure was excision with limbal conjunctival autograft (55.6%). In the 8 eyes that underwent simple excision, recurrence of the dermoid was seen in 1 patient and development of pseudopterygium in 5 patients. Dermoid was the histopathological diagnosis in 96.3% of the excised tissues. Twenty-four (24) patients (63.2%) presented with ambylopia with BCVA <20/30, and regardless of type of intervention done, only 7 participants (23.3%) had a visual acuity better than 20/30 during their latest consult. Conclusion Ocular dermoid is a common congential anomaly usually presenting as an isolated, unilateral mass at the inferotemporal limbal area. Aside from the obvious effect on the cosmetic appearance of the eye, a more pressing concern is the high incidence of amblyopia. Timely excision with limbal conjunctival autograft is highly recommended coupled with aggressive amblyopia therapy.
Recurrence ; Amblyopia

OBJECTIVE

This study described the ophthalmic symptoms and signs in patients with nasopharyngeal carcinoma (NPCA).

This was a retrospective, cross-sectional, descriptive study involving patients with histologically-confirmed NPCA seen in two subspecialty eye clinics in a single referral hospital from January 2014 to December 2018. Chart review obtained data on symptoms and ophthalmic findings of patients with NPCA on the first visit. Descriptive statistics was used to analyze the data.

There were 36 patients in the study. There were 27 males (75%) and mean age was 47 years (Range: 13 - 83). Delay to consult was marked, with 28 patients (78%) presenting later than three months; 19 (53%) had invasion to distant sites on presentation. Almost all of the patients (35/36 or 97%) had either diplopia or blurring of vision, with nasal symptoms as the most common extra-ophthalmic accompanying symptom. Multiple cranial nerve palsies, particularly optic nerve plus at least one ocular motor nerve, was a prominent feature. The combination of nasal symptoms with ophthalmoparesis was noted in 24 patients (67%) and was identified as a red flag for NPCA.

Blurred vision and diplopia were the most common ocular complaints of patients with NPCA who were evaluated at the ophthalmology department of a tertiary hospital. Blurred vision is frequently from optic nerve involvement while diplopia is due to ophthalmoparesis secondary to multiple ocular motor cranial nerves involvement. Male patients in their 40s who present with combination of optic neuropathy or ocular motor palsies should be probed for presence of otologic or nasal symptoms as well as neck masses as these are the common presentation of NPCA in the ophthalmology clinics.

We report a 39-year-old male who had generalized tonic-clonic seizure with loss of awareness. Investigations led to a diagnosis of a left temporal lobe tumor. He underwent resection of the mass with consequent loss of brain tissue in the temporal lobe and was found to have a complete right homonymous hemianopia in the immediate postoperative period. Macular ganglion cell analysis on optical coherence tomography (OCT) showed homonymous thinning affecting the inferonasal sector in the right eye and inferotemporal sector in the left eye. This case demonstrates transsynaptic retrograde degeneration through the interruption of the inferior optic radiation, and its corresponding effect on the structure and function of the affected retinal field. Temporal lobe lesions may cause not only a homonymous visual f ield defect contralateral to the side of the lesion but also result to homonymous sectoral thinning of the macular ganglion cell complexes in both eyes located ipsilateral to the side of the lesion.
Human ; retinal ganglion cells ; hemianopsia ; temporal lobe

Humans ; Intervertebral Disc Displacement/surgery* ; Skull ; Lumbar Vertebrae/surgery* ; Blindness ; Treatment Outcome ; Intervertebral Disc Degeneration/surgery*

Objectives: Staphylococcal blepharitis is a common ocular condition that can cause significant visual morbidities due to corneal complications. This study described the clinical profile of patients with staphylococcal blepharitis seen in a tertiary referral eye center, and determined the frequency and the type of corneal complications, the possible reasons for the delay in diagnosis, and the management prior to the consult. Methods: This study was a single-center, five-year retrospective case series design. The charts of all patients from January 2016 to December 2021 with the diagnosis of staphylococcal blepharitis seen at the External Disease and Cornea Clinic of the Philippine General Hospital that have fulfilled the inclusion and exclusion criteria were included. The data extracted were age, sex, chief complaint, laterality, time of onset of symptoms to consult, previous consults, lid and lid margin findings, conjunctival and corneal findings, pre- and post-treatment uncorrected distance visual acuity, duration of follow-up, and treatments received. Results: Fifty-five (55) charts out of 107 charts with a diagnosis of staphylococcal blepharitis were included. Eighty percent (80%) or 44 patients had bilateral disease. Ninety-nine (99) eyes of 55 patients were analyzed. The median age of the study population was 19 years. Sixty-seven percent (67%) were female, and 33% were male. The mean duration of follow-up at the External Disease and Cornea Clinic was 10.8 ± 14.61 months. Corneal opacity, eye redness, and blurring of vision comprised 70% of the reasons for consult. The mean time from the onset of symptoms to consult was 18.36 ± 25.69 months. Sixty-seven percent (67%) had prior consults elsewhere and 45% came in with a different diagnosis. Seventy-eight (78) eyes had fibrin or crust on the lashes. Fifty percent (50%) of the eyes had concomitant conjunctivitis, while 30% had meibomitis. Fifty-eight percent (58%) of patients had corneal complications. Seventy-two percent (72%) of eyes had bilateral involvement. The median age of patients with corneal complications subgroup was 13 years. The most common corneal complications noted were neovascularization, phlyctenulosis, pannus formation, and marginal infiltrates or ulcers. Twenty-two percent (22%) of all study eyes had visually disabling corneal complications like corneal ulcer, descemetocele, corneal perforation, and corneal scar. Ninety percent (90%) of the patients received standard medical treatment and three patients underwent penetrating keratoplasty. The mean uncorrected distance visual acuity at initial consult of eyes with corneal complication was 20/55 (LogMAR 0.43 ± 0.51) and 20/35 (LogMAR 0.25 ± 0.40) after treatment (p = 0.032). Conclusion Staphylococcal blepharitis was most prevalent among young female patients, and it affected both eyes. Almost all patients manifested the typical lid margin lesions. Nearly 60% of the patients presented with corneal complications and 22% had corneal lesions that were potentially blinding. Close to 50% had delay in treatment due to misdiagnosis.
blepharitis ; staphylococcus ; cornea ; blindness

Objective: To analyze the clinical characteristics of patients with Mucopolysaccharidosis ⅣA (MPS ⅣA). Methods: A retrospective study was conducted on 111 patients with MPS ⅣA in Xinhua Hospital of Shanghai Jiao Tong University School of Medcine from December 2008 to August 2020, confirmed by enzyme activity and genetic testing. General situation, clinical manifestations and enzyme activity test results were analyzed. According to the clinical manifestations, it can be divided into severe, intermediate and mild group. The independent sample t test was used to compare the birth body length and weight of children with that of normal boys and girls, and group comparisons of enzyme activities were evaluated by median test. Results: One hundred and eleven unrelated patients, 69 males and 42 females, were classified into 3 subtypes: severe (n=85), intermediate (n=14), and mild (n=12). The age at symptom onset were 1.6 (1.0, 3.0) years, and at diagnosis were 4.3 (2.8, 7.8) years. Skeletal manifestations were observed in all patients and consisted mainly of pectus carinatum (96/111, 86.5%), motor dysfunction (78/111, 70.3%), spinal deformity (71/111, 64.0%), growth retardation (64/111, 57.7%), joint laxity (63/111, 56.8%) and genu valgum (62/111, 55.9%). Eighty-eight patients (88/111, 79.3%) with MPS ⅣA were also along with non-skeletal manifestations, mainly including snoring (38/111, 34.2%), coarse faces (34/111, 30.6%), and visual impairment (26/111, 23.4%). The most common skeletal manifestation was pectus carinatum (79 cases), and non-skeletal manifestation was snoring (30 cases) and coarse faces (30 cases) in severe patients, pectus carinatum (13 cases) and snoring (5 cases) in intermediate type, motor dysfunction (11 cases) and snoring (3 cases) and visual impairment (3 cases) in mild patients. The height and weight of severe patients began to fall below -2 s at 2-<5 years and 5-<7 years, respectively. At the age of 10-<15 years, the standard deviation score of the height of severe patients reached (-6.2±1.6) s in males and (-6.4±1.2) s in females, and the score of weight got (-3.0±1.1) s in males and (-3.5±0.5) s in females. The height of intermediate patients began to fall below -2 s at the age of 7-<10 years, and the standard deviation score of height were -4.6 s and -3.6 s in 2 males, and -4.6 s and -3.8 s in 2 females at the age of 10-<15 years. The weight remained within -2 s in 72.0% (18/25) of intermediate patients compared to age-matched healthy children. In the mild patients with MPS ⅣA, the mean standard deviation score of height and weight was within -2 s. The enzyme activities of mild patients (2.02 (1.05, 8.20) nmol/(17 h·mg)) were both significantly higher than that of intermediate (0.57 (0.47, 0.94) nmol/(17 h·mg)) and severe (0.22 (0, 0.59) nmol/(17 h·mg)) patients (Z=9.91, 13.98, P=0.005, 0.001), and the enzyme activity of intermediate patients was significantly higher than that of severe patients (Z=8.56, P=0.010). Conclusions: The clinical manifestations of MPS ⅣA are charactered by pectus carinatum, motor function impairment, spinal deformity and growth retardation. The clinical characteristics, growth rate and enzyme activity differ among the 3 subtypes of MPS ⅣA.
Male ; Child ; Female ; Humans ; Adolescent ; Mucopolysaccharidosis IV ; Pectus Carinatum ; Retrospective Studies ; Snoring ; China ; Mucopolysaccharidoses ; Growth Disorders ; Vision Disorders

Central nervous system involvement in primary Sjögren's syndrome (pSS) is less common and usually presents as white matter lesions, neuromyelitis optica spectrum disorder (NMOSD), or transverse myelitis. NMOSD is an immune-mediated inflammatory demyelinating disease of the central nervous system with a high rate of relapse and significant disability. Studies have shown that patients with pSS combined with NMOSD have more severe symptoms and poorer prognosis. Here, we present a case of critical illness in pregnancy-associated NMOSD combined with Sjögren's syndrome. The patient was a 30-year-old pregnant woman with a history of Sjögren's syndrome who was diagnosed with NMOSD. She received combination therapy with steroids, intravenous immunoglobulin (IVIG), and hydroxychloroquine during pregnancy, resulting in partial resolution of numbness below the waist. However, due to irregular medication adherence outside the hospital setting, she developed weakness in her right lower limb accompanied by inability to move it, while her left lower limb still had some mobility but occasional numbness along with urinary and fecal incontinence. Ten days later, she was admitted to the emergency department where an emergency cesarean section was performed to deliver a healthy baby boy. However, her condition worsened postpartum as she developed high fever accompanied by bilateral lower limb paralysis and weakness along with loss of voluntary control over urination and defecation. The patient underwent ano-ther course of treatment consisting of steroids and IVIG; however there was limited improvement in symptoms observed after this intervention. Following administration of rituximab for the first time, the patient developed urinary tract infection which was successfully managed before continuing regular infusions. In later stages the patient could walk slightly with a limp and regained control over urination and defecation, allowing her to resume normal activities. This case suggests that combination therapy with steroids, IVIG, and hydroxychloroquine should be considered for the patients with pregnancy-associated NMOSD combined with Sjögren's syndrome. Rituximab can significantly improve symptoms such as postpartum paralysis in patients with NMOSD, however, there may be a risk of infection associated with its use.
Adult ; Female ; Humans ; Pregnancy ; Cesarean Section/adverse effects* ; Critical Illness ; Hydroxychloroquine/therapeutic use* ; Hypesthesia/complications* ; Immunoglobulins, Intravenous/therapeutic use* ; Inflammation/complications* ; Neuromyelitis Optica/diagnosis* ; Paralysis/complications* ; Pregnancy Complications/therapy* ; Rituximab/therapeutic use* ; Sjogren's Syndrome/complications* ; Steroids/therapeutic use* ; Vision Disorders

OBJECTIVES: To investigate the characteristics and objective assessment method of visual field defects caused by optic chiasm and its posterior visual pathway injury. METHODS: Typical cases of visual field defects caused by injuries to the optic chiasm, optic tracts, optic radiations, and visual cortex were selected. Visual field examinations, visual evoked potential (VEP) and multifocal visual evolved potential (mfVEP) measurements, craniocerebral CT/MRI, and retinal optical coherence tomography (OCT) were performed, respectively, and the aforementioned visual electrophysiological and neuroimaging indicators were analyzed comprehensively. RESULTS: The electrophysiological manifestations of visual field defects caused by optic chiasm injuries were bitemporal hemianopsia mfVEP abnormalities. The visual field defects caused by optic tract, optic radiation, and visual cortex injuries were all manifested homonymous hemianopsia mfVEP abnormalities contralateral to the lesion. Mild relative afferent pupil disorder (RAPD) and characteristic optic nerve atrophy were observed in hemianopsia patients with optic tract injuries, but not in patients with optic radiation or visual cortex injuries. Neuroimaging could provide morphological evidence of damages to the optic chiasm and its posterior visual pathway. CONCLUSIONS Visual field defects caused by optic chiasm, optic tract, optic radiation, and visual cortex injuries have their respective characteristics. The combined application of mfVEP and static visual field measurements, in combination with neuroimaging, can maximize the assessment of the location and degree of visual pathway damage, providing an effective scheme for the identification of such injuries.
Humans ; Optic Chiasm/pathology* ; Visual Pathways/pathology* ; Visual Fields ; Evoked Potentials, Visual ; Random Amplified Polymorphic DNA Technique ; Hemianopsia/complications* ; Vision Disorders/pathology* ; Optic Nerve Injuries/diagnostic imaging* ; Brain Injuries, Traumatic/diagnostic imaging*

OBJECTIVES: To study the virtual reality-pattern visual evoked potential (VR-PVEP) P100 waveform characteristics of monocular visual impairment with different impaired degrees under simultaneous binocular perception and monocular stimulations. METHODS: A total of 55 young volunteers with normal vision (using decimal recording method, far vision ≥0.8 and near vision ≥0.5) were selected to simulate three groups of monocular refractive visual impairment by interpolation method. The sum of near and far vision ≤0.2 was Group A, the severe visual impairment group; the sum of near and far vision <0.8 was Group B, the moderate visual impairment group; and the sum of near and far vision ≥0.8 was Group C, the mild visual impairment group. The volunteers' binocular normal visions were set as the control group. The VR-PVEP P100 peak times measured by simultaneous binocular perception and monocular stimulation were compared at four spatial frequencies 16×16, 24×24, 32×32 and 64×64. RESULTS: In Group A, the differences between P100 peak times of simulant visual impairment eyes and simultaneous binocular perception at 24×24, 32×32 and 64×64 spatial frequencies were statistically significant (P<0.05); and the P100 peak time of normal vision eyes at 64×64 spatial frequency was significantly different from the simulant visual impairment eyes (P<0.05). In Group B, the differences between P100 peak times of simulant visual impairment eyes and simultaneous binocular perception at 16×16, 24×24 and 64×64 spatial frequencies were statistically significant (P<0.05); and the P100 peak time of normal vision eyes at 64×64 spatial frequency was significantly different from the simulant visual impairment eyes (P<0.05). In Group C, there was no significant difference between P100 peak times of simulant visual impairment eyes and simultaneous binocular perception at all spatial frequencies (P>0.05). There was no significant difference in the P100 peak times measured at all spatial frequencies between simulant visual impairment eyes and simultaneous binocular perception in the control group (P>0.05). CONCLUSIONS VR-PVEP can be used for visual acuity evaluation of patients with severe and moderate monocular visual impairment, which can reflect the visual impairment degree caused by ametropia. VR-PVEP has application value in the objective evaluation of visual function and forensic clinical identification.
Humans ; Evoked Potentials, Visual ; Vision, Ocular ; Vision, Binocular/physiology* ; Vision Disorders/diagnosis* ; Virtual Reality