ObjectiveTo observe the clinical characteristics and optical coherence tomography (OCT) features of pseudopapilledema (PPE) combined with peripapillary hyper-reflective ovoid mass-like structures (PHOMS) in children. MethodsA retrospective observational study. From October 2019 to May 2021, total 22 eyes from 12 children diagnosed as PPE combined with PHOMS in the Neuro-ophthalmology Department of The First Hospital of Xi’an (Affiliated of The First Hospital of Northwest University) were recruited. Among the children, 6 were male and 6 were female. The average age was (10.6±2.7) years. The average course from disease onset to diagnosis of PPE combined with PHOMS was (8.0±7.5) months. All patients underwent best corrected visual acuity (BCVA), relative afferent papillary defect (RAPD), Ishihara's test, fundus photography, OCT, fundus autofluorescence (FAF), ocular B-mode ultrasound, visual field and patternvisual evoked potential (P-VEP). The clinical and OCT characteristics of the patients were observed. ResultsThe anterior segments of the patients were normal. The intraocular pressures and Ishihara's test were all normal. All RAPD were negative. Total 22 eyes, BCVA was 1.0 in 21 eyes and one eye was 0.12. The fundus photography revealed blurred optic discs margin, showing mild to moderate edema-like elevation with more prominent in the nasal parts, presenting as a “C” shape halo. No obvious abnormal fluorescence was observed in FAF. The OCT scan of involvement eyes showed an elevated appearance in vary degrees, and the sharply marginated ovoid hyper-reflective mass-like structures which laterally herniated into the peripapillary region under retinal nerve fiber layer and above the Bruch membrane were detected with consecutive nasal enlargement scanning, corresponding to the nasal parts in the fundus photography. The higher degree of elevation, the larger the volume. Macular retina pigment epithelium layer and ganglion cell thickness were normal. Ocular B-mode ultrasound showed that the head of the optic nerve in the posterior wall of the eyeball (in front of the optic disc) was elevated in all affected eyes, and there was no strong signal echo in it. Visual field examination showed physical blind spot enlargement in 3 eyes and visual field defect in 2 eyes. P-VEP examination showed that the peak was slightly delayed in 3 eyes and the amplitude was slightly reduced in 3 eyes. ConclusionsEnlarged nasal optic disc OCT scan can improve the detection rate of PHOMS. PHOMS were detected bilaterally in the cases with binocular PPE while only in the effected eye in the cases of monocular PPE; the higher degree of PPE, the lager volume of PHOMS. PHOMS were could contribute to the diagnosis of PPE in children.
Idiopathic intracranial hypertension (IIH) is a neurological disease, characterized by increased intracranial pressure and papilledema, and often associated with headache, transient loss of vision and pulsatile tinnitus. IIH typically occurs in women of childbearing age. Over 90.0% of patients are with obesity or over weighted. Loss of sensory visual function is the major morbidity associated with IIH and some patients even develop into blindness. Most patients will have varied degrees of visual impairment, or even a few become blind. Frisén grading system, visual field examination and spectral-domain optical coherence tomography can be used to evaluate and monitor the IIH papilledema functionally and morphologically. In recent years, IIH treatment trials in other countries confirmed that, weight loss and low-salt diet combined with acetazolamide treatment has a clear improvement for IIH patients with mild visual impairment. In-depth understanding of the etiology, clinical manifestations, diagnostic criteria and the main treatment has important clinical significance for IIH patients
ObjectiveTo observe the clinical and imaging features of infiltrative optic neuropathy (ION) secondary to extraocular malignant tumors. MethodsA retrospective case study. From January 2017 to October 2022, 26 eyes of 20 patients with ION secondary to extraocular malignancies and 32 eyes of 16 patients with early papilloedema (EP) secondary to intracranial metastatic carcinoma were included in the study. All eyes underwent best corrected visual acuity (BCVA), fundus color photography, orbital and/or craniocerebral magnetic resonance imaging (MRI). A total of 54 eyes were examined by visual field examination, among which ION and EP were 22 and 32 eyes, respectively. Clinical and imaging features of the affected eye were retrospectively analyzed. ResultsAmong 26 eyes of 20 ION patients, there were 13 males and 7 females, with the mean age of (52.8±16.9) years. There were 10 patients of hematologic malignancy, 7 patients of periorbital malignancy, 2 patients of lung cancer, 1 patient of gastric cancer, 1 patient of breast cancer and 1 patient of prostate cancer. Two patients of nasal lymphoma were recorded as hematologic malignancies and periorbital malignancies. Sixteen patients had a history of systemic or periorbital malignancy, among which 4 patients reported that they had been "clinically cured". Optic neuritis was diagnosed in 15 patients. Among the 16 patients with EP, 5 were males and 11 were females, with the mean age of (47.9±12.3) years. The primary malignant tumors were lung cancer, breast cancer, leukemia, gastric cancer, ovarian cancer, colon cancer and rectal cancer in 7, 2, 2, 2, 1, 1, 1, respectively. In 26 eyes of ION, 20 eyes complained of blurred vision or peripheral vision occlusion and progressive aggravation; no obvious visual symptoms in 6 eyes. BCVA was light sensing to 1.0 with a median of 0.3, including light sensing and light sensing in 4 eyes. Optic disc edema was observed in 19 eyes; no obvious abnormality in 7 eyes. Visual field examination showed that in 22 eyes, normal or mild enlargement of blind spot in 3 eyes, arcuate scotoma in 4 eyes, annular scotoma in 6 eyes, tubular visual field or concentric contraction of visual field in 6 eyes, and diffuse depression in 3 eyes. MRI showed optic nerve enlargement with sheath enhancement in all ION eyes. Among 32 eyes of EP, 28 eyes showed recurrent transient amaurosis, and the other 4 eyes showed horizontal diplopia. BCVA ranged from 0.8 to 1.5, with a median of 1.0. All EP patients showed different degrees of optic disc hyperemia and edema by fundus examination. The visual field examination showed normal or mild enlargement of the physiological blind spot. MRI showed thickening of the optic nerve and widening of the intrathecal space, but no obvious enhancement of the optic nerve and its intrathecal membrane, and obviously enhanced space-occupying lesions in the brain parenchyma, accompanied by compression and edema of the surrounding brain tissue and midline displacement. ConclusionsION secondary to extrocular malignant tumors mainly manifested as mild visual symptoms and obvious optic disc edema. MRI showed thickened optic nerve and strengthened sheath, and no obvious abnormality in optic nerve parenchyma.