2016年 · 第52卷第12期

This issue focuses on the clinical and basic research of neuroophthalmology. The responsible editorial committee responsible for the planning, composition and review of this issue is Professor Zhong Yong.

traumatic optic neuropathy (TON) is a common type of ocular trauma in clinical practice. It mainly refers to optic neuropathy caused by sudden blunt external force in the frontal and temporal regions above the orbit^[1,2]。 Its incidence rate accounts for 0.5% ~5.0% of closed craniocerebral trauma, and more than 90% of them are indirect injury of optic nerve in the intracanal segment. With the rapid development of transportation, tourism, factory and mining industries, the incidence of TON is increasing. Among the causes of TON, car accident injury occupied the first place (50% ~65%), followed by fall injury and fall injury (13% ~28%)^[3,4]。 The most common injury sites were the lateral and temporal parts of the eyebrow arch, followed by the periorbital and cranial areas. The consequences of TON are more serious, 68% ~78% of patients have no light sensation after injury^[2,3,4]。 Therefore, aggressive treatment should be given as early as possible to save visual function.

Glucocorticoid (hereinafter referred to as hormone) ophthalmic preparations are currently one of the most commonly used and effective drugs for the treatment of ocular inflammatory reaction. In corneal and ocular surface inflammatory reaction diseases, rational application of hormonal ophthalmic preparations can quickly and effectively control the inflammatory reaction and disease. However, irregular use may lead to poor curative effect and even serious side effects, such as prolonging the disease and aggravating corneal infection. It is emphasized that ophthalmologists should master the indications and usage of hormonal ophthalmic preparations, which has important clinical significance for the treatment of corneal and ocular surface diseases. Therefore, the Keratology Group of Ophthalmology Branch of Chinese Medical Association discussed and formed a consensus on the application of hormone ophthalmic preparations in corneal and ocular surface diseases, which provided a reference for clinical ophthalmologists to treat related diseases.

Example 1The child was a 7-year-old female. Due to the loss of vision in his left eye for nearly 2 years and exophthalmos for more than 3 months, he went to the Department of Ophthalmology of the Second Hospital of Tianjin Medical University on May 15, 2008. The child's previous systemic condition was acceptable, the development was normal, the nutritional status was acceptable, there were no obvious abnormalities in both eyes, and there was no history of orbital tumor in the family. Ophthalmological examination: the visual acuity of the right eye was 1.0, and no obvious abnormalities were found in the anterior and posterior segments; The visual acuity of the left eye was 0.1, the exophthalmos was about 1 mm, the orbital pressure was +2, and the papilla was edema. The rest of the examinations were normal. Orbital MRI showed that the anterior 2/3 of the optic nerve in the left eye was swollen, with a size of about 12 mm ×11 mm ×19 mm, T₁WI showed medium signal (Figure 1a), T₂WI is high signal. Diagnosis of left eye optic nerve glioma, to transcranial orbital resection of the tumor, later transferred to our hospital, gamma knife treatment. Four months after treatment, pain in the orbital area, visual acuity decreased to 0.01, exophthalmos significantly increased, and eyelid edema (Figure 1b), MRI showed that the tumor was larger than before treatment, about 16 mm ×16 mm ×22 mm in size (Figure 1c), intravenous drip of hypertonic fluid, oral administration of prednisone, orbital pain gradually subsided. MRI revealed tumor shrinkage after 1 year. After 3 years of ophthalmic examination, the visual acuity of the left eye was 0.02, the eyeball was 1 mm protruding from the contralateral side (Figure D), the orbital pressure was +1, the eyeball movement in all directions was unrestricted, the edge of the optic papilla was clear and light, and the central retinal artery and vein were thin (Figure 1e), MRI suggested that the tumor had shrunk more recently. MRI after 7 years showed that the tumor was significantly smaller than before treatment (Figure 1f）。

The child was a 9-year-old male. Due to the ptosis of his right eye for 1 week, he came to the ophthalmology department of Cangzhou Central Hospital in December 2012. Ophthalmic examination after admission: the visual acuity of the right eye was 1.0, the exophthalmos was 17 mm, the upper eyelid margin covered 1/2 of the pupil, the ptosis was obviously light in the morning and heavy in the evening, and the ptosis was obviously improved after rest. The visual acuity of the left eye was 1.0, the exophthalmos was 22 mm, the upper eyelid was retracted and delayed, and the position of the upper eyelid of the left eye did not change in morning light and evening weight. The corneas of both eyes are transparent, the pupils are round, about 2.5 mm in diameter, there is light reflection, the fundus examination is normal, and the intraocular pressure is normal. Eye movement is unrestricted. No obvious extraocular muscle abnormalities were found on orbital CT. Positive test for neostigmine. Whole body examination: the white blood cell count in routine blood examination was 11.85×10⁹/L. Chest CT showed right middle lobe pneumonia; The thymus gland is slightly full, and there is no exact abnormality in density. CT of the head showed no obvious abnormalities. The electrocardiogram showed sinus tachycardia, 121 beats/min, and no abnormalities in myocardial enzyme spectrum were found. Follow-up history The child had a history of fever, mild cough, no phlegm, asthma and chest pain 5 days before admission, and a history of irritability, hyperhidrosis, polyphagia, etc. Therefore, the thyroid function was checked. Thyroid stimulating hormone receptor antibody: 6.04 IU/L, free triiodothyronine: 10.26 ng/L, free thyroxine: 3.87 mg/L, thyroid stimulating hormone: 0.005 IU/L, serum total triiodothyronine: 297.2 mg/L, serum total thyroxine: 19.15 g/L, thyroid color ultrasound return: thyroid volume increased. Physical examination: Second degree thyroid enlargement, soft texture, heart rate 110 beats/min, uniform rhythm, left eye exophthalmia positive. Endocrinology consultation, Graves' disease and thyroid associated ophthalmopathy (TAO) were diagnosed clearly, and methimazole tablets and atenolol oral administration were recommended. Pediatric consultation, mycoplasma pneumonia is considered, and azithromycin is recommended for anti-inflammation. There were no abnormalities in other physical examinations of the whole body, and there were no manifestations of myasthenia gravis. Therefore, the diagnosis of ocular myasthenia gravis (OMG), Graves disease, TAO and mycoplasma pneumonia in the child was clear. Treatment with pyridostigmine bromide, prednisolone, methimazole tablets, atenolol and azithromycin was observed after 2 weeks. The height of right eyelid fissure was 10 mm and the height of left eyelid fissure was 12 mm at the time of discharge. After discharge, oral prednisone tablets, pyridostigmine bromide and methimazole were continued. After using prednisone tablets and pyridostigmine bromide for 25 days, the right eyelid fissure was stable in height, which was 10 mm, and there was no change in morning light and evening weight. Thyroid function was checked after 3 months with methylmercaptoimidazole: free triiodothyronine: 5.42 ng/L, free thyroxine: 1.25 mg/L, thyroid stimulating hormone: 0.585 IU/L, thyroid stimulating hormone receptor antibody 5.50 IU/L, and thyroid function was checked after 5 months: free triiodothyronine: 4.39 ng/L, free thyroxine: 1.02 mg/L, thyroid stimulating hormone: 1.52 IU/L. Up to the follow-up date of June 6, 2015, the ocular condition was good, myasthenia gravis did not convert to generalized form, and the thyroid function was normal.

Crouzon syndrome, also known as hereditary familial craniofacial hypoplasia, is a rare genetic disease named after the first report by French physician Crouzon in 1912^[1]。 The clinical features of the disease include craniosynostosis, mid-facial dysplasia and exophthalmos. Most ophthalmologists have insufficient understanding of this disease, which is easily neglected in clinical diagnosis and treatment. The author encountered a family with Crouzon syndrome in 2014, which is now reported as follows, and the relevant literature is reviewed to raise awareness and avoid missed diagnosis and misdiagnosis.

The patient was a 42-year-old female. Because the visual acuity of his left eye suddenly decreased for 16 h, he was seen in the Department of Ophthalmology, Affiliated Hospital of Guangdong Medical University. Systemic examination: mild elevation of blood triglycerides, mild fatty liver. Eye examination: the visual acuity of the right eye is 1.5, and the left eye is manual before the eye; IOP was 12 mmHg (1 mmHg =0.133 kPa) in the right eye and 13 mmHg in the left eye; The cornea of both eyes is transparent, the aqueous humor is clear, and the iris texture is clear. The pupil diameter of the right eye is 3 mm, and the direct response to light is normal, while the pupil diameter of the left eye is 4 mm, and the direct response to light is slow. Right fundus normal; For the left fundus, seeQuintessence Picture 1。 FFA examination: the boundary of the optic papilla is unclear, the retina is scattered with bleeding to block the fluorescence, the retinal artery is full, the venous return is slow, the temporal retina is a large capillary without perfusion area, the posterior pole capillary is dilated, and there are small non-perfusion areas in between, and the late fluorescein leakage is obvious. OCT examination is presented inQuintessence Picture 2。 Clinical diagnosis: retinal artery occlusion combined with venous occlusion in left eye.

Pupillary examination is an important item in ophthalmology and neurology. This examination has the advantages of being convenient, non-invasive and not relying on expensive equipment. Because the size and activity of the pupil are controlled by the autonomic nervous system, pupil examination can reflect visual function information that patients cannot subjectively control. In some ophthalmic diseases, pupil changes may be the only positive sign, and pupil examination is an important objective basis for evaluating related diseases. In addition, pupil examination requires low cooperation with patients, and pupil examination is still feasible for infants, young children, the elderly and seriously ill patients. Mastering the correct method of pupil examination is of great significance to the clinical diagnosis of ophthalmic diseases.