2015年 · 第51卷第02期

pigmentary dispersion syndrome (PDS) is characterized by depigmentation in the middle and peripheral part of the iris and pigmentation in the anterior part of the eye, including the trabeculae, which can lead to an increase in intraocular pressure and the appearance of pigmented glaucoma. The disease mostly occurs in young and middle-aged men, and is closely related to myopia. The incidence of PDS was 2.45% among white people^[1]Although the incidence rate has not been reported in China, it is not very rare clinically.

The patient was a 75-year-old male. On July 16, 2013, he went to the Department of Ophthalmology of Peking University People's Hospital for treatment due to intermittent eye swelling in his right eye for nearly 1 year and poor intraocular pressure control with two intraocular pressure lowering drugs. One year ago, the patient developed intermittent eye swelling in his right eye 1 month after cataract surgery, without nausea and vomiting. The ophthalmology examination of Peking University People's Hospital showed that the intraocular pressure of his right eye was 27 mmHg (1 mmHg =0.133 kPa), the cornea was clear, and there were a few pigmented retrocorneal deposits, but no abnormalities were found. Carteolol hydrochloride eye drops were given to the right eye twice a day, and tobramycin and dexamethasone eye drops (medication after cataract surgery) were stopped. After regular follow-up visits, the intraocular pressure fluctuated between 25 and 30 mmHg, so the patient was treated with selective laser trabeculoplasty in his right eye 10 months ago. After multiple follow-up visits, the intraocular pressure fluctuated between 25 and 40 mmHg, during which 2 or 3 kinds of intraocular pressure lowering drugs (carteolol hydrochloride eye drops, levobunolol hydrochloride eye drops, brinzolamide eye drops) were used. In 2002, the patient underwent YAG laser peripheral iridotomy due to angle-closure glaucoma in both eyes. No intraocular pressure lowering drugs were used in both eyes after operation, and the intraocular pressure was normal after regular reexamination. From December 2010 to May 2012, the intraocular pressure was 19-22 mmHg after multiple reexaminations. The cornea was clear, the anterior chamber was not shallow, the peripheral iris laser incision was unobstructed, the lens was NC2, the fundus optic papilla boundary was clear and positive after mydriasis, the optic cup was shallow, the cup-to-disc ratio was 0.5-0.6, the disc edge was normal, the retina was leopard-shaped, and the visual field examination showed no glaucoma-specific changes. On June 28 and July 12, 2012, phacoemulsification and IOL implantation were performed in the left and right eyes under local anesthesia (AcrySoft IQ IOL, 22.50 D and 22.00 D, respectively). The left eye went smoothly intraoperatively; After implantation of IOL in the right eye, the posterior capsule ruptured when viscoelastic was injected. Continuous annular capsulorhexis of the posterior capsule was performed. IOL was implanted in the ciliary sulcus. The operation was smooth, and no mydriatic drug was used after the operation. After operation, the left eye recovered smoothly, and the intraocular pressure was maintained at about 13 mmHg. The condition of the right eye is described above.

The patient was a 19-year-old female. On March 7, 2008, I came to the Department of Ophthalmology of Jinan Second People's Hospital for treatment due to the protrusion of my left eyeball since childhood, which gradually aggravated for 3 years. General examination: Scattered irregular coffee spot-like changes (>6 places) on the whole body skin with a diameter of 0.5~6.0 cm, and scoliosis deformity (Figure 1), a 1 cm ×1 cm size subcutaneous nodule was seen in the middle of both forearms, which was soft in texture, without tenderness, with clear boundaries and acceptable mobility; The anterolateral radiographs of the chest showed curvature and deformity of the spine, and the heart and lung were roughly normal; MRI scan of the brain showed no obvious abnormalities. Eye examination: visual acuity 1.0 in the right eye, no light sensation in the left eye; No abnormalities were found in the anterior segment and fundus of the right eye; Pulsatile exophthalmos in the left eye, obviously limited movement in all directions of the eyeball, no palpable mass in the eyelid, mixed congestion, corneal edema, several large blisters on the surface, expansion and thinning of the temporal sclera, and transparent pigment membrane above (Figure 2)。 Under the slit lamp microscope, the depth of the anterior chamber was moderate, brown-yellow hamartoma (Lisch nodule) was seen on the surface of the iris, the pigment collar of the iris was everted above the nose, the pupil was not round, and it was displaced to the upper temporal (Figure 3), the rest of the eyes are unclear. IOP: 15 mmHg (1 mmHg =0.133 kPa) in right eye, T +2 in left eye; B-ultrasound of left eye showed scleral staphyloma; Orbital CT scan showed that the left eyeball was about 2.9 cm ×4.4 cm in size, protruding, the left superior and lateral rectus muscles were thickened, the optic nerve was not abnormal, the left sphenoid large wing bone was missing, and the right eye was not abnormal (Figure 4)。 Admission diagnosis: neurofibromatosis type 1 (NF-1). On March 20, 2008, the contents of the left eye were removed under general anesthesia (the anterior sclera was incised 3 mm away from the limbus at 3 o'clock, and a circle was drawn with the diameter of the line connecting the limbus at 9 o'clock, and the corneal tissue and part of the sclera in this circular area were cut off). The tissue was removed and sent for pathological examination after operation. The wound healed well after surgery. Histopathological examination (Figure 5): Conventional paraffin section, HE staining, observation under light microscope, one side of anterior iris adhesion and iris atrophy were seen. On the other side, the pigment epithelium of the pupil margin was everted, the iris atrophy, and the pupil sphincter was cystic lesion, but no neurofibroma lesion was seen; Local chronic inflammation of the cornea, abnormal development of the chamber angle; Choroidal vasodilatation, hyperemia, atrophy of retinal ganglion cell layer and nerve fiber layer.

The patient was a female, 45 years old. On February 22, 2010, he came to Tianjin Medical University Eye Hospital for treatment due to swelling and tearing in his right eye for 3 years, aggravated eyelid swelling, ptosis, and headache on the right side for 4 months. MRI examination showed a tumor above the right orbit. After treatment with 40 mg of prednisone acetate, the appearance of both eyes was consistent. After the dose of prednisone acetate was reduced to 5 mg, the condition recurred, so he was admitted to the hospital with "right orbital mass". The patient denied previous history of ophthalmic disease, systemic disease, and family disease. Admission physical examination: general condition is good. The visual acuity was 0.8 in the right eye and 1.0 in the left eye. The upper eyelid of the right eye is swollen and slightly drooping, the eyelid margin is located above the pupil margin, and the lifting is slightly limited. The cornea was clear, the anterior chamber was clear, the lens was clear, and no obvious abnormalities were found in the fundus. The eyeball is prominent and displaced downward. Exophthalmia: 16 mm in the right eye, 14 mm in the left eye and 105 mm in the orbital distance. Eye movement: Upward rotation was limited, and no abnormalities were observed in the eye movement in all directions. IOP 15.6 mmHg. Palpation: Flat hard mass can be palpable at the upper orbital margin, the surface is not smooth, pushed immobile, orbital pressure (+ +), and no mass is palpable around the orbit. The anterior segment and fundus of the left eye were roughly normal. Cervical lymph nodes were unpalpable. B-mode ultrasound showed that the hypoechoic area above the right external orbit was flat, with clear boundaries and uneven internal echoes. Color Doppler flow imaging showed that the blood flow signal in the tumor was rich, with thick strip-like blood flow signal, staghorn-like bifurcation at the front end, and blue area in the red blood flow signal (Figure 1)。 Color Doppler power imaging showed more obvious bifurcation of blood flow. Orbital CT examination showed irregular high-density mass shadow in the upper part of the right external orbit, with clear and homogeneous boundary, close to the upper wall of the external orbit, and surrounding the upper eye of the external orbit, showing a casting shape, and the relationship with the lateral rectus muscle was not clearly distinguished. There was no obvious bone destruction in the orbital wall, and the inferior eye ball was displaced (Figure 2)。 Orbital MRI examination showed abnormal signal area above the right eyeball, the location, shape and extent were the same as those of CT; The signal intensity showed medium signal in T1WI, but still showed medium signal in T2WI, and the enhancement was not obvious. Clinical diagnosis: right intraorbital mass.

The child was a 3-year-old male. Due to the discovery of left orbital mass since childhood, there was no obvious change in the mass within 3 years, and he went to the Department of Ophthalmology of Peking University Third Hospital on June 2, 2012. The child had no other symptoms of general discomfort. Family history and genetic history are unknown. Ophthalmic examination: Binocular visual acuity examination did not cooperate, and no obvious abnormality was found in the right eye. The left eye is incomplete closure, the left upper eyelid is everted, the bulbar conjunctiva is obviously congested and edema, and an ellipsoidal mass is visible in the eye socket. The mass protrudes from the orbit, is tough to touch, has no adhesion to the surrounding tissues, and the structure of the eyeball is invisible (Figure 1)。 Orbital CT examination: Huge fat-containing space in the left orbit, and the left eyeball compression deformation became small (Figure 2)。 Orbital magnetic resonance imaging examination: lipid space-occupying lesions in the left orbit (Figure 3)。 Preliminary diagnosis: (1) left orbital mass (nature to be investigated); (2) Atrophy of the left eye?

The patient was a 45-year-old male. He went to Tianjin Eye Hospital because his right eye visual acuity decreased for 2 weeks. Eye examination: visual acuity was 0.9 in the right eye and 0.9 in the left eye; There was no obvious abnormality in the anterior segment examination of both eyes; For right fundus examination, seeQuintessence Picture 1In A, see B in Essence Picture 1 for OCT examination. Clinical diagnosis: congenital fovea of the right eye.

The patient was a 48-year-old male. He went to the Department of Ophthalmology of the People's Liberation Army General Hospital because of conscious decline of vision in his right eye. Eye examination: right eye visual acuity 0.7; No obvious abnormalities were observed in the anterior segment of the right eye; For right fundus examination, seeQuintessence Picture 1AndQuintessence Picture 2。 Clinical diagnosis: partial defect of the right eye papilla and choroid.

The patient was a 32-year-old male. Because his right eye had a sense of occlusion for several months, he went to the Department of Ophthalmology, the Second People's Hospital of Yunnan Province. Ophthalmic examination: visual acuity was 0.8 in the right eye and 1.0 in the left eye; The cornea of both eyes was transparent, the depth of the anterior chamber was normal, the pupil was equal size and round, and the direct and indirect response to light was normal. A calcified tumor was seen in front of the retina of the right eye, which was white, turbid and nodular, consisting of a large number of glittering calcium globules and appeared mulberry-like; No obvious abnormalities were observed in the optic papilla, macular area and blood vessels (Quintessence Picture 1)。 Clinical diagnosis: Retinal astrocytic hamartoma of the right eye.

The 20th National Symposium on Eye Trauma and the First Asia-Pacific Eye Trauma Conference were held in Shenzhen, Guangdong Province from October 30 to November 2, 2014. This conference was academically supported by the Ocular Trauma Group of Ophthalmology Branch of Chinese Medical Association and hosted by Shenzhen Eye Hospital. A total of 398 papers were submitted and more than 500 representatives attended. It was the largest grand event in all previous academic symposiums on ocular trauma. As the "First Asia-Pacific Eye Trauma Conference" held at the same time, not only many famous ophthalmologists from all over the country participated, but also invited experts and scholars from India, Singapore, Taiwan Province and Macao to give special lectures.