Jianhua Yan

Retinoblastoma is a curable intraocular malignancy in children. However, in clinical practice, retinoblastoma can sometimes be misdiagnosed and mismanaged, leading to extraocular extension and even death. In this report, a series of 3 cases are related that emphasize the conditions and consequences resulting from misdiagnosis and mismanagement of retinoblastoma. The clinical features, imaging findings, histopatholigical examination, and management in 3 case reports of children with misdiagnosed retinoblastoma are presented. Two of the cases received pars plana vitrectomy after being misdiagnosed with Coats disease or ocular blunt trauma, whereas the third case received evisceration after being misdiagnosed with suppurative endophthalmitis. When the diagnosis of retinoblastoma had been confirmed after a second surgery was performed in our hospital, only 2 of the cases received adjuvant orbital radiotherapy. All 3 cases died of systemic tumor metastases. Intraocular surgical procedures should be avoided in any equivocal case until the possibility of latent retinoblastoma is eliminated.We strongly recommend that early enucleation be executed as soon as possible followed by postoperative adjuvant therapy under conditions wherein an intraocular surgery was inadvertently performed in an eye with retinoblastoma.

To present rare benign orbital tumors with bone destruction in children who could not be diagnosed pre-surgically and may simulate malignant ones. A retrospective review of cases. Clinical, operative and pathological records in all children with a diagnosis of benign orbital tumors who showed remarkable bone destruction at a tertiary Ophthalmic Center in China between Jan 1, 2000 and Dec 31, 2009 were reviewed. All patients had definitive histopathologic diagnosis. Eight patients with benign orbital tumors showed obvious bone destruction, including six cases of eosinophilic granuloma, one case of leiomyoma and one case of primary orbital intraosseous hemangioma. Among them, three patients were females and five patients were males. Tumors were unilateral in all cases, with both the right and left side affected equally. Age ranged from 3 to 7 years (mean 4.1 years). Symptom duration ranged from 1 to 5 weeks (mean 4.8 weeks). Eyelid swelling and palpable mass were the most common complaint. There was no evidence for multifocal involvement in cases with eosinophilic granuloma. Among six patients with eosinophilic granuloma, two were treated with low dose radiation (10 Gy), three received systemic corticosteroid and one was periodically observed only after incisional biopsy or subtotal curettage. There was no postoperative therapeutic intervention in the two patients with leiomyoma and intraosseous hemangioma. All eight patients regained normal vision without local recurrence after a mean follow-up time of 32.8 months. Benign orbital tumors such as isolated eosinophilic granuloma, leiomyoma and primary orbital intraosseous hemangioma may show remarkable bone destruction.

Objective To determine the primary sites, clinical features, treatment, and outcome of 46 patients with cancer metastatic to the orbits in southern China. Methods Retrospective review of medical records at a tertiary center in southern China between Jan 1, 1991 and Dec 31, 2008. Results The study included 35 males and 11 females, with an age range of 3–78 years (average 47 years). All of the patients came from southern China. The left orbit was affected more frequently than the right orbit (3:2), and no cases were bilaterally involved. The most common primary cancer that metastasizes to the orbit is nasopharyngeal carcinoma (30.34%), followed by lung cancer (8.70%), liver cancer (6.52%), breast carcinoma (4.35%), renal cell cancer (4.35%), medullary thyroid carcinoma (2.17%), lacrimal gland carcinoma (2.17%), stomach carcinoma (2.17%), mediastinum carcinoma (2.17%), other forms (6.52%), and unknown carcinomas (30.34%). Orbital metastasis was the first sign of systemic cancer in 24 patients (52.17%). The five most common clinical findings at the initial examination were abnormal eye position (65.22%), proptosis (63.04%), increased orbital pressure (54.35%), diplopia (54.35%), and limited ocular motility (52.17%), followed by swollen eyelid (50%), visible mass or swelling (46%), blurred vision (41%), conjunctival hyperemia, and edema (35%), pain (28%), blepharoptosis (20%), papilledema (17%), and vasodilatation of the retina (7%). Primary treatment included surgical excision, radiotherapy, systemic chemotherapy, and observation. Among the patients (78% of 46) with sufficient follow-up time (mean 20.7 months), 30.56% of them died of disseminated metastasis. Twenty-five percent of them were in remission with no active metastases and 27.78% of them were living with active metastases. Overall, the mean survival time was 18 months (range, 5–38 months) after diagnosis of orbital metastasis. Conclusions The most common primary cancer that metastasizes to the orbit is nasopharyngeal carcinoma (30.34%), followed by lung cancer (8.70%) and liver cancer (6.52%) in southern China. Orbital metastasis can display a variety of clinical features and should be considered in patients with known systemic cancer. The systemic prognosis is generally poor.

To analyze the clinical features and therapeutic outcome in patients with strabismus due to orbitotomy. Retrospective case series. Nine cases of strabismus following orbital operation seen between March 1998 and July 2003 in Zhongshan Ophthalmic Center, Sun Yat-sen University were reviewed retrospectively. All of the 9 cases were treated by strabismus surgery. Among them, 5 cases were male and 4 were female, with their age ranging from 12 to 40 years (average 26.4 years). The right eye was involved in 4 cases and the left one in 5 cases. Most of them belonged to restrictive strabismus secondary to the formation of surgical scar, accounting for 66.7% of them. The methods of surgery included the excision of fibrous tissue around the involved extraocular muscle, the routine recession and resection and the Jensen procedure, combined with the fixation suture. Six cases obtained excellent results. Two cases were slightly undercorrected and one case was remarkably undercorrected. Of the 4 cases with diplopia before operation, the diplopia disappeared in 2 cases after surgery. The other 2 cases had diplopia only in one direction far away from the primary position. All of the ocular movement improved obviously after strabismus surgery. Most of the deviation happened after orbitotomy was restrictive strabismus caused by the muscle fibrosis and scar formation around the muscle. Excision of fibrotic tissue, combined with the routine recession and resection, Jensen procedure, and the fixation suture can have quite satisfactory results.

To investigate the inhibition of proliferation of retinoblastoma cells (SO-Rb50) and the differentiation of tumor cells into normal cell types by all trans retinoic acid (ATRA). This was an experimental study. SO-Rb 50 cells were treated with ATRA at different concentrations and growth curves were plotted. IC50 was analyzed by MTI method. Cell cycles before and after drug treatment were analyzed using flow cytometry. Morphologic characters of living cells and hematoxylin and eosin (HE) stained cells were observed microscopically before and 20 days after drug treatment The cell markers of NSE, vimentin, GFAP were detected with immunohistochemical method before and 20 days after drug treatment. Cell growth was inhibited by ATRA treatment dose-dependently from 0 to 1 x 10(-5) mol/L and the cell viability was decreased. The IC50 of ATRA measured by MTT was approximately 14.05 microm/L. Treated with 1 x 10(-5) mol/L ATRA, the G0/G1 stage cells increased from 56.5% before treatment to 66.6%-81.0% after treatment, whilst S stage cells decreased from 33.1% to 22.3%-15.9%. Cells could attach to poly-lysine coated cover slides and formed small colonies. Some cells changed from round in shape to oval or fusiform shapes. The ratio of nuclear to cytoplasm decreased after the treatment of ATRA. Some cells extended long or short processes 20 days after treatment. Immunohistochemical studies showed that cells were stained moderately positive, strongly positive and weakly positive by NSE, vimentin and GFAP antibodies, respectively before treatment. After the ATRA treatment, cells were stained strongly positive, strongly positive and moderately positive by NSE, vimentin and GFAP antibodies, respectively. ATRA significantly inhibits the proliferation of SO-Rb 50 cells and cells arrested at G0/G1 stage of cell cycle. ATRA induces differentiation of retinoblastoma cells towards the neurons and gliocytes.

To investigate the dose-effect relationship of strabismus surgery in patients with thyroid associated ophthalmopathy (TAO). Thirty cases (32 eyes) with TAO treated with extraocular muscle surgery by the author seen between Jan 1999 and March 2006 in Zhongshan Ophthalmic Center, Sun Yat-sen University, China were reviewed retrospectively. Out of the 30 cases (32 eyes), 19 cases were male and 11 were female, with their age ranging from 22 to 68 (mean 49.4 years). The right eye was involved in 11 cases and the left one in 21 cases. The extraocular muscle surgery included recession of inferior rectus muscle (10 muscles), recession of superior rectus muscle (10 muscles), recession of medial rectus muscle (6 muscles), recession of inferior rectus muscle combined with medial rectus muscle (2 eyes), recession of superior rectus muscle combined with medial rectus muscle (1 eye), tenotomy of superior rectus muscle (3 muscles). Thirty cases obtained excellent results, and the rate of ocular alignment was 93.75%. By calculating the dose-effect on 28 successfully corrected (<or= +/- 5 degrees) cases who were undergone recession, we found out that the deviation of (3.93 +/- 1.67) degrees on average can be corrected by one millimeter recession of rectus muscle surgery. The corrected amount in superior rectus muscle was minimum, which was (2.63 +/- 0.83) degrees /mm, and the maximum was in medial rectus muscle, which was (5.33 +/- 1.46) degrees /mm; the corrected amount in inferior rectus muscle was (4.11 +/- 1.55) degrees /mm. If the preoperative deviation was equal to or less than 15 degrees, the corrected amount on average was (2.30 +/- 1.09) degrees /mm; if the preoperative deviation was in the range of 16 degrees to 30 degrees , the corrected amount on average was (3.56 +/- 0.79) degrees /mm, and if the preoperative deviation was more than 30 degrees, the corrected amount on average was (6.02 +/- 1.01) degrees /mm. The patients with strabismus in TAO whose corrected amount of deviation by one millimeter recession of rectus muscle on average was greater than that of patients with other kinds of strabismus. The larger the deviation, the more the corrected amount of one millimeter recession of rectus muscle. It was difficult to estimate the surgical effect on patients with strabismus in TAO before surgery.

Purpose: To report an unusual case of cavernous hemangioma with rapidly developing proptosis. Methods: Case report. The clinical features, imaging findings and orbital surgery were presented. Results: A 54-year-old man presented with rapidly developing proptosis and a mass in the lower eyelid of his left eye for 8 h. The left eye had 5 mm of proptosis. Color Doppler ultrasonography and computed tomography disclosed a well-defined soft tissue mass in the left anterior orbit, which extended along the floor of the orbit and sized 1.7 × 2.2 cm without bone destruction. An anterior orbitotomy was performed. At surgery a red-purple, well-defined and cone-shaped mass and a hematoma, which was found on the lateral part of the mass, were removed. The histopathological evaluation of the mass revealed cavernous hemangioma. Conclusions: Patient with a rapidly developing proptosis might have the possibility of orbital cavernous hemangioma.

Background Strabismus with large deviation in Graves’ ophthalmopathy is relatively common in developing countries, such as China. However, little information is available in the literature on this condition. We report here our surgical results of strabismus with large deviation in Graves’ ophthalmopathy. Patients and methods The surgical management of strabismus in 27 patients with Graves’ ophthalmopathy with large angle (≥25°) were retrospectively analyzed. The strabismus surgery included recession or free tenotomy of involved rectus muscle, recession of rectus plus resection of ipsilateral antagonist, and recession of rectus plus recession of contralateral antagonist. The patients were followed up for an average of 1.2 years. Results Among 27 patients, 17 were male and 10 were female, with their age ranging from 28 to 68 years old (mean: 51.9 years). There were 12 cases with excellent results, 10 with good results, 3 with fair results, and 2 with poor results. Only one surgery was required in all cases except three who needed re-operation. Based on the rectus muscles involved, just one rectus muscle recession combined with traction suture could correct the strabismus with large angle in 20 patients; two muscles were required in 9 patients and three in 1 patient. Four patients had surgical overcorrection of strabismus. Eight patients had complicated eyelid retraction postoperatively. Conclusions The surgical management of strabismus with large deviation in patients with Graves’ ophthalmopathy was remarkably good. Just one rectus muscle recession combined with traction suture could correct the large angle strabismus in 74% (20/27) of cases. The choice of surgical procedure and some special attention in performing surgery are discussed.

It is well known that mast cells (MC) are involved in fibrosis and many forms of chronic inflammation. Chronic inflammatory cells infiltration and fibrosis in various orbital tissues are the main histopathologic features in patients with idiopathic orbital inflammatory pseudotumor (IOIP). Whether MC is involved in the course of chronic inflammatory conditions of IOIP is not yet clear. We sought to investigate the distribution of MCs in samples of IOIP and to explore the possible role of MC in the course of its pathegenesis. Immunohistochemistry with tryptase monoclonal antibody (a specific mast-cell surface marker) was used in 53 different subtypes of IOIP specimens including 19 of the lymphocyte infiltrative type, 22 of the fibrotic type, 12 of the mixed type and 4 specimens of normal orbital tissue. The number of positive stained MC was counted by light microscopy. The differences of the number of MC between various subtypes of IOIP were analyzed. The average number of positive stained MC in the normal control group was 33.33 ± 4.72 /mm³, whereas the average numbers of positive stained MC in the lymphocyte infiltrative subtype group, the mixed subtype group and the fibrotic subtype group were 306.35 ± 55.81 /mm³, 662.93 ± 115.28 /mm³ and 813.44 ± 146.56 /mm³, respectively. Compared with the normal control, the number of MC increased significantly in all three subtypes of IOIP samples (P < 0.01). The number of MC in fibrotic subtype IOIP was the largest, followed by mixed subtype IOIP, and the lymphocyte infiltrative subtype IOIP (P < 0.05). The MC were distributed mainly around small vessels and in collagen fibers. Mast cells may play an underappreciated role in the fibrosis of IOIP.

Purpose: To report a rare case of linear nevus sebaceous syndrome in a little Chinese girl with a new ocular finding. Methods: Case report. The clinical manifestations, ophthalmic features and central nervous system abnormalities associated with this neurocutaneous syndrome are presented. Results: A little girl, aged 1 year and 8 months, had several linear or oval nevi on her forehead and parietal scalp. Three round well-defined subcutaneous masses, approximately 1 cm in size, were present on the occipital, parietofrontal and right mastoid region, respectively. A round choroidal hemangioma, approximately 4-5 PD in diameter, was found in the superotemporal portion of the right fundus without retinal detachment. A round mass, 8 × 8 mm in size and tough in consistency, was located in the left upper eyelid, together with a 1/2 coloboma of the middle upper eyelid. Extensive choristomas of the superior bulbar conjunctiva and nearly the whole cornea were found in the left eye. CT-scans of both the brain and orbit disclosed left cerebral hypoplasia, dysplasia of sphenoid bone and temporal bone, and multiple calcifications of posterior sclera of both the right and the left eye. The mass on the left upper eyelid was removed and pathological examination confirmed the diagnosis of eyelid choristoma. Conclusions: Patients with linear nevus sebaceous syndrome may have choroidal hemangioma.