Combined Transcranial-supraorbital and Transconjunctival Approach for Optic Nerve Coloboma with Ophthalmic Dysplasia Associated with Rheumatoid Arthritis.

We report a 59-year-old woman with optic nerve coloboma and ophthalmic dysplasia associated with rheumatoid arthritis. She experienced progressive visual dysfunction over the course of several years and presented with headache and pain in the left eye. Since infancy the visual acuity of her left eye had been compromised and her eyesight worsened gradually until she was blind in the left eye. Macroscopic observation showed a reddish lesion on the sclera thought to be due to rheumatoid arthritis (RA). Magnetic resonance imaging and computed tomography disclosed a well-defined cystic lesion at the left retro-bulbar optic nerve within the optic nerve sheath. We selected the combined transcranial-supraorbital and transconjunctival approach to remove the eyeball after detaching the optic nerve. This technique was successful and the placement of an ocular prosthetic was cosmetically acceptable.

Frontalis suspension with an expanded polytetrafluoroethylene sheet for congenital ptosis repair.

Congenital ptosis with poor levator function is most often repaired with a frontalis suspension procedure. Autogenous fascia lata grafting is generally effective, with low rates of infection and granuloma formation. However, contraction of the grafted fascia lata may cause eyelash inversion, tarsal deformity, and/or lagophthalmos. Conversely, several synthetic suspensory materials have been used for frontalis suspension, among which polytetrafluoroethylene has been reported to be comparable to the fascia lata. However, in some studies using polytetrafluoroethylene strips or sutures, complications such as infection and granuloma formation were a significant problem. This study evaluated the outcomes of frontalis suspension with an expanded polytetrafluoroethylene sheet for congenital ptosis repair in 97 patients (130 eyelids). No ptosis recurrence was reported in an average follow-up of 31.6 months (range: 6-102 months). Six of the 130 eyelids (4.6%) had complications. Based on these results, frontalis suspension with an expanded polytetrafluoroethylene sheet for congenital ptosis repair can be considered safe and effective and be recommended for clinical use.

Adhesiotomy with grafting of fat and perifascial areolar tissue for adhesions of extraocular muscles after trauma or surgery.

PURPOSE: To investigate the effect of adhesiotomy with grafting of fat and perifascial areolar tissue (A-GFPAT) on eyes with restricted eye movements after trauma or surgery. DESIGN: Single-center retrospective interventional, consecutive case series. METHODS: Twenty-four eyes of 24 patients that underwent A-GFPAT were studied. The changes in the eye movements was evaluated by the Hess area ratio (HAR %) and in the binocular single vision (BSV) field scores. RESULTS: The HAR % was significantly improved by the A-GFPAT, from 50.9 ± 32.0% (±SD) to 66.4 ± 28.2% at the final visit (Student's t test; P < 0.01). Nine of the 24 cases (37.5%) had an improvement of the final HAR % by >10%. The preoperative mean BSV field score was 15.4 ± 13.3, which improved significantly to 25.9 ± 10.5 after the A-GFPAT (P < 0.001). Thirteen cases (56.5%) had an improvement of the final BSV score by more than 5 points. There were improvements of both HAR % (50%) and BSV score (54.5%) by more than 50% after A-GFPAT in patients with old orbital fractures. All patients who had been treated with Lactosorb earlier had an improvement of the BSV score, while those treated with endoscopic transmaxillary reduction and balloon technique had a lower chance of improvement (20%). CONCLUSIONS: Our new technique of A-GFPAT leads to improvements of eye movements that had been limited by the adhesion of orbital soft tissue and periorbita with less adverse effects. We recommend our surgical procedure for eyes with restrictive eye movements or pain caused by adhesions following trauma or ocular surgery.

Trochlea surgery for acquired Brown syndrome.

PURPOSE: To describe surgical treatment of acquired Brown syndrome by trochlea reconstruction and trochlea adhesiotomy. METHODS: The medical records of patients with acquired Brown syndrome who underwent intraoperative forced duction testing under direct view of the trochlea from 2010 to 2012 were retrospectively reviewed. In all cases, the site of restricted movement was identified, after which either the trochlea was reconstructed or the trochlear adhesion was removed. Surgical results were assessed by means of the pre- and postoperative Hess chart scores and binocular single vision test scores. RESULTS: Six eyes of 6 patients were included. In 4 patients the trochlea was reconstructed; in 2, the adhesion was removed. A significant improvement in the Hess chart scores was observed postoperatively in 5 of the 6 patients (P = 0.047). Binocular single vision test scores also significantly improved in 4 of 5 patients (P = 0.019). No iatrogenic superior oblique muscle paresis was observed in any of the patients. CONCLUSIONS: Our technique of isolating the source of limitation of elevation and treating with trochlea reconstruction or adhesion removal successfully treated acquired Brown syndrome in these patients.

[A case of atresia of the nasolacrimal passage with cleft lip and palate].

INTRODUCTION: Congenital nasolacrimal duct obstruction with facial malformation may accompany atresia of the nasolacrimal duct. We report a case of congenital bony obstruction of the nasolacrimal duct with cleft lip and palate. CASE: A 2-year-old boy with obstruction of both lacrimal system. Computed tomography (CT) showed lacrimal system aplasia on the right side, and bony obstruction of the nasolacrimal duct on the left side. The patient had a history of cleft lip and palate. We performed external dacryocystorhinostomy (EX-DCR) at 6 years of age because he developed dacryocystitis. We performed EX-DCR again at 8 years of age and endonasal DCR (EN-DCR) at 9 years of age due to recurrence. At the time of writing, 6 years after the last surgery, his postoperative course is excellent. CONCLUSIONS: Epiphora with cleft lip and palate may suggest the possibility of the atresia of the nasolacrimal duct. Congenital bony obstruction of the nasolacrimal duct with facial malformation may recur postoperatively after DCR due to nasal hypoplasia, which should be followed up for a long period.

A case of Muir-Torre syndrome with multiple cancers of bilateral eyelids and breast.

We report a case of Muir-Torre syndrome (MTS) with a very rare combination of cancers, involving bilateral eyelid cancers and breast cancer. A 71-year-old female with a history of breast cancer from 18 years prior presented with bilateral eyelid tumors. One of her siblings had lung cancer, and another had pancreatic cancer. She underwent excisional biopsy of the eyelid tumors and histopathology revealed sebaceous carcinoma of the right eyelid and basal cell carcinoma of the left. She was diagnosed with MTS: a skin cancer associated with visceral malignancy. Immunohistochemical tests for mutS homolog 2 showed a lack of expression in both eyelid carcinomas.

[Characterization and treatment of three cases with complications after frontalis suspension using autogenous fascia lata].

BACKGROUND: To report three cases of severe complications after frontalis suspension using autogenous fascia lata CASES: All three cases showed lagophthalmos and eyelash inversion after frontalis suspension using autogenous fascia lata. During surgery, we found that the tarsus was raised upwards and distorted by the fascia lata which had contracted and adhered to the surrounding tissue. We excised the adhesion of the fascia lata. In case 1, we transplanted the fat and perifascial areolar tissue under the cicatrized orbicularis. In cases 2 and 3, we flattened the distorted tarsus with nylon mattress sutures. In case 2, we added a frontalis suspension using Gore-Tex one year later. In case 3, we fixed Gore-Tex between the tarsus and the released fascia lata. CONCLUSIONS: Lagophthalmos and eyelash inversion due to contraction of the fascia lata may be complications of frontalis suspension using the fascia lata. Prolonged follow-up after the surgery is essential, and if such complications occur appropriate treatment is required.

Comparison of nylon monofilament suture and polytetrafluoroethylene sheet for frontalis suspension surgery in eyes with congenital ptosis.

PURPOSE: To compare nylon monofilament suture with polytetrafluoroethylene sheet for frontalis suspension surgery to treat eyes with congenital ptosis. DESIGN: Retrospective, nonrandomized, comparative, interventional case series. METHODS: We reviewed the medical records of 49 patients who had undergone 79 eyelid frontalis suspension surgeries to treat congenital ptosis. All of the patients were younger than 16 years and had congenital ptosis with poor levator muscle function. They were treated with frontalis suspension surgery with either a nylon suture or a polytetrafluoroethylene sheet and were followed up for at least 1 year. A single rhomboid loop sling was used for the nylon suture surgery. For the polytetrafluoroethylene sheet, an incision was made in the eyelid crease, and one end of the sheet was fixed to the tarsus and the other was fixed to the frontalis muscle. The main outcome measures were postoperative recurrences and complications. RESULTS: We evaluated 37 eyelids of 25 patients after nylon suture surgery and 42 eyelids of 31 patients after polytetrafluoroethylene sheet surgery. Among these, 9 eyelids of 7 patients were included in both groups. The median postoperative follow-up period was 32 months in both groups. The recurrence rates were 62.2% for the nylon suture group and 0% for the polytetrafluoroethylene sheet group (P < .001). The postoperative complication rates were 0% for the nylon suture group and 7.1% for the polytetrafluoroethylene sheet group (P > .05). CONCLUSIONS: Frontalis suspension using a polytetrafluoroethylene sheet with direct tarsus and frontalis muscle fixation is a reasonable technique with low rates of recurrences and complications.

Targeting CD9 produces stimulus-independent antiangiogenic effects predominantly in activated endothelial cells during angiogenesis: a novel antiangiogenic therapy.

The precise roles of tetraspanin CD9 are unclear. Here we show that CD9 plays a stimulus-independent role in angiogenesis and that inhibiting CD9 expression or function is a potential antiangiogenic therapy. Knocking down CD9 expression significantly inhibited in vitro endothelial cell migration and invasion induced by vascular endothelial growth factor (VEGF) or hepatocyte growth factor (HGF). Injecting CD9-specific small interfering RNA (siRNA-CD9) markedly inhibited HGF- or VEGF-induced subconjunctival angiogenesis in vivo. Both results revealed potent and stimulus-independent antiangiogenic effects of targeting CD9. Furthermore, intravitreous injections of siRNA-CD9 or anti-CD9 antibodies were therapeutically effective for laser-induced retinal and choroidal neovascularization in mice, a representative ocular angiogenic disease model. In terms of the mechanism, growth factor receptor and downstream signaling activation were not affected, whereas abnormal localization of integrins and membrane type-1 matrix metalloproteinase was observed during angiogenesis, by knocking down CD9 expression. Notably, knocking down CD9 expression did not induce death and mildly inhibited proliferation of quiescent endothelial cells under conditions without an angiogenic stimulus. Thus, CD9 does not directly affect growth factor-induced signal transduction, which is required in angiogenesis and normal vasculature, but is part of the angiogenesis machinery in endothelial cells during angiogenesis. In conclusion, targeting CD9 produced stimulus-independent antiangiogenic effects predominantly in activated endothelial cells during angiogenesis, and appears to be an effective and safe antiangiogenic approach. These results shed light on the biological roles of CD9 and may lead to novel antiangiogenic therapies.

Visual improvement following trans-Tenon's retrobulbar triamcinolone acetonide infusion for polypoidal choroidal vasculopathy.

PURPOSE: To report a case of polypoidal choroidal vasculopathy (PCV) that was successfully treated by sub-Tenon's infusion of triamcinolone acetonide (TA). METHODS: A 56-year-old Japanese man had PCV in his left eye with vision of 20/32. Fluorescein angiography demonstrated a hyper-fluorescent spot, and indocyanine green angiography showed marked leakage of dye from the polypoidal vessel. Optical coherence tomography showed anterior protrusion of highly reflective layers and subretinal fluid. RESULTS: The patient underwent trans-Tenon's retrobulbar infusion of 12 mg TA. Subsequently, the reddish-orange lesion decreased in size and elevation with complete resolution of the serous retinal detachment, and visual acuity improved to 20/20. Visual acuity remained good through a 15-month follow-up during which the patient was free of endophthalmitis and elevated intraocular pressure. CONCLUSIONS: Trans-Tenon's retrobulbar TA infusion is potentially effective in treating PCV.