Ioannis Mavrikakis

The incidence of irreversible retinal toxicity in patients treated with hydroxychloroquine a reappraisal

PURPOSE To define the risk of hydroxychloroquine (HCQ)-related retinal toxicity in patients with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) who are receiving recommended dosages of the drug (< or =6.5 mg/kg/day). DESIGN Prospective cohort study, from 1985 to 2000. PARTICIPANTS Greek patients with RA (n = 335) and SLE (n = 191) treated with HCQ, 400 of whom had completed at least 6 years of treatment. METHODS Ophthalmologic evaluation was performed every 6 months from 1985 to 1995, and yearly thereafter. This consisted of best-corrected visual acuity, color vision testing, static central visual field testing, fundoscopy, electroretinography, and fluorescein angiography, when indicated. MAIN OUTCOME MEASURES Fundus lesions attributed to HCQ. RESULTS No HCQ retinal toxicity was noted in any of the 526 patients during the first 6 years of treatment. Two (3.4%) of the first 58 long-term (>6 years) treated patients developed HCQ-related maculopathy at 8 and 6.5 years of treatment, despite regular ophthalmologic evaluation. On follow-up 7 and 9 years after cessation of HCQ treatment, both patients had stable eye disease. No HCQ retinal toxicity was observed in the subsequent 342 patients who were treated for >6 years. Overall, the incidence of HCQ-related retinopathy in 400 patients who were treated with recommended dosages of the drug for a mean of 8.7 years was reduced to 0.5%. CONCLUSIONS After a baseline ophthalmic examination to confirm the absence of preexisting fundus pathology, patients with normal renal function may receive HCQ at a maximal daily dosage of 6.5 mg/kg and continue safely for 6 years. However, annual screening is recommended in patients who have taken the drug, even in recommended doses, for >6 years.

Management of canalicular obstructions.

Purpose of review The successful treatment of canalicular obstructions continues to represent a therapeutic challenge. Conjuctivodacryocystorhinostomy with a Lester Jones bypass tube is the standard treatment in these situations. Although a high rate of anatomic success can be achieved with this procedure, a relatively high rate of complications and poor patient satisfaction have been reported. Because of this, alternative techniques have emerged. Herein, we review all these techniques described in the literature. Recent findings Increasing interest in minimally invasive surgery and developments in fiberoptic technology have fueled advances in endocanalicular surgery. Canalicular trephination for obstructions at any level of the canalicular system and laser and balloon canaliculoplasty for focal canalicular and common canalicular stenoses may offer a well tolerated, fast and easy alternative, with potentially better patient satisfaction when compared with conjuctivodacryocystorhinostomy. Summary The alternative techniques to conjuctivodacryocystorhinostomy are less invasive, and respect and utilize any healthy and functional part of the canalicular system. The correct choice of the technique for a given canalicular obstruction, and therefore the long-term success, depends on the site and degree of the obstruction.

B-cell targeted therapy with rituximab for thyroid eye disease: closer to the clinic.

The management of thyroid eye disease (TED) remains a therapeutic challenge. The current established therapies are unsatisfactory in one-third of the patients and have many limitations. Rituximab (RTX) is a CD20+ B-cell-depleting monoclonal antibody approved for the treatment of non-Hodgkin lymphomas, chronic lymphocytic leukemia, and rheumatoid arthritis. The early experience with RTX suggests that it is a promising alternative therapy for TED. Rituximab may compare favorably to the conventional glucocorticoid therapy and causes less collateral damage than retrobulbar orbital radiation and decompression surgery. In addition, the preliminary studies on RTXs proposed mechanism of action have revealed new insights into the pathogenic role of B-cells in TED. We summarize the current literature on the clinical application of RTX in TED and discuss its putative mechanisms of action.

Opaque intraocular lens implantation: a case series and lessons learnt

Purpose To report the use of opaque intraocular devices in three patients with complex neuro-ophthalmic symptoms. Methods A case series of three patients with neuro-ophthalmic symptoms requiring occlusion of one eye when alternative methods had failed to control symptoms. Morcher (Stuttgart, Germany) opaque intraocular implants were used in all patients. Results All three patients observed an improvement in symptoms following opaque intraocular device implantation. One patient (Case 2) required multiple devices for symptom relief. Conclusion Opaque intraocular occlusive devices are an increasingly popular choice for clinicians in patients with intractable diplopia but we highlight their use in patients with other complex neuro-ophthalmic symptoms. We learned a number of useful lessons in these patients as summarized in this case series.

Nonsurgical management of silent sinus syndrome with hyaluronic acid gel.

A 44-year-old woman was referred with 3 mm of left enophthalmos and a deep superior sulcus. She was diagnosed 6 months earlier with silent sinus syndrome and had undergone a left middle-meatal antrostomy with improvement of her hypoglobus but not of her enophthalmos and superior sulcus deformity. Two milliliters of Restylane SubQ (Q-Med AB, Uppsala, Sweden) was injected as a single bolus in the intraconal and extraconal posterior orbit via a standard transcutaneous inferotemporal approach using a primed green 21-gauge needle. Immediate reduction of enophthalmos by approximately 2 mm and superior sulcus improvement were noted. This remained stable at 6 months. The symptoms and signs of silent sinus syndrome can be effectively corrected with this minimally invasive nonsurgical option. To our knowledge, the use of hyaluronic acid gel for the treatment of enophthalmos in silent sinus syndrome has not been previously reported.

Kaposi's Sarcoma of the Bulbar Conjunctiva in an Immunocompetent Patient.

Kaposi’s sarcoma is an uncommon endothelial malignant tumor, first described by Moricz Kaposi in 1872. It is commonly encountered in patients with acquired immunodeficiency syndrome (AIDS), immunosuppression or organ transplantation. Conjunctival Kaposi’s sarcoma is a rare tumor, mostly in patients with AIDS. We present a selected case with a superior bulbar conjunctival lesion in a patient who is HIV negative and immunocompetent, with an unremarkable general medical history. The lesion was surgically excised with no adjunctive treatment, and histological examination confirmed the diagnosis. There was no recurrence or metastasis 5 years after surgery.

Orbital Lobular Panniculitis in Weber-Christian Disease: Sustained Response to Anti-TNF Treatment and Review of the Literature

Weber-Christian disease is a febrile, relapsing, non-suppurative panniculitis of unknown etiology. Lobular panniculitis is the essential feature in biopsy specimens and evolves through three recognizable stages. We report a case of Weber-Christian disease with bilateral orbital involvement, at different stages, affecting the orbital fat along with enophthalmos in one orbit, and the upper preaponeurotic fat pad in the other. Weber-Christian disease was refractory to treatment with conventional immunosuppressive regimens; however, early inflammatory-but not chronic fibrotic-orbital lesions responded dramatically to anti-tumor necrosis factor (TNF) therapy. A literature review revealed five additional cases of orbital Weber-Christian disease, none treated with anti-TNF antibodies. Of these, four presented initially with proptosis, representing early stages of inflammation, and two subsequently developed enophthalmos, representing late, inactive stage of the disease. Although orbital Weber-Christian disease is rare, ophthalmologists need to be aware of this entity. Depending on the stage of inflammation, Weber-Christian disease should be included in the differential diagnosis of both proptosis and enophthalmos. Anti-TNF antibodies can successfully treat patients at the early inflammatory stage.

Suturing techniques and postoperative management in penetrating keratoplasty in the United Kingdom

Aims To report on the suturing techniques and aspects of postoperative management in penetrating keratoplasty in the United Kingdom. Methods A postal questionnaire was sent to 137 ophthalmic consultants identified from a Royal College of Ophthalmology database as having a special interest in anterior segment surgery. The questionnaire surveyed surgeon preferences for surgical and suturing technique for penetrating keratoplasty surgery, and the postoperative care of corneal grafts. Results In all, 68% of questionnaires were completed and returned: 73% of respondents used a Flieringa ring or equivalent, 94% routinely used cardinal sutures, with 50.5% removing them at the end of the procedure. The most common suturing technique for routine penetrating keratoplasty was a single continuous suture (35%). In these cases, a 10/0 nylon suture was used by 89%. Sixty-six percent changed their technique in high-risk cases, 52% used a 3-1-1 knot, and 75% made a distinction between a reef and granny knot, with 76% using a reef. Thirty percent buried the knots within the donor material, and 29% within the host tissue. Twenty-five percent had no routine time for graft suture removal, but 41% removed them between 1 and 2 years post-surgery. After suture removal, 98% used steroids and 88% used topical antibiotics. Thirty-four percent stopped topical steroids before suture removal, with 38% stopping topical steroids more than 3 months prior to suture removal. Conclusion This survey demonstrates that there is considerable variation in suturing techniques and postoperative care for penetrating keratoplasty. These significant variations in practice need to be considered when interpreting outcomes and research.

Isolated orbital fibrous dysplasia associated with ipsilateral keratoconus.

Introduction: Fibrous dysplasia is a primary orbital bone tumor, described as a benign disorder in which proliferation of fibrous tissue and osteoid replaces and distorts the bone from which it derives. Unilateral keratoconus is a rare entity. Herein, we report a case of an extensive ethmoidal fibrous dysplasia associated with ipsilateral keratoconus, and review the literature on the subject. Materials and methods: A 22-year-old man presented with left painless proptosis evolving over 10 years. There was associated ipsilateral epiphora and gradual visual loss. On examination his visual acuity was 20/20 OD and CF OS. His left globe was displaced lateraly 12 mm, with 9 mm of proptosis. The extraocular movements were normal. Left nasolacrimal duct obstruction was noted. Clinical signs of keratoconus were present only in the left cornea. High resolution corneal topography confirmed unilateral keratoconus and a CT scan showed an ovoid mass with a well defined sclerotic margin arising from the left ethmoid sinus and invading the orbit. Results: The patient underwent resection of the lesion via a modified Lynch incision. Complete regression of proptosis was observed immediately after surgery. Histopathological examination revealed irregular trabeculae of woven bone in different levels of maturation in a moderately cellular fibrous matrix without nuclear atypia. Trabeculae were without osteoblastic rimming or osteoclastic resorption. There has been no recurrence over a 2-year follow-up period. Conclusion: To our knowledge, the coexistence of unilateral isolated craniofacial fibrous dysplasia with ipsilateral keratoconus has not been reported so far.

Single-suture brow lift in VII palsy.

To the Editor: VII nerve palsy is associated with a multitude of ocular and periocular disorders, including lagophthalmos, dry eye, paralytic ectropion of the lower eyelid, and brow ptosis. The latter may be esthetically disfiguring due to induced asymmetry between the affected and unaffected side and the ipsilateral loss of forehead wrinkles. In addition, brow ptosis may cause upper eyelid fullness and corneal erosions from elongated brow hair. A number of procedures have been described for correcting brow ptosis associated with VII palsy, such as direct, transblepharoplasty, midforehead or endoscopic brow lift. Although effective, these procedures are time-consuming and may require sedation or general anesthesia and special instrumentation. In the case of direct brow lift, which is commonly employed for paralytic brow ptosis, damage to the supraorbital nerve, brow hair loss, and the creation of a disfiguring scar have been reported. Furthermore, because patients with VII palsy may require additional corrective procedures, such as the insertion of a gold weight to correct lagophthalmos or the correction of paralytic ectropion (Fig. A–C), the duration of brow ptosis correction is important since it may constitute one part of a combined corrective procedure. A previous study has described a minimally invasive brow suspension technique for facial paralysis, using 4.0 prolene sutures. In that study, a Hewson suture retriever was used to direct 3 sutures from a scalp incision to the suprabrow area. The sutures were then anchored to exposed galea-pericranium of the scalp incision site. In an attempt to further simplify and shorten the duration of brow lift in cases with VII palsy we have employed a single 2.0 prolene suture to lift the ptotic brow. A simulation for the effect of the procedure can be obtained preoperatively by using adhesive tapes, such as Steristrips (3M, Maplewood, MN, U.S.A.) to elevate the ptotic brow (Fig. C). The procedure is easily performed on an outpatient basis under local anesthesia and usually lasts for only a few minutes. The frontal area above the ptotic brow is marked and infiltrated with local anesthetic (Fig. D). A Wright’s fascia needle is used to pass the suture at a deep supraperiosteal plane (Fig. E). A variety of suture paths can be employed but our familiarity with the Fox pentagon path for the correction of blepharoptosis has lead us to commonly use a similar approach, i.e., a pentagon with a base along the superior brow border (with stab incisions located at the medial and lateral ends of the brow), and apex located close to the hairline, where the suture is secured with a deeply buried multiple knot. The intermediate passage points are placed at the midforehead area (Fig. D). The degree of suture tightening is decided intraoperatively based on the level of the fellow brow. All stab incisions are closed with 7.0 vicryl sutures (Fig. F). Although a variety of materials, well known from ptosis correction, such as polytetrafluoroethylene or silicone slings, may theoretically be employed, we have used prolene because it is cheaper and it does not stretch. We feel that the thread brow lift described is better tolerated by patients than the direct brow lift, which is much more interventional and may be associated with frontal hypesthesia or a visible scar. Some degree of temporary skin puckering caused by suture tightening was observed only in the immediate postoperative intervals (Fig. F). For the surgeon, the thread brow lift is faster and much easier to perform, which may be important in cases where combined procedures are undertaken, with additional surgical steps such as paralytic ectropion correction or gold weight insertion. The main disadvantage of thread brow lift by the technique described may be the risk of brow ptosis recurrence due to cheese-wiring of the suspending material through tissues, although the risk may be less in cases with passages of slings though deeper planes, for thicker material (such as the 2.0 prolene suture) or for porous integrated materials, such as expanded polytetrafluoroethylene. A comparative study of the minimally invasive single-suture brow lift described with the “gold-standard” direct brow lift in paralytic brow ptosis could help in determining its advantages and disadvantages in the everyday clinical practice.