Scleral contact lenses for the management of complicated ptosis.

PURPOSE: To present the management of three patients suffering from ptosis of various etiologies, with scleral contact lenses. MATERIAL AND METHODS: Three patients (five eyes) with ptosis resulting from levator dehiscence due to long-term rigid gas permeable contact lens wear for keratoconus, phthisis bulbi, and myopathy due to Kearns-Sayre syndrome were identified during a 2-year period. They were fitted with scleral contact lenses in order to provide cosmesis by lifting the upper eyelid with the bulk of the lens, and simultaneously provide vision correction where applicable. RESULTS: The scleral contact lenses provided comfortable wear, significantly improved cosmesis as both palpebral aperture and marginal reflex distance were increased, and visual acuity was also subjectively and objectively improved. Two of the patients opted for the scleral contact lenses, whereas the parents of the third patient, a 10-year-old girl with Kearns-Sayre syndrome, chose to undergo ptosis surgery due to handling issues of the scleral contact lenses. CONCLUSION: Scleral contact lenses can be a useful addition to the treatment option for patients with complicated ptosis.

Corneal topography with upper eyelid platinum chain implantation using the pretarsal fixation technique.

PURPOSE: To determine the effect of upper eyelid platinum chain implantation, with the pretarsal fixation technique, on corneal astigmatism. METHODS: This is a prospective, cohort study. Fifteen eyes of 15 patients underwent upper eyelid platinum chain implantation, with the pretarsal fixation technique, for facial nerve palsy. Information recorded included patient demographics, etiology for facial palsy, weight of the implant, time from onset of paresis to upper eyelid platinum chain implantation, associated surgical procedures, and preoperative and postoperative corneal topography measurements. RESULTS: Of the 15 patients studied, 10 were male and five were female. The mean age was 55.9 ± 13.8 years (range, 33-87 years). The most common etiology for facial palsy was acoustic neuroma. The weight of the implant ranged from 0.6 to 1.6gr (median 1.2gr). The time from onset of paresis to upper eyelid platinum chain implantation varied from 1 week to 3 months (median 1 month). Four patients had an associated procedure to correct the effect of paralytic ectropion. There was no statistically significant difference in with the rule astigmatism before and after platinum chain implantation. CONCLUSIONS: Upper eyelid platinum chain implantation, with the pretarsal fixation technique, does not appear to cause significant change in corneal astigmatism. This is contrary to data for pretarsal gold weight implantation, which does induce significant with the rule astigmatism.

Self-retaining bicanaliculus stents as an adjunct to 3-snip punctoplasty in management of upper lacrimal duct stenosis: a comparison to standard 3-snip procedure.

PURPOSE: To evaluate the efficacy of self-retaining bicanaliculus stents when used as an adjunct to 3-snip punctoplasty in comparison with standard 3-snip procedure between fellow eyes. METHODS: Prospective, randomized, comparative study. Thirty-two eyes of 16 consecutive patients with acquired, nontraumatic stenosis of the proximal lacrimal duct were randomly distributed into 2 equal study groups. Lower punctal stenosis was a standard prerequisite. Group A (16 eyes) received a simple 3-snip punctoplasty, while group B (16 fellow eyes) underwent a modified 3-snip punctoplasty combined with insertion of self-retaining bicanaliculus stents. Duration of follow ups: 6 months. Anatomical, functional, and subjective parameters were evaluated. RESULTS: A statistically significant difference in anatomical success rates was observed in favor of group B (p = 0.011) 6 months postoperatively. Complete relief from epiphora was noted in 8 eyes of group B compared with 2 eyes of group A (p = 0.057). In regard to mixed (absolute and partial) functional success rates, group B exceeded with statistically significant difference (15 eyes group B vs. 6 eyes group A; p = 0.002). A nasolacrimal duct obstruction was subsequently diagnosed in 3 of the nonpatient eyes. Reintroduction of silicone tubes was required in 4 eyes of group B. CONCLUSIONS: The use of self-retaining bicanaliculus stents seems to improve anatomical, functional, and subjective scores when combined with standard 3-snip punctoplasty in patients with acquired upper lacrimal duct stenosis.

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 RTX's 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.

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.

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.

Monocanalicular intubation in external dacryocystorhinostomy.

PURPOSE: To examine the feasibility of monocanalicular intubation in external dacryocystorhinostomy (EX-DCR). METHODS: Monocanalicular intubation using the mini-Monoka tube (S1-1500u, FCI Ophthalmics, Marshfield Hills, MA, U.S.A.) was performed in 18 patients undergoing EX-DCR. The tube was placed at the superior canaliculus in 16 cases and at the inferior canaliculus in 2 cases and was removed 3 months postoperatively. All cases were followed for at least 6 months postoperatively. RESULTS: The mini-Monoka was well tolerated in all patients. One case of spontaneous tube dislocation and 2 cases of presumed tube migration were recorded on the 15-day interval. Success rates on the 6-month interval were 88.88% (16/18), 94.44% (17/18), and 66.66% (12/18), concerning patency upon irrigation, resolution of mucopurulent epiphora, and resolution of watery epiphora, respectively. CONCLUSIONS: Monocanalicular intubation using mini-Monoka in EX-DCR is safe and effective. The technique may have advantages over bicanalicular intubation, such as easier insertion and removal, easier probing and irrigation though the unintubated canaliculus and, more importantly, less risk of punctal or corneal damage.

Primary, unilateral ocular adnexal lymphoma: disease progression and long-term survival.

PURPOSE: This investigation aimed to improve our understanding of the disease characteristics and clinical course for primary unilateral ocular adnexal lymphoma (OAL). METHODS: In this retrospective case series, all consecutive biopsy confirmed cases of lymphoma confined to a single ocular adnexa (unilateral stage 1E) during a 30-year period were included. All histologic classification conformed to the current World Health Organization classification. Descriptive statistics and standard survival analyses were performed. RESULTS: 122 patients were included in this study. The most common primary unilateral OAL were indolent B-cell lymphomas (mucosa-associated lymphoid tissue (MALT)-type marginal zone, follicular and small lymphocytic lymphoma) representing 80% of cases. Typically aggressive lymphomas were found in only 7% of patients (diffuse large B-cell lymphoma, immunoblastic lymphoma, diffuse large cell lymphoma, and mantle cell lymphoma). Overall, 24.4% of patients experienced progression of their disease after initial therapy, the majority occurring within 5 years. For the 80% of OAL of indolent B-cell type, progression free and disease specific survivals were 71 and 98% at 5 years and 61 and 90% at 10 years, respectively. Diffuse large B-cell lymphoma was more likely to progress overall (p < 0.01) and progress earlier (log rank, p < 0.01). Additionally, these patients were also more likely to succumb to disease (p < 0.01) in a shorter interval (log rank, p < 0.01). CONCLUSION: Most primary unilateral OALs are indolent lymphomas with good prognosis for survival and freedom from progression. However, a minority present with more aggressive lymphomas (diffuse large B-cell lymphoma, mantle cell lymphoma, immunoblastic lymphoma, and diffuse large cell lymphoma), carrying a more guarded prognosis.

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.

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.

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.

The role of thrombosis as a mechanism of exacerbation in venous and combined venous lymphatic vascular malformations of the orbit.

PURPOSE: To describe venous thrombosis as a mechanism of clinical change in venous and combined venous lymphatic malformations of the orbit and to attempt histopathologically to distinguish the various vascular components of these lesions using immunohistochemistry with CD31 and D2-40 antibodies. DESIGN: Retrospective, comparative, interventional case series. PARTICIPANTS: Twelve patients with clinically and radiologically well-documented episodes of thrombosis in venous malformations (n = 7; group A) and combined venous lymphatic malformations (n = 5; group B). INTERVENTION: Surgical excision of lesion in selected patients, 2 from group A and 5 from group B. MAIN OUTCOME MEASURES: Age at presentation, gender, onset, symptoms and signs, investigative findings (imaging and histopathologic review), management, and outcome. RESULTS: In group A, 4 patients were male and 3 were female, and in group B, 4 patients were female and 1 was male. The mean age+/-standard deviation at presentation was 57.6+/-10.9 years (range, 45-71 years) and 11+/-11.6 years (range, 1.5-26 years), respectively. The pattern of onset was acute in all cases. The most common signs and symptoms in group A were pain (n = 7), proptosis (n = 6), and nausea (n = 5), whereas in group B they were periorbital swelling (n = 5), proptosis (n = 5), and ecchymosis (n = 4). The immunohistochemistry results were positive for vascular (CD31) and lymphatic (D2-40) endothelium in all of the specimens. The combined venous lymphatic lesions divided themselves into 2 main categories based on the D2-40 findings in relationship to percentage of lymphatic vessels. These were lesions that were either lymphatic dominant (n = 3) or venous dominant (n = 2). CONCLUSIONS: Clinically, the 2 groups behave differently. Group A lesions present in adults with acute pain, proptosis, and nausea and may resolve spontaneously on follow-up. Intervention may be required in cases of severe pain, proptosis, or dysfunction. Group B lesions present early in life with frequent bouts of periorbital swelling, progressive proptosis, and ecchymosis. Therefore, early intervention is advised. Finally, when requesting imaging for such lesions, early- and late-phase contrast imaging should be used because thrombosis typically is better demonstrated in the late phase. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Congenital orbital fibrosis: a distinct clinical entity.

PURPOSE: To describe congenital orbital fibrosis as a distinct clinical entity, and highlight its constellation of features. MATERIAL AND METHODS: Retrospective, noncomparative, interventional case series of 4 patients with congenital orbital fibrosis. One patient underwent exploration of the orbit, release and biopsy of scar tissue. Two patients underwent strabismus surgery. One of these also underwent upper eyelid lengthening. Blepharoptosis, eyelid retraction, enophthalmos, proptosis, presence of a diffusely infiltrating orbital mass with secondary involvement of extraocular muscles, and dysplasia of the affected bony orbit were identified. RESULTS: One patient presented with eyelid retraction, 1 with ptosis, and the remaining 2 had normal eyelid height. Furthermore, one patient demonstrated true enophthalmos. Of the remaining 3, 1 had symmetrical exophthalmometry, and 2 presented with proptosis. The later 2 patients presented with ipsilateral facial hypoplasia, accompanying orbital wall dysplasia and decreased orbital volume, confirmed on computed tomography. In addition, all patients demonstrated an irregular, retrobulbar mass located medially within the orbit, incorporating the medial rectus muscle. CONCLUSION: Congenital orbital fibrosis is a nonfamilial, nonprogressive, unilateral, distinct clinical entity, characterized by the presence of a diffusely infiltrating orbital mass with secondary involvement of extraocular muscles resulting in variable symptomatology due to the cicatricial process.

Medpor lower eyelid spacer: does it biointegrate?

PURPOSE: To report the histopathologic findings of explanted Medpor lower eyelid spacers (LES) in complicated cases. MATERIALS AND METHODS: Four cases of lower eyelid retraction due to thyroid orbitopathy (n = 2), facial nerve palsy (n = 1), and post-enucleation socket syndrome (n = 1) were treated with Medpor LES. RESULTS: All implants were removed between 6 months to 2 years following their original insertion due to exposure, poor stability, or contour. Histopathology of the implants showed fibrosis and vascularization although clinically, at the time of removal, did not appear vascularized. In addition, immunohistochemistry was positive for Factor VIII related antigen and CD34, thus highlighting the presence of vessels in the pores and around the implant. CONCLUSION: To our knowledge, we are the first to report histopathologic findings of explanted high-density porous polyethylene implants from the lower eyelid in humans. Although this study shows that Medpor LES does biointegrate, we advocate using it sparingly due to associated complications such as exposure, poor stability, and contour.

A modified technique of retrograde intubation dacryocystorhinostomy for proximal canalicular obstruction.

PURPOSE: To describe a modification of the retrograde intubation dacryocystorhinostomy (DCR) in patients with proximal canalicular obstruction. MATERIALS AND METHODS: Interventional case report of a 43-year-old female with a nine-month history of left epiphora following a road traffic accident involving the proximal lower canaliculus. An external DCR approach was performed. Following the creation of a lower canalicular pseudopunctum, the O'Donoghue silicone stent was introduced through the common ostium, out through the pseudopunctum of the lower canaliculus, and returned through the punctum of the normal upper canaliculus down through the common ostium into the nose. RESULTS: The patient experienced complete resolution of symptoms and on her last follow-up, two years later, her lower canaliculus was patent to syringing. CONCLUSION: This modification of the retrograde intubation DCR is an effective technique which decreases the intraoperative time needed to insert the tubes and minimises further trauma to the newly created punctal area.