Rituximab for the treatment of IgG4-related orbital disease: experience from five cases.

PURPOSE: To review the clinical efficacy and safety of rituximab for treatment of IgG4-related orbital disease (IgG4-ROD). DESIGN: Retrospective multicentre interventional case series. METHODS: Chart review for five cases of biopsy-confirmed IgG4-ROD (IgG4+>10/HPF, ratio of IgG4+/IgG+>40%) treated with rituximab. Information retrieved included the dosing schedule, adverse events and the magnitude, temporality, and duration of the clinical response. RESULTS: All cases of IgG4-ROD were either steroid dependent or steroid resistant. Rituximab doses for induction therapy included two doses of 1000 mg at 2-weekly intervals, and four doses at 375 mg/m(2) at weekly intervals. Two months after starting rituximab, three cases achieved complete clinical resolution and two cases achieved partial clinical resolution. Complete radiological resolution occurred in one case, and partial radiological resolution in three cases. Three cases received rituximab maintenance therapy and one case was commenced on mycophenolate. No relapse occurred during a mean follow-up of 33 months (range: 7-65 months). One disease relapse occurred when the dosing interval of rituximab maintenance therapy was extended to 6-monthly intervals; remission was swiftly achieved with rituximab reinduction therapy. The only adverse effects reported were one episode of fatigue lasting 1 week and two episodes of orbital discomfort. CONCLUSION: Rituximab may be an effective treatment option for IgG4-ROD that is steroid dependent or steroid intolerant. Rituximab therapy resulted in swift clinical and radiological improvement, many months free of relapse, and few side effects.

'The Microhand': a new concept of micro-forceps for ocular robotic surgery.

PURPOSE: To test the feasibility of retinal manipulations using a new micromanipulator (Microhand) for ocular robotic microsurgery. METHODS: Pneumatically actuated four-finger microhands were developed at UCLA with micro electromechanical systems (MEMS) technology to mimic a human hand for small object manipulation. Microhands with four 4 mm finger lengths were used for this study to lift caliper weights and fresh retinal tissue of porcine cadaver eyes to find the maximum force at a given pressure and feasibility of the microhands for retinal manipulation in real surgery. RESULTS: A full closure of the microhand used for caliper weight lifting was achieved under 65 psi (448 kPa) of air pressure. The four-fingered microhand was able to develop about 20 mN of total lifting force and 5 mN per finger at 80 psi (551 kPa), and was strong enough to displace and lift the retina of pig eyes. CONCLUSIONS: The microhand is able to apply calibrated forces to ocular tissues and is suitable for ocular microsurgical procedures. This new tool would be useful in the development of robotic microsurgery.

Robotic ocular surgery.

BACKGROUND: Bimanual, three-dimensional robotic surgery has proved valuable for a variety of surgical procedures. AIMS: To examine the use of a commercially available surgical robot for ocular microsurgery. METHODS: Using a da Vinci surgical robot, ocular microsurgery was performed with repair of a corneal laceration in a porcine model. The experiments were performed on harvested porcine eyes placed in an anatomical position using a foam head on a standard operating room table. A video scope and two, 360 degrees -rotating, 8-mm, wrested-end effector instruments were placed over the eye with three robotic arms. The surgeon performed the actual procedures while positioned at a robotic system console that was located across the operating room suite. Each surgeon placed three 10-0 sutures, and this was documented with still and video photography. RESULTS: Ocular microsurgery was successfully performed using the da Vinci surgical robot. The robotic system provided excellent visualisation, as well as controlled and delicate placement of the sutures at the corneal level. CONCLUSIONS: Robotic ocular microsurgery is technically feasible in the porcine model and warrants consideration for evaluation in controlled human trials to deploy functioning remote surgical centres in areas without access to state-of-the-art surgical skill and technology.

Mikulicz's disease: a new perspective and literature review.

PURPOSE: To report the clinical and pathophysiologic features of two patients with Mikulicz's disease and to further characterize recommendations for diagnosis and management with a review of the literature. METHODS: Retrospective nonrandomized consecutive case series, Jules Stein Eye Institute, David Geffen School of Medicine at UCLA. RESULTS: Mikulicz's disease is characterized by symmetric lacrimal, parotid, and submandibular gland enlargement with associated lymphocytic infiltrations. The authors noted two cases of Mikulicz's disease. The diagnosis of Mikulicz's disease was based on the following criteria: 1) symmetric and persistent swelling of the lacrimal glands and either or both of the major salivary glands (parotid and submandibular); and 2) the exclusion of other diseases that may mimic this presentation, such as sarcoidosis, viral infection, or lymphoproliferative disorders. CONCLUSIONS: Mikulicz's disease is a condition in which there is bilateral lacrimal and salivary gland swelling that is not associated with other systemic conditions. The condition is self-limiting and most often, the diagnosis is a clinical one. Previously, Mikulicz's disease was often considered as a subtype of Sjögren's syndrome (SS). Clinical and immunologic differences between Mikulicz's disease and SS may warrant further consideration of Mikulicz's disease as a specific autoimmune phenomenon separate from SS, and Mikulicz's disease may be amenable to different treatment modalities than those employed in patients with SS.

Investigation and endoscopic treatment for functional and anatomical obstruction of the nasolacrimal duct system.

The purpose of this study was to assess the results of endonasal DCR in patients with a functional obstruction of the nasolacrimal system. The study design was a prospective non-randomized interventional case series. A prospective series of 102 consecutive endoscopic DCRs were evaluated with preoperative dacrycytography (DCG) and lacrimal scintillography. Evaluation of the DCG and scintillography allowed the patients to be classified as either having an anatomical or functional obstruction of the nasolacrimal system. The powered endoscopic DCR was performed between January 1999 and December 2001. Patients were followed up for a minimum of 12 months after surgery. In the 70 powered endoscopic DCRs for anatomical obstruction, 68 (97%) have remained asymptomatic with a free flow of fluorescein from the conjunctiva to the nose. In the patients who were classified as having a functional obstruction, 27 of 32 were asymptomatic (84%) with a free flow of fluorescein into the nose. To conclude, powered endoscopic DCR produces excellent results in patients with anatomical obstruction and good results in patients with functional obstruction of the nasolacrimal system.

Mechanical endonasal dacryocystorhinostomy with mucosal flaps.

AIMS: To describe and assess the efficacy of mechanical endonasal dacryocystorhinostomy (MENDCR). This is a new technique that involves creation of a large rhinostomy and mucosal flaps. The study involved a prospective non-randomised interventional case series with short perioperative follow up. METHOD: A prospective series of 104 consecutive endonasal DCRs performed from January 1999 to December 2001 were entered into the study. Patients included in the study had nasolacrimal duct obstruction and had not had previous lacrimal surgery. The technique involved anastomosis of nasal mucosal and lacrimal sac flaps and a large bony ostium. Surgery was performed by two surgeons (AT/PJW). Follow up assessment included nasoendoscopy as well as symptom evaluation. Success was defined as anatomical patency with fluorescein flow on nasoendoscopy and patency to lacrimal syringing. The average follow up time was 9.7 months (range 2-28, SD 6.7 months). RESULTS: There were 104 DCRs performed on 86 patients (30 male, 56 female). The average age of the patients was 59 years (range 3-89, SD 24.1 years). Common presentations were epiphora (77%) and/or mucocele (19%). Septoplasty (SMR) was required in 48 DCRs (46%) and 13 DCRs (12.5%) needed other endoscopic surgery in conjunction with the lacrimal surgery. The surgery was successful in 93 cases (89%). Of the 11 cases that were classified as a failure six patients was anatomically patent but still symptomatic and another two had preoperative canalicular problems. The anatomical patency with this new technique was thus 95% (99 of 104 DCRs). CONCLUSION: MENDCR involves creation of a large ostium and mucosal preservation for the construction of flaps. The anatomical success is 95% and is similar to external DCR and better then other endonasal approaches. The authors suggest that creation of a large ostium as well as mucosal flaps improves the efficacy of this endonasal technique.

Secondary haemorrhage after dacryocystorhinostomy.

OBJECTIVE: To describe the incidence, clinical features, possible risk factors and outcome of patients suffering secondary haemorrhage after dacryocystorhinostomy (DCR). METHODS: Records of all patients undergoing external DCR by one surgeon (AAM) over a 6-year period were analysed retrospectively. RESULTS: After 293 DCR operations in 260 patients, 10 patients had a secondary haemorrhage (one after bilateral DCR), usually between days 4 and 10 post-operatively (mean 6.8 days). Six of the 10 were hospitalized for 2-5 days (mean 2.8 days), all of whom had nasal packing, intravenous antibiotics and intravenous fluids; none required blood transfusion. Three patients had immune system defects, three had used non-steroidal anti-inflammatory drugs (NSAID) and four had no obvious risk factors. All 10 had a successful outcome following DCR (compared to an overall failure rate of 25/293 (8.5%): 16 anatomical and nine functional). CONCLUSIONS: Secondary haemorrhage after DCR was found to occur after 3.8% of DCR. Risk factors include being immunocompromised and taking NSAID prior to surgery. The haemorrhage did not adversely affect surgical outcome.