Orbital extranodal marginal zone lymphoma arising during pregnancy: a case report.

A 37-year-old, previously healthy woman presented during her first trimester of pregnancy with a two-week history of rapidly progressive proptosis in the left eye. Clinical examination revealed limited left supraduction and diplopia in upward gaze. Orbital magnetic resonance imaging showed a medial orbital mass adjacent to the globe with secondary proptosis. Pathologic examination of a biopsied specimen of the orbital mass and subsequent immunophenotyping by flow cytometry revealed an extranodal marginal zone B-cell lymphoma. Clinical and histological features as well as a review of the literature are described.

Primary Lacrimal Gland Oncocytoma Associated With Hypervascularity: A Case Report With Imaging and Immunohistochemical Characterization.

Oncocytoma is a rare benign neoplasm with few cases reported to involve the orbit. When affecting the orbit, oncocytomas have very slow growth and often evade early diagnosis. The diagnostic evaluation of these orbital tumors is not fully understood. The authors present a case of a benign oncocytoma arising in the lacrimal gland associated with significant vascularity and intraoperative bleeding. The authors provide immunohistochemical analysis of this rare orbital tumor and insights in surgical planning.

Rapidly Enlarging Hidradenoma of the Eyelid.

Hidradenoma is a rare benign neoplasm, with few cases reported to involve the eyelid. When affecting the eyelid, hidradenomas may mimic other benign or malignant lesions. Rarely, a benign hidradenoma may transform into a malignant hidradenoma and metastasize. The authors present a case of a benign hidradenoma arising from the eyelid, presenting with rapid growth, ulceration, and bleeding, suggestive of a malignant lesion.

Posteriorly Displaced Orbital Implant Causing Intractable Anophthalmic Socket Pain.

Anophthalmic socket pain is a rare complication of enucleation. The authors present a patient presenting with intractable anophthalmic socket pain due to a posteriorly displaced orbital implant. The patient's pain localized to the V1 and V2 orbitofacial dermatomes, and we suspect compression of the frontal and zygomatic branches of the ophthalmic and maxillary nerves, respectively, as the underlying etiology of the patient's pain. Removal of the implant and placement of a dermis fat graft was effective at alleviating the patient's symptoms.

Topical Corticosteroid Use Associated With Increased Degree of Ptosis and Rate of Ptosis Repair Failure.

PURPOSE: To compare the degree of ptosis and the risk of ptosis repair failure among patients with and without a history of topical corticosteroid use. METHODS: Retrospective, case-controlled study examining topical corticosteroid use among adults with ptosis who underwent external levator advancement/resection (ELR) or Müller muscle conjunctival resection with at least 3 months postoperative follow-up. Comparative statistical analyses of surgical outcomes were performed amongst patients with and without history of topical corticosteroid use. RESULTS: A total of 240 patients (406 eyelids) met study criteria, of which 36 patients (44 eyelids) had history of topical corticosteroid use. Mean preoperative margin reflex distance was 0.20 mm and 0.58 mm for topical corticosteroid and non-corticosteroids users (p = 0.01). Mean preoperative levator function was 9.78 mm and 10.38 mm for topical corticosteroid and non-corticosteroid users (p = 0.02). The rate of ptosis repair failure was 30% and 16% in patients with and without a history of topical corticosteroid use (odds ratio 2.25, 95% confidence interval 1.10-4.55; p = 0.03). The rate of recurrence per surgical type in eyelids with and without history of topical corticosteroid use was: external levator advancement/resection 11/27 (41%) and 48/266 (18%) (odds ratio = 3.12, confidence interval 1.36-7.15 0; p = 0.01); Müller muscle conjunctival resection 2/17 (12%) and 9/96 (9%) (odds ratio 1.29, confidence interval 0.25-6.56; p = 0.76). CONCLUSIONS: Topical corticosteroid use is associated with more severe presenting ptosis and increased rates of ptosis repair failure. Compared to Müller muscle conjunctival resection, there is a significantly higher rate of ptosis repair failure in patients undergoing external levator advancement/resection.

Polytetrafluoroethylene frontalis suspension in blepharospasm with eyelid apraxia: an effective and well-tolerated adjunct to botulinum toxin therapy.

Purpose: To report the efficacy of polytetrafluoroethylene (PTFE) frontalis suspension for blepharospasm with eyelid apraxia and postoperative botulinum toxin requirements. Methods: Retrospective chart review of patients with blepharospasm and eyelid apraxia who underwent frontalis suspension. The primary outcome was a surgical success, defined by surgeon- and patient-reported success in postoperative eyelid opening. Comparative statistical analyses of botulinum toxin dosage and treatment intervals were performed amongst patients before and after frontalis suspension ptosis repair. Results: Five patients (10 eyelids) met the study criteria, of which 40% were female. Mean age was 63.2 years. All patients had successful surgical outcomes based on physician-reported and patient reported satisfaction with the postoperative eyelid opening. Average follow-up was 14 months. Preoperative botulinum toxin treatments averaged 80.4 units (range 32-110, SD 33.2) to the periocular region over an average of 9.6-week intervals. Postoperative botulinum toxin treatments averaged 61.4 units (range 24-110, SD 34.7) to the periocular region over an overage of 9.8-week intervals. No patients experienced postoperative exposure keratopathy, extrusion of the sling, or postoperative infection. Conclusions: Frontalis suspension using PTFE suture in the setting of blepharospasm with eyelid apraxia was found to be a safe and effective procedure. Frontalis suspension in this population is not a substitute for botulinum toxin treatment but may allow for reduced treatment dosage. Frontalis suspension appears to increase patient functionality with improved eyelid opening in patients with blepharospasm with eyelid apraxia.

Ophthalmic changes in patients with hemifacial atrophy (Parry-Romberg syndrome).

PURPOSE: Parry-Romberg syndrome (PRS) is a rare condition characterized by progressive, unilateral facial atrophy. We hypothesize that patients with this condition may have involvement of the ocular structures. Here, we report our ophthalmic, clinical and anatomical findings in an observational study of six patients with long-standing PRS. METHODS: Patients diagnosed with PRS were invited to participate in a clinic visit during which the following tests were administered and data recorded: best-corrected vision, refractive error, Ishihara color plates, Hertel exophthalmometry, gonioscopy, complete slit-lamp and dilated fundus examination, Intra Ocular Lens Master measurements and keratometry. Two-sample T tests were used to compare data between affected and unaffected eyes, as well as affected eyes and a normative population. RESULTS: Six patients underwent complete eye examinations. The mean spherical equivalent of the affected eye was + 3.83 D, while that of the unaffected eye was + 0.13 D. The atrophic hemiface averaged 2.8 mm of enophthalmos on Hertel exophthalmometry. The axial length of the atrophic eye was 0.91 mm shorter than the unaffected eye. Compared to normative data, in patients with PRS, the difference between eyes was statistically different for each of the following variables: visual acuity, spherical equivalent, corneal diameter, axial length and flat and steep keratometry. CONCLUSIONS: This is one of the first quantitative, exploratory studies with ophthalmic measurements in patients with PRS. Our results suggest the globe may demonstrate atrophic changes similar to other soft tissues in the face known to be affected by this condition.

Intraorbital Arteriovenous Fistula From the Superficial Temporal Artery.

Intraorbital arteriovenous fistula is a rare vascular disease characterized by an acquired arteriovenous communication without direct cavernous sinus involvement. Intraorbital arteriovenous fistula may develop slowly and present similarly to other insidious orbitopathies, such as carotid-cavernous fistula. The authors present a case of a superficial temporal artery to superior ophthalmic vein fistula arising in the absence of trauma or prior surgery. This is the first report of a spontaneous intraorbital arteriovenous fistula arising between these vessels and further describes the rare occurrence of intraorbital arteriovenous fistula.

Ophthalmic Changes Following Maxillectomy With or Without Postoperative Radiation Therapy.

PURPOSE: To evaluate ophthalmic complications and quantitatively assess anatomic changes following maxillectomy with or without adjuvant radiotherapy in patients with midface malignancy. METHODS: Twenty-four patients who underwent maxillectomy for sinus and/or paranasal cancer were included for retrospective review. Patients with complete ophthalmic examinations were evaluated for postoperative findings corresponding to sequelae of treatment. When available, anatomical changes including eyelid position were quantified from preoperative and postoperative full-face photos using computer software. RESULTS: The most common complications identified in patients after maxillectomy were retraction/ectropion (50%), epiphora (29%), and exposure keratopathy/dry eye syndrome (25%). Patients treated with maxillectomy with adjuvant radiation therapy were more frequently found to have ophthalmic complications following treatment. In patients with available postoperative photos (n = 10), the mean ipsilateral margin reflex distance (MRD)2 and inferior scleral show were 8.4 mm and 2.4 mm, respectively. In patients with available preoperative photos (n = 5), the mean change in MRD2 and inferior scleral show following maxillectomy was 3.4 mm and 2.8 mm, respectively. CONCLUSION: Patients undergoing maxillectomy for the treatment of head and neck malignancy may be at significant risk for development of specific periocular complications. Lower eyelid malposition was the most significant postoperative quantitative eyelid change following maxillectomy, which may be exacerbated by adjuvant radiotherapy and inferior orbital rim removal.

Outcomes of Endoscopic Dacryocystorhinostomy in Secondary Acquired Nasolacrimal Duct Obstruction: A Case-Control Study.

INTRODUCTION: Secondary acquired nasolacrimal duct obstruction (SANDO), where the obstruction is caused by a known process, is becoming more commonly treated by endoscopic dacryocystorhinostomy (endoDCR). This study evaluates outcomes of endoDCR for cases of SANDO in comparison to endoDCR outcomes treating primary acquired nasolacrimal duct obstruction (PANDO). METHODS: All patients undergoing endoDCR from 2006 to 2015 at a tertiary referral center were reviewed for etiology of nasolacrimal duct obstruction (NLDO) and success of procedure. Inclusion criteria were preoperative determination of NLDO via probing and irrigation, greater than 60-day follow up with assessment of duct patency via probing and irrigation after silicone tube removal, and postoperative survey for recurrent epiphora. Exclusion criteria were cases treating partial NLDO, patients undergoing concurrent conjunctivodacryocystorhinostomy, and patients lost to follow up or with incomplete records. Preanalysis stratification was performed for the following preexisting conditions: PANDO, granulomatosis with polyangiitis, sarcoidosis, midfacial radiotherapy, radioactive iodine therapy, severe prolonged sinusitis, prior failed DCR, and midface trauma. The primary outcome was postoperative anatomical patency of the nasolacrimal system with resolution of epiphora. Fisher-Freeman-Halton exact tests were performed comparing each SANDO group to the PANDO group with statistical significance set at p < 0.05. RESULTS: Fifty-nine cases of NLDO underwent endoDCR in this series, 21 of which had a diagnosis consistent with SANDO. All patients treated with the aggressive perioperative management protocol the authors describe achieved anatomical patency. Patients with history of radioactive iodine therapy and radiotherapy had statistically worse outcomes compared with patients with PANDO, p = 0.011, p = 0.045, respectively. Stratified groups with increased intranasal sinus inflammation trended toward worse outcomes, but were not statistically different. CONCLUSIONS: EndoDCR is a viable approach for the treatment of SANDO. In patients with history of radiotherapy or radioactive iodine therapy, however, endoDCR demonstrated decreased success rates as compared with patients with PANDO. Modifications of operative and postoperative management based on underlying etiology of NLDO may improve outcomes of endoDCR in SANDO cases. The authors describe an aggressive protocol for perioperative management of patients with pathologic nasal inflammation undergoing endoDCR.

Lateral Wall Orbital Decompression: Comparison of Outcomes in Rim Sparing and Temporary Rim Removal Techniques.

PURPOSE: To compare exophthalmos reduction in lateral orbital decompressions performed via rim sparing versus temporary rim removal techniques. METHODS: The authors performed a retrospective chart review of all patients who underwent simple lateral or combined medial and lateral wall orbital decompression between 2005 and 2013 by a single surgeon. Nineteen patients (33 orbits) were identified for inclusion in the study. Decompression procedures (1 or 2 orbital walls) involved either a rim sparing or a temporary rim removal technique. Preoperatively, all patients had stable exophthalmos defined as ≤1 mm change in exophthalmos over 2 consecutive visits. Measurements were taken again at the 3 to 4 months postoperative visit. Exclusion criteria were acute or unstable exophthalmos, exophthalmos secondary to malignancy, and patients lost to follow up. RESULTS: There were no significant differences in exophthalmos reduction for rim sparing versus temporary rim removal techniques in any of the groups studied. Simple lateral decompression procedures achieved 3.7 and 4.4 mm of exophthalmos reduction in rim sparing versus temporary rim removal techniques, respectively (P = 0.49). Exophthalmos reduction in combined medial and lateral wall orbital decompression was 4.1 mm for rim sparing and 3.5 mm for temporary rim removal techniques (P = 0.75). CONCLUSION: In our experience, orbital decompression approached through rim sparing or temporary rim removal techniques achieves similar results in simple lateral and combined medial and lateral decompressions. Though these techniques generate similar outcomes, temporary rim removal provides for improved visibility and access to deep orbital structures.

Endoscopic dacryocystorhinostomy following head and neck radiation therapy.

This article determines the efficacy of endoscopic dacryocystorhinostomy (endoDCR) in patients who have undergone adjuvant external beam radiation therapy (XRT) following head and neck cancer resection. A retrospective chart review was performed on all patients who underwent endoDCR between 2006 and 2014 at a tertiary referral center. Cases were reviewed and selected for the following inclusion criteria: history of adjuvant sinonasal XRT following head and neck cancer resection, preoperative probing and irrigation demonstrating nasolacrimal duct obstruction (NLDO), postoperative probing and irrigation following silicone tube extubation. Exclusion criteria included active dacryocystitis, postoperative follow-up of less than 4 months, presence of epiphora prior to XRT, lack of probing/irrigation at preoperative or postoperative visit, and lid malposition including ectropion, facial palsy, and/or poor tear pump. Six patients (7 eyes) met the selection criteria. EndoDCR was performed at a mean time of 30 months following last radiation treatment (range, 3-71 months). Mitomycin C was used in 4/7 cases. Silicone tube removal occurred between 3-8 months postoperatively. Five out of 6 patients had postoperative sinonasal debridement and nasal saline/corticosteroid irrigation. Five out of 6 patients (83%) had both resolution of epiphora and anatomic patency confirmed by probing and irrigation. Our experience suggests that endoDCR procedures can be effective in patients with NLDO following prior sinonasal XRT for head and neck neoplasms. Postoperative management with sinonasal debridement and combined saline/corticosteroid nasal irrigation may help to improve surgical success in patients with increased post-radiotherapy mucosal inflammation.

Intralesional Injection of Bevacizumab for the Treatment of an Apical Orbital Cavernous Venous Malformation.

Surgical excision of orbital cavernous venous malformations located in the orbital apex is challenging due to difficulty obtaining surgical exposure and higher risk of morbidity. Intralesional bevacizumab injection has been utilized for the treatment of choroidal and intracranial cavernous venous malformations. A 52-year-old woman with an orbital apical mass consistent with a cavernous venous malformation causing decreased visual acuity, diminished color vision, and visual field loss was treated with intralesional bevacizumab injected under direct surgical visualization. Subsequently, she demonstrated improved visual acuity, color vision, and slow but dramatic visual field improvement over one year. Injection of bevacizumab may be a viable alternative treatment for orbital cavernous venous malformations.