J. Javier Servat

Bilateral Lacrimal Gland Disease: Clinical Features of 97 Cases

OBJECTIVE Bilateral lacrimal gland (LG) disease is a unique presentation that can result from varied causes. We reviewed the diagnoses, clinical features, and outcomes of 97 patients with this entity. DESIGN Case series. PARTICIPANTS Ninety-seven patients with bilateral LG disease. METHODS Retrospective review and statistical analysis using analysis of variance and the Fisher exact test. MAIN OUTCOME MEASURES Patient demographics, clinical features, diagnostic testing, diagnosis, and treatment. RESULTS Patient age ranging from 8 to 84 years (mean, 46 years). The predominant gender was female (77%), and race included black (49%), white (38%), and Hispanic (12%) patients. Diagnoses fell into 4 categories: inflammatory (n = 51; 53%), structural (n = 20; 21%), lymphoproliferative (n = 19; 20%), and uncommon (n = 7; 7%) entities. The most common diagnoses included idiopathic orbital inflammation (IOI; n = 29; 30%), sarcoidosis (n = 19; 20%), prolapsed LG (n = 15; 15%), lymphoma (n = 11; 11%), lymphoid hyperplasia (n = 8; 8%), and dacryops (n = 5; 5%). Inflammatory conditions were more likely in younger patients (P<0.05) and in those with pain (P<0.001) and mechanical blepharoptosis (P<0.01) at presentation, whereas lymphoma was more common in older patients (P<0.001) without active signs of inflammation at presentation. Black patients were more likely to have sarcoidosis (P<0.01). Laboratory results showed high angiotensin converting enzyme level being significantly more likely in patients with sarcoidosis (P<0.05). However, sensitivity was limited to 45%, with 25% of patients diagnosed with IOI also demonstrating positive results. Corticosteroid therapy was the treatment of choice in 38 cases, corresponding to resolution of symptoms in 29% and improvement in an additional 32%. Overall, chronic underlying disease was found in 71% of patients, among whom 26% achieved a disease-free state, whereas 3% succumbed to their underlying disease. CONCLUSIONS The cause of bilateral lacrimal gland disease most commonly was inflammatory, followed by structural and lymphoproliferative. Patient characteristics and clinical presentations were key features distinguishing between competing possibilities. Despite local control with corticosteroids or radiotherapy, underlying disease continued in 71% of patients and led to death in 3%.

Bilateral Corneal Perforations and Autoproptosis as Self-Induced Manifestations of Ocular Munchausen's Syndrome

Purpose: To report a patient with bilateral corneal perforations and autoproptosis in a case of ocular Munchausen’s syndrome. Design: Case report. Participant: A 26-year-old white male referred to the oculoplastics service with one month history of decreased vision bilaterally and painful right eye. Multiple eyelid scars and right corneal opacity were noted. The patient was previously seen at another institution for rapid loss of vision in both eyes. Interventions: An orbit decompression among many procedures failed to controlled extreme pain and proptosis. Main Outcome Measures: Resolution of proptosis, stabilization of vision, pain resolution. Results: Three weeks after enucleation of the right eye was offered, patient presented with spontaneous left ruptured globe. After multiple episodes of self-mutilation and infections, both eyes were exenterated. Conclusions: Munchausen syndrome can be seen with ophthalmic manifestations and should be considered in the differential diagnosis when ocular abnormalities cannot be explained after a thorough evaluation. Recognition of this psychiatric disease is not only important for correct medical diagnosis and treatment, but also essential in protecting the patients from unnecessary invasive and aggressive medical procedures.

Periocular Necrotizing Fasciitis Causing Blindness

IMPORTANCE Periocular necrotizing fasciitis is a rare but potentially devastating disease, accompanied by high rates of morbidity and mortality. OBSERVATIONS We report 5 cases of periocular necrotizing fasciitis resulting in severe vision loss, 3 of which required exenteration to contain the disease and only 1 of which recovered vision. Three cases were caused by group A streptococcus; 1, by methicillin-resistant Staphylococcus aureus; and 1, by Streptococcus anginosus constellatus. CONCLUSIONS AND RELEVANCE Providers should maintain a high clinical suspicion for necrotizing fasciitis and distinguish it from more common forms of cellulitis. As seen in these 5 cases, periocular necrotizing fasciitis may cause severe visual loss more often than previously recognized. To our knowledge, this is also the first report of Streptococcus anginosus constellatus causing necrotizing fasciitis.

A solution to the Jones tube continuous positive airway pressure (CPAP) dilemma

A 49-year-old woman, who had previously undergone bilateral Jones tube placement, began nasal continuous positive airway pressure for obstructive sleep apnea. The patients use of continuous positive airway pressure was limited by intolerance of the transfer of air through the Jones tube to her ocular surface resulting in irritation and discomfort. A change from nasal continuous positive airway pressure to a full face mask, including both Jones tubes in the pressure circuit, resolved the problem.

Spontaneous Periocular Ecchymosis in Children: Differential Diagnosis and Current Trends in Evaluation and Management

While periocular ecchymosis commonly develops following surgery or traumatic injury to the orbit, the spontaneous appearance of periocular ecchymosis in children can indicate the presence of life-threatening conditions including pediatric malignancies (neuroblastoma, rhabdomyosarcoma, leukemia) and hematologic disorders (aplastic anemia, thrombocytopenia). Vascular malformations (capillary hemangioma, lymphangioma, orbital varix), inflammatory conditions (orbital myositis, amyloidosis), pertussis, and Blue rubber bleb nevus syndrome are benign differential considerations with visual complications in certain instances. Since spontaneous periocular ecchymosis (SPE) can be encountered by pediatric subspecialists both within and outside ophthalmology, the authors present a review of the current literature integrating the clinical features, latest diagnostic investigations, and updates in management for the entities that cause spontaneous periocular ecchymosis in children. A comprehensive and current understanding of the differential diagnosis elicited by this unique ocular finding will aid the clinician in managing long-term visual consequences and coordinating with appropriate pediatric subspecialists.

A comparison of surgical outcomes with the use of 2 different biodegradable multipoint fixation devices for endoscopic forehead elevation.

Purpose: To assess the efficacy and rates of inflammatory cyst formation with the use of 2 different biodegradable multipoint fixation devices, Endotine versus Ultratine, for endoscopic forehead elevation. Methods: The charts of 138 patients who underwent endoscopic forehead elevation by the same surgeon (E.H.B.) were reviewed retrospectively. Data regarding patient demographics, type of biodegradable fixation device, loss of fixation in the immediate and late postoperative period, inflammatory cyst formation of the fixation device, additional complications, and reoperation status were collected from the charts. Patients implanted with Endotine and Ultratine were compared postoperatively for early and late loss of elevation (efficacy), inflammatory cyst formation, additional complications, and reoperation rates. Results: Of the 276 fixation devices implanted (117 Endotine and 159 Ultratine), 11 inflammatory cysts were found, all of them in the Ultratine group (p = 0.0037). Four patients required reoperation due to early loss of fixation. Three of these cases were in the Endotine group, and 1 case was observed in the Ultratine group (p = 0.18). A total of 4 patients had late loss of fixation, all of them in the Ultratine group (p = 0.08). Scalp hypoesthesia and/or dysesthesia beyond the sixth postoperative month were observed in 3.62% of the cases. Postsurgical neuralgia was observed in 2.17%, and focal alopecia was seen in 1.44%. Conclusions: The presence of inflammatory cysts with the implantation of the Ultratine device is statistically higher than with Endotine. No statistical significant difference was found in either early or late fixation loss between the 2 groups.

Cutaneous melanoma metastatic to the orbit: review of 15 cases.

Purpose: To describe the clinical features, management, and outcomes of 15 patients with cutaneous melanoma metastatic to the orbit. The authors review emerging treatments for metastatic melanoma and their ocular implications. Methods: Retrospective chart review of 15 patients with orbital metastasis from cutaneous melanoma. Results: At presentation of the orbital metastasis, systemic metastatic cutaneous melanoma was present in 13 (87%) patients. The mean interval from diagnosis of cutaneous melanoma to orbital metastasis was 40 months (median, 37 months; range, 0–117 months). The most common presenting signs were dysmotility (63%), proptosis (56%), and blepharoptosis (19%). Four patients (25%) presented with pain. Metastasis involved extraocular muscle in 6 orbits (35%), intraconal space in 4 (24%), extraconal space in 7 (41%), and lacrimal sac in 1 (6%). The tumor was unifocal in all cases, unilateral in 13 patients (87%), and bilateral in 2 (13%). The mean tumor basal dimension was 20 × 20 mm and mean thickness was 16 mm. Treatments included complete surgical excision in 1 patient (6%), external beam radiotherapy (EBRT) in 7 (47%), systemic chemotherapy in 8 (53%), and immunotherapy in 5 (33%). Orbital tumor control was achieved in 2 orbits (18%) following focal therapy alone (excision or EBRT), 4 (36%) following systemic therapy alone (chemotherapy or immunotherapy), and 3 (27%) following combination focal plus systemic therapy. Three patients required exenteration. Survival rates at 1 year/2 years were 100%/0% following focal therapy, 50%/25% following systemic therapy, and 100%/66% following combination therapy. Conclusions: Cutaneous melanoma metastatic to the orbit tends to involve muscle (35%) or intraconal soft tissue (24%) as a painless (75%), circumscribed (87%) mass. Treatment with systemic chemotherapy and/or immunotherapy resulted in orbital tumor control in 80% of cases. Overall survival was 25.1 months.

The relationship between sex and symmetry in thyroid eye disease

Purpose To examine the relationships between sex and symmetry in the context of disease activity, severity, and thyroid status in thyroid eye disease. Methods Retrospective chart review of 31 men and 31 women with untreated thyroid eye disease. Subjective complaints, smoking status, thyroid status, and objective findings pertinent to the clinical activity score (CAS) and “NO SPECS” classification were recorded. Overall disease asymmetry was defined as having simultaneous asymmetry of both more than one symptom and more than one external finding. Asymmetry was compared across sex and thyroid status. CAS and NO SPECS severity were compared across sex, symmetry, and thyroid status. Results Asymmetric appearance was reported by 58% of men and 19% of women. Asymmetric proptosis (>2 mm difference) was seen in 45% of men and 23% of women (P=0.036). Overall asymmetry was seen in 55% of men and 19% of women (P=0.017). Thyroid status and sex had a combined effect on symmetry, as 15 of 16 hyperthyroid females (94%) demonstrated symmetric disease. Average NO SPECS severity was 3.5 (standard deviation [SD] 1.4) in men and 3.3 (SD 1.1) in women (P=0.51), and was 3.8 (SD 1.4) in asymmetric patients versus 3.2 (SD 1.3) in symmetric patients (P=0.08). The CAS was higher in asymmetric than symmetric patients (1.84 versus 0.97; P=0.012). Conclusion Men demonstrated more asymmetric disease (proptosis and overall asymmetry) than women, while hyperthyroid females demonstrated more symmetry than euthyroid and hypothyroid males and females. NO SPECS severity score was unaffected by sex, thyroid status, or symmetry. Asymmetric patients demonstrated higher clinical activity scores.

Demodex Folliculitis Presenting as Periocular Vesiculopustular Rash

Abstract Purpose: To report an unusual case of Demodex folliculitis presenting as periocular vesiculopustular rash. Design: Case report. Results: A 68 year-old woman presented with a unilateral periocular rash that was initially treated by her primary ophthalmologist with topical steroids and antivirals. Slit-lamp examination revealed severe bilateral blepharitis, right greater than left, with waxy sleeves around the eyelashes. The diagnosis of Demodex infestation was considered. Treatment with daily lid scrub with polyhexamethylene biguanide (PHMB), 1,2-hexanediol and 1,2-octanediol (OCuSOFT PLUS) and erythromycin ointment twice a day resulted in complete resolution of the symptoms after 4 weeks. Conclusions: Ophthalmologists should be aware of Demodex and consider it in the differential diagnosis of periocular skin lesions.

Surgical Anatomy of the Eyelid

Proper diagnosis and management of eyelid disorders, both functional and cosmetic, hinge upon a thorough understanding of the location of critical eyelid structures and the anatomic relationships between them.