Histologic Evaluation of Nonvisual Afferent Sensory Upper Eyelid Proprioception.

PURPOSE: Recent research has suggested a possible role for proprioception in ipsilateral frontalis activation in the setting of ptosis; however, there has not been any robust histologic or anatomic evidence to support this theory. To further elucidate proprioceptive structures in the eyelid, this investigation uses validated histologic techniques to explore the presence of proprioceptive structures or afferent neural networks in the Levator Palpebrae Superioris (LPS) and Müller muscle. METHODS: Müller muscle and LPS samples were evaluated by a laboratory with extensive experience with the histology of extraocular muscle proprioception. Immunofluorescence and confocal laser scanning microscopy were used to analyze the tissue samples. RESULTS: Thirty-four Müller muscle samples and 10 LPS samples were analyzed. Golgi tendon bodies and muscle spindles were not identified in the Müller muscle and LPS samples. This result is expected in the Müller muscle given that these structures are not typically present in smooth muscle, but noteworthy in the skeletal muscle of the LPS. Previously undescribed synaptophysin-positive free nerve terminals within the intermuscular connective tissue of the Müller muscle were identified. CONCLUSIONS: The nerve terminals identified are anatomically consistent with free nerve endings present in the extraocular muscles that have been implicated in proprioception. These findings advance our current knowledge of the ultrastructure of Müller muscle and the LPS and suggest a possible mechanism for proprioception in the upper eyelid that may have a role in ipsilateral brow elevation in the setting of ptosis.The authors describe proprioception in the upper eyelid: A histologic analysis.

Comparison of Revision Rates of Anterior- and Posterior-Approach Ptosis Surgery: A Retrospective Review of 1519 Cases.

PURPOSE: To compare revision rates for ptosis surgery between posterior-approach and anterior-approach ptosis repair techniques. METHODS: This is the retrospective, consecutive cohort study. All patients undergoing ptosis surgery at a high-volume oculofacial plastic surgery practice over a 4-year period. A retrospective chart review was conducted of all patients undergoing posterior-approach and anterior-approach ptosis surgery for all etiologies of ptosis between 2011 and 2014. Etiology of ptosis, concurrent oculofacial surgeries, revision, and complications were analyzed. The main outcome measure is the ptosis revision rate. RESULTS: A total of 1519 patients were included in this study. The mean age was 63 ± 15.4 years. A total of 1056 (70%) of patients were female, 1451 (95%) had involutional ptosis, and 1129 (74.3%) had concurrent upper blepharoplasty. Five hundred thirteen (33.8%) underwent posterior-approach ptosis repair, and 1006 (66.2%) underwent anterior-approach ptosis repair. The degree of ptosis was greater in the anterior-approach ptosis repair group. The overall revision rate for all patients was 8.7%. Of the posterior group, 6.8% required ptosis revision; of the anterior group, 9.5% required revision surgery. The main reason for ptosis revision surgery was undercorrection of one or both eyelids. Concurrent brow lifting was associated with a decreased, but not statistically significant, rate of revision surgery. Patients who underwent unilateral ptosis surgery had a 5.1% rate of Hering's phenomenon requiring ptosis repair in the contralateral eyelid. Multivariable logistic regression for predictive factors show that, when adjusted for gender and concurrent blepharoplasty, the revision rate in anterior-approach ptosis surgery is higher than posterior-approach ptosis surgery (odds ratio = 2.08; p = 0.002). CONCLUSIONS: The overall revision rate in patients undergoing ptosis repair via posterior-approach or anterior-approach techniques is 8.7%. There is a statistically higher rate of revision with anterior-approach ptosis repair.

Atypical presentations of orbital cellulitis caused by methicillin-resistant Staphylococcus aureus.

PURPOSE: To evaluate the epidemiologic and clinical features of orbital cellulitis caused by methicillin-resistant Staphylococcus aureus (MRSA). DESIGN: Multicenter, retrospective case series. PARTICIPANTS: Fifteen patients with culture-positive MRSA orbital cellulitis. METHODS: All recent cases of orbital cellulitis at several hospitals and surgical centers were reviewed, and cases with culture-positive MRSA from aspirates were identified. The data collected and analyzed retrospectively included patient demographics, medical history, presenting sign, imaging results, surgical procedure performed, surgical culture results, visual acuity at presentation and last follow-up, and duration of antibiotics. MAIN OUTCOME MEASURES: Presenting sign, radiographic evidence of paranasal sinus disease, radiographic evidence of multiple orbital abscesses, presence or absence of antecedent upper respiratory infection, and final visual acuity. RESULTS: Fifteen cases were identified. The mean patient age was 31.9 years (standard deviation, 24.2 years). Lid swelling was the presenting sign in 14 of 15 patients. No patients had a preceding upper respiratory infection, and only 1 patient had antecedent eyelid trauma. Only 3 of 15 patients had documented adjacent paranasal sinus disease on imaging. Lacrimal gland abscess or dacryoadenitis was the presenting finding in 5 of 15 patients. Multiple orbital abscesses were identified in 4 of 15 patients by computed tomography or magnetic resonance imaging. Fourteen of 15 cases required surgical intervention. Four of 15 cases had loss of visual acuity to light perception or worse. All 4 of these cases had a delay in referral for surgical intervention. CONCLUSIONS: In these 15 patients with MRSA orbital cellulitis, the typical clinical setting of orbital cellulitis was absent; chiefly, there was no identified antecedent upper respiratory illness, nor was there a preceding traumatic injury. Lid swelling in the absence of recent upper respiratory illness, lacrimal gland focus, multiple orbital abscesses, and lack of adjacent paranasal sinus disease may be predictive factors that suggest MRSA as the causative organism of orbital cellulitis. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Ophthalmic methicillin-resistant Staphylococcus aureus infections: sensitivity and resistance profiles of 234 isolates.

To identify the sensitivity and resistance profiles of ophthalmic Methicillin-resistant Staphylococcus aureus (MRSA) in a large, diverse demographic. The electronic database of a large health maintenance organization was searched for patients who had an ophthalmic bacterial culture performed from 2002 to 2008 which grew MRSA. Data collected included culture source, sensitivity and resistance profiles, and whether the infection was community or nosocomially-acquired. In this retrospective study, not all isolates were tested for all the antibiotics. All isolates tested for vancomycin, trimethoprim/sulfamethoxazole, and rifampin were sensitive to these antibiotics and none were resistant. Gentamycin and chloramphenicol had the next highest sensitivity to resistance ratios, followed by tetracycline and trimethoprim (without sulfamethoxazole). Community acquired MRSA is becoming more frequent and the most common ophthalmic manifestation is eyelid involvement. The sensitivities to antibiotics of the ophthalmic MRSA isolates in this study are consistent with that seen in the literature.

Analysis of Varicella-Zoster Virus in Temporal Arteries Biopsy Positive and Negative for Giant Cell Arteritis.

IMPORTANCE: Giant cell arteritis (GCA) is the most common systemic vasculitis in elderly individuals. Diagnosis is confirmed by temporal artery (TA) biopsy, although biopsy results are often negative. Despite the use of corticosteroids, disease may progress. Identification of causal agents will improve outcomes. Biopsy-positive GCA is associated with TA infection by varicella-zoster virus (VZV). OBJECTIVE: To analyze VZV infection in TAs of patients with clinically suspected GCA whose TAs were histopathologically negative and in normal TAs removed post mortem from age-matched individuals. DESIGN, SETTING, AND PARTICIPANTS: A cross-sectional study for VZV antigen was performed from January 2013 to March 2015 using archived, deidentified, formalin-fixed, paraffin-embedded GCA-negative, GCA-positive, and normal TAs (50 sections/TA) collected during the past 30 years. Regions adjacent to those containing VZV were examined by hematoxylin-eosin staining. Immunohistochemistry identified inflammatory cells and cell types around nerve bundles containing VZV. A combination of 17 tertiary referral centers and private practices worldwide contributed archived TAs from individuals older than 50 years. MAIN OUTCOMES AND MEASURES: Presence and distribution of VZV antigen in TAs and histopathological changes in sections adjacent to those containing VZV were confirmed by 2 independent readers. RESULTS: Varicella-zoster virus antigen was found in 45 of 70 GCA-negative TAs (64%), compared with 11 of 49 normal TAs (22%) (relative risk [RR] = 2.86; 95% CI, 1.75-5.31; P < .001). Extension of our earlier study revealed VZV antigen in 68 of 93 GCA-positive TAs (73%), compared with 11 of 49 normal TAs (22%) (RR = 3.26; 95% CI, 2.03-5.98; P < .001). Compared with normal TAs, VZV antigen was more likely to be present in the adventitia of both GCA-negative TAs (RR = 2.43; 95% CI, 1.82-3.41; P < .001) and GCA-positive TAs (RR = 2.03; 95% CI, 1.52-2.86; P < .001). Varicella-zoster virus antigen was frequently found in perineurial cells expressing claudin-1 around nerve bundles. Of 45 GCA-negative participants whose TAs contained VZV antigen, 1 had histopathological features characteristic of GCA, and 16 (36%) showed adventitial inflammation adjacent to viral antigen; no inflammation was seen in normal TAs. CONCLUSIONS AND RELEVANCE: In patients with clinically suspected GCA, prevalence of VZV in their TAs is similar independent of whether biopsy results are negative or positive pathologically. Antiviral treatment may confer additional benefit to patients with biopsy-negative GCA treated with corticosteroids, although the optimal antiviral regimen remains to be determined.

Prevalence and distribution of VZV in temporal arteries of patients with giant cell arteritis.

OBJECTIVE: Varicella-zoster virus (VZV) infection may trigger the inflammatory cascade that characterizes giant cell arteritis (GCA). METHODS: Formalin-fixed, paraffin-embedded GCA-positive temporal artery (TA) biopsies (50 sections/TA) including adjacent skeletal muscle and normal TAs obtained postmortem from subjects >50 years of age were examined by immunohistochemistry for presence and distribution of VZV antigen and by ultrastructural examination for virions. Adjacent regions were examined by hematoxylin & eosin staining. VZV antigen-positive slides were analyzed by PCR for VZV DNA. RESULTS: VZV antigen was found in 61/82 (74%) GCA-positive TAs compared with 1/13 (8%) normal TAs (p < 0.0001, relative risk 9.67, 95% confidence interval 1.46, 63.69). Most GCA-positive TAs contained viral antigen in skip areas. VZV antigen was present mostly in adventitia, followed by media and intima. VZV antigen was found in 12/32 (38%) skeletal muscles adjacent to VZV antigen-positive TAs. Despite formalin fixation, VZV DNA was detected in 18/45 (40%) GCA-positive VZV antigen-positive TAs, in 6/10 (60%) VZV antigen-positive skeletal muscles, and in one VZV antigen-positive normal TA. Varicella-zoster virions were found in a GCA-positive TA. In sections adjacent to those containing VZV, GCA pathology was seen in 89% of GCA-positive TAs but in none of 18 adjacent sections from normal TAs. CONCLUSIONS: Most GCA-positive TAs contained VZV in skip areas that correlated with adjacent GCA pathology, supporting the hypothesis that VZV triggers GCA immunopathology. Antiviral treatment may confer additional benefit to patients with GCA treated with corticosteroids, although the optimal antiviral regimen remains to be determined.

The smooth surface tunnel porous polyethylene enucleation implant.

BACKGROUND AND OBJECTIVE: To describe early clinical results with the porous polyethylene smooth surface tunnel (SST) enucleation implant. PATIENTS AND METHODS: Uncontrolled, prospective interventional case series of patients undergoing enucleation with placement of the SST implant. This implant consists of a porous polyethylene sphere with a smooth anterior surface containing pre-drilled tunnels to facilitate direct suturing of the rectus muscles to the implant without use of an implant wrap. Postoperatively, socket healing was assessed, and prosthesis and socket motility were evaluated by the surgeon using an ordinal scale (0 = no motility to 4 = excellent motility). RESULTS: Thirty patients received the SST implant, with a mean follow-up of more than 23 months. Two cases of exposure occurred and were managed surgically without the need for explantation. Mean socket motility was 3.1 on a 0 to 4 ordinal scale, with mean prosthesis motility of 2.8. CONCLUSION: The SST implant provides satisfactory socket motility and is generally well tolerated in the anophthalmic socket without the need for wrapping material.

Trends in ophthalmic manifestations of methicillin-resistant Staphylococcus aureus (MRSA) in a northern California pediatric population.

PURPOSE: To determine pediatric clinical trends of ocular and periocular methicillin-resistant Staphylococcus aureus (MRSA) in a large northern California healthcare system. METHODS: This study was a retrospective cross-sectional review of all pediatric cases (aged 0-18) with culture-positive ophthalmic MRSA isolates identified between January 2002 and December 2009. Medical record review included history, presentation, infection site, acquisition (community or nosocomial), antibiotic sensitivity/resistance, treatment, and clinical outcome. Incidence was classified by year, sex, and age. Parameters were analyzed for statistical significance by trend and χ(2) analysis. RESULTS: A total of 399 ocular and periocular MRSA cases were included. Cases trended upward from 2002 to 2009, peaking in 2006. Of the 137 pediatric cases (0-18 years), 58% were community acquired. Conjunctivitis was the predominant presentation (40%), followed by stye/chalazion (25%), orbital cellulitis/abscess (19%), dacryocystitis (11%) and brow abscess (3%). Significant predictors for ocular infection with MRSA included male sex (61%), neonates (38%), and multiple infection sites on the body (38%). Resistance was high to bacitracin (80.9%) and ofloxacin (48.3%) but remained low for trimethoprim/sulfamethoxazole (8.7%). Topical therapy was effective in 29% of cases; oral antibiotics, in 47%. Intravenous therapy was required in 12% of cases and incision/drainage or surgery in 19%. Initial oral antibiotic treatment, primarily cephalosporins (24%), was ineffective in 37% of patients. There was a significant increase in resistance to antibiotic therapy (P < 0.001) during the study period. No patients developed permanent visual impairment. CONCLUSIONS: Pediatric ocular and periocular MRSA is increasing in incidence and resistance in our patient population. Outcomes can be improved by early recognition, proper antibiotic selection, and obtaining cultures and sensitivities when resistant or severe ocular infections are present.

Broad fascia fixation enhances frontalis suspension.

PURPOSE: To report the use of a modified frontalis suspension procedure for congenital ptosis patients with minimal to no levator function. METHODS: Retrospective, non-comparative case series of frontalis suspension by 2 surgeons over a 21-year period. RESULTS: Frontalis suspension with broad fascia fixation was performed on 48 eyelids of 25 patients. All patients had visual obscuration due to blepharoptosis with only 0 mm to 2 mm of measurable levator function. Age ranged from 3 to 13 years. Minimum length of follow-up was 6 months (range, 6-174 months). The palpebral fissure was increased in all patients, improving their head position and unmasking their visual axis in primary gaze. Postoperatively, all patients developed transient exposure symptoms. There were no migrations, extrusions, infections, or granulomas. All patients achieved a satisfactory postoperative result based on eyelid position, function, and contour. CONCLUSIONS: This modified frontalis suspension procedure maximizes frontalis muscle recruitment, creating powerful eyelid elevation with stable effect over time. The technique minimizes other procedures' pitfalls and advances in cosmesis are achieved with enhanced eyelid crease formation and adjustable eyelid contour.

Hyaluronic acid gel (Restylane) filler for facial rhytids: lessons learned from American Society of Ophthalmic Plastic and Reconstructive Surgery member treatment of 286 patients.

PURPOSE: To review injection techniques and patient satisfaction with injection of Restylane in various facial areas by American Society of Ophthalmic Plastic and Reconstructive Surgery members. METHODS: Data from 286 patients treated with Restylane in nine American Society of Ophthalmic Plastic and Reconstructive Surgery practices were abstracted to a spreadsheet for analysis. RESULTS: Nine practices performed Restylane injections for 8.8 months on average (range, 2 to 28 months). Average practice volume per patient was 1.2 ml (range, 0.7 to 2.1 ml). Nine of nine practices injected the nasolabial and melolabial folds, 9 of 9 practices injected the lips, and 6 of 9 injected the glabella. Only 2 of 9 practices injected other fillers concurrently. Botox was injected concurrently by 8 of 9 practices. On a scale of 1 to 10, physicians rated average patient discomfort during Restylane injection 4.6 with topical anesthesia and 2.1 with injectable lidocaine, with or without topical anesthesia. The end point for injection was determined by visual cues, volume of injection, extrusion of the product, and palpation. "Problematic" complications, including bruising, swelling, bumpiness, and redness each had an incidence of 5% or less. Patient satisfaction on a scale of 1 to 10 had an average rating of 8.1, compared with that of Botox injection (8.9), upper blepharoplasty (8.9), and collagen injection (6.6). The source of Restylane patients was estimated to be existing Botox patients (45%); existing non-Botox patients (18%); word of mouth (14%); and new patients for other services (13%). CONCLUSIONS: Injection techniques, volume, end points, and anesthesia vary for different facial areas and between practices. Patients experience mild to moderate injection discomfort that is lessened with injectable lidocaine. Self-limited problems occur in about 5% of patients. Physician-determined patient satisfaction is perceived to be higher than that of collagen injection but slightly lower than that of botulinum toxin injection. The major source of Restylane patients was from existing practice patients, especially botulinum toxin patients.

Orbital rhabdomyosarcoma metastatic to the contralateral orbit: a case report.

OBJECTIVE: To report a rare presentation of metastatic orbital rhabdomyosarcoma and the corresponding findings on magnetic resonance imaging (MRI). DESIGN: Interventional case report. RESULTS: A 29-year-old white man was diagnosed with rhabdomyosarcoma of the left sinus and orbit for which he was treated with chemotherapy and radiation. Eighteen months after diagnosis, he returned with subacute right eye pain and dysmotility of his extraocular muscles. MRI revealed solitary enlargement of the right medial rectus muscle, and thyroidopathy was suspected. Over the next 2 months, symptoms progressed, and proptosis developed. MRI showed infiltration of seven extraocular muscles. A biopsy of right orbital tissues and the right medial rectus muscle was performed. Special tissue typing confirmed metastatic alveolar rhabdomyosarcoma. The patient underwent palliative radiation therapy and chemotherapy, but he ultimately died of disseminated disease. CONCLUSIONS: Rhabdomyosarcoma can rarely metastasize to the extraocular muscles. Earlier recognition of orbital metastasis through radiographic and biopsy findings, along with prompt and aggressive treatment, may prevent fulminant spread of rhabdomyosarcoma.

The role of supraorbital nerve biopsy in cutaneous malignancies of the periocular region.

PURPOSE: To describe clinical situations in which a biopsy or resection of the supraorbital nerve may play a role in patients in whom perineural invasion secondary to cutaneous head and neck malignancies is suspected. METHODS: The clinical records of 230 patients with squamous cell carcinoma (SCCA) of the skin of the head and neck who were treated at the University of Texas M.D. Anderson Cancer Center between April 1994 and March 2001 were reviewed. Thirty-five patients were identified as having primary lesions on the forehead skin. Of these, 8 patients had microscopic or clinical evidence of perineural invasion. Four of these 8 patients had undergone a supraorbital nerve biopsy or resection in the course of their treatment. Although all 4 patients were classified as having had SCCA, 1 patient had a variant of undifferentiated SCCA that more appropriately should have been classified as lymphoepithelioma-like carcinoma. The clinical history was reviewed in each case. The median follow-up time for this cohort was 47 months (range, 24 to 72 months). RESULTS: The 4 cases reported here illustrate that a supraorbital nerve biopsy can help confirm the diagnosis of perineural invasion, identify the extent of tumor infiltration, and help in staging of particularly aggressive cutaneous malignancies of the periocular region. Access to the proximal (orbital) portion of the nerve can be accomplished through an anterior orbitotomy. CONCLUSIONS: A biopsy of the peripheral branches of the trigeminal nerve may be indicated as part of the staging or to confirm the diagnosis of perineural invasion in patients with SCCA or other locally aggressive cutaneous malignancies of the head and neck.

Feasibility of preoperative lymphoscintigraphy for identification of sentinel lymph nodes in patients with conjunctival and periocular skin malignancies.

PURPOSE: To determine the feasibility of preoperative lymphoscintigraphy for identification of sentinel lymph nodes (SLNs) in patients with conjunctival and periocular skin tumors and to determine the patterns of lymphatic drainage from such tumors. METHODS: We retrospectively reviewed the records of all patients with biopsy-confirmed conjunctival and periocular skin malignancies who underwent lymphoscintigraphy with or without SLN biopsy between January 1999 and June 2000. Patients underwent lymphoscintigraphy with 0.3 to 1 mCi of technetium Tc-99m sulfur colloid in a volume of either 0.2 mL or 1 mL. Images were taken as soon as the first SLNs were detected through the camera and every 15 minutes thereafter. Intraoperative mapping and SLN biopsy was performed 1 to 2 days after lymphoscintigraphy unless the patient refused or there were medical contraindications to the procedure. RESULTS: The study included 7 patients with malignant melanoma of the conjunctiva or periocular skin and 1 patient with Merkel cell carcinoma of the eyelid. On lymphoscintigraphy, at least 1 SLN was identified in 7 of the 8 patients. Although all lesions located in the lateral half of the ocular adnexa drained to at least one SLN in the parotid (preauricular) area, there was some variability in the drainage patterns of lesions located in the medial half of the ocular adnexa. A smaller injection volume (0.2 mL) was adequate for detecting the nodes draining the area of injection and led to less spread of technetium to the surrounding areas. Six patients underwent SLN biopsy. In all but one, the nodes identified during surgery corresponded with those visualized on lymphoscintigraphy. CONCLUSIONS: Preoperative lymphoscintigraphy successfully identifies SLNs in most patients with conjunctival and periocular skin malignancies. Smaller injection volumes (0.2 mL) appear to be adequate for identification of the sentinel nodes and lead to less spread to surrounding tissues.

Use of bioglass for orbital volume augmentation in enophthalmos: a rabbit model (oryctolagus cuniculus).

PURPOSE: To investigate the clinical and histologic response of Novabone-C/M as an osteoproductive alloplastic implant for volume augmentation in the orbit in the treatment of enophthalmos and to compare its outcome alone versus its use in combination with autogenous bone or Medpor granules. METHODS: Novabone-C/M, a bioactive silicone glass material, was implanted in the subperiosteal space of the left orbit of 12 New Zealand White rabbits. The animals were divided into 3 groups, each with 4 animals, based on the material implanted in the orbit: group 1, Novabone alone; group 2, Novabone plus Medpor granules; and group 3, Novabone plus autogenous bone fragments. All rabbits were studied clinically, radiographically, and histologically at 1-, 3-, and 6-month intervals. Animals underwent preoperative and postoperative computed tomography (CT) with 3-dimensional reconstruction, proptosis measurements, and volumetric analysis. Orbit specimens were studied histologically with mineralized bone stain (MIBS) to look for bone formation, reactivity, infection, implant resorption, and migration. RESULTS: There were no signs of significant inflammation or infection. Subcutaneous migration of the implant was seen radiographically but not clinically in groups 1 and 3. Induced proptosis averaged 2.5 mm (at 1 month) and showed regression in all groups over a 6-month period but was not statistically significant. Implant volume was markedly reduced in all groups, averaging 69% in group 1, 37% in group 2, and 59% in group 3 at 6 months. New bone formation and bone remodeling was present in all 3 groups at 3 months and only in group 2 at 6 months. The rate and amount of implant remodeling and bone formation was greatest in the Novabone/Medpor group (group 2). CONCLUSIONS: Bioglass particulate is biocompatible, easy to use in the orbit, and stimulates bone growth. Bioglass is associated with volume loss and migration over 6 months and may not provide adequate volume augmentation in the orbit when used alone for the treatment of enophthalmos. The duration and amount of bone formation may be enhanced when Novabone is used in conjunction with Medpor.

Outcomes in patients with adenoid cystic carcinoma of the lacrimal gland.

PURPOSE: To evaluate the outcomes among patients with adenoid cystic carcinoma of the lacrimal gland treated at various stages of their disease at a tertiary care cancer center. METHODS: A retrospective case series of 20 patients with adenoid cystic carcinoma of the lacrimal gland treated at a single institution between 1952 and 2002. Clinical records were available for all 20 patients; histologic sections from 12 of the 20 patients were available for review. Disease-free survival was measured from the completion of treatment; overall survival was measured from the date of initial diagnosis. RESULTS: The study included 6 men and 14 women. The mean age at diagnosis was 39.5 years. The median follow-up time was 34 months (range, 6 to 264 months). The local/regional treatment modalities included exenteration with bone removal and radiation therapy (RT) in 5 patients, exenteration with RT (no bone removal) in 8 patients, exenteration (no RT or bone removal) in 1 patient, exenteration with bone removal (no RT) in 1 patient, local resection with RT in 3 patients, and local resection without RT in 2 patients. Overall, 16 patients had RT as part of their treatment regimen. Seven patients (35%) had local recurrence. Sixteen patients (80%) had distant metastasis during the study period. At the time of this report, 13 (65%) of the patients had died of disease. The median disease-free survival for the entire group was 18 months. Eight patients had a predominantly basaloid histologic pattern. Ten patients had verifiable histologic evidence of perineural invasion. CONCLUSIONS: This study underscores the generally grave prognosis for patients with adenoid cystic carcinoma of the lacrimal gland and the difficulty in making any conclusive recommendations for local therapy for this disease.