Surgical correction of blepharoptosis in patients with myasthenia gravis.

PURPOSE: To describe the results of surgical correction of blepharoptosis in a series of patients with myasthenia gravis (MG). METHODS: In this retrospective case series, we reviewed the medical records of all patients with MG who did not respond to medical therapy and underwent surgical correction for blepharoptosis at the Mayo Clinic between 1985 and 1999. The primary outcome measure was change in interpalpebral eyelid fissure height. RESULTS: Sixteen blepharoptosis procedures were performed on 10 patients with MG. Eight of the 10 patients had ocular MG. Two of the 10 patients had systemic MG. Of the 16 procedures performed, 9 were external levator advancements (ELA), six were frontalis slings, and one was a tarsomyectomy. Patients were followed postoperatively for an average of 34 months (range, 14-126 months). The amount of ptosis was quantified pre- and postoperatively for seven of the nine eyelids that underwent ELA. For these seven eyelids (five patients), there was a statistically significant improvement in the mean interpalpebral eyelid fissure height from 3.7 mm preoperatively to 7.8 mm postoperatively, with a mean difference of 4.1 mm (95% confidence interval 1.9 mm to 6.25 mm, p = 0.0038). Postoperative complications included worsened diplopia in one patient with ELA and exposure keratopathy in one patient with frontalis sling. Two of the ELA eyelids developed recurrent ptosis requiring additional surgery more than 2 years after the initial procedure. CONCLUSIONS: Blepharoptosis surgery can achieve eyelid elevation in patients who have failed to respond to medical therapy for MG. Potential complications include worsened diplopia and exposure keratopathy.

Surgical correction of blepharoptosis in patients with myasthenia gravis.

PURPOSE: To describe the results of surgical correction of blepharoptosis in a series of patients with myasthenia gravis. METHODS: We reviewed the medical records of all patients with myasthenia gravis who underwent surgical correction for blepharoptosis at the Mayo Clinic between 1985 and 1999. The primary outcome measure was change in interpalpebral eyelid fissure height. RESULTS: Eighteen blepharoptosis procedures were performed on 11 patients with myasthenia gravis. Eight of the 11 patients had ocular myasthenia gravis, and 3 had systemic myasthenia gravis. Of the 18 procedures performed, 11 were external levator advancements (ELA), 6 were frontalis slings, and 1 was a tarsomyectomy. Patients were followed up postoperatively for an average of 34 months (range, 9 to 126 months). The amount of ptosis was quantified preoperatively and postoperatively for 9 of the 11 eyelids that underwent ELA. For these eyelids, there was a statistically significant improvement in the mean interpalpebral eyelid fissure height, from 4.2 mm preoperatively to 8.1 mm postoperatively, with a mean difference of 3.9 mm (95% confidence interval, 2.3 to 5.5 mm; P = .0005). Postoperative complications included worsened diplopia in 1 patient who underwent ELA and exposure keratopathy in 1 patient who underwent a frontalis sling procedure. Two of the eyelids that underwent ELA developed recurrent ptosis, requiring additional surgery more than 2 years after the initial procedure. CONCLUSION: Surgical correction of blepharoptosis is an appropriate treatment option in patients with myasthenia gravis who fail medical therapy. Potential complications include worsened diplopia and exposure keratopathy.

Follow-up of patients with essential blepharospasm who underwent eyelid protractor myectomy at the Mayo Clinic from 1980 through 1995.

PURPOSE: To determine the efficacy of eyelid protractor myectomy (subtotal excision of the orbicularis oculi, the corrugator supercilii, and the procerus muscles) for the treatment of essential blepharospasm, and to evaluate the need for and the effectiveness of botulinum toxin (BT) injections in these patients. METHODS: The medical records of all patients who underwent eyelid protractor myectomy at the Mayo Clinic (Rochester, MN) from 1980 through 1995 were reviewed. The Health Status Questionnaire was used to assess overall medical and mental health, and a questionnaire specific to eyelid spasms was developed. RESULTS: Fifty-four white patients, of whom 32 (59%) were women, underwent myectomy. The average age at diagnosis of essential blepharospasm was 64 years (median, 65 years; range, 43 to 84 years), whereas the average age at the time of myectomy was 66 years (median, 66 years; range, 51 to 85 years). Of the 14 patients who were treated with BT injections before myectomy, the average interval between the initial injection and surgery was 21 months (median, 20 months; range, 2 to 51 months). Patients who had been treated with BT injections before myectomy were more likely to receive injections postoperatively than were those patients who had not been treated with BT (p < 0.001). Twenty patients were treated with BT injections after myectomy; the overall probability of receiving BT five years after surgery was 46%. Time from myectomy to treatment with BT varied considerably; mean, 880 days; median, 659 days; range, 3 to 4221 days. Postoperative follow-up for those patients who did not receive BT after myectomy ranged from 2 to 5935 days (mean, 2354 days; median, 1722 days). Although the probability of receiving BT injections after myectomy was not associated with age or sex, there was a significant association with the time interval during which the myectomy had been performed (related to the availability of BT as an adjunctive therapy). Of the 41 patients who were alive when the study was conducted, 32 (78%) completed a follow-up survey. Thirty of those (94%) said myectomy provided short-term and long-term benefits. Of the 11 patients who received BT injections before and after myectomy, six (55%) said the toxin was more effective in ameliorating eyelid spasms after surgery and four (36%) required injections less frequently after myectomy. Results from the Health Status Questionnaire showed no significant differences between patients who underwent myectomy and control subjects. CONCLUSIONS: Eyelid protractor myectomy provides subjective benefit to patients with essential blepharospasm and decreases the long-term need for BT injections in approximately 50% of these patients. Although the probability of receiving postoperative BT paralleled its availability, patients who received both preoperative and postoperative BT perceived either increased efficacy of the toxin injections, longer-lasting effects, or both, after myectomy. Patients with severe disability from blepharospasm benefited more from myectomy than did patients with relatively mild symptoms.

Unusual orbital foreign bodies.

PURPOSE: To describe the clinical features of patients with unusual orbital foreign bodies. METHODS: The clinical histories, preoperative photographs, imaging studies, and surgical pathologic findings of five patients with unusual orbital foreign bodies are presented. Additionally, published reports pertaining to similar cases were reviewed. RESULTS: The five patients treated by the authors had good outcomes. CONCLUSIONS: An orbital foreign body may be overlooked because a small penetrating wound may be accompanied by minimal or no signs of inflammation early in the clinical course. A careful history and physical examination, imaging studies, and a strong suspicion are helpful for establishing the diagnosis of an orbital foreign body.

Mental health-related calls to the CDC National AIDS Hotline.

The CDC National AIDS Hotline provides confidential HIV-related information and referrals to anonymous callers, twenty-four hours a day. As part of a continuing quality improvement assessment of caller informational needs, 302 randomly selected anonymous overnight calls to the Hotline were evaluated for mental health-related content. Of 302 calls, 34 calls (11.3%) were mental health-related, in that callers spoke about specific mental health-related topics or requested mental health referrals, and 14 calls (4.6%) included signs or symptoms from the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV) potentially indicative of mental illness. The results suggest that training in recognizing and referring mental health calls might be useful for Hotline workers. The results also serve as a reminder for all health care professionals and organizations of the potential for overlap between patients' mental health-related needs and patients' HIV-related needs.

National AIDS Hotline: HIV and AIDS information service through a toll-free telephone system.

The National AIDS Hotline (NAH), a service of the Centers for Disease Control (CDC), is an information resource for the population of the United States, its Territories, and Puerto Rico concerning the human immunodeficiency virus (HIV) and acquired immunodeficiency syndrome (AIDS). Since its inception in 1983, NAH has grown to be the world's largest health-related hotline service. NAH has received an average of more than 1.4 million calls per year since October 1987. Services of NAH include responding to the public's questions about HIV and AIDS and providing referrals to State and local resources. All services, including HIV and AIDS publications, are provided free of charge. The public contacts NAH 24 hours a day, 7 days a week, through a toll-free telephone system. Services are available to English-speaking, Spanish-speaking, and deaf populations. Each service has its own telephone number--English-speaking, 1-800-342-2437; Spanish-speaking, 1-800-344-7432; TTY service for the deaf, 1-800-243-7889. NAH employs approximately 170 information specialists to answer calls. The facility uses modern telecommunications technology to effectively manage and direct calls to 43 work stations. Each work station is supported by a personal computer that allows access to CDC's National AIDS Clearinghouse data bases for referrals and publication ordering. NAH ensures that information provided to the public is current, accurate, and consistent with approved government policy. Quality assurance reviews address call management, delivery of information, and content of calls.

Optic nerve sheath meningoceles. Clinical and radiographic features in 13 cases with a review of the literature.

Thirteen patients with dilated intraorbital optic nerve sheaths with an expanded, patulous cerebrospinal fluid (CSF) space were studied with high-resolution computed tomography (CT) or magnetic resonance imaging (MRI). Eleven patients had bilateral findings. Headache or visual complaints, or both, were present in all patients. Signs of optic nerve dysfunction were present in eight patients. Three patients had visual acuity worse than 20/200. Cerebrospinal fluid pressure was mildly elevated in two patients. Three patients underwent a surgical procedure; visual acuity improved in one. The authors propose the term meningocele for this condition and suggest MRI with fat-suppression techniques and off-axis sagittal views as the radiographic procedure of choice.

Treatment of blepharospasm with botulinum toxin.

Many therapeutic modalities, including medications, excision of the muscles used in closure of the eyelids (myectomy), and selective extirpation of branches of the facial nerve (neurectomy), have been used for the management of blepharospasm. Because of limited effectiveness and undesirable side effects, none of these treatments has been completely satisfactory. Recent reports about injection of botulinum toxin indicate that it is safe and effective for most patients. Relief from blepharospasm, however, is usually transient, and repeated injections are usually necessary. The current availability of effective therapy for blepharospasm emphasizes the importance of prompt diagnosis and referral of affected patients to physicians knowledgeable in the use of botulinum toxin and other therapeutic approaches.

Orbital exenteration at the Mayo Clinic. 1967-1986.

Orbital exenteration was performed in 102 patients at the Mayo Clinic during the 20-year period from 1967 through 1986. The surgical procedure was performed for mucormycosis in one patient and for pain and deformity after a severe facial burn in another; in the remaining 100 patients, exenteration was used to treat a neoplastic disorder. Although 19 different neoplasms were encountered, squamous cell carcinoma, basal cell carcinoma, and melanoma constituted 70% of the total. In 82 patients with no known residual tumor or metastases at operation, the 1-year survival rate was 88.6%, the 5-year rate was 56.8%, and the 5-year rate free of recurrence or metastases was 48.3%. In 18 patients with known residual tumor or metastases at exenteration, 55.0% were alive 1 year postoperatively, and the 5-year survival rate was 25.8%. Unusual findings in this series included two patients with metastatic basal cell carcinoma and one patient with a metastatic thyroid Hürthle cell carcinoma.

Isolated metastasis to optic nerve from medulloblastoma.

Isolated involvement of the optic nerve with metastatic tumor is uncommon. A 19-year-old man had a midline cerebellar medulloblastoma; a gross total removal was performed. He received postoperative radiation therapy to the whole brain, posterior fossa, and craniospinal axis. A progressive optic neuropathy developed 28 months later with radiologic evidence of an enlarged optic nerve. There was no evidence of metastatic disease elsewhere. An optic nerve biopsy showed metastatic medulloblastoma. An intramedullary metastasis developed 48 months after the primary diagnosis, and the patient died 5 months later.

Ocular pigmented spots and eyelid myxomas.

Ophthalmic abnormalities are part of a recently recognized syndrome that includes myxomas, spotty pigmentation, and endocrine overactivity. The complex is familial with mendelian dominant mode of inheritance. The 63 patients (27 males and 36 females) we have identified with this complex had the following ophthalmic abnormalities: facial and eyelid lentigines in 44 patients (70%); pigmented lesions on the caruncle or conjunctival semilunar fold in 17 patients (27%); and eyelid myxomas in ten patients (16%). Because these findings usually preceded the most serious component of the syndrome, cardiac myxoma, early recognition of their significance is important.

Myectomy for essential blepharospasm.

Essential blepharospasm is a progressive, bilateral, involuntary facial spasm. In this study, we conducted a prospective analysis of myectomy for the treatment of this condition in 21 patients. The procedure consisted of extirpation of the muscles that close the eyelids (protractors), which include the pretarsal, preseptal, and orbital components of the orbicularis oculi, as well as the procerus and corrugator supercilii muscles of the brow. In addition, the muscles that open the eyelids (retractors) were strengthened (by resection of the frontalis muscle and repair of disinsertion of the levator aponeuroses). The most common symptoms that necessitated myectomy were difficulty with driving (20 patients), watching television (17 patients), reading (15 patients), and eating (5 patients). Overall, the 21 patients had a 70% mean decrease in symptoms postoperatively. Five patients required an additional operation for management of residual blepharospasm.

A clinicopathologic study of primary adenoid cystic carcinoma of the lacrimal gland.

A retrospective study of 26 patients with the diagnosis of primary adenoid cystic carcinoma of the lacrimal gland was performed with use of randomly selected tissue from each tumor. An attempt was made to determine any associations among clinical presentation, survival, and tumor histologic patterns. We found that lower tumor grades were associated with a predominantly Swiss-cheese (cribriform) pattern. Both lower tumor grade and Swiss-cheese pattern were associated with longer survival. The basaloid (solid) pattern or bone invasion was not associated with a shorter survival. Between men and women, there were no differences in clinical presentation, histologic patterns, or survival. Survival was not affected by surgical or radiation therapy.

Management of blepharospasm.

From 1950 through 1984, 123 patients underwent surgical treatment of blepharospasm at the Mayo Clinic. During this period, four different operations (proximal and distal neurectomy and two types of myectomy) were used. Significant recurrent or residual blepharospasm was observed more frequently (P less than 0.01), and need for subsequent operations was greater (P less than 0.01), among patients who had undergone distal neurectomy than among those who had had myectomy. These data support the view that myectomy is a more effective procedure than neurectomy. Initial results with botulinum toxin injection seem to indicate that it is an effective short-term treatment for blepharospasm. However, its long-term efficacy and safety need further study, as does the role it should play in combination with myectomy.

Sebaceous carcinoma of the eyelid: pitfalls in diagnosis.

Sebaceous carcinoma of the eyelid and adnexa commonly simulates other entities both clinically and pathologically. Between 1905 and 1981, 43 patients with sebaceous carcinoma of the eyelid were seen at the Mayo Clinic. Prior to establishing the diagnosis of sebaceous carcinoma, patients commonly carried multiple clinical diagnoses (mean = 2.2) and multiple microscopic diagnosis (mean = 1.7). The most common clinical diagnosis was chalazion. The patients' average age at the onset of symptoms was 61.5, with females predominating. The upper eyelid was most commonly involved. The conjunctival surface was involved alone in a statistically greater number of patients with metastatic disease (P less than 0.05). Second primary malignancies and/or radiation exposure was found in 11 patients, or 25% of our series. Of the second primary malignancies, malignancies of the skin and breast were most common. Sebaceous carcinoma should be suspect in atypical presentations of common disorders and in cases in which clinical and pathologic findings are not well correlated. If sebaceous carcinoma of the eyelid is a diagnostic consideration, we recommend a frozen section with fat stain together with a full-thickness lid biopsy. Surveillance for second primary malignancies may be warranted.

Facial nerve akinesia.

In ophthalmology, increasing attention has been directed to methods of local anesthesia and akinesia that provide complete analgesia and immobilization of the eye conducive to work with the surgical microscope, particularly in operations for cataracts. The volume of anesthetic agent often used to achieve these goals may approach the toxic level of the drug in some patients. A method of producing akinesia of the temporal, zygomatic, and buccal divisions of the facial nerve that requires only a small volume of anesthetic is described. In our experience, this method can, in many cases, provide akinesia of the orbicularis oculi equal in effect to some established methods of infiltrative akinesia that require large doses of anesthetic.

Management of blepharospasm.

From 1950 through 1984, 123 patients underwent surgical treatment of blepharospasm at the Mayo Clinic. During this period, four different operations (proximal and distal neurectomy and two types of myectomy) were used. Significant recurrent or residual blepharospasm was observed more frequently (p less than 0.01) and need for subsequent operations was greater (p less than 0.01) among patients who had undergone distal neurectomy than among those who had had myectomy. These data support the view that myectomy is a more effective procedure than neurectomy. Initial results with botulinum toxin injection seem to indicate that it is an effective short-term treatment for blepharospasm. However, its long-term efficacy and safety need further study, as does the role it should play in combination with myectomy.