Oral methylphenidate for the treatment of refractory facial dystonias.

Oral methylphenidate (Ritalin, Novartis) has been reported to alleviate symptoms of benign essential blepharospasm in an off-label application. This series presents 3 patients with refractory periorbital and facial dystonias, including blepharospasm, apraxia of eyelid opening, and oromandibular dystonia unresponsive to standard treatments who experienced a response to oral methylphenidate therapy. While the mechanisms for facial dystonias have not been elucidated, there is evidence to suggest that they are on the spectrum with Parkinson disease. Given the role of dopamine loss in the pathogenesis of Parkinson, the authors' speculate that methylphenidate may be acting on the pathway directly involved in facial dystonias. To the authors' knowledge, this is the first report of a case of successful treatment of blepharospasm refractory to upper eyelid myectomy with methylphenidate monotherapy.

Lateral Transorbital Endoscopic Access to the Hippocampus, Amygdala, and Entorhinal Cortex: Initial Clinical Experience.

BACKGROUND/AIMS: Transorbital approaches traditionally have focused on skull base and cavernous sinus lesions medial to the globe. Lateral orbital approaches to the temporal lobe have not been widely explored despite several theoretical advantages compared to open craniotomy. Recently, we demonstrated the feasibility of the lateral transorbital technique in cadaveric specimens with endoscopic visualization. We describe our initial clinical experience with the endoscope-assisted lateral transorbital approach to lesions in the temporal lobe. METHODS: Two patients with mesial temporal lobe pathology presenting with seizures underwent surgery. The use of a transpalpebral or Stallard-Wright eyebrow incision enabled access to the intraorbital compartment, and a lateral orbital wall 'keyhole' opening permitted visualization of the anterior temporal pole. RESULTS: This approach afforded adequate access to the surgical target and surrounding structures and was well tolerated by the patients. To the best of our knowledge, this report constitutes the first case series describing the endoscope-assisted lateral transorbital approach to the temporal lobe. We discuss the limits of exposure, the nuances of opening and closing, and comparisons to open craniotomy. CONCLUSION: Further prospective investigation of this approach is warranted for comparison to traditional approaches to the mesial temporal lobe.

Traumatic arteriovenous fistula of the superficial temporal artery: a histopathologic report.

Traumatic aneurysms, pseudoaneurysms, and arteriovenous (AV) fistulas of the superficial temporal artery (STA) are uncommon, accounting for only 0.5% to 2.0% of all aneurysms subjected to surgery. In the literature that relates to the STA, the general term "aneurysm following traumatic injury" often includes a wide array of pathologic entities, including proper aneurysms, pseudoaneurysms, and AV fistulas. In 75% of cases, such aneurysms are due to blunt trauma. Within this group, STA AV fistulas constitute an exceedingly rare subset. The case presents the occurrence of a traumatic AV fistula of the STA following blunt trauma (karate kick) and provides the first description of the histologic appearance of this lesion.

Blepharospasm and apraxia of eyelid opening associated with anti-Hu paraneoplastic antibodies: a case report.

PURPOSE: To report a case of blepharospasm associated with anti-Hu paraneoplastic antibodies that was treated successfully with botulinum toxin A. DESIGN: Case report. PARTICIPANTS: A 57-year-old man had altered mental status and a 20-pound weight loss at presentation. Evaluation revealed an occult small-cell lung cancer. Despite initiating appropriate chemotherapy, his mental status worsened and over the course of several weeks, he was unable to open his eyes because of forceful orbicularis contractions. Neuroimaging and cerebrospinal fluid studies found no evidence of intracranial metastases. However, his paraneoplastic panel was positive for anti-Hu antibodies. He was diagnosed with paraneoplastic encephalitis and blepharospasm. INTERVENTION: Intravenous Solu-Medrol (Pharmacia & Upjohn Co, Bridgewater, NJ) and periocular injections of botulinum toxin A. MAIN OUTCOME MEASURES: Ocular disease control. RESULTS: Intravenous Solu-Medrol improved his mental status, but did not change his ocular symptoms. Subsequent botulinum toxin A injections allowed spontaneous eyelid opening. CONCLUSIONS: Although paraneoplastic blepharospasm is rare, it is an important diagnosis to be aware of because paraneoplastic disorders often herald an occult tumor. This is the only case of paraneoplastic blepharospasm that the authors know of that was the result of anti-Hu antibodies as well as the only case that was treated with botulinum toxin A.

Optic nerve biopsy in the management of progressive optic neuropathy.

BACKGROUND: In cases of progressive optic neuropathy, diagnostic uncertainty often persists despite extensive work-up. Optic nerve biopsy (ONB) can be considered, especially when visual decline of the affected or fellow eye ensues despite empiric therapy. We aimed to evaluate both diagnostic and therapeutic utilities of ONB based on the long-term experience at a tertiary care institution. METHODS: This was a retrospective chart review of biopsies over 20 years at a single institution involving intrinsic or adherent optic nerve masses. Main outcome measures included the impact of tissue sampling on reaching a diagnosis and on guiding treatment. Secondary measures included vision in the eye of the ONB and the fellow eye. RESULTS: Fifteen patients with a mean age of 51.7 ± 17.4 years underwent biopsies. At the time of biopsy, visual acuity was no light perception in 8 (53%) eyes, light perception to counting fingers in 5 (33%), and 20/400 or better in 2 (13%). The fellow eye of 7 patients (47%) experienced some degree of sequential vision loss before biopsy. Seven specimens included en bloc biopsy of the nerve, 7 contained the dural sheath (usually with a portion of the optic nerve), and 1 only of the compressive mass. Six patients (40%) had tumors. Six of 8 inflammatory lesions biopsied required further clinical data to arrive at specific diagnoses. In one case, a clinical diagnosis could not be made. No patients experienced further vision loss in the fellow eye at last follow-up (median, 8 months). CONCLUSIONS: In diverse circumstances of progressive optic neuropathy, ONB can be beneficial in establishing the diagnosis. ONB can help direct specific local or systemic treatment, particularly when infectious or inflammatory etiologies are identified. ONB, if considered early in the disease course, can potentially halt or prevent vision loss when the fellow eye is threatened.

Presence of Erdheim-Chester disease and Langerhans cell histiocytosis in the same patient: a report of 2 cases.

The histiocytic disorders Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD), can both present with multisystem involvement, with the central nervous system and the bone, skin, neuroendocrine, cardiac, respiratory, and gastrointestinal systems potentially affected. The 2 entities occasionally can be difficult to distinguish. Both rarely affect the orbit and the central nervous system, and although there are rare reports of patients with coexistent LCH and ECD, there are no reported cases of the 2 diseases that involve both the orbital and neuroendocrine systems. We report 2 such cases, and review the literature of cases of LCH and ECD occurring in the same patient. The presentation of LCH and ECD in certain patients suggests a possible abnormality in the common CD34 progenitor cell. The coexistence of the 2 disease states should be suspected in patients with atypical presentations of either disorder.

Orbital lymphoma in the setting of idiopathic CD4+ lymphocytopenia (HIV-negative AIDS).

A 43-year-old Asian man with idiopathic CD4+ lymphocytopenia (human immunodeficiency virus [HIV]-negative acquired immunodeficiency syndrome [AIDS]) noted blurred vision OD. Visual acuity was 20/30 OD and 20/15 OS. The right eye showed features of axial proptosis (2 mm), blepharoptosis, upgaze restriction, conjunctival chemosis, color desaturation, and choroidal folds. The OS was unremarkable. There was no lymphadenopathy. Orbital MRI showed a well-circumscribed mass indenting the globe. Excision revealed diffuse large-B-cell lymphoma. Systemic evaluation was negative and adjuvant external beam radiotherapy to the right orbit was performed. Idiopathic CD4+ lymphocytopenia is a rare hematologic condition characterized by depletion of CD4+ T cells in HIV-negative patients. This condition closely resembles AIDS but HIV serology is negative. Similar to AIDS, patients are at risk for opportunistic infections and neoplasia, including systemic lymphoma. This is the first case, to the authors' knowledge, of orbital lymphoma with idiopathic CD4+ lymphocytopenia.

Tarsal dermoid cyst: clinical presentation and treatment.

The authors present 2 cases of eyelid dermoid cyst attached to tarsus in pediatric patients. Both patients were infants who presented with a firm, nontender upper eyelid mass firmly adherent to tarsus. In both cases, the lesion was excised en bloc, and histopathology revealed a dermoid cyst. To the authors' knowledge, there are no previously reported cases of tarsal dermoid cyst. These cases demonstrate the importance of including dermoid cyst in the differential diagnosis of a tarsus-based eyelid mass. Misdiagnosis may lead to incision and curettage, resulting in spillage of cyst contents and the risk of severe inflammation and scarring.

Transient descent of the contralateral eyelid in unilateral ptosis surgery.

PURPOSE: To report novel findings regarding contralateral eyelid height (i.e., intraoperative descent, followed by postoperative elevation) during unilateral ptosis surgery and to comment on their relevance in surgical planning. METHODS: Twelve adults with unilateral ptosis underwent levator advancement surgery. During surgery, eyelid height was set to the contralateral preoperative margin reflex distance value, rather than intraoperative level. The margin reflex distance of both eyes was measured before, during, and after surgery. RESULTS: The mean preoperative margin reflex distance on the ptotic side was 0.63 mm versus 3.83 mm contralaterally. No patient demonstrated a Hering phenomenon preoperatively. In each case, the goal was to elevate the ptotic eyelid to the contralateral preoperative height. For the ptotic eyelid, this resulted in a mean intraoperative margin reflex distance of 4 mm. Simultaneously, the contralateral side was noted to drop in each case, to a mean margin reflex distance of 1.67 mm. Postoperatively, at a mean follow up of 1.25 weeks, the mean margin reflex distance values were 3.88 mm and 3.83 mm for the operated and unoperated sides, respectively (Pearson correlation coefficient = 0.88, p < 0.05). At a mean follow-up of 4.35 months, the mean margin reflex distance values were 3.80 mm and 3.83 mm for the operated and unoperated sides, respectively (Pearson correlation coefficient = 0.96, p < 0.05). No patient had greater than 0.5 mm of asymmetry, and no patient requested postoperative adjustment. Had intraoperative symmetry been obtained with a postoperative contralateral return to preoperative height, a mean 42.1% of postoperative height asymmetry would have resulted between the 2 eyelids. CONCLUSIONS: During unilateral levator advancement surgery, the authors noted that the contralateral eyelid temporarily droops, and this Hering-like effect reverses postoperatively. The authors recommend that by raising the operated eyelid to the height of the contralateral side's preoperative (rather than intraoperative) height, excellent postoperative eyelid height and symmetry can be obtained.

Botulinum toxin as novel treatment for excess mucous membrane graft secretions.

Salivary gland secretion is an unusual complication of mucous membrane grafting. The authors report a case in which botulinum toxin was successfully used to treat graft secretions after buccal mucous membrane autograft for recurrent pterygium repair.

Management of an expansile orbital mass: Plexiform neurofibroma decompression by orbitozygomatic approach.

Neurofibromatosis types 1 (NF-1) and 2 are significant entities to otolaryngologists because they frequently involve head and neck skin, brain, skull base, orbits, and surrounding neural and vascular structures. We present the case of a 52-year-old male with progressive, marked unilateral proptosis due to a multilobulated orbital mass, secondary to biopsy-proven plexiform neurofibroma (PN). Acute worsening of proptosis leading to corneal abrasion, diplopia, and pain required debulking surgery, for which an orbitozygomatic approach was utilized. Genetic testing for NF-1 revealed no mutation. This rare case of NF-negative orbital PN and multidisciplinary treatment considerations for expansile orbital tumors are discussed.

Metastatic breast cancer to 4 eyelids: a clinicopathologic report.

A 47-year-old woman presented complaining of progressive, painless swelling of all 4 eyelids for 6 weeks. Her medical history was significant for breast cancer 14 months prior. Examination results showed a diffuse, firm thickening of all 4 eyelids with mild erythema of the overlying skin. Orbital computer tomography revealed extensive preseptal infiltration of soft tissue of all 4 eyelids, which enhanced with contrast. Results of a bilateral biopsy of the upper eyelids demonstrated extensive infiltration of the orbicularis muscle. Histologic features were consistent with metastatic breast cancer. Results of both the primary breast cancer biopsy taken 14 months previously and the eyelid biopsies were the same histologic type. Presentation of metastatic breast cancer to the eyelids is rare, but a recurrence must be considered in any patient with a history of breast cancer, despite the length of tumor-free survival. Bilateral involvement should not exclude metastases from diagnostic consideration, but rather, the diagnosis requires a high degree of clinical suspicion and recognition of the various cutaneous forms.

Sarcoid-like granulomatous orbital inflammation induced by interferon-alpha treatment.

A 56-year-old black woman presented with bilateral orbital swelling in the lacrimal gland region 4 months after initiation of interferon-alpha and ribavirin treatment for occupationally-acquired hepatitis C infection. Histopathologic evaluation of bilateral lacrimal gland biopsy specimens revealed granulomatous inflammation. All other tests were negative for sarcoidosis including angiotensin converting enzyme level and chest x-ray. The use of interferon-alpha as an antiviral and/or chemotherapeutic agent is increasing. Therefore recognition of its possible orbital side effects is important for the oculoplastic surgeon. To the authors' knowledge, this is the first case of orbital granulomatous involvement associated with interferon-alpha therapy.

Waldenström macroglobulinemia of the orbit.

A 77-year-old man with known Waldenström macroglobulinemia was examined for unilateral proptosis and periorbital swelling. The patient was found to have an orbital mass involving the lateral wall. Histologic and immunophenotypic matching of the orbital mass to the patient's systemic findings were used to diagnose the orbital mass as a Waldenström macroglobulinemia tumor.

Langerhans cell histiocytosis of the orbit 10 years after involvement at other sites.

Langerhans cell histiocytosis within the orbit generally occurs in children and is marked by periorbital pain and swelling. Although these lesions respond well to minimal local curettage and concomitant corticosteroid injection, this disorder can be extremely aggressive and destructive. Recurrences usually occur within 12 months to 18 months after diagnosis. Herein, we discuss a patient who initially had mastoid and maxillary lesions and developed unilateral orbital involvement with periorbital pain and edema after a 10-year disease-free interval, and review other cases of late recurrence. The patient's symptoms resolved after curettage and injection of intralesional steroids. Clinicians should be aware that Langerhans cell histiocytosis may reappear at other sites, including the orbit, even after several years of quiescence.

D2-40 expression demonstrates lymphatic vessel characteristics in the dural portion of the optic nerve sheath.

PURPOSE: To investigate the presence or absence of lymphatic vessels in the human optic nerve by means of immunohistochemistry. METHODS: Use of selective molecular markers to differentiate lymphatics from blood vessels in optic nerve specimens obtained after enucleation or exenteration procedures. Specifically, the lymphatic-specific monoclonal antibody D2-40 was used to identify lymphatic endothelial cells in contrast to CD-34 identification of endothelial cells of blood vessels. Optic nerves obtained from 10 enucleation and 2 exenteration specimens submitted for routine pathology. Paraffin-embedded sections of human optic nerve were immunostained with the lymphatic specific endothelial marker D2-40 and the vascular specific endothelial marker CD-34, using a double-staining method. RESULTS: Immunostaining with the lymphatic selective marker D2-40 positively demonstrated lymphatic vessels in the dura mater of the optic nerve. CD-34 counter-immunostaining identified blood vessels as separate vascular structures within the optic nerve meninges and adjacent ocular tissues. Positive D2-40 staining of the arachnoid mater in a nonvascular pattern was identified. CONCLUSIONS: Vessels with features compatible with lymphatic vessels were demonstrated by means of a selective monoclonal immunohistochemical marker for lymphatic endothelium in the dura mater of the human optic nerve.

Newly recognized ocular side effects of erlotinib.

An 85-year-old man had a bilateral periorbital rash and conjunctivitis leading to lower eyelid ectropion and epiphora within 6 weeks of treatment with erlotinib (Tarceva, Genentech, Inc., San Francisco, CA, and OSI Pharmaceuticals, Melville, NY), a second-line antineoplastic agent. The treatment was discontinued secondary to toxicity, and the periorbital rash completely resolved within 6 weeks of cessation of the drug. To our knowledge, the periorbital rash resulting in bilateral lower eyelid ectropion associated with epiphora is a newly recognized side effect of erlotinib that is completely reversible with discontinuation of the drug. The rash and ectropion should be treated palliatively, and surgical intervention should be avoided unless the patient cannot be removed from treatment.