Extranodal Rosai-Dorfman Disease of the Orbit: Clinical Features of 8 Cases.

PURPOSE: To report on the presentation, radiography, histology, and treatment of 8 cases of extranodal Rosai-Dorfman disease involving the orbit. METHODS: Multicenter retrospective case series. RESULTS: Five males and 3 females had a median age of 10 years (range 2-78 years). Presenting signs and symptoms included proptosis, periorbital pain, palpable mass, blepharoptosis, decreased vision, diplopia, impaired extraocular motility, and afferent pupillary defect. Four patients had bilateral orbital disease, while 4 had unilateral disease. Six cases were extraconal, 1 was intraconal, and 1 was both intra- and extra-conal. Four cases had only extranodal disease without lymphadenopathy (3 of which had localized orbital disease). Diagnosis was confirmed by exam, orbital, and/or systemic radiography, and biopsy in all cases. Treatment strategies included excision or debulking, systemic corticosteroids, chemotherapy, radiotherapy, observation or a combination thereof. At last follow up, 4 patients were disease free, while 4 had residual improved disease. CONCLUSIONS: Rosai-Dorfman disease of the orbit is a rare clinical entity. Purely extranodal disease is rare, with isolated orbital disease being exceedingly rare. This study is unique in that 4 of 8 patients had strictly isolated extranodal disease of the orbit. A large majority of the cases had disease in the extraconal space, contrasting with previous reports. In addition, lacrimal gland disease, particularly bilateral involvement, was prominent in the current study. Although there is no consensus on treatment, surgical excision should be attempted if plausible in symptomatic patients especially if the orbit represents a localized site of disease.

Management of postblepharoplasty chemosis.

Clinically significant chemosis occasionally complicates lower eyelid blepharoplasty. In this report, the etiologic components of postblepharoplasty chemosis are discussed. The time course and duration of chemosis vary according to the underlying cause. Early, late, and prolonged chemosis are managed with different strategies. Diagnostic and therapeutic algorithms for chemosis assessment and management are presented.

Epstein-Barr virus associated smooth muscle tumors in post transplant pediatric patients two cases of rare locations, and review of the literature.

Epstein-Barr virus (EBV) may present few or no symptoms in immunocompetent individuals; however, in immunocompromised patients as in the case of AIDS and post-transplant patients, the virus occasionally stimulates neoplastic transformations. Epstein-Barr virus may play a role in the development of smooth muscle tumors (SMT). In the case of Epstein-Barr associated smooth muscle tumors (EBV+SMT), the virus is thought to be the leading factor to the tumorigenic pathway. We report two pediatric patients (6 and 13 years old) who underwent liver transplantation and developed EBV+SMT in the colon and orbit. These two cases represent rare locations for this kind of lesion.

Concepts in eyelid biomechanics with clinical implications.

The inner canthal orbicularis drives spontaneous blinking of the eyelids and is richly innervated by a branching network of twigs based largely on the buccal branch of the facial nerve. This segment of the orbicularis is relatively resistant to denervation from subciliary incisional approaches. Combined with proper eyelid tension, contraction of this segment of orbicularis results in the normal physiologic blink mechanism. The authors discuss how to achieve proper eyelid tension and position intraoperatively during the treatment of patients with a range of orbital morphologies.

The open brow lift.

The open brow lift procedure is discussed in terms of relevant surgical anatomy, preoperative evaluation, and detailed surgical technique for pretrichial coronal forehead lift with hair-bearing temporal lift, direct incisional brow lift, and coronal brow lift. Complications are discussed, and information is presented on patient evaluation and expectations, with a discussion of what patients can expect before and after brow lift surgery.

Diffuse symmetric meningioma en plaque mimicking metabolic disease of bone.

A 50-year-old African American woman with right proptosis and decreased vision demonstrated symmetric, bilateral thickening of the sphenoid wings, calvarium, and skull base on computed tomography. Histopathologic evaluation of the hyperostotic lesion was consistent with meningotheliomatous meningioma. The patient underwent uncomplicated right frontal orbital craniectomy with postoperative radiation. The presentation of en plaque meningioma, when diffuse and symmetric, can mimic a metabolic disease of bone.

Non-surgical treatments of primary, non-melanoma eyelid malignancies: a review.

The diagnosis and management of periocular cutaneous malignancies are essential components of an ophthalmologist's practice. Skin cancers comprise nearly one-third of newly diagnosed malignancies. Furthermore, the incidence of skin cancer appears to be increasing. Multiple treatment modalities exist for periocular cutaneous malignancy. Surgical extirpation, often with the combined expertise of a Mohs micrographic surgeon, is typically the first line therapy and is often curative in the periocular region, depending on a variety of factors, including tumour histology, specific location, depth of invasion and surgical technique. However, there are instances where a less invasive, non-surgical treatment option is warranted, including patients who are poor surgical risks or those with diffuse disease. The purpose of this article is to review the literature and describe the non-surgical treatment options, indications and efficacies for non-melanoma primary eyelid malignancies.

Bilateral circumferential macular folds in inflicted childhood neurotrauma.

PURPOSE: To report an infant with inflicted neurotrauma who exhibited bilateral circumferential macular folds. METHODS: Bedside ophthalmic examination and fundus photography using RetCam-II (Clarity Medical Systems, Pleasanton, CA). RESULTS: Both pupils were miotic and nonreactive. External and anterior segments showed no abnormality. The fundus showed bilateral diffuse multilaminar retinal, subhyaloid, and vitreous hemorrhages. Distinctive macular folds were noted bilaterally. CONCLUSION: Circumferential macular folds are most commonly found in abused infants, although it has been suggested that they may be associated with Terson syndrome and severe crush injury.

Magnetic resonance imaging evidence of optic nerve glioma progression into and beyond the optic chiasm.

Optic nerve glioma has been a topic of great interest and controversy, particularly with respect to its tendency to invade the optic chiasm. This case report demonstrates histologically verified optic nerve glioma in an otherwise healthy 5-year-old girl. MRI revealed sequential prechiasmal, chiasmal, and postchiasmal disease during the untreated course of 18 months. The authors also discuss the results obtained with a specific immunohistochemical technique, the mindbomb homolog 1 (MIB-1) labeling index, and speculate that this information, if known early in the course of disease, might influence early surgical decision-making.

Metastatic endometrial carcinoma resulting in orbital apex compression.

A 63-year-old woman presented subacutely with signs of orbital apex and cavernous sinus disease in the setting of widespread, untreated metastatic carcinoma of the uterus. MRI revealed a destructive lesion of the left sphenoid wing with compression of the orbital apex and with possible extension in the cavernous sinus. The underlying diagnosis was confirmed with repeat endometrial biopsy, revealing well-differentiated endometrial adenocarcinoma. Given the supporting clinical evidence for metastatic disease and the radiologic characteristics of the lesion, the decision was made to not perform a confirming orbital biopsy.

Compressed helium injury to the orbit resulting in pneumocephalus.

A 33-year-old woman received a conjunctival laceration after accidental contact with the flexible outlet tip of a tank of compressed helium while filling balloons. The gas discharged during the contact, blowing compressed helium into her right orbit, with intracranial extension. The patient was asymptomatic, except for a transient headache. She was treated with prophylactic antibiotics and observed overnight, then discharged without complication. A literature search reveals that the usual outcome for this mechanism of injury is favorable.

Cysticercosis isolated to the orbit.

A 55-year-old woman who underwent orbital fat excision for cosmesis was incidentally found to have an isolated orbital cysticercus identified by histopathologic examination. The ensuing workup was negative for disseminated cysticercal infection and further treatment was deferred. Although uncommon, orbital cysticercosis should be considered in the differential diagnosis of an asymptomatic orbital mass. Symptomatic orbital myocysticercosis has been effectively treated with albendazole at a dosage of 30 mg/kg for 15 days combined with simultaneous low-dose steroids.