Faculty Bibliography

PURPOSE OF REVIEW/OBJECTIVE:Optic pathway gliomas (OPG) are the most common tumor of the anterior visual pathway and can involve the optic nerve, chiasm, tract, and optic radiations. They are typically benign lesions, often pilocytic astrocytomas, which are diagnosed in childhood. We review the epidemiology, clinical presentation, diagnosis, and management of these lesions in patients with and without neurofibromatosis type 1 (NF-1). RECENT FINDINGS/RESULTS:Most commonly, patients diagnosed with OPG have NF-1 especially if the lesions are bilateral. Such lesions tend to have a relatively indolent course and at least 50% of patients have no evidence of visual loss. Rarely, children without NF-1 may sporadically develop OPG with such lesions often having a more aggressive nature and greater propensity for visual dysfunction. The gold standard for diagnosis and follow-up are thorough neuro-ophthalmic examinations with specific attention to visual acuity. Management must be individualized and may comprise conservative follow-up, chemotherapy, radiation and/or surgical intervention. SUMMARY/CONCLUSIONS:OPG may range in their behavior based upon the nature of the tumor (NF-1 or sporadic). Current guidelines recommend following patients with regular clinical examinations. Management of these lesions is highly individualized based upon the nature and extent of the lesion, visual function and side-effect profile of the treatment. Clinicians should be aware of the available options to determine which may be best suited for their patient.

Orbital schwannomas are typically slow-growing benign tumors that can cause gradual loss of vision, proptosis, and limitation of ocular motility. The authors present an atypical case of a rapidly growing orbital apex schwannoma in a patient with preexisting vision loss secondary to presumed sarcoidal optic neuritis. Contrary to the slowly progressive nature of a typical orbital schwannoma, the lesion was observed to enlarge from radiologically undiscernible to 3.5 cm over 4 years.

With improvements in systemic therapy, patients with cancer treated with whole-brain radiotherapy (WBRT) are living long enough to develop late toxicities, including dry eye syndrome. In general practice, dose to the lacrimal gland (LG) is not constrained (maximum constraint <40 Gy) in WBRT. The purpose of this study was to measure dose to the LG in WBRT and determine methods for reducing radiation exposure. We conducted a retrospective review of 70 3-dimensional (3D) conformal plans; thirty-six plans with a radiation prescription of 30 Gy in 10 fractions and 34 plans with a prescription of 37.5 Gy in 15 fractions. LGs were contoured in accordance with Freedman and Sidani (2015). Biological effective dose (BED)3 maximum constraints were calculated from 40 Gy and 20 Gy to be 32.17 Gy (30 Gy) and 36.70 Gy (37.5 Gy). Both regimens demonstrated supraorbital blocking by 3 methods: T1, bordering the supraorbital ridge; T2, no contact with supraorbital ridge; and T3, coverage of the supraorbital ridge. Mean dose for the plans with a 30-Gy prescription and the plans with a 37.5-Gy prescription was 27.5 Gy and 35.2 Gy, respectively (p ≤ 0.0001). BED3 maximum constraint (D_max) was violated 16 of 26 (61.5%) in T1 (average D_max: 32.2 Gy), 13 of 28 (46.4%) in T2 (average D_max: 32.1 Gy), and 5 of 18 (27.8%) in T3 (average D_max: 31.8 Gy) for the 30-Gy prescription. D_max was violated in 32 of 32 (100%) in T1 (average D_max: 40.1 Gy), 22 of 22 (100%) in T2 (average D_max: 40.3 Gy), and 14 of 14 (100%) in T3 (average D_max: 39.4) for the 37.5 Gy prescription. Average D_max for the 37.5-Gy prescription was highly significant in favor of T3 (p = 0.0098). Patients who receive WBRT may develop dry eye syndrome as a late toxicity. Constraints are commonly violated with a prescription of 37.5 Gy. Methods to reduce dose include T3 supraorbital blocking, an easily implementable change that may dramatically improve patient quality of life.

Classically, granuloma annulare (GA) is a cutaneous disorder localized to the dorsum of the hands and/or feet in children and young adults. Very rarely it can present on the face and rarer still on periorbital structures such as the eyelid and orbital rim. Diagnosis hinges on clinical presentation and histological features, such as palisading granulomas with central destruction of collagen, presence of mucin and lymphohistiocytic infiltration. The etiology of this condition remains unknown, but may involve a delayed-type hypersensitivity reaction, malignancy and/or infection. Herein is the first reported case of an intraorbital GA in an 86-year-old male patient who presented with right eye proptosis.

We report a case of orbital myositis of the superior rectus muscle-levator complex masquerading as nonspecific orbital inflammation but corresponding in location to a known braided polyester "chicken suture" placed 20 years earlier during strabismus surgery. The orbital inflammation was refractory to oral steroids but resolved promptly on surgical removal of the suture material. Although suture material is known to cause foreign body granulomatous reactions, to our knowledge this is the first reported case of a deep, diffuse orbital inflammation attributable to chicken suture placed during strabismus surgery.

PURPOSE:: Identify a reproducible measure of axial globe position (AGP) for multicenter studies on patients with thyroid eye disease (TED). METHODS:: This is a prospective, international, multicenter, observational study in which 3 types of AGP evaluation were examined: radiologic, clinical, and photographic. In this study, CT was the modality to which all other methods were compared. CT AGP was measured from an orthogonal line between the anterior lateral orbital rims to the cornea. All CT measurements were made at a single institution by 3 individual clinicians. Clinical evaluation was performed with exophthalmometry. Three clinicians from each clinical site assessed AGP with 3 different exophthalmometers and horizontal palpebral width using a ruler. Each physician made 3 separate measurements with each type of exophthalmometer not in succession. All photographic measurements were made at a single institution. AGP was measured from lateral photographs in which a standard marker was placed at the anterior lateral orbital rim. Horizontal and vertical palpebral fissure were measured from frontal photographs. Three trained readers measured 3 separate times not in succession. Exophthalmometry and photography method validity was assessed by agreement with CT (mean differences calculation, intraclass correlation coefficients [ICCs], Bland-Altman figures). Correlation between palpebral fissure and CT AGP was assessed with Pearson correlation. Intraclinician and interclinician reliability was evaluated using ICCs. RESULTS:: Sixty-eight patients from 7 centers participated. CT mean AGP was 21.37 mm (15.96-28.90 mm) right and 21.22 mm (15.87-28.70 mm) left (ICC 0.996 and 0.995). Exophthalmometry AGP fell between 18 mm and 25 mm. Intraclinician agreement across exophthalmometers was ideal (ICC 0.948-0.983). Agreement between clinicians was greater than 0.85 for all upright exophthalmometry measurements. Photographic mean AGP was 20.47 mm (10.92-30.88 mm) right and 20.30 mm (8.61-28.72 mm) left. Intrareader and interreader agreement was ideal (ICC 0.991-0.989). All exophthalmometers' mean differences from CT ranged between -0.06 mm (+/-1.36 mm) and 0.54 mm (+/-1.61 mm); 95% confidence interval fell within 1 mm. Magnitude of AGP did not affect exophthalmometry validity. Oculus best estimated CT AGP but differences from other exophthalmometers were not clinically meaningful in upright measurements. Photographic AGP (right ICC = 0.575, left ICC = 0.355) and palpebral fissure do not agree with CT. CONCLUSIONS:: Upright clinical exophthalmometry accurately estimates CT AGP in TED. AGP measurement was reliably reproduced by the same clinician and between clinicians at multiple institutions using the protocol in this study. These findings allow reliable measurement of AGP that will be of considerable value in future outcome studies.

Infectious orbital complications after strabismus surgery are rare. Their incidence is estimated to be 1 case per 1,100 surgeries and include preseptal cellulitis, orbital cellulitis, subconjunctival and sub-Tenon's abscesses, myositis, and endophthalmitis. This report describes the case of an otherwise healthy 3-year-old boy who underwent bilateral medial rectus recession and disinsertion of the inferior obliques. A few days after surgery, the patient presented with bilateral periorbital edema and inferotemporal chemosis. A series of CT scans with contrast revealed inferotemporal orbital collections OU. The patient immediately underwent transconjunctival drainage of fibrinous and seropurulent collections in the sub-Tenon's space and experienced rapid improvement a few days later. The patient is reported to be in stable condition in a follow-up examination performed more than a year after the reported events.

PURPOSE:: The natural history of thyroid eye disease follows a biphasic course featuring an initial active stage followed by a durable quiescent stage. Reactivation of thyroid eye disease is defined by recurrence of inflammatory signs and symptoms after a period of stability lasting at least 6 months. It is thought to be rare and is poorly studied. The goal of this study was to define the incidence and characteristics of recurrent thyroid eye disease. METHODS:: A retrospective chart review of 415 visits of patients with thyroid eye disease was performed between 2006 and 2012. Recurrent cases were identified by subjective historical accounts of disease recurrence, review of prior orbital imaging, and photographic evidence. RESULTS:: Among 415 cases of thyroid eye disease, 65 cases of recurrence (15.7%) were identified. Most cases of reactivation occurred within the first 10 years after the initial episode of thyroid eye disease. The mean age of patients at the first event was 42.2 years and 52.6 years at the second event. Eighteen patients had identifiable events that may have triggered disease recurrence. The recurrence rate was higher among patients that had been smokers during their first episode of thyroid eye disease (22%) compared with the nonsmokers (14.6%). CONCLUSIONS:: Although uncommon, recurrence of thyroid eye disease does not appear to be as rare as previously believed. A better understanding of the natural history of thyroid eye disease is important as it impacts patient education and management.

We present two cases of infants with a similar constellation of clinical findings: retro-orbital infantile hemangioma (IH), internal carotid artery (ICA) arteriopathy, and intracranial IH. In both cases, intracranial vascular anomalies and hemangiomas were found incidentally during evaluation of unilateral proptosis. Neither infant had evidence of cutaneous segmental IH of the face or neck, which might have provided a clue to the diagnosis of PHACE syndrome or of intracranial hemangiomas. In one case, intracranial involvement was particularly extensive and function threatening, with mass effect on the brain parenchyma. These cases serve to highlight the fact that clinical findings of proptosis, globe deviation, and strabismus should prompt immediate imaging to confirm the presence of orbital IHs and to exclude other diagnoses. Moreover, based on our cases and the embryologic origin of the orbit as a unique developmental unit, patients with confirmed retro-orbital IHs should undergo evaluation for anomalies associated with PHACE syndrome. Patients with orbital IHs and an additional major criterion for PHACE syndrome should be considered to have definite, and not just possible, PHACE syndrome.