Selumetinib in the Treatment of Orbital Plexiform Neurofibroma: A Case Report.

A 4-year-old boy with a known diagnosis of neurofibromatosis 1 (NF1) and a diffusely infiltrative plexiform neurofibroma (PN) of the left orbit was started on selumetinib treatment for progressively worsening amblyopia. The patient first presented with new-onset left ptosis at 11 months old. He subsequently developed refractory anisometropic amblyopia of the left eye, in addition to clinically significant left proptosis and hypoglobus that interfered with glasses wear for his amblyopia treatment.

Teprotumumab and Hearing Loss: Case Series and Proposal for Audiologic Monitoring.

Purpose: To present a protocol for audiologic monitoring in the setting of teprotumumab treatment of thyroid eye disease, motivated by 4 cases of significant hearing loss, and review the relevant literature.

Methods: Cases of hearing loss in the setting of teprotumumab were retrospectively elicited as part of a multi-institutional focus group, including oculoplastic surgeons, a neurotologist and an endocrinologist. A literature review was performed.

Compound Coherent Plane-Wave Ultrasound Imaging of Vascular Malformations of the Orbit.

Purpose: Prior color-flow Doppler ultrasound studies of the eye have been performed with systems that exceed US Food and Drug Administration permissible ophthalmic ultrasonic energy limits. The authors report a study of orbital vascular malformations using a novel, Food and Drug Administration compliant, ultrafast compound coherent plane-wave ultrasound device to produce power Doppler images.

Methods: Using a Verasonics Vantage 128 ultrasound engine and a user-developed MATLAB program with a 5-MHz linear-array probe, compound coherent plane-wave ultrasound data were collected on patients with orbital vascular malformations.

Doxycycline Injection for Sclerotherapy of Lower Eyelid Festoons and Malar Edema: Preliminary Results.

Purpose: To investigate the safety and efficacy of direct, intralesional doxycycline hyclate injection for improving the appearance of cosmetically significant lower eyelid festoons and malar edema.

Methods: An Institutional Review Board approved, retrospective review was performed of 15 consecutive patients with malar edema and/or festoons injected with doxycycline hyclate at a concentration of 10 mg/ml. Pre- and postinjection photographs were reviewed and graded on a scale of 0 to 3 (0: no festoon; 1: small festoon; 2: medium festoon; 3: large festoon) by 2 masked physician observers.

Predominant Contribution of Superior Rectus-Levator Complex Enlargement to Optic Neuropathy and Inferior Visual Field Defects in Thyroid Eye Disease.

Purpose: To compare extraocular muscle volumes in thyroid eye disease patients with and without compressive optic neuropathy.

Methods: A retrospective review of 44 orbital CT scans (28 orbits without compressive disease and 16 orbits with compressive optic neuropathy) was conducted. The extraocular muscle volumes, summated soft tissue volumes, and optic nerve volumes were calculated at a section in the posterior 1/3 of the orbit.

The Columbia Thyroid Eye Disease-Compressive Optic Neuropathy Diagnostic Formula.

Purpose: Diagnosing thyroid eye disease-compressive optic neuropathy (TED-CON) is challenging, particularly in cases lacking a relative afferent pupillary defect. Large case series of TED-CON patients and accessible diagnostic tools are lacking in the current literature. This study aims to create a mathematical formula that accurately predicts the presence or absence of CON based on the most salient clinical measures of optic neuropathy.

Nasal Glioma: A Rare Cause of Congenital Inner Canthal Swelling.

Nasal glioma, encephalocele, and ectopic brain are rare congenital anomalies. The terminology applied to these entities has been historically confusing. In many cases, the terms overlap and may be employed synonymously although some authors emphasize their differences.

IgG4-related orbital disease masquerading as thyroid eye disease, vice versa, or both?

A 40 year-old male presented after one year of unilateral, progressive, steroid-responsive, orbital inflammatory disease causing proptosis, extraocular muscle (EOM) restriction, and compressive optic neuropathy. The development of anti-thyroidal antibodies prompted the diagnosis of thyroid eye disease (TED); however, the prolonged active phase, remarkable reversibility of ophthalmic features with high-dose corticosteroids, unilaterally of disease, uncharacteristic EOM involvement (including both obliques), and the absence of autoimmune thyroid disease provoked consideration of alternative diagnoses. Inferior oblique biopsy stained positive for IgG4 with histologic features atypical of TED.

Ophthalmic Timolol Hallucinations: A Case Series and Review of the Literature.

Purpose: Systemic absorption and central nervous system (CNS) penetration of timolol drops are a well-studied phenomenon, resulting in common side effects such as bradycardia, bronchospasm, fatigue, and confusion. More serious CNS side effects, such as psychosis and depression, however, are rarely attributed to eye drops. We report a case series in which patients developed visual hallucinations secondary to topical ocular timolol use.

Patterns of visual field changes in thyroid eye disease.

This article provides a systematic description of visual field changes in thyroid eye disease-compressive optic neuropathy (TED-CON). A retrospective, non-comparative chart review of patients with TED-CON and documented Humphrey Visual Field 24-2 or 30-2 testing was conducted with IRB approval. Ninety-six visual fields in 68 patients were classified into 7 broad categories (superior, inferior, diffuse, temporal, nasal, central/paracentral, enlarged blind spot) and 17 mutually exclusive patterns from the Ocular Hypertension Treatment Study (OHTS) or "other.

Enhanced Fasanella-Servat Procedure for the Graded Repair of Blepharoptosis.

Purpose: To describe in detail a technique for a modified Fasanella-Servat procedure that provides a simplified method for graded Mueller muscle excision with minimal and controlled tarsal excision.

Methods: A retrospective study of 71 patients (102 eyelids) who underwent the modified Fasanella-Servat procedure is reported. Measurements include the preoperative, post-phenylephrine, and postoperative margin-to-reflex distance-1, and postoperative upper eyelid height symmetry as determined by the absolute difference between right- and left-sided margin-to-reflex distance-1.

Low-Cost 3D Printing Orbital Implant Templates in Secondary Orbital Reconstructions.

Purpose: Despite its increasing use in craniofacial reconstructions, three-dimensional (3D) printing of customized orbital implants has not been widely adopted. Limitations include the cost of 3D printers able to print in a biocompatible material suitable for implantation in the orbit and the breadth of available implant materials. The authors report the technique of low-cost 3D printing of orbital implant templates used in complex, often secondary, orbital reconstructions.

Atypical Rapidly Enlarging Orbital Schwannoma.

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.

Low-Flow Arterialized Venous Malformations of the Orbit.

Purpose: Orbital vascular malformations are classified by their hemodynamic properties, either high or low flow. Low-flow lesions may be simple venous, lymphatic, or combined lymphaticovenous malformations. The authors report a series of cases in which predominantly low flow, venous lesions were unexpectedly noted to have arterial feeders.

Retained strabismus suture material masquerading as nonspecific orbital inflammation.

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.

Wyburn-Mason Syndrome Associated With Cutaneous Reactive Angiomatosis and Central Retinal Vein Occlusion.

Retinal venous occlusive events are a rare complication of arteriovenous malformations of the retina found in Wyburn-Mason syndrome. The authors present a case of a 28-year-old man diagnosed with Wyburn-Mason syndrome and cutaneous reactive angiomatosis, a reactive angioproliferative disorder induced by vascular occlusion. He developed a central retinal vein occlusion complicated by macular edema and received treatment with intravitreal bevacizumab, which led to resolution of the edema.

Malignant rhabdoid transformation of a longstanding, aggressive, and recurrent orbital angiomyxoma.

A 47-year-old woman presented with a medial orbital tumor initially diagnosed as either a myxoid neurofibroma or myoepithelioma. Over 30 years the tumor recurred seven times and was serially debulked. Careful histopathologic analysis coupled with immunohistochemical studies performed on the last two biopsies established the rare diagnosis of a locally aggressive angiomyxoma (because of its local infiltrative growth) with myofibroblastic features (smooth muscle actin and calponin positivity and desmin negativity).

Reactivation of thyroid associated orbitopathy following trauma with intraorbital foreign body.

A 63-year-old female with mild, bilateral, stable thyroid-associated orbitopathy sustained trauma resulting in glass foreign bodies embedded on the left ocular surface and left lateral orbital extraconal and intraconal space. After 2 orbitotomies including a failed attempt to remove the intraconal foreign body and poor response to oral steroids, she developed severe, progressive left periorbital edema and 9 mm of relative proptosis. Serial, post-operative imaging demonstrated worsening inflammatory changes along the surgical tract, which slowly improved over several months, with simultaneously worsening proptosis and enlargement of the left inferior and medial rectus muscles consistent with worsening thyroid orbitopathy.

Microdebrider use in orbital surgery.

Purpose: To report the novel use of a sinus microdebrider for the removal of tissue during orbital surgery.

Methods: This retrospective study reviewed the logs of 3 surgeons to identify patients who required orbital surgery during which the surgeon chose to use a sinus microdebrider with an open sky technique as a means of removing portions of the orbital tissue. Collected data included patient demographics, clinical examinations, pathologic diagnoses, radiologic studies, operative reports and, when available, photographs and intra-operative video.

Infantile hemangiomas: A review.

Infantile hemangiomas (IH) are the most common eyelid and orbital tumors of childhood. Although they are considered benign lesions that have a generally self-limited course, in the periocular region, they have the potential to cause amblyopia, strabismus, and severe disfigurement. The decision for treatment can be a source of anxiety for patients, parents, and physicians alike.