Faculty Bibliography

PURPOSE/OBJECTIVE: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. RESULTS:An aggregate of 4 cases of teprotumumab-associated hearing loss documented by formal audiologic testing were identified among 3 clinicians who had treated 28 patients. CONCLUSIONS:Teprotumumab may cause a spectrum of potentially irreversible hearing loss ranging from mild to severe, likely resulting from the inhibition of the insulin-like growth factor-1 and the insulin-like growth factor-1 receptor pathway. Due to the novelty of teprotumumab and the lack of a comprehensive understanding of its effect on hearing, the authors endorse prospective investigations of hearing loss in the setting of teprotumumab treatment. Until the results of such studies are available, the authors think it prudent to adopt a surveillance protocol to include an audiogram and tympanometry before, during and after infusion, and when prompted by new symptoms of hearing dysfunction.

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. METHODS: A retrospective case series of 5 orbital reconstructions utilizing a technique of 3D printed orbital implant templates is presented. Each patient's Digital Imaging and Communications in Medicine data were uploaded and processed to create 3D renderings upon which a customized implant was designed and sent electronically to printers open for student use at our affiliated institutions. The mock implants were sterilized and used intraoperatively as a stencil and mold. The final implant material was chosen by the surgeons based on the requirements of the case. RESULTS: Five orbital reconstructions were performed with this technique: 3 tumor reconstructions and 2 orbital fractures. Four of the 5 cases were secondary reconstructions. Molded Medpor Titan (Stryker, Kalamazoo, MI) implants were used in 4 cases and titanium mesh in 1 case. The stenciled and molded implants were adjusted no more than 2 times before anchored in place (mean 1). No case underwent further revision. CONCLUSIONS: The technique and cases presented demonstrate 1) the feasibility and accessibility of low-cost, independent use of 3D printing technology to fashion patient-specific implants in orbital reconstructions, 2) the ability to apply this technology to the surgeon's preference of any routinely implantable material, and 3) the utility of this technique in complex, secondary reconstructions.

BACKGROUND: Congenital nasolacrimal duct obstruction (NLDO) is a common condition causing excessive tearing in the first year of life. Infants present with excessive tearing or mucoid discharge from the eyes due to blockage of the nasolacrimal duct system, which can result in maceration of the skin of the eyelids and local infections, such as conjunctivitis, that may require antibiotics. The incidence of nasolacrimal duct obstruction in early childhood ranges from 5% to 20% and often resolves without surgery. Treatment options for this condition are either conservative therapy, including observation (or deferred probing), massage of the lacrimal sac and antibiotics, or probing the nasolacrimal duct to open the membranous obstruction at the distal nasolacrimal duct. Probing may be performed without anesthesia in the office setting or under general anesthesia in the operating room. Probing may serve to resolve the symptoms by opening the membranous obstruction; however, it may not be successful if the obstruction is due to a bony protrusion of the inferior turbinate into the nasolacrimal duct or when the duct is edematous (swollen) due to infection such as dacryocystitis. Additionally, potential complications with probing include creation of a false passage and injury to the nasolacrimal duct, canaliculi and puncta, bleeding, laryngospasm, or aspiration. OBJECTIVES: To assess the effects of probing for congenital nasolacrimal duct obstruction. SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (CENTRAL), which contains the Cochrane Eyes and Vision Trials Register (2016, Issue 8); MEDLINE Ovid (1946 to 30 August 2016); Embase.com (1947 to 30 August 2016); PubMed (1948 to 30 August 2016); LILACS (Latin American and Caribbean Health Sciences Literature Database; 1982 to 30 August 2016), the metaRegister of Controlled Trials (mRCT) (www.controlled-trials.com), last searched 14 August 2014; ClinicalTrials.gov (www.clinicaltrials.gov), searched 30 August 2016; and the WHO International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en), searched 30 August 2016. We did not use any date or language restrictions in the electronic searches for trials. SELECTION CRITERIA: We included randomized controlled trials (RCTs) that compared probing (office-based or hospital-based under general anesthesia) versus no (or deferred) probing or other interventions (observation alone, antibiotic drops only, or antibiotic drops plus massage of the nasolacrimal duct). We did not include studies that compared different probing techniques or probing compared with other surgical procedures. We included studies in children aged three weeks to four years who may have presented with tearing and conjunctivitis. DATA COLLECTION AND ANALYSIS: Two review authors independently screened studies for inclusion and independently extracted data and assessed risk of bias for the included studies. We analyzed data using Review Manager software and evaluated the certainty of the evidence using GRADE. MAIN RESULTS: We identified two RCTs and no ongoing studies; one of the included RCTs was registered. The studies reported on 303 eyes of 242 participants who had unilateral or bilateral congenital nasolacrimal duct obstruction. For both included studies, the interventions compared were immediate office-based probing to remove the duct obstruction versus deferred probing, if needed, after 6 months of observation or once the child reached a certain age.The primary outcome of the review, treatment success at 6 months, was reported partially in one study. Treatment success was not reported at this time point for all children in the immediate probing group; however, 77 of 117 (66%) eyes randomized to deferred probing had resolved without surgery 6 months after randomization and 40 (34%) eyes did not resolve without probing. For children who had unilateral NLDO, those randomized to immediate probing had treatment success more often than those who were randomized to deferred probing (RR 1.41, 95% CI 1.12 to 1.78; 163 children; moderate-certainty evidence). Treatment success for all children was assessed in the study at age 18 months; as an ad hoc analysis in the included study, results were presented separately for children with unilateral and bilateral NLDO (RR 1.13, 95% CI 0.99 to 1.28 and RR 0.86, 95% CI 0.70 to 1.06, respectively; very low-certainty evidence).In the other small study (26 eyes of 22 children), more eyes that received immediate probing were cured within one month after surgery compared with eyes that were randomized to deferred probing and analyzed at age 15 months (RR 2.56, 95% CI 1.16 to 5.64). We considered the evidence to be low-certainty due to imprecision from the small study size and risk of bias concerns due to attrition bias.One study reported on the number of children that required reoperation; however, these data were reported only for immediate probing group. Nine percent of children with unilateral NLDO and 13% with bilateral NLDO required secondary procedures.One study reported cost-effectiveness of immediate probing versus deferred probing. The mean cost of treatment for immediate probing was less than for deferred probing; however, there is uncertainty as to whether there is a true cost difference (mean difference USD -139, 95% CI USD -377 to 94; moderate-certainty evidence).Reported complications of the treatment were not serious. One study reported that there were no complications for any surgery and no serious adverse events, while the other study reported that bleeding from the punctum occurred in 20% of all probings. AUTHORS' CONCLUSIONS: The effects and costs of immediate versus deferred probing for NLDO are uncertain. Children who have unilateral NLDO may have better success from immediate office probing, though few children have participated in these trials, and investigators examined outcomes at disparate time points. Determining whether to perform the procedure and its optimal timing will require additional studies with greater power and larger, well-run clinical trials to help our understanding of the comparison.

Inflammatory myofibroblastic tumor is a rare entity characterized by the presence of myofibroblasts and inflammatory cells within a fibrous stroma. It typically occurs in the pediatric population. The most common site of occurrence is the lung though it has been reported throughout the body. Although rare, it has been reported in the orbit. The clinical course is ill defined in the literature; here we report a case of pediatric IMT with delayed spontaneous regression.

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. METHODS: A retrospective chart review of patients identified by the authors as having orbital varices with arterial components was conducted. The authors identified 7 such cases. After careful review, 2 cases were excluded due to inconclusive neuroradiographic findings. The authors review the clinical, radiologic, histopathologic, and surgical information from the remaining 5 cases and discuss their clinical significance. RESULTS: All 5 cases were most consistent with variceal lesions: 3 as clinically distensible lesions and 2 as thrombosed lesions. Additional arterial feeder vessels were noted by angiography (3) or intraoperative visualization (2). The arterial contribution varied from faint vessels to distinct branches of the ophthalmic artery. Ages ranged from 13 to 61 years without predilection for gender. Treatments consisted of excision, embolization, and observation. Two poignant cases are highlighted: the first illustrating that an angiogram in isolation of its clinical picture can be misleading and result in treatment intervention with undue risk, and the second illustrating that inadequate treatment of unrecognized arterial components may contribute to recurrences. CONCLUSIONS: Low-flow orbital variceal lesions may have less prominent, arterial components. This type of combined arterialized venous malformation is largely unrecognized in the ophthalmic literature. Correct identification of these lesions is critical in providing safe, effective, and durable treatment.

A raised erythematous eyelid lesion that appeared in a 31-year-old man was diagnosed as an arteriovenous malformation (AVM), with confirmatory Doppler ultrasound demonstrating high arterial flow. Surgical excision, aided by electrocautery for extensive hemorrhage, resulted in an acceptable cosmetic result. Histopathology of the excised lesion showed collapsed capillary channels lined by endothelium. AVM is rarely encountered in the eyelid.

The authors describe an esthesioneuroblastoma (olfactory neuroblastoma) that occurred within the nasal cavity and brain in a 31-year-old man. Following excision, the tumor recurred in the left orbit and in mediastinal lymph nodes. Treatment included orbital excision and systemic chemotherapy. Histopathology showed a high-grade neuroepithelial tumor with positive immunohistochemical markers for neuroendocrine and epithelial components, an unusual combination raising issues concerning taxonomy.

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.

PURPOSE:: Some patients with severe benign essential blepharospasm (BEB) become clinically refractory to chemodenervation with botulinum toxin. In these patients, surgical myectomy is an effective additive treatment for the management of this disabling condition. The purpose of this study is to determine how myectomy for BEB alters subsequent botulinum toxin administration. METHODS:: A retrospective chart review of 27 patients undergoing surgical myectomy for the treatment of refractory BEB was conducted. The frequency and dosage of botulinum toxin were compared between preoperative and postoperative treatments. Paired Student t tests were used to determine significance (alpha = 0.05). RESULTS:: The mean interval between botulinum treatments was 10.1 weeks preoperatively and 15.7 weeks postoperatively (p < 0.001). The mean quantity of botulinum toxin administered was 68 units prior to myectomy and 64.9 units postoperatively (p = 0.227). When comparing the quantity of botulinum toxin injected into the upper eyelid region only (site of myectomy), there was a significant decrease in the dose administered preoperatively and postoperatively (46.8 units vs. 39.9 units, p = 0.00310). CONCLUSIONS:: Upper eyelid myectomy is an effective treatment modality for BEB in those patients who are refractory to botulinum toxin injections. Myectomy for BEB decreases the morbidity, botulinum toxin treatment frequency, and long-term expense associated with this disabling condition.