Novel teprotumumab treatment of severe thyroid dermopathy; ototoxicity as an adverse side effect.

Pretibial myxedema, more generally thyroid dermopathy, results from mucopolysaccharide accumulation in the dermis, typically between the knee and dorsal foot. Thyroid dermopathy presents in Graves disease, but can occur in Hashimoto thyroiditis, primary hypothyroidism, and euthyroid patients. Treatment of thyroid eye disease with teprotumumab is established in the literature, with few case reports also showing improvement in pretibial myxedema. Reported is a 76-year-old man with thyroid eye disease and pretibial myxedema treated with teprotumumab; improvement was demonstrated in both conditions. He developed "muffled" hearing as an adverse effect, a complication not widely published in the dermatology literature. At 18 months post-treatment, his symptoms are stable without recurrence, but hypoacusis persists. Given the long-term efficacy and side-effects, dermatologists should recognize the potential benefits and risks of using teprotumumab for thyroid dermopathy. A baseline audiogram may be considered prior to therapy. Additionally, longitudinal data is needed to document the benefits and risks of this novel therapy.

Sino-orbital osteochondroma with malignant transformation to osteosarcoma.

Purpose: Osteosarcoma is an aggressive malignant osteogenic tumor that commonly arises in long bones of pediatric populations. Primary osteosarcomas of the head and neck are rare, comprising less than 0.5% of malignancies in this region, usually affecting the mandible or maxilla. Here we present an extraordinary case of a rare benign osteochondroma of the ethmoid sinus and bilateral orbits evolving to an intermediate grade osteosarcoma. Observations: An 80-year-old woman with a history of right orbital tumor resection 20 years ago presented to our clinic with right eye proptosis and palpable bony prominence of the right orbit and nasal bridge. Partial resection demonstrated sino-orbital osteochondroma. Relapse a year later prompted repeat partial resection with unchanged histology. The patient was followed clinically until an abrupt relapse four years after initial presentation. Imaging demonstrated a large bony mass involving the right orbit, ethmoid and frontal sinuses, and anterior cranial fossa. Repeat debulking confirmed transformation to intermediate grade osteosarcoma. Conclusions: Osteochondroma is an extremely rare tumor in the orbit with only three cases previously reported. This patient is the first known case of benign osteochondroma of the orbit undergoing malignant transformation to osteosarcoma. Rapid progression of orbital osteochondroma should raise the suspicion of malignant transformation to osteosarcoma and prompt biopsy. Our patient subsequently underwent palliative radiation treatment and is stable with no gross progression.

Unusual Case of a Caruncular Oncocytoma.

ABSTRACT: Oncocytomas are benign neoplasms that are most often found in the salivary glands and kidneys. Ocular oncocytoma typically present as an asymptomatic dark blue papule on the lacrimal caruncle. Histologically, the tumor is composed of oncocytes, cells notable for eosinophilic cytoplasmic granules representing large quantities of dysfunctional mitochondria. The neoplastic proliferation may be partially or totally encapsulated and may be surrounded by myoepithelial cells. Discussed is the pathophysiology and histopathology of an ocular oncocytoma from the right lacrimal caruncle of a 68-year-old man. The patient complained of a foreign body sensation and tearing associated with a purpuric 1 × 1-mm papule of the right caruncle that had been present for 6 months. An excisional biopsy showed an oxyntic neoplasm with small cystic glandular spaces associated with goblet cells and mucin, confirmed by mucicarmine stain. Given the rarity of ocular oncocytomas and presence of mucin, exclusion of an endocrine mucin-producing sweat gland carcinoma was necessary. Peripheral myoepithelial cells noted on p63 immunostaining are seen in both endocrine mucin-producing sweat gland carcinoma and oncocytomas. However, endocrine mucin-producing sweat gland carcinoma is excluded by the presence of goblet cells, the uniform oxyphilic cytoplasm of the epithelial cells, and the negative immunohistochemical staining for neuron-specific enolase, chromogranin, synaptophysin, estrogen receptor, and progesterone receptor. Complete surgical excision is the treatment of choice for oncocytomas. This case highlights the clinical and histopathological presentation of ocular oncocytomas and raises awareness of this rare entity for both the practicing dermatologist and dermatopathologist.

Caruncular Dacryops After Cataract Surgery With Histopathological Characterization.

ABSTRACT: Caruncular dacryops is a rare cyst of lacrimal tissue most commonly found in the palpebral lobe of the lacrimal gland. The exact cause of dacryops is unclear, although it may be congenital or associated with trauma, infection, or inflammation. The pathophysiology and histology of a caruncular dacryops of the left lacrimal gland in a 68-year-old woman is reported. The patient presented 3 months after a cataract surgery with symptoms of irritation and pruritus of the left eye. A 6- × 6-mm clear cyst of the lacrimal gland prolapsing into the lateral fornix was noted on physical exam. Histological examination of the cyst showed a double layer of flat cuboidal epithelium surrounded by fibrous tissue with minor lacrimal glands present. Periodic acid Schiff-diastase and mucicarmine staining showed the presence of goblet cells dispersed in the epithelial cells. Complete excision of the cyst is the treatment of choice for most caruncular dacryops, and our case remains without recurrence after excision. This case highlights the clinical and histological presentation of caruncular dacryops and raises awareness of their incidence following presumed trauma during cataract surgery.

Endometriosis of the Eyelid, an Extraordinary Extra-abdominal Location Highlighting the Spectrum of Disease.

Cutaneous endometriosis is an uncommon dermatologic disorder predominantly seen in young women. Most commonly, it presents within a region of a previous surgical scar, often in relation to a gynecologic procedure on the abdomen or in close proximity to the umbilicus. The typical clinical presentation is that of papules or nodules with monthly cyclical pain and size variation. Histologically, the lesions are composed of endometrial stroma and glands. The pathophysiology is not well understood but is believed to be due to metastasis or seeding of endometrial cells. When this uncommon disorder occurs outside of the most typical clinical setting, it may cause some diagnostic difficulty. In this report, we present the first known case of cutaneous endometriosis on the eyelid.

Severe Oculofacial Sequelae of Cutaneous Blastomyces dermatitidis.

This study reports a case of Blastomyces dermatitidis soft tissue infection resulting in a disfiguring lower eyelid ectropion from cicatricial and postinflammatory cutaneous changes. Primary treatment included intravenous amphotericin B followed by long-term oral itraconazole, which resulted in complete remission of the disease without debridement, after which cicatricial ectropion was repaired surgically with scar release, full-thickness skin graft, and temporary Frost tarsorraphy. Cutaneous blastomycosis may cause severe oculofacial sequelae, ranging from eyelid ectropion to widespread facial cicatrix, and may mimic other more common infectious processes, in addition to malignancy. Recommended antifungal therapy includes induction with intravenous amphotericin B and a long course of oral antifungals, preferably coordinated in conjunction with an infectious disease specialist. Ectropion repair should be delayed until the inflammatory response has completely healed. If the ocular surface is compromised or nearby ocular structures are threatened, primary debridement and repair may be indicated.

Orbital osteoma: clinical features and management options.

PURPOSE: This study reviews the clinical presentation and management of 11 cases of sino-orbital osteoma. METHODS: The medical records of patients with primary (originating from orbital bone) and secondary (originating from the paranasal sinuses) orbital osteoma from the academic practices of 4 surgeons (A.V.C., M.J.L., P.J.D., V.D.D.) were reviewed for clinical presentation and course, pathologic study, and radiologic reports. A Medline search of English-language literature on orbital osteomas was conducted for comparison with these findings. RESULTS: Eleven cases of primary (1) and secondary (10) orbital osteoma were reviewed, with a mean follow up of 16 months. Seven patients were women. Ages ranged from 15-68 years, with a median of 40 years. Presenting complaints included slowly progressive globe displacement, palpable bony nodule, pain, and diplopia. Surgery was performed in 10 cases. Surgical approach varied according to location and size of each lesion and was performed in combination with otolaryngology and neurosurgery services as needed. Reconstruction included sculpting osteomatous bone to natural orbital contours, repair of orbital wall defects with implants, and obliteration of frontal sinus. Lesions demonstrated mixed compact, cancellous, and fibrous histologic subtypes. CONCLUSIONS: Osteomas are the most common tumor of the paranasal sinuses (noted in up to 3% of coronal CT images), but secondary extension in or primary involvement of the orbit is rare. A variety of surgical approaches led to successful outcomes in this series. Complete surgical removal is not always necessary, and partial sculpting may relieve symptoms and cause less surgical morbidity in selected cases.

Multidetector CT of nasolacrimal canal morphology: normal variation by age, gender, and race.

PURPOSE: To accurately characterize anatomical age, gender, and racial differences in normal nasolacrimal canal morphology and to describe a new, accurate technique for measuring nasolacrimal canal parameters based on high-resolution CT. METHODS: The CT images of 72 patients were reviewed retrospectively and grouped according to age, gender, and race. Patients with history of long-term sinusitis, facial trauma, or nasolacrimal duct pathology were excluded from study. Variations in nasolacrimal duct length, minimal and maximal diameter, and other metrics were recorded and compared across each category. All patient records were deidentified to protect privacy. RESULTS: Nasolacrimal canal length and volume were significantly greater in men than those in women. A trend was noted for greater canal cross-sectional diameter in patients older than 50 years when compared with that in younger patients. Significant differences in canal diameters were noted at the canal apex and base of older patients compared with those of younger patients. The cross-sectional area of the canal base was greater in black patients than that in Caucasian patients. CONCLUSIONS: Although some significant age, gender, and racial variations of nasolacrimal canal morphology exist, these do not fully explain the epidemiology of primary acquired nasolacrimal duct obstruction. The new method described here for measuring nasolacrimal canal metrics is straightforward and theoretically more accurate than those previously defined in the literature.

Vertical vergence adaptation produces an objective vertical deviation that changes with head tilt.

PURPOSE: To document the cyclovertical ocular motor mechanism used for vertical fusion in healthy subjects, and to explore whether vertical vergence training in healthy individuals can produce objectively confirmed vertical deviations that change with head tilt, revealing a basic mechanism that can produce a pattern of misalignment in an otherwise normal ocular motor system that is similar to superior oblique muscle paresis (SOP). METHODS: Seven subjects with normal orthoptic examinations were adapted to vertical image disparities using our tilting haploscopic eye-tracking apparatus presenting concentric circle targets without torsional cues. Static eye positions were recorded with head straight and when tilted 45 degrees to the left and right, during both binocular and monocular viewing. RESULTS: Vertical fusional vergence was accompanied by a cycloversion, with the downward-moving eye intorting and the upward-moving eye extorting, implicating primary involvement of the oblique extraocular muscles. After adaptation to the slowly increasing vertical target separation, all subjects developed a temporary vertical deviation in the straight ahead position that increased with head tilt to one side and decreased with head tilt to the other side. CONCLUSIONS: These results not only show that head-tilt-dependent changes in vertical deviation are not necessarily pathognomonic for SOP, but also, and more importantly, suggest mechanisms that can mimic SOP and suggest a possible role for vertical vergence training in reducing deviations and thus the amount of head tilt required for fusion. Ultimately, vertical vergence training may provide an adjunct or alternative to extraocular muscle surgery in selected cases.

Arcanobacterium hemolyticum orbital cellulitis: a rare but aggressive disease.

The clinical presentation and course of orbital cellulitis in a young adult resulting from Arcanobacterium hemolyticum frontal sinusitis are presented in detail. This case illustrates the importance of a multidisciplinary approach for refractory and aggressive orbital cellulitis. A high level of suspicion for A. hemolyticum must be maintained in such cases, because it has proven to be a rare but aggressive, potentially occult, and life-threatening pathogen.

Metastatic pancreatic carcinoma to the orbital apex presenting as a superior divisional third cranial nerve palsy.

Metastatic tumors to the orbit are rare, especially from a primary pancreatic carcinoma. A 59-year-old man presented with 4 weeks of right eye pain and eyelid swelling. There was right upper eyelid ptosis associated with a right supraduction deficit consistent with a superior divisional third cranial nerve (CN III) palsy. Magnetic resonance imaging revealed a right orbital apex lesion. A right orbital exenteration was performed for intractable and severe pain. Surgical pathology demonstrated a poorly differentiated carcinoma that was ultimately felt to be derived from the pancreas. In this report, we describe the clinical and neurological imaging findings of a superior divisional CN III palsy as the presenting manifestation of a presumed metastatic pancreatic carcinoma to the orbital apex, and review the neuroanatomy of CN III with particular emphasis on the anatomical bifurcation of the nerve into a superior and inferior division.

Clinical usefulness of orbital and facial Time-Resolved Imaging of Contrast KineticS (TRICKS) magnetic resonance angiography.

PURPOSE: The clinical usefulness of orbital and facial Time-Resolved Imaging of Contrast KineticS (TRICKS) MRI was assessed quantitatively and qualitatively. METHODS: A retrospective chart review of the years 2001 to 2011 was conducted at the University of Wisconsin, Madison. Patients were selected based on tumor location (extracranial head and neck) and evaluation with TRICKS imaging at any stage of the clinical course. The TRICKS protocol presented in this article allows measurement of specific tumor characteristics including lesion morphology, vascular anatomy, flow dynamics within the lesion, rapidity and sequential nature of contrast enhancement, and lesion distensibility. Cases involving changes in diagnoses and/or management as a result of TRICKS interpretation are presented in detail. RESULTS: The imaging records of 49 patients were reviewed. Most lesions were located in or around the orbit (32 lesions, 65%). Benign vascular tumors comprised the largest subcategory (32 lesions, 65%) in the cohort. Of the 20 cases with histopathological data, interpretation of MRI with TRICKS magnetic resonance angiography successfully predicted 17 (85%). Characteristic enhancement patterns for the most commonly encountered lesions are presented, in addition to a qualitative analysis of how TRICKS contributed to select cases. TRICKS helped clarify diagnosis and/or redirect management in 19 of the 49 cases in this series. CONCLUSION: To the best of the authors' knowledge, this is the largest study to date of patients evaluated with TRICKS MRI/magnetic resonance angiography for orbital and facial tumors. MRI with TRICKS magnetic resonance angiography imaging protocol offers a minimally invasive, safe, and effective diagnostic instrument in selected patients with clinical diagnostic uncertainty and in those patients requiring high-resolution vascular mapping for management planning.

Comparison of complication rates of porous anophthalmic orbital implants.

BACKGROUND AND OBJECTIVE: Porous anophthalmic orbital implants are used widely. This study evaluates risk factors for porous anophthalmic orbital implant complications and compares complication rates of hydroxyapatite, porous polyethylene, and polyglactin mesh-wrapped aluminum oxide implants. PATIENTS AND METHODS: The records of 105 patients (110 eyes) who received porous anophthalmic orbital implants for any indication were reviewed retrospectively. Complications were recorded and correlated with potential risk factors, including implant material. All patient records were de-identified to protect privacy. RESULTS: Porous polyethylene and aluminum oxide implants were associated with higher exposure rates (porous polyethylene: odds ratio 6.1 [1.29, 29.1]; aluminum oxide: odds ratio 6.0 [1.58, 23.1]; P = .004) and higher overall complication rates compared to hydroxyapatite implants. CONCLUSION: Implant material may be a risk factor for several anophthalmic clinical outcomes.