Jeremy D. Clark

CD56 Negative Extranodal NK/T-cell Lymphoma of the Orbit Mimicking Orbital Cellulitis

ABSTRACT Introduction: To report a case of Extranodal NK/T-cell lymphoma of the orbit mimicking orbital cellulitis. Case Description: A 52-year-old healthy male presented to our institution after 3 months of treatment for sinusitis with antibiotics and steroids. The patient was transferred due to the presence of an “orbital abscess” on CT with orbital signs that not responding to antibiotics. Clinical examination was significant for decreased vision in the affected orbit of 20/50, a trace RAPD OS, elevated IOP of 30 OS, proptosis and grossly decreased motility with diplopia, periorbital edema and chemosis. Dilated funded exam was unremarkable. CT imaging demonstrated a left sided pan-sinusitis, a medial “orbital process” with proptosis and erosion of the cribiform plate. The patient was taken for an emergent orbital exploration for histopathologic diagnosis. Intraorbital and sinus biopsy was consistent with extranodal NK/T-cell lymphoma, with extension into the skull base and left orbital space. The patient was started on radiation therapy followed by chemotherapy. Comments: The authors demonstrate how the acute presentation of an aggressive extranodal NK/T-cell lymphoma can present in a similar fashion as orbital cellulitis. Additionally, the case highlights that a unilateral pansinusitis with involvement of the skull base and orbit is likely due an aggressive malignant process in an immune competent patient.

Radiographic evidence of prominent retro and suborbicularis oculi fat in thyroid-associated orbitopathy

ABSTRACT Purpose: To compare the radiological differences in retro-orbicularis oculi fat (ROOF) and suborbiculars oculi fat (SOOF) among patients with thyroid-associated orbitopathy (TAO) and normal subjects using computed tomography (CT).Methods: A retrospective analysis of orbital CTs was performed in 39 consecutive patients, who were imaged between October 2005 and June 2009. Bilateral orbital CTs of 16 patients with a final report significant for thyroid orbitopathy and 23 normal subjects were evaluated. All of the CTs consisted of 0.75 mm thick axial slices with 1.5 mm coronal reconstructions. Using the axial soft tissue windows, the ROOF and SOOF tissues were identified. The maximum ROOF thickness was measured perpendicular to the frontal bone, immediately superior to the supraorbital rim. Similarly, the maximum SOOF thickness was measured perpendicular to the zygomatic bone, immediately inferolateral to the infraorbital rim. The radiologist was blinded to the CT reports while conducting the measurements. Multivariable analysis of the two groups was then performed for comparison.Results: Seventy-eight ROOF and SOOF measurements were obtained from 16 TAO patients with and 23 patients without TAO. The female-to-male ratio was 6:1 in the TAO group and only 3:2 among the normal subjects. The mean axial ROOF thicknesses was 3.8 ± 1.9 for TAO patients and 2.8 ± 1.0 mm in patients without TAO, while the SOOF thickness was 4.4 ± 1.0 and 3.4 ± 1.0 mm in the 2 groups, respectively. To account for bilateral measurements, the mixed model analysis was used. After controlling for age, gender, and ethnicity, the mean TAO ROOF and SOOF thicknesses were 1.0 mm (p = 0.04) and 0.9 mm (p < 0.01) greater than the control group, respectively.Conclusion: Retro-orbicularis oculi fat (ROOF) and suborbicularis oculi fat (SOOF) hypertrophy are two additional radiologic CT measurements that may be valuable in establishing the diagnosis of thyroid-associated orbitopathy.

Recession and Extirpation of the Lower Eyelid Retractors for Paralytic Lagophthalmos.

Purpose:To describe a technique to correct lower eyelid malposition and lagophthalmos due to facial nerve palsy. Methods:Chart review was performed and identified 13 patients with facial nerve palsy, who presented with paralytic eyelid malposition and were treated with recession and extirpation of the lower eyelid retractors between September 2012 and March 2014 by 1 surgeon (HBL). Results:A total of 12 eyelids in 11 patients met inclusion criteria. Mean preoperative MRD2 was 10 mm (range, 9.0–12.0 mm) and the mean postoperative MRD2 was 7 mm (range, 5.0–9.0 mm). The MRD2 improved an average of 3.0 mm in each patient (range, 2.0–4.0 mm). Patients had an average of 6.9 mm (range, 4.0–10.0 mm) of lagophthalmos preoperatively, which improved to 2.1 mm (range, 0.0–4 mm). The amount of lagophthalmos improved an average of 4.8 mm in the patients. There were no complications encountered in the patients. All patients had a subjective improvement in ocular comfort. Conclusions:The authors’ surgical technique is effective in addressing lower eyelid malposition and ocular surface disease in paralytic lagophthalmos.

Nodular Fasciitis of the Orbit.

A 13-month-old boy was presented with new onset proptosis of the right eye. CT scan and MRI showed an enhancing mass in the right superior orbit with local bone remodeling and erosion. A craniotomy was performed for biopsy and sub-total resection. Histopathology and immunohistochemistry confirmed the lesion to be nodular fasciitis. Nodular fasciitis lesions are classically found in the anterior ocular adnexa, especially in pediatric patients. This is the first reported case of nodular fasciitis arising in the posterior orbit of a child younger than 16.

Foreign Body Granuloma Following Orbital Reconstruction with Porous Polyethylene.

Porous polyethylene is commonly used in the orbit for fracture repair and anophthalmic reconstruction; it reportedly has a good safety profile and integrates well into host tissue. Foreign body reaction to porous polyethylene has been reported in facial tissue, but to our knowledge, not in the orbit. The authors report 2 cases of foreign body inflammatory giant cell reaction in patients who underwent orbital fracture repairs with porous polyethylene implants.

Orbital cellulitis and corneal ulcer due to Cedecea: First reported case and review of the literature

ABSTRACT Cedecea is a gram-negative bacterium from the family Enterobacteriaceae, rarely associated with human infection. We report the first case of an orbital cellulitis and corneal ulcer due to Cedecea in a patient who sustained a motor vehicle accident and was then found to have a retained wooden orbital foreign body.

Hairpulling causing vision loss: a case report

ABSTRACT Subperiosteal extension of a subgaleal hematoma (SGH) to the orbit is a reported, but rare complication of trauma. This report details a 13-year-old African-American male who originally presented to the emergency department after trauma with headache and was found on CT imaging to have a contained subgaleal hemorrhage. He presented 2 days later with increased pain and proptosis of the left eye with findings of decreased visual acuity, elevated intraocular pressure, proptosis, and complete external ophthalmoplegia. Repeat imaging revealed enlargement of the SGH with subperiosteal extension into the left orbit. He required an emergent lateral canthotomy with inferior and superior cantholysis, followed by surgical drainage of the subperiosteal and SGH. Hematologic workup for coagulopathy was negative. The authors urge point-of-care providers to consider ophthalmic evaluation for patients with large SGHs where orbital extension and vision loss may occur. Furthermore, SGH causing orbital compartment syndrome may develop in patients who have normal blood work and clotting factors.

Ophthalmologic Injuries as a Complication of Maxillofacial Surgery

Managing traumatic injuries to the orbit and periorbital structures requires an individualized approach to these complex patients. The ideal surgical and medical plan is not always clear. The orbital specialist must use the most updated and appropriate recommendations from our own and other subspecialty studies to help guide this decision-making process.

Ophthalmic considerations in patients with Pfeiffer syndrome

Purpose We report here a newborn male infant with striking features consistent with severe Pfeiffer syndrome type II, including cloverleaf skull deformity with pansynostosis, extreme proptosis, upper extremity contractures, broad big toes and thumbs with varus deviation and genetic mutation in the FGFR2 gene. The authors review the ophthalmic complications in Pfeiffer syndrome and discuss the unique surgical strategies used for obtaining adequate corneal coverage in these unique patients. Observations Ophthalmic considerations in Type 2 Pfeiffer Syndrome include vision loss secondary to increased intracranial pressure, and extreme proptosis as a result of orbitostenosis and midfacial retrusion. Our patient has undergone multiple ophthalmic/oculoplastic, neurosurgical, and midfacial surgeries as a result of corneal deterioration due to extreme exorbitism. Conclusions and importance It is important for ophthalmologists to be aware of the ophthalmic complications associated with patients with craniosynostosis syndromes. Our case identifies the importance of close communication between ophthalmology and plastic reconstructive surgery to help formulate the most successful plan in treating corneal decompensation and proptosis in Pfeiffer Syndrome patients.

Pigmented Epithelioid Melanocytoid Tumor of the Ocular Adnexa

An 8-year-old girl with a history of microphthalmia in the right eye presented with a left medial upper eyelid mass with a dark blue-green nodule that could be seen through the skin but did not appear to involve the overlying epidermis. A biopsy demonstrated a pigmented epithelioid melanocytoid tumor with rare mitoses arising in association with a congenital nevus and positive margins. Due to the pathological findings, the patient underwent excision of the tumor with 5-mm margins and a sentinel lymph node biopsy. The re-excision of the upper eyelid margins demonstrated residual benign congenital melanocytic nevus, but did not reveal residual melanocytic lesion. The parotid sentinel node biopsy revealed benign and cytologically mature nevus nests in the capsule and septa, but there was no evidence of involvement with the pigmented epithelioid melanocytoid tumor. The patient underwent successful eyelid reconstruction and had no evidence of recurrence or metastasis after 2 years.