Imran Jivraj, Gino R. Somers, Michel J. Belliveau, David Malkin, Dan D. DeAngelis
This case highlights the management of orbital rhabdomyosarcoma in a child with Li Fraumeni syndrome (LFS). Treatment with chemotherapy and eventual orbital exenteration enabled margin-free control of the tumor. Radiation therapy was avoided to reduce the risk of inducing additional malignancy. Reactive orbital hyperostosis was observed postoperatively and was confirmed with surgical biopsy of the orbital roof. In this case, systemic surveillance imaging, which is necessary in patients with LFS, revealed an adrenal cortical carcinoma.
Ruth K. Jones, Zanna I. Currie, Sachin M. Salvi
Microphthalmic cysts are rare. Although small cysts can be left in situ to promote orbital expansion, large cysts require drainage or surgical excision. Complete surgical excision is notoriously difficult, and incomplete excision may result in cyst reformation. We describe a novel method of using fibrin glue to aid successful complete removal of a large recurrent microphthalmic cyst in a 6-year-old child who previously had multiple drainage and surgical attempts.
Stacy M. Scofield-Kaplan, Elizabeth K. Weidman, Gul Moonis, Lora R. Dagi Glass
Detection of wooden foreign bodies within the orbit can be difficult on imaging, including computed tomography (CT). When visible, wood appears iso- or hypodense and can mimic air or fat. We report the case of a 3-year-old boy with progressive orbital cellulitis following a penetrating wood injury to the right lower lid. CT imaging revealed a linear hyperdensity contiguous with an orbital abscess. He underwent a right anterior orbitotomy with abscess drainage, during which exploration revealed a 2.3 cm retained wooden foreign body. The appearance of wood as hyperdense on imaging is unusual. In the appropriate context, high clinical suspicion for retained wood should be maintained with any irregularity on CT imaging.
Anagha Medsinge, Christin Sylvester, Elizabeth Tyler-Kabara, Susan Tonya Stefko
Osteopetrosis is a rare disorder of bone remodeling characterized by defective resorption leading to excessive skeletal mass including optic canal. Compression of the optic nerve from the narrowed optic canal is the most common cause of vision loss in children with osteopetrosis. We report the case of a 6-month old girl with osteopetrosis who underwent bilateral optic canal decompression via endoscopic transcaruncular approach for progressive deterioration of visual function secondary to compressive optic neuropathy from narrowed optic canals. The patient showed improvement in visual function postoperatively.
James A. Garrity, Daniel A. Greninger, Noha S. Ekdawi, Eric A. Steele
To describe surgical management and outcomes for large-angle esotropia of ≥50Δ secondary to Graves ophthalmopathy using combined initial nonadjustable medial rectus recessions and lateral rectus resections.
The medical records of consecutive patients undergoing strabismus surgery for large-angle esotropia secondary to Graves ophthalmopathy from 1995 to 2012 by a single surgeon at each of two institutions was performed. Patient characteristics, surgical technique, and pre- and postoperative measurements of ocular alignment were analyzed. A modified Gorman diplopia scale was used to assess outcome.
Of 38 patients, 36 had bilateral nonadjustable medial rectus recessions and lateral rectus resections as initial treatment for esotropia, and 6 patients underwent simultaneous vertical muscle surgery. Mean preoperative horizontal deviation was 60Δ and mean preoperative vertical deviation was 10Δ. Of the 38 patients, 19 (50%) reached the primary outcome, including 5 of 6 (85%) who had no preoperative vertical strabismus. The indications for reoperation were vertical strabismus in 13 of 21 patients (62%), residual esotropia in 7 of 21 (33%), and consecutive exotropia in 1 of 21 (5%). With a median follow-up of 13.2 months after first surgery, 32 of 38 patients (84%) reached the secondary outcome.
Combining nonadjustable medial rectus recessions with lateral rectus resections can be a beneficial primary treatment for large-angle esotropia in patients with Graves ophthalmopathy, especially in those patients with small or no associated vertical strabismus.
Deep Parikh, Rand Rodgers, Sylvia Kodsi
We report the case of a 13-year-old boy who presented with a 2-month history of left eyelid swelling, ecchymosis, and epiphora. Magnetic resonance imaging revealed a lobulated lesion in the region of the left lacrimal sac extending to the left nasolacrimal duct. Biopsy revealed diffuse large B-cell lymphoma of the lacrimal sac. Chemotherapy was initiated, consisting of rituximab, methotrexate, cytarabine, doxorubicin, cyclophosphamide, and vincristine. The lesion resolved within weeks of treatment, and the patient remained disease free at 1 year. Primary orbital lymphoma is rare in children; primary diffuse large B-cell lymphoma of the lacrimal sac in a child has not been reported previously.
Ahmad S. Alfaar, Guillermo Chantada, Ibrahim Qaddoumi
To the Editor: We read with interest the article by Shehata and colleagues, which suggests that measuring serum and aqueous humor levels of survivin and TGF-B1 may be beneficial for detection and follow-up in patients with retinoblastoma (RB).1 Targeting survivin does improve chemosensitivity in patients with RB.2,3 Nevertheless, survivin is not cancer specific and is involved in many essential physiological processes, which makes it a poor choice for targeted therapy.4
Bipasha Mukherjee, Manideepa Banerjee, Sumita Agarkar
A dermoid cyst is a choristoma that arises from the displacement of ectoderm to a subcutaneous location along embryonic closure lines. Rarely dermoids have a deep intraorbital component that is connected to a superficial component through a defect in bone, also known as dumbbell dermoid. We report the case of a young boy who was misdiagnosed having chronic osteomyelitis of the zygoma, based on the clinical and radiological features. On surgical exploration he was found to have a dumbbell-shaped dermoid with a bony defect. Histopathological features were consistent with a diagnosis of dermoid cyst with chronic inflammation.
Jenna May Kim, Marez Megalla, Martha Howard, John Sinard, Renelle Pointdujour-Lim
We report the case of a 76-year-old man who presented with left eye pain, eyelid swelling, and restricted extraocular motility 3 days after strabismus surgery. Computed tomography of the orbits did not show drainable abscesses, but magnetic resonance imaging on postoperative day 9 showed choroidal detachment with retrobulbar abscess and dural enhancement despite broad-spectrum intravenous antibiotic use. Following incision and drainage of the abscess, which grew methicillin-resistant Staphylococcus aureus, the patient showed improvement in visual acuity, pain, and extraocular motility. This is the first report of choroidal detachment occurring in the setting of orbital cellulitis after strabismus surgery.
Brock Alonzo, Beth Edmunds, Allison R. Loh
Glaucoma drainage devices are commonly used in the surgical treatment of children with glaucoma. We report a case of unusually large reservoir formation causing proptosis, exposure keratopathy, and motility deficits. This case highlights the importance of B-scan and axial length measurements in differentiating proptosis from buphthalmos in children with glaucoma. Reducing the reservoir improved the proptosis and resolved the motility and exposure issues.