Ruchi Mittal, Devjyoti Tripathy
Orbitopalpebral cysts are most commonly associated with microphthalmic eyes (microphthalmos with cyst). We report a 15-year-old girl with a large orbitopalpebral cyst in the absence of associated microphthalmos. The patient presented with a massive swelling involving the left lower eyelid overhanging the left cheek and occupying much of the left orbital cavity. The globe was not visible on clinical examination. No history of trauma or surgery preceding the development of the cyst was reported by the patient or her father. Imaging showed a small eyeball displaced superiorly along the anterior part of the orbital roof. On excision of the cyst, a normal-sized globe with a cataractous lens was noted. Histopathological examination revealed the cyst to be of conjunctival origin.
Jenna Tauber, Daniel J. Polla, Sunju Park
Kearns-Sayre syndrome (KSS) is a rare mitochondrial DNA (mtDNA) deletion syndrome that typically presents before 20 years of age and is characterized by chronic progressive external ophthalmoplegia, pigmentary retinopathy, and a combination of cardiac conduction defects, cerebellar ataxia, and elevated cerebrospinal fluid protein levels. The mtDNA defects interfere with oxidative phosphorylation and can affect a number of cellular energy processes in various organs. We report the case of a 15-year-old girl with KSS that was uniquely associated with bilateral, symmetrical exophthalmos.
David Somsen, Gena Heidary
Orbital cellulitis is extremely uncommon following strabismus surgery. When it occurs, the infection has been reported to present from day 1 to within 1 week following surgery and has the potential for significant morbidity. We report the case of a 6.5-year-old boy presenting with unilateral orbital cellulitis growing group A Streptococcus pyogenes on postoperative day 1, after uncomplicated bilateral medial rectus recessions. The patient had two contacts with streptococcal pharyngitis at the time of surgery but was completely asymptomatic himself. We hypothesize that these contacts may have led to the rapid onset of his orbital cellulitis.
Nathaniel L. Simmons, Richard M. Robb, David J. Tybor, Aubrey L. Gilbert
We analyzed clinical and histopathologic data of 97 pediatric patients who underwent excision of dermoid cysts. On review, 16.5% of the sample population demonstrated localized chronic inflammatory changes, including the presence of giant cells and epithelial disruption. These features were considered indicative of prior cyst rupture. Age at time of initial presentation was significantly older and cyst size was significantly larger in patients with histopathologic signs of previous rupture. Longer time to presentation and time to excision were associated with increased odds of spontaneous rupture.
Allister S. Lee, Timothy J. Sullivan
To report our experience in the diagnosis and management of invasive fungal infections with orbital involvement in children from a subtropical population.
The medical records of children (<18 years of age) with orbital mycosis and treated by the senior author (TJS) from 1995 to 2017 in multiple pediatric tertiary centers were reviewed retrospectively.
Six patients (aged 12 weeks to 15 years) were included in this series. Four patients had confirmed infection with isolated pathogens, including mucormycosis (3) and Exserohilum (2). One patient rapidly deteriorated and died before biopsy could be performed; however, the patient was presumed to have invasive fungal disease. Four patients had underlying hematological malignancy, and 1 presented in diabetic ketoacidosis. Orbital apex syndrome was observed in one patient. All patients received liposomal amphotericin B and five underwent at least one debridement surgery. One patient proceeded to orbital exenteration and survived. The overall survival rate was 67%.
Orbital mycoses can affect children of all ages. Immunocompromised patients are particularly at risk, and mortality rates are high. In a subtropical population, these infections may be caused by a different spectrum of fungi compared to other climate zones. We believe extensive surgical debridement, including exenteration may still be necessary in the management of this disease in a young population, particularly if there is extensive orbital involvement.
Lana del Porto, Anne-Marie Hinds, Naz Raoof, Christen Barras, Indran Davagnanam, Joanne Hancox, Gillian Adams
To determine the extent of superior oblique enlargement in thyroid eye disease (TED) by comparing the cross-sectional superior oblique areas of TED patients with those of unaffected control subjects.
The medical records of TED patients treated for strabismus from January 2005 to January 2016 were reviewed retrospectively for demographic and surgical data. The cross-sectional superior oblique area was compared to age-matched controls on high-resolution orbital computed tomography (CT) using a standardized protocol.
A total of 46 TED patients and 18 controls were included. The mean superior oblique cross-sectional area in TED subjects was 250% larger than in controls (22.88 ± 6.64 mm2 vs 9.32 ± 1.85 mm2. The mean cross-sectional area was >3 standard deviations from the mean of the control group in 96% of TED patients.
Superior oblique enlargement in TED may occur more frequently than generally recognized, challenging the notion that TED is primarily a disease of the rectus muscles.
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.
Frank L. Brodie, Khashayar Nattagh, Vinil Shah, Vivek Swarnakar, Shezhang Lin, Tatiana Kelil, Derrick Gillan, Dylan Romero, Alejandra G. de Alba Campomanes
Children with craniofacial malformations frequently require spectacles but have difficulty finding an acceptable fit with current offerings of pediatric spectacle frames. We describe a novel method for creating custom 3D-printed spectacle frames based on a 3D reconstruction of a prior computed tomography scan. This method offers the ability to create better-fitting spectacles to children who are not served by “off the rack” frames.
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.