Giant Orbital Hydrocystoma in Children: Case Series and Review of the Literature

Objective: To describe the clinical features, ancillary diagnostic studies, and treatment outcomes in a cohort of pediatric patients with giant orbital hydrocystomas.

Design: Retrospective case series.

Participants: Pediatric patients with giant orbital hydrocystomas treated in the practice of one surgeon (PDL).

Methods: A retrospective review of the clinical charts of pediatric patients with orbital hydrocystoma was performed and diagnostic information collected. Results were reviewed and compared with reported clinical data in the literature.

Main Outcome Measures: Clinical presentation and histopathological findings of pediatric orbital hydrocystomas.

Results: Three pediatric cases of giant orbital hydrocystoma were encountered, each with an unusual feature, including deep orbital location, occurrence following trauma, and eccrine pathology.

Conclusion: Giant orbital hydrocystomas may present in the pediatric population. Ophthalmologists should be cognizant of this entity when evaluating a child with a large, cystic orbital mass.     

Abducens nerve palsy after orbital decompression

Introduction: Double vision after decompression surgery for Thyroid Eye Disease (TED) is well described in the literature and the incidence ranges from 0 to 64%. The Mechanisms for new onset diplopia after orbital decompression are poorly understood. Common theories include: Fibrosis of muscles, displacement of the muscle cone, and reactivation of the TED.

Aim: We present two cases with Abducens nerve palsy after uncomplicated secondary orbital decompression surgery.

Results: Two patients with inactive TED, who were followed for an average of 2 years prior to uneventful secondary decompression surgery, presented at the first postoperative visit with double vision and limitation of abduction in the recently operated eye. Magnetic resonance imaging(MRI) was done in both cases and revealed no abnormal bleeding or scaring.

Discussion: Our two cases of Abducens palsy following reoperative orbital decompression may be due to ischemic neuropathy caused by postoperative hemorrhage or inflammation.     

Juvenile Xanthogranuloma–A Rare Cause of Unilateral Orbital Swelling in an Elderly Patient

Objective: To report the clinical and pathologic features of an elderly patient with a unilateral orbital swelling and proptosis caused by Juvenile Xanthogranuloma diagnosed and confirmed by orbital biopsy.

Design: Interventional case report.

Participants: One patient.

Intervention: Steroids (Medrol dose pack) and radiation.

Main Outcome Measures: Unusual clinical presentation and pathological features of Juvenile Xanthogranuloma in the orbit.

Conclusions: Juvenile Xanthogranuloma affecting one orbit is very rare with unilateral involvement in an elderly patient. Steroids and radiation therapy were very effective in treatment and provided impressive results.     

A Case Series and Review of Bisphosphonate-associated Orbital Inflammation

Purpose: To report the largest series of new cases to date of bisphosphate-associated orbital inflammation.

Methods: A retrospective case review of patients with orbital inflammation following treatment with systemic bisphosphonate.

Results: Six patients over an 18-month period (2 males, 4 females) with an average age of 62.2 years had onset of orbital inflammatory symptoms 1–11 days after intravenous bisphosphonate infusion or, in 1 case, 4 weeks after initiation of oral bisphosphonate therapy. Imaging revealed diffuse orbital involvement in 3 cases, isolated lateral rectus muscle involvement in 2 cases, and superior rectus–levator involvement in 1 case. Two patients’ symptoms resolved spontaneously within 2 weeks, and 3 responded rapidly and completely to corticosteroid therapy. The 1 patient on oral bisphosphonate had a slower but complete response to corticosteroid treatment.

Conclusion: Clinicians should be aware of the association between acute orbital inflammation and recent treatment with systemic bisphosphonate medication.     

Regression of a Paraganglioma Tumor of the Orbit

Purpose: To describe a clinical case of an orbital paraganglioma that displayed regression after biopsy alone.

Methods: Case report.

Results: A 75-year-old female was examined for a right orbital tumor suspected to be metastatic breast carcinoma. An orbital biopsy was performed with significant hemorrhage encountered requiring extensive cautery. There was apparent clinical regression of the tumor with no signs of proptosis or eye movement restriction two years after this patient’s biopsy. Histology was consistent with paraganglioma (glomus tumor).

Conclusion: Although we cannot rule out spontaneous regression of this unique tumor, we postulate that tissue necrosis caused by the use of cautery induced regression. Unless encapsulated and easily accessible, we suggest that the best management of this rare tumor is that of observation after being found negative for malignancy by biopsy given their propensity for slow progression and in rare cases, regression.     

Complications and outcomes of grafting of posterior orbital fat into the lower lid-cheek junction during orbital decompression

Purpose: To report the complications of grafting of excised posterior orbital fat into the lower lid-cheek junction at the time of orbital decompression surgery.

Methods: Retrospective review of consecutive patients undergoing orbital decompression combined with grafting of posterior orbital fat to the pre-malar and lateral canthal area (FG). A second group of consecutive patients undergoing orbital decompression but no orbital fat grafting (NoFG) were also studied as a form of comparative control. Standard patient data, including age, sex, visual acuity, degree of proptosis, operative details, diplopia or any other complications was collected. Independent assessment of pre- and post-operative photographs graded the lower lid-cheek junction.

Results: Thirty-four orbits of 29 patients, of which 21 orbits underwent orbital decompression with orbital fat grafting (FG). There were no intraoperative complications, postoperative infections, or visual loss. Complications relating to fat grafting included prolonged swelling in 3 (17%) patients at 3 months, in 1 case lasting 6 months, lower lid lumps in 3 (17%), and fat seepage in 1 (6%). The FG group achieved a greater improvement in the appearance of the lower-lid-cheek junction at 12 months in comparison to NoFG. Mean grade improvement 1.24 ± 1.09 vs 0 ± 0.82 (p = 0.025). Median follow-up was 20 months (range 6–30 months).

Conclusion: Grafting of excised orbital fat during orbital decompression can improve the appearance of the lower lid-cheek junction in patients being treated for thyroid orbitopathy. However, 24% of patients will experience swelling and/or lumpiness requiring several months to settle or further fat excision.     

Bisphosphonate-Induced Orbital Inflammation: A Case Series and Review

Introduction: To present a series of patients with bisphosphonate induced orbital inflammation, and to review the clinical presentation, radiological features, treatment options and outcomes.

Methods: We present a multicentre, retrospective case series review of patients with a clinico-radiological diagnosis of bisphosphonate induced orbital inflammation and review all the reported cases of this complication in the literature.

Results: Four new patients with bisphosphonate induced orbital inflammation were added to the 25 cases in the literature. Intravenous zoledronate was the commonest precipitant (22/29, 75.9%) and inflammation occurred 1–28 (mean 3) days post-infusion. Orbital imaging identified orbital inflammation in 22/29 cases and extra-ocular muscle enlargement in 8/29. Five patients presented with reduced vision of which one – with anterior ischaemic optic neuropathy – did not resolve. The vision resolved in all except one patient, with most requiring steroid treatment.

Conclusions: Bisphosphonates have a pro-inflammatory effect, which can precipitate orbital inflammation. This rare, but potentially serious complication of bisphosphonate treatment should be considered by clinicians using bisphosphonate treatment and by ophthalmologists seeing patients with orbital inflammatory disease.     

“The influence of orbital vector on involutional entropion and ectropion”

Purpose: This study aimed to evaluate the effect of orbital vector and other biometric parameters (i.e. axial globe length, axial globe projection) on the development of involutional entropion or ectropion.

Methods: In this cross-sectional study, 167 eyes from 132 patients were included. Of these eyes, 128 had involutional entropion and 39 had involutional ectropion, all of lower lids. The axial globe projection was measured using Hertel exophthalmometry; axial globe length was assessed by A-mode ultrasound; and orbital vector was determined clinically. Patient-specific categorical variables and continuous variables were compared using the chi-square test and the two-sided t test, respectively. Correlations were derived using the Pearson correlation.

Results: The percentage of females was 59% and 33.3% in the entropion group and in the ectropion group, respectively. A significant association was found between the gender and type of eyelid malposition (p = 0.015). Exophthalmometry reading was greater in the ectropion group than in the entropion group (17.7 ± 2.5 versus 10.8 ± 3.7 mm, respectively; p < 0.001). There was no significant correlation between axial globe length and exophthalmometry reading. Positive orbital vectors were observed in 87.5% of eyelids with involutional entropion. Negative orbital vectors were observed in 92.3% of eyelids with involutional ectropion (p < 0.001). Patients with negative orbital vectors showed greater axial globe projection than patients with positive orbital vectors (18.0 ± 2.1 versus 10.6 ± 3.5 mm, respectively; p < 0.001).

Conclusions: There is an association between orbital vector measurement and involutional entropion and ectropion. Measuring the orbital vector may help predict the development of these lid malpositions.     

Treatment of Dural Carotid-Cavernous Fistulas via the Medial Ophthalmic Vein

Aim: To present a novel approach to treatment of dural carotid-cavernous fistulas via the medial ophthalmic vein.

Design: Retrospective case series.

Material and Methods: In this retrospective case series, we present 2 patients (3 eyes) with Type C dural CCFs, who had failed cannulation via the conventional transfemoral route and the transorbital superior ophthalmic vein approach. They subsequently underwent CCF occlusion via an anterior orbital approach through the medial ophthalmic veins, at the Department of Ophthalmology, National University Hospital Singapore. CCF occlusion was confirmed intraoperatively using angiography. Both patients were evaluated postoperatively for best-corrected visual acuity and resolution of clinical signs and symptoms.

Results: Successful occlusion of CCFs via the medial ophthalmic veins were achieved in all three orbits, with excellent visual and cosmetic outcomes postoperatively.

Conclusion: Dural CCFs may potentially lead to severe visual dysfunction and should be diagnosed and treated promptly. When all venous routes have been exhausted, the transorbital approach via the medial ophthalmic vein remains an excellent and viable alternative to access the fistula. Close cooperation between the orbital, anesthetic and radiological teams is essential in ensuring success of the operation.     

Intralesional corticosteroid injections as treatment for non-infectious orbital inflammation

A retrospective single-center chart review identified 19 patients treated with intralesional corticosteroid injection between 2007 and 2015 for orbital inflammatory disease. Patients were treated in the office, or intraoperatively at the time of orbital exploration and biopsy, with 5–40 mg of triamcinolone acetonide. Average follow up time was 2.44 years.

Information recorded included visual acuity and eyelid position measurements, pre-injection treatment, post-injection treatment, complications, and exam findings.

Nine of the identified patients received intraoperative injections, 9 in the office setting, and one received an injection in each. Eight patients (42.1%) were on oral corticosteroids prior to the injection. All patients showed initial clinical improvement after 1–2 injections and no more than a 1–2 line difference in visual acuity. Overall, six patients (31.6%) required repeat injections due to recurrence of inflammation, and seven (36.8%) required additional oral corticosteroids beyond the initial taper.

Patients pre-treated with oral corticosteroids were more likely to need repeat injections (83.3% vs 16.7% respectively, p = 0.04).

Compared to injections performed in the office setting, intraoperative injections were associated with fewer repeat injections (0% vs. 60.0% respectively, p = 0.01) and less need for post-injection oral steroids (0% vs. 58.3% respectively, p < 0.01). There were no systemic or permanent side effects observed from the injections. Two patients (10.5%) had transient post-injection intraocular pressure elevation, which resolved.

Intralesional orbital corticosteroid injection is an effective first-line and adjunctive treatment for localized and diffuse non-infectious orbital inflammation.     

Lymphocytic Meningitis in Patients with Sympathetic Ophthalmia

Purpose: This study aimed at reporting lymphocytic meningitis in patients diagnosed with sympathetic ophthalmia (SO).

Methods: In this single-center retrospective observational case series, we reviewed cases diagnosed with SO. We analyzed the patients’ inciting injuries, the characteristics of uveitis and the cerebrospinal fluid (CSF) analyses.

Results: Nine patients were diagnosed with SO and CSF analyses were available in all cases. Four cases had lymphocytic pleocytosis, 3 of which showed marked CSF inflammation with more than 300 lymphocytes/mm³. The inciting event in these 3 patients was a globe perforation injury, whereas 4 patients without meningitis had SO following a surgical intervention.

Conclusions: In this case series of patients with SO, lymphocytic meningitis was a common finding. The prevalence of meningitis in patients with SO and its value for the diagnosis of the disease needs to be further studied.     

Transcutaneous Endoscopic Orbital Surgery: A Less Morbid Alternative to Lateral Orbitotomy

Background: Retrobulbar lesions of the orbit situated lateral to the optic nerve are difficult to access. In this article, the authors would like to present a new perspective to approach these lesions where the goals of surgery are met with minimal complications.

Methods: A retrospective analysis of patients’ charts was performed.

Results: For approaching retrobulbar lesions an endoscopic technique was developed. Four patients (2 male and 2 female patients) presenting with retrobulbar lesions lateral to the optic nerve have been included in this study. Two patients underwent endoscopic drainage for orbital abscess and two patients were successfully biopsied endoscopically. No intra-operative complications were noted. Open procedures such as lateral orbitotomy were avoided in all the cases.

Conclusion: Transcutaneous orbital endoscopy is a safe and a versatile technique to approach retrobulbar lesions lateral to the optic nerve. It can be used as an effective alternative to lateral orbitotomy for well selected cases. One can obtain adequate material for histopathological examination and also drain deep-seated orbital abscess using this technique. It would be possible with increasing experience to use this technique for extended applications.     

Endogenous Endophthalmitis in Patients with MRSA Septicemia: A Case Series and Review of Literature

Purpose: To report the presentation, predisposing factors, clinical features and outcome in 6 eyes of 3 patients with endogenous endophthalmitis secondary to methicillin resistant staphylococcus aureus (MRSA) septicaemia.

Methods: Retrospective review of case records of 3 patients who were treated for endogenous endophthalmitis secondary to MRSA septicaemia in a tertiary referral institution.

Results: All three patients had systemic predisposition to endogenous endophthalmitis (diabetes, urosepsis). Two patients presented within 1 week of onset of visual symptoms and the third after 3 months. Blood culture was positive for MRSA in all patients. Visual and anatomical improvement was noted in two patients who presented early. The third patient’s visual outcome despite early treatment with intravitreal antibiotics and vitrectomy was not satisfactory.

Conclusion: Endogenous endophthalmitis by MRSA is a rare but serious condition. Early and specific therapy based on reliable detection of the underlying microorganism is needed for good anatomical and functional outcome.     

Ipilimumab-induced Ocular and Orbital Inflammation—A Case Series and Review of the Literature

Purpose: Ipilimumab, a monoclonal antibody directed against the immune protein cytotoxic T-lymphocyte antigen-4 (CTLA-4), characteristically induces side effects called “immune-related adverse events” (IRAE). Although ophthalmic involvement is rare, we report 7 cases of eye and orbit complications related to ipilimumab therapy.

Methods: We performed a retrospective review of patients with metastatic melanoma who developed ipilimumab-related ocular or orbital inflammation who were seen at our institutions.

Results: Seven patients were identified: 4 patients had orbital inflammation, 2 had uveitis, and 1 had peripheral ulcerative keratitis. Four patients developed inflammation after the second ipilimumab infusion, 2 after the third infusion and 1 after the first infusion. All 4 patients with orbital inflammation were treated with systemic corticosteroids. Two patients with uveitis were treated with topical steroids, but were also treated with systemic corticosteroids for other IRAE, including colitis and hypophysitis. The patient with keratitis was treated with topical corticosteroids alone with resolution of inflammation. All 7 patients discontinued ipilimumab therapy, 5 due to systemic IRAE and 2 due to tumor progression. Five of 7 patients had tumor progression on ipilimumab therapy.

Conclusions: Ocular and orbital inflammation may occur in patients with metastatic melanoma receiving ipilimumab, is frequently accompanied by other IRAEs, and resolves with corticosteroid treatment, often leaving no long-term sequelae.     

Use of Aqueous Humor and Flow Cytometry in Ocular Sarcoidosis Diagnosis

Purpose: To report the use of flow cytometry on aqueous fluid to diagnose sarcoidosis in a patient with recurrent granulomatous anterior uveitis.

Methods: Case report.

Results: Flow cytometry on aqueous fluid demonstrated a CD4/CD8 ratio >9.5, consistent with a diagnosis of sarcoidosis.

Conclusions: Flow cytometry on aqueous fluid may offer an additional pathway for diagnosing sarcoid anterior uveitis.     

Leuconostoc as a Cause of Endophthalmitis Post-intravitreal Injection of Ranibizumab

Purpose: The authors report a case of endophthalmitis after intravitreal injection of ranibizumab.

Methods: After searching for extensive laboratory tests to isolate the etiologic agent

Results: The agent was determined as Leuconostoc mesenteroides, gram-positive cocci, vancomycin resistant.

Discussion: Considerations regarding this bacterium were done by calling attention to its rarity, difficulty of isolation, and action on secondary comorbidities as opportunistic pathogen.     

Varicella Zoster-related Occlusive Retinal Vasculopathy—A Rare Presentation

Purpose: To report a case of occlusive retinal vasculopathy following varicella zoster infection in an immunocompetent adult.

Design: Observational case report.

Methods: A patient with defective vision following chickenpox was evaluated with fluorescein angiography, spectral domain optical coherence tomography and fundus auto fluorescence.

Results: Fundus showed multiple cotton wool spots and a well-demarcated zone of retinal ischemia in the posterior pole with normal optic disc without any evidence of anterior or posterior uveitis. Fluorescein angiography, spectral domain optical coherence tomography and fundus auto fluorescence findings revealed occlusive vasculopathy as the cause of defective vision.

Conclusions: We report a hitherto undescribed case of purely occlusive vasculopathy following varicella zoster infection without features of vasculitis or anterior and posterior uveitis in an immunocompetent individual.     

Hyaluronidase allergy mimicking orbital cellulitis

Purpose: Hyaluronidase enzyme is a common additive with local anesthetic agent to facilitate faster permeation of the anesthetic in periocular tissues during ophthalmic surgery. We report a series of five subjects presenting with clinical features mimicking orbital cellulitis following peribulbar anesthesia and consequently diagnosed with hyaluronidase hypersensitivity.

Setting: The study was conducted at a tertiary eye care center in Southern India.

Design: It was a retrospective interventional case series.

Methods: We retrospectively reviewed the case records of patients diagnosed as and treated for hyaluronidase allergy from 2011 to 2015. The presenting features included periocular edema, proptosis, and restriction of ocular movements. The symptoms appeared immediately after the injection to as late as 6 days after the surgery. All patients underwent comprehensive ophthalmic evaluation, relevant investigations, and dermal allergy tests. All five patients tested positive for hyaluronidase. Patients were treated with antihistaminics, systemic steroids, and emergency orbital decompression, when required. In majority of the patients, symptoms resolved in 3–5 days. Clinically, hyaluronidase allergy may mimic orbital cellulitis, which in the context of a recent intraocular surgery may be alarming for both the patient and the surgeon. However, with prompt intervention, the prognosis is extremely favorable in cases of hyaluronidase allergy. It is important for ophthalmic surgeons and anesthetists to recognize and differentiate this entity from the more serious vision threatening conditions.     

Endogenous Endophthalmitis in the American and Korean Population: An 8-year Retrospective Study

Purpose: To study the clinical features of endogenous endophthalmitis (EE) in sample patient populations from the USA and South Korea over an 8-year period.

Methods: We reviewed data from 128 eyes of 60 American and 48 Korean patients diagnosed with EE and compared their clinical characteristics.

Results: Fungemia and liver abscess were the most common extraocular infection sources among American (26.7%) and Korean patients (33.3%), respectively. Klebsiella pneumoniae and Candida species were the most common pathogens of EE in the Korean and the American patients, respectively. Endophthalmitis caused by fungi had a better visual prognosis than that caused by bacteria (p = 0.001). Vitrectomy was beneficial for eyes with EE due to virulent bacteria presenting with worse than counting finger vision.

Conclusions: The predisposing conditions and responsible organisms for EE vary in different regions of the world. The visual prognosis was strongly influenced by the underlying pathogen.     

Stroke Due to Cerebral Vasculitis in a Patient with Relentless Placoid Chorioretinitis

Purpose: To report the first case of stroke in a patient with relentless placoid chorioretinitis.

Methods: Observational case report.

Results: A 20-year-old female with newly diagnosed relentless placoid chorioretinitis was urgently evaluated for unilateral paresthesias. She was found to have acute bilateral pontine strokes and cerebral vasculitis on magnetic resonance imaging of the brain and cerebral angiography.

Conclusions: We report the first case of stroke due to cerebral vasculitis in a patient with relentless placoid chorioretinitis. This case emphasizes the need for timely evaluation of neurological symptoms in patients with this ocular diagnosis.