Orbital inflammation in older children is most commonly associated with infection, predominantly of the sinuses. However, additional differential diagnoses should be considered, especially if the child does not clinically improve with a course of antibiotics. We present a case of orbital inflammation associated with intraocular inflammation in a previously healthy adolescent with no specific etiology, despite an extensive evaluation. Pertinent clinical findings, laboratory tests, and clinical course are presented in this unusual case of idiopathic orbital inflammation with panuveitis in both eyes.
A 14-year-old boy presented with a 2-month history of waxing and waning bilateral eye redness, pain, eyelid swelling, and photophobia worsening in the 4 days prior to presentation. The patient reported increased fatigue, nausea, anorexia, and a 9-pound weight loss in the past 2 months. The patient was treated initially by his pediatrician and an outside ophthalmologist with antibiotic ointment for a presumed conjunctivitis followed by oral antibiotics for presumed sinusitis as the cause of his periorbital inflammation and systemic symptoms.
On initial examination, his visual acuity was 20/20−1 in the right eye and 20/20−1 in the left eye. There was no proptosis, but ballottement of the left globe demonstrated mild resistance to retropulsion. Intraocular pressures were 14 mm Hg in the right eye and 20 mm Hg in the left eye. Examination of the right eye revealed no eyelid edema or erythema, trace conjunctival injection, and 1+ cell in the anterior chamber. The left eye had mild eyelid edema and erythema, 1+ diffuse conjunctival injection, fine keratic precipitates, and 2+ cell in the anterior chamber. Extraocular motility demonstrated −1 abduction and adduction deficits in the right eye and −2 abduction and adduction and −1 supraduction and infraduction deficits in the left eye (Figure 1).
External photographs of the patient on the day of presentation demonstrating −1 abduction and adduction deficits of the right eye and −2 abduction and adduction and −1 supraduction and infraduction deficits of the left eye.
A dilated fundus examination revealed 1+ vitreous cell in the right eye, 3+ vitreous cell in the left eye, bilateral disc edema, macular folds, no sheathing of the vessels, and no retinal lesions (Figure 2).
Fundus photographs showing optic disc edema and macular striae bilaterally. OD = right eye; OS = left eye
A fluorescein angiogram highlighted papillitis with leakage surrounding the optic nerve in both eyes, but no evidence of vasculitis. Magnetic resonance imaging with contrast showed infiltrative enhancing soft tissue density in the orbital fat bilaterally, scleral enhancement, and optic disc edema and enhancement, all greater in the left eye than in the right eye (Figure 3).
(A) Initial magnetic resonance imaging (MRI) (T1-weighted post-contrast, fat saturated view) showing bilaterally enhancing soft tissue density in the intraconal fat and prominence of the optic discs and enhancement of the sclera bilaterally. (B) Repeat MRI shows improved orbital inflammation and near complete resolution of optic disc and scleral enhancement.
Laboratory work-up demonstrated an unremarkable complete blood cell count, including normal white cell count of 8,500 cells, antinuclear antibody, anti-neutrophil cytoplasmic antibody, thyroid stimulating hormone and free thyroxine, angiotensin-converting enzyme (41 u/L), lysozyme (9.6 µg/mL), QuantiFERON gold, fluorescent treponemal antibody absorption (FTA-Abs) test, blood cultures, erythrocyte sedimentation rate (21 mm), and C-reactive protein (18.5 mg/dL). Lyme titers drawn by his pediatrician were reportedly negative according to the patient's parents.
The patient was diagnosed as having bilateral idiopathic orbital inflammation with associated panuveitis and given 40 mg prednisone daily (1 mg/kg/day dose for 39.9 kg child) with significant improvement in his examination over the ensuing 48 hours. His eyelid edema, optic nerve edema, and macular striae substantially improved. The cortico-steroid was gradually tapered by 10 mg every 2 to 3 weeks and his visual acuity returned to baseline at 20/15 in both eyes. Repeat magnetic resonance imaging 2 months after treatment initiation showed resolution of enhancement in the retrobulbar fat, scleritis, and near complete resolution of the optic disc enhancement (Figure 3).
This study describes a case of bilateral idiopathic orbital inflammation in a pediatric patient presenting with orbital inflammatory symptoms, bilateral panuveitis, and macular edema. This clinical entity is rare and many pediatricians and ophthalmologists are not aware of this unique presentation. Our case is notable for the bilaterality of the panuveitis on presentation and prolonged period of systemic and ocular symptoms, which resulted in dramatic improvement when recognized and treated as idiopathic orbital inflammation. The clinician should be mindful of this disease when evaluating patients with panuveitis accompanied by orbital signs because prompt diagnosis may allow for more appropriate directed treatment with rapid resolution of symptoms.
Idiopathic orbital inflammation is a nonspecific inflammatory process. Pediatric cases are relatively uncommon and account for only 6% to 16% of when recognized and treated as idiopathic orbital inflammation cases.1,2 In children it is particularly important to rule out other disease processes such as orbital cellulitis, leukemia, histiocytosis, rhabdomyosarcoma, retinoblastoma, neuroblastoma, and lymphangioma prior to the initiation of treatment.3
Pediatric cases of idiopathic orbital inflammation may present with features not characteristically seen in adult cases. Pediatric idiopathic orbital inflammation is more likely to be bilateral on presentation and has a high rate of recurrence and morbidity.2,4–6 In a case series of 29 patients, Mottow-Lippa et al. reported that 45% of patients had recurrent disease.2 Additionally, pediatric cases of idiopathic orbital inflammation are often associated with systemic symptoms, which can be difficult to interpret, and laboratory work-up may reveal peripheral eosinophilia. The systemic symptoms, reported in up to 50% of pediatric patients, include upper respiratory tract infection symptoms, headache, vomiting, anorexia, abdominal pain, fever, and weight loss.4,7
As demonstrated in this case, uveitis may rarely be a coexisting condition. Idiopathic orbital inflammation is seldom considered in the standard differential of pediatric uveitis; however, uveitis is seen in up to 25% of pediatric patients with idiopathic orbital inflammation and portends a poor prognosis, often with recurrent disease.1 In Bloom et al.'s published study of 2 patients, 1 had bilateral uveitis and optic nerve edema and the other had unilateral iritis at presentation; both had recurrences requiring repeat initiation of steroids.5 Hertle et al. similarly comment on uveitis being seen early in the clinical course in pediatric patients in their case reports of 2 patients with iritis at presentation.6
Systemic steroid therapy is the established first-line treatment for idiopathic orbital inflammation, starting at a dose of 1.0 to 1.5 mg/kg/day in adults or children and slowly tapered over weeks to months.8–10 In most cases, there is significant improvement within 24 to 48 hours with the initiation of steroids. In the pediatric population it is also important to keep in mind the side effects of long-term steroid use, such as growth suppression, glucose intolerance, and pituitary–adrenal axis suppression, particularly when treating recalcitrant or recurrent disease. Alternative treatments include low-dose irradiation and immunosuppressive agents. Radiation is usually reserved for patients in whom steroids are contraindicated or who are unresponsive to steroids.11 Data on the use of immunosuppressive agents are limited; however, there are reports of use of low dose cyclosporine, methotrexate, and anti-tumor necrosis factor alpha agents such as rituximab in cases of steroid dependence.10,12,13 Biopsy is recommended in the case of recurrent or refractive disease, chronic disease, discrete mass on imaging, or for patients with a history of carcinoma.10
- Blodi FC, Gas JD. Inflammatory pseudotumour of the orbit. Br J Ophthalmol. 1968;52:79–93. doi:10.1136/bjo.52.2.79 [CrossRef]
- Mottow-Lippa L, Jakobiec FA, Smith M. Idiopathic inflammatory orbital pseudotumor in childhood: II. Results of diagnostic tests and biopsies. Ophthalmology. 1981;88:565–574. doi:10.1016/S0161-6420(81)34984-7 [CrossRef]
- Stevens JL, Rychwalski PJ, Baker RS, Kielar RS. Pseudotumor of the orbit in early childhood. J AAPOS. 1998;2:120–123. doi:10.1016/S1091-8531(98)90076-3 [CrossRef]
- Mottow LS, Jakobiec FA. Idiopathic inflammatory orbital pseudotumor in childhood: I. Clinical characteristics. Arch Ophthalmol. 1978;96:1410–1417. doi:10.1001/archopht.1978.03910060164013 [CrossRef]
- Bloom JN, Graviss ER, Byrne BJ. Orbital pseudotumor in the differential diagnosis of pediatric uveitis. J Pediatr Ophthalmol Strabismus. 1992;29:59–63.
- Hertle RW, Granet DB, Goyal AK, Schaffer DB. Orbital pseudotumor in the differential diagnosis of pediatric uveitis. J Pediatr Ophthalmol Strabismus. 1993;30:61.
- Belanger C, Zhang KS, Reddy AK, Yen MT, Yen KG. Inflammatory disorders of the orbit in childhood: a case series. Am J Ophthalmol. 2010;150:460–463. doi:10.1016/j.ajo.2010.05.012 [CrossRef]
- Snebold NG. Orbital pseudotumor syndromes. Curr Opin Ophthalmol. 1997;8:41–44. doi:10.1097/00055735-199712000-00007 [CrossRef]
- Yuen SJA, Rubin PAD. Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol. 2003;121:491–499. doi:10.1001/archopht.121.4.491 [CrossRef]
- Jacobs D, Galetta S. Diagnosis and management of orbital pseudotumor. Curr Opin Ophthalmol. 2002;13:347–351. doi:10.1097/00055735-200212000-00001 [CrossRef]
- Marwaha G, Macklis R, Singh AD. Radiation therapy: orbital tumors. Dev Ophthalmol. 2013;52:94–101. doi:10.1159/000351084 [CrossRef]
- Suhler EB, Lim LL, Beardsley RM, et al. Rituximab therapy for refractory orbital inflammation: results of a phase 1/2, dose-ranging, randomized clinical trial. JAMA Ophthalmol. 2014;132:572–578. doi:10.1001/jamaophthalmol.2013.8179 [CrossRef]
- Zacharopoulos IP, Papadaki T, Manor RS, Briscoe D. Treatment of idiopathic orbital inflammatory disease with cyclosporine-A: a case presentation. Semin Ophthalmol. 2009;24:260–261. doi:10.3109/08820530903392639 [CrossRef]