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Orbital Mass in a Child

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Orbital Mass in a Child Orbital Mass in a Child Edward G. Buckley, MD Banks Anderson Professor of Ophthalmology Duke University Orbital Tumors/Lesions in Children • Malformations • Choristomas - Hemangioma, Lymphangioma, Varix, Neurofibroma • Primary Neoplasms - Glioma, Rhabdomyosarcoma, fibrous dysplasia • Secondary tumors – Astrocytoma, medulloepithelioma • Metastatic tumors – Neuroblastoma, Wilm’s , Ewing’s sarcoma • Leukemias / Lymphoma – Burkitt’s • Histiocytoses/Xanthogranuloma- esosinophilic granuloma • Inflammations- Pseudotumor, myositis • Infections – orbital cellulitis How to evaluate a child with orbital mass? Neuro-imaging ! Which neuro-imaging test is best ? CT scans are superior in most cases MRI may be desirable in certain cases when optic nerve dysfunction is present How to evaluate an orbital mass in a child? Ways to: How to determine: Classify ? Seriousness ? Categorize ? Urgency ? Compartmentalize ? Morbidity ? Is it Rapidly Expanding ?….. • Cellulitis/abscess • Pseudotumor/myositis • Hemangioma • Rhabdomyosarcoma • Neuroblastoma • Lymphoma • Eosinophilic granuloma Bilateral ?….. • Optic nerve glioma • Neuroblastoma • Leukemia • Lymphoma • Pseudotumor/myositis • Eosinophilic granuloma Eyelid echymosis ?…. • Neuroblastoma • Ewing’s sarcoma • Leukemia • Eosinophilic granuloma • Lymphanigoma Present at birth ?...... Microphthalmos with cyst Varix Teratoma Optic nerve glioma Capillary hemangioma Retinoblastoma Lymphangioma Neuroblastoma Dermoid cyst Neurofibroma Meningoencephalocele Juvrnile xanthogranuloma Intermittent ?.... Lympangioma Dermoid cyst Varix Inflammatory Pseudotumor Mucocele An illusion ?... 1. Unilateral high axial myopia 2. Actual enophthalmos of other eye 3. Upper lid retraction By imaging appearance ?..... Tumors that are well circumscribed on neuroimaging • Cavernous hemangioma • Schwannoma • Fibrohistiocytoma • Neurofibroma • Hemangiopericytoma • Dermoid cyst Simple approach to Orbital Mass in Children Age - young vs older Speed of growth - fast: days to week(s) slow: months to year(s) Simple approach to Orbital Masses in Children AGE Young Older Infection Infection Fast Leukemia Myositis Neuroblastoma Rhabdomyosarcoma Lymphoma Growth Dermoid cyst Ewing’s sarcoma Glioma Fibrous dysplasia Slow Hemangioma Mucocele Lymphangioma Eosinophilic granuloma Fast/young….. 15 month old 2 week history of proptosis No other complaints Normal birth No childhood illnesses Normal development Fast/young Vision – CSM, localizes 1mm bead OU easily Pupils – no afferent defect External - 6mm proptosis OS EOM – slight decrease OS Fundus – Venous engorgement OS Normal nerve Fast/young WBC – 150,000 Diagnosis: Chloroma Fast/young Post Chemotherapy Slow/Young . 6 month old . ? Proptosis OD . Mid optic atrophy Slow/Young Slow/Young Child < 6 y.o. with gradual, painless, progressive, unilateral axial proptosis with visual loss Optic nerve glioma (juvenile pilocytic astrocytoma) slow-growing tumor Decreased visual acuity with a RAPD CT scan or MRI - “fusiform” enlargement of the ON associated with NF1 Dx if bilateral Systemic evaluation and genetic counselling for NF is essential NF 1 - Optic Nerve Glioma Fast/young….. Fast/young….. Fast/young….. Unilateral proptosis, pain, fever, decreased ocular motility, erythema, and edema of the eyelids Infectious orbital cellulitis usually bacterial extended posterior to orbital septum meningitis cavernous sinus thrombosis staphylococci. streptococci. anaerobes, and Haemophilus influenza (in children under 5 years of age) most common source -- ethmoid sinusitis intravenous antibiotics Fast/older….. • 15 year old female • 2 week history of ? Diplopia • Mild ? eye pain on movement • No other symptoms Doesn’t want to go to school --- Exam week ! Fast/older…. Motility Ow! It hurts. Doesn’t want to move her eyes ! Fast/older…. Motility Recommendation: Observe Fast/older I told you I had an eye problem !! Orbital Myositis Lessons Learned Pain • Uncommon in benign strabismus • Pain on movement = myositis/inflammatory Incomitance Fast/older….. • 8 year old • Notes “funny” right eye x 2 weeks • Head position to left • Otherwise fine ! Motility Case Report Diagnosis ? a) Right Duanes syndrome b) Right internuclear ophthalmoplegia c) Myasthenia gravis d) Right orbital mass Radiology: “probable hemangioma / lymphangioma” Case Report Management ? a) Excisional Biopsy b) Oral Steroids c) Observation d) External beam radiation Case Report 2 weeks later……. Rhabdomyosarcoma Child with rapidly progressive unilateral proptosis, displacement of the globe inferiorly, and edema of upper eyelid? Rhabdomyosarcoma most common primary orbital malignancy of childhood malignant growth of striated muscle tissue rapidly progressive mass in the superior orbit with proptosis, globe displacement, and eyelid swelling average age of presentation is 7 years Prompt diagnosis with orbitotomy and biopsy is crucial overall mortality is 60% once the disease has extended to orbital bones Current Rx with radiation + chemo have lowered mortality rates to 5 to 10% Fast/older….. Fast/older….. Persistent proptosis or progression of infection despite adequate antibiotic Rx Orbital subperiosteal abscess • CT scan confirm diagnosis locate the abscess • surgical drainage and continued intravenous antibiotics Fast/older….. Slow/young….. Slow/young….. Capillary Hemangioma Systemic corticosteroids 3 weeks, 6/15/2008 1/2009 7/13/2008, steroids initiated Mechanism of Action? • Early (1-3 days): vasoconstriction due to decreased release of nitric oxide – Inhibit vasodilation by adrenaline via beta receptors vasoconstriction reduction of blood flow to the hemangioma • Intermediate: blocking of pro-angiogenic signals (VEGF, bFGF, MMP2/9) growth arrest – MMP 2/9 regulated via beta receptors • Late: induction of apoptosis in proliferating endothelial cells tumor regression – Disengage inhibition of apoptosis caused by beta agonists Storch CH, Hoeger PH. Propranolol for infantile haemangiomas: insights into the molecular mechanisms of action. Br J Dermatol. 2010 Aug;163(2):269-74. Epub 2010 May 8. Safety • > 40 years of clinical experience in infants and young children • No documented case of death or serious cardiovascular morbidity <6 yrs • BUT… among beta blockers– high risk of side effects – Bradycardia – Hypotension – Bronchospasm (ask about asthma or episodes of wheezing) – Hypoglycemia (stop if decreased food intake for ANY reason) • Check other medications (salicylates, sulfonylureas, quinine) • Preoperative & nighttime fasting • Give during day, followed by feeding – Hyperkalemia – Sweats – Cold & mottled extremities – Diarrhea 50 days of life 60 days after initiation 4 months of life After propranolol Orbital Hemangioma 3 weeks later 2 months later 10 months after initiation Timolol 4 months old Guo S, Ni N. Topical treatment for capillary hemangioma of the eyelid using beta-blocker solution. Arch Ophthalmol. 2010 Feb;128(2):255-6. 5 weeks after initiation Summary…… Simple approach to Orbital Mass in Children AGE Young Older Infection Infection Fast Leukemia Myositis Neuroblastoma Rhabdomyosarcoma Lymphoma Growth Dermoid cyst Ewing’s sarcoma Glioma Fibrous dysplasia Slow Hemangioma Mucocele Lymphangioma Eosinophilic granuloma .
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