Pediatric Orbital and Ocular Disclosures Masses • No financial disclosure relative to this presentation Bernadette L. Koch, M.D. Departments of Radiology and Pediatrics Cincinnati Children’s Hospital Medical Center University Hospital Cincinnati, Ohio @CincyKidsRad facebook.com/CincyKidsRad Objectives Normal Bony Orbit Anatomy • Review imaging characteristics of the • Bony walls most common non-infectious/ non- • SOF – CN III, IV, V1, V2, SOV inflammatory orbital and ocular masses • IOF – IOV in children • Optic canal – ON, OA, sympathetic plexus Soft Tissue Anatomy Orbit Optic Nerve Sheath Complex • Muscle cone • Optic nerve –SR, IR, MR, LR •Fibrous annulus of Zinn to –Disc to chiasm anterior sclera • Sheath –SO, IO, levator palpebrae –Continuous with –SR complex = SR + LP intracranial meninges • Preseptal/postseptal –Orbital septum - Periosteal • Subarachnoid space reflection from bony orbit to tarsal plates –Barrier to spread of disease Normal Anatomy of Globe Ocular masses • Lens • Retinoblastoma Aqueous & vitreous segment • Retinal astrocytoma • Iris • Melanoma Anterior & posterior chamber • Vascular lesion Sturge Weber • Posterior hyaloid membrane • Medulloepithelioma Lines vitreous cavity • Metastasis • Retina – inner sensory, outer • Hamartoma pigmented • Osteoma • Uvea = choroid, ciliary body, iris • Primary intraocular lymphoma • Sclera/Cornea Ocular masses Leukocoria = white pupillary • Retinoblastoma reflex indicating ocular disease • Retinal astrocytoma • Retinoblastoma • Melanoma • Retinal astrocytoma • Vascular lesion Sturge Weber • Choroidal osteoma, mets, melanoma • Medulloepithelioma • Coat’s disease • Metastasis • Long standing retinal detachment • Cataract • Hamartoma • Vitreous hemorrhage • Osteoma • Sclerosing endophthalmitis • Primary intraocular lymphoma • PHPV – persistent hyperplastic primary vitreous • ROP – retinopathy of prematurity Retinoblastoma Retinoblastoma • Most common intraocular malignancy children • Clinical –Arises from neuroectodermal cells –Leukocoria 60% • 1/20,000 live births; M=F –Strabismus 20% • 300-350 new cases in US/year –Uveitis, cellulitis, hyphema, heterochromia, • Average age at diagnosis 18 months, glaucoma, buphthalmos –Undiagnosed late stage proptosis –Majority present prior to 3 years of age • 95% calcified • 60-90% sporadic • CT good delineation of calcification • 10-40% have a family history –SWI nearly as good, US best –Most with germline RB1 mutation (inactivation of • MRI tumor suppressor gene at chromosomal locus –better evaluation for adjacent spread and 13q14) intracranial disease Retinoblastoma Rbl growth patterns • 2/3 unilateral, 1/3 bilateral • Trilateral = bilateral globe + pineal • Endophytic (45%) + vitreous seeding +/- (pineoblastoma) retinal detachment –Or suprasellar • Exophytic (45%) + retinal detachment & • Pathology –Flexner-Wintersteiner rosettes subretinal exudate •ring of cuboidal cells surrounding • Mixed (10%) central lucency •Attempt at retinal differentiation • Diffuse infiltrating form – rare, older children, may not have Ca++ Courtesy Beth Ey, M.D. International Classification for Retinoblastoma Intraocular Retinoblastoma • Group A – < 3 mm in retina and not near optic disc or foveola • Group B – > 3m mm in retina or < 3 mm close to optic disc /foveola • Group C 3 wk old red sclera and eye drainage – Well-defined tumors + small amts. subretinal/vitreous seeding • Group D – Large or poorly defined + widespread subretinal/vitreous seeding +/- retinal detachment • Group E – Very large, extends near anterior globe, hemorrhage, glaucoma or other features of non-salvageable eye Trilateral Rbl Rbl additional findings Globe Prosthesis • Anterior segment CE more aggressive behavior • CE of optic nerve sheath –suspicious for, but not diagnostic of ON extension, especially if only mild CE Expected heterogeneous CE hydroxyapatite prosthesis DDx Globe Calcification Retinal Astrocytoma • Rare retinal tumor • Tuberous sclerosis most common Phthisis bulbi & prosthesis • Enhancing, calcified mass +/- ocular detachments, hemorrhage Drusen TS astryocytic hamartoma BB Ocular Melanoma • Most common primary ocular malignancy in adults • 5% of all melanomas • Uvea >>> conjunctiva – Choroidal 90%, ciliary body 7%, iris 3% • Present with floaters, dec. periph. vision, frequent change in correction 4 year – 3 mo. redness & eye drainage • Dome/mushroom shaped (choroidal) mass Courtesy Dr. Cornelius – Hyperdense CT, hyperintense T1, Ophth exam: proptosis, retinal detachment, hypointense T2, moderate CE lesion with internal pigmentation – Amelanotic hypointense T1 • +/- choroidal or retinal detachment Courtesy Dr. Wiggins Histopathology – choroidal melanoma Choroidal lesion in Sturge-Weber Medulloepithelioma • Congenital vascular lesion • Rare, most common in first –Low flow vascular lesion, not really hemangioma or decade hamartoma • Non-pigmented, vascular • Solitary or diffuse –hyperdense, intensely mass with CE and some enhancing cystic elements • Hyperintense T1/T2 • + pial angiomatosis, large CP • Most common - ciliary body, and sequelae of venous optic head, optic nerve ischemia Extraocular orbital masses ISSVA Classification • Hemangioma • Neoplasm • Vascular malformations –Rhabdomyosarcoma –Lymphatic, venous, mixed –Lymphoma/leukemia venolymphatic –Neuroblastoma mets malformation –Optic nerve glioma • Langerhan cell histiocytosis –Optic nerve sheath • Nasolacrimal Duct Mucocele meningioma • Dermoid/epidermoid cyst • Thyroid ophthalmopathy • Idiopathic orbital inflamm. • Sarcoid International Society for the Study of Vascular Anomalies Mulliken and Glowaki Plast Reconstr Surg 1982;69:412 Infantile Hemangioma Infantile Hemangioma • Benign neoplasm proliferating endothelial cells –Glut-1 positive in all phases • Intense enhancement • Present shortly after birth • +/- flow voids –Proliferative phase with high flow vessels • enlarge up to 2 years • + fatty infiltration –Involutional phase with fatty replacement during involutional • spontaneous regression up to 9 years phase • 60% in H & N –Parotid, orbit, nasal, suglottic, anterior neck, posterior neck • Tx - expectant • Majority single in subQ tissue waiting, propranalol, • Occasionally multiple, trans-spatial, deep steroids, rarely surgical excision PHACE Syndrome PHACE Syndrome • Posterior fossa (DWM) • Hemangiomas H&N • Arterial –Stenosis, occlusion, aneurysm • Cardiovascular 2 different patients –Coarctaion aorta, cardiac anomalies • Eye • Supra-umbilical and sternal clefts Vascular Malformations Lymphatic Malformation • Congenital malformations • Lymphatic malformation • Unilocular or transpatial • Present at birth • Venous malformation • Micro or macrocystic • Sudden increase in size • Grow commensurate with • Arteriovenous malformation –URI, hemorrhage, the child • Capillary malformation puberty • Subcategorized by type • Mixed malformation – most • Spontaneous hemorrhage of endothelial channel • Enhancement – septations common VLM only unless mixed IC Vascular Anomalies + periorbital LM/VLM Idiopathic Orbital Inflammation • 33 pts. neonate – 39 years • Nongranulomatous • Imaging types –Focal mass = tumefactive • 22 (70%) had IC vascular inflammation –EOM enlargement = myositic anomalies –? etiology • involves tendinous insertions –DVA (n=20; 61%) • Painful proptosis –Diffuse orbital inflammation –CCM (n=2; 6%) • Responsive to steroids –Optic neuritis –DAVM (n=2; 12%) –Uvea, sclera, lacrimal gland, • 20% bilateral mixed –Pial AVM (n=1; 3%) • M = F –Orbital apex/cavernous sinus = –Sinus pericranii (n=1; 3%) • IR most common Tolosa-Hunt syndrome Bisdorff et al. AJNR 28:355-341, 2007 • 5-25% IgG4-RD Idiopathic Orbital Inflammation Thyroid Ophthalmopathy • Painless proptosis • 80% bilateral • IR>MR>SR>LR • Euthyroid, hyper OR hypothyroid • Fusiform enlargement EOM’s • Spares tendinous insertion Dermoid/Epidermoid Cysts Dermoid/Epidermoid Cysts • 80% H&N dermoid cysts –orbit, oral, nasal region • Epidermoid (epith lining) –Similar to fluid –+/- diff. restriction • Dermoid (skin appendages) –Fluid, fat +/- Ca++ –+/- diff. restriction Episcleral dermolipoma Langerhan Cell Histiocytosis • Proliferation of histiocytic cells • CE soft tissue mass • Smooth lytic bone defect • +/- CNS involvement –Infundibular enlargement and CE –Enhancing or nonenhancing intraparenchymal or extra-axial lesions • DDx –Rhabdo, mets, NBL Rhabdomyosarcoma Rhabdomyosarcoma • Most common childhood soft tissue sarcoma • 8 – 10 year old • 40% occur in head and neck • M > F –Orbit • May present with rapidly –Parameningeal – 55% IC extension progressive proptosis • middle ear, paranasal sinus and nasal cavity, nasopharynx, infratemporal • Well–defined fossa/pterygopalatine/parapharyngeal • Noncalcified –All other head and neck sites • Variable CE • scalp, parotid gland, oral cavity, larynx, oropharynx, cheek, hypopharynx, • +/- bone destruction thyroid/parathyroid, neck Lymphoma • Intraconal/extraconal • Lacrimal gland common • ON sheath • Well defined or diffuse • +/- osseous erosion • Variable CE • If bilateral consider NHL Leukemia Neuroblastoma • Subperiosteal collections • Most common malignancy < 1 yo • Majority adrenal gland and • ON-sheath complex infiltration retroperitoneum • Intra-or extraconal shaggy masses • Hematogenous spread to orbit • Chloroma/granulocystic sarcoma – “raccoon eyes” • ? related to obstruction of –Focal mass leukemic cells palpebral branches of facial and •AML ophthalmic vessels •+/- bony erosion • Bilateral typical • Subperiosteal soft tissue masses • Spiculated periosteal reaction Optic Pathway