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Clinicopathologic Correlation Case

Clinicopathologic Correlation Case. June 8 th , 2011 Audrey Chan, PGY-3 University of Pittsburgh Medical Center Department of Ophthalmology Pathologist: Dr Csaba Galambos. KJ. 7 week old infant presents to ED with unequal pupils noticed incidentally by mother

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Clinicopathologic Correlation Case

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  1. Clinicopathologic Correlation Case June 8th, 2011 Audrey Chan, PGY-3 University of Pittsburgh Medical Center Department of Ophthalmology Pathologist: Dr Csaba Galambos

  2. KJ • 7 week old infant presents to ED with unequal pupils noticed incidentally by mother • No change in visual behavior noted by mother • Healthy birth history – SVD full term; mother healthy except for prophylactic use of acyclovir for herpes simplex but without active lesions • Normal development; healthy siblings

  3. Exam • Blinks to light • Soft to palpation OU • OD: non-reactive pupil 6mm dilated • OS: 5-2 briskly reactive • No afferent pupillary defect • Orthophoric, full extraocular movements

  4. Exam • Lids/Lashes: Normal OU; no ptosis • Conj/Sclera: White/Quiet OU • Cornea: Clear OU • AC: D/Q OU • Iris: non reactive OD; OS normal • Lens: clear • Vitreous: clear • Disc: c/d 0.2 normal, no pallor or edema OU • Macula: Normal OU • Periphery: Normal OU

  5. MRI/MRA Head and Orbits revealed a right orbital mass that was intraconal with extraconal extension. Mass was distinct from the optic nerve and muscles. There was no bony defect or connection to the brain. There was heterogenous signal intensity of the lesion post-contrast without robust enhancement

  6. KJ • Lesion was presumed to be most likely a capillary hemangioma though its radiographic features were atypical (without robust enhancement), and less likely, a rhabdomyosarcoma • KJ was started on empiric oral propranolol and monitored for 4 days • When there was no improvement over 4 days, the lsion was biopsied to obtain definitive diagnosis

  7. Low magnification of hematoxylin-eosin stained section (HE x4) shows soft tissue fragment with low cellularity and abundant eosinophilic stroma 4x Hematoxylin Eosin stain

  8. Large neuronal cell with perinuclear clearing and rim of deeply basophilic granular cytoplasmic 20x Hematoxylin Eosin Stain

  9. The glial and neuronal elements are highlighted by the S-100 immunostain 20x s-100 antibody immunostain

  10. The positive GFAP stain indicates glial origin of the stromal tissue Glial fibrillary acid protein 20x

  11. Nuclei are positive staining with PGP 9.5 immunostain, a pan-neuronal marker 20x PGP9.5 Stain

  12. Heterotopic neuroglial tissue in the orbit • Defined as non-teratomatous neural tissue isolated from the central nervous system (CNS) without any direct connection to the brain or spinal cord • Very rare in the orbit: Review of the literature shows 15 reported cases since 1980 • CNS heterotopia more common in the midline face, in or around the nasal cavity (aka nasal gliomas) • Usually found in pediatric population, though there are 2 adult cases reported • Present with proptosis, strabismus and lid swelling most commonly

  13. Heterotopic neuroglial tissue in the orbit • Surgically, these lesions can be difficult to excise in toto. There is low incidence of recurrence following complete excision, less than 10% • Visual prognosis after non-traumatic surgical resection is excellent • Most reported cases occur in isolation without other systemic abnormalities

  14. KJ – POW#1 • Continues to blink to light appropriately OU • Pupillary function starting to return OD • Mother has noticed intermittent in-turning of the right eye

  15. References • Elder JE, Chow CW, Holmes AD. Heterotopic brain tissue in the orbit: case report. Br J Ophthalmol 1989; 73: 928-931. • Kiratli H, Sekeroglu MA, Tezel GG. Orbital heterotopic glial tissue presenting as exotropia. Orbit 2008; 27: 165-168. • Bajaj MS, et al. Glial heterotopia of the orbit and extranasal region: an unusual entity. Clin Experiment Ophthalmol 2005; 33: 513-515. • Harmon HL, et al. Orbtial ganglioglioma rising from ectopic neural tissue. Am J Ophthalmol 2000; 129:109-111. • Mihora LD, et al. Ectopic orbital brain diagnosed 20 years after symptomatic presentation. Orbit 2009; 28: 185-187. • Price KM, et al. Ectopic brain in the orbit presenting as disc edema in an adult. Orbit 2009; 28: 74-77. • Ghose S et al. Orbital ectopic glial tissue in relation to medial rectus: a rare entitity. Clin Experiment Ophthalmol 2005; 33: 67-69. • Vemuganti GK, Shekar GC. Ectopic brain presenting as orbital and conjunctival mass: a case report. Orbit 2000; 18: 305-310 • Park KA, Kim HJ, Kim YD. Ectopic brain in the orbit with congenital adduction deficit and simultaneous abduction. Ophthal Plas Reconstr Surg 2007; 23: 244-246. • Wilkins RB, et al. Heterotopic brain tissue in the orbit. Arch Ophthalmol 1987; 105: 390-392. • Altissimi G, et al. Central nervous system tissue heterotopia of the nose: case report and review of the literature. Acta otorhinolaryng italica 2009; 29: 218-221.

  16. Thank you • Dr. Csaba Galambos • Dr. Jenny Will • Dr. Joseph Paviglianti

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