Avey Orbital Inflammatory Disease ASHNR 2016.Pptx

Orbital Inflammatory Disease Disclosure

I have no financial disclosures that would be a potential conflict of interest with this presentation.

I will not be discussing off label uses of medications or unapproved uses of a commercial product or device.

Greg Avey, MD

Goals Organization

Review orbital infections and inflammatory Pertinent orbital anatomy. Preseptal / Periorbital Cellulitis conditions. Idiopathic orbital inflammatory disease. Postseptal / Orbital Cellulitis Discuss the Chandler classification of orbital Subperiosteal Abscess Review remaining infection. Knowledge of this system aids inflammatory diseases on Cavernous Sinus communication with our referring physicians. an anatomic basis. Dacryoadenitis Dacryocystitis Review potential complications of orbital Globe infections – what to watch out for! Optic Nerve Myositis

Organization Osteology

Pertinent orbital anatomy. Preseptal / Periorbital Constructed of 7 separate bones Cellulitis Idiopathic orbital inflammatory disease. Postseptal / Orbital Cellulitis Zygomatic Subperiosteal Abscess Review remaining Maxillary Cavernous Sinus inflammatory diseases on Frontal an anatomic basis. Dacryoadenitis Dacryocystitis Lacrimal Globe Ethmoid Optic Nerve Sphenoid Myositis Palatine Orbital Septum Orbital Septum

Originates at the Originates at the Orbicularis Oculi confluence of the confluence of the Levator Palpebrae periorbita and periorbita and Septum Orbitale periosteum. Superior Rectus periosteum. Inserts at or near the Inserts at or near the tarsal plates, deep to the tarsal plates, deep to the orbicularis oculi muscle. orbicularis oculi muscle. Inferior Rectus Separates the orbital fat Separates the orbital fat from the subcutaneous Inferior Oblique from the subcutaneous Inferior Oblique fat - barrier for infection. fat - barrier for infection. Septum Orbitale

Globe and Optic Nerve Orbital Septum Anterior Chamber Iris Originates at the Orbicularis Oculi Lens confluence of the Vitreous Chamber periorbita and Septum Orbitale periosteum. Dural Sheath CSF Inserts at or near the tarsal plates, deep to the Optic Nerve orbicularis oculi muscle. Separates the orbital fat from the subcutaneous Inferior Oblique fat - barrier for infection. Septum Orbitale

Globe and Optic Nerve Globe and Optic Nerve

Anterior Chamber Iris Lens

Vitreous Chamber

Dural Sheath CSF Optic Nerve Globe and Optic Nerve Venous Anatomy Iris Cornea The venous system in and about the orbit is “valveless”, Sclera allowing bidirectional flow and can be a conduit for spread of infection. Two main venous channels: Ciliary Body Uvea Retina Superior ophthalmic vein

Originates near superior oblique, courses posteriorly and laterally through orbit to enter the cavernous sinus.

Inferior ophthalmic vein

Drains a venous plexus along the floor of the orbit, can Choroid terminate in the pterygoid plexus, superior orbital vein or cavernous sinus. Lamina Cribrosa

Superior Ophthalmic Veins

Superior Ophthalmic Veins Superior Ophthalmic Veins Superior Ophthalmic Veins

Inferior Ophthalmic Veins

Superior Ophthalmic Veins Superior Ophthalmic Veins Superior Ophthalmic Veins Cavernous Sinus

Before we go on to anatomic SOV Cavernous sites... The image cannot be displayed.Sinus Your computer may not have enough memory to open the image, or the image may have been corrupted. Idiopathic Orbital Inflammatory Disease Restart your computer, and then open the ﬁle again. If the red x still appears, (IOID) will be a recurring differential at you may have to delete the image and then insert it again. every anatomic site. What is it?

“Benign, noninfective clinical syndrome Facial Veins characterized by features of nonspecific inflammatory conditions of the orbit without identifiable local or systemic cause.”

Arch Ophthalmol. 2003;121(4):491-499.

Idiopathic Orbital Inflammatory Disease Mimics of IOID

The disease previously known as IgG4 Related Systemic Lupus “pseudotumor”. Disease. Erythematosus 5% of orbital conditions, behind thyroid Thyroid Orbitopathy Scleroderma orbitopathy, and lymphoproliferative disease. Sarcoidosis Giant Cell Arteritis Diagnosis of exclusion, no definitive pathologic Amyloidosis Lymphoma or clinical criteria. Granulomatosis with Metastatic disease My pragmatic definition: No response to abx, Polyangiitis Orbital Cellulitis typically responds to steroids, no identifiable Crohns disease systemic autoimmune condition.

Arch Ophthalmol. 2003;121(4):491-499. IgG4 Related Disease IgG4 Related Disease

Tumor like fibroinflammatory condition which can affect Head and Neck diseases with IgG4 contributions multiple organ systems. Idiopathic Orbital Inflammatory Disease Often associated with elevated serum IgG4 levels, and/ Idiopathic Hypertrophic Pachymeningitis or IgG4 positive lymphocytes on biopsy. Mikulicz Syndrome (Salivary and Lacrimal Glands) First reported in association with autoimmune Riedel’s Thyroiditis pancreatitis, now associated with almost every organ system. Kuttner’s Tumor (Submandibular Glands)

Idiopathic Orbital Inflammatory Disease Idiopathic Orbital Inflammatory Disease

Tolosa Hunt –

Description:

“Episodic orbital pain associated with paralysis of one or more of the third, fourth and/or sixth cranial nerves which usually resolves spontaneously but tends to relapse and remit.”

Arch Ophthalmol. 2003;121(4):491-499. Arch Ophthalmol. 2003;121(4):491-499.

Idiopathic Orbital Inflammatory Disease Idiopathic Orbital Inflammatory Disease

Tolosa Hunt – Tolosa Hunt – Diagnostic criteria: Diagnostic criteria:

One or more episodes of unilateral orbital pain One or more episodes of unilateral orbital pain

Paresis of one or more of cranial nerves III, IV or VI and/or Paresis of one or more of cranial nerves III, IV or VI and/or demonstration of granuloma by MRI or biopsy demonstration of granuloma by MRI or biopsy

Paresis coincides with the onset of pain or follows it within 2 Paresis coincides with the onset of pain or follows it within 2 weeks weeks

Pain and paresis resolve within 72 hours when treated Pain and paresis resolve within 72 hours when treated adequately with corticosteroids adequately with corticosteroids

Other causes have been excluded by appropriate Other causes have been excluded by appropriate investigations investigations

European Journal of Radiology, Volume 45, Issue 2, February 2003, Pages 83–90 European Journal of Radiology, Volume 45, Issue 2, February 2003, Pages 83–90 Normal Cavernous Sinus

29 yo female: L retro-orbital pain Concave, & progressive cranial neuropathies Uniformly smooth * 3, 4, V1, V2, & 6 Enhancing borders.

Case Courtesy of Christopher Wood, MD

Chandler Classification Chandler Classification

Classification of orbital infection based on anatomic site of involvement, development of a defined abscess, and etiology.

Chandler I through Chandler V.

The categories are not sequential.

The Chandler classification has been very effective in helping guide appropriate diagnosis and therapy for orbital infections. I II III IV V Preseptal Postseptal Subperiosteal Orbital Cav. Sinus Cellulitis Cellulitis Abscess Abscess Thrombosis

Inflammation Purulent

Chandler Classification Chandler Classification 32% 19% 48% <1% <1%

I II III IV V I II III IV V Preseptal Postseptal Subperiosteal Orbital Cav. Sinus Preseptal Postseptal Subperiosteal Orbital Cav. Sinus Cellulitis Cellulitis Abscess Abscess Thrombosis Cellulitis Cellulitis Abscess Abscess Thrombosis Inflammation Purulent

Inflammation Purulent Le TD, Liu ES, Adatia FA, Buncic JR, Blaser S. The effect of adding orbital computed tomography findings to the Chandler criteria for classifying pediatric orbital cellulitis in predicting which patients will require surgical intervention. J AAPOS. 2014;18(3):271-7. Anatomic Sites Preseptal / Periorbital Cellulitis

Preseptal / Periorbital Cellulitis Infection of the soft tissues anterior to the orbital Postseptal / Orbital Cellulitis septum. Subperiosteal Abscess Most common in pediatric patients – often Cavernous Sinus subsequent to trauma, acne or insect bite. Dacryoadenitis Dacryocystitis If treated, it is relatively uncommon to spread Globe into the postseptal orbit. Optic Nerve Typically caused by staph, strep, or HiB. Myositis

Preseptal / Periorbital Cellulitis Preseptal Cellulitis

Most are treated as outpatients.

The imaging goals are to exclude postseptal cellulitis, identify drainable abscess, exclude intracranial complications and causative sinus disease.

Anatomic Sites Postseptal Cellulitis

Preseptal / Periorbital Cellulitis Often secondary to sinus infection – spreading Postseptal / Orbital Cellulitis to the orbit via veins and/or through the thin Subperiosteal Abscess bony septae. Cavernous Sinus Clinical hallmarks of proptosis, chemosis, Dacryoadenitis opthalmoplegia and visual acuity loss. Dacryocystitis Infection in a closed space – increased Globe pressure, lack of drainage. Optic Nerve Initial tx is usually medical : abx, +/- steroids, +/- Myositis sinus irrigation, +/- sinus vasoconstrictors. Postseptal Cellulitis Postseptal Cellulitis

Often secondary to sinus infection – spreading Typically inpatient tx. Repeat CT and/or MRI to the orbit via veins and/or through the thin with persistent symptoms or worsening bony septae. exopthalmos. Clinical hallmarks of proptosis, chemosis, Surgical decompression is typically advised with opthalmoplegia and visual acuity loss. persistent or worsening symptoms after 48 Infection in a closed space – increased hours. pressure, lack of drainage. Imaging goals are to document abscess Initial tx is usually medical : abx, +/- steroids, +/- formation, venous thrombosis, cavernous sinus sinus irrigation, +/- sinus vasoconstrictors. thrombosis.

Postseptal Cellulitis Postseptal Cellulitis

15 yo with IDDM 1, poorly controlled 15 yo with IDDM 1, poorly controlled

Postseptal Cellulitis Postseptal Cellulitis

15 yo with IDDM 1, poorly controlled 15 yo with IDDM 1, poorly controlled Postseptal Cellulitis Postseptal Cellulitis

15 yo with IDDM 1, poorly controlled 67 yo F with facial pain and swelling, DM II, Renal Transplant

Postseptal Cellulitis Postseptal Cellulitis

67 yo F with facial pain and swelling, DM II, Renal Transplant 67 yo F with facial pain and swelling, DM II, Renal Transplant

Anatomic Sites Subperiosteal Abscess

Preseptal / Periorbital Cellulitis Typically from extension of ethmoid Postseptal / Orbital Cellulitis sinusitis through the lamina papyracea. Subperiosteal Abscess The abscess characteristics guide medical Cavernous Sinus versus surgical tx. Dacryoadenitis Dacryocystitis Globe Optic Nerve Myositis Subperiosteal Abscess Subperiosteal Abscess

Medical treatment can be attempted if the Surgical decompression is typically via endoscopic abscess sinus surgery, with drainage into the sinonasal cavity. is medial in location. This can be a challenge in young patients. is less than 1 cm in size. Alternative procedure involves a medial canthotomy is not dental or frontal sinus in origin. to access the abscess. presents without optic nerve compromise. Imaging assists with guidance, abscess presents without gas within the abscess. characterization, and exclusion of remote abscess pockets which might warrant an external approach.

Subperiosteal Abscess Subperiosteal Abscess

Subperiosteal Abscess + Gas Subperiosteal + Epidural Abscess

7 year old with headache and left “eye swelling” 14 year old with headache, fever, and left periorbital swelling. Subperiosteal + Epidural Abscess Anatomic Sites

Preseptal / Periorbital Cellulitis Postseptal / Orbital Cellulitis Subperiosteal Abscess Cavernous Sinus Dacryoadenitis Dacryocystitis Globe Optic Nerve Myositis

14 year old with headache, fever, and left periorbital swelling.