Pathology of Regions Adjacent to Paranasal Sinuses (Orbital/ Lacrimal)

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Pathology of Regions Adjacent to Paranasal Sinuses (Orbital/ Lacrimal)

Rhinology
Author Credentials 

Kristin Seiberling, MD
Nadia Chan, MD
Associate professor
Otolaryngology Head and Neck Surgery
Loma Linda University
Loma Linda, CA

Module Summary

There are many pathologies that can affect the orbit and lacrimal system. These include nasolacrimal duct obstruction, Graves orbitopathy, complications from acute sinusitis, orbital apex lesions, and optic neuropathy. It is of utmost importance to understand the anatomy of the bony orbit as well as the fissures, foramens, and canals in which important neurovascular structures traverse. This will guide understanding of surgical technique and the exposure that is required. It is also important to recognize that some conditions can be treated conservatively with aggressive medical therapy first, although surgery is often a required adjunct for complete therapy.

Module Learning Objectives 
  1. Describe the anatomy of the lacrimal system and orbit.
  2. Recognize the different disease pathologies related to the lacrimal system including nasolacrimal obstruction.
  3. Explain the surgical management of thyroid eye disease including medial, inferior and lateral orbital wall decompression.
  4. Describe Chandler classification of orbital and periorbital infections as complications of acute sinusitis.
  5. Recognize the broad differential for orbital apex lesions causing compression.
  6. Cite the surgical approaches for DCR, orbital decompression and optic nerve decompression.
  7. Summarize the risks and benefits to the above mentioned surgical procedures.

Anatomy

Learning Objectives 
  1. To understand the bony composition of the orbital walls, orbital foramen, fissures and position of the extraocular muscles.
  2. To understand different depths of infection into/around the orbit as a complication from acute sinusitis.
  3. To understand the lacrimal anatomy including the secretory and excretory systems.
  4. Describe the position of the lacrimal sac and nasolacrimal duct relative to the middle and inferior turbinates.
  5. Recognize the intranasal landmarks important to successful endoscopic DCR.
References 
  1. Wormald PJ, Kew J, Van Hasselt A. Intranasal anatomy of the nasolacrimal sac in endoscopic dacyrocstorhinostomy. Otolaryngol Head Neck Surg. 2000 Sep;123(3):307-10.

Pathogenesis

Learning Objectives 
  1. Be familiar with the causes of acquired tearing from either hypersecretion of tears or impairment of drainage.
  2. Be familiar with the causes of nasolacrimal obstruction which include midfacial trauma, sinus disease, prior surgery, systemic inflammatory disease, and previous episodes of inflammation of the lacrimal sac.
  3. Describe the ophthalmopathy associated with thyroid dysfunction and Graves disease.
  4. Understand that acute sinusitis can have orbital complications.
  5. Recognize broad differential of orbital apex lesions.
    1. Infectious (fungal, bacterial, viral)
    2. Inflammatory (orbital pseudotumor, sarcoidosis, optic neuritis, thyroid orbitopathy, giant cell arteritis, Churg-Strauss syndrome, Wegeners granulomatosis, systemic lupus erythematosus)
    3. Neoplasm (meningioma, schwannoma, optic glioma, lymphoma, rhabdomyosarcoma, hemangiopericytoma, metastatic disease)
    4. Extrinsic compression (fibrous dysplasia, mucocele, Paget’s disease, subperiosteal hematoma, primary or metastatic osseous tumor)
    5. Vascular (venous malformation, lymphovascular malformation, ophthalmic artery aneurysm, carotid cavernous fistula/AVM, cavernous sinus thrombosis, sickle cell anemia, orbital varix)
    6. Trauma-related/iatrogenic (penetrating injury, orbital apex/optic canal fracture, foreign body, sinonasal surgery, orbitofacial surgery)
  6. Describe the differences between direct and indirect optic neuropathy.
References 
  1. Bartley G. Acquired lacrimal drainage obstruction: An etiologic classification system, case review and review of literature. Ophthalmic Plast Reconstr Surg. 1992;8(4):237-42.
  2. Kazim M, Goldberg R, Smith T. Insights into the pathogenesis of thyroid associated orbitopathopathy. Arch Ophthalmol. 2002 Mar;120(3):380-6.
  3. Vohra S, Escott E, Stevens D, Branstetter B. Categorization and characterization of lesions of the orbital apex. Neuroradiology. 2011; 58:89-107.
  4. Sarkies N. Traumatic optic neuropathy. Eye 2004;18,1122-1125.

Basic Science

Learning Objectives 

Understand that thyroid eye disease is considered an autoimmune process resulting from thyroid-stimulating hormone.

References 
  1. Tsui S, Naik V, Hoa N, Hwang C. Evidence for an association between Thyroid stimulating hormone and Insulin-like growth factor I receptors: A talke of two antigens implicated in Graves disease. J Immunol. 2008;181(6) 4397-4405.

Incidence

Learning Objectives 

Know the incidence of thyroid eye disease.

References 
  1. Bartley GB, Fatourechi V, Kadrmas EF. Chronology of Graves’ ophthalmopathy in an incidence cohort. Am K Ophthalmol. 1996;121(4):426-434.

Genetics

Learning Objectives 

Be familiar with the possible role of autoimmune disease and thyroid eye disease.

References 
  1. S, Naik V, Hoa N, Hwang C. Evidence for an association between thyroid stimulating hormone and Insulin-like growth factor I receptors: A talke of two antigens implicated in Graves disease. J Immunol. 2008;181(6) 4397-4405.

Patient Evaluation

Learning Objectives 
  1. Be able to identify physical exam findings of preseptal cellulitis, orbital cellulitis, subperiosteal abscess, orbital abscess, and cavernous sinus thrombosis.
  2. Be able to describe the various diagnostic tests available to confirm nasolacrimal duct obstruction.
  3. Be familiar with lacrimal system irrigation/probing and the prescence of either a partial or complete obstruction
References 
  1. Javed M, Kamal S, Gupta A. Simple vs complex congenital nasolacrimal duct ostructions: etiology, management and outcomes. Int Forum Allergy Rhinol. 2015;5(2):174-177.

Imaging

Learning Objectives 
  1. Discuss the usefulness and differences of lacrimal scintigraphy and contrast dacryocystography in the work up of nasolacrimal duct obstruction.
  2. Understand the role of MRI and CT scan in the work up and treatment of Thyroid eye disease, orbital apex lesions, and optic nerve compression.
  3. To be able to identify the location of critical structures on CT scan including the optic canal and carotid artery, as well as infectious etiologies on contrast-enhanced CT scan.
References 
  1. Wearne M, Pitts J. Comparison of dacyrocytography and lacrimal scintography in the diagnosis of functional nasolacrimal duct obstruction. Br J Ophthalmol. 1999 Sep; 83(9):1032-1035.

Treatment

Learning Objectives 

To understand that the degree of recession of the exophthalmos achieved by orbital decompression is directly related to the number of walls surgically decompressed.

References 
  1. Kikkawa DO, Pornpanich K, Cruz RC. Graded orbital decompression based on severity of proptosis. Ophthalmology. 2002;109(7):1219-1224.

Medical Therapies

Learning Objectives 
  1. Recognize that the majority of patients with thyroid eye disease require only conservative therapy and correction of the thyroid dysfunction may aid in improving the orbitopathy.
  2. Be familiar with the role of oral steroids and orbital radiation in the treatment of orbital inflammation and compressive optic neuropathy associated with thyroid eye disease.
  3. Discuss the role of oral steroids and treatment outcomes in traumatic optic neuropathy.
  4. Know when to administer oral steroids vs. offer surgical therapy for periorbital or orbital infections.
References 
  1. Gorman CA, Garrity JA. A prospective, randomized, double-blind, placebo controlled study of orbital radiotherapy for Graves’ ophthalmopathy. Ophthalmology. 2001;108(9):1523-1534.
  2. Kazim M. Perspective-Part II:radiotherapy for Graves orbitopathy: the Columbia University experience. Ophthal Plast Reconstru Surg. 2002;18(3):173-174.
  3. Sosin M, De La Cruz C, Mundinger G. Treatment outcomes following traumatic optic neuropathy. Plastic and Reconstructive Surgery. 2016;137(1):231-238.
  4. Radovani P, Vasili D, Xhelili M, Dervishi J. Orbital Complications of Sinusitis. Balkan Med J. 2013;30(2):151-154.

Pharmacology

Learning Objectives 

Be familiar with non-surgical treatment options in thyroid eye disease which includes oral steroids, radiation and immunomodulators.

References 
  1. Ginter A, Migliori ME. The role of biological agents and immunomodulators in treatment strategies for thyroid eye disease: An evidence-based review. RI Med J (2013). 2016 Jun 1;99(6):26-9.
  2. Balaji P, Dale M. Treatment of severe thyroid eye disease: A survey of American society of ophthalmic plastic and reconstructive surgery (ASOPRS). Ophthalmic Plast Reconstr Surg. 2015 Mar-Apr;31(2):127-31.

Surgical Therapies

Learning Objectives 
  1. Discuss the success rates between endoscopic DCR to the traditional techniques.
  2. Be able to describe the steps involved in endoscopic DCR.
  3. To be able to discuss the various surgical approaches to decompress the orbit by removing bone and fat, including: transcranial, coronal flap, transconjunctival/transcaruncular, transantral and endonasal.
  4. To be able to understand the sinus exposure needed for endoscopic medial orbital wall decompression.
  5. Identify and preserve the inferomedial orbital strut when performing an endoscopic medial orbital wall decompression.
  6. Recognize that surgical decompression of the orbit is often performed with the ophthalmology team when involving the inferior and lateral walls.
  7. To describe the endonasal access needed for optic nerve decompression.
  8. To discuss when incision of the optic nerve sheath is necessary during optic nerve injury.
References 
  1. Tsirbas A, Davis G, Wormald PJ. Mechanical endonasal dacyrocystorhinostomy versus external dacyrocystorhinostomy. Ophthalmic Plast Reconstr Surg. 2004;20(1):50-56.
  2. Ben Simon GJ, Joseph J, Lee S. Schwarcz RM. External versus endoscopic dacryocystorhinostomy for acquired nasolacrimal duct obstruction in a tertiary referral center. Ophthalmology. 2005;112(8):1463-1468.
  3. Xu R, Chen F, Zuo K Ye X, Yang Q, Shi J, Chen H, Li H. Endoscopic optic nerve decompression for patients with traumatic optic neuropathy: Is nerve sheath incision necessary. ORL J Otorhinolaryngol Relat Spec. 2014;76(1):44-9.
  4. http://www.american-rhinologic.org/videos (Surgical dissection videos on the ARS website, for members. ARS membership is FREE for residents.)

Case Studies

  1. 8-year-old healthy female presents to the ER with 4 day history of sinus pressure between her eyes. She was seen by her pediatrician and prescribed amoxicillin for suspected otitis media and acute sinusitis. After 2 days on amoxicillin, patient develops left eye pain and swelling around the left eye. She develops a fever to 103oF. On examination, bilateral tympanic membranes are normal with no evidence of otitis media or externa. Her right eye appears normal with 20/20 vision. Her left eye is proptotic, exhibits purulent discharge, and has 20/50 visual acuity. In addition, the left eye exhibits restricted mobility in lateral directions and upward gaze. What further procedures are necessary, if any? What imaging modality would you choose? How would you treat this patient?
    1. CT paranasal sinus with IV contrast is imaging modality of choice to evaluate for infectious cause. In this case, patient has a subperiosteal abscess.
    2. Treatment options include external drainage vs. endoscopic drainage via medial orbital wall decompression.
  2. 53-year-old woman is referred from the ophthalmologist for Graves’ disease with right exophthalmos. She has increasing proptosis of the right eye, with resulting diplopia, blurry vision, and tearing. Besides these orbital symptoms, her Graves’ disease is well-controlled. What would you offer this patient? If surgery is to be performed, what approach would you use?
    1. Combination approach with ophthalmologist for orbital decompression.
    2. Can start with endoscopic medial orbital wall decompression and release of periorbita, +/- endoscopic inferior orbital wall decompression and external lateral orbital wall decompression if necessary.
  3. 60-year-old woman presents with 6-month history of excessive watery discharge from her right eye, with the discharge occasionally becoming thick mucus. She reports occasional tenderness and swelling at the medial canthus area. No changes in vision. Ophthalmologist performed canaliculus probing and irrigation, and found that there is stenosis of the nasolacrimal duct. What is the etiology of nasolacrimal duct stenosis? What is the next step in treatment?
    1. Idiopathic, infectious, inflammatory, neoplastic, traumatic, and mechanical causes for obstruction.
    2. External or endonasal dacryocystorhinostomy is indicated.

Complications

Learning Objectives 

Untreated orbital infections can cause bacteremia, meningitis, blindness.

  1. Complications from Graves’ ophthalmopathy (GO)
    1. Mild GO: Eyelid retraction, proptosis, chemosis, periorbital edema, altered ocular motility
    2. Severe GO: Exposure keratopathy, diplopia, compressive optic neuropathy and possible eventual visual loss
  2. Periorbital and orbital complications from acute sinusitis, classified by the Chandler system:
    1. Preseptal cellulitis (eyelid edema, erythema, and tenderness)
    2. Orbital cellulitis (1 + chemosis, proptosis, extraocular movement restriction, decreased visual acuity)
    3. Subperiosteal abscess (same as 2 but more severe symptoms)
    4. Orbital abscess (3 + papilledema)
    5. Cavernous sinus thrombosis (4+ headache, high fever, dilated or sluggish pupils, hypesthesia around face/eyes, seizures)
  3. Specific surgical complications:
    1. DCR: scarring within the nose, leading to blockage of surgical opening and resultant recurrence of epiphora; wound infection; orbital hematoma; injury to extraocular muscle.
    2. Endoscopic orbital decompression: loss of vision; diplopia; inadequate decompression of the eye; orbital hematoma; V2 paresthesia, vertical dystopia; bleeding; CSF leak; meningitis; epiphora; sinusitis.
    3. Endoscopic optic nerve decompression: same as endoscopic orbital decompression, with additional risk of carotid artery injury.
References 
  1. Steibel-Kalish H, Robenshtok E, Hasanreisoglu M et al. Treatment Modalities for Graves’ Ophthalmopathy: Systemic Review and Metaanalysis. J Clin Endocrinol Metab. 2009 Aug;94(8):2708-16.
  2. Radovani P, Vasili D, Xhelili M, Dervishi J. Orbital Complications of Sinusitis. Balkan Med J. 2013;30(2):151-154.
  3. Ayoob M, Mahida K, Ul-ain Q, Dawood Z. Outcome and Complications of Endoscopic Dacryocystorhinostomy without Stenting. Pak J Med Sci. 2013;29(5):1236-1239.
  4. Kasperbauer J, Hinkley L. Endoscopic Orbital Decompression for Graves’ Ophthalmopathy. Am J of Rhinology. 2005;19(6):603-606.

Review

Review Questions 
  1. Describe the tests that can be performed to evaluate nasolacrimal duct obstruction.
  2. What treatment (medical and su gical) can be offered to patients with Graves hyperthyroidism with resulting orbitopathy?
  3. What is the Chandler classification of orbital infection and what are the symptoms of each?
  4. Name the 6 main categories in the differential or orbital apex lesions.
  5. Describe the endoscopic approach to medical orbital wall decompression as well as optic nerve decompression and the anatomic exposure that is required for both.
Resources 

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