surgery 4

capstrone project report

assesment
opthalmology (cogan syndrome)
By Ankit Gurjar
cogan syndrome
• Cogan syndrome is a rare autoimmune disease that affects the eyes and inner
ears. Symptoms of the syndrome include irritation and pain in the eyes,
decreased vision, hearing loss, and vertigo. Other symptoms may include joint or
muscle pain or inflammation of the blood vessels
• Cogan syndrome primarily affects the eyes and the inner ears.
• Cogan’s syndrome is seen in middle aged adults.
• The features include interstitial keratitis, acute tinnitus, vertigo and deafness.
• Cogan’s syndrome primarily affects the large vessels—especially the aorta, which
carries oxygenated blood from the heart to the rest of the body.
causes
• Cogan syndrome is an autoimmune disease. Specifically, the immune systems of people
with Cogan syndrome begin to attack the tissues of the eyes and the inner ears. In some
cases, the autoimmune response may also be directed against blood vessels.The exact
reason that people with Cogan syndrome have an autoimmune response against the
tissues of the eyes and the inner ears is not well-understood. For some people, it may be
that symptoms of Cogan syndrome begin after an infection.
• For some people, Cogan’s syndrome develops after an infection. It is not known to run in
families.
• chronic interstitial keratitis and vestibuloauditory dysfunction remain the hallmarks of
Cogan’s syndrome. The etiology is unknown, but is believed to involve autoimmunity to
the inner ear and cornea. HLA-B17, HLA-A9, HLA-Bw35, and HLA-Cw4 have been
correlated with an increased incidence of Cogan’s syndrome.
sighn and symptoms
• The first symptoms typically either affect only the inner ears or only the eyes but
often progress to affect both the eyes and the ears.
• interstitial keratitis- Eye redness and pain, decreased or blurred vision, sensitivity
to light, and excess tear production.
• ears may include sensorineural hearing loss, ringing in the ears (tinnitus) and
dizziness (vertigo).
• vasculitis- fatigue, headaches, joint pain, rashes, arm pain, weight loss
Heart murmurs or other heart problems may develop.
• shortness of breath
• enlarged lymph nodes
epidemiology
• The actual number of patients suffering from Cogan’s syndrome could well be
substantially higher because many cases are incompletely or wrongly diagnosed
as idiopathic hearing loss/deafness, autoimmune inner ear disease, or idiopathic
recurring keratitis. Since these disorders are frequently treated with
corticosteroids, the typical or complete clinical picture of Cogan’s syndrome may
often be blurred.
• Cogan's syndrome is a rare disease, which primarily affects young adults; reports
that establish the age of onset as ranging from 3 to 50 years have been published.
• The average age of disease onset is 29 years. No gender predilection seems to
exist in most of the literature series. In 25% of the patients, the eye and the ear
can be affected simultaneously and in another 10% of the cases, systemic
vasculitis can complicate the course of the disease
clinical presentation
• Typical Cogan syndrome manifests with interstitial keratitis and audiovestibular
symptoms similar to Meniere disease (tinnitus, vertigo, and hearing loss). Auditory
symptoms can precede or follow eye disease, usually within two years.

• Atypical Cogan syndrome presents with other types of inflammatory eye disease (such as
uveitis, scleritis, and optic neuritis), audiovestibular symptoms that may not resemble
Meniere disease, and longer intervals between the onset of ocular and vestibuloauditory
disease. However, this distinction may not carry prognostic significance.

• A substantial minority of patients also develop systemic vasculitis. Common systemic

symptoms include headache, arthralgia/arthritis, myalgia, fever, and fatigue. The most
characteristic cardiovascular manifestation is aortitis.
Diagnosis-
• clinical diagnosis - it includes all these listed below
• chief complaint- red eye, photophobia, blurred vision, vertigo
• Signs and symptoms- Redness, pain in the eyes and extreme sensitivity to bright
light, Interstitial keratitis vertigo, tinnitus.
• medical and social history- we will ask for any other disease, smoking drinking
etc,
• physical diagnosis and inspection
• imaging techniques
• Cogan’s syndrome is a clinical diagnosis based on the clinical findings and
exclusion of infectious causes. Most patients initially present with eye symptoms.
• slit lap examination- we commonly notice limbal edema and stromal opacity and
fine vasculat loops.
• in some cases MRI scan and CT scan to get a better look at your eyes and ears
Blood test to rule out other disease
Pathology
• Recent experiences strongly suggest that Cogan's syndrome is an autoimmune disease,
mediated by means of a hypersensitivity response to one or more infectious agents
associated with vasculitisSeveral authors have noted an immediately preceding upper
respiratory tract infection. Thus, it is quite probable that a virus infection prompts an
antibody response that develops a cross-immunity with similar proteins in the tissues of
the audiovestibular system, eye, and occasionally other organs as well. Temporal bone
pathology includes endolymphatic hydrops, atrophy of the organ of Corti, plasma cell
and lymphocytic infiltration of the spiral ligament, osteoneogenesis of the round
window, spiral ganglion cell degeneration, cystic degeneration of the stria vascularis,
middle ear effusion, demyelination of the eighth cranial nerve and vasculitis of the
internal auditory artery.
• Typical laboratory markers of systemic autoimmune disease (e. g. , a n t i nu clear
antibodies, antineutrophil - cytoplasmatic antibodies) could be detected in a very small
number of patients . Several patients tested for antibodies to corneal antigens were
found to be positive.
• Lymphocyte transformation has reportedly been detected when the patient's
lymphocytes are exposed to corneal antigen, scleroprotein, and inner ear antigen,
suggesting the presence of cell-mediated autoimmune reactivity
Differential Diagnosis
Management
Treatment-
• Cogan syndrome is a systemic disease that can lead to severe functional
impairments; therefore, clinical management and treatment must involve by
different specialists whose competencies should be coordinated
• the development of systemic vasculitis require systemic immunosuppressive
therapy. Corticosteroids at high dosages remain the mainstay at the beginning
and during the acute phases of the disease
Pharmacological treatment
• Corticosteroids
• Cyclophosphamide
• Methotrexate
• Azathioprine
• Cyclosporine A
• Biological therapy- Biological therapy for the treatment of autoimmune diseases
consists of genetically engineered proteins derived from human genes.
• The use of biological therapy in Cogan syndrome patients included for anti-TNFα
agents, rituximab (RTX) and tocilizumab (TCZ).
Prognosis
• Many people with Cogan's syndrome have permanent visual or hearing damage.
The condition is fatal in less than 10% of patients. However, most patients are
able to manage their symptoms and limit complications of the disease with
treatment.
• The long-term outlook for people with Cogan syndrome also depends on the
involvement of the blood vessels. If the blood vessels are affected by the
autoimmune response associated with Cogan syndrome and the treatment is not
effective, symptoms such as stomach pain or headaches may persist. Life-
threatening complications of Cogan syndrome are rare but may include an
aneurysm of the aorta.
• Permanent hearing loss is more common than permanent vision loss, and hearing
loss may not be improved with the use of medications. The medications used to
treat Cogan syndrome may have side-effects, so the risks and benefits of each
medication must be considered.
patient case presentation
• Chief Complaint: Red left eye for the past two months with recent onset of
vertigo and otalgia.

• History of Present Illness: 42-year-old Caucasian female complains of an

annoying red left eye for the past two months. On occasion, the left eye has been
"aching" with a "pressure sensation". Despite a course of antibiotics to treat a
suspected sinusitis, her symptoms persisted and gradually worsened with time. In
the past month she also developed vertigo, nausea, otalgia, diminished appetite,
as well as progressively worsening vision. Acetaminophen provided moderate
relief from her pain. She denied any hearing loss, diplopia, fever, cough,
rhinorrhea, otorrhea, tinnitus, and vomiting. She presented to the University of
Iowa in the spring of 2007 after having been seen by several doctors and
frustrated with her persisting and progressive symptoms.
• Past Ocular History: Unremarkable for surgery or trauma. Other history as noted above.

• Medical History: History of hypothyroidism, Type II diabetes mellitus, nonalcoholic

steatohepatitis, polycystic ovarian sydrome, uterine fibroids, fibromyalgia, Celiac's disease,
and borderline hypertension.

• Medications: Trazodone, lorazepam, meperidine, levothyroxine, albuterol, fluoxetine,

montelukast, and cyclobenzaprine. Additionally, the patient periodically takes a daily
multivitamin, fish oil, acetaminophen, and calcium and magnesium supplements..

• Family History: No history of coagulopathy.

• Social History: Non-contributory.

• Ocular Examination: Moderate photophobia noted during the exam.
• Visual Acuity, with correction at distance: Right eye 20/20; Left eye 20/20
• Extraocular motility: Full,
• Pupils: No relative afferent pupillary defect (RAPD)
• Intraocular pressure: Normal, OU
• Slit lamp examination: Limbal edema and stromal opacity were noted as well as several fine
vascular loops extending into the mid-stroma of the cornea There is mild vascular injection,
especially inferiorly and at the episclera covered by the lower lid (not visualized in this Figure),
associated with tenderness of the globe.
• Dilated fundus exam: Normal, OU
clinical course
• The patient appears to have interstitial keratitis and episcleritis. Interstitial
keratitis is an uncommon chronic, non-ulcerative inflammation of the corneal
stroma that may be seen in isolation or may be associated with uveitis or
episcleritis, as was the case with this patient. Symptoms include pain, tearing,
photophobia, gradual blurring of vision.
• his Laboratory studies showed leukocytosis, increased ESR, and increased CRP.
• Some systemic associations may include life-threatening aortitis (up to 10%),
inflammatory bowel disease, pericarditis, abdominal pain, fatigue, weight loss,
arthralgia, myalgia, fever, headache, and urticaria
treatment
• Anterior ocular inflammation usually treated with topical corticosteroids
• Mydriatics may be added for comfort
• Topical or oral NSAIDs may be helpful for episcleritis and scleritis

• Systemic corticosteroids may be required for posterior ocular inflammation

• Vestibuloauditory dysfunction (especially hearing loss) is treated with 1-2mg/kg/day of

prednisone for 2-6 months duration

• Steroid-sparing agents (methotrexate, cyclophosphamide, azathioprine, tacrolimus) may

be considered
• Systemic vasculitis is treated with prednisone (usually 1 mg/kg/day)