Congenital SO palsy vs Acquired SO palsy I’m a 3rd yr resident Kim EK. This case is supervised by associate Professor Sin sun young. R3 김은경/ AP. 신선영

Case 1 C.C) Diplopia associated with head trauma Onset : 1 month ago - Struck on the head by a soccer ball Onset : 1 month ago Systemic disease(-) Trauma Hx.(+) A 24 year old male visited our clinic with diplopia which had been developed since he was struck on the head by a soccer ball. This picture was taken at the fisrt timing of the visit. He had no history of systemic disease

RSO palsy RIO overaction RSO underaction He presented with right hypertropia in the primary position, and his right eye showed overaction of inferior oblique and underaction of superior oblique. Right hypertropia was more in the left down gaze. Form this 9 - cardinal photos we could suspect he had RSO palsy. RSO underaction

Congenital RSO palsy vs Acquired RSO palsy After then we had to differentiate congenital or acquired type of RSO palsy in order to decide whether brain imaging was required or not.

Other Clues Final Diagnosis Congenital Rt. SO palsy 1. Facial asymmetry (+) 2. Old photo : Head tilt to left shoulder 3. Large vertical fusional amplitude As you can see, facial asymmetry was present and may be result of chronic head tilt , and large vertical fusional amplitude was also present. These findings are suggestive of a congenital RSO palsy. Final Diagnosis Congenital Rt. SO palsy

Case 2 F/64 C.C) Head tilt to right shoulder Blurring caused head tilt to Lt. shoulder Onset : 5 ~6 months ago Gradual progression Facial asymmetry(+) DM(-), HBP(-) Trauma Hx.(-) A 64 year old female visited our clinic complaining of head tilt to right shoulder and blurring vision caused head tilt to the Lt.shoulder over the previous 6 months. Her symptoms had gradually developed, and facial asymmetry was present at the timing of the first visiting. She had no history of DM & HBP & head trauma.

LIO overaction Mild LSO underaction She presented with 16 prism dioptor left hypertropia in the primary position, and her left eye showed over-action of inferior oblique and mild underaction of superior oblique. Left hypertropia was more in the right down gaze. Mild LSO underaction

Excyclotorsion Fundus photos showed excyclotorsion.

Parks’ 3 step test LSO palsy 20Δ LHT 16Δ LH(T) 10Δ LHT 20 Δ LH 25Δ LHT 10 Δ LHT Lt.eye SR IO IO SR Rt.eye For identifying the paretic muscle, the Parks three test was used. Left hypertropia increased in downgaze and right gaze and also on left head tilt. These findings indicate a paretic oblique muscle is LSO. IR SO SO IR LSO palsy

Impression r/o Acquired Lt. SO palsy r/o Decompensated congenital Lt. SO palsy Again we had to differentiate congenital or acquired type of LSO palsy.

Clues Final Diagnosis Congenital Lt. SO palsy 1. Facial asymmetry (+) Hypoplastic Rt.facial structrue 2. Gradual progression 3. No Hx. of head trauma 4. Large vertical fusional amplitude Even though she had no history of head trauma, considering of her age we decided to perform bain imaging. CT images showed hypoplasty of Rt.facial structure and atrophy of LSO muscle. According to her history , diplopia had developed gradually and large vertical fusional amplitude was present , we could guess she had congenital LSO palsy. Final Diagnosis Congenital Lt. SO palsy

Case 3 C.C) Binocular diplopia Head tilt to right shoulder M/14 C.C) Binocular diplopia Head tilt to right shoulder Onset : 4 months ago , suddenly Facial asymmetry(-) DM(-), HBP(-), Systemic disease(-) Trauma Hx.(-) A 14 year old male complained of sudden onset of diplopia and head tilt to right shoulder over the previous 4 months. He had no facial asymmetry at the timing of visiting and he had no history of DM & HBP & head trauma.

LIO overaction LSO underaction He presented with 12 prism dioptor left hypertropia in the primary position, and his left eye showed over-action of inferior oblique and underaction of superior oblique. Left hypertropia was more in the right down gaze. LSO underaction

Fundus photos showed excyclotorsion.

Parks’ 3 step test LSO palsy 16Δ LHT 12Δ LHT 8Δ LHT 25Δ RHT 10Δ RHT Lt.eye SR IO IO SR Rt.eye Left hypertropia increased in downgaze and right gaze and also on left head tilt. These findings imply a paretic oblique muscle is LSO. IR SO SO IR LSO palsy

decompensated congenital SOP In this patient, Small vertical fusional amplitude Old photo : no head tilt decompensated congenital SOP Recommend BMRI In this patient , since he presented with small vertical fusional amplitude, and there was no head tillt in his old photos, we could exclude decompensated congenital SOP , so we decided to perform braing MRI.

Final Diagnosis Acquired Lt. SO palsy due to 4th nerve palsy Carvernous hemangioma in midbrain Brain MRI of confirmed carvernous[kǽvərnəs] hemangioma[himæ̀ndƷióumə] in mid brain. So the following diagnosis was acquired LSO palsy associated with brain tumor. Final Diagnosis Acquired Lt. SO palsy due to 4th nerve palsy

Thank you !

“Cause & Clinical features” Review Congenital SO palsy vs Acquired SO palsy “Cause & Clinical features”

Anatomy Origin : orbital apex above AZ Trochlea (tendon: longest, 26 mm length) Insertion : sup.temp. Q of eyeball Primary action: intorsion (ant. 1/3) Secondary action: depression & abduction (post. 2/3)  Paresis: excyclotropia , hypertropia , esotropia

The Four Golden Rules of the patient with a vertical strabismus A vertical strabismus is caused by a superior oblique palsy until proved otherwise. A superior oblique palsy is congenital until proved other wise. A superior oblique palsy is traumatic if not congenital If the superior oblique palsy is not congenital, decompensated congenital or traumatic in origin, a neurologic consultation to exclude intracranial neoplasm and other acquired lesion is essential

Superior Oblique Palsy Cause Superior Oblique Palsy

Superior Oblique Palsy Congenital SOP Approximately 3/4 of SOP cases Cause Unknown SO tendon abnormalities SO muscle volume reduction

Superior Oblique Palsy Acquired SOP Cause Trauma: most common Inflammation Infection Vascular malformation Infarction Tumor Myasthenia gravis Surgery : ethmoidectomy, orbital surgery, blepharoplasty

Superior Oblique Palsy Clinical Features Superior Oblique Palsy

Superior Oblique Palsy Clinical features Congenital vs Acquired Congenital : greater vertical deviations little or no subjective torsion * Decompensated congenital SO palsy : aging  weaken fusional control  manifest tropia, diplopia - Acquired : smaller vertical deviations relatively larger degree of excyclotorsion 나이가 들어감에 따라 fusional control 약화

congenital vs. acquired Duration of symptom long short Vertical fusion amplitude large small Diplopia +/- + Head tilt if covering each eye Facial asymmetry: hemifacial hypotrophy IOOA/SOUA Severe/mild Old photo(Head tilt) yes no

Superior Oblique Palsy Clinical features “ Fusional vergence amplitude (FVA) “ The difference in the power needed to turn the eyes from their far point to their near point of convergence Also called fusional amplitude , vergence ability

Superior Oblique Palsy Clinical features “ Fusional vergence amplitude (FVA) “ Congenital - large fusional amplitude, average 16 PD (some, 30 PD or more) - symptomatic (diplopia) : age-related reduction in FVA Acquired - very little fusional amplitude

Superior Oblique Palsy Clinical features “ Diplopia “ Vertical diplopia Some degree of horizontal or torsional diplopia - Vertical : worse in gaze directed toward the uninvolved eye associated with IOOA common in acquired cases congenital cases: 25% - Torsional : most pronounced on down gaze(in the field of action of SO) bilateral SO palsy

Superior Oblique Palsy Clinical features “Head tilt” - head tilt to sound eye, 70% * Paradoxical head tilt head tilt to affected side wide separation of double image * Absence of head tilt inabilty to obtain SBV by any head position presence of large fusional amplitude reduced VA in one eye

Superior Oblique Palsy Clinical features “Facial asymmetry” Secondary to chronic head tilting ¾ of patients with congenital SO palsy Head tilt away from the affected side Not specific for SO palsy lateral canthus shallow edge of mouth midfacial hypoplasia on the side of head tilt

Thank you !