Case conference -strabismus- Department of Ophthalmology and Visual Science, College of Medicine, The Catholic University of Korea, Seoul, Korea. R3 SY Kim/ AP. SY Shin Let us begin the case on “strabismus”.

C/C Diplopia Case 1 (M/46) Onset ) 2mos ago “뒤로 넘어지면서 T.SAH 발생한 이후 3.19. 2009 C/C Diplopia Onset ) 2mos ago “뒤로 넘어지면서 T.SAH 발생한 이후 복시가 발생 했어요.” A 46-year old male patient visited our clinic. He complained of diplopia, after falling backwards 2 months ago. T.SAH had occurred at that time. The diplopia was Vertical and torsional.

SO underaction D&V OU) SOUA -2.0 -1 -2 This figure is 9 gaze photo. On Primary position, the left eye is slightly elevated. On adduction, both eyes have difficulty looking downwards. EOM limitation was -2. It means superior oblique underaction SO underaction -1 -2

Lt inferior movement limitation Lancaster test This shows the Lancaster test. Left inferior movement limitation is suspected. Excyclotorsion is present. Lt inferior movement limitation Excyclotorsion

Disc/Fovea relation Excyclotorsion This is the Fundus photo. We can see the Disc fovea relation. Fovea is under this line. This is the excyclotorsion. Excyclotorsion

Problem list Vertical & Torsional diplopia 외상 후 갑작스런 발생 The patient’s problem lists are ; Vertical & torsional diplopia on Lancester test. The fact that the symptom occurred suddenly after trauma.

Vertical Diplopia – Work up PLAN) History taking & Inspection Brain MRI & Orbit imaging Systemic evaluation c TFT Prism cover test Parks’ 3 step test 5. Bielshowsky head tilt test Presence of diplopia/strabismus at childhood. Variability & fatigability For vertical diplopia workup The Plan includes ; Detailed history taking Presence of diplopia/strabismus at childhood. Variability & fatigability of the symptom. Inspection of facial asymmetry and head tilt. Verifying brain tumor, aneurysm and muscle anomaly by Brain MRI / Orbit MRI. Systemic evaluation including TFT. the paralyzed muscle can be found through Prism cover test.

Vertical Diplopia – Differential diagnosis DVD(Dissociated vertical dissociation) Primary inferior oblique overaction Cranial nerve pasly, III, IV, and VI Skew deviation Brown's Syndrome Thyroid-related ophthalmopathy Duane’s Retraction Syndrome Myasthenia Gravis Orbital Blow‐out Fracture Post‐Surgical (“iatrogenic”) • strabismus surgery • retinal detachment repair • cataract surgery 11. Tumor causes of strabismus: • Brain tumor • aneurysm • Vertical diplopia can cause the following disease.

No Variablity, fatigability Vertical Diplopia – Differential diagnosis DVD(Dissociated vertical dissociation) Primary inferior oblique overaction Cranial nerve pasly, III, IV, and VI Skew deviation Brown's Syndrome Thyroid-related ophthalmopathy Duane’s Retraction Syndrome Myasthenia Gravis Orbital Blow‐out Fracture Post‐Surgical (“iatrogenic”) • strabismus surgery • retinal detachment repair • cataract surgery 11. Tumor causes of strabismus: • Brain tumor • aneurysm • History taking is important No Variablity, fatigability Surgery Hx (-) History taking is important. Myasthenia gravis is unlikely because the symptoms show no variability, fatigability. There was no Surgery Hx. So These are also unlikely.

No Variablity, fatigability Vertical Diplopia – Differential diagnosis DVD(Dissociated vertical dissociation) Primary inferior oblique overaction Cranial nerve pasly, III, IV, and VI Skew deviation Brown's Syndrome Thyroid-related ophthalmopathy Duane’s Retraction Syndrome Myasthenia Gravis Orbital Blow‐out Fracture Post‐Surgical (“iatrogenic”) • strabismus surgery • retinal detachment repair • cataract surgery 11. Tumor causes of strabismus: • Brain tumor • aneurysm • History taking is important No Variablity, fatigability Surgery Hx (-) History taking is important. Myasthenia gravis is unlikely because the symptoms show no variability, fatigability. There was no Surgery Hx. So These are also unlikely.

No Variablity, fatigability Vertical Diplopia – Differential diagnosis DVD(Dissociated vertical dissociation) Primary inferior oblique overaction Cranial nerve pasly, III, IV, and VI Skew deviation Brown's Syndrome Thyroid-related ophthalmopathy Duane’s Retraction Syndrome Myasthenia Gravis Orbital Blow‐out Fracture Post‐Surgical (“iatrogenic”) • strabismus surgery • retinal detachment repair • cataract surgery 11. Tumor causes of strabismus: • Brain tumor • aneurysm • History taking & Brain imaging need Orbit CT normal TFT normal No Variablity, fatigability Surgery Hx (-) if Brain MRI, Orbit MRI are normal. These are also unlikely. Brain imaging : T-SAH

No Variablity, fatigability Vertical Diplopia – Differential diagnosis DVD(Dissociated vertical dissociation) Primary inferior oblique overaction Cranial nerve pasly, III, IV, and VI Skew deviation Brown's Syndrome Thyroid-related ophthalmopathy Duane’s Retraction Syndrome Myasthenia Gravis Orbital Blow‐out Fracture Post‐Surgical (“iatrogenic”) • strabismus surgery • retinal detachment repair • cataract surgery 11. Tumor causes of strabismus: • Brain tumor • aneurysm • History taking & Brain imaging need Orbit CT normal TFT normal No Variablity, fatigability Surgery Hx (-) The others need prism cover test and parks’ 3 step test. Brain imaging : T-SAH

No Variablity, fatigability Vertical Diplopia – Differential diagnosis DVD(Dissociated vertical dissociation) Primary inferior oblique overaction Cranial nerve pasly, III, IV, and VI Skew deviation Brown's Syndrome Thyroid-related ophthalmopathy Duane’s Retraction Syndrome Myasthenia Gravis Orbital Blow‐out Fracture Post‐Surgical (“iatrogenic”) • strabismus surgery • retinal detachment repair • cataract surgery 11. Tumor causes of strabismus: • Brain tumor • aneurysm • Prism cover test Parks’ 3 step test History taking & Brain imaging need Orbit CT normal TFT normal No Variablity, fatigability Surgery Hx (-) The others need prism cover test and parks’ 3 step test. Brain imaging : T-SAH

Prism cover test RHT XT at upgaze ET at downgaze = V pattern !! Prism cover test was done. It showed mostly RHT, and XT at upgaze, ET at downgaze. So It is V-pattern strabismus. RHT worsened on Rt head tilt. So Bielshowsky head tilt test was positive. But on Lt head tilt RHT was gone, and showed LHT instead. It shows positive Reversing Bielshowsky test. Bielshowsky head tilt test(+) Reversing Bielshowsky head tilt test(+)

Parks’ three step test 에 적용 Prism cover test Parks’ three step test 에 적용 6XT 2RHT 7RHT 9LHT 6RHT 7ET 9LHT 6ET 6ET Prism cover test result is applied to Parks’ three step test 25ET 3RHT

Parks’ three step test 1단계 : RHT ? or LHT ? on primary position 2단계 : 오른쪽 주시 또는 왼쪽 주시시 어느 쪽이 더욱 심한가? 3단계 : 머리를 오른쪽 또는 왼쪽으로 기울일 때 어느 쪽이 더욱 심한가?• Parks’ three step test is examining which muscle was paralized. Step1 is examining whether it is RHT or LHT on primary position, Step2 is finding the worse side : Rt gaze or Lt gaze, Step3 is finding the worse side on head tilt.

Parks’ three step test algorithm There was RHT. RHT worsened on Lt gaze and Rt tilt.

Parks’ three step test algorithm

Parks’ three step test algorithm

Parks’ three step test algorithm

Parks’ three step test algorithm

Parks’ three step test algorithm The conclusion is RSO palsy. But there was also LHT, and LHT worsened on Rt gaze and Lt tilt. This means LSO palsy

RSO palsy ? LSO palsy ? or Both? CASE DIAGNOSIS Then, is this RSO palsy, LSO palsy, or both? RSO palsy ? LSO palsy ? or Both?

Superior oblique palsy Vertical diplopia – Differential diagnosis Bilateral ?? DVD Primary inferior oblique overaction Cranial nerve pasly, III, IV, and VI Skew deviation Brown's Syndrome Duane’s Retraction Syndrome • Superior oblique palsy And then we think bilateral or unilateral Congenital or acquired........ We found that it is superior oblique palsy, and then we must think whether bilateral or unilateral, congenital or acquired.

Monocular vs. Bilateral Palsy Superior oblique palsy Monocular vs. Bilateral Palsy Bilateral Superior Oblique Palsy – Characteristics 1) History of trauma (70%) 2) Spontaneous torsional diplopia , none or minor vertical diplopia 3) "V" Pattern, ET down gaze 4) Extorsion >15 degrees 5) Reversing (or nearly so) Bielshowsky head tilt test 6) Chin down, eyes up posture Bilateral superior oblique palsy characteristics are 1…. The patient suits 1.2.3.5

Monocular vs. Bilateral Palsy Superior oblique palsy Monocular vs. Bilateral Palsy Bilateral Superior Oblique Palsy – Characteristics 1) History of trauma 2) Spontaneous torsional diplopia , none or minor vertical diplopia 3) "V" Pattern, ET down gaze 4) Extorsion >15 degrees 5) Reversing (or nearly so) Bielshowsky head tilt test 6) Chin down, eyes up posture Double Maddox rod test Double Maddox rod test must be done to see if excyclotorsion is over 15 degrees

Double Maddox Rod test 정면주시시 15' 내회선 후 중화 하방주시시 20' 내회선 후 중화 15-20 degrees of excyclotorsion was found in double maddox rod test. This finding is appropriate for bilateral superior oblique palsy.

Superior oblique palsy Vertical diplopia – Differential diagnosis Bilateral ?? DVD Primary inferior oblique overaction Cranial nerve pasly, III, IV, and VI Skew deviation Brown's Syndrome Duane’s Retraction Syndrome • Superior oblique palsy Thus, bilateral superior oblique palsy is suspected.

Superior oblique palsy Vertical diplopia – Differential diagnosis DVD Primary inferior oblique overaction Cranial nerve pasly, III, IV, and VI Skew deviation Brown's Syndrome Duane’s Retraction Syndrome • Bilateral !! Superior oblique palsy Thus, bilateral superior oblique palsy is suspected.

Superior oblique palsy Vertical diplopia – Differential diagnosis DVD Primary inferior oblique overaction Cranial nerve pasly, III, IV, and VI Skew deviation Brown's Syndrome Duane’s Retraction Syndrome • Bilateral !! Congenital ?? Superior oblique palsy We must consider congenital superior oblique palsy as well.

Congenital vs. acquired Late onset, Trauma Hx , Torsional diplopia, Superior oblique palsy Congenital vs. acquired Torsional diplopia 가 없음 2) Long history of head tilt 3) Facial asymmetry which we are convinced is secondary to early and longstanding head tilt.  4) Lax superior oblique tendon confirmed by the superior oblique traction test done at surgery - in some cases the superior oblique tendon was shown to be absent! Old photo Late onset, Trauma Hx , Torsional diplopia, No facial asymmetry… Congenital superior oblique palsy shows no Torsional diplopia. It has Long history of head tilt, Facial asymmetry which we are convinced is secondary to early and longstanding head tilt, Lax superior oblique tendon confirmed by the superior oblique traction test done at surgery. This patient shows Late onset, Trauma Hx , Torsional diplopia, No facial asymmetry. Acquired superior oblique palsy can be concluded.

Superior oblique palsy Vertical diplopia – Differential diagnosis DVD Primary inferior oblique overaction Cranial nerve pasly, III, IV, and VI Skew deviation Brown's Syndrome Duane’s Retraction Syndrome • Bilateral !! Acquired!! Superior oblique palsy Acquired superior oblique palsy can be concluded.

그는 Torsional diplopia 에 If little vertical deviation but large extorsional component Consider Harada-Ito procedure 그는 Torsional diplopia 에 괴로워하고 있었으며 외회선을 바로 잡는 수술인 Harada Ito 수술을 하기로 결심하였다. If there is little vertical deviation but large extorsional component, Harada-Ito procedure can be considered . The patient was suffering of Torsional diplopia, and decided to take Harada-Ito operation, which adjusts the excyclotorsion.

Harada-Ito Anterior displacement of ½ SO tendon Harada-Ito operation is Anterior displacement of ½ SO tendon. Dr. G.Vicente

Harada-Ito Anterior displacement of ½ SO tendon So the surgery strengthens incyclotorsion function Dr. G.Vicente

수술 후 회선 감소 POD 3일 PREOP After surgery, excyclotorsion markedly decreased.

Case Review -Bilateral Superior Oblique Palsy- = 4th nerve palsy (used as synonyms) Department of Ophthalmology and Visual Science, College of Medicine, The Catholic University of Korea, Seoul, Korea. R3 SY Kim/ AP. SY Shin Let us begin the case on “strabismus”.

복시 단안 복시 교정되지않은 굴절이상(원시, 난시), 망막이상 양안 복시 한눈 가리면 소실, 수평 or 수직 사시 검사의 시작점

복시환자를 처음 만났을때 진성복시인지 발병 시기 동반 질환/신경학적 증상 영상의학검사가 필요한지? Urgent transfer or not 프리즘안경 or 수술적 치료가 필요한지

마비사시와 비마비사시

Inspection 머리기울임 안면비대칭, 척추 측만 한눈감음 융합을 위한 머리 돌림, 턱위치 이상 안검하수

마비사시라고 판단되면 문진 & 시진 뇌, 안와 영상촬영 전신질환 및 갑상성 질환 Prism cover test Parks’ 3 step test Bielshowsky head tilt test 견인검사, 근력검사 눈운동속도검사 For vertical diplopia workup The Plan includes ; Detailed history taking Presence of diplopia/strabismus at childhood. Variability & fatigability of the symptom. Inspection of facial asymmetry and head tilt. Verifying brain tumor, aneurysm and muscle anomaly by Brain MRI / Orbit MRI. Systemic evaluation including TFT. the paralyzed muscle can be found through Prism cover test.

마비사시의 영상검사 뇌 촬영 MRI MRA 안와촬영 마비근육의 크기 감소 외안근의 크기 증가 숨겨진 안와골절

사시각이 작을때 오래 가린다 4, 6, 8 정도의 작은 프리즘을 대어 해리시킨다. 양안복시인데 사시양이 작은 경우에는 사시를 재기가 곤란하다 오래 가린다 4, 6, 8 정도의 작은 프리즘을 대어 해리시킨다. 복시방향에 맞게 프리즘을 대어 하나로 보이는지, 더 선명하게 보이는지 물어본다.

선천마비, 오래된 마비와 최근마비

Superior oblique palsy

Superior oblique palsy

Superior oblique palsy, Lt

Superior oblique palsy

Superior oblique palsy

Superior oblique palsy Congenital Acquired

Superior oblique palsy 4th nerve palsy

Superior oblique palsy 뇌줄기의 뒤로 나가는 유일한 뇌신경 보호되지 않는 상태로 가장 길게 두개내로 주행  머리외상으로 인한 외안근마비중 활차신경마비가 가장 많다.

환자의 증상 이것은 좌안이다. . The chorioretinal lesions appeared hypofluorescent in the early phases of the study; leakage occurs in the later phases. This is the left eye FAG, leakage occurs in the later phases.

환자의 안구 운동 상태

SOP 의 진단 1단계 : 제일 눈 위치에서 어느 쪽이 상사시? Parks’ three step test 1단계 : 제일 눈 위치에서 어느 쪽이 상사시? 2단계 : 오른쪽 주시 또는 왼쪽 주시시 어느 쪽이 더욱 심한가? 3단계 : 머리를 오른쪽 또는 왼쪽으로 기울일 때 어느 쪽이 더욱 심한가?• Let me introduce parks’ three step test. Step1 is examining whether it is RHT or LHT on primary gaze, Step2 is finding the worse side : Rt gaze or Lt gaze, Step3 is finding the worse side on head tilt.

SOP 의 진단 Parks’ three step test

SOP 회선의 양 측정 Double Maddox Rod test Disc Fovea relation 15-20 degrees of excyclotorsion was found in double maddox rod test. This is appropriate for bilateral superior oblique palsy.

Monocular vs. Bilateral Palsy Superior oblique palsy Monocular vs. Bilateral Palsy Bilateral Superior Oblique Palsy – Characteristics 1) History of trauma (70%) 2) Spontaneous torsional diplopia , none or minor vertical diplopia 3) "V" Pattern, ET down gaze 4) Extorsion >15 degrees 5) Reversing (or nearly so) Bielshowsky head tilt test 6) Chin down, eyes up posture Bilateral superior oblique palsy characteristics are 1…. The patient suits 1.2.3.5

Congenital vs. acquired Superior oblique palsy Congenital vs. acquired Torsional diplopia 가 없음 2) Long history of head tilt 3) Facial asymmetry which we are convinced is secondary to early and longstanding head tilt.  4) Lax superior oblique tendon confirmed by the superior oblique traction test done at surgery - in some cases the superior oblique tendon was shown to be absent! Old photo Congenital superior oblique palsy shows no Torsional diplopia. It has Long history of head tilt, Facial asymmetry which we are convinced is secondary to early and longstanding head tilt, Lax superior oblique tendon confirmed by the superior oblique traction test done at surgery. This patient shows Late onset, Trauma Hx , Torsional diplopia, No facial asymmetry. Acquired superior oblique palsy can be concluded.

SOP image LSO OK RSO ? absent

SOP image RSO clearly smaller than LSO

RSO atrophic

SOP 의 치료 마비사시의 치료는 선천마비 – 소아의 마비사시에 대한 수술은 4,5세에 행하여지는 것이 일반적이나 이상두위 등으로 마비에 대한 보상이 되지 않을 때나 매우 심한 마비 증상을 보일 때는 수술을 일찍 하기도 한다. 후천마비 – 1)마비의 원인을 밝히며 그 원인에 대한 치료가 선생되어야한다. 2)마비가 발생되고부터 또는 원인이 되는 질환을 치료하변서 최소한 6~8개월 동안 상태가 고정될 때까지 자연적인 회복여부를 충분히 관찰하여야 한다. 3) 증상을 완화시켜서 양안 단일시를 가능하도록 이상두위를 유지하게 하거나 프리즘을 일시적으로 이용한다.(프리즘은 대항근수축을 억제하는 데 도움이 된다.)

SOP 의 치료 1. 비수술적 치료 Prism : 편위가 10프리즘이옵터 이하일 때 복시를 제거하기 위한 가장 효과적인 방법. Botulinum toxin : 마비근의 대항근에 주입함으로써 효과적으로 수축을 예방하고 사시각을 감소시킬 수 있다.

SOP 의 치료 2. 수술적 치료 1) 심한 머리기울임이 있는 경우 2) 심한 수직사시가 있는 경우 3) 복시가 있는 경우 4) 얼굴의 비대칭이 있는 경우 마비안의 하전을 시도하는 견인검사가 양성이면 상직근 수축을 의심하여 이 근육을 4~5mm 후전하고 사근에 대한 수술을 한다. 견인검사가 음성이면 비마비안의 하직근을 후전시키는것이 좋다. BSOP 일때 외회선사시가 유일한 장애이고 수직편위가 없으면 하라다-이토 수술을 한다.

SOP 의 치료 -눈의 주시방향에 따라 가장 큰 상사시가 나타나는 위치에 따른 분류 (우안 마비기준 ) - 분류7 : 거짓브라운증후군을 나타내며 안와내의 도르래에 대한 직접적인 손상 원인

SOP 의 치료

Harada-Ito Anterior displacement of ½ SO tendon Harada-Ito operation is Anterior displacement of ½ SO tendon. Dr. G.Vicente

Harada-Ito Anterior displacement of ½ SO tendon So the surgery strengthens incyclotorsion function modulating the direction Dr. G.Vicente

Harada-Ito Anterior displacement of ½ SO tendon So harada ito procedure strengthen incyclotorsion function using anterior displacement Dr. G.Vicente

Harada-Ito Anterior displacement of ½ SO tendon So harada ito procedure strengthen incyclotorsion function using anterior displacement Dr. G.Vicente