Orbital Disorders I have the following financial interests or - PowerPoint PPT Presentation

11/11/17 Relevant Financial Disclosures Orbital Disorders • I have the following financial interests or relationships to disclose: Joseph L. Demer, MD, PhD Consultancy on unrelated topic from Alcon Japan 1. Arthur L. Rosenbaum Chair of Pediatric Ophthalmology Professor of Neurology Grant support from USPHS, National Eye Institute, 2. EY008313 for this work Rectus Pulley System: Topics Inner Gimbal of the Eye Common Non-neurological Causes of Strabismus: CHILDREN: Congenital malpositioning of structurally sound rectus pulley system. OLDER ADULTS: Acquired rectus pulley malpositioning due to connective tissue degeneration. Novel Neurological Causes of Strabismus: Compartmental lateral rectus palsy. Compartmental superior oblique palsy. Demer Orbital Disorders AAPOS'18 Presentation 1

11/11/17 Kono, R., Poukens, V., and Demer, J. L. Quantitative analysis of the structure of the human extraocular muscle pulley system, Invest. Ophthalmol. Vis. Sci. 43: 2923-2932 , 2002 . Congenital A Pattern Pulley Heterotopy Childhood Onset Pulley Het erotopy -4 -4 34 cases (from prospective imaging data set of 577 total strabismus cases and 157 normal controls) +4 +4 MRI ALWAYS shows robust LR-SR band. Congenital A Pattern Pulley Heterotopy Demer Orbital Disorders AAPOS'18 Presentation 2

11/11/17 Congenital A Pattern Pulley Heterotopy Congenital A Pattern Pulley Heterotopy Lid fissures usually correlate. Congenital V Pattern Pulley Heterotopy Congenital V Pattern Pulley Heterotopy +4 +4 -4 -4 Demer Orbital Disorders AAPOS'18 Presentation 3

11/11/17 Congenital V Pattern Pulley Heterotopy Plication of LR-SR Band Insertional Transposition With or without scleral posterior fixation sutures. Lid fissures sometimes correlate. Note: Always induces adverse torsion (Kushner 2013). Age-Related Degeneration Late Onset Pulley Heterotopy of LR-SR Band 28+cases Diplopia onset: 68 ± 12 (std. dev.) years MRI NEVER shows robust LR-SR band. It is usually ruptured. Sagging Eye Syndrome Demer Orbital Disorders AAPOS'18 Presentation 4

11/11/17 MRI Shows Age-Related Degeneration Bilaterally Symmetrical LR Sag: of LR-SR Band Divergence Paralysis Esotropia (DPE) Older Older Young 1. Symmetrical reduction of abduction by both LR’s. Orthotropic Strabismic 2. Symmetrical infraduction by both LR’s. Cases of Asymmetric LR Sag Causes Cyclovertical Strabismus Divergence Paralysis Esotropia (DPE) = Age-related Distance Esotropia Criteria 1.ET at distance, vertically comitant 2.Fusion at Near 3.Normal Abduction Saccades Patients- 11 1.Age: 72 ± 11 years 2.Gender: 7 women and 4 men 3.Distance ET: 11.5 ± 10.6 D (SD) 4.Near E’: 1.3 ± 3.1 D Exclusions: 1.Orbital Trauma 2.Restrictive Strabismus (i.e. thyroid) 1. Asymmetrically greater infraducting action of one LR. 3.Prior strabismus surgery 2. Greater excyclotropia of more affected LR. 4.High myopia Demer Orbital Disorders AAPOS'18 Presentation 5

11/11/17 Cases of Cyclovertical Magnetic Resonance Imaging Strabismus (CVS) Surface coils Central target fixation Image Planes: Patients- 17 Quasi-coronal planes for pulley positions 1.Age: 68 ± 2 years Axial planes for horizontal rectus lengths 2.Gender: 10 women and 7 men Controls 3.Distance HT: 9.9 ± 9.4 D (SD) Younger Normal: 52 orbits, 28 subjects Normal eye exam Exclusions: Age 23 ± 5 years 1.Superior Oblique Palsy Age-Matched Older Normal: 25 orbits, 14 subjects 2.Orbital Trauma 3.Restrictive Strabismus (i.e. thyroid) No ocular disease (pseudophakia allowed) Age 65 ± 5 years 4.Prior strabismus surgery 5.High myopia 6.Skew deviation Superior Sulcus Defect in SES Blepharoptosis in SES 29% had ptosis and high lid crease. 64% of subjects 29% had prior blepharoplasty, brow lift, or face lift surgery . Demer Orbital Disorders AAPOS'18 Presentation 6

11/11/17 Rectus Pulleys Heterotopic in SES Always Asymmetrical > 1 mm P = 0.02 OD-OS Symmetrical Excyclo Excyclo < 0.5 mm 7 ± 5° 12 ± 6° P = 0.8 P = 0.01 14/22 31/34 Ruptured Ruptured Demer Orbital Disorders AAPOS'18 Presentation 7

11/11/17 Horizontal Rectus Muscle Elongation in SES Medial Rectus Recession for DPE in SES Graded Vertical Rectus Tenotomy For Cyclovertical Strabismus in SES Avoids surgery near the fragile LR-SR band. Partial tenotomy at margin of scleral insertion MR recession dose: Reduces EOM tension 1. Doubling the measured distance ET. 2D for 40% to 6D for 80%. 2. Recess MR based on surgical dose table of Parks et al. for doubled ET. You Get All But You Get Less Demer Orbital Disorders AAPOS'18 Presentation 8

11/11/17 Heavy Eye Syndrome SES is NOT “Heavy Eye Syndrome” “Heavy Eye Syndrome” “Sagging Eye Syndrome” • Inferotemporal shift of lateral rectus. • Inferonasal shift of lateral rectus, • Temporal shift of inferior rectus. hugging the globe. • No shift of superior rectus. • Nasal shift of inferior rectus. • Lateral rectus shifted away from • Nasal shift of superior rectus globe. “Sagging Eye Syndrome” “Sagging Eye Syndrome” Age-related degeneration of LR-SR band allows lateral rectus pulley to Adnexal “sag” strongly correlates with pulley sag. shift and tilt inferolaterally. Diagnosis does NOT require imaging in most cases. Rectus EOMs elongate. Divergence paralysis ET treatable by large MR recessions. When symmetrical: divergence paralysis esotropia. Cyclovertical strabismus treatable by partial vertical rectus tenotomy. When asymmetrical: ipsilateral hypotropia and excyclotropia SES is probably a major cause of adult acquired strabismus . (Note: SO palsy causes ipsilateral hypertropia and excyclotropia). Demer Orbital Disorders AAPOS'18 Presentation 9

11/11/17 MRI Shows Dual Compartments of Compartmental Pathology of Normal Lateral Rectus Muscle Horizontal Rectus Muscles Human Abducens Nerve Has Longitudinal Fissure In Human Lateral Rectus Muscle Superior & Inferior Divisions Frequent congenital cranial dysinnervation disorders: Duane syndrome Congenital fibrosis Occasionally in normal subjects . Lateral Rectus Demer Orbital Disorders AAPOS'18 Presentation 10

11/11/17 Abducens Nerve Has Superior & Human Medial Rectus Motor Nerve Has Superior & Inferior Divisions Inferior Divisions Demer Orbital Disorders AAPOS'18 Presentation 11

11/11/17 Patient WS Patient WS Left Superior Compartment Lateral Rectus Palsy Left Superior Compartment Lateral Rectus Palsy Incomitant Esotropia Limited Abduction Pre-operative Patient WS Superior Left Superior Compartment Lateral Rectus Palsy LR Atrophy Improved 3. Recess left MR 5 mm. 1. Plicate superior LR tendon 10 mm Post-operative 2. Transpose superior LR tendon 1/2 tendon width Demer Orbital Disorders AAPOS'18 Presentation 12

11/11/17 Ipsilesional Hypotropia in 15/39 Cases Trochlear Nerve Branches in Adult Superior Oblique Muscle (Confirmed in 4 specimens) Medial Lateral Le, A., Poukens, V., Ying, H., Rootman, D., Goldberg, R. A., and Demer, J. L. Compartmental innervation of the superior oblique muscle in mammals. Invest. Ophthalmol. Vis. Sci. 56: 6237-6246, 2015. Le, A., Poukens, V., Ying, H., Rootman, D., Goldberg, R. Le, A., Poukens, V., Ying, H., Rootman, D., A., and Demer, J. L. Compartmental innervation of the Goldberg, R. A., and Demer, J. L. Compartmental innervation of the superior superior oblique muscle in mammals. Invest. Ophthalmol. Vis. Sci. 56: 6237-6246, 2015. oblique muscle in mammals. Invest. Ophthalmol. Vis. Sci. 56: 6237-6246, 2015. Alan Le, Robet Goldberg, Dan Rootman, Adult Human Superior Oblique Adult Human Superior Oblique Medial Lateral Medial Lateral Demer Orbital Disorders AAPOS'18 Presentation 13

11/11/17 Superior Oblique Compartments Torsional Vertical Alan Le, Robet Goldberg, Dan Rootman, Adult Human Superior Oblique Medial Lateral Le, A., Poukens, V., Ying, H., Rootman, D., Goldberg, R. A., and Demer, J. L. Compartmental innervation of the superior oblique muscle in mammals. Invest. Ophthalmol. Vis. Sci. 56: 6237-6246, 2015. Palsy Normal 36 Anisotropic Atrophy 62 Cases of SO Atrophy 26 Isotropic Anisotropic Isotropic Atrophy Atrophy Atrophy Shin, S. Y. and Demer, J. L. 16 Normal Superior oblique extraocular muscle shape in superior Controls oblique palsy. Am. J. Normal Ophthalmol. 159: 1169-1179, 2015. Demer Orbital Disorders AAPOS'18 Presentation 14

11/11/17 Both Axes Major Axis Reduced Preserved Shin, S. Y. and Demer, J. L. Superior oblique extraocular muscle shape Shin, S. Y. and Demer, J. L. Superior oblique extraocular muscle shape in superior oblique palsy. Am. J. Ophthalmol. 159: 1169-1179, 2015. in superior oblique palsy. Am. J. Ophthalmol. 159: 1169-1179, 2015. Shin, S. Y. and Demer, J. L. Superior oblique extraocular muscle shape Shin, S. Y. and Demer, J. L. Superior oblique extraocular muscle shape in superior oblique palsy. Am. J. Ophthalmol. 159: 1169-1179, 2015. in superior oblique palsy. Am. J. Ophthalmol. 159: 1169-1179, 2015. Demer Orbital Disorders AAPOS'18 Presentation 15