HEALTHY LIMBS, FEET & SPINE P A U L S P O N S E L L E R M D - PowerPoint PPT Presentation

HEALTHY LIMBS, FEET & SPINE P A U L S P O N S E L L E R M D M D A D A M B I T T E R M A N M D M D

TOPICS Spine ne iss ssues ues Dural l Ectasia sia Hips ps 2 1/14/2019

Fatigue Back Sight Heart I. MAIN HEALTH CONCERNS IN MFS Rao, Sponseller 2016 ht gue Heart 0 1 2 3 4 5 6 7 8 Spine & Back Sight Fatigue Diff. Concentration Feet Depression Hip Ribs & Breast Bone Dural Ectasia Lungs Diff. Learning Striae Hernia

MAIN SOURCES OF SKELETAL PAIN -FURTHER RESEARCH NEEDED ON THESE Back 6 Hip Neck 5 Knee 4 Shoulder der Feet et 3 2 1 Back Pain Hip Pain Neck Pain Headache Knee Pain Shoulder Pain Ankle Pain Elbow Pain

II. SPINE ISSUES Scoliosis osis Kyphosi hosis Slippi pping ng (Spondyl ndyloli olisthesis) thesis) # M A R FA N C O N F E R E N C E # C R E AT I N G C O N N E C T I O N S

Scoliosis in Marfan Syndrome 3 years Mild later Scoliosis Scoliosis osis affects cts 2/3 of MF Does not ot always s worsen 10% need brace or surge rgery Cur urves es increas rease e fa faster er Increase creased d back k pain 97 °

WILL BRACE HELP SCOLIOSIS? Straight ighten en cur urve? e? – No No “Hold” it? – Maybe be Results sults of braci cing: ng:  20% successes  80% failures Same me princip ciple le for all connect nnective tiss ssue e disor order ders!! !!

BRACE RECOMMENDATIONS Conside sider r brace at earlier er point nt  15-20 o curves in young children Avoid in “low - yield” situations  Bigger curves, reluctant kids

INFANTILE MARFAN SYNDROME Requiring quiring surg rger ery before age 9 Fusi sion on arrests ests trunk unk growth th Solution: “Growing” rods (internal brace)

GROWING ROD RESULTS IN MARFAN 10 Marfan fan childr ldren en  80 o curves  10 cm length in 5 years  No infections

MAGNETICALLY CONTROLLED GROWING RODS (“MAGEC”) If No n need for MRI 11 1/14/2019

ADOLESCENT SCOLIOSIS SURGERY: MFS VERSUS IDIOPATHIC (TYPICAL) Multicen icenter er, Case-Cont ontrol ol 34 Marfan pa patie ients 68 AIS pa patie ients  Matched 1:2 for age, gender and degree of major deformity January 14, 2019 12

RESULTS Marfan n vs. AIS:  More Rod Complications (3 vs 1, p = 0.007)  More Reoperations (8 vs 0, p = 0.01)  More Intraoperative CSF Leaks (3 vs 0, p = 0.01)  Progression of Unfused Thoracic Curves (3 v vs 1, p = 0.107) January 14, 2019 13

SPONDYLOLISTHESIS Not ot more common, , but more likely to slip L4 L4-5, L5-S1 S1 Grades es 2-5 Posteri erior r fusio sion n only

BONY CHANGES Pedi dicles s thin in Lamin ina thin in Vertebra brae scallop loped

IIC. DURAL ECTASIA Present nt in over 50% of pati tients nts  LDS similar Visua sual criteri eria:  dural diameter S1> L4  scalloping > 4 mm  anterior meningocele What is Dura? • Tough tube protecting spinal cord • Contains clear fluid - CSF

HOW DOES DURAL ECTASIA DEVELOP? Spina nal Flui uid d pulsa sati tions ons Nl Dilata tati tion n of the weak dura ra Scalloping/ oping/er erosio osion n of the vertebra ral body DE Signa naling ng effects cts?

DOES DURAL ECTASIA CAUSE PAIN? YES, BUT… Case-co contr ntrol ol study udy 30-50 50 y/o  DE greater in pain group  But not all with DE had pain! Possi sible ble reason sons s for pain:  pressure on bone, soft tissues  folding of dura or nerve roots Treat atme ment? nt?

SIGNS/SYMPTOMS ASSOCIATED WITH DURAL ECTASIA Very Low Back pain n (91%) Headaches hes (77%) Leg pain n (64%) Pelvic c & Perine neal pain n (30%)

DOES DURAL ECTASIA GET WORSE? 11 yr follow up 12 pati tient nts  repeat MRI/CT/pain score  age 52y/o (42-60)

60Y/O WOMAN WITH SEVERE DE (ANTERIOR MENINGOCELE) 1998 2009

48Y/O MALE WITH MODERATE DE 1998 2009

RESULTS No st statisti tically ally si signi nifica icant nt differen erence ce in:  dural ectasia size noted after 11 yrs  Symptoms (pain scores) 12 10 8 1998-99 6 2009 4 2 0 L4 L5 S1 S2

IV. HIPS NEONATAL HIP DISLOCATION IN MARFAN Inciden idence ce abou out t 2 % Pa Pavlik Harness ss not ot success cessful Surprisi singl ngly good respon onse se (3/4) 4) to closed sed reducti ction on and hip p spica ca cast

HIPS “ PROTRUSIO ” Deep hip p socket et  Center-edge angle  Medial displacement Bone more plastic stic under er load  With growth  Mechanism:  Activation of osteoclasts? Are there re ot other r reasons ns for hip p degenera rati tion? n?

RESULTS- PROTRUSIO STUDY Pai Pain only y presen ent t in ~5% of 300 0 hips  Role of prophylactic surgery seems limited Few w peop ople le needed eded hip replacem acement ent Hip replacem acemen ent t has good od results sults

UNANSWERED QUESTIONS Mechani nism sm for osteo eope peni nia/o /ost steo eopor porosi osis Pathogene nesis sis of dysm smor orphi phic changes es Mechani nism/tre sm/treatm atment ent of  Skeletal pain,  fatigue,  disability