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

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• I. MAIN HEALTH CONCERNS IN MFS

Heart Sight ht Back Fatigue gue

Hernia

• Diff. Learning

Striae Ribs & Breast Bone Dural Ectasia

• Diff. Concentration

Lungs Depression Hip Feet Fatigue Spine & Back Sight Heart 8 7 6 5 4 3 2 1

Rao, Sponseller 2016

MAIN SOURCES OF SKELETAL PAIN

• FURTHER RESEARCH NEEDED ON THESE

Back Hip Neck Knee Shoulder der Feet et

Ankle Pain Elbow Pain Shoulder Pain Knee Pain Headache Neck Pain Hip Pain Back Pain 6 5 4 3 2 1

• II. SPINE ISSUES

Scoliosis

• sis

Kyphosi hosis Slippi pping ng (Spondyl ndyloli

• listhesis)

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

97° 3 years later Mild Scoliosis

Scoliosis

• sis affects

cts 2/3 of MF Does not

• t always

s worsen 10% need brace or surge rgery

Cur urves es increas rease e fa faster er Increase creased d back k pain

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

• rder

ders!! !!

BRACE RECOMMENDATIONS

Conside sider r brace at earlier er point nt

• 15-20o 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

• n arrests

ests trunk unk growth th Solution: “Growing” rods (internal brace)

GROWING ROD RESULTS IN MARFAN

10 Marfan fan childr ldren en

• 80o curves
• 10 cm length in 5 years
• No infections

MAGNETICALLY CONTROLLED GROWING RODS (“MAGEC”)

If No n need for MRI

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ADOLESCENT SCOLIOSIS SURGERY: MFS VERSUS IDIOPATHIC (TYPICAL) Multicen icenter er, Case-Cont

• ntrol
• l

34 Marfan pa patie ients 68 AIS pa patie ients

• Matched 1:2 for age, gender and degree of

major deformity

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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)

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SPONDYLOLISTHESIS

Not

• t 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

• ns

Dilata tati tion n of the weak dura ra Scalloping/

• ping/er

erosio

• sion

n of the vertebra ral body Signa naling ng effects cts?

Nl DE

DOES DURAL ECTASIA CAUSE PAIN? YES, BUT…

Case-co contr ntrol

• l 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

2009 1998

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)

2 4 6 8 10 12 L4 L5 S1 S2

1998-99 2009

• IV. HIPS

NEONATAL HIP DISLOCATION IN MARFAN

Inciden idence ce abou

• ut

t 2 % Pa Pavlik Harness ss not

• t success

cessful Surprisi singl ngly good respon

• nse

se (3/4) 4) to closed sed reducti ction

• n 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

• ther

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

• ple

le needed eded hip replacem acement ent Hip replacem acemen ent t has good

• d results

sults

UNANSWERED QUESTIONS

Mechani nism sm for osteo eope peni nia/o /ost steo eopor porosi

• sis

Pathogene nesis sis of dysm smor

• rphi

phic changes es Mechani nism/tre sm/treatm atment ent of

• Skeletal pain,
• fatigue,
• disability