Scheuermann’s Kyphosis:

Introduction to Problem and Recent Literature Review

Daniel M. Sciubba, MD

Professor of Neurosurgery, Oncology and Orthopaedic Surgery

Johns Hopkins University

Department of Neurological Surgery

Email: dsciubb1@jhmi.edu

Disclosure

• Research

– AANS, CNS, NASS, NREF, SRS, AOSpine, DOD & JHMI research grants

• Teaching

– Honoraria (AANS, AOSpine, Depuy, Medtronic, Globus, Stryker, Orthofix)

• Editorial Board– Journal of Neurosurgery, Spine

• Study Groups

– Spine Tumor Knowledge Forum (AOSpine)

– International Spine Study Group (ISSG)

• Consulting– Medtronic, Depuy-Synthes, Stryker, Nuvasive, Stryker

Kyphosis:

What is ‘normal’

kyphosis?

• T1 - T12 male & female < 40º

– Roaf (1960) : 20º - 40º

– Rocher (1965) : 35º

– Stagnara (1982) : 30º - 50º

– Oxborrow et al (SRS 1997) :15º- 45º

Holger Werfel Scheuermann

(1877-1960)

• Danish orthopedic surgeon who first

described the entity "Scheuermann's

Disease", relating to disabling back pain

caused by a juvenile spine kyphosis.

• Scheuermann correctly attributed this

"disease" to vertebral endplate deformities

which he believed were due to ossification

failures.

November 1, 2017 4

Kyphosis:

• Congenital

• Postural

• Scheuermann’s Disease

•http://www.back.com/causes-developmental-scheuermann.html

Postural Kyphosis

• Usually < 60°

• “round shoulders”

• “slouching”

• Flexible

• No wedging, no end plate irregularities

• Non-structural: complete reduction with hyperextension

(bending backwards)

Scheuermann’snormal

November 1, 2017 8

Scheuermann’s Kyphosis

• Scheuermann (1921):

– Male > Female ratio 7.3:1

– Prevalence 0.4-8% pop.

• Bradford (1973) : 1:2

• Halal (1978) and Findlay (1989):

– ? Autosomal dominant transmission

• Damborg (SRS 2004) – prevalence

– 2.8% M:F 3.6:2.1

– Monozygotic twins 2-3x greater

than Dizygotic twins

Pathological Process ?

• Thickened A.L.L (bowstring effect)

• Wedging of disc and vertebral body

• Altered endochondral ossification of end plate cartilage

• Decreased collagen & increased proteoglycan

Clinical Presentation

• Occurs when healthy, rectangular shaped vertebrae

become wedge shaped.

• Symptoms are similar to other types of kyphosis:

1. Severe back pain with sports and other activities

2. A rigid curve of the spine that gets worse when bending and only

partially corrects itself when standing

3. Difficulty standing erect

4. Chest pain or difficulty breathing caused by decreased lung

capacity

5. Tight hamstring muscles in the legs

6. Forward posture of the head and neck

November 1, 2017 11

Radiographic Findings

• Apex – usually between T7 & T9

• Negative sagittal balance

– C7 plumbline > 2cm posterior to sacral promontory

– Compensatory hyperlordosis of cervical & lumbar spine

– Lumbar spondylolithesis

• Sorenson criteria:

– > 5° anterior wedging

– At least 3 adjacent vertebral bodies

• (Drummond (2), Bradford (1))

– Schmorl’s nodes, irregular endplates, disc space narrowing

Natural History

• Benign course

• < 75° at maturity– No long-term difficulties

• > 75° at maturity– May seek medical attention due to cosmesis

and/or back pain

– Over compensation lumbar/cervical

• Physical signs & symptoms– prepubertal growth spurt

Natural History and Long-Term Follow-up

• 37-year follow-up study of untreated Scheuermann’s

disease was performed of 80 patients, compared to

a sample of the general Finnish population

(n=3,935).

– Scheuermann patients had a higher risk for back

pain and disability score.

– The degree of thoracic kyphosis was not

associated with back pain, quality of life, or

general health. (Ristolainen et al. Eur Spine J

2012).

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Treatment Options:

Non-operative:• Reassurance

• Bracing

Operative:• Posterior correction

• Anterior correction

• Combined correction

Non-operative Treatment:

• Observation: – Asymptomatic, non-progressive

deformity, recommended first for most patients

• Postural exercises:– Extension muscle strength; Prescribed

with brace treatment

• Serial Casting:– For rigid, larger curves

– Prior to initiating bracing treatment

• Bracing– Prerequisites: Adequate curve

flexibility, sufficient growth remaining, patient compliance, Sachs – 45-75 deg

Intensive Physical Therapy

Effect of Intensive Rehabilitation on Pain in Patients with

Scheuermann’s Disease

Weiss HR, Dieckmann J,Gerner HJ Stud Health Technol Inform

2002;88:254-257

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• 351 patients, from 17-21 years old with kyphotic angle 53-64o, underwent intensive PT.

• Pain was significant between sex and location of disease: – Numeric pain scale: 2.1 for females with thoracolumbar Scheuermann’s,

1.9 for thoracic (p<0.05)

– 1.4 for males with thoracolumbar Scheuermann’s, 1.8 for males with Scheuermann’s.

• Following intensive rehabilitation, there was pain reduction between 16-32% across all patients.

Bracing

• If curve progresses/severe curve - designed to hold spine

straight with shoulders pulled back and chin upright.

• Takes pressure off vertebrae, allowing growth of bony area in

front of vertebrae to catch up with growth in back

• Usually worn for 16-24 hours a day for one year. Only used in

patients who are still growing and is not effective for adults.

• Similar to bracing for scoliosis, but needs to extend up higher

proximally than for idiopathic scoliosis to control kyphosis.

(upper thoracic/over shoulders/neck base)

• Apex – T8 or above (Milwaukee); T9 or below (molded TL

space)

• Adolescents find bracing difficult - uncomfortable, hot, rigid,

unattractive, self-conscious. (Milwaukee brace)

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Bracing Outomes

• 50% correction should be expected with brace treatment

• Gradual loss of correction will occur when the use of the brace

is discontinued.

– 15° loss of correction at 18 months after brace therapy. Sachs, et al.,[26]

– poor success rate for brace treatment in patients in whom kyphosis was

greater than 75°.

• generally not indicated in the skeletally mature patient because

it does not alter the natural history of curvature progression.

November 1, 2017 19

Current Literature on Bracing

• 56 adolescents, mean age 14 y.o., with

thoracic Scheuermann’s kyphosis were

treated with the kyphologicTM brace.

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– The average Stagnara angle at presentation

was 55.6o and in-brace correction was 36% of

initial angle (p<0.001). Mean in-brace Stagnara

angle was 39o. (Weiss et al. Scoliosis 2009).

• A retrospective study was conducted of 120 patients, with different angles of

Scheuermann’s kyphosis, treated with Milwaukee brace and physiotherapy.

– Mean angle of kyphosis at presentation was 63o.

– Mean angle of kyphosis was 36.5o after treatment for all patietnts

– Mean improvement for patients <75o at presentation was 25.3o (p<0.001)

– Mean improvement for patients at or >75o at presentation was 26.7o

(p<0.001). (Etemadifar et al. J Craniovertebr Junction) Spine 2017).

Risk of Progression

• 197 patients with Scheuermann’s kyphosis were treated with

Milwaukee brace. (Bradford et al. JBJS 1975)

– 96% had progression pre-bracing

– 70% had progression after maturity

– In those that completed treatment for over 3 years (n=75),

40% had improvement in kyphosis and 45% had

improvement in vertebral wedging.

• Failures were for (1) patient cooperation; (2) dorsal vertebral wedging

greater than 10o in more than 1 vertebra; (3) the presence of skeletal

maturation; (4) initial dorsal kyphosis >65o.

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15 Year Old Girl With Kyphosis

64° 1/05 Brace 3/05 44° 3/06

Indications for Surgery

• Pain in kyphotic area (TL & L > Thoracic)

• Pain in compensatory cervical / lumbar lordosis

• Curves > 75º or rapidly progressing curves > 60º

• Altered respiratory function (>110º curves)• Stagnara 1982

Operative Treatment

Goals:• Reduction of thoracic hyperkyphosis

• Sagittal balance maintenance/ correction

• Pain relief

Options:• Anterior Alone

• Combined - Open

• Combined – Thoracoscopic

• Posterior Alone – Current Trend: Posterior alone

– Reserve ant. Release/ fusion for larger stiffer curves

Anterior Surgery Alone

Interbody structural

support• Femoral ring allograft

• Titanium surgical mesh and autograft

• Synthetic ramps/cages

• Instrumentation

Anterior - Scheuermann’s

• 28 patients

• Age 14 – 36

• Indications pain / cosmetic

deformity

• Minimum follow up 2 years

(range 2 – 6)

• Pre-op 88º (range 74º - 98º)

• Post-op 47º (range 46º - 50º)

• Final follow-up 48º - 55º

Gaines RW, Marks DS, Thompson AG, Ohlin A, Moller H, Min K, Finch G. SRS Annual meeting, Buenos Aires 2004

Follow-up

(2 yrs)

Posterior Surgery

• Bradford (1975), Kostuick (1990) loss of correction /pseudarthrosis

• Cotrel (1978), Hall (1990) use of more powerful compression systems reduced loss of correction

• Ponte (1985) routine re-exploration at 3 months reduced pseudarthrosis

• Ponte (1996) multi level closing wedge osteotomy

• Harms (1998) multi segmental pedicle screws

• Lenke (2005) multi level pedicle screw with posterior osteotomy

Lenke

Posterior Alone

• Kyphosis corrects to < 50° on hyperextension in skeletally immature

patients

• Elderly patient when the goal is pain relief and not deformity correction

• Potential posterior complications:

– loss of correction, pseudarthrosis, failure of instrumentation, neuro

deficit, excessive blood loss, infection, sagittal imbalance

Fusion Levels:Upper vertebral level of the kyphosis (T1 or T2)

First lordotic segment distally

Basic Construct

– Double-Rod Multisegment Hook Systems

• Two double level claws (pediculo-transverse) above

• Pedicle hook and two-laminar claw below

• All pedicle screws

– Correction achieved by:

• Cantilever mechanism

• Segmental compression following segmental posterior column shortening by multiple osteotomies

Posterior Alone

Posterior: T2-L2 and 9 SPO

Slide borrowed from Larry Lenke

Pre-opPost-op

Lim et al (2004):– Review of 23 consecutive

patients:• 20 pts underwent combined AP

fusion with ant release & posterior multisegmental instrumentation

• 3 pts underwent PSF with multisegmental instrumentation

– Kyphosis: 83°→ 46 °→ 51° at follow up

– Complications: 43% minor; 17% major; 0% life threatening

– Multisegmental instrumentation- safe & effective treatment

Combined: Open Technique

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Surgical Treatment of Scheuermann’s Kyphosis Using a Combined Antero-posterior Strategy and Pedicle Constructs: Efficacy,

Radiographic and Clinical Outcomes in 111 Cases

Koller at al Eur Spine J 2014

• Consecutive series of Scheuermann Kyphosis patients treated with anterior release and posterior fusion.

• 111 Patients, mean age 23 years old, were included for an average follow-up of 24 months.

• Average Cobb angle at fusion was 68o; 37o post-operatively. – Preoperative bending films were highly correlated with

postoperative and follow up Cobb angles (r=0.7, p<0.05)

– Increased junctional kyphosis angle (JKA + 1) was associated with a higher risk of revision surgery (p=0.049).

– Increased fused segments was negatively correlated with SRS-24 self-image scores (r=-0.5, p<0.05).

Herrera-Soto et al (2005):– Review of 19 skeletally mature

patients (17.4 yrs) having undergone video-assisted thoracoscopic anterior release combined with PSF

– 8.3 disc released anteriorly; 13 levels fused posteriorly

– Kyphosis: 84.8°→ 43.7 °→ 45.3° at follow up

– Complications: pleural effusions (2); pneumothorax (2); inferior hook out (1)

– Viable treatment option for more severe & rigid curves

Combined technique:

Thoracoscopic

Johnston et al (2005):• Retrospective review

• 27 patients with 24-56 months FU

– Group 1 (20 pts) → PSF only

– Group 2 (7 pts) → Ant. release & fusion of 5-7 apical segments

• No difference in sagittal plane measures nor correction (Group 1=53%, Group 2=46% correction)

• No difference in correction from immediate post op & fu

Posterior vs AP Combined

Lee et al (2005):

• Results of 18 posterior only pts to 21 AP combined pts (AP)

– Similar average age, pre-op kyphosis, PSF levels, SRS scores

• Kyphosis correction at FU

– 51.8% (PO) vs 38.5% (AP), p<0.001

• Complications → 0/18 pts (PO) vs 8/21 pts (AP)

• Posterior only achieved & maintained better correction & had less

complications

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Operative Management of Scheuermann’s Kyphosis in 78 Patients

Lonner et al. Spine 2007

• 78 patients with Scheuermann’s Kyphosis were included in this study.

• Mean age 16.7 years old.

– Overall mean preoperative Cobb kyphosis 78.8o; mean postoperative Cobb 51.4o.

• 42 underwent combined anteroposterior

– Mean preoperative Cobb 82.6o for this group; mean postoperative Cobb 55.8o

• 36 had posterior alone

– Mean preoperative Cobb 74.4o; mean postoperative Cobb 46.2o.

• Proximal and distal junction kyphosis >/=10o occurred in 25 (32.1%) and 4 (5.1%), respectively.

– Among PJK, the magnitude of junctional kyphosis correlated directly with pelvic incidence and indirectly with percent correction.

Posterior w/ SPO vs. AP

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Scheuermann’s Kyphosis: Comparison Between the Posterior

Approach Associated with Smith-Peterson Osteotomy and Combined

Anterior-Posterior Fusion

Temponi et al. Rev Bras Ortop 2011

• 28 patients split into two groups:

• Group 1: AP– Mean age 19 years, preoperative kyphosis 77.6o, postoperative kyphosis

35.8o, and average correction of 53.2%.

– Mean postop pain VAS=0.6

• Group 2: Posterior + SPO – Mean age 27.3 years, preoperative kyphosis 72.9o, postoperative

kyphosis 44.3o, and average correction 39.3%.

– Mean postop pain VAS=0.5

• Both techniques were adequate in treating SK.

• Deformity correction and pain was greater in AP

Surgical Controversy

• Comparison of Scheuermanns kyphosis correction posterior only pedicle screws versus anterior release and posterior hybrid

• Lee SS Lenke LG Bridwell KH et al SRS 2005

• ‘Better radiological correction with pedicle screws only, 38% implant complication rate with AP Hybrid BUT no clinical difference between the groups at 2 years’

• Operative management of Scheuermanns kyphosis in 78 patients• Newton PO, Sponseller P, Lenke LG, Crawford AH et al SRS 2005

• ‘Loss of correction was less with anterior / posterior BUT the degree of correction with posterior only screws was better’

• Surgical correction of sagittal plane deformity in Scheuermanns kyphosis

• Grevitt MP Mehdian SH Webb JK et al SRS 2001

• ‘Combined anterior and posterior surgery gave better overall correction and maintenance of correction than posterior pedicle screw alone’ (Stiffer curves – bend to >45º - AP / flexible curves – bending to <45º posterior only)

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Complications of Spinal Fusion for Scheuermann Kyphosis: A Report of the Scoliosis Research Society Morbidity and

Mortality Committee

Coe et al. Spine 2010

• Retrospective multicentre database study. 683 procedures involving spinal fusion for Scheuermann Kyphosis were included

• Mean patient age was 21 years. 73% were </= 19 years old.

• 338 (49%) were posterior spinal fusions (PSF)

• 73 (11%) were anterior spinal fusions (ASF)

• 272 (40%) same-day ASF+PSF

• 99 complications were reported (14%)

– Wound infection (3.8%)

– Acute neurologic complication rate (1.9%) including 4 spinal cord injuries (0.6%)

– Mortality rate was 0.6%

• Complications were more common among adults (22%) compared with pediatric patients (12%) (p=0.002)

• Overall incidence of complications did not differ significantly between the PSF (14.8%) and same-day ASF/PSF (16.9%) (p=0.5)

Junctional Kyphosis

• Complications of surgical management of Scheuermanns kyphosis

• Crawford AH, Morley TR et al SRS 1998

• ‘Post operative distal junctional kyphosis was reduced by including the first lordotic disc in the fusion’

• Operative management of Scheuermanns kyphosis in 78 patients

• Newton PO, Sponseller P, Lenke LG, Crawford AH et al SRS 2005

• ‘Proximal junctional kyphosis related to pelvic incidence’

• Proximal junctional kyphosis in patients following treatment for Scheuermanns kyphosis – what are the risks?

• Sun EC et al

• 27% PJK at 5 years was not related to magnitude of curve correction but to flavum or ligament disruption and UIV level

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Anterior Release for Scheuermann’s Disease: A Systematic Literature Review and Meta-analysis

Yun C, Shen CL. Eur Spine J 2017

• Systematic review for outcomes of anterior release and posterior fusion (AP), or posterior-only surgery (PO).

• 23 studies met inclusion criteria, with a total of 711 patients. Mean follow-up was 42.4 months.

• Pooled correction loss

– AP: 4.1 (3.4, 4.8)

– PO: 3.8 (3.3, 4.4)

• Median blood loss

– AP: 2156 ml (910-3150)

– PO: 823 ml (620-1086)

• Median operative time

– AP: 325 min (307-662)

– PO: 243 (141-378)

• PJK

– AP: 17.7% (0-31.3%)

– PO: 6.3% (0-16.7%)

• DJK

– AP: 21% (0-33.3%)

– PO: 6.4% (0-37.5%)

• Self-image improvement

– AP: 63.5% (31.3-100%)

– PO: 63.5% (31.3-100%)

• Pain relief

– AP: 70.7% (31.3-100%)

– PO: 51.7% (33.3-100%)

• Need for reoperation

– AP: 16.4% (6.7-40.9%)

– PO: 10.3% (0-22.7%)

• PO and AP had comparable correction loss. Meta-analysis regression demonstrated correction loss decreased over each decade—possibly due to improvements in instrumentation and surgical technique.

• PO group had advantages in blood loss, surgical time, PJK and DJK

Outcomes

The Natural History and long term follow-up of Scheuermanns kyphosis

Murray PM, Weinstein SL et al JBJS 75A 1993:236-248

• 67 patients with 32 years follow-up

• No difference in pain / ADL / Fatigue / Self esteem /

• employment / sick leave for back pain or sciatica /

• psychiatric disorders compared to controls

• The site and intensity of pain were different, they had jobs with less physical requirement compared to controls and had less extension

• Kyphosis of <100 had normal pulmonary function

Outcomes

• Long term follow-up of patients treated for Scheuermanns Kyphosis

• Soo C L, Esses S I SRS Ottowa 1996

• 63 pts.14 year FU. No difference between Observation

• Bracing / Surgery in any of the assessed domains

• Average increase in kyphosis of 22º in the surgical group at final FU (note Harrington posterior instrumentation only)

• 94% all patients, regardless of treatment regime, worked without restriction

• Patients treated by bracing or surgery reported that their self image was better however there was no radiological support for this

• Pts with >70° kyphosis had inferior functional results

Sagittal Stable Vertebrae (SSV) Method

Distal Fusion Level Selection in Scheuermann’s Kyphosis: A

Comparison of Lordotic Disc Segment Versus the Sagittal Stable

Vertebrae

Kim HJ et al Global Spine J 2017

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• 44 patients divided into 2 groups:

– Group 1 (n=26) had distal lowest instrumented vertebra (LIV) distal to or at the level of the

sagittal stable vertebrae (SSV)

– Group 2 (n=18) LIV was proximal to the SSV

• Mean follow-up 3.1 years, no difference in demographics between groups

• Group 1 had greater average lordotic disc angle below LIV compared to

Group 2 (p=0.02).

• Subgroup analysis demonstrated extending fusions to the SSV rather than

first lordotic disc resulted in fewer distal LIV complications requiring revision

vs. fusing proximal to SSV

Thank You