CONGENITAL MUSCULAR TORTICOLLIS US의 임상 적용

성균관의대

강북삼성병원 재활의학과

이 용택

강의내용

• CMT의 정의

• 임상상

• CMT에서의 US 임상적용

• 검사방법

• Case

Torticollis

• 1912, Tubby

“a deformity by lateral inclination of the head

to the shoulder, with torsion of the neck and

deviation of the face”

Latin word for twisted neck

• Sign of an underlying disease

process, not a specific diagnosis

• Congenital torticollis

• Ocular abnormalities

• Strabismus, congenital nystagmus

• Acquired torticollis in older

children

• Trauma, infection, tumor

• Torticollis in adults

• Neck muscle injury

• Muscle spasm

Torticollis

Congenital Muscular Torticollis

•1969, MacDonald

•SCM tumor (SMT): Mass (+)

•Muscular torticollis (MT): Mass (-), tightness (+)

•1950, Hulbert

•Postural torticollis (POST): Mass (-), tightness (-)

Fine needle aspiration Bx.

Reactive skeletal muscle cell (large arrows)

Spindled fibroblast (small arrows)

Pediatr Radiol 1998;28:230-233

Etiology • Unclear

• Intrauterine mechanical factor

• Intrauterne crowding: small uterus in the firstborn, breech,

oligohydroaminos

• Birth trauma Hx. : 30-60%

• Fibrosis from peripartum bleeding

• Breech presentation, forceps delivery, difficult delivery

• Occlusion of the venous outflow

• Compartment syndrome

Curr Opin Pediatr 2006;18:26-29

Clinical Features • MC congenital problems of

MSK in neonates & infants

• Incidence: 0.3-2.0%

• Clinical symptom

• Neck mass

• Shortened SCM & limitation of

ROM

• Ipsilateral head tilt

• Contralateral rotation of the face and

chin

• Associated Sx

• DDH

• Plagiocephaly

• Facial asymmetry

Curr Opin Pediatr 2006;18:26-29

Clinical Course • 50 -70 % tumor

• Spontaneously resolve during the first year of life

• Early & intensive treatment (Celayir et.al) • Good response

• No surgery, mean Tx duration 3.2 m

• Some cases • 10 %Persistent functional asymmetry

(Binder et al.)

• Craniofacial growth • Facial asymmetry

• Deformational plagiocephaly: 사두증

• Spine growth during childhood • Scoliosis

→ Surgical release

• Any limitation of rotation • Early physical therapy

• f/u every 3 month

Plagiocephaly

Diagnosis

• Clinical Dx. • Hx.

• Palpation of neck mass

• Limited neck ROM

• Imaging study for DDx. • Nonmuscular causes > 18% of the torticollis

• US

Curr Opin Pediatr 2006;18:26-29

Clinical Features

• 821 pts. with CMT (< 1 yr)

• Δ passive rotation > 10° • Standardized measure of PROM

• Standardized manual stretching program

• 3 times a week

Outcome of Manual Stretching

Treatment

• Manual stretching

• Safe and effective < 1yr

• Prognosis factor

• Patients’ age

• Initial rotational deficit

• Surgery

• Recommend for resistant

cases after 6 m manual

stretching

JBJS Am 2001;83:679-687

Findings of US

• 36 CMT patients (< 6 mo) • Echo-texture

• Hyperechoic

• Heterogeneous (49%) vs. homogeneous (51%)

• Hypo-echoic rim

• Well-defined margin of the mass

• Sternal portion (57%) vs. clavicular portion (0%)

• Sternal & clavicular portion (43%)

Pediatr Radiol 1992;22:356-360

J Pediatr Surg 1997; 32: 1648-1651

Extent of fibrosis on US vs. Prognosis

• 197 CMT (1mo ~ 16yr)

• 362 US exam • Sternal (24%), Sternal & clavicle (76%)

• clavicular portion (0%)

• Transverse scan • Homogeneous hyperechoic lesion (36.7 %)

• Heterogeneous, hyper & isoechoic (63.3%)

• Fibrosis L/M ratio • OP (63%) vs. Non-op (55%), p < 0.05

• Longitudinal scan • 32 operative group

• 81.3 % fibrotic lesion in the entire length of muscle

• 256 CMT pts ( 9d~16y)

• 4 Type according to extent of

fibrosis

• Average f/u: 6 mo

• Op

• Prominent clinical feature after

physiotherapy for 6 mo

• Age > 1 yr

Arch Phys Med Rehabil 1999; 80: 637-641

Extent of fibrosis on US vs. Prognosis

Normal

Type 1

Type 3

Type 4

Arch Phys Med Rehabil 1999; 80: 637-641

Type 2

Arch Phys Med Rehabil 1999; 80: 637-641

Type 1 Type 2 Type 3 and 4

Ultrasound Med Biol 2000; 26: 1237-1241

US findings vs. Clinical Severity

• 436 CMT pts. (< 1yr) • US before any treatment

• SMT, MT, POST group

• 4 groups according to rotation limitation

Clin Orthop Relat Res2002; 403: 179-186

Longitudinal F/U of Extent of fibrosis

• 73 Pts (1mo~ 11yr)

• Type 1~4

• Mean 8 mo F/U

Irreversible !!

J Pediatr Orthop 2005; 25: 812-814

Extent of fibrosis on US vs. Prognosis

• 26 CMT Pts with palpable neck mass (1 w~ 16 w)

• Type 1~2

• All vaginal delivery (4 traumatic) • Complete recovery of torticollis 1-6 w after Dx. (mean 2.8 w)

• Palpable mass disappearance 2-8.5 w (mean 3.5 w)

• US mass disappearance 3-10 w (mean 5.5 w)

• F/U after 6 months • Clinical and US abnormality (-)

• OP (-)

Normal Type 1 Type 2 Type 3

Extent of fibrosis on US vs. Etiology

• 67 CMT pts (< 3 m) • palpable neck mass

• Δ passive rotation > 10°

• Type 1~3, Type 4 (-)

J Pediatr Surg 2011;46:514-519

J Pediatr Surg 2011;46:514-519

• 21 breech presentation + C-sec

• *OR (95% CI): 6.393 (1.398-29.225)

• P = 0.017

J Pediatr Surg 2011;46:1526-1531

Extent of fibrosis on US vs. PTx. Duration

• 50 CMT pts (< 3 m.) • palpable neck mass

• Δ passive rotation > 10°

• Type 1~3, Type 4 (-)

• Standardized manual stretching • Treatment failure

• ᇫrotation > 6° at 10 m.

• No improvement after 6 m.

• Success rate: 98 %

The Clinical Features and Outcome of Sono-negative Congenital Torticollis with Limitation of Passive Range of Motion

An Ju-ha, MD, Yong-Taek Lee*, MD, Soo Kyoung Cho, MD

Number of cases

ᇫRotatio

n (°)

ᇫLateral

Flex (°) Age at

presentation (month)

Duration of Treatment

(month)

23 9.13 (0~20)

13.26 (0~25)

4.23 (1.2~6.0)

1.13 (0.5~2.0)

• 23 Pts with normal US (< 6 mo )

• Δ Passive rotation or Δ lateral flexion > 10 ̊

Normal US vs. Prognosis

2009 대한재활의학회 추계학술대회

AJNR 2013; www.ajnr.org

US Thickness Ratio vs. ROM

• 48 CMT pts (mean 3.9m)

• Standardized manual stretching • Clinical score

• ᇫrotation &ᇫlateral flexion

• SCM thickness ratio • Excellent intra- &

interobserver agreement

• Most correlated w clinical improvement

• Overcome of false-positive aggravation

Methods of US

• Position

• Supine with small pillow placed under the shoulders

• Head slightly rotated to the opposite side

• Equipment

• 5-13 MHz real-time linear-array transducer

• Transverse scan

• Perpendicular to the long axis of the muscle

• Origin (clavicle and sternal head), Belly, Insertion (mastoid process)

• Maximal A-P diameter

• Longitudinal scan

Methods of US

• Location

• Sternal or clavicular head

• Lower, middle or upper thirds

• Internal echo-texture

• Homogeneous/Heterogeneous

• Hyperechoic/isoechoic/hypoechoic

• Margin of mass

• Definable/poorly visualized

• Both side

• Normal: the findings were similar to the uninvolved SCM

SCM

US Findings

CASE 1: 고 OO 8y/M

CASE 2: 5y/M

• GA 40+6자, 3543gm, vacuum delivery

• 출생 시부터 오른쪽으로 고개 기운 증상으로 외부병원 내원하여 US • Neck mass 1.75 cm/0.5 cm

• TYPE 3

• 생후 80일 경 재활치료 위해 본원 내원 • Neck mass 3.5 cm/0.5 cm,

• Plagiocephaly (-)

• Rotation 100/45, lateral flexion 50/20

• PTx 6개월 지속

• 생후 9개월 • Neck mass 2 cm/0.5 cm

• Rotation 100/70, lateral flexion 60/25

• Op 의뢰

• 생후 13개월 • Neck mass 1.7 cm/0.7 cm

• Rotation 100/90, lateral flexion 50/45

• 증상 갑자기 좋아져 observation 하기로 함

• 4세 증상 유지 중

• 5세 RT SCM muscle이 도드라져 내원함.

CASE 2: 최 OO 5y/M

CASE 2: 최 OO 5y/M

CASE 3 장 OO 2mo/M • Rotation 95/65,

lateral flexion 60/40

• 4개월 PTx

• Rotation 95/90, lateral flexion 50/45

“Take Home Message”

• CMT는 SCM의 involvement와 LOM이 증명되어야 함.

• US는 P/E의 보완 및 CMT의 DDx.를 위해 꼭 필요함.

• 초음파 상의 Fibrosis의 정도가 임상상을 반영하므로 F/U에

적용할 수 있다.

• Thickness ratio, Fibrosis의 정도.

• 향후 fibrosis의 정도를 객관적으로 측정할 수 있는 방법에

대한 연구 필요.

• Fibrosis의 정도와 임상양상과의 관계에 대한 연구가 더 필요함.

THE END 경청해 주셔서 감사합니다^^