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Torticolis

머리가 한 쪽으로 기울어져 있는 상태.
진단이 아니며 증상이다.
대부분은 1주 내 회복되는 근성 사경(muscular torticolis) 이다.
후인두농양 등의 급성 감염, 경추손상, 뇌종양 등의 질환 감별이 필요하다.

A. 정의

Torticolis
"Torus (twisted)" + "Collum (neck)"
머리 : 한 쪽으로 tilting
턱 : 머리와 반대쪽으로 돌아감.
선천성과 후천성으로 나뉨

B. Pathophysiology

B1. Congenital 

SCM의 muscular fibrosis (대개)
신경학적 또는 골격이상 (덜 흔함)
Facail assymmetry +

B2. acquired 

SCM또는 Trapezius의 손상 또는 염증
경추부 근육 sapsm 또는 cervical muscle irritation

C. 소아에서 후천성 사경의 감별진단

Trauma
Infection
C1-C2 subluxation C1-C2 dilsocation Clavicel Fracture Cervical spine Fx. Muscle injury Ligamentous injury Spinal epidural hematoma
URI  Cervical adenitis  Pharyngitis  Retropharyngeal abscess  Upper lobe pneumonia  Cervical osteomyelitis  Cervical diskitis  Tuberculosis  Lemierre SD
Tumors
Ocular
Post. Fossa Intraspinal Osteoid osteoma Eosinphillic granuloma
Spuerior oblique palsy  Congenical nystagmus  Refractive errors  Strabismus
Neurologic
Miscellaneous
MG Poliomyelitis Dystonia Kernicterus Huntington Chorea Wilson Dz Neuritis of spinal accessory nerve
Myositis or fibromyositis  Gastroesophageal refulx  Jevenile idiopathic arthritis  Spasmus nutans  Benign paroxysmal torticolis  Drug-induced  Ligamentous laxity  Functional torticolis

D. Life-threatening causes

Retropeitoneal abscess
Suppurative jugular thrombophlebitis (Lemierre SD)
Cervical spine injury
Spinal epidural hematoma
CNS tumor

D1. Retropahryngeal abscess

Age : 2~4 yrs (m/c)
경부신전제한 + 동공 상부편위
Fever, irritability, dsyphagia, drooling, odynophagia, resp. distress (stridor/tachypnea)

D2. Suppurative Jugular Thrombophlebitis (Lemierre;s SD)

인두염의 합병증
Fusobacterium necrophorum
인두염 선행 --> 지속적인 발열 & 항생제 투여에도 증상 악화 및 경부 부종 시 의심!
발열 39도 이상, Rigors, Resp distress 동반
국소적 경부, 인두부 통증
SCM 의 자극 --> 사경 유발
Neck CT/E 필요함.

D3. Cervical spine injury

8세 미만에서는 드물다.
높은 운동에너지가 가해진 메커니즘
8세 미만에서는 전형적으로 상부 경추를 침범함.
8세 이상에서는 성인처럼 하부 경추가 취약함
징후 : 경부 통증, 압통, 사경, stiffness
다수는 신경학적 이상이 없다.

D4. Spinal epidural hematoma

드물지만 경미한 손상으로도 발생이 가능하다.
자연발생하는 경우도 있다.
갑작스럽고 설명할 수 없는 경부 통증, 사경 + 사지 위약 또는 기타 신경학적 이상

D5. CNS tumor

Posterior fossa의 종양
구토, 두통, ataxia, vision disturbance, papiledema, cranial nerve deficit
Diplopia를 교정하기 위해 머리를 기울이려는 시도함.
Accesory nerve 자극 시 neck stiffness 발생

E. 흔한 원인들

Muscle injury or inflammation
Acute infections
Atalnto-axial rotatory subluxation (AARS)

E1. 근성 사경 (muscular torticolis)

소아 후천성 사경의 가장 흔한 원인
SCM이나 Trapezius 의 손상이나 염증이 원인
경미한 손상, viral illness 또는 특정 Hx. 없이도 발생하는 경우 있음.
갑작스럽게 발생 - 자고 일어나서 종종 발견됨.
사경 + 경부 통증 & distress
SCM/Trapezius 에 국소 압통
C-spine에 ROM 제한
인두, 경부 임파선, CNS, C-spine XR : 정상
전형적으로 1주 이내 보존적 치료로 호전됨.

E2. Acute infection

급성 감염이 compensatory muscle spasm 과 동반되면 발생
소아 후천성 사경의 두 번째로 흔한 원인임.
Viral or Stretococcal pharyngitis, cervical lumphadenitis, parapharyngeal abscess, viral URI 등으로 발생
Osteomyelitis, promyositis, diskitis, uberculosis, RUL pn. 도 가능함.

E3. Atlantaxial rotatory subluxation (AARS)

C1과 C2의 회전성 아탈구
Retropharyngeal edema -> laxity of ligaments & capsular structures -> rotatory deformity
비외상성 AARS : Grisel's syndrome involves the subluxation of the atlantoaxial joint from inflammatory ligamentous laxity following an infectious process.
Age : 6~12 (m/c)
경증 외상, 인두 수술, 경부의 염증(SCM의 연축 유발), URI
통증, 사경, Neck ROM제한
Axis의 spinous process에 압통 있을 수도
C2 nerve 지배 부위(한쪽 후두부) 통증 호소할 수도
경추 고정 조치 및 C-spine XR검사 필요
불안정성과 관련된 상황
다운 증후군, Morquio 증후군, Larsen 증후군
말판 증후군, 경추 관절염
XR에서 C1에 비해 C2 odontoid process가 비대칭
Open mouth view에서 가장 잘 보이나, 촬영이 힘듬.
1주 이내의 급성기, 경증인 경우
--> Soft neck collor, rest, analgesics
심한 경우
--> Cervial traction, immobilization, surgical intervention

F. Other causes

Ocular torticolis
Spasmus nutans
Benign paroxysmal torticolis
Dystonic reaction
Sandifer SD

F.1. Ocular torticolis

Strabismus
Tilt head to avoid diplopia

F.2. Spasmus nutans

대개 6개월 ~12개월에서 발생
Nystagmus, head nodding, torticolis

F.3. Benign paroxysmal torticolis

반복적인 머리 기울임
구토, 창백, irritability, ataxia, drowsiness와 동반
출생 수 개월 사이에 대개 발생
좌 우를 랜덤하게 바뀜
Seizure와 유사하거나 posterior fossa tumor와 감별 - EEG, 신경영상검사

F.4. Dystonic reaction

Phentothiazines, Carbamazepine, Phenytoin
Acute spasm of muscle groups
: Fixed uuper gaze, neck twisting, grimace, clenced jaw, 말하기 어려움
치료 : diphenhydramine or diazepam

F.5. Sandifer SD

GERD가 목의 연축과 비정상 자세와 동반된 경우
간헐적으로, 좌우 방향 변경
치료: antireflux 치료

G. 증상 및 접근방법

G.1. urgent evaluation

심각한 경추 손상 - 즉시 경추고정 + 신경학적 검사
발열, 침흘림, 협착음 - Retropharyngeal abscess
두통, 구토, 신경학적 이상, Ataxis -> 후두와 뇌종양 또는 spinal EDH
발열, 인두염, 빈호흡, IJV 부위 압통 -> suppurative jugular thrombophlebitis