Congenital Muscular Torticollis

An Overview

Introduction

▪ Congenital Muscular Torticollis (CMT) is a congenital deformity characterized by unilateral shortening of the sternocleidomastoid muscle resulting in lateral inclination of the neck associated with contralateral torsion

▪ It is a relatively common recognized infantile abnormality and its incidence varies from 0.3% to 2.0% live births

▪ CMT is recorded as is the third most common congenital musculoskeletal anomaly after dislocation of the hip and clubfoot

▪ CMT is often associated with other congenital deformities such as(DDH) with a coexistence rate estimated as high as 14.9%

▪ Other coincident lesions less frequently recorded include tibial torsion, clubfoot, calcaneovalgus foot, flexible pes planus, metatarsus adductus, and hallux valgus

▪ If torticollis persists, patient will develop scoliosis and the facial/head asymmetry known as plagiocephaly.

Aetiology

▪ Postulated that fetal position abnormalities, intrauterine or perinatal compartment syndrome and birth trauma ensuing a difficult delivery embody the main causes

▪ Other possible causes encountered are hereditary and venous or arterial occlusion which may create fibrous tissue within the sternocleidomastoid

▪ Other possible causes encountered are hereditary and venous or arterial occlusion which may create fibrous tissue within the sternocleidomastoid

Diagnosis

▪ Diagnosis is based mainly on past medical history and clinical examination of the infant

▪ A meticulous prenatal history record is essential and detects complicated labor and the coexistence of previous birth trauma such as clavicular fracture.

▪ The presence of perinatal asphyxia, jaundice seizures, medication, gastroesophageal reflux disease (GERD) or Sandifer’s syndrome are also recorded

▪ Children with CMT can be assigned to one of three groups

1.Children with a palpable swelling or pseudotumorof the sternocleidomastoid;

2. Children with SCMtightness but no tumor;

3. Children with all features

▪ Associated congenital musculoskeletal conditions i.e. hip dysplasiais also investigated.

▪ Ophthalmological examination may reveal extra ocular muscle imbalance as the causing factor of torticollis

▪ Ultrasonographic imaging is a useful diagnostic tool with important diagnostic and prognostic application.with high sensitivity and specificity of 95.83% and 83.33%, respectively.

▪ Magnetic resonance imaging (MRI) is a modern radiologic examination with increasing role in CMT diagnosis that have been found to be correlated with histopathological findings [19].

Differential Diagnosis

Klippel –fail syndrome, grisel syndrome

Neurological and psychiatric causes of torticollis

Unilateral hearing difficulty which results in otogenic torticollis,

Neoplasms

Infections and systematic diseases such as rheumatoid arthritis

Management▪

▪ Manual passive stretching of the sternocleidomastoid muscle before the age of 12 months is the most effective mode of physical therapy

▪ A Program of gentle stretching exercise should include flexion extension, lateral bending away from involved side and rotation toward it

▪ Streching exercise shoud be continued until full neck rotation achieved

▪ Cervical orthosis may be an adjunct and support for children whose lateral head tilt dosesn’t resolve with exercise or older children wiyh no longer tolerate strecthing

Non Operative Management

▪ Botulinum toxin (Botox) could enhance the effectiveness of stretching on the side of the contracture and allow strengthening of overstretched and weakened muscles on the opposite side of the neck. 

▪ This method is safe and effective in children and adolescents with cerebral palsy especially in ambulatory patients

Operative Management

▪ Surgical release may be considered in children older than 12-18 months of age with CMT resistant to conservative treatment or in case of facial asymmetry and plagiocephaly development

▪ Surgical techniques to lengthen tight SCMs include unipolar release, bipolar release,endoscopic release,and subperiosteal lengthening.

▪ Surgical lengthening of the contracted SCM is mandatory in only 3% of the cases .

▪ Surgery is highly recommended when a restriction of movement up to thirty degrees is present, as well in cases complicated with deformities of facial bones

▪ A potential complication of the surgical approach is an injury of the accessory nerve . The rate of relapse is up to 1.2%.

▪ The optimal time for surgical intervention is referred between 1 and 4 years although favourable results have been also described for patients 10 years or older at the time of surgery .

▪ Surgical techniques to lengthen tight SCMs include unipolar release, bipolar release, endoscopic release,and subperiosteal lengthening.

▪ For aged more than 6 years old, recommend bipolar release .

TECHNIQUE

▪ Cervical spine should be reviewed– bony anomalies or cervical scoliosis

▪ In fixed deformities, positioning of the head can be difficult for anestesiologist. Flexible fiberoptic intubation shoud be considered

▪ The ear taped anteriorly and hair around the mastoid process is shaved

Pre Operative Planning

Positioning

▪ Supine postion , General anestesia

▪ Sanbag placed to elevate the shoulder on the affected side

▪ Draping should permit correction to be evaluated by bending the neckthe neck is bent toward the unaffected side and the head rotated to affected side - the SCM muscle kept under tension and the origin and insertion can be clearly identified

Incission and dissection

▪ For release the distal pole SCM, Tranverse incision 3-4long incision 1 cm superior the clavicle and the two head the SCM Muscle

▪ The subcutaneus tissue and platysma muscle are divided inthe lline incission and the tendon sheats of clavicular and sternal head exposed

▪ For proximal pole exposure , 2-3 cm horizontal incisision is made just distal the tip of mastoid process

▪ The dissection is carried deeper until the periosteum of mastoid process exposed. Inserton of muscle exposed subperiosteally

Distal Unipolar Release

▪ Release the sternal , some times clavicular head the SCM Muscle

▪ A Transverse Incission placed pararelly zand 1 cm proximal to the clavicle between calvicular and strenal head of the SCM

▪ An Incission that overlies ove rthe clavicle –hipertrofic sccar,A higher –jeopardize jugular vein

▪ Two head OF SCM Identified. Surrounding fascia is cleared and strenal head or both head is undermined with curved clamp

▪ The Muscle are elevated with the help of clamp and divide with cautery. Altenatively sternal head can be lengthened by Z Plasty

▪ About 5 -10 mm muscle tendon excised to prevent contracture and fibrous formation.

▪ Check bending neck kontralateral and rotating lareal side while palpating area with finger tip to identified remaining tight

Bipolar Release

▪ Bipolar relaese include the relaese of the mastoid isertion of SCM Muscle along with the distal released

▪ The procedure start with a distal incision

▪ The insertion of the muscle is identified anteriorly and posterorly

▪ Dissection starts subperiosteally from mastoid processus to avoid facial nerve anteriorly and the anterior branch of the great auricular nerveinferiorly

▪ Release the clavicular head with lengtheneing of the sternal head by z plasty may approprate in older children ti provide simetrical aapearence post operative

Post operative Care

▪ Immobilization the head and neck a slightly over corrected postion with thermoplastic custom made brace or pinless for 3 weeks

▪ The Brace is removed 3 weeks and passive stretching is recommeded as well as active strengthening exercise

▪ Exercised continued for 3-6 weeks

Out come

▪ Early conservative mangement succesful in over 90 % children with CMT who are younger than 1 year

▪ In resistenat cases –still controversy

▪ Cheng et al –excellent result operatedon at age 6 monts to 2 years with nipolar release

▪ Canale et al better with bipolar release, although the difference not significan

▪ Wirth et al reported satisfactory result in 48 of 55 patient with bipolar relaese with low reccurance

Complication

▪ Wound Breakdown

▪ Hematoma

▪ Residual lateral band

▪ Neurovascular damage

▪ Hypertohic Scar

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