Torticollis cap 60.pdf

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CHAPTER 60

TorticollisSpencer W. Beasley

There are many causes of torticollis in childhood (Table 60-1),but most are rare. The most common cause of torticollis is

tightness and shortening of one sternomastoid muscle, a con-dition that occurs in about 0.4% of all births. Typically, atabout 3 weeks of age, a visible or palpable swelling developsin part or all of the muscle; this swelling is calleda sternomas-toid tumor. It affectsthe right side in about 60%,1 is bilateral in2%to 8%,2,3 and often persists for up to 1 year. Olderchildrenmay present with a fibrotic, shortened sternomastoid muscle,which is presumed in many to be the legacy of a previouslyunrecognized sternomastoid tumor.

History

Alexander the Great may have had torticollis, according toPlutarch.4 Antyllus is said to have performed tenotomies in350 AD, but the first authenticated division of the sternoclei-domastoid was by Minnus in Amsterdam in 1641.5 A sterno-cleidomastoid tumor was described by Heusinger in 1826.6

Torticollis was also a subject of interest to Dupuytren.7

Etiology

Although often referred to as congenital torticollis, the ster-nomastoid mass and the torticollis are rarely noticeable atbirth. Little is known about the etiology of sternomastoid

fibrosis, although several theories have been put forward toexplain the condition. It may be due to an idiopathic intrauter-ine embryopathy8 or could be the manifestation of an intra-uterine positional disorder producing sternocleidomastoidcompartment syndrome.9 The high incidence of obstetric diffi-culties, such as breechpresentation andthe need for assistedde-livery,10,11 may be the result rather than the cause of theshortened sternomastoid muscle, as was initially thought. Thereis no report of a sternomastoid tumor detected by antenatal

ultrasonography.12 Concomitant hip dysplasia is common.10

Pathology

The basic abnormality on histology is fibrous replacement ofmuscle bundles.13 The lesion, called fibromatosis colli, is of-ten classified with other types of fibromatoses, such as theDupuytren contracture and plantar fibromatosis. Jones8 hasdescribed endomysial fibrosis involving the deposition of col-lagen and fibroblasts around individual muscle fibers that un-dergo atrophy. The sarcoplasmic nuclei are compacted to formgiant cells that appear to be multinucleated. The maturity ofthe fibrous tissue i n neonates suggests that the disease may be-

gin before birth8,14,15 and may therefore contribute to the fre-quency of obstetric difficulties during delivery. Thereportedincidence of breech deliveries is about 20% to 30%16muchhigher than the normal incidence. About 60% of affected in-fants are involved in a complicated birth,16 which suggeststhat the fibrosis may affect the position of the fetus in uteroand perhaps even prevent normal engagement of the headin the maternal pelvis.

The natural history of untreated sternomastoid fibrosis iscomplete resolution in 50% to 70% of patients at 6 monthsof age. In about 10%, the tumor and sternomastoid shorteningpersist beyond 12 months of age.2,17 The severity and distri-bution of the fibrosis within the sternomastoid muscle is var-iable and has led to a variety of classifications based either on a

palpable localized sternomastoid tumor or thickening andshortening of the whole muscle or on the basis of ultrasono-graphic findings.18,19 The systems of classification have someprognostic significance in that localized lesions within thesternomastoid (clinically or ultrasonographically) are morelikely to resolve spontaneously than those involving the wholemuscle. In older children with torticollis, the appearance ofdegenerating fibers is more consistent with disuse atrophyproduced by limitation of movement caused by the fibrosis.

Clinical Features

STERNOMASTOID TORTICOLLIS

The tumor is a hard, spindle-shaped, painless, discrete swell-ing usually about 1 to 3 cm in diameter within the substance ofone sternomastoid muscle. Almost always, it first becomes ev-ident at about 3 weeks after birth. Obvious head tilt or torti-collis tends to develop later.12 In infants, the head is rotated tothe side opposite the tumor, with only slight flexion of thehead to the affected side (Fig. 60-1).

In other patients, the sternomastoid tumor is less discrete,and the sternomastoid appears to be thickened and tight-ened along its whole length. The shortening of the musclerestricts rotation and lateral flexion of the head (Fig. 60-2)

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The rotational component of the action of the sternomas-toid is easy to measure. It is assessed by standing behindthe childs head and passively rotating the head while itis held between both hands. The sternomastoid muscle isstretched to its maximum length by rotation to the sideof the affected muscle. Where the muscle is fibrotic, it can-not be stretched to its full length, and rotation to the ipsi-lateral side is restricted.

Older children withtorticolliscompensate forthe more pro-nounced tilt by elevating one shoulder to enable the eyes tokeep as level as possible (Fig. 60-3). Such compensation isnot seen in infants, because there is no need for them to main-

taintheireyes in a horizontal plane until they stand up.

20

More-over, older children do not turn their heads to the contralateralside as much, because they tend to compensate by twisting theneck and back to keep their eyes pointing forward.

DIFFERENTIAL DIAGNOSIS

Initial clinical assessment must establish whether the wry neckis caused by shortness of one sternomastoid muscle or bysome other condition. In sternomastoid fibrosis, the anteriorborder of the muscle stands out as a tight band, although insome small infants in whom the neck is relatively short, themuscle may be difficult to see readily. For this reason, the fulllength of the muscle must be palpated to determine whetherthere is an area of thickening or fibrosis along part or all of itslength. In about two thirds, there is a definite localized swell-ing (tumor) in the muscle; in the remainder, the whole muscleappears to be affected. There is no role for plain radiographywhere the sternomastoid is tight or shortened.21Although notrequired for diagnosis, appearance on ultrasonography mayhelp predict(to a degree) the likelihood of spontaneous reso-lution.1,19,22 Inexperienced ultrasonographers, worried by theinfiltrative and ill-defined appearance, may recommend

TABLE 60-1

Causes of Torticollis in Infants and Children

Cause Comment

Sternomastoid tumor Common; appears at 3 weeks of age

Abnormal position in utero Tends to improve with age

Cervical hemivertebrae Structural; confirmed on plain radiograph

Cervical lymphadenitis/abscess Acute; usually occurs in first 2 years of life

Retropharyngeal abscess31,58 and pyogenic cervical spondylitis59 Acute; signs of toxicity, cervical pain

Posterior fossa tumors61 A rare cause; headaches, vomiting, and other neurologic signs present61

Acute atlantoaxial subluxation May occur after tonsillectomy30

Atlantoaxial rotatory subluxation Significance disputed58,59; diagnosed on dynamic CT

Spasmodic with Sandifer syndrome59 Due to gastroesophageal reflux

Congenital absence of sternomastoid Unilateral, extremely rare60

Postural Familial

CT, computed tomography.

Neutral position Limited rangeNormal range

FIGURE 60-2 Restriction of rotation of the head secondary to shortening of the sternomastoid muscle as viewed from above the head. The black barsrepresent the right sternomastoid muscle and show that its inability to lengthen limits rotation to that side.

FIGURE 60-1 Appearance of a right sternomastoid tumor in infancy; thehead is turned to the contralateral side.

764 PART V HEAD AND NECK
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further imaging or biopsy of the tumor; this is not indicatedin the presence of the typical palpable mass within the sterno-mastoid and torticollis. It is possible to diagnose a sternomas-toid torticollis on magnetic resonance imaging (MRI)22,23 andcomputed tomography (CT),24 but neither alters managementand should not be performed routinely.23,25

An obvious mass or fibrosis of the muscle may not alwaysbe noticeable in idiopathic torticollis, but in such instances,alternative diagnoses must be sought (see Table 60-1 andFig. 60-4).20,26

A squint may cause head tilt from imbalance in rotation ofthe eyes. The squint may not be obvious at first because the tiltcompensates for the abnormal position of the eyes. When thehead is straightened passively, the squint becomes apparent.Occasionally, sternomastoid fibrosis may occur coincidentallywith ocular torticollis.

Posterior fossa tumors may compress the brainstem at theforamen magnum and produce acute stiffness of the neck thatcauses it to be held to one side. The neck is frozen in this po-sition and is difficult to move actively or passively. The pres-ence of a central nervous system tumor may be known already,but occasionally, acute torticollis is the first manifestation.Careful neurologic examination may show abnormalities ofthe lower cranial nerves and cerebellar function, and the caus-ative lesion is demonstrated on CT or MRI.

Hemivertebrae involving the cervical spine may produce atilt of the head that is evident from birth and does not pro-gress. Vertebral lesions can be identified clinically by inspec-tion and palpation of the dorsal cervical spines and confirmedon plain radiographs of the neck.

Acute torticollis has been attributed to atlantoaxial rotatorysubluxation as determined on dynamic CT,27,28 but othersdoubt the existence or significance of these findings and sug-gest that CTscans are notnecessary at the initialexamination.29

Atlantoaxial subluxation has been reported after tonsillec-tomy.30 Acute torticollis can also result from inflammatoryconditions of the neck, including retropharyngeal abscess,31

and can be a symptom of acute lymphoblastic leukemia.32

Secondary Effects of Torticollis

Table 60-2lists the secondary effects of torticollis.

FIGURE 60-3 Appearance of torticollis as a result of sternomastoid fibro-sis in an older child. The eyes are kept horizontal, but the shortened ster-nomastoid muscle causes compensatory elevation of the shoulder. (FromBeasley SW, et al: Pediatric Diagnosis. London, Chapman & Hall, 1994, withpermission.)

FIGURE 60-4 Torticollis caused by atlanto-occipital subluxation aftetonsillectomy. Notice that there is no tightness of the sternomastoid mus-cle on either side. (From Beasley SW, et al: Pediatric Diagnosis. Chapman &Hall, London, 1994, with permission.)

TABLE 60-2

Secondary Effects of Torticollis

Infants Plagiocephaly

Hemifacial hypoplasia

Older children Compensatory scoliosis

765CHAPTER 60 TORTICOLLIS
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PLAGIOCEPHALY

In small infants with torticollis and fixed rotation of the head,gravity deforms the relatively soft head as it lies in the same po-sition for a prolonged period. Flattening of one occiput leads to

secondary flattening of the contralateral forehead (Fig. 60-5).This asymmetric skull deformity is called plagiocephaly and de-velops in the first few months of life.33 It is best observed fromabove the head. Once the child begins to sit up or the torticollisresolves, the plagiocephaly tends to resolveas well.34 Itmaytakeseveral years to disappear, and a few children have a slightpermanent deformity. It is possible that many children with pla-giocephaly have had unrecognized torticollis during infancy.35

HEMIFACIAL HYPOPLASIA

Progressive facial deformity is seen when one sternomastoidmuscle immobilizesthe face for a longtime.The malareminenceon the side of the face limited by the fibrotic muscle grows moreslowly than the normal side does9 and causes progressive asym-metry (Fig.60-6). This inhibition of growth of the mandible and

maxilla embodies an important principle of pediatrics: Normalgrowth of bones depends on normal muscular movement.

The degree of hypoplasia of one side of the face can be deter-mined by the angle between the plane of the eyes and the plane

of the mouth. Normally these lines are parallel, but they forman angle to each other when the face is asymmetric. The devel-opment of hemifacial hypoplasia is one indication for surgery;division of the tight sternomastoid muscle allows resolutionof the skeletal abnormality and subsequent normal growth.9

Significant hemifacial hypoplasia takes about 8 months todevelop8 but is more often recognized at about 3 to 4 years ofage.17 It becomes less obvious with ongoing growth once thetorticollis has resolved.

POSTURAL COMPENSATION

When children are old enough to walk, the eyes are kept hor-izontal to facilitate balance and horizontal eye movement. Thechild compensates for the short fibrous sternomastoid by el-evating the ipsilateral shoulder (see Fig. 60-3). In addition,there may be compensatory cervical and thoracic scoliosis.Adjacent muscles, such as the trapezius, may be wastedbecause of relative inactivity.8

Conservative Management

Sternomastoid fibrosis resolves spontaneously in the vast ma-jority of infants. Therefore surgery is required only rarely, inthose in whom the torticollis has not resolved. The valueofmanipulation of the head and neck has not been proven,12 al-though it is widely used and may have some benefit in the firstyear of life.36 Physiotherapy and regular neck exercises appearto be safe36 and may make the parents feel that something isbeing done for their infant. Unintentional snapping duringmanipulation has been reported with no apparent deleteriouseffect on outcome.37 Some clinicians advocate early institu-tion of intensive passive neck range-of-motion stretchingexercises and have reported high rates of resolution,9,3840

while others believe that there is no convincing evidence thatthese measures alter the natural history of the condition.

Others consider it important to encourage parents to placetoys andotherdesirable objects on the ipsilateral side to encour-age the infant to turn toward the affected side.8 Again, this

Gravity

Tightmuscle

Headheld

rotated

Halfocciput

and faceflattened

Plagiocephaly

FIGURE 60-5 Plagiocephaly.

FIGURE 60-6 Hemifacial hypoplasia on the right side.

766 PART V HEAD AND NECK
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strategy probably helps the parent more than the infant, but isunlikelyto do anyharm.Attemptsto putthe infant to sleep withtheheadfacingtowardthe affectedside tendto fail, particularlyifthe muscle is tight. Botulinum toxin injection appears to be in-effective in patientspresenting in late childhood or adulthood,41

but results are more encouraging in younger children.42

In most cases, reassurance is all that is required. The passageof time is probably as effective as the various manipulationswhen torticollis is due to sternomastoid fibrosis.

Operative Treatment

INDICATIONS FOR SURGERY

Indications for surgery include1. Persistent sternomastoid tightness limiting head rotation in

children more than 12 to 15 months of age43

2. Persistent sternomastoid tightness with progressive hemi-facial hypoplasia

3. Diagnosis in children older than 1 year44

OPERATIVE TECHNIQUEThe procedure is performed under general anesthesia with la-ryngeal or endotracheal intubation, according to the expertiseand preference of the pediatric anesthetist. The child is placedsupine with the shoulders elevated and the neck rotated tothecontralateral side. The muscle is bestdivided at its lowerend,4,9 although division at its upper end,45 at both ends,4648

or in its midportion8,49 have all been described. Endoscopictenotomy of the muscle is also feasible5053 including througha transaxillary approach.54,55

A 3- to 4-cm transverse incision is made in a skin creaseabout1 cm above the sternal and clavicular heads of the affected ster-nomastoid (Fig. 60-7). The platysma is divided with diathermyso that no bleeding occurs in thelineof the incision. The externaljugular vein can be retracted if it is within the field of view.The tight fibrosisof the two heads of sternocleidomastoid are di-vided with diathermy near their lower end. Tightness of thecervicalfascia betweenthe sternomastoidand trapezius is usuallypalpable once the sternomastoid has been divided, and this

fasciaalsoshouldbedivided(Fig.60-8).Thisisdoneunderdirectvision to avoid damage to other structures, particularly thespinal accessory nerve and brachial plexus.

The wound is infiltrated with bupivacaine or other localanesthetic agent. The platysma is closed with continuous4-0 absorbable suture and the skin with subcuticular 5-0Monocryl absorbable suture. No drains are required.

The procedure canbe performed as a day case, and no post-operative restriction of movement is necessary. Full range ofthe neck is normally achieved within 1 week of surgery. Phys-iotherapy is usually unnecessary, although some advocate anextended period of physiotherapy postoperatively.46 In olderchildren, restoration of a full range of movement may takelonger, and the final cosmetic appearance is less certain.56

COMPLICATIONS

A hematoma may develop if hemostasis was inadequate at thetime of surgery. Diathermy dissection keeps blood loss to aminimum. Larger superficial veins may require ligation anddivision if they cannot be retracted.

Incomplete division of both heads of the sternocleidomas-toid muscle or failure to divide the cervical fascia over the pos-terior triangle of the neck may produce persistent torticollis.Careful inspection and palpation of the neck for residual tight-ness and bands at the time of surgeryshould prevent this com-plication from occurring. Recurrent torticollis is rare after

surgical treatment and is seen in less than 3% of patients.57

FOLLOW-UP

Patients should be monitored until (1) the torticollis has re-solved completely, (2) there is full range of movement of thehead and neck, and (3) the sternomastoid muscle feels nor-mal. In an older child with secondary scoliosis, follow-up, in-cluding radiologic studies, if required, should continue untilthe scoliosis has resolved.

The complete reference list is available online at wwwexpertconsult.com.

Sternomastoid

Accessory

nerve

Trapezius

Brachial plexus(immediately deep to fascia)

FIGURE 60-8 Division of the sternomastoid and investing cervical fasciato the anterior border of the trapezius.

Clavicle

Sternomastoid

FIGURE 60-7 Skin incision for low division of the sternomastoid muscle.

767CHAPTER 60 TORTICOLLIS
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