Congenital Muscular TorticollisA Spectrum of Disease

James K. Bredenkamp, MD; Larry A. Hoover, MD; Gerald S. Berke, MD; Anthony Shaw, MD

\s=b\Sternocleidomastoid muscle fibrosishas been recognized for centuries, but itspathogenesis and treatment remains con-troversial. Pseudotumor of infancy is afirm fibrous mass in the sternocleidomas-toid muscle appearing at 2 to 3 weeks ofage. Congenital muscular torticollis is lesscommon and appears later in life. Pseudo-tumor and congenital muscular torticollisprobably represent different manifesta-tions of sternocleidomastoid muscle fibro-sis. Pseudotumor will usually resolve withconservative therapy; however, some pa-tients will subsequently develop torticol-lis. Congenital muscular torticollis usuallyrequires surgical release of the sternoclei-domastoid muscle to achieve a good cos-metic result and to prevent plagiocephaly,facial asymmetry, and scoliosis. This re-port provides guidelines for the manage-ment of congenital muscular torticollis andpseudotumor of infancy based on the au-thors' experience and review of the med-ical literature. Representative case histo-ries from the neonate through the adult arepresented, and the pathogenesis, diagno-sis, treatment, and prognosis are dis-cussed.(Arch Otolaryngol Head Neck Surg.

1990;116:212-216)

Congenital muscular torticollis(CMT) is a fibrous contraction of

the sternocleidomastoid (SCM) mus¬cle, which causes the head to be drawnto the side so that the chin is rotatedand points to the opposite side.Pseudotumor of infancy (POI) is a

firm, fibrous mass appearing at 2 to 3weeks of age and is located in the SCMmuscle distally.1 Both disorders are

Accepted for publication March 3, 1989.From the Divisions of Head and Neck Surgery

(Otolaryngology) (Drs Bredenkamp, Hoover, andBerke) and Pediatric Surgery (Dr Shaw), UCLASchool of Medicine, Los Angeles, Calif, and theDivisions of Head and Neck Surgery (Otolaryn-gology) (Dr Hoover) and Pediatric Surgery (DrShaw), Olive View Medical Center, Sylmar, Calif.Read before the Paul H.Ward, MD, Society, Los

Angeles, Calif, April 16, 1988.Reprint requests to Division of Head and Neck

Surgery, UCLA School of Medicine, Los Angeles,CA 90024 (Dr Hoover).

characterized by SCM muscle fibrosis,and their pathogenesis remains un¬known. The incidence of POI is lessthan 0.4% of all newborns.2 Theseinfants are usually normal at birth,but, at 2 to 3 weeks of age, a fusiformSCM muscle swelling appears. Themass will often persist for 2 to 3months, then slowly disappear.1 How¬ever, 10% to 20% of patients with POIwill go on to develop either a progres¬sive or delayed torticollis, and 25% willhave a minor residual degree of per¬sistent asymmetry and tightness ofthe SCM muscle.3·4 In contrast to POI'searly presentation, CMT may appearat any time, usually at 3 to 4 years ofage, and may or may not be associatedwith a history of POL Congenital mus¬cular torticollis may cause significantcraniofacial asymmetry and cervical/lumbar scoliosis.4 Men and women areaffected equally, and there is no racialpredilection.Historically, the wry neck deformity

has been recognized for centuries. Theterm wry neck is derived from old andmiddle English, meaning to wind or totwist. Greek and Roman authorsnamed this condition caput obstipurnto indicate the abnormal posturing ofthe head and, in the German medicalliterature, this term has been usedsynonymously with torticollis.4 JeanFroissart, in the 14th century, coinedthe term au tort col from the Latinwords tortus, meaning twisted, andcollum, meaning neck. From this, themore common term, torticollis, isderived.4The finding of a torticollis deformity

in a particular patient is a sign, ratherthan a specific diagnosis. Nearly 80 en¬tities have been reported to cause

torticollis,5 and these are outlined inTable 1. Torticollis can result from anyprocess that damages or irritates themuscles, ligaments, or bones of theneck. Furthermore, any lesion of the

brain, spinal cord, or tracts governinghead position and orientation, as wellas ocular and psychiatric distur¬bances, can present with torticollis.Acquired torticollis, especially second¬ary to a head and neck inflammatoryprocess, is the most common cause oftorticollis in children. Adult-onset tor¬ticollis usually is a spastic torticollis.Congenital muscular torticollis is thethird most common form of torticollis,and it is the most common congenitaltype.5 Congenital muscular torticollisis the third most common congenitalmusculoskeletal anomaly, after club-foot and congenital hip dysplasia.6Likewise, POI in the absence of a tor¬ticollis deformity must be differenti¬ated from other pediatrie neck masses.Figure 1 outlines the diagnostic ap¬proach to a patient presenting withtorticollis.

PATHOGENESISA number of authors have at¬

tempted to explain the development offibrosis and/or tumor in the SCM mus¬cle. Intrauterine mechanical factorsand birth injury have been hypothe¬sized to produce torticollis.4 The highincidence of obstetrical complications(sevenfold incidence of breech deliv¬ery) in patients with CMT,4·7 and the20% association of clubfoot and con¬

genital hip dislocation, supports thistheory.4·6 However, the majority of in¬fants with SCM muscle fibrosis havehad unremarkable gestations and de¬liveries, and Jones4 has even describedseveral infants with POI who had beendelivered by cesarean section. Stro-meyer, in 1838, believed that the tumorwas a hematoma caused by rupture ofthe muscle.4 Hemosiderin, however,has never been demonstrated in patho¬logic specimens, and, clinically, thereis little evidence to support his theory.7Anoxic injury to the SCM muscle wasfirst proposed by Mikulicz8 in 1895,

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Torticollis

Acute Chronic

CervicalImmobilization

f? Trauma

No

Yes

Evaluation forHead and NeckInflammatoryProcess

No

R/O Drugs, GEReflux, Psychiatric(Table 1)

Yes

RoentgenogramR/O FractureReferral

R/O C1-C2Subluxation

Spasmotic

Refer forNeurologicEvaluation

Positive(Table 1)

Positive(Table 1)

Static

OcularVestibularExamination

NeurologicExamination

CervicalRadiographsPlus Examination

Fibrosed SCMNormal Roentgenograms

of Vertebrae

CMT

Other CongenitalTorticollis(Table 1)

Fig 1.—

Diagnostic workup of patients with torticollis. Question mark indicates questionable; R/O,rule out; GE, gastroesophageal; SCM, sternocleidomastoid; and CMT, congenital musculartorticollis.

then later by Völcker9 in 1902, and isstill the most widely accepted theory.Middleton10 produced histologicallysimilar "tumors" by ligation of veinsdraining the sartorius muscle in dogs,and inferred that venous obstructionand anoxia during parturition was thecause of POL The pathologic changesfound in Volkmann's ischemie contrac¬ture are similar to those found in CMT4and further supports the anoxic injurytheory. The various theories of SCMmuscle fibrosis have never completely

explained all the peculiarities of thiscondition, and, thus, a precise expla¬nation for all cases remains obscure.Torticollis is not simply a static,

fixed deformity, but has far-reachingeffects on facial and cranial growth,development of the spine, and, possi¬bly, ocular and vestibular develop¬ment. Ombrédanne11 observed thatchildren, in an attempt to compensatefor torticollis, elevated the affectedshoulder girdle and laterally shiftedthe head toward the affected side. This

Table 1.—Differential Diagnosis ofTorticollis

CongenitalMuscular torticollisC1-C2 articular malformation:atlantoaxial dislocation,rotatory subluxation23

Klippel-Feil syndrome2"Sprengel's deformity2''Congenital postural torticollis—transient,present at birth, secondary toabnormal fetal position"5

AcquiredTrauma"Nasopharyngeal" torticollis26Grisel's disease/C1-C2 subluxation2728DrugsSandifer's syndrome:gastroesophageal reflux29

PsychiatricNeurologicSyringomyelia30Dystonia5Herniated cervical disks5Any posterior fossapathologic finding5

Spastic torticollis31Ocular strabismus/paresis of

extraocular movements14VestibularCongenital nystagmus5Paroxysmal torticollis of infancy:episodic head tilt, vomiting, pallor,and dizziness32

involuntary compensation may lead tothe development of cervical scoliosis,compensatory lumbar scoliosis, andsubsequent chronic pain. Cranialasymmetry, or plagiocephaly, has beenfound in a third of infants and childrenwith CMT.4 This cranial deformityprobably results from the asymmetricpressure on the cranium during sleep,with resultant remodeling of the facialbones. Consequently, the long axis ofthe typical elliptical skull is shiftedfrom the normal midsagittal plane toone that is angulated. Facial hemihy-poplasia and asymmetry is also com¬

monly found in patients, and probablyresults from compression of the facialskeleton by the shortened muscle.Characteristically, there is flatteningof the occiput contralaterally, and de¬pression of the malar prominence ip-silaterally, with downward displace¬ment of the ear, eye, and mouth on theaffected side.12 Because remodeling ofthe face occurs as long as there is bonyskeletal growth, these skeletal defor¬mities may improve followingsurgery.13 Ocular14 and vestibular im¬pairment may occur as the child triesto compensate for the head's abnormalorientation in space.

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REPORT OF CASESPatients with CMT and POI may present

to the clinician at various stages, dependingon age and duration of disease. Table 2 rep¬resents the authors' experience in treatingpatients with SCM muscle fibrosis. The agerange for patients with CMT was 4 years to22 years, and there was a 3:2 male-to-femalepredominance. All patients had good re¬

sults, with follow-up ranging from 6months to 10 years (Figs 2 through 7). Thefollowing case reports serve to illustratethe pertinent manifestations of SCM mus¬

cle fibrosis as it presents at different ages.Case 1.—A 6-week-old girl was noted to

develop the typical fusiform SCM musclemass of POI at 3 weeks of age (Fig 2). Thechild was the product of a normal preg¬nancy and spontaneous vaginal delivery.She had no other associated anomalies, andher siblings were normal. A fine-needle as¬

piration of the mass showed fibrosis. Themother was instructed on the exercises shecould give the infant at home, and theinfant has complete resolution of the mass.Case 2.—A 10-year-old girl with CMT

was noted by her mother to have progres¬sive head tilt to the left side and chin rota¬tion to the right side since age 7 years.There was no history of POL On physicalexamination, the child had a 50% decreaseof lateral head tilt to the right side, eleva¬tion of her left shoulder and scapula (Fig 3,left), cervical scoliosis to the right side, andcompensatory lumbar scoliosis to the leftside. Magnetic resonance imaging of theneck demonstrated fibrosis of the affectedSCM muscle, and x-ray films of the skullshowed facial asymmetry and hemihy-poplasia of the cranium on the left side (Fig4). At surgery, a very firm fibrotic SCMmuscle, along with some platysmal band-

ings, was found. The SCM muscle wasdivided distally with marked improvementin range of motion. Postoperatively, shewas given both passive and active physicaltherapy, with a good postoperative resultnoted 1 year later (Fig 2, right). However,facial asymmetry still persists 1 year post¬operatively. A complete neuro-ophthalmo-logic and vestibular examination preopera¬tively and postoperatively showed no dys¬function.Case 3.—A 22-year-old woman presented

with a history of progressive right-sidedCMT since early childhood. There was no

history of POL She had compensatory sco¬liosis and hemifacial microsomia. She hadnot been troubled by the CMT until re¬cently, when she began experiencing per¬sistent neck and shoulder pain, at whichtime she sought medical attention. Ini¬tially, she had release of the SCM muscledistally. Because persistent tethering wasnoted intraoperatively, a second incisionwas made behind the ear for release of themastoid attachment of the SCM muscle.She has had complete resolution of her neckdeformity and shoulder pain at follow-up 1year later.

COMMENTPathogenesis

Congenital muscular torticollis isbelieved to be related to POI. Canale etal15 reported that a third of their pa¬tients treated for CMT had a history ofPOI. Ippolito et al16 reported a 25% oc¬currence of POI in their patients sur¬gically treated for CMT. Although POIis reported to be localized to the distalSCM muscle, Reye17 demonstrated ex¬tension of muscle fibrosis outside theboundaries of the palpable resectedpseudotumors. Thus, some cases ofPOI may actually represent a more

generalized process of fibrosis, makingprogression to CMT more likely. Reye17also found SCM muscle fibrosis unilat¬erally in four infants who died of othercauses in whom no localized tumormass was noted ante mortem. The lackof a local manifestation (ie, tumormass) may explain why only some

children with CMT give a history ofhaving POL18 Children with CMT show

Table 2.—Authors' Experience in Treating Patients With SCM Muscle Fibrosis*

Patient No. Age, y/SexPathologicFinding Treatment Outcome

Follow-upTime, y

6 wk/F POI Conservative(exercises)

Resolved

10/F CMT Distal release Good (Fig 3)22/F CMT Bipolar release Good4/M CMT Distal release Good (Fig 5)12/M CMT Distal release Good (Fig 6) 6 mo6/M CMT Distal release Good (Fig 7) 10

CMT indicates congenital muscular torticollis; POI, pseudotumor of infancy; and SCM, sternocleidomastoid.

Fig 2.—Case 1. Six-week-old infant with right-sided sternocleidomastoid muscle fusiformswelling (arrows) consistent with pseudotumorof infancy.

Fig 3.—Case 2. Left, Ten-year-old girl with left-sided congenital muscular torticollis. Note obviousfacial asymmetry with downward displacement of the eye, ear, and mouth on the left side. Right,Results 1 year postoperatively after distal release of sternocleidomastoid muscle.

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Fig 4.—Case 2. Anterior-posterior skull roent¬genogram showing hemihypoplasia of the leftside of the face with depression of the malareminence, fore-shortened left mandibular ra¬mus, and increased left mastoid development.

fibrosis, but to a greater degree andmaturation than that found in infantswith POL4 Pseudotumor of infancy andCMT probably represent merely a dif¬ferent manifestation of SCM musclefibrosis.Despite the uncertainty of the

pathogenesis of POI and CMT andtheir relationship to each other, theirtreatment is less controversial. Withconservative therapy, good results canbe achieved in most patientswith POL7Jones4 found that half of his patientswith POI treated conservatively hadcomplete resolution, a third had someresidual subclinical muscular fibrosis,and less than 20% had either a pro¬gressive or delayed torticollis, whicheventually required division of themuscle. Binder et al19 reported similarresults. Table 3 outlines the standardexercises recommended for the conser¬vative therapy of POL Although theyare of unproven benefit, they are sim¬ple, actively involve the parents, andare harmless, if done with care. If thechild has progressive fibrosis and tor¬ticollis with or without associatedcraniofacial abnormalities by 12 to 18months of age, surgical release of themuscle is recommended.15 Results aregenerally excellent in most cases whensurgery is performed at an early age,

Fig 5.—Case 4. Left, Four-year-old child with left-sided congenital muscular torticollis preoper¬atively. Note obvious facial asymmetry. Right, Release distally of sternocleidomastoid muscle onthe left side postoperatively.

Fig 6.—Case 5. Left, Twelve-year-old child with right-sided congenital muscular torticollis. Notethat primary compensation is by elevation of right shoulder. Right, Release distally of sternoclei¬domastoid muscle on the right side.

Fig 7.—Case 6. Left, Six-year-old boy with left-sided congenital muscular torticollis 6 monthspostoperatively following release distally of the sternocleidomastoid muscle with obvious facialasymmetry. Right, Follow-up results at 10 years postoperatively. The facial asymmetry that wasnoted at 6 months postoperatively, (left) is now not apparent at age 16.

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Table 3.—

Exercises Recommended for the Conservative Therapy of POI*Passive rotation of the head and neck.Positioning of crib toys so that the neck will be stretched when trying to reach or grasp.The mother should suckle the baby so that the infant must actively stretch the affected muscle toreach the breast.

Encourage the infant to sleep with the opposite side of the head down to passively stretch themuscle and also to prevent flattening of the head on the side of the tumor.

By age 3 months, the headrighting response can be used to strengthen the opposite muscle.* POI indicates pseudotumor of infancy.

thus halting or reversing craniofacialasymmetry (Fig 7, left and right).13

Surgical TreatmentChildren and adults with CMT usu¬

ally require surgical treatment. Mostagree that nonoperative managementafter the age of 1 year is rarelysuccessful.15 The patient's age at oper¬ation, the duration of the disease, andthe severity of the deformity beforethe operation are the major factorsaffecting both cosmetic and functionaloutcome.16 Surgery prior to adoles¬cence, or even later, however, often re¬sults in significant improvement.The surgical treatment of CMT be¬

gan in Roman times with subcutane¬ous tenotomy.4 Open tenotomy was

popularized by Dupuytren in 1822,"and since that time many authors havewritten of the virtues of the varioussurgical therapies.42021 The ideal sur¬gical procedure for CMT is one thatoffers the greatest chance for success,is easy to perform, can be done withminimal complications, and gives thebest cosmetic and functional results.All patients in this series were treatedwith initial release of the SCM muscledistally and lysis of associated fibroustissue and platysmal banding. The pa¬tient's neckmobility was then assessedin the operating room. Patient 3, theonly adult who had the most severe fi¬brosis in this series, required bipolarrelease. The authors believe that thisapproach is as successful as the morecomplicated procedures and that it re¬sults in good cosmesis and function.Total excision of the muscle probablyoffers no significant advantage over

simple muscular release, may result ina poor cosmetic result, and places thespinal accessory nerve at great risk.Muscle lengthening techniques are of¬ten technically difficult, generally of¬fer no advantage over simple release,and rarely yield better cosmetic re¬

sults. All patients in this series were

treated postoperatively with eitherformal or informal passive and activephysical therapy. No patients hadsplints or casts placed, were placed intraction, or had elaborate postopera¬tive fixation, as has been suggested byothers.21,22

ConclusionsThe authors advocate the following

therapeutic approach for infants withPOI and children and adults withCMT.1. Nonoperative therapy of infants

younger than 1 year of age, unlessthere is progressive deformity withtreatment (Table 3).2. Persistent fibrosis and torticollis

after 12 to 18 months of age should betreated surgically.3. Torticollis is a sign, not a disease,

and requires appropriate diagnosticevaluation (Fig 1 and Table 1).4. Unipolar, distal release is per¬

formed initially. Intraoperative as¬

sessment of neck motility is used todetermine the need for bipolar releaseor more extensive dissection, whichmay occasionally be necessary in se¬

vere, long-standing torticollis.5. Physical therapy is recommended

following release.6. Good results can be expected in

children. Adults with long-standingtorticollis are less likely to have reso¬lution of all stigmata, but are gener¬ally satisfied with the improvement.

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