Torticollis and Plagiocephaly PT932

Torticollis and Plagiocephaly
PT932: Pediatrics
center850008549640July 16, 2018
Megan Spitler and Emily Stamps 1000000July 16, 2018
Megan Spitler and Emily Stamps

Health Condition
Congenital muscular torticollis (CMT) is the third most common congenital musculoskeletal condition behind hip dislocation and clubfoot, affecting 0.3-16% of newborns.1 CMT is considered a postural deformity that is characterized by a lateral head tilt toward and a chin rotation away from a unilaterally shortened sternocleidomastoid muscle (SCM).2 Research has found that CMT can be associated with asymmetries among the face, skull, trunk, pelvis, and feet.1 Research has determined three etiological factors that may occur; prenatal, perinatal, or postnatal. These have been found to contribute to the asymmetries observed in infants diagnosed with congenital muscular torticollis. Prenatal factors include incidents that occur in utero such as an ischemic injury, head position in utero, and intrauterine crowding. Perinatal factors are those that occur during the birthing process, such as breech birth position or assisted delivery via a vacuum or forceps. Lastly, postnatal factors may include the presence of hip dysplasia, positional preferences, and the presence of plagiocephaly.1
Once CMT has been diagnosed, there are three categories in which it can be classified; postural, muscular, and SCM mass. Postural CMT is the result of an infant’s positional preference but does not result in muscle or passive range of motion restrictions. Muscular CMT is characterized by sternocleidomastoid muscle tightness and passive range of motion restrictions. The most severe form of CMT is SCM mass where there is fibrotic thickening of the SCM muscle leading to limitation in passive range of motions.2
Plagiocephaly is a condition in which an infant’s skull is flattened in an area in response to repeated pressure on that specific side of the head.3 As a consequence to the “Back to Sleep” campaign in 1992, which recommended infants be put on their back when sleeping to prevent sudden infant death syndrome (SIDS), the incidence of plagiocephaly has increased.1 Two types of plagiocephaly exist; congenital and developmental. Congenital plagiocephaly is caused by craniosynostosis, a condition where the infant’s sutures between the skull bones grow together too early.4 Positional, or deformational plagiocephaly is the more common type which may or may not be present at birth but is developed during infancy. This type is most often caused by an infant sleeping regularly in one position. “Studies show 20-25% of infants who sleep on their back develop some degree of plagiocephaly”.3 Other causes of positional plagiocephaly include small intrauterine space, prematurity, and muscular torticollis. Decreased intrauterine space may be seen with multiple births, mothers with smaller pelvic frames, or when a baby is in breech position. Premature babies are at risk of developing plagiocephaly because the last 10 weeks of pregnancy are crucial for the cranial bones to become stronger and harder. When an infant is premature, they end up spending extensive time in a fixed position while in the neonatal intensive care unit, thus, increasing their risk of developing a flattened area.4
Plagiocephaly is also commonly associated with muscular torticollis; approximately 80-90.1% of children with CMT develop plagiocephaly.1 This again is caused by the infant not being able to change the position of their head and neck and favoring a specific position. In cases of CMT, plagiocephaly can be seen at birth or it may be acquired within the first 3 months of infancy development. When plagiocephaly is seen with CMT at birth, the plagiocephaly may be concordant (same side) or discordant (opposite side) to the muscular torticollis. However, acquired plagiocephaly is always concordant to the muscular torticollis.1 The risk factors for developing CMT and plagiocephaly together are the same risk factors considered for each condition individually: large birth weights, male gender, breech position at birth, multiple births, small pelvic framed mothers, difficult labor and delivery, use of vacuum or forceps birth assistance, and maternal uterine abnormalities.1
Medical Implications
Newborns receive several medical checkups during their first year of life. The American Academy of Pediatrics recommends pediatricians to begin checking for head dysmorphia at one week of age, skull deformities at one month 2, and assess for developmental hip dysplasia at 10 weeks of age10. Neck symmetry or “torticollis checks” are not currently performed by pediatricians until the 2 months checkup.2 During this time, the pediatrician will provide the initial screening as well as parent education on proper positioning and stretching. If asymmetry is noted, however the recommended stretching and positional changes have not resulted in resolution of the asymmetry, the pediatrician will refer the infant to physical therapy for early intervention.2 There is a 98% chance that an infant will achieve normal neck range of motion within a month and a half treatment if initial intervention is started within the first month. However, if intervention is prolonged to after 6 months of age, episode of care may be lengthened to 9-10 months of intervention and fewer reports of achieving normal ranges.2
Developmental Implications
Developmental delays may be detected at 2 months of age in infants with CMT. The early months are crucial for development, and CMT may negatively impact the development of vision, sensory processing, feeding, and fine and gross motor skills. Examples of these delays may include, “decreased head control, limited visual tracking, limited reaching on affected side, preference for rolling to one side only, delayed sitting, asymmetrical crawling, delayed walking, poor balance, difficulty feeding, and decreased tolerance to movement in space, such as swinging or sliding”.5 According to observational data from the Congenital Muscular Torticollis Clinical Practice Guidelines, up to 25% of infants with postural CMT may have temporary motor asymmetry. Due to the risk of impaired movements, developmental exercises should be incorporated in physical therapy treatment to promote symmetrical patterns in weight bearing postures and to prevent the development of movement impairments in prone, sitting, crawling, and walking. However, with adequate physical therapy, these motor delays should be resolved by 2 years of age.2
There are few reported cases of untreated CMT, however unresolved or reoccurring CMT has implications for older children or adults who may later undergo a form of invasive intervention to intervene. The tightness in the SCM may progress to a contracture over time or even contribute to cervical scoliosis. Untreated CMT can also lead to limited shoulder mobility which will impact upper extremity use and development by limiting exploration via reaching toward midline.8 Movement compensation is not uncommon in this population, presenting with shoulder elevation, adducted arm and elbow flexion, and a medial rotation at the hip and supinated foot on the contralateral side.9
Plagiocephaly is regarded as a cosmetic condition and there is little evidence supporting any developmental delays or secondary impairments. However, in addition to head shape distortion, facial appearance may be adversely affected by ear displacement and/or mandibular and maxillary deformations which may result in the child experiencing social or psychological risks, such as teasing and bullying.6
Equipment
When conservative treatment has not proven beneficial for CMT, orthotics may be implemented as an adjunct in the plan of care. A Tubular Orthosis for Torticollis (TOT) collar is an assistive device that helps obtain, maintain, or restrain motion.1 The neck orthotic stimulates movement away from and prevents movement toward the tilted head position.2 The collars are used on “infants with CMT aged 4 to 4.5 months who demonstrate adequate head control in supported sitting, and who demonstrate > 5-6 degrees of head tilt.”2 However, although used routinely, there is lack of evidence supporting the isolated use of the TOT collar compared to other interventions.2 Other devices used, however not showing significant evidence, include soft foam collars and custom fabricated cervical orthoses post-surgery 2, and a contralateral torticollis postural positioning device.1
As with CMT, when positional plagiocephaly has not responded to conservative measures, a custom-made molding headband or helmet is considered. A dynamic orthotic cranioplasty (DOC) band, also known as the “cranial orthosis” is designed by a certified orthotist and is most effective when initiated at or before 6 months of age.1,7 The goal of the helmet is to relieve pressure on the affected side of the head and allow for better pressure distribution. The helmet is fitted to promote the bones of the skull to expand into the flat areas as the infant’s head grows. The helmet should be worn by the infant for 23 hours a day to allow for skin care and passive neck range of motion exercise. For optimal outcomes, there is a continued process of modification done by the orthotist over the 3-6 months of use. According to the literature, the use of a helmet in infants with positional plagiocephaly has more successful outcomes in reshaping the infant’s head than repositioning.7
PT Considerations
Examination of an infant referred to physical therapy for congenital muscular torticollis should be thorough and include screens for asymmetries in musculoskeletal and non-musculoskeletal systems. Conducting an interview with the caregiver or parent of the infant to obtain an extensive history overview is an important component of the examination allowing for a complete understanding of the condition. A history of pregnancy, delivery, the infant’s head postures and preferences, family history of torticollis or developmental conditions, as well as progression of developmental milestones are all necessary pieces of information.2 Developmental assessment screens such as the Alberta Infant Motor Scale (AIMS) or the Peabody Developmental Motor Scales (PDMS) can assist a physical therapist in determining the presence of developmental delays.2 The AIMS is valid and reliable for this population of infants between 1 and 18 months of age, while the PDMS extends to 72 months of age.2
Screening of the neurological, musculoskeletal, integumentary and cardiopulmonary system should be included in the examination of infants with CMT.2 According to the APTA’s Clinical Practice Guidelines for Congenital Muscular Torticollis, a systems review includes the following items:
Neurological screen: Screening for abnormal or asymmetrical tone, retention of primitive reflexes, resistance to movement, cranial nerve integrity, brachial plexus injury, pain, temperament, achievement of milestones, visual screenings of eye tracking in all directions.
Musculoskeletal screen: Screening for asymmetries in face, skull and spine shape, shoulder and hip alignment, rib cage formation, craniofacial shape, passive range of motion of neck and palpation of the SCM.
Integumentary screen: Screening for symmetrical skin folds of the hip and cervical regions, color and condition of the skin as well as assessing for sign of pressure or trauma.
Gastrointestinal screen: Screen includes asking the caregiver or parent if there is a history of reflux or constipation, as well as asking about a preference in feeding side.
Cardiopulmonary screen: Screening for symmetrical coloration, rib cage expansion and clavicle movement, history of or presence of acute respiratory
tract distress, and if the infant presents with appropriate vocalization without wheezing.
The severity of torticollis can be classified into eight grades that are based on the infants age, the presence of a SCM mass, and the difference in cervical rotation with passive motion. This classification system allows for clarity within research and communication among clinicians.2 Severity level grade 1, also termed early mild, is when an infant 0-6 months of age presents with only postural preference or a difference in passive cervical rotation less than 15 degrees between right and left sides. An infant that presents with a difference of 15 to 30 degrees is considered a grade 2, or early moderate severity. If the difference is greater than 30 degrees or if there is a SCM mass present, the infant is classified as early severe, grade 3. Grades 4 and 5 are used when the infant is diagnosed later than 6 months of age with only postural preference or less than 15 degrees difference in passive cervical rotation; grade 4 is when the infant is 7-9 months of age, while grade 5 is from 10-12 months of age. Grade 6 is late severe, where an infant between 7-9 months presents with more than 15 degrees of passive cervical rotation difference between sides or an infant 10-12 months presents with 15-30 degrees difference. Late extreme, or grade 7, is used for a 7-12-month-old presenting with a SCM mass or a 10-12-month-old with more than 30 degrees of passive cervical rotation difference between sides. The last grade, 8, is very late severity and encompasses toddlers that are diagnosed older than 12 months of age presenting with any asymmetry such as postural preference, SCM mass or any degree of difference between sides in passive cervical rotation.2
An infant’s severity classification, age of treatment initiation, comorbidity presence, rate of change, and adherence to home exercise programs contribute to the infants prognosis of treatment for congenital muscular torticollis.2 Research by Demirbilek, Atayurt et al. revealed treatment prior to 3 months old resulted in full resolution of CMT in 100% of participants.2 Predictors of poor or fair outcomes for treatment of CMT include initiation of intervention after 1 year old, the presence of a SCM tumor, and an initial head rotation greater than 15 degrees from neutral.10
Plan of Care
The development of a plan of care for an infant with CMT should be client-centered and holistic. It is important to consider all aspects of development as they pertain to the infant’s experiences and exploration rather than limiting treatment to the body structure alone.2 The plan should focus on the infant’s development rather than solely on biomechanical measures of changes. Discontinuation of the plan of care occurs when the infant has met these criteria: when passive range of motion is within 5 degrees of the non-affected side, presents with symmetrical active range patterns, demonstrates age appropriate motor development, no observable head tilt, and the parent or caregiver understands what to monitor as the child continues to develop.2
Treatment for congenital muscular torticollis should be a systematic approach that encompasses the whole infant and his or her development. Weight-bearing postures and facilitating movement patterns in prone, sitting, crawling and walking can be used to allow for appropriate developmental milestone progress. Manual stretching and positioning in side lying or prone with the infant’s head turned to the affected side are important components to improve neck range of motion in a passive form.2 The therapist may also use active motion of the neck to encourage strengthening of the muscles as well as stretching. Sitting in an upright position, practicing rolling, or side lying can assist in practicing righting reactions while strengthening the muscles involved. Trunk strength and range of motion should also be addressed; the use of prone positioning is an appropriate treatment that encourages trunk extensor strengthening as well as trunk flexor elongation.2
Parent or caregiver education is a large contributor to patient prognosis and success in treatment of CMT and plagiocephaly. Emphasis on ‘tummy time’ positioning and symmetrical handling for feeding can be implemented in the home exercise program to improve CMT and plagiocephaly. Focuses should be on environmental adaptations for positioning to promote stretching and relief of pressure on the infant’s head and neck.2 Some ideas include positioning in the crib or changing table, adapting the car seat, and using toys to encourage head or neck positioning.2
An infant reassessment is recommended about 3 to 12 months after discontinuation of care .2 An examination for recurrence of CMT as well as for evidence of atypical development should include screening for postural preference, and the assessment of movement and structural symmetries in the neck, face, head, trunk, hips, and upper and lower extremities. Performing the developmental screen of choice from the initial evaluation of the infant should occur at the discontinuation visit as well as the reassessment visit. Developmental delays may not become evident until the infant is in early school ages, thus continuation of reassessment is highly recommended by the American Physical Therapy Association.2 A referral to a specialist or pediatrician should be made by the physical therapist when there are indications that progress is not occurring as anticipated, or if within 4-6 weeks of conservative treatment there is no observable resolution of neck, head or trunk asymmetries noted in the initial examination.2
Treatment
After a referral has been made and conservative management has not been effective after 6 months of treatment, the pediatrician and the caregiver may decide that an invasive intervention is the next best option. These are commonly considered when the infant is beginning treatment after 1 year of age and presents with significant neck range of motion restrictions or SCM mass.2 Botulinum toxin is a fairly new form of treatment10 that encourages muscle relaxation with the goal of greater compliance of the muscle to stretching by decreasing the SCM muscles ability to respond to firing.10 Botox injections are used for adults with cervical dystonia, as well as other muscular conditions, however it is not formally approved for use in the infant population.2
Surgical release of the SCM is a largely invasive surgery that requires physical therapy management postoperatively as well as preoperative management. Procedural options includes a tendon lengthening method, a unipolar release of the distal SCM attachment, or a bipolar release of both attachment sites.2 In order for the infant to qualify for this procedure, they must meet the following criteria: they must have persisting and progressing limitations in cervical range of motion greater than 15 degrees, the presence of a SCM mass with a persistent observable head tilt, and must be greater than 12 months of age in combination with a late diagnosis.2 Postoperative physical therapy management is similar to conservative management with emphasis on stretching, range of motion, positioning, strengthening of trunk and cervical muscles as well as additional scar management.2 Surgical complications are rare for this procedure, however, there are always risks that must be considered when deciding on an invasive intervention, especially for an infant or toddler..10 Damage to the nervous or vascular system may occur as a result of sternocleidomastoid proximity to the brachial plexus, external and anterior jugular veins, carotid arteries, and facial nerve.10
Family Impact
Parent/caregiver education is crucial in the management and successful treatment of CMT and plagiocephaly. “Almost 80% of skull growth occurs before 12 months of age, affording only a small window of opportunity to provide nonsurgical treatment to correct deformational plagiocephaly”, emphasizing early diagnosis and intervention as the key to a successful treatment.1 As mentioned earlier, 98% of infants with CMT who start physical therapy interventions before 1 month of age will achieve normal or near normal range of motion within one and a half months. However, if the start of intervention is delayed, then the episode of care is lengthened and has been correlated with decreased outcomes.2
Due to the restricted time frame of opportunity, a child with CMT and plagiocephaly will primarily impact the family’s time. When an infant is not at physical therapy, it will be the family/caregiver’s role to continue reinforcement or improvement of neck range of motion, strength, and postural control throughout the day.1 The family/caregiver needs to be instructed on techniques to encourage movement toward the affected CMT side and repositioning techniques that will discourage the infant from lying on the flattened area of the skull. Other than time and education needed for the family, many parents become concerned with how CMT and plagiocephaly may impact their child’s appearance, peer interactions, and development.6 With proper family/caregiver education, physical therapy intervention, and a quality relationship with the child’s pediatrician, these two conditions have successful outcomes with treatment which reinforces the reason why early diagnosis and intervention are key to management of CMT and plagiocephaly.

References:
Palisano R, Orlin M, Schreiber J. Campbell’s Physical Therapy for Children. 5th ed. Elsevier; 2017.

Kaplan S, Coulter C, Sargent B. PHYSICAL THERAPY MANAGEMENT OF CONGENITAL MUSCULAR TORTICOLLIS: AN EVIDENCE-BASED CLINICAL PRACTICE GUIDELINE FROM THE AMERICAN PHYSICAL THERAPY ASSOCIATION ACADEMY OF PEDIATRIC PHYSICAL THERAPY. Academy of Pediatric Physical Therapy. June 2018. http://www.pediatricapta.org/userfiles/files/CMT-CPG_PublicDraft_May_2018.pdf. Accessed July 1, 2018.

Plagiocephaly | Boston Children’s Hospital. Boston Childrens Hospital. http://www.childrenshospital.org/conditions-and-treatments/conditions/p/plagiocephaly. Accessed July 16, 2018.

Stanford Children’s Health. Stanford Children’s Health – Lucile Packard Children’s Hospital Stanford. http://www.stanfordchildrens.org/en/topic/default?id=deformational-plagiocephaly-90-P01834. Accessed July 16, 2018.

Congenital Muscular Torticollis: An Overview. Hospital for Special Surgery. https://www.hss.edu/conditions_congenital-muscular-torticollis.asp#plagiocephaly. Accessed July 14, 2018.

COLLETT B, BREIGER D, KING D, CUNNINGHAM M, SPELTZ M. Neurodevelopmental Implications of “Deformational” Plagiocephaly. Journal of Developmental and Behavioral Pediatrics. 2005;26(5):379-389.

Steinbok P, Mortenson P. A Clinician’s Guide to Positional Plagiocephaly. BC Children’s Hospital. 2008.

Ragsdale A. What Happens When Torticollis Goes Untreated. North Shore Pediatric Therapy. https://nspt4kids.com/parenting/what-happens-when-torticollis-goes-untreated/. Published September 7, 2015. Accessed July 16, 2018.

Knutsen RM. Tackling Torticollis. Rehab Insider. http://rehab-insider.advanceweb.com/tackling-torticollis/. Published September 5, 2017. Accessed July 16, 2018.

Luther BL. Congenital Muscular Torticollis. Orthopaedic Nursing. 2002;21(3):21-29. doi:10.1097/00006416-200205000-00005.