II. Definition

  1. Head deformity in infant related to fixed position

III. Epidemiology: Incidence in U.S.

  1. 1992: 1 per 300 healthy infants
  2. 1999: 1 per 60 healthy infants
  3. Increase due to back positioning for SIDS prevention
  4. Head shape abnormality persists in 2-8% of those over age 2 years
    1. Roby (2012) Otolaryngology

IV. Pathophysiology

  1. External pressure from prolonged fixes head positioning
  2. Infant susceptible due to rapidly developing skull

V. Risk factors

  1. Prematurity
  2. Hypotonic muscle disorders
  3. Congenital Torticollis
  4. Intrauterine Constraint (Twins, Oligohydramnios, Large for Gestational Age)
  5. Ventriculoperitioneal shunt
  6. Developmental Delay

VI. Signs

  1. Forehead protrudes on side of occiput flattening
  2. Ear on flattened side moves anterior to other ear
  3. Eyes appear to have unequal positioning
  4. Bald spot may be present on flattened side
  5. Viewed from above, head shape is a parallelogram
  6. Measured with diagonals (occiput to contralateral frontal forehead)
    1. Difference between diagonal measurements normally <4 mm
    2. Difference of 6-8 mm is visible

VII. Differential Diagnosis: Synostotic Plagiocephaly

  1. Critical to differentiate from positional deformity
  2. Findings that suggest Synostotic Plagiocephaly
    1. Lambdoid Suture with palpable ridge
    2. Ear on flattened side more posterior than other side
    3. Forehead does not protrude
    4. No signs of external pressure (e.g. bald spot)

VIII. Imaging: Indicated for unclear or refractory cases

  1. No routine imaging unless suspicion for Craniosynostosis, neurologic findings
  2. Skull XRay or CT Head with 3D Reconstruction
    1. Check Sutures for Synostotic Plagiocephaly
  3. Ultrasound also effective in lambdoid Suture evaluation
    1. Sze (2003) Pediatr Radiol 33(9):630-6 [PubMed]
  4. Cervical Spine XRay indications
    1. Neurologic or ocular findings
    2. Torticollis refractory to physical therapy

IX. Management

  1. General
    1. Teach prevention at Well Child Visits (see below)
    2. Evaluate head shape for deformity at Well Child Visit
    3. Identify mild deformity early (e.g. 2 month check)
  2. Step 1: Physical therapy to relieve Torticollis
    1. Parent education
    2. Sternocleidomastoid stretches
  3. Step 2: If no improvement at 4-8 weeks
    1. Check for Synostotic Plagiocephaly (See XRay above)
    2. Custom molded head orthosis (skull-molding helmet)
      1. Off-the-shelf helmets (non-custom helmets) do not appear effective
      2. Helmets worn for 23 of 24 hours daily, and follow-up every 2 weeks for Orthotic adjustment
      3. Typically most effective if started by 6 months of age, and used for 3-4 months
      4. Vles (2000) J Craniofac Surg 11(6):572-4 [PubMed]
  4. Step 3: Refer if refractory or possible synostosis
    1. Craniofacial surgery or
    2. Pediatric neurosurgery

X. Complications

  1. Cosmetic deformity: Facial asymmetry (10% of cases)
  2. Persistent occipital flattening (33% of cases)
  3. Developmental Delay in some persistent cases
    1. Mild delay: 8-20% of persistent deformity cases
    2. Significant delay: 9-13% of persistent cases
    3. Panchal (2001) Plast Reconstr Surg 108(6):1492-8 [PubMed]

XI. Prevention: Education at Well Child Visits

  1. Regular supervised play in prone position
  2. Approach infant for feeding from alternating sides
  3. Avoid prolonged sitting in Car Seat or swing

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Ontology: Postural plagiocephaly (C0432129)

Definition (SNOMEDCT_US) Distortion of the infantile cranium secondary to the application of prenatal and postnatal external forces.
Concepts Congenital Abnormality (T019)
SnomedCT 254024005
English Positional plagiocephaly, Postural plagiocephaly, Postural plagiocephaly (disorder)
Spanish plagiocefalia postural (trastorno), plagiocefalia postural

Ontology: Plagiocephaly, Nonsynostotic (C1450010)

Definition (MSH) A deformity of the SKULL that is not due to bone fusion (SYNOSTOSIS), such as craniosynostoses, and is characterized by an asymmetric skull and face. It is observed with an increased frequency in INFANTS after the adoption of supine sleeping recommendations to prevent SUDDEN INFANT DEATH SYNDROME.
Concepts Acquired Abnormality (T020)
MSH D049068
Swedish Plagiocefali, icke-synostotisk
Czech plagiocefalie nonsynostotická, plagiocefalie bez synostózy, Polohová plagiocefalie
Finnish Ei-synostoottinen plagiokefalia
Russian PLAGIOTSEFALIIA NESINOSTOZNAIA, ПЛАГИОЦЕФАЛИЯ НЕСИНОСТОЗНАЯ
English Positional plagiocephaly, Plagiocephaly, Nonsynostotic [Disease/Finding], Plagiocephaly, Deformational, Plagiocephalies, Deformational, Plagiocephaly, Positional, Plagiocephalies, Positional, Deformational Plagiocephalies, Deformational Plagiocephaly, Positional Plagiocephalies, Positional Plagiocephaly, Nonsynostotic Plagiocephaly, Plagiocephaly, Nonsynostotic
Spanish Plagiocefalia posicional, Plagiocelalia sin Sinostosis, Plagiocefalia no Sinostótica
Dutch positionele plagiocefalie, Plagiocefalie, non-synostotische
Italian Plagiocefalia posizionale, Plagiocefalia non dovuta a sinostosi
German Positions-Plagiocephalie, Nicht-synostotische Plagiozephalie, Nicht-synostotische Schiefköpfigkeit, Plagiozephalie, nicht-synostotische, Schiefköpfigkeit, nicht-synostotische
French Plagiocéphalie posturale, Plagiocéphalie sans synostose, Plagiocéphalie positionnelle
Portuguese Plagiocefalia posicional, Plagiocefalia não Sinostótica
Japanese トウイセイシャトウショウ, 頭位性斜頭症, 斜頭蓋-非骨癒合性, 非骨癒合性斜頭蓋
Polish Skośnogłowie niekościozrostowe
Hungarian Positionalis plagiocephalia
Norwegian Ikke-synostotisk plagiocefali, Plagiocefali, ikke-synostotisk