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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