Definitions
Plagiocephaly is often used as an umbrella term for both craniosynostosis and positional plagiocephaly, but we would do well to be precise with these terms.
- Craniosynostosis: Premature fusion of one or more cranial sutures; types include
- trigonocephaly = premature fusion of metopic suture
- scaphocephaly = premature fusion of sagittal suture (most common form)
- plagiocephaly = unilateral coronal or lambdoidal synostosis
- kleeblattschädel (aka cloverleaf deformity) = premature fusion of multiple sutures
- acrocephaly = combined sagittal, coronal, and lambdoid synostosis
- brachycephaly = bilateral coronal synostosis
- Positional plagiocephaly (aka deformational plagiocephaly): Flattened head shape resulting from extrinsic forces acting on an intrinsically normal skull (i.e., not craniosynostosis because all sutures remain open)
Note that the remainder of this blog post largely focuses on positional plagiocephaly.
Clinical Diagnosis… From Above!
- Look down at the patient from above.
- Identify the side of flattening.
- Identify if and which ear is anteriorly positioned.
Note that with positional plagiocephaly, the flattened side corresponds with the anterior positioning of the ipsilateral ear. As such, in positional plagiocephaly, the head shape is a parallelogram.
DDx
Positional plagiocephaly should be differentiated from other conditions, particularly congenital muscular torticollis (CMT) and craniosynostosis.
CMT:
- Postural deformity of neck
- Head tilt to one side with chin rotated to the opposite side
- Reduced cervical range of motion
- Palpable SCM tightness or mass
Craniosynostosis:
- Consider the pathophysiology of craniosynostosis: the premature synostosis (i.e., union/fusion) of at least one cranial suture leads to compensatory skull growth in parallel to the affected suture (e.g., fusion of sagittal suture results in skull elongation, aka scaphocephaly—see figure above)
Complications from craniosynostosis include increased intracranial pressure, inhibition of brain growth, impairments in cognitive and neurodevelopment function (e.g., developmental delay, poor feeding, and weight gain), cranial nerve deficits, poor self-esteem and social isolation. Craniosynostosis is associated with multiple genetic syndromes, including Crouzon syndrome, Apert syndrome, Pfeiffer syndrome, and Carpenter syndrome.
Interventions for positional plagiocephaly
Position and handling:
- Increased tummy time
- Supervised time with supported sitting up or cuddling upright
- Adjusting infants head position during feeds, holding, carrying
Environmental adaptations:
- Place infant at a different end of the crib each time going to sleep
- Infants prefer to face the room to look around
- Place crib-safe toys or mirrors on desired side of infant
- Limit time in car seats, swings, and other infant devices
Helmet or band therapy (cranial orthosis):
- Helmet: surrounds the asymmetrical head with a symmetrical mold
- Band: more proactive, applies gentle pressure to area where head growth not wanted, leaving space for area of desired growth
- Adjusted weekly or bi-weekly
Physical Therapy:
- 2016 Congress of Neurological Surgeons systematic review suggested that the additional of PT to repositioning education is more effective than repositioning education alone
Blog post based on Med-Peds Forum talk by Julia Ding, PGY4