POSITIONNAL
DEFORMITIES
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Plagiocephaly : when should we be concerned ? |
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The term plagiocephaly, a unilateral flattening of the skull, is used
without reference to its aetiology.[1]
There are two types to distinguish:
- Plagiocephaly resulting from fusion
(synostosis) of a coronal or lambdoid suture. In these
craniosynostoses, the deformity of the cranium is visible from birth.
- Positional or postural plagiocephaly, in which
the flattening is secondary to a pre or post-natal mechanical
pressure on the skull. Positional plagiocephaly is most commonly a
posterior flattening of the skull and only rarely affects its anterior
aspect. It is more common in boys (71% of cases) and in twins. [2] It
is associated with a congenital torticollis in 37% of cases. [3]
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Scenario
1. A child presents with a posterior flattening of the skull; the
likelihood is that this is a positional plagiocephaly, by far the most
common diagnosis. |
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- It
is important to enquire as to the position of the child when put to bed
and to determine whether there is a congenital torticollis.
- A congenital torticollis should be treated with physiotherapy
- Instructions on positioning must be provided as soon as the diagnosis is made :[4]
- Ensure that the child does not sleep on the flattened side of the skull
- Encourage prone positioning while the child is awake
- Reserve the use of hard-backed child seats for carrying or transporting the child
- Encourage
rotation of the child’s head away from the affected side; arrange
the bed in such a way that the light approaches it from the unaffected
side. Similarly, objects of interest, toys, mobiles, family pictures
should be positioned on the unaffected side of the child.[5]
If the above simple advice is not followed, the deformity will show no
improvement. The best opportunity for improvement is within the first
six months of life and this then diminishes progressively until 18
months of age. Beyond 18 months, no further improvement can be expected.
In cases of diagnostic uncertainty, AP and lateral skull X rays are
required; the absence of a lambdoid suture suggests a synostosis and a
non-urgent appointment with a craniofacial surgeon must be arranged.
This single posterior suture craniosynostosis does not result in a
significant differential growth of brain and skull and its surgical
treatment is therefore optional with essentially an aesthetic
indication.[6]
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Scenario
2. A child presents with an anterior (frontal) flattening of the skull;
this suggests an anterior plagiocephaly as a result of a premature
uni-coronal synostosis. |
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The deformity is usually obvious, with the combination of a unilateral
frontal flattening, with a elevated, yet set-back ipsilateral
eyebrow and a deviation of the nose towards the affected side (in the
case of an anterior positional plagiocephaly, the forehead is flat, but
the ipsilateral eyebrow region is depressed and the nose deviates to
the unaffected side).
Antero-posterior and lateral X rays must be acquired to confirm the
diagnosis and the child should be referred to a cranio-facial surgeon.
There is no chance of spontaneous improvement of the deformity when the
cause is premature synostosis. In the case of a positional aetiology
improvement is variable. The treatment of the deformity is surgical and
involves remodelling of the frontal bone between the ages of 6 and 18
months. The indication is essentially aesthetic as uni-coronal
craniosynostosis has only minimal risk of raised intra-cranial pressure
resulting from differential growth of the brain and the skull.
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Summary |
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Strange as it may seem, posterior postional plagiocephaly, so
frequently seen since the 1994 consensus to ensure newborn infants are
put on their backs to sleep, is the clinical entity of most concern, as
its improvement is directly related to the precocity with which
treatment is begun.
Craniosynostoses responsible for plagiocephaly necessitate additional
investigations at the discretion of the surgeon (radio-imaging, genetic
studies) and classically undergo surgery, after the age of 6
months. Early diagnostic studies (before 3 months of age) are unhelpful
and can result in diagnostic errors. They intensify the anxiety of the
parents and constitute an unnecessary expense.
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References |
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- Arnaud E, Marchac D, Renier D.
[Diagnosis of facial and craniofacial asymmetry]. Ann Chir Plast Esthet
2001;46(5):410-23.
- Littlefield TR KK, Pomatto JK, Beals SP.
Multiple-birth infants at higher risk for development of deformational
plagiocephaly: II. is one twin at greater risk? Pediatrics
2002;109(1):19-25.
- Hollier L, Kim J, Grayson BH, McCarthy JG.
Congenital
muscular torticollis and the associated craniofacial changes. Plast
Reconstr Surg 2000;105(3):827-35.
- Neufeld S, Birkett S. What to do about flat
heads:
preventing and treating positional occipital flattening. Axone
2000;22(2):29-31.
- Cartwright CC. Assessing asymmetrical infant
head shapes. Nurse Pract 2002;27(8):33, 35-6, 39.
- Ellenbogen RG, Gruss JS, Cunningham ML. Update
on
craniofacial surgery: the differential diagnosis of lambdoid
synostosis/posterior plagiocephaly. Clin Neurosurg 2000;47:303-18.
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