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What is craniosynostosis?
- Craniosynostosis
is a term that refers to the early fusing of one or more of the
sutures in the skull during fetal development. The skull is composed
of multiple bones that are separated by sutures or openings. If
any of these sutures fuse prematurely, the skull will expand in
the direction of the open sutures resulting in an abnormal head
shape.
- Premature
closure of a single suture can be caused by space constraints
in utero. This is seen with twins more often than with a single
fetus. Single suture closure is usually not associated with a
syndrome and not genetic in nature.
- Premature closure
of multiple sutures, including facial sutures, are seen in individuals
with a craniosynostosis syndrome.
- The most
common craniosynostosis syndromes are Crouzon, Pfeiffer and Apert.
In these syndromes, several sutures in the head are fused resulting
in abnormal skull shapes. Bones in the face also are fused, resulting
in a flat midface and protruding eyes.
- Children
with Apert syndrome also have syndactyly (webbing) of the hands
and feet.
- Crouzon syndrome
occurs in approximately 1 in 100,000 births and severity varies
considerably. Pfeiffer syndrome occurs in approximately 1 in 25,000
births and also varies in severity. Apert syndrome occurs in approximately
1 in 100,000 births, and is much less variable from case to case
than either Crouzon or Pfeiffer syndrome.
How is craniosynostosis
treated?
- A child with
a non-syndromic single suture synostosis will usually not have
any functional problems such as difficulties with breathing or
feeding. Surgical release of the closed suture may be necessary
and if so, would usuallly be done during the first year of life.
- The child
with a craniosynostosis syndrome should be seen immediately after
birth by the nurse from the Craniofacial Center to establish adequate
feeding and breathing. Shortly after discharge from the hospital,
the child should be seen for a full team evaluation.
- The skull
abnormalities require early attention. Timing of surgery is variable,
but would usually be done before the baby is six months old.
- If a cleft
palate is present, additional feeding difficulties may be present
(see Cleft Palate Only)
- An infant
should be evaluated by a pediatric pulmonary physician to address
any breathing difficulties.
- Audiology
(hearing test) and speech evaluations are important to insure
good speech and language development.
- Evaluation
by an ophthalmologist is important, especially if the eyelids
do not adequately protect the eyes.
- For a child
with Crouzon, Pfeiffer or Apert syndrome, a surgical procedure
called a midface advancement brings the cheekbones forward to
provide greater protection for the eyes and to improve the child's
airway. Because the upper jaw is usually too small, a child with
Crouzon, Pfeiffer or Apert syndromes will have an "under
bite" and severely crowded teeth. Orthodontic treatment (braces)
is therefore necessary. Timing for the midface advancement and
orthodontics is based on a number of factors. They may be done
early in life or after growth is completed, depending on the particular
needs of the child.
- A child with
a craniosynostosis syndrome may encounter social and emotional
challenges unique to these syndromes. Support organizations and
resources are available for children and their families (see Support
Information).
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