What are the advantages of spring assisted surgery?
Spring assisted surgery has advantages in a specific subgroup of
patients with craniosynostosis, in particular those with sagittal
-The operation is much shorter
-The scar is shorter and less noticeable
-Only a small part of the skull needs to be exposed
-Only a fraction of dura is exposed compared to traditional surgery
-There is very little bleeding
-The need for a blood transfusion is rare
-Recovery and hospital stay is much shorter
The new skull shape take several weeks to develop
The springs must be removed about 4-6 months later. This is a minor
The springs can be felt beneath the scalp whilst they are present
There is excellent long term data available now confirming the
safety and efficacy of spring surgery. These can be provided to
referring medical professionals on request. Some of these are listed
Is surgery necessary for single suture craniosynostosis?
Rationale for surgery in ‘isolated’ single suture
For many years a significant body of international opinion has
believed that the prime reason for operating in cases of single
suture craniosynostosis is for cosmetic reasons. Quite recently
it is becoming increasingly accepted that the risk of raised intracranial
pressure and the possibility of significant psychological sequelae
are higher than previously thought in these children. 1,2
Intracranial pressure (ICP)
The brain grows rapidly after birth and by the first year of age
it is approximately 80% of adult size. The cranial sutures are designed
to allow rapid skull expansion as the brain expands. If a suture
fuses prematurely, rapid brain growth still occurs but will push
out in another direction causing an external skull deformity. If
the brain requires a higher tissue tension to displace the adjacent
bones then there will be a higher pressure within the cranial cavity
and within the brain tissue. Consequences of raised ICP may include
mental delay and blindness.
Incidence of raised ICP in single suture craniosynostosis.
Raised ICP is seen in at least half of all cases with multiple
suture synostosis. In single suture synostosis the rate is lower.
The London group showed 24% (of 83 cases) had raised intracranial
pressure.3 The rate was 17% (of 18 cases) of scaphocephaly. 4
The Paris group showed 14% (of 75 cases) had definitely raised
intracranial pressure. However a further 62% were borderline with
pressure higher than expected but not in the range that is definitely
The above studies contained a large number of children and used
intracranial ICP monitors. More sensitive is the use of SPECT scanning,
not readily available in this country. It has recently been shown
that abnormalities in brain blood flow were present adjacent to
the site of a single fused suture in 71% of cases and that these
brain perfusion defects were corrected after early reconstructive
surgery. The numbers in the study were small (7 cases). The authors
recommended early surgical intervention to prevent brain compromise
secondary to abnormal blood flow. 6
A study of 10 children with single-suture craniosynostosis showed
that cerebral glucose metabolism (a measure of local brain function)
was lower than normal in the posterior occipital cortex. It consistently
increased after cranial remodeling. 7
Methods of detecting raised ICP
Currently the only accurate method for determining raised intracranial
pressure is an intracranial pressure monitor. It is well recognized
that ICP varies throughout the day, being highest during REM (rapid
eye movement) sleep, and so ideally monitoring should be performed
overnight for a minimum of 12 hours. This is unnecessary for children
with craniosynostosis who undergo early surgery unless there is
a later concern about raised ICP.
Radiological tests (such as xrays and CT scans) may sometimes show
the effects of raised ICP such as a “copper beaten”
skull, erosion of the dorsum sella, widened sutures and narrowing
of the basal cisterns. However the sensitivity of radiology for
detecting raised ICP is very low and it is not recommended to screen
for raised ICP in children with craniosynostosis.8,9
Symptoms sometimes seen with raised ICP include vomiting and headaches.
However in most cases the effects are chronic and subtle with no
readily identifiable symptoms. Headache, the most classic symptom
of raised ICP, is often not able to be vocalized in young children
and the presence of headache in cases of proven raised ICP is inconsistent.
Symptoms are more obvious in older children. The Philadelphia group
reported 16 patients with scaphocephaly who had delayed treatment
(average age 3.2 years). 25% had obvious symptoms of raised ICP.
Blindness with raised ICP
Blindness is a devastating consequence of craniosynostosis and
it is a well recognized problem in complex craniosynostosis where
its onset may be rapid and with little warning.10 Cases of blindness
in single-suture craniosynostosis are uncommon with a few isolated
case reports.11 I saw one case when I worked at the Scandinavian
craniofacial unit in Sweden in a 4 year old girl with untreated
Blindness is uncommon because it tends to develop in later years
and most cases of single-suture synostosis have a cranial decompression
procedure performed during the first year of life. It is more common
with cases who have minimal cranial involvement that go unnoticed
until late childhood.12,13 Of 290 children treated for craniosynostosis
at Birmingham, 9 (3%) had visual failure at presentation as the
result of raised ICP.12 In 7 children this was the late presenting
symptom of an undiagnosed deformity. 3 of the 9 children had a single-suture
Raised intracranial pressure (ICP) is known to cause papilloedema
(oedema of the optic disc) and if prolonged, optic atrophy. Its
presence in the younger child reliably indicates raised ICP however
chronic and gradual increases in ICP may occur without papilloedema.15
Sustained papilloedema eventually leads to optic atrophy and visual
loss. The visual loss tends to be rapid and with little warning.
Although visual loss is more likely to occur during the period of
rapid brain growth, there are several known cases of sudden visual
loss in young adults with craniosynostosis with no warning.16
Mental function with raised ICP
In summary the literature suggests that in single-suture synostosis
the rate of raised ICP is s and the incidence of cognitive disability
also appears high. Currently it cannot be proven that the raised
ICP causes cognitive dysfunction or that surgical intervention alters
cognitive outcome. 17
Prolonged elevated ICP is believed to be detrimental to mental
function however studies investigating the relationship are complicated
by the problem of objective testing particularly in young children.
A critical review of 17 studies investigating the relationship
between single-suture synostosis and neurodevelopment demonstrated
a three- to five fold increase in the risk for cognitive deficits
or learning and language disabilities. There is no difference between
the various sutures (metopic, coronal or sagittal) 18
Some of these studies included the following;
The Paris group showed (in 396 cases) that the main predictive
factor of mental outcome was the initial developmental level and
most patients had a good outcome whether or not they had surgery.19
A Harvard study (16 cases) showed 50% of patients with scaphocephaly
had reading or spelling disabilities. 20
In a more recent longitudinal study (84 children with single-suture
craniosynostosis) the incidence of mental retardation was two to
three times higher than normal. In those children who were not retarded
and had reached school age, 47% had learning difficulties. Rates
of retardation may increase relative to normal expectations as children
The London group showed no increased prevalence of global cognitive
impairment in scaphocephaly however there was an increased risk
of developing speech and language impairment. 22
The Oklahoma group (21 cases of single-suture synostosis) showed
43% were normal, 48% had mild delay and 9% significant delay. 23
While there are pathophysiological mechanisms to explain why brain
restriction may affect cognitive development currently there is
no conclusive evidence that shows surgery affects the ultimate intellectual
outcome. This may be due to limitations of current testing methods.
The effect of surgery on preserving mental function in single-suture
synostosis is controversial. While some studies have described a
possible deterioration in mental function if surgery is not performed,
other studies appear to show no increased incidence of developmental
delay in the absence of surgery.
Psychological sequelae of abnormal head shapes
There are few studies of large numbers of children who have grown
into adulthood with unoperated craniofacial deformities. The incidence
of severe psychological distress at some point during adolescence
is 35-45% for patients with cleft lip and palate. In a Swiss study
of unoperated scaphocephaly patients (30 children who had reached
an average age of 9 years), quality of life and psychological adjustment
was within the normal range. 24 For many years, the neurosurgical
team in Adelaide did not operate in cases of scaphocephaly believing
it to be only of cosmetic concern. 25 In the 34 cases studied there
was a high incidence of anxiety amongst parents and children. As
these patients have subsequently been followed into their 30s they
have had 3 times the expected incidence of relationship breakdowns,
difficulty maintaining employment and poor self esteem.16
From a practical point of view many occupations and recreational
activities require the use of hats, helmets, glasses and protective
eyewear. This may be difficult with cranial shapes well outside
the normal range.
Modern Craniofacial Surgery
Today craniofacial surgery is a mature discipline with acceptably
low rates of serious complications. In the Wellington and Christchurch
regions it is undertaken by a well trained team who have spent time
in international centres of excellence during their training. We
are one of the few centres in the world who currently offer spring
assisted skull reshaping to suitable patients, a novel technique
associated even lower complication rates.
Families face a difficult decision when choosing whether or not
to have surgery for their child with craniosynostosis. Where parents
are adamant that they do not want their child to undergo surgery,
we may recommend intracranial ICP monitoring to exclude the possibility
of raised intracranial pressure.
This information sheet is intended for patients of Mr Charles Davis.
It is a summary only and not an exhaustive review of this topic
and it is not intended to replace a full discussion about any patients
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