Information on Fontanelles Separation Surgery

I had Fontanelles Separation Surgery in 1964 when I was 2 years old... My Mom was fond of telling me throughout my childhood and beyond that "we ate chicken" to pay for my operation and that if I didn't have the operation that I would have grown up retarded and died by the time I was 25.........

Definition of Craniosynostosis

A congenital (present at birth) disorder in which premature closure

of the sutures (gaps) on the skull results in an abnormally shaped

head. The severity of symptoms and shape of the skull depend on which

bones are affected. Synostosis means a union of adjacent bones.

Premature ossification of a suture, or synostosis, is sometimes

attributed to intrauterine restraint of head growth by twinning

or by early engagement of the head in the pelvis.

It is commonly seen in craniofacial disorders such as Crouzon, Apert,

Pfeiffer, or Saethrre-Chotzen syndromes, or in the setting of fetal

exposure to phenytoin, valproic acid, or other teratogens. Most often,

however, there is no identifiable cause. Sporadic cases typically

involve a single suture; syndromic cases typically demonstrate more

complex, multi-sutural involvement, often bilateral coronal synostosis.

Synonyms include: craniostenosis, plagiocephaly, scaphocephaly, and trigonocephaly.

Description of Craniosynostosis

Craniosynostosis consists of deformities of the skull resulting

from premature closure of the gaps, or sutures, between the skull

bones.

Most cases of craniosynostosis occur in a family with no history

of the condition and children with craniosynostosis are otherwise

healthy and have normal intelligence.

Normally, the bones of the skull are not joined at birth, allowing

the head to grow evenly. In individuals with craniosynostosis, the

sutures where the skull bones meet have closed, or close prematurely.

As a result, the expanding skull bones grow abnormally, and the

abnormal skull shape becomes more pronounced as the infant grows.

The shape is dependent on which sutures have closed, and various

abnormalities have specific names.

Acrocephaly, oxycephaly, turricephaly denote a pointed

(high) head, caused by the premature closure of all sutures.

Brachycephaly denotes an abnormally short, squat skull,

caused by the premature closure of the two coronal sutures,

which cross the top front portion of the skull, width-wise.

Dolichocephaly and scaphocephaly denote an abnormally long

front-to-back distance of the skull, caused when the sagittal suture,

which runs lengthwise along the top of the skull, is closed.

Sagittal synostosis (scaphocephaly) is the most common type.

It affects the main (sagittal) suture on the very top of the head.

The early closing forces the head to grow long and narrow, instead

of wide. Babies with this type of craniosynostosis tend to have a

broad forehead. It is more common in boys than girls.

Plagiocephaly denotes a somewhat lopsided, asymmetric, pointed

appearance, caused by premature closure of sutures that cross the

top of the skull widthwise (coronal sutures in the front, lambdoidal

sutures in the back), on only one side.

Trigonocephaly denotes a triangular shape at the top of the

skull, caused by the closing of the metopic suture, which runs

lengthwise along the top front of the skull, forward of the sagittal

suture and anterior fontanelle (the "soft spot" at the top front

portion of an infant's skull).

The expansion of the head is driven from within by growth of the

brain, and craniosynostosis causes deformity by restricting expansion

of the head in the dimension perpendicular to the affected suture.

Compensatory pressure by the growing brain results in expansion, or

bossing, in adjacent areas of the skull. The characteristic patterns

of deformity caused by synostosis of each individual suture are

readily recognized by the trained eye. Thus, physical examination

is the gold standard for evaluation of this problem.

Misinterpretation of skull radiographs and computed tomographic

(CT) scans is a common source of confusion and alarm for parents.

CT scanning of the skull and brain has a role principally for

surgical planning and to rule out any associated cerebral abnormality

or hydrocephalus, which are extremely uncommon in sporadic synostosis.

Without surgical intervention, the skull deformities caused by

craniosynostosis persist through life. Adults with untreated

craniosynostosis are easily recognizable. Sagittal synostosis

causes elongation and narrowing of the skull that can attain

grotesque proportions in childhood and are only partly mitigated

by normal enlargement of the facial skeleton and thickening of the

neck muscles later in life. Unilateral coronal synostosis causes

asymmetry of the forehead and orbits and rotation of the nose

that do not disappear with maturation. The natural history of

nonsyndromic metopic synostosis is uncertain. Older children

and adults with trigonocephaly are not seen, and some surgeons

have abandoned the treatment of this condition.

Of greater concern to parents than the persistence of deformity

into adulthood are social issues during the school years.

Children who are different in any way tend to become the objects

of ridicule and ostracism by their peers, and the skull deformities

caused by craniosynostosis do not escape the attention of schoolmates.

Parents are usually willing to subject their infant children to

surgery in order to remove obstacles to social integration, but

such an indication for surgery places heavy demands on the surgical

team with respect both to safety and aesthetic results. Treatment of craniosynostosis requires not only surgical expertise but also the

highest quality support in pediatric anesthesia and pediatric

intensive care.

The goal of the modern surgical treatment of craniosynostosis is

immediate correction of the skull deformity. In the past surgical

intervention was limited to synostectomy, so-called "reopening" of

the affected suture. The rationale for this approach was that removal

of the synostotic constraint on skull development would allow

subsequent brain growth to remodel the skull. Treatment by

synostectomy put a very high premium on early diagnosis, in order

to take maximal advantage of remaining brain growth potential.

However sound the theory may have been, the results were poor,

and contemporary surgeons have abandoned synostectomy in favor

of a variety of techniques for active remodeling of the skull.

Patients now look better in the recovery room. This shift in

surgical philosophy has been boosted by an explosion of new

instruments and materials that have allowed intervention not

at the earliest possible moment but at the point in skull

development mechanically most satisfactory for reconstruction.

Postponement of surgery until 6 to 9 months of age increases

the margin of safety as well.

Causes and Risk Factors of Craniosynostosis

Craniosynostosis is estimated to occur in one in 2,000 live births.

The cause is unknown in most children. However, genetic syndromes

account for 10 to 20 percent of cases.

More than 50 craniosynostosis syndromes and more than 20 conditions

in which craniosynostosis is a secondary or occasional feature have

been described.

Craniosynostosis should not be confused with much more common, mild

changes in skull shape that result from prolonged periods in one

position. This condition is called positional skull flattening.

With the current recommendation for infants to sleep on their backs,

some infants develop a flattened occiput (back of the skull).

Similarly, some children develop lateral flattening if they lay

on their side too long without rotation. Parents should change

the infant’s position while awake to reduce the risk of flattening.

This is a benign condition and rarely requires any intervention

except change in position more frequently and occasionally a helmet.

Symptoms of Craniosynostosis

There is rarely any neurologic deficit. Mental retardation may occur

in these disorders and is more likely in cases where the closure

of the sutures is greatest.

Diagnosis of Craniosynostosis

Most of the cases are evident at birth, but they may also be

identified at routine “well” child examinations during the first

year of life. Compute Tomography (CT scanning) can identify the

abnormal suture more effectively than standard plain film radiographs. Furthermore, the CT scans can be used to develop a full picture of the abnormality though three dimensional reconstructions.

Treatment of Craniosynostosis

Among the majority who do not exhibit neurologic complications or

mental retardation, there are few complications caused by this

disorder other than cosmetic and sociopsychological problems. The craniosynostoses may be treated surgically by removing the affected

suture(s). These are complex procedures that require extensive

planning, employing CT scan three-dimensional reconstructions and a

team of physicians including neurosurgeons and anesthesiologists.

Correction is usually performed in the first year of life.