What is Craniosynostosis (CRAY-nee-o-SIN-os-Toe-sis)

It is very common for a newborn baby to have a misshapened

head immediately after birth. This can be caused by the position
of the baby in the womb or by the pressure of the birth canal
during delivery. In these cases, the shape should begin to resolve
in the first couple of days of life. A persistent misshapened head
should be evaluated for craniosynostosis, a relatively common
condition occurring in as many as one in one thousand births.
To understand craniosynostosis, its important to first appreciate
the changes happening inside a newborns brain. Immediately
following birth, the babys brain undergoes an extremely rapid
phase of growth and development. It is calculated that the
volume and size of the newborns brain will double in size in nine
months and triple in size in 36 months. In order to accommodate
such rapid brain growth, your babys skull cap must expand
rapidly as well. Rather than being one single large piece of bone,
his/her skull is made up of several bones (frontal, parietal,
occipital, squamosal) which are held together by fibrous-like
hinges called sutures. These sutures respond to brain growth by
stretching and producing new bone, thereby allowing the skull
to grow along with the underlying brain.
Should any of these sutures close or fuse before birth or soon
after, while the brain is growing, craniosynostosis occurs. The
term cranial stenosis and simply synostosis are used to describe
this condition. Because the brain is normal, it continues to grow
at its programmed rapid rate. However, the closed suture delays
proper and parallel bone growth which leads the brain to take the
path of least resistance and ultimately the shape of the brain,
skull and even face become distorted. Each suture premature
closure will lead to a specific abnormal head shape and unique set
of problems.
Misshapened Head
If you are concerned about your newborns misshapened head,
we are happy to provide an initial opinion at no-cost through
email with photographs. Please contact us for instructions.
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Types of Craniosynostosis

Premature skull suture closure can be classified in several ways. Single suture synostosis refers to a condition where only one suture is affected, all others
are open and typically the babies are otherwise normal. Multiple synostosis can involve the closure of one, two, three or more sutures. If only the sutures are
closed and no other problems can be found, then the classification of non syndromic multisuture synostosis is applied. If several sutures are closed and
other well-known systemic problems are found, then the term syndromic multisuture synostosis is used. Common syndromes include: Crouzon, Apert, and
Pfeiffer.
Single Suture Synostosis Sagittal
The sagittal suture is located on the midline, on top of the head and extends from the soft spot towards the back of the head. When the head is
palpated, a ridge can be felt along the suture. Because the skull does not grow properly from side to side, the brain grows forward towards the forehead and
backwards (Sagittal Suture Craniosynostosis Case 1). As a result, the head has marked forehead protrusion and bulging (Sagittal Suture Craniosynostosis
Case 2). It becomes long and narrow; the back of the head becomes prominent and pointed. Often times the top of the head arches into a point and the
temples become pinched in and narrow (Sagittal Suture Craniosynostosis Case 3). The overall shape is known as scaphocephaly . This is the most common
type of single suture synostosis and most commonly affects males. The majority of cases are "sporadic," meaning they do not exhibit a specific pattern of
inheritance.
View video animation of the treatment Sagittal Suture Synostosis
Coronal Suture Synostosis
The coronal suture is located on the side of the head and extends from the soft spot to an area just in front of the ear. It allows the forehead
and the frontal lobe to grow and expand forward. When the suture closes prematurely, the condition is known as anterior plagiocephaly . On the side of
involvement, the following deformities develop: the forehead is recessed and flattened; the eye socket is elevated and tilted (known as vertical dystopia );
the eyeball and eyelids protrude (proptosis ); the nose deviates to the opposite side; theres deviation of the top of the head in relation to the face and the
baby tends to tilt the head to the side in order to prevent him/her from seeing double if untreated (Coronal Suture Craniosynostosis Case 4).
View video animation of the treatment Coronal Suture Synostosis
Metopic Suture Synostosis
This midline suture is located in the middle of the forehead and extends from the soft spot to the root of the nose. It allows both frontal lobes to
expand forward and sideways as well as the eye socket to move to either side. Premature closure leads to the condition known as trigonocephaly . The baby
develops a midline ridge and the forehead slants sharply backwards giving the forehead the appearance of a triangle when viewed from above (Metopic
Suture Craniosynostosis Case 6). The eyes are too close together, a condition known as hypotelorism . The eyes and eye lids bulge forward (proptosis ) and
the back of the head protrudes markedly as the brain grows in that direction.
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craniosynostosis/#sthash.HjaEvi40.dpuf
Lambdoidal Suture Synostosis
Premature closure of the lambdoid suture is the least common of
all and has been frequently confused with positional molding of
the head. Closure leads to posterior plagiocephalus with flattening
of the back of the head on the affected side, protrusion of the
mastoid bone and lowering of the affected ear. It may also cause
the skull to tilt sideways.
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craniosynostosis/#sthash.HjaEvi40.dpuf
Traditional Treatment

Surgical treatment of craniosynostosis began in the 1890's and multiple procedures have been developed and advocated for the treatment of this condition.
In modern times, surgical correction of craniosynostosis is carried out by surgical craniofacial teams performing a variety of procedures which often times
leads to successful correction of the associated deformities.
The "traditional" operation is carried out by making a scalp incision from ear-to-ear, mobilizing the scalp to expose the skull, total or sub-total skull removal,
which is followed by reshaping and replacement of the skull with a variety of materials.
Surgery typically takes several hours (3-7), and often requires blood transfusions with hospitalization of three-five days. Extensive postoperative swelling is
often seen and can be associated with some pain and discomfort.
Resection or removal of the affected suture has been tried in the past with mixed results. Although results with these procedures have indeed improved, the
patients have paid a higher price in terms of increased swelling, pain, bleeding, blood transfusions rates, increased complications and longer hospitalizations.
Even though we have performed traditional surgery for many years with very good results, our goals have been to decrease complications, surgical trauma
and need for transfusions while obtaining excellent results that compare favorably or are even better than traditional surgery results.
- See more at: http://www.craniosynostosis.net/traditional-
treatment/#sthash.yTGCF9ly.dpuf
Endoscopic Craniectomy (Craniosynostosis Surgery)

A Less Invasive Technique for Babies with Craniosynostosis
The basic and fundamental principle with our treatment approach is to operate on the patient with craniosynostosis as early as possible. Best results are obtained when the baby receives the procedure by 12 weeks of age.
However, very good outcomes can be obtained for older babies, with appropriate postoperative helmet therapy.
Our approach is to release the prematurely closed suture and to allow the rapidly growing brain to remodel the skull and face to a normal shape. Depending on which suture is affected, our procedures have been designed to
provide the most optimal results. Following surgery, the desired shape of the head and face is attained with the use of custom made helmets that are worn continuously over the ensuing months.
SAGITTAL SUTURE SYNOSTOSIS
The endoscopic treatment of sagittal craniosynostosis is done via two small incisions. One is placed behind the anterior fontanel (soft spot) and the other is placed further back on the head. The endoscopes are used to expose
the areas above and below the skull, including the affected suture. Also, with endoscopic aid, the involved bone is removed, thereby freeing the brain and the skull to expand normally.
CORONAL SYNOSTOSIS
Dissection endoscopic release of the closed coronal suture is performed via a small single incision located halfway between the soft spot and the ear on the involved side. In all cases, only a very small amount of hair is
removed. The stenosed suture is resected with bone cutting scissors and instruments. Unlike traditional treatment, facial and orbital swelling is not seen and minimal pain is experienced by the patients.
METOPIC SUTURE SYNOSTOSIS
The treatment of metopic suture synostosis is done via a single incision placed behind the hair line and across the mid-line. The endoscopes are used to elevate the scalp over the suture from the anterior fontanel down to the
root of the nose (nasion). Once a small opening is made on the skull, the endoscopes are inserted under the bone and used to visualize the bone under the affected suture. A small strip of bone (0.7mm) is typically removed
from the anterior fontanel to nasion, thereby releasing the closed stenosed suture.
LAMBDOID SUTURE SYNOSTOSIS
For releasing stenosed lambdoid sutures, two 1" incisions are made in the back of the head. One is made over the midline and the second one behind the affected ear. In a similar fashion, the stenosed lambdoid suture is
removed with the aid and visualization of an endoscope. As with other sutures, the incisions are closed with subcutaneous absorbable sutures which do not require subsequent removal.
View a presentation on our approach to
Endoscopic Treatment of Craniosynostosis.






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Post-Operative Cranial Remolding Orthosis (Cranial Helmets)

Your infants cranial helmet treatment will be provided by the STAR Cranial Center of Excellence. The STAR Cranial Center of Excellence exclusively utilizes cranial helmets manufactured by Orthomerica
Products, Inc, the largest cranial remolding manufacturer in the world. Currently, Orthomerica is one of only two manufacturers
that have FDA clearance to fabricate cranial helmets. These highly specialized custom cranial helmets require multiple design options, clinical expertise, and technology in order to achieve optimal clinical outcomes for this vulnerable
patient population. The STAR Cranial Center of Excellence has a working relationship with UT Health
Science Center (UTHSC) but is not associated with UTHSC.
The custom post-operative cranial remolding orthosis (cranial helmet) is a Class II device regulated by the FDA, which requires
stringent quality, safety, and labeling information. Cranial helmets can only be manufactured by companies that have 510k clearance from the FDA. In June of 2009, Orthomerica Products, Inc was awarded FDA clearance for its cranial
helmets. It is important to note that the FDA does not consider these devices to be investigational.

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