Coronal Synostosis Facts

The coronal  sutures are located on either side of  the head and adjoin the soft spot in the middle. Their function is to allow the frontal lobes of the brain to grow and move forward. This in turn allows the forehead, eyes, eyebrows and nose to also move forward and downward. Premature closure results in a number of deformities related to these structures' inability to advance normally.

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Side view of the newborn skull shows the location of the left coronal suture highlighted in red.

Top view of the skull depicts the  location of the coronal sutures on either side of the soft spot and extending to the sides.

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Coroanl skull AP
Coronall skull AP

The premature closure of the right coronal suture prevents the front right side of the skull from moving forwards (red arrows) leading to flattening of the right side of the forehead. The brain takes the path of least resistance and begins to grow  more on the left side (green arrows) leading to frontal bossing of the right.

Front view of a newborn skull with right coronal synostosis. The right eye socket is elevated and pulled upwards giving the appearance on skull x-rays of the so called "harlequin eye".

The nose is pushed to the opposite left  side (green arrow), the right eye socket is elevated and moves higher than the left one (red arrow), the right side of the face is pushed inwards (blue arrows) while the left  side of the forehead is pushed outwards (yellow arrows).

Presentation

The classical presentation consists of coronal craniosynostosis consists of :

1. Elevation of the eye on the affected side (vertical dystopia)

2. Deviation of the nose to the opposite side

3. Flattening of the forehead on the affected side (frontal plagiocephaly)

4. Protruding eye on the affected side (proptosis)

5.  Overgrowth of forehead on the opposite side.

6.  Head deviates to the side (cranial scoliosis)

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Two month old male with significant cranial-facial deviation (scoliosis) as evidenced by the angulation of a line going from the root of the nose to the mid chin and top of the head as seen in coronal synostosis

Cranial scoliosis (red line) and vertical dystopia (left eye elevated above right eye) as demonstrated by the black line in infant girl with left coronal synostosis.

Radiology

Metopic synostosis is a clinical diagnosis, meaning that it is made by examining the patient and identifying the associated deformation of the head and face. CT  scans and X rays are not necessary to make the diagnosis. However, the images clearly show the skull changes related to this condition.

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CORONAL CT AP 2

3-D CT  reconstruction scan of a 2 month old male with left coronal synostosis. Elevated left orbit and deviation of nose to the right are noticed.

3-D CT reconstruction of 6 week old female with right coronal synostosis. Also seen are the nasal deviation and vertical dystopia (uneven orbits)

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Skull radiograph of 2.5 month old female who presented with left coronal synostosis. The "harlequin" sign is seen on the left orbit.

Top view of a 3 month old female with right coronal synostosis. The fused coronal suture is evident on the right side leading to the anterior fontanelle (soft spot).

Endoscopic Surgery

The goal of the surgery is to simply release and open the closed suture to allow the brain to resume its normal growth pattern and revert to a normal shape. Since the brain of an infant grows very rapidly, doubling in size during the first year of life, performing the procedure at an early age is of utmost importance. By using minimally invasive, endoscopic assisted techniques, such procedure can be done safely in very young babies. Once released, normalization of the head is aided with the use of custom made helmets (cranial orthosis) during the following year.

 

 

Positioning in Surgery

The patient is placed flat on the operating table (supine position) witht he head being placed on on a specially designed head holder.

Coronal position

Skin Incision

A single small (inch or less) incision is placed behind the hairline and on the side of the closed suture. Access to the entire suture is obtained by mobilizing the scalp with the aid on an endoscope and lighted scalp retractors.

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Correction of the vertical dystopia and the harlequin sign after endoscopic treatment of left coronal synostosis

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Day of Surgery

Two months after surgery

Six months after surgery

RESULTS 

Number of Patients:                                         150

Time Range:                                 March 1997 to September 2018

Average Blood Loss:                                         35 ml's

Average Blood Transfusion Rate:                       2.1%

Average Length of Hospitalization:                1.0 days

Average Surgical Time:                                 45 minutes

Number of Re-operations:                                None

Number of cases converted to CVR:                None