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Pediatric Craniosynostosis

Dr. Pincus and Peds Team

The Department of Neurosurgery at UF provides a full range of pediatric neurosurgical services.

  • Pediatric Neurosurgery

Several cases of craniosynostosis are treated each year at the University of Florida under the guidance of:


Drawing: Pediatric Skull Sutures

What is Craniosynostosis?

At birth the skull is made up of multiple bones held together by seams called sutures. The sutures should remain open as long as the brain continues to grow, enabling the skull to expand and properly accommodate the brain.s growth. Craniosynostosis is a congenital anomaly caused by early closure of one or more of these sutures, resulting in limited or distorted head grown. Craniosynostosis is also called synostosis or cranial stenosis. In rare cases, in addition to abnormal head shape, craniosynostosis may result in raised pressure inside the skull. Eighty to ninety percent of craniosynostosis cases involve a single suture, however craniosynostosis associated with a genetic syndrome may involve multiple sutures. Not all cases of multiple suture synostosis are associated with a genetic syndrome.


Types of Craniosynostosis


Drawing: Metopic CraniosynostosisMetopic synostosis

The metopic suture is located between the anterior fontanelle (soft spot) and the nose. Early closure of this suture results in trigoncephaly, or a triangularly shaped head. Infants will often have a prominent ridge down the middle of the forehead, a pointed forehead, and eyes that seem too close together.


Drawing: Saggital CraniosynostosisSagittal synostosis

This is the most common type of craniosynostosis. The sagittal suture is located between the anterior fontanelle and the posterior fontanelle. Early closure of this suture results in scaphocephaly, which literally means .boat shaped head.. Infants will often have a protruding forehead, an elongated head, and a bony protuberance at the back of the skull.


Drawing: Coronal Craniosynostosis

Coronal synostosis

Coronal synostosis may occur on either side or may be bilateral. Infants will have an elevation of the eye socket, flattening of the ridge of the eye and displacement of the nose on the affected side.


Drawing: Lambdoid Craniosynostosis

Lambdoid synostosis

Lambdoid synostosis is rare and is commonly confused with positional molding. It may occur on either side or may be bilateral.


What are common signs and symptoms of Craniosynostosis?

The most common sign of Craniosynostosis is an abnormal head shape. Routine skull x-rays and CT scans are not recommended, as most cases can be diagnosed by a neurosurgeon based on physical exam only. Symptoms are rare, but in some cases headaches can occur due to increased intracranial pressure.


What is the treatment for Craniosynostosis?

Most infants born with Craniosynostosis will need surgery to repair their skull deformity. Children with mild synostosis may not need surgery. There are two different surgical procedures to correct skull deformity.

Endoscopic synostosis repair involves one or two small incisions made near the fused suture. There is no need to shave any hair for this procedure. An endoscope, or small camera, is used to visualize the affected suture. Portions of the skull bone are removed over the fused suture through the small incisions. Most patients will have minimal eye swelling after the procedure. This procedure can be performed on patients up to 6 months of age. We have been using this technique for craniosynostosis since 2003. Less than 25% of our patients have required a blood transfusion with this procedure. The hospital stay is one night.

cranial molding deviceFollowing surgery patients are fitted for a cranial molding device (helmet) and will wear the helmet 23 hours a day for 9 to 12 months. Advantages of this procedure are less smaller incisions, less blood loss and a shorter hospital stay.

For more information, view this article, which appeared in Neurosurgical Focus: Endoscopic-assisted repair of craniosynostosis

 

Right coronal synostosis before and after endoscopic repair
Right coronal synostosis before and after endoscopic repair

Sagittal synostosis before and after endoscopic repair
Sagittal synostosis before and after endoscopic repair

Metopic synostosis before and after endoscopic repair
Metopic synostosis before and after endoscopic repair

Cranial vault remodeling is performed on infants with craniosynostosis. This procedure can also be performed on younger children. Surgical reconstruction is usually performed by our craniofacial team that includes Dr. David Pincus and Dr. Brent Seagle, a board certified plastic surgeon. An incision is made on the top of the head that runs from ear to ear. It is usually not necessary to shave hair for this procedure. The abnormal bone is removed and remodeled to create a normal appearing head. The bone is put back in place with screws and plates that will dissolve in 12 to 18 months. A small drain is left in place for several days. Most patients will have eye swelling that will resolve in 5 to 7 days. Approximately 60% of our patients will require a blood transfusion with this procedure. Most patients will spend 3 to 5 days in the hospital.

Metopic synostosis before and after Cranial Vault Remodeling
Metopic synostosis before and after Cranial Vault Remodeling

Children with a genetic syndrome, such as Apert.s or Crouzon.s Syndrome, need a genetic consult.


What to expect if my child needs surgery

  • Endoscopic Synostosis Repair
  • Cranial Vault Remodeling

For more information contact our office at 352-273-9000.


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