Craniosynostosis is a condition in which the bones of the skull fuse together prematurely. Our skulls are not made up of one single bone.  Rather, several different bones fit together to make up the skull. The areas where the bones meet one another are called "sutures" .  As a baby grows, the brain rapidly increases in size.  The growing brain pushes on the bones of the skull causing the skull bones to expand or grow. When one of these sutures is fused too early, growth is restricted in that area. This growth restriction in one area may lead to overgrowth in other areas. This process results in an abnormally shaped skull. Different suture fusion patterns lead to predictable head shapes: Scaphocephaly, plagiocephaly and trigonocephaly are the most common. "Plagiocephaly", from the greek meaning "flat head", can be a confusing term because it does not necessarily result from craniosynostosis. Any asymmetric flattening of the forehead or back of the head should be examined by an experienced craniofacial plastic surgeon, neurosurgeon or neurologist so that deformational plagiocephaly can be differentiated from true craniosynostosis.

Drawing of a skull demonstrating the various sutures that may be affected by craniosynostosis. Each pattern will result in a characteristic head shape. The shape may be symmetric or asymmetric

True craniosynostosis requires surgery for correction while deformational plagiocephaly, if addressed early enough, will rarely require surgery.

James T. Goodrich, M.D., PhD is the Director of Pediatric Neurosurgery. He is internationally recognized pediatric neurosurgeon. His office telephone number is (718) 920-4197. You can also visit his website by clicking here.

Ira Richmond Abbott, M.D. is an internationally recognized leader in Pediatric Neurosurgery. He recently joined the faculty at the Children's Hospital at Montefiore and we are fortunate to have his expertise available to us at The Center for Craniofacial Disorders. Read aqbout him here. He can be reached at (718) 920-7400.

Scaphocephaly (Sagittal Synostosis)

Scaphocephaly is an early closure of fusion of the sagittal suture. This suture runs front to back, down the middle of the top of the head. This fusion causes a long, narrow skull. The skull is long from front to back and narrow from ear to ear.

Plagiocephaly (Unicoronal Synostosis)

Plagiocephaly results when one of the coronal sutures fuses prematurely. The coronal sutures travel across the top of the skull. The soft spot (fontanel), is located between the right and left coronal sutures. When viewed from above, the forehead on the affected side is flat and recessed when compared to the other side which may bulge further forward than normal. This happens to compensate for the brain's inability to grow on the flat side. The fused suture prevents forwrd growth of the brain on the flat side. Looking at the child's face, the brow on the affected side is usually higher than the other side, and the tip of the nose may appear to be deviated to the other side. Plagiocephaly is estimated to occur in about one in 3500 births.

Almost all children affected with plagiocephaly require surgical treatment. There are a few surgeons who are treating this condition with an endoscopic procedure and then having the child wear a helmet or headband for as long as one year. This procedure is often referred to as "minimally invasive" but is, nevertheless, a significant surgical procedure. Dr. Goodrich and I have each helped to advance and develop endoscopic techniques in craniofacial surgery, and I have been involved in the development of endoscopic techniques in plastic surgery (review abbreviated curriculum vitae), we do not believe that this is the best treatment for this craniosynostosis, and we do not perform it in any patients. We believe that spending an additional 45 minutes in the operating room prevents the need for a child to wear a headband or helmet, and this slightly longer procedure may actually put your child at less risk, with better results. Removal of a fused suture, leaving a large area of brain unprotected, is an old procedure and has been associated with relapse or recurrence of synostosis with an abnormal headshape in about 85% of cases. Endoscopic removal of a fused suture is basically the same but is able to performed through two or more incisions, and the head-shape is corrected through the use of a molding helmet. The technique that we utilize allows us to reshape the head directly, and not leave any area of the brain unprotected by the child's own bone. These issues should be discussed in greater depth with your craniofacial surgeon.

In general, a single operation is all that is necessary to treat this condition. A very small percentage of children end up undergoing a second smaller operation later in life to correct residual deformities. It is unclear whether or not this is due to an inadequate initial correction, an inherent growth disturbance (the bones are not growing normally afterwards), or a combination of the two.

Trigonocephaly (Metopic Synostosis)

Trigonocephaly is a fusion of the metopic (forehead) suture. This suture runs from the top of the head down the middle of the forehead, toward the nose. Early closure of this suture may result in a prominent ridge running down the forehead. Sometimes, the forehead looks quite pointed, like a triangle, with closely placed eyes (hypotelorism).