Scaphocephaly: What is it? Symptoms, Causes, Diagnosis and Treatment

Also called sagittal synostosis, it is the most common type of craniosynostosis, which occurs when the bones of a baby’s head fuse abnormally.

The shape of the head

One of the most common signs of scaphocephaly is an elongated head shape.

The sagittal suture is the long suture that runs from the front to the back of the head, and if this suture fuses too quickly, the result is a long, thin head shape.

Pronounced (scafe-oh-kef-all), the name scaphocephalic is derived from the Greek word shape, which means light rowboat or skiff and kephale meaning head.

Together, the term describes a long, narrow head shape that resembles the bottom of a boat.

Other common signs of scaphocephaly are an eyebrow developed on the front and a very developed occipital area on the back of the head.

Symptoms

Scaphocephaly is typically a head shape that is elongated and relatively narrow. The head will appear longer and thinner from the side and top view.

 

The forehead will appear bulbous from the front view, while the back face will be narrower. Also, if you run your hand over the top of the head, you can feel a ridge.

This is due to the premature fusion of the suture in the upper part of the head, also known as a sagittal suture. In more severe cases, the asymmetry of the face may also be present.

What is sagittal craniosynostosis?

Sagittal craniosynostosis, or scaphocephaly, occurs when certain bones in a child’s skull fuse prematurely.

At birth, a child’s skull comprises several separate bones with growth plates (also called ‘sutures’) between them.

The brain can grow and expand in size because the skull is not yet a solid piece of bone. Typically, the sutures of the skull close during adulthood.

In some children, the bones of the head fuse earlier than they should. Sagittal craniosynostosis refers to the premature closure of the suture from front to back in the upper part of the head.

Babies usually identify with irregular forms of the head shortly after birth. When this happens, the bones put pressure on the brain and can prevent it from growing normally.

Scaphocephaly also causes a child’s head to look deformed. We recommend that parents treat their children early, preferably before six months.

Causes

Scaphocephaly can occur in a healthy baby for no known reason. Scaphocephaly has also been linked to:

  • Apert syndrome.
  • Síndrome de Beare-Stevenson.
  • Crouzon’s syndrome.
  • Jackson-Weiss Syndrome.
  • Muenke syndrome.
  • Pfeiffer’s syndrome.

Scaphocephaly, also called dolichocephaly, is a congenital disability characterized by an asymmetric distortion or shape of the head.

Scaphocephaly frequently occurs in premature babies. Cases of scaphocephaly that start in the uterus may result from some factors, including the position of the baby’s head during pregnancy.

The transport of multiple (twins, triplets) where there is less space for each baby to grow and often the heads are forced against the pelvis or the mother’s ribs for a long time a small or deformed uterus, complications during childbirth.

The leading cause of scaphocephaly is the result of external forces due to pressure on the sides of the skull in the first months after birth.

It is commonly seen in babies who spend time in the neonatal intensive care unit (NICU). Lateral positioning is standard in the NICU to provide easy access to monitors.

When the leading cause of scaphocephaly comes from internal forces, it is called sagittal synostosis.

Sagittal synostosis results from premature fusion of the sagittal suture; the suture extends anteroposteriorly along the top of the head.

When the sagittal suture closes prematurely, it does not allow average growth of the head.

Therefore, growth will be limited transversally, and the head will grow more anteroposteriorly and remain narrow. It is the most common form of synostosis, with statistics ranging from 1 in 2000 to 1 in 5000 births.

Treatment

Traditional surgery for sagittal craniosynostosis involves the release (reopening) of the fused suture and enlargement of the skull by opening the coronal and lambdoid sutures.

When bones are prematurely fused, surgery is the only way to separate them. Skilled surgeons offer scaphocephaly surgery, both open and endoscopic. Your surgeon will recommend the most appropriate type of surgery for your child.

The types are:

  • Repair of endoscopic craniosynostosis.
  • Repair of open craniosynostosis.