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Multisuture Synostosis

Multisuture Synostosis is the name for several rare types of a condition called craniosynostosis. These conditions cause the joints in the bones in a baby’s skull to fuse too early. These are congenital defects, and they cause problems related to bone growth and the shape of the heads of affected babies. This condition can happen all by itself, or it can be present along with other craniofacial syndromes. When not treated, this condition can cause head deformity, pressure on the brain, seizures, and developmental delays.

Symptoms

The symptoms of Multisuture Synostosis are:

  • Missing, full-feeling, or bulging fontanel on the newborn’s skull
  • Bony ridges along the edges of the fontanels that are affected
  • Abnormal appearance and development of the bones of the forehead, eye sockets, cheekbones, and the jaw
  • Prominent blood vessels in the scalp
  • Poor feeding
  • Projectile vomiting
  • Seizures

Causes & Risk Factors

The causes of this condition are not well understood. Genetic disorders in other parts of the body can cause this condition to be present, but some patients do not have other genetic problems and only have Multisuture Synostosis. It is thought that a combination of genes and environmental factors leads to this condition.

The risk factors for having Multisuture Synostosis are having conditions like Carpenter Syndrome, Crouzon Syndrome, Pfeiffer syndrome, or Saethre-Chotzen Syndrome.

Diagnosis

There are various kinds of Multisuture Synostosis. When a diagnosis is made, the exact type of Multisuture Synostosis will be confirmed.

  • Sagittal – the premature fusion of the sagittal suture that runs across the top of the skull
  • Coronal – a premature fusion of one of the coronal structures that extend from the ear to the top of the skull
  • Metopic – a premature fusion of the suture that runs from the top of the bridge of the nose through the midline of the forehead
  • Lambdoid – the premature fusion of the lambdoid structure along the back of the head. This is the rarest of the various kinds of Multisuture Synostosis.

The diagnosis process typically begins when parents or doctors notice that the child’s head is not growing evenly or is beginning to appear crooked or lumpy. In some cases, the roughness at the edges of the fontanels is felt first before there are changes to the appearance of the skull.

Imaging studies will be necessary for these patients, and MRI and CT scans are usually both ordered. The absence of visible seams in the skull will often verify the location of the defect and confirm the diagnosis of this condition. Genetic testing can also help confirm a diagnosis of Multisuture Synostosis.

Treatment & Prevention

Mild cases of these conditions will usually not require medical intervention or treatment. Your child might need to wear a protective helmet to help shape the head during the early years of development. Surgery is often the primary treatment for this condition for more serious cases. Some patients will require more than one surgery to correct the defects caused by Multisuture Synostosis.

Surgery can be done through endoscopy, but more serious defects will often require open surgery. Open surgery is typically only done on patients who are six months of age and older. Helmet therapy might still be necessary following surgical intervention.

Prevention of these conditions is not usually possible. Genetic testing that is completed before a woman becomes pregnant might show genes that indicate a risk of the related conditions that can lead to Multisuture Synostosis. Genetic counselling can let parents know how likely it will be that any children they have will have Multisuture Synostosis.

 
 
 

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