What Is Craniosynostosis?
The word "craniosynostosis" refers to a type of birth defect that involves the premature fusion, or closure, of one or more “seams” on a baby’s skull. These seams, or what surgeons call sutures, connect individual skull bones. When these sutures are open, or patent, they allow the skull to expand evenly when the brain grows in infancy. When a suture has closed too early, the skull cannot expand in a uniform fashion. In some cases, this may lead to increased pressure on the brain. This is called elevated intracranial pressure, which can produce signs and symptoms such as headache, irritability, visual changes and developmental delay. Elevated intracranial pressure occurs approximately 13 percent of the time when a single suture is affected, and up to 42 percent of the time when multiple sutures have fused, or closed, early.
Trigonocephaly, a fusion of the forehead suture, is one form of craniosynostosis.
The Importance of a Proper Diagnosis
Craniosynostosis is usually diagnosed by a specialist experienced in examining a child’s head shape, such as a pediatric neurosurgeon or pediatric plastic surgeon. The diagnosis is often made using special X-ray tests, such as computed tomography (CT) scans, which can confirm the diagnosis by revealing the fused suture.
Proper diagnosis is important because craniosynostosis should not be confused with plagiocephaly, a term that refers to a crooked head shape without a fused suture. This distinction is critical because craniosynostosis is usually treated with surgery, whereas plagiocephaly is treated with nonsurgical therapy.
Treatment Options for Craniosynostosis
If surgery is recommended for your child, our specialists will present a detailed discussion of the treatment options with you. Typically, surgery is performed in infancy (at 3 to 9 months of age), depending upon the technique. Our priority is to perform surgery when it is safest for your child and to minimize surgical and postsurgical risks.
Here are some of the surgical options available:
- Modified Strip Craniectomy
This surgery involves removing the affected suture as a strip and making special cuts on the bones of the skull to allow the skull to reshape itself. The surgery may involve a conventional technique using a bicoronal (ear-to-ear) incision or an endoscopic technique, which is performed through a limited incision (see below). The advantage of a modified strip craniectomy is that it requires less time than other techniques, such as the calvarial vault reshaping technique (see below). This means patients have less bleeding and a shorter hospital stay. The disadvantage is that the modified strip craniectomy may only be effective for certain types of craniosynostosis, such as the sagittal type (front-to-back suture), and may require more extensive surgery later on. For this operation to be effective, the surgery should be performed no later than 3 months of age. After surgery, patients must wear a helmet for several months to help mold the head.
- Calvarial Vault Remodeling
This technique involves temporarily removing the skull bones (called a craniotomy), reshaping them, and replacing them to form the skull in their new shape and arrangement. The reshaped bones are secured using synthetic plates and dissolving screws, and sometimes dissolving sutures. A clear advantage of this versatile type of reconstruction is that it allows the surgeon to precisely reshape an area of, or the entire, skull. Also, the bones are reshaped in one surgery, and no helmet is required after surgery. The disadvantage of this approach is that it requires a large, bicoronal incision and more operative time, and results in more blood loss and a longer hospital stay. This surgery is usually performed after 6 months of age, when a child’s skull bones have grown thicker. In some cases, however, this type of surgery may be performed between 3 to 6 months of age.
- Endoscopic Surgery
In this operation, a special "telescope" called an endoscope is used to help the surgeon see the operative field inside the skull. It can be combined with a strip craniectomy technique, but not with the remodeling technique. This allows the surgeon to make one or two small incisions in the scalp, thereby avoiding the large, and sometimes noticeable, bicoronal scar. This is the main advantage of this technique. However, this less invasive surgery may be less precise and also does not allow the surgeon to fully control bleeding in areas of the skull. Like the modified strip craniectomy surgery, endoscopic craniectomy requires a helmet after surgery and should be performed even earlier, by 3 months of age. The long-term aesthetic outcomes from this type of surgery are largely unknown, compared with more traditional techniques, such as cranial vault reshaping.
After the surgery, your child will be admitted to a pediatric intensive care unit (PICU) for dedicated nursing care and monitoring. Depending on the length of surgery, your child’s surgeon and anesthesiologist may choose to keep a breathing tube in as a precaution. In such cases, the breathing tube will be removed when deemed safe by one of the intensive care physicians.
Your surgeon will take off your child’s wound dressing and drain care. Usually, a postoperative CT scan is needed to check the skull. A typical hospital stay ranges from three to five days.
When your child is discharged from the hospital, you will receive printed instructions with information on caring for your child’s incision(s) at home, your child’s medication regimen, and contact information in case of an emergency or questions. Further instructions will be provided at your child’s first postoperative outpatient visit, which is typically two to three weeks after surgery.
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