Single-suture Craniosynostosis factsheet
Introduction
Craniosynostosis is when one of the flexible bands of tissue, or sutures, in a baby’s skull closes too early. Sutures normally stay open at birth to make it easier for your baby’s head to pass through the birth canal and allow the skull to grow as the brain develops. When a suture closes too soon, it can cause:
- changes in the shape of the skull
- pressure inside the head
- developmental delays.
The most common form is single-suture craniosynostosis, which happens when only one suture closes early. It usually happens on its own and is not linked to genetic syndromes.
Signs and symptoms
The signs of single-suture craniosynostosis depend on which suture has closed:
- Sagittal Suture - runs from the front soft spot to the back of the head. If it closes too early, the head becomes long and narrow, a shape called scaphocephaly. This is the most common type.
- Metopic Suture - runs from the top of the head to the nose. If it closes too early, it causes a pointed, triangular-shaped forehead and eyes that appear close together. This is called trigonocephaly. Trigonocephaly is different from metopic ridging, which is a temporary ridge on the forehead.
- Unicoronal - runs from ear to ear. If one side fuses early, it causes an uneven forehead. One side may look flat while the other bulges. The eyebrow may lift, and the nose may tilt.
- Lambdoid - found at the back of the skull. If it closes too soon, it can cause uneven flattening at the back of the head. This is different from positional plagiocephaly.
Diagnosis
A specialist doctor can diagnose single-suture craniosynostosis with:
Treatment
Single-suture Cranial Synostosis is usually treated with surgery to help reshape the skull and, in some cases, relieve pressure inside the head. Surgery is recommended for most children to prevent the skull shape from worsening as they grow.
The type of surgery depends on your child’s age and the specific condition. There is no medical treatment for this condition.
Children may not need surgery if they:
- are diagnosed late and have no signs of pressure in the skull
- have a very mild case.
Strip craniectomy
Strip craniectomy involves removing a small strip of bone from the closed suture to allow the skull to grow properly.
The surgery takes less than an hour, and children usually go home the next day.
This surgery works best for single-suture craniosynostosis and is most effective for babies between 2.5 and 4 months old. Helmet therapy is used afterwards to shape the skull as it grows.
Benefits
- less invasive
- only one or two small scars
- low risk of blood loss and blood transfusion
- high success rate, which can help avoid more invasive surgery.
Complications:
- risk of incomplete correction
- helmet therapy can cause discomfort
- risk of infection
- there may be a need for additional treatments as your child grows.
Fronto Orbital Advancement (FOA) surgery
FOA reshapes the forehead and eye sockets. The bones are removed, reshaped, and replaced using dissolving plates and screws. FOA is used when craniosynostosis affects the front of the skull and the forehead. This surgery takes a few hours and is done by plastic surgeons and neurosurgeons.
After the surgery, your child will stay in the hospital for 3 to 5 days.
Advantages:
- major improvement in forehead shape
- allows the brain to grow
- low risk of complications
Complications:
- risk of infection or bleeding
- risk of damaging the dura, or outer lining of the brain
- risk of asymmetry or scarring
- may need further surgeries as your child grows.
Spring cranioplasty
Spring cranioplasty is used for sagittal craniosynostosis when strip craniectomy and helmet therapy are not an option. Springs are placed to widen the skull and removed after three months. It works best for babies aged 5–7 months.
Advantages:
- less invasive than other surgeries
- low risk of blood loss and blood transfusion
- high success rate, which can help avoid more invasive surgery.
Complications:
- risk of incomplete correction
- risk of infection in the springs
- may need further surgeries as your child grows.
Total calvarial remodelling
Total calvarial remodelling reshapes the entire skull by removing, reshaping and reattaching the affected bones.
This procedure is generally used for children who are too old for strip craniectomy or spring cranioplasty.
Advantages
- effectively corrects severe differences in the shape of the skull
- allows room for the brain to grow
- creates a more normal skull shape.
Complications
- is a major surgery and usually needs blood transfusions
- risks include infection, asymmetry, damaging the dura or lining of the brain
- more treatments may be needed as your child grows.
Management
Helmet therapy
Helmet therapy helps reshape your baby’s head as they grow. It starts a few days after strip craniectomy surgery.
Your baby will wear the helmet for 23 hours a day until they are around 9 months old.