How is it treated?

Treatment of Craniosynostosis - Mayo Clinic

Four Options for correcting Metopic Craniosynostosis

1. No treatment
2. Cranio Vault Reconstruction Surgery
3. Endoscopic Surgery
4. Helmet Molding Therapy

Option One - No Treatment or Adopting the "Wait and See" Approach

The Metopic suture is the only calvarial suture which normally closes during infancy. Upon closure, a palpable and visible ridge often forms which can be confused with Metopic Craniosynostosis. Metopic ridging may be treated nonsurgically while metopic craniosynostosis is treated surgically.

Mild cases of Craniosynostosis — those that involve only one suture and no underlying syndrome — may require no treatment. Skull abnormalities may become less obvious as the baby grows and develops hair.

Differentiating between the two is paramount; however, consensus is lacking about where a clear diagnostic threshold lies. Physical examination and CT scan characteristics may help to differentiate between physiological closure of the Metopic suture with ridging and Metopic Craniosynostosis.

A benign metopic ridge does not require surgical treatment. It is very important that a qualified surgeon can distinguish between the two. Accurate diagnosis is paramount given the fact that surgical interventions present a significant risk to the patient.

Metopic Ridge a less severe form of Craniosynostosis - Cappkids.org

Option Two - Cranio Vault Reconstruction Surgery (CVR) or Open Cranial Vault Expansion Surgery (CVE)

For most babies, however, surgery is the primary treatment for Craniosynostosis. The type and timing of surgery depend on the type of Craniosynostosis and whether there's an underlying syndrome that needs treatment.

The purpose of surgery is to relieve pressure on the brain, create room for the brain to grow normally and improve your child's appearance. A team that includes a specialist in surgery of the head and face (craniofacial surgeon) and a specialist in brain surgery (neurosurgeon) often performs the procedure.

A team made of a Craniofacial Plastic Surgeon and a Neurosurgeon will remodel or reshape the bones of the skull expanding and enlarging the space within the vault to allow room for the baby's brain to grow while attempting to restore a more natural appearing shape of the skull.

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.

The surgeon makes an incision in the baby's scalp and cranial bones, then reshapes the affected portion of the skull. Sometimes plates and screws, often made of material that is absorbed over time, are used to hold the bones in place. Surgery, which is performed during general anesthesia, usually takes hours.

After surgery, the baby remains in the hospital for at least three days. Some children may require a second surgery later because the Craniosynostosis recurs.

CVR is designed to correct deformities in the facial and skull bones; often works best in children who are less than 1 year old, since the bones are still soft and pliable, making them easier to manipulate
may be recommended in much younger infants, including newborns, depending on the severity of their symptoms; can also be performed in older children (although the older the child, the more complex the surgery); is usually complete after a single surgery (but in some cases, may need to be repeated over time, depending on the seriousness of the condition).

Option 3 - Endoscopic Surgery

Endoscopic surgery. This less invasive form of surgery isn't an option for everyone. But in certain cases, the surgeon may use a lighted tube (endoscope) inserted through one or two small scalp incisions over the affected suture. The surgeon then opens the suture to enable the baby's brain to grow normally.

The minimally invasive, endoscopic-assisted craniosynostosis surgery utilizes a small camera to assist with removal of the abnormal bone that causes skull deformity through one or two one-inch incisions. The surgery is performed in one to two hours, children rarely need a blood transfusion, and they typically go home the next day.

Success using the minimally invasive endoscopic-assisted approach is early diagnosis and treatment. his minimally invasive procedure is typically performed on infants younger than three months of age, since it depends on extremely rapid brain growth to help reposition the cranial bones.

Removal of the prematurely closed suture (the abnormal bone) when the child is between two and six months of age allows the skull to develop a more normal shape as the child grows – this is just one of the options of a minimally invasive procedure.

The use of a helmet for 6-12 months after surgery helps facilitate this bone remodeling.

The downsides include the need for the helmet, and more frequent follow-up in the 6 to 9 months following the operation for helmet adjustments and visits with the surgeon.

It is important that the family find a Cranial Band Provider with Endoscopic and Surgery experience for post operatice care.

Option Four - Helmet Molding Therapy or  Cranial Orthosis

Helmet molding therapy, or cranial orthosis, is a type of treatment where an infant is fitted with a special helmet to correct the shape of the skull. Cranial remolding helmets are usually made of a hard outer shell with a foam lining. Gentle, persistent pressures are applied to capture the natural growth of an infant's head, while inhibiting growth in the prominent areas and allowing for growth in the flat regions. As the head grows, adjustments are made frequently. The helmet essentially provides a tight, round space for the head to grow into. So, even if the child continues to rest their head on one side, the helmet will provide a cushioning to prevent the head from further flattening or molding.

If the child is diagnosed with deformational plagiocephaly, brachycephaly, or scaphocephaly and is less than 12 months old, they may be prescribe cranial remolding to correct the shape of the child’s head. In addition, children who have undergone endoscopy to correct a craniofacial disorder will often be prescribed helmet therapy for the first year after surgery to further correct the shape of the head.

The helmet is that it is worn 23 hours a day. It can be taken off during bathing. The rest of the time, the infant should constantly be in the helmet — whether playing, sleeping, or feeding. This can be shocking to hear as a parent as you think of the baby’s formative months being spent wearing a helmet. One thing we like to emphasize is that the helmet is usually not uncomfortable for the baby.

The average treatment with a helmet is usually three months. The amount of time the child will wear a helmet depends on their age and severity of their condition. Careful and frequent monitoring is required to ensure that the skull is reshaping correctly. Helmets must be prescribed by a licensed physician.

The frequency of follow-up visits depends on the severity of the child’s condition. As the child is being fitted with a molding helmet, and adjustments are made, you will make several regular visits to the company that provides the helmet.

Sources: 

"The American Association of Neurological Surgeons." AANS. American Association of Neurological Surgeons, Sept. 2005. Web. 07 Jan. 2016. <http://www.aans.org/patient%20information/conditions%20and%20treatments/craniosynostosis%20and%20craniofacial%20disorders.aspx>.

"Facts about Craniosynostosis." Centers for Disease Control and Prevention. Centers for Disease Control and Prevention, 10 Dec. 2015. Web. 07 Jan. 2016. <http://www.cdc.gov/ncbddd/birthdefects/craniosynostosis.html>.

BUROKAS, Laura, MS, APN, CPNP/CCNS. "Craniosynostosis: Caring for Infants and Their Families." The Essential Neurosurgery Companion August 2013 33.4 (2013): 39-50. Aacn.org. American Association of Critical Care Nurses, 04 Aug. 2013. Web. 07 Jan. 2016. <http://www.aacn.org/wd/Cetests/media/C134.pdf>.

"Treatment Options | CAPPS." CAPPS Treatment Options Comments. Craniosynostosis and Positional Plagiocephaly Support, Inc., n.d. Web. 07 Jan. 2016. <https://www.cappskids.org/know-your-options/>.

Meulen, Jacques Van Der. "Metopic Synostosis." Child's Nervous System. Springer-Verlag, 08 Aug. 2012. Web. 07 Jan. 2016. <http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3413823/>.

"Craniosynostosis." Treatments and Drugs. Mayo Clinic Staff, 30 Sept. 2013. Web. 07 Jan. 2016. <http://www.mayoclinic.org/diseases-conditions/craniosynostosis/basics/treatment/con-20032917>.

"Treatments for Metopic Synostosis (Trigonocephaly) in Children." Metopic Synostosis (Trigonocephaly) Treatments | Boston Children's Hospital. Boston Children's Hospital, n.d. Web. 07 Jan. 2016. <http://www.childrenshospital.org/conditions-and-treatments/conditions/m/metopic-synostosis-trigonocephaly/treatments>.

"Helmet Molding Therapy." Helmet Molding Therapy - John Hopkins Pediatric Neurosurgery. John Hopkins Medacine, n.d. Web. 8 Jan. 2016. <http://www.hopkinsmedicine.org/neurology_neurosurgery/centers_clinics/pediatric_neurosurgery/conditions/craniosynostosis/helmet-molding-therapy.html>.