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Diagnosis and Treatment of Craniosynostosis (CRS)

Introduction

The bones that make up a newborn’s skull are connected by sutures, which are flexible strips of fibrous material that enable the skull, and thus the brain within it, to grow apace. The premature fusion of one or more of those sutures is called craniosynostosis (CRS). Untreated, the disorder can inhibit the brain and head from growing normally and can put pressure on sensitive brain tissue.

As the mechanisms underlying the disorder become better understood, and as surgical practices evolve, important advances are being made in the treatment of CRS. In the past, most cases required open surgery, but today many patients can be treated with minimally invasive surgical techniques that necessitate only small incisions in a patient’s scalp. These techniques have made CRS treatment safer, faster and easier to recover from. Improvements in post-surgical techniques for protecting the brain while giving it plenty of room to grow have also helped our young patients heal more quickly and completely.

Much of this progress has occurred here at Lucile Packard Children’s Hospital Stanford, where some of the world’s top clinicians and scientists continue to advance the understanding and treatment of CRS. Packard Children’s has a diverse team of specialists trained to address every aspect of what can be a complex disorder with psychological, developmental and social components as well as cosmetic, neurologic and orthopedic ones. 

Diagnosing CRS 

Some cases of CRS are identified in ultrasound images before a child is born. Most cases, however, are detected in the first months of a child’s life by pediatricians who notice characteristic abnormalities such as smaller than normal head size or head elongation, bulging, protrusions, or asymmetry. Sometimes, symptoms other than head size and shape will cause parents to bring their baby to the doctor, who may then diagnose CRS. Those symptoms, which may be the products of increased pressure in the brain caused by skull growth not keeping pace with brain growth, may include sleepiness, prominent veins on the scalp, irritability, high-pitched crying, difficulty feeding, projectile vomiting, seizures, bulging eyes and developmental delays. Of course, many of these symptoms could have other causes. If in doubt, promptly consult a pediatrician.

There are numerous types of CRS, and they require various treatment approaches. Pediatricians typically refer a patient whom they suspect of having the disorder to a specialist. To zero in on a more precise diagnosis, the specialist will likely request the patient’s complete prenatal and birth history and ask if the patient has a family history of CRS or other head or face abnormalities. CRS can sometimes be associated with broader developmental delays, and the specialist will ask about the patient’s cognitive and behavioral milestones. The physician will also measure the dimensions of the child’s head, feel along the skull’s sutures, and occasionally order X-rays or CT scans, or both.

If an underlying syndrome is suspected, genetic tests may be conducted in an attempt to identify the genetic anomaly.

Treating CRS

Children who are over 6 months old are usually treated with traditional open surgery. This approach requires making long incisions in the scalp to reveal and remove the fused sutures and allow reshaping of the skull. Once adjusted, the new skull bone positions are held in place with absorbable plates and screws. Although a single surgery is usually sufficient, follow-up surgeries may be required in complicated cases to adjust a baby’s face and head shape as they grow.

Babies diagnosed before they are 6 months old can often be treated with endoscopic surgery using a technique that requires only very small incisions in the scalp. An endoscope, which is a long, thin, flexible tube-like surgical instrument equipped with a camera and light source, is inserted into an incision and the surgeon uses instruments fed through the endoscope to cut and remove the sutures along with strips of bone on either side of them. This endoscopic approach allows for a shorter recovery time, results in less bruising and scarring, requires a shorter operation with less anesthesia, and typically also entails less blood loss and thus no need for transfusion. The endoscopic option is one clear benefit of early recognition and diagnosis of CRS.

Post-surgery care 

A child will usually spend the first night after open surgery in the intensive care unit for observation and then two or three more days in the hospital before being released home. Patients who have had endoscopic surgery can usually go home after only one night in the hospital.

After endoscopic surgery, a custom-tailored helmet is often used to protect and gradually reshape the patient’s head. Pads in the helmet, which will be adjusted every few weeks, put gentle but persistent pressure on the skull, slowly reforming it into a more symmetrical, normal shape while allowing for normal brain growth and skull fusion. For up to a year after surgery, most patients wear their helmets all the time except while bathing. Children, and their parents, quickly acclimate to the helmets, which are neither uncomfortable nor burdensome.

The CRS team: Treating the whole patient 

The CRS treatment team at Packard Children’s includes four dedicated pediatric neurosurgeons, two pediatric plastic and craniofacial surgeons, and a pediatric nurse practitioner certified in plastic and reconstructive surgery. The team works closely with experts on psychiatric, social, and learning issues to help anticipate, identify, and address any issues that may accompany CRS, including cognitive and behavioral concerns. Developmental problems are more likely to arise if the CRS results not from defects in the sutures themselves but from another underlying problem that is limiting brain growth. In such cases, it is especially important for CRS experts to work closely with clinicians in other specialties who can also continue to help our young patients after their surgical issues are resolved.

We focus on what matters most: The best possible outcome

Craniosynostosis patients from around the world receive the best available treatment at Lucile Packard Children’s Hospital Stanford. Every child is unique, and our world-class surgeons, who have decades of combined experience, do whatever they can to address each patient’s specific needs. From our state-of-the-art pediatric surgical facilities to our family-oriented care rooms, our whole team works with precision, passion and compassion toward one goal: the best possible outcome for each young patient.

 

 

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