Premature Fusion of Sutures in the Brain Craniosynostosis: Premature Fusion of Sutures in the Brain

Patient’s History Who is the patient? What is important about their history? 2 month old baby No previous diagnostic imaging Physician suggested Skull X-rays after a physical examination of the baby

Patient Care Is there anything which would impact the procedure? What is it and why is it important to consider? While doing X-Rays on babies, a few techniques such as “bunny baby” can be used For skull and facial work, immobilizers will be used The technologist has to be extra careful while making the exposure in order to compensate for any movement Repeating X-Rays should be avoided

Some forms of immobilizers used for pediatric Skull imaging

This patient had fused Lambdoid, Frontal and Sagittal Sutures Sutures of the Skull This patient had fused Lambdoid, Frontal and Sagittal Sutures

Pathological Condition What is the patient’s pathology? Is the pathology expected? Craniosynostosis is the premature fusing of the sutures in the brain This is what occurs in a newborn’s brain: Following birth, the baby’s brain undergoes an extremely rapid phase of growth and development. Newborn’s brain will double in size in nine months and triple in size in 36 months To accommodate for such rapid brain growth, the baby’s skull cap must expand rapidly as well Rather than being one large piece of bone, the skull is made up of several bones (frontal, parietal, occipital) which are held together by fibrous material called “sutures.” These sutures respond to brain growth by “stretching” and allowing the skull to grow along with the underlying brain.

When these sutures close too early, it causes Craniosynostosis. In some cases, it causes pressure on the baby's brain, which can cause damage. The baby will be affected depending on how many sutures close too soon: If only one suture closes too soon, the baby's brain usually develops normally, but the head has an odd shape. This is the common case. If more than one suture closes too soon, the baby's brain may not be able to grow as fast as it should. If severe pressure builds up around the brain, it may cause brain damage, seizures, blindness, and developmental delays. This is the rare case.

Types of Craniosynostosis Several types of Craniosynostosis occur depending on the number and which suture is affected: Single suture Synostosis: refers to a condition where only one suture is affected and all others are open. Multiple Synostosis can involve the closure of one, two, three or more sutures. Sometimes this problem can occur along with other systemic problems, which is then called “syndromic multisuture synostosis”. Some common syndromes include: Crouzon, Apert, and Pfeiffer.

Comparison of the Normal Skull to the different types of Craniosynostosis

The patient had an affected Sagittal and Lambdoid Suture so they suffered from Scaphocephaly and Plagiocephaly

Sagittal Synostosis The sagittal suture is located on the midline: on top of the head and extends from the soft spot towards the back of the head. When the skull does not grow properly from side to side, the brain grows forward towards the forehead and backwards. The head becomes long and narrow; the back of the head becomes prominent and pointed. The overall shape is known as Scaphocephaly. This is the most common type of single suture synostosis and most commonly affects males. 

Coronal Synostosis The coronal suture is located on the side of the head and extends from the soft spot to an area just in front of the ear. When this suture closes prematurely, the condition is known as Anterior Plagiocephaly. On the side of involvement, the following deformities develop: the forehead is flattened; the eye socket is elevated and tilted, the nose deviates to the opposite side; the baby tends to tilt the head to the side in order to prevent him/her from seeing double if untreated.

Lambdoid Synostosis Premature closure of the Lambdoid suture is the least common. Closure of this suture leads to Posterior Plagiocephalus with flattening of the back of the head on the affected side, protrusion of the mastoid bone and lowering of the affected ear. It may also cause the skull to tilt sideways.

Causes There is no set cause for this problem However, it is likely in: Babies who lie in a breech position while in the uterus. Babies whose mothers smoke or live at a high altitude during pregnancy. Babies who are twins.

Symptoms The most common sign is an oddly shaped head at birth or by the time the child is a few months old. For example, if the skull is long and narrow, flat and broad or the baby has a misshapen nose or jaw. Sometimes, this disease causes pressure to build up on the baby's brain. This can cause brain damage and cause the baby to develop more slowly than other children. If there is pressure buildup, the baby may: Start vomiting Become sluggish and sleep more and play less Have swollen eyes or has problems moving the eyes or following objects Have problems hearing

Diagnosis If the baby has an odd-shaped head at birth, the doctor will do a physical checkup The doctor will: Measure your baby's head. Feel the sutures and soft spots on the skull. Feel the top and sides of the head, where sutures are located, for unusual ridges or bumps. The doctor may also order a skull X-ray, or CT scan Imaging studies: X-rays of the baby’s skull will show whether any sutures have fused. Fused sutures are identifiable by their absence, because they're invisible once fused, and by the ridging of the suture line.

Treatment & Follow Up What treatment is the patient receiving? Treatment will depend on the severity of Craniosynostosis: If it only involves one suture and no underlying syndrome , it may require no treatment. Skull abnormalities may become less obvious as your infant grows and develops hair However, if more than one suture is involved then Surgery is recommended: 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 of a Craniofacial Surgeon and a specialist in brain surgery (neurosurgeon) will often perform the procedure. There are two types of surgeries: Traditional Surgery Endoscopic Surgery

Treatment In Traditional surgery, the surgeon makes an incision in the infant'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. Endoscopic surgery is a less invasive procedure. In some cases, the surgeon may use an endoscope which is inserted through one or two small scalp incisions over the affected suture. The surgeon then opens the suture to enable your baby's brain to grow normally. Endoscopic surgery usually takes about an hour, causes less swelling and blood loss.

If the baby only has Positional Plagiocephaly which is flattened or misshapen areas on the head, they do not require surgery. Treatment will be as simple as occasionally repositioning the child's head while sleeping or, in some cases, wearing a cranial band to mold the skull.

Animated Video http://www.craniosynostosis.net/video-gallery/procedure-animations http://www.craniosynostosis.net/craniosynostosis-before-after-results-photos/sagittal-suture-craniosynostosis/