Susan Durham, MD, MS, a professor of clinical neurological surgery at the Keck School of Medicine of USC, discusses the diagnosis and treatment of many types of craniosynostosis and helps practicing physicians differentiate craniosynostosis from other head shape anomalies commonly seen in infants.
Yeah. My name is Susan Durham. I am the division chief of pediatric neurosurgery at Children's Hospital, Los Angeles. I'll be talking about head shapes and infants. When is it really cranial? Sin Ost Assis. The three objectives from this lecture our first to identify the most common types of cranial sin ost Asus in Children. To understand the surgical procedures used for correction of the most common types of cranial sin ost, Asus and three to differentiate between positional play Joseph Aly and Cranial Sin Ost Assis Cranial sin Ost assis is premature ossification and obliteration of the skull suitors and the suitors involved determined the resultant head shape of the child. The epidemiology shows us that the incidents in simple non syndrome IQ cranial sin ost assis is one in 2000 births in syndrome IQ sin Ost, Asus It's approximately one and 25,000 births. Sagittal sin Ost assis is the most common type within 40 to 60% of cases Corona LSA anastomosis both by Corona land unilateral coronal make up about a quarter of the cases. The topics anastomosis accounts for about 10% of all cases with lamb DeutTel sin Hostos is being the most rare at about 5%. Occasionally, Children will have two or more cranial suitors that refused again, about 4 to 8% of all cases. The cranial suitors are well defined on the skull. The MMA topics future runs between the two frontal bones and is the only cranial suitor that you don't see in adults but only see in Children, particularly in infants. There are two Corona, LSU, Cher's Ah, sagittal suture running down the mid line of the skull and paired lamb DeutTel searchers in the posterior portion of the skull. Here's some fun facts about skull growth. Bone growth is driven by the expanding growth of the brain. Ah, small head typically means that a child has a smaller sized brain head size the brain. The head is actually 35% of the adult size. When a child is born by the time a child is seven, the head is actually 90% of the size. It's going to be a zone adult. The MMA topics future. The future that runs between the two frontal bones is the Onley suitor that completely closes over with bone, and this bony fusion is usually complete by age two. Suitors do not fuse after complete growth of the head. There's always fibers tissue intervening in crane ius anastomosis, bony fusion of the searcher occurs vehicles. Law tells us that cranial growth occurs parallel to the few searcher. This is a picture of a child with a fused sagittal searcher. You can see that the head is long and narrow. There is decreased growth, which is perpendicular to the fused future, and there's exaggerated growth that's parallel to the fused future. The diagnosis of Kronos Anastomosis is typically a clinical diagnosis. Infants are born with a characteristic skull shape that is typically present at birth and becomes exaggerated over time. Radiographic studies include plane skull X rays, which are typically of little value. Ah, CT head with three D reconstruction will accurately demonstrate which cranial suitors refused. Ultrasound is an emerging technology that can actually help diagnosed fusion of the involved futures. The treatment of creamiest anastomosis is typically two rationales for treatment. The first is one of aesthetics, and this is the most common indication in single future creamiest anastomosis. Because the cranial deformity worsens with head growth, this often has involved facial involvement with facial asymmetry. and also has a significant psychological impact to the child and the family. The functional rationale for treatment are much more common in syndrome IQ Children. These Children may often have increased intracranial pressure, PayPal, oedema and decreased visual acuity. They often have hydrocephalus as well as risks for corneal exposure. Surgical timing for repair of cranial sin acidosis really depends on when the child is diagnosed. We like to take advantage of the exponential growth face of the skull, which occurs in the 1st 18 months of life. Normally, a child's head grows approximately two centers meters per month for the first six months of life and one centimeter a month for 6 to 12 months of life. Early diagnosis is key in determining what type of surgery should be done. When Children are diagnosed before three months of age, they can often be a candidate for the endoscopic assisted procedures. At this point, the skull is thin and malleable, and there's little need for extensive bone work. These Children are typically placed in a cranial molding helmet post operatively for a period of 6 to 12 months. Unfortunately, most Children are diagnosed after three months of age, making them, not the best candidates for endoscopic approaches. I'd like to review the different types of creating a sin. Ost Assis Sagittal Sinus tose ISS ISS fusion of the sagittal searcher, which runs along the midline of the skull. This is the most common type of cranial sin ost Asus and occurs most commonly in males compared to females at a 4 to 1 ratio. This is often described as Scelfo Cifelli or delay Kochav Aly, which comes from the Greek word Scaife, meaning boat. It is characterized by a boat like cranium. Sagittal Sinus doses typically have an exaggerated anterior posterior elongation of the skull, a high forehead as well as both frontal and occipital. Boston in sagittal Sinus tose issue typically see an absent sagittal suture. You see an increased AP length of the skull and you see a narrow by temporal distance. And this picture demonstrates a three D reconstruction of a seat of a C T scan of a child, which demonstrates sagittal Sinus doses and complete fusion of the sagittal suture. The treatment for sagittal Sinus doses varies by the time of diagnosis. If a child is diagnosed early, typically before 3 to 4 months of age. Their candidate for an endoscopic suits your ectomy, followed by a molding helmet therapy. At age 4 to 6 months, Children typically glow what's undergo what's called an open calve aerial remodeling. And this is a surgery that's performed to reduce the AP dimension of the skull and we barrel stave the temporal areas, allowing for by temporal widening. At late diagnosis, which is approximately 1 to 2 years of life. These Children could be candidates for cranial distraction, whereas a very late diagnosis after two years of age, this requires a much more complex Calvario vault remodeling with frontal orbital advancements. A complete Calvario vault remodeling in bone grafting, which is a much larger surgery to dio. This is an example of an endoscopic repair, which is typically done prior to three months of age. It's a simple shooter ectomy of the sagittal suture. The width of the suitor that's removed is approximately 3 to 5 centimeters, and there's an option for adding barrel stave ost iata mies bilaterally. The advantage of this approach is that it's less blood loss in a young infant. There's a shorter operative time, as well as anesthesia time and a shorter length of stay. The drawbacks to this procedure at this age are there's limited visibility for what we're doing and also requires helmet compliance. Post operatively between 6 to 12 months of helmet therapy. Afterwards, the picture on the right demonstrates the incisions that are made on the cranium. There's two small incisions that are made, which allow us access to the few future underneath, and the second picture is that of a typical cranial molding helmet orthe Asus, that is worn by the Children Post operatively. The typical Calvario remodeling, which is typically performed at 3 to 6 months of age, has been described in a variety of methods, Um, depending on the shape of the skull and the frontal versus occipital Boston versus the degree of by temporal narrowing. There are many different options for Cal burial vault remodeling for treatment of sagittal soonest Asus that have been described over the years. Here's a typical picture of a pre and postoperative head shape in sagittal Sinus toe sis. The pictures on the left are a top down view and a side view of a child with sagittal Sinus toe sis, and you can see that the head is very narrow and very long, and the pictures on the right are about two weeks post operatively from a Calvario vault remodeling, and you can see that the head is shorter and wider and much more round than when they started when they before, prior to the having their surgery. The second type of of Kronos anastomosis that's commonly seen is Corona LSI Anastomosis, and this is seen anywhere between 20 to 30% off all cases. And this is a fusion of one or both of the Corona LSU Cher's. This type is more common in females. Unilateral Corona LSA Knost assis is seen about twice as commonly is bilateral, So Knost Asus Unilateral is typically isolated. Bilateral corona LSA anastomosis is often syndrome IQ. The picture on the right is a picture of a fused Coronas future, and it demonstrates the compensatory cranial remodeling as a result, as a result of the Corona suitor, be infused with contra lateral forehead bossing, and it's a lateral forehead flattening unilateral Corona LSI anastomosis again shows it's a lateral forehead flattening as well as contra lateral forehead. Bossing the ipsa lateral eye is more open. The Ipsa lateral brow is higher These Children often have a C shaped facial deformity, and the base of the nose and the chin typically point away from the affected side. Unilateral Corona LSI anastomosis can be treated and disk optically again before three months of age, requiring these Children to wear a helmet post operatively for anywhere between six and 12 months. The picture on the top on the right demonstrates the bony cuts that are made for repair of Corona LSI anastomosis unilaterally. Traditionally, these air treated with a tradition with a bilateral frontal orbital advancement, and this is done between six and nine months of age. And in this type of surgery, the entire frontal bone, as well as the frontal orbital bar are removed and remodeled to, uh, alleviate the flattening and the hyperplasia on the affected side. The third type of cranial sin ost Asus Esma topics in aust Asus and this is fusion of them a topic searcher. This is the on Lee Soo chur, diffuse normally in the first year of life. This head shape is typically called Trygg anencephaly, which is named after Greek Trigon on meaning triangle, and you can see that the head shape is characterized by a keel shaped triangular forehead. The pictures on the right or a three D c T scan of a child with my topic since August, Asus. And you can appreciate the triangle shape of the forehead and the how the eyes air close together. This is called Hypo Terrorism. Thes Children often have by temporal narrowing hype Attila Riz um, and a midline ridge along the forehead. The treatment of MMA topics anastomosis varies depending on the severity of the soonest Asus and head shape deformity. Typically, these air mild they do not have any by temporal narrowing, and they only have a small, palpable ridge on the forehead. In these cases, it's tip. These Children are typically treated with reassurance to the parents and followed and told that this is a normal skull variant in moderate cranial prosthesis of the topics Future without significant temporal narrowing or hyper, Tillery is, um, these Children can often be a candidate for a less lesser involved surgery called an endoscopic a topic strip craniectomy again. This would need to be done within the first three months of life and Children that have severe by temporal narrowing and cranial deformities. These Children typically undergo a frontal orbital reconstruction as well as an anterior cranial vault remodeling where again the frontal bone is removed as well as the orbital bandeau, or frontal orbital bar. And both of these are reshaped to reduce the triangular shape of the forehead. Here is a pre and postoperative picture of a child with the topics in Aust, Asus and the preoperative picture. You could really appreciate the triangular shape to the forehead and the post operative picture shows nice by temporal widening and a more normal shaped head. Lam DeutTel See anastomosis is probably the most rare occurring in anywhere between 1 to 5% of Children. There is typically flattening of the OcciPet and the Ipsa lateral ear is deviated, inferior early. There is a pronounced contra lateral parietal bulge, and these Children are often confused with positional play Geo Sefa Lee and it's important to be able to differentiate Lambda Witelson Ost Assis from positional play Geo safely positional play. Just Shefali, often called Flathead, is probably the most common referral to a pediatric neurosurgeon or a pediatric craniofacial surgeon. This comes from the Greek word play Geos, meaning oblique and stuff early, meaning head and this is an asymmetric cranial and facial deformity. Positional play Joe Shefali typically is caused by back sleeping, where a child prefers to sleep on one side of the head rather than the other toward a call. Us. Where the head does not rotate normally often causes positional play JSF elite, as does prolong stays in the neonatal intensive care units, where Children are often nursed on their backs as well as limited mobility. And Children with developmental delays that don't develop the ability to roll and sit up proper sit up at the usual times. The treatment for positional play Josepha Lee is typically conservative counter positioning, which means teaching the parents methods to not have the child always laying on the affected side, as well as physical therapy, which may help with torta Collis and allowing the child to move the neck more freely. Tow. Avoid sleeping on the affected side and in severe cases. Sometimes a molding helmet is used, which is an external orthe Asus, which will actually works to reshape the head over a period of about 3 to 6 months. Molding helmets are typically most effective when used in the first year of life differentiation between positional play JSF early in lamb DeutTel sanest Assis is important. This is a schematic of a Vertex view or a top down view, looking at the difference in head shapes between positional play Joseph Feli and Lamb Doyle's anastomosis. Both of these illustrate flattening in the right occipital with positional play. Joseph Aly. When you look down at the child's head, everything on the right side is deviated anterior early so the right ear will be moved forward as well as the right forehead and sometimes the right cheekbone in lamb. DeutTel, sanest Asus. It's the opposite. Even though the right side might be flat, the right ear is actually deviated, inferior Lee and post eerily and the contra lateral forehead is pushed forward. And there's typically a strong compensatory contra lateral parietal bulge, which again can be confused with positional play. Joseph Lee. This is another schematic demonstrating the difference between positional molding and lamb droids. Anastomosis, which again demonstrates the position of the ear and the position of the frontal pra tuberous, which is quite different between the two Ah posterior view of the head is often very helpful in differentiating between positional play Josepha Lee and Limb DeutTel Sin Ost Asus in positional play Josepha Lee, the ears are level and lamb deuttel sin ost acis. The involved here is deviated, inferior early, and there's often a very large protuberance of the mass toid and the skull base on the affected side, as well as a strong contra lateral bulge in the parietal region region on the Contra lateral side. And you can see that the head shape looks very different when viewed from behind in posterior in um lam DeutTel sanest Asus versus Positional play Josepha Lee. So, in conclusion, head shape abnormalities are very common. Pediatric diagnosis. Most can be diagnosed by clinical history and exam alone. Radiographic imaging the three D C T, which is the diagnostic test of choice, should only be done when the diagnosis is in question, or if there's other clinical suspicion that one would need to image the brain. There's very limited role for skull X rays in this diagnosis. Early diagnosis and treatment is important. There's less result in skull base and facial, a symmetries which are often difficult to correct even after the child has had surgery. And this really requires a multidisciplinary team approach both by pediatric neurosurgeons as well as pediatric plastic surgeons and craniofacial surgeons. Thank you
