Structural Defects: Craniosynostosis (Nursing)

by Elizabeth Stone, PHD, RN, CPEN, CHSE, FAEN

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    00:00 Hello. This is the lecture on craniosynostosis.

    00:05 We're going to start by going over the definition and physiology, we'll discuss the causes of it, the manifestations of it and what each type looks like, and then we'll discuss the basic treatments for it. Let's start with definition.

    00:21 Craniosynostosis is a premature closure of a suture or sutures of the skull.

    00:28 The sutures of the skull are basically the gaps between the regions of the skull.

    00:34 Typically in the normal newborn head, they end up fusing together in a certain predictable order.

    00:41 In craniosynostosis, something fuses prematurely. Again, it may be one suture or it may be more than 1.

    00:50 These are the terms that represent that various sutures or borders or junctions in between the regions of our skull.

    00:58 You may not see this very often, these terms, you'll usually see them more in radiology reports, but it's helpful to see them here so you can understand the condition.

    01:08 In craniosynostosis, the skull is forced to grow in a way it typically does not grow in order to accommodate brain expansion.

    01:16 This is actually a fairly common condition. It occurs in about 1 to every 2000 to 2500 births.

    01:26 This is the normal closure of the suture lines between the skull bones.

    01:32 Generally by about 2 months of age or 8 weeks, the posterior fontanelle has closed, but the anterior fontanelle remains open.

    01:43 There is a fibrous material that exist in the junctions in these sutures lines and also in the fontanelle.

    01:49 Eventually it becomes bone through a process called ossification, but that happens gradually as the infant's brain needs to grow.

    02:00 At about 6 months, there is this fibrous union so basically the regions are starting to come together in the posterior area.

    02:11 Then around 18 months or by 18 months, the anterior fontanelle is closing.

    02:16 You may not feel it any more at this point.

    02:21 An astounding almost 70% of the adult brain weight is achieved by about 18 months of age.

    02:28 This may be why many toddlers especially seem very top heavy and very clumsy, they fall a lot and they often hit their heads.

    02:36 and that's really just because their heads are so heavy in proportion to the rest of their body.

    02:44 By the age of about 12 years, the sutures or the borders in between the regions of the skull are not separable by increased intracranial pressure.

    02:55 And what this means is the skull is not going to be as forgiving to small increases in pressure as a result of, for example, brain injury.

    03:06 Craniosynostosis occurs if the bone somewhere in the head fuse together before the brain is fully developed.

    03:14 This is animation that's going to show you in real time what could happen if one of the sutures closes early.

    03:21 We're going to watch the suture at the top near that ear at the top close prematurely and you'll see how the brain shifts in the direction to how it grows and it can also affect the head shape.

    03:34 Basically it displaces it away from diffuse suture.

    03:39 Premature suture closure inhibits the normal growth and development of the brain and causes it to shift in this way.

    03:46 So that's the way it might look if that suture close prematurely.

    03:52 This is the way it might look on the outside.

    03:54 We're going to look at each type of craniosynostosis a little more closely in a moment.

    04:02 These are the types of craniosynostosis. I'm going to show you how each one presents.

    04:08 The sagittal suture runs along the top of the head across the fontanelle.

    04:13 Sagittal craniosynostosis is actually the most common type. It's also known as scaphocephaly.

    04:21 When this happens, when the sagittal suture closes prematurely, it forces the head to grow kind of long and narrow just like the picture on the far left.

    04:32 The next one, unicoronal means one of the coronal sutures close prematurely.

    04:38 It's also known as anterior plagiocephaly.

    04:42 The coronal sutures run from each ear to the sagittal suture at the top of the head.

    04:49 This type, the unicoronal craniosynostosis, basically occurs when one coronal suture closes prematurely and the infant may, as a result, have a flattened forehead on the side that closed early.

    05:05 Sometimes, both coronal sutures will close prematurely.

    05:10 When this happens, the head can grow broad and short and this is likely to result in increased ICP, which makes sense because when you have more than 1 area of the skull that's closing prematurely, there's even less room for growth of the brain so it's more likely that pressure is going to build up.

    05:29 This one is also known as brachiocephaly.

    05:32 Then there are 2 types that are fairly rare. One is metopic craniosynostosis.

    05:41 So the metopic suture typically runs from the nose to the sagittal suture at the top of the head.

    05:47 Closing early may result in a triangular looking head like this.

    05:51 It's also called trigonocephaly which can be kind of easier to remember if you think of the triangular shape that it results in.

    06:01 This is one of the rarest types and often requires surgical correction.

    06:06 One of the other rare types of craniosynostosis is lambdoid craniosynostosis.

    06:12 It's also known as posterior plagiocephaly.

    06:16 The lambdoid suture typically runs along the back side of the head.

    06:19 If it closes early, the head can be flattened on the back side.

    06:25 Alright, let's review the causes of craniosynostosis.

    06:29 The causes and risk factors for this condition are largely unknown.

    06:33 There are genetic and environmental components that are both suspected, and sometimes this condition can be part of a syndrome or a cluster of defects.

    06:44 Maternal thyroid disease and some fertility medications may also contribute to the rest of craniosynostosis in the baby.

    06:51 Alright, let's go over the diagnostic findings and the manifestations of this condition.

    06:57 The manifestation basically depends on which sutures closed, how early they closed, and whether or not the brain has had a room to grow whether there has been a brain damage as a result of it.

    07:10 Some signs that can lead to the diagnosis of craniosynostosis are a firm raised edge where sutures are supposed to be flat, so where they close too early; the absence of a soft spot or fontanelle where one is supposed to be present; and/or slow or no growth in the head size over time.

    07:35 Usually this condition is detected during routine check-ups because the newborn and the infant are all having head circumference measurements and had assessments done at every check-up.

    07:47 A CT scan can confirm the diagnosis once it's suspected.

    07:53 Let's go over the treatment.

    07:56 The treatment basically depends on the timing and when it's caught and then what the goals are so the goals will be set according to the signs and symptoms and the effects it's had on the child.

    08:09 Usually, surgery is indicated or needed and the timing of that surgery depends on which sutures were closed.

    08:17 The goals of the surgery include relieving any pressure on the brain, correcting the actual condition, this craniosynostosis, and then allowing for proper growth of the brain and the head after that point.

    08:31 Cranial vault remodelling is the surgery done to treat this condition.

    08:37 Most infants who have it require this particular surgery.

    08:41 Usually only one surgery is required when the child is about 4-6 months old.

    08:47 About 10% of the children may need a second surgery at some point to correct some kind of minor deformity or a possible relapse of the condition, but most of them have pretty good outcomes as long as the condition is caught early before there is too much pressure or any damage to the brain.

    09:08 Occasionally, you may see a child wearing a helmet to help treat this condition as well.

    09:14 This special helmet may also be used to help reshape the skull.

    09:19 This is the NCSBN Clinical Judgment Measurement model.

    09:23 It's a framework being used now for many NCLEX next generation questions and case studies.

    09:29 You may hear about it in nursing school and you may have some tests questions that use this framework so we're going to connect a little bit of this content from the lecture to this measurement model specifically the first 2 steps of it, recognizing cues and analyzing cues.

    09:45 In order to be able to accurately recognize cues and analyze cues in an infant who may have craniosynostosis, you have to first be familiar what a normal newborn head looks like.

    09:57 We have a whole lecture dedicated to that and I'll tell you all about it and show you how to measure a head circumference.

    10:04 Then, you also have to be able to assess for signs and symptoms of craniosynostosis and be able to understand what they are and when you might see them.

    10:14 For example, if you don't feel or see a fontanelle on an infant that should have one, that might be a sign that they have craniosynostosis.

    10:25 If you feel a raise from edge at where you think is a suture line between 2 regions of the skull, that might be another indicator.

    10:33 And if you see a misshapen head, if you know the misshapen head, there should be no misshaping of the head after the initial couple of days after birth.

    10:42 The head should be round and should be even and symmetrical.

    10:45 That might be another cue.

    About the Lecture

    The lecture Structural Defects: Craniosynostosis (Nursing) by Elizabeth Stone, PHD, RN, CPEN, CHSE, FAEN is from the course Neurologic Disorders – Pediatric Nursing.

    Included Quiz Questions

    1. Premature closure of at least one of the sutures of the skull.
    2. Delayed closure of at least one fontanelle.
    3. Delayed closure of at least one of the sutures of the skull.
    4. Premature closure of at least one fontanelle.
    1. It is a relatively common condition.
    2. It prevents the brain from growing normally.
    3. There are different types.
    4. The causes are largely unknown.
    5. It does not affect the head shape.
    1. Sagittal
    2. Unicoronal
    3. Lambdoid
    4. Metopic
    1. A firm raised edge where the suture closed.
    2. Decreased intracranial pressure.
    3. Rapid head growth.
    4. Larger than average head circumference.
    1. Cranial vault remodeling
    2. Skull reshaping helmets
    3. Ventriculoperitoneal shunt
    4. External ventricular drain

    Author of lecture Structural Defects: Craniosynostosis (Nursing)

     Elizabeth Stone, PHD, RN, CPEN, CHSE, FAEN

    Elizabeth Stone, PHD, RN, CPEN, CHSE, FAEN

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