Baby Flat Head Syndrome

Baby Flat Head Syndrome - Treatment & Prevention - Tips & Advice

Craniosynostosis Treatment

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In minor cases of craniosynostosis no treatment may be required, or helmeting may be recommended for a short period of time. In general, however, the preferred treatment for craniosynostosis is surgery. There are three main goals for all craniosynostosis surgeries:

  • Improving physical appearance
  • Allowing the brain to grow freely
  • Relieving pressure on the brain

Age considerations

Ideally, a child with craniosynostosis will undergo surgery before they turn 1. If your child has a severe case, however, surgery may be necessary as early as 1 month of age. In cases that are not diagnosed until significantly later in life, but that present with complications such as swelling on the brain or seizures, surgery may also be considered.

When a very young infant receives cranial surgery, the procedure may be less invasive than what would be required to treat the same problem at a later stage. Since a child’s skull grows more slowly with age, and the sutures begin to fuse between 1-2 years of age, surgeries at or beyond this point may be much more complex.

Most craniofacial specialists and neurosurgeons agree that the ideal time for a child to undergo surgery for craniosynostosis is between 3 and 8 months of age.

Available Methods

Traditional Surgery

Traditional surgical correction involves the correction of a craniosynostosis by moving the affected skull bone in a way that creates a more normal, rounded appearance. This type of surgery can be very long due to its complexity; lasting up to eight hours. It is not recommended for children under six months of age, due to the potential for blood loss.

A blood transfusion may be necessary during this type of surgery, so if either you or your partner is a blood-type match, you might look into donating blood for your child ahead of the procedure. Facial swelling is common after this type of surgery. Cranial vault remodeling (CVR), strip craniectomy, and Barrell Stave craniectomy are the most common traditional surgical methods for craniosynostosis. Of these, CVR is the most successful procedure, even for older children.

The benefit of this type of surgery is the lack of need for any follow up procedures, unless the craniosynostosis returns. Children who have traditional surgery do not need to wear cranial helmets following the procedure, although they may have plates, screws or other hardware installed as part of the procedure.

Endoscopic Surgery

Endoscopic surgery is best for infants younger than 3 months of age, although it can be performed on babies as old as 6 months. It is a minimally invasive procedure that allows your child to go home within 24 hours on average. During endoscopic surgery, a neurosurgeon or craniofacial surgeon re-opens the closed suture on your baby’s head, allowing the bones to grow normally again. The surgery itself lasts about an hour, and involves very little blood loss. Swelling can occur following this surgery, but is usually minor.

This type of surgery can result in bone thickening where the bone was cut, and is prone to a higher rate of recurrence than traditional surgical techniques. A new variation on this procedure attempts to prevent craniosynostosis from recurring by placing springs in the re-opened fissures. These springs need to be removed in a second surgery, when your doctor believes your child is ready.

A cranial helmet is required after this type of surgery, and your doctor will discuss the frequency of re-fittings and follow-up visits that your child needs, based on their particular case. Generally, your child will need to see his or her specialist at least once every three months for the first year following the surgery.

Outlook

The number of sutures initially involved in the craniosynostosis, the complexity of the surgery (if applicable), your child’s health prior to the procedure, and the presence of any atrophies in the brain all affect the post-operation outcome for your baby. Unless your child is suffering from syndromic craniosynostosis however, the prognosis following surgery is usually good.

Genetic counseling may be recommended, in order to make sure that your child is not suffering from a hereditary disorder. Frequent well-child visits will be necessary to monitor the growth of your child’s brain, skull and facial bones in order to make sure that they are growing normally following the procedure. Craniosynostosis can re-occur in some cases.

Additionally, reconstructive surgeries that repair facial deformities may be necessary or cosmetically recommended based on the factors surrounding your baby’s particular case.

What is Craniosynostosis and How is it Diagnosed?

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What is craniosynostosisIf your infant has a misshapen or flattened head, it is very important that you get an accurate diagnosis of the cause.  Although the majority of head flattening is caused by external pressure on an area of the baby’s head, it is possible that there could be a more serious cause, such as craniosynostosis. This article explores what craniosynostosis is, and how it is diagnosed. Craniosynostosis is a rare but serious condition.

Definition

Craniosynostosis is the premature closing of your baby’s skull fissures. As new parents, we worry so much about our children’s soft spots, but when they close early, significant problems can result.

The complications of craniosynostosis can leave babies with oddly-shaped heads, and in severe cases can cause swelling in the cranial cavity, headaches, vomiting, increasing head circumference, poor feeding, irritability, developmental delays, structurally-related speech impediments, hearing problems, reduced vision, loss of vision, breathing difficulties, and even seizures.

There are two types of craniosynostosis; primary and secondary. Primary craniosynostosis occurs in 2-8% of cases, when a child’s bones fuse together prematurely. This can keep the brain from growing correctly. Secondary craniosynostosis is the result of the brain’s lack of growth (microcephaly), and is more common than primary craniosynostosis.

The particular bones that fuse together early in a baby’s brain can also affect the treatment and outcome of the condition. The most common type of craniosynostosis is saggital (50-58% of cases), the second most frequent is coronal (20-29% of cases), the third is metopic (4-10% of cases). The least common type of craniosynostosis is lambdoid, which affects 2-4% of cases.

Craniosynostosis varies in severity, as well as in cause. Simple craniosynostosis affects only one of the sutures in a baby’s skull. Complex craniosynostosis involves more than one skull fissure, and syndromic craniosynostosis is synostosis that occurs in conjunction with other skeletal deformities in the body.

If your child has just been diagnosed with craniosynostosis, please skip to the end of the book. Several resources and contact information for support groups has been provided in hyperlink format. You are not alone, and sometimes when things get rough, it’s good to talk to someone who has been in your position.

Saggital craniosynostosis

The appearance of saggital craniosynostosis – scaphocephalic craniosynostosis – is identical to that of positional scaphocephaly. Children with this condition develop an elongated and narrow skull.  This happens when the fissure that runs between the front and back of the head closes too early.

Coronal craniosynostosis

Coronal craniosynostosis occurs when one or both of the bony seams on the sides in the front of the skull close early.

Unilateral coronal craniosynostosis (Unicoronal craniosynostosis [one-sided]) can be confused with deformational plagiocephaly, a condition which does not require surgical correction, since the coronal suture isn’t fused. Both conditions can cause plagiocephaly, or ‘flat head,’ a condition which creates a flat spot on one side of the skull.

Brachycephalic craniosynostosis is another name for bilateral coronal craniosynostosis. Children with this condition can look like children with positional brachycephaly, but with craniosynostosis, the skull fissures have closed.

Metopic craniosynostosis

Trigonocephaly, or metopic craniosynostosis, is the result of the skull fissure in a baby’s forehead closing too soon. Children with trigonocephaly often have eyes that appear too narrow and angled upwards towards the outside. Their faces can take on a sharply triangular appearance, hence “trigono”- cephaly.

Lambdoid craniosynostosis

The least common type of simple craniosynostosis is lambdoid craniosynostosis. This condition occurs when one or both of the two rear side fissures of the skull fuse early. It is also described as posterior plagiocephalic craniosynostosis, and looks similar to posterior positional plagiocephaly.

Pansynostosis

Complex craniosynostosis which involves the early closure of three or more sutures is referred to as pansynostosis, which means “all one bone”. Babies with pansynostosis can look very different from one another. Some will have what is called clover-leaf skull, where the different bones of the skull bulge outward, and look like a clover-leaf. Others will looks microcephalic, with little to no head growth. Oftentimes, this condition is the result of dwarfism.

Frequency

According to Medscape.com, 0.04-0.1% of babies in the United States are born with craniosynostosis.

Causes

There are many reasons that a child may be born with craniosynostosis. A baby’s poor position in the womb, specific medications taken by the mother during pregnancy (valporic acid or phenytoin), a pre-existing defect or malformation in the fissures themselves, metabolic disorders, neuromuscular conditions, sugar-processing disorders, and genetics have all been tied to craniosynostosis.

Craniosynostosis can appear as a symptom for other conditions, in which case it is referred to as syndromic craniosynostosis. If the craniosynostosis develops in an other-wise healthy child, it is considered nonsyndromic craniosynostosis. It is always important to rule out any potentially related conditions if your child has been diagnosed with craniosynostosis – some of the potentially related syndromes and disorders are dangerous if untreated. There over 150 conditions which are associated with craniosynostosis. The following is a list of some of the conditions which sometimes accompany craniosynostosis:

  • Apert’s syndrome
  • Cranio-fronto-nasal dysplasia
  • Crouzon’s disease
  • Hurler’s syndrome
  • Morquio’s syndrome
  • Muenke (P250) syndrome
  • Pfieffer’s syndrome
  • Saethre-Chotzen syndrome

Diagnosis

An infant with craniosynostosis is usually diagnosed as a result of physical exam by the doctor, or a parent’s concern about their child’s unusual head shape. Because the severity of craniosynostosis can vary significantly, some children go undiagnosed for several years until further complications such as blurred vision or increasing pressure on the brain develop. Symptoms of true craniosynostosis (not positional cephalic disorders) include:

  • Absence of a soft spot (fontanel) at birth
  • Early disappearance of the baby’s soft spot
  • Seizures
  • Bulging eyes
  • Raised hard ridges on the skull
  • Unusual head shape
  • Decreased head circumference
  • Increased head circumference
  • Obvious scalp veins
  • Slow head growth
  • No head growth
  • Developmental delays
  • Increasing irritability
  • Projectile vomiting
  • High-pitched crying
  • Poor feeding habits

Craniosynostosis can be confused with cephalic disorders such as positional plagiocephaly or brachycephaly. As a result, it is important that your doctor use diagnostic imaging when deciding what type of craniosynostosis your baby has. For example, in the past children with positional scaphocephaly were frequently misdiagnosed as having closed saggital sutures – an indication of scaphocephalic craniosynostosis.  An accurate diagnosis of the cause of any flat spots on your baby’s head is critical.

For complete information on all causes of flat head syndrome including craniosynostosis, and how to prevent and treat these conditions, please see our ebook.

Baby head Shape – What is Normal?

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normal baby head shape.“Normal” head shape can be seen in the figure to the left. This picture is only a guide, however – normal head shape includes a range of sizes and minor deviations from this idealized form.

Acquired cephalies, or types of flat head syndrome, are normally diagnosed using a set of skull measurements. Occasionally there are visible differences in the position of an ear or the appearance of the face as well.

Any diagnosis of cephalic deformation has to rule out the possibility of craniosytosis – a condition which results from the skull bones fusing together before the skull has finished growing. Craniosytosis requires surgery, so it is important if your baby’s head appears to be misshapen to see a doctor to determine the cause.

Degrees of severity

Mild cephalic deformities may go unnoticed by parents and doctors, or may be easily dismissed as a cosmetic problem. A baby who has a mild cephalic malformation will have a skull with only slight flattening or elongation. Diagnosis will require measurements of the skull to be taken.

An infant with a moderate cephalic malformation can be diagnosed by sight, although measurements should be taken to determine the extent of the condition. An infant with a severe cephalic deformity exhibits an extreme degree of flattening on one part of the skull. Measurements, x-rays and other diagnostic imaging tests may be performed to rule out craniosytosis.

Diagnosis and Abnormal Head Measurements

The presence of plagiocephaly, brachycephaly, and scaphocephaly is generally determined by abnormal skull measurements. Plagiocephaly, brachycephaly and scaphocephaly can be identified using a craniometer or a medical laser designed for the same basic purpose. A craniometer looks a lot like the protractors we all used in elementary school for drawing circles and measuring angles, and depends on subjective decisions by your baby’s practitioner. Lasers provide a more accurate and less subjective measurement, and their popularity is growing as a result.

When evaluating for plagiocephaly, craniometers and lasers are used to take two measurements of your baby’s skull, and the severity of plagiocephaly is determined by the difference in these measurements. The measurements are essentially like what you would see if you drew and X on the top of your baby’s head. Mild plagiocephaly has a difference of up to 9mm. Moderate plagiocephaly is determined by a 10-19mm difference, and severe plagiocephaly exhibits a 20mm or greater difference.

One of the most common measurements taken in the evaluation of brachycephaly and scaphocephaly is the cephalic ratio, or cephalic index, which is a rather fancy term for the measurement taken by dividing the size of the widest part of a baby’s skull by the longest part of the skull, in millimeters and multiplying by 100. There is no consensus as to what the ‘normal’ cephalic ratio of an infant should be, however.

Normal measurements

In 1979, one of the first studies which tried to resolve the question of what a normal head shape is resulted in a 78-78.5% ratio, with gender being an important factor. Since that point, over 10 different ratios have been proposed as indicative of a significant brachycephalic cranial deformity, but most researchers and doctors propose a number in the low 90th percentile, ranging from 90-93% as a reliable marker for severe cranial deformities and a starting margin for clinical studies of cranial remolding therapies.

Scaphocephaly is generally considered to be a cephalic index measurement below 78%.  There are many ‘normal’ head shapes, and your doctor may determine that your baby doesn’t necessarily have brachycephaly, scaphocephaly or plagiocephaly if their head mesaurements are close to the ‘normal’ point and either you or your partner comes from a family with head shapes that are similar to your infant’s.

For complete information on flat head syndrome in babies including how to tell normal from abnormal head shape, as well as how to prevent and treat these conditions, please see our ebook.

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