15 Signs Of Chiari Malformation In Babies

This is one such disorder that isn’t spoken about nearly as much as it should be, but it’s definitely a debilitating condition that can be seriously difficult to deal with for adults, let alone babies.

Chiari malformation, formerly known as Arnold-Chiari malformation, refers to when the back of the brain, lowest part of the brain doesn’t sit where it’s supposed to be. This specific part of the brain – the cerebellar tonsils – isn’t meant to be in contact with anything and is supposed to just lie in the brain’s cerebrospinal fluid, untouched by any bone. But in the case of this malformation, the cerebellar tonsils are displaced; it’s in contact with the spinal cord and pushes down into it, through an area called the foramen magnum. The fact that this area of the brain is connected to the base of the skull at the spinal cord can cause a number of issues – rarely issues that could potentially be fatal, but problems that could cause the sufferer a great deal of discomfort.

There are four types of Chiari malformations, each of which are characterized differently and manifest in different ways. Chiari can be hereditary and your baby could be born with it. It can be hard to notice whether or not your baby has Chiari malformation, because of course, there’s that small issue of communication. But there are still signs to look out for. These are 15 signs which together, could indicate Chiari malformation in your baby, so take heed and be aware of the symptoms.

15 Cognition Is Off 

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Cognitive problems – problems with memory and concentration, may be a sign of Chiari, in particular type 1 Chiari. Now of course, a young baby hasn’t yet accumulated memories, so this isn’t a sign you should look out for the moment your baby comes out of the womb. In actual fact Chiari is diagnosed in the majority people who have this malformation, when they’re in their late teens or have reached adulthood. But as your baby begins to get older, if he/she is falling behind in terms of natural development, it could very well be a sign. So if your baby is having serious problems with memory, concentration and as he/she gets slightly older, has trouble communicating and using words, it may be due to Chiari.

Of course there are a host of other issues which may cause these symptoms, so just because your baby seems more interested with the left over toilet roll then you, don’t instantly jump to conclusions. But this combined with the other symptoms in this article could point to Chiari.

14 Misshapen Skull

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Chiari malformation can be hereditary and can occur in the womb, or the effects may start to become apparent as your baby develops. Again, it’s due to the obstruction and consequently the build-up of CSF in the brain, which leads to a build-up of pressure. This can cause frequent and severe headaches, but it can even change the shape of the skull entirely.

Of course, when looking for signs of Chiari malformation in your baby, it’s easier to tell that you baby has a misshapen skull than spot a headache for example. Your baby’s skull may be abnormally shaped, or it could just be bigger than usual, due to the pressure changes and excess CSF which have forced the skull to change its shape

This build-up of pressure is known as hydrocephalus, is associated mainly with type 2 Chiari malformation and is one of the most serious effects of Chiari, which if left untreated could actually prove to be fatal.

13 Headaches

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With Chiari malformation, headaches occur because that lower region of the brain pushing into the spinal canal obstructs the flow of cerebrospinal fluid – more specifically CSF outflow. This alters the body’s circulatory system as a whole and leads to an improper balance of CSF in the brain. It’s this build-up of fluid in the brain that increases pressure and can lead to serious headaches.

Now, your baby isn’t going to come straight up to you and complain that his or her head his throbbing. You’d think that headaches are quite difficult to spot in your baby, but there are certain signs; just a general indication that your baby’s not feeling well is a common sign. But your baby may convey his or her feelings of being unwell by crying; if it’s a headache issue, your baby’s more likely to moan and groan – balling his/her eyes out isn’t going to help that thumping feelinSo look for frequent headaches and whether they’re connected to something else such as earache.

12 Swallowing Problems

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Another common symptom associated with type 1 Chiari is having difficulty with swallowing. It may feel as if something is obstructing the esophagus, so your baby might gag when drinking or eating, may vomit and could choke which could be very alarming.

These swallowing problems can be attributed to the increased pressure in the brain due to the obstruction and thus accumulation of CSF. This could result in a misshapen skull or it could cause other structures to change, some of which could have an adverse impact on your body’s swallowing mechanisms. One common change is that the tonsils are slightly lower than they normally should be. This could mean that they protrude down through the hole in the base of the skull – known as the foramen magnum – and cause gagging and swallowing problems, problems that aren’t life-threatening but can be seriously distressing for anyone, let alone a baby.

11 Sneezing, Coughing, Straining

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These aren’t symptoms of Chiari as such. But it’s what happens after you baby sneezes, strains or coughs that you need to look out for.

We’ve mentioned that one of the most common signs of Chari malformation is headaches. These could just come on randomly, but very often they’re triggered by something. It’s not uncommon for your baby to sneeze or cough now and again, and often it’s not anything to worry about. But just keep an eye on your baby after he/she sneezes or coughs and look for any adverse reactions. Very often in the case of Chiari, sneezing and coughing can lead to headaches, that dull throbbing feeling at the back of the head, which could get progressively worse and cause your baby an immense amount of distress. So if your baby begins to feel unwell after a little cough or sneeze, it could be a sign that he/she is suffering from a headache – look out for these triggers.

10 Immature Spines

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Now all babies and young children have immature spines – they have immature bone structures full stop. But you should be able to tell that your baby’s back is straight, that the spine is doing its job. The spine is one of the first structures to actually develop, along with other vital organs such as the brain and heart, during the fifth week of pregnancy. That’s because the spine is vitally important, not just because it holds you upright, but also because it acts as a highway for all the nerves and fibres which connect different part of the body to the brain.

Infantile Scoliosis could very well point to Chiari. This may have been started because of a flattening of the skull, or it may cause a flattening of the skull and cause Chiari – either way it’s a very serious problem. The distinct curvature of the spine may straighten out as your baby grows, but still, observe and keep an eye on it.

9 Bone Defects

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This is a take on Immature Spine, but it’s more about the overall bone development of the spine and surrounding structures. Spina bifida refers to lack of spine development – not just improper development such as spine curvature – and abnormalities in its protective covering.

These malformations are usually prevalent in those with type 2 Chiari and could include a number of problems. In some instances, the lowest part of the spine extends out into a sac-like opening. In other cases, the bones holding the spine together and surrounding the spine just don’t form properly at all which could lead to serious back problems and even paralysis.

You’ll know if your baby experiences paralysis, there’s no question about that. But when it comes to back problems, you’re going to have to wait for your baby to grow up a little bit and begin walking before signs become apparent. If your baby has problems walking, or experiences pain when upright, it could be due to spinal problems, so notify your doctor.

8 Severe Back Pain

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Syringomyelia is a congenital malformation relating to Chiari type 1. It’s a chronic disorder and can be seriously debilitating.

Chiari malformations occur when the lower part of the brain pushes down into the spinal cord, through the little opening. This obstructs CSF flow, and alters the normal CSF input-output, which can cause a build-up of pressure in addition to many other problems. One such problem that it can cause is syringomyelia.

So Chiari can obstruct and trap CSF in the brain. But because the flow is obstructed, the CSF has to go somewhere. CSF isn’t usually found in the spinal cord – it usually bathes the spinal cord, surrounding it and cushioning it. But in the case of syringomyelia, it enters the spinal cord, which can stretch the spinal cord – can cause a number of problems if this occurs in babies because the spinal cord is still developing – and can mess with the intricate system of nerves and fibres which in turn can cause a whole host of problems.

Symptoms can be as simple as back pain, improper development, or they could be more severe. In some instances, paralysis could occur, loss of pain – so if your baby doesn’t feel the difference between hot and cold for example – and strength – not being able to use arms, lift them or move them about due to muscle weakness.

7 Wide Spinal Cord

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Hydromyelia and syringomyelia are very similar, however they can be differentiated from one another.

Hydromyelia is the name given to the widening of the spinal cord – more specifically the central canal of the spinal cord that runs longitudinally, top to bottom, through the spinal cord. I know we’ve just mentioned in the previous section that CSF bathes the spinal cord and isn’t found inside it. Well, the central canal is the exception and is the only bit of the interior of the spinal cord that contains CSF. In the case of hydromyelia, because the spinal cord widens, it allows more CSF into the central canal, causing a build-up of pressure in certain regions of spine which could cause a number of problems, including general back pain and nerve problems.

Symptoms of hydromyelia in your baby could be severe back and upper arm pain, and extreme weakness of the arms, stiffness in the legs. Hydromyelia is usually always associated with those who are born with Chiari malformation 2.

6 Spasticity or Baby Jerking

Spasticity in babies could be due to any number of problems, but usually involve issues with the portion of the brain – the largest part of the brain called the cerebrum – that’s responsible for controlling involuntary muscle movements, and problems with the spinal cord.

It can be very distressing to see your baby jerk around with spastic movements, but the underlying cause could be Chiari.

We’ve just discussed hydromyelia and syringomyelia, both of which involve CSF entering the spinal cord. When CSF enters the spinal cord – the all-important highway of nerve bundles and different fibres – could be affected. CSF fluid could accumulate in certain regions of the spinal cord and pool together, putting pressure on the spinal structure but also on bundles on nerves, damaging nerve cells and their all-important connections to the brain and the rest of the body. Spasticity as a result of Chiari could develop over time, or it could be noticeable immediately when your baby is born.

5 Seeing Is Believing 

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The brainstem is responsible for a number of vital physiological functions, so Chiari could affect different systems.

The main symptom is headaches, often a throbbing feeling at the back of the head, but these could then radiate behind the eyes and worsen – or start – because of coughing or sneezing. Your baby could display signs of having a headache by being especially irritable, having trouble sleeping, and sometime head banging could indicate a headache. If the headache radiates behind the eyes, it could also cause your baby to tightly close his/her eyes, because light, or hypersensitivity to bright lights may cause more pain and make matters worse.

There are also other visual problems you can look out for. It’s hard to tell whether your baby is experiencing double vision, but nystagmus – jerky eye movements back and forth – is a common sign, as is having difficulty tracking objects and following them with normal eye movements.

4 Baby Can’t Breathe Right 

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Sleep apnea is a disorder which refers to the fact that your baby is having trouble breathing at night when trying to sleep. Sleep apnea and a variety of other respiratory problems can be caused because of Chiari, because of the intricate involvement of the brainstem in the way we breathe.

Breathing is something you don’t even think about doing because the brain takes care of everything. Oxygen and carbon dioxide go through the brainstem. You brain then deciphers the concentration of each in the blood and adjusts your breathing accordingly so you either breathe more to take in more oxygen, or less, at night for example when not as much oxygen is required. When sleeping, breathing is completely reliant on the brainstem performing this function, so if there’s pressure in this region due to the build-up of CSF fluid and Chiari, you can imagine the impact this will have on respiratory functions. Blood gasses won’t be able to be measured as well, so breathing can’t be assessed and will become affected.

Periodic breathing – rapid breaths, no breaths, short breaths, gasping and just overall irregular breathing patterns is a sure sign of sleep apnea.

3 Ringing In The Ears

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If the baby has a headache or just keeps rubbing its ear, it could point to tinnitus, characterized by a ringing or buzzing in the ears.

Tinnitus may be one of the first symptoms of Chiari. This is because sometimes not just CSF, but tissues could extend down through that hole in the spinal cord which could affect a range of different functions. Tinnitus may also occur due to the pressure that’s built up in the brain. Head pressure issues could result in pressure changes in the ear which could in turn cause tinnitus. Worst case scenario, it could even cause total hearing loss – obviously noticeable in a baby because he/she won’t respond, won’t react when you say something.

Ear problems could also cause dizziness, problems balancing and clumsiness; but this is what babies do anyway, so these are the symptoms to look out for when your child gets slightly older.

2 Baby’s Not Sleeping 

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Again, babies have irregular sleeping patterns – they’re often awake when you’re trying to sleep and vice versa, but if your baby doesn’t seem to be sleeping at all, or can’t seem to get to sleep, it could be a symptom.

Sleep apnea -which we’ve already touched upon – could of course cause a lack of sleep, but it could also be as a result of a number of other functions which have been affected by Chiari. Swallowing problems, respiratory problems, headaches, arms and upper body pain, neck pain, numbness, tinnitus – you try and sleep with a constant buzzing, tingling sound in your ears! – and just overall pain and a general feeling of being unwell could prevent your baby from sleeping.

Let’s just reiterate, that lack of sleep could of course be caused by any number of things, not solely Chiari. But lack of sleep combined with these other symptoms could mean that your baby is suffering from this malformation.

1 It’s More Than Just Sickness 

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Again, sickness alone of course doesn’t point straight to Chiari. Little ones can be sick due to any number of reasons, so think about why the baby is being sick. Is it due to an aversion to a certain type of food, new formula? Or is because of some other underlying issues, such as pulsating headaches, ear aches, swallowing problems and gagging, or just unpleasant pain? Recognize the signs and think about the reasons for the sickness.

Nausea, vomiting and abdominal pain are also common signs of Chiari. Chiari can be extremely difficult to diagnose, especially in young ones, because the malformation usually becomes apparent later in life during adulthood. The malformation also causes a diverse range of symptoms, so your doctor will be very reluctant to put your baby through an MRI scan just because he/she is vomiting, and has one or a couple of other problems. It’s even harder to spot in babies, because of course, the communication factor isn’t there. But if you’ve read this article and find yourself ticking off the symptoms one by one, it’s time to notify the doctor of your concerns and get the baby checked out.

Sources: RareDiseases.org, Ncbi.nlm.nih.gov

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