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Craniosynostosis : Causes, Types, Symptoms, Diagnosis and Treatment

 What is craniosynostosis?

Craniosynostosis happens once a baby’s os bones fuse too early. It causes issues with the form of the baby’s os. With early treatment, most youngsters do not experience the other craniosynostosis symptoms. Babies might bear helmet medical aid or surgery to correct the os form.

A newborn baby’s os consists of many bones that match along. Usually, newborns have areas referred to as sutures between their os bones. The sutures let the os size grow to accommodate the baby’s growing brain. Once the bones of the os are united along either at birth or fuse timely, the condition is termed craniosynostosis.


The sutures of the os fuse round the brain at around age two years. Once a baby has craniosynostosis, one or a lot of those sutures hardens too early and closes before the baby reaches age two.

In places wherever sutures have united too early, a baby’s head might stop growing. In alternative areas, wherever sutures haven’t united, the baby’s head can still grow. As a result, babies with craniosynostosis usually have heads that are asymmetrical (misshapen).


If a baby has multiple sutures that shut too early, the brain may not have enough space to grow. As a result, these babies may expertise a buildup of pressure within the os (intracranial pressure).


What is craniosynostosis



Explanation of medical terms and concepts Craniosynostosis

Craniosynostosis (kray-nee-o-sin-cos-TOE-sis) may be a anomaly within which one or a lot of of the fibrous joints between the bones of your baby's os (cranial sutures) shut untimely (fuse), before your baby's brain is totally fashioned. Brain growth continues, giving the top a distorted look.

Normally, throughout infancy the sutures stay versatile, giving your baby's brain time to grow. within the front of the os, the sutures meet within the giant soft spot (fontanel) on high of the top. The anterior orifice is that soft spot you are feeling simply behind your baby's forehead. consecutive largest is at the rear (posterior). Both sides of the os feature a small orifice.

Craniosynostosis typically involves premature fusion of one os suture, however will involve over one amongst the sutures in your baby's os (multiple suture craniosynostosis). In rare cases, craniosynostosis is caused by bound genetic syndromes (syndromic craniosynostosis).

Treating craniosynostosis involves surgery to correct the form of the top and permit for traditional brain growth. Early identification and treatment enable your baby's brain adequate area to grow and develop.

Although neurologic harm will occur in severe cases, most kids have traditional psychological feature development and bring home the bacons} good cosmetic results when surgery. Early identification and treatment square measure key.

 Craniosynostosis is a condition that occurs when there is an error in the formation of skull sutures The skull sutures are joints between the different plates of bone in the baby's skull Usually these joints close up during the first few months after birth but sometimes they do not form and remain open Surgeons can correct this problem by putting clips on each side of the brain and then closing the openings with small plates or screws Some patients have to return to have their head reshaped as they grow older This may include removing excess tissue from around areas where bones fused together prematurely.

Craniosynostosis is a birth defect that results in the fusion of one or more of the joints between two or more of the skull bones This can result in an abnormal shape to the head and limit growth While craniosynostosis can occur at any age it most often occurs within the first six months after birth It can affect one bone (unicameral) or multiple bones (multicameral) Craniosynostosis may be present at birth but not noticeable until later childhood when growth spurts occur and pressure on the skull increases Sometimes a child's skull changes.

Symptoms Craniosynostosis

The primary symptom of craniosynostosis could be a distorted bone. If babies receive early surgical operation, they'll not experience the other craniosynostosis symptoms.

The signs of craniosynostosis are typically noticeable at birth, however they will become additional apparent throughout the primary few months of your baby's life. Signs and severity rely upon what number sutures are unit united and once in brain development the fusion happens. These will include:

  • A misshapen skull, with the shape depending on which of the sutures are affected

  • An abnormal feeling or disappearing fontanel on your baby's skull

  • Development of a raised, hard ridge along affected sutures

  • Slow or no growth of the head as your baby grows

Types of craniosynostosis

There are many styles of craniosynostosis. Most involve the fusion of one os suture. Some advanced types of craniosynostosis involve the fusion of multiple sutures. Most cases of multiple suture craniosynostosis area unit coupled to genetic syndromes and area unit known as syndromic craniosynostosis.

The term given to every style of craniosynostosis depends on what sutures are affected. styles of craniosynostosis include:

  • Sagittal (scaphocephaly). Premature fusion of the suture that runs from the front to the rear at the highest of the os forces the top to grow long and slim. Mesial craniosynostosis leads to a head form known as malformation and is the commonest kind of craniosynostosis. 

  • Coronal. Premature fusion of 1 of the wreath sutures (unicoronal) that run from every ear to the highest of the bone might cause the forehead to flatten on the affected aspect and bulge on the unaffected aspect. It conjointly results in turning of the nose and a raised orbital cavity on the affected aspect. Once each wreath sutures fuse untimely (bicoronal), the pinnacle includes a short and wide look, usually with the forehead leaning forward. 

  • Metopic. The metopic suture runs from the highest of the bridge of the nose up through the midplane of the forehead to the anterior orifice and also the fibrous joint. Premature fusion offers the forehead a triangular look and widens the rear a part of the top. This is often conjointly known as trigonocephaly. 

  • Lambdoid. Lambdoid synostosis is a rare type of craniosynostosis that involves the lambdoid suture, which runs along the back of the head. It may cause one side of your baby's head to appear flat, one ear to be higher than the other ear and tilting of the top of the head to one side.

Other reasons for a misshapen head

A distorted head does not continually indicate craniosynostosis. As an example, if the rear of your baby's head seems planar , it can be the result of disbursing an excessive amount of time on one aspect of his or her head. This could be treated with regular position changes, or if vital, with helmet medical care (cranial orthosis) to assist reshape the top to an additional traditional look.

When to see a doctor

Your doctor can habitually monitor your child's head growth at well-child visits. confer with your specialist if you have got considerations concerning your baby's head growth or form.

Causes Craniosynostosis

In most babies, consultants cannot establish one acknowledged reason behind craniosynostosis. Sometimes, craniosynostosis happens as a result of an unpredictable (random) point mutation (change), or it should run in families. immaturity could be a risk issue for craniosynostosis.

In alternative cases, some factors throughout physiological state increase a baby’s risk for developing craniosynostosis. These embody

Fertility medications like clomiphene (Clomid®).

Maternal thyroid unwellness (developing thyroid unwellness whereas pregnant).

Often the cause of craniosynostosis is not known, but sometimes it's related to genetic disorders.

  • Nonsyndromic craniosynostosis is the most common type of craniosynostosis, and its cause is unknown, although it's thought to be a combination of genes and environmental factors.

  • Syndromic craniosynostosis is caused by bound genetic syndromes, like Apert syndrome, Pfeiffer syndrome or Crouzon syndrome, which might have an effect on your baby's bone development. These syndromes typically conjointly embody alternative physical options and health issues. 

Complications

If untreated, craniosynostosis may cause, for example:

  • Permanent head and facial deformity

  • Poor self-esteem and social isolation

The risk of increased pressure inside the skull (intracranial pressure) from simple craniosynostosis is small, as long as the suture and head shape are fixed surgically. But babies with an underlying syndrome may develop increased intracranial pressure if their skulls don't expand enough to make room for their growing brains.

If untreated, increased intracranial pressure can cause:

  • Developmental delays

  • Cognitive impairment

  • No energy or interest (lethargy)

  • Blindness

  • Eye movement disorders

  • Seizures

  • Death, in rare instances

Diagnosis Craniosynostosis

Healthcare suppliers sometimes will diagnose craniosynostosis by feeling for soft spots on your baby’s head, feeling for ridges that signify united bone sutures and menstruation the top circumference.

If the scale of your baby’s head isn't growing of course, the care supplier can check for craniosynostosis. It’s necessary to recollect that a small-sized head may be thanks to many alternative reasons moreover. Your baby might have an Associate in Nursing X-ray or CT scan of the top to substantiate this designation.

Craniosynostosis needs analysis by specialists, like a pediatric sawbones or a specialist in plastic and surgical operation. designation of craniosynostosis might include:

  • Physical exam. Your doctor will feel your baby's head for abnormalities such as suture ridges, and look for facial deformities.

  • Imaging studies. A computed axial tomography (CT) scan or resonance imaging (MRI) of your baby's os will show whether or not any sutures have consolidated. Bone ultrasound imaging is also used. consolidated sutures square measure recognizable by their absence, as a result of they are invisible once consolidated, or by the ridging of the suture line. An optical device scan and images additionally want to create precise measurements of the os form. 

  • Genetic testing. If your doctor suspects an underlying genetic syndrome, genetic testing may help identify the syndrome.

Treatment Craniosynostosis

Many types of craniosynostosis need surgery. The operation is supposed to alleviate pressure on the brain, correct the craniosynostosis, and permit the brain to grow properly. Once required, an operation is sometimes performed throughout the primary year of life. But, the temporal order of surgery depends on that sutures area unit closed and whether or not the baby has one amongst the genetic syndromes which will cause craniosynostosis.

Babies with terribly delicate craniosynostosis may not like surgery. because the baby gets older and grows hair, the form of the bone will quieten down noticeable. Sometimes, special medical helmets will be worn to facilitate mildew the baby’s bone into an additional regular form.

Each baby born with craniosynostosis is totally different, and also the condition will vary from delicate to severe. Most babies with craniosynostosis are otherwise healthy. Some youngsters, however, have organic process delays or intellectual disabilities, as a result of either the craniosynostosis has unbroken the baby’s brain from growing and dealing unremarkably, or as a result of the baby encompasses a genetic syndrome that caused each craniosynostosis and issues with however the brain works. A baby with craniosynostosis can have to be compelled to see an attention supplier often to form positive that the brain and bone area unit is developing properly. Babies with craniosynostosis will typically get pleasure from early intervention external icon services to assist with any organic process delays or intellectual issues. Some youngsters with craniosynostosis might have problems with vanity if they're involved with visible variations between themselves and alternative youngsters. Parent-to-parent support teams can also be helpful for brand spanking new families of babies with birth defects of the top and face, as well as craniosynostosis.

Mild cases of craniosynostosis might not like treatment. Your doctor might suggest a specially shaped helmet to assist reshape your baby's head if the bone sutures area unit is open and also the head form is abnormal. During this state of affairs, the shaped helmet will assist your baby's brain growth and proper the form of the bone.

However, for many babies, surgery is the primary treatment. The sort associate degreed temporal order of surgery depends on the sort of craniosynostosis and whether or not there is an underlying genetic syndrome. generally over one surgery is needed.

The purpose of surgery is to correct the abnormal head form, cut back or forestall pressure on the brain, produce area for the brain to grow unremarkably, and improve your baby's look. This involves a method of coming up with surgery.

Surgical planning

Imaging studies will facilitate surgeons develop a surgery arrangement. Virtual surgical designing for treatment of craniosynostosis uses high-definition 3D CT scans and MRIs of your baby's os to construct a computer-simulated, individualized surgical arrangement. supported that virtual surgical arrangement, bespoken templates are made to guide the procedure.

Surgery

A team that features a specialist in surgery of the pinnacle and face (craniofacial surgeon) and a specialist in operation (neurosurgeon) usually performs the procedure. Surgery is done by examination or open surgery. Each variety of procedure usually turns out superb cosmetic results with low risk of complications.

  • Endoscopic surgery. This minimally invasive surgery is also thought-about for babies up to age half-dozen months. employing a lighted tube and camera (endoscope) inserted through tiny scalp incisions, the Dr. removes the affected suture to change your baby's brain to grow commonly. Compared with associate degree open procedure, scrutiny surgery features a smaller incision, usually involves solely a one-night hospital stay and typically doesn't need an introduction. 

  • Open surgery. Generally, for babies older than six months, open surgery is completed. The operating surgeon makes AN incision within the scalp and os bones, then reshapes the affected portion of the os. The os position is controlled in situ with plates and screws that square measure absorbed. Open surgery generally involves a three- or four-day hospital keep, and transfusion is sometimes necessary. It's typically a one-time procedure, however in advanced cases, multiple open surgeries square measure usually needed to correct the baby's head form. 

Helmet therapy

After examination surgery, workplace visits at certain intervals are needed to suit a series of helmets to assist form your baby's os. The MD can confirm the length of helmet medical care supported however quickly the form responds to treatment. If open surgery is completed, no helmet is required after. 

Coping and support

When you learn that your baby has craniosynostosis, you will experience a spread of emotions, as well as anger, fear, worry, sorrow and guilt. you will not understand what to expect, and you will worry regarding your ability to worry for your baby. The most effective counterpoison for concern and worry is info and support.

Consider these steps to organize yourself and to worry for your baby:

  • Find a team of trusted professionals. You'll need to make important decisions about your baby's care. Medical centers with craniofacial specialty teams can offer you information about the disorder, coordinate your baby's care among specialists, help you evaluate options and provide treatment.

  • Seek out other families. Talking to folks that are coping with similar challenges will offer you data and emotional support. raise your doctor concerning support teams in your community. If a bunch is not for you, perhaps your doctor will place you up-to-date with a family UN agency that has prohibited craniosynostosis. otherwise you could also be able to notice cluster or individual support on-line. 

  • Expect a bright future. Most children have traditional psychological feature development and come throughs} good cosmetic results once surgery. Early diagnosing and treatment square measure key. Once required, early intervention services are facilitated with organic process delays or intellectual disabilities. 

Preparing for your appointment

In some cases, your baby's podiatrist might suspect craniosynostosis at a routine well-baby visit. In alternative cases, you will build a meeting as a result of you have got considerations concerning your baby's head growth. Your doctor will refer you to a specialist for diagnosing and treatment

Here's some data to assist you make preparations for your appointment. If doable, bring a friend or friend with you. A sure companion will assist you bear in mind data and supply emotional support.

What you can do

Before the appointment, make a list of:

  • Any signs you've noticed, such as raised ridges, a change in the shape of your baby's face or head, or the absence of soft spots on your baby's head

  • Questions to ask your doctor

Questions to ask might include:

  • What's the most likely cause of my baby's symptoms?

  • Are there other possible causes?

  • What kinds of tests does my baby need? Do these tests require any special preparation?

  • What treatments are available, and which do you recommend?

  • Are there alternatives to the treatment you're recommending?

  • What are the risks involved with surgery?

  • Who will perform the surgery if it's needed?

  • What happens if we choose not to have the surgery right now?

  • Will the abnormal shape of the skull affect the functioning of my baby's brain?

  • What is the likelihood of future children having the same condition?

  • Are there brochures or other printed material that I can have?

  • What websites do you recommend?

Don't hesitate to ask other questions during the appointment.

What to expect from your doctor

Your doctor is likely to ask you questions, such as:

  • When did you first notice the changes in your baby's head?

  • How much time does your baby spend on his or her back?

  • In what position does your baby sleep?

  • Has your baby had any seizures?

  • Is your baby's development on schedule?

  • Were there any complications during your pregnancy?

  • Do you have a family history of craniosynostosis or genetic conditions such as Apert syndrome, Pfeiffer syndrome or Crouzon syndrome?

Your doctor will ask additional questions based on your responses. Preparing and anticipating questions will help you make the most of your time with the doctor.

General summary

Yes craniosynostosis can be corrected In most instances of craniosynostosis the bones in a baby's head will want to fuse too early and prevent the normal growth of the skull When this happens a surgeon must cut out part of the fused skull bone that is restricting growth so that it can open up again This surgery allows the baby’s brain enough room to grow normally

When should craniosynostosis be treated?

Most pediatric neurosurgeons maintain that it is crucial to see the child early and determine whether or not surgery is needed If a baby has unequal head-growth skull deformities or mental impairment treatment should begin as early as possible to prevent potential costs later on

Does mild craniosynostosis need surgery?

Craniosynostosis is a condition in which certain skull bones fuse together before they are supposed to This causes the head of the patient to grow abnormally often leading to an elongated or misshapen skull Mild craniosynostosis may not require treatment since there are many ways it can be managed without surgery Sometimes however mild craniosynostosis does need surgical intervention for correction of deformities and prevention of future health problems The decision whether or not to operate depends upon a number of factors including family history and severity of the symptoms

Can a helmet fix craniosynostosis?

Yes a helmet can help alleviate the pain and discomfort associated with craniosynostosis a condition that results in fused skull bones The pressure and irritation of the expanding brain against the rigid bone can cause serious complications if not promptly treated Although it may seem counterintuitive to use a helmet to fix something like this helmets provide stability and relieve pressure on the head They also prevent further damage from occurring inside the skull by distributing forces throughout the entire helmet structure instead of concentrating them at one point

At what age is craniosynostosis diagnosed?

Craniosynostosis can be diagnosed at any age however the most common time for diagnosis is between 6 weeks and 1 year of age The closure of the cranial sutures usually occurs during an infant's first year If these sutures close prematurely craniosynostosis may be present In order to diagnose craniosynostosis a physician will typically perform a physical examination evaluate the child's head circumference growth curve measure various parts of the head with calipers obtain x-rays and compare them with those of a healthy newborn or infant

What happens if craniosynostosis is left untreated?

Craniosynostosis occurs when the sutures of an infant’s skull fuse together prematurely This can happen for a variety of reasons including developmental abnormalities improper oxygen flow to the brain or exposure to certain chemicals during pregnancy When craniosynostosis is left untreated it can lead to complications such as vision problems and mental retardation Craniosynostosis is diagnosed through x-ray imaging of the head and might be treated with surgery if other interventions don’t work

What causes a baby's skull to close too soon?

When the head is still growing at birth a premature baby has an underdeveloped brain The skull hasn't yet closed and this immaturity leaves it susceptible to infection Premature babies are also susceptible to respiratory distress syndrome which prevents normal oxygen uptake by their bloodstream causing damage to the baby's lungs This can result in lifelong lung problems or even death Both infections and respiratory distress syndrome are treated with antibiotics and may be reversed if caught early enough but they can have serious long-term effects on a premature baby's development

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Craniosynostosis : Causes, Types, Symptoms, Diagnosis and Treatment

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