Noonan syndrome and scoliosis

Noonan syndrome is a congenital disorder that can impact the formation and development of several areas of the body.

Characterised by a variety of distinctive features – including facial abnormalities, stunted height and heart defects – Noonan syndrome is caused by a mutation of one or more genes.

According to the NHS, it's estimated that the number of children born with Noonan syndrome is somewhere between 1 in 1000 and 1 in 2500. The condition affects all ethnicities and sexes equally.

Notably, children with Noonan syndrome are also more susceptible to spinal conditions such as scoliosis.

 

Scoliosis as a result of Noonan syndrome

In 2001, a Korean study carried out by the Department of Orthopaedic Surgery in Seoul National University Hospital scientifically proved the correlation between scoliosis and Noonan syndrome.

Of the 60 patients with Noonan syndrome included in this study, 30% were found to have spinal deformities. Of those patients, two patients had congenital deformities, while the remaining 16 were diagnosed with scoliosis.

Based on the evidence provided, the study concluded that scoliosis with an associated thoracic lordosis occurs more frequently in Noonan syndrome than had been previously reported.

Today the relationship is well-documented, and early assessment of children with Noonan syndrome is recommended to ensure prompt detection and advanced treatment of scoliosis symptoms.

 

Treating scoliosis in Noonan syndrome patients

We at the Scoliosis SOS Clinic treat scoliosis in people of all ages and backgrounds. Our non-surgical treatment courses are ideal for all manner of individuals, including those with Noonan syndrome.

Combining the tried and tested Schroth method with an assortment of additional complementary techniques, our ScolioGold programme is designed to promote natural correction of asymmetric posture without invasive surgery. Our treatment method has an unrivalled level of success, providing significant relief to most patients within just a few weeks.

Better still, our methods are continuously evolving in line with medical breakthroughs and non-surgical development, allowing us to modify and improve our programme as the scientific study of scoliosis advances.

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Sad dog waiting

The people of the UK are very lucky to have the National Health Service, but it's fair to say that the NHS is far from perfect.

Issues like crowded waiting rooms, bed shortages and understaffing have plagued hospitals across the nation for decades. These problems are largely caused by insufficient NHS funding.

However, for scoliosis sufferers seeking spinal treatment on the NHS, one negative outweighs all others: the notorious length of NHS waiting lists.

 

NHS Waiting Times

Unfortunately, a lot of NHS services come with interminable waiting times. Even a simple X-ray can take weeks to arrange, while surgical operations and other treatments can roll into the months, depending on the perceived urgency.

According to www.nhs.uk, the maximum waiting time for non-urgent, consultant-led treatments is 18 weeks from the day your appointment is booked through the NHS e-Referral Service or the hospital receives your referral letter.

If the hospital cancels your operation at the last minute for non-clinical reasons, they should offer another binding date within 28 days; however, if your operation is cancelled before the day of admission, the hospital is not obliged to provide an alternative option within 28 days.

 

Luck of the Draw?

This NHS patient information pamphlet on scoliosis surgery simply states that "there is usually a wait before your operation, and the amount of time can vary".

You may be seen sooner if your case is especially urgent, but in most cases, the length of your wait will come down to luck.

 

Hospital waiting room

The Effects of Waiting Times on Scoliosis

A study published in Orthopaedic Proceedings reported that, according to an independent investigation of 61 scoliosis patients, the average waiting time for scoliosis surgery was actually ten months.

Worse still, the extended waiting time resulted in 20% of those patients suffering "significant curve progression" as a result, and 10 of the 61 patients (approximately 16%) required more extensive surgery than originally planned.

The report concluded that long waiting times can have a detrimental effect on the surgical management of scoliosis patients.

 

ScolioGold: An Alternative to Waiting

The stats and figures surrounding NHS waiting times can be rather alarming for people with scoliosis, especially for those awaiting surgical treatment.

But there may be another way. The Scoliosis SOS Clinic run regular scoliosis treatment courses, allowing you to choose your preferred treatment dates instead of spending months on a waiting list.

After your initial consultation, you will be given a summary of the clinical diagnosis, informed of the proposed treatment (including expected outcomes) and given an overview of our estimated timescales.

Our therapists treat scoliosis patients using the ScolioGold method, which encompasses a variety of proven non-surgical techniques such as the Schroth method and FITS.

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Hyperkyphosis

Hyperkyphosis (often just called kyphosis) is a curvature of the spine that occurs in the upper back, resulting in a hunched or stooped appearance.

It affects approximately 8% of the general population, and while it is most common in older people, it can affect men and women of all ages.

Watch our video to learn everything you need to know about hyperkyphosis:

Kyphosis is sometimes known as 'dowager's hump', particularly if the patient is getting on in years. 'Hunchback' is a somewhat derogatory term for a person with kyphosis.

 

Hyperkyphosis vs Scoliosis: What's the Difference?

Hyperkyphosis and scoliosis are two different curvatures of the spine.

We treat both conditions here at the Scoliosis SOS Clinic, and they can occur together (see kyphoscoliosis). But they're not the same thing.

Kyphosis vs scoliosis

The difference lies in the direction of the curvature:

  • Hyperkyphosis: Causes the upper* region of the spine to curve forwards, making the patient's upper back look unusually rounded or hunched.

  • Scoliosis: Causes the spine to curve sideways, forming a 'C' or 'S' shape.

*Excessive forward curvature of the lower spine is known as hyperlordosis. Read our Curvatures of the Spine guide for more information.

 

How Does Hyperkyphosis Affect the Body?

Kyphosis patients can usually be recognised by their visibly hunched backs, but this is just one of the many ways in which hyperkyphosis can affect one's body.

Kyphosis patients

Other symptoms of kyphosis include:

  • Back pain
  • Stiffness and discomfort
  • Reduced mobility / flexibility
  • Fatigue
  • Poor body image

But that's not all. A severe kyphotic spinal curve can even interfere with the body's most fundamental inner workings, such as the respiratory and digestive systems.

> How does hyperkyphosis affect breathing?

If hyperkyphosis is not treated and the spinal curve continues to get worse over time, there is a risk that it may eventually begin to adversely affect the patient's ability to breathe. This happens because especially severe spinal deformities inevitably end up warping other parts of the skeleton, including the rib cage; this leaves the lungs with less room to inflate, resulting in compromised breathing.

> How does hyperkyphosis affect the digestive system?

Severe hyperkyphosis can also impact on the patient's ability to digest food normally. Again, this is due to the knock-on effect that a pronounced spinal curve can have on other parts of the body. In the case of the digestive system, problems may arise because the patient's internal organs are being squashed together, potentially obstructing the passage of food through the intestines. Acid reflux is also fairly common among people with advanced hyperkyphosis.

> How does hyperkyphosis affect the nervous system?

In some cases, the distortion of the body due to hyperkyphosis can end up impinging on a nerve. Depending on where in the body this happens, nerve compression can lead to:

  • Persistent aches/pains
  • Numbness or tingling sensations
  • Certain body parts feeling weak
  • Loss of bladder/bowel control

The good news is that all of these consequences are relatively rare and do not arise in the majority of mild to moderate cases of hyperkyphosis.

 

What Causes Hyperkyphosis?

Kyphosis can develop for a number of different reasons, and some forms of this condition are more preventable than others.

Here are some of the most common causes of hyperkyphosis (and who they're most likely to affect):

  • Bad Posture - If you persistently slouch forward or lean back when seated, you may notice that your spine starts to develop a visible curvature over time. Desk workers are particularly prone to the sort of postural problems that can lead to hyperkyphosis.

  • Scheuermann's Disease - Scheuermann's disease typically occurs during the growth spurt that accompanies puberty. If you have this condition, it means that your vertebrae (the bones that make up your spine) develop into a wedge shape, creating a forward spinal curve. Learn more about Scheuermann's disease here.

  • Congenital Issues - While rare, it is sometimes the case that a baby's spine will develop incorrectly in the womb, and this can mean that hyperkyphosis is present from birth. This is called congenital kyphosis, and when it does occur, it usually begins within the first 6-8 weeks of embyronic development.

  • Osteoporosis - Human beings (especially women) often lose bone density as they get older, a condition known as osteoporosis. The resulting bone weakness can lead to a range of different problems, including curvature of the spine. Learn more about osteoporosis here.

  • Spinal Injury - Certain accidents and injuries can impact the spine, resulting in hyperkyphosis in some cases.

 

How to Prevent Hyperkyphosis

While hyperkyphosis can be treated, it is often impossible to prevent it from developing altogether.

Scheuermann's kyphosis and congenital kyphosis cannot be prevented with lifestyle changes. Good posture will reduce your risk of developing postural kyphosis, and a healthy diet and weight-bearing exercises can help to prevent kyphosis from developing as a result of osteoporosis.

If you want to prevent hyperkyphosis, here's what we recommend:

  • Avoid rounding your shoulders and make an effort to observe your posture when sitting, walking or standing.

  • Perform exercises which increase bone mass - rebounding on a trampoline is very effective for this, and is even used by astronauts preparing for space travel.

  • Eat a diet rich in Calcium and Vitamin D, such as spinach, fatty fish and fortified foods.

  • Perform exercises to improve your posture at home, especially if you work in a job that requires you to sit for long periods of time or lift heavy objects. Here are some exercises to get you started.

  • Seek physical therapy from a qualified professional who will be able to identify the cause of your poor posture.

 

Hyperkyphosis Treatment

As with scoliosis, there are a number of different hyperkyphosis treatment methods in use, including both surgical and non-surgical options.

Congenital Kyphosis Treatment

The most prevalent treatment methods are:

  • Bracing - During adolescence, bracing may be required to stunt the progression of the patient's kyphosis in moderate to severe cases. Bracing aims to ensure that the degree of the curvature does not develop any further than it already has. The patient may be required to continue wearing the brace until their spine stops growing at around 16 years of age. Learn more about how a hyperkyphosis brace works here.

  • Pain Management - As is the case for many health problems, pain management is often a central part of hyperkyphosis treatment. Painkillers such as ibuprofen or paracetamol can help to relieve the aches and pains that derive from having a curvature of the spine. If the patient is in a lot of pain, stronger pain relief medications may be prescribed.

  • Physical Therapy - Though it does require some work on the patient’s part, physiotherapy is a great way to treat hyperkyphosis. Physical therapy programmes such as our own ScolioGold method can straighten the back, reduce pain, and improve the patient's quality of life in general - see before and after photos here.

  • Surgery - If the curvature becomes so severe that the patient is having difficulty going about their day, surgery may be recommended. Spinal fusion is the standard surgical procedure for hyperkyphosis - this involves fusing the vertebrae together to correct the spine's curvature. Method rods, screws, hooks and bone grafts are used during the operation to fuse the bones together. The operation takes 4-8 hours, and a back brace may need to be worn for up to 9 months while your spine heals.

 

Matthew from Exeter: A Kyphosis Case Study

Matthew Ellison came to the Scoliosis SOS Clinic in 2018. Our treatment course helped to reduce his back pain, and he actually grew by 3.8cm during his time here!

Read Matthew's story in full here. Matthew is just one of the many hyperkyphosis patients we've helped - if you'd like to find out more about our physical therapy courses, please give us a call on 0207 488 4428.

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Osteogenesis imperfecta

The phrase osteogenesis imperfecta may look like a mouthful, but there's a chance you may be familiar with this condition under a different name.

Osteogenesis imperfecta (OI) is commonly known as brittle bone disease: a genetic condition that prevents adequate production of collagen, resulting in under-developed bones that are naturally more susceptible to fractures.

According to the Osteogenesis Imperfecta Foundation, osteoporosis is an almost universal consequence of this (learn about osteoporosis here). Sadly, it's not the only possible consequence that people with OI sometimes have to deal with.

 

The relationship between osteogenesis imperfecta and scoliosis

While the likelihood of being diagnosed with both osteogenesis imperfecta and scoliosis is extremely rare (0.1 in a million), it's far from an impossibility. In fact, many within the medical community theorise that there is a relationship between the two conditions.

It's believed by some medical professionals that OI can actively contribute to the development of scoliosis. As such, the chances of developing scoliosis are, in theory, likely to increase marginally if you already have OI.

 

Scientific studies on OI and scoliosis

Findings presented by James J. McCarthy at the American Academy of Orthopaedic Surgeons' 74th Annual Meeting in 2007 appeared to confirm this theory. The study focused on childhood OI in particular, and was carried out retroactively.

Of the 288 osteogenesis imperfecta patients studied, 83 were later diagnosed with scoliosis. This represented a 28.8% incidence of scoliosis in existing OI sufferers. What's more, those who underwent corrective surgery for their scoliosis had a high rate of complication.

Meanwhile, a further study published in the Journal of the American Academy of Orthopaedic Surgeons in 2017 noted that, while scoliosis is rarely discovered in OI patients under six years old, it can progress rapidly thereafter.

The journal went on to note that the most common type of scoliosis curve found in OI sufferers was a single thoracic curve (present in 97% of scoliosis patients with type I OI). Meanwhile, 58% of scoliosis patients with type III OI had curves in the thoracic region.

Healthy bone vs brittle bone

Treating scoliosis patients with osteogenesis imperfecta

Due to the fragility of OI patients' bones, scoliosis treatment can be tricky. Manoeuvrability may be limited, and stress placed on the bones could prove to be dangerous.

What's more, younger patients may also exhibit confidence issues and lack of trust in treatment providers, particularly if they have suffered multiple bone fractures in the past.

Here at the Scoliosis SOS Clinic, our aim is to provide safe and effective non-surgical treatment for people with scoliosis. For those who also suffer from osteogenesis imperfecta, our ScolioGold programme is a fantastic way of treating the symptoms of scoliosis without aggravating the symptoms of OI.

From muscular imbalance and trunk rotation to breathing and posture improvement, our specialist treatment courses (led by trained professionals) can be truly life-changing for those who have both OI and scoliosis. The treatment can even be modified to involve fun, interactive exercises for younger patients.

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Scoliosis Explained Infographic