HNPP · Physical Health

Is surgery worth it with HNPP?

HNPP hereditary neuropathy surgery carpal tunnel

Carpal tunnel syndrome seems to be a common occurrence with many HNPP sufferers, and the symptoms sometimes overlap when it comes to nerve damage around the wrists. Not to mention, foot issues such as high arches and unstable ankles appear for some with this particular condition. And the obvious question for those newly-diagnosed tends to be can surgery help stop or relieve the symptoms?

Most people with HNPP have attacks of numbness, tingling and muscle weakness in the leg or foot, which can make walking, climbing stairs and driving difficult or impossible, and can result in foot drop – the inability to lift the front part of the foot because of muscle weakness.

While others can simultaneously have weakness in the elbow, wrist or hands, which can result in wrist drop and loss of sensation in the index finger and thumb (symptoms similar to those of carpal tunnel syndrome), making fine movements such as fastening buttons, typing and gripping impossible.

Disclaimer: Please ask your medical practitioner or doctor for more information. This article is based on various research, journals and testimonies.

What is carpal tunnel syndrome?

Carpal tunnel syndrome or CTS is a common condition that causes a tingling sensation, numbness and sometimes pain in the hand and fingers. These sensations usually develop gradually and start off being worse during the night. They tend to affect the thumb, index finger and middle finger.

The symptoms of CTS also tend to develop gradually, and usually start off being worse at night or early in the morning.

Any repetitive actions of the hand or wrist can aggravate the symptoms, as can keeping your arm or hand in the same position for a prolonged period of time.

Symptoms include:

  • A dull ache and discomfort in the hand, forearm or upper arm
  • A burning, prickling sensation (paraesthesia) in the hand similar to pins and needles
  • Dry skin, swelling or changes in the skin colour of the hand becoming less sensitive to touch (hypoaesthesia)
  • Weakness and wasting away (atrophy) of the muscles at the base of the thumb
  • Pain may spread from your hand up to your forearm and elbow.

CTS ends up becoming a symptom of HNPP, as muscle strain isn’t the cause, rather nerve issues present itself in the form of carpal tunnel syndrome.

Will CTS surgery help?

The problem with getting surgery for CTS is that it may relieve symptoms in the short term, but evidence suggests that it is likely to return after a period of time. A study for The Musculoskeletal Journal of Hospital for Special Surgery presented a 38-year-old HNPP sufferer with complaints of numbness in the first three digits of both hands and weakness of both hands after doing yoga. She was seen by a hand surgeon after wrist splints and anti-inflammatories had no benefit.

“The doctor told me I would have instant relief, which I did. My recovery time about 3 weeks. Disadvantages: I lost 30% strength in my right hand.”

Case from Inspire Forum for Charcot-Marie Tooth

Ten weeks after the diagnosis, she underwent carpal tunnel and Guyon’s canal (surgery to decompress the ulnar nerve) releases, which yielded significant improvement in symptoms within a week. However, after several years, the symptoms returned and instead her hand was left permanently weakened by the surgery.

The authors of the report conclude: “Typically, carpal tunnel release is of little benefit, and ulnar nerve transposition at the elbow may worsen symptoms.

“However, in cases with severe symptoms, debility, and/or deformity, surgical intervention should be considered on an individual basis and in this patient appears to have been beneficial.”

Carpal_tunnel_splint

This shows that surgery should be undertaken on a case by case basis. So you really need to weigh up your options, as it may relieve pain now, but create issues at a later stage.

Another case study featured in the report Is Carpal Tunnel Warranted for HNPP? also warned about having multiple surgeries. Authors Nicholas Earle and Douglas W. Zochodne conducted extensive research on the role of carpal tunnel decompression surgery for those with HNPP.

“[We] do not recommend multiple decompression procedures in an attempt to modify the natural history of HNPP.”

Is carpal tunnel decompression warranted for HNPP?
Nicholas Earle and Douglas W. Zochodne 2013

Two patients with HNPP underwent nerve decompression and surgery for CTS. Despite seeing some improvements, there were issues during surgery for one of the patients in terms of symptoms of HNPP posing itself.

The authors state that even though carpal tunnel decompression could be offered to patients who have “appropriate, persistent, and significant symptoms”, they add: “we do not recommend multiple decompression procedures in an attempt to modify the natural history of HNPP”.

Surgery for anyone increases the risk of nerve damage, from lying still. Individuals with HNPP are at a much greater risk. Nevertheless, knowing about the susceptibility to pressure palsies can help the surgical team avoid causing them by positioning you to avoid pressure palsies.

Correcting foot deformities

I do feel uncomfortable with the word ‘deformity’ but I’ll use this to describe the next lot of symptoms.

It is more common for those with hereditary neuropathies such as Charcot Marie-Tooth disorder, to undergo operations to correct the positioning of a foot. Clawed toes can be straightened, high arched feet can be flattened, and unstable ankles can be tightened.

Blausen_0411_FootAnatomy

For some people with HNPP, the toes begin to claw, causing pain on the ball of the foot, which develops as the deformity places downward pressure on the heads of the metatarsal bone and the protective fat pad beneath them is pulled away by the upturning toes.

Walking becomes painful and wearing shoes becomes difficult, hence many wear cushioned orthotics and special insoles to help alleviate symptoms. Over time, the deformity becomes fixed and conservative treatment no longer helps.

Foot drop, however, is more frequent as Glenn Pfeffer, MD, is the Director of the Foot and Ankle Center at Cedars-Sinai Medical Center reports. In an interview to the Hereditary Neuropathy Foundation, Dr Pfeffer says the muscles that lift up the foot and ankle weakens due to motor nerve issues, eventually causing the foot to drop. While it isn’t a long term solution, the doctor says surgery for patients with CMT and foot drop has “made great advances in the past decade”. But he only recommends surgery after conservative measures such as stretches, physiotherapy, as well as orthotics no longer work.

On a CMT UK forum on Health Unlocked, one person had undergone surgery for foot drop, consisting of four operations and then two subsequent procedures. The online user said: “Weigh up how much of handicap it is now, how much does it hurt and ask your surgeon about the long term future of your foot.”

While others have had more positive outcomes, with another netizen, who had high arches corrected, saying: “Was so incredible. It was life changing for me.”

Either way, whatever you choose to do, it’s important to also remember to provide your medical practitioner, surgeons, nurses and anyone involved with details of your condition, as you may need to have special positioning during surgery.

During surgery

In the Journal of Association of Anaesthetists of Great Britain and Ireland, a case of a woman with HNPP, who underwent a biopsy under general anaesthesia, was left with weakness and decreased sensation of her left arm due to incorrect positioning.

The study added that approximately 16 per cent of all anaesthesia-related claims in the United States was down to peripheral nerve injuries. The ulnar nerve is damaged most commonly (0.33 per cent of general anaesthetics); this is three times as common as injury to other nerves. Hence it is essential that you let your medical team know about your condition well in advance.

In the end, it is your choice if you want to go ahead with surgery, but the more knowledge you have beforehand on what to expect, the better the decision you will make and the outcome will be.

4 thoughts on “Is surgery worth it with HNPP?

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