Friday, October 23, 2015

Radial Deficiency, Doing Fine

As a surgeon, I have a bias that surgery can help most of the time.  And I feel this one because I have seen it help in so many children.  Surgery can help function and it can help appearance- both are important.  And as a surgeon, I feel fortunate to be able to offer surgery to kids and families.

However, surgery is not the answer for every child and sometimes the best path is different. Sometimes it is therapy, sometimes watchful waiting, and sometimes support in other ways.  Kids with birth differences of the upper extremity almost universally adapt well, learn to function in their own way, and generally can accomplish the tasks that are necessary to go about their day.  It is my job to know when I can help but also know when to avoid the recommendation for surgery.  

And this can be really, really difficult.  Because I want to do something to help each child.  And families come to me (and surgeons like me everywhere) because, if something can be done, they want to do it for their child.  Some families push harder than others, but most want something done if it at all can help.  But we, as surgeons, need to be realistic and honest in our approach.

Below is a great kid with a great family who came to me for the first time at age 12.  He had been observed elsewhere and moved to St Louis and asked for our opinion and, if appropriate, surgery. Radial longitudinal deficiency comes in many different varieties with differing severity.  This is a relatively severe presentation.  The family of this child was hopeful that surgery could be done to help him function better and easier.  His arms are short and his elbow don't bend.  His fingers work reasonably well, especially the small finger and ring fingers.  But the stiff elbows make many tasks tough including eating without aids.  But, he does reasonably well and HE is not interested in surgery.

Severe radial longitudinal deficiency.  Note the bent wrist position and lack of elbow motion.  But the shoulder work well. The patient's face is blinded for privacy.

Severe radial longitudinal deficiency.  Note the bent wrist position and lack of elbow motion.  

Severe radial longitudinal deficiency.  Note the lack of elbow joint formation.
Severe radial longitudinal deficiency.  Note the lack of normal elbow joint formation.

The temptation for the surgeon is to straighten the wrists.  However, when the elbows don't bend, the angled position of the wrists actually can help.  Straightening the wrists would only take the hands further from the midline and further from the body.  And, unfortunately, restoring elbow motion is not really possible at this age and with his elbow development.  Therefore, we agreed with the patient and did not recommend surgery.  Not easy for me to say and not easy for the family to hear.  We will, of course, follow him regularly and if certain issues arise, we may have therapy or adaptive recommendations.  But, at least for now, no surgery.

Charles A. Goldfarb, MD
My Bio at Washington University

Sunday, October 11, 2015

Rare Bone Tumor- Fibrous Dysplasia

Fibrous dysplasia is a bone tumor which is related to a genetic abnormality leading to abnormal bone formation.  Normal bone is replaced by a fibrous tissue.  It can present at any age- young kids, adolescence, or even adulthood.  It is a bone tumor but it is benign growth. Rarely, the tumors can become malignant, but this only happens in about 1 in 20 of kids with fibrous dysplasia.  McCune Albright Syndrome includes kids with fibrous dysplasia, endocrine disorders (such as early puberty), and skin lesions (cafe au lait spots).

Fibrous Dysplasia typically presents in the legs but can occasionally present in the upper extremity, typically the humerus bone.  It may involve only one bone or can involve many bones.  The bone develops abnormally, expands, and can break due to the weakened nature of the bone.  The bone can be painful or the pain can develop from a fracture of the weakened bone  Bones can bow due to the weakening caused by the fibrous tissue of fibrous dysplasia.  X-rays have the classic appearance of ground glass and bone specimens examined under a microscope show a 'chinese letter' pattern.

Treatment for fibrous dysplasia depends on many different factors.  Fractures may require surgical treatment although they can heal without surgery.  Bowing of the long bones can cause problems and sometimes has to be addressed with bone realignment (or osteotomy).

Medications called bisphosphonates which may have a role in treatment of fibrous dysplasia.  These come in different forms including pills.  These have been reported to help pain and limit deformity but the true expectations continue to be clarified.

Here is one of our patients followed over time with fibrous dysplasia of the humerus.  The early xrays from 2010 of the left humerus show a fracture, which healed without surgery.  Recent xrays from October 2016 show some progression of the fibrous dysplasia over time.

Early xray from 2010 of patient with fibrous dysplasia
Later in 2010, the humerus fractures due to weakness of bone in fibrous dysplasia.  The bone broke after a fall. 

Healing humerus after fracture in fibrous dysplasia

The most recent xray of humerus with fibrous dysplasia.

Charles A. Goldfarb, MD
My Bio at Washington University

Young Adult with Multiple Hereditary Exostoses

Multiple Hereditary Exostosis is an uncommon condition with the growth of multiple benign tumors or bone/cartilage growths.  These can be called osteochondromas or exostoses.  It has been estimated to be present in 1 in 50,000 children.  Here is a great reference- NLM site. The growths can present in several different ways.
1. Bumps which are not painful
2. Bumps which are painful, often related to pressure on cutaneous nerves or other structures
3. Bumps which cause problems with bone growth.  These are usually in the forearm or lower leg- segments with 2 bones.  Altered growth of one of these bones affects the other bone.

There is a strong hereditary component to Multiple Hereditary Exostoses.  It can be inherited in an autosomal dominant fashion, related to the EXT1 and 2 genes.  Typically, we, in the United States, see these children at a relatively young age and certainly by the beginning of puberty.  The osteochondromas grow rapidly at the time of rapid bone growth.

We often have the chance to care for children from other countries, sometimes thanks to the generosity and coordination of the World Pediatric Project.  This amazing organization helps many children with orthopedic and non orthopedic conditions.  Check out there website HERE.  We recently took care of a patient from Central America with Multiple Hereditary Exostoses.  He had not undergone any previous treatment.  Here are some pictures:
Multiple Hereditary Exostoses.  Note the lesions on the right wrist and both legs.

Multiple Hereditary Exostoses

Here are some x-rays showing the bumps.
Multiple Hereditary Exostoses.  Note the bumps on the bone.

Multiple Hereditary Exostoses.  Note the big bump on the outside of the humerus.
This patient was taken to the operating room for removal of the painful bumps.
Multiple Hereditary Exostoses, the excision of 3 lesions.
Multiple Hereditary Exostoses.  One large osteochondroma prior to removal.

Charles A. Goldfarb, MD
My Bio at Washington University

Thursday, October 1, 2015

Finger Bump

There are many reasons for bumps, cysts and masses in the fingers.  I have previous blogged HERE on some of these growths.  There is another common finger bump that should be discussed, the retinacular cyst.  The retinacular cyst is a type of cyst- a fluid filled sac- that grows from the flexor tendon sheath.

Here are a link that explain the general concepts of a ganglion cyst
Hand Society Explanation of Ganglion Cyst

Retinacular cysts can affect patients of any age.  These cysts appear as a pea- sized bump, where the finger meets the hand.  The are really a nuisance and usually cause pain with grasping (such as the steering wheel or a hammer).  Pressure causes discomfort as well.  As noted in the article by Hutchinson and Wang, these can disappear or resolve with time.  But in some patients, the cysts don't go away and do cause pain.  In those patients (a minority), treatment is a reasonable option.  This can include aspiration (sucking fluid out of cyst) or surgical excision.  Aspiration has been proven cost effective but is a challenge in kids.

Article by Wang and Hutchinson.  This article reviews ganglion cysts in the hand in children (7 were retinacular cysts).  Most resolved and typically within a year.

Surgery is simple and safe with a removal of the cyst off of the flexor tendon sheath.  The nerves and arteries are typically safe as the cyst is usually fairly central (in the middle of the finger).  When required, this is a satisfying surgery.

Article by Jebsen and Spencer.  This article reviews the outcomes after surgery for the retinacular cyst and reports very good results.

Retinacular cyst in the typical location at the base of the finger.  See the small cyst deep in the incision.

Charles A. Goldfarb, MD
My Bio at Washington University

Wednesday, September 23, 2015

Syndactyly Treatment: What's Next?

The basic treatment of syndactyly has not changed in many years.  The essential surgical care includes the creation of a web space (the 'commissure') with a flap and zig- zag incisions to the tips of the fingers to allow separation without straight- line scars (which can contract).  There is no doubt that our understanding of syndactyly has improved over the years and there have been many technical advances including variations on the commissural flap and modifications based on the exact type of syndactyly. Reports on reconstructing syndactyly without skin grafts have offered exciting new potential although many congenital hand surgeons continue to use grafts suggesting that results are not universally ideal.    I have written many times on syndactyly- check for those posts HERE.

Recently, Dr Landi reported outcomes using a new concept in the treatment of syndactyly.

This article describes the use of a hyalruonic acid scaffold to fill in the defects instead of skin grafting for syndactyly reconstruction.  The results are very good.  Article link  This material has become available in the United States and I wanted to share a recent case of ours.

This one year old child has bilateral cleft hands and cleft feet. He has a complete, cutaneous (skin only) syndactyly of the ring and small fingers bilaterally.  

Cleft hand with ring/ small finger syndactyly

Cleft hand with ring/ small finger syndactyly, palm view

In an effort to improve function and independence of the two fingers, he was brought to the operating room for bilateral syndactyly reconstruction.  We discussed using skin grafts but elected to proceed with the hyaluronic acid scaffold.  The 'hyalomatrix' works as a hydrophilic gel which allows cellular migration onto its 3D scaffold.    Skin cells can then migrate to allow healing.  

Hyaluronic acid scaffold
Hyaluronic acid scaffold- shiny side later peels off.

Here are a few pictures after the surgery.

Syndactyly reconstruction with hyaluronic acid scaffold, palm view
Syndactyly reconstruction with hyaluronic acid scaffold, dorsal view
It is too soon to know if this material will dramatically change the treatment of syndactyly.  But it does seem to be an important step forward to minimize or eliminate the need for skin grafts (with the accompanying scar).  More to come on this treatment.

Charles A. Goldfarb, MD
My Bio at Washington University

Saturday, September 5, 2015

Amniotic Constriction Band

Amniotic Constriction Band commonly affects the hand and feet.  I have previously posted on the topic on several occasions as can be viewed HERE.  I wanted to share a few recent cases to demonstrate the different types of Amniotic Constriction Band.

This young child has Amniotic Constriction Band affecting one foot and one hand as depicted below.  The toes are characteristically short and there is a syndactyly (joining of the digits) as well.  As classically noted, the syndactyly is fenestrated such that the tip of the digits are joined but there is an opening closer to the foot.  The hand has a great thumb and a reasonable index finger but the finger is tethered to the remnants of the other digits.

Amniotic Constriction Band of the foot

Amniotic Constriction Band of the hand

Amniotic Constriction Band of the hand, view of the palm

Here is another case of Amniotic Constriction Band demonstrating a slightly different appearance. First, here is the hand prior to surgery.  The thumb is somewhat short, the index finger short and the first webspace (between the thumb and index finger) is tight.  The index finger is bulbous as may be seen in this condition.
Amniotic Constriction Band of the hand, view of the palm.  The pinky is normal but the other digits are short.

Amniotic Constriction Band of the hand.  Note the abnormal index finger with bulbous index finger.

Amniotic Constriction Band of the hand, view of abnormal index finger

Tight first web space with 2 bands of contracture.  Amniotic Constriction Band.
The decision for surgery is based primarily on function but also should consider appearance.  Mom and family were concerned about the length of the thumb and the appearance of the hand.  Certainly, I agreed with the concerns about the thumb because the short thumb and tight webspace limit large object grasp.  And, the bulbous nature of the index finger also limits both function and appearance.  We therefore offered reconstructive surgery.  The webspace was limited in two planes and therefore we used a more complex flap than typical.
Flap raised to reconstruct the first webspace in Amniotic Constriction Band
Flap raised to reconstruct the first webspace in Amniotic Constriction Band. The flap has been laid across the tight web.
Reconstructed hand with deeper webspace and contoured index finger Amniotic Constriction Band

Reconstructed hand with deeper webspace and contoured index finger Amniotic Constriction Band.  The pinky finger is held by the instrument.
Reconstructed hand in Amniotic Constriction Band

Charles A. Goldfarb, MD
My Bio at Washington University

Wednesday, August 19, 2015

3D Progress

I have posted several times previously on our work with 3D Printed Prosthetics, the posts are compiled here.  Sydney is one of our patients who has been a big part of this process.  She recently received the latest prosthetic version, a 3D Printed, Myoelectric device and fabricated for about $110. Today, there was a great deal of press on Sydney and her new 'arm'.  Here are a few of the links:

KSDK link


Fox2 Link

In addition, Sydney and I talked just after she got the new prosthetic.  She wasn't yet a pro at using the hand but still lots of interesting things to say.  Check it out

Charles A. Goldfarb, MD
My Bio at Washington University