The Wrist in Arthrogryposis

Arthrogryposis is different in every child.  While there may be a classic appearance- internal rotation of the arms, elbow straight (extended), wrists flexed, and limited finger and thumb motion- the variability between kids makes treatment a challenge.  I have previously commented on arthrogryposis and general treatment concepts (my own thoughts guided by my experience and […]

The Wrist in Arthrogryposis

Arthrogryposis is different in every child.  While there may be a classic appearance- internal rotation of the arms, elbow straight (extended), wrists flexed, and limited finger and thumb motion- the variability between kids makes treatment a challenge.  I have previously commented on arthrogryposis and general treatment concepts (my own thoughts guided by my experience and […]

The Power of a Positive Outlook

It is amazing how much my patients teach me every day.  The power of positive thinking and the power of a positive outlook mean so much.  This newspaper piece highlights this attitude. http://www.ajc.com/news/ap/florida/sixth-graders-turns-tables-on-bullying/nTBgZ/

Unusual Combination

We have come a long way in understanding congenital hand and upper extremity abnormalities.  Dr Swanson, more than 40 years ago, published a classification system that was adopted by most of us who treat patients with birth anomalies.  This classification system was based purely on appearance of the extremity but allowed us to communicate better […]

Carpenter Syndrome

Carpenter Syndrome is one rare type of acrocephalopolysyndactyly, Type 2.  It is closely related to acrocephalosyndactyly, with the most notable type of this group of disorders being Apert syndrome.  These syndrome all have craniosynostosis (premature fusion of the suture lines of the skull) and syndactyly, with or without polydactyly as well.  Carpenter syndrome has been […]

Clavicle Pseudoarthrosis

Clavicle pseudoarthrosis (meaning fake joint) is a rare condition.  It occurs when the normal growth centers of the clavicle do not join (fuse) during development.  It is almost always right sided, a fact which is may be related to pressure from the subclavian artery.  Clavicle pseudoarthrosis is generally painless but it causes a large prominence […]

Central Deficiency with 2-3 fingers

Central deficiency (cleft hand) is classically as I have shown in previous blogs.  https://congenitalhand.wustl.edu/2012/05/cleft-hand-surgery.html We have previously published our experience with the classic central deficiency, that is the hand with a limited thumb- index web space, 3 fingers (index, ring, and small finger), and a large cleft http://www.ncbi.nlm.nih.gov/pubmed/18984341 However, there are many different varieties of cleft […]

Hemihypertrophy

Hemihypertrophy is a difficult diagnosis due to its rarity and the variety of associated conditions.  Basically, the diagnosis means that one extremity (or one side of the body) is enlarged compared to the other side.  As little as a 5% difference can be diagnostic but usually families seek care when the difference is more notable. […]

Too much about me

I have been asked on several occasions by patients (or prospective patients) to provide more information on the blog about my professional career.  Enclosed, please find a variety of links including local and regional publications and videos.  I have included links to several books and articles. First, my basic bio from Washington Universityhttp://www.ortho.wustl.edu/content/Patient-Care/2637/Find-a-Physician/Listing-of-Faculty/Charles-Goldfarb-MD/Bio.aspx Others:1) Great […]

Severe Ulnar Deficiency

Ulnar deficiency, as described in a previous post has characteristic findings at the elbow, forearm, and hand.  https://congenitalhand.wustl.edu/2011/09/ulnar-longitudinal-deficiency-basics.html Most classifications describe 4 types of ulnar deficiency (or ulnar longitudinal deficiency- ULD).  These classifications only address the bony deficiency (and not the important other issues such as muscles, tendons, nerves, etc).  While there are a number […]

Severe Symbrachydactyly and Nubbins

Symbrachydactyly, as noted previously, https://congenitalhand.wustl.edu/2011/12/normal.html  means “short, webbed fingers.”  The use of this label is clearly appropriate for some children who (brace yourself for this truth) have short and webbed fingers (i.e., a syndactyly).  The term symbrachydactyly has further meaning as it has implications for etiology (i.e., cause). I believe symbrachydactyly occurs due to difficulties with the AER […]

Limb Formation

I have been remiss in that I have not given an overview of limb formation.  Mind you, this is  a topic that is constantly changing- we learn more each year about the process, the proteins, the timing, etc.  So this is a hand surgeon’s perspective in 2012.  There are others with a much better understanding […]

“Simple” Extra Digits

An extra finger on the pinky side of the hand is one of the most common birth anomalies of the hand.  The official name is ulnar polydactyly or postaxial polydactyly.  It is typically seen in African Americans and is usually passed on as an autosomal dominant trait.  OMIM has numerous details: http://omim.org/entry/174200?search=postaxial%20polydactyly&highlight=postaxial%20polydactyly In caucasians, it […]

September 2012 Travels

It has been too long since I last blogged.  Several catch- up blogs are on the way.  In the meanwhile, I have been to several interesting events in the last month. First, we had our annual St Louis Shriners Hospital Hand Camp.   There were approximately 18 children with their families, 10 junior counselors, and […]

Symbrachydactyly Web Deepening

Web space deepening is a straightforward surgical technique to provide a deeper space between fingers.  There are several variations of the technique of web space deepening, typically based on the number of skin flaps that are utilized.  These techniques include 2- flap, 4- flap, 5- flap and other variants; precision is required for all.  A […]

Recurrent Radial Longitudinal Deficiency

The best treatment for the forearm/ wrist deformity in Radial Longitudinal Deficiency remains uncertain as I have previously outlined in a previous blog posting https://congenitalhand.wustl.edu/2012/05/form-and-function-in-radial-deficiency.html .  The problem is that the anatomical deficiencies in Radial Longitudinal Deficiency limit our ability to sufficiently correct the underlying deformity.  There is a lack of muscles to extend (bend back) the wrist and a […]

Clinodactyly

Clinodactyly is a curvature of a finger (or thumb).  The bend is in relation to the next finger rather than towards the palm or towards the top of hand.  This is not to be confused with camptodactyly as we discussed in a previous post, https://congenitalhand.wustl.edu/2012/03/camptodactyly.html .  Additional information, as always, is available from our website, http://ortho.wustl.edu/content/Patient-Care/3220/SERVICES/Hand-Wrist/Congenital-Hand-Disorders.aspx Small finger […]

Finger Lengthening

Lengthening a finger or thumb may be a good treatment option for a number of conditions including symbrachydactyly, constriction band syndrome, and ulnar deficiency.  Essentially any congenital or traumatic condition that leads to a shortened digit (or digits) may interfere with function.  If lengthening that digit will help pinch or large object grasp (i.e., soda […]

Arthrogryposis: General Thoughts

Arthrogryposis– What Works and What Is Still a Challenge I want to restate how happy our arthrogryposis/ amyoplasia (AMC)(I use both of these terms together but these are very different diagnoses and may have different treatments) patients and families have been with three procedures.  1)  External rotation osteotomy of the humerus.  If the arms are really […]

Radioulnar Synostosis

Radioulnar synostosis literally means a bony union between the two forearm bones.  Normally, the ulna bone acts as a straight “post” to anchor the wrist to the elbow.  The radius bone rotates around the ulna to allow the forearm to turn palm up and palm down.  This rotation is helpful for daily activities and allows […]

Fixator for Radial Longitudinal Deficiency (RLD)

We continue to favor a two- stage centralization procedure for RLD.  In the first stage, we apply a circular fixator to allow a slow, painless stretching of the soft tissues.  We know that in RLD, all of the radial (i.e., thumb) sided structures are abnormal and often tight.  By using the fixator before surgery, we […]

Cleft Hand Surgery

Cleft hand (Central Deficiency) reconstruction is a challenging surgical procedure.  This child’s hand demonstrates some of the difficulties.  There are 3 problems in the hand.  First, the space between the thumb and the index finger is narrowed which limits his ability to grasp large objects.  Despite his young age (2 years), mom and dad have […]

Sprengel Deformity

Sprengel deformity is an uncommon condition in which the scapula (shoulder blade) on one side is higher in the neck than the other side.  Normally, during prenatal development, both scapula begin high in the neck and slowly descend toward their normal resting position in the upper back.  If, for unclear reasons, one scapula does not […]

Arthrogryposis Arm Rotation

I want to share several other photographs to relate how happy our amyoplasia patients/ families have been with an external rotation osteotomy of the humerus (obviously in situations in which the arms are markedly internally rotated).  In this procedure, we cut the arm bone and rotate it out of the internally rotated position and put […]

Rubinstein- Taybi Syndrome

Rubinstein- Taybi syndrome is a rare disease related to a gene defect causing a protein abnormality.  Most cases are sporadic without other known affected family members.  One of the tell-tale signs of Rubinstein- Taybi syndrome is the presence of broad, deviated thumbs (clinodactyly).  Patients are typically short statured and may have slow motor and cognitive […]

Robinow Syndrome

Robinow syndrome is an exceedingly rare condition which may be transmitted as an autosomal dominant or recessive condition.   Patients are short stature with the forearm segments classically most notably short (mesomelia).  The fingers and toes are also short (brachydactyly) and may be curved as well (clinodactyly).   There are classically spinal malformations, craniofacial anomalies, […]

Difficult Extra Thumbs

As with most medical conditions, congenital anomalies of the upper extremity have a wide range of severity.  No two conditions are the same although most can be categorized into groups with similar findings.  Patients with an extra thumb (also called duplicated thumb, split thumb, or radial polydactyly) can be severely affected as demonstrated below.  These […]

Form and Function in Radial Deficiency

There are a variety of factors which affect appearance and function in patients with radial deficiency (RLD).  Some of the major factors to consider include (not necessarily in order as it will vary from patient to patient): Factors affecting appearance                               […]

Books

This list will be updated on a regular basis with books that send the right message to kids with congenital differences.  I welcome any feedback or suggestions. 1. Harry and Willy and Carrothead by Judith Casely.2. Oliver’s High Five by Beverly Swerdlow Brown3. Elmer by David McKee4. Why Me? by Julie Parker

Great Function in Ulnar Deficiency

Take a look at this amazing 5 year old with a marked, bilateral ulnar deficiency.  While he has good shoulder motion, he does not have an elbow joint on either side.  He has great wrist motion which helps make up for his lack of elbow motion.  He has 2 fingers on the right hand and […]

Diagnosis and Evaluation

So your child has a congenital anomaly of the upper extremity.  The first step in moving forward is assuring the correct diagnosis.  While this sounds obvious, it is not always easy.  Some conditions are over- diagnoses such as constriction band (amniotic band) syndrome and some are under-diagnosed, such as symbrachydactyly.  The correct diagnosis is important […]

Camptodactyly

Camptodactyly is the position of flexion of the proximal interphalangeal (PIP) joint. Translated from Greek, camptodactyly means “bent finger.”  It may be first noted in the newborn, as a congenital camptodactyly, or it may present or progress in adolescence.   There are a number of anatomical structures that have been described as “causing” camptodactyly including abnormal […]

World Symposium on Congenital Malformation

Wow!  I had to write to share my recent experience at the World Symposium on Congenital Malformation of the Hand and Upper Limb.  This meeting is held once every three years and this year was in the United States in Dallas Texas, hosted by Marybeth Ezaki and Scott Oishi and the Texas Scottish Rite Hospital. […]

External Fixator- Piece of Cake

There are two different types of fixators that we use in the care of kids with upper extremity differences.  One is a unilateral frame and is just on one side of the bone.  It usually has 4 pins sticking into the bone and a rail outside the skin.  It is useful for lengthening bone- done […]

The Thumb in Arthrogryposis

Thumb posture is a common difficulty in children with arthrogryposis.   The thumb assumes a flexed (bent) posture at the metacarpophalangeal (MCP) joint and also may be adducted (tight towards the index finger).   As the thumb is positioned across the palm, grasping with the fingers is a challenge (which is especially troublesome given that finger motion […]

Central Deficiency, Severe

Central deficiency (cleft hand) may present in many different ways.  Sometimes, there is just long (or middle) finger absence and other times more than one digit may be missing.  The thumb- index web space is often affected, ranging from a mild narrowing to a complete syndactyly/ merging of the space. In this young patient, both […]

Amniotic Constriction Band

The cause of amniotic constriction band is uncertain.  Some believe it is a developmental issue (a “dysplasia” resulting from abnormal development of the structures) and others believe that it is a “deformity” that results from banding or pressure on previously  normal structures.  Either way, at the time of birth, a variety of differences can be […]

Central Deficiency

Central deficiency is also called cleft hand.  It typically includes a missing long finger (middle finger) and a narrow thumb- index web space.  Severity varies and some patients have loss of additional digits or a syndactyly of the ring and small fingers.  Like many congenital anomalies, central deficiency is considered from both a functional and […]

Recurrent Syndactyly

Recurrent Syndactyly This 8 year old male was surgically treated with syndactyly reconstruction utilizing skin grafts 6 years ago (in his hometown).  He did well although the skin between the fingers gradually crept distally towards the fingertips.  The skin growth limited the spread of the fingers. Creep of web space after syndactyly correction He and […]

Toe Polydactyly

Toe Polydactyly I am a congenital hand surgeon and, therefore, treat children with birth abnormalities of the upper extremity.  However, because abnormalities of the feet, including extra toes and toe syndactyly, are similar to hand anomalies, I also treat children with some birth differences of the feet.  Toe polydactyly (extra toes) is less common and […]