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                               […]