Wednesday, October 10, 2018

Untreated Cleft Hand

Cleft hand is one of the most notable birth anomalies of the upper extremities.  The appearance is distinctive although function can be really well maintained.  One of the founding fathers of the discipline of hand surgery, Sterling Bunnell, labeled cleft hand, "a functional triumph and a social disaster".

There are other names for cleft hand.  The geneticists call it split hand (often along with split foot).  EEC is a syndrome with cleft hand as a part- ectrodactyly (missing digit) and ectrodermal dysplasia.  It can also be associated with cleft lip and palate.  It may be genetic or random and it may affect one hand, both hands and, especially in genetic conditions, the feet.   We continue to learn more about cleft hand from a genetic standpoint.

There are two classification systems on cleft hand.  My former partner and mentor, Paul Manske, classified cleft hand based on the quality of the thumb web space.  This is relevant due to the need to reconstruct this web space if too tight.  Dr Ogino, a friend who advanced our understanding of cleft hand through lab and patient research, classified patients on the basis of the number of missing digits.  Together, these classification systems really help our understanding of each patient and help us plan treatment.

Families with other members affected may have a different outlook and approach to the evaluation and treatment of cleft hand.  If left alone, children can function well using the cleft for large object grasp no matter the size of the first (thumb- index) webspace.  This picture and video are of a child without functional limitation in a family with others with cleft hand.  The family wishes to avoid surgery for now.

As a surgeon, I know what surgery can offer: improved appearance and a better thumb grasp with enlargement of the first web space.  However, I also understand why every family may not chose surgery.  My role, as I see it, is to share my experience and help guide each family to the best decision for them.  Most of my families would chose surgery for these cleft hands, but not all.

Cleft hand with large cleft and tight first web space bilaterally.




This video shows these same cleft hands with dramatic instability of the index finger MCP joints.  The videos also demonstrate the limitations of the first web space with limited space available for thumb function.  The instability can become an issue with strength and grasp although surgical reconstruction can be helpful.  There can also be instability of the ring finger MCP joints.

Cleft hand is a complex and striking disorder.  Surgery can absolutely be beneficial for the child, for their function, and for their appearance but every decision is family- centered.

Charles A. Goldfarb, MD
My Bio at Washington University
Email me: congenitalhand@wudosis.wustl.edu