Madelung deformity

Madelung deformity is a rare congenital deformity of the wrist. The deformity
is characterized by a palmar subluxation of the hand, a prominent distal ulna,
and volar angulation of the distal radial epiphysis. Currently, we know that
the deformity is caused by an abnormal growth arrest of the distal radial
epiphysis leading to volar and ulnar tilting of the radial articular surface,
and palmar bowing of the distal radius. Because of its progressive nature, the
deformity can lead to wrist pain, restricted range of motion (ROM), and loss of
grip strength, heavily interfering with daily activities. Additionally,
patients can complain about the visible deformity caused by prominence of the
distal ulna. Madelung deformity often occurs bilaterally, is most often
diagnosed in adolescent females, and has been associated with genetic disorders
such as Léri-Weill dyschondrosteosis (LWD) and Turner syndrome.

Representing less than 2% of pediatric hand deformities, our current
understanding of this condition is limited. This is also reflected in patient
care, as multiple surgical procedures have been proposed to correct the
deformity, without a current consensus. Therefore, nearly 200 years after its
original description, a significant knowledge gap remains, with respect to
etiology, surgical management and outcomes of Madelung deformity patients,
compromising patients' access to optimal care.

To find the best treatment for patients it is of the utmost importance to
understand the complex anatomical situation and corresponding wrist
biomechanics. Due to recent innovations it is now possible to capture wrist
anatomy during movement using 4-Dimensional (4D) Computed Tomography (CT)
imaging, a technique which our group has applied in multiple previous studies.

Investigate anatomy, kinematics, clinical outcomes, and functional outcomes in
Madelung deformity patients.

In this study we will answer the following 3 questions:
1) What is the anatomical situation of the wrist in patients with Madelung
deformity, and how does this differ in comparison to healthy wrists?
2) How is the movement of carpal bones in patients with Madelung deformity, and
how does this differ in comparison to healthy wrists?
3) What are the clinical and functional outcomes before and after different
surgical treatment options?

The answer to these questions can be used to better understand the
pathophysiology of Madelung deformity. In addition, our data can be used to
identify the optimal treatment plan through quantification and comparison of
objective/subjective outcome measurements. Ideally, our results will lead to a
personalized treatment protocol.

Observational cohort study.

Not applicable.