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Madelung Deformity Treatment & Management

  • Author: Paul M Lamberti, MD; Chief Editor: Jeffrey D Thomson, MD  more...
 
Updated: Jun 02, 2016
 

Approach Considerations

Operative treatment of Madelung deformity (MD) is indicated for pain relief and cosmetic improvement. These indications are consistent among many authors. Range of motion, especially in pronation and supination, is usually only minimally improved and varies in different operative series.

In the skeletally immature patient with clear evidence of MD, the most likely cause of pain is tension within Vickers ligament. Release of the ligament alone or in combination with an osteotomy is indicated. In the skeletally mature patient, the congruency of the radiocarpal joint and that of the distal radioulnar joint (DRUJ) are assessed. If an osteotomy will result in a secondarily congruous joint, then an osteotomy is indicated. However, if radiocarpal congruity is not possible to obtain, a radioscaphocapitate arthrodesis is indicated. Similarly, if DRUJ congruity is not possible, then a Darrach or Sauve-Kapandji procedure is indicated.

No specific contraindications to surgery exist other than those associated with any elective surgical procedure.

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Medical Therapy

Nonoperative management may be helpful in skeletally mature individuals with MD and mild-to-moderate short-term wrist pain. If the pain is predominantly related to the DRUJ, then a sugar tong–type splint may relieve joint irritation from overactivity. If radiocarpal pain is the primary problem, then a volar splint may decrease symptoms. Patients may eventually decrease their manual activity levels to a point where their symptoms are manageable without surgery. In contrast, the younger and skeletally immature patient with clear evidence of MD has pain that is caused by the tension within the Vickers ligament, and splintage will most likely not have a satisfactory result.

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Surgical Therapy

The surgical decision is based on the following four factors:

  • Patient's age and the growth remaining in the distal radius
  • Severity of the deformity
  • Severity of the symptoms
  • Clinical and radiographic findings

Operative treatment can be divided into three broad categories as follows:

  • Procedures that correct the primary deformity of the radius
  • Procedures that attempt to decrease pain and increase range of motion by making a compensatory change in the ulna
  • Procedures that address both

Multiple procedures address the deformity in the radius. Conceptually, these can be broken into the following three types:

  • Procedures that change the growth or anatomy at the physis
  • Procedures that change the bony anatomy of the metaphysis
  • Procedures that are considered salvage-type joint-sacrificing procedures

The goal of ulnar procedures is to change the relationship of the relatively long ulna to the radius.

Vickers physiolysis

When the deformity is noticed early and significant growth remains, changing the growth pattern of the distal radial physis to correct the deformity is possible. In 1992, Vickers and Nielsen described the lesion in the volar and ulnar distal radius as both bony and ligamentous, and they stated that it is an inherent failure of focal growth and structural tethering of further growth. They described an ulnar-volar release for MD of the physis, called physiolysis (see the images below).

Postoperative anteroposterior radiograph of wrist Postoperative anteroposterior radiograph of wrist of patient B following Vickers physiolysis. Vickers ligament and the ulnar abnormal physis have been excised.
Postoperative lateral radiograph of the wrist of p Postoperative lateral radiograph of the wrist of patient B following Vickers physiolysis.

This allows, then, normal and compensatory growth to correct the deformity, much like the bony bridge resection of Langenskiöld. Vickers and Nielsen used the procedure of resecting the bony and ligamentous lesion (the first to correct MD using remaining growth to correct the deformity). They were the first to describe a ligamentous lesion as part of the pathology and also were the first to use the volar approach to address it.

Through a volar flexor carpi radialis (FCR) approach, the pronator is mobilized, and the distal radius is exposed. An osteotome is used to make a longitudinal split 5 mm from the DRUJ on the radius, and parallel transverse 1-mm sections are made until normal physis is identified. Some physis is left to overhang the metaphysis. Fat harvested from the forearm is packed into the bone defect. Completely excising the tethering ligament (Vickers ligament) between the lunate and the radius is critical. (See the images below.)

Intraoperative photo of Vickers ligament, outlined Intraoperative photo of Vickers ligament, outlined in red.
Intraoperative color photograph of Vickers ligamen Intraoperative color photograph of Vickers ligament. The ligament is outlined in the previous image.

The Vickers ligament originates on the radius in a fossa that is seen radiographically as a flame-shaped radiolucency distal to a bone spur on the ulnar aspect of the distal metaphysis (see the images below). Dannenberg noted this characteristic as an area of osteopenia in his original radiographic criteria in 1938. Carter and Ezaki noted it in 91% of their cases.

Preoperative posteroanterior radiograph of wrist f Preoperative posteroanterior radiograph of wrist from patient C.
Preoperative lateral radiograph of wrist from pati Preoperative lateral radiograph of wrist from patient C.

The Vickers ligament is fibrous and fibrocartilaginous and 5-7 mm thick. It inserts into the anterior surface of the lunate and the anterior radioulnar ligament portion of the triangular fibrocartilage complex (TFCC). Most likely, it is a secondary pathologic structure to a primary bony derangement and may even be a coalescence of normal structures. This ligament may be an etiologic factor in MD pain. Following operative release of this ligament, patients state soon after surgery that their pain is largely relieved.

Osteotomy of radius

If the deformity has progressed in an older child and remaining growth is insufficient, several procedures can be used to correct the position of the distal radiocarpal joint surface. They generally consist of a biplane osteotomy, either closing or opening wedge, which corrects the position of the joint surface and brings the radius and ulna into a more proper position. If a positive ulnar variance remains, an ulna-shortening procedure can be performed.[23, 24, 25]

A less commonly used method is distraction histogenesis with the Ilizarov technique. Several authors have described experience with the technique of radial osteotomy and subsequent angular and length correction over time.[26, 27] The goal is slow correction of the distal radius to make a more congruous DRUJ. Children treated with this method are either skeletally mature or nearly mature.

Half pins are commonly selected over tensioned thin wires to allow better range of motion during distraction. Three or four rings are used, with fewer complications and less pain noted when three are used. The first goal after fixation placement and radial osteotomy is angular correction over a 2-week period. The second goal is lengthening of approximately 1.5 cm over the next 2 weeks, followed by consolidation for 3 weeks. The entire process in the distractor takes 8 weeks.

In 2000, Carter and Ezaki reported a combined procedure using a Vickers ligament release and a dome-shaped osteotomy of the radius to correct all of the aspects of the radial deformity, including the radial and volar translation of the distal metaphysis.[19] This procedure is performed with a volar approach, and the Vickers ligament is excised entirely.

A dome-shaped osteotomy is made in the metaphysis so that the articular surface not only decreases in its radial inclination but also translates as it rotates. This translation reestablishes articular support to the lunate. A second maneuver translates the articular surface dorsally. The prominent volar bone is then removed, and the osteotomy is pinned into position with two Steinman pins. It not only corrects the deformity but also decreases pain and increases range of motion. (See the images below.)

Preoperative plan prior to Carter-Ezaki dome osteo Preoperative plan prior to Carter-Ezaki dome osteotomy. Dorsal translation of the distal radius is depicted.
Preoperative plan prior to Carter-Ezaki dome osteo Preoperative plan prior to Carter-Ezaki dome osteotomy. Rotation of the distal radius to accomplish both ulnar translation and normalization of radial tilt is depicted.
Postoperative lateral radiograph. Note dorsal tran Postoperative lateral radiograph. Note dorsal translation of distal radius after Carter-Ezaki dome osteotomy.
Postoperative posteroanterior radiograph with Kirs Postoperative posteroanterior radiograph with Kirschner-wire fixation in place. Note combination of ulnar translation of distal radius and correction of radial tilt towards normal after Carter-Ezaki dome osteotomy.

Volar ligament release with distal radial dome osteotomy has been shown to yield lasting correction of Madelung deformity.[28]

Radioulnar length adjustment

In MD, the ulna grows normally and becomes longer than the radius. Because the radius is volar, the ulna appears to be subluxated dorsally. The incongruence at the DRUJ and the impingement of the radius on the ulna in supination may cause pain and contribute to decreased range of motion in supination. To allow unrestricted rotation, several ulnar procedures have been described. These include ulnar shortening, ulnar head resection and a Sauve-Kapandji-type (Lauenstein) DRUJ arthrodesis, and ulnar pseudoarthrosis. Several authors have advocated both radial and ulnar procedures.

Ulnar resection

The Darrach procedure long has been a treatment option for MD. This construct in isolation may leave the carpus unstable, especially in light of the increased ulnar and volar slope of the radial articular surface. The carpus therefore tends to slide off of the ulnar side of the wrist. Several authors have devised procedures to solve this problem. The Sauve-Kapandji (Lauenstein) procedure may be a viable option for MD in that when the ulnar head is preserved, less chance exists for ulnar migration of carpus.

In 1975, Ranawat studied 13 wrists in 8 patients with MD. His operative indications were pain and limitation of motion. He did not consider deformity alone to be an indication for operation. Mild deformity was treated with a Darrach procedure, and severe deformity was treated with Darrach plus a biplane radial osteotomy. In patients in whom a Darrach procedure was performed, the carpus tended to translate ulnarward. A radial osteotomy was theorized to improve the muscle balance about the wrist and to provide better axial bone support for the carpus on the radius in the absence of the ulnar head.

In 1993, Watson et al described another combined procedure in which the radial osteotomy was performed with both a closing wedge technique on the radial aspect of the metaphysis and an opening wedge on the ulnar aspect with the radial bone wedge. This technique preserved radial length but also required an ulnar head resection. Pain was significantly relieved in all 15 wrists.

White and Weiland described a combination procedure in a series of wrists with MD of posttraumatic etiology.[29] The procedure included a volar closing wedge osteotomy along with a distal radioulnar arthrodesis and ulnar pseudoarthrosis proximal to the arthrodesis. The osteotomy of the radius improved the mechanics of the radiocarpal joint and prevented radiocarpal arthritis.

This procedure alone may not improve pain on supination/pronation due to the incongruent DRUJ. Resection of the distal ulna as in the Darrach procedure may solve this problem, but ulnar translation of the carpus may result. Fusing the DRUJ maintained carpal stability. Increase in the patient's range of motion was minimal in any plane, but pain was significantly relieved, the carpus was stable, and the cosmetic result was satisfactory.

In the skeletally mature patient who presents with pain and in whom physical examination demonstrates limitation of motion and severe radiocarpal joint incongruity, a salvage procedure may be indicated. When incongruity is severe, any surgical manipulation of the joint surfaces would be difficult and the joint would not be stable for smooth articulation. For long-term pain relief and a stable joint, a radiocarpal arthrodesis and distal ulna resection is indicated.

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Postoperative Care

Postoperative management depends on what is done operatively. Cast immobilization is necessary for 6-8 weeks after an osteotomy of the radius or ulna to allow for bony healing. Hand therapy is necessary in children who are not able to regain range of motion on their own after 2-3 months without restrictions.

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Complications

In the author's experience, no specific complications after operative correction have arisen as problematic. However, isolated cases of postoperative wound infections, reflex sympathetic dystrophy, and recurrent deformity after continued growth are reported in the literature.

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Long-Term Monitoring

Monitor these children until surgical issues are resolved and then yearly until skeletal maturity is reached. After a physiolysis procedure, yearly radiographs can be used to document improvement in the position of the distal radial articular surface with growth.

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Contributor Information and Disclosures
Author

Paul M Lamberti, MD Principal Surgeon, Lamberti Orthopedic and Hand Surgery, LLC

Paul M Lamberti, MD is a member of the following medical societies: American Medical Association, American Society for Surgery of the Hand

Disclosure: Nothing to disclose.

Coauthor(s)

Terry R Light, MD Dr William M Scholl Professor and Chair, Department of Orthopedic Surgery, Loyola University School of Medicine; Attending Surgeon, Department of Orthopedic Surgery and Rehabilitation, Loyola University Medical Center

Terry R Light, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Orthopaedic Association, Mid-America Orthopaedic Association, Illinois Association of Orthopaedic Surgeons, Association of Bone and Joint Surgeons, American Association for Hand Surgery, American College of Surgeons, American Society for Surgery of the Hand

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

George H Thompson, MD Director of Pediatric Orthopedic Surgery, Rainbow Babies and Children’s Hospital, University Hospitals Case Medical Center, and MetroHealth Medical Center; Professor of Orthopedic Surgery and Pediatrics, Case Western Reserve University School of Medicine

George H Thompson, MD is a member of the following medical societies: American Orthopaedic Association, Scoliosis Research Society, Pediatric Orthopaedic Society of North America, American Academy of Orthopaedic Surgeons

Disclosure: Received none from OrthoPediatrics for consulting; Received salary from Journal of Pediatric Orthopaedics for management position; Received none from SpineForm for consulting; Received none from SICOT for board membership.

Chief Editor

Jeffrey D Thomson, MD Associate Professor, Department of Orthopedic Surgery, University of Connecticut School of Medicine; Director of Orthopedic Surgery, Department of Pediatric Orthopedic Surgery, Associate Director of Clinical Affairs for the Department of Surgical Subspecialties, Connecticut Children’s Medical Center; President, Connecticut Children's Specialty Group

Jeffrey D Thomson, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons

Disclosure: Nothing to disclose.

Additional Contributors

Charles T Mehlman, DO, MPH Professor of Pediatrics and Pediatric Orthopedic Surgery, Division of Pediatric Orthopedic Surgery, Director, Musculoskeletal Outcomes Research, Cincinnati Children's Hospital Medical Center

Charles T Mehlman, DO, MPH is a member of the following medical societies: American Academy of Pediatrics, American Fracture Association, Scoliosis Research Society, Pediatric Orthopaedic Society of North America, American Medical Association, American Orthopaedic Foot and Ankle Society, American Osteopathic Association, Arthroscopy Association of North America, North American Spine Society, Ohio State Medical Association

Disclosure: Nothing to disclose.

References
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Preoperative wrist posteroanterior radiograph of a 13-year-old girl (patient A) with dyschondrosteosis and Madelung deformity of the wrist.
Preoperative lateral wrist radiograph of patient A.
Lateral radiograph of elbow of patient A, depicting a dysplastic proximal radius. This is characteristic of dyschondrosteosis.
Preoperative anteroposterior radiograph of wrist of patient B. This patient has primary Madelung deformity (no sign of dyschondrosteosis).
Preoperative lateral radiograph of wrist of patient B. The flame-shaped radiolucency in the metaphysis of the radius is occupied by the fibrocartilaginous Vickers ligament.
Postoperative anteroposterior radiograph of wrist of patient B following Vickers physiolysis. Vickers ligament and the ulnar abnormal physis have been excised.
Postoperative lateral radiograph of the wrist of patient B following Vickers physiolysis.
Postoperative anteroposterior radiograph from patient A following biplane osteotomy of distal radius and ulnar shortening procedure.
Postoperative lateral radiograph from patient A following biplane osteotomy of radius and ulnar shortening procedure.
Preoperative photograph of 17-year-old girl (patient C) with idiopathic Madelung deformity.
Preoperative photograph of 17-year-old girl (patient C) with idiopathic Madelung deformity.
Preoperative posteroanterior radiograph of wrist from patient C.
Preoperative lateral radiograph of wrist from patient C.
Intraoperative photo of Vickers ligament, outlined in red.
Intraoperative color photograph of Vickers ligament. The ligament is outlined in the previous image.
Postoperative result in patient C. Compare with preoperative appearance.
Postoperative lateral radiograph. Note dorsal translation of distal radius after Carter-Ezaki dome osteotomy.
Postoperative posteroanterior radiograph with Kirschner-wire fixation in place. Note combination of ulnar translation of distal radius and correction of radial tilt towards normal after Carter-Ezaki dome osteotomy.
Preoperative plan prior to Carter-Ezaki dome osteotomy. Dorsal translation of the distal radius is depicted.
Preoperative plan prior to Carter-Ezaki dome osteotomy. Rotation of the distal radius to accomplish both ulnar translation and normalization of radial tilt is depicted.
 
 
 
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