Growth disturbance of the volar-ulnar distal radial physis | Tethered by Vickers ligament | Linked to Leri-Weill dyschondrosteosis and SHOX defects | Bilateral, female, adolescent
HOW THE DISTAL RADIUS DEFORMS
Critical Must-Knows
- Core lesion: a partial growth arrest of the volar and ulnar corner of the distal radial physis, so the radius keeps growing on its dorsal-radial side but not its volar-ulnar side - this twists the joint surface and lets the carpus slide volar and ulnar
- Vickers ligament: an abnormal thick band running from the volar distal radius to the lunate. It tethers the volar physis and is the structure released at surgery
- Who gets it: typically an adolescent girl with bilateral wrists, presenting with deformity, a prominent ulnar head, pain, and loss of forearm rotation and wrist extension
- The big association: Leri-Weill dyschondrosteosis (a mesomelic short-stature dwarfism) caused by SHOX gene defects - but many cases are idiopathic, post-traumatic, post-infective, or part of other dysplasias
- Treatment depends on skeletal maturity: in the growing child, release Vickers ligament and free the radial physis (physiolysis) to slow progression; in the mature, symptomatic wrist, a dome osteotomy of the radius corrects the deformity, and severe joint damage may need a salvage procedure
Clinical Pearls
- "Madelung deformity is the classic exam wrist that is bilateral, female, adolescent and associated with short stature - say all four
- "The deforming tether is Vickers ligament running from the volar radius to the lunate; releasing it is the key growth-modulating step in children
- "Think SHOX and Leri-Weill dyschondrosteosis, but remember Madelung-like deformity can also follow trauma, infection, or other skeletal dysplasias (the reverse Madelung points the other way)
- "Match the operation to the skeleton: physiolysis and ligament release while growing; osteotomy once mature
Clinical Imaging
What the wrist looks like on X-ray
Critical Madelung Deformity Exam Points
The One-Line Description
A partial growth arrest of the volar-ulnar distal radial physis, tethered by an abnormal Vickers ligament (volar radius to lunate). The radius tilts in the coronal and sagittal planes, the carpus slides volar and ulnar, and the ulna looks relatively long and prominent. Classic patient: adolescent girl, bilateral wrists, short stature.
Know the SHOX Link
The headline association is Leri-Weill dyschondrosteosis, a mesomelic short-stature dwarfism caused by SHOX gene defects. But do not say all Madelung deformity is genetic - it can also be idiopathic, post-traumatic, post-infective, or part of other dysplasias (for example multiple hereditary exostoses, Turner syndrome).
Treatment Follows the Skeleton
In the growing child: release the Vickers ligament and free the radial physis (physiolysis), sometimes with an ulnar epiphysiodesis, to slow progression. In the skeletally mature, symptomatic wrist: a dome osteotomy of the distal radius corrects all three planes; severe joint destruction may need a salvage procedure (for example ulnar shortening, distal-radioulnar-joint reconstruction, or arthrodesis).
Do Not Confuse the Names
Madelung deformity is the wrist condition. Madelung disease (benign symmetric lipomatosis) is a completely different fatty-neck condition - same surname, unrelated. A reverse Madelung deformity tilts the radius the opposite way (dorsal, not volar).
Memory aids
MADELUNGThe Classic Patient and Lesion
| M | Mesomelic short stature Leri-Weill dyschondrosteosis, the key association |
| A | Adolescent presentation Pain-free as a child, presents in the teens |
| D | Distal radius physeal arrest Volar-ulnar corner stops growing |
| E | Eponymous Vickers ligament Abnormal volar tether, radius to lunate |
| L | Lunate subsidence Carpus sinks into the V-notch, pyramid carpus |
| U | Ulna prominent and long Relative ulnar overgrowth, dorsal prominence |
| N | Not unilateral usually Typically bilateral wrists |
| G | Girls more than boys Strong female predominance |
| M | Mesomelic short stature Leri-Weill dyschondrosteosis, the key association | E | Eponymous Vickers ligament Abnormal volar tether, radius to lunate | N | Not unilateral usually Typically bilateral wrists |
| A | Adolescent presentation Pain-free as a child, presents in the teens | L | Lunate subsidence Carpus sinks into the V-notch, pyramid carpus | G | Girls more than boys Strong female predominance |
| D | Distal radius physeal arrest Volar-ulnar corner stops growing | U | Ulna prominent and long Relative ulnar overgrowth, dorsal prominence |
Hook:Spell MADELUNG and you have the whole clinical picture: a short adolescent girl with a tethered, arrested distal radius and a prominent ulna in both wrists.
RIVThree Planes of Deformity
| R | Radial inclination increased Coronal plane - joint tilts toward the ulna |
| I | Inclined volar (palmar tilt) Sagittal plane - carpus drawn towards the palm |
| V | Vickers ligament tethers it The structure driving the volar tether and arrest |
| R | Radial inclination increased Coronal plane - joint tilts toward the ulna |
| I | Inclined volar (palmar tilt) Sagittal plane - carpus drawn towards the palm |
| V | Vickers ligament tethers it The structure driving the volar tether and arrest |
Hook:RIV - Radial inclination up, Inclined volar, Vickers tether - the three things every Madelung X-ray and viva answer needs.
GROWChoosing the Operation
| G | Growing child Skeletal immaturity changes everything |
| R | Release Vickers + physiolysis Growth modulation while bone is still growing |
| O | Osteotomy when mature Dome osteotomy corrects established deformity |
| W | Wrecked joint - salvage Ulnar shortening, DRUJ reconstruction or fusion |
| G | Growing child Skeletal immaturity changes everything | O | Osteotomy when mature Dome osteotomy corrects established deformity |
| R | Release Vickers + physiolysis Growth modulation while bone is still growing | W | Wrecked joint - salvage Ulnar shortening, DRUJ reconstruction or fusion |
Hook:GROW: while the child can still GROW you modulate growth; once growth is over you osteotomise; if the joint is wrecked you salvage.
Overview
Madelung deformity is a developmental abnormality of the wrist in which the distal radius grows in the wrong shape because part of its growth plate stops working. It is named after Otto Madelung, who described it in the 19th century. The typical patient is an adolescent girl who develops a bent-looking wrist with a prominent bump on the little-finger side (the ulnar head), often in both wrists, with pain and reduced movement.
The simplest way to understand it is to think of the distal radius growth plate as having a corner that fails. The volar (palmar) and ulnar corner of the physis stops growing, while the rest of the radius carries on. The result is a joint surface that tilts towards the ulna and towards the palm, a carpus that slides into the gap that forms, and an ulna that ends up looking too long because the radius has shortened on that side. Running across the front of the failing physis is an abnormal thick band, the Vickers ligament, which tethers the lunate to the volar radius and is central to both the pathology and the surgery.
For the exam, Madelung deformity is a favourite because it links genetics (the SHOX gene and Leri-Weill dyschondrosteosis), growth-plate biology, three-dimensional deformity, and a maturity-dependent treatment decision. Get the classic picture, the Vickers ligament, and the growing-versus-mature treatment split, and you have answered most of what an examiner will ask.
Anatomy and Pathophysiology
The distal radius normally grows from a physis (growth plate) that contributes most of the length of the radius. In Madelung deformity, the volar-ulnar part of this physis is abnormal and arrests early, while the dorsal-radial part keeps growing. This asymmetric growth twists the distal radius:
- In the coronal plane, the joint surface tilts so its ulnar side is lower - this is increased radial inclination, and it points the carpus towards the ulna.
- In the sagittal plane, the joint surface tilts towards the palm - increased volar (palmar) tilt - so the carpus and lunate are carried forwards (palmar carpal displacement).
- Because the radius shortens on the affected side while the ulna grows normally, the ulna becomes relatively long and its head sits prominent and often dorsal at the wrist.
The carpus adapts to this distorted radius. The lunate sinks into the notch that forms between the tilted radius and the ulna (lunate subsidence), and the proximal carpal row takes on a wedge or pyramid/triangle shape pointing proximally - a classic descriptive sign on the X-ray.
The Vickers ligament is the abnormal structure at the centre of it all. According to PubMed, histopathological study of patients with Leri-Weill dyschondrosteosis and a confirmed SHOX abnormality showed widespread disorganisation of the radial growth plate - the normally neat parallel columns of cartilage cells are disrupted - and confirmed that the Vickers ligament blends with the triangular fibrocartilage complex (TFCC) (Munns et al., Hand Surg 2001, DOI). This abnormal volar tether is thought to bowstring across the volar physis, both reflecting and reinforcing the local growth disturbance.
Clinical Pearl
The core sentence to memorise: "Madelung deformity is a partial arrest of the volar-ulnar distal radial physis, tethered by an abnormal Vickers ligament running from the volar radius to the lunate, producing increased radial inclination, increased volar tilt, lunate subsidence, and a relatively prominent ulna."
Aetiology and Genetics
Not all Madelung deformity has the same cause. It is useful to split it into a genetic/dysplastic group, where the physis is intrinsically abnormal, and an acquired group, where something damages a previously normal physis.
Causes of Madelung and Madelung-Like Deformity
| Group | Examples | Key point |
|---|---|---|
| Genetic - Leri-Weill dyschondrosteosis | SHOX gene defect (deletion or mutation), mesomelic short stature | The classic association; bilateral, female predominant, true Vickers ligament |
| Other dysplasias / syndromes | Turner syndrome, multiple hereditary exostoses, other skeletal dysplasias | Madelung-like deformity as part of a wider skeletal picture |
| Acquired - trauma | Physeal injury to the volar-ulnar distal radius | Post-traumatic arrest mimics the genetic shape |
| Acquired - infection / other | Osteomyelitis affecting the physis, prior radiation | Damaged physis behaves like an arrest |
| Idiopathic | No identifiable cause or syndrome | Common - do not assume every case is genetic |
The SHOX gene (short-stature homeobox gene) sits in the pseudoautosomal region at the tip of the X and Y chromosomes. Losing one working copy (haploinsufficiency) causes Leri-Weill dyschondrosteosis, a mesomelic (middle-segment) short-stature dwarfism in which Madelung deformity is the most characteristic limb feature. According to PubMed, SHOX haploinsufficiency accounts for around 80% of genetic causes of Leri-Weill dyschondrosteosis, and SHOX-deficient bone shows a disrupted columnar growth plate with an expanded hypertrophic layer and altered programmed cell death of the cartilage cells - the cellular basis of the deformity (Seki et al., Clin Pediatr Endocrinol 2014, DOI).
Clinical Pearl
If the examiner shows a short adolescent girl with bilateral Madelung wrists, link it to SHOX and Leri-Weill dyschondrosteosis and mention that you would consider endocrine/genetic referral. But add the caveat that Madelung-like deformity can also be acquired (trauma, infection) or part of other dysplasias - this shows you are not pattern-matching blindly.
Classification
There is no single universally used classification, but examiners expect you to be able to categorise by cause and to describe the deformity in a structured way. A practical scheme groups Madelung deformity by aetiology, direction, severity, and radiographic measurement:
Dysplastic / genetic (Leri-Weill dyschondrosteosis and SHOX, other dysplasias), post-traumatic, post-infective, and idiopathic. Naming the group guides whether you look for short stature, a family history, or a previous injury.
Clinical Presentation
The classic story is an adolescent girl (commonly early teens) who notices that her wrists look bent and that there is a bump on the ulnar side (the prominent ulnar head). It is frequently bilateral, and there may be a family history and short stature if there is underlying Leri-Weill dyschondrosteosis.
Symptoms:
- Deformity and cosmetic concern - often the main reason for presenting, especially the prominent dorsal ulnar head.
- Pain - typically ulnar-sided wrist pain, worse with loading and activity, often emerging in adolescence as the deformity matures.
- Stiffness - loss of wrist extension and especially loss of forearm rotation (supination), and loss of radial deviation.
- Weakness of grip in symptomatic wrists.
Examination findings:
- A characteristic wrist contour with the hand looking displaced towards the palm and the ulnar head prominent dorsally (the "bayonet" or step look).
- Reduced range of movement, particularly forearm rotation and wrist extension.
- The forearm may look mesomelically short (shortened middle segment) if there is dyschondrosteosis; check the other wrist and the patient's height.
Clinical Pearl
Madelung deformity is a leading congenital cause of ulnar-sided wrist pain and loss of supination in an adolescent. If you are given that triad with a prominent ulnar head and bilateral involvement, Madelung deformity should be at the top of your list.
Investigations
Posteroanterior and lateral wrist X-rays are the cornerstone and usually diagnostic. Look for:
- Increased radial inclination (the joint surface tilting towards the ulna).
- Increased volar tilt and palmar carpal displacement on the lateral view.
- Lunate subsidence into a V-shaped notch between radius and ulna, with the proximal carpal row taking a pyramid/triangle shape.
- A relatively long, dorsally prominent ulna and changes in the sigmoid notch.
- Bowing of the radial shaft and shortening.
Measurements such as ulnar (radial) tilt, lunate subsidence, lunate fossa angle, and palmar carpal displacement quantify the deformity and let you follow progression and judge surgical correction.
Do not stop at the wrist
A bilateral Madelung deformity in a short adolescent is a clue to an underlying skeletal dysplasia. Examine the whole child (height, limb proportions, the other limbs) and have a low threshold for genetic/endocrine referral - the wrist may be the presenting sign of Leri-Weill dyschondrosteosis.
Management
The single most important decision is skeletal maturity: a growing physis can be modulated, whereas an established deformity in a mature wrist can only be corrected by cutting and realigning bone. Severity and symptoms then refine the plan.
Many patients - especially those with mild, painless deformity - never need surgery. The natural history is unpredictable, so a reasonable approach is observation with serial radiographs, activity modification, analgesia, and splinting for symptom control. According to PubMed, mild asymptomatic deformity warrants a period of non-surgical management with serial X-rays because the natural history is uncertain and many patients never require surgery (Kozin and Zlotolow, J Hand Surg Am 2015, DOI).
Matching Treatment to the Patient
| Situation | Typical approach | Aim |
|---|---|---|
| Mild, painless, any age | Observation, serial X-rays, splint/analgesia | Avoid unnecessary surgery; many never progress |
| Growing child, progressive or symptomatic | Vickers ligament release + radial physiolysis (+/- ulnar epiphysiodesis) | Slow progression and let remaining physis grow |
| Mature wrist, symptomatic deformity | Dome osteotomy of distal radius (+/- ulnar shortening) | Correct deformity, offload the ulnar wrist, restore rotation |
| Severely damaged joint / sigmoid notch | Salvage: ulnar procedure, partial arthrodesis, DRUJ reconstruction | Reliable pain relief when reconstruction is not possible |
Clinical Pearl
The examiner wants to hear the maturity split out loud: "If she is still growing I would release the Vickers ligament and free the radial physis to modulate growth; if she is skeletally mature with a symptomatic deformity I would correct it with a dome osteotomy of the distal radius, addressing the ulna as needed." That single sentence carries most of the marks.
Clinical Relevance
Madelung deformity sits at the crossroads of paediatric hand surgery, genetics, and growth-plate biology, which is exactly why it is a recurring exam topic. It teaches the principle that a physeal growth disturbance produces a predictable three-dimensional bony deformity, and that timing surgery to skeletal maturity changes the whole strategy - growth modulation while the physis is open, correction once it is closed. It also rewards the candidate who looks beyond the wrist: recognising the link with SHOX and Leri-Weill dyschondrosteosis turns an isolated wrist problem into a diagnosis that may need genetic and endocrine input. In practice, the everyday skills it tests - reading the deformity on plain films, deciding who needs surgery, and choosing between ligament release, physiolysis, osteotomy, and salvage - are the same judgements that come up across deformity correction in the upper limb.
Complications
Complications of Madelung Deformity and Its Treatment
| Complication | Why it happens | Key point |
|---|---|---|
| Progressive deformity | Ongoing asymmetric physeal growth and tethering | Natural history is unpredictable; serial X-rays in mild cases |
| Ulnar-sided wrist pain | Abnormal radioulnar mechanics, TFCC and DRUJ loading | Common indication for surgery; main complaint in adolescents |
| Loss of forearm rotation and grip | Distorted distal radioulnar joint and sigmoid notch | Supination and wrist extension are typically most affected |
| Early degenerative change | Joint incongruity and chronic abnormal loading | May ultimately need salvage (arthrodesis, DRUJ reconstruction) |
| Recurrence / continued progression after physiolysis | Growth modulation does not fully normalise the physis | Long-term studies show further surgery may be needed after growth |
| Osteotomy-related problems | Nonunion, malunion, hardware irritation, neurovascular risk | Standard risks of distal radius corrective osteotomy |
Evidence
According to PubMed, the following studies support the modern, maturity-dependent approach to Madelung deformity.
Surgical Management of Madelung Deformity: A Systematic Review
- Systematic review of 25 studies (all case series, level IV evidence) describing surgery for Madelung deformity
- The main indication for surgery was wrist pain, not deformity alone
- Procedures fall into radial lengthening/correction, ulnar shortening, or a combination of both
- All studies reported postoperative pain reduction and most reported improved range of motion, but inconsistent outcome reporting prevented pooling of results
Long-Term Outcomes After Vickers Ligament Release and Growth Modulation
- Six skeletally immature patients (8 wrists) with symptomatic Madelung deformity treated by Vickers ligament release and distal radial physiolysis, median follow-up 10.6 years
- Pain relief was durable - 6 of 8 wrists were completely pain-free at final follow-up
- Range of motion was preserved compared with before surgery
- Lunate subsidence still progressed over time and 2 of 6 patients needed a later procedure (one radial dome osteotomy, one ulnar shortening osteotomy)
Dome Osteotomy with Vickers Ligament Section in Children: Esthetic and Functional Outcomes
- Retrospective series of 6 wrists treated with distal radial dome osteotomy plus Vickers ligament section
- Radiographic correction achieved across ulnar tilt, lunate subsidence, lunate fossa angle, and palmar carpal displacement
- Good postoperative motion preserved in flexion, extension, pronation, and supination
- Low residual pain and disability (median DASH 0), with high patient satisfaction
Radioscapholunate Arthrodesis with a Neo-DRUJ as a Salvage Option
- Six wrists with a severely abnormal sigmoid notch treated by radioscapholunate arthrodesis with construction of a neo-distal radioulnar joint, compared with 7 reverse wedge osteotomies
- No difference in postoperative pain, grip strength, or most range of motion between the two salvage approaches
- Quality of life and Michigan Hand Outcomes Questionnaire scores were similar between groups
- Extension was reduced after the arthrodesis-with-neo-DRUJ procedure
Exam Viva Scenarios
Use these scenarios to practise clinical reasoning and management decisions
Adolescent Girl with Bilateral Bent Wrists (~4 min)
"A 14-year-old girl is referred with deformity of both wrists and a prominent bump on the little-finger side. She is short for her age. The examiner asks for your diagnosis and assessment."
Diagnosis: The picture of a short adolescent girl with bilateral wrist deformity and a prominent ulnar head strongly suggests Madelung deformity. This is a partial growth arrest of the volar-ulnar distal radial physis, tethered by an abnormal Vickers ligament, classically associated with Leri-Weill dyschondrosteosis from SHOX gene defects.
History: I would ask about pain (typically ulnar-sided), loss of movement and function, the cosmetic concern, the rate of progression, family history, and any previous wrist trauma or infection that could give an acquired Madelung-like deformity.
Examination: I would assess the wrist contour and the prominent dorsal ulnar head, measure range of motion (looking for reduced wrist extension and forearm supination), check grip, and examine the whole child - her height and limb proportions - because bilateral disease with short stature points to an underlying dysplasia.
Investigation: PA and lateral wrist radiographs are usually diagnostic, showing increased radial inclination, increased volar tilt, palmar carpal displacement, lunate subsidence with a pyramid-shaped carpus, and a relatively long ulna. I would consider MRI to confirm the Vickers ligament, and genetic/endocrine referral for possible SHOX-related Leri-Weill dyschondrosteosis.
Choosing the Operation by Skeletal Maturity (~4 min)
"Two patients have symptomatic Madelung deformity: a 10-year-old with open physes and a 19-year-old who is skeletally mature. The examiner asks how your surgical plan differs and why."
Principle: The key determinant is skeletal maturity. While the physis is open I can modulate growth; once it is closed I must correct the established deformity directly.
The growing child (10-year-old): If she has progressive or symptomatic deformity with growth remaining, I would release the Vickers ligament and perform a radial physiolysis to free the diseased volar-ulnar physis, sometimes with an interposition graft, and consider an ulnar epiphysiodesis to stop the ulna outgrowing the radius. The aim is to slow progression and let the remaining physis grow more normally. I would counsel the family that this gives good pain relief and preserves motion, but that some radiographic progression can continue and a later osteotomy may still be needed.
The mature patient (19-year-old): Growth modulation is no longer possible, so I would correct the deformity with a dome osteotomy of the distal radius for three-dimensional realignment, fixed with a plate, often combined with an ulnar-sided procedure such as an ulnar shortening osteotomy to address the long, prominent ulna and the distal radioulnar joint. If the joint or sigmoid notch is severely damaged, I would consider a salvage option such as a partial wrist arthrodesis.
Both: Mild, painless deformity can be observed with serial X-rays regardless of age - surgery is driven mainly by pain and progression.
MADELUNG DEFORMITY
Clinical summary
The Core Lesion
- •Partial arrest of the volar-ulnar distal radial physis
- •Abnormal Vickers ligament tethers volar radius to lunate
- •Increased radial inclination + volar tilt + palmar carpal displacement
- •Lunate subsidence, pyramid carpus, relatively long prominent ulna
Classic Patient and Association
- •Adolescent girl, bilateral wrists, short stature
- •Leri-Weill dyschondrosteosis from SHOX gene defects
- •But also post-traumatic, post-infective, idiopathic, other dysplasias
- •Presents with ulnar-sided pain and loss of supination/extension
Investigations
- •PA + lateral wrist X-rays - usually diagnostic
- •Measure radial tilt, lunate subsidence, palmar carpal displacement
- •MRI to show Vickers ligament and plan; CT/3D for osteotomy planning
- •Genetic/endocrine referral if syndromic (SHOX testing)
Treatment by Maturity
- •Mild/painless: observe with serial X-rays, splint, analgesia
- •Growing child: Vickers release + physiolysis (+/- ulnar epiphysiodesis)
- •Mature wrist: dome osteotomy of distal radius (+/- ulnar shortening)
- •Destroyed joint: salvage (partial arthrodesis, DRUJ reconstruction)
Guidelines, Registries and Global Practice
- No high-level guideline or registry governs Madelung deformity - it is rare, so the evidence base is case series (level IV) and a systematic review of them, and practice is guided by paediatric hand surgery experience rather than randomised trials.
- Consistent global principle: treat symptoms and progression, not the X-ray alone. Mild, painless deformity is observed with serial radiographs everywhere; surgery is reserved for pain, functional loss, or progressive deformity.
- Maturity-based strategy is universal: growth modulation (Vickers ligament release with radial physiolysis, sometimes ulnar epiphysiodesis) in the skeletally immature wrist, and corrective osteotomy (commonly a distal radial dome osteotomy, with ulnar-sided procedures as needed) once mature. Salvage arthrodesis is reserved for severely damaged joints.
- Multidisciplinary input: because of the strong link with SHOX-related Leri-Weill dyschondrosteosis, bilateral disease in a short child prompts genetic and endocrine assessment in addition to orthopaedic care - a point valued internationally.