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MANNERFELT'S SYNDROME

Introduction

Some researchers and authors define Mannerfelt syndrome1 solely as absence of flexion at the interphalangeal (IP) joint of the thumb. Others expand the definition to include absence of flexion at the distal IP joints in the index and long fingers. The conflation may occur because once the flexor pollicis longus (FPL) tendon to the thumb ruptures, the flexor digitorum superficialis (FDS), and profundus (FDP) tendons to the index and long fingers become vulnerable, and their risk of rupture increases. More often the cause is systemic, resulting from rheumatoid arthritis (RA). Because tendon rupture can be painless, patients may be slow to seek medical attention, and diagnosis is delayed. 1,2,3

Pathophysiology

  • The FPL and FDP are vulnerable when subluxation and spur formation are present at the trapeziometacarpal (TM) or scaphotrapezial joint as well as the volar radiocarpal joint.
  • Rupture occurs due to carpal irregularities, flexor tenosynovitis, or volar carpal bone subluxation into the carpal tunnel.
    • Rarely, causes of FPL tendon rupture are iatrogenic, for example, after a screw or wire misplacement during TMjoint arthrodesis, after volar distal radius plate fixation, after steroid injections at the carpal tunnel or flexor tendon sheath.2,3,4

Related Anatomy

  • The most common site of FPL rupture is at the volar scaphoid.
  • Untreated, an FPL rupture can be followed by rupture(s) of the FDP and FDS of the index finger and occasionally the FDP to the long finger.

Incidence and Related Conditions

  • RA-related hand pathology includes a number of different injuries, but tenosynovitis and tendon ruptures (causes of Mannerfelt syndrome) are common.   
  • Non-RA causes (ie, iatrogenic, mechanical) of Mannerfelt syndrome are rare.
  • Abrasive surfaces in the carpal tunnel that cause tendon fraying can result from trauma such as fractures, wrist dislocations (eg, scaphoid nonunion, Bennett fracture, Colles’ fracture) or STT osteoarthritis.
  • Mannerfelt sydrome may also be associated with diffuse flexor tennosynovitis with palpable crepitus at the proximal edge of the transverse carpal ligament i.e. trigger wrist. 2,3,4

Differential Diagnosis

  • Anterior interosseous nerve (AIN) syndrome
  • Carpal tunnel syndrome
  • FDP rupture
  • IP joint arthritis
  • Trigger thumb 
ICD-10 Codes

  • MANNERFELT'S SYNDROME

    Diagnostic Guide Name

    MANNERFELT'S SYNDROME

    ICD 10 Diagnosis, Single Code, Left Code, Right Code and Bilateral Code

    DIAGNOSISSINGLE CODE ONLYLEFTRIGHTBILATERAL (If Available)
    MANNERFELT'S SYNDROME M66.342M66.341 

    ICD-10 Reference

    Reproduced from the International statistical classification of diseases and related health problems, 10th revision, Fifth edition, 2016. Geneva, World Health Organization, 2016 https://apps.who.int/iris/handle/10665/246208

Clinical Presentation Photos and Related Diagrams
Clinical Photos
  • Right handed 62 y.o. male complaining of weak pinch and inability to flex thumb and index
    Right handed 62 y.o. male complaining of weak pinch and inability to flex thumb and index
  • Male with long standing RA complaining of poor pinch and grip. He can not flex the thumb, index or long fingers.
    Male with long standing RA complaining of poor pinch and grip. He can not flex the thumb, index or long fingers.
  • Right handed female with arthritic index DIP and sudden loss of thumb flexion.
    Right handed female with arthritic index DIP and sudden loss of thumb flexion.
Symptoms
Tendency to drop objects secondary to weak pinch
Absent active flexion of the thumb at the IP joint
Stiffness of the wrist and thumb with palmar radial swelling
Associated carpal tunnel syndrome symptoms can occur
Typical History

Fortunately, patients tend to detect loss of thumb function early. On presentation, they will complain of loss of thumb IP flexion and a weakened pinch. Patients may also mention a grinding sound or sensation at the wrist. The medical history may include RA, previous hand surgery or multiple steroid injections.

Positive Tests, Exams or Signs
Work-up Options
Images (X-Ray, MRI, etc.)
Imaging in Mannerfelt Syndrome
  • Oblique wrist X-ray showing osteophytes that caused attritional FPL rupture
    Oblique wrist X-ray showing osteophytes that caused attritional FPL rupture
Treatment Options
Treatment Goals
  • Improve hand function by reconstruction of flexor tendons with or without thumb IP arthrodesis.
  • Prevent the rupture of additional flexor tendons.
Conservative
  • If the flexor pollicis longus is completely ruptured then conservative non-surgical therapeutic measures are not going to manage the problem.  If the flexor tendon function can not be assessed then treating the flexor tennosynovitis and joint stiffness may help the diagnostic progress.  Treatment options in this situation may include:
    1. Cortisone injection and intermittent immobilization
    2. Hand therapy
    3. Occupational therapy 
Operative
  • Operative treatment should include a tenosynovectomy the flexors; it will help preserve, restore function and usually prevent rupture of intact flexor tendons. 
  • Operative treatment after flexor tendon rupture:
    • Tenosynovectomy with finger DIP or thumb IP joint arthrodesis
    • Tendon transfer of the ring FDS tendon to the FPL
    • Tendon grafting (PL) to reconstruct the FPL
  • Surgery should be delayed in patients with active disease
  • Surgery is followed by 6-week immobilization of the thumb and wrist after an IP arthrodesis
    • The wrist should be at mild flexion
    • The MP joint should be at 40° of flexion
    • The IP joints are also in a functional degree of flexion
    • After 6-weeks, a gentle range-of-motion program—without resistance—is started; resistance is added at 12 weeks. Normal activity is progressively resumed.
  • Surgery is followed by 6-week hand therapy rehab protocol after tendon transfer or tendon grafting.
  • Surgery should also include exploration of the carpal tunnel to remove any osteophytes and to repair the volar wrist capsule, if possible, in order to prevent future flexor tendon ruptures. 
Treatment Photos and Diagrams
Surgical Treatment of Mannerfelt Syndrome
  • Incision for tenosynovectomy and flexor tendon reconstruction with second incision for harvesting PL graft (arrow)
    Incision for tenosynovectomy and flexor tendon reconstruction with second incision for harvesting PL graft (arrow)
  • 1 - Ring FDS transfer to distal ruptured FPL; 2 - Median Nerve; 3 - proximal ruptured flexor tendon ends; 4 - Distal ruptured flexor tendon ends
    1 - Ring FDS transfer to distal ruptured FPL; 2 - Median Nerve; 3 - proximal ruptured flexor tendon ends; 4 - Distal ruptured flexor tendon ends
  • Floor of carpal tunnel with probe on volar carpal bone osteophyte that caused the attritional ruptures. Adjacent clamp on volar wrist capsule.
    Floor of carpal tunnel with probe on volar carpal bone osteophyte that caused the attritional ruptures. Adjacent clamp on volar wrist capsule.
  • Mannerfelt surgical reconstruction complete: 1 - drain in carpal tunnel; 2 - incision for MP joint arthrodesis; 3 - K-wire fixation for MP joint arthrodesis
    Mannerfelt surgical reconstruction complete: 1 - drain in carpal tunnel; 2 - incision for MP joint arthrodesis; 3 - K-wire fixation for MP joint arthrodesis
  • Chronic severe RA with multiole flexor tendon ruptures (I,II,III): 1 -Ring FDS for FPL reconstruction; 2 - Excised not salvageable flexor tendons; 3 - Palmar longus (PL) graft for ring flexor tendon reconstruction
    Chronic severe RA with multiole flexor tendon ruptures (I,II,III): 1 -Ring FDS for FPL reconstruction; 2 - Excised not salvageable flexor tendons; 3 - Palmar longus (PL) graft for ring flexor tendon reconstruction
CPT Codes for Treatment Options

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Common Procedure Name
Flexor tenosynovectomy
CPT Description
Radical excision of bursa, synovia or wrist, or forearm tendon sheaths (e.g. tenosynovitis, fungus, Tbc, or other granulomas, rheumatoid arthritis); flexors
CPT Code Number
25115
CPT Code References

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CPT 2021 Professional Edition: Spiralbound

Complications
  • Attenuation of the transferred tendon
  • Infection
  • Joint stiffness
  • Loss of motion due to improper tensioning of the transferred tendon or post-operative sccarring
  • Nerve injury
  • Re-rupture of the tendon
  • Skin or wound breakdown
Outcomes
  • In the patient with RA, results are highly dependent on the status of the disease.
  • In all patients, results are highly dependent on the patient’s ability to cooperate with postoperative splinting and rehabilitation.
    • Supervision by a hand therapist is key to good outcomes
    • With good medical management, controlled RA, and a motivated patient, good results can be expected
Key Educational Points
  • Because FPL rupture can be painless, discovery is important, as successive rupture of the FDS and FDP tendons can occur.
  • Rupture of the FPL tendon is more disabling than rupture of the EPL tendon. 
  • In patients with RA, chronic synovitis compromises structural integrity of the tendons and produces degenerative changes that can lead to Mannerfelt syndrome. 
  • Bony protrusions are common in patients with RA, and they may cause abrasion and eventual attritional flexor tendon rupture.
References

Cited and New Articles

  1. Mannerfelt L, Norman O. Attrition ruptures of flexor tendons in rheumatoid arthritis caused by bony spurs in the carpal tunnel. A clinical and radiological study. J Bone Joint Surg Br 1969;51(2):270-7. PMID: 5770406Case Report
  2. Kanaya K, Kanaya K, Iba K, Yamashita T. Attrition rupture of the flexor pollicis longus tendon after arthrodesis of the trapeziometacarpal joint: a case report. Hand Surgery 2015;20(3):474-76. PMID: 26388014
  3. Netscher DT, Badal JJ. Closed flexor tendon ruptures. J Hand Surg Am 2014;39(11):2315-23. PMID: 25442746.
  4. Trieb K. Treatment of the wrist in rheumatoid arthritis. J Hand Surg Am 2008;33(1):113-23. PMID: 18261675

Reviews

  1. Netscher DT, Badal JJ. Closed flexor tendon ruptures. J Hand Surg Am 2014;39(11):2315-23. PMID: 25442746
  2. Trieb K. Treatment of the wrist in rheumatoid arthritis. J Hand Surg Am 2008;33(1):113-23. PMID: 18261675

Classic

  1. Mannerfelt L, Norman O. Attrition ruptures of flexor tendons in rheumatoid arthritis caused by bony spurs in the carpal tunnel. A clinical and radiological study. J Bone Joint Surg Br 1969;51(2):270-7. PMID: 5770406
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