2004-03-14
PIPJ: fingers > 45 deg flexion need secondary release (Ritchie JHSB 2004)
Sources of contracture:
PIPJ release in DD (Paul Smith)
Steps
Author: Alphonsus Chong
Overview Proliferative Fibroplasia involving fascia of palm and digits leading to nodules, cords and contractures
Epidemiology
• True incidence unknown - influenced by geography • Males predominantly (M:F, 10:1) - typically white caucasian of north european descent • extremely rare under 13 years of age • More common in temperate zones • More common in Northern Europeans, Scandinavians
Pathology:
Etiologic factors
• multifactorial with genetic factors of primary importance • some factors: 1 traumatic - increased incidence a/w occupational hand trauma (vibration) 2 Neoplastic - immunocytochemical studies show relationship between sarcoma and DC; growth factors involved - FGF, TGFalpha, EGF 3 Genetic - AD with variable penetrance 4 ?autoimmune component - T-cell mediated HI disorder; controversial 5 nordic theory - "viking disease" to modern europeans: celts, caucasians of northern europe descent 6 Risk factors: Diabetes Mellitus, Chronic alcoholism (believed to be related to liver involvement - lipid metabolism), epilepsy (?medication related), Chronic pulmonary disease, HIV infection, heavy smoker
Molecular biology and pathophysiology
• Similarities between DC nodules and wound healing (based on immunocytochemistry and cell culture tests); BUT DC is sporadic and progressive • Myofibroblasts (MF)- ? characteristics of both fibroblasts and smooth muscle cells ? origin uncertain - likely to be modulated contracture fibroblast ? contractile - PG; lysophosphatidic acid ? alpha- smooth muscle actin ? Features differentiating it from fibroblasts: ? larger ? indented nucleus ? cytoplasmic actin microfilaments ? macrophage source of GF and free radicals • ECM - synthesis and deposition of fibronectin (important in MF-MF and cell-stroma binding) leads to transmission of cellular contraction; increased PG, collagenespecially type III:I • Mechanical strain leads to cell culture proliferation
Presentation
Initially presents with nodule in the palmar fascia
Clinical types
Dupuytren's diathesis
• Young patient with severe disease • recurrent even after surgery • associated with palmar knuckle, plantar and penile fibromatosis • no family history • radial side • ectopic progression
Differential diagnoses
• isolated trigger finger • Palmar induration • isolated digital dupuytren's
Non-surgical Management
1 Calcium channel blockers 2 Collagenase - big trial 3 Radiation 4 Intralesional steroids 5 Intralesions gamma interferon 6 External distraction - e.g. Messina's TEC device - few advantages, increased complications
Surgery when indicated
1 Severity of contracture 1 Hueston's table-top test 2 MP joint flexion contracture => 30 degrees 3 any IPJ flexion contracture 2 Infection of web space or flexor pits
Surgical options
1 Fasciotomy 2 Partial fasciectomy 3 Total fasciectomy 4 Dermafasciectomy - for Dupuytren's diathesis 5 Open palmar fasciectomy - McCash 6 Arthroplasty 7 Amputation - only if all else has failed; mainly for reasons of hygiene
Complications of surgery
• Recurrence 2-60% • Skin necrosis • hematoma • neurovascular injury • infection • digital flexion
Historical Notes
References