Dupuytrens contracture

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Dupuytren's contracture
Dupuytren's contracture of the fourth digit (ring finger).
ICD-10 M72.0
ICD-9 728.6
OMIM 126900
DiseasesDB 4011
MedlinePlus 001233

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Editors-In-Chief: Mohamed Riad, M.D.[1], Matthew I. Leibman, M.D.[2]; Mark R. Belsky, M.D.[3]; David E. Ruchelsman, M.D.[4]Kiran Singh, M.D. [5]

Synonyms and keywords: Dupuytren contracture I, included; DUPC1; palmar fascial fibromatosis; palmar fibromatosis

Overview

Dupuytren's contracture (also known as Morbus Dupuytren) is an abnormal thickening of the hand (palmar fascia) causing curling of fingers and impaired function of the fingers, especially the little and ring fingers.. It is named after the famous surgeon Baron Guillaume Dupuytren, who described an operation to correct the affliction. It is thought that Dupuytren's contracture is the result of microvascular angiopathy. The incidence of Dupuytren's contracture increases with age; the median age at diagnosis is 50-70 years. men are affected more often than women. Common risk factors in the development of Dupuytren's contracture include family history, diabetes, antiepileptic medications, liver disease, smoking, alcoholism and manual work. Dupuytren’s disease is progressive in nature with no available definitive cure.

Historical Perspective

Pathophysiology

Pathogenesis

Associated Conditions

Causes

The cause of Dupuytren's contracture has not been identified.

Epidemiology and Demographics

Prevalence

Age

Race

  • Dupuytren's contracture usually affects individuals of the northern Europeans race (Icelandic and Scandinavian populations).

Gender

Risk Factors

Common risk factors in the development of Dupuytren's contracture include:

Natural History, Complications and Prognosis

Natural History

Complications

Prognosis

Diagnosis

Diagnostic Study of Choice

Dupuytren’s disease is primarily diagnosed based on the clinical presentation.

History and Symptoms

  • Symptoms of Dupuytren's disease include abnormal thickening of the hand (palmar fascia) causing curling of fingers and impaired function of the fingers, especially the little and ring fingers.
  • It usually has a gradual onset, often beginning as a tender lump in the palm.
  • It often starts as a nodule, usually in line with the ring finger.
  • Over time, pain associated with the condition tends to go away, but tough bands of tissue may develop.
  • These bands, which are the source of the reduced mobility commonly associated with the condition, are visible on the surface of the palm, and may appear similar to a small callus.
  • It commonly develops in both hands, and has no connection to dominant- or non-dominant hands, nor any correlation with right- or left-handedness.
  • The contracture sets on very slowly, especially in women. However, when present in both hands, and when there is associated foot involvement, it tends to accelerate more rapidly.

Physical Examination

Treatment

Medical Therapy

  • Pharmacologic medical therapies for Dupuytren's disease include pain management and corticosteroid injection into the nodules,
  • In some cases, repeated corticosteroid injections may cause softening of the nodules and slow down the disease progression.
  • Most cases with simple nodules and without significant functional impairment benefit from conservative medical therapy.

Interventions

Collagenase clostridium histolyticum (CCH) injections

  • The mechanism of action is to weaken the contracted cord by breaking down collagen.
  • It is done in an outpatient practice but requires another clinic visit to snap the cord.
  • I t is generally safe; however several complications were reported, such as tendon rupture and pulley injury.
  • CCH injectiont is available in only North America after its withdrawal from European, Australian, and Asian markets.

Surgery

Surgery is not the first-line treatment option for patients with Dupuytren's disease. Surgery is usually reserved for patients with either:

The choice of the type of surgery depends the following variables:

  • Severity of the disease
  • Individual characteristics (such as age, occupation, degree of functional disability)
  • Patient and/or physician preference

Surgical options include:

  • Percutaneous needle fasciotomy (PNF): involves division of the cord using a needle. It is generally effective and safe with no complications; however tendon and digital nerve and vessel damage were reported in some cases. Its efficacy is low in the management of extensive contractures or contractures involving the proximal interphalangeal joints. The recurrence rate is high.
  • Fasciectomy either partial (segmental aponeurectomy) or complete (limited fasciectomy): involves excision of the cord with higher rate of complications PNF.
  • Dermofasciectomy: a more invasive procedure that involves excising the whole diseased tissue, along with the overlying subcutaneous fat and skin. It is reserved for more severe cases with extensive skin involvement when the other surgical options have failed. The complication rate is the highest among the three surgical options. The recurrence rate is low.


External links

http://www.nlm.nih.gov/medlineplus/ency/article/001233.htm

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