Thiemann's disease

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Classification according to ICD-10
M93.2 Osteochondrosis dissecans
ICD-10 online (WHO version 2019)

The disease Thiemann is a very rare Aseptic osteonecrosis on fingers , less commonly, the big toe .

Synonyms are: Thiemann syndrome; Thiemann disease; Aseptic osteonecrosis of the phalangeal epiphyses; Familial osteoarthropathy of the fingers; osteochondrosis of the phalangeal epiphyses; Osteochondrosis, aseptic type Thiemann; Thiemann-Fleischner's disease; Latin Acrodysplasia epiphysearia ; English Thiemann Epiphyseal Disease; Osteoarthropathy Of Fingers, Familial

The first description comes from the year 1909 by the German surgeon H. Thiemann.

distribution

The frequency is not known. The disease occurs in families with an autosomal dominant inheritance or sporadically.

root cause

The cause is not known. In addition to a disorder of ossification (osteochondrosis), a youthful form of osteonecrosis is also discussed.

It is not certain whether it is actually osteonecrosis; the histology shows normal vessels and no inflammatory reactions. A bone fracture may have preceded the bone necrosis due to trauma or stress.

Clinical manifestations

Clinical criteria are:

  • Manifestation mostly between the ages of 8 and 14
  • Usually the epiphyses of the 2nd and 3rd fingers in the middle and distal phalanx, also in the metatarsophalangeal joint, as well as the metatarsal joint of the big toe and the first metatarsal bone are affected
  • Painless to painless joint swelling of the interphalangeal joints
  • Tension pain and partially restricted mobility
  • sometimes shortening or misalignment when the growth plate is damaged

diagnosis

The x-ray shows a fissure or dissolution of the epiphysis in the affected areas.

Differential diagnostics

Must be distinguished is a stress-related Epiphysenfraktur that rheumatoid arthritis is a osteoarthritis or septic osteonecrosis.

therapy

Treatment is conservative, the disease is usually self-limiting. With immobilization, pain relief and anti-inflammatory medication, the symptoms usually recede completely.

literature

Individual evidence

  1. ↑ a b c Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
  2. ↑ a b c Thiemann disease. In: Orphanet (Rare Disease Database).
  3. ↑ a b c d e f C. Lutter: Thiemann's disease. In: e.Medpedia
  4. ^ H. Thiemann: Juvenile epiphyseal disturbance. In: ROEFO Vol. 14, 1909, pp. 79-87
  5. ↑ Radiopaedia
  6. ^ P. Mangat, AS Jawad: Case number 32: Thiemann's disease. In: Annals of the rheumatic diseases. Volume 64, number 1, January 2005, pp. 11-12, doi: 10.1136 / ard.2004.024836 , PMID 15608298 , PMC 1755196 (free full text).
  7. ↑ C. Radtke, A. Steiert, P. Vogt: Thiemann's disease or epiphyseal PIP joint fractures at D3 in young sport climbers? A case report from identical twins. In: German Society for Hand Surgery. 54th Congress of the German Society for Hand Surgery. Düsseldorf, 10-12 October 2013. German Medical Science GMS Publishing House, Düsseldorf. [1]

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