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Coxa plana(LCPD)

MedGen UID:
6035
Concept ID:
C0023234
Disease or Syndrome
Synonyms: LCPD; Legg-Calve-Perthes disease; Legg-Calve-Perthes syndrome; Legg-Perthes Disease; Osteochondritis deformans; Perthes disease
SNOMED CT: Perthes disease - osteochondritis of the femoral head (111255008); Coxa plana (240241003); Pseudocoxalgia (111255008); Legg-Calve-Perthes disease (111255008)
 
Gene (location): COL2A1 (12q13.11)
OMIM®: 150600
Orphanet: ORPHA2380

Definition

Legg-Calve-Perthes disease is characterized by loss of circulation to the femoral head, resulting in avascular necrosis in a growing child. Clinical pictures of the disease vary, depending on the phase of disease progression through ischemia, revascularization, fracture and collapse, and repair and remodeling of the bone. The disease occurs more frequently in boys, and most patients tend to be shorter than their peers. Both familial and isolated cases of LCPD have been reported (summary by Chen et al., 2004). [from OMIM]

Clinical features

Short stature
MedGen UID:
87607
Concept ID:
C0349588
Finding
Abnormality of the teeth
MedGen UID:
424982
Concept ID:
CN000160
Finding
Any abnormality of the teeth.
Arthralgia
MedGen UID:
409545
Concept ID:
C1963066
Finding
Delayed skeletal maturation
MedGen UID:
505344
Concept ID:
CN002495
Finding
A decreased rate of skeletal maturation. Delayed skeletal maturation can be diagnosed on the basis of an estimation of the bone age from radiographs of specific bones in the human body.
Avascular necrosis of the capital femoral epiphysis
MedGen UID:
428180
Concept ID:
CN005076
Finding
Avascular necrosis of the proximal epiphysis of the femur occurring in growing children and caused by an interruption of the blood supply to the head of the femur close to the hip joint. The necrosis is characteristically associated with flattening of the femoral head, for which reason the term coxa plana has been used to refer to this feature in the medical literature.
Cartilage destruction
MedGen UID:
452007
Concept ID:
CN117663
Finding
Skeletal muscle atrophy
MedGen UID:
505481
Concept ID:
CN002890
Finding
The presence of skeletal muscular atrophy (which is also known as amyotrophy).
Avascular necrosis of the capital femoral epiphysis
MedGen UID:
428180
Concept ID:
CN005076
Finding
Avascular necrosis of the proximal epiphysis of the femur occurring in growing children and caused by an interruption of the blood supply to the head of the femur close to the hip joint. The necrosis is characteristically associated with flattening of the femoral head, for which reason the term coxa plana has been used to refer to this feature in the medical literature.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCoxa plana
Follow this link to review classifications for Coxa plana in Orphanet.

Recent clinical studies

Etiology

Accadbled F, Pailhé R, Launay F, Nectoux E, Bonin N, Gicquel P; SOFCOT
Orthop Traumatol Surg Res 2014 Oct;100(6):647-9. Epub 2014 Sep 10 doi: 10.1016/j.otsr.2014.03.029. [Epub ahead of print] PMID: 25217031
Richards BS, Coleman SS
J Bone Joint Surg Am 1987 Dec;69(9):1312-8. PMID: 3440790
Chakirgil GS, Isitman AT, Ceten I
Orthopedics 1985 Dec;8(12):1495-504. PMID: 3832036
Clarke NM, Harrison MH
J Bone Joint Surg Am 1983 Jan;65(1):13-8. PMID: 6848528
Friberg S
Acta Orthop Scand 1975 May;46(2):234-45. PMID: 1146517

Diagnosis

Katz JF, Siffert RS
Clin Orthop Relat Res 1975 Jan-Feb;(106):75-85. PMID: 1126092
Specht EE
West J Med 1974 Apr;120(4):287-94. PMID: 4595380Free PMC Article
Bohr H
Acta Orthop Scand 1973;44(4):417-25. PMID: 4771276
Edgren W
Acta Orthop Scand Suppl 1965:Suppl 84:1-129. PMID: 5850148

Therapy

Accadbled F, Pailhé R, Launay F, Nectoux E, Bonin N, Gicquel P; SOFCOT
Orthop Traumatol Surg Res 2014 Oct;100(6):647-9. Epub 2014 Sep 10 doi: 10.1016/j.otsr.2014.03.029. [Epub ahead of print] PMID: 25217031
Catterall A
Mod Trends Orthop 1972;6:122-47. PMID: 4669849

Prognosis

Accadbled F, Pailhé R, Launay F, Nectoux E, Bonin N, Gicquel P; SOFCOT
Orthop Traumatol Surg Res 2014 Oct;100(6):647-9. Epub 2014 Sep 10 doi: 10.1016/j.otsr.2014.03.029. [Epub ahead of print] PMID: 25217031
Richards BS, Coleman SS
J Bone Joint Surg Am 1987 Dec;69(9):1312-8. PMID: 3440790
Chakirgil GS, Isitman AT, Ceten I
Orthopedics 1985 Dec;8(12):1495-504. PMID: 3832036
Marklund T, Tillberg B
J Bone Joint Surg Br 1976 Feb;58(1):25-30. PMID: 1270492
Specht EE
West J Med 1974 Apr;120(4):287-94. PMID: 4595380Free PMC Article

Clinical prediction guides

Accadbled F, Pailhé R, Launay F, Nectoux E, Bonin N, Gicquel P; SOFCOT
Orthop Traumatol Surg Res 2014 Oct;100(6):647-9. Epub 2014 Sep 10 doi: 10.1016/j.otsr.2014.03.029. [Epub ahead of print] PMID: 25217031
Sangkaew C, Piyapittayanun P
J Med Assoc Thai 2012 Oct;95 Suppl 10:S12-9. PMID: 23451432
Smith RB, Nevelös AB
Acta Orthop Scand 1980 Jun;51(3):445-9. PMID: 7446024
Friberg S
Acta Orthop Scand 1975 May;46(2):234-45. PMID: 1146517
Willner S
Acta Orthop Scand 1975 Apr;46(1):102-7. PMID: 1136725

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