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ペルテス病

個人契約の方の無料トライアル・お申込み

施設契約の方の見積り・お問い合わせ

整形股関節

著者: 薩摩眞一兵庫県立こども病院整形外科

監修: 酒井昭典産業医科大学整形外科学教室

著者校正/監修レビュー済：2023/06/22

患者向け説明資料

改訂のポイント：

定期レビューを行った（変更なし）。

概要・推奨

学童前期に跛行が継続し膝周辺の疼痛を訴える場合には本疾患も鑑別に挙げ、股関節に可動域制限や疼痛がないかを調べ、ときに応じてX線撮影を行うことも念頭に置く（推奨度2）。

MRIは早期診断のみならず、壊死範囲の広がりを評価するために必須の検査である（推奨度1）。

病態・疫学・診察

疾患情報（疫学・病態）

ペルテス病は1910年にLegg^[1]、Calvé^[2]、 Perthes^[3]ら3人により相次いで報告された。

病態は、何らかの原因により起こった大腿骨近位骨端部の阻血性壊死で、いわゆる骨端症の1つと位置づけられる。

その原因については外傷説、炎症説、内分泌異常説、血液凝固系異常説など種々の報告がなされているが定説はない。

4～8歳、いわゆる学童期の男児に多い。

予後に影響を与える因子としては、発症年齢と壊死範囲が重要と考えられている。すなわち年長児発症例ほど、壊死範囲が広いほど予後が不良となる可能性が高い。

問診・診察のポイント

問診：

跛行の有無を確認する。

これより先の閲覧には個人契約のトライアルまたはお申込みが必要です。

最新のエビデンスに基づいた二次文献データベース「今日の臨床サポート」。
常時アップデートされており、最新のエビデンスを各分野のエキスパートが豊富な図表や処方・検査例を交えて分かりやすく解説。日常臨床で遭遇するほぼ全ての症状・疾患から薬剤・検査情報まで瞬時に検索可能です。

まずは15日間無料トライアル

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文献

Legg AT: An obscure affection of the hip joint. Boston Med Surg J 162: 202-204, 1910.

Calvé : u rune forme particulare de pseudo-coxalgia greffee sur des deformations caracteristiques de l’extremite superiure due femur. Revue de Chirurgie 30: 54-84, 1910.

Perthes GC: Uber arthritis deformans juvenilis. Deutsche Zeitschrift fur Chirurgie 107: 11-59, 1910.

A Catterall
The natural history of Perthes' disease.
J Bone Joint Surg Br. 1971 Feb;53(1):37-53.

Abstract/Text

PMID 5578764

J A Herring, J B Neustadt, J J Williams, J S Early, R H Browne
The lateral pillar classification of Legg-Calvé-Perthes disease.
J Pediatr Orthop. 1992 Mar-Apr;12(2):143-50.

Abstract/Text To determine the predictive value of a new classification system for Legg-Perthes, 93 hips in 86 patients with radiographic follow-up to maturity were reviewed. All patients were treated by bracing at the Texas Scottish Rite Hospital from 1970 to 1980. Hips were classified during the fragmentation stage of disease into three groups based on radiolucency in the lateral pillar of the femoral head. Final radiographs were reviewed at skeletal maturity, and the outcome was determined according to the Stulberg classification. Group A had a uniformly good outcome (100% Stulberg I and II results); Group B had a good outcome in patients who were less than 9 years at onset (92% Stulberg I and II, 8% Stulberg III results), but a less favorable outcome in patients who were greater than 9 years at onset (30% Stulberg II, 50% Stulberg III, and 20% Stulberg IV results). In Group C, the majority of femoral heads became aspherical in both age groups (29% Stulberg II, 52% Stulberg III, and 19% Stulberg IV results). The group C hips also had a longer duration of fragmentation and reossification stages. Members of the Legg-Perthes study group agreed 78% of the time when applying the classification to unknown radiographs. The classification group was a stronger determinant than age of onset in predicting final outcome. This classification system is easy to apply during the active stage of the disease and has a high correlation in predicting the amount of flattening of the femoral head at skeletal maturity.(ABSTRACT TRUNCATED AT 250 WORDS)

PMID 1552014

John A Herring, Hui Taek Kim, Richard Browne
Legg-Calve-Perthes disease. Part I: Classification of radiographs with use of the modified lateral pillar and Stulberg classifications.
J Bone Joint Surg Am. 2004 Oct;86-A(10):2103-20.

Abstract/Text BACKGROUND: Accurate and reliable radiographic classifications of the relative severity and outcome of Legg-Calve-Perthes disease are essential in the study of that disease. As part of a prospective multicenter study, we sought to define more clearly the lateral pillar classification of severity and the Stulberg classification of outcome; we sought especially to define the borderlines between classification groups.
METHODS: We performed interobserver and intraobserver trials of the lateral pillar and Stulberg classifications using sets of twenty radiographs chosen from a prospective study of 345 hips. To establish reliable definitions of the lateral pillar classification, we added a new, intermediate group termed the B/C border group, which includes femoral heads with a thin or poorly ossified lateral pillar and those with a loss of exactly 50% of the original height of the lateral pillar. The resulting classification consists of four groups: A, B, B/C border, and C. In our application of the classification system of Stulberg et al., we defined a class-II femoral head as round and fitting within 2 mm of a circle on both anteroposterior and frog-leg lateral radiographs. We defined a Stulberg class-III femoral head as out of round by more than 2 mm on either view and a Stulberg class-IV femoral head as one with at least 1 cm of flattening of the weight-bearing articular surface. To assess interobserver and intraobserver agreement, we performed two trials of each classification with six orthopaedic surgeons reviewing twenty radiographs or pairs of radiographs.
RESULTS: In the first trial of the lateral pillar classification, there was 81% agreement per radiograph and the average weighted kappa was 0.71. In the second trial, there was 85% agreement per radiograph and the weighted kappa averaged 0.79. Intraobserver reliability testing showed a 77% match between Trials 1 and 2, an average weighted kappa of 0.81, and an average generalizability coefficient of 0.91. In Trial 1 of the Stulberg classification, there was 91% agreement per radiograph and an average weighted kappa of 0.82. In Trial 2, there was 92% agreement per radiograph and an average weighted kappa of 0.82. Intraobserver reliability testing showed an 89% match between Trials 1 and 2, an average weighted kappa value of 0.88, and an average generalizability coefficient of 0.92.
CONCLUSIONS: The interobserver and intraobserver trials of these classifications produced kappa values and generalizability coefficients in the excellent range. The modified lateral pillar classification and the redefined Stulberg classification are sufficiently reliable and accurate for use in studies of Legg-Calve-Perthes disease.

PMID 15466719

S D Stulberg, D R Cooperman, R Wallensten
The natural history of Legg-Calvé-Perthes disease.
J Bone Joint Surg Am. 1981 Sep;63(7):1095-108.

Abstract/Text Two groups of patients who had Legg-Calvé-Perthes disease were studied. The first group of patients consisted of eighty-eight patients (ninety-nine affected hips) followed in three hospitals for an average of forty years. The second group consisted of sixty-eight patients (seventy-two affected hips), all of whose radiographs from the onset of disease to maturity were available and all of whom had been treated in one hospital. The patients in this second group were followed for an average of thirty years. Each hip in both study groups could be placed into one of five classes of deformity based on its radiographic appearance at maturity. Each class showed a characteristic pattern of involvement during the active stages of the disease and had a specific long-term clinical and radiographic course. The clinical and radiographic course of an involved hip subsequent to childhood was related to the type of congruency that existed between the femoral head and acetabulum. Three types of congruency were recognized: (1) spherical congruency (Class-I and II hips) - in hips in this category arthritis does not develop; (2) aspherical congruency (Class-III and IV hips) - mild to moderate arthritis develops in late adulthood in these hips; and (3) aspherical incongruency (Class-V hips) - severe arthritis develops before the age of fifty years in these hips.

PMID 7276045

薬剤監修について：
オーダー内の薬剤用量は日本医科大学付属病院薬剤部部長伊勢雄也　以下、渡邉裕次、井ノ口岳洋、梅田将光および日本医科大学多摩永山病院副薬剤部長林太祐による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。

※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。

※同効薬・小児・妊娠および授乳中の注意事項等は、海外の情報も掲載しており、日本の医療事情に適応しない場合があります。

※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適応の査定において保険適応及び保険適応外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適応の記載の一部を割愛させていただいています。
（詳細はこちらを参照）

著者のCOI（Conflicts of Interest）開示:
薩摩眞一 : 特に申告事項無し[2025年]
監修：酒井昭典 : 講演料（旭化成ファーマ（株），帝人ヘルスケア（株））[2025年]

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