Dysplasia epiphysealis capitis femoris: Meyer dysplasia / Displasia epifisaria de la cabeza femoral: displasia de Meyer

Introduction: Epiphyseal dysplasia of the femoral head (EDFH) is defined as an alteration in the development of the child’s hip, characterized by delayed ossification of the proximal femoral epiphysis. Methods: Herein, we present six cases of epiphyseal dysplasia of the femoral head (EDFH), seen by the principal author (EVA) within the last six years with minimum follow up at 15 months. Results: The cases were all diagnosed as casual findings. None of the children had symptoms or clinical signs in the hip, only one had a history of hip pain for five days, two months prior, which was diagnosed at the time as transient synovitis. Discussion: Among the differential diagnoses, the main one is Perthes disease, which is differentiated by several parameters like earlier age onset for EDFH (in children below 4 years of age), bilateralism (50% vs. 10%), and a calmer presentation in Meyer’s dysplasia. The evolution in Meyer’s dysplasia is toward improving radiographic changes. None of the patients revealed incongruence of the hip or early degenerative changes, indicating an excellent prognosis. Many authors think it is a variant of the normal ossification of the femoral head.

Introducción: La displasia epifisaria de la cabeza femoral (DECF) es definida como una alteración del desarrollo de la cadera en el niño, caracterizada por un retraso en la osificación con irregularidad en el núcleo de osificación de la epífisis proximal del fémur. Métodos: Se presentan 6 casos de displasia epifisaria de la cabeza femoral (DECF), atendidos por el autor principal (EVA) en los últimos 6 años, con un seguimiento mínimo de 15 meses. Resultados: Todos los casos fueron diagnosticados como un hallazgo casual. Ninguno de los niños tenía síntomas ni signos clínicos en la cadera, solo uno tenía antecedente de dolor de cadera durante 5 días, dos meses antes, el cual fue diagnosticado en su momento como sinovitis transitoria. Discusión: Entre los diagnósticos diferenciales, el principal es la Enfermedad de Perthes, el cual se diferencia en varios parámetros como son edad de inicio más temprano para la DECF (en niños menores de 4 años), bilateralidad (50% vs. 10%) y presentación más silente en la displasia de Meyer. La evolución en la displasia de Meyer es hacia la mejoría de los cambios radiológicos. En ningún paciente se ha observado incongruencias de la cadera o cambios degenerativos tempranos, indicando un excelente pronóstico. Muchos autores piensan que es una variante de la osificación normal de la cabeza femoral.

Prognostic Value of Modified Lateral Pillar Classification in Legg-Calve-Perthes Disease

BACKGROUND: To evaluate the usefulness of the modified lateral pillar classification as a prognostic factor in Legg-Calve-Perthes disease (LCPD). METHODS: Thirty nine patients diagnosed with lateral pillar C in LCPD from May, 1977, to October, 2001 were reviewed, and their skeletal maturity was followed. The mean follow up duration was 12 years and 7 months (4 years, 6 months to 24 years, 9 months). Lateral pillar C classification was divided into C1 (50-75% collapse of the lateral pillar) and C2 (> 75%). All radiological and clinical prognostic factors were evaluated. The final results were evaluated according to the Stulberg classification. RESULTS: Twenty one and 18 of the affected hips were in groups C1 and C2, respectively. According to the Stulberg classification, the final results of group C1 were better than those of C2 (p = 0.002). Patients with more head-at-risk signs had significantly poorer outcomes. CONCLUSIONS: The modified lateral pillar classification has significant value for predicting the prognosis of LCPD.

Claudicação na criança / Limping in children

A claudicação na criança é uma queixa frequente nos consultórios dos pediatras, dos ortopedistas pediátricos e nos pronto-socorros. Diversas são as causas para essa condição e identificá-las torna-se um desafio. Quanto maior a idade do paciente, melhor é a coleta da anamnese e mais completo se torna o exame físico, facilitando o raciocínio médico na busca da origem do distúrbio. Para facilitar a abordagem, três grupos etários podem e devem ser considerados. No grupo etário infantil, de um aos três anos de idade, os diagnósticos mais prováveis incluem: sinovite transitória, artrite séptica, desordens neurológicas (paralisia cerebral (PC) branda e distrofia muscular), displasia do desenvolvimento do quadril (DDQ), coxa vara, artrite reumatoide juvenil (ARJ) e neoplasias (osteoma osteoide, leucemia); no grupo etário escolar, dos quatro aos 10 anos de idade, além dos diagnósticos acima, doença de Legg-Calvé-Perthes, menisco discoide, discrepâncias de comprimento dos membros inferiores e "dor do crescimento"; nos adolescentes, dos 11 aos 15 anos de idade: epifisiólise, DDQ, condrólise, síndromes de overuse, osteocondrite dissecante e coalizão tarsal. A proposta deste trabalho é atualizar o leitor em relação à abordagem do paciente pediátrico que apresenta claudicação e discutir as suas prováveis causas.

Limping in children is a common complaint at pediatric, pediatric orthopaedic offices and in emergency rooms. There are several causes for this condition, and identifying them is a challenge. The older the patient, the better the anamnesis and more detailed the physical examination will be, enabling an easier medical assessment for searching the source of the disorder. In order to make the approach easier, three age groups can and should be considered. Among infants (1 to 3 years old), diagnosis will most likely be: transitory synovitis, septic arthritis, neurological disorders (mild brain palsy (BP) and muscular dystrophy), congenital hip dislocation (CHD), varus thigh, juvenile rheumatoid arthritis (JRA) and neoplasias (osteoid osteoma, leukemia); in the scholar age group, between 4 and 10 years old, in addition to the diagnoses above, Legg-Calvé-Perthes disease, discoid meniscus, inferior limbs discrepancy and unspecific muscular pain; in adolescents (11 to 15 years old): slipped capital femoral epiphysis, congenital hip dislocation, chondrolysis, overuse syndromes, dissecans osteochondritis, and tarsal coalition. The purpose of this study is to provide an update on how to approach pediatric patients presenting with limping, and to discuss its potential causes.

Fator V de Leiden na doença de Legg-Calvé-Perthes / Leiden's V-factor in Legg-Calvé-Perthes disease

Trobofilias hereditárias têm sido implicadas na patogênese da doenca de Legg-Calvé-Perthes. Uma investigação do fator de risco hereditário mais comum para hipercoagulabilidade - a mutação no gene do fator V (fator V de Leiden) - foi conduzida em 20 pacientes com Legg-Calvé-Perthes e 214 controles sadios. A prevalência do fator V de Leiden foi maior nos pacientes com Legg-Calvé-Perthes que no grupo controle (30 vs. 1,87 por cento). A razão de chances (odds ratio) para o desenvolvimento de Legg-Calvé-Perthes foi de 22,5 (p<0,05; intervalo de confiança: 5,68- 89.07). Estes dados sugerem, o fator V de Leiden como fator de risco hereditário para hipercoagulabilidade associada ao desenvolvimento da doença de Legg-Calvé-Perthes.

Inherited tendency to hypercoagulability has been suggested as a cause of vascular thrombosis resulting in Legg-Calvé-Perthes disease. An investigation of the most common inherited risk factor for hypercoagulability - the mutation in the V-factor gene (Leiden's V-factor) - was carried out among 20 Patients diagnosed with Legg- Calvé-Perthes disease. Patients were compared with 214 healthy controls. The prevalence of the Leiden's V-factor was higher in patients with Legg-Calvé-Perthes disease than in controls (30 percent vs. 1,87 percent). The odds ratio for the development of Legg-Calvé-Perthes disease in the presence of the Leiden's V-factor mutation was 22,5 (p<0,05; confidence interval: 5,68-89.07). These data suggest the Leiden's V-factor as an inherited risk factor for hypercoagulability associated with the development of Legg-Calvé-Perthes disease.

Efficacy of Gadolinium Enhanced MR Imaging for the Diagnosis of Legg-Calve-Perthes Disease

PURPOSE: The purpose of this study was to evaluate the efficacy of gadolinium enhanced MR imaging for making the diagnosis of Legg-Calve-Perthes (LCP) disease. MATERIALS AND METHODS: We studied the gadolinium enhanced MR images of 14 hips in 12 children who had the diagnosis of LCP disease. We retrospectively analyzed the extent of necrosis, the epiphyseal revascularization pathways and the metaphyseal changes. RESULTS: The absence of enhancement on gadolinium enhanced MRI was noted in all cases of LCP disease. Diffuse absence of enhancement was observed in 9 femoral epiphyses. Two of them showed normal bone marrow signal intensity on the T1 and T2-weighted images. Focal absence of enhancement was observed in 5 femoral epiphyses. Enhanced MRI showed better epiphyseal revascularization in the lateral column (five cases), in the lateral and medial columns (four cases) and in the transphyseal pathway (three cases). Metaphyseal change was observed in two cases. CONCLUSION: Gadolinium enhanced MRI allows detection of LCP disease and an accurate analysis of the different revascularization patterns, and this helpful for predicting the prognosis.

Long-term outcome of Legg-Perthes disease after Salter innominate osteotomy / 中国矫形外科杂志

[Objective]To observe radiographic morphology of femoral head at skeletal maturity of the Salter innominate osteotomy for treatment of patients with Legg-Perthes disease and evaluate sphericity of the femoral head and congruity of the hip.Based on system review of literatures,thes attempted to determine the indications and methods of treatment.[Method]Sixteen patients of Legg-Perthes disease were treated by Salter innominate osteotomy before 1994.The mean age of patients was six years and seven months at operation.Of them,only were 12 patients followed up 10 years and ten months avergely and mean ages of patients were 17 years and 2 months at last follow-up.Growth of plate of femoral head and Y cartilage of acetabulum was closed,indicating skeletal maturity.[Result]According to Stulberg classification of outcome,there were four cases in Stulberg class Ⅰ and six patient in class Ⅱ and one patient in class Ⅲ and one patients in class Ⅳ and no case in class Ⅴ.Lower limb of shortening was averagely 1.5 cm(ranged 0.8~2.0 cm)and the range of motion of hips was almost normal and no abnormal gait was observed.[Conclusion]The Salter innominate osteotomy could keep sphericity of the femoral head and congruity with acetabulum of the hip for children with Legg-Perthes disease and also could be in aid of preventing premature arthritis of hip.

Uso de la oxigenación hiperbárica en la enfermedad de Perthes / Use of the hyperbaric oxygenation in Perthes disease

Se realizó un estudio longitudinal prospectivo a 39 niños diagnosticados de la enfermedad de Perthes en el servicio de oxigenación hiperbárica del Hospital Manuel Ascunce Domenech de la ciudad de Camagüey, en el período comprendido entre diciembre 1995-1998, con el objetivo de valorar la eficacia de la terapéutica con oxígeno hiperbárico en esta afección. Este se administró a dos atmósferas absolutas durante 50 minutos de isopresión en cámaras monoplazas OKA-MT soviéticas. Se dieron un total de 35 sesiones en tres ciclos: el primero de 15 sesiones, el segundo de 10 sesiones y el tercero de 10 también, con un intervalo de 45 días cada uno y seis meses de duración total. Se compararon los exámenes clínicos y gammagráficos antes y después de su aplicación. Aquellos casos que permanecieron sin curar fueron reevaluados a los 10-12 meses, se procesaron los datos a través del utilitario estadístico Microstat. Los parámetros analizados se normalizaron significativamente una vez concluida la investigación.

A longitudinal, prospective study was performed in the service hy hyperbaric oxygenation of "Manuel Ascunce Domenech" Prov. Hospital od Camagüey City whitin the period from december 1995 to 1998 in 39 children diagnosed with Perthes disease with the aim of evaluate the efecctiveness of the terapeutic with hyperbaric oxygen , this was administred to 2 absolute atmospheres during 50 minutes of isprossure in soviet single seaters OKA_MT. A total of 35 sessions, second 10 sessions in the three cicles: first 15 sessions, second 10 sesssions and third 10 sessions with and interval of 45 days each and 16 months of total duration. Clinical and gammagraphic examinations were compared before and after its application. Those cases that remained without cure were reevaluated at 10 to 12 months, data were procesed through the estatistic data base MICROSTAT. Parameters analyzed werenormaly standarized after concluding the investigation.

The long-term results of intertrochanteric varus medial-displacement osteotomy for Perthes disease / 中华骨科杂志

Objective To observe the long term results of intertrochanteric varus medial displacement osteotomy(IVMDO) for Perthes disease. Methods Thirty eight patients with Perthes disease treated with IVMDO were reviewed. The results were evaluated based on a criteria made by the authors including clinical and radiographic parameters. The duration of follow up ranged from 3 to 15 years, with an average of 7 years. Results Fifteen patients were evaluated as having excellent result, 17 good, 3 fair and 3 poor respectively. The overall excellent or good rate was 84.2%. Considering the relationship between the outcome and staging of the disease, the overall excellent or good rate was 94.7% in stage Ⅱ lesion, 85.7%in stage Ⅲ lesion, and 40.0% in stage Ⅳ lesion. Conclusion The treatment of Perthes disease with IVMDO has the advantages of simple manipulation, less trauma and good results, and is worthy of populariztion.

Long-term follow-up results of Perthes disease in older children treated with surgical containment methods / 中华骨科杂志

Objective To evaluate the long-term results of Chiari osteotomy in the treatment of Legg-Calv?-Perthes disease for the patients of 8 years old or above and discuss the factors that influence the prognosis of this disease. Methods There were 116 hips of 103 patients of Perthes disease treated with Chiari osteotomy between June 1986 and December 2002. All patients were 8 years old or above. 72 hips of these cases acquired follow-up, the meaning follow-up duration was 97 months(range, 38-196 months). According to lateral pillar classification, 30 hips were classified as group B, 22 hips as B/C group, 20 hips as C group. There were 50 hips in male, 22 hips in female, 47 patients between 8 and 10 years old, 22 cases between 10 and 14 years old. X-ray showed 25 hips with subluxation of femoral head preoperatively. Results According to the revised Stulberg classification system: (1)31 hips had Stulberg classⅠ,Ⅱ outcome, in which B group accounted for 19 of 30 hips, the hips in group C had the worst results: of 20 hips, only 3 had a Stulberg class Ⅰ or Ⅱ outcome, the results for the hips in lateral B/C border group were intermediate between those groups B and C. 33 hips had Stulberg class Ⅲ, in which B group had 10 hips, B/C group had 11 hips, C group had 12 hips. 8 hips had Stulberg class Ⅳ or Ⅴ, B group had 1 hip, B/C group had 2 hips, C group had 5 hips. (2)All cases had a satisfied containment, no one had subluxation after Chiari osteotomy. (3)48% of male patients had a Stulberg class Ⅰor Ⅱ outcome, in contrast, only 23% of female had the comparable outcome. (4)55% of the group of 8 to 10 years old patients had a Stulberg class Ⅰ or Ⅱ outcome, in comparison with 17% from the group of 10 to 14 years old cases. Conclusion The patients older than 8 years at the onset of disease and in the lateral pillar B or C group should be treated with surgical containment, Chiari osteotomy can fulfill such task. The gender, the age of onset, especially the lateral pillar classification strongly correlate with the outcome of Perthes disease, and the latter can help us to evaluate the severity degree of the disease and the prognosis.

Costo del tratamiento quirúrgico de la enfermedad de Perthes / Cost of the surgical treatment of Perthes' disease / Coût du traitement chirurgical de la maladie de Perthes

Se estudiaron 10 pacientes con la enfermedad de Perthes, intervenidos quirúrgicamente mediante el sistema AO, para comprobar cuánto cuesta el tratamiento de esta afección. Luego del análisis efectuado para determinar los gastos de los diferentes centros de costos que intervienen en este proceso, se arribó a conclusiones. Así, se logró conocer y planificar los recursos necesarios y modificar incluso algunos esquemas terapéuticos que encarecían este tipo de cirugía.

10 patients suffering from Perthes disease and who were operated on by the AO system were studied to check how much the treatment of this affection costs. Conclusions were reached after having made the analysis to determine the expenses of the different cost centers taking part in this procedure. Thus, it was possible to know and to plan the necessary resources and to modify even some therapeutic schemes making this surgery more expensive.

On a étudié 10 patients qui avaient la maladie de Perthes, opérés par le système AO, afin de vérifier quel est le coût du traitement de cette affection. Après l'analyse pour déterminer les dépenses des différents centres de coût intervennant dans ce processus on a fait des conclusions. On est arrivé a connaître et planifier les ressources nécessaires et modifier, même, certains schémas thérapeutiques qui faisaient élever les prix de ce type de chirurgie.

Legg-Perthes Disease Associated with Cornelia de Lange Syndrome: A Case Report / 대한정형외과학회잡지

The Cornelia de Lange syndrome is characterized by severe growth and mental retardation; typical facies; low-pitched, weak, growling cry, and various bone abnormalities, as was first described by Cornelia de Lange in 1933. On the other hand, Legg-Perthes disease is the condition, in which avascular necrosis of the femoral head develops, as was first described by Legg, Calve and Perthes in 1910. Review of the worlds literature disclosed only one case of Legg-Perthes disease associated with Cornelia de Lange syndrome. We experienced a case of Legg-Perthes disease associated with Cornelia de Lange syndrome in a 8 years old boy, which was treated surgically for Legg-Perthes disease. A brief review of literature was made.

Plasma Somatomedin in Children with Perthes' Disease / 대한정형외과학회잡지

No abstract available in English.