The External Genitalia Score (EGS): A European Multicenter Validation Study.

CONTEXT: Standardized description of external genitalia is needed in the assessment of children with atypical genitalia. OBJECTIVES: To validate the External Genitalia Score (EGS), to present reference values for preterm and term babies up to 24 months and correlate obtained scores with anogenital distances (AGDs). DESIGN, SETTING: A European multicenter (n = 8) validation study was conducted from July 2016 to July 2018. PATIENTS AND METHODS: EGS is based on the external masculinization score but uses a gradual scale from female to male (range, 0-12) and terminology appropriate for both sexes. The reliability of EGS and AGDs was determined by the interclass correlation coefficient (ICC). Cross-sectional data were obtained in 686 term babies (0-24 months) and 181 preterm babies, and 111 babies with atypical genitalia. RESULTS: The ICC of EGS in typical and atypical genitalia is excellent and good, respectively. Median EGS (10th to 90th centile) in males < 28 weeks gestation is 10 (8.6-11.5); in males 28-32 weeks 11.5 (9.2-12); in males 33-36 weeks 11.5 (10.5-12) and in full-term males 12 (10.5-12). In all female babies, EGS is 0 (0-0). The mean (SD) lower/upper AGD ratio (AGDl/u) is 0.45 (0.1), with significant difference between AGDl/u in males 0.49 (0.1) and females 0.39 (0.1) and intermediate values in differences of sex development (DSDs) 0.43 (0.1). The AGDl/u correlates with EGS in males with typical genitalia and in atypical genitalia. CONCLUSIONS: EGS is a reliable and valid tool to describe external genitalia in premature and term babies up to 24 months. EGS correlates with AGDl/u in males. It facilitates standardized assessment, clinical decision-making and multicenter research.

Delayed gadolinium-enhanced MRI of cartilage (dGEMRIC) and morphologic MRI of cartilage in the long-term follow-up after Legg-Calvé-Perthes disease (LCPD).

INTRODUCTION: The purpose of the present study was to evaluate the feasibility of delayed gadolinium-enhanced magnetic resonance imaging of cartilage (dGEMRIC) in the detection of cartilage changes versus morphologic imaging in the long-term course of Legg-Calvé-Perthes disease (LCPD). METHODS: A total of 31 hips in 26 patients (mean age, 30.0years; range, 18-54years) who were diagnosed with LCPD in childhood were included. Twenty-one radiographically normal contralateral hips served as controls. dGEMRIC indices of femoral and acetabular cartilage in the weight-bearing zone. Cartilage morphology was classified on radial PD-weighted images according to the modified Outerbridge classification. RESULTS: Mean dGEMRIC values of cartilage were significantly lower in hips after LCPD than in the radiographically normal contralateral hips (513±100 ms vs. 579±103 ms; P=0.026). In 24 out of 31 LCPD hips and in 4 out of 21 radiographically normal contralateral hips, morphological cartilage changes were noted. Analysis of variance analysis revealed a significant influence of Outerbridge grading on decreased T1-values (P=0.031). CONCLUSION: Our results suggest that dGEMRIC at 1.5 T is suitable to assess cartilage quality changes in the long-term follow-up after LCPD. The evaluation of biochemical cartilage quality with dGEMRIC may provide additional information about early cartilage changes occurring without visible alterations of cartilage morphology.

Delayed gadolinium-enhanced magnetic resonance imaging of cartilage in the long-term follow-up after Perthes disease.

BACKGROUND: Aim of this study was to assess the glycosaminoglycan content in hip joint cartilage in mature hips with a history of Legg-Calvé-Perthes (LCPD) disease using delayed gadolinium-enhanced magnetic resonance imaging of cartilage (dGEMRIC). METHODS: Thirty one hips in 27 adults (mean age: 30.5+/-10.9 y) with LCPD in childhood were included. Mean follow-up after diagnosis was 24.5+/-11.4 years. Clinical symptoms and standard radiographic parameters were evaluated. dGEMRIC indices were calculated as T1(Gd) mean values in four coronal MRI slices medially, centrally, and laterally. For comparison, the morphologically normal appearing contra-lateral hips (21 hips) were assessed. RESULTS: The following T1(Gd) values for the LCPD group were noted: medial (507+/-100 ms), central (543+/-104 ms), and lateral (553+/-105 ms). The total T1(Gd) mean value was 534+/-104 ms. The difference between LCPD and normal hips was statistically significant only for the medial compartment (P=0.018). CONCLUSIONS: Hip joint cartilage after LCPD shows a significant glycosaminoglycan loss in the medial compartment while this decrease is less apparent centrally and laterally. dGEMRIC allows direct assessment of cartilage matrix biochemistry and may depict the complex damage pattern of hip joint cartilage after LCPD spatially and qualitatively better than other radiographic methods. LEVEL OF EVIDENCE: Prognostic study, Level II-1 (retrospective study).

Reproducibility of dGEMRIC in assessment of hip joint cartilage: a prospective study.

PURPOSE: To investigate the reproducibility of dGEMRIC in the assessment of cartilage health of the adult asymptomatic hip joint. MATERIALS AND METHODS: Fifteen asymptomatic volunteers (mean age, 26.3 years +/- 3.0) were preliminarily studied. Any volunteer that was incidentally diagnosed with damaged cartilage on MRI (n = 5) was excluded. Ten patients that had no evidence of prior cartilage damage (mean age, 26.2 years +/- 3.4) were evaluated further in this study. The reproducibility of dGEMRIC was assessed with two T1(Gd) exams performed 4 weeks apart in these volunteers. The protocol involved an initial standard MRI to confirm healthy cartilage, which was then followed by dGEMRIC. The second scan included only the repeat dGEMRIC. Region of interest (ROI) analyses for T1(Gd)-measurement was performed in seven radial reformats. Statistical analysis included the student's t-test and intra-class correlation (ICC) measurement to assess reproducibility. RESULTS: Overall 70 ROIs were studied. Mean cartilage T1(Gd) values at various loci ranged from 560.9 ms to 684.4 ms at the first set of readings and 551.5 ms to 662.2 ms in the second one. The mean difference per region of interest between the two T1(Gd)-measurements ranged from 21.4 ms (3.7%) to 45.0 ms (6.8%), which was not found to be statistically significant (P = 0.153). There was a high reproducibility detected (ICC range, 0.667-0.915). Intra- and Inter-observer analyses proved a high agreement for T1(Gd) assessment (0.973 and 0.932). CONCLUSION: We found dGEMRIC to be a reliable tool in the assessment of cartilage health status in adult hip joints.

Clinical presentation of young adults after Legg-Calvé-Perthes disease.

The purpose of the present study was to review the clinical and radiological outcome of young adult individuals who had Legg-Calvé-Perthes disease (LCPD) during child age. After a mean follow-up of 18.7 +/- 6.2 years, 25 young adults with various morphological deformity grades were assessed clinically and radiologically. Deterioration of hip joint function and onset of pain were found to manifest early in the intermediate follow-up of LCPD and to correlate with the residual hip deformity.