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Reliability of haemophilia early arthropathy detection with ultrasound (HEAD-US) in children: a comparative magnetic resonance imaging (MRI) study

Domen Plut

Domen Plut

  • Clinical Radiology Institute, University Medical Centre LjubljanaLjubljana, Slovenia
  • Faculty of Medicine, University of LjubljanaLjubljana, Slovenia
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Plut, Domen
, 
Barbara Faganel Kotnik

Barbara Faganel Kotnik

Department of Haematology and Oncology, Children’s Hospital, University Medical Centre LjubljanaLjubljana, Slovenia
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Kotnik, Barbara Faganel
, 
Luka Pusnik

Luka Pusnik

Faculty of Medicine, University of LjubljanaLjubljana, Slovenia
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Pusnik, Luka
, 
Peter Slak

Peter Slak

  • Clinical Radiology Institute, University Medical Centre LjubljanaLjubljana, Slovenia
  • Faculty of Medicine, University of LjubljanaLjubljana, Slovenia
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Slak, Peter
, 
Ziga Snoj

Ziga Snoj

  • Clinical Radiology Institute, University Medical Centre LjubljanaLjubljana, Slovenia
  • Faculty of Medicine, University of LjubljanaLjubljana, Slovenia
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Snoj, Ziga
 und 
Vladka Salapura

Vladka Salapura

  • Clinical Radiology Institute, University Medical Centre LjubljanaLjubljana, Slovenia
  • Faculty of Medicine, University of LjubljanaLjubljana, Slovenia
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Salapura, Vladka
  
20. Okt. 2022
Reliability of haemophilia early arthropathy detection with ultrasound (HEAD-US) in children: a comparative magnetic resonance imaging (MRI) study's Cover Image
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Artikel-Kategorie: Research Article
Online veröffentlicht: 20. Okt. 2022
Seitenbereich: 471 - 478
Eingereicht: 23. Aug. 2022
Akzeptiert: 04. Sept. 2022
DOI: https://doi.org/10.2478/raon-2022-0040
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© 2022 Domen Plut, Barbara Faganel Kotnik, Luka Pusnik, Peter Slak, Ziga Snoj, Vladka Salapura, published by Sciendo
This work is licensed under the Creative Commons Attribution 4.0 International License.

Figure 1

Concordance plot for depicting agreement between US and MRI scores for all three joints. Equal size of the fields denotes perfect agreement. The plots demonstrate overall an excellent agreement between the methods. It can also be observed, that in most cases of discordance, US slightly undervalued the progression of the joint disease.
Concordance plot for depicting agreement between US and MRI scores for all three joints. Equal size of the fields denotes perfect agreement. The plots demonstrate overall an excellent agreement between the methods. It can also be observed, that in most cases of discordance, US slightly undervalued the progression of the joint disease.

Figure 2

Anterior transverse US images over the distal humeral epiphysis in a 7-years and 16-years old healthy boys. A wavy osteochondral surface consisting of the convex capitellum and the concave trochlea is shown. Note the age-dependent anatomic differences: subchondral bony surface in the younger child (A) shows physiological irregularities (thick arrow); the articular cartilage, which appears as a uniform hypoechoic band overlying the subchondral bone (thin arrows), is thinner in the older child (B).
Anterior transverse US images over the distal humeral epiphysis in a 7-years and 16-years old healthy boys. A wavy osteochondral surface consisting of the convex capitellum and the concave trochlea is shown. Note the age-dependent anatomic differences: subchondral bony surface in the younger child (A) shows physiological irregularities (thick arrow); the articular cartilage, which appears as a uniform hypoechoic band overlying the subchondral bone (thin arrows), is thinner in the older child (B).

Figure 3

PD weighted MRI of ankles in sagittal plane. Image (A) shows an ankle with no signs of haemophilic arthropathy in an 11-years old boy, while image (B) shows a severely affected ankle in a 17-years old boy. The thin arrow marks a talar osteochondral defect, while the thick arrow marks synovial hypertrophy with hemosiderin deposition.
PD weighted MRI of ankles in sagittal plane. Image (A) shows an ankle with no signs of haemophilic arthropathy in an 11-years old boy, while image (B) shows a severely affected ankle in a 17-years old boy. The thin arrow marks a talar osteochondral defect, while the thick arrow marks synovial hypertrophy with hemosiderin deposition.

Figure 4

An example of good concordance between HEAD-US and MRI in a 7-years old child. US image of the femoral trochlea in the transverse plane is shown (A). T2* weighted MR image in the transverse plane (B) of the same knee is shown for comparison of the corresponding structures. The smooth bone surface and normal thickness trochlear joint cartilage with homogenous structure are shown (white arrow); the corresponding intact structures are shown on MR image. On MRI, there were also no additional arthropathic changes in the parts of the joint not visualized by the US. The images show a perfect concordance between US and MRI findings in this knee with no signs of haemophilic arthropathy.
An example of good concordance between HEAD-US and MRI in a 7-years old child. US image of the femoral trochlea in the transverse plane is shown (A). T2* weighted MR image in the transverse plane (B) of the same knee is shown for comparison of the corresponding structures. The smooth bone surface and normal thickness trochlear joint cartilage with homogenous structure are shown (white arrow); the corresponding intact structures are shown on MR image. On MRI, there were also no additional arthropathic changes in the parts of the joint not visualized by the US. The images show a perfect concordance between US and MRI findings in this knee with no signs of haemophilic arthropathy.

Figure 5

An example of a lesion causing a discordance between the US and MRI. A T2* weighted MR image of an ankle of a 16-years old boy in the sagittal plane is shown. A small subchondral cyst covered with intact cortical bone and articular cartilage (white arrow) is shown. MRI demonstrates a defect which cannot be visualized by US.
An example of a lesion causing a discordance between the US and MRI. A T2* weighted MR image of an ankle of a 16-years old boy in the sagittal plane is shown. A small subchondral cyst covered with intact cortical bone and articular cartilage (white arrow) is shown. MRI demonstrates a defect which cannot be visualized by US.

Descriptive statistics for US and MRI assessment scores

Joints Statistic US MRI
% of zeros 85 75
Elbows Median Mean 00.35 01.05
SD 0.93 2.82
% of zeros 100 100
Knees Median Mean
SD
% of zeros 65 70
Ankles Median Mean 00.8 01.6
SD 1.2 2.2
% of zeros 83.3 81.7
Overall Median Mean 00.38 00.88
SD 0.92 2.16

Characteristics of subjects included in the study

Age: mean; range (years) 11.5; 6–17
Age at the start of prophylaxis: mean; range (years) Primary prophylaxis – 5 patients: Secondary prophylaxis – 5 patients: 3; 0.8–6.6 2.2; 0.83.8; 2.5 –3.8 –6.6
Duration of prophylaxis: mean; range (years) 9.1; 3.2–14.7
Haemophilia Joint Health Score (HJHS): mean; range 0.9; 0–7
Number of previous joint bleeds per patient: mean; range 16.2; 0–83
Number of previous joint bleeds per joint: mean; range 2.5; 0–71
Number of previous joint bleeds: Elbows Knees Ankles Overall
0 (number of joints) 14 7 9 30
1–4 (number of joints) 5 12 8 25
> 5 (number of joints) 1 1 3 5

Measures of diagnostic accuracy for detection of haemophilic arthropathy by US (HEAD-US) in comparison to MRI (IPSG MRI score) as the reference standard

Specificity 81.8%
Sensitivity 98%
Positive predictive value 90%
Negative predictive value 96%

The results of the correlation analysis

US vs MRI Pearson’s correlation coefficient (r)
Elbows Knees Ankles
Total score 0.849 1 0.842
Synovium 0.841 1 0.722
Cartilage 0.829 1 0.546
Bone 0.499 1 0.478
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