Chronic nonbacterial osteomyelitis in childhood: five ... · Chronic nonbacterial osteomyelitis in...
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Chronic nonbacterial osteomyelitis in childhood: five years’ experience of imaging at a tertiary hospital Mare Lintrop 1,2 Jaanika Ilisson 3 , Chris Pruunsild 3, 4 1 Radiology Clinic, Tartu University Hospital; 2 Radiology Clinic, Institute of Clinical Medicine, Faculty of Medicine, University of Tartu; 3 Children's Clinic, Tartu University Hospital; 4 Children’s Clinic, Institute of Clinical Medicine, Faculty of Medicine, University of Tartu Key words Chronic nonbacterial osteomyelitis, CNO, chronic recurrent multifocal osteomyelitis, CRMO, whole body MR, WBMR Conclusions Ø In the recent years CNO has been more recognized and diagnosed in our institution and mean time to the CNO diagnosis has decreased. Ø Symmetricity, multifocality and particularly specific patterns of lesions appear suggestive to CNO. Ø For imaging of CNO MRI should be preferred instead of CT and nuclear imaging. Ø WBMR is a valuable and radiation-free imaging modality of choice in patients with clinical multifocal symptoms and in detection of subclinical lesions. Objective Ø Chronic nonbacterial osteomyelitis (CNO) is a relatively rare autoinflammatory bone disease of unknown etiology characterized by sterile bone inflammation. Ø Chronic recurrent multifocal osteomyelitis (CRMO) is the most severe form of CNO. Ø CNO mainly occurs in children and adolescents, predominantly affects the metaphyses of the long bones, but single or multiple lesions can occur at any site of the skeleton. Ø Delays in diagnosis may lead to prolonged courses of antibiotics, unnecessary radiation exposure from multiple radiographs or bone scans and repeated surgery including bone biopsies. Ø The aim of the study was to determine patient characteristics, clinical presentation, pattern of bone involvement and imaging strategies of patients with CNO. Methods Ø We did retrospective analysis of the electronic medical records of children diagnosed with CNO at the Tartu University Hospital Childrens’ Clinic and department of Maxillofacial Surgery from 2012 to 2017. Ø The complete record of each patient — including demographic, clinical, laboratory and histological data — was reviewed. Ø All performed images were analysed for the presence and number of bone lesions. Ø To classify patients the CNO clinical score proposed by Jansson et al. in 2009 was applied. Results Ø Patients characteristics (Table 1). A total of nine children, five boys and four girls, with CNO/CRMO were enrolled in the study. The median age at the onset of first symptoms was 11 ± 3.9 (range 5.3-17.1) years and the median age at diagnosis was 12.4 (range 5.5 – 17.5) years. The median delay to the CNO diagnosis was 5 (range 1-17) months. There was not significant difference in the median diagnostic delay between multifocal and unifocal involvement groups. The median time to diagnosis of the first two patients diagnosed in 2012 was 15 months versus 3.9 months in other seven patients diagnosed in later years what confirms improvement in the diagnostics skills and knowledge. 56% of patients with unifocal disease and/or mandibular involvement were biopsied and in all cases biopsy confirmed the diagnosis of CNO. Ø Clinical presentation. All patients complained about pain, six had localized swelling and only one presented with fever. One child had associated Crohn’s disease, none of the patients had pustulosis. Five patients presented with clinically unifocal disease but two of them had radiologically more than one lesion. Median CNO clinical score at the diagnosis was 47 ± 10. Ø Skeletal involvement according to MRI and radiographs is summarized in the Table 2. Ø Imaging strategies. Although in some cases also CT, bone scintigraphy and PET-CT was done, in this study we reviewed only radiographs which are usually the first radiological assessment tool in everyday practice, and targeted MRI and WBMR images. For follow-up of patients with multifocal CNO whole body MR was used. Table 2. Skeletal involvement according to MRI and radiographs. Altogether 41 bony lesions were identified with MRI. Lesions were located in the spine (19), mandibula (5), tarsals (5), tibias (3), phalanges (4), femur (2), fibulas (2), metacarpal and rib. 14 lesions could not be detected on radiographs. 7 subclinical sites were diagnosed by whole body imaging. Chronic recurrent multifocal osteomyelitis (CRMO) in an 6-year-old girl with a 10-month history of ankle pain. 1 – AP radiographs of the ankles show multiple lytic lesions with surrounding sclerosis in the metaphyses of the tibias (asterisks) and right fibula. 2 - A fragment from whole body MR (WBMR) STIR sequence image demonstrates high signal intensity lesions in distal tibial metaphyses. Similar lesions in the right distal femoral and left proximal tibial metaphyses, as well as in distal tibial epiphyses and in right talus were not clearly detectable on radiographs. Chronic recurrent multifocal osteomyelitis (CRMO) in an 6-year-old girl. 1 - AP chest radiograph shows expansion and irregular sclerosis in the anterior shaft of the left 9th rib (asterisk). 2 – A fragment from whole body MR (WBMR) STIR sequence image shows high signal lesion in the 9th left rib (#) Chronic nonbacterial osteomyelitis in a 7-year old boy with a 4-month history of right mandibular pain and swelling. 1-3 - Expansion and edema of right mandibular body, mandibular angle and surrounding soft tissues (T2 hyperintense (1), T1 hypointense (2) with Gd enhancement (3)) with marked periosteal reaction is seen. 4-5 - Follow-up MRI T2/T1 STIR images eight months later show positive dynamics. Table 1. Characteristics of patients.
Transcript of Chronic nonbacterial osteomyelitis in childhood: five ... · Chronic nonbacterial osteomyelitis in...
Chronic nonbacterial osteomyelitis in childhood:five years’ experience of imaging at a tertiary hospital
Mare Lintrop1,2 Jaanika Ilisson3, Chris Pruunsild3, 4
1Radiology Clinic, Tartu University Hospital; 2Radiology Clinic, Institute of Clinical Medicine, Faculty of Medicine, University of Tartu; 3Children's Clinic, Tartu University Hospital; 4 Children’s Clinic, Institute of Clinical Medicine, Faculty of Medicine, University of Tartu
Key wordsChronic nonbacterial osteomyelitis, CNO, chronic recurrent multifocal osteomyelitis, CRMO, whole body MR, WBMR
ConclusionsØ In the recent years CNO has been more
recognized and diagnosed in our institution and mean time to the CNO diagnosis has decreased.
Ø Symmetricity, multifocality and particularly specific patterns of lesions appear suggestive to CNO.
Ø For imaging of CNO MRI should be preferred instead of CT and nuclear imaging.
Ø WBMR is a valuable and radiation-free imaging modality of choice in patients with clinical multifocal symptoms and in detection of subclinical lesions.
ObjectiveØ Chronic nonbacterial osteomyelitis (CNO) is a
relatively rare autoinflammatory bone disease of unknown etiology characterized by sterile bone inflammation.
Ø Chronic recurrent multifocal osteomyelitis (CRMO) is the most severe form of CNO.
Ø CNO mainly occurs in children and adolescents, predominantly affects the metaphyses of the long bones, but single or multiple lesions can occur at any site of the skeleton.
Ø Delays in diagnosis may lead to prolonged courses of antibiotics, unnecessary radiation exposure from multiple radiographs or bone scans and repeated surgery including bone biopsies.
Ø The aim of the study was to determine patient characteristics, clinical presentation, pattern of bone involvement and imaging strategies of patients with CNO.
MethodsØ We did retrospective analysis of the electronic medical records of children diagnosed with CNO at the Tartu University
Hospital Childrens’ Clinic and department of Maxillofacial Surgery from 2012 to 2017.
Ø The complete record of each patient — including demographic, clinical, laboratory and histological data — was reviewed.
Ø All performed images were analysed for the presence and number of bone lesions.
Ø To classify patients the CNO clinical score proposed by Jansson et al. in 2009 was applied.
ResultsØ Patients characteristics (Table 1).
A total of nine children, five boys and four girls, with CNO/CRMO were enrolled in the study.The median age at the onset of first symptoms was 11 ± 3.9 (range 5.3-17.1) years and the median age at diagnosis was 12.4(range 5.5 – 17.5) years. The median delay to the CNO diagnosis was 5 (range 1-17) months.There was not significant difference in the median diagnostic delay between multifocal and unifocal involvement groups. The median time to diagnosis of the first two patients diagnosed in 2012 was 15 months versus 3.9 months in other seven patientsdiagnosed in later years what confirms improvement in the diagnostics skills and knowledge.56% of patients with unifocal disease and/or mandibular involvement were biopsied and in all cases biopsy confirmed thediagnosis of CNO.
Ø Clinical presentation.All patients complained about pain, six had localized swelling and only one presented with fever. One child had associated Crohn’sdisease, none of the patients had pustulosis. Five patients presented with clinically unifocal disease but two of them had radiologicallymore than one lesion. Median CNO clinical score at the diagnosis was 47 ± 10.
Ø Skeletal involvement according to MRI and radiographs is summarized in the Table 2.Ø Imaging strategies. Although in some cases also CT, bone scintigraphy and PET-CT was done, in this study we reviewed
only radiographs which are usually the first radiological assessment tool in everyday practice, and targeted MRI and WBMR images.For follow-up of patients with multifocal CNO whole body MR was used.
Table 2. Skeletal involvement according to MRI and radiographs.
Altogether 41 bony lesions were identified with MRI.Lesions were located in the spine (19), mandibula (5), tarsals (5), tibias (3), phalanges (4), femur (2), fibulas (2), metacarpal and rib.14 lesions could not be detected on radiographs.7 subclinical sites were diagnosed by whole body imaging.
Chronic recurrent multifocal osteomyelitis (CRMO) in an 6-year-old girl with a 10-month history of ankle pain.1 – AP radiographs of the ankles show multiple lytic lesions with surrounding sclerosis in the metaphyses of the tibias (asterisks) and right fibula.2 - A fragment from whole body MR (WBMR) STIR sequence image demonstrates high signal intensity lesions in distal tibial metaphyses. Similar lesions in the right distal femoral and left proximal tibialmetaphyses, as well as in distal tibial epiphyses and in right talus were not clearly detectable on radiographs.
Chronic recurrent multifocal osteomyelitis (CRMO) in an 6-year-old girl.1 - AP chest radiograph shows expansion and irregular sclerosis in the anterior shaft of the left 9th rib (asterisk).2 – A fragment from whole body MR (WBMR) STIR sequence image shows high signal lesion in the 9th left rib (#)
Chronic nonbacterial osteomyelitis in a 7-year old boy with a 4-month history of right mandibular pain and swelling.1-3 - Expansion and edema of right mandibular body, mandibular angle and surrounding soft tissues (T2 hyperintense (1), T1 hypointense (2) with Gd enhancement (3)) with marked periosteal reaction is seen.4-5 - Follow-up MRI T2/T1 STIR images eight months later show positivedynamics.
Table 1. Characteristics of patients.
