Introduction Pure androgen-secreting adrenocortical tumors are a rare but important cause of peri... more Introduction Pure androgen-secreting adrenocortical tumors are a rare but important cause of peripheral precocious puberty. Case presentation Here, we report a pure androgen-secreting adrenocortical tumor in a 2.5-year-old boy presenting with penile enlargement, pubic hair, frequent erections, and rapid linear growth. We confirmed the diagnosis through laboratory tests, medical imaging, and histology. Furthermore, genetic testing detected a pathogenic germline variant in the TP53 gene, molecularly confirming underlying Li–Fraumeni syndrome. Discussion Only 15 well-documented cases of pure androgen-secreting adrenocortical tumors have been reported so far. No clinical or imaging signs were identified to differentiate adenomas from carcinomas, and no other cases of Li–Fraumeni syndrome were diagnosed in the four patients that underwent genetic testing. However, diagnosing Li–Fraumeni syndrome is important as it implies a need for intensive tumor surveillance and avoidance of ionizing ...
Traumatic pseudoaneurysms of the superficial temporal artery (STA) are uncommon. The appearance o... more Traumatic pseudoaneurysms of the superficial temporal artery (STA) are uncommon. The appearance of a pulsatile preauricular mass after a traumatism is highly suggestive of the diagnosis. Diagnostic techniques are echo-doppler, CT-scan and arteriography. The most common treatment is surgical. Embolization may prove to be an alternative to surgery in some cases. We report a rare case of pseudoaneurysm arising from STA caused by blunt injury.
Numerous issues must be addressed when developing standard operating procedures for clinical rese... more Numerous issues must be addressed when developing standard operating procedures for clinical research studies involving chest computed tomography of lung disease in patients with cystic fibrosis (CF). Study success depends on the provision of adequate funding and the identification of personnel with the necessary expertise to conduct the study, along with clear guidelines that detail the CT operating procedure at each site, including breathing maneuvers, and image reconstruction. Close coordination of the quality assurance process between sites and the central review organization is required to maintain protocol adherence. The data transfer process must ensure the integrity and security of the data to comply with patient privacy regulations, and study outcome measures are best assessed with a scoring system or other structured method of imaging data analysis. The recommendations provided are designed to serve as a valuable reference guide for planning clinical research studies of patients with CF involving chest CT.
In adult patients with cystic fibrosis (CF), the lung clearance index (LCI) derived from the mult... more In adult patients with cystic fibrosis (CF), the lung clearance index (LCI) derived from the multiple breath washout relates to both acinar and conductive ventilation heterogeneity. The latter component predicts an association between LCI and the number of bronchial segments affected by bronchiectasis. Here, we experimentally demonstrated this association in patients with CF, and also examined an ancillary group of patients with non-CF bronchiectasis. We conclude that lung disease severity in terms of number of bronchial segments results in an associated LCI increase, likely constituting a portion of LCI that cannot be reversed by treatment in patients with CF lung disease.
Introduction: Aspergillus fumigatus (Af) causes various pulmonary entities ranging from aspergill... more Introduction: Aspergillus fumigatus (Af) causes various pulmonary entities ranging from aspergilloma in lung cavities (1), allergic or hypersensitive reactions (ABPA) (2), chronic necrotizing aspergillosis in chronic lung disease (3) to invasive infection in immunocompromised hosts (4). ABPA is reported in 2 15% of adult CF patients, fulfilling 4 minimal criteria. Patients: In the past 6 months, we had 2 young CF patients, a 9 year old male and a 12 year old girl with PA, and overlapping criteria of entity 2 and 3. Results: the boy had normal lungfunction, total IgE 2330 KIU/1, and elevated Afspecific IgE and IgG, a large peripheral infiltrate, with eosinophilic granulomatous reaction and fungal hyphy on transthoracic needle biopsy (sputa were negative). The girl has an FEV1 60% (without asthma symptoms), normal IgE, hemoptysis and repeated positive sputum cultures for Af, being the only pathogen cultured, including bacteria and also a large peripheral infiltrate. Both responded very well to voriconazole with disappearance of the infiltrates. Oral steroids given to the first patient resulted in steroid related diabetes. Conclusions: PA is also seen in young CF patients, ABPA criteria are not always fulfilled and clinical entities of PA overlap. Diagnosis may be difficult and the causal role of Af in lung infiltrates is difficult to asses (needing biopsy in case 1). Treatment guidelines are not uniform and the use of oral steroids includes risk for important side-effects.
Isolated tracheoesophageal fistula (H-TOF) is a rare type of tracheoesophageal anomaly and is in ... more Isolated tracheoesophageal fistula (H-TOF) is a rare type of tracheoesophageal anomaly and is in most cases diagnosed in the neonatal period because of choking and cyanosis during feeding. Diagnosis may be delayed even until adulthood because of nonspecific and sometimes intermittent symptoms, and because false-negative results of all diagnostic tools are not uncommon. We report a 10-year-old child with H-TOF, whose symptoms had nearly disappeared completely between the ages of 4 and 10 years. Diagnosis was only possible after the recurrence of the symptoms at the time of an episode of bronchitis, profound interrogation of the child's medical history, and questioning of the results of a former diagnostic work-up. In this article, we discuss the potential pitfalls in both clinical diagnosis and diagnostic work-up.
Purpose To optimize our standard full-spine radiography with respect to diagnostic quality and do... more Purpose To optimize our standard full-spine radiography with respect to diagnostic quality and dose. Methods A phantom study was performed to establish an optimal posterior-anterior view (PA) full spine protocol having the lowest dose with non-inferior quality compared to standard. We then applied this protocol in 40 pediatric patients (group B). The radiographs were scored on six criteria by a pediatric radiologist and orthopedist and compared to the scores of 40 PA full spine radiographs performed in 2013 with standard protocol (group A). Radiation dose was assessed by dose area product (DAP) and effective dose (E). Statistical analysis included independent samples t test, Mann-Whitney U test and intra-class correlation coefficient (ICC). Results An optimized protocol was defined (0.2 mm Cu filter, 0.87 relative exposure, with grid). Mean age was 13.3 ± 1.6 years for group A and 13.4 ± 1.7 years for group B. For group B, the mean DAP was 47.0 µGy m 2 with an E of 0.13 mSv. For group A, the mean DAP was 85.3 µGy m 2 with an E of 0.24 mSv. This represents a dose reduction of 45%. Mean image quality scores for group A (27.9 ± 2.4) and group B (28.1 ± 2.3) were similar (p = 0.612). Interobserver agreement was observed to be excellent (ICC 0.92). Conclusion This study demonstrates that a low-dose full-spine radiograph can be performed in patients with idiopathic scoliosis without loss of image quality. Graphical abstract These slides can be retrieved under Electronic Supplementary Material.
Poster: "ECR 2016 / C-0649 / An optimal protocol for full-spine radiography" by: "... more Poster: "ECR 2016 / C-0649 / An optimal protocol for full-spine radiography" by: "C. Ernst1, N. Buls1, A. Laumen1, E. ASSENOVA2, S. Assouti3, G. Van Gompel1, J. de Mey4; 1Brussels/BE, 2LEDE/BE, 3Antwerpen/BE, 4Brussel/BE"
JBR-BTR: organe de la Société royale belge de radiologie (SRBR) = orgaan van de Koninklijke Belgische Vereniging voor Radiologie (KBVR)
Symmetrical lesions in the basal ganglia are accepted as the characteristic radiological feature ... more Symmetrical lesions in the basal ganglia are accepted as the characteristic radiological feature of methanol toxicity. A case of chronic deliberate repeated methanol ingestion is presented. The typical MRI findings of basal ganglia lesions on T1 and T2 weighted images, FLAIR, diffusion and T1-weighted post Gadolinium images are presented. To our knowledge, this is the first case in which late diffusion and post contrast MRI images, showing necrotic sequelae due to methanol ingestion, are reported.
Introduction Pure androgen-secreting adrenocortical tumors are a rare but important cause of peri... more Introduction Pure androgen-secreting adrenocortical tumors are a rare but important cause of peripheral precocious puberty. Case presentation Here, we report a pure androgen-secreting adrenocortical tumor in a 2.5-year-old boy presenting with penile enlargement, pubic hair, frequent erections, and rapid linear growth. We confirmed the diagnosis through laboratory tests, medical imaging, and histology. Furthermore, genetic testing detected a pathogenic germline variant in the TP53 gene, molecularly confirming underlying Li–Fraumeni syndrome. Discussion Only 15 well-documented cases of pure androgen-secreting adrenocortical tumors have been reported so far. No clinical or imaging signs were identified to differentiate adenomas from carcinomas, and no other cases of Li–Fraumeni syndrome were diagnosed in the four patients that underwent genetic testing. However, diagnosing Li–Fraumeni syndrome is important as it implies a need for intensive tumor surveillance and avoidance of ionizing ...
Traumatic pseudoaneurysms of the superficial temporal artery (STA) are uncommon. The appearance o... more Traumatic pseudoaneurysms of the superficial temporal artery (STA) are uncommon. The appearance of a pulsatile preauricular mass after a traumatism is highly suggestive of the diagnosis. Diagnostic techniques are echo-doppler, CT-scan and arteriography. The most common treatment is surgical. Embolization may prove to be an alternative to surgery in some cases. We report a rare case of pseudoaneurysm arising from STA caused by blunt injury.
Numerous issues must be addressed when developing standard operating procedures for clinical rese... more Numerous issues must be addressed when developing standard operating procedures for clinical research studies involving chest computed tomography of lung disease in patients with cystic fibrosis (CF). Study success depends on the provision of adequate funding and the identification of personnel with the necessary expertise to conduct the study, along with clear guidelines that detail the CT operating procedure at each site, including breathing maneuvers, and image reconstruction. Close coordination of the quality assurance process between sites and the central review organization is required to maintain protocol adherence. The data transfer process must ensure the integrity and security of the data to comply with patient privacy regulations, and study outcome measures are best assessed with a scoring system or other structured method of imaging data analysis. The recommendations provided are designed to serve as a valuable reference guide for planning clinical research studies of patients with CF involving chest CT.
In adult patients with cystic fibrosis (CF), the lung clearance index (LCI) derived from the mult... more In adult patients with cystic fibrosis (CF), the lung clearance index (LCI) derived from the multiple breath washout relates to both acinar and conductive ventilation heterogeneity. The latter component predicts an association between LCI and the number of bronchial segments affected by bronchiectasis. Here, we experimentally demonstrated this association in patients with CF, and also examined an ancillary group of patients with non-CF bronchiectasis. We conclude that lung disease severity in terms of number of bronchial segments results in an associated LCI increase, likely constituting a portion of LCI that cannot be reversed by treatment in patients with CF lung disease.
Introduction: Aspergillus fumigatus (Af) causes various pulmonary entities ranging from aspergill... more Introduction: Aspergillus fumigatus (Af) causes various pulmonary entities ranging from aspergilloma in lung cavities (1), allergic or hypersensitive reactions (ABPA) (2), chronic necrotizing aspergillosis in chronic lung disease (3) to invasive infection in immunocompromised hosts (4). ABPA is reported in 2 15% of adult CF patients, fulfilling 4 minimal criteria. Patients: In the past 6 months, we had 2 young CF patients, a 9 year old male and a 12 year old girl with PA, and overlapping criteria of entity 2 and 3. Results: the boy had normal lungfunction, total IgE 2330 KIU/1, and elevated Afspecific IgE and IgG, a large peripheral infiltrate, with eosinophilic granulomatous reaction and fungal hyphy on transthoracic needle biopsy (sputa were negative). The girl has an FEV1 60% (without asthma symptoms), normal IgE, hemoptysis and repeated positive sputum cultures for Af, being the only pathogen cultured, including bacteria and also a large peripheral infiltrate. Both responded very well to voriconazole with disappearance of the infiltrates. Oral steroids given to the first patient resulted in steroid related diabetes. Conclusions: PA is also seen in young CF patients, ABPA criteria are not always fulfilled and clinical entities of PA overlap. Diagnosis may be difficult and the causal role of Af in lung infiltrates is difficult to asses (needing biopsy in case 1). Treatment guidelines are not uniform and the use of oral steroids includes risk for important side-effects.
Isolated tracheoesophageal fistula (H-TOF) is a rare type of tracheoesophageal anomaly and is in ... more Isolated tracheoesophageal fistula (H-TOF) is a rare type of tracheoesophageal anomaly and is in most cases diagnosed in the neonatal period because of choking and cyanosis during feeding. Diagnosis may be delayed even until adulthood because of nonspecific and sometimes intermittent symptoms, and because false-negative results of all diagnostic tools are not uncommon. We report a 10-year-old child with H-TOF, whose symptoms had nearly disappeared completely between the ages of 4 and 10 years. Diagnosis was only possible after the recurrence of the symptoms at the time of an episode of bronchitis, profound interrogation of the child's medical history, and questioning of the results of a former diagnostic work-up. In this article, we discuss the potential pitfalls in both clinical diagnosis and diagnostic work-up.
Purpose To optimize our standard full-spine radiography with respect to diagnostic quality and do... more Purpose To optimize our standard full-spine radiography with respect to diagnostic quality and dose. Methods A phantom study was performed to establish an optimal posterior-anterior view (PA) full spine protocol having the lowest dose with non-inferior quality compared to standard. We then applied this protocol in 40 pediatric patients (group B). The radiographs were scored on six criteria by a pediatric radiologist and orthopedist and compared to the scores of 40 PA full spine radiographs performed in 2013 with standard protocol (group A). Radiation dose was assessed by dose area product (DAP) and effective dose (E). Statistical analysis included independent samples t test, Mann-Whitney U test and intra-class correlation coefficient (ICC). Results An optimized protocol was defined (0.2 mm Cu filter, 0.87 relative exposure, with grid). Mean age was 13.3 ± 1.6 years for group A and 13.4 ± 1.7 years for group B. For group B, the mean DAP was 47.0 µGy m 2 with an E of 0.13 mSv. For group A, the mean DAP was 85.3 µGy m 2 with an E of 0.24 mSv. This represents a dose reduction of 45%. Mean image quality scores for group A (27.9 ± 2.4) and group B (28.1 ± 2.3) were similar (p = 0.612). Interobserver agreement was observed to be excellent (ICC 0.92). Conclusion This study demonstrates that a low-dose full-spine radiograph can be performed in patients with idiopathic scoliosis without loss of image quality. Graphical abstract These slides can be retrieved under Electronic Supplementary Material.
Poster: "ECR 2016 / C-0649 / An optimal protocol for full-spine radiography" by: "... more Poster: "ECR 2016 / C-0649 / An optimal protocol for full-spine radiography" by: "C. Ernst1, N. Buls1, A. Laumen1, E. ASSENOVA2, S. Assouti3, G. Van Gompel1, J. de Mey4; 1Brussels/BE, 2LEDE/BE, 3Antwerpen/BE, 4Brussel/BE"
JBR-BTR: organe de la Société royale belge de radiologie (SRBR) = orgaan van de Koninklijke Belgische Vereniging voor Radiologie (KBVR)
Symmetrical lesions in the basal ganglia are accepted as the characteristic radiological feature ... more Symmetrical lesions in the basal ganglia are accepted as the characteristic radiological feature of methanol toxicity. A case of chronic deliberate repeated methanol ingestion is presented. The typical MRI findings of basal ganglia lesions on T1 and T2 weighted images, FLAIR, diffusion and T1-weighted post Gadolinium images are presented. To our knowledge, this is the first case in which late diffusion and post contrast MRI images, showing necrotic sequelae due to methanol ingestion, are reported.
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