Potential interstitial lung abnormalities on chest X-rays prior to symptoms of idiopathic pulmonary fibrosis.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) often has significant diagnostic delay. At present it is not well-known what factors associate with time to diagnosis and if this is associated with survival after the diagnosis. There has also been increasing attention for interstitial lung abnormalities on chest CT-scans. In this study we assessed what factors associate with time to diagnosis in patients with IPF, and whether early stages of pulmonary fibrosis can be seen on chest X-rays prior to the start of symptoms. METHODS: In this retrospective study, 409 Dutch patients with IPF were included. Clinical characteristics, including patient demographics, medical history, time of start of symptoms, time of first visit to pulmonologist, and any previous radiographic imaging reports were collected from patient records. RESULTS: In 96 patients (23%) a chest X-ray was available that had been made prior to the start of symptoms (median of 50.5 months (IQR 26.3-83.3 months)), and this showed potential interstitial lung abnormalities in 56 patients (58%). The median time from the start of symptoms to the final diagnosis was 24.0 months (interquartile range 9.0-48.0 months). In a multivariate model that corrected for diffusion capacity of the lung for carbon monoxide, forced vital capacity, sex, and age at diagnosis, time to diagnosis did not associate with survival (hazard ratio 1.051 (95% CI 0.800-1.380; p = 0.72)). CONCLUSIONS: There is a significant diagnostic delay for patients with IPF, but longer time to diagnosis did not associate with survival. Interstitial lung abnormalities were seen in more than half of the patients in whom a chest X-ray had been made prior to the start of symptoms. This illustrates that a computed tomography scan should be strongly considered for analysis of unexplained abnormalities on a chest X-ray. This could facilitate early detection and possibly prevention of disease progression for patients with pulmonary fibrosis.

Chest Computed Tomography-Based Scoring of Thoracic Sarcoidosis: Inter-rater Reliability of CT Abnormalities.

PURPOSE: To determine inter-rater reliability of sarcoidosis-related computed tomography (CT) findings that can be used for scoring of thoracic sarcoidosis. MATERIALS AND METHODS: CT images of 51 patients with sarcoidosis were scored by five chest radiologists for various abnormal CT findings (22 in total) encountered in thoracic sarcoidosis. Using intra-class correlation coefficient (ICC) analysis, inter-rater reliability was analysed and reported according to the Guidelines for Reporting Reliability and Agreement Studies (GRRAS) criteria. A pre-specified sub-analysis was performed to investigate the effect of training. Scoring was trained in a distinct set of 15 scans in which all abnormal CT findings were represented. RESULTS: Median age of the 51 patients (36 men, 70%) was 43 years (range 26 - 64 years). All radiographic stages were present in this group. ICC ranged from 0.91 for honeycombing to 0.11 for nodular margin (sharp versus ill-defined). The ICC was above 0.60 in 13 of the 22 abnormal findings. Sub-analysis for the best-trained observers demonstrated an ICC improvement for all abnormal findings and values above 0.60 for 16 of the 22 abnormalities. CONCLUSIONS: In our cohort, reliability between raters was acceptable for 16 thoracic sarcoidosis-related abnormal CT findings. KEY POINTS: • Thoracic sarcoidosis is common; knowledge on reliability of CT scoring is limited. • Scoring CT abnormalities in pulmonary sarcoidosis can achieve good inter-rater agreement. • CT scoring validation in thoracic sarcoidosis is important for diagnostic and prognostic studies.

Comparison of Imaging Strategies with Conditional versus Immediate Contrast-Enhanced Computed Tomography in Patients with Clinical Suspicion of Acute Appendicitis.

OBJECTIVES: To compare the diagnostic accuracy of conditional computed tomography (CT), i.e. CT when initial ultrasound findings are negative or inconclusive, and immediate CT for patients with suspected appendicitis. METHODS: Data were collected within a prospective diagnostic accuracy study on imaging in adults with acute abdominal pain. All patients underwent ultrasound and CT, read by different observers who were blinded from the other modality. Only patients with clinical suspicion of appendicitis were included. An expert panel assigned a final diagnosis to each patient after 6 months of follow-up (clinical reference standard). RESULTS: A total of 422 patients were included with final diagnosis appendicitis in 251 (60 %). For 199 patients (47 %), ultrasound findings were inconclusive or negative. Conditional CT imaging correctly identified 241 of 251 (96 %) appendicitis cases (95 %CI, 92 % to 98 %), versus 238 (95 %) with immediate CT (95 %CI, 91 % to 97 %). The specificity of conditional CT imaging was lower: 77 % (95 %CI, 70 % to 83 %) versus 87 % for immediate CT (95 %CI, 81 % to 91 %). CONCLUSION: A conditional CT strategy correctly identifies as many patients with appendicitis as an immediate CT strategy, and can halve the number of CTs needed. However, conditional CT imaging results in more false positives. KEY POINTS: • Conditional CT (CT after negative/inconclusive ultrasound findings) can be used for suspected appendicitis. • Half the number of CT examinations is needed with a conditional strategy. • Conditional CT correctly identifies as many patients with appendicitis as immediate CT. • Conditional imaging results in more false positive appendicitis cases.

Chronic thromboembolic pulmonary hypertension.

Chronic pulmonary thromboembolic disease is an important cause of severe pulmonary hypertension, and as such is associated with significant morbidity and mortality. The prognosis of this condition reflects the degree of associated right ventricular dysfunction, with predictable mortality related to the severity of the underlying pulmonary hypertension. Left untreated, the prognosis is poor. Pulmonary endarterectomy is the treatment of choice to relieve pulmonary artery obstruction in patients with chronic thromboembolic pulmonary hypertension and has been remarkably successful. Advances in surgical techniques along with the introduction of pulmonary hypertension-specific medication provide therapeutic options for the majority of patients afflicted with the disease. However, a substantial number of patients are not candidates for pulmonary endarterectomy due to either distal pulmonary vascular obstruction or significant comorbidities. Therefore, careful selection of surgical candidates in expert centres is paramount. The current review focuses on the diagnostic approach to chronic thromboembolic pulmonary hypertension and the available surgical and medical therapeutic options.

CT evaluation of chronic thromboembolic pulmonary hypertension.

The educational objectives of this article are to provide an overview of the computed tomography (CT) findings in chronic thromboembolic pulmonary hypertension. This article reviews the key imaging findings at CT in patients with chronic thromboembolic pulmonary hypertension. After reading this article, the reader should have an improved awareness of the condition, its imaging features, and the CT imaging features associated with surgically accessible disease.

The usefulness of repeated CMR and FDG PET/CT in the diagnosis of patients with initial possible cardiac sarcoidosis.

BACKGROUND: Cardiac sarcoidosis (CS) diagnosis is usually based on advanced imaging techniques and multidisciplinary evaluation. Diagnosis is classified as definite, probable, possible or unlikely. If diagnostic confidence remains uncertain, cardiac imaging can be repeated. The objective is to evaluate the usefulness of repeated cardiac magnetic resonance imaging (CMR) and fluorodeoxyglucose positron emission tomography (FDG PET/CT) for CS diagnosis in patients with an initial "possible" CS diagnosis. METHODS: We performed a retrospective cohort study in 35 patients diagnosed with possible CS by our multidisciplinary team (MDT), who received repeated CMR and FDG PET/CT within 12 months after diagnosis. Imaging modalities were scored on abnormalities suggestive for CS and classified as CMR+/PET+, CMR+/PET-, CMR-/PET+ and CMR-/PET-. Primary endpoint was final MDT diagnosis of CS. RESULTS: After re-evaluation, nine patients (25.7%) were reclassified as probable CS and 16 patients (45.7%) as unlikely CS. Two patients started immunosuppressive treatment after re-evaluation. At baseline, eleven patients (31.4%) showed late gadolinium enhancement (LGE) on CMR (CMR+) and 26 (74.3%) patients showed myocardial FDG-uptake (PET+). At re-evaluation, nine patients (25.7%) showed LGE (CMR+), while 16 patients (45.7%) showed myocardial FDG-uptake (PET+). When considering both imaging modalities together, 82.6% of patients with CMR-/PET+ at baseline were reclassified as possible or unlikely CS, while 36.4% of patients with CMR+ at baseline were reclassified as probable CS. Three patients with initial CMR-/PET+ showed LGE at re-evaluation. CONCLUSION: Repeated CMR and FDG PET/CT may be useful in establishing or rejecting CS diagnosis, when initial diagnosis is uncertain. However, clinical relevance has to be further determined.

Profiles of US and CT imaging features with a high probability of appendicitis.

OBJECTIVES: To identify and evaluate profiles of US and CT features associated with acute appendicitis. METHODS: Consecutive patients presenting with acute abdominal pain at the emergency department were invited to participate in this study. All patients underwent US and CT. Imaging features known to be associated with appendicitis, and an imaging diagnosis were prospectively recorded by two independent radiologists. A final diagnosis was assigned after 6 months. Associations between appendiceal imaging features and a final diagnosis of appendicitis were evaluated with logistic regression analysis. RESULTS: Appendicitis was assigned to 284 of 942 evaluated patients (30%). All evaluated features were associated with appendicitis. Imaging profiles were created after multivariable logistic regression analysis. Of 147 patients with a thickened appendix, local transducer tenderness and peri-appendiceal fat infiltration on US, 139 (95%) had appendicitis. On CT, 119 patients in whom the appendix was completely visualised, thickened, with peri-appendiceal fat infiltration and appendiceal enhancement, 114 had a final diagnosis of appendicitis (96%). When at least two of these essential features were present on US or CT, sensitivity was 92% (95% CI 89-96%) and 96% (95% CI 93-98%), respectively. CONCLUSION: Most patients with appendicitis can be categorised within a few imaging profiles on US and CT. When two of the essential features are present the diagnosis of appendicitis can be made accurately.

Real-Time 3D fluoroscopy guidance during needle interventions: technique, accuracy, and feasibility.

OBJECTIVE: Real-time 3D fluoroscopy guidance using cone beam CT with dedicated needle path planning software is a promising new interventional technique. The objective of this study was to evaluate the procedure and to assess the accuracy and feasibility of this technique for use in needle interventions. SUBJECTS AND METHODS: All procedures were performed using a flat panel-based fluoroscopy system capable of acquiring cone beam CT images and dedicated needle path-planning software. This new technology allows the use of fluoroscopy coregistered with a 3D data set reconstructed from the acquired attenuation information. The needle trajectory is planned in the 3D data set using the needle path-planning software. The calculated trajectory is then projected on to the real-time fluoroscopy image. Fluoroscopy time, accuracy, technical success of the procedures, median procedure time, and complications were recorded in 145 interventions. RESULTS: One hundred forty-five needle interventions were performed in 139 patients using real-time 3D fluoroscopy guidance. Procedures were divided into five groups according to anatomic region: upper thoracic (n = 19; 13.1%), lower thoracic (n = 18; 12.4%), upper abdominal (n = 65; 44.8%), lower abdominal (n = 13; 9.0%), and musculoskeletal (n = 30; 20.7%). Thirty needle interventions were therapeutic, and 115 were diagnostic biopsies. All interventions were within the predefined 5-mm safety margin and achieved 100% technical success. A histopathologic diagnosis could be made in 91.4% of the diagnostic biopsies. The median interventional procedure time was 28.5 minutes, and the median fluoroscopy time was 2 minutes 58 seconds. There were minor complications in six patients (4.3%) and one major complication (0.7%). CONCLUSION: Real-time 3D fluoroscopy guidance is a new, promising, and feasible technique providing high accuracy in needle interventions.

[Complaints of back pain in childhood: find curable causes]. / Rugpijnklachten op de kinderleeftijd: behandelbare oorzaken opsporen.

Three children, a 13-year-old boy and a 3-year-old and 6-year-old girl, were presented to the hospital with back pain, caused by Scheuermann's disease, spondylodiscitis and sickle cell disease, respectively. The boy with Scheuermann's disease received exercise therapy, the spondylodiscitis was treated with antibiotic therapy and the girl with sickle cell disease was given hyperhydration and folic acid. Although back pain is a common problem in children and teenagers, it is infrequently reported in the clinic. In contrast to back pain in adults, the same complaint in childhood is more often caused by a serious disorder which should be treated. Various causes of back pain in children can be distinguished: mechanical problems, infections of the lumbar spine, neoplasia, inflammation, and other causes, such as sickle cell disease. A child or adolescent presenting to the clinic with complaints of back pain deserves a careful detailed evaluation of the history, appropriate physical examination and additional investigation. Alarm symptoms are an increase in back pain, age below 4 years, pain during the night, restriction in function, systemic complaints or neurological deficits.

TINF2 Gene Mutation in a Patient with Pulmonary Fibrosis.

Pulmonary fibrosis is a frequent manifestation of telomere syndromes. Telomere gene mutations are found in up to 25% and 3% of patients with familial disease and sporadic disease, respectively. The telomere gene TINF2 encodes an eponymous protein that is part of the shelterin complex, a complex involved in telomere protection and maintenance. A TINF2 gene mutation was recently reported in a family with pulmonary fibrosis. We identified a heterozygous Ser245Tyr mutation in the TINF2 gene of previously healthy female patient that presented with progressive cough due to pulmonary fibrosis as well as panhypogammaglobulinemia at age 52. Retrospective multidisciplinary evaluation classified her as a case of possible idiopathic pulmonary fibrosis. Telomere length-measurement indicated normal telomere length in the peripheral blood compartment. This is the first report of a TINF2 mutation in a patient with sporadic pulmonary fibrosis, which represents another association between TINF2 mutations and this disease. Furthermore, this case underlines the importance of telomere dysfunction and not telomere length alone in telomere syndromes and draws attention to hypogammaglobulinemia as a manifestation of telomere syndromes.

[CT strategy for patients with suspected acute appendicitis; comparison of conditional and immediate CT]. / CT-strategie bij vermoeden van acute appendicitis; vergelijking van conditionele en directe CT.

OBJECTIVE: To compare the diagnostic accuracy of conditional CT strategy, i.e. CT if ultrasound findings are negative or inconclusive, with immediate CT strategy for patients with suspected appendicitis. DESIGN: Subanalysis of a prospective multicenter diagnostic accuracy study. METHOD: Only data of patients with signs of appendicitis based on medical history, physical examination, and laboratory tests were analyzed. All patients underwent both ultrasound and CT. Images of each were read by different observers who were blinded to the results of the other imaging modality. The observer then selected the most likely diagnosis. These diagnoses were compared with the reference standard, i.e. final diagnoses as assigned by an expert panel based on all available data and at least 6 months of follow-up. RESULTS: A total of 422 patients with suspected appendicitis were included. In 251 patients the final diagnosis was acute appendicitis (59%). In 199 patients (47%), ultrasound findings were inconclusive or negative. Use of conditional CT strategy resulted in correctly identified number of correctly identified patients with appendicitis, i.e. 96% (95% CI 93-98), versus 95% identified by immediate CT (95% CI 91-97). However, conditional CT strategy resulted in more false positive diagnoses compared with immediate CT (39 versus 22), had an accompanying lower specificity of 77% (95% CI 70-83) versus 87% (95% CI 81-91), and a lower positive predictive value of 86% (95% CI 81-90) versus 92% (95% CI 87-95). CONCLUSION: Use of a conditional CT strategy results in exactly the same number of patients with correctly identified acute appendicitis while halving the number of CTs needed. However, conditional strategy results in more false positive diagnoses.

Multifocal inflammatory demyelinating neuropathy: a distinct clinical entity?

BACKGROUND: Several patients have been reported with an asymmetric sensory or sensorimotor demyelinating neuropathy not fulfilling the diagnostic criteria for chronic inflammatory demyelinating polyneuropathy or multifocal motor neuropathy. OBJECTIVE: To present the clinical, electrophysiologic, radiologic, and pathologic features of six patients with an asymmetric sensory or sensorimotor demyelinating neuropathy. RESULTS: All six patients were initially affected in only one limb; in four patients the neuropathy progressed to other limbs in an asymmetric fashion during several years. On electrophysiologic examination, evidence of multifocal demyelination and conduction block in motor and sensory nerves was found in all patients. MRI of the brachial plexus revealed swollen nerves and an increased signal intensity on T2-weighted imaging in four patients. A biopsy sample taken from the brachial plexus of one patient revealed evidence of inflammation. All patients showed a beneficial response to IV immunoglobulin treatment. Thirty-four similar patients have been reported previously, many of whom were initially diagnosed as having various other (nontreatable) diseases. CONCLUSIONS: The authors propose calling this neuropathy "multifocal inflammatory demyelinating neuropathy" and considering it as a distinct clinical entity to facilitate early diagnosis of this treatable disorder.

Magnetic resonance imaging of the brachial plexus in patients with multifocal motor neuropathy.

We studied whether magnetic resonance (MR) imaging of the brachial plexus is useful to distinguish multifocal motor neuropathy (MMN) from lower motor neuron disease (LMND) and whether abnormalities resemble those of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We compared MR images of the brachial plexus of nine patients with MMN with scans from five patients with CIDP, eight patients with LMND, and 174 controls. In two patients with MMN, and in three patients with CIDP, the MR images showed an increased signal intensity on the T2-weighted images of the brachial plexus. Two other patients with MMN demonstrated a more focal, increased signal intensity on the T2-weighted images, occurring in one patient only in the axilla, and in the other patient in the axilla and in the ventral rami of the roots. MR images of the brachial plexus of eight patients with LMND were normal. The distribution of the MR imaging abnormalities corresponded with the distribution of symptoms of the patients: asymmetrical in MMN and symmetrical in CIDP. These findings demonstrate that MR imaging abnormalities of the brachial plexus in patients with MMN resemble those seen in CIDP and may be useful to distinguish MMN from LMND.

Malignant stromal tumour of the rectum: findings at endorectal ultrasound and MRI.

This case report describes the findings on endorectal ultrasound and MRI in a patient with a giant malignant stromal tumour of the rectum. A review of imaging characteristics and histopathological findings as described in the literature is presented.

Transmanubrial osteomuscular sparing approach for sulcus superior tumours.

OBJECTIVE: The authors report their initial experience with the transmanubrial osteomuscular sparing approach for resection of sulcus superior tumours. The feasibility of this technique is evaluated. PATIENTS: Between February 2000 and March 2002 three patients with sulcus superior tumours were surgically treated using the transmanubrial osteomuscular sparing approach. The first two patients had a non-small cell carcinoma of the upper lobe. In the third patient a pathological diagnosis of a plasmocytoma of the first rib was made. In two cases the first thoracic root was resected. RESULTS: In two patients a complete R0 resection was achieved. However, an additional posterolateral thoracotomy was necessary in two patients because the costovertebral angle was difficult to address. In one patient final histologic examination found microscopically positive margins. CONCLUSION: We believe that the transmanubrial osteomuscular sparing technique enables us to approach and control the subclavian vessels and brachial plexus in an oncologically responsible way and permits a radical resection of tumours invading the thoracic inlet.

[Diagnostic image (200). A woman with acute pain between the scapulae]. / Diagnose in beeld (200). Een vrouw met acute pijn tussen de schouderbladen.

A 71-year-old woman presented with an acute onset of interscapular pain due to an intramural haematoma of the ascending part of the aorta. She was treated with an emergency ascending aorta graft replacement.

[An 84 year old man with gastric outlet obstruction]. / Een 84-jarige man met onverklaarde maagretentie.

An 84-year-old man was admitted with 4 days of postprandial emesis. Gastroscopy revealed the presence of a large gallstone in the duodenal bulb causing gastric outlet obstruction. The patient was diagnosed with Bouveret's syndrome. Treatment consisted of gastrotomy with removal of the gallstone.