Adult Hirschprung disease: radiographic findings

Hirschprung disease is usually diagnosed in infancy. Occasionally patients reach adulthood without diagnosis or treatment. Four cases of adult Hirschprung disease are described. The principal radiographic findings are a markedly dilated, feces-filled colon above the zone of transition; a narrowed rectum; a cone- or funnel-shaped zone of transition; and a mosaic colonic pattern caused by collapsed redundant mucosa after colonic cleansing. In an adult, identification on a barium enema examination of an abrupt, smooth transition zone in the rectum with proximal colonic dilatation, in conjunction with an appropriate clinical history, should suggest the diagnosis of adult Hirschprung disease.     

Erdheim-Chester disease: radiographic findings in five patients

We present the case histories of five patients with Erdheim-Chester disease, a rare lipoidosis that has several typical radiographic features. In all the patients, the diaphyses and metaphyses of the extremities demonstrated a symmetric pattern of diffuse or patchy increased density, a coarsened trabecular pattern, medullary sclerosis, and cortical thickening. The epiphyses were spared in four patients and partially involved in one. The axial skeleton was involved in one patient. Radiotracer ^99mTc accumulated in areas of radiographic abnormalities in all patients. In one patient, MRI demonstrated an abnormal signal, corresponding to radiographic abnormalities. The signal was hypointense to muscle on T1-weighted sequences and heterogeneously hyperintense and hypointense to normal bone marrow on T2-weighted sequences. Xanthogranulomatous lesions infiltrated the retroperitoneum in one patient, the testes in one patient, the eyelids in one patient, and the orbits in two patients.     

Nasopharyngeal carcinoma: clinical and radiographic findings in children

Nasopharyngeal carcinoma (NPC) in childhood occurs so infrequently that it is not suspected in affected children until the disease has been present for a long time and local spreading has occurred. The survival rates are therefore quite poor. Six children with NPC are described. A massive local lymph node spread simulating lymphoma was present in half of the patients; in the other half the disease was more subtle, presenting with epistaxis and CNS involvement. If an evaluation of the nasopharynx were part of the initial physical examination in children, the diagnosis of NPC would be made earlier and survival rates would improve.     

Paracardiac pleural Castleman disease: radiographic and MR findings

Castleman disease is a rare benign lymphoid tumour of uncertain aetiology that usually appears as a solitary mediastinal mass. We report a rare case of Castleman disease in the right paracardiac pleural space, occurring in a young woman with non-specific chest discomfort. MRI showed a well-defined, oval mass that was slightly hyperintense on T(1) weighted images, inhomogeneously hyperintense on T(2) and enhanced T(1) weighted images. The patient underwent radical tumour resection and has remained well for 8 years.     

Endoscopic retrograde intrahepatic cholangiogram: radiographic findings in intrahepatic disease.

Endoscopic retrograde intrahepatic cholangiograms were evaluated in 107 patients and correlated with intrahepatic diagnoses determined by liver biopsy. Included were normal livers (six), cirrhosis (38) portal fibrosis (14), cholangitis (22), metastases (11), and miscellaneous diagnoses (16). Results suggest that differentiation of the normal from the abnormal intrahepatic biliary system using the endoscopic retrograde intrahepatic cholangiogram is possible, and that certain patterns of abnormality prevail within given disease categories. The cholangiogram in cirrhosis is marked by ductular stenosis, diminished arborization, tortuosity, and approximation of the intrahepatic ducts. Sclerosing cholangitis demonstrates focal stenoses with concomitant ectasias and frequent similar involvement of the extrahepatic system. Chronic cholangitis and portal fibrosis are frequently associated with extrahepatic obstructing lesions and increased intrahepatic ductal caliber, but demonstrate no distinguishing intrahepatic characteristics. Intrahepatic metastases, polycystic liver disease, and primary hepatic neoplasm produce mass effects consisting of ductal displacement, narrowing, and obstruction. The potential of endoscopic retrograde intrahepatic cholangiography in evaluating the intraheptic biliary tree is significant; specifically in separating normal from abnormal, in distinguishing between intrahepatic processes, and as an adjunct to liver biopsy in determining the extent and location of intrahepatic abnormalities.     

Pulmonary manifestations of Hodgkin's disease: radiographic and CT findings

The aim of this study was to assess the radiological and CT findings in patients with pulmonary Hodgkin's disease and to analyse to what extent CT provides more diagnostic information. In 37 patients with 41 episodes of pulmonary manifestation of Hodgkin's disease (histological diagnosis: 11, clinical diagnosis: 30) 39 radiographs and 33 CT scans were analysed by two readers in consensus. Pulmonary nodules were recorded in 77% of radiographs (CXR) and 88% of CT scans. Nodules were multiple in 67% (CXR) and 86% (CT) and bilateral in 43% (CXR) and 66% (CT) of cases, respectively. Nodule size ranged from 2 to 100 mm. Of the nodules, 83% at radiography and CT, respectively, were < or =30 mm, and again 83% at radiography and CT, respectively, were irregularly marginated. Diffuse infiltration with and without nodules was less common. With pulmonary manifestations at initial diagnosis of Hodgkin's disease there was always hilar or mediastinal lymphadenopathy. Of 20 episodes, in which radiograph and CT had been obtained within 8 days, CT demonstrated pulmonary involvement when chest radiography was normal in 3 cases and demonstrated more lesions in 12 cases. The typical appearance of pulmonary HD consisted of multiple, irregularly marginated pulmonary nodules. Diffuse infiltration was less common. Computed tomography was superior to radiography not only in characterization of lesions but could also demonstrate pulmonary involvement when the radiograph was normal and should, therefore, be used liberally in addition to radiography.     

Pulmonary involvement with Erdheim-Chester disease: radiographic and CT findings

OBJECTIVE: We determined the chest radiographic and CT findings of Erdheim-Chester disease with pulmonary involvement. MATERIALS AND METHODS: We retrospectively reviewed the radiologic images of 15 patients with biopsy-proven Erdheim-Chester disease. Nine patients had chest radiographic images and CT scans that were available for review. Six men and three women were studied (age range, 25-70 years; mean age, 56 years). Two radiologists interpreted all images by consensus. Lung parenchyma was assessed for the type and distribution of disease. Bronchi, pleurae, hila, and mediastinal and extrathoracic structures were evaluated for abnormalities. Pathologic specimens from all patients were reviewed and correlated with radiologic findings. RESULTS: Eight of nine patients had thoracic images with abnormal findings. The most common radiographic pattern was reticular interstitial opacities with fissural and interlobular septal thickening. CT revealed regions of ground-glass attenuation and centrilobular nodular opacities. Typically, extrapulmonary findings included pleural effusions (6/8 patients), pericardial fluid or thickening (4/8), and extrathoracic infiltrative soft-tissue masses (4/8). CONCLUSION: The most common findings of Erdheim-Chester disease with pulmonary involvement include an interstitial process characterized by smooth interlobular septal thickening and centrilobular nodular opacities, fissural thickening, and pleural effusions. On CT, six of nine patients had pericardial fluid and thickening or extrathoracic soft-tissue masses. Such findings are characteristic of Erdheim-Chester disease with pulmonary involvement. Definitive diagnosis requires correlating skeletal findings and lung biopsy findings.     

Dentomaxillofacial manifestations of Gaucher's disease: preliminary clinical and radiographic findings

Objectives

A wide variety of manifestations is presented in patients with Gaucher''s disease (GD), including bone, haematology and visceral disturbances. This study was conducted to ascertain the main maxillofacial abnormalities by means of clinical survey, panoramic and cone beam CT (CBCT); to compare the patient''s group with an age–sex matched control group; and to correlate clinical and radiological data.

Methods

Ten patients previously diagnosed with GD were submitted to clinical and radiological surveys (CBCT and panoramic radiographs). The examination consisted of anamnesis, extra- and intraoral examinations and analyses of each patient''s records. Imaging data were collected from the point of view of 3 observers, and the results compared with a healthy group (20 individuals) by means of statistical analysis (Fisher''s exact test).

Results

Gaucher patients had significantly more manifestations than otherwise healthy carriers. The most prevalent findings were enlarged marrow spaces, generalized osteopenia and effacement of jaw structures (mandibular canal, lamina dura and mental foramen). Here we describe a case in which thickening of the maxillary sinus mucosa was observed on CBCT rather than opacification of the sinus as seen on panoramic radiographs. Pathological fractures, root resorption and delay on tooth eruption were not observed.

Conclusions

A poor relationship could be observed between clinical and radiological data. Patients showed important bone manifestations, which require careful diagnostic and surgical planning whenever necessary. Although panoramic radiographs have shown significant differences, CBCT is more effective in pointing out differences between patients and a control group, thus showing it as an important tool for evaluation of Gaucher patients.     

Hand and wrist arthropathies of hemochromatosis and calcium pyrophosphate deposition disease: distinct radiographic features

Radiographic features of hand and wrist involvement in 26 patients with hemochromatosis and in 26 patients with idiopathic calcium pyrophosphate dihydrate (CPPD) crystal deposition disease were compared. Two radiologists independently examined the radiographs without knowledge of the specific group to which the patient belonged. The results of this study clearly establish that structural joint diseases in the two disorders are not identical. Characteristic findings allow the radiologist to favor one diagnosis over the other. These radiographic differences indicate that the arthropathy of hemochromatosis is related to factors additional to the presence of CPPD crystals, specifically, the more prevalent narrowing of the metacarpophalangeal joint spaces, including those in the fourth and fifth digits, peculiar hook-like osteophytes on the radial aspect of the metacarpal heads, and less prevalent separation of the scaphoid and the lunate.     

小儿腐蚀性食管炎病因与X线表现的对比研究

目的　分析小儿腐蚀性食管炎X线表现特点 ,探讨其食管损伤程度与腐蚀剂关系。方法　 16例小儿腐蚀性食管炎 ,其中男 14例 ,女 2例 ,年龄 1～ 11岁 ,平均 3 4岁。依据其吞服药物性质 ,剂量与X线表现 ,对其发病部位 ,损伤程度逐一进行对比研究。结果　 16例食管狭窄范围平均为10 7cm ,占食管平均总长度的 6 3 4 %,狭窄部位直径 0 1～ 1 2cm。病变累及中段或中下段者 13例 ,占 81 3%。碱性腐蚀剂与酸性腐蚀剂所致食管平均狭窄长度占食管平均总长度的百分比分别为70 5 %和 42 0 %,两者间有显著性差异 (t=2 35 9,P <0 0 5 )。结论　小儿腐蚀性食管炎的狭窄程度与腐蚀剂的性质、浓度及吞服剂量呈正相关 ,发病部位以中段及中下段多见 ,强碱腐蚀剂所致食管损伤范围较酸性腐蚀剂更广泛。     

Acid corrosive esophagitis: radiographic findings

Thirty-nine esophagograms of 24 patients after ingestion of muriatic acid (27% HCI) in suicide attempts were reviewed. All esophagograms were obtained in the acute, subacute, and chronic phases. In the acute and subacute phases, the radiographic findings consisted of mucosal edema, submucosal edema or hemorrhage, ulcerations, sloughing of the mucosa, atony, and dilatation. Strictures of the esophagus were present in the chronic phase. These radiographic findings were not different from those found in alkaline corrosive esophagitis. The severity of the corrosive esophagitis is considered related to the concentration, amount, viscosity, and duration of contact between the caustic agent and the esophageal mucosa.