赫什朋病的X线诊断(附49例分析)

目的：提高对赫什朋病特征性X线征象的认识。方法：49例患儿均行钡剂灌肠检查，并经手术病理证实，将其X线征象狭窄段、移行段、24h钡剂残留情况及狭窄段肠管不规则收缩进行统计学处理，计算其敏感性、特异性、阳性预告值、假阳性率、阴性预告值、假阴性率、约登指数。结果：49例中，短段型(17／49)、常见型(24／49)、长段型(8／49)；赫什朋病39例，非赫什朋病10例，它们的敏感性、特异性、阳性预告值、假阳性率、阴性预告值、假阴性率、约登指数，狭窄段分别是95％、30％、84％、70％、60％、5％、25％；移行段分别是74％、90％、97％、10％、47％、26％、64％；24h钡剂残留情况分别是79％、60％、89％、40％、43％、21％、39％；狭窄段肠管不规则收缩分别是31％、100％、100％、0、27％、69％、31％。结论：在赫什朋病的钡剂灌肠X线征象中，移行段最高可靠，狭窄段最常见，狭窄段肠管不规则收缩的特异性最高，24h钡剂残留对早期不典型的赫什朋病的诊断具有重要意义，常见型X线诊断最可靠。     

Hirschsprung disease: accuracy of the barium enema examination

To determine the relative accuracy of the various radiologic signs of Hirschsprung disease (HD), we retrospectively reviewed both radiographs obtained after a barium enema and the medical records of 62 children who had surgery to prove or exclude the diagnosis of HD. The visualization of a rectosigmoid transition zone was highly predictive of HD, but nonvisualization did not rule out HD. A false positive transition zone at the splenic flexure was seen in four neonates who had small left colon syndrome rather than HD. Retention of barium seen on radiographs obtained 24 hours after a barium enema was not a specific sign, but it was the only sign of HD in seven neonates, including two who had total colonic aganglionosis. Anal manipulation prior to the barium enema examination did not affect the diagnostic value of that procedure. We conclude that the single most reliable radiographic sign of HD is the presence of a rectosigmoid transition zone. Statistically, the use of three radiographic features combined (rectosigmoid transition zone, retention of barium, and stool mixed with barium) correlated better with the presence or absence of HD than did any of these features alone. A comparison of 24 and 48 hour postevacuation radiographs may help to differentiate HD from meconium plug syndrome.     

The barium-enema examination and rectal manometry in Hirschsprung disease.

Barium-enema studies and rectal manometry were clearly shown complementary in diagnosing Hirschsprung disease. Of 27 patients studied, 19 had positive barium-enema and rectal manometric studies and were proved histologically to have Hirschsprung disease. Three had positive barium-enemas but normal rectal manometric studies in the first 10 days of life; later manometric studies were positive, and all 3 were subsequently proved to have Hirschsprung disease. Three patients had negative barium-enema and positive manometric studies and were proved to have low segment Hirschsprung disease. If both studies indicate the disease, rectal biopsy is not necessary before surgery.     

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.     

Ultrasound diagnosis of Hirschsprung disease in the infant with distended abdomen

It is possible to diagnose Hirschsprung disease by real-time ultrasound, as demonstrated by the case of an apparently asymptomatic 1-month-old baby boy who was found to have a distended abdomen at well-baby check-up.     

超声对先天性巨结肠术后并发症的诊断价值

目的探讨经腹壁联合经会阴超声对先天性巨结肠儿童术后并发症的诊断价值。资料与方法回顾性分析行超声检查的114例先天性巨结肠术后患儿的超声声像图资料,以手术、病理结果和临床特征为诊断“金标准”,计算超声检查的敏感度、特异度、阳性预测值、阴性预测值,采用Kappa检验评价超声检查与诊断“金标准”的一致性。结果114例患儿中,23例(20.2%)出现手术相关并发症,包括巨结肠相关小肠结肠炎(HAEC)6例,吻合口漏2例,各种瘘道形成3例,吻合口裂开2例,脱出肠管扭转2例,吻合口狭窄2例,病变段残留4例,术后出血2例。超声发现19例并发症,漏诊2例HAEC、1例吻合口裂开和1例脱出肠管扭转。超声检查的敏感度、特异度、阳性预测值、阴性预测值分别为82.6%、100.0%、100.0%、95.8%。超声检查与诊断“金标准”的一致性较好(Kappa=0.883,P<0.001)。结论经腹壁联合经会阴超声检查对先天性巨结肠术后并发症的监测有一定的诊断价值。     

Sudden unexpected early neonatal death due to undiagnosed Hirschsprung disease enterocolitis: a report of two cases and literature review

Hirschsprung enterocolitis (HEC) is an uncommon, albeit well known, complication of Hirschsprung disease (HD). It is multifactorial and can appear in different age groups, but is particularly important in the neonatal period where it is characteristically seen in full-term neonates. Two cases of HEC are reported that were diagnosed at post-mortem examination, which presented as early sudden neonatal death, with a review the literature on fatal Hirschsprung enterocolitis. Case 1 was a 4-day old male neonate who was found unwell, struggling to breath, and with green vomitus. He was taken to hospital and pronounced dead a short time later. According to the parents meconium was passed on the first day. Post-mortem examination demonstrated necrotizing enterocolitis with isolated bowel perforation. Histology disclosed unsuspected HD. Case 2 was a 2-day old male neonate who was found wheezing with green vomitus. He arrived floppy, cyanosed, and in shock at the hospital and died a few hours later. Meconium was not passed, according to the parents. Post-mortem examination revealed necrotizing enterocolitis. There was also recto-sigmoidal aganglionosis and acetylcholinesterase staining confirmed HD. HEC is a multifactorial and sometimes recurrent complication of HD which characteristically develops in full-term neonates. Presentation with early sudden neonatal death is rare but should be considered in the diagnostic work-up of sudden deaths in this age group.     

MR imaging of the prostate gland: normal anatomy

MR images of the male pelvis in 55 subjects were analyzed retrospectively for depiction of the zonal anatomy of the prostate gland as related to different repetition (TR) and echo (TE) times, slice thickness, plane of imaging, chronologic age of the patient, and different magnetic field strengths. With imagers operating at 0.35 and 1.5 T, T2-based tissue-contrast images were needed for the demonstration of the internal anatomy of the prostate gland. The display of zonal anatomy was improved when continuous 0.5-cm slices were used. Evaluating sequential sections, the peripheral, central, and transition zones could be differentiated. The peripheral zone showed higher signal intensity than either the central or transition zone and was discerned in the coronal, sagittal, and transverse planes. The central zone was of low signal intensity and was well displayed in the coronal and sagittal planes. The central zone was seen in 31 of the 32 young men (aged 25-35 years) but in only eight of the 23 older men (aged 40 years and older). The transition zone had intrinsic MR parameters similar to the central zone, and the two could be distinguished from each other only by the knowledge of their respective anatomic location. The low-intensity transition zone blended with the periurethral glands and the preprostatic sphincter. The transition zone was of homogeneous low signal intensity in young men but varied in size and signal intensity in older men. Such a detailed display of the prostate zonal anatomy offers a unique potential for the evaluation of prostatic physiology and disease.     

Haddad syndrome presenting with abdominal distension associated with long-segment aganglionosis

Haddad syndrome is a form of neurocristopathy characterized by a combination of congenital central hypoventilation syndrome and Hirschsprung disease (HD). Although Haddad syndrome is extremely rare, awareness of the combination of long-segment HD in Haddad syndrome as well as radiographic manifestations may help to ensure a timely diagnosis as well as to facilitate optimal treatment of this unusual condition. We report a case of Haddad syndrome with long-segment, intestinal aganglionosis in a newborn infant. This report emphasizes the features of HD in children with Haddad syndrome and suggests that specific attention be given to its interpretation on plain radiographs.     

Mucosal detail at CT virtual reality: surface versus volume rendering

PURPOSE: To evaluate computed tomographic virtual reality with volumetric versus surface rendering. MATERIALS AND METHODS: Virtual reality images were reconstructed for 27 normal or pathologic colonic, gastric, or bronchial structures in four ways: the transition zone (a) reconstructed separately from the wall by using volume rendering; (b) with attenuation equal to air; (c) with attenuation equal to wall (soft tissue); (d) with attenuation halfway between air and wall. The four reconstructed images were randomized. Four experienced imagers blinded to the reconstruction graded them from best to worst with predetermined criteria. RESULTS: All readers rated images with the transition zone as a separate structure as overwhelmingly superior (P <.001): Nineteen cases had complete concurrence among all readers. The best of the surface-rendering reconstructions had the transition zone attenuation equal to the wall attenuation (P <.001). The third best reconstruction had the transition zone attenuation equal to the air attenuation, and the worst had the transition zone attenuation halfway between the air and wall attenuation. CONCLUSION: Virtual reality is best with volume rendering, with the transition zone (mucosa) between the wall and air reconstructed as a separate structure.     

Distribution of gastric ulcers by double contrast barium meal with endoscopic correlation

The double contrast barium meals and endoscopic examinations of 246 patients with 264 benign gastric ulcers were reviewed with regard to the location of these ulcers. Seventy-four per cent of ulcers were found in the antral portion of the stomach within 7 cm of the pyloric canal. A similar study in the United Kingdom revealed that the majority of ulcers were in the upper body of the stomach. Since true gastric ulcers almost always arise in a pyloric type of mucosa, or in the zone of transition from pyloric to body type mucosa, perhaps this junction zone lies higher in a United Kingdom population than in North Americans. Possibly the higher transition zone and the documented higher frequency of gastric malignancy in the United Kingdom are related.     

T2WI积分法对前列腺移行区癌诊断及鉴别诊断的价值

目的 探讨T2WI积分法对移行区前列腺癌(PCa)诊断及鉴别诊断的价值.方法 回顾性分析经病理证实的43例移行区PCa和91例前列腺增生(BPH)患者的T2WI图像,将T2WI影像征象分为主要征象和次要征象,并赋予不同积分,通过受试者工作特征(ROC)曲线评价不同积分对其诊断价值.结果 在-1~10共11个积分段中,移行区PCa的敏感度随着积分的增加而降低,特异度和阳性预测值逐渐增高.依据ROC曲线,积分≥4.5时为最佳诊断值,其敏感度为81%,特异度为73.3%,阴性预测值为90.9%,准确度为70.4%;积分≥8.5时,特异度和阳性预测值均为100%;在积分≥0.5和≥1.5时,阴性预测值均为100%.结论 T2WI积分能对移行区PCa进行量化分析,对提高临床诊断和指导治疗具有重要的价值.     

Bilateral primary xanthoma of the humeri with pathologic fractures: A case report

Xanthomas are rare bone tumors that occur more often in the appendicular skeleton and typically appear radiographically benign,with a narrow zone of transition and a sclerotic rim.We report the case of a 57-year-old woman with hyperlipidemia presenting with bilateral shoulder pain after minor trauma.Radiographic and histopathologic investigation demonstrated intraosseous xanthoma with atypical features,including multifocality,a wide zone of transition and pathologic fractures-characteristics more commonly associated with aggressive lesions such as multiple myeloma or metastasis.The diagnosis,imaging,and histological appearance of xanthoma of bone are reviewed.     

前列腺外周带癌T 2 WI影像特征的多因素分析

目的：探讨前列腺外周带癌 T2 WI 影像特征的诊断价值。方法回顾性分析经病理证实的56例外周带前列腺癌的T2 WI图像,另选取75例前列腺增生、7例前列腺炎作为对照组,应用χ2检验对各影像特征进行筛选,再采用多因素Logistic回归分析。结果 T2 WI上病灶的信号强度、形态、均匀度、患侧外周带体积、患侧外周带与移行区分界、前列腺包膜在外周带前列腺癌与对照组的差异均有统计学意义(P<0.05),病灶的边界、包膜在2组间的差异无统计学意义(P>0.05)。多因素Logistic回归发现影响外周带前列腺癌的危险因素为病灶的形态、患侧外周带体积、前列腺包膜、外周带与移行区分界。结论 T2 WI 上病灶的形态、患侧外周带体积及前列腺包膜是诊断外周带前列腺癌的独立危险因素。     

Multi-detector CT features of acute intestinal ischemia and their prognostic correlations

Acute intestinal ischemia is an abdominal emergency occurring in nearly 1% of patients presenting with acute abdomen. The causes can be occlusive or non occlusive. Early diagnosis is important to improve survival rates. In most cases of late or missed diagnosis, the mortality rate from intestinal infarction is very high, with a reported value ranging from 60% to 90%. Multi-detector computed tomography (MDCT) is a fundamental imaging technique that must be promptly performed in all patients with suspected bowel ischemia. Thanks to the new dedicated reconstruction program, its diagnostic potential is much improved compared to the past and currently it is superior to that of any other noninvasive technique. The increased spatial and temporal resolution, high-quality multi-planar reconstructions, maximum intensity projections, vessel probe, surface-shaded volume rending and tissue transition projections make MDCT the gold standard for the diagnosis of intestinal ischemia, with reported sensitivity, specificity, positive and negative predictive values of 64%-93%, 92%-100%, 90%-100% and 94%-98%, respectively. MDCT contributes to appropriate treatment planning and provides important prognostic information thanks to its ability to define the nature and extent of the disease. The purpose of this review is to examine the diagnostic and prognostic role of MDCT in bowel ischemia with special regard to the state of art new reconstruction software.     

Frequency and relevance of the "small-bowel feces" sign on CT in patients with small-bowel obstruction

OBJECTIVE: We sought to determine the incidence of the "small-bowel feces" sign (SBFS) in patients with small-bowel obstruction (SBO) and whether it can be used to accurately locate the point of obstruction. SUBJECTS AND METHODS: From November 2002 until March 2003, 34 consecutive adult patients with CT findings of small-bowel obstruction were prospectively evaluated. The CT findings used to diagnose small-bowel obstruction were a dilated proximal small bowel and a collapsed distal small bowel and colon. CT scans were evaluated to determine the degree of obstruction (mild, moderate, or high-grade), the presence or absence of the SBFS (defined as particulate-type material in the dilated small bowel), the location of the SBFS in relation to the transition zone, and the cause of the obstruction. Mild obstruction was defined as a slight discrepancy between the caliber of the proximal and that of the distal small bowel; moderate SBO was defined as a discrepancy of 50% or more between the calibers of the proximal and the distal small bowel; and high-grade SBO was considered to be present if the distal small bowel and the colon had collapsed. The cause of the obstruction was determined from surgical findings or a combination of CT findings, follow-up barium studies, and clinical assessment. RESULTS: The SBFS was present in 19 (55.9%) of 34 patients with SBO. The degree of SBO was mild in six, moderate in 11, and high-grade in 17 of the patients. The SBFS was present in one of the six patients (16.6%) with mild, eight (72.7%) of the 11 with moderate, and 10 (58.8%) of the 17 with high-grade SBO. In all patients in whom the SBFS was present, the particulate material could be traced to the point of transition and was most conspicuous in the transition zone. The length of fecallike material ranged from 2 to 25 cm and was longer in moderate and high-grade SBO than in mild SBO. The cause of the SBO was an adhesion in 20 patients, a hernia in four patients, Crohn's disease in four patients, a tumor in three patients, and other miscellaneous causes in three patients. CONCLUSION: When present on CT, the SBFS can be used to help locate the transition zone in patients with SBO. The sign is present more frequently in patients with moderate and high degrees of SBO.     

Gastrointestinal Emergency in Neonates and Infants: A Pictorial Essay

Gastrointestinal (GI) emergencies in neonates and infants encompass from the beginning to the end of the GI tract. Both congenital and acquired conditions can cause various GI emergencies in neonates and infants. Given the overlapping or nonspecific clinical findings of many different neonatal and infantile GI emergencies and the unique characteristics of this age group, appropriate imaging is key to accurate and timely diagnosis while avoiding unnecessary radiation hazard and medical costs. In this paper, we discuss the radiological findings of essential neonatal and infantile GI emergencies, including esophageal atresia and tracheoesophageal fistula, hypertrophic pyloric stenosis, duodenal atresia, malrotation, midgut volvulus for upper GI emergencies, and jejunoileal atresia, meconium ileus, meconium plug syndrome, meconium peritonitis, Hirschsprung disease, anorectal malformation, necrotizing enterocolitis, and intussusception for lower GI emergencies.     

Exercise-associated intestinal ischemia

Ischemic bowel disease exhibits a complex spectrum of clinical presentations and in the athlete the disease may be superimposed on dehydration, hyperthermia, and exhaustion. Physicians caring for athletes should be aware of the manifestations of ischemic bowel disease and the optimum methods of diagnosis and treatment. Abdominal pain and diarrhea are typical initial symptoms of ischemia and these symptoms generally limit further damage. However, symptoms may be overridden in cases of extreme athletic competition or other significant endurance events such as combat. Athletes and coaches should be aware of the danger of ischemic bowel disease. Patients or athletes with recurrent symptoms of abdominal pain and diarrhea during exercise may be at increased risk for ischemic damage. However, no underlying anatomic abnormalities have been noted. Ischemic hemorrhagic gastritis is generally reversible and may be controlled with effective acid blockade. Ischemic colitis generally presents with pain, diarrhea, and bleeding. It is usually mild but may require volume and transfusion support, rarely progressing to need for resection or stricture. Severe presentations with intestinal infarction are rare but potentially life threatening. The athlete is usually able to ultimately resume his or her activities without restriction.