成人先天性巨结肠症的X线表现与病理对照分析(附4例报告)

探讨成人先天性巨结肠症的临床、Ｘ线表现特点及其与病理的关系。材料与方法，回顾性分析４例ＡＨＤ的临床、Ｘ线表现并与病理学所见进行对照。结果全部病全 有慢性便秘症状、Ｘ线表现与小儿Ｈｉｒｓｃｈｐｒｕｎｇ’ｓ病相仿，惟前者结肠狭窄段较短。     

Radiology in chronic constipation in childhood

One hundred thirteen children referred for chronic constipation were examined by means of diagnostic work-up including anal inspection, rectal exploration, weekly bowel frequency evaluation, measurement of total and segmental intestinal transit times (TITT, SITT), contrast enema, anorectal manometry (ARM), suction rectal biopsy for histochemistry. Final diagnosis were: chronic functional "simple" constipation in 53 children; chronic functional constipation and soiling in 32; Hirschsprung's disease in 18. In 10 children, initially referred for constipation, TITT was in the normal range so they underwent no further examination. Conclusions are that bowel frequency identifies a real gastrointestinal problem, but definite diagnosis of constipation is relied on TITT. In the assessment of chronic constipation nature, ARM is more sensitive than radiology. Suction rectal biopsy is reliable in detection of aganglionosis: its accuracy can be improved by histochemical or biochemical determination of Acetylcholinoesterase.     

Bowel perforation in Hirschsprung's disease

A review of the medical records of 45 infants less than 1 year old with Hirschsprung's disease identified two (4.4%) who presented with bowel perforation. A literature survey was done to evaluate the relationship between bowel perforation of the bowel early in the course of Hirschsprung's disease indicates that: the infant under 4 months old is at the greatest risk; the majority of cases (62%) were associated with long-segment or total colonic Hirschsprung's disease; the most common sites of perforation were the proximal colon (68%) and appendix (17%); in cases with a short or intermediate length of aganglionic bowel, the perforation was proximal to or at the site of transition, but in 84% of infants with total colonic aganglionosis the perforation was situated in aganglionic bowel. Hirschsprung's disease should be a prime differential consideration in a young infant with penumoperitoneum caused by distal bowel perforation.     

Bilateral hydrosalpinx in adolescent girls with Hirschsprung's disease: association of two rare conditions

OBJECTIVE: The purpose of our study was to illustrate three cases of bilateral hydrosalpinx in postpubertal girls operated on for Hirschsprung's disease and to discuss the possible cause: iatrogenic or congenital. We identified bilateral hydrosalpinx in three postpubertal sexually inactive girls with Hirschsprung's disease treated, respectively by Duhamel, Soave pull-through, and Martin procedures. No history of surgical complications or pelvic inflammation had been reported. CONCLUSION: Hirschsprung's disease is rare in girls and bilateral hydrosalpinx is also extremely uncommon in sexually inactive adolescents. We think there may be a possible common cause: either a postsurgical complication or a congenital defect of the autonomous innervation in the context of a neurocristopathy. Because of the rarity of both conditions, the association is unlikely to be coincidental. The cause of this association is unclear and further studies are required to find its prevalence and to estimate the possible impact on fertility.     

出口梗阻型便秘的外科治疗

目的　探讨出口梗阻型便秘 (outlet obstruction constipation,OOC)的诊断方法、手术指征及术式选择。方法　本组65例均经肛门指诊、排粪造影和结肠传输试验检查 ,术前获得诊断 :直肠前膨出 40例 ,直肠粘膜内脱垂或内套叠 42例 ,肛门内括约肌失弛缓症 2 3例 ,耻骨直肠肌肥厚 2 1例 ,盆底痉挛综合征 9例 ,盆底下降 9例 ,骶直分离 1 3例 ;根据检查结果分别采用 :直肠前膨出修补术、直肠粘膜固定术、直肠粘膜部分切除加固定术、肛门内括约肌部分切除术、耻骨直肠肌部分全切、耻骨直肠肌部分全切加闭孔内肌自体移植术。结果　治愈 5 9例 (90 .8% ) ,好转 4例 (6.1 % ) ,无效 2例 (3 .1 % )。结论　对出口梗阻型便秘患者必须进行肛门指诊、排粪造影和结肠传输试验三项基本检查 ,根据这三项检查一般都能明确诊断。手术疗效确切 ,但术式选择应根据三项具体检查结果而定     

Imaging of constipation in infants and children

The aims of this review article are to present epidemiology, important definitions, clinical considerations, and etiologic and pathogenetic aspects of constipation in infants and children. Anatomy, physiology, and pathophysiology of the the anorectum are described. Special attention is given to the indications for diagnostic imaging, imaging techniques, and imaging findings with different causes of constipation. Other diagnostic modalities, such as anorectal manometry, electromyography, and biopsy techniques are briefly discussed. The central question as to whether diagnostic imaging is needed for the diagnostic workup of infants and children suffering from constipation can be answered affirmatively. Especially the combination of barium enema or defecography and anorectal manometry allows definition of those infants and children who do not need biopsy and surgery for Hirschsprung's disease. The special role of defecography in this context is underlined. Received 16 May 1997; Revision received 5 August 1997; Accepted 6 August 1997     

The diagnostic role of abdominal CT imaging findings in adults intussusception: focused on the vascular compromise

Intussusception is defined as telescoping of one segment of the gastrointestinal tract into an adjacent one. Unlike that in children, adult intussusception is a relatively rare condition. More than 90% of patients with adult intussusception have been reported to have an organic cause, with benign or malignant tumors for accounting for approximately 65% of the cases. In general, the diagnosis is easily made by means of computed tomography (CT) or magnetic resonance (MR) imaging. The imaging appearance of a bowel-within-bowel configuration with or without contained fat and mesenteric vessels, is pathognomonic. As the intussusceptum enters into the intussuscipiens, the mesentery is carried forward and trapped between the overlapping layers of bowel. The twisting or severe constriction of the mesenteric vessels may result in vascular compromise with subsequent edematous thickening of the involved bowel. In these circumstances, ischemic necrosis may develop if timely intervention is not undertaken. Therefore, determination of the presence or absence of intestinal necrosis in intussusception is important in patient management. On CT, the presence of well-known diagnostic CT criteria for strangulated obstruction (especially severe engorgement or twisting of the mesenteric vessels) as well as evidence of loss of the layered pattern, accumulation of extraluminal fluid collection, and bowel perforation, may suggest the diagnosis of intestinal necrosis. CT and MR imaging are limited in determining the primary disease causing intussusception. However, CT and MR provide excellent pre-operative evaluation, including the possible extension and/or dissemination of a malignant tumor. CT and MR imaging may also be useful in suggesting the presence of vascular compromise.     

Rotatory subluxation of the scaphoid

Rotatory subluxation of the scaphoid is an uncommon abnormality which is not widely understood. It can be difficult to diagnose and, if incorrectly managed, can lead to a severe and disabling radio-carpal arthritis. While complicating many forms of wrist trauma, its place in the lunate-perilunate dislocation spectrum being especially important, the abnormality may occur in the absence of trauma, such as with rheumatoid arthritis; its association with Kienb?ck's disease is documented in this paper. The mechanisms underlying the subluxation are discussed and the importance of early diagnosis is stressed. Eight cases of rotatory subluxation of the scaphoid are reported to illustrate the presentations, symptomatology and complexities of diagnosis.     

Antral mucosal diaphragms in adults.

A group of 53 patients with the diagnosis of antral mucosal diaphragm is reported. In over 50% of the cases the diaphragm was considered an asymptomatic lesion. In addition, an autopsy study in 29 unselected cases is reported with demonstration of five antral mucosal diaphragms in different forms. On the basis of these findings, it is evident that the antral mucosal diaphragm in the adult is not an acquired lesion secondary to peptic ulcer disease, but may be congenital. In addition, techniques of radiologic evaluation of the diaphragm for significant narrowing of the central aperture using the compressed barium tablet, and for significant gastric outlet obstruction using the "barium burger" examination, are described.     

The distended neonate

A prospective study, followed by a retrospective audit, was made of the radiographs and clinical features of 225 consecutive neonates with abdominal distension presenting during a period of 4 years. More than one major cause for the distension was found in a third of cases. Excluding cases of ano-rectal atresia, diagnostic difficulties were encountered in 31% of cases and, occasionally, the diagnosis was revised several times as investigations proceeded. Neuhauser's sign of 'bubbly' meconium is unreliable, being found in cases of meconium ileus, ileal atresia, Hirschsprung's disease and necrotising enterocolitis. Concomitant small-bowel atresia should be suspected in all cases of meconium ileus; intestinal malrotation should be considered in association with duodenal and intestinal atresias. In the absence of an obvious anatomical defect, it is difficult to differentiate between the various causes of functional obstruction by radiological means alone and all such infants should be referred to a specialised centre for intensive investigation and treatment. Very often, the skills of a neonatologist, paediatric surgeon and, especially, an experienced pathologist will be required in addition to those of the radiologist.     

Congenital aganglionosis of the entire colon in neonates.

A report of six cases and their radiological diagnosis in neonates. Total colonic aganglionosis (TCA) is a variety of what is commonly known as long segment Hirschsprung's disease. This is not a rare condition, but is often unrecognized. It has a high mortality, frequently with a complicating enterocolitis. Aganglionosis of some part of the colon should be suspected in all babies who show obstructive plain film changes. In TCA the barium enema changes may easily be passed as unremarkable. However, free ileal reflux during the examination, with a transition point in the ileum, and retention of barium in the entire colon after the examination, are diagnostic.     

Neuroradiologic abnormalities in Marchiafava-Bignami disease of benign evolution.

Marchiafava-Bignami disease has been recognized since 1903, but only recently has it become possible to achieve a probable diagnosis before death occurs. Imaging of the central nervous system with MR and CT have contributed significantly to such a diagnosis. Two cases of the disease are reported in patients, aged 33 and 59 years, with benign evolution of neurologic symptoms the diagnosis was confirmed by neurologic imaging with MR and CT and both were studied with evoked responses. Reversibility of the disease and possible prognostic indicators in these and other patients reported in the literature are discussed. The important role that diagnostic procedures, especially MR imaging, play in the management of this disease is emphasized.     

Colonic contractions and diverticular disease

Contractions of the left colon, augmented by ceruletide, a drug which mimics the postprandial production of cholecystokinin, were studied fluoroscopically on single and double contrast barium enema in patients with diverticular disease (with and without constipation) and, as a control, in patients without abnormalities of the colon (with and without constipation). Patients with diverticular disease showed contractions more often than controls. Propulsion showed a difference between controls and patients, but this difference did not reach statistical significance. However, propulsion was statistically significantly more often seen in subjects without constipation. Compared with controls without constipation, segmentation and retropulsion occured more often in patients, in constipation, and in patients without constipation. Segmentation, but not retropulsion, occured more often in constipated patients than in constipated controls. These findings suggest that segmentation is of more importance than retropulsion in diverticular disease and that both retropulsion and segmentation are of importance in constipation.     

The role of ultrasound and isotope scanning in the management of irritable hips.

Patients (n = 181) with the irritable hip syndrome were reviewed. Four of these were found to have Perthes disease and 3 cases had septic arthritis. Ultrasonography provides accurate information as to the presence or absence of an effusion in children with an irritable hip syndrome. The likelihood of a positive result is higher in the early course of the disease process (i.e. within 3 days). Bone scanning, if done routinely will help in the early diagnosis of Perthes disease. Recurrence of the symptoms occurred in 18% of patients and most of them were within 12 months of the first onset of symptoms.     

骨纤维异常增殖症X线诊断(附35例分析)

目的:探讨骨纤维异常增殖症的X线表现特征并阐明其鉴别诊断要点。材料与方法:本文报道了35例经病理证实的骨纤维异常增殖症,其中,单骨型29例,多骨型6例;男19例,女16例;年龄于4～49岁,其中30岁以下者30例(86％)。结果:骨纤维异常增殖症X线表现特征大致可分为四种类型:1)囊状膨胀性改变;2)磨玻璃状改变;3)栅栏状改变;4)虫蚀样改变。另外,病变好发于四肢长骨(32/35)。病理性骨折发生5例,全部见于5岁以下儿童,且位于骨干侧的骨皮质。结论:本病依据其X线特征通常不难作出诊断,但在某些情况下,需与骨肿瘤、非骨化性纤维瘤、韧带性纤维瘤,以及骨囊肿等加以鉴别。     

排粪造影对功能性出口梗阻便秘的诊断

目的　探讨排粪造影对功能性出口便秘的诊断价值。方法　对 5 0 0例功能性出口便秘患者进行排粪造影检查。女性 3 49例 ,男性 15 1例 ,年龄 14～ 78岁 ,平均 3 6.7岁。病史在 2个月～ 3 8年。主要临床症状 :排便困难、不适、腹痛、腹胀 ,排便时间延长 ,肛门坠胀及解不净 ,多数病人需长期服泻药及灌肠帮助排便。排粪造影是给病人灌入 40 0～ 60 0ml硫酸钡混悬液 ,病人侧坐在排便装置上 ,在X线电视下作动态观察、摄片。结果　通过对 5 0 0例患者排粪造影检查 ,发现有直肠前突 14 4例、直肠黏膜脱垂 75例、直肠内套叠 5 6例、盆底痉挛综合征 10 2例、耻骨直肠肌肥厚 65例、会阴下降 12 1例、正常 2 9例 ,以上症状多数由两种或两种以上同时出现。结论　排粪造影对功能性出口便秘的诊断具有重要价值。     

Chronic recurrent multifocal osteomyelitis

Chronic recurrent multifocal osteomyelitis is a recognized condition that usually affects children and adolescents. It generally has a self-limited course, causing significant symptoms but leaving few, if any, residual changes in most cases. The authors undertook a retrospective review of the radiographs and medical records of 11 patients with chronic recurrent multifocal osteomyelitis. They found that not all patients have complete resolution of disease, and a small minority may be left with residual bone changes. Chronic recurrent multifocal osteomyelitis was originally reported in Switzerland; most reported cases are from Europe and Scandinavia. The disease is being recognized with increasing frequency in North America.     

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.     

Nekrotisierende Enterokolitis (NEC) beim Neugeborenen

Necrotizing enterocolitis (NEC) is a disease of the premature neonate that requires early therapy, sometimes even surgery and therefore early diagnosis. In general, plain radiography of the abdomen and sonography are valuable imaging techniques for diagnosis of NEC. COURSE OF NEC: The disease starts with distension of small bowel loops. Furthermore the children develop pneumatosis in the bowel wall, which is a nonspecific pattern. If the NEC persists, the air passes into the portal vein system, causing a pneumoportogram. Finally, the disease can lead to perforation. In doubtful cases, the plain radiogram must be repeated within a period of 6 h. With sonography distension of the bowel, thickening of and pneumatosis in the bowel wall, the pneumoportogram and free intraperitoneal fluid can be easily depicted. The most common complications of NEC are intestinal stenosis and strictures that can lead to ileus. DIFFERENTIAL DIAGNOSIS: Focal perforation of ileum, volvulus and Hirschsprung's disease are some of the differential diagnoses.     

Agenesis of the right lobe of the liver

In five patients with agenesis of the right lobe of the liver, computed tomography or sonography demonstrated absence of the right lobe with compensatory hypertrophy of other segments. In three patients, the caudate lobe was also absent. The radiologic differential diagnosis includes lobar atrophy due to cirrhosis or hilar cholangiocarcinoma. Nineteen other cases have been reported in the world literature since 1956. This interesting congenital anomaly may be associated with biliary tract disease (12 patients), portal hypertension (seven patients), and other congenital anomalies (four patients), or it may be an incidental finding (five patients).