Ultrasonography of Crohn disease in children

US is increasingly performed in Crohn disease (CD) in children as a first line imaging modality. It reduces the use of other more invasive examinations such as endoscopy, CT or contrast enema. We describe bowel ultrasonography technique, normal bowel appearances on US and pathological patterns in CD. We discuss the current role and limitations of bowel US in CD in children including diagnosis, extent of disease, assessment of disease activity, follow-up and detection of complications. The diagnostic accuracy of US is discussed according to the literature and compared to other imaging modalities. US is currently used for screening in children with the suspicion of inflammatory bowel disease (IBD) with a good negative predictive value. In follow-up, US has a role in monitoring medical treatment by evaluating disease activity, extent of disease and for detecting complications.     

MDCT angiography of pediatric vascular diseases of the abdomen, pelvis, and extremities

Multi-detector-row computed tomography (MDCT) enables rapid, noninvasive, high-resolution, and three-dimensional imaging of pediatric vascular diseases. In this paper, we explore the adaptation of the MDCT angiographic principles to pediatric patients for vascular diseases of the abdomen, pelvis, and extremities. Special emphasis is placed on the practical aspects of how to perform these studies. Optimizations of scan parameters, contrast medium usage, radiation dose, and three-dimensional image processing are discussed in detail. We provide practical guidance on how to choose between MR angiography and CT angiography. Finally, we review important pediatric vascular diseases, categorized into traumatic injuries, inherited vascular diseases, congenital vascular diseases, vasculitides, and surgical planning and assessment. In each category, we discuss how CT angiography can be tailored to maximize its clinical benefits.     

The place of interventional radiology in Crohn disease in children

The interventional radiologist plays an important role in the treatment of complicated Crohn disease in children. Interventional radiology includes percutaneous drainage of abscesses and insertion of suitable intravenous lines. A multidisciplinary approach is mandatory for the management of these patients.     

Pelvic MRI in children with Crohn disease and suspected perianal involvement

MRI is an important imaging tool in evaluation of adult and pediatric patients with Crohn disease. Pelvic MRI, in particular, has become the method of choice to evaluate for perianal fistulas and associated complications of Crohn disease. MRI can define the extent and location of perianal fistulas and abscesses, as well as provide critical information for operative management. In this pictorial essay, we describe useful MRI techniques for evaluation of perianal complications in pediatric patients with Crohn disease. We review pertinent anatomy and illustrate typical examples of perianal fistulas with and without abscess. We show one case of clinically suspected perianal fistula that was actually a pilonidal sinus.     

Orocaecal transit time in patients with Crohn disease

Orocaecal transit time (OCTT) using the lactulose hydrogen breath test was investigated in 16 patients (age 10–19.4 years) with active Crohn disease (CD). Disease activity was assessed by the paediatric CD activity index (PCDAI). OCTT was prolonged in all patients (mean 149.9 min, SD 32.7) relative to healthy age-matched controls (mean 56.9 min, SD 11.1). PCDAI was increased (median 48.8, range 32.5), indicating moderate to severe disease in all patients. A close correlation between OCTT and PCDAI (Spearmanr=0.90) was observed. Following nutritional therapy with a semi-elemental diet over a period of 6 weeks OCTT and PCDAI declined, OCTT becoming normal in 4 and PCDAI in 4/12 patients respectively. OCTT provides a sensitive, noninvasive method for the assessment and followup investigations in patients with CD.     

Isolated cerebral thrombo-embolism and Crohn disease

Arterial thrombo-embolism is an unusual complication in inflammatory bowel disease in adults and children. Cerebral arterial disease has been reported on only a few occasions. There are only two previous case reports of arterial disease occurring in a child with Crohn disease. However in both instances the arterial disease was part of a generalised Takayasu arteritis which resulted in multi-organ involvement. This report describes a 14-year-old boy who developed seizures before a histological diagnosis ofCrohn disease was made. These seizures were the result of a vascular lesion which was confined to the right middle cerebral artery.Conclusion Crohn disease as well as ulcerative colitis should be considered as a possible cause of cerebrovascular accidents in children     

CT and MRI of paediatric Crohn disease

Over the past two decades there has been considerable evolution in cross-sectional imaging modalities for the evaluation of Crohn disease (CD) in children. CT and MRI have contributed to conventional techniques so that now radiology has an even greater role in the management of CD, monitoring disease progression and detecting complications. The role of CT and MRI, their limitations, and the various imaging features that the radiologist should be aware of are discussed in this review.     

Crohn disease in systemic lupus erythematosus: a case report

A 15-year-old girl with systemic lupus erythematosus (SLE) in remission for 3 years showed abdominal pain and bloody stools. No evidence for exacerbation of SLE was obtained. An air-contrast barium enema examination and colonoscopy revealed findings typical of Crohn disease. Despite the rarity of the combination, patients with SLE showing gastrointestinal manifestations might need evaluation for Crohn disease.Abbreviation SLE systemic lupus erythematosus     

Crohn disease with sclerosing cholangitis and liver cirrhosis in adolescence

A 17-year-old patient suffering from Crohn disease (CD) and liver cirrhosis is presented. At an advanced stage of the disease, he died of a concomitant urosepsis. Autopsy showed that the liver cirrhosis was caused by sclerosing cholangitis. This very rare complication of CD in adolescence is discussed.     

Association of glycogen storage disease 1b and Crohn disease: results of a North American survey

Glycogen storage disease (GSD)-1b has been associated with neutropenia and abnormalities of neutrophil function. Many individuals with GSD-1b manifest chronic gastrointestinal inflammation. Our study was performed to precisely establish the type, frequency, and spectrum of gastrointestinal disease in patients with GSD-1b. We established a medical database of 36 individuals affected with GSD-1b, utilizing patients at our center, disease registries, and direct contacts with North American referral centers specializing in genetic disorders. Records were reviewed, focusing on documentation of gastrointestinal involvement. Gastrointestinal symptoms or manifestations were present in approximately 75% of the patients, including chronic abdominal pain and diarrhea, perianal fistula or abscess, and oral aphthous ulceration. Of patients identified, 28% had documented inflammatory bowel disease (IBD) and an additional 22% of patients had symptoms highly suggestive of IBD, but had not undergone comprehensive diagnostic evaluation. Gastrointestinal involvement was found to be indistinguishable from idiopathic Crohn disease (CD) by detailed review of clinical, radiologic, endoscopic and histopathologic findings. Many patients had significant improvement or resolution of gastrointestinal disease in response to G-CSF treatment. The expression of CD in patients with GSD-1b may be delayed or prevented by G-CSF treatment.Conclusion: a strong association exists between glycogen storage disease type 1b and inflammatory bowel disease. A high index of suspicion for Crohn disease should be applied in evaluating patients with glycogen storage disease type 1b and intestinal symptoms. These results provide further support for the hypothesis that some forms of inflammatory bowel disease may result from impaired mucosal innate immunity. Additional investigations into the intestinal disease in glycogen storage disease type 1b may be directly relevant to the etiology and treatment of idiopathic Crohn disease. Published online: 13 August 2002 On behalf of the North American Glycogen Storage Disease Study Group Members of the North American Glycogen Storage Disease Study Group: John Bellmont, Steve Cederbaum, Y.T. Chen, Peter Chenaille, Sechin Cho, George E. Hoganson, R. D. Holmes. Eric Hoover, Simon Horslen, James Keating, Douglas Kerr, Mark Ludman, Martin G. Martin, Dietrich Matern, Seymour Packman, Howard Parsons, Thomas Roe, Phil Rosenthal, C. R. Scott, Liz Shorey, Alfred E. Slonim, Charles Stanley, Micheline T. Ste-Marie, Robert D. Steiner, Holley Arp Swain, Steven Werlin, Joseph I. Wolfsdorf, and R. C. Woodman.     

In vitro release of eosinophil cationic protein from peripheral eosinophils reflects disease activity in childhood Crohn disease and ulcerative colitis

The aim of this study was to compare in vitro release of eosinophil cationic protein (ECP) from peripheral blood eosinophils during active phases of childhood Crohn disease (CD) and ulcerative colitis with phases of remission. Ten children with CD and nine children with ulcerative colitis were investigated during 55 and 56 clinical visits, respectively. Each patient was investigated during at least one phase of clinically active disease and one phase of remission. Disease activity was assessed by means of the Paediatric Crohn Disease Activity Index (PCDAI) in Crohn disease and according to the clinical activity index of Rachmilewitz in ulcerative colitis. On an intra-individual basis, in vitro ECP release was significantly higher (P < 0.001) during active phases of CD and ulcerative colitis than in phases of remission (CD:median: 24.5 μg/l, range 16.0–61.2 versus median: 5.7 μg/l, range 2.0–16.7; ulcerative colitis: 14.8 μg/l, 8.3–39.8 versus 4.9 μg/l, 2.0–9.9). On an inter-individual basis, in CD and ulcerative colitis a strong and highly significant correlation was observed between disease activity indices and in vitro release of ECP from peripheral eosinophils (r = 0.89, P < 0.0001 and r = 0.82, P < 0.0001, respectively). Conclusion These results show that in vitro ECP release from peripheral eosinophils reflects disease activity in both CD and ulcerative colitis and therefore can be used as a new appropriate laboratory parameter for assessment of disease activity in chronic inflammatory bowel disease. Received: 26 October 1996 and in revised form: 8 April 1997 / Accepted: 15 April 1997     

Magnetic resonance imaging of the small bowel in children with idiopathic inflammatory bowel disease: evaluation of disease activity

Background  Examinations using ionizing radiation are frequently used in the evaluation of disease activity in children affected by idiopathic inflammatory bowel disease (IBD). Objective  To develop an MR imaging protocol without the need for fluoroscopic insertion of an enteral tube and to assess the disease activity in children with IBD. Materials and methods  Included in the study were 37 children (22 girls and 15 boys; age range 7–15 years, mean 11.67 years) with IBD who underwent MR imaging of the small bowel. Of these 37 children, 32 had Crohn disease and 5 had indeterminate colitis. A water solution containing herbal fibres was administered orally or through a nasogastric tube. Patients were imaged on a 1.5-T MR scanner with T1-weighted and Τ2-weighted sequences followed by a dynamic study using 3-D T1-W images after intravenous administration of gadolinium. Results  The percentage enhancement of the bowel wall was significantly increased in patients with abnormal C-reactive protein (CRP) values compared to patients with CRP values in the normal range (P<0.001). A relatively weak but significant correlation between percentage enhancement of the bowel wall and CRP values was noted during all phases of enhancement. Conclusion  This MR imaging protocol is a safe and well-tolerated method for evaluating disease activity and extraintestinal manifestations of IBD in children.     

Scatter and transmission doses from several pediatric X-ray examinations in a nursery

Background While several studies have investigated the dose from scattered radiation from X-ray procedures in a pediatric nursery, they examined scatter from chest procedures only, or the types of examination were not specified.Objective The aim of this study was to collect scatter and transmission data from several types of X-ray examinations.Materials and methods Using a "newborn" anthropomorphic phantom and an ion chamber, a series of scatter and transmission dose measurements were performed using typical exposure factors for chest, chest and abdomen, skull, skeletal long bone and spine procedures. The phantom was inside a crib for all exposures.Results The maximum scatter dose measured at 1 m from the field center was about 0.05 µGy per exposure for lateral skulls. Transmission doses for lateral exams were around 0.1 µGy per exposure at 1 m from the isocenter.Conclusions The study demonstrated that scatter dose to other patients in a neonatal unit is not significant, assuming the distance between adjacent cribs is in the order of 1 m. Transmission doses are also low provided the beam is fully intercepted by the cassette. For an average workload the dose received by imaging technologists would be small.     

Surgical treatment of Crohn disease in children and adolescents; how conservative can the paediatrician be?

Thirty-eight children (21 male, 17 female, age 3–18 years), treated for Crohn disease in two Dutch university centres, were retrospectively studied in order to evaluate the results of conservative treatment and to find out in what way surgical treatment in this age group may have differed from treating adults with this disease. Both groups had an equal distribution of age and sex. Diarrhoea with discharge of blood and mucus, abdominal pain, nausea/vomiting, weight loss, fever and general discomfort were the most frequent presenting symptoms. Twenty-three children (60%) showed signs of malabsorption; 4 children (10%) had growth retardation. In 27 children (70%), 63 surgical procedures were performed (2.4 operations per child). There was no surgical mortality. Most operations were performed for ileocolitis and colon-only localizations needed most re-operations. Of the surgical procedures performed, 55% were excisional procedures. Already 3 years after the onset of symptoms, 50% of all children had had their first resection, whereas in adults, 50% of the patients undergo surgery 8 years after disease onset. Eight children were treated with split ileostomy. In only one of these children, operated for non-toxic colitis and severe steroid-dependent growth retardation, could the colon eventually be saved. The time between the onset of symptoms and the first operation seems to be shorter in children compared to adults. Severe malabsorption and growth retardation are additional specific indications for surgery for Crohn disease in childhood. The latter combined with non-toxic colitis, may perhaps be the only indication left for performing split ileostomy in Crohn disease.     

Assessment of coronary artery aneurysms in paediatric patients with Kawasaki disease by multidetector row CT angiography: feasibility and comparison with 2D echocardiography

Background Transthoracic ECHO is the locally accepted method for coronary surveillance of patients with Kawasaki disease but it may have limited visualization in the older child. Objective To assess the feasibility of multidetector CT (MDCT) angiography in the follow-up of coronary artery aneurysms in children with previous Kawasaki disease. Materials and methods Six children (5 boys, 1 girl; mean age 11.5 years) with known Kawasaki disease and coronary artery involvement underwent CT coronary angiography using 16-detector MDCT. The visualized lengths and diameter of all coronary segments were measured. The number, size and location of coronary artery aneurysms were recorded and compared with recent ECHO. Results Twelve coronary artery aneurysms (seven saccular, five fusiform) were identified by MDCT angiography. One saccular aneurysm at the junction of the distal right coronary artery and posterior descending artery was not detected by ECHO while the remaining six in proximal segments were detected by both modalities. Two of five fusiform aneurysms were not detected by ECHO due to their small sizes. Excellent agreement was found between CT and ECHO for maximal diameter and length of the visualized aneurysms. Conclusions MDCT angiography accurately defines coronary artery aneurysms. It is more sensitive for detecting aneurysms at distal coronary segments and fusiform aneurysms of small size. Presented at the 5th International Pediatric Radiology Conjoint Meeting, Montreal, Canada, May 2006.     

Enteroclysis in older children and teenagers

Background

Enteroclysis (EC) has been widely and successfully used for evaluation of the small bowel in adults for about 30 years. However, despite recently improved intubation and examination techniques, in many paediatric radiology centres it is still not the preferred conventional barium study for the evaluation of small bowel pathology in children.

Objective

To share our 10 years of experience and review the feasibility of EC in 83 older children and teenagers, in terms of both technique and pathological findings.

Materials and methods

Between 1996 and 2006, EC was performed by the standard technique described by Herlinger to 83 children between 7 and 18 years of age. The indication for the study was jointly decided by the paediatric radiologist and the clinician. None of the examinations was converted to follow-through studies because of patient refusal or technical failure. Morphological changes, mucosal abnormalities, luminal abnormalities, perienteric structures, the location of the disease, indirect findings regarding the bowel wall and functional information were evaluated.

Results

All the children tolerated the procedure without difficulty. Out of 83 patients, 63 had abnormal findings. The spectrum of diagnoses were Crohn disease (n?=?23), nonspecific enteritis (n?=?10), malabsorption (n?=?8), intestinal tuberculosis (n?=?6), intestinal lymphoma (n?=?5), Peutz-Jegher syndrome (n?=?3), adhesions (n?=?2), Behçet disease (n?=?2), back-wash ileitis due to ulcerative colitis (n?=?2), common-variable immune deficiency (n?=?1) and lymphangiectasis (n?=?1).

Conclusion

EC can easily be performed in children over 7 years of age and when performed using a correct technique it shows high diagnostic performance without any complications in the evaluation of small bowel diseases in older children and teenagers.

     

儿童炎症性肠病治疗现状和进展

炎症性肠病是一种多因素的肠道慢性炎症性疾病,主要分为溃疡性结肠炎、克罗恩病和未定型炎症性肠病.炎症性肠病的发病率逐年增加,并且呈现低龄化的趋势,儿童炎症性肠病越来越普遍.由于儿童正处于生长发育较快的特殊时期,导致儿童炎症性肠病与成人在临床表现、疾病类型、治疗方案、并发症等问题上有很多不同.该文对炎症性肠病尤其是儿童炎症性肠病的治疗现状和进展进行阐述.     

克罗恩病和健康儿童血清细胞因子水平的病例对照研究

目的 观察克罗恩病（CD）患儿血清细胞因子水平,探讨细胞因子水平紊乱在儿童克罗恩病发病中的作用。 方法 纳入2013年1月至2015年12月在温州医科大学育英儿童医院（我院）儿内科住院的确诊CD患儿（病例组,根据CD活动度分为不同亚组）和同期在我院门诊体检的健康儿童,采用流式分析技术检测晨起空腹静脉血血清中IL-2、IL-4、IL-6、IL-10、TNF-α、IFN-γ和IL-17A的水平。 结果 病例组32例,男18例,女14例,年龄（8.5±4.3）岁,其中CD轻度活动亚组12例,CD中重度活动亚组20例;对照组30名,男18名,女12名,年龄（10±3.5）岁。病例组和对照组年龄、性别比较差异无统计学意义（P>0.05）。病例组患儿血清IL-2、IL-4、IL-6、TNF-α和IL-17A水平高于对照组,血清IL-10和IFN-γ水平低于对照组,差异均有统计学意义（P＜0.05）。此外,根据儿童CD活动指数（PCDAI）评分为中重度活动亚组患儿的血清IL-2、TNF-α和IL-17A水平高于轻度活动亚组患儿,差异有统计学意义（P＜0.05）。 结论 与健康儿童相比,CD患儿存在明显的免疫相关细胞因子水平变化,而且疾病活动度不同其细胞因子水平亦有差异,提示细胞因子水平紊乱在儿童CD发病及病情的发展中有重要作用。     

儿童克罗恩病诊断和治疗的10年临床经验总结

目的分析儿童克罗恩病（Crohn’s disease,CD）临床特点、总结10年来的诊治经验,促进对儿童CD的认识,提高诊断治疗水平。方法对1996至2005年诊断为CD的9例患儿的临床资料及诊治结果进行分析。结果本组9例,男8例,女1例,年龄6—13岁。平均病程10个月。病变单独累及小肠者3例,小肠和大肠同时受累者6例。腹痛和腹泻是最常见的胃肠道症状。主要的肠道外表现为体重下降、低蛋白血症、轻度贫血、发热和低钙血症。结肠镜下主要的病变有溃疡、节段性病变、鹅卵石样改变、多发性息肉和肛周病变。1例行胶囊内镜检查见典型纵形裂隙样溃疡和鹅卵石样改变。内镜活检组织病理检查以非特异性黏膜慢性炎症为主,2例见非干酪样肉芽肿（22．2％）。2例手术标本均未检出非干酪样肉芽肿。7例患儿予5氨基水杨酸（5-ASA）口服,部分加用抗生素和营养治疗,2例加用肾上腺皮质激素。现3例患儿维持长期缓解。结论儿童CD诊断需特别强调排除感染性腹泻。CD患儿常见的体重下降和营养障碍可为诊断提供重要线索。合适的剂型和较大剂量的5-ASA[30—50mg／（kg·d）]对诱导和维持缓解有较好疗效。