Radiographic reduction of intussusception in patients with cystic fibrosis

Cystic fibrosis (CF) is a genetic disorder affecting approximately one in 2,500 births in the United States. Nearly 1% of patients with CF will develop intussusception, which is commonly ileocecal and felt to be secondary to inspissated feces. These patients generally present before the age of ten. Once the diagnosis of intussusception is confirmed on ultrasound or CT, surgery has been the mainstay of treatment in this patient group. We propose the use of air and contrast enemas as effective and beneficial non-operative approach in these patients. Clinical and imaging findings in four children with known CF who presented with intestinal intussusception, average age 13.25 +/- 5.3 years (range 8-18 years) were reviewed. Patients were diagnosed using ultrasound (n = 3) or abdominal CT (n = 1). All patients suffered from an ileocolic intussusception. Air and Gastrografin(R) enemas were used in an attempt to reduce the intussusception. There were six separate successful intussusception reductions in four patients. Three patients required multiple attempts (2.3 +/- 0.6). Air enemas were used initially in all cases. Gastrografin(R) was used successfully following the failure of air enema in one patient. One patient suffered three separate intussusceptions over a period of 18 months, which were all successfully reduced using air. There were no complications and the patients tolerated the procedure well. Intussusception is an uncommon but serious complication in children with CF. While surgical reduction has been the mainstay of treatment for these patients, we demonstrated that reduction of an intussusception using air or contrast can be accomplished safely, without anesthesia, and should be the initial treatment option.     

婴儿阑尾盲肠多重套叠并急性阑尾炎一例并文献复习

目的 探讨婴儿期阑尾盲肠多重套叠并急性阑尾炎的临床病理和诊治方法.方法 回顾性分析1例阑尾盲肠多重套叠并急性阑尾炎8月龄婴儿的临床资料,并通过PubMed、ProQuest、中国知网(CNKI)、万方和维普数据库中检索2016年5月前的报道并复习相关文献资料,对各类儿童阑尾套叠进行系统性综述.结果 本例阑尾盲肠多重套叠并急性阑尾炎经手术诊断,整复套叠后切除阑尾并经病理证实,术后顺利康复.共检索到215例阑尾套叠中儿童52例,阑尾盲肠套叠136例,仅3例阑尾自身套叠中有2例为儿童;检索到肠套叠并阑尾炎66例,儿童59例,其中小于1岁的患儿27例,回结型肠套叠43例,此外,检索到回肠盲肠型套叠7例和盲肠盲肠型套叠并阑尾炎1例均为成人患者,所有病例均行手术治疗而痊愈.儿童阑尾盲肠多重套叠并急性阑尾炎者未见报道.结论 婴儿阑尾盲肠多重套叠并急性阑尾炎罕见,如套叠未导致回盲瓣排空完全梗阻则无典型症状体征,诊断困难,易误、漏诊.因此,对经保守治疗不能解除肠梗阻且不能解释其原因时,只要B型超声提示“同心圆征”或“套筒征”就应果断行手术探查以明确诊断并及时治疗.     

Intussusception of the vermiform appendix: a preoperative diagnosis in an adolescent girl

Intussusception of the vermiform appendix (IVA) in a 14-year-old girl is reported. The diagnosis was made preoperatively, which is rare: only five other cases have been reported. IVA can present with variable symptoms. This patient presented with episodes of recurrent severe paroxysmal pain and vomiting, each episode being separated by several uneventful weeks. The diagnosis of IVA could be made at the fourth attack of colic by the radiological visualization of a "spiral shell" filling defect at the bottom of the cecum that was reducible by intravenous injection of a spasmolytic. Laparotomy with appendectomy rescued the patient from these distressing episodes. Although IVA is an uncommon condition, one should be aware of its existence and include it in the differential diagnosis of acute abdominal syndromes. When confronted with a clinical picture of intussusception in older children or adolescents, one should especially remember this entity as a differential diagnosis with other causes of intussusception, especially tumors.     

Delta-F508 cystic fibrosis mutation is not linked to intussusception: implications for rotavirus vaccine

Objective: To determine whether the cystic fibrosis (CF) delta-F508 gene mutation predisposes to intussusception, and so selects infants who should not receive rotavirus vaccine. METHODS: Stored neonatal screening blood spots, retrieved from 81 infants with intussusception and from 79 controls, were tested for the presence of the CF delta-F508 gene mutation. RESULTS: Prevalence of the mutation was similar in blood specimens from intussusception patients and from controls. Conclusion: Testing for the CF delta-F508 mutation at birth is unlikely to identify infants predisposed to intussusception, and therefore is not relevant to rotavirus vaccine programs.     

Cecal polyp and appendiceal intussusception in a child with recurrent abdominal pain: diagnosis by colonoscopy

A 7-year-old boy developed recurrent abdominal pain. He was eventually discovered to have an inverted appendix via colonoscopy. At surgery, a polyploid intracecal mass was palpated at the base of the partially invaginated appendix. Resection of the cecal mass (histologically, a juvenile polyp) and appendix was easily accomplished. The types of clinical presentation and treatment of children with appendiceal intussusception are discussed.     

A right lower quadrant mass in cystic fibrosis: A diagnostic challenge

In a cystic fibrosis (CF) patient a right lower quadrant (RLQ) mass may be a difficult diagnostic problem. Most frequently it is due to a distal intestinal obstruction syndrome [8, 12] also called meconium ileus equivalent [11], but the possibility of intussusception and appendiceal abscess should also be considered. We describe three CF patients with an appendiceal abscess seen in a 4-year period. All three patients had a palpable RLQ mass. Chronicity and obliteration of the appendiceal lumen with abnormally viscid mucus may lead to concealed perforation and be responsible for the atypical presentation.     

小儿肠套叠合并肠坏死的超声表现及诊断价值

目的：肠套叠合并肠坏死是空气灌肠复位的绝对禁忌症,目前对小儿肠套叠合并肠坏死的超声检查表现及特点报道较少,该研究重点探讨其超声表现,及时指导临床医生正确选择复位方式。方法：回顾性分析2004~2006年手术复位的48例肠套叠合并肠坏死病例的超声图文资料。结果：①肠套叠的类型与肠坏死密切相关,回回结型最易导致肠坏死;②套叠部肠壁明显增厚,套叠中心部常伴有肿大的淋巴结、肿大的阑尾;③肠套叠内积液、套叠远端肠管扩张伴肠壁增厚、肠蠕动减弱或消失是肠壁大片坏死的表现;④继发性肠套叠也是导致肠坏死的一个重要因素,部分可探及原发病灶的声像;⑤腹腔积液是所有肠套叠伴肠坏死的共同表现。结论：当肠套叠伴肠坏死时有一些较为明显的声像图特征,可间接提示肠坏死的可能,掌握这些超声表现能及时指导临床医生正确选择复位方式。     

Adenoviral appendicitis presenting clinically as acute appendicitis.

The vermiform appendix may react as part of a generalized viral infection, but histological documentation of appendiceal viral infection is rare. Adenovirus has been described in association with mesenteric adenitis and ileocecal intussusception, but to our knowledge there are no well-documented cases of adenoviral appendiceal infection presenting clinically as acute appendicitis without intussusception. We reviewed the pathology records of all appendectomies performed at our institution from 2001 through 2005. All incidental appendectomies and appendices with acute appendicitis or other pathological findings were excluded. We selected all negative appendices with lymphoid hyperplasia and reviewed hematoxylin and eosin-stained slides. Representative sections of each of these cases were immunostained with adenovirus antibody. Eight hundred seventy-seven appendectomies were performed during the study period. Of these, there were 94 cases that had a clinical diagnosis of acute appendicitis and that were pathologically negative. Sixty-three of the 94 cases had lymphoid hyperplasia and were stained for adenovirus. We identified 2 positive cases, which also showed epithelial proliferation and viral inclusions. One involved a 6-year-old male and the other involved a 5-year-old female, without intussusception. Adenovirus can infect the appendix and clinically mimic acute appendicitis without intussusception. We recommend that all negative appendices be evaluated for lymphoid hyperplasia and epithelial viral changes and possibly be stained with immunoperoxidase staining if indicated. We speculate that adenovirus may play a role in the pathogenesis of acute appendicitis.     

小肠缺血再灌注损伤在肠套叠患儿的临床观察

目的 观察肠套叠患儿血清一氧化氮（NO）浓度和肠黏膜细胞凋亡的相关性，分析其对临床症状及预后的影响。方法急性单纯性阑尾炎组为对照，检测术前血清NO和超氧化物歧化酶（SOD）浓度；组织切片黏膜细胞TUNEI。染色阳性细胞数和caspase-3平均灰度值。结果 肠套叠组血清NO浓度明显升高（P〈0．05）；血清SOD浓度与对照组无差异。阑尾组织匀浆MDA含量高于对照组；肠套叠组阑尾黏膜内TUNEI。染色阳性细胞数、caspase-3平均灰度值均高于对照组（P〈0．05）。肠套叠组血清NO浓度与术前血白细胞计数呈正相关（r=0．82，P〈0．001）。白细胞及血清NO浓度均升高的患儿，术后恢复相对较慢，高热持续时间延长，腹泻、腹胀等并发症增加。结论 肠套叠相关的缺血再灌注损伤伴随血清NO浓度升高，与术前血白细胞计数呈正相关；肠黏膜损伤及细胞凋亡程度影响患儿术后恢复。     

急性肠套叠手术脱套后再灌注损伤

选择２５例经手术治疗的急性肠套叠患儿的受累阑尾，测定其组织脂质过氧化物（ＬＰＯ）含量，并以１４例择期剖腹手术患儿的正常阑尾作为对照。手术前和术后１２ｈ分别测定两组患儿血中乳酸脱氢酶（ＬＤＨ）、肌酸激酶（ＣＫ）活性及内毒素（ＥＴＸ）含量。结果急性肠套叠组的ＬＰＯ含量显著高于对照组（Ｐ＜０．０５），ＬＤＨ、ＣＫ活性及ＥＴＸ浓度术后较术前明显升高（Ｐ＜０．０５）。表明急性肠套叠脱套术后发生了缺血后再灌注损伤，术后血中内毒素浓度的升高与再灌注损伤有关。     

Colonic diverticulitis causing partial bowel obstruction in a child with cystic fibrosis

Children and adolescents with cystic fibrosis (CF) may manifest bowel pathology with resulting bowel obstruction. Recognized causes of bowel obstruction in CF patients include meconium ileus, intussusception, distal intestinal obstruction syndrome and postoperative adhesions. Additionally, the development of colonic strictures in children with CF has recently been described. We report an unusual cause of partial obstruction of the ascending colon in a child with CF due to pathologically proven diverticulitis. Received: 17 March 1997 Accepted: 24 July 1997     

Clinicopathologic spectrum and management of neonatal intussusception

The etiology, clinical presentation, and management of neonatal intussusception differs markedly from the more common infantile variety. This report summarizes the clinical course of three neonates with intussusception seen during the past 5 years. Bilious emesis, rectal bleeding, and abdominal distension were the cardinal symptoms in these neonates. A contrast enema was diagnostic in one patient but hydrostatic reduction of the intussusception was not possible. One patient had a Meckel's diverticulum and another had an ileocecal duplication cyst. In-utero intussusception of a Meckel's diverticulum was the cause of a gap-type ileal atresia in the third patient. Surgical excision of the causative lesion was performed in all three patients.     

Surgical aspects of intussusception due to lymphoma in children]

BACKGROUND: Intussusception due to lymphoma is a challenging condition for pediatric surgeons. The aim of this study is to report seven cases of this entity and to discuss its management. CASE REPORT: Six boys and one girl, 3-15-years-old, were admitted for intussusception secondary to a lymphoma. All patients underwent laparotomy: biopsy of massive abdominal tumor 6 and 8 weeks following resection of an intussusception (two cases), ileal resection of non-reductible intussusception (one case), right hemicolectomy for tumor of the appendix (one case), tumorectomy of localized ileal tumor (two cases), enlarged mesenteric lymph node biopsy associated with simple reduction of intussusception (one case). All children were successfully treated with protocol chemotherapy with a 15-month to 13-year follow-up. No relapse was observed. CONCLUSION: Surgeons should be aware of operative sights of ileal lymphomas. Diagnosis of lymphoma may be difficult after manual reduction of intussusception. A sample of any abnormality (mesenteric lymph node, peritoneal fluid) should be taken. Intestinal resection allows to reduce the intensity of chemotherapy but must be as limited as possible: ileal resection in cases of complicated intussusception, tumorectomy "in sano" in cases of ileal parietal isolated tumor. Reduction of intussusception alone (with no resection of ileal tumor) seems to be effective if diagnosis of lymphoma is possible from peripheral samples (peritoneal fluid, pleural effusion, mesenteric lymph node, bone marrow biopsy...).     

Intussusception secondary to anaphylactic reaction to salmon roe (ikura)

Anaphylactic food reaction often involves gastrointestinal symptoms, such as vomiting and abdominal pain, but to date, there have been no publications documenting the association between food hypersensitivity and intussusception. Herein is reported the case of a 2‐year‐old boy with intussusception accompanied by anaphylactic food reaction. The patient without known allergies complained of severe abdominal pain following ingestion of salmon roe for the first time. Dyspnea, wheezing and generalized urticaria also developed. Subsequently, he had stools containing jelly‐like blood with mucus. At hospital arrival, physical examination identified an abdominal mass in the right lower quadrant; imaging confirmed the diagnosis of colo‐colic intussusception. This patient was successfully treated with enema and no pathological findings were identified via radiology. Laboratory results supported the presence of IgE‐mediated allergy to salmon roe in the present patient. To our knowledge, this is the first report to describe the possible association between intussusception and a hypersensitive food reaction.     

Perforated appendicitis and appendicolith in a child presenting as intussusception: a case report

Simultaneous diagnoses of intussusception and appendicitis in the same patient have rarely been described in the pediatric literature. A case of a 30-month-old boy is presented with an initial diagnosis of intussusception that was successfully reduced by air contrast enema. When the patient's condition deteriorated, a diagnostic search surprisingly revealed a missed perforated appendicitis with an appendicolith that had been part of the intussusception. The patient's hospitalization and surgical course is described along with a discussion of the intermingling of intussusception and appendicitis in a young child. This case illustrates the need to consider alternative diagnoses when a patient's course takes an unexpected and confusing turn.     

Unexpected finding of laparoscopic appendectomy: appendix MALT lymphoma in children

Mucosa-associated lymphoid tissue (MALT) lymphomas comprise a group of indolent B-cell non-Hodgkin lymphomas (NHL), which are rare in pediatric age. The clinical presentation of MALT lymphomas varies according to the location of the lymphoma. We report on a case of MALT lymphoma involving the appendix in a 6-year-old girl. A 6-year-old girl was referred to our institution in May 2005 with a diagnosis of appendicitis. The abdominal ultrasound showed slight effusion in the pelvic fossa. The patient underwent laparoscopic appendectomy using the three-trocar technique. The appendix appeared moderately hyperaemic with slight enlargement of the two-thirds of the distal portion. The postoperative course was uneventful and the girl was discharged on day 1 without any complication. The morphological and immunohistochemical examination showed typical findings of low-grade MALT lymphoma (positivity for CD20, no immunostaing for CD5 and CD10, positivity for anti-λ light chain and low positivity for Ki-67). Further extensive examinations (abdominal MRI, gastroscopy, colonscopy and capsule endoscopy of the ileum) revealed that the lymphoma was limited to the distal two-third of the appendix (stage IA) and was not associated with any specific infection. At a recent follow-up the patients appeared to be doing well. Appendiceal MALToma is a rather uncommon pathology and, to our knowledge, there is only one report of appendiceal intussusception associated with appendiceal maltoma. According to our experience, low-grade MALToma can be managed by simple appendectomy. The histological examination should be the rule whenever an appendectomy is performed in children.     

Perforated appendicitis: an underappreciated mimic of intussusception on ultrasound

Background

We encountered multiple cases in which the US appearance of ruptured appendicitis mimicked intussusception, resulting in diagnostic and therapeutic delay and multiple additional imaging studies.

Objective

To explore the clinical and imaging discriminatory features between the conditions.

Materials and methods

Initial US images in six children (age 16 months to 8 years; 4 boys, 2 girls) were reviewed independently and by consensus by three pediatric radiologists. These findings were compared and correlated with the original reports and subsequent US, fluoroscopic, and CT images and reports.

Results

All initial US studies demonstrated a multiple-ring-like appearance (target sign, most apparent on transverse views) with diagnostic consensus supportive of intussusception. In three cases, US findings were somewhat discrepant with clinical concerns. Subsequently, four of the six children had contrast enemas; two were thought to have partial or complete intussusception reduction. Three had a repeat US examination, with recognition of the correct diagnosis. None of the US examinations demonstrated definite intralesional lymph nodes or mesenteric fat, but central echogenicity caused by debris/appendicolith was misinterpreted as fat. All showed perilesional hyperechogenicity that, in retrospect, represented inflamed fat “walling off” of the perforated appendix. There were four CTs, all of which demonstrated a double-ring appearance that correlated with the US target appearance, with inner and outer rings representing the dilated appendix and walled-off appendiceal rupture, respectively. All six children had surgical confirmation of perforated appendicitis.

Conclusion

Contained perforated appendicitis can produce US findings closely mimicking intussusception. Clinical correlation and careful multiplanar evaluation should allow for sonographic suspicion of perforated appendicitis, which can be confirmed on CT if necessary.     

Duplication of the caecum in a neonate simulating intussusception

A case of caecal duplication in a 36-h-old neonate is described. The patient presented with vomiting and an abdominal mass and the barium enema finding was initially suggestive of intussusception. At laparotomy a duplication cyst completely obstructing the lumen was found. This was considered to have given the radiological picture simulating intussusception.     

CT findings in children with Meckel diverticulum

Background  Approximately 0.04% of the general population will present with a complication related to Meckel diverticulum. The classic teaching is that symptomatic children with Meckel diverticulum present with painless rectal bleeding and are evaluated with a radionuclide scan. Our subjective experience is that we see children with Meckel diverticulum who present with abdominal pain and are evaluated by CT. Objective  We reviewed the findings on CT in children with pathologically proven Meckel diverticulum to identify characteristic patterns of presentation. Materials and methods  Databases were searched (2004–2008) for all children who had a pathologic diagnosis of Meckel diverticulum and a CT scan performed prior to surgery. Demographics, pathology, and CT features were reviewed. CT features reviewed included: soft-tissue stranding, abnormal calcifications, bowel obstruction, free air, free peritoneal fluid, cystic mass, intussusception, obvious lead point, location, and whether a normal appendix was identified. The frequency of Meckel diverticulum encountered on CT scans was compared to that found during the same period of time on technetium pertechnetate studies. Results  The review identified 16 subjects (mean age 9.5 years, M:F 9:7). CT findings included: soft-tissue stranding in nine (56%), small-bowel obstruction (SBO) in nine (56%), intussusception in three (19%), free fluid in ten (63%), cystic mass in four (25%), calcification in none (0%), free air in one (6%), and no abnormalities in two (13%). A normal appendix was identified in only five children (31%). There were three basic patterns of presentation of abnormalities: SBO only in five, intussusception with SBO in three, or cystic mass with inflammatory stranding in four (one with SBO). Also, 2.3 times more Meckel diverticulum was encountered on CT than on technetium pertechnetate studies. Conclusion  Meckel diverticulum is currently more commonly encountered in children on CT performed for abdominal pain than on technetium pertechnetate studies. There are three categories of appearance on CT: SBO only, intussusception, or a cystic inflammatory mass.     

An interactive teaching device simulating intussusception reduction

Intussusception is relatively uncommon, occurring in 0.5 to 2.3 cases per 1,000 live births in the USA. Radiology residents, therefore, have few opportunities to participate in intussusception reduction during training, and practicing radiologists encounter it infrequently. Training is essential, as successful reduction avoids surgery. The judgment involved in reducing an intussusception is best gained with experience. We developed a training device that simulates fluoroscopic intussusception reduction with air. The device consists of a doll that contains a cylinder with similar stress and strain characteristics to the human colon. The trainee pumps air into the cylinder through a rectal tube using a standard hand-held air reduction pump. A sensor measures the pressure within the chamber and transmits readings to a computer, which displays images from actual intussusception reductions based on the pressure maintained within the device. A random component in the software gives the user a new experience each time and models uncertainties in the actual reduction process, including perforation. This intussusception reduction simulator can enhance resident education, giving residents the opportunity to practice this technique before employing it on a real patient. The simulator can also help practicing radiologists become more comfortable with intussusception air reduction.