Abdominal symptoms in patients with hematological malignancies can occur due to an array of pathologies. Two diagnoses with similar presentation albeit, generally opposite treatment modalities, are typhlitis (inflammation of cecum) and acute appendicitis. Both diagnoses have to be kept in mind in such a patient presenting with right lower quadrant (RLQ) pain. Sagacious clinical judgment along with the aid of radiological imaging may help in differentiating between the two conditions. We present a case of a young male with chronic myeloid leukemia (CML) on imatinib, diagnosed and started on therapy four years earlier, who presented with symptoms of RLQ pain not typical of acute appendicitis. The accurate diagnosis was made with the assistance of ultrasound (US) imaging and prompt surgical therapy was instituted followed by a smooth postoperative recovery.

Neutropenic enterocolitis (NEC) is a life-threatening disease with substantial morbidity and mortality, seen primarily in patients with hematologic malignancies. The frequency of NEC has increased with the widespread use of chemotherapeutic agents such as the taxanes, which cause severe gastrointestinal mucositis. Neutropenic patients with fever and abdominal symptoms (cramping, pain, distention, diarrhea, GI bleeding), should undergo evaluation of the abdomen for bowel wall thickening of >4 mm, the hallmark of NEC. Clostridium difficile infection should be ruled out, as well as other etiologies such as graft-versus-host disease. Complications include bacteremia, which is often polymicrobial, hemorrhage, and bowel wall perforation/abscess formation. Management includes bowel rest, correction of cytopathies and coagulopathies, and broad spectrum antibiotics and antifungal agents. Surgical intervention may be necessary to manage complications such as hemorrhage and perforation and should be delayed, if possible, until recovery from neutropenia.

Gastrointestinal (GI) manifestations of leukemia occur in up to 25% of patients at autopsy, generally during relapse. Its presence varies with the type of leukemia and has been decreasing over time due to improved chemotherapy. Gross leukemic lesions are most common in the stomach, ileum, and proximal colon. Leukemia in the esophagus and stomach includes hemorrhagic lesions from petechiae to ulcers, leukemic infiltrates, pseudomembranous esophagitis, and fungal esophagitis. Lesions in the small and large bowel are usually hemorrhagic or infiltrative. Infiltration of lymphoreticular organs, mainly spleen, liver, and lymph nodes, is more prominent in chronic than acute leukemia. Neutropenic enterocolitis, a necrotizing process involving the cecum, ascending colon, and terminal ileum, is increasing in incidence due to greater intensity of chemotherapy. Distension of bowel leads to mucosal breaches, permitting entry of organisms that grow profusely in the absence of neutrophils. Ischemic necrosis follows, leading to perforation and/or peritonitis. Patients present with fever, abdominal pain, diarrhea, nausea, vomiting, abdominal distension and tenderness. Ultrasound and computed tomography scans show thickening of the bowel wall. Treatment is supportive with surgery for necrosis and perforation. The main GI causes of death in leukemia are hemorrhage, infection, and necrotizing enterocolitis.

Gastrointestinal complications in pediatric acute myeloid leukemia (AML) have not been systematically described in the literature. Our objective was to describe complications related to the small and large bowel in children with AML. Literature searches were conducted of Ovid Medline from 1950 to November 2009 and EMBASE from 1980 to November 2009. We included any study design that described gastrointestinal complications in children and/or adults with AML. Common gastrointestinal complications were typhlitis and enterocolitis. Less common complications included appendicitis, pneumatosis intestinalis, and perianal infections. Both leukemia infiltration and intensive chemotherapy likely play a role in the etiology of these conditions. There is a paucity of carefully conducted studies that describe the natural history of typhlitis and enterocolitis and evidence is needed to help guide the management of gastrointestinal complications. Gastrointestinal complications are relatively common in children with AML. Conduction of carefully performed cohort studies is needed to better understand the spectrum of symptoms and expected consequences of gastrointestinal complications. Randomized trials are required to develop evidence-based guidelines for the management of gastrointestinal complications in pediatric AML.

There is a paucity of data on the sonographic criteria for the diagnosis of intestinal tuberculosis. The purpose of this study was to further characterize the potential sonographic signs of intestinal tuberculosis and to increase the diagnostic sensitivity and specificity of ultrasound.

Seven patients with a final diagnosis of gastrointestinal tuberculosis and a control group of 18 patients suffering from tuberculosis limited to the lungs were prospectively evaluated for sonographic criteria of intestinal tuberculosis and the findings were compared with those in 50 healthy controls.

The following signs of intestinal tuberculosis were detectable: asymmetric thickened bowel wall (100%), intramural abscesses (86%), fistula (43%), extramural abscesses (29%), mesenteric thickening (29%), "white bowel" sign (29%), hypoechoic edema of Kerckring's folds with mesenterial thrombosis (14%), enlarged mesenteric lymph nodes with inhomogeneous echotexture and circumscribed hypoechoic spots <3 mm (86%), ascites (29%) and enlarged spleen (14%). These signs were exclusively present in patients with intestinal tuberculosis as compared with patients with tuberculosis limited to the lungs or with healthy controls. We could confirm the endoscopically reported right-sided prevalence of these wall thickenings. In contrast to the reported literature, a much higher prevalence of these sonographic signs was found as they were present in all patients. Six of 7 patients (86%) showed enlarged mesenteric lymph nodes. This was particularly interesting as mesenteric lymph nodes have not been described as being enlarged in the majority of other differential diagnoses of the ileocecal region.

The combination of bowel-wall thickening of the ileocecal region with intramural abscesses with or without fistula, abscesses and mesenteric thickening accompanied by enlarged mesenteric lymph nodes was highly predictive of intestinal tuberculosis.

Neutropenic enterocolitis (NE) must be recognized in patients with fever, neutropenia, and abdominal pain. Classically, NE has been described in patients with hematologic malignancies treated with intensive chemotherapy. Current interest in NE has increased due to recent cases associated with newer, more intensive chemotherapy in solid tumors. This review discusses pathology, clinical presentation, and treatment of NE. Ultrasonography or CT scans are the best radiographic studies to confirm the diagnosis. Management options, including antimicrobial therapy, surgery, and supportive care, are discussed. Chemotherapy incorporating the taxane family of drugs (paclitaxel and docetaxel) associated with NE is also reviewed with observations regarding the earlier onset of the disease in the first weeks following chemotherapy. Even with currently recommended therapy, a high mortality rate, approximating 45%, can occur. Best outcomes for NE rely upon understanding of risks for the condition, prompt empiric therapy with broad-spectrum antimicrobial agents, systemic antifungal therapy, and meticulous attention to supportive care.

To investigate the sonographic features at time of diagnosis and follow-up in patients with neutropenic enterocolitis.

The sonographic findings in 14 patients with neutropenic enterocolitis were described and evaluated regarding symptoms and clinical outcome.

In all patients with neutropenic enterocolitis, the ileocoecal region was involved with wall thickening >10 mm. A transmural inflammatory pattern, hypervascularity of the thickened bowel wall and free abdominal fluid were the common findings. The sonographically revealed thickness of the bowel wall was associated with lethal outcome (P<0.03). In the 11 surviving patients,the improvement of clinical symptoms was accompanied by progressive reduction of intestinal wall thickness.

High-end sonography of the bowel is a helpful tool for diagnosis,assessment of prognosis and follow-up of patients with neutropenic enterocolitis.The ultrasonographically revealed bowel thickness reflects the severity and the course of the disease, and seems to be predictive for the clinical outcome.

Neutropenic enterocolitis (typhlitis) is an unusual acute complication of neutropenia, most often associated with leukaemia and lymphoma and characterized by segmental caecal and ascending colonic ulceration that may progress to necrosis, perforation, and septicaemia. We present a unique case of an 8-year-old girl with recently diagnosed infectious mononucleosis having findings consistent with typhlitis on abdominal CT.

Typhlitis is increasingly recognized in children undergoing chemotherapy but is poorly characterized. The authors investigated the demographic, clinical, and imaging (ultrasonography and computed tomography [CT] scans) variables related to the diagnosis, risk, and outcome of typhlitis.

The authors reviewed the records of patients who had typhlitis (bowel wall thickness > or = 0.3 cm plus clinical findings) during treatment at St. Jude Children's Research Hospital (Memphis, TN) between 1990 and 2001. They assessed whether duration of typhlitis was related to bowel wall thickness, extent of colonic involvement, ascites, demographics, primary diagnosis, symptoms of typhlitis, or duration of neutropenia. To identify risk factors for typhlitis, the authors compared the demographic data and previous drug therapy of 78 patients who had typhlitis and 1231 identically treated children who did not.

Of 3171 children, 83 (2.6%) developed typhlitis. Frequent symptoms were abdominal pain (91%), fever (84%), abdominal tenderness (82%), and diarrhea (72%). Twelve percent of the patients were not neutropenic. Duration of typhlitis was associated with bowel wall thickness measured by ultrasonography (n = 68; P = 0.05) but not CT scan (n = 48; P = 0.67) and was associated with duration of neutropenia (P = 0.02), fever (P = 0.01), and abdominal tenderness (P = 0.04). Age >16 years at cancer diagnosis was the only demographic factor associated with typhlitis (P = 0.03). Two patients died of typhlitis.

Ultrasonography was a useful imaging modality for children with suspected typhlitis. The classic triad of abdominal pain, fever, and neutropenia may be absent. The severity of typhlitis was related to the duration of neutropenia and the presence of fever or abdominal tenderness.

We performed a study to determine if colour Doppler findings may help to identify the cause of wall thickening in acute non-diverticular diseases of the colon. The study group included 66 patients admitted to the emergency department with a final diagnosis of infectious colitis (n=23), inflammatory colitis (n=10), ischaemic colitis (n=23) and malignant tumours (n=10). The following ultrasound features were assessed: maximal wall thickness, wall stratification, arterial flow in the colonic wall and arteriolar resistive index. Higher values of wall thickness were observed in malignant tumour (18.2+/-6.2 mm, p<0.001). Moderately thickened wall (6.6+/-1.3 mm, p< or =0.06), preserved stratification (90% versus 46% in the remainder of the study population) and lower resistive index (0.51+/-0.10, p< or =0.05) were significantly related to inflammatory colitis. Absence of arterial flow was more frequently observed in ischaemia (43% versus 12% in the remainder of the study population). In conclusion, despite some overlap, both ultrasound and colour Doppler features are helpful in the differential diagnosis of colonic thickening related to non-diverticular colonic lesions.

The purpose of this study was to investigate the initial clinical features and subsequent outcomes in patients with adult leukemia with typhlitis or neutropenic enterocolitis. A retrospective review of 10 episodes of neutropenic enterocolitis in nine patients (age range, 21-71 years) with acute leukemia from March 1, 1990, through February 28, 2002, was conducted. In clinical presentations, fever appears in all patients, followed by abdominal pain or tenderness (90%) and diarrhea (60%), respectively. In particular, three cases were coincidentally diagnosed as leukemia before any chemotherapy. The most common diagnostic modality used for the diagnosis of neutropenic enterocolitis was computed tomography (CT) scan (seven episodes). Medical treatments, including broad-spectrum antibiotics, bowel rest, and total parenteral nutrition, were applied to seven patients. Laparotomy with bowel resection was performed on two patients with bowel necrosis and severe peritonitis. Of all nine cases, six were fatal as a result of sepsis, a common complication of neutropenic enterocolitis. As the incidence of neutropenic enterocolitis increases in patients with acute leukemia, EPs should be alert and make an early diagnosis of this rapidly deteriorated and life-threatening disease.

To assist the radiologist in differentiating the colitis in children, this review proposes a systematic US approach to the disease, presents the US aspect of the normal colon and describes three distinctive US patterns reflecting the intramural extension of the histopathological changes. Each pattern corresponds to one or several diseases producing alterations in the same layer(s). Stratified thickening suggests an inflammatory mucosal process resulting from infection (as in advanced appendicitis or in infectious colitis) or to inflammation (as in IBD). Nonstratified thickening with loss of the haustral folds reflects a marked submucosal infiltrate. Color Doppler is required to distinguish between an inflammatory disease (as advanced CD or neutropenic colitis) and an ischemic colitis (HUS in children). Nonstratified thickening with preservation of the length of the haustral folds is the sign of an intraluminal deposit due to PMC. Correlating the sonographic pattern and the anatomic distribution of the disease with the clinical and laboratory findings often permits to propose a specific diagnosis.

Abdominal infections are an important cause of morbidity and mortality in neutropenic patients. We present a retrospective series of 16 patients, mostly with acute leukemia, who developed severe abdominal infections during chemotherapy-induced neutropenia between 1991 and 1997. The frequency among patients with acute leukemia was 2.35% (13 of 553). Thirteen patients presented with enterocolitis and 3 patients presented with cholecystitis. Eight patients died. Bacteremia was present in 6 patients, 4 patients suffered from proven or strongly suspected fungal infections, and 1 patient suffered from cytomegalovirus infection. Early surgical management was required in a patient with intestinal obstruction, whereas other patients could be managed conservatively. Two patients with acute cholecystitis were treated with antibiotics until the end of neutropenia and then were resected. Severe abdominal injections in neutropenic patients, which are often fatal, were caused by nonbacterial microorganisms in one-fourth of the cases and could be managed conservatively in most instances.

Abdominal pain, respiratory infections, and skeletal trauma are among the most common reasons for imaging infants and children in the emergency department. The types of pathology and the imaging findings for the various causes of these conditions often differ significantly from those seen in adults with similar symptoms. Although radiographs remain the primary imaging tool, ultrasound, helical CT, and MR imaging play an increasingly important role in solving diagnostic problems in emergency pediatrics. This article highlights some of the common diagnostic dilemmas and discusses current trends in the use of advanced imaging in pediatric patients.

This article focuses on salient points in the evaluation of abdominal pain in infants and children. Specifically, the authors address appendicitis and abdominal pain associated with either vomiting, constipation, or gastrointestinal bleeding. A discussion of common abdominal masses, urologic, and gynecologic problems, and considerations in the evaluation of immunologically suppressed or neurologically impaired children, and children with recurrent abdominal pain is also presented. The authors establish logical, focused approaches to the initial evaluation and management of abdominal pain and suggest criteria for timely surgical referral.

Neutropenic colitis is a clinicopathological syndrome characteristically seen as a complication of chemotherapy for haematological malignancy. This review explores the pathogenesis of the condition and appraises the options for management.

A Medline search was carried out and all relevant papers were reviewed.

There are many case reports but few published series, so experience is mainly anecdotal. Both medical and surgical management have been successful; it is not possible to compare treatment groups formally.

The greater use of aggressive multiagent chemotherapy regimens may increase the frequency with which the condition is encountered. Heightened awareness of neutropenic colitis may prompt diagnosis and a better understanding of the pathophysiology may help guide clinical management.

The treatment of acute leukemia in childhood has been increasingly successful. Concurrently, severe leukemia-related gastrointestinal complications have become more common.

We evaluated the findings of the abdominal ultrasound (US) examinations of 52 children with acute lymphoblastic leukemia (ALL) who had severe clinical symptoms indicating infection or abdominal complication during chemotherapy treatment or after the cessation of such treatment and assessed the impact of these findings on patients' subsequent treatment and survival.

Our study presents ten cases of typhlitis with a prevalence of 9%, all of which were rapidly diagnosed by US and had a favourable outcome. We also found focal intra-abdominal parenchymal lesions in six children, five of them due to fungal infection and one due to leukemic infiltration. Several other intra-abdominal pathologies significant for the patients' treatment are also reported.

We believe that abdominal US is a useful, rapid, safe, and accurate imaging method for children with ALL suspected to suffer from leukemia- or chemotherapy-related gastrointestinal complications. More invasive imaging methods are seldom needed.

According to our results, abdominal US gives the necessary information in most of the cases and provides prompt diagnosis, which may prevent possible fatal complications.

We report an unusual case of acute lymphoblastic leukemia (ALL) that presented as right lower quadrant pain in a 17-year-old boy. Ultrasonographic findings were consistent with typhlitis. The clinical and imaging symptoms resolved upon treatment with antibiotics and conservative care, only to recur after initiation of chemotherapy. Familiarity with the clinical presentation and imaging findings of typhlitis is important for its correct diagnosis and management.

The sonographic features of total neutropenic colitis in a 14-year-old girl with osteogenic sarcoma are presented. Sonography disclosed characteristic diffuse thickening of the colonic wall with hyperechoic bowel mucosa. Serial sonograms were performed to monitor the progress of the disease.

Neutropenic colitis is a complication of the treatment of hematologic malignancies and, less commonly, of other disease entities. The septic, inflammatory process has a predilection for the terminal ileum and right colon. While the pathogenesis is not clear, mucosal injury caused by several different mechanisms and local opportunistic infection play significant roles. An association has been recognized between neutropenic colitis and sepsis caused by C. septicum. Patients present with fever, diarrhea, and acute abdominal pain and tenderness often localized in the right lower quadrant. Sonography and CT are helpful in demonstrating colonic wall thickening and pericolic fluid. Peritoneal lavage has been used to exclude perforation in these critically ill patients. Although there has been debate about whether medical or operative management is best, the optimal initial therapy includes supportive care with gastric decompression, fluid and blood product replacement, and broad-spectrum antibiotics. The indications for surgery include continued intestinal bleeding despite correction of coagulopathy and pancytopenia, free intraperitoneal air, and uncontrolled sepsis. At operation, a right colectomy with ileostomy and mucous fistula or, in selected patients, primary anastomosis is the procedure of choice. Timely return of functioning neutrophils and the eventual prognosis of the primary disease are crucial to the overall success or failure of treatment of neutropenic colitis.

The decision to operate for abdominal pain in patients with leukopenia can be exceedingly difficult. Surgical exploration may be the only effective way to differentiate acute appendicitis from other causes, but it involves considerable risk of infectious complications due to immunosuppression. Leukemic patients, who presented significant RLQ pain, had been indicated for operation, despite having advanced disease or having had received chemotherapy or steroids. Four adult leukemia patients, complicated by acute appendicitis, were reviewed. Two patients were in induction chemotherapy, one receiving salvage chemotherapy due to relapse and the other was in conservative treatment. Two patients were acute myelocytic leukemia (AML), one had acute lymphocytic leukemia (ALL), and the other had aleukemic leukemia. All patients underwent appendectomy and recovered without complication. Our experience supports the theory that the surgical management of appendicitis in acute leukemia is the most effective way, in spite of leukopenia.

Neutropenic colitis is recognized as a rare complication of chemotherapy in haematological malignancies. By contrast, the complication is less well known in relationship to chemotherapy for solid malignancies. There are very few examples reported and this paper adds three further cases and reviews the literature. We emphasize that although some cases of neutropenic colitis may be managed medically, full thickness involvement of the bowel wall may lead to perforation and require surgery. The pathogenesis of this progression is discussed and it is concluded that clinical awareness is important in the diagnosis. The signs of peritonitis or septicaemia are indications for excisional surgery with the formation of a temporary ileostomy.

Review of the consultation records of the Gastrointestinal Surgical Oncology service at Roswell Park Memorial Institute from 1982 to 1987 revealed 22 patients with a clinical diagnosis of neutropenic enterocolitis. Ninety-one percent of the patients had hematologic malignancies, and 95% were receiving cytotoxic chemotherapy. Sixteen patients were treated nonsurgically; 11 died. Of those 11 cases, autopsies were performed in 9. At autopsy, the clinical diagnosis was confirmed in four cases; four cases were found to have normal intestinal tracts, and one case had a small bowel volvulus. In none of the four cases for which autopsy proved neutropenic enterocolitis was transmural bowel necrosis or perforation found. Laparotomy was performed in six patients; three survived. The clinical diagnosis was verified in four of the six patients. Neutropenic enterocolitis must be considered a diagnosis of exclusion. Care of these patients should be individualized. Nonoperative management with bowel rest, decompression, nutritional support, and broad spectrum antibiotics is recommended initially. Operative intervention is recommended for those with perforation or those whose condition deteriorates clinically during close, frequent observation.

A review of pediatric autopsy results at the Texas Children's Hospital, Baylor College of Medicine from 1970 through 1987 was conducted. Thirty-three cases of typhlitis were identified in patients with acute leukemia and two cases each in patients with lymphoblastic lymphoma and aplastic anemia. Patients ranged in age from 10 months to 17 years. Fifty-seven percent were male and 43% were female. All were myelosuppressed. A postmortem incidence rate of 24% was determined for patients with acute leukemia. Common symptoms included abdominal pain and distention in 78% of patients and acute lower gastrointestinal bleeding in 35%. Abdominal radiographs varied in spectrum from a nonspecific bowel gas pattern to frank right colonic pneumatosis intestinalis. Thirty-three patients received chemotherapy within 30 days before onset of abdominal symptoms. All patients were febrile (greater than 38.5 degrees C), and 33 received broad-spectrum antibiotics. Three patients received amphotericin B. Premortem, 84% of organisms cultured from blood were bacterial whereas 16% were fungal. Fungal pathogens accounted for 53% of new microorganisms seen at autopsy. Postmortem examination showed typhlitis in the following anatomic distributions: (1) confined to the cecum; (2) involving the cecum and ileum; (3) involving the cecum, ileum, and ascending colon; or (4) involving the cecum, with sporadic ulcers throughout the intestine. This review includes clinical and postmortem features of typhlitis and current strategies for diagnosis and management.

The sonographic findings in a patient with typhlitis are presented. Sonography showed a solid mass with a "target" sign in the right lower quadrant, continuous with the ascending colon. In addition to suggesting the diagnosis, sonography was helpful in the follow-up examination of the patient because it was able to assess the gradual decrease in the thickness of the bowel wall and ultimate resolution of the inflammatory process.

Three cases of histologically confirmed neutropenic enterocolitis, each presenting as an acute abdomen in patients with leukaemia are presented. All three patients presented with fever and abdominal pain within 14 days of completing a course of chemotherapy. Signs of peritonitis localized to the right iliac fossa developed in each patient, in spite of aggressive antibiotic therapy and bowel rest. All three patients were found to have non-viable caecum at laparotomy and were treated by right hemicolectomy. Primary ileocolic anastomosis was performed in one patient, who recovered following a stormy postoperative course owing to sepsis. Two patients underwent formation of an ileostomy with distal mucous fistula and each recovered with minimal postoperative complications; secondary anastomosis was performed electively in both cases. The difficulty in diagnosing neutropenic enterocolitis preoperatively is discussed and the place of non-operative management is reviewed but we recommend surgical intervention as a means of ensuring removal of a localized septic focus until marrow regeneration occurs.