Pediatric GIST presenting as anemia

Gastrointestinal stromal tumors (GIST) are tumors of the gastrointestinal (GI) tract originating from the myenteric ganglion cells (interstitial cells of Cajal), that are very rare in children and adolescents. The most common clinical manifestation is acute or chronic, overt or occult GI bleeding although these tumors are asymptomatic in 10–30% of patients. We report a case of gastric GIST in a 11-year-old girl presenting with an iron deficiency refractory anemia without gastrointestinal symptoms and stool evidence of GI bleeding that caused a slight diagnostic delay.     

Antenatal detection of a communicating duodenal duplication.

We report a case of a subhepatic cystic mass diagnosed in utero by antenatal ultrasonography (US) at 15 weeks' gestation which subsequently proved to be a communicating duodenal duplication. In this male foetus, the differential diagnosis was choledochal cyst, congenital biliary atresia, foregut duplication or omentum cyst. Neonatal US examination lead to a diagnosis of duodenal duplication, also confirmed by barium gastrointestinal series. He was operated on day 8 and recovered uneventfully. We discuss the accuracy of antenatal US in the diagnosis of such right upper quadrant cystic masses. Now that antenatal findings are becoming increasingly sensitive in the detection of foetal anomalies, and parents need to be informed about the suspected pathology and its prognosis, we tried to determine, in the light of this case and a review of the literature, how antenatal US findings can offer more accuracy in the diagnosis of duodenal duplication.     

Massive gastrointestinal haemorrhage in isolated intestinal Henoch-Schonlein purpura with response to intravenous immunoglobulin infusion

There is increasing recognition that Henoch-Schonlein purpura may present in an atypical form in which gastrointestinal symptoms may predominate, and classic cutaneous changes may be delayed or absent. This may lead to significant diagnostic delay. We report the case of a 9-year-old girl who presented acutely with life-threatening gastrointestinal haemorrhage from multiple intestinal sites, with no skin rash and only mild evidence of renal involvement. Henoch-Schonlein purpura was confirmed by finding IgA deposition on vessels within gastric and duodenal mucosa, while immunohistochemistry also identified dense focal T cell infiltration in gastric mucosa and within duodenal epithelium. After initial stabilisation, the patient became shocked due to further gastrointestinal haemorrhage. Isotope bleeding scan identified multiple bleeding sites. Her endoscopically confirmed gastritis was sufficiently severe to preclude corticosteroids, and she was thus treated with intravenous immunoglobulin. This therapy induced prompt and sustained resolution of symptoms, and she has remained well since. Our patient's response concords with previous reports in corticosteroid-resistant cases to suggest that severe intestinal Henoch-Schonlein purpura may respond preferentially to intravenous immunoglobulin (IVIG) therapy. In severe cases where there is significant gastritis, IVIG provides an effective alternative to corticosteroids that may be employed as first-line therapy.     

Sonographic windsock sign of a duodenal web

We report a neonate with a duodenal web demonstrating the windsock appearance on US. In neonates, duodenal web is rare and its windsock appearance is also rarely seen. The windsock sign of duodenal web has been a well-known finding on upper gastrointestinal series. The corresponding windsock appearance may be demonstrated on US. Duodenal web can, therefore, be accurately diagnosed by identifying the sonographic windsock sign even in neonates. Received: 18 May 2000/Accepted: 5 July 2001     

Gastrointestinal stromal tumor in a child and review of the literature

Gastrointestinal stromal tumors (GIST) are c-KIT positive neoplasm that occur predominantly in adults. A 13-year-old boy who presented with massive gastrointestinal hemorrhage was subsequently diagnosed with GIST of the stomach. The tumor originated from the lesser curvature with a narrow base and no infiltration into surrounding organs. Two metastatic lesions in the liver were also found. Total excision of the gastric lesion was performed followed by adjuvant imatinib mesylate chemotherapy. He has had no signs of recurrence during 8 months of follow-up. GIST in children are rare and their behavior is notoriously difficult to predict. We report our experience and review the literature.     

Stricture of the duodenum and jejunum in an abused child

We report a case of abdominal injury secondary to child abuse in which the child had both a duodenal hematoma and contained perforations of the duodenum and proximal jejunum. These injuries were evaluated by both CT scan and upper gastrointestinal (GI) series. The child's nausea and vomiting persisted despite conservative treatment; after 3 weeks a repeat upper GI series demonstrated high-grade duodenal obstruction. An exploratory laparotomy was performed and a calcified, fibrotic mesentery and strictures in the distal duodenum and proximal jejunum were found. To our knowledge, this unusual complication of blunt abdominal trauma has not been described in association with child abuse. Received: 31 May 1996 Accepted: 16 September 1996     

Malrotation in Children with Symptoms of Gastrointestinal Allergy and Psychosomatic Abdominal Pain

Three children with malrotation 4.5, 5 and 9 years old at operation are presented. Their preliminary diagnoses were gastrointestinal (GI) allergy, GI allergy with colon irritabile and psychosomatic abdominal pain. They were treated on an outpatient basis under these diagnoses for more than two years before their malrotations were discovered. In two children radiology did not demonstrate any signs of intestinal obstruction and in one of these children repeated radiological examinations were necessary for the diagnosis of malrotation. Operative findings were chronic volvulus with compromised blood flow and obstructive duodenal bands. The third child suffered acute strangulation ileus with gangrene of the intestines before diagnosis was made. We conclude that special consideration must be given to the possibility of malrotation when treating and radiologically investigating children with GI disturbances.     

Malrotation in children with symptoms of gastrointestinal allergy and psychosomatic abdominal pain

Three children with malrotation 4.5, 5 and 9 years old at operation are presented. Their preliminary diagnoses were gastrointestinal (GI) allergy, GI allergy with colon irritable and psychosomatic abdominal pain. They were treated on an outpatient basis under these diagnoses for more than two years before their malrotations were discovered. In two children radiology did not demonstrate any signs of intestinal obstruction and in one of these children repeated radiological examinations were necessary for the diagnosis of malrotation. Operative findings were chronic volvulus with compromised blood flow and obstructive duodenal bands. The third child suffered acute strangulation ileus with gangrene of the intestines before diagnosis was made. We conclude that special consideration must be given to the possibility of malrotation when treating and radiologically investigating children with GI disturbances.     

A similar high level of immunoglobulin A and immunoglobulin G class milk antibodies and increment of local lymphoid tissue on the duodenal mucosa in subjects with cow's milk allergy and recurrent abdominal pains

In previous studies, we have reported endoscopic and histological alterations locally on the gastrointestinal (GI) tract associated with a gastrointestinal type of cow's milk allergy. In this study, we sought to further characterize endoscopic, and immunological findings in these children. We also hypothesized that the same type of immune responses might also be found in children with unexplained and recurrent abdominal pains. We did a gastroduodenoscopy for persistent GI symptoms, examined the mucosal histology of the small intestine and measured the antibodies to whole cow's milk and its fractions with an enzyme‐linked immunosorbent assay (ELISA) in a consecutive series of 22 subjects with untreated and 14 with treated cow's milk allergy (CMA) and 44 with recurrent abdominal pains (RAP). The immunological findings of the study subjects were compared with 54 controls. Lymphonodular hyperplasia (LNH) of the duodenum was the main endoscopic finding in 11 subjects (50%) with untreated and 5 (36%) with treated CMA. It was also found in 6 of 44 subjects with RAP. Compared with the controls, the patients with CMA showed significantly higher levels of IgA class antibodies to whole milk (p = 0.003) and βLG (p < 0.0001). Of the IgG class antibodies to βLG (p = 0.032), BSA (p < 0.0001) and αCAS (p < 0.0001) were significantly higher. The patients with LNH of the duodenal bulb as the main endoscopic finding showed significantly higher values of IgG class antibodies to βLG (p = 0.01) and αCAS (p = 0.005). Interestingly, the patients examined for RAP showed a similar increment in the pattern of whole milk and specific milk protein antibodies as the CMA children. In conclusion this study showed that gastrointestinal CMA beyond infancy is significantly associated with high levels of IgG and IgA class antibodies to milk and its fractions. As high levels of these antibodies and LNH of the duodenal bulb were also found in subjects with RAP, the study further suggests that gastrointestinal CMA might be one major reason for RAP.     

Henoch-Schonlein purpura presenting duodenal involvement similar to superior mesenteric artery syndrome in a girl

Henoch-Schonlein purpura (HSP) is an inflammatory vasculitis involving the skin, joints, gastrointestinal (GI) tract, and kidneys. This is the first case report describing a 5-year-old girl with HSP presenting duodenal involvement which might be associated with superior mesenteric artery syndrome (SMAS).     

Laparoscopic treatment of duodenal obstruction: report on first experiences in Latin America

BACKGROUND: There are few reports in the literature on the use of a laparoscopic approach for duodenal obstruction, particularly for duodenal atresia. We report here the results of 4 cases treated laparoscopically, and discuss the safety, feasibility and long-term results of this approach. PATIENTS AND METHODS: Four pediatric patients presented with duodenal obstruction, one with duodenal atresia, one with annular pancreas, and two with duodenal obstruction due to Ladd's bands but without malrotation. Diagnosis was made by clinical evaluation, simple X-ray film in the Ladd's patients, and contrast gastroduodenal X-ray series for the annular pancreas. All procedures were performed using 3-mm instruments and 3 trocars. Two duodeno-duodenal anastomoses were performed and two lyses of Ladd's bands; all procedures were carried out laparoscopically. RESULTS: An upper GI contrast excluded obstruction or leakage in all patients 5 - 7 days after surgery and feedings were started. The patient with annular pancreas died of cardiovascular complications after one months. The other 3 patients are asymptomatic and tolerating feedings after a follow-up of 36 months. CONCLUSIONS: We conclude that a laparoscopic approach for duodenal obstruction can be performed safely and effectively and achieves a short hospital stay. Laparoscopic lysis of Ladd's bands is easy to perform, but duodenoduodenostomy requires advanced laparoscopic skills.     

Blue rubber bleb nevus syndrome

Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare condition characterized by multiple venous malformations involving the skin and internal organs. The gastrointestinal tract is always involved and intestinal haemorrhage is the most frequent clinical manifestation associated with iron deficiency anaemia. We describe a 10‐year‐old girl who, since birth, presented numerous venous malformations all over her body and a lymphangioma in the right leg. At the age of 5 years, she also had a severe episode of gastric bleeding requiring a blood transfusion. From this episode, she is suffering from chronic anaemia and this is the reason for admission into our hospital. The endoscopic examination of the gastrointestinal tract revealed multiple giant venous malformations in the oesophagus, stomach, duodenum and in all visible sections of the colon. Endoscopy is the gold standard technique for the diagnosis of BRBNS with GI lesions and also allows immediate therapeutic measures such as argon plasma coagulation, laser photocoagulation, sclerotherapy or band ligation. In addition, pharmacological treatments based on corticosteroids, interferon alfa, vincristine or octreotide have been described for BRBNS. Conclusion: Blue Rubber Bleb Nevus Syndrome is a congenital cutaneous and gastrointestinal haemangiomatosis. Its morbidity and mortality depends on involvement of visceral organs and particularly on GI bleeding. The treatment is based on pharmacological or surgical therapy. Overall, the most important step is the follow‐up to the presence and the evolution of GI lesions and the possible bleeding.     

Gastrointestinal pathology in neonates: new imaging strategies

The mainstay of imaging of gastrointestinal (GI) pathology in infants has always been and still is the plain radiograph of the abdomen and conventional contrast studies. In this review emphasis is placed on the situations where there are new imaging strategies and alternative modalities of imaging, including US, CT, MRI and radionuclide studies. This review will deal with GI pathology in the newborn and in the older neonate. It will also refer to any new approaches to imaging GI pathology in the premature infant. Finally the review will address how antenatal diagnosis of gastrointestinal tract abnormalities has changed the imaging strategy and management of the neonate.     

Duodenal haematoma following endoscopy as a marker of coagulopathy

Background

Intramural duodenal haematomas (IDHs) are a rare complication of endoscopic biopsy but can cause significant morbidity and mortality, including duodenal obstruction, hospitalization and needing intravenous feeding. They are extremely uncommon in those with normal haematology.

Objective

To describe the occurrence of IDHs following endoscopic biopsy in our institution.

Materials and methods

We identified three patients who developed a post biopsy IDH during an 18-month period (2010–2012) in a retrospective search of our hospital pathology and imaging databases.

Results

All three children had complex medical problems and presented with gastrointestinal symptoms including severe abdominal pain, reflux, poor feeding and abnormal gut transit time. All underwent normal upper GI endoscopy with duodenal biopsy. Following endoscopy, they presented with intermittent GI obstruction with severe abdominal pain, distension and bilious vomiting or symptoms of pancreatitis, had imaging features of IDH and were managed conservatively making a full recovery. Initial haematology including platelet counts were normal, but two children were subsequently found to have platelet dysfunction and the third to have an unclassified coagulopathy.

Conclusion

IDHs may be the presenting factor in children with unsuspected bleeding problems. We present these findings to raise awareness of the imaging features and clinical impact of these cases because better understanding of these risk factors may help to avoid these complications in children in the future.     

Gastrointestinal tract imaging in children: current techniques

Imaging of the gastrointestinal (GI) tract in children continues to evolve, with new techniques, both radiological and non-radiological, being added to the repertoire. This article provides a summary of current imaging techniques of the GI tract (primarily the upper GI tract) and the relationship between those techniques. It covers the upper GI series and other contrast studies, US, CT and MRI. Note is also made of the contribution now made by capsule endoscopy (CE). Abdominal emergency imaging is not covered in this article.     

Periampullary duodenal duplication cyst masquerading as a choledochocele

Enteric duplication cysts are rare congenital anomalies of unclear etiology. While they can occur anywhere in the gastrointestinal tract, they typically occur in the ileum or ileocecal region and very rarely in the duodenum. Here, we report a case of a periampullary duodenal duplication cyst in a 13-year-old male who presented with clinical and laboratory evidence of small bowel obstruction, hepatitis, and pancreatitis. Based on radiologic imaging, the patient was thought to have a type III choledochal cyst (choledochocele) within the duodenal lumen. Intraoperative findings and postoperative pathological evaluation, however, revealed that the lesion was a duodenal duplication cyst masquerading as a choledochal cyst. Interestingly, the duplication cyst was communicating with the common bile duct, simultaneously causing biliary and small bowel obstruction.     

Utility of sonographic assessment of the position of the third part of the duodenum using water instillation in intestinal malrotation: a single-center retrospective audit

Background

Intestinal malrotation and particularly volvulus are potentially devastating conditions. Upper gastrointestinal (UGI) contrast studies have been considered the gold standard for diagnosis. However the use of ultrasonography (US) has been increasingly described. We describe a method for delineating the duodenal anatomy with US as a means to exclude malrotation.

Objective

To report our experience using US to assess intestinal rotation.

Materials and methods

We conducted a retrospective audit of US scans performed at a tertiary referral centre to exclude malrotation for paediatric surgery between 2008 and 2011.

Results

One hundred thirty-nine infants were included, of whom 114 had a normal US scan. Of the 114, nine had subsequent upper gastrointestinal contrast studies that confirmed the initial results; there were no false-negatives. There were abnormal US scans in four infants associated with midgut volvulus and malrotation; there were no false-positives. The other 21 US scans were equivocal, and 11 of these had a confirmatory UGI contrast study; only one required surgery to correct malrotation.

Conclusion

US has been a safe and effective tool in the assessment of intestinal rotation at our institution. The main advantages of US imaging are its lack of ionising radiation and its rapid and accurate diagnosis of volvulus.     

Gastrointestinal stromal tumors (GIST) in children and adolescents: A comprehensive review of the current literature

Standards for the management of gastrointestinal stromal tumors (GIST) in children do presently not exist. Thus a systematic review and summary of the current literature was conducted serving as a basis for the further development of optimal management strategies for childhood GIST within a cooperative network. Presently 21 cases with familial GIST, and more than 100 pediatric cases each with Carney triad or sporadic GIST have been published so far. An international prospective registration based on national registries has recently started to acquire more clinical and molecular data and to develop appropriate management strategies for children and adolescents with GIST. Pediatr Blood Cancer 2009; 53:1171–1179. © 2009 Wiley‐Liss, Inc.     

National estimates of hospital utilization by children with gastrointestinal disorders: analysis of the 1997 kids' inpatient database

OBJECTIVES: To identify and to generate national estimates of the principal gastrointestinal (GI) diagnoses associated with hospital utilization and to describe national hospital utilization patterns associated with pediatric GI disorders. STUDY DESIGN: We analyzed a nationwide and stratified probability sample of 1.9 million hospital discharges from 1997 of children 18 years and younger, weighted to 6.7 million discharges nationally. Principal GI diagnoses were identified through the use of the Clinical Classification Software and Major Diagnostic Categories. RESULTS: In 1997 in the United States, there were 329,825 pediatric discharges associated with a principal GI diagnosis, accounting for more than 2.6 billion US dollars in hospital charges and more than 1.1 million hospital days. Appendicitis, intestinal infection, noninfectious gastroenteritis, abdominal pain, esophageal disorders, and digestive congenital anomalies combined accounted for 75.1% of GI discharge diagnoses, 64.2% of GI hospital charges, and 68.0% of GI hospital days. Excluding normal newborn infants and conditions related to pregnancy, GI disorders were the third leading cause of hospitalization. CONCLUSIONS: GI disorders are a leading cause of hospitalization of children. A minority of GI conditions account for the majority of measures of utilization. Children are hospitalized for GI conditions and at institutions that are distinct from adults.     

Incidental GIST after appendectomy in a pediatric patient: a first instance and review of pediatric patients with CD117 confirmed GISTs

A 7-year-old boy underwent uncomplicated laparoscopic appendectomy for acute appendicitis. Incidentally, he was found to have a spindle cell tumor with CD117 immunopositivity, consistent with gastrointestinal stromal tumor (GIST) in the appendix. Although commonly reported in adults, pediatric GISTs are rare gastrointestinal malignancies that occur in only 1.4–2.7 % of children and adolescents [1]. Due to the paucity of reports, data are insufficient to adequately characterize tumor behavior, recurrence, and survival. We present the first case of pediatric GIST in the appendix. In addition, a review of the literature for CD117 confirmed pediatric GISTs was conducted to summarize its clinical features and current treatment options.