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Home , Gastric volvulus, Intestinal atresia, Pyloric stenosis, Pylorus

Michael Moore MB BCh, Ruth Lim MD, Sjirk J Westra MD and Katherine Nimkin MD IMAGING OF THE IN CHILDREN: Beyond Pyloric Pediatric Radiology , Massachusetts General Hospital, Boston, MA • Harvard Medical School, Boston, MA

INFLAMMATORY CONDITIONS cont. CONGENITAL/DEVELOPMENTAL CONDITIONS ACQUIRED CONDITIONS NEOPLASTIC CONDITIONS INTRODUCTION EOSINOPHILIC Figure 8 Figure 11 Figure 13 Figure 15 Gastric abnormalities in children are relatively uncommon. Imaging of the stomach in children is frequently 4 year old with acute onset of . Eosinophilic is an uncommon condition and rarely seen in A 13 year old female with a known diagnosis of tuberous sclerosis presented for routine screening 12 year old girl with acute An 18 year old male with a family history of polyposis syndrome. performed on the vomiting infant; hypertrophic (HPS) is a relatively common entity seen in infancy. It may occur alone or, more commonly, as part of a more abdominal MR to follow known renal angiomyolipomas. and this setting. We present imaging studies of the stomach in a variety of less common pathologic conditions. diffuse gastroenteritis involving the in particular. This vomiting. A double contrast upper GI barium study demonstrates multiple polyps throughout heterogeneous group of eosinophilic gastroenteropathies includes the gastric fundus and body (arrows). No small bowel polyps were seen. Biopsy at Chest radiograph (a) shows large left effusion, CT of the abdomen (a) reveals eosinophilic , , and gastritis. They have revealed fundic polyps and hamartomatous-type polyps with focal left basilar lung opacity, and elevated left hemi- (arrows) in common a similar pathologic process - eosinophilic inflammation low grade dysplasia. Colonoscopy confirmed multiple adenomatous polyps and the diaphragm. Image from UGI series (b) shows and a heterogeneous mass of the gut. Diagnosis is made by pathological demonstration of eosinophilic infiltration, which may be patchy and patient underwent a total colectomy. INFLAMMATORY CONDITIONS (arrow) to the left of the GE junction filling the stomach (b, arrows). involve different layers of the bowel wall, and by the exclusion of other causes of eosinophilia, such as parasites, infla- with close approximation of the pylorus and GE Trichobezoar with gastric a b c mmatory bowel disease, and vasculitis. Peripheral eosinophilia may be present in up to 50% of patients. The exact junction. Mesenteroaxial was found at perforation was found at Figure 1 GARDNER SYNDROME pathophysiologic cause is unknown and the role of allergens is debated. a b surgery. a b surgery. 17 week old female, s/p at age 11 weeks for pyloric stenosis, who Gardner syndrome is a rare autosomal dominant disorder characterized by the triad of colonic polyposis, multiple osteomas and mesenchymal tumors of On barium studies, eosinophilic gastritis may demonstrate a lacy mucosal pattern affecting the gastric antrum or, presents with persistent vomiting. a b c d 2 year old with cerebral palsy and 9 year old girl presents the skin and soft tissues. Symptoms are usually evident by the 20th year of age, but they may present anytime between 2 months and 70 years. In general more commonly, a marked nodular appearance in the gastric antrum with relative sparing of the body and fundus. chronic abdominal pain. with a small bowel the cutaneous and bone abnormalities develop approximately 10 years prior to polyposis. The gastrointestinal manifestations of Gardner syndrome Eosinophilic gastritis may mimic idiopathic hypertrophic pyloric stenosis demonstrating the typical sonographic A heterogeneous, T2 bright (a) rounded mass (arrow) is seen in the left supra-renal area, demonstrating marginal obstruction 3 days after include colonic adenomatous polyps, gastric and small intestinal adenomatous polyps and peri-ampullary carcinomas. Gastric fundic gland polyps occur findings of thickened and elongated pyloric channel. Hummer-Ehret et. al. have suggested that enhancement on T1 fat-saturated image (b). An ultrasound (c) demonstrates an echogenic mass in the same area surgical removal of gastric in approximately 90% of affected individuals. Most of these lesions are hyperplastic and carry no malignant potential. However, adenomatous polyps and demonstration of abnormal thickening of the mucosa and submucosal layers on ultrasound in the setting of a (arrows). These findings were concerning for an adrenal angiomyolipoma. However, abdominal CT with oral contrast bezoar. their progression to gastric cancer have rarely been described. thickened muscular layer may be indicative of eosinophilic infiltration. A reported association of IHPS and eosinophilic (d), performed previously at another institution, demonstrates this mass to be a gastric diverticulum (arrow), a finding confirmed at a later MR, when air was seen within the “mass”. gastroenteritis raises interesting questions about the possible etiologic relationship between the two entities. Two images from UGI series show the greater CT of the abdomen shows small (c) secondary curvature of the stomach lying superior to Figure 16 Figure 5 GASTRIC DIVERTICULA to retained trichobezoar in the the lesser curvature. Organoaxial volvulus A 14 year old female presented with abdominal pain and vomiting. c d (d, arrow). 17 year old male presents with vomiting and weight loss. Gastric diverticula (GD) are the least common of all gastrointestinal diverticula. The majority was found at surgery. c d a b (75%) are located within a few centimeters of the gastroesophageal junction, usually on the lesser curvature or posterior aspect. Although there is a wide variety in shape and size, typical diverticula There is elongation and narrowing of the pyloric channel which appears asymmetric and changeable TRICHOBEZOAR Images from an air are 1–6cm in diameter. These juxtacardiac diverticula are congenital in origin and covered by all during the examination. Pyloric muscle thickening is present (arrows), ranging from 3.5-5mm. Gastric volvulus is an abnormal rotation of the stomach leading to partial or total obstruction. contrast UGI show layers of the normal gastric wall. The remaining (25%) gastric diverticula are smaller in size and Trichobezoars are most commonly found in young females, typically in the setting of an underlying Endoscopy confirmed eosinophilic allergic gastritis. Patient improved with dietary modification. Depending on the axis of rotation, it may be classified into organoaxial, mesenteroaxial and mixed. marked narrowing located in the pylorus or gastric antrum. GD may be seen at cross-sectional imaging including psychiatric disorder. Trichobezoar formation occurs when ingested hair strands are retained in the In mesenteroaxial (MA) volvulus, the stomach rotates around an imaginary (short) axis passing and irregularity of the ultrasound, CT and MR and may be mistaken for more sinister supra-renal pathology. folds of the because their slippery surface prevents propulsion by . This through the greater and lesser curvatures such that the greater and lesser curvatures are in their Figure 2 pylorus & duodenal large quantity of hair becomes matted together and assumes the shape of the stomach, usually as a cap (arrows). Biposy usual positions relative to each other but there is reversal of the relationship of the gastroesophageal 8 week old male infant with vomiting and . Figure 9 single mass. at UGI endoscopy junction and pylorus. This creates a narrow pedicle about which the stomach can twist, leading A neonate presented with intolerance of feeds and recurrent non- confirmed Crohn’s to gastric obstruction and and presents as an acute emergency. In organoaxial (OA) Rapunzel syndrome is a rare form of trichobezoar. It is named after a charming tale written in 1812 a b c d a b c disease. bilious vomiting. volvulus, the stomach rotates around an imaginary (long) axis passing between the esophago- by the Brothers Grimm about a young maiden, Rapunzel, with long tresses who lowered her hair to Coronal HASTE (a) and axial T2 (b) MR images of the abdomen show a mass arising from the lesser curvature of the stomach (arrows). gastric junction and the pylorus, such that the greater curvature is positioned superior to and to the ground from high in her prison tower to permit her young prince to climb up to her window and T1 enhanced axial image shows enhancing liver lesions (c, arrows). PET image (d) reveals increased uptake in the gastric and liver lesions (arrows). A plain abdominal radiograph demonstrates marked distension of the stomach with Figure 6 the right of the lesser curvature. The stomach might flip upward along its long axis, but because it rescue her. Originally described in 1968, more than 25 cases have since been reported in the literature, Pathology: Gastrointestinal stromal tumor (GIST) of the stomach with liver metastases. an absence of distal bowel gas. Diagnosis: Pyloric Atresia 8 year old male presents with bright red blood per does not twist on itself there is no risk of ischemia. Obstruction, however, can occur. Organoaxial with variable clinical features and varied diagnostic criteria. The typical diagnostic features include volvulus is the most common whilst the mixed variety is extremely rare and difficult to differentiate and abdominal pain (a) and endoscopy PYLORIC ATRESIA a trichobezoar with a tail, extension of the tail at least to the jejunum, and symptoms suggestive GIST photo (b). both radiologically and intraoperatively. of obstruction. All the cases reported in the literature are females with the exception of one male Congenital pyloric atresia (CPA) accounts for less than 1% of intestinal a patient who ate his sisters’ hair. Gastrointestinal stromal tumors (GIST) are uncommon mesenchymal tumors of the gastrointestinal (GI) tract. Pediatric GIST cases are rare and have been On barium studies, organoaxial (OA) volvulus is diagnosed when the greater curvature of the atresias. The gastric outlet obstruction may be secondary to a true reported to represent only 1.4% of all GIST cases. Cases of pediatric GIST can be sporadic, familial, or found in association with Carney’s triad (GIST, pulmonary a b stomach lies superior to the lesser curvature and the pylorus points inferiorly. Mesenteroaxial (MA) UGI (a) shows nodular fold thickening in the gastric antrum atresia or caused by a membrane at the pylorus or gastric antrum. The atresia may be an isolated chondroma, and paraganglioma) or neurofibromatosis type 1. Pediatric GIST is a unique subtype with a tendency to present with gastric multinodular volvulus is diagnosed when the pylorus is displaced superiorly and toward the left (shortening Figure 14 Pyloric muscle thickening is present measuring 4-5mm with change in thickness during the exam, (arrows). UGI endoscopy photograph shows thickened antral abnormality or may be associated with epidermolysis bullosa/aplasia cutis congenita and other or multifocal disease. Gastric GISTs in children have mainly epithelioid morphology, often occur in the antrum, and have a somewhat unpredictable the distance between the pylorus and gastroesophageal junction), and the pylorus overlaps the Newborn presents with bilious vomiting. atypical for pyloric stenosis. There are prominent mucosal and submucosal layers (arrows). Upper fold (arrow) and biopsy confirmed Crohn’s disease. Terminal gastrointestinal atresias. The presence of associated anomalies is a contributing factor for the but slow course of disease. They have malignant potential, but their behavior is often difficult to predict. In children, two peaks of incidence have been a b gastroesophageal junction or gastric fundus. GI endoscopy showed hemorrhagic and ulcerated mucosa in the gastric antrum and colonoscopy was also present. reported high mortality. demonstrated - <1-year-old and between 10 and 15 years old. Pediatric GIST affects females more commonly than males, as opposed to adult GIST where revealed evidence of allergic . Pyloromyotomy was not performed and patient improved with A high index of suspicion is necessary to avoid confusion of CPA with , particularly males are more commonly affected. dietary modification. CROHN’S DISEASE Figure 12 on X-rays. The plain abdominal X-ray shows a solitary bubble due to a gastric entrapment of air. A On MR imaging, gastric GIST often present as a large exophytic mass with variable T1 and T2 signal corresponding to areas of necrosis and hemorrhage. The frequency of pediatric Crohn’s disease involvement of the upper GI tract is often underestimated. Gastric lesions long stretched-out peak at the pylorus is regarded as a pathognomonic sign of complete pyloric A 12 month old male infant presented with vomiting and a palpable mass in the mid-abdomen. Figure 3 Careful evaluation for abnormal increased T2 signal may help in identifying multifocal disease within the gastric wall. detected at endoscopy are often not reported on UGI examinations. Endoscopic and histological involvement closure. A double bubble may, however, be seen in cases with prolapse of a pyloric membrane 6 week old male with pyloric stenosis, for comparison. has been reported with rates varying between 30% and 80%. Upper GI tract Crohn’s disease is most common in into the , thus imitating duodenal atresia. The possibility of associated intestinal atresias the stomach (67%) and said to occur in the gastric antrum with greater frequency then the body of stomach. The must always be kept in mind and to exclude or locate associated colonic atresia, a preoperative duodenum is reported to be involved in 12-22% of patients. Findings on UGI series may help to localize disease prior barium enema is advocated. The presence of calcification on plain abdominal X-ray should raise Scout film (a) and UGI (b) reveals complete duodenal obstruction (arrow). Malrotation with to upper endoscopy. In many cases, granulomas are only found on upper endoscopy and histologic findings are the possibility of associated heredity multiple (HMIA). volvulus was found at surgery. 2 weeks following surgery the neonate remained intolerant Figure 17 equivocal in the colon. of feeds as a result of a secondary duodenal stenosis. An attempt was made at passing a naso-jejunal feeding tube under fluoroscopic guidance (c). Inadvertant gastric perforation 10 year old girl with abdominal pain and weight loss. Figure 10 occurred (d). Figure 7 2 year old with vomiting. a A 1 year old male presented with a prolonged history of vomiting a b c and failure to thrive. There was a background of recurrent CT image with oral contrast shows marked thickening of the gastric wall with luminal narrowing. Image from an UGI series shows mass effect on the greater curvature of the gastric More typical uniform muscular hypertrophy in a classic superficial skin and respiratory tract infections. An enhanced CT of the abdomen demonstrates an ectopically placed spleen in the midline as well as an obstructed Diagnosis: Burkitt Lymphoma antrum. Diagnosis: Gastric Duplication Cyst a a case of HPS. stomach with evidence of pneumatosis (arrow). GASTRIC DUPLICATION CYSTS BURKITT LYMPHOMA WANDERING SPLEEN with GASTRIC VOLVULUS Figure 4 Gastric duplications are rare, comprising only 4-9% of intestinal Primary malignant tumors of the stomach are uncommon in children and mainly consist of lymphoma and sarcoma. The majority of primary gastric a Wandering spleen is a rare condition characterized by the absence or underdevelopment of one 17 year old female with recurrent vomiting and peripheral eosinophilia. Upper GI barium study demonstrates concentric narrowing of the gastric antrum duplications, are more common in girls and occur along the greater lymphomas are high-grade large B-cell non-Hodgkin’s lymphomas (NHL). Among Caucasian children under the age of 15 years, lymphomas account for a (arrow). Subsequent investigations confirmed chronic granulomatous disease. or all of the ligaments that hold the spleen in its normal position in the left upper quadrant of curvature in most instances. Most gastric duplications are attached nearly 10% of all the malignant disease, of which 40% are histologically NHL. In the pediatric population 30-50% of all NHL will be of Burkitt type. Burkitt the abdomen. Most cases in children present when the child is younger than 1 year and there is b c d to the wall of the stomach and have been classified as tubular or cystic, the latter usually not Lymphoma (BL) is a highly aggressive neoplasm of mature B cells, is one of the fastest growing tumors with a potential tumor-cell doubling time of < 24 a male predominance. Splenic torsion, complicating 64% of pediatric cases of wandering spleen, CHRONIC GRANULOMATOUS DISEASE communicating with the stomach. Distal antral or pyloric cysts can present with gastric outlet hours and 90% of these will typically present with an abdominal tumor. In childhood, primary of the stomach is extremely rare, accounting for < 2% of is usually clockwise and can cause vascular congestion, infarction, and even gangrene of the GASTRIC PERFORATION obstruction, mimicking hypertrophic pyloric stenosis. Ectopic pancreatic tissue is present in 37% pediatric lymphoma cases. The few reported cases of gastric BL suggest that these tumors present as large masses with extensive local infiltration, but Chronic granulomatous disease of childhood is an inherited disorder of neutrophil phagocytosis characterized by spleen. Wandering spleen and gastric volvulus share a common cause, the absence or laxity of of gastric duplications. Neonatal gastric perforation is a rare condition, associated with high mortality and morbidity and an without a predilection for significant nodal or distant disease. recurrent infections with catalase-positive organisms. The disorder is much more common in males than females intraperitoneal visceral ligaments. (>80:20) and most patients present within the first 2 years of age. CGD can involve the from acute surgical emergency that must be promptly recognized and treated. Such patients usually present Gastric duplications typically present with partial obstruction before 12 months of age and an the to the rectum but the most common gastrointestinal manifestation is chronic antral gastritis leading with acute onset of rapidly progressive abdominal distension and massive pneumoperitoneum. The upper abdominal mass is often palpable on examination. Ultrasound displays the cystic nature, to progressive narrowing and gastric outlet obstruction. Upper GI series reveals narrowing of the antropyloric majority of cases present within the first 7-10 days of life. Although gastric distension, instrumentation, anatomic location and characteristic inner echogenic mucosal and outer hypoechoic muscle CONCLUSION lumen secondary to chronic inflammation and fibrosis. Sonographic evaluation demonstrates circumferential wall and necrotizing have been cited as predisposing factors to neonatal gastric perforation, layers of gastrointestinal duplication cysts. Computerized tomography (CT) is useful in defining a b c thickening at the antrum. Histologically, there is inflammation with granuloma formation and infiltration of lipid- most perforations appear to be truly spontaneous. the nature of the cyst and its relationship to other structures. The cyst usually has an attenuation Multimodality imaging, including upper GI series, CT, US, MRI and PET are useful tools when evaluating less common Ultrasound (a) reveals antral wall thickening (arrow). Contrast enhanced CT abdomen (b, c) shows antral laden histiocytes involving the , , smooth muscle, and serosa. No organisms are usually similar to that of water, and a rim of calcification may be visible, even in children. abnormalities of the stomach in children. wall thickening (arrows) and massive ascites. Upper endoscopy confirmed eosinophilic gastritis. Ascitic isolated from these lesions. fluid contained 95% eosinophils. Patient responded well to steroids and dietary restriction.

REFERENCES

Eosinophilic Gastroenteritis Pediatric Crohn’s Chronic Granulomatous Disease Gastric Diverticula Gastric Duplication Cysts Pyloric atresia Gastric Volvulus Trichobezoars Neonatal Gastric Perforation Gardner Syndrome GIST Burkitt Lymphoma

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