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Acquired Disorders
of GIT in children
Radiology
Acquired disorders of the stomach
Acquired disorders of the stomach in
children are not common and can be
conceptualized as disorders with
• an underlying congenital predisposition
(e.g., gastric volvulus),
• as inflammatory (e.g., peptic ulcer
disease), and
• as tumors or tumor-like conditions.
Radiologic point of view
• Fluoroscopic contrast studies remain the
mainstay of diagnostic examinations for most
abnormalities of the stomach.
• Depending on the specific concern and
underlying clinical conditions, the stomach
can also be evaluated with cross-sectional
imaging such as ultrasound and computed
tomography (CT), as well as nuclear medicine
studies.
Spontaneous Gastric Perforation
• Etiology: Spontaneous perforation of the stomach is an
uncommon event mainly seen in the neonatal period as
a cause of pneumoperitoneum. The etiology is
unknown, but possibilities include sudden gastric
distension with a degree of ischemia attributable to
perinatal hypoxia, a more distal bowel obstruction, and
congenital focal absence of the muscle of the gastric
wall. Beyond the neonatal period, perforation is rare
and usually secondary to trauma (tubes, catheters),
surgery (fundoplication), caustic ingestion, or peptic
ulcer. The most common presenting manifestations of
perforation include sudden onset of abdominal
distension, ileus, respiratory distress, and, less
frequently, cyanosis, fever, vomiting, and bloody stool.
Spontaneous Gastric Perforation
• Imaging: Abdominal radiography is the imaging
method of choice when perforation of the
gastrointestinal (GI) tract is suspected. As in any
other type of GI perforation, abdominal radiographs
will typically demonstrate free intraperitoneal air. A
reported suggestive sign of gastric perforation is the
lack of an air-fluid level in the stomach in a
horizontal beam view, and relative paucity of gas in
the distal bowel.
• Treatment and Follow-up: Gastric perforations
require surgical repair. At surgery, most of the
perforations are linear and are found high in the
stomach along the greater curvature.
Gastritis and Gastropathy
• Gastritis is a nonspecific term that refers to
the presence of inflammatory cells within the
gastric wall; the diagnosis is made
histologically, as the mucosa may appear
normal at endoscopy.
• It differs from gastropathies, which demonstrate
evidence of epithelial damage and regeneration but
have little in the way of inflammatory infiltrate, and
are often associated with specific conditions such as
portal hypertensive gastropathy.
Gastritis and Gastropathy
• In children, inflammatory changes in the
stomach can result from several etiologies,
including: infections (e.g., secondary
to Helicobacter pylori), severe stress (e.g.,
during a serious illness), chemicals (e.g.,
ingestion of corrosive substance), eosinophilic
gastritis, hypertrophic gastropathy (Ménétrier
disease of childhood), and secondary to other
systemic entities such as Crohn disease and
chronic granulomatous disease.
Peptic Ulcer Disease. Etiology
• Peptic ulcer disease represents ulceration of the gastric
or duodenal mucosa resulting from, on the one hand,
an imbalance between the mucosal protective
mechanisms and, on the other, the aggressive factors of
acid and pepsin production, injury, and infection.
• The gel layer, a protective bicarbonate and mucous
barrier lining the stomach, is approximately 0.2 to
0.5 mm in thickness and consists of 95% water and 5%
mucin glycoprotein. Breaches in this gel layer,
secondary to H. pylori or antiinflammatory drugs, result
in a continuum of damage to the underlying mucosa,
with ulceration occurring when damage extends to the
muscular layer.
Peptic Ulcer Disease
• Peptic ulcer disease in children may
be primary or secondary (induced by drugs, alcohol,
stress, or metabolic disease), with each form having
different manifestations and prognostic
implications.
• H. pylori has been recognized as a common human
pathogen associated with both inflammatory and
malignant conditions of the upper gastrointestinal
tract, and affects nearly all children with peptic
ulcer disease. Primary peptic ulcers are associated
with H. pylori infection.
Peptic Ulcer Disease
Primary and secondary peptic ulcers
According to the site - gastric or duodenal
Gastric ulcers are mostly seen in neonates and young
children: duodenal ulcers are more common after the
neonatal period and tend to be secondary to systemic
illness or chronic intake of medications such as non-
steroidal antiinflammatory agents.
Zollinger-Ellison syndrome causes secondary peptic
ulcer disease, often with multiple ulcerations caused
by increased acid generated by a gastrin-producing
tumor (Fig. 1)
Zollinger-Ellison syndrome secondary to a pancreatic gastrinoma in a 10 year-
old-boy. The coronal image of a contrast-enhanced computed tomography
shows marked segmental thickening of the gastric fundus and body
(arrowheads). Two hypodense lesion in the liver indicate metastases (arrows).
Peptic Ulcer Disease. Symptoms
• Vary with age; infants and young children
present with feeding problems and vomiting.
• In some patients, the first sign of peptic ulcer
disease may be upper or lower GI
hemorrhage or acute severe abdominal pain
due to perforation.
• Pain can be nocturnal or occur early in the
morning. Unlike in adults, the pain is neither
precipitated nor relieved by meals or antacid
use
Peptic Ulcer Disease. Imaging
• Endoscopy has assumed the primary role in the
diagnosis of ulcer disease over the past two decades,
while the role of the radiologist has dramatically
decreased and is now limited to the incidental case,
as UGI contrast studies have been demonstrated to
have a high false-negative rate for ulcer detection.
• However, these studies, as an initial tool to evaluate
the child with abdominal pain and vomiting, may
incidentally demonstrate the ulcer. Perforated ulcers
may be incidentally identified on CT in the
evaluation of a child with acute abdominal pain (Fig.
2)
Perforated duodenal ulcer in 16-year-old boy on nonsteroidal antiinflammatory regimen
for previous knee injury and surgery, presenting with acute onset of abdominal pain
while in school.
A - A contrast-enhanced computed tomography scan shows marked thickening of the
duodenal wall and small amount of free air (arrow).
B- The slightly more cephalad image shows fluid about the duodenum and additional
free air extending toward the area of the falciform ligament (arrow).
Peptic Ulcer Disease.
Treatment and Follow-up
Current therapy has been proven to be effective and
is based on medications that decrease acid
production.
In the cases of H. pylori infection, a combination
therapy:
a histamine-2 blocker or proton pump inhibitor,
Antibiotics.
Bismuth - necessary to eradicate the causative
organism and prevent both recurrence and
malignant complications
Hypertrophic Gastropathy (Ménétrier
Disease)
• Hypertrophy of the gastric rugal folds,
in association with protein-losing
enteropathy, in childhood is
labeled Ménétrier disease,
or hypertrophic gastropathy of
childhood. The clinical, pathologic, and
etiologic factors of this disease in children
differ from those of the adult form. In
adults, the disease is chronic and
premalignant..
Imaging
• Diagnosis is most commonly made with UGI contrast
studies demonstrating thickened gastric mucosal folds
in the fundus and body, sparing the antrum and
pylorus, with normal appearance of the small bowel.
Ultrasound has also been successfully used in
diagnosis.
• On the CT scan, similar findings of thickened rugal
folds in the fundus and body of the stomach can be
seen, with sparing of the antrum (Fig. 3).
• Endoscopy confirms the diagnosis.
• Differential diagnosis includes eosinophilic gastritis,
primary gastric lymphoma, gastric carcinoma,
inflammatory pseudotumor, gastric varices, Zollinger-
Ellison syndrome, lymphangiectasia, and anisakiasis if
there is a history of ingestion of raw fish.
Hypertrophic Gastropathy
• In children, the disease is self-limiting, with a peak
age of presentation of 5 years. Presentation
includes acute vomiting, diarrhea, upper
abdominal pain, anorexia.
• Peripheral edema is usually present and may be
associated with ascites and pleural effusions.
Rarely, signs of GI bleeding occur with coexisting
ulceration of the gastric rugae.
• The etiology of the disease remains unknown;
however, it has been previously associated with
several infectious agents, including
cytomegalovirus, H. pylori, mycoplasma, herpes
virus, and Giardia lamblia
Ménétrier disease.Contrast-enhanced coronal computed tomography
reformation shows the typical thickening of the gastric folds in the
fundus of the well distended stomach in a 4-year-old boy presenting
with 10 days of vomiting with streaks of blood, palpebral edema, and
hypoalbuminemia. Ménétrier disease confirmed with endoscopy and
gastric biopsy.
Ménétrier disease.Treatment and
Follow-up
• The disease in children is self-limiting
• After correction of hypoproteinemia,
complete clinical recovery usually occurs
within 2 to 4 weeks, although symptoms
found on radiologic and endoscopic
assessment may require months to
resolve.
Chronic Granulomatous Disease (CGD)
Etiology
• is a hereditary disorder of neutrophil function, which is
typically inherited as an X-linked recessive disorder, but
three autosomal recessive defects have also been
identified.
• The genetic alteration leads to a defect in activation of
the NADPH (nicotinamide adenine dinucleotide
phosphate-oxidase) molecule within the phagocyte,
preventing the formation of free radical superoxide in
the “respiratory burst,” and resulting in survival of
catalase-positive organisms within the phagocytes, with
chronic inflammatory reaction and granuloma
formation.
Chronic Granulomatous Disease (CGD)
• In the stomach, narrowing of the gastric
antrum is a distinctive manifestation of
CGD, occurring in 16% of cases. Gastric
outlet obstruction occurs in the X-linked
recessive form more commonly than in
the autosomal recessive form and
presents at a mean age of 44 months,
usually with severe vomiting.
Inflammatory Bowel Disease
• The incidence is equal in males and females, and the
peak onset is in adolescence or early adulthood. Both
ulcerative colitis and Crohn disease represent a chronic
inflammatory process without a known specific cause.
• Ulcerative colitis primarily involves the colon, whereas
Crohn disease involves primarily the small intestine.
The distinction between ulcerative colitis and Crohn
disease defies classification in as many as 10% of
patients. In children, the presentation may be
nonspecific, leading to a delay in diagnosis that ranges
from months to years.
• Clinical and laboratory markers for active disease are
inadequate; therefore repeated imaging is common,
especially in patients with Crohn disease
Chronic ulcerative colitis
• is an idiopathic inflammatory disease of the colon
that typically affects older children and young
adults; an infantile form has been described that is
devastating and often fatal.
• The disease is characterized by mucosal
inflammation, edema, ulceration, and it is
accompanied by submucosal edema in the early
stages and fibrosis in the later stages.
• Transmural disease is uncommon. The disease may
be localized in the distal colon, or it may spread to
involve the entire colon and the terminal ileum.
Skip areas are not characteristic, and their presence
should raise the diagnosis of Crohn disease.
Infantile ulcerative colitis in a 3-week-old girl with rectal bleeding.Frontal (A) and
lateral (B) projections during barium enema show tubular narrowing of the rectum and
sigmoid colon (arrows) with an abrupt transition zone. The findings were suggestive of
aganglionosis, but biopsy revealed ulcerative colitis.
Symptoms
• Bloody diarrhea may appear explosively in as many
as one third of affected patients, but the majority
come to medical attention with progressive chronic
diarrhea.
• Occasional patients are seen with toxic megacolon,
in which marked dilation of the large bowel,
primarily the transverse colon, is seen.
• Many children are first seen with
nongastrointestinal symptoms, of which severe
growth retardation is the most common and
clinically striking.
Symptoms
• Arthritis may precede the colon symptoms;
typically, it is monoarticular or pauciarticular and
affects large joints, although seronegative
spondyloarthropathy is seen in some affected
males.
• Skin rashes, uveitis, digital clubbing, stomal ulcers,
and hepatic dysfunction (primary sclerosing
cholangitis, autoimmune hepatitis) occur in a
variable number of children, but less frequently
than in adults. Patients with ulcerative colitis for 10
years or longer are at risk for colonic carcinomas,
which arise in areas of dysplastic mucosa rather
than in adenomatous polyps, and may be multiple.
Crohn Disease
• The disease can affect the colon and the small
intestine.
• Two features that favor the diagnosis of Crohn
disease over ulcerative colitis are the frequent
sparing of the rectum, and the presence of skip
areas in Crohn disease.
• Colonoscopy is often the initial examination in
patients with suspected Crohn colitis, because it
allows visualization of early changes and permits
biopsy for diagnosis. Capsule endoscopy is
commonly used in both adult and pediatric practice
to visualize small-bowel abnormalities.
Imaging of Ulcerative Colitis
• Abdominal radiographs are most often
nonspecific; typically, they show an
absence of recognizable stool from
affected colonic segments, and they may
show evidence of mucosal edema or
“thumbprinting” .
• Patients with toxic megacolon should not
undergo contrast enemas because of the
high risk of perforation
Ulcerative colitis in a 14-year-old girl.
A - Abdominal radiograph shows “thumbprinting” of the distal transverse colon,
suggesting submucosal edema. B - Double-contrast enema shows granularity and
irregularity of the colonic mucosa. Small ulcerations are seen throughout the
transverse colon and the descending colon. The entire colon was involved. C - Coned-
down view of the splenic flexure shows multiple areas of pseudopolyps.
Ulcerative colitis
• Double-contrast barium enema, formerly the
diagnostic imaging procedure of choice, has been
replaced by colonoscopy with biopsy.
• Ulcerative colitis always affects the rectum, with
contiguous proximal involvement. Skip areas do not
occur, although different parts of the colon may not be
equally affected. The terminal ileum may become
secondarily affected when there is proximal colonic
involvement; terminal ileal involvement is known
as backwash ileitis ( Fig -7). Ultimately, the colonic wall
becomes stiff, shortened, and tubular—the “lead
pipe” colon—secondary to fibrosis of the submucosa
(Fig.5).
• Late-stage disease produces presacral thickening, and
retroperitoneal fibrosis is a rare complication.
Ulcerative colitis with pseudopolyposis and “backwash ileitis” in a 15-
year-old boy. Multiple, round, marginal filling defects appear in the
transverse and descending portions of the colon, reflecting retained
islands of normal mucosa between areas of denuded mucosa. The
terminal ileum (arrows) is rigid and lacks a normal mucosal pattern.
Ulcerative colitis in a teenage girl. The
radiographs show both acute and chronic changes
of the colon resulting from ulcerative colitis.
A - Abdominal radiograph shows that the
transverse colon is very distended, and the left
colon has a fixed, narrow “lead pipe”
appearance.
B - In the lower abdomen, the right colon is
edematous, as seen by thumbprinting
(arrows). C -The enema reveals the featureless
and nondistensible left colon (“lead pipe”),
resulting from fibrosis in chronic ulcerative colitis.
• Computed tomography (CT) or magnetic resonance
imaging (MRI) can be performed to investigate disease
activity (abdominal pain, fever, or other symptoms), to
diagnose complications, or to identify associated liver
or biliary disease.
• When ulcerative colitis is active, cross-sectional imaging
shows colonic wall enhancement with preservation of
the smooth outer contour of the bowel ( Fig ).
• Surrounding fat stranding, mesenteric adenopathy,
ascites, and, when perforation occurs, abscesses may
also be evident, but extramural changes are much less
common than in Crohn disease. In chronic ulcerative
colitis, fatty changes may occur in the submucosa.
Pelvic image from abdominal-pelvic computed tomography in a 12-
year-old with known ulcerative colitis.The examination shows a smooth
outer wall with marked mucosal enhancement of the rectosigmoid
Crohn Disease
• As with ulcerative colitis, double-contrast barium
enemas are seldom used today for diagnosis or
monitoring of disease activity. Characteristic aphthous
ulcers are small and superficial, seen as an elevated
edematous halo with a central umbilication caused by
barium in the shallow ulcer crater. Eventually, the
inflammation becomes transmural, and the
characteristic “rose thorn” configuration develops from
deep ulcers that extend into the thickened bowel wall.
A “cobblestone” pseudopolyposis pattern, similar to
that seen in the small intestine, may be apparent: areas
of edematous mucosa separated by areas of denuded
mucosa and deep ulcerations.
Crohn Disease
• Small-bowel follow-through (SBFT)
examinations can identify complications
of diseases that affect the colon, and
sequelae such as enteric fistulae .
• Enteroclysis is helpful in unmasking focal
areas of disease activity, such as
strictures
Active Crohn disease with fistula formation on small-bowel follow-through.The
fistula (arrow) extends between the ileum and the medial wall of the cecum.
Crohn disease in a 12-year-old girl.The fluoroscopic enteroclysis image
shows a stricture of the terminal ileum (arrow). The stricture led to
proximal obstruction and dilation and required surgical resection.
Extent of extramural inflammatory changes and affected loops of bowel can be
identified, as can development of abscess or colonic strictures
Active Crohn disease in a 19-year-old. CT shows the distended ileum (arrow) proximal to the
thick-walled ileal loops and mesenteric stranding and vascular engorgement, resulting from
active inflammation. Mural thickening and vascular engorgement of colonic segments is
apparent. Positive oral contrast in the lumen interferes with evaluation of mucosal
Magnetic resonance enterography of active Crohn disease in an 8-year-old boy.
A -T2-weighted axial image of the pelvis shows several thickened, contiguous loops of
terminal ileum that represent active Crohn disease.
B- After gadolinium administration, bright signal enhancement appears in the bowel
wall relative to normal adjacent colon and rectum.

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Acquired Disorders GIT child Radiology.pptx

  • 1. Acquired Disorders of GIT in children Radiology
  • 2. Acquired disorders of the stomach Acquired disorders of the stomach in children are not common and can be conceptualized as disorders with • an underlying congenital predisposition (e.g., gastric volvulus), • as inflammatory (e.g., peptic ulcer disease), and • as tumors or tumor-like conditions.
  • 3. Radiologic point of view • Fluoroscopic contrast studies remain the mainstay of diagnostic examinations for most abnormalities of the stomach. • Depending on the specific concern and underlying clinical conditions, the stomach can also be evaluated with cross-sectional imaging such as ultrasound and computed tomography (CT), as well as nuclear medicine studies.
  • 4. Spontaneous Gastric Perforation • Etiology: Spontaneous perforation of the stomach is an uncommon event mainly seen in the neonatal period as a cause of pneumoperitoneum. The etiology is unknown, but possibilities include sudden gastric distension with a degree of ischemia attributable to perinatal hypoxia, a more distal bowel obstruction, and congenital focal absence of the muscle of the gastric wall. Beyond the neonatal period, perforation is rare and usually secondary to trauma (tubes, catheters), surgery (fundoplication), caustic ingestion, or peptic ulcer. The most common presenting manifestations of perforation include sudden onset of abdominal distension, ileus, respiratory distress, and, less frequently, cyanosis, fever, vomiting, and bloody stool.
  • 5. Spontaneous Gastric Perforation • Imaging: Abdominal radiography is the imaging method of choice when perforation of the gastrointestinal (GI) tract is suspected. As in any other type of GI perforation, abdominal radiographs will typically demonstrate free intraperitoneal air. A reported suggestive sign of gastric perforation is the lack of an air-fluid level in the stomach in a horizontal beam view, and relative paucity of gas in the distal bowel. • Treatment and Follow-up: Gastric perforations require surgical repair. At surgery, most of the perforations are linear and are found high in the stomach along the greater curvature.
  • 6. Gastritis and Gastropathy • Gastritis is a nonspecific term that refers to the presence of inflammatory cells within the gastric wall; the diagnosis is made histologically, as the mucosa may appear normal at endoscopy. • It differs from gastropathies, which demonstrate evidence of epithelial damage and regeneration but have little in the way of inflammatory infiltrate, and are often associated with specific conditions such as portal hypertensive gastropathy.
  • 7. Gastritis and Gastropathy • In children, inflammatory changes in the stomach can result from several etiologies, including: infections (e.g., secondary to Helicobacter pylori), severe stress (e.g., during a serious illness), chemicals (e.g., ingestion of corrosive substance), eosinophilic gastritis, hypertrophic gastropathy (Ménétrier disease of childhood), and secondary to other systemic entities such as Crohn disease and chronic granulomatous disease.
  • 8. Peptic Ulcer Disease. Etiology • Peptic ulcer disease represents ulceration of the gastric or duodenal mucosa resulting from, on the one hand, an imbalance between the mucosal protective mechanisms and, on the other, the aggressive factors of acid and pepsin production, injury, and infection. • The gel layer, a protective bicarbonate and mucous barrier lining the stomach, is approximately 0.2 to 0.5 mm in thickness and consists of 95% water and 5% mucin glycoprotein. Breaches in this gel layer, secondary to H. pylori or antiinflammatory drugs, result in a continuum of damage to the underlying mucosa, with ulceration occurring when damage extends to the muscular layer.
  • 9. Peptic Ulcer Disease • Peptic ulcer disease in children may be primary or secondary (induced by drugs, alcohol, stress, or metabolic disease), with each form having different manifestations and prognostic implications. • H. pylori has been recognized as a common human pathogen associated with both inflammatory and malignant conditions of the upper gastrointestinal tract, and affects nearly all children with peptic ulcer disease. Primary peptic ulcers are associated with H. pylori infection.
  • 10. Peptic Ulcer Disease Primary and secondary peptic ulcers According to the site - gastric or duodenal Gastric ulcers are mostly seen in neonates and young children: duodenal ulcers are more common after the neonatal period and tend to be secondary to systemic illness or chronic intake of medications such as non- steroidal antiinflammatory agents. Zollinger-Ellison syndrome causes secondary peptic ulcer disease, often with multiple ulcerations caused by increased acid generated by a gastrin-producing tumor (Fig. 1)
  • 11. Zollinger-Ellison syndrome secondary to a pancreatic gastrinoma in a 10 year- old-boy. The coronal image of a contrast-enhanced computed tomography shows marked segmental thickening of the gastric fundus and body (arrowheads). Two hypodense lesion in the liver indicate metastases (arrows).
  • 12. Peptic Ulcer Disease. Symptoms • Vary with age; infants and young children present with feeding problems and vomiting. • In some patients, the first sign of peptic ulcer disease may be upper or lower GI hemorrhage or acute severe abdominal pain due to perforation. • Pain can be nocturnal or occur early in the morning. Unlike in adults, the pain is neither precipitated nor relieved by meals or antacid use
  • 13. Peptic Ulcer Disease. Imaging • Endoscopy has assumed the primary role in the diagnosis of ulcer disease over the past two decades, while the role of the radiologist has dramatically decreased and is now limited to the incidental case, as UGI contrast studies have been demonstrated to have a high false-negative rate for ulcer detection. • However, these studies, as an initial tool to evaluate the child with abdominal pain and vomiting, may incidentally demonstrate the ulcer. Perforated ulcers may be incidentally identified on CT in the evaluation of a child with acute abdominal pain (Fig. 2)
  • 14. Perforated duodenal ulcer in 16-year-old boy on nonsteroidal antiinflammatory regimen for previous knee injury and surgery, presenting with acute onset of abdominal pain while in school. A - A contrast-enhanced computed tomography scan shows marked thickening of the duodenal wall and small amount of free air (arrow). B- The slightly more cephalad image shows fluid about the duodenum and additional free air extending toward the area of the falciform ligament (arrow).
  • 15. Peptic Ulcer Disease. Treatment and Follow-up Current therapy has been proven to be effective and is based on medications that decrease acid production. In the cases of H. pylori infection, a combination therapy: a histamine-2 blocker or proton pump inhibitor, Antibiotics. Bismuth - necessary to eradicate the causative organism and prevent both recurrence and malignant complications
  • 16. Hypertrophic Gastropathy (Ménétrier Disease) • Hypertrophy of the gastric rugal folds, in association with protein-losing enteropathy, in childhood is labeled Ménétrier disease, or hypertrophic gastropathy of childhood. The clinical, pathologic, and etiologic factors of this disease in children differ from those of the adult form. In adults, the disease is chronic and premalignant..
  • 17. Imaging • Diagnosis is most commonly made with UGI contrast studies demonstrating thickened gastric mucosal folds in the fundus and body, sparing the antrum and pylorus, with normal appearance of the small bowel. Ultrasound has also been successfully used in diagnosis. • On the CT scan, similar findings of thickened rugal folds in the fundus and body of the stomach can be seen, with sparing of the antrum (Fig. 3). • Endoscopy confirms the diagnosis. • Differential diagnosis includes eosinophilic gastritis, primary gastric lymphoma, gastric carcinoma, inflammatory pseudotumor, gastric varices, Zollinger- Ellison syndrome, lymphangiectasia, and anisakiasis if there is a history of ingestion of raw fish.
  • 18. Hypertrophic Gastropathy • In children, the disease is self-limiting, with a peak age of presentation of 5 years. Presentation includes acute vomiting, diarrhea, upper abdominal pain, anorexia. • Peripheral edema is usually present and may be associated with ascites and pleural effusions. Rarely, signs of GI bleeding occur with coexisting ulceration of the gastric rugae. • The etiology of the disease remains unknown; however, it has been previously associated with several infectious agents, including cytomegalovirus, H. pylori, mycoplasma, herpes virus, and Giardia lamblia
  • 19. Ménétrier disease.Contrast-enhanced coronal computed tomography reformation shows the typical thickening of the gastric folds in the fundus of the well distended stomach in a 4-year-old boy presenting with 10 days of vomiting with streaks of blood, palpebral edema, and hypoalbuminemia. Ménétrier disease confirmed with endoscopy and gastric biopsy.
  • 20. Ménétrier disease.Treatment and Follow-up • The disease in children is self-limiting • After correction of hypoproteinemia, complete clinical recovery usually occurs within 2 to 4 weeks, although symptoms found on radiologic and endoscopic assessment may require months to resolve.
  • 21. Chronic Granulomatous Disease (CGD) Etiology • is a hereditary disorder of neutrophil function, which is typically inherited as an X-linked recessive disorder, but three autosomal recessive defects have also been identified. • The genetic alteration leads to a defect in activation of the NADPH (nicotinamide adenine dinucleotide phosphate-oxidase) molecule within the phagocyte, preventing the formation of free radical superoxide in the “respiratory burst,” and resulting in survival of catalase-positive organisms within the phagocytes, with chronic inflammatory reaction and granuloma formation.
  • 22. Chronic Granulomatous Disease (CGD) • In the stomach, narrowing of the gastric antrum is a distinctive manifestation of CGD, occurring in 16% of cases. Gastric outlet obstruction occurs in the X-linked recessive form more commonly than in the autosomal recessive form and presents at a mean age of 44 months, usually with severe vomiting.
  • 23. Inflammatory Bowel Disease • The incidence is equal in males and females, and the peak onset is in adolescence or early adulthood. Both ulcerative colitis and Crohn disease represent a chronic inflammatory process without a known specific cause. • Ulcerative colitis primarily involves the colon, whereas Crohn disease involves primarily the small intestine. The distinction between ulcerative colitis and Crohn disease defies classification in as many as 10% of patients. In children, the presentation may be nonspecific, leading to a delay in diagnosis that ranges from months to years. • Clinical and laboratory markers for active disease are inadequate; therefore repeated imaging is common, especially in patients with Crohn disease
  • 24. Chronic ulcerative colitis • is an idiopathic inflammatory disease of the colon that typically affects older children and young adults; an infantile form has been described that is devastating and often fatal. • The disease is characterized by mucosal inflammation, edema, ulceration, and it is accompanied by submucosal edema in the early stages and fibrosis in the later stages. • Transmural disease is uncommon. The disease may be localized in the distal colon, or it may spread to involve the entire colon and the terminal ileum. Skip areas are not characteristic, and their presence should raise the diagnosis of Crohn disease.
  • 25. Infantile ulcerative colitis in a 3-week-old girl with rectal bleeding.Frontal (A) and lateral (B) projections during barium enema show tubular narrowing of the rectum and sigmoid colon (arrows) with an abrupt transition zone. The findings were suggestive of aganglionosis, but biopsy revealed ulcerative colitis.
  • 26. Symptoms • Bloody diarrhea may appear explosively in as many as one third of affected patients, but the majority come to medical attention with progressive chronic diarrhea. • Occasional patients are seen with toxic megacolon, in which marked dilation of the large bowel, primarily the transverse colon, is seen. • Many children are first seen with nongastrointestinal symptoms, of which severe growth retardation is the most common and clinically striking.
  • 27. Symptoms • Arthritis may precede the colon symptoms; typically, it is monoarticular or pauciarticular and affects large joints, although seronegative spondyloarthropathy is seen in some affected males. • Skin rashes, uveitis, digital clubbing, stomal ulcers, and hepatic dysfunction (primary sclerosing cholangitis, autoimmune hepatitis) occur in a variable number of children, but less frequently than in adults. Patients with ulcerative colitis for 10 years or longer are at risk for colonic carcinomas, which arise in areas of dysplastic mucosa rather than in adenomatous polyps, and may be multiple.
  • 28. Crohn Disease • The disease can affect the colon and the small intestine. • Two features that favor the diagnosis of Crohn disease over ulcerative colitis are the frequent sparing of the rectum, and the presence of skip areas in Crohn disease. • Colonoscopy is often the initial examination in patients with suspected Crohn colitis, because it allows visualization of early changes and permits biopsy for diagnosis. Capsule endoscopy is commonly used in both adult and pediatric practice to visualize small-bowel abnormalities.
  • 29. Imaging of Ulcerative Colitis • Abdominal radiographs are most often nonspecific; typically, they show an absence of recognizable stool from affected colonic segments, and they may show evidence of mucosal edema or “thumbprinting” . • Patients with toxic megacolon should not undergo contrast enemas because of the high risk of perforation
  • 30. Ulcerative colitis in a 14-year-old girl. A - Abdominal radiograph shows “thumbprinting” of the distal transverse colon, suggesting submucosal edema. B - Double-contrast enema shows granularity and irregularity of the colonic mucosa. Small ulcerations are seen throughout the transverse colon and the descending colon. The entire colon was involved. C - Coned- down view of the splenic flexure shows multiple areas of pseudopolyps.
  • 31. Ulcerative colitis • Double-contrast barium enema, formerly the diagnostic imaging procedure of choice, has been replaced by colonoscopy with biopsy. • Ulcerative colitis always affects the rectum, with contiguous proximal involvement. Skip areas do not occur, although different parts of the colon may not be equally affected. The terminal ileum may become secondarily affected when there is proximal colonic involvement; terminal ileal involvement is known as backwash ileitis ( Fig -7). Ultimately, the colonic wall becomes stiff, shortened, and tubular—the “lead pipe” colon—secondary to fibrosis of the submucosa (Fig.5). • Late-stage disease produces presacral thickening, and retroperitoneal fibrosis is a rare complication.
  • 32. Ulcerative colitis with pseudopolyposis and “backwash ileitis” in a 15- year-old boy. Multiple, round, marginal filling defects appear in the transverse and descending portions of the colon, reflecting retained islands of normal mucosa between areas of denuded mucosa. The terminal ileum (arrows) is rigid and lacks a normal mucosal pattern.
  • 33. Ulcerative colitis in a teenage girl. The radiographs show both acute and chronic changes of the colon resulting from ulcerative colitis. A - Abdominal radiograph shows that the transverse colon is very distended, and the left colon has a fixed, narrow “lead pipe” appearance. B - In the lower abdomen, the right colon is edematous, as seen by thumbprinting (arrows). C -The enema reveals the featureless and nondistensible left colon (“lead pipe”), resulting from fibrosis in chronic ulcerative colitis.
  • 34. • Computed tomography (CT) or magnetic resonance imaging (MRI) can be performed to investigate disease activity (abdominal pain, fever, or other symptoms), to diagnose complications, or to identify associated liver or biliary disease. • When ulcerative colitis is active, cross-sectional imaging shows colonic wall enhancement with preservation of the smooth outer contour of the bowel ( Fig ). • Surrounding fat stranding, mesenteric adenopathy, ascites, and, when perforation occurs, abscesses may also be evident, but extramural changes are much less common than in Crohn disease. In chronic ulcerative colitis, fatty changes may occur in the submucosa.
  • 35. Pelvic image from abdominal-pelvic computed tomography in a 12- year-old with known ulcerative colitis.The examination shows a smooth outer wall with marked mucosal enhancement of the rectosigmoid
  • 36. Crohn Disease • As with ulcerative colitis, double-contrast barium enemas are seldom used today for diagnosis or monitoring of disease activity. Characteristic aphthous ulcers are small and superficial, seen as an elevated edematous halo with a central umbilication caused by barium in the shallow ulcer crater. Eventually, the inflammation becomes transmural, and the characteristic “rose thorn” configuration develops from deep ulcers that extend into the thickened bowel wall. A “cobblestone” pseudopolyposis pattern, similar to that seen in the small intestine, may be apparent: areas of edematous mucosa separated by areas of denuded mucosa and deep ulcerations.
  • 37. Crohn Disease • Small-bowel follow-through (SBFT) examinations can identify complications of diseases that affect the colon, and sequelae such as enteric fistulae . • Enteroclysis is helpful in unmasking focal areas of disease activity, such as strictures
  • 38. Active Crohn disease with fistula formation on small-bowel follow-through.The fistula (arrow) extends between the ileum and the medial wall of the cecum.
  • 39. Crohn disease in a 12-year-old girl.The fluoroscopic enteroclysis image shows a stricture of the terminal ileum (arrow). The stricture led to proximal obstruction and dilation and required surgical resection.
  • 40. Extent of extramural inflammatory changes and affected loops of bowel can be identified, as can development of abscess or colonic strictures Active Crohn disease in a 19-year-old. CT shows the distended ileum (arrow) proximal to the thick-walled ileal loops and mesenteric stranding and vascular engorgement, resulting from active inflammation. Mural thickening and vascular engorgement of colonic segments is apparent. Positive oral contrast in the lumen interferes with evaluation of mucosal
  • 41. Magnetic resonance enterography of active Crohn disease in an 8-year-old boy. A -T2-weighted axial image of the pelvis shows several thickened, contiguous loops of terminal ileum that represent active Crohn disease. B- After gadolinium administration, bright signal enhancement appears in the bowel wall relative to normal adjacent colon and rectum.