A previously healthy 2-year-old girl was seen by her pediatrician with a history of increasingly poor appetite and decreased energy and activity over the prior 2 months. She had occasional vomiting and foul-smelling stools. On physical examination, the child appeared pale and emaciated. Her weight had dropped from the 60th percentile at 19 months to below the 25th.
Timothy J. Carroll, MD, PhD
Janice M. Gallant, MD
from the Department of Radiology, Fletcher Allen Healthcare,
A previously healthy 2-year-old girl was seen by her
pediatrician with a history of increasingly poor appetite and
decreased energy and activity over the prior 2 months. She had
occasional vomiting and foul-smelling stools. On physical
examination, the child appeared pale and emaciated. Her weight had
dropped from the 60th percentile at 19 months to below the 25th.
She was lethargic, but cooperative. Her abdomen was protuberant,
soft, and nontender. There was no organomegaly. An abdominal
ultrasound was performed (Figure 1). A subsequent computed
tomography (CT) scan demonstrated no abnormality (not shown).
Small bowel intussusception associated with celiac sprue
An ultrasound of the abdomen was normal except for a target
appearance to the bowel in the mid-abdomen (Figure 1). A focal
segment of bowel showed a hypoechoic rim of thickened bowel wall
measuring 6 to 7 mm and heterogeneous echogenic structures within
the lumen, which appeared to be small bowel loops. These findings
are classically compatible with intussusception.
A repeat examination (not shown) performed 45 minutes later
revealed no abnormality, suggesting intermittent intussusception.
There was no tenderness, ascites, or fluid-filled bowel.
A gluten-free diet was started empirically (on the mother's
initiative following an Internet search). The patient improved
within 2 days: vomiting ceased, appetite improved, and she returned
to normal activities. Laboratory studies were performed for
endomysial antibodies IgA and anti-gliadin antibodies; IgG and IgA
were strongly positive with endomysial IgA titer 1:10,240, gliadin
IgG 108, and gliadin IgA >500. At follow-up, physical
examination showed a normal, active 2-year-old child with a 9-oz
weight gain. A biopsy, the diagnostic test of choice for celiac
sprue, was not performed at the mother's request because of her
child's dramatic improvement on dietary therapy. Because the
laboratory results were strongly suggestive of a diagnosis of
celiac sprue, this was the presumptive diagnosis, and the patient's
progress was followed on a gluten-free diet with continuing good
Intussusception is easily diagnosed by ultrasound. Sonographic
appearance can be variable in detail but most characteristically
shows a target or donut configuration of bowel with a hypoechoic
rim and a dense central echogenic core and no central movement in
This pattern is known as the crescent-in-donut sign.
Studies have shown this to be a sensitive and specific sign of
Intussusception in adults is much less common than in children,
and it is more often caused by a specific gastrointestinal (GI)
abnormality, usually a neoplasm.
Adult intussusception is not an uncommon complication of sprue, but
it is asymptomatic.
In the pre-CT and ultrasound GI radiology literature, it was
considered a specific sign of celiac disease.
Celiac sprue, or gluten-sensitive enteropathy, is an autoimmune
reaction to the gliadin fraction of gluten protein found in wheat,
rye, oats, and barley. It does not present until gluten-containing
foodstuffs have been introduced into the diet. Typically this means
the disorder presents between 6 months and 2 years, the same age
range as idiopathic intussusception, but it can manifest at any
age. It affects more than 1 in 10,000 live births. The incidence
appears to be much higher in Europe than in the United States. It
is a malabsorption syndrome whose pathophysiology is the
obliteration of intestinal villi and drastic reduction in
absorptive surface area. Common signs and symptoms include
diarrhea, failure to thrive, vomiting, anorexia, abdominal
distension, and foul stools. Abdominal pain is rare. Affected
children are often irritable and unhappy. There is no cure. The
only treatment is a gluten-free diet that is a lifetime commitment
that restricts patients to corn and rice.
Until recently, diagnosis of sprue has depended on serial
small-bowel biopsy that shows the characteristic histologic changes
and interval improvement with cessation of gluten consumption.
Antigliadin antibody tests, the first serologic tests, were not
very reliable. Newer IgA-endomysial antibody tests have approached
a sensitivity and specificity of 100%. Biopsy is still the
definitive test, in particular if a repeat biopsy returns to normal
following a gluten-free diet. The serologic tests normalize over 6
to 12 months following institution of a gluten-free diet, and this
has been accepted as an alternative way to follow the resolution of
sprue, instead of repeat biopsy.
The case of a childhood small-bowel intussusception apparently
caused by sprue has been rarely reported and is usually reported
There is one U.S. based report.
One study using ultrasound in 17 pediatric sprue patients did not
mention observing intus-susception.
The largest prospective study of ultrasound for suspected
intussusception showed many cases of clinically suspected
intussusception that were not confirmed by radiologic study.
The fraction of these cases that represent intermittent or
transient cases with spontaneous resolution is unknown.
Infantile intussusception is considered a life-threatening
emergency that requires immediate radiological and possible
This is a case of a child with an unexpected finding of transient
intussusception on ultrasound apparently associated with active