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I am Smirniotopoulos
and this is MedPix® case number 10834
We have no significant financial nor
other disclosures to make. This case was contributed by
Eva Rubio. The patient is a 9-year-old girl who presents with abdominal pain
and feeding intolerance. A plain radiograph of
the abdomen demonstrates a mass in the left upper quadrant. If we look at a
slightly enlarged view,
we can see the contours of lung on both sides the central silhouette of the
heart.
We can see a soft tissue mass below the diaphragm that
is displacing partially air-filled bowel loops.
This is in the expected location and has the approximate shape of
the patient's stomach. If we look next at the CT scan,
we can see the liver and spleen in a heterogeneous
mottled mixed air and soft tissue opacity
in the location of the fundus of the stomach. Additional CT scans
demonstrate this lesion involving the body
the stomach and extending towards the location of the duodenum bulb.
Here a 3rd CT section outlines this
heterogeneous mass in the expected location of the stomach.
so the findings in this case are a plain radiograph
showing a left upper quadrant mass displacing bowel loops
downward and toward the right; and a CT scan shows a heterogeneous mass with
concentric rings of
mixed air and soft tissue attenuation in the expected location
of the patients stomach. We have several things to consider in the
differential diagnosis,
including an abscess, or a phlegmon, and emphsematous gastritis.
But, because the air is not within the wall of the stomach but within the
lumen of the stomach we know that this patient must have some other condition.
Here is an operative photograph. We can see the patient's navel.
We can see the retractors opening into the stomach;
and, we can see that this is exactly where the lesion should be.
Here is the resected specimen in this patient; and, it looks like a perfect cast
of the patient's stomach. If we look at this very carefully you can see the
fundus,
the body, and the antrum of the stomach and duodenal bulb.
We can also see what appears to be blonde hair and the location
of the second and third portions of the duodenum. This is a patient that has a
trichobezoar or hairball.
Because they hair is extending out stomach into the
small bowel
it is sometimes called Rapunzel syndrome.
This was proven by surgical resection. So this is a case pf
Rapunzel syndrome. Well what is Rapunzel syndrome?
It is a trichobezoar which is composed of hair.
It may be human, animal, doll hair, or wig hair.
It becomes matted into a cast of the stomach by the gastric churning.
The extension of the bezoar into the small bowel
transforms it into what is called Rapunzel syndrome -
after the Grimm's fairy tale of Rapunzel, who let her hair down
out the window to escape from the tower prison. Gastric acid denatures the protein
turn the hair in the stomach dark.
But, the hair in the duodenum continues to be a lighter color and looks blonde.
These patients may have halitosis, appetite loss,
abdominal pain, or even a palpable mass. Rapunzel syndrome is often associated
with psychiatric conditions including trichotillomania (compulsive hair-eating)
or hair-pulling, trichopagia or hair-
eating. Only about one percent of patients who have trichophagia actually
will develop a trichobezoar.
the condition is much more common in female patients - presumably because they
have longer hair.
It a typically presents between 13 and 20 years of age.
So this young girl had a long history of chewing on her doll's hair
and the trichobezoar extending into the small bowel qualifies this
as a Rapunzel syndrome. Just as a reminder,
National Hair Ball Awareness Day takes place annually
on April 27 and this poster is courtesy of the National Museum
of Health and Medicine. I'm Jim Smirniotopoulos,
and I approve this message. Thank you very much for your kind attention.