Keywords
trichobezoar - Rapunzel syndrome - intussusceptions - contrast-enhanced computed tomography
Bezoars are formed by indigestible material within the gastrointestinal tract that
eventually transforms into an agglomerate nondigestible mass. They are classified
according to their contents as phytobezoars (composed of fruit fibers or plants),
lactobezoars (composed of milk), trichobezoars (concretions of hair), and pharmacobezoars
(composed of medications).[1] Trichobezoars are associated with psychiatric illnesses such as trichotillomania
and trichophagia.[2] Approximately 1% of patients suffering from trichophagia develop trichobezoar.[3] Rapunzel syndrome is characterized by the presence of a trichobezoar in the stomach
with a tail that extends beyond the pylorus and causes mechanical bowel obstruction.[4] We here report a case of Rapunzel syndrome with small bowel complications.
Case Report
A 7-year-old female child presented to the emergency room with severe colicky epigastric
pain and nonbilious vomiting for 5 days. The patient had a past medical history of
trichotillomania and trichophagia. No signs of mental retardation. On general examination,
the patient appeared pale. She was afebrile, and all other vital signs were normal
for her age (heart rate: 115 beats/min, respiratory rate: 18 breaths/min, blood pressure:
110/70 mm Hg, oxygen saturations: 99% on room air, oxygen saturations: 100% on room
air, and capillary refill: <2 seconds). On abdominal examination, a firm nontender
mass was palpated, extending from epigastrium to left hypochondrium. Approximately
7.2 × 5.5 cm2 firm, nontender, well-delineated lump occupying the epigastrium with a smooth surface
was noted. There was no rigidity or guarding. Routine blood and urine investigations
performed demonstrated mild anemia. The rest of the parameters were within normal
limits (hemoglobin: 9 g%, total leukocyte count: 9500/cumm, differential leucocyte
count neutrophils: 75%, lymphocytes: 22%, eosinophils: 02%, macrophages: 01%, red
blood cell: hypochromic and microcytic, platelets: 2.5 lakh/cumm). Urine examination,
liver function tests, and serum electrolytes were within normal limits. Blood urea
22 mg% and serum creatinine 0.8% mg%. Total serum proteins 3.5 g%, albumin 1.2 g%,
globulin 2.3 g%, blood glucose 105 mg%, iron 23 microgm%, ferritin 11 microgm%, and
total iron binding capacity raised. She appeared well nourished and weighed 24.1 kg.
Her height is 121 cm
The plain radiograph demonstrated a distended stomach gas shadow. Routine ultrasound
abdomen showed nonspecific echogenicity with intense acoustic shadow within the stomach
and pylorus region. Upper gastrointestinal endoscopy revealed a large trichobezoar
([Fig. 1A, B]) filling the gastric lumen and obscuring the distal end of the stomach. Contrast-enhanced
computed tomographic scan of the abdomen and pelvis performed demonstrated a nonenhancing,
well-circumscribed heterogeneous filling defect with a typical “mottled gas pattern”
due to entrapped air in the body and pylorus of the stomach and tail extending into
the duodenum, jejunum, and proximal part of the ileum without any attachment to the
bowel wall with thickening of the wall of the duodenum ([Figs. 2]
[3]
[4]). In the proximal jejunum, telescoping of the small bowel loops was observed, suggestive
of jejuno-jejunal intussusceptions ([Fig. 5]). In addition, we noted dilated small bowel loops proximal to the obstruction and
bowel wall thickening. Findings were consistent with a large trichobezoar causing
jejuno-jejunal intussusceptions. Due to its large size, endoscopic removal was not
feasible, and exploratory laparotomy was performed under general anesthesia.
Fig. 1 (A, B) Upper gastrointestinal endoscopy images demonstrating large trichobezoar in the
body of the stomach extending into the antrum and pylorus obstructing the further
passage of endoscope.
Fig. 2 Axial oral and intravenous contrast-enhanced computed tomographic scan of abdomen
demonstrating a heterogenous density lesion (black arrow) within the lumen of stomach
with “mottled” appearance and clearly outlined by intraluminal oral contrast coincidental
gall bladder calculi (asterisk).
Fig. 3 Axial oral and intravenous contrast-enhanced computed tomographic scan of abdomen
demonstrating a heterogenous density lesion (black arrows) within the lumen of duodenum
with “mottled” appearance and clearly outlined by intraluminal oral contrast. Note
the dilated proximal jejunal loop (white arrow) secondary to the distal jejuno-jejunal
intussusceptions and duodenal wall thickening (black asterisk).
Fig. 4 Axial oral contrast-enhanced computed tomographic scan of abdomen demonstrating the
telescoping of the jejunal loops suggestive of jejuno-jejunal intussusception (white
arrow) with thickening of adjacent jejunal loops.
Fig. 5 Coronal multiplanar reformation of oral and contrast-enhanced computed tomographic
of abdomen demonstrating the trichobezoar (black arrows) outlined by oral contrast
media and contents in the stomach, duodenum, and proximal jejunum.
A midline laparotomy incision was given, and a duodenojejunal (DJ) junction was noted
to the left of the L1 vertebra. Masses were palpated at the stomach and small bowel.
Two concealed perforations were pointed out at the DJ junction and 40 cm distal to
the DJ junction. Trichobezoar was successfully removed through a separate enterotomy
incision at the antimesenteric border of the distal jejunal perforation segment as
the perforation site is close to mesentery ([Fig. 6]). DJ junction perforation was closed using 3–0 silk in two layers. Resection and
end-to-end anastomosis were performed at the distal jejunal perforation site. Given
trichotillomania, the patient was further referred for psychiatric evaluation. Postsurgical
follow-up was uneventful, and no significant complications occurred.
Fig. 6 Operative (A) and postextraction (B) images demonstrating the trichobezoar extraction by laparotomy and enterotomy, and
postoperative specimen.
Discussion
Rapunzel was a fairy tale character with long hair. Because of the resemblance of
the tail of a trichobezoar extending into the small intestine to the hair of Rapunzel,
this condition was named Rapunzel syndrome.[5] There have been only 45 cases of Rapunzel syndrome reported, and less than 15 cases
were reported with small bowel complications.[6]
Trichobezoar is a complex mass made of swallowed hair and makes up less than 6% of
all bezoars.[7] Human hair is resistant to digestion, and the ingested hair pieces are mixed with
mucus and food particles over a long period, forming a thin encapsulated mass. In
young females, trichobezoars are often associated with psychiatric illnesses such
as trichotillomania (hair-pulling) and trichophagia (hair swallowing).[8] Trichotillomania involves pulling the hair to the point of alopecia and is mainly
performed on the scalp, although eyelashes, eyebrows, and the axilla are all susceptible.
Only 30% of these patients will also engage in trichophagia, and of those that do,
only 1% will eventually develop a trichobezoar that requires surgical extraction.[3] Patients with trichobezoar usually present with nausea, vomiting, abdomen pain,
gastric ulcers, hematemesis, perforation, and intestinal obstruction.[9] Small bowel intussusception with or without small bowel obstruction(transient) may
be an associated complication. Regardless of the cause, it is important to remember,
particularly in recurrent cases, that bezoars may seldom be the undeclared cause of
intussusception. In some cases, the intussusceptions may be at multiple sites and
are transient.[10]
Nour et al reported a rare case of Rapunzel syndrome with generalized edema. Protein-losing
enteropathy, poor intake, malabsorption, and bacterial overgrowth may contribute to
hypoalbuminemia that occurs insidiously over a long period.[11]
The abdominal radiograph is nonspecific and may demonstrate a distended stomach shadow
with an intragastric mottled gas pattern outlined by fundal gas, which may resemble
a food-filled stomach. Barium studies may show an intraluminal filling defect with
a mottled gas pattern without attachment to the bowel wall. Transabdominal ultrasound
may demonstrate a hyperechoic curvilinear mass associated with posterior acoustic
shadowing within the stomach and pylorus region.[12] Computed tomography is a better radiological modality for demonstrating the size
and configuration of the bezoar. It shows the entire length of the bezoar as an intragastric
well-circumscribed mass consisting of a “mottled gas pattern” or “compressed concentric
rings” pattern due to the presence of entrapped air and food debris and tail extending
up to the duodenum or jejunum with oral contrast material dispersed within the mass
and surrounding it. Contrast-enhanced computed tomography scans can differentiate
the bezoar from the gastrointestinal tumors such as a gastrointestinal stromal tumor
extending into the stomach lumen and other bezoars like phytobezoar. Oral contrast
can trace a normal stomach wall separately from the lesion.[13] We can notice mucosal edema and wall thickening in the duodenum and jejunum. Oral
contrast demonstrates intussusceptions.[14]
With its limitations, magnetic resonance imaging is less beneficial than computed
tomography for diagnosing trichobezoar. The upper gastrointestinal endoscopy may demonstrate
a complex mass of hair within the stomach and detect other complications such as gastric
inflammation and ulcers.
Enzymatic degradation and medical treatment of trichobezoars are futile as they resist
them. Endoscopic removal of trichobezoars is mostly ineffective. The large size and
dense composition of the bezoar limit its endoscopic fragmentation. On endoscopic
removal, we should consider the possibility of migration of lysed bezoar fragments
into the small bowel, causing a secondary obstruction. Surgical removal through laparotomy
is the treatment of choice for trichobezoars given its high success rate, low complication
rate, low complexity, and potential to examine the small bowel and management of intussusception.
Long-term psychiatric counselling is essential to reduce the risk of recurrence of
trichotillomania and trichophagia.[15]
Conclusions
We present a case of Rapunzel syndrome with small bowel complications. In young females
with underlying psychiatric illness presenting with the features of small bowel obstruction,
one should exclude the possibility of trichobezoar. Computed tomography is superior
to other radiological imaging modalities for diagnosing trichobezoars as it helps
diagnose and demonstrate mechanical bowel complications.
