Introduction:
This syndrome is named after the girl with the long tresses in the fairy tale written by the Grimm Brothers in 1812. The Rapunzel syndrome was first reported in the literature by Vaughan et al. in 1968.1
For centuries bezoars have been known to occur in the form of undigested masses found in the stomach of animals and humans. Nevertheless, this finding has become more common in humans as a result of more frequent manipulation of the
gastrointestinal tract.2 From the increase in the incidence of this finding and the current ease
with which knowledge is disseminated today, it is clear that the Rapunzel syndrome, two cases of which were
reported by Vaughan et al., remains rare, a finding of phytobezoars being more common.2
This fact was recently confirmed in a literature review performed in 2007 that identified 27 cases
of Rapunzel syndrome described between 1968 and 2006.3 A few other reports on this syndrome
were published in the medical literature after this date.4-6
There are several different forms of presentation of this syndrome; however, in general, it involves
the presence of a gastric trichobezoar with a long tail extending beyond the duodenum, as found in the
case reported here. The factor responsible for this syndrome is the compulsion of patients to pull out
their own hair and swallow it, processes referred to as trichotillomania and trichophagia, disorders that
affect young girls with or without known psychiatric disorders.3-4
Case Presentation:
A 13 year old female came to surgery OPD with the history of abdominal mass associated with
epigastric pain, sense of fullness, vomiting after meals for the last 6 months. On examination she
was found mildly dehydrated, abdominal palpation (Fig.01) revealed a well defined mass occupying the
upper 1/3rd of the abdomen, the mass was not tender and was firm in consistency measuring
approx 8cm X 10 cm. On further evaluation, a microcytic anaemia was detected, while all the laboratory
parameters were within the normal range. An abdominal ultrasound revealed a superficially located broad
band of high amplitude echoes along the anterior wall of mass with sharp, clean post acoustic shadowing
and a hyperechoic curvilinear dense strip with acoustic shadowing and no through transmission. An upper
gastrointestinal contrast study was eventually performed which showed a grossly distended stomach with
irregular filling defects extending into the proximal gut (Fig.02), mottled filling defect and a plain
abdominal CT reported a mobile intragastric mass consisting of compressed concentric rings, with a high
density pattern due to the presence of entrapped air and food debries (Fig.03).
The patient was referred to Surgery, and through upper mid line incision the stomach
was opened (Gastrotomy fig.04) between two Vicryl stay suture. A huge Trichobezoar was identified which
took the shape of the stomach (Figure 05). There was a long tail of hair extending through the pylorus
into the small bowel (fig.06). By this feature the diagnosis was clear of a Rapunzel syndrome.Hairball
was removed intact and stomach was closed in double layers using continuous vicryl and silk sutures.
The patient had an uneventful postoperative course and was discharged after six days. The parents were
advised to visit pediatric psychiatry for follow up.
 |
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Fig. 1: Palpable abdominal mass |
Fig. 2: GI Contrast study |
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Fig. 3: CT image |
Fig. 4: Gastrostomy |
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Fig. 5: Trichbezoar removed |
Fig. 6: Rapunzel |
Discussion:
The common presentation of trichobezoar is in young females usually with an underlying
psychiatric disorder. In our case the presentation is in a very young age with hair extending down to
the small bowel, causing symptoms, which could mimic gastrointestinal infections and infestation
especially in endemic areas. The commonly accepted definition of Rapunzel syndrome is that of a
gastric trichobezoar with a tail extending to the jejunum, ileum or the ileocecal junction.
Majority of cases of trichobezoar present late, due to the low index of suspicion by
the physician. Of 131 collected cases of trichobezoar, a palpable abdominal mass was present in (87.7%),
abdominal pain (70.2%), nausea and vomiling (64.9%), weakness and weight loss (38.1%), constipation or
diarrhoea (32%) and haematemesis (6.1%). The laboratory investigations revealed low haemogiobin in about
62% (average).7
Patient with Trichotillomania (a psychological condition that involves strong urges to
pull hair), around 30% will engage in trichophagia, and of these, only 1% will go on to eat their hair
to the extent requiring surgical removal.8 Less than half of the patients give a history of
trichophagia. There has been few cases of recurrence following successful surgery.9
The early detection of trichophagia and trichobezoar depends on an effective screening
for trichotillomania and related behaviours, in order to prevent a possibly life-threatening condition
with important medical and surgical morbidity. Such effort must include a better collaboration between
medical and surgical specialties, dealing with particular aspects of therapeutic relationship regarding
shame and guilt as well as considering that trichophagia may be more often present than the majority of
clinicians, psychiatrists in particular, would expect.
Conclusion:
A trichobezoar represents a serious surgical condition. It is important to consider
such diagnosis in face of suggestive symptoms, even if signs of trichotillomania are not present.
The discrepancies between the prevalence of trichotillomania and trichobezoars due to trichophagia may be
due to issues related to self-selection of patients and symptom severity. Such issues may also be important
in the study of impulsive-compulsive spectrum models and to their relevance to impulse control disorders.
Acknowledgement: Dr A.K. Chaturvedi, Professor & HOD
References:
- Vaughan ED, Sawyers JL, Scott HW. The Rapunzel syndrome: an unusual complication of intestinal bezoar. Surgery. 1968;63(2):339–343.
- Andrus CH, Ponsky JL. Bezoars: classification, pathophysiology, and treatment. American Journal of Gastroenterology. 1988;83(5):476–478..
- Naik S, Gupta V, Naik S, et al. Rapunzel syndrome reviewed and redefined. Digestive Surgery. 2007;24(3):157–161.
- Pérez E, Sántana JR, García G, et al. Perforación gástrica en adulto por tricobezoar (síndrome de Rapunzel) Cirugía Española. 2005;78(4):268–270.
- Koç O, Yildiz FD, Narci A, Sen TA. An unusual cause of gastric perforation in childhood: trichobezoar (Rapunzel syndrome). A case report. European Journal of Pediatrics. 2009;168(4):495–497.
- Mohite PN, Gohil AB, Wala HB, Vaza MA. Rapunzel syndrome complicated with gastric perforation diagnosed on operation table. Journal of Gastrointestinal Surgery. 2008;12(12):2240–2242.
- Naik S, Gupta V, Naik S, Rangole A, Chaudhary AK, Jain P, Sharma AK. Rapunzel syndrome reviewed and redefined. Dig Surg 2007;24:157–161. doi: 10.1159/000107716
- Frey AS, McKee M, King RA, Martin A. Hair Apparent: Rapunzel Syndrome. Am J Psychiatry. 2005;162:242–248. doi: 10.1176/appi.ajp.162.2.242.
- Memon SA, Mandhan P, Qureshi JN, Shairani AJ. Recurrent Rapunzel syndrome – a case report. Med Sci Monit. 2003;9:CS92–4.
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