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2018 ; 18
(1
): 125
Nephropedia Template TP
gab.com Text
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English Wikipedia
Rapunzel syndrome: an infrequent cause of severe iron deficiency anemia and
abdominal pain presenting to the pediatric emergency department
#MMPMID29614986
Cannalire G
; Conti L
; Celoni M
; Grassi C
; Cella A
; Bensi G
; Capelli P
; Biasucci G
BMC Pediatr
2018[Apr]; 18
(1
): 125
PMID29614986
show ga
BACKGROUND: Iron deficiency anemia (IDA) and abdominal pain are commonly seen in
a pediatric emergency department (8 and 18% incidence respectively in our
center). They are manifestations of a wide variety of diseases ranging from
benign to immediately life-threatening. Trichobezoar is an under-diagnosed entity
that has to be considered in children and adolescents, expecially female,
suffering from trichotillomania (compulsion to pull hair) and trichophagy
(compulsion to swallow hair). When undiagnosed, gastric bezoars may cause gastric
ulceration, perforation, haemorrhage and obstruction. CASE PRESENTATION: To
underline the importance of including this pathology in the differential
diagnosis of IDA and abdominal pain, we present the case of a 14 year-old girl
with a huge trichobezoar which completely filled the stomach and extended into
the small bowel. Since trichobezoar has an extension to the small bowel, it is
classified as Rapunzel syndrome. As the bezoar couldn't be removed by endoscopy,
the girl underwent surgical intervention. The patient passed through a gradual
re-feeding, with iron and vitamins supplementation, and through a psychiatric
counselling. CONCLUSION: The Rapunzel syndrome is a rare entity that may be
complicated by life-threatening events. A prompt diagnosis and an appropriate
therapy can reduce comorbidities. Gradual re-feeding with supplementation of
micronutrients allows adequate catch-up weight with normalization of
haematochemical nutritional parameters. Since many of these patients suffer from
psychiatric pathology such as PICA with emotional problems and mental
retardation, psychological/psychiatric counselling plays an important role in
order to prevent bezoar recurrence.