Short Communication 297
Rapunzel’s Syndrome – A Rare Phenomenon Das Rapunzel-Syndrom – ein seltenes Phänomen
▼
Rapunzel Syndrome is a rare phenomenon describing a trichobezoar which extends through the stomach into the small intestine or even colon. It is named after the fairy tale of the Grimm brothers and was first reported under that name in 1968 (Vaughan ED et al., Surgery 1968; 63: 339– 343), whereas Rolleston describes already in 1924 a similar case of a girl who died from a “hair ball of the stomach” in 1917 (Rolleston JD, Proc R Soc Med. 1924; 17 (Sect Study Dis Child): 5–8). He himself cited a first recorded case of Trichobezoar from Baudamant in 1779 (Baudamant M, Hist Soc Roy Med 1777–1779; 2: 262–263). From 1968–2006 27 cases were reported world-wide, with only one case in a male patient (Naik S et al., Digestive Surgery 2007; 24: 157–161). Since then more and more case reports have followed, with 53 publications listed in PubMed from 2007 by now (PubMed: “Rapunzel Syndrome case report” 14.05.2014). Most of the cases affect patients between 13 and 20 years of age (Gonuguntia V et al., Clin Med Res 2009; 7: 99–102), often in the course of psychiatric disorders. A history of trichotillomania and trichotillophagia is often neglected. The swallowed hairs, which are resistant to digestion due to their smooth surface, accumulate in the stomach and form a growing mass. This condition may remain asymptomatic for years. Besides of unspecific abdominal symptoms like vomiting, nausea, loss of weight and malnutrition it may in later stages also cause life-threatening complications like bowel-obstruction, -perforation or peritonitis (Gorter RR et al., Pediatr Surg Int 2010; 26: 457–463). Therefore an early diagnosis is crucial. Diagnostic procedures may include labaratory tests, ultrasound, barium contrast study, abdominal x-ray, CT-scan, MRI and endoscopy. Gold standard of the therapy remains the open surgical removal, as endoscopic and laparoscopic approaches are most often not successful. Moreover the open surgery allows the examination of the whole bowel system in order to determine satellites (Gorter RR et al., Pediatr Surg Int 2010; 26: 457–463). Medical treatment and enzymatic degra-
Fig. 1 Physical examination with a palpable mass in the left upper abdominal region.
Fig. 2 Gastrografin swallow shows a mass not infiltrating the borders of the stomach.
Fig. 3 Oesophagogastroduodenoscopy revealed a trichobezoar beginning in the antrum and extending to the duodenum.
Rickert AT et al. Rapunzel’s Syndrome… Klin Padiatr 2014; 226: 297–298 · DOI 10.1055/s-0034-1385867
Downloaded by: Georgetown University Medical Center. Copyrighted material.
Introduction
298 Short Communication
Conclusion
▼
Although rare, trichobezoars should be taken into consideration in patients presenting with unspecific abdominal symptoms and respective diagnostic procedures should be initiated to prevent life-threatening complications. Often underlying psychiatric disorders need to be treated to prevent relapse.
Central statements
Fig. 4 The bezoar was removed during open laparotomic surgery.
dation was also reported as ineffetive (Jensen AR et al., J Pediatr Surg 2005; 40: 1364–1365, Coulter R et al., South Med J. 2005; 98: 1042–1044).
Our Case
▼
An 11-year-old Chinese girl was referred to our hospital by her pediatrician because a palpabel mass was noticed in the left upper ▶ Fig. 1). abdominal region 4 days before (●
The clinical examination revealed no further abnormalities, especially no signs of alopezia or malnutrition. She only complained about a reduced appetite, no weight loss and no further abdominal symptoms had been noticed. There was no known history of a psychiatric disorder. Blood tests showed normal findings, no anemia, no vitamin deficiencies and normal levels of liver and pancreatic enzymes. The performed ultrasound of the abdomen revealed an
Rickert AT et al. Rapunzel’s Syndrome… Klin Padiatr 2014; 226: 297–298 · DOI 10.1055/s-0034-1385867
– Rapunzel Syndrome is a rare but important differential diagnosis of unspecific abdominal symptoms. – Gold standard of therapy is open surgery. – Underlying is in most cases a psychiatric disorder which requires psychiatric assessment and therapy.
Conflict of interest: The authors have no conflict of interest to disclose. A. T. Rickert, G. Düker, A. Heydweiller, J. Strohm, M. Born, I. Franke; Bonn, Germany
Downloaded by: Georgetown University Medical Center. Copyrighted material.
extremely dilated stomach with echogene content. The gastrografin-swallow showed that the mass was not infiltrating the borders ▶ Fig. 2). of the stomach (● Only on explicit questioning, the patient admitted a history of trichotillophagia some years ago. She was not able to tell wether she was still doing that. In the subsequently performed gastroduodenoscopy a trichobezoar was found, beginning in the antrum and extending to the ▶ Fig. 3). duodenum (● An endoscopic mobilisation of the bezoar was unsuccessful, so the patient under▶ Fig. 4). went open laparotomic surgery (● She recovered quickly and could be discharged from hospital 8 days after surgery.
Rapunzel’s Syndrome – A Rare Phenomenon Das Rapunzel-Syndrom – ein seltenes Phänomen
▼
Rapunzel Syndrome is a rare phenomenon describing a trichobezoar which extends through the stomach into the small intestine or even colon. It is named after the fairy tale of the Grimm brothers and was first reported under that name in 1968 (Vaughan ED et al., Surgery 1968; 63: 339– 343), whereas Rolleston describes already in 1924 a similar case of a girl who died from a “hair ball of the stomach” in 1917 (Rolleston JD, Proc R Soc Med. 1924; 17 (Sect Study Dis Child): 5–8). He himself cited a first recorded case of Trichobezoar from Baudamant in 1779 (Baudamant M, Hist Soc Roy Med 1777–1779; 2: 262–263). From 1968–2006 27 cases were reported world-wide, with only one case in a male patient (Naik S et al., Digestive Surgery 2007; 24: 157–161). Since then more and more case reports have followed, with 53 publications listed in PubMed from 2007 by now (PubMed: “Rapunzel Syndrome case report” 14.05.2014). Most of the cases affect patients between 13 and 20 years of age (Gonuguntia V et al., Clin Med Res 2009; 7: 99–102), often in the course of psychiatric disorders. A history of trichotillomania and trichotillophagia is often neglected. The swallowed hairs, which are resistant to digestion due to their smooth surface, accumulate in the stomach and form a growing mass. This condition may remain asymptomatic for years. Besides of unspecific abdominal symptoms like vomiting, nausea, loss of weight and malnutrition it may in later stages also cause life-threatening complications like bowel-obstruction, -perforation or peritonitis (Gorter RR et al., Pediatr Surg Int 2010; 26: 457–463). Therefore an early diagnosis is crucial. Diagnostic procedures may include labaratory tests, ultrasound, barium contrast study, abdominal x-ray, CT-scan, MRI and endoscopy. Gold standard of the therapy remains the open surgical removal, as endoscopic and laparoscopic approaches are most often not successful. Moreover the open surgery allows the examination of the whole bowel system in order to determine satellites (Gorter RR et al., Pediatr Surg Int 2010; 26: 457–463). Medical treatment and enzymatic degra-
Fig. 1 Physical examination with a palpable mass in the left upper abdominal region.
Fig. 2 Gastrografin swallow shows a mass not infiltrating the borders of the stomach.
Fig. 3 Oesophagogastroduodenoscopy revealed a trichobezoar beginning in the antrum and extending to the duodenum.
Rickert AT et al. Rapunzel’s Syndrome… Klin Padiatr 2014; 226: 297–298 · DOI 10.1055/s-0034-1385867
Downloaded by: Georgetown University Medical Center. Copyrighted material.
Introduction
298 Short Communication
Conclusion
▼
Although rare, trichobezoars should be taken into consideration in patients presenting with unspecific abdominal symptoms and respective diagnostic procedures should be initiated to prevent life-threatening complications. Often underlying psychiatric disorders need to be treated to prevent relapse.
Central statements
Fig. 4 The bezoar was removed during open laparotomic surgery.
dation was also reported as ineffetive (Jensen AR et al., J Pediatr Surg 2005; 40: 1364–1365, Coulter R et al., South Med J. 2005; 98: 1042–1044).
Our Case
▼
An 11-year-old Chinese girl was referred to our hospital by her pediatrician because a palpabel mass was noticed in the left upper ▶ Fig. 1). abdominal region 4 days before (●
The clinical examination revealed no further abnormalities, especially no signs of alopezia or malnutrition. She only complained about a reduced appetite, no weight loss and no further abdominal symptoms had been noticed. There was no known history of a psychiatric disorder. Blood tests showed normal findings, no anemia, no vitamin deficiencies and normal levels of liver and pancreatic enzymes. The performed ultrasound of the abdomen revealed an
Rickert AT et al. Rapunzel’s Syndrome… Klin Padiatr 2014; 226: 297–298 · DOI 10.1055/s-0034-1385867
– Rapunzel Syndrome is a rare but important differential diagnosis of unspecific abdominal symptoms. – Gold standard of therapy is open surgery. – Underlying is in most cases a psychiatric disorder which requires psychiatric assessment and therapy.
Conflict of interest: The authors have no conflict of interest to disclose. A. T. Rickert, G. Düker, A. Heydweiller, J. Strohm, M. Born, I. Franke; Bonn, Germany
Downloaded by: Georgetown University Medical Center. Copyrighted material.
extremely dilated stomach with echogene content. The gastrografin-swallow showed that the mass was not infiltrating the borders ▶ Fig. 2). of the stomach (● Only on explicit questioning, the patient admitted a history of trichotillophagia some years ago. She was not able to tell wether she was still doing that. In the subsequently performed gastroduodenoscopy a trichobezoar was found, beginning in the antrum and extending to the ▶ Fig. 3). duodenum (● An endoscopic mobilisation of the bezoar was unsuccessful, so the patient under▶ Fig. 4). went open laparotomic surgery (● She recovered quickly and could be discharged from hospital 8 days after surgery.
