Dirk Schaper, A. Jassoy, W. Lässig (Klinik für Kinderchirurgie Krankenhaus St. Elisabeth und St. Barbara Halle und Institut für Radiologie und Klinik für Kinder- und Jugendmedizin Städtisches Krankenhaus Martha-Maria Halle-Dölau gGmbH )  

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14 Years  

Female  

GI-Stomach  

physical  

In-patient admittance because of reoccuring nausea, vomiting and stomach aches for 6 months. In the pediatrician's office, an epigastric tumor was palpated which was 15 cm in size. The 14 year-old girl was never seriously ill before this, and shows no signs of distress in schoolwork. She has been referred to a dermatologist due to loss of hair.  

Bezoars are conglomerates of undigestable materials like hair (i.e. Hairballs), and are usually located in the stomach. Physiologically, one usually observes this in birds (wool) or in cats. The undigestable left-overs are usually vomited. Bezoars in humans are differentiated due to their make-up. Most common are phytobezoars, which consist of undigestable plant material (cellulose, lignine), the second most common form are trichobezoars, which are usually due to trichophagia (eating of hair) combined with a psychological illness (trichotillomania). Also to the classifications of bezoars are the following: Lactobezoars of premature newborns, the pharmakobezoars due to the clumping of medicinal tablets/capsules. Aside from these four main groups, observations of mixed-types have been made.
The Rapunzel-Syndrome is when the the bezoar has a "pig-tail" which continues though the gut. This may be observed throughout the entire small intestines and can have an increasingly obstructive effect. Furthermore, gastrointestinal symptoms occur and the bezoar itself.  

<- view MRI 1

MRI 1: T2-weighted HASTE sequence, coronal.
Large inhomogeneous, hypointense mass in the T2-weighted image, which fills almost the entire stomach, and continues to the duodenum.






<- view MRI 2

MRI 2: T2-weighted HASTE sequence, coronal.
Large inhomogeneous, hypointense mass in the T2-weighted image, which fills almost the whole stomach, and continues to the duodenum.






<- view MRI 3

MRI 3: T2-weighted HASTE sequence, transversal: Here, a large, inhomogeneous, hypointense mass (which has sharp margins), which almost completely fills the stomach. There is distal extension through the pylorus into the duodenum.
There is a small fluid level to the right and lateral to the mass and air ventral to the mass inside the stomach.




<- view MRI 4

MRI 4: T1 weighted 3D-GE-Sequence with fat-saturation and before constrast application. Conspicuous fat saturation in the T1-weighted image, which shows inhomogeneous, hypointense mass in the stomach lumen without evidence of contrast uptake after Gd-DTPA application. Some air in the stomach to the right and lateral of the mass.




<- view MRI 5

MRI 5: T1 weighted 3D-GE-Sequence with fat-saturation after contrast application, transversal. Conspicuous fat saturation in the T1-weighted image, which shows an inhomogeneous, hypointense mass in the stomach lumen without evidence of contrast uptake after Gd-DTPA application. Some air in the stomach to the right and lateral of the mass. There is intense contrast uptake in the stomach wall.




<- view OP-Situs 1

OP-Situs 1: Specimen





<- view Pathology 1

Pathology 1: Specimen

 

Surgery / Histo  

Phytobezoar, Diosphyrobezoar, Pharmacobezoar  

After diagnostic imaging, initial attempt to reduce the size and removal of the bezoar endoscopically. Because of the size and consistency of the bezoar, it was not possible, and a gastrotomy and removal of the bezoar followed. In this case, this was a trichobezoar with a "pigtail" that reached into the duodenum - about 15 cm in size. The bezoar weighed 1700 grams. After wound healing, the patient was transferred to the clinic for child- and adolescent psychiatry.  

In our case, the endoscopic treatment was not possible, so that we decided upon a surgical intervention. In accordance to Alik et al., this was a Rapunzel Syndrome grade I°.  

Bezoars are known since the 12th century, where they were described initially in India. Because of their rarity and the uncertain pathogenesis, bezoars were given magical properties. Even the name, bezoar, comes from the arabic word "Bedzehr" - the persian "Padzahr" - or the hebrew "Beluzaar" - all meaning "opposite material." This, of course, increased the mystical meaning. In the mideval times, bezoars were seen as being valuable, and were even plated in gold. Today, bezoars are more or less a potential medical problem, whose complication rates should not be underestimated.
Next to the displacement related gastrointestinal symptoms, ileus situations may occur. Furthermore, ulcerations, strangulations and hemorrhages may result and have been described. The name, "Rapunzel Syndrome" was coined by Vaughan et al. in 1968, who referred to the Brothers Grimm fairytale.  

1. Medline:
Gockel,I., C.Gaedertz,H.-J.Hain,U.Winckelmann,M.Albani,D.Lorenz
Das Rapunzel-Syndrom
Chirurg 2003 74:753-756

2. Medline:
M.K.Sanders
Bezoars: From mystical charms to medical and nutritional management
Practical Gastroenterology 13 2004

3. Medline:
Balik,E.,I.Ulman,C.Taneli,M.Demircan
The Rapunzel syndrome. A case report and review of the literature
Eur J Pediatr Surg 3 (1993) 171-173

4. Medline:
Vaughan,E.D.,J.L.Sawyers,H,W.Scott
Rapunzel syndrome: An unusual complication of intestinal bezoar
Surgery 63: 339-343  

Bezoar, trichobezoar, hairball, foreign object, stomach, trichophagia, trichotillomania, hair eating, Rapunzel syndrome,, child, childhood, pediatric radiology  

Dirk Schaper, A Jassoy, W Lässig. Rapunzel syndrome grade I° due to trichobezoar. PedRad [serial online] vol 6, no. 1.
URL: www.PedRad.info/?search=20060104123424