Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 6  |  Issue : 2  |  Page : 200-204  

An adolescent girl with Rapunzel syndrome: Case report with review of literature


Department of Medicine, Padmashree Dr. D. Y. Patil Medical College, Hospital & Research Centre, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune, India

Date of Web Publication10-Apr-2013

Correspondence Address:
Pradnya M Diggikar
Department of Medicine, Pad. Dr. D. Y. Patil Medical College, Sant Tuka Ram Nagar, Pimpri, Pune
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.110315

Rights and Permissions
  Abstract 

Trichobezoar is a collection of dense mass of hair in stomach. When this extends into any part of the small intestine it is called Rapunzel Syndrome (RS). We report here, a case of RS in an adolescent girl who had presented with epigastric pain and swelling. Gastroscopy confirmed the presence of trichobezoar. She underwent gastrotomy and a large dense mass of hair extending up to first part of duodenum was removed. Her parents revealed their daughter's impulsive nature of scalp-hair pulling. Following surgery, Psychiatric consultation was sought to prevent recurrence. Trichobezoar is a very rare cause of upper abdominal mass and should be considered if there is a very strong history of impulsive hair pulling. Surgical removal of a large trichobezoar is the only treatment of cure and psychiatric treatment that prevents its recurrence.

Keywords: Bezoar, Rapunzel Syndrome trichobezoar, trichophagia, trichotillomania


How to cite this article:
Diggikar PM, Satpathy PK, Kakrani AL, Laddha M. An adolescent girl with Rapunzel syndrome: Case report with review of literature. Med J DY Patil Univ 2013;6:200-4

How to cite this URL:
Diggikar PM, Satpathy PK, Kakrani AL, Laddha M. An adolescent girl with Rapunzel syndrome: Case report with review of literature. Med J DY Patil Univ [serial online] 2013 [cited 2024 Mar 28];6:200-4. Available from: https://journals.lww.com/mjdy/pages/default.aspx/text.asp?2013/6/2/200/110315


  Introduction Top


''Rapunzel'' was the name of the maiden in the ''Grimm brothers'' fairy tale in 1812, whose long hair flowed out of her prison tower allowing her prince to rescue her. [1] Because of the resemblance of the tail of trichobezoar extending into the small intestine to the hair of Rapunzel, this condition is given the name-Rapunzel Syndrome (RS). [2]

RS was first described in the literature by Vaughan et al. in 1968, [2] in which a dense compact mass of hair (trichobezoar) was found in the stomach with extension into intestine through duodenum in patients with a history of psychiatric disorder. Trichotillomania is the habit of hair pulling and trichophagia is the morbid habit of chewing the hair.

Bezoars are compact masses formed of indigestible materials found in stomach. The term ''Bezoar" is believed to originate from the Arabic word ''Badzehr'', Persian word ''Padzahr'' or Turkish word ''Panzehir'', all of which mean substance that act as ''antidote'' or counter poison. [3] Bezoars are classified according to the content of the bezoar, such as trichobezoar(hair), phytobezoar (vegetable fibres), lactobezoar (milk products), pharmacobezoar (drugs), diospyrobezoars (persimmon fibres), cotton bezoars (cotton fibres). [4] Trichobezoars were first described by Baudomont in 1779. [5]

The presentation of RS forms a wide spectrum, varying from asymptomatic state to gastrointestinal ulceration, obstruction or perforation in young psychiatric patients.


  Case Report Top


A 15-year-old adolescent girl presented with loss of scalp hair of 2 months duration; upper abdominal pain and swelling of both lower limbs of 1 month duration. She was apparently in good health 2 months before whence her parents noticed lack of scalp hair growth and gradual loss of scalp hair mostly from the sides. There was no itching and discharge from the scalp. She did not use any unusual ''hair tonic'' or ''conditioner'' nor any drugs. One month prior to admission she developed mild dull aching constant upper abdominal pain without any food-pain relation, flatulence or retrosternal burning sensation. Soon she noticed mild slowly increasing upper abdominal swelling, gradual loss of appetite and occasional vomiting, and noticed slowly developing bilateral pedal oedema. There was no jaundice, hematemesis, fever, breathlessness, palpitation, cough, oliguria, and hematuria. Her diet was mixed and her bowel movements were normal. Her past and family history was unremarkable. She belonged to poor socioeconomic status. She was adequately immunized, unmarried with normal menstrual history.

Clinically, anxious looking with normal vital parameters, BMI-17kg/m 2 , mild anemia, mild bilateral pitting pedal oedema extending up to the knees. There was no jaundice, lymphadenopathy, or raised JVP. Abdominal examination revealed small epigastric swelling moving with respiration, normal skin texture, and absent distended veins. A 6 × 4cm 2 firm, non-tender, well-delineated lump occupying the epigastrium, with smooth surface, non-pulsatile, moving well with respiration, no rigidity or guarding. No other abnormal intra abdominal mass, no succussion splash, no free fluid, no bruit, normal bowel sounds, hernial sites free. PR examination-normal.

Other systemic examination-normal

Scalp-non-scarring, non-scaly frontal, temporal baldness with irregular outline, broken hairs with variable hair length-all features suggestive of traumatic alopecia (Patients profile photograph [Figure 1]). Mother gave very reliable history of her daughter's intermittent aggressive behavior with impulsive pulling of scalp hair followed by chewing, for the past few months.
Figure 1: Photograph of patient with loss of hair

Click here to view


A provisional diagnosis of trichotillomania, trichophagia with a strong possibility of trichobezoar with nutritional deficiency was entertained.

Laboratory Investigations-Hb-9 gm%, TLC-8500/cumm, DLC P-77%, L-20%, E-02%, M-01%; RBC-hypochromic, microcytic. Platelets-2.5 lakh/cumm. Urine examination-normal, LFT-normal, blood urea 25mg%, Sr. Creatinine 0.8% mg%, Sr. Electrolytes-normal. Total serum proteins 3.7 gm%, Sr. Albumin 1.2 gm%, Sr. Globulin-2.5 gm%. Plasma glucose 109mg%. Sr. Iron 23 microgm%, Sr. Ferretin-11 microgm%, TIBC was raised.

Abdominal USG-Hyperechoic curvilinear strip with dense acoustic shadow in the epigastric region [Figure 2].
Figure 2: USG abdomen showing hyperechoic curvilinear strip with dense acoustic shadowing in the epigastric region

Click here to view


Barium Studies-Distended stomach with flocculations of barium giving it a mottled appearance with dense acoustic shadow in stomach [Figure 3].
Figure 3: Barium study showing distended stomach with flocculation of barium giving it a mottled appearance

Click here to view


Upper G.I. endoscopy-A mass made up of black hair with entangled food and fibrous materials occupying the entire stomach and extending into first part of duodenum.

MRI Abdomen-A large area of hypointense signal intensity seen in stomach on all pulse sequences, shaped according to gastric lumen and extending into proximal part of duodenum [Figure 4].
Figure 4: MRI abdomen showing a large area of abnormal signal intensity in stomach as hypointense region extending into the proximal portion of the duodenum

Click here to view


She underwent elective gastrotomy. A large trichobezoar, occupying fundus, body, greater curvature, lesser curvature extending through the pylorus into the first part of duodenum was removed [Figure 5]a and 5b.
Figure 5: (a) Lump of hair lying inside the exposed stomach cavity. (b) Completely extracted trichobezoar from the stomach and its tail extending into the first part of duodenum, resembling Rapunzelæs hair

Click here to view


Gastric biopsy specimen histopathology revealed chronic non-specific gastritis. She made uneventful recovery; mild iron deficiency anemia and severe hypoalbuminemia were managed by oral iron replacement and parenteral albumin, respectively. Psychiatric consultation was taken and she was diagnosed as trichotillomania and managed successfully using the combination of pharmacotherapy and a package of behavior therapy. She has been on regular psychiatric OPD follow up for the last 5 years and there is no recurrence.

Final Diagnosis-Trichotillomania, Rapunzel Syndrome.


  Discussion and Review of Literature Top


We present here an adolescent girl withRS, an uncommon form of trichobezoar. RS was first described in literature by Vaughan et al. in 1968 [2] and recent literature review reveals about 100 odd cases of RS (Medline search till Sept. 2012), mostly reported by surgeons, pediatricians, and gastroenterologists. [6],[7],[8],[9],[10],[11],[12],[13] Very few cases are reported in psychiatric literature. An explanation for such disparity is due to the fact that most cases of trichotillomania are referred to psychiatrist early before the development of RS. [14],[15]

The formation of trichobezoar is complex. It occurs in psychiatric patients with trichotillomania and trichophagia. [15] The formation of trichobezoar in these patients depends upon the quantity and duration of trichophagia, on an average only 1% patients with trichophagia develop trichobezoar. [8],[16],[17] The slippery surface of the hair tufts resist the gastric peristaltic propulsion and tends to collect in the gastric mucosal folds. As more and more hair accumulates, a ball of hair is formed which becomes too large to escape and results in gastric atony and subsequently takes the shape of stomach. [9],[18],[19] Gastric mucous accumulates over the bezoar to make it glistening black. Decomposition and fermentation of fat over the bezoar imparts putrid smell to the patient's breath. [10] The acidic contents of the stomach denature hair protein and give the bezoar a jet black color. [11]

The clinical presentation forms a very wide spectrum depending on the duration and quantity of trichophagia. The commonest presenting symptoms are abdominal pain, nausea, early satiety, and vomiting. [6],[7],[8],[9],[10],[11],[12],[13] An upper abdominal mass remains the commonest presenting sign. [18] The signs and symptoms are due to mechanical effect of the mass and malabsorption of different nutrients. Other less common presenting features are gastrointestinal ulceration, obstruction, haemorrhage, perforation with peritonitis, acute pancreatitis, obstructive jaundice, and gastric emphysema. [12],[20],[21] Other malabsorption related complications include protein losing enteropathy, iron deficiency, and megaloblastic anemia. [19] The gastrointestinal perforation and peritonitis are largely responsible for mortality which is about 30%. [8] Our patient presented with upper abdominal mass, iron deficiency anemia, and severe hypoalbuminemia.

A high index of suspicion for trichobezoar arises from the classical clinical presentation in the background of psychiatric illness. [7],[8] Various diagnostic modalities confirm the diagnosis. Abdominal ultrasounds reveal an intraluminal mass with hypoechoic arc-like surface and dense acoustic shadow. [22] An abdominal CT scan shows a well-circumscribed lesion, composed of concentric whorls of different densities with pockets of air enmeshed within it, appears in the region of the stomach. Oral contrast fills the more peripheral interstices of the lesion and a thin band of contrast circumscribed it. [23] The absence of significant post intravenous contrast enhancement precludes a neoplastic lesion. [24] MRI abdomen shows a large mass of hypointense signal in stomach on all pulse sequences shaped according to gastric lumen with extension into small intestine in case of RS. [23] The gold standard for diagnosis is gastro-duodenal endoscopy. The presence of a dark greenish brown or black intragastric mobile mass with a slimy surface and a strong odor due to decomposition of various organic residue interspersed with hair is confirmatory. [25]

The management of RS includes the removal of the trichobezoar and prevention of recurrence by psychiatric treatment of the underlying psychiatric illness. Removal of trichobezoar is done either by surgery or by endoscopy. Endoscopic removal is more effective for small trichobezoars, also for phytobezoars and lactobezoars but not for large trichobezoars as in RS. Specialized bezotomes (device that pulverize bezoars) and bezotriptors (devices that fragment bezoars by acoustic waves) have been used to fragment large and solid trichobezoars. [26] Surgical removal is recommended in a very large trichobezoar causing perforation or hemorrhage [8]

Surgical removal is done traditionally by upper midline laparotomy and gastrotomy. [7],[8] Minimally invasive surgery is now available for small to moderate size trichobezoars. [6] Various other methods like lithotripsy, intragastric administration of pancreatic lipase, cellulose, acetylcystine are met with various success. [27] Our patient underwent midline laparotomy and gastrotomy for the removal of trichobezoar extending into duodenum.

Recurrence of trichotillomania and trichophagia as a cause of RS is prevented by managing the underlying psychiatric ailment using a combination of pharmacotherapy and a package of behavior therapy. [14],[28],[29],[30] Our patient was managed by both pharmacotherapy and behavior therapy and is on constant follow up and there has been no recurrence.


  Conclusion Top


RS, a gastric trichobezoar with intestinal extension, is quite uncommon, and should be considered strongly in a young patient with abdominal pain, and non-tender abdominal mass with history of trichotillomania. Surgical removal is the gold standard treatment of choice. Psychiatric treatment prevents its recurrence.

 
  References Top

1.Grimm Brothers: Rapunzel. Translated by Godwin-Jones R., Richmond, Virginia Common Wealth University Department of Foreign language; 1994-1999.  Back to cited text no. 1
    
2.Vaughn ED Jr., Sawyers JL, Scott HW Jr. The Rapunzel syndrome-an unusual complication of intestinal bezoar. Surgery 1968;63:339-43.  Back to cited text no. 2
    
3.Williams RS. The fascinating history of bezoars. Med J Aust 1986;145:613-4.  Back to cited text no. 3
    
4.Andrews CH, Ponsky JL. Bezoars: Pathophysiology, and treatment. Am J Gastroenterol 1988;83:476-8.  Back to cited text no. 4
    
5.Shorter E. A historical dictionary of Psychiatry. New York: Oxford University Press; 2005.  Back to cited text no. 5
    
6.Naik S, Gupta V, Naik S, Rangole A, Chaudhary AK, Jain P, et al. Rapunzel syndrome reviewed and redefined. Diag Surg 2007;24:157-61.  Back to cited text no. 6
    
7.Hirugada ST, Talpallikar MC, Deshpande AV, Gavali JS, Borwankar SS. Rapunzel syndrome with a long tail. Indian J Pediatr 2001;68:895-6.  Back to cited text no. 7
    
8.Phillips MR, Zaheer S, Grugas GT. Gastric trichobezoar: Case report and literature review. Mayo Clinic Proc 1998;73:653-6.  Back to cited text no. 8
    
9.Deslypere JP, Praet M, Verdonk G. An unusual case of the trichobezoar: The Rapunzel syndrome. Am J Gastroenterol 1982;77:467-70.  Back to cited text no. 9
    
10.Sidhu BS, Singh G, Khanna S. Trichobezoar. J Indian Med Assoc 1993;91:100-1.  Back to cited text no. 10
    
11.Sharma V, Sahi RP, Misra NC. Gastro-intertinal bezoar. J Indian Med Assoc 1991;89:338-9.  Back to cited text no. 11
    
12.Jiledar, Singh G, Mitra SK. Gastric perforation secondary to recurrent trichobezoar. Indian J Pediatr 1996;63:689-91.  Back to cited text no. 12
    
13.Sharma NL, Sharma RC, Mahajan VK, Sharma RC, Chauhan D, Sharma AK. Trichotillomania and Trichophagia leading to trichobezoar. J Dermatol 2000;27:24-6.  Back to cited text no. 13
    
14.Tiago S, Nuno M, João A, Carla V, Gonçalo M, Joana N. Trichophagia and Trichobezoar. Clin Pract Epidemiol Ment Health 2012;8:43-5.  Back to cited text no. 14
    
15.Bouwer C, Stein DJ. Trichobezoars in Trichotillomania: Case report and literature review. Psychosom Med 1998;60:658-60.  Back to cited text no. 15
    
16.Irving PM, Kadirkamanathan SS, Priston AV, Blanshard C. Education and imaging. Gastrointestinal: Rapunzel syndrome. J Gastroenterol Hepatol. 2007;22:2361.  Back to cited text no. 16
    
17.Christenson GA, Mackenzie TB, Mitchell JE. Characteristics of 60 adult hair pullers. Am J Psychiatry 1991;148:365-70.  Back to cited text no. 17
    
18.DeBakey M, Ochsner A. Bezoars and Concretions: A comprehensive review of literature with analysis of 303 collected cases and presentation of 8 additional cases. Surgery 1938;4:934-63.  Back to cited text no. 18
    
19.Gonuguntla V, Joshi DD. Rapunzel Syndrome: A comprehensive review of an unusual case of Trichobezoar. Clin Med Res 2009;7:99-102.  Back to cited text no. 19
    
20.Klipfel AA, Kessler E, Schein M. Rapunzel Syndrome causing gastric emphysema and small bowel obstruction. Surgery 2003;133:120-1.  Back to cited text no. 20
    
21.Kohler JE, Millie M, Neuger E. Trichobezoar causing pancreatitis: First reported case of Rapunzel syndrome in a boy in North America. J Pediatr Surg 2010;47e:17-9.  Back to cited text no. 21
    
22.Newman B, Girdany BR. Gastric trichobezoar-sonographic and compound tomographic appearance. Pediatr Radiol 1990;20:526-7.  Back to cited text no. 22
    
23.Sharma UK, Sharma Y, Chhetri RK, Makaju RK, Chapagain S, Shrestha R. Epigastric mass in a young girl: Trichobezoar: Imaging Diagnosis. Nepal Med Coll J 2006;8:211-2.  Back to cited text no. 23
    
24.Morris B, Shah ZK, Shah P. An intragastrictrichobezoar: Computerised tomographicc appearance. J Postgrad Med 2000;46:94-5.  Back to cited text no. 24
[PUBMED]  Medknow Journal  
25.De Backer A, Van Nooten V, Vandenplas Y. Huge gastric trichobezoar in a 10 year old girl: Case report with emphasis on endoscopy in diagnosis and therapy. J Pediatr Gastroenterol Nutr 1999;28:513-5.  Back to cited text no. 25
    
26.Wang YG, Seitz U, Li ZL, Soehendra N, Qiao XA. Endoscopic management of huge bezoars. Endoscopy 1998;30:371-4.  Back to cited text no. 26
    
27.Groenwalk CB, Smoot RL Farley DR.A football sized gastric mass in a healthy teen. Contemp Surg 2006;62:531-4.  Back to cited text no. 27
    
28.Kaur H, Chavan BS, Raj L. Management of tricchotillomania. Indian J Psychiatry 2005;47:235-37.  Back to cited text no. 28
[PUBMED]  Medknow Journal  
29.Swedo SE, Leonard HL, Rapoport JL, Lenane MC, Goldberger EL, Cheslow DL. A double blind comparison of clomipramine and desipramine in the treatment of trichotillomania (hair pulling). N Eng J Med 1989;321:497-501.  Back to cited text no. 29
    
30.Kumar V, Khatri AK, Pandey M, Shukla VK, Gangopadhyay AN. Recurrent trichobezoar: First reported case. Indian J Pediatr 1996;63:257-8.  Back to cited text no. 30
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


This article has been cited by
1 Compulsion beyond hairpulling
Dimple Gupta, Avisha Mahla, Akansha Bhardwaj, Nimmi Jose
International Journal of Advanced Medical and Health Research. 2023; 10(1): 50
[Pubmed] | [DOI]
2 Rapunzel Syndrome: A Case of Trichobezoar with Small Bowel Complications
Ramakrishna Narra, Anusha Guntamukkala, Chanda Bhaskara Rao, Tanveer Begum
The Surgery Journal. 2022; 08(04): e293
[Pubmed] | [DOI]
3 Trichobezoar obstructive syndrome in a pediatric patient: the Rapunzel Syndrome
Alessia DE FEO, Salvatore MIRESSI, Iolanda CAPALDO, Stefano CALABRESE, Sonia TAMASI, Rosanna MAMONE, Giovanni GAGLIONE, Paolo QUITADAMO, Massimo ZECCOLINI
Journal of Radiological Review. 2022; 9(1)
[Pubmed] | [DOI]



 

Top
   
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
Abstract
Introduction
Case Report
Discussion and R...
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed5945    
    Printed126    
    Emailed0    
    PDF Downloaded329    
    Comments [Add]    
    Cited by others 3    

Recommend this journal