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CASE REPORT |
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Year : 2013 | Volume
: 6
| Issue : 2 | Page : 200-204 |
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An adolescent girl with Rapunzel syndrome: Case report with review of literature
Pradnya M Diggikar, Prasanna K Satpathy, Arjun L Kakrani, Mukesh Laddha
Department of Medicine, Padmashree Dr. D. Y. Patil Medical College, Hospital & Research Centre, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune, India
Date of Web Publication | 10-Apr-2013 |
Correspondence Address: Pradnya M Diggikar Department of Medicine, Pad. Dr. D. Y. Patil Medical College, Sant Tuka Ram Nagar, Pimpri, Pune India
Source of Support: None, Conflict of Interest: None | Check |
DOI: 10.4103/0975-2870.110315
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 |
Introduction | | |
''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 | | |
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.
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
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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
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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
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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
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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 | | |
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 | | |
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 | | |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
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