Gastric masses {Trichobezoar with Rapunzel Syndrome}

 
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Question :
Charu Tiwari, Neha Sisodiya Shenoy, Mukta Waghmare, Kiran Khedkar, Hemanshi Shah
Department of Pediatric Surgery, Topiwala Nair Medical College and BYL Nair Hospital, Mumbai, Maharashtra, India.

Address for Correspondence: Prof Hemanshi Shah, Professor & Head, Department of Paediatric Surgery, TNMC & BYL Nair Hospital, Mumbai, Maharashtra - 400008, India. E-mail: hemanshisshah@gmail.com

Case 1: A 10 year female child presented with abdominal pain, nausea and vomiting associated with abdominal lump and weight loss. A non-tender mobile mass was palpated in the epigastric region. Abdominal ultrasound (USG) showed an 8 x 7 x 6cm echogenic mass with posterior acoustic shadow. An upper gastrointestinal contrast study showed a mobile intraluminal filling defect in the stomach. The patient underwent mini-laparotomy with gastrotomy and removal of the mass. Case 2: A 7 year old girl was admitted with an asymptomatic epigastric lump. A non-tender mobile mass could be palpated in epigastric region. USG showed a 12 x 8 x 6cm echogenic mass. CECT abdomen suggested a well distended stomach with mixed density lesion surrounded by oral contrast. Upper gastrointestinal endoscopy confirmed a large bezoar. The patient underwent laparotomy with gastrotomy and removal of bezoar. It had a tail like extension in the duodenum (Figure 1). Both patients were discharged after appropriate parental and psychiatric counselling.

What is the diagnosis_?
Expert Opinion :
Discussion:
Both patients had history of trichophagia and the second patient also had history of eating nails. The mass removed was trichobezoar. A trichobezoar is a conglomerate of trapped hair mainly originating in the stomach. {1,2} It is seen in young females. {1,2} The hair being enzyme-resistant and smooth, cannot be digested and accumulates between the gastric mucosal folds leading to formation of a hair ball together with entrapped food and mucus. {1,3} It is commonly associated with mental retardation and psychiatric disturbances like trichotillomania and trichophagia predominantly seen in emotionally disturbed or mentally retarded youngsters. {1,2,4} Predisposing risk factors include delayed gastric emptying, prior gastric surgery, peptic ulcer disease, chronic gastritis, Crohn’s disease, carcinoma of the gastrointestinal tract, dehydration and hypothyroidism. {1,5,6}
Presentation can be asymptomatic or with dyspepsia, anorexia, nausea, vomiting, colicky abdominal pain, bowel disturbances and weight loss. {4} A palpable mobile mass in epigastric region may be present. {2} Complications include gastrointestinal bleeding {caused by ulceration in the gastric mucosa due to pressure necrosis induced by the bezoar}, perforation, gastric emphysema, iron deficiency and megaloblastic anemia mandating early removal. {1,2,4} An unusual form of bezoar extending from stomach to the small intestine or beyond has been described as Rapunzel syndrome {2} which was seen in our second patient. USG, contrast study of the gastrointestinal tract, CECT scan and endoscopy help in diagnosis. {1}
Removal by endoscopic fragmentation is generally ineffective in large trichobezoars. {1} Specialized bezotomes and bezotriptors have been reported to fragment large and solid trichobezoars. Surgical excision can be done by laparotomy, mini-laparotomy or by laparoscopic techniques. {1,2,4} Successful pharmacotherapy for bezoars with cola, papain and cellulose and prokinetic agents like itopride, mosapride and metoclopramide has been reported. {1,2} Parental counseling, appropriate psychiatric treatment and long-term follow-up are mandatory to prevent recurrence.
References:
1. Iwamuro M, Okada H, Matsueda K, Inaba T, Kusumoto C, Imagawa A et al. Review of the diagnosis and management of gastrointestinal bezoars. World J Gastrointest Endosc. 2015 Apr 16` 7{4}: 336–345.
2. Czerwinska K, Bekiesinska-Figatowska M, Brzewski M, Gogolewski M, Wolski M. Trichobezoar, Rapunzel Syndrome, Tricho-Plaster Bezoar – A Report of Three Cases. Pol J Radiol. 2015` 80: 241–246.
3. Gorter RR, Kneepkens CM, Mattens EC, Aronson DC, Heij HA. Management of trichobezoar: case report and literature review. Pediatr Surg Int. 2010`26:457–463.
4. Kaushik NK, Sharma YP, Negi A, Jaswal A. Images - gastric trichobezoar. Indian J Radiol Imaging 1999`9:137-9.
5. Kumar GS, Amar V, Ramesh B, Abbey RK. Bizarre metal bezoar: a case report. Indian J Surg. 2013`75:356–358.
6. LaFountain J. Could your patient’s bowel obstruction be a bezoar__? Today’s Surg Nurse. 1999`21:34–37.

Correct Answers : yes  99%

Last Shown : May 2017
 
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