Medicine
®
CLINICAL CASE REPORT
A Case Report Large Trichobezoar Causing Rapunzel Syndrome Soon Chul Kim, MD, PhD, Seong Hun Kim, MD, PhD, and Sun Jun Kim, MD, PhD
Abstract: Rapunzel syndrome is very rare gastric foreign bodies that occur in children. It is a severe condition of a gastric trichobezoar with a long tail that passes into the small intestine. Here, we present the case of an 8-year-old girl with Rapunzel syndrome due to a very large (7 7 30 cm) gastric trichobezoar. The patient had trichotillomania and trichophagia for 1 year prior to presentation. Ideally, small bezoars are removed through a minimally invasive method, such as endoscopic fragmentation. However, large trichobezoars, including those in Rapunzel syndrome, can only be managed with open surgical extraction, despite the large scars that may result. We report a case of Rapunzel syndrome with a large bezoar that was surgically removed after it was endoscopically cauterized with argon plasma. Endoscopic precutting was used to effectively reduce the size of the bezoar. (Medicine 95(22):e3745) Abbreviation: CT = computed tomography.
INTRODUCTION
A
bezoar is an indigestible accumulation of foreign materials in the gastrointestinal tract. Because of the stomach’s large capacity, gastric bezoars do not become symptomatic until they are very large. The masses are classified according to their contents. Phytobezoars include fruit fibers or plants, lactobezoars are composed of milk, trichobezoars are concretions of hair, and pharmacobezoars are composed of medications.1 Trichobezoars are rare conditions that consist of hair bundles in the stomach or small bowel. In young women, trichobezoars are associated with psychiatric disorders such as trichotillomania (hair pulling) and trichophagia (hair swallowing). The
Editor: Vasile Valeriu Lupu. Received: January 22, 2016; revised: March 31, 2016; accepted: April 22, 2016. From the Department of Pediatrics (SCK, SJK) and Department of Internal Medicine (SHK), Chonbuk National University Medical School and Hospital, Research Institute of Clinical Medicine of Chonbuk National University - Biomedical research Institute of Chonbuk National University Hospital, Jeonju, Korea. Correspondence: Sun Jun Kim, Department of Pediatrics, Chonbuk National University Hospital, Jeonju, Korea (e-mail: [email protected]). Financial disclosure statement: no party having a direct interest in the results of the research or no organization with which we are associated has or will confer a benefit to us regarding this study. Funding: this study was performed by the approval of the Institutional Review Board of Chonbuk National University Research Council (CUH 2016-03-010) The authors have no conflicts of interest to disclose. Copyright # 2016 Wolters Kluwer Health, Inc. All rights reserved. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial License 4.0 (CCBY-NC), where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially. ISSN: 0025-7974 DOI: 10.1097/MD.0000000000003745
Medicine
Volume 95, Number 22, June 2016
Rapunzel syndrome is a rare type of trichobezoar that extends into the small intestine. Some types of bezoars, including small trichobezoars, can be removed after endoscopic fragmentation.1 However, very large trichobezoars, such as those in a patient with Rapunzel syndrome, are resistant to endoscopic fragmentation due to the dense hair mass. Therefore, these masses must be surgically removed, despite the large scars that result. Here, we report the case of Rapunzel syndrome with a large trichobezoar. After endoscopic shrinkage, this mass was removed surgically through a small incision.
Case Report An 8-year-old girl visited our pediatric department with epigastric discomfort for 2 weeks. The patient had no past medical history or drug history. The physical examination revealed a distended gastric area with normal bowel sounds and a soft abdominal wall. A solid mass was palpable in the epigastric region. However, there was no tenderness or rebound tenderness. The patient had intermittent small hard stools, which included some hairs. Her parents described that the patient had habits of hair pulling and chewing for 2 years. She did not show any signs of anxiety, depression, or mental retardation during admission. There were hairless regions on her scalp in the parietal areas, bilaterally. On plain abdominal x-ray (Figure 1) and computed tomography (Figure 2), the stomach was distended and filled with a large solid mass. The patient had stable vital signs. Her height and weight were both in the 25th percentile. Laboratory evaluations revealed a WBC count of 7150 / mL, hemoglobin of 13.2 g/dL, platelets of 443,000/mL, and CRP 0.04 mg/L. Other laboratory findings, including electrolytes, acid balance, liver function tests, and renal function tests, were within normal limits. An upper gastrointestinal endoscopy was performed. The inner cavity of the stomach was filled with a large trichobezoar composed of hair bundles. The bezoar had a long tail, which extended into the duodenal bulb. Endoscopic removal was not possible because of the mass’ large size. However, endoscopic removal was attempted. When this failed, a longitudinal anterior incision was made for gastrostomy. The procedure was performed by a pediatric surgeon under general anesthesia. An adult standard gastroscope (GIFH 260; Olympus; 9.8 mm diameter) was used. Electronic coagulation with a snare failed to cut the trichobezoar, and increased the risk of injuring the gastric mucosa. Instead, we were able to burn and cut the bezoar using argon plasma coagulation (effect 1 of forced mode and 60–80 W; ERBE VIO, 300D). However, smoke obscured the view, making this process lengthy (Figure 3). In order to secure a clear view, saline irrigation and frequent suctions were performed. The volume of the trichobezoar was reduced over the course of 1 hour, after which fragmentation was stopped due to the large amount of argon plasma used, and time expended. Finally, surgical gastrostomy was performed. The gastric trichobezoar was removed successfully within 1 hour. The maximum diameter of the mass was www.md-journal.com |
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Volume 95, Number 22, June 2016
similar result in electrogastrography was noted. Huge bezoar or gastrotomy might affect this result. However, the patient was treated with itopride, a prokinetic medication, for 2 months. The followed result of electrogastrography was improved. She was discharged without complications after 7 days. The patient was advised to recommend child psychiatric evaluation and treatment of the child to the family.
DISCUSSION
FIGURE 1. The abdominal simple x-ray. It shows the stomach filled with a huge soft tissue density of solid mass.
4 cm and its length was 30 cm. The thickest portion had been debulked from 7 to 4 cm with endoscopic precutting (Figure 4, arrow). Although we failed to completely remove the mass using endoscopic techniques, the argon plasma cauterization effectively reduced its size. Therefore, the incision scar (length in 4 cm) was smaller than originally expected. The patient underwent electrogastrography for the evaluation of gastric motility before the surgical procedure. There was bradygastria, with 90% of bradygastria and 10% of normal slow waves in both pre- and postprandial states. After the surgical procedure, the
Undigested foreign materials are aggregated by gastric fluid in the stomach. The word ‘‘bezoar’’ is derived from the Arabic word ‘‘badzehr,’’ which means ‘‘antidote.’’ Ancient people believed that bezoars from animals had medicinal properties.2 Currently, bezoars are known to be harmful and should be removed. Bezoars are named according to their components: phytobezoars, trichobezoars, lactobezoars, phamacobezoars, polybezoars, diospyrobezoars, or biliary bezoars. The most common type is the phytobezoar, which is composed of cellulose, hemicelluloses, and other proteins.1 Trichobezoars, or hairballs, are rare. The first reported trichobezoar was described in a 16-year-old boy by Baudamant in 1779.3 ‘‘Rapunzel’’ is a fairy tale in the collection by the Brothers Grimm. The heroine in the story had very long, blond hair. The first case of Rapunzel syndrome was published by Vaughan et al in 1968.4 In total, 49 case reports of Rapunzel syndrome were reviewed (Table 1). The age was from 4 to 19 years. Most girls had underwent surgical removal and had uneventful postoperative course. There were 3 fatal cases. Only 1 case was boy5 (Table 1). Gastric bezoars are formed by the synergic effect of multiple factors, the most important of which is the ingestion of indigestible material. Long-standing trichotillomania and trichophagia are relevant factors with regard to trichobezoars. There is a high incidence of depression or anxiety in young girls with trichobezoars.6 Retained hairs between the gastric folds are denatured and oxidized by gastric juice. After food and mucus accumulates, bacterial colonization occurs in the mass. Halitosis occasionally results. Proline-rich proteins from the parotid glands play a role in binding tannins and bezoar concretions.7 Another factor of bezoar formation is decreased
FIGURE 2. The abdominal computed tomography. The cavity of stomach is filled with a large heterogeneous mass (length in 7 cm). The gastric mass extends into the duodenal bulb over the pyloric canal (black arrow).
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Medicine
Volume 95, Number 22, June 2016
FIGURE 3. Upper gastrointestinal endoscopy. The view is obscured smoke from the burning hair.
gastric motility.8 The classical method of evaluating gastric motility is to measure the gastric emptying time during a barium swallow test and scintigraphy. Scintigraphy revealed that the patient did not have delayed gastric emptying time with solid food. However, electrogastrography showed preprandial bradygastria. There were normal gastric myoelectrical patterns in the postprandial state. Electrogastrography is a noninvasive method of recording gastric myoelectrical signals. In the human stomach, the normal slow wave frequency is 3 cycles per minute (3 cpm). According to the frequencies, the results are divided into normal, bradygastria, and tachygastria. It has been proposed that abnormalities in gastric myoelectrograms are associated with gastric dysmotility.9,10 Prokinetics can accelerate the gastric emptying, and therefore normalize the myoelectrogram.11 Therefore, this noninvasive tool is helpful in the diagnosis and management of gastric motility. The method of bezoar removal depends on its type, component, size, and location. In phytobezoars, Coca-Cola irrigation or administration is effective for fragmentation.
The Rapunzel Syndrome and Endoscopic Procedure
The carbon dioxide gas and the strong acidity of Coca-Cola are able to effectively dissolve food particles. The sodium bicarbonate in Coca-Cola has mucolytic effects.12 The CocaCola was introduced via nasogastric tube, direct injection, or spray through endoscopy. One group found that Coca-Cola and Coca-Cola zero had equally effective dissolvability, and significantly higher phytolytic activity than water, digestive enzymes, and papain.13 The papain enzyme from the papaya plant has been used to dissolve phytobezoars. However, it has not been used recently due to adverse side effects, including mucosal injury in the gastrointestinal tract.13 Endoscopic devices such as forceps, snares, and argon plasma coagulation are useful for bezoar fragmentation. After Coca-Cola injection, endoscopic fragmentation is the best therapeutic regimen for gastric phytobezoars. Trichobezoars, or hairballs, are not easily dissolved with pharmacotherapy or using endoscopic trials. Open surgery or laparotomy has been the treatment of choice for large trichobezoars. Unfortunately, surgery may have postoperative complications, such as perforation, pneumonia, bleeding, intussusception, wound infections, or unsightly scarring.14 Laparoscopic-assisted techniques have been suggested to reduce the complication rate.14 Recently, 1 group reported that they removed an 8 4 cm trichobezoar endoscopically. First, they fragmented the bezoar into 10 pieces using a polypectomy snare and argon plasma coagulation.15 There is no case report of Rapunzel syndrome treated with endoscopic removal completely (Table 1). Despite using the same tools and similar methods, we were not able to fragment the trichobezoar completely in our patient. Larger trichobezoars tend to have different densities and components compared to those of smaller masses. In the literature, almost all dense and large trichobezoars have failed to be removed endoscopically.15– 17 We checked the dissolubility of the removed bezoar in vitro. The bezoar was divided into 4 pieces. We analyzed the dissolubility with coca-cola, Sprite (Cider), soda water, and distilled water. Both Sprite and carbonated water are carbonated beverages. Distilled water was used as the control solution. The solutions were exchanged every 12 hours. Each bezoar fragment was incubated for 7 days. We measured changes in weight, density, and form. However, there were no interval changes in any item after the 7 days. Iwamuro et al investigated phytobezoar fragmentation using coca-cola and coca-cola zero in vitro.
FIGURE 4. A gross image of the removed trichobezoar. The arrows indicate the burning site. Endoscopic coagulation debulked the maximal diameter from 7 to 4 cm. Copyright
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TABLE 1. The Case Reports of Rapunzel Syndrome in the Literature First Author
Year
Age/ Gender
Treatment
Outcome
Vaughan ED Deslypere JP Wolfson PJ Hassan AA Balik E Duncan ND Pul N Uroz Tristan J Seker B Senapati MK Dalshaug GB Singla SL Kaspar A Hirugade ST Klipfel AA Gockel I Memon SA Frey AS Quraishi AH Ventura DE AI-Wadan AH Mathai J Naik S Henry PY Western C Rabie ME Emre AU Koc O Kibria R Mateju E Gonuguntla V Gorter RR Raikar S Chogle A Patel R Rajakannu M Bashir EA Dorn HF Cook SL Singh S Tiwary SK Neychev V Middleton E Petrovic G Phavichitr N Veloso N Belsky J Islek A Meier CM
1968 1982 1987 1989 1993 1994 1996 1996 1996 1997 1999 1999 1999 2001 2003 2003 2003 2005 2005 2005 2006 2007 2007 2007 2008 2008 2008 2009 2009 2009 2009 2010 2010 2010 2010 2010 2010 2010 2011 2011 2011 2012 2012 2012 2012 2013 2014 2014 2015
13/F 14/F 5/F 5/F 15/F 5/F 12/F 8/F 6/F 8/F 7/F 9/F 12/F 6/F 14/F 4/F 12/F 7/F 5/F 5/F 18/F 5/F 16/F 10/F 14/F 11/F 18/F 14/F 6/F 4/F 5/F 12/F 12/F 3/F 13/F 15/F 8/F 17/F 9/F 5/F 15/F 16/M 3/F 19/F 10/F 7/F 6/F 5/F 7/F
Enterotomy Autopsy Gastrotomy Gastrotomy Gastrotomy Enterotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Enterotomy Enterotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Autopsy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Autopsy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy
Alive Died Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Died Alive Alive Alive Alive Alive Alive Alive Alive Alive Died Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive
After 12 hours of incubation, the group found that both cocacola and coca-cola zero had excellent dissoluble effects. The group questioned whether other carbonated beverages have the same lytic effects against phytobezoars.13 We confirmed that carbonated beverages such as coca-cola are unable to fragment trichobezoar.
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CONCLUSION Trichobezoars tend to occur in patients at a mean age of approximately 8 years. Complete endoscopic removal through fragmentation is the most ideal management for trichobezoars, especially to avoid large scars after open surgery. Inevitably, large and dense gastric trichobezoars, including Rapunzel syndrome, are removed surgically. We suggest that the endoscopic debulking method using argon plasma burning be performed preoperatively to reduce the surgical complications of such Rapunzel syndromes. REFERENCES 1. Iwamuro M, Okada H, Matsueda K, et al. Review of the diagnosis and management of gastrointestinal bezoars. World J Gastrointest Endosc. 2015;7:336–345. 2. Williams RS. The fascinating history of bezoars. Med J Aust. 1986;145:613–614. 3. Grant JE, Odlaug BL. Clinical characteristics of trichotillomania with trichophagia. Compr Psychiatry. 2008;49:579–584. 4. Vaughan ED Jr, Sawyers JL, Scott HW Jr. The Rapunzel syndrome. An unusual complication of intestinal bezoar. Surgery. 1968;63:339–343. 5. Kohler JE1, Millie M, Neuger E. Trichobezoar causing pancreatitis: first reported case of Rapunzel syndrome in a boy in North America. J Pediatr Surg. 2012;47:e17–e19. 6. Duke DC, Keeley ML, Geffken GR, et al. Trichotillomania: a current review. Clin Psychol Rev. 2010;30:181–193. 7. Matsumoto K, Kadowaki A, Ozaki N, et al. Bile acid-binding ability of kaki-tannin from young fruits of persimmon (Diospyros kaki) in vitro and in vivo. Phytother Res. 2011;25:624–628. 8. Dikicier E, Altintoprak F, Ozkan OV, et al. Intestinal obstruction due to phytobezoars: an update. World J Clin Cases. 2015;3:721–726. 9. Brzana RJ, Koch KL, Bingaman S. Gastric myoelectrical activity in patients with gastric outlet obstruction and idiopathic gastroparesis. Am J Gastroenterol. 1998;93:1803–1809. 10. Christensen CJ, Johnson WD, Abell TL. Patients with cyclic vomiting pattern and diabetic gastropathy have more migraines, abnormal electrogastrograms, and gastric emptying. Scand J Gastroenterol. 2008;43:1076–1081. 11. Rothstein RD, Alavi A, Reynolds JC. Electrogastrography in patients with gastroparesis and effect of long-term cisapride. Electrogastrography in patients with gastroparesis and effect of long-term cisapride. Dig Dis Sci. 1993;38:1518–1524. 12. Ladas SD, Kamberoglou D, Karamanolis G, et al. Systematic review: Coca-Cola can effectively dissolve gastric phytobezoars as a first-line treatment. Aliment Pharmacol Ther. 2013;37:169–173. 13. Iwamuro M, Kawai Y, Shiraha H, et al. In vitro analysis of gastric phytobezoar dissolubility by Coca-Cola, Coca-Cola zero, cellulase, and papain. J Clin Gastroenterol. 2014;48:190–191. 14. Tudor EC, Clark MC. Laparoscopic-assisted removal of gastric trichobezoar; a novel technique to reduce operative complications and time. J Pediatr Surg. 2013;48:e13–e15. 15. Benatta MA. Endoscopic retrieval of gastric trichobezoar after fragmentation with electrocautery using polypectomy snare and argon plasma coagulation in a pediatric patient. Gastroenterol Rep. 2015;13:1–3. 16. Czerwin´ska K, Bekiesin´ska-Figatowska M, Brzewski M, et al. Trichobezoar, rapunzel syndrome, tricho-plaster bezoar—a report of three cases. Pol J Radiol. 2015;80:241–246. 17. Fallon SC, Slater BJ, Larimer EL, et al. The surgical management of Rapunzel syndrome: a case series and literature review. J Pediatr Surg. 2013;48:830–834.
Copyright
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®
CLINICAL CASE REPORT
A Case Report Large Trichobezoar Causing Rapunzel Syndrome Soon Chul Kim, MD, PhD, Seong Hun Kim, MD, PhD, and Sun Jun Kim, MD, PhD
Abstract: Rapunzel syndrome is very rare gastric foreign bodies that occur in children. It is a severe condition of a gastric trichobezoar with a long tail that passes into the small intestine. Here, we present the case of an 8-year-old girl with Rapunzel syndrome due to a very large (7 7 30 cm) gastric trichobezoar. The patient had trichotillomania and trichophagia for 1 year prior to presentation. Ideally, small bezoars are removed through a minimally invasive method, such as endoscopic fragmentation. However, large trichobezoars, including those in Rapunzel syndrome, can only be managed with open surgical extraction, despite the large scars that may result. We report a case of Rapunzel syndrome with a large bezoar that was surgically removed after it was endoscopically cauterized with argon plasma. Endoscopic precutting was used to effectively reduce the size of the bezoar. (Medicine 95(22):e3745) Abbreviation: CT = computed tomography.
INTRODUCTION
A
bezoar is an indigestible accumulation of foreign materials in the gastrointestinal tract. Because of the stomach’s large capacity, gastric bezoars do not become symptomatic until they are very large. The masses are classified according to their contents. Phytobezoars include fruit fibers or plants, lactobezoars are composed of milk, trichobezoars are concretions of hair, and pharmacobezoars are composed of medications.1 Trichobezoars are rare conditions that consist of hair bundles in the stomach or small bowel. In young women, trichobezoars are associated with psychiatric disorders such as trichotillomania (hair pulling) and trichophagia (hair swallowing). The
Editor: Vasile Valeriu Lupu. Received: January 22, 2016; revised: March 31, 2016; accepted: April 22, 2016. From the Department of Pediatrics (SCK, SJK) and Department of Internal Medicine (SHK), Chonbuk National University Medical School and Hospital, Research Institute of Clinical Medicine of Chonbuk National University - Biomedical research Institute of Chonbuk National University Hospital, Jeonju, Korea. Correspondence: Sun Jun Kim, Department of Pediatrics, Chonbuk National University Hospital, Jeonju, Korea (e-mail: [email protected]). Financial disclosure statement: no party having a direct interest in the results of the research or no organization with which we are associated has or will confer a benefit to us regarding this study. Funding: this study was performed by the approval of the Institutional Review Board of Chonbuk National University Research Council (CUH 2016-03-010) The authors have no conflicts of interest to disclose. Copyright # 2016 Wolters Kluwer Health, Inc. All rights reserved. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial License 4.0 (CCBY-NC), where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially. ISSN: 0025-7974 DOI: 10.1097/MD.0000000000003745
Medicine
Volume 95, Number 22, June 2016
Rapunzel syndrome is a rare type of trichobezoar that extends into the small intestine. Some types of bezoars, including small trichobezoars, can be removed after endoscopic fragmentation.1 However, very large trichobezoars, such as those in a patient with Rapunzel syndrome, are resistant to endoscopic fragmentation due to the dense hair mass. Therefore, these masses must be surgically removed, despite the large scars that result. Here, we report the case of Rapunzel syndrome with a large trichobezoar. After endoscopic shrinkage, this mass was removed surgically through a small incision.
Case Report An 8-year-old girl visited our pediatric department with epigastric discomfort for 2 weeks. The patient had no past medical history or drug history. The physical examination revealed a distended gastric area with normal bowel sounds and a soft abdominal wall. A solid mass was palpable in the epigastric region. However, there was no tenderness or rebound tenderness. The patient had intermittent small hard stools, which included some hairs. Her parents described that the patient had habits of hair pulling and chewing for 2 years. She did not show any signs of anxiety, depression, or mental retardation during admission. There were hairless regions on her scalp in the parietal areas, bilaterally. On plain abdominal x-ray (Figure 1) and computed tomography (Figure 2), the stomach was distended and filled with a large solid mass. The patient had stable vital signs. Her height and weight were both in the 25th percentile. Laboratory evaluations revealed a WBC count of 7150 / mL, hemoglobin of 13.2 g/dL, platelets of 443,000/mL, and CRP 0.04 mg/L. Other laboratory findings, including electrolytes, acid balance, liver function tests, and renal function tests, were within normal limits. An upper gastrointestinal endoscopy was performed. The inner cavity of the stomach was filled with a large trichobezoar composed of hair bundles. The bezoar had a long tail, which extended into the duodenal bulb. Endoscopic removal was not possible because of the mass’ large size. However, endoscopic removal was attempted. When this failed, a longitudinal anterior incision was made for gastrostomy. The procedure was performed by a pediatric surgeon under general anesthesia. An adult standard gastroscope (GIFH 260; Olympus; 9.8 mm diameter) was used. Electronic coagulation with a snare failed to cut the trichobezoar, and increased the risk of injuring the gastric mucosa. Instead, we were able to burn and cut the bezoar using argon plasma coagulation (effect 1 of forced mode and 60–80 W; ERBE VIO, 300D). However, smoke obscured the view, making this process lengthy (Figure 3). In order to secure a clear view, saline irrigation and frequent suctions were performed. The volume of the trichobezoar was reduced over the course of 1 hour, after which fragmentation was stopped due to the large amount of argon plasma used, and time expended. Finally, surgical gastrostomy was performed. The gastric trichobezoar was removed successfully within 1 hour. The maximum diameter of the mass was www.md-journal.com |
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Volume 95, Number 22, June 2016
similar result in electrogastrography was noted. Huge bezoar or gastrotomy might affect this result. However, the patient was treated with itopride, a prokinetic medication, for 2 months. The followed result of electrogastrography was improved. She was discharged without complications after 7 days. The patient was advised to recommend child psychiatric evaluation and treatment of the child to the family.
DISCUSSION
FIGURE 1. The abdominal simple x-ray. It shows the stomach filled with a huge soft tissue density of solid mass.
4 cm and its length was 30 cm. The thickest portion had been debulked from 7 to 4 cm with endoscopic precutting (Figure 4, arrow). Although we failed to completely remove the mass using endoscopic techniques, the argon plasma cauterization effectively reduced its size. Therefore, the incision scar (length in 4 cm) was smaller than originally expected. The patient underwent electrogastrography for the evaluation of gastric motility before the surgical procedure. There was bradygastria, with 90% of bradygastria and 10% of normal slow waves in both pre- and postprandial states. After the surgical procedure, the
Undigested foreign materials are aggregated by gastric fluid in the stomach. The word ‘‘bezoar’’ is derived from the Arabic word ‘‘badzehr,’’ which means ‘‘antidote.’’ Ancient people believed that bezoars from animals had medicinal properties.2 Currently, bezoars are known to be harmful and should be removed. Bezoars are named according to their components: phytobezoars, trichobezoars, lactobezoars, phamacobezoars, polybezoars, diospyrobezoars, or biliary bezoars. The most common type is the phytobezoar, which is composed of cellulose, hemicelluloses, and other proteins.1 Trichobezoars, or hairballs, are rare. The first reported trichobezoar was described in a 16-year-old boy by Baudamant in 1779.3 ‘‘Rapunzel’’ is a fairy tale in the collection by the Brothers Grimm. The heroine in the story had very long, blond hair. The first case of Rapunzel syndrome was published by Vaughan et al in 1968.4 In total, 49 case reports of Rapunzel syndrome were reviewed (Table 1). The age was from 4 to 19 years. Most girls had underwent surgical removal and had uneventful postoperative course. There were 3 fatal cases. Only 1 case was boy5 (Table 1). Gastric bezoars are formed by the synergic effect of multiple factors, the most important of which is the ingestion of indigestible material. Long-standing trichotillomania and trichophagia are relevant factors with regard to trichobezoars. There is a high incidence of depression or anxiety in young girls with trichobezoars.6 Retained hairs between the gastric folds are denatured and oxidized by gastric juice. After food and mucus accumulates, bacterial colonization occurs in the mass. Halitosis occasionally results. Proline-rich proteins from the parotid glands play a role in binding tannins and bezoar concretions.7 Another factor of bezoar formation is decreased
FIGURE 2. The abdominal computed tomography. The cavity of stomach is filled with a large heterogeneous mass (length in 7 cm). The gastric mass extends into the duodenal bulb over the pyloric canal (black arrow).
2
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#
2016 Wolters Kluwer Health, Inc. All rights reserved.
Medicine
Volume 95, Number 22, June 2016
FIGURE 3. Upper gastrointestinal endoscopy. The view is obscured smoke from the burning hair.
gastric motility.8 The classical method of evaluating gastric motility is to measure the gastric emptying time during a barium swallow test and scintigraphy. Scintigraphy revealed that the patient did not have delayed gastric emptying time with solid food. However, electrogastrography showed preprandial bradygastria. There were normal gastric myoelectrical patterns in the postprandial state. Electrogastrography is a noninvasive method of recording gastric myoelectrical signals. In the human stomach, the normal slow wave frequency is 3 cycles per minute (3 cpm). According to the frequencies, the results are divided into normal, bradygastria, and tachygastria. It has been proposed that abnormalities in gastric myoelectrograms are associated with gastric dysmotility.9,10 Prokinetics can accelerate the gastric emptying, and therefore normalize the myoelectrogram.11 Therefore, this noninvasive tool is helpful in the diagnosis and management of gastric motility. The method of bezoar removal depends on its type, component, size, and location. In phytobezoars, Coca-Cola irrigation or administration is effective for fragmentation.
The Rapunzel Syndrome and Endoscopic Procedure
The carbon dioxide gas and the strong acidity of Coca-Cola are able to effectively dissolve food particles. The sodium bicarbonate in Coca-Cola has mucolytic effects.12 The CocaCola was introduced via nasogastric tube, direct injection, or spray through endoscopy. One group found that Coca-Cola and Coca-Cola zero had equally effective dissolvability, and significantly higher phytolytic activity than water, digestive enzymes, and papain.13 The papain enzyme from the papaya plant has been used to dissolve phytobezoars. However, it has not been used recently due to adverse side effects, including mucosal injury in the gastrointestinal tract.13 Endoscopic devices such as forceps, snares, and argon plasma coagulation are useful for bezoar fragmentation. After Coca-Cola injection, endoscopic fragmentation is the best therapeutic regimen for gastric phytobezoars. Trichobezoars, or hairballs, are not easily dissolved with pharmacotherapy or using endoscopic trials. Open surgery or laparotomy has been the treatment of choice for large trichobezoars. Unfortunately, surgery may have postoperative complications, such as perforation, pneumonia, bleeding, intussusception, wound infections, or unsightly scarring.14 Laparoscopic-assisted techniques have been suggested to reduce the complication rate.14 Recently, 1 group reported that they removed an 8 4 cm trichobezoar endoscopically. First, they fragmented the bezoar into 10 pieces using a polypectomy snare and argon plasma coagulation.15 There is no case report of Rapunzel syndrome treated with endoscopic removal completely (Table 1). Despite using the same tools and similar methods, we were not able to fragment the trichobezoar completely in our patient. Larger trichobezoars tend to have different densities and components compared to those of smaller masses. In the literature, almost all dense and large trichobezoars have failed to be removed endoscopically.15– 17 We checked the dissolubility of the removed bezoar in vitro. The bezoar was divided into 4 pieces. We analyzed the dissolubility with coca-cola, Sprite (Cider), soda water, and distilled water. Both Sprite and carbonated water are carbonated beverages. Distilled water was used as the control solution. The solutions were exchanged every 12 hours. Each bezoar fragment was incubated for 7 days. We measured changes in weight, density, and form. However, there were no interval changes in any item after the 7 days. Iwamuro et al investigated phytobezoar fragmentation using coca-cola and coca-cola zero in vitro.
FIGURE 4. A gross image of the removed trichobezoar. The arrows indicate the burning site. Endoscopic coagulation debulked the maximal diameter from 7 to 4 cm. Copyright
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TABLE 1. The Case Reports of Rapunzel Syndrome in the Literature First Author
Year
Age/ Gender
Treatment
Outcome
Vaughan ED Deslypere JP Wolfson PJ Hassan AA Balik E Duncan ND Pul N Uroz Tristan J Seker B Senapati MK Dalshaug GB Singla SL Kaspar A Hirugade ST Klipfel AA Gockel I Memon SA Frey AS Quraishi AH Ventura DE AI-Wadan AH Mathai J Naik S Henry PY Western C Rabie ME Emre AU Koc O Kibria R Mateju E Gonuguntla V Gorter RR Raikar S Chogle A Patel R Rajakannu M Bashir EA Dorn HF Cook SL Singh S Tiwary SK Neychev V Middleton E Petrovic G Phavichitr N Veloso N Belsky J Islek A Meier CM
1968 1982 1987 1989 1993 1994 1996 1996 1996 1997 1999 1999 1999 2001 2003 2003 2003 2005 2005 2005 2006 2007 2007 2007 2008 2008 2008 2009 2009 2009 2009 2010 2010 2010 2010 2010 2010 2010 2011 2011 2011 2012 2012 2012 2012 2013 2014 2014 2015
13/F 14/F 5/F 5/F 15/F 5/F 12/F 8/F 6/F 8/F 7/F 9/F 12/F 6/F 14/F 4/F 12/F 7/F 5/F 5/F 18/F 5/F 16/F 10/F 14/F 11/F 18/F 14/F 6/F 4/F 5/F 12/F 12/F 3/F 13/F 15/F 8/F 17/F 9/F 5/F 15/F 16/M 3/F 19/F 10/F 7/F 6/F 5/F 7/F
Enterotomy Autopsy Gastrotomy Gastrotomy Gastrotomy Enterotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Enterotomy Enterotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Autopsy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Autopsy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy Gastrotomy
Alive Died Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Died Alive Alive Alive Alive Alive Alive Alive Alive Alive Died Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive
After 12 hours of incubation, the group found that both cocacola and coca-cola zero had excellent dissoluble effects. The group questioned whether other carbonated beverages have the same lytic effects against phytobezoars.13 We confirmed that carbonated beverages such as coca-cola are unable to fragment trichobezoar.
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Volume 95, Number 22, June 2016
CONCLUSION Trichobezoars tend to occur in patients at a mean age of approximately 8 years. Complete endoscopic removal through fragmentation is the most ideal management for trichobezoars, especially to avoid large scars after open surgery. Inevitably, large and dense gastric trichobezoars, including Rapunzel syndrome, are removed surgically. We suggest that the endoscopic debulking method using argon plasma burning be performed preoperatively to reduce the surgical complications of such Rapunzel syndromes. REFERENCES 1. Iwamuro M, Okada H, Matsueda K, et al. Review of the diagnosis and management of gastrointestinal bezoars. World J Gastrointest Endosc. 2015;7:336–345. 2. Williams RS. The fascinating history of bezoars. Med J Aust. 1986;145:613–614. 3. Grant JE, Odlaug BL. Clinical characteristics of trichotillomania with trichophagia. Compr Psychiatry. 2008;49:579–584. 4. Vaughan ED Jr, Sawyers JL, Scott HW Jr. The Rapunzel syndrome. An unusual complication of intestinal bezoar. Surgery. 1968;63:339–343. 5. Kohler JE1, Millie M, Neuger E. Trichobezoar causing pancreatitis: first reported case of Rapunzel syndrome in a boy in North America. J Pediatr Surg. 2012;47:e17–e19. 6. Duke DC, Keeley ML, Geffken GR, et al. Trichotillomania: a current review. Clin Psychol Rev. 2010;30:181–193. 7. Matsumoto K, Kadowaki A, Ozaki N, et al. Bile acid-binding ability of kaki-tannin from young fruits of persimmon (Diospyros kaki) in vitro and in vivo. Phytother Res. 2011;25:624–628. 8. Dikicier E, Altintoprak F, Ozkan OV, et al. Intestinal obstruction due to phytobezoars: an update. World J Clin Cases. 2015;3:721–726. 9. Brzana RJ, Koch KL, Bingaman S. Gastric myoelectrical activity in patients with gastric outlet obstruction and idiopathic gastroparesis. Am J Gastroenterol. 1998;93:1803–1809. 10. Christensen CJ, Johnson WD, Abell TL. Patients with cyclic vomiting pattern and diabetic gastropathy have more migraines, abnormal electrogastrograms, and gastric emptying. Scand J Gastroenterol. 2008;43:1076–1081. 11. Rothstein RD, Alavi A, Reynolds JC. Electrogastrography in patients with gastroparesis and effect of long-term cisapride. Electrogastrography in patients with gastroparesis and effect of long-term cisapride. Dig Dis Sci. 1993;38:1518–1524. 12. Ladas SD, Kamberoglou D, Karamanolis G, et al. Systematic review: Coca-Cola can effectively dissolve gastric phytobezoars as a first-line treatment. Aliment Pharmacol Ther. 2013;37:169–173. 13. Iwamuro M, Kawai Y, Shiraha H, et al. In vitro analysis of gastric phytobezoar dissolubility by Coca-Cola, Coca-Cola zero, cellulase, and papain. J Clin Gastroenterol. 2014;48:190–191. 14. Tudor EC, Clark MC. Laparoscopic-assisted removal of gastric trichobezoar; a novel technique to reduce operative complications and time. J Pediatr Surg. 2013;48:e13–e15. 15. Benatta MA. Endoscopic retrieval of gastric trichobezoar after fragmentation with electrocautery using polypectomy snare and argon plasma coagulation in a pediatric patient. Gastroenterol Rep. 2015;13:1–3. 16. Czerwin´ska K, Bekiesin´ska-Figatowska M, Brzewski M, et al. Trichobezoar, rapunzel syndrome, tricho-plaster bezoar—a report of three cases. Pol J Radiol. 2015;80:241–246. 17. Fallon SC, Slater BJ, Larimer EL, et al. The surgical management of Rapunzel syndrome: a case series and literature review. J Pediatr Surg. 2013;48:830–834.
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