Rapunzel Syndrome Causing Gastric Outlet Obstruction in A 12-Year-Old Girl: Report of A Case and Review of the Literature

Bilge Gördü

doi:10.4274/atfm.galenos.2023.03064

Case Report

12 Yaşındaki Bir Kız Çocukta Mide Çıkım Tıkanıklığına Neden Olan Rapunzel Sendromu: Olgu Sunumu ve Literatürün Gözden Geçirilmesi

Year 2024, Volume: 77 Issue: 1, 113 - 118, 28.03.2024

Bilge Gördü

Abstract

Rapunzel sendromu (RS) midede biriken saçların ince barsakta obstrüksiyona neden olabilecek şekilde uzandığı nadir bir durumdur. Mide çıkımında ve barsakta tıkanıklığına neden olabilir. En sık görülen klinik belirtiler karın ağrısı, ele gelen mobil kitle ve kusmadır. Trikotilomani, trikofaji, dikkat eksikliği ve hiperaktivite bozukluğu öyküsü olan 12 yaşında kız hasta, mide çıkım tıkanıklığı nedeniyle başvurdu. Batın ultrasonunda mideyi tamamen dolduran ve duodenumun üçüncü kısmına uzanan bezoar tespit edildi. Trikobezoarın gastrotomi yoluyla çıkarılması tercih edildi. Burada, bu nadir
görülen tablonun klinik özelliklerini ve tedavi seçeneklerini tartışmak amacıyla 12 yaşında RS’li bir kız çocuğunu sunuyoruz.

Anahtar Kelimeler: Bezoar, trikobezoar, Rapunzel Sendromu, çocuk

Keywords

Bezoar, trikobezoar, Rapunzel Sendromu, çocuk

Ethical Statement

Informed consent was obtained from the patient’s family.

Supporting Institution

Project Number

Thanks

References

1. Gorter RR, Kneepkens CM, Mattens EC, et al. Management of trichobezoar: case report and literature review. Pediatr Surg Int. 2010;26:457-463.
2. Vaughan ED Jr, Sawyers JL, Scott HW Jr. The Rapunzel syndrome. An unusual complication of intestinal bezoar. Surgery. 1968;63:339-343.
3. Gonuguntla V, Joshi DD. Rapunzel syndrome: a comprehensive review of an unusual case of trichobezoar. Clin Med Res. 2009;7:99-102.
4. Naik S, Gupta V, Naik S, et al. Rapunzel syndrome reviewed and redefined. Dig Surg. 2007;24:157-161.
5. Hirugade ST, Talpallikar MC, Deshpande AV, et al. Rapunzel syndrome with a long tail. Indian J Pediatr. 2001;68:895-896.
6. Frey AS, McKee M, King RA, et al. Hair apparent: Rapunzel syndrome. Am J Psychiatry. 2005;162:242-248.
7. Hoover K, Piotrowski J, St Pierre K, et al. Simultaneous gastric and small intestinal trichobezoars--a hairy problem. J Pediatr Surg. 2006;41:1495- 1497.
8. Miltenberger RG. Habit Reversal Treatment Manual for Trichotillomania. In: Woods, D.W., Miltenberger, R.G. (eds) Tic Disorders, Trichotillomania, and Other Repetitive Behavior Disorders. Springer, Boston, MA. 2001;170-195.
9. Singh S, Wakhlu A, Pandey A, et al. Complicated Rapunzel syndrome mimicking intussusception. BMJ Case Rep. 2011;2011:bcr0820103277.
10. Malpani A, Ramani SK, Wolverson MK. Role of sonography in trichobezoars. J Ultrasound Med. 1988;7:661-663.
11. Ratcliffe JF. The ultrasonographic appearance of a trichobezoar. Br J Radiol. 1982;55:166-167.
12. Narra R, Guntamukkala A, Rao CB, et al. Rapunzel syndrome: a case of trichobezoar with small bowel complications. Surg J (N Y). 2022;8:e293-e296.
13. Chun SW, Lee K, Park YJ, et al. [Rapunzel syndrome removed with enteroscopy in a child]. Korean J Gastroenterol. 2019;74:42-45.
14. Kim SK, Beck AR. Multiple small bowel perforations: an unusual complication of trichobezoar. Mt Sinai J Med. 1972;39:293-299.
15. Nirasawa Y, Mori T, Ito Y, et al. Laparoscopic removal of a large gastric trichobezoar. J Pediatr Surg. 1998;33:663-665.
16. Obinwa O, Cooper D, Khan F, et al. Rapunzel syndrome is not just a mere surgical problem: A case report and review of current management. World J Clin Cases. 2017;5:50-55.
17. Flaherty DC, Aguilar F, Pradhan B, et al. Rapunzel syndrome due to ingested hair extensions: Surgical and psychiatric considerations. Int J Surg Case Rep. 2015;17:155-157
18. Kumar M, Maletha M, Bhuddi S, et al. Rapunzel syndrome in a 3-yearold boy: A menace too early to present. J Indian Assoc Pediatr Surg. 2020;25:112-114.
19. Kim JS, Nam CW. A case of rapunzel syndrome. Pediatr Gastroenterol Hepatol Nutr. 2013;16:127-130.
20. Khanna K, Tandon S, Yadav DK, et al. Rapunzel syndrome: a tail too long to tell! BMJ Case Rep. 2018;2018:bcr2018224756
21. Lalith S, Gopalakrishnan KL, Ilangovan G, et al. Rapunzel Syndrome. J Clin Diagn Res. 2017;11:TD01-TD02 .
22. Mazzei A, Centonze A, Aloi IP, et al. Rapunzel syndrome: endoscopy, laparotomy, or laparoscopy? J Indian Assoc Pediatr Surg. 2021;26:66-67.
23. Kyin C, Patel P, Casas-Melley A, et al. Acute case of trichobezoar diagnosed from computed tomography and 3D images: rapunzel syndrome reexamined. Cureus. 2023;15:e35597.
24. Czerwińska K, Bekiesińska-Figatowska M, Brzewski M, et al. Trichobezoar, rapunzel syndrome, tricho-plaster bezoar - a report of three cases. Pol J Radiol. 2015;80:241-246.
25. Raikar S, Wali P, Khan S. Recurrence of Rapunzel syndrome. J Pediatr. 2010;157:343-343.e1.
26. Winsett F, Ting J, Harting MT. Rapunzel syndrome: a rare case requiring surgical management of acute pancreatitis. Pancreas. 2019;48:e38-e39.
27. Rogers HK, Phadke O, Glaser AM. The curious case of Rapunzel syndrome: a rare non-trichobezoar. Am J Gastroenterol. 2018;113:783-784.
28. Al Wadan AH, Al Kaff H, Al Senabani J, et al. ‘Rapunzel syndrome’ trichobezoar in a 7-year-old girl: a case report. Cases J. 2008;1:205.
29. Chogle A, Bonilla S, Browne M, et al. Rapunzel syndrome: a rare cause of biliary obstruction. J Pediatr Gastroenterol Nutr. 2010;51:522-523.
30. Nwankwo E, Daniele E, Woller E, et al. Trichobezoar presenting as a gastric outlet obstruction: A case report. Int J Surg Case Rep. 2017;34:123-125.

Rapunzel Syndrome Causing Gastric Outlet Obstruction in A 12-Year-Old Girl: Report of A Case and Review of the Literature

Year 2024, Volume: 77 Issue: 1, 113 - 118, 28.03.2024

Bilge Gördü

Abstract

Rapunzel syndrome (RS) is a rare condition presenting with hair accumulation in the stomach that extends to small intestine. It may cause gastric outlet and intestinal obstruction. The most common clinical signs are abdominal pain, palpable mobile mass and vomiting. A 12-year-old girl with a history of trichotillomania, trichophagia, attention deficit and hyperactivity disorder presented with gastric outlet obstruction. A bezoar completely filling the stomach and reaching to third part of the duodenum was detected in abdominal ultrasound. Removal of the trichobezoar via gastrotomy was the treatment of choice. Herein we report a 12-year-old girl with RS to discuss the clinical features and treatment options in this rare presentation.

Key Words: Bezoar, trichobezoar, Rapunzel Syndrome, child

Keywords

Bezoar, trichobezoar, Rapunzel Syndrome, child

Ethical Statement

Informed consent was obtained from the patient’s family.

Supporting Institution

Project Number

Thanks

References

1. Gorter RR, Kneepkens CM, Mattens EC, et al. Management of trichobezoar: case report and literature review. Pediatr Surg Int. 2010;26:457-463.
2. Vaughan ED Jr, Sawyers JL, Scott HW Jr. The Rapunzel syndrome. An unusual complication of intestinal bezoar. Surgery. 1968;63:339-343.
3. Gonuguntla V, Joshi DD. Rapunzel syndrome: a comprehensive review of an unusual case of trichobezoar. Clin Med Res. 2009;7:99-102.
4. Naik S, Gupta V, Naik S, et al. Rapunzel syndrome reviewed and redefined. Dig Surg. 2007;24:157-161.
5. Hirugade ST, Talpallikar MC, Deshpande AV, et al. Rapunzel syndrome with a long tail. Indian J Pediatr. 2001;68:895-896.
6. Frey AS, McKee M, King RA, et al. Hair apparent: Rapunzel syndrome. Am J Psychiatry. 2005;162:242-248.
7. Hoover K, Piotrowski J, St Pierre K, et al. Simultaneous gastric and small intestinal trichobezoars--a hairy problem. J Pediatr Surg. 2006;41:1495- 1497.
8. Miltenberger RG. Habit Reversal Treatment Manual for Trichotillomania. In: Woods, D.W., Miltenberger, R.G. (eds) Tic Disorders, Trichotillomania, and Other Repetitive Behavior Disorders. Springer, Boston, MA. 2001;170-195.
9. Singh S, Wakhlu A, Pandey A, et al. Complicated Rapunzel syndrome mimicking intussusception. BMJ Case Rep. 2011;2011:bcr0820103277.
10. Malpani A, Ramani SK, Wolverson MK. Role of sonography in trichobezoars. J Ultrasound Med. 1988;7:661-663.
11. Ratcliffe JF. The ultrasonographic appearance of a trichobezoar. Br J Radiol. 1982;55:166-167.
12. Narra R, Guntamukkala A, Rao CB, et al. Rapunzel syndrome: a case of trichobezoar with small bowel complications. Surg J (N Y). 2022;8:e293-e296.
13. Chun SW, Lee K, Park YJ, et al. [Rapunzel syndrome removed with enteroscopy in a child]. Korean J Gastroenterol. 2019;74:42-45.
14. Kim SK, Beck AR. Multiple small bowel perforations: an unusual complication of trichobezoar. Mt Sinai J Med. 1972;39:293-299.
15. Nirasawa Y, Mori T, Ito Y, et al. Laparoscopic removal of a large gastric trichobezoar. J Pediatr Surg. 1998;33:663-665.
16. Obinwa O, Cooper D, Khan F, et al. Rapunzel syndrome is not just a mere surgical problem: A case report and review of current management. World J Clin Cases. 2017;5:50-55.
17. Flaherty DC, Aguilar F, Pradhan B, et al. Rapunzel syndrome due to ingested hair extensions: Surgical and psychiatric considerations. Int J Surg Case Rep. 2015;17:155-157
18. Kumar M, Maletha M, Bhuddi S, et al. Rapunzel syndrome in a 3-yearold boy: A menace too early to present. J Indian Assoc Pediatr Surg. 2020;25:112-114.
19. Kim JS, Nam CW. A case of rapunzel syndrome. Pediatr Gastroenterol Hepatol Nutr. 2013;16:127-130.
20. Khanna K, Tandon S, Yadav DK, et al. Rapunzel syndrome: a tail too long to tell! BMJ Case Rep. 2018;2018:bcr2018224756
21. Lalith S, Gopalakrishnan KL, Ilangovan G, et al. Rapunzel Syndrome. J Clin Diagn Res. 2017;11:TD01-TD02 .
22. Mazzei A, Centonze A, Aloi IP, et al. Rapunzel syndrome: endoscopy, laparotomy, or laparoscopy? J Indian Assoc Pediatr Surg. 2021;26:66-67.
23. Kyin C, Patel P, Casas-Melley A, et al. Acute case of trichobezoar diagnosed from computed tomography and 3D images: rapunzel syndrome reexamined. Cureus. 2023;15:e35597.
24. Czerwińska K, Bekiesińska-Figatowska M, Brzewski M, et al. Trichobezoar, rapunzel syndrome, tricho-plaster bezoar - a report of three cases. Pol J Radiol. 2015;80:241-246.
25. Raikar S, Wali P, Khan S. Recurrence of Rapunzel syndrome. J Pediatr. 2010;157:343-343.e1.
26. Winsett F, Ting J, Harting MT. Rapunzel syndrome: a rare case requiring surgical management of acute pancreatitis. Pancreas. 2019;48:e38-e39.
27. Rogers HK, Phadke O, Glaser AM. The curious case of Rapunzel syndrome: a rare non-trichobezoar. Am J Gastroenterol. 2018;113:783-784.
28. Al Wadan AH, Al Kaff H, Al Senabani J, et al. ‘Rapunzel syndrome’ trichobezoar in a 7-year-old girl: a case report. Cases J. 2008;1:205.
29. Chogle A, Bonilla S, Browne M, et al. Rapunzel syndrome: a rare cause of biliary obstruction. J Pediatr Gastroenterol Nutr. 2010;51:522-523.
30. Nwankwo E, Daniele E, Woller E, et al. Trichobezoar presenting as a gastric outlet obstruction: A case report. Int J Surg Case Rep. 2017;34:123-125.

There are 30 citations in total.

Details

Primary Language	English
Subjects	Pediatric Surgery
Journal Section	Articles
Authors	Bilge Gördü 0000-0002-7095-7225
Project Number	-
Publication Date	March 28, 2024
Published in Issue	Year 2024 Volume: 77 Issue: 1

Cite

APA	Gördü, B. (2024). Rapunzel Syndrome Causing Gastric Outlet Obstruction in A 12-Year-Old Girl: Report of A Case and Review of the Literature. Ankara Üniversitesi Tıp Fakültesi Mecmuası, 77(1), 113-118. https://doi.org/10.4274/atfm.galenos.2023.03064
AMA	Gördü B. Rapunzel Syndrome Causing Gastric Outlet Obstruction in A 12-Year-Old Girl: Report of A Case and Review of the Literature. Ankara Üniversitesi Tıp Fakültesi Mecmuası. March 2024;77(1):113-118. doi:10.4274/atfm.galenos.2023.03064
Chicago	Gördü, Bilge. “Rapunzel Syndrome Causing Gastric Outlet Obstruction in A 12-Year-Old Girl: Report of A Case and Review of the Literature”. Ankara Üniversitesi Tıp Fakültesi Mecmuası 77, no. 1 (March 2024): 113-18. https://doi.org/10.4274/atfm.galenos.2023.03064.
EndNote	Gördü B (March 1, 2024) Rapunzel Syndrome Causing Gastric Outlet Obstruction in A 12-Year-Old Girl: Report of A Case and Review of the Literature. Ankara Üniversitesi Tıp Fakültesi Mecmuası 77 1 113–118.
IEEE	B. Gördü, “Rapunzel Syndrome Causing Gastric Outlet Obstruction in A 12-Year-Old Girl: Report of A Case and Review of the Literature”, Ankara Üniversitesi Tıp Fakültesi Mecmuası, vol. 77, no. 1, pp. 113–118, 2024, doi: 10.4274/atfm.galenos.2023.03064.
ISNAD	Gördü, Bilge. “Rapunzel Syndrome Causing Gastric Outlet Obstruction in A 12-Year-Old Girl: Report of A Case and Review of the Literature”. Ankara Üniversitesi Tıp Fakültesi Mecmuası 77/1 (March 2024), 113-118. https://doi.org/10.4274/atfm.galenos.2023.03064.
JAMA	Gördü B. Rapunzel Syndrome Causing Gastric Outlet Obstruction in A 12-Year-Old Girl: Report of A Case and Review of the Literature. Ankara Üniversitesi Tıp Fakültesi Mecmuası. 2024;77:113–118.
MLA	Gördü, Bilge. “Rapunzel Syndrome Causing Gastric Outlet Obstruction in A 12-Year-Old Girl: Report of A Case and Review of the Literature”. Ankara Üniversitesi Tıp Fakültesi Mecmuası, vol. 77, no. 1, 2024, pp. 113-8, doi:10.4274/atfm.galenos.2023.03064.
Vancouver	Gördü B. Rapunzel Syndrome Causing Gastric Outlet Obstruction in A 12-Year-Old Girl: Report of A Case and Review of the Literature. Ankara Üniversitesi Tıp Fakültesi Mecmuası. 2024;77(1):113-8.

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