Godlewska, Joanna

Górecki, Wojciech

Orpiszewska, Justyna

Pediatria i Medycyna Rodzinna

Polish

Jagiellonian Univeristy Repository

64Joanna Godlewska, Justyna Orpiszewska, Wojciech Górecki© Pediatr Med Rodz 2021, 17 (1), p. 64–66© Medical Communications Sp. z o.o. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License (CC BY-NC-ND). Reproduction is permitted for personal, educational, non-commercial use, provided that the original article is in whole, unmodified, and properly cited.DOI: 10.15557/PiMR.2021.0010Received: 13.05.2020Accepted: 28.07.2020Published: 05.03.2021Recurrent trichobezoar in a patient with Rapunzel syndromeNawrotowy trichobezoar u pacjentki z zespołem RoszpunkiDepartment of Paediatric Surgery, Jagiellonian University Medical College, University Children Hospital, Kraków, PolandCorrespondence: Joanna Godlewska, Department of Paediatric Surgery, Jagiellonian University Medical College, Wielicka 265, 30-663 Kraków, Poland,  tel.: +48 12 333 91 53, e-mail: joanna.godlewska@gmail.comRapunzel syndrome is a condition where a trichobezoar is formed in the stomach and proximal intestine due to hair ingestion. A 6-year-old girl presented to emergency department with abdominal pain, vomiting and a palpable epigastric mass. Laparotomy was performed for gastric foreign body; a trichobezoar that filled the stomach, duodenum and proximal small intestine was removed. Postoperative course was uncomplicated; the patient was discharged for further out-patient follow-up and psychological care. After 7 months, the girl presented with a recurrence. A recurrent trichobezoar was removed via laparotomy. The girl was started on psychiatric treatment and iron substitution for anaemia. Ten weeks after discharge, follow-up gastroscopy was negative for gastric foreign body. There are no guidelines for follow-up after trichobezoar removal. Since the disease may be recurrent, follow-up endoscopy should be considered in order to enable an early diagnosis and less invasive treatment.Keywords: trichobezoar, Rapunzel syndrome, gastrointestinal foreign bodyZespół Roszpunki jest schorzeniem, w którym na skutek powtarzalnego spożywania włosów dochodzi do powstawania trichobezoaru wypełniającego żołądek i bliższy odcinek jelita cienkiego. Sześcioletnia dziewczynka zgłosiła się na szpitalny oddział ratunkowy z powodu bólu brzucha, wymiotów i badalnego oporu w nadbrzuszu. Ustalono rozpoznanie ciała obcego w żołądku i drogą laparotomii usunięto trichobezoar będący odlewem żołądka, dwunastnicy i bliższego odcinka jelita cienkiego. Pacjentkę wypisano w 5. dobie pooperacyjnej z zaleceniem kontroli ambulatoryjnej i opieki psychologicznej. Po 7 miesiącach dziewczynka zgłosiła się z nawrotem objawów. Wykonano laparotomię i usunięto nawrotowy trichobezoar. Włączono leczenie psychiatryczne oraz substytucję żelaza. Po 10 tygodniach od wypisu przeprowadzono kontrolną gastroskopię, w której nie stwierdzono obecności ciała obcego. Obecnie brak jest wytycznych odnośnie do postępowania pooperacyjnego po usunięciu trichobezoaru. Ze względu na możliwość nawrotowego przebiegu choroby należy rozważyć planową kontrolę endoskopową, która może pozwolić na wcześniejsze rozpoznanie nawrotu i zastosowanie mniej inwazyjnego leczenia.Słowa kluczowe: trichobezoar, zespół Roszpunki, ciało obce w przewodzie pokarmowymAbstractStreszczenieRecurrent trichobezoar in a patient with Rapunzel syndrome65DOI: 10.15557/PiMR.2021.0010PEDIATR MED RODZ Vol. 17 No. 1, p. 64–66INTRODUCTIONA bezoar is a gastrointestinal foreign body resulting from deposition of indigestible matter. Bezoars are formed in the context of gastrointestinal dysmotil-ity, altered anatomy resulting from surgical interventions, and in psychiatric conditions manifesting with eating dis-orders (pica). Rapunzel syndrome is a condition where re-peated hair ingestion (trichophagia) leads to the formation of a trichobezoar in the stomach and duodenum, some-times extending into the small intestine.CASE REPORTA previously healthy 6-year-old girl presented to our emer-gency department in November 2017 with abdominal pain and vomiting. On the previous day, the girl’s parents no-ticed a protruding mass in her epigastric region. Physical examination and radiography (abdominal sonography and X-ray) yielded a diagnosis of gastric foreign body (Fig. 1). Laparotomy and gastrotomy resulted in the removal of a trichobezoar with a total length of 73 cm, filling the stom-ach, duodenum and proximal small intestine (Figs. 2, 3).The postoperative course was uncomplicated; the patient was discharged on day five postoperatively. She was referred for psy-chological care and recommended outpatient surgical follow-up.Four months later, the girl presented again in the emergen-cy department with abdominal pain. Physical examination, abdominal sonography and abdominal X-ray were normal; she was discharged home.After another 3 months (7 months postoperatively), the girl presented with another episode of abdominal pain and vomiting, a palpable epigastric mass and mass effect on ab-dominal X-ray. A recurrent trichobezoar was removed via laparotomy and gastrotomy from the stomach, duodenum and the first intestinal loop (Figs. 4, 5).The recovery was uneventful. The consulting psychiatrist started the girl on SSRI (selective serotonin re-uptake inhib-itor) treatment. Iron substitution for iron-deficiency anae-mia was introduced. Social worker opinion was obtained. The patient was discharged home after 5 days with recom-mendation of outpatient psychiatric treatment, surgical control and gastroenterology consultation.Ten weeks after discharge, a follow-up gastroscopy was performed, which was negative for gastric foreign body. The patient was well and asymptomatic. Pharmacotherapy is not longer required, but she continues behavioural therapy.DISCUSSIONRapunzel syndrome is a rare disease, most commonly af-fecting young girls and women suffering from psychiatric disorders(1). Without efficient treatment of trichophagia, re-currences may occur. Only few cases of recurrent trichobe-zoars have been reported in the literature; they mostly con-cern adult patients(2–4).The treatment of a bezoar producing acute symptoms is usually surgical via laparotomy and gastrotomy. There are reports of laparoscopic removal(5) as well as endoscopic fragmentation of a bezoar with either endoscopic removal Fig. 1.  Abdominal X-ray at presentationFig. 2.  Intraoperative view (first operation)Fig. 3.  Operative specimen (first operation)Joanna Godlewska, Justyna Orpiszewska, Wojciech Górecki66DOI: 10.15557/PiMR.2021.0010 PEDIATR MED RODZ Vol. 17 No. 1, p. 64–66or leaving the fragmented mass for natural evacuation via the gastrointestinal tract(6). However, these less invasive methods have limited application in intestinal obstruction and a large mass. In patients with no acute symptoms, there are reports of successful dissolution of bezoars with carbon-ated drinks such as Coca-Cola®, beer or sparkling mineral water(7), with enzymatic substances(8), with or without en-doscopic assistance. Thus, elective treatment may help avoid invasive surgical procedures.Due to the rare occurrence of trichobezoars, especially in the recurrent form, there are no widely accepted standards for patient monitoring after bezoar removal. Psychiatric and psychological care are the mainstay of treatment and are aimed at controlling trichophagia. Routine endoscop-ic control may permit early detection of recurrence, which in consequence could allow successful conservative man-agement of a small mass and avoidance of repeated surgi-cal intervention.CONCLUSIONSThe described case is an example of an atypical, recurrent course of Rapunzel syndrome. There are no guidelines for follow-up after trichobezoar removal. Since the disease may be recurrent, follow-up endoscopy should be considered in order to enable an early diagnosis and less invasive treat-ment. We warn both paediatricians and family practitioners about the possibility of recurrent disease and underline the importance of continuous psychological support due to the possibility of persistent behavioural disorder.Conflict of interestAuthors do not report any financial or personal connections with other persons or organisations, which might negatively affect the contents of this publication and/or claim authorship rights to this publication.References1. Chana RS, Viral A, Kumar R: Gastrointestinal bezoars in chil-dren with special reference to recurrent trichobezoar. J Indian Assoc Pediatr Surg 2004; 9: 25–29.2. Morales-Fuentes B, Camacho-Maya U, Coll-Clemente FL et al.: Trichotillomania, recurrent trichobezoar and Rapunzel syndrome: case report and literature review. Cir Cir 2010; 78: 265–266.3. Kırpınar I, Kocacenk T, Koçer E et al.: Recurrent trichobezoar due to trichophagia: a case report. Gen Hosp Psychiatry 2013; 35: 439–441.4. Kiernan MF, Kamat S, Olagbaiye F: Cows-feet soup: a rare cause of recurrent trichobezoar. BMJ Case Rep 2012; 2012: bcr0220125787.5. Bustos R, Gheza F, Masrur M: Snapshot in surgery: how do you approach this 12-year-old girl? Clin Case Rep 2018; 6: 1389–1390.6. Nunes G, Patita M, Barosa R et al.: Small bowel obstruction after endoscopic fragmentation of a gastric bezoar: an unexpected complication. Turk J Gastroenterol 2018; 29: 616–618.7. Iwamuro M, Yamauchi K, Shiraha H et al.: All carbonated bev-erages effectively dissolve phytobezoars. Clin Res Hepatol Gas-troenterol 2018; 42: e66–e67.8. Cerezo Ruiz A, Domínguez-Jiménez JL, Uceda-Vaño A: Cellu-lase, Coca-Cola®, pancreatin and ursodeoxycholic acid in the dis-solution of gastric bezoars: why not all together? Rev Esp Enferm Dig 2018; 110: 472–473.Fig. 4.  Intraoperative view (second operation)Fig. 5.  Operative specimen (second operation)

Recurrent trichobezoar in a patient with Rapunzel syndrome

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Recurrent trichobezoar in a patient with Rapunzel syndrome

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