Rapunzel syndrome in children: a retrospective review of ten cases combined with literature review in a tertiary referral center.

PURPOSE: Rapunzel syndrome is an uncommon condition in children, and its clinical features remain unclear. This study presents the largest single-center series of pediatric cases to date, with the objective of documenting the clinical characteristics and treatment approaches for children with Rapunzel syndrome. METHODS: A retrospective study was conducted in children with Rapunzel syndrome from 2019 to 2023. We recorded age, gender, symptoms, locations of bezoar, complications, and treatment options. RESULTS: Ten patients with Rapunzel syndrome were included. The median age was 9.1 years, with all of whom were female. The most common clinical symptoms were upper abdominal mass (90%), abdominal pain (80%), and nausea and vomiting (50%). Complications occurred in six cases (60%), including small bowel obstruction (20%), severe gastric dilatation (10%), intestinal perforation (10%), choledochodilation (10%), acute pancreatitis with cholecystitis (10%). Preoperative ultrasonography suggested low-echoic foreign bodies continuing to the jejunum or ileocecal region in five cases (50%). Preoperative gastroscopy attempted in four cases (40%) to remove the foreign bodies, all of which failed. All patients underwent surgical treatment, with nine cases undergoing gastric incision foreign body removal, and one case undergoing gastric incision foreign body removal combined with intestinal perforation repair. All patients recovered well. No recurrence was observed during follow-up. CONCLUSION: The accuracy of ultrasound diagnosis in identifying Rapunzel syndrome is high; however, it may lead to misdiagnosis if not complemented with the patient's medical history. Endoscopic presents a heightened treatment risk and a reduced success rate. The condition commonly presents with severe complications, thus making laparotomy a safe and effective option for intervention.

Successful endoscopic treatment of a huge trichobezoar in a 10-year-old girl.

A 10-year-old girl was admitted to our hospital due to acute pancreatitis. Computed tomography showed an intra-gastric mass containing multiple small air bubbles. Ultrasound showed a well-circumscribed large oval mass with a broad acoustic shadow. Endoscopy revealed a huge trichobezoar with many movable hairs, being judged by the cause of acute pancreatitis. Due to the parents' strong preference not to leave any surgical scars on their daughter, the patient underwent endoscopic treatment. The trichobezoar grasped with a snare was too large to pass through the esophageal-gastric junction. In addition, the outer layer of the trichobezoar was too hard to be cut with conventional endoscopic devices but was successfully cut with a FlushKnife. The content of the trichobezoar was much softer than its hard surface but needed appropriate counter-traction to be torn off the tissue. Two alligator forceps via a dual-channel multi-bending scope were able to give sufficient counter-traction to the inner tissue of the trichobezoar, successfully removing the trichobezoar through piece-by-piece tearing off. All the endoscopic procedures took seven hours for the complete trichobezoar removal. The total weight of the dissected mass was 180 g. The girl resumed eating on the next day and was discharged on the third day. Physicians should note that a medical team with full endoscopic expertise can remove huge trichobezoars using a FlushKnife, a dual-channel multi-bending scope, and two alligator forcepses.

Expect the unexpected: bezoar-caused gastric perforation in the 19-year-old patient, after traffic accident.

A 19-year-old female involved in a traffic accident presented to the Emergency Room (ER) with no trauma-related symptoms but a palpable mass in the epigastrium. Imaging revealed a massive trichobezoar causing gastric perforation. Urgent laparotomy was performed, and a 1.5-kilogram bezoar was removed, along with repairing coexisting gastric ulcers. The patient had a history of trichophagia, suggesting a psychiatric association. This case highlights the potential of trichobezoars to cause gastric perforation, even in patients admitted for unrelated reasons. CT-scan proves effective in diagnosing such cases. While a traffic accident might be a plausible cause, the presence of a bezoar can elevate the risk of complications. Psychiatric evaluation is recommended when trichophagia is identified. The study underscores the need for vigilance in unexpected scenarios, demonstrating the importance of multidisciplinary approaches in managing such cases.

Trichotillomania, Trichophagia and Trichobezoar in a Male Paediatric Patient: A Case Report and Literature Review.

INTRODUCTION AND IMPORTANCE: Trichotillomania and tricophagia, characterized by compulsive hair-pulling and subsequent ingestion which results in a compact mass of hair called trichobezoar. It represents an uncommon psychiatric disorder, especially in young children. CASE PRESENTATION: This case report describes a distinctive and rare occurrence of trichotillomania, tricophagia and trichobezoar in a 11-year-old male child. Concerns raised by the parents regarding noticeable hair loss, who initially presented to medical outdoor patient with complaints of abdominal pain on and off from the last one year. He had a history of pica and weight-loss. He was then diagnosed with a gastric trichobezoar for which he was operated upon and a giant trichobezoar was retrieved from his stomach. Post-operatively patient remained admitted in ward and was discharged home on fifth post-operative day and sent for psychiatry evaluation. CLINICAL DISCUSSION: Trichotillomania and tricophagia often have roots in psychosocial stressors, anxiety, and depression. Children may engage in hair-pulling as a coping mechanism, especially in response to familial or environmental stressors. The literature emphasizes the importance of understanding the psychosocial context to tailor interventions effectively. CONCLUSION: Trichotillomania and tricophagia is very rare in paediatric population and if presents a multidisciplinary team comprising of a paediatrition, paediatric surgeon and paediatric psychiatrist should be involved and if diagnosed with a gastric trichobezoar should be removed surgically in order to prevent complications.

A Rare Case of Trichobezoar Revealing Undiagnosed Celiac Disease.

Trichobezoar is a relatively rare condition in children, mainly observed in young girls with psychiatric disorders. While documented cases of trichobezoar associated with celiac disease exist, such occurrences remain uncommon in the literature. The association between the two can be explained either by behavioral disorders resulting from a deficiency in iron and folic acid or directly by celiac disease. Treatment is predominantly surgical, and psychological support plays a crucial role in preventing the likelihood of recurrence. We present an unusual case involving the discovery of gastric trichobezoar in a 15-year-old girl who had undiagnosed celiac disease. The condition manifested after she experienced abdominal pain and pallor.

Gastrointestinal trichobezoars in the pediatric population: a retrospective study.

BACKGROUND: Trichobezoar is an extremely rare condition characterized by a foreign body in the gastrointestinal tract (GIT) among children. The foreign body may exist in the digestive tract for several years, and it becomes evident if complications develop. The current study aimed to present 21 cases of GIT trichobezoars. METHODS: Retrospective analysis of children who were diagnosed with trichobezoars between August 2012 and December 2022. Patient demographics, clinical presentation, diagnosis, and therapy were collected and analyzed.Twenty-one patients had GIT trichobezoars. Data were collected and analyzed retrospectively. RESULTS: Twenty-one patients were identified. All patients were female. Their mean age at admission was 8.9 ± 1.9 years. Furthermore, 19 (90.5%) patients presented with abdominal pain, 16 (76.2%) with vomiting, and 13 (61.9%) with a palpable mass. Sixteen patients underwent gastroduodenoscopy. Among them, 15 had gastric trichobezoars. Moreover, 12 patients underwent computed tomography scan. Eight patients presented with gastric and small intestinal BZs, one presented with increased small intestinal contents with dilation, and one presented with abundant gastric contents. Then, 20 patients underwent surgery. Among them, five underwent laparoscopic-assisted minilaparotomy (LAML), and the rest underwent laparotomy. The results showed that 10 (50%) patients had gastric trichobezoars; 7 (35%), Rapunzel syndrome; and 3 (15%), small bowel trichobezoars. Two patients developed superficial wound infection postoperatively. One patient had a recurrent gastric trichobezoar. CONCLUSION: Trichobezoar should be considered in young girls with a history of hair eating or those with hair in the vomit or feces. Timely diagnosis and aggressive treatment are the keys to reducing complications and improving prognosis. Laparoscopic-assisted minilaparotomy is a safe, feasible, and effective surgical method for treating trichobezoars.

Trichobezoar Found Accidently while Diagnosing Resistance to Thyroid Hormone.

Introduction: Bezoars are masses of indigestible foreign material in the gastrointestinal tract, usually in the stomach. These materials could be indigestible fruits, hair, milk products, or tablets. In children, the most common type of bezoar is trichobezoar (formed from hair). Case Presentation: We describe a female patient who has been complaining about deterioration of mood, collapse without losing consciousness, scotomas, and cardiac arrhythmia for 2 years. Based on the results of thyroid hormone, resistance to thyroid hormone (RTH) was suspected. Physical examination during hospitalization revealed a palpable upper abdominal mass. Several diagnostic examinations were performed. The abdominal ultrasound showed acoustic shadowing caused by a pathological structure in the upper abdomen. Therefore, the contrast X-ray of the digestive tract revealed a deficit of contrast with an irregular shape in the stomach body and the pylorus region. Due to these results, a gastroscopy was performed, which revealed a large trichobezoar of the stomach. The trichobezoar was surgically removed without complications. Conclusion: The case presented shows that these nonspecific symptoms and laboratory test suggesting RTH require multi-path diagnostics and the cooperation of many specialists, ultimately giving a surprising diagnosis. It is crucial to interpret diagnostic examinations with regard to the patient's physical condition. Diagnosis of trichobezoar requires a detailed search of causes to avoid another incident.

A case of Rapunzel syndrome.

Rapunzel syndrome is a rare disorder characterized by a trichobezoar in the gastroduodenal tract caused by trichophagia. Diagnosis was confirmed by upper endoscopy and treatment was surgical.

Endoscopic Removal of a Recurrent Trichobezoar in an Adolescent: A Case Report.

Trichobezoar, a rare condition of intragastric hair accumulation is commonly associated with an underlying psychological condition. Removal of the bezoar either endoscopically or surgically (laparoscopy or laparotomy) with concurrent psychiatric assessment and treatment is the mode of treatment. We present a 10-year-old child with recurrent trichobezoar, who was managed surgically the first time, and subsequently endoscopic removal was done on recurrence of bezoar after 3 months. We also present the difficulties encountered during endoscopic bezoar removal.

When the Gut Tells a Story: Bezoars in a Neglected Autistic Child.

This case study delves into the unique presentation of bezoars in a 14-year-old autistic female who exhibited chronic diarrhea and abdominal pain. While trichobezoars, masses formed from ingested hair, are rare, they are predominantly seen in young females and are associated with psychiatric conditions. Through rigorous diagnostic procedures, including a computed tomography imaging of the abdomen and pelvis (CTAP) scan, fecal impaction, and multiple bezoars, including hair and non-biological items, were identified. The background revealed significant neglect, emphasizing the importance of a comprehensive approach that integrates medical, surgical, and psychosocial care.

Rapunzel Syndrome: A Rare Case of Phyto-Trichobezoar in an Indian Girl.

Phyto-trichobezoar is a rare disorder characterized by the formation of mass in the gastrointestinal tract (GIT) by the ingested inedible material, mostly hair and thread. The ingestion of hair is a rare psychiatric disorder called trichotillomania, which is more common in girls. An 11-year-old girl presented with clinical features of GIT obstruction, which were diagnosed to be Rapunzel syndrome by computed tomography. The phyto-trichobezoar was removed en masse by the anterior gastrotomy, which extended beyond the duodenum. The girl, after recovery, was counseled and treated for subclinical depression.

Histopathologic diagnosis and patient characteristics in cats with small intestinal obstructions secondary to trichobezoars.

OBJECTIVES: The aim of the present study was to describe the distribution of gastrointestinal histopathology findings associated with gastrointestinal obstructions secondary to trichobezoar formation in cats. METHODS: A total of 100 surgical gastrointestinal biopsies were obtained from 44 cats with gastrointestinal obstructions secondary to a trichobezoar. Medical records, including signalment, type and duration of clinical signs, surgical reports and histopathologic analysis, were reviewed for each cat. RESULTS: Biopsies taken near the site of the trichobezoar were more likely to show neutrophilic inflammation and mucosal erosion/ulceration compared with biopsies taken elsewhere in the small intestine. Lymphoplasmacytic and mixed lymphocytic and eosinophilic populations were the most common histopathologic findings from all biopsies followed by alimentary small cell lymphoma. Biopsy samples were more likely to represent a diagnosis of alimentary lymphoma in cats older than 10 years. CONCLUSIONS AND RELEVANCE: Gastrointestinal biopsies taken at the time of surgery in cats with trichobezoar obstructions may represent an important diagnostic tool for further evaluation of potential feline chronic enteropathy. Biopsies taken at the site of the obstruction should be interpreted cautiously as the presence of a trichobezoar may induce an acute inflammatory reaction. The resultant histologic interpretation at this site may not represent the chronic state of the intestinal mucosa, supporting the utility of obtaining multiple biopsies orad and aborad to the obstruction.

Trans-umbilical Intragastric Surgery Using Two Alexis Wound Retractors for a Large Trichobezoar in a Four-Year-Old Girl: A Case Report.

Trichobezoars are difficult to remove endoscopically and often require surgery. We performed trans-umbilical intragastric surgery using two Alexis wound retractors with successful results in a pediatric patient with a trichobezoar. This method is a safe and cosmetically favorable option for the removal of large trichobezoars and does not require special techniques or instruments. It also contributes to the reduction of postoperative complications such as wound infection and intra-abdominal abscess.

Respiratory and intestinal trichobezoar in a female: An unusual case report and literature review.

Trichobezoars are hairballs in the gastrointestinal tract that usually develop due to the consumption of hair after pulling it. However, some rare case reports suggest that trichobezoar can also occur in the respiratory system. In this context, we present an unusual case of a 25-year-old woman who experienced dyspnea, productive cough, and leukocytosis. The patient was found to have a trichobezoar in her respiratory tract, accompanied by the presence of hair in her digestive tract.

Revelation of Rapunzel syndrome: A rare case report of gastric trichobezoar-induced acute purulent peritonitis.

INTRODUCTION AND IMPORTANCE: The Rapunzel syndrome embodies an uncommon variation of trichobezoar, predominantly observed among individuals with a history of psychiatric conditions, trichotillomania, and trichophagia. This combination of factors predisposes to the eventual formation of gastric bezoars. Trichobezoars, infrequent gastric masses composed of hair, can lead to complications if left untreated. CASE REPORT: A 19-year-old female hairdresser with a history of trichophagia sought medical attention due to abdominal pain and episodes of vomiting. With an elevated body temperature and abdominal rigidity, imaging revealed the presence of a trichobezoar, accompanied by pneumoperitoneum and intraperitoneal effusion. Urgent surgical intervention confirmed the diagnosis of generalized purulent acute peritonitis, triggered by a gastric perforation caused by a 20 cm trichobezoar with an extension into the duodenum, which defines the Rapunzel syndrome. Gastrotomy was performed to remove the trichobezoar, followed by thorough peritoneal lavage. CLINICAL DISCUSSION: Trichobezoar is a rare condition that involves the abnormal accumulation of solid substances, particularly hair, within the stomach. Known as "Rapunzel syndrome," it can extend into the duodenum or jejunum. Commonly associated with emotional disorders, trichobezoar can lead to symptoms like epigastric discomfort, vomiting, and weight loss. Diagnosis is through endoscopy, and treatments include fluid intake, endoscopic extraction, chemical dissolution, and surgical removal. Surgical intervention is often preferred, with laparoscopic approaches considered. Psychiatric management is often required for patients. CONCLUSION: This case underscores the uncommon Rapunzel syndrome presentation, emphasizing timely surgical measures and multidisciplinary care for trichobezoars causing acute peritonitis.

A Rare Case of Rapunzel Syndrome Presenting with Perforation Peritonitis.

Rapunzel syndrome with gastric perforation is an extremely rare presentation of trichobezoars of the stomach. Trichobezoars may vary greatly in presentation ranging from benign symptoms like vague abdominal pain and anorexia to grave complications like perforation peritonitis. A sincere evaluation of any underlying psychiatric illnesses, usually trichotillomania and trichophagia, holds the key to preventing recurrences in patients of trichobezoar. A 15-year-old adolescent female presented with signs and symptoms of enteric perforation with a history of trichotillomania and trichophagia. Exploratory laparotomy of the patient revealed anterior gastric perforation with a huge gastric trichobezoar that extended into the duodenum and jejunum, hence establishing the diagnosis of Rapunzel syndrome.

Mallory-Weiss syndrome from giant gastric trichobezoar: A case report.

BACKGROUND: Mallory-Weiss syndrome (MWS), representing a linear mucosal laceration at the gastroesophageal junction, is a quite frequent cause of upper gastrointestinal bleeding, usually induced by habitual vomiting. The subsequent cardiac ulceration in this condition is likely due to the concomitance of increased intragastric pressure and inappropriate closure of the gastroesophageal sphincter, collectively inducing ischemic mucosal damage. Usually, MWS is associated with all vomiting conditions, but it has also been described as a complication of prolonged endoscopic procedures or ingested foreign bodies. CASE SUMMARY: We described herein a case of upper gastrointestinal bleeding in a 16-year-old girl with MWS and chronic psychiatric distress, the latter of which deteriorated following her parents' divorce. The patient, who was residing on a small island during the coronavirus disease 2019 pandemic lockdown period, presented with a 2-mo history of habitual vomiting, hematemesis, and a slight depressive mood. Ultimately, a huge intragastric obstructive trichobezoar was detected and discovered to be due to a hidden habit of continuously eating her own hair; this habit had persisted for the past 5 years until a drastic reduction in food intake and corresponding weight loss occurred. The relative isolation in her living status without school attendance had worsened her compulsory habit. The hair agglomeration had reached such enormous dimensions and its firmness was so hard that its potential for endoscopic treatment was judged to be impossible. The patient underwent surgical intervention instead, which culminated in complete removal of the mass. CONCLUSION: According to our knowledge, this is the first-ever described case of MWS due to an excessively large trichobezoar.

Successful endovascular treatment of superior mesenteric artery-duodenal fistula secondary to Rapunzel syndrome.

Rapunzel syndrome is a rare clinical entity in pediatric patients with a history of trichotillomania and trichophagia that has only been mentioned a few times in the literature. It is characterized by abnormal gastric bezoar formation that sometimes extends to the duodenum, jejunum, or colon. Here, we present a case of a 16-year-old previously healthy female patient who had prolonged hospitalization due to complications related to a significant gastric bezoar that led to massive bleeding due to a superior mesenteric artery (SMA)-duodenal fistula successfully treated with stent graft placement. Undiagnosed trichobezoar can lead to rare and unexpected complications, such as SMA-duodenal fistula, with life-threatening hemorrhagic shock. Prompt activation of massive transfusion protocol and endovascular control of the hemorrhage was vital to successfully treating our patient.