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.

Unveiling a Silent Obstructor: Phytobezoar in the Third Duodenal Segment.

We present a case of obstruction in the third portion of the duodenum secondary to a phytobezoar in an adult patient with no surgical history and without a vegan diet. High intestinal obstruction due to a phytobezoar is rarely described in the literature, posing a diagnostic challenge when evaluating potential differentials in the emergency setting. Subsequently, we conduct a review focusing on tomographic findings and the surgical specimen, highlighting key points to consider when addressing such pathologies.

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.

A rare cause of intestinal obstruction in children trichobezoar: How to diagnose?

BACKGROUND: Trichobezoar is a rare clinical condition in children, which is caused by the accumulation of swallowed hair mass in the digestive tract. This condition is most common in young women with psychiatric histories who suffer from trichotillomania (TTM), where they have an irresistible urge to pull out their hair. Diagnosis and treatment of this pathology, which is already extremely rare, and its variable clinical presentations are challenging. The aim of this study was to increase awareness of trichobezoar in the differential diagnosis of signs of intestinal obstruction in children and to evaluate the diagnosis and management of this rare pathology. METHODS: The clinical data of six patients who were treated for trichobezoars in the pediatric surgery department of our hospital between 2009 and 2022 were retrospectively analyzed. RESULTS: Six female patients were treated with the diagnosis of trichobezoar during this period. Patients were diagnosed with the help of anamnesis, physical examination, abdominal ultrasonography (USG), and finally, endoscopy. USG can predict the intestinal wall infiltration and the tail extended to the duodenum through pylorus in the series. All patients were evaluated with contrast-enhanced abdominal radiography. Five surgical interventions were performed in four of the cases. In a case who underwent surgery twice, the distal intestinal satellite bezoar was not noticed in the first operation. Two patients were diagnosed to have trichobezoar, but surgery was not required. These patients were younger and had early-onset TTM (before 10 years old). The patients were followed for an average of 10.8 years and no recurrence was detected. CONCLUSION: Trichobezoar is a rare cause of intestinal obstruction in children with fatal complications when diagnosed late. Failure to follow an algorithm for the management of the disease causes difficulties in the diagnosis and treatment. Especially in pa-tients with a known psychiatric history, whole abdominal USG and laparoscopy performed with awareness can prevent unnecessary examinations.

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.

Phytobezoar-Induced Mechanical Ileus and Incipient Intussusception: A Case Report.

Phytobezoars constitute conglomerates of indigested plant fibers and are a rare cause of acute mechanical ileus. They exhibit an increased prevalence in the elderly population and people with specific predisposing conditions. Radiological imaging can often set a definitive diagnosis and dictate the optimal therapeutic approach, combined with the patient's clinical status. An 81-year-old male presented with deteriorating clinical symptoms of intestinal obstruction, and an exploratory laparotomy was performed following inconclusive radiological findings; multiple phytobezoars and incipient intussusception were revealed intraoperatively. A patient's medical history can often raise clinical suspicion of phytobezoars. However, a careful etiological investigation is imperative in all cases of mechanical ileus in advanced ages; early detection and dissolution of phytobezoars, when applicable, can reduce the need for surgical interventions.

A Single-center 12-year Experience of Patients with Gastrointestinal Bezoars.

Background/Aims: Gastrointestinal (GI) bezoars are relatively rare diseases with clinical characteristics and treatment modalities that depend on the location of the bezoars. This study evaluated the clinical characteristics and treatment outcomes in patients with GI bezoars. Methods: Seventy-five patients diagnosed with GI bezoars were enrolled in this study. Data were collected on the demographic and clinical characteristics and the characteristics of the bezoars, such as type, size, location, treatment modality, and clinical outcomes. Results: Among the 75 patients (mean age 71.2 years, 38 males), 32 (42.6%) had a history of intra-abdominal surgery. Hypertension (43%) and diabetes (30%) were common morbidities. The common location of the bezoars was the stomach in 33 (44%) and the small intestine in 33 (44%). Non-surgical management, including adequate hydration, chemical dissolution, and endoscopic removal, was successful in 2/2 patients with esophageal bezoars, 26/33 patients with gastric bezoars, 7/9 patients with duodenal bezoars, and 20/33 patients with small intestinal bezoars. The remaining patients had undergone surgical management. Conclusions: The management of GI bezoars requires multidisciplinary approaches, including the appropriate correction of fluid and electrolyte imbalances, chemical dissolution, and endoscopic and surgical treatments.

Recurrent Rapunzel Syndrome in an Adolescent Female.

Bezoars are concrete masses found within the gastrointestinal tract that can lead to obstructions. One of the most common forms of bezoars is trichobezoar, composed of swallowed hair. Many bezoars are confined to the stomach; however, a small occurrence of trichobezoars extends past the pylorus and into the duodenum, or small bowel, termed Rapunzel syndrome. In the literature, there have been few cases of recurrent Rapunzel syndrome. Our case is a 13-year-old female with recurrent Rapunzel syndrome requiring three operative interventions.

Intestinal Obstruction Due to Reassembly after Endoscopic Crushing of a Bezoar.

An 87-year-old woman visited her primary-care doctor because of nausea and epigastric pain. Esophagogastroduodenoscopy (EGD) revealed a giant bezoar in her stomach. She was referred to our hospital after carbonated beverage dissolution proved ineffective and underwent endoscopic mechanical crushing. After crushing, the symptoms disappeared, and she began eating. Later, however, the crushed fragments reassembled in the duodenal bulb and caused intestinal obstruction. The patient underwent emergency EGD for crushing once more, and all of the fragments were extracted from the body. This case highlights the need for bezoars to be removed from the body after crushing in order to avoid reassembly.

An atypical death from Rapunzel syndrome: a case report.

Trichotillomania is a psychiatric disorder characterized by recurring urges to pulling out hairs, eyelashes, or down in other parts of the body. Trichophagia, which is the urge to ingesting the pulled-out hairs, can cause Rapunzel syndrome, an unusual disorder where gastric trichobezoars can be found in the small intestine. Trichobezoars, amorphous masses composed of undigested food formed by hairs, can obstruct the gastrointestinal tract up to simulating symptoms typical of bowel obstruction. Rapunzel syndrome, named after Grimm's tale, may cause death, especially in the pediatric population, being it seldom over the age of 6; moreover, developing countries and environmental and familiar issues are listed as uncertain risk factors. The present case report deals with the death of a 4-year-old female occurred after lunch and following a series of vomit events; while no traumatic or pathological findings were revealed at the external examination, the autopsy revealed three large trichobezoars localized in the stomach and the small intestine. Despite death was due to gastrointestinal obstruction for multiple trichobezoars and collateral bronchoaspiration of dietary material, histological findings were totally non-specific, meaning that it is sometimes difficult to conclude that death is related to the primary pathological condition.

An Unusual Small Bowel Phytobezoar Successfully Resolved by Double-balloon Enteroscopy.

We herein report a very unusual case of small bowel obstruction caused by phytobezoar in a 69-year-old woman who consumed a large amount of bracken. The patient presented with nausea and vomiting. Computed tomography revealed an air-filled foreign body in the jejunum that had likely caused the small bowel obstruction. A fibrous foreign body diagnosed as a phytobezoar was detected using double-balloon enteroscopy. The obstruction was successfully resolved by crushing the phytobezoar repeatedly using a snare. Small bowel obstructions caused by phytobezoars are often treated with surgical interventions. However, endoscopic fragmentation using a snare is a minimally invasive treatment alternative.

Small bowel obstruction secondary to a plastic bezoar.

We present a case of a small bowel obstruction secondary to a rare plastic bezoar. A man in their early 20s with autism and an intellectual disability presented with symptoms of small bowel obstruction. CT revealed very subtle signs and, despite passage of gastrografin, ongoing clinical suspicion led to operative management which confirmed the diagnoses of plastic bezoar.

An Unusual Case of Primary Ileal Trichobezoar Causing Intussusception.

BACKGROUND Intussusception, which is the prolapse of one section of intestine into another, is a common cause of small-bowel obstruction in pediatric patients. Bezoars are concretions of ingested foreign material. Trichobezoars, which are bezoars made of hair, occur in the context of trichotillomania, the compulsive pulling of hair, and trichophagia, the eating of hair. If gastric trichobezoars grow to sufficient size, an intestinal extension can serve as a lead point for intussusception to occur. Rarely, hair passes completely through the stomach and forms a trichobezoar within the small bowel. This obstruction can also create lead points and cause intussusception. This is one of the few reported cases of intussusception due to a primary intestinal bezoar. CASE REPORT We present the case of an 8-year-old boy with an unknown history of trichophagia and a preliminary diagnosis of appendicitis. Upon imaging, a bowel obstruction related to a small-bowel intussusception was discovered to be the probable cause. A diagnostic laparoscopy revealed an ileo-ileal intussusception caused by an ileal bezoar. Conversion to exploratory laparotomy assisted in removing the causative bezoar. The patient recovered without postoperative complications. CONCLUSIONS We report a case of an isolated intestinal trichobezoar causing intussusception in a boy. While intussusception secondary to a trichobezoar most commonly occurs due to 'Rapunzel syndrome,' this case shows that it is possible for an intestinal trichobezoar to form without the presence of 'Rapunzel syndrome.' This unique cause of intussusception presented as a small-bowel obstruction, requiring evacuation of the bezoar.