Rapunzel syndrome: an uncommon tale of a long hairy tail - case report and review of literature

Trichobezoar is a rare clinical entity in which a ball of hair accumulates within the alimentary tract. When the tail of the trichobezoar extends into the small intestine, this condition is called Rapunzel syndrome (RS). A 14-year-old female presented with pain abdomen and vomiting for 2 weeks, and a history of trichotillomania and trichophagia, and an epigastric lump. A contrast enhanced computerized tomography (CECT) of the abdomen showed a grossly distended stomach with a heterogeneous mass containing trapped air with underlying normal mucosa suggestive of trichobezoar, with its tail extending into the proximal jejunum suggestive of RS. During laparotomy, a giant trichobezoar was seen in the stomach with its tail extending beyond the duodenum into the proximal jejunum. The entire specimen was delivered out intact. On follow up, she has no surgical complications, and was on behaviour therapy. Trichobezoars form when ingested hair strands become retained in the folds of the gastric mucosa and becomes entangled, forming a ball too large to exit the stomach. Trichotillomania and trichophagia are seen in many of these patients. Patients present with abdominal pain, vomiting, gastric outlet obstruction, and an epigastric mass. In the case of RS, complete removal without breakage and distal migration is important. RS should be considered as a differential diagnosis in a young girl with abdominal pain, vomiting, anaemia and upper abdominal lump. Early diagnosis prevents complications. Surgical removal is treatment of choice. Trichobezoar often coexists with psychiatric illness. Psychiatric evaluation, counselling and treatment are helpful in preventing recurrence.

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A68 DISTAL MIGRATION OF A PERCUTANEOUS GASTROJEJUNAL DUODOPA INFUSION TUBE DUE TO BEZOAR FORMATION AT THE JEJUNAL TUBE TIP

Journal of the Canadian Association of Gastroenterology ◽

10.1093/jcag/gwab002.066 ◽

2021 ◽

Vol 4 (Supplement_1) ◽

pp. 30-31

Author(s):

O Akman ◽

D Duerksen

Keyword(s):

Gastric Tube ◽

Rare Complication ◽

Symptom Control ◽

Tube Placement ◽

Surgical Removal ◽

Proximal Jejunum ◽

Surgical Extraction ◽

Distal Migration ◽

Jejunal Tube ◽

Gastroduodenal Ulceration

Abstract Background Continuous intestinal infusion of levodopa/carbidopa intestinal gel (LCIG) for the treatment of advanced Parkinson’s Disease (PD) leads to less variability in plasma drug levels and improved symptom control. Percutaneous Gastrojejunostomy (PEG-J) tube placement has a high placement success rate; however, delayed tube malfunctions occur in approximately 58% of cases within two years. A rare complication is bezoar formation at the jejunal tube tip. Aims To present a case of bezoar formation at the jejunal tip of a PEG-J tube that caused distal migration of the tube with gastroduodenal ulceration and required surgical extraction. Methods Full chart review was conducted including clinical notes, laboratory results, radiographic imaging, endoscopy reports, and surgical reports. A relevant literature review was conducted. Results A 57-year-old male with severe PD underwent endoscopic guided PEG-J tube insertion for continuous infusion of LCIG; intestinal administration was effective for symptom control. Two years later, he noted that the gastric tube had retracted approximately 15 cm into the stoma without external manipulation of the apparatus. Attempts to externally pull the tube back into position were unsuccessful. The patient underwent Gastroscopy (EGD) with fluoroscopy. Contrast was used to confirm placement of the jejunal tip within the jejunum, but also showed migration of the gastric tip into the duodenum. A gastroscope was used to reposition the gastric tube in the stomach; the jejunal tube was visualized to be under traction. The bumper on the apparatus was re-positioned and external tape was used to further secure the apparatus and prevent migration. A month later the tube had migrated again; repeat EGD showed the jejunal tube to be under traction with some resultant ulceration of the pyloric channel and duodenal bulb where the tube had been pressing against the mucosa. The jejunal tube could not be pulled back and appeared to be fixed distally. A CT scan was obtained to assess for complications and a coiled tip was seen in the proximal jejunum. Surgical extraction of the malfunctioning tube was required. At laparotomy, the coiled tip of the feeding tube was successfully removed via enterotomy. The tube tip had coiled around itself and was encased with food materials, creating a large bezoar that was being pulled distally by peristalsis. The patient subsequently underwent insertion of a new GJ tube for ongoing administration of LCIG and has been doing well since. Conclusions Bezoar formation at the jejunal tip of LCIG PEG-J tubes is a rare complication and can lead to distal migration and traction related gastroduodenal ulceration. Surgical removal may be required. Funding Agencies None

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Rapunzel Syndrome in a Seven-Year-Old Girl

Indian Journal of Surgery ◽

10.1007/s12262-021-02788-0 ◽

2021 ◽

Author(s):

Aviral Gupta ◽

Sarvesh C. Mishra ◽

Vijay D. Upadhyay ◽

Pujana Kanneganti

Keyword(s):

Abdominal Pain ◽

Gold Standard ◽

Gastrointestinal Endoscopy ◽

Case Reports ◽

Abdominal Mass ◽

Upper Gastrointestinal Endoscopy ◽

Surgical Removal ◽

Rare Entity ◽

Rapunzel Syndrome ◽

Upper Gastrointestinal

AbstractRapunzel syndrome is a rare entity with less than hundred case reports cited in the literature. In this, there is presence of a trichobezoar in the stomach which extends into the small intestine or beyond. It can typically cause abdominal pain and nausea, but can also present as an asymptomatic abdominal mass, progressing to abdominal obstruction and perforation. Many of these patients have associated psychiatric disorder. The gold standard for diagnosis is upper gastrointestinal endoscopy and treatment is surgical removal. Herein, we present surgical images of Rapunzel syndrome in a seven-year-old girl.

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Psychological factors addressed in cognitive behaviour therapy for paediatric functional abdominal pain: Which are most important to target?

Journal of Health Psychology ◽

10.1177/1359105317694488 ◽

2017 ◽

Vol 24 (9) ◽

pp. 1282-1292 ◽

Cited By ~ 4

Author(s):

Shelley MC van der Veek ◽

Else de Haan ◽

HHF Derkx ◽

Marc A Benninga ◽

Frits Boer

Keyword(s):

Abdominal Pain ◽

Psychological Functioning ◽

Cognitive Behaviour Therapy ◽

Behaviour Therapy ◽

Child Functioning ◽

Functional Abdominal Pain ◽

Negative Cognitions ◽

Passive Coping ◽

Cognitive Behaviour ◽

Positive Cognitions

The effectiveness of cognitive behaviour therapy for paediatric functional abdominal pain leaves room for improvement. We studied which factors addressed in cognitive behaviour therapy relate most strongly to the physical and psychological functioning of children with functional abdominal pain and are thus most important to target. Questionnaires were filled out by 117 children with functional abdominal pain and their parents. Multiple regression analyses showed that children’s passive coping and parental and children’s positive cognitions relate to child functioning. Negative cognitions and parental solicitous behaviour were unrelated to child functioning. Cognitive behaviour therapy for functional abdominal pain may benefit most from changing children’s passive coping and promoting positive cognitions.

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Eosinophilic Esophagitis in Brazilian Pediatric Patients

Clinical Medicine Insights Pediatrics ◽

10.4137/cmped.s12733 ◽

2013 ◽

Vol 7 ◽

pp. CMPed.S12733 ◽

Cited By ~ 8

Author(s):

Mayra Isabel Correia Pinheiro ◽

Luciano Pamplona De Góes Cavalcanti ◽

Rodrigo Schuler Honório ◽

Luïs Hélder De Alencar Moreno ◽

Mayara Carvalho Fortes ◽

...

Keyword(s):

Abdominal Pain ◽

Topical Corticosteroid ◽

Eosinophilic Esophagitis ◽

Pediatric Patients ◽

Erosive Esophagitis ◽

Normal Mucosa ◽

Patient Treatment ◽

Therapeutic Approaches ◽

Endoscopic Findings ◽

The Mean

We examined 11 pediatric patients with eosinophilic esophagitis with a tardy diagnosis. The symptoms were initially thought to be related to other diseases, leading to the use of inadequate therapeutic approaches. The patients were between 3 and 17 years old (mean 7.8 ± 3.8 years), and 8 of the patients were male. Common symptoms included abdominal pain, regurgitation, difficulty in gaining weight, vomiting, dysphagia, and coughing. The mean age for the onset of symptoms was 4.3 ± 2.9 years. Endoscopic findings included normal mucosa in five (45%) patients, thickening of the mucosa with longitudinal grooves in three (27%), erosive esophagitis in two (18%), and a whitish stippling in one (9%) patient. Treatment included the use of a topical corticosteroid for 10 patients. In eight (73%) cases, the treatment made the symptoms disappear. Ten patients underwent histopathological management after treatment, with a decrease in the number of eosinophils.

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Bouveret syndrome: a rare cause of gastric outlet obstruction

BMJ Case Reports ◽

10.1136/bcr-2020-240236 ◽

2021 ◽

Vol 14 (4) ◽

pp. e240236

Author(s):

Christopher Smith ◽

Shailendra Singh ◽

Paul Vulliamy ◽

Samrat Mukherjee

Keyword(s):

Abdominal Pain ◽

Gastric Outlet Obstruction ◽

Gallstone Disease ◽

Outlet Obstruction ◽

Chronic Cholecystitis ◽

Fistula Formation ◽

Bladder Drainage ◽

Blood Tests ◽

History Of ◽

Bouveret Syndrome

Bouveret syndrome is a rare cause of gastric outlet obstruction. It is characterised by the presence of an obstructing gallstone in the pylorus or proximal duodenum, which has travelled to its obstructing position via an acquired fistula. Our case involves a 73-year-old man presenting to the acute surgical take with a 2-day history of right-sided abdominal pain and vomiting. His medical history included perforated cholecystitis treated with antibiotics and percutaneous gall bladder drainage, 1 year earlier. Examination and blood tests were suggestive of gastric outlet obstruction. CT abdomen and pelvis demonstrated a large gallstone obstructing the duodenum, confirming a diagnosis of Bouveret syndrome. The patient improved following gastrolithotomy, and was discharged 2 weeks postoperatively. Fistula formation is a complication of chronic cholecystitis and therefore Bouveret syndrome should be considered in patients with a background of gallstone disease presenting with gastric outlet obstruction.

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LINGUAL BRAKE ANALYSIS AND EVALUATION IN CHILDREN IN THE AMAZON FACULTY - IAES

10.36106/0340386 ◽

2021 ◽

pp. 10-11

Author(s):

Darlemcris da Fonseca Vieira ◽

Anselmo Junio Pedroso Matos

Keyword(s):

Statistical Data ◽

Complete Removal ◽

Pediatric Dentistry ◽

Surgical Removal ◽

Cross Sectional ◽

Analysis And Evaluation ◽

Speech Therapist ◽

Data Program ◽

Adipose Cells ◽

Important Structure

The lingual brake is an important structure for the musculoskeletal growth of the baby's jaws, inuencing the act of sucking, speaking and feeding. There is an alteration called ankyloglossia (Ankylos: Linked and Glossa: Tongue), dened as a congenital malformation that alters mobility and lingual function. Brake change occurs when a portion of the tissue that should have suffered apoptosis during embryonic development remains on the underside of the tongue, restricting its movement. The lingual brake is formed by a connective tissue rich in elastic bers, lined with nonkeratinized stratied paved epithelium, containing adipose cells, muscle bers and blood vessels. Any anatomical or functional brake deciency can have an impact on functions based on their severity. Its diagnosis is not difcult, as it is very visible and can be done through the "Tongue Test". Surgical removal of the brake is necessary when it causes gingival retraction, diastema, hindering orthodontic, speech therapist, prosthetic and aesthetic treatment. It is advisable to wait for the individual's growth process, especially between 6 months and 6 years of life. The treatment performed for this anomaly is the frenectomy, which consists in the complete removal of the brake insert. The objective of the present study was to conduct a research that evaluated the lingual brake of children, and the need for surgical treatment at the pediatric dentistry clinic at Amazon Faculty - IAES through a questionnaire. It was a cross-sectional, descriptive and observational study, which evaluated the lingual brake of children. Data were analyzed quantitatively and qualitatively using the statistical data program. The software used for data analysis was Epi Info version 7.2.2.6 for windows. It was concluded that of the 50 lingual brakes evaluated, 14% presented alteration, with a preference for females. It was clear the need for an early diagnosis, besides the need for a multidisciplinary team

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Rapunzel syndrome causing partial gastric outlet obstruction requiring emergency laparotomy

BMJ Case Reports ◽

10.1136/bcr-2019-232904 ◽

2020 ◽

Vol 13 (1) ◽

pp. e232904

Author(s):

Robert Lyons ◽

Granit Ismaili ◽

Michael Devine ◽

Haroon Malik

Keyword(s):

Ct Scan ◽

Gastric Outlet Obstruction ◽

Epigastric Pain ◽

Outlet Obstruction ◽

Postoperative Recovery ◽

Emergency Laparotomy ◽

Rapunzel Syndrome ◽

History Of

A 16-year-old girl with a background of childhood trichophagia presented with a 2-day history of epigastric pain and associated anorexia with vomiting. An epigastric mass was palpable on examination. A CT scan revealed an intragastric trichobezoar, extending into the duodenum consistent with Rapunzel syndrome with evidence of partial gastric outlet obstruction and a possible perforation. The patient underwent an urgent laparotomy and extraction of the trichobezoar. The bezoar was removed without complication and no intraoperative evidence of perforation was detected. After an uncomplicated postoperative recovery, she was discharged home with psychiatric follow-up.

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Congenital band adhesion causing a proximal jejunal obstruction: an uncommon presentation and diagnosis

BMJ Case Reports ◽

10.1136/bcr-2019-229235 ◽

2019 ◽

Vol 12 (7) ◽

pp. e229235

Author(s):

David Ashley Cruise ◽

Kim Goddard

Keyword(s):

Abdominal Pain ◽

Acute Onset ◽

Greater Omentum ◽

Proximal Jejunum ◽

Complete Obstruction ◽

Uncommon Presentation ◽

Surgical History ◽

Crampy Abdominal Pain ◽

Contrast Swallow ◽

Jejunal Obstruction

A 20-year-old woman with no medical or surgical history presented with acute onset crampy abdominal pain on a background of uninvestigated similar chronic abdominal pain. She became obstructed during her admission and a contrast swallow showed a complete obstruction at the level of the proximal jejunum. A diagnostic laparoscopy revealed a congenital band adhesion from the greater omentum to the proximal jejunum to be the cause, and dissection of the band relieved her obstruction. This case presents a rare cause of mechanical obstruction, and highlights the seriousness of investigating obstructive symptoms even in atypical patient populations.

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Giant Hepatic Hemangioma Presenting as Gastric Outlet Obstruction

International Surgery ◽

10.9738/cc170.1 ◽

2013 ◽

Vol 98 (1) ◽

pp. 19-23 ◽

Cited By ~ 13

Author(s):

Cemalettin Aydin ◽

Sami Akbulut ◽

Koray Kutluturk ◽

Aysegul Kahraman ◽

Cuneyt Kayaalp ◽

...

Keyword(s):

Weight Loss ◽

Abdominal Pain ◽

Gastric Outlet Obstruction ◽

Benign Tumor ◽

Significant Proportion ◽

Outlet Obstruction ◽

Left Lobe ◽

Hepatic Hemangioma ◽

Giant Hepatic Hemangioma

Abstract Hemangioma, a most frequently encountered primary benign tumor of the liver, is generally determined incidentally during the course of radiologic tests for other reasons. Most lesions are less than 3 cm and a significant proportion of patients are asymptomatic, although the size and location of the lesion in some patients may be associated with the onset of symptoms. Pressure on the stomach and duodenum of giant hemagiomas developing in the left lobe of the liver, in particular, may result in the development of abdominal pain, nausea, vomiting, and feeling bloated, which are characteristic of a gastric outlet obstruction. A 42-year-old man presented with findings of gastric outlet obstruction and weight loss as a result of a giant hepatic hemangioma.

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