Rapunzel Syndrome is an uncommon presentation of trichobezoar, involving strands of swallowed hair extending as a tail through the small intestine, beyond the stomach. This was first described in 1968 and until 2003 only thirteen cases have been reported.
A 55-year old man of East Indian descent presented to the surgical team via the emergency department in Trinidad, West Indies, with an acute abdomen and small bowel obstruction. He had a recent psychiatric history and patchy alopecia as well as a family history of schizophrenia. A supine abdominal radiograph revealed a small bowel obstruction as well as an unusual air shadow in the left hypochondrium in the region of the duodeno-jejunal flexure associated with the stomach bubble, which resembled a 'comma'. At laparotomy, the stomach and third part of the duodenum were distended and contained a large mass of dark, foul-smelling hair that occupied the stomach, crossed the pylorus and extended into the small bowel. A retroperitoneal perforation of the third part of duodenum was found and repaired. The large trichobezoar was removed via a gastro-enterotomy and the patient made a slow, but complete recovery.
We wish to report another case of Rapunzel Syndrome and describe an unusual radiologic sign associated with a retroperitoneal perforation of the third part of duodenum – the comma sign. To the best of our knowledge this is the first reported case of a spontaneous retroperitoneal perforation of the third part of duodenum associated with Rapunzel Syndrome.
Rapunzel Syndrome is a rare trichobezoar, involving strands of swallowed hair extending as a tail through the duodenum, beyond the stomach. Trichobezoar usually occur in patients with history of trichotillomania, characterized by compulsive behavioral disorder of pulling own hairs, combined with trichophagia that consists of ingesting that hairs. It typically occurs in stomach and rarely affects the small intestine. Trichobezoars are more common in adolescent females. Common presentation is gastrointestinal tract obstruction with nausea and vomiting, gut perforation, acute pancreatic necrosis, obstructive jaundice, hypochromic anemia, vitamin B12 deficiency, weight loss, an abdominal mass, or other serious problems. Intestinal obstruction due to trichobezoar is extremely rare. We are here reporting a large trichobezoar in a 3-year-old male child who presented to the emergency department with thread protruding from mouth with no sign of hair loss on body.
Rapunzel; trichobezoar; trichotillomania
Bezoars are concretions of foreign materials that impair gastrointestinal motility or cause intestinal obstruction in the stomach, small intestine or bowel of humans or animals. There are many types of them such as phyto, lacto and trichobezoars. Although bezoars are not rare, multiple giant bezoars which totally fill the stomach lumen and have extension to the small intestine (Rapunzel syndrome) are very rare. This is a case report of a young girl who had a history of trichophagia and presented with partial gastric and intestinal obstructive signs. The patient was healthy, and her physical exam was almost normal and the only positive thing in her past medical history was trichophagia from several years ago. She had a big trapped bobble in her stomach and several air-fluid levels in abdominal radiograph and was investigated with endoscopy which confirmed the diagnosis of a huge gastric trichobezoar.
Bezoars; Gastric Outlet Obstruction
We report the unusual case of a 45-year-old woman who presented with multiple episodes of small bowel obstruction. Initial exploratory lap-roscopy did not reveal an etiology of the obstruction. Subsequent upper endoscopy identified a non-obstructing gastric trichobezoar which could not be removed endoscopically but was not thought to be responsible for the small bowel obstruction given its location. One week postoperatively, the patient experienced recurrence of small bowel obstruction. Repeat endoscopy disclosed that the trichobezoar was no longer located in the stomach and upon repeat laparotomy was extracted from the mid-jejunum. In the following 8 months, the patient had no further episodes of small bowel obstruction. Consequently, gastric bezoars should be included in the differential diagnosis of recurrent small bowel obstruction.
Bezoar is a tightly packed collection of undigested material that is unable to exit the stomach. Most bezoars are of indigestible organic matter such as hair-trichobezoars; or vegetable and fruit–phytobezoars; or a combination of both. Trichobezoars commonly occur in patients with psychiatric disturbances who chew and swallow their own hair. In very rare cases, the Rapunzel syndrome hair extends through the pylorus into the small bowel causing symptom and sign of partial or complete gastric outlet obstruction. A case report of trichobezoar in the stomach causing Rapunzel syndrome in a 12-year-old female is reported.
Rapunzel; syndrome; female; giant; trichobezoar
Trichobezoars are caused by hair ingestion. The usual presentation of a trichobezoar is with early satiety and malnutrition. Obstructive symptoms and manifestations of gastric outlet obstruction may occur. The diagnosis may be suspected in young females with malnutrition, who have a history of trichophagia.
We report a case of 12-year-old female admitted to the emergency room for abdominal pain. On physical examination, she was cachectic and an epigastric mass was palpated. An exploratory laparotomy was conducted. A giant trichobezoar was palpated in the stomach and was removed through an anterior gastrostomy.
There were no complications postoperatively and the patient was referred to a psychiatrist.
Rapunzel syndrome is a rare type of trichobezoar with an extension of the hair into the small bowel. Clinical presentation is deceptive and vague ranging from abdominal mass to gastrointestinal symptoms.
We present a 7 years old girl with Rapunzel syndrome, where the trichobezoar was not suspected at all especially with negative history of trichophagia. In majority of the cases the diagnosis was made very late in the history of the disease, at a stage where surgery is the only cure for this syndrome.
In the paediatric age group with a long history of gastrointestinal symptom, endoscopy is a diagnostic as well as a therapeutic modality and may reduce surgery in trichobezoars.
There is little information available concerning trichobezoars in the nonhuman primate literature.
We evaluated 118 cases of trichobezoar in baboons over a 29 year period at the Southwest National Primate Research Center.
The anatomic locations affected in decreasing order were the stomach, small intestine, cecum, esophagus, and colon. The most common clinical history was weight loss. The most frequent associated pathology included gastrointestinal inflammation and ulceration, emaciation, peritonitis, intussusception, pneumonia, and aspiration. Trichobezoars were the cause of death in 9 baboons and the reason for euthanasia in 12. Females were 2.14 times more likely than males to be affected. The greater the percentage of group housing time, the more likely the baboon was to develop trichobezoars.
The baboon may present a useful model to evaluate the etiology, genetic predisposition, physiopathology, neurobiology, and treatment response of trichobezoars.
Stomach; hairball; trichophagia; trichotillomania; hair pulling; nonhuman primate; Papio
A bezoar is an intraluminal mass formed by the accumulation of undigested material in the gastrointestinal tract. A trichobezoar is a bezoar made up of hair and is a rare cause of bowel obstruction of the proximal gastrointestinal tract. They are seen mostly in young women with trichotillomania and trichotillophagia and symptoms include epigastric pain, nausea, loss of appetite and bowel or gastric outlet obstruction. We herein describe a case of a trichobezoar that presented as a gastric outlet obstruction and was subsequently successfully removed via a laparotomy.
Obstruction; Bezoar; Trichobezoar; Trichotillomania
Trichobezoars (hair ball) are usually located in the stomach, but may extend through the pylorus into the duodenum and small bowel (Rapunzel syndrome). They are almost always associated with trichotillomania and trichophagia or other psychiatric disorders. In the literature several treatment options are proposed, including removal by conventional laparotomy, laparoscopy and endoscopy. We present our experience with four patients and provide a review of the recent literature. According to our experience and in line with the published results, conventional laparotomy is still the treatment of choice. In addition, psychiatric consultation is necessary to prevent relapses.
Trichobezoar; Trichotillomania; Rapunzel syndrome; Therapy
A rare case of 13 years old female child with recurrent trichobezoar stomach which needed reoperation for its removal is reported. Patient also had trichotillomania and mental retardation. She showed satisfactory response to therapy with fluoxetine.
Trichobezoar; trichotillomania; fluoxetine
Bezoars are concretions of undigested material in the gastrointestinal tract, most commonly in the stomach. Duodenojejunal localization of bezoars is exceptional. We report a case of a 27-year-old woman who experienced nausea, vomiting and severe abdominal pain for one week. By palpation a mobile and sensitive mass, 15 × 15 cm, was detected, which filled the upper quadrant. Results of gastric endoscopy were normal. X-ray and ultrasonography suggested a bezoar. A laparotomy revealed that the jejunum was fissured by the trichobezoar ball. This trichobezoar mass was totally excised by intestinal resection.
To our knowledge, this is the first reported case of duodenojejunal fissuration caused by trichobezoar in an adult. Among patients with high subocclusif syndrome, duodenojejunal bezoar should remain a possibility in differential diagnosis.
Duodenojejunal flexure; intestinal partial obstruction; surgery; trichobezoar
The Rapunzel syndrome is an unusual form of trichobezoar found in patients with a history of psychiatric disorders, trichotillomania (habit of hair pulling) and trichophagia (morbid habit of chewing the hair), consequently developing gastric bezoars. The principal symptoms are vomiting and epigastric pain. In this case report, we describe this syndrome in a young girl.
A Pakistani girl presented with acute abdominal pain and raised serum amylase and alkaline phosphatase concentrations. She was found to have a gastric trichobezoar with a tail extending to the mid-ileum. The altered biochemical parameters returned to normal after surgical removal of the bezoar. Irritation of ampulla of Vater by the bezoar tail is believed to have caused transient pancreatitis.
Trichobezoar consists of a compact mass of hair occupying the gastric cavity to a various extent. When the trichobezoar extends past the duodenum it is better referred to as Rapunzel Syndrome. Although trichobezoars are well described in terms of surgical diagnostic and procedure, there are only but very scarce reports on psychiatric literature, usually associated with trichotillomania. The authors present a clinical case of trichobezoar and discuss the most relevant aspects concerning this entity.
Presentation of psychiatric and surgical data concerning the case report. Previously reported cases are also mentioned.
Report of a 27-year-old female patient with a trichobezoar submitted to surgical removal, with a prior intervention 4 years before also due to trichobezoar, and with unknown psychiatric antecedents or follow-up.
A trichobezoar represents a serious surgical condition. It is important to consider such diagnosis in face of suggestive symptoms, even if signs of trichotillomania are not present. The discrepancies between the prevalence of trichotillomania and trichobezoars due to trichophagia may be due to issues related to self-selection of patients and symptom severity. Such issues may also be important in the study of impulsive-compulsive spectrum models and to their relevance to impulse control disorders.
Trichobezoar; Rapunzel Syndrome; Trichotillomania; Trichophagia.
Hypertrophic gastropathy (Ménétrièr's disease) is a clinical and pathological curiosity. It is often seen in association with a variety of other disease processes. The authors here present a previously undescribed association of Ménétrièr's disease with trichobezoar in the stomach and small intestine.
This report describes a laparoscopic approach for the removal of a large gastric bezoar.
Background and Objectives:
Gastric bezoars are a rare clinical entity, most commonly observed in patients with mental or emotional illness. Large bezoars can be difficult to remove laparoscopically without extending a port incision.
We report the case of a large symptomatic trichobezoar with Rapunzel syndrome that occurred in a 17-year-old girl who had trichotillomania.
The bezoar was removed laparoscopically, in piecemeal fashion, through a gastrotomy port. This procedure did not require an extension of any incision, nor did it require the contents of the stomach to directly touch the incision, thereby reducing the risk of infection. The patient was discharged home, on the fourth postoperative day, free of any complications.
This case illustrates the safety of the laparoscopic approach in the removal of large gastric bezoars. In considering use of this approach, the potentially long operative time must be weighed against the benefits of both minimal risk of infection and minimal incisions.
Trichobezoar; Gastric outlet obstruction; Rapunzel syndrome; Trichotillomania
Laparoscopic removal of a large gastric trichobezoar is technically feasible through small incisions without requiring port-site extension or limited midline laparotomy.
Standard treatment of large gastric bezoars not amenable to medical or endoscopic management is surgical removal. The optimal operative approach, laparotomy versus laparoscopy, is a contested subject. Though laparoscopic removal has been described, it remains a relatively new technique for surgical management with outcome literature limited to case reports. In addition, currently described laparoscopic techniques often involve limited midline laparotomy incisions or >3 cm extensions of port sites.
The following describes the case of a 4-year-old girl with a large gastric trichobezoar.
The gastric trichobezoar was successfully removed through a 12-mm left lower quadrant trocar incision cosmetically hidden within a skin crease.
This case, along with accumulating literature, supports the use of laparoscopy to treat large gastric bezoars.
Bezoar; Trichobezoar; Laparoscopy
Primary small bowel bezoars are rare and cause acute abdomen due to small bowel obstruction (SBO). A 69-year-old Japanese man presented with epigastric pain associated with fullness. Physical examination of the abdomen showed no marked signs of peritoneal irritation. An erect X-ray film of the abdomen showed small bowel obstruction. Computed tomography (CT) showed a dilated small bowel loop proximal to the site of the obstruction. Retrograde double balloon enteroscopy (DBE) was performed and showed yellow, hard bezoars blocking the distal ileum. At surgery, a bezoar was found impacted in the distal ileum, and enterotomy with extraction was performed. After 9 days, the patient was discharged from our hospital in satisfactory condition. DBE also appears to be a safe and useful diagnostic tool in patients with SBO, and the findings of DBE influence the strategy of therapy in patients in whom the cause of SBO could not be determined by conventional radiography.
Trichobezoar which were traditionally managed by open surgical retrieval are now often managed by minimally invasive surgical approach. Removal of a large trichobezoar by laparoscopy, however, needs an incision (usually 4-5 cm in size) for specimen removal and has the risk of intra-peritoneal spillage of hair and inspissated secretions.
MATERIALS AND METHODS:
The present paper describes a modified laparoscopy-assisted technique with temporary gastrocutaneopexy for the effective removal of a large trichobezoar using a camera port and a 4-5 cm incision (which is similar to that needed for specimen removal during laparoscopy).
Three patients with large trichobezoar were managed with the described technique. The average duration of surgery was 45 (30-60) min and the intraoperative blood loss was minimal. There was no peritoneal spillage and the trichobezoar could be retrieved through a 4-5 cm incision in all patients. All had an uneventful recovery and at a median followup of 6 months had excellent cosmetic and functional results.
The described technique is a minimally invasive alternative for trichobezoar removal. There is no risk of peritoneal contamination and the technical ease and short operative time in addition to an incision limited to size required for the specimen removal, makes it an attractive option.
Limited incision; trichobezoar; bezoar; laparoscopy; trichotillomania; rapunzel syndrome; gastric; hair ball
A 9-year-old girl presented with a chief complaint of abdominal pain. Esophagogastroduodenal endoscopy (EGD) identified a long and large gastric trichobezoar extending into the duodenum. We attempted endoscopic retrieval after informed consent was obtained from the patient’s mother. Initially, a gasper with 5-prolongs, commonly used for retrieval of endoscopically excised polyps, failed to remove the whole trichobezoar. When a net was used instead, it proved impossible to remove the trichobezoar completely. Therefore, we withdrew the scope from the mouth, leaving the net grasping the tricobezoar firmly in the stomach. Subsequently, we were able to retrieve about 70% of the trichobezoar manually by grasping the snare part of the net directly. A second pass found no deep laceration or perforation endoscopically. The remaining trichobezoar was completely retrieved with the net. The procedure was completed within 15 min. The retrieved specimens were 34 cm in length and 100 g in weight. The patient was discharged uneventfully 5 d thereafter. She was advised to visit a psychiatrist to avoid suffering from a relapse. Follow-up EGD showed no trichobezoar, and the patient’s frontal hair grew back.
Gastric bezoar; Trichobezoar; Endoscopic retrieval; Grasper; Retrieval net
Bezoars usually present as a mass in the stomach. The patient often has a preceding history of some psychiatric predisposition. Presentation could be in the form of trichophagy followed by trichobezoar (swallowing of hair leading to formation of bezoar), orphytobezoar (swallowing of vegetable fibres). Rapunzel syndrome is a condition where the parent bezoar is in the stomach and a tail of the fibres or hair extends in to the jejunum. Presentation as intestinal obstruction due to a bezoar in the intestine without a parent bezoar in the stomach is rare, therefore we report it here.
A 35 year old lady tailor with a previous history of receiving treatment for depression on account of being infertile- years after her marriage, presented to the surgical emergency department with features of acute intestinal obstruction. Exploratory laparotomy and enterotomy revealed a cotton bezoar in the terminal ileum without a parent bezoar in the stomach. She was managed by resection of the affected segment of the ileum and end-to-end anastomosis of the bowel. In the postoperative period the patient gave a history of ingesting cotton threads whenever she was depressed.
Presence of cotton bezoar is rare and an intestinal bezoar in the absence of parent bezoar in the stomach is still rarer.
cotton bezoar; ileum
Intersigmoid hernia is a rare internal hernia presenting with symptoms of bowel obstruction. Preoperative diagnosis is uncommon but computerised tomography (CT) may show signs to suggest internal hernia.
PRESENTATION OF CASE
A 63-year-old female presented with abdominal pain, vomiting and absolute constipation. Examination revealed a tense distended abdomen. A plain abdominal radiograph showed features of small bowel obstruction. Conservative management was initiated without success and a CT scan was performed which showed a dilated distal oesophagus, stomach and small bowel with a non-dilated length of distal ileum and large bowel. Internal hernia was suggested as a possible cause and the patient underwent a laparotomy where a loop of small bowel was found to be strangulated and gangrenous within the intersigmoid fossa. The gangrenous bowel was resected, an end-to-end anastamosis was performed and the fossa was closed. The patient made an uneventful recovery.
Hernias of the sigmoid mesocolon account for 6% of internal hernias with internal hernias themselves causing between 0.2 and 4.1% of intestinal obstruction. This report presents a case of intersigmoid hernia, a rare internal hernia which should be suspected in patients presenting with acute obstruction, no past surgical history and no external hernia. Patients with these symptoms should receive an urgent CT scan to facilitate early surgery and minimise strangulation and prevent bowel resection.
Intersigmoid hernia presents with acute obstruction, no past surgical history and no external hernia. Urgent CT scanning and early surgery may minimise strangulation, conserve bowel and reduce patient morbidity and mortality.
Intersigmoid hernia; Intersigmoid fossa; Sigmoid mesocolon hernia; Internal hernia; Intestinal obstruction
Gallstone ileus, an uncommon complication of cholelithiasis, is described as a mechanical intestinal obstruction due to impaction of one or more large gallstones within the gastrointestinal tract. The clinical presentation is variable, depending on the site of obstruction, manifested as acute, intermittent or chronic episodes. A 51-year-old female patient was referred to our hospital with 3 events of intestinal obstruction during the previous 7 d. At admission, there were clinical signs of intestinal obstruction; abdominal film demonstrated dilated bowel loops, air-fluid levels and a vague image of a stone in the inferior left quadrant. Once stabilized, a laparotomy was performed. Surgical findings were distention of the jejunum and ileum proximal to a palpable stone in the ileum as well as gallstones and a cholecystoduodenal fistula in the gallbladder. An enterolithotomy, repair of the cholecystoduodenal fistula and cholecystectomy were performed. The postoperative course was uneventful. There is no uniform surgical procedure for this disease. When the patient is too ill or when biliary surgery is not advisable, an enterolithotomy is the best option. The one-stage procedure should be the offered to adequately stabilized patients when local and general conditions, such as good cardiorespiratory and metabolic reserve permit a more prolonged surgical procedure.
Gallstone ileus; Cholecystoduodenal fistula; Intestinal obstruction
Obturator hernia is an extremely rare type of hernia with relatively high mortality and morbidity. Its early diagnosis is challenging since the signs and symptoms are non specific.
PRESENTATION OF CASE
Here in we present a case of 70 years old women who presented with complaints of intermittent colicky abdominal pain and vomiting. Plain radiograph of abdomen showed acute dilatation of stomach. Ultrasonography showed small bowel obstruction at the mid ileal level with evidence of coiled loops of ileum in pelvis. On exploration, Right Obstructed Obturator hernia was found. The obstructed Intestine was reduced and resected and the obturator foramen was closed with simple sutures. Postoperative period was uneventful.
Obturator hernia is a rare pelvic hernia and poses a diagnostic challenge. Obturator hernia occurs when there is protrusion of intra-abdominal contents through the obturator foramen in the pelvis. The signs and symptoms are non specific and generally the diagnosis is made during exploration for the intestinal obstruction, one of the four cardinal features. Others are pain on the medial aspect of thigh called as Howship Rombergs sign, repeated attacks of Intestinal Obstruction and palpable mass on the medial aspect of thigh.
Obturator hernia is a rare but significant cause of intestinal obstruction especially in emaciated elderly woman and a diagnostic challenge for the Doctors. CT scan is valuable to establish preoperative diagnosis. Surgery either open or laproscopic, is the only treatment. The need for the awareness is stressed and CT scan can be helpful.
Obturator hernia; Intestinal obstruction; Computed Tomography scan; Laproscopy