Rapunzel syndrome and gastric Peutz Jeghers (hamartomatous) polyps: case report

Rapunzel syndrome is an extremely rare complication of a trichobezoar. These females have a history of trichophagia and trichotillomania. Peutz Jeghers syndrome (PJS) is an uncommon autosomal dominant syndrome with a variable to high penetrance that leads to the development of the polyps within the gastrointestinal mucosa. This case report of a 25-year-old deaf and dumb female presented with pain and lump in upper abdomen, vomiting, nausea, loss of appetite, loss of weight. An exploratory laparotomy with anterior gastrotomy was performed and a giant trichobezoar with tail extending into the duodenum was removed. There were multiple polyps in the lower stomach excised. Histopathology reports showed Peutz Jeghers (hamartomatous polyps). She recovered well and was discharged on the 10th day. Patient was advised regular follow up.

Intestinal intussusception due to “Rapunzel syndrome” in an adult

The bezoars are collections of foreign bodies or ingested material that accumulate over time and are often located in the stomach or small intestine,but can be found throughout the digestive tract. Most bezoars are of indigestible organic matter such as trichobezoars (hair); or vegetable and fruit- phytobezoars; or a combination of both but other rare substances have also been described in literature. In some cases, the trichobezoar extends through the pylorus into duodenum, jejunum or even into the colon. Rapunzel syndrome is a rare manifestation of a trichobezoar, which occurs when strands of swallowed hair extend beyond the pylorus of the stomach, into the intestine as a tail. It was first described by Vaughan et al in 1968. Trichobezoars can block the intestinal tract and, if left untreated, can be a life-threatening emergency requiring surgery. Patients are often women, under the age of 20 and have insidious non-specific abdominal symptoms and staggered presentations over months to years. The diagnosis of this type of pathology is usually a challenge for the attending physician, so we present a case report of a 38-year-old female with gastric-intestinal occlusion secondary to trichobezoar.

Síndrome de Rapunzel: hallazgos y sus complicaciones

Se reporta el caso de una niña de 10 años que acude al cuarto de urgencias con historia de tricofagia de 3 años de evolución, dolor abdominal y datos de abdomen agudo al examen físico. En la radiografía de abdomen se reconoce la cámara gástrica distendida con un patrón de gas moteado intragástrico, delineado por gas y en la tomografía de abdomen y pelvis, se visualiza masa intragástrica bien circunscrita heterogénea en patrón de gas moteado y datos de pancreatitis necrotizante. Se requirió manejo quirúrgico encontrándose tricobezoar que se extendía hasta la segunda porción del duodeno. (provisto por Infomedic International)

We report the case of a 10-year-old girl who came to the emergency room with a history of trichophagia of 3 years of evolution, abdominal pain and data of acute abdomen on physical examination. The abdominal X-ray showed a distended gastric chamber with an intragastric speckled gas pattern, delineated by gas, and the CT scan of the abdomen and pelvis showed a well-circumscribed heterogeneous intragastric mass with a speckled gas pattern and evidence of necrotizing pancreatitis. Surgical management was required and trichobezoar was found extending to the second portion of the duodenum. (provided by Infomedic International)

El largo camino de la tricotilomania al síndrome de Rapunzel / The Long Road from Trichotillomania to Rapunzel Syndrome

Introducción: La tricotilomania, la tricofagia y el tricobezoar son afecciones entrelazadas e interconectadas. Estas deben tenerse en cuenta especialmente en féminas jóvenes que a menudo tienen una enfermedad psiquiátrica o trastorno social subyacente y es posible que la historia sea difícil de obtener. Objetivo: Presentar un caso portador de tricobezoar gástrico, por su baja incidencia en nuestro medio y a nivel mundial. Caso clínico: Paciente femenina de 12 años de edad, con antecedentes de retraso psicomotor, sin seguimiento o tratamiento previo. Acudió a consulta traída por su madre que aduce aumento de volumen en hemiabdomen superior de 5 años de evolución con pérdida de peso insidiosa. Los estudios radiológicos revelaron un tricobezoar gástrico que se corrobora, asociado a un síndrome de Rapunzel, en la laparotomía exploratoria. La paciente evolucionó satisfactoriamente y es seguida por grupo multidisciplinario. Conclusiones: Los bezoares gástricos son entidades poco frecuentes. Para el diagnóstico es necesario un alto índice de sospecha de conjunto con estudios radiográficos y/o endoscópicos. El tratamiento estará determinado por el tipo de bezoar, tamaño y consistencia. La opción quirúrgica es la más usada, con el consecuente seguimiento interdisciplinario para evitar recurrencias(AU)

Introduction: Trichotillomania, trichophagia and trichobezoar are intertwined and interconnected conditions. These should be considered, especially in young women who often have an underlying psychiatric illness or social disorder and possibly a history difficult to get. Objective: To report a case of gastric trichobezoar due to its low incidence in our environment and worldwide. Clinical case report: We report the case of a 12-year-old female patient with a history of psychomotor retardation, with no prior follow-up or treatment, who came to consult with her mother, complaining of increased volume in the upper abdomen, of 5 years of evolution with malignant weight loss. Radiological studies reveal a gastric trichobezoar that is corroborated, associated with Rapunzel syndrome, in the exploratory laparotomy. The patient evolved satisfactorily and a multidisciplinary group assisted her. Conclusions: Gastric bezoars are rare entities. Diagnosis requires high level of suspicion together with radiographic and/or endoscopic studies. The treatment will be determined by the type of bezoar, size and consistency. The surgical option is the most used, requiring interdisciplinary follow-up to avoid recurrences(AU)

Tricobezoar: reporte de caso / Case report: Trichobezoar

Reportamos el caso de una adolescente femenina que ingresa al Hospital Mario Catarino Rivas en San Pe- dro Sula con antecedente de tricofagia desde hace 5 años. Ingresa con historia de dolor abdominal epigás- trico de 3 meses de evolución con náuseas y vómitos de alimentos ocasionales junto a pérdida de aproxima- damente 6 kilogramos de peso en los últimos 3 meses. Paciente fue ingresada y se completaron estudios diagnosticándose tricobezoar gástrico con poste- rior intervención quirúrgica: laparotomía media supraumbilical accediendo al estómago sobre el cual se le realizó una gastrostomía antral de 6 cm de longitud por donde se extrae el bezoar en su to- talidad. Se cierra posteriormente la gastrotomía.

Síndrome de Rapunzel como complicación en una paciente lúpica / Rapunzel syndrome as a complication in a lupus patient

El lupus eritematoso sistémico es una enfermedad que causa un gran número de complicaciones de origen psicológico; estas pueden llevar a patrones inadecuados de conducta que pueden poner en riesgo la vida de los pacientes como el síndrome de Rapunzel. Se describen los elementos clínicos y diagnósticos de este síndrome mediante el caso de una paciente femenina de 25 años de edad, con diagnóstico de lupus eritematoso sistémico. Acude a consulta con manifestaciones clínicas e imagenológicas que permiten llegar al diagnóstico de síndrome de Rapunzel como complicación psicológica en el curso del lupus eritematoso sistémico. Se procede a realizar la intervención quirúrgica donde se halló una masa tumoral constituida por cabello humano. En la actualidad, la paciente se mantiene en seguimiento por las especialidades de reumatología, psiquiatría y psicología, y su recuperación y evolución han sido satisfactorias. El lupus eritematoso condiciona conductas que pueden ser perjudiciales para la salud de los pacientes. Es importante la identificación precoz de conductas como la tricotilomanía y el seguimiento psicológico especializado en pacientes con esta enfermedad(AU)

Systemic lupus erythematosus is a systemic disease that generates a large number of complications of psychological origin; these can lead to inappropriate behavior patterns that can be life-threatening for patients. To describe the clinical and diagnostic elements of Rapunzael syndrome. Clinical case: a 25-year-old female patient with a diagnosis of systemic lupus erythematosus who consulted with clinical and imaging manifestations that led to the diagnosis of Rapunzel syndrome as a psychological complication in the course of systemic lupus erythematosus. Lupus erythematosus conditions a series of behaviors that can be detrimental to the health of patients; early identification of behaviors such as trichotillomania and specialized monitoring in psychology in patients with this disease is important. The clinical evolution of the patient has been very favorable and her general condition has recovered(AU)

Síndrome de Rapunzel: mito o realidad / Rapunzel Syndrome: Myth or Reality

Resumen Se presenta el caso de una paciente de 35 años, quien ingresa al servicio de urgencias de un hospital de tercer nivel, por causa de un cuadro de 6 meses de saciedad precoz, pérdida de peso y, en el momento del examen físico de ingreso, se observan signos de desnutrición. En consecuencia, se realiza una endoscopia de las vías digestivas altas en las que se descubre un tricobezoar que ocupa toda la cámara gástrica y se extiende más allá de la tercera porción del duodeno. Mediante una tomografía axial computarizada (TAC) de abdomen, se confirma la presencia de una lesión que ocupa la cámara gástrica y el duodeno, por lo que la paciente es conducida a cirugía. Allí se encuentra el tricobezoar que confirma el diagnóstico de síndrome de Rapunzel. Luego de la cirugía, la paciente presenta una adecuada evolución posoperatoria, con resolución de los síntomas de ingreso. Asimismo, se inicia una intervención por nutrición y psicología, a fin de manejar la desnutrición y el trastorno psiquiátrico de base.

Abstract A 35-year-old patient was admitted to the emergency department of a third-level hospital following six months of early satiety and weight loss. Upon physical admission, the patient had signs of malnutrition. Upper digestive tract endoscopy found a trichobezoar that occupied the entire gastric chamber and extended into the third portion of the duodenum. This diagnosis was confirmed by an abdominal CT scan. Surgery confirmed the diagnosis of Rapunzel Syndrome. Following surgery the patient's evolution was good and symptoms resolved. Nutritional and psychological interventions were initiated to manage malnutrition and an underlying psychiatric disorder.

Trichobezoars: a hairy cause of intestinal obstruction

Trichobezoars are an infrequent form of bezoar found in the stomach or intestine, created from ingested hair. Their most frequent location is in the stomach but they may extend through the pylorus into the small bowel. This condition is known as Rapunzel syndrome. Trichobezoar with Rapunzel syndrome is an uncommon diagnosis in children. Authors report 3 cases of Rapunzel syndrome with a large bezoar in paediatric age group that presented with features of acute intestinal obstruction. They were managed with surgical exploration.

Gastric trichobezoar and Rapunzel syndrome: a case report

Giant gastric trichobezoars are unusual form of foreign body found in stomach of mostly young adolescent females which may lead to morbidity and high mortality 30%, if goes unnoticed. These females have history of trichophagia or trichotillomania. This report is of a 12- year old young adolescent female presented with epigastric pain and mass. An exploratory laparotomy with anterior gastrotomy was performed and a giant trichobezoar with a very large tail of 2.5 ft was removed, weighing 2.52 kg. She recovered well and was discharged on 7th post-operative day. Patient was advised for psychiatric follow up.

Tricobezoar: presentación de caso / Trichobezoar: presentation of case

Los tricobezoares pertenecen a la clasificación de los bezoares, tumores abdominales formados por el acumulo de diversas sustancias que pueden ocasionar la oclusión intestinal, se presenta un paciente, femenina, que acuda al médico por presentar dolor abdominal y vómitos, en el examen físico abdominal se palpa una masa de aproximadamente 5 cm en el epigastrio que aparece y desaparece con los movimientos intestinales, se plantea inicialmente la posibilidad de padecer de una neoplasia abdominal, se realiza una endoscopia en donde informe que, en el fondo gástrico, cuerpo y antro está ocupado por cabello y restos alimenticios mal digeridos, la cual es removida mediante una laparotomía sobre el área que ocupaba la tumoración en el intestino delgado extrayendo un tricobezoar de 35 cm de longitud por 10 de ancho(AU)

The trichobezoars belong to the classification of bezoars, abdominal tumors formed by the accumulation of various substances that can cause intestinal occlusion, a patient, female, who comes to the doctor due to abdominal pain and vomiting, in the abdominal physical examination is presented palpa a mass of approximately 5 cm in the epigastrium that appears and disappears with bowel movements, initially poses the possibility of suffering from an abdominal neoplasm, an endoscopy is performed where it reports that, in the gastric fundus, body and antrum is occupied by hair and badly digested food remains, which is removed by a laparotomy on the area occupied by the tumor in the small intestine, extracting a trichobezoar measuring 35 cm long by 10 cm wide(AU)

Síndrome de Rapunzel en una adolescente: causa de suboclusión intestinal / Rapunzel syndrome in teenager: Intestinal subocclusion cause

Resumen Objetivo: Presentar un caso de síndrome de Rapunzel. Esta es una enfermedad poco frecuente que consiste en una variante de tricobezoar gástrico que se extiende hasta la segunda porción del duodeno. Caso clínico: Paciente femenina adolescente con antecedentes de atresia tricuspídea tratada quirúrgicamente. Presentó dolor abdominal de 3 días de evolución en el hipocondrio izquierdo irradiado al hipocondrio derecho, con distensión abdominal, náuseas y vómitos. Al examen físico presentó un perímetro abdominal de 58 cm y ruidos hidroaéreos disminuidos. La laparotomía exploratoria con gastrostomía anterior mostró una masa de cabellos entrelazados de aproximadamente 40 cm de longitud, compatible con tricobezoar, que se extendió desde el fondo gástrico hasta la segunda porción, llamado síndrome de Rapunzel, provocando suboclusión intestinal. Conclusión: El diagnóstico temprano permite evitar complicaciones, que pueden ser graves. Se recomienda un manejo multidisciplinario con el servicio de psiquiatría para controlar la enfermedad de base.

Abstract Aim: Present a case of Rapunzel syndrome, this is a rare disease that is a variant of gastric tricobezoar which extends to the second portion of duodenum. Case report: Female patient with tricuspid atresia treated surgically. Abdominal pain three days of evolution on the left upper quadrant radiating to the right upper quadrant with abdominal distension, nausea and vomiting. Abdominal circumference was 58 cm, decreased bowel sounds. The exploratory laparotomy presenced of a mass formed by interlocking hair, approximately 40 cm long with a compatible tricobezoar that spread from the gastric fundus to the duodenum called Rapunzel síndrome and caused partial bowel obstruction. Conclusion: Early diagnosis can prevent complications, which can be severe, a multidisciplinary management is recommended in psychiatry service to control the underlying disease.

Stomach trichobezoar (rapunzel syndrome) with iatrogenic intussusception

We present a rare case of stomach trichobezoar complicated with iatrogenic intussusception noted intra-operatively after failed attempt of endoscopic removal in a 13-year-old girl. At presentation, she had gastric outlet obstruction with anaemia. Endoscopy established the diagnosis of trichobezoar. Surgical removal was warranted after failed endoscopic removal. Her postoperative course was uneventful. Detailed history and careful examination disclosed trichotillomania and associated trichophagia. Psychiatric referral was sought with the intention to prevent future recurrence.

Rapunzel Syndrome: Trichobezoar in a 15-Year-Old Female Patient: A Case Report.

Rapunzel syndrome is a type of trichobezoar, a very rare to find in clinical settings, wherein the hairball tail extends in small bowel. Patient may present with varying signs ranging from vague abdominal pain to signs of intestinal obstruction. This case describes averagely built, averagely nourished young female of 15 years of age, no e/o anemia, jaundice, lymphadenopathy; per abdominal examination revealed an elliptical lump in epigastric region extending to umbilical region, lump was not freely mobile and non-tender. Early gastroscopy is indicated in patients with long history of vague abdominal pain, weight loss and hair loss in young females.

A rare case of paediatric trichotillomania complicating into Rapunzel syndrome: a case report

Background: Trichotillomania is a psychological condition that involves strong urge to pull one’s hair. Trichophagia is disorder of eating of hair. Bezoar is collection of undigested material such as hair, drugs and plastic or combinations. Rapunzel syndrome is a rare type of trichobezoar, which has mass of hair lodged in stomach extending up to ileum. In majority, diagnosis is made very late, at a stage where surgery is the only option. Case description: A 9 year old girl was brought with complaints of loss of hair on scalp since 8 months, decreased appetite, and constipation, weight loss since 5 months and vomiting since 2 weeks. Patient had paediatric consultations without much benefit; patient was referred to dermatologist and paediatric surgeon. Ultrasound showed mass in abdomen. Patient was operated to find a mass extending from stomach to terminal ileum. Patient was referred to a Psychiatrist and diagnosis of Trichotillomania was made. Discussion: Trichobezoar is common in young females, often with an underlying psychiatric disorder, they present late due to the low index of suspicion. As this condition is a rarity in paediatric age group and very little progress to Rapunzel syndrome, this paper discusses about the systemic team management of the above individual. Conclusion: High index of suspicion is needed to diagnose this condition in paediatric age group.

A Case of Rapunzel Syndrome / 대한소아소화기영양학회지

Rapunzel syndrome refers to a very rare condition in which swallowed hair forms a gastric trichobezoar that has a long tail extending into the small bowel. We describe a case of Rapunzel syndrome in an 8-year-old girl who presented with abdominal mass, epigastric pain and vomiting. Abdominal computed tomography scan showed a markedly dilated stomach filled with coarse heterogeneous materials. Upper gastrointestinal endoscopy revealed a huge hairy ball with a tail extending through the pylorus. We performed a surgical laparotomy and successfully removed a huge trichobezoar with a long tail extending into the middle portion of jejunum. Psychiatric consultation with review showed her past history of trichotillomania and trichophagia 4 years ago. But her parents denied further psychiatric therapy and she was lost to the follow-up. Rapunzel syndrome should be included in the differential diagnosis in children with chronic abdominal pain and trichophagia.

Rapunzel Syndrome : A Rare Cause of Vomiting and Pain Abdomen.

Bezoar is an agglomeration of food or foreign material in intestinal tract. Contiguous extension of trichobezoar into the small bowel can lead to the Rapunzel syndrome. We present rare case of Rapunzel syndrome in 16 year old female who presented with recurrent vomiting.

Tricobezoar gástrico: caso clínico / Gastric trichobezoar: case report

Los Bezoares del tracto digestivo representan una patología poco frecuente y con presentaciones clínicas que dependen del tipo de bezoar. Esta patología ocurre en estómagos normales a causa de ingestión de sustancias u objetos que no atraviesan el píloro y como complicación secundaria a cirugía gástrica. El tricobezoar corresponde a un bezoar constituido por pelo; la sospecha clínica de esta entidad se establece en pacientes jóvenes habitualmente de sexo femenino que tienen masa epigástrica palpable y en algunas ocasiones baja de peso negando la ingestión de cabello. El diagnóstico se puede realizar con exámenes contrastados del tracto digestivo y mediante endoscopía, siendo este último el método de elección. El tratamiento puede ser endoscópico o quirúrgico y depende de las características del bezoar. Se presenta un caso clínico y se revisa la literatura relacionada con esta patología.

Digestive tract bezoars are a rare pathology with clinical presentations depending on the type of bezoar. This condition occurs in normal stomachs due to ingestion of substances or objects that do not pass through the pylorus as a secondary complication to gastric surgery. The tricobezoar corresponds to a bezoar composed of hair. The clinical suspicion of this entity is established in young patients usually female with palpable epigastric mass and sometimes weight loss denying the ingestion of hair. The diagnosis can be made through contrasting reviews of the digestive tract or through endoscopy, the latter being the method of choice. The treatment may be endoscopic or surgical depending on the characteristics of the bezoar. We present a case report and review of the literature regarding this disease.

Síndrome de Rapunzel: reporte de caso y revisión de la literatura / Rapunzel syndrome: a case report and literature review

Los tricobezoares son una condición poco común que usualmente ocurre en mujeres jóvenes con una historia de tricotilomanía y tricofagia. En la actualidad, la mayoría de los casos se presentan en pacientes con antecedentes de cirugía gástrica, o con alteraciones en la función pilórica; clínicamente pueden cursar asintomáticos durante meses o años o presentarse como una entidad aguda acompañada por sus complicaciones. En el presente artículo se presenta el caso clínico de una paciente embarazada a quien se le realizó diagnóstico de tricobezoar gastroduodenal - síndrome de Rapunzel (una variedad compleja de este bezoar que involucra estómago, duodeno e intestino); se presenta una revisión de la literatura al respecto.

The presence of trichobezoars is a rare condition which usually occurs in young women who have histories of trichotillomania and trichophagia. Nowadays, the majority of cases occur in patients with a history of gastric surgery, or pyloric function alteration. They may be clinically asymptomatic for months or years or may present acute symptoms accompanied by severe complications.This article presents the case of a pregnant patient who was diagnosed with a case of Rapunzel syndrome a is presented. This complex variety of gastroduodenal trichobezoar involves the stomach, duodenum and intestine. The article also reviews the literature about the Rapunzel syndrome.

Presentación de un caso pediátrico de tricobezoar gigante-Sìndrome de Rapunzel- / Submitted a pediatric case of giant trichobezoar Rapunzel Syndrome

La ingestión de sustancias no digeribles puede formar cuerpos extraños en el estómago denominados bezoares. Es una circunstancia poco frecuente generalmente vinculada a trastornos de conducta. Las manifestaciones principales son: desnutrición, anemia, palidez, síntomas digestivos vagos, dispepsia post-plandial y tumoración abdominal. Presentamos una niña de 13 años que consultó por edema facial y de miembros inferiores. Ingresó con signos de enfermedad crónica, palidez cutánea y de mucosas y cabellos quebradizos y signos de enfermedad crónica. Al examen se palpó en epigastrio una masa de consistencia duro-pétrea en forma de herradura, de aproximadamente 20 x 15cms.; se realizó radiografía simple de abdomen con insuflación gaseosa del estómago observándose una masa intragástrica. El diagnóstico de tricobazoar fue corroborado por endoscopía. El tratamiento fue la exéresis quirúrgica...

Tricobezoar gástrico y síndrome de Rapunzel en una adolescente / GastricTrichobezoar and Rapunzel syndrome in an adolescent

Se describe el caso clínico de una adolescente de 17 años de edad, con malas condiciones sociales, ingresada en el Hospital San Juan de Dios, municipio de Challapata (Bolivia), por presentar epigastralgia, náuseas y vómitos. Se realizó endoscopia digestiva superior y se confirmó un cuadro de oclusión intestinal alta por tricobezoar gástrico en forma de síndrome de Rapunzel, por lo cual fue operada de urgencia. La evolución posoperatoria resultó favorable y egresó a los 7 días, con recomendaciones de asistir a consultas externas de cirugía general y psicología

The case report of a 17 year-old adolescent , with poor social conditions, admitted at San Juan de Dios Hospital, Challapata municipality (Bolivia), for presenting epigastralgia, nauseas and vomits is described . An upper digestive endoscopy was carried out and a case of upper intestinal occlusion due to gastric tricobezoar simulating a Rapunzel syndrome was confirmed, reason why she had an emergent surgery. The postoperative clinical course was favorable and she was discharged after 7 days, with recommendations of attending the out patient department of general surgery and psychology