An unusual case report of rapunzel syndrome trichobezoar in a 3-year-old boy.

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.

INTRODUCTION

Infants and children, particularly if mentally disturbed or abnormal, may acquire the habit of swallowing foreign material which if it persists may lead to the formation of a bezoar in the gastrointestinal (GI) tract. This foreign material may be vegetable or any other substance. If it contains hair, it is known as a trichobezoar. Trichobezoar usually occurs in patients with history of trichotillomania, characterized by recurrent pulling out of one's hair resulting in noticeable hair loss, increasing sense of tension immediately preceding or when resisting hair pulling, and pleasure or relief when pulling out the hair. In addition, the symptoms of trichotillomania cannot be better accounted for by another psychiatric or medical disorder (e.g., schizophrenia, dermatitis) and must also cause clinically significant distress or impaired social or occupational functioning.[1] The site from which hair is most frequently pulled is the scalp, but hair may be pulled from eyelashes, eyebrows, the pubic region, or other parts of the body.[2] Trichobezoars make up to 55% of all bezoars, 90% occur in adolescent females, probably as a consequence of their long hair, though they may occur in both sexes. We, here, are reporting a case of large trichobezoar in a 3-year-old male child and such cases are rarely reported in literature.

CASE REPORT

A 3-year-old male child was referred to casualty department with a thread protruding from mouth. On examination, he was 14 kg in weight and 88 cm in height. No sign of malnutrition was apparent and there was no alopecia. Abdominal examination revealed a non-tender smooth abdominal mass in the left upper quadrant emerging from beneath the left costal margin and extending over the midline. Rigid esophagoscopy was done and a bunch of hair about 50 g was found in esophagus which was extended into stomach. Laparotomy was planned and was carried out through an upper midline incision. A large 10×4 cm J-shaped foul-smelling black bunch of hairs, about 200 g, was retrieved through a longitudinal anterior gastrotomy [Figures 1 and 2]. There was also a daughter bezoar of 6×4 cm in size and about 100 g in jejunum about 30 cm away from duodenojejunal junction which was retrieved through enterotomy. Tail of the daughter bezoar was seen to have gone through the duodenum to jejunum. Abdomen was closed and patient was shifted to post-anesthesia care unit and then to surgical ward. His postoperative recovery was good and uneventful. He was discharged on 10th postoperative day. Psychiatric consultation was advised and child was followed up for a period of 1 year. No recurrence occurred.

Figure 1

Bunch of hairs removed from patient (Weight 350 g)

Figure 2

Trichobezoar retrieved from stomach by gastrostomy with daughter trichobezoar (Weight 200 g)

DISCUSSION

Vaughan et al. in 1968 first described Rapunzel Syndrome, a rare presentation of trichobezoar, involving strands of swallowed hair extending as a tail through the duodenum, beyond the stomach.[3] Rapunzel syndrome was named after the maiden in the Grimm brothers’ fairy tale of 1812 whose long hair flowed out of her prison tower allowing her prince to rescue her.[45] The common presentation of trichobezoar is in a young female usually with an underlying psychiatric disorder, mainly trichophagia.[6] Ingested hair gets collected in the stomach and form a mass in the stomach and mostly these masses do not dislodge. Sometimes, these masses or part of them pass through the duodenum into the intestine and may cause ulceration, partial or total obstruction, intestinal perforation, and peritonitis. These patients may present with nausea, vomiting, anorexia, weight loss, vague abdominal pain, or constipation but most of them are asymptomatic.[78] Personal history, psychiatric disorders in family, previous bezoars as well as physical examination of a palpable mass, halitosis, and patchy hair loss aids in diagnosis. Presentation of trichobezoar is usually late, due to the low index of suspicion by the clinician. A palpable abdominal mass is present in 87.7%, abdominal pain in 70.2%, nausea and vomiting in 64.9%, weakness and weight loss in 38.1%, constipation or diarrhea in 32%, and hematemesis in 6.1%.[9] Abdominal mass is usually well defined and mobile in 90% cases and may be indentable (Lamerton's sign). Various imaging modalities help in detection of bezoars.[1011] On conventional radiography, a mass of opaque soft tissue is visible in a swollen stomach.[10-12] Edge of the bezoar may be seen as calcification.[1012] The ultrasonography has limited role in diagnosis, it shows a typical curvilinear trichobezoar with bright echogenic band but high echogenicity of hair and the presence of multiple acoustic interfaces created by trapped air and food limit the ultrasonography of the trichobezoar.[10-13]

Both contrast radiography and upper GI endoscopy are the diagnostic procedures of choice.[1013] The upper GI contrast radiography confirms the existence of the trichobezoar and also detect other complications such as gastric ulcers. Furthermore, the upper GI endoscopy is also used for retrieval of proximal minor trichobezoars.[10] The computed tomography is the most useful diagnostic tool because it reveals the localization of the bowel obstruction. A mottled gas pattern in the mass is reported characterizing the bezoar, and it is supposed to be air bubbles retained within the bezoar.[1314] Recently, magnetic resonance imaging (MRI) has been recommended for the small intestinal disease. MRI visualization of bezoars can be improved by breath holding with fast imaging techniques. MRI picture of bezoar is a mass in the small bowel containing mottled and confluent low signal intensities on both T1- and T2-weighted images.[14] In the early stages, endoscopic removal is not without risk of bowel perforation and should be resolved for small trichobezoars only. Other methods like endoscopic biopsy devices, chemical dissolution, mechanical fragmentation, and laser-ignited mini-explosive technique were used successfully for small bezoars.[15] Laparoscopic techniques are becoming popular and large bezoars can be milked into the caecum before removal. Open surgery is still the corner stone of large trichobezoar removal, especially if it has an extension into the bowel, which is often missed. Recurrence of Rapunzel Syndrome has been reported and it is mainly due to underlying emotional stress trigger which was not corrected.[1617] However, treatment and psychological support of the mental as well as physical disorder is important for prevention of its recurrence. Parental counseling is also a regular part of treatment to prevent recurrence. Pharmacotherapy of trichotillomania remain inconsistent, some patients seem to respond to fluoxetine or other serotonin reuptake inhibitors.[18] The patient's long-term prognosis is excellent if behavioral therapy is used to control trichophagia, and psychological/psychiatric follow-up is maintained.

CONCLUSION

Trichobezoars, especially Rapunzel syndrome, is very rarely encountered. The present case was diagnosed in a 3-year-old boy with no visible alopecia. Protruding thread from mouth as in present case may lead to high suspicion of bezoar and radiological investigations may clarify the diagnosis. After definitive surgical treatment, pediatric psychiatric consultation should be opted for any mental disorder and for prevention of further recurrence.