A Convoluted Tale of Rapunzel Tail: With Dislodged Daughter Bezoar as Lead Point for Intussusception

ABSTRACT

Rapunzel syndrome (RS) can have a varied clinical presentation, spanning from being asymptomatic to having a fatal outcome. If left untreated, complications are inevitable. This case highlights an amalgamation of varied presentations of RS with a daughter bezoar acting as a lead point for intussusception, its management, and favorable result. The purpose of this discussion is to throw the focus on the blend of diverse clinical findings and complications of RS with a daughter bezoar; and the importance of early surgical intervention followed by psychological support and psychiatric treatment, as a key to a successful outcome.

INTRODUCTION

Trichobezoars are composed of hair or hair-like fibers, and are invariably associated with psychiatric disorders, such as compulsive pulling of body hair (trichotillomania) with subsequent ingestion of pulled hair (trichophagia) and gastric dysmotility.[1] The term “Rapunzel syndrome” (RS) is used when hair extends beyond the stomach into the small bowel in the form of a tail.[2] It can have vivid clinical presentations which can range from being asymptomatic, or a subtle one like malnutrition, and can present as intestinal obstruction, intestinal perforations, peritonitis, etc.[3] We report a case of RS with a dislodged daughter trichobezoar, causing an ileoileal intussusception. To the best of our knowledge, such a presentation has not been reported so far.

CASE REPORT

A 5-year-old girl presented with complaints of nonprojectile, nonbilious vomiting, diffuse abdominal pain, loose stools, and low-grade fever for 10 days.

As per her mother, she was in the habit of pulling and consuming her hair and cloth fibers for the past 6–7 months, for which no psychiatric treatment was received.

The patient was conscious, developmentally normal for age, and pale but hemodynamically stable. An irregular patch of hair loss (alopecia) and a patch of irregular hair length were observed on the right side of her scalp. On palpation, a large epigastric mass (10 cm × 8 cm) was noted; which was smooth surfaced, firm in consistency.

Biochemical investigations revealed anemia and hypoalbuminemia. A plain radiograph of the abdomen showed a grossly distended stomach with mottled density. Contrast-enhanced computed tomography (CECT) abdomen, upper gastrointestinal (GI) endoscopy was suggestive of a large trichobezoar in the stomach.

On exploration, approximately 300 ml of turbid, foul-smelling fluid was drained from the peritoneal cavity. Gastric trichobezoar (B1) was noted with the tail of the bezoar palpable throughout the duodenum, well into the mid jejunum. Multiple perforations were noted from proximal to mid jejunum. Distal jejunum and proximal ileum were bezoar free. Furthermore, a daughter trichobezoar (B2) was noted in the distal ileum (approximately 40 cm proximal to the ileocecal junction, leading to an antegrade ileoileal intussusception) [Figure 1]. The daughter’s trichobezoar was protruding through the perforated site of intussusception [Figure 2a].

Figure 1.

Line diagram showing Rapunzel (B1) and daughter bezoar (B2) acting as a lead point for intussusception; and multiple perforations (red dots)

Figure 2.

Intraoperative photographs showing. (a) Daughter trichobezoar (lead point) protruding out through the perforation in intussusception (black arrow). (b) Excised Rapunzel specimen

Anterior gastrostomy was performed and trichobezoar was retrieved in toto [Figure 2b]. Unhealthy diseased jejunal and ileal segments (along with intussusception) were resected and bowel continuity was restored. The immediate postoperative period was uneventful. Psychiatric consultation was taken, and the patient was discharged on tablet fluoxetine for obsessive-compulsive disorder on the 10^th postoperative day. At present, the patient is doing well over a close follow-up of 2 years with no recurrence of trichotillomania, trichophagy, and abdominal complaints, and is attached to the psychiatry outpatient department.

DISCUSSION

The term “Bezoar” refers to the collection of hair or any other matter in GI tract. The word bezoar is derived from the Arabic word “badzehr.”[4]

It is most commonly found in children and adolescents, but all age groups can be affected.[1] This entity is more common in young females with psychiatric disorders.[1] However, only a minority of patients have severe psychiatric disorders.[1]

The most common site is the stomach as hair gets easily entrapped within the gastric folds and limited peristaltic movement and pyloric sphincter effect lead to their accumulation.[5] Over a period, indigestible hair gets matted with each other and takes the shape of the stomach leading to bezoar formation.[5]

Interestingly, it can extend as a tail into the small bowel known as RS.[1] It is named after the imprisoned German fairy tale protagonist, who used to let down her extremely long hair for the prince to climb up and rescue her.[1] It was first reported by Vaughan et al. in 1968.[6]

Daughter trichobezoars are formed when fragments of the bezoar become detached and migrate to the small bowel.[7]

The clinical presentation of trichobezoar is quite varied. Patients may present with a palpable mass in the abdomen (87.7%), abdominal pain (70.2%), nausea and vomiting (64.9%), weight loss (38.1%), malnutrition, anemia, and early satiety.[1]

These can amount to serious complications including gastric ulceration, bleeding, intussusception, small bowel obstruction, and perforation.[8]

Meticulous history taking and clinical examination are key to the diagnosis. Patches of alopecia along with abdominal complaints should raise an alarm in favor of trichobezoar. A plain abdominal radiograph is the first investigation and profuse amount of mottled material.[3] Ultrasonography has a limited role in diagnosis as hair is highly echogenic.[9] The upper GI contrast study and CECT scan may reveal a mass or filling defect.[9]

There have been sporadic case reports of trichobezoars complicated by intussusception and peritonitis.[1,3,10] In the index case, during exploration, along with gastric trichobezoar, the child was found to have a perforated distal ileoileal intussusception with the daughter trichobezoar as the lead point. Such a blend of complications; with a daughter bezoar amounting to intussusception has not been reported so far.

Exploratory laparotomy is considered the treatment of choice for trichobezoars with a high success rate.[9]

In RS, the patient may need to undergo gastrostomy and/or enterotomy for bezoar retrieval.[1]

However, surgical management will address only the tip of the iceberg. Psychiatric management is of paramount importance to address the root cause and prevent recurrences.

CONCLUSION

RS must be contemplated in a patient of trichobezoar presenting with intestinal obstruction. This is the first case report of a combination of RS with daughter bezoar acting as a lead point for intussusception, in English literature. This clinical scenario is potentially life-threatening condition and hence, the importance of timely intervention cannot be underrated. Psychological support and psychiatric treatment are the cornerstones for preventing recurrences.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given their consent for images and other clinical information to be reported in the journal. The guardian understands that patient’s name and initials will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.