Rapunzel Syndrome: A Case Report

Case Report

Rapunzel Syndrome: A Case Report from Al-Dabba Hospital, Northern State, Sudan

Hassan M. E. Eltigani¹, Sara O. A. Ahmed^*1

Abstract

Background: Trichobezoar is accumulation of hair in the stomach, which may fill up the stomach and take its full shape. Sometimes it extends down to the small and large intestine; thus forming the “Rapunzel syndrome”. Occasionally, small pieces dislodge from the mother hairball and travel down to the small intestine causing intestinal obstruction.

Case Description: A 16-year-old girl presented with abdominal mass and features of intestinal obstruction. Medical history indicated that she tended to pull her hair (trichotillomania) and eat it (trichophagia). The condition was diagnosed on clinical grounds with the help of plain abdominal X-rays, which showed features of intestinal obstruction.  The patient was operated on in AL-Dabba District Hospital, Northern State, Sudan. A huge hairball was delivered from the stomach, and parts of the hairball were found and removed from the small intestine. Postoperatively, the patient made good recovery, and was referred for the care of the Psychiatrist.

Keywords:  Rapunzel syndrome, bezoar, trichobezoar, trichophagia

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1AL-Dabba District Hospital, Al-Dabba City, Northern State. Sudan.
* Corresponding author: Sara O. A. Ahmed
AL-Dabba District Hospital, Al-Dabba City, Northern State. Sudan.
Email: saraosman19990@gmail.com

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:Introduction 

Trichobezoar is formation of hairball in the stomach, which may be seen in young patients with psychiatric illness, mostly females who tend to eat their hair. The hair forms a ball-like mass which may occupy the full stomach. When this hairball extends into the duodenum and jejunum it constitutes the Rapunzel syndrome.(1) When materials accumulate in the stomach, they form Bezoars. Trichobezoar is a variety of Bezoars with an accumulation of hair in the stomach. With time the peristalsis causes it to be enmeshed to a ball, and may extend to reach the small and large intestines.(2) 

Rapunzel syndrome is trichobezoar with downward extension to the small and 

large bowel. Sometimes it may lead to partial or complete intestinal obstruction 

.when small pieces dislodge from the mother hairball down to the intestine

(خطأ! الإشارة المرجعية غير معرّفة.)

Only 64 cases of Rapunzel syndrome have been reported in the literature since 1968, when first described by Vaughan et al.(3) Presenting symptoms usually include abdominal pain, nausea, vomiting, abdominal distension, constipation, obstruction, peritonitis and weight loss.(4)

Rapunzel syndrome usually occurs in female patients with psychiatric disorders in whom trichotillomania (pulling their hair) and trichophagia (eating their hair) are noticed.(2,5) Treatment can be through surgical intervention (laparotomy/laparoscopy) or via endoscopic intervention.(6)

:Case description

This is a case of a 16-year-old secondary school female student originally from Omdurman, who reported to our hospital in Al-Dabba with complains of abdominal pain and vomiting for four days. It started four days earlier when she developed epigastric pain colicky in nature, seven out of ten on the severity scale, on and off, aggravated by taking food or drinks and relieved by fasting, vomiting

. and taking analgesics. Later on, the pain was all over the abdomen

The pain was associated with vomits many times; greenish in color, large in amount, offensive in odor, and did not contain blood. Later, the vomiting turned yellowish and was associated with upper abdominal distension, absolute constipation for two days, loss of appetite and weight loss. The patient was not

. known to have any chronic illness

Patient had similar complaints when she was 8 years old, sought medical advice and was treated for gastritis and stomach infection. She had the habit of chewing her hair since she was 4years old. There is a positive family history of

. this morbid habit of chewing hair in a second-degree relative

On general examination, she looked ill, pale not jaundiced, in pain and dehydrated. Her vitals showed: respiratory rate: 26 cycle /min, blood pressure: 100/60 mmHg

Abdominal examination revealed normal abdominal temperature, distended in the supra-umbilical part, no dilated veins. Tenderness was elicited all over the abdomen but mainly in the epigastric area with a palpable epigastric mass and exaggerated bowel sounds at supra-umbilical area. A palpable mobile mass was noted in the epigastrium. Bowel sounds were exaggerated. On digital examination

. the rectum was empty

:Operation Findings

The patient was referred to our unit as a probable surgical abdomen. History and physical examination revealed a clinical picture of high intestinal obstruction. Investigations included complete blood count, urine general and renal profile with electrolytes were all normal. She underwent abdominal X–ray erect (Figure 1) and supine (Figure 2). Being a peripheral district hospital we did not have the facilities of Endoscopies and CT-Scan. The first author (HE) had previously come across a similar case with upper intestinal obstruction in a central hospital where investigations were available and revealed the diagnosis

. of intestinal obstruction because of Trichobezoar

Figure 1: Erect abdominal X- ray 

Figure 2: Supine abdominal X- ray

Both figures show dilated small bowel loops and fluid levels which is

. pathognomic of small bowel obstruction

intestinal obstruction and prepared the patient for laparotomy

Under general anesthesia, and through upper mid-line incision the abdominal cavity was reached. There was a palpable mass that filled the stomach and took its shape. This confirmed our clinical judgment of trichobezoar. The proximal jejunum was massively dilated (15cm), the obstructing mass was followed down the jejunum and jejunotomy was done. The obstructing agent, a dislodged segment from the mother hairball was removed (Figure 3), the jejunum was closed. Then the stomach was opened longitudinally and the huge mass of the trichobezoar weighting about 400g was delivered (Figure 4). Naso-gastric tube was inserted for suction, and the stomach closed. Two drains, pelvic and epigastric, were put and

. the abdomen closed in layers

The patient made an uneventful recovery and was seen by the psychiatrist with

. regular visits till discharge

Figure 3: The dislodged segment of the hairball removed from the small

. intestine

Figure 4: The mother hairball, which was occupying and taking the shape of

. the whole stomach

:Discussion

Our patient admitted chewing her hair. We made our diagnosis on clinical grounds supported by abdominal X.ray (erect and supine). CT-Scan when available would show more details. Sono-graphic features are not diagnostic but a hyper-echoic curve line at the outer margin may suggest the diagnosis. Upper gastrointestinal endoscopy confirms the diagnosis and could be therapeutic for removing small

. bezoar-like foreign bodies  

Trichobezoar was described in all age groups; the disorder has female preponderance among the age group of 10-19 years. The symptoms depend on the size and time of presentation, the presenting symptoms of our case were similar to the most known in the literature; abdominal pain and vomiting. On the other hand, the case satisfies two of the three common features of Rapunzel syndrome, the trichobezoar with tail and the extension of the tail at least to the jejunum.(4) The weight of the bezoar that we had removed was about 400g. This can be regarded as a huge one as compared to what had been described in the literature.(7)

Thirteen cases of Rapunzel syndrome were reported in the literature where long hair strands extended from the gastric mass to the small bowel duodenum or jejunum to reach the caecum.(4) This is the fourth case to be reported in Sudan and the second in the Northern State.(8)

During Gastrostomy and the hairball removal from the duodenum, the small bowel should be examined on the table for additional bezoar at these sites. Psychiatric counseling to the family members since the case may recur.(5,5)

:Conclusion

History and clinical examination are important to diagnose intestinal obstruction because of Rapunzel syndrome, especially in an area without imaging (CT scan) or

. endoscopy

In addition, the current situation in Sudan, where the country is going through the turmoil of an internal war, makes it difficult for patients in remote areas to travel to big hospitals for sophisticated investigations. Throughout the path of the

. management, we went on clinical grounds supported with only abdominal X-ray

:Conflict of interest 

None 

:Ethical consideration 

Informed consent was obtained from the patient and her parents for the purpose

. of publication

:Acknowledgement

We sincerely thank Dr. Ahmed Salah, the General Surgery Registrar, for diligently participating in the patient’s follow-up until discharge. We also appreciate the help and care of Dr. Anas Sir Alkhatim the physician, and Dr. Hassan Ismail the Psychiatrist. Additionally, we commend the theatre team, anesthesiologists, and scrub nurses who worked tirelessly under challenging circumstances. Special thanks go to Dr. Egbal Ahmed Basheir Abukaraig for her close supervision

. throughout the case study writing

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