Rapunzel Syndrome: Endoscopy, Laparotomy, or Laparoscopy?

Sir,

We read with a great deal of interest the article recently published on your journal by Kumar et al.[1] The authors report the youngest case of Rapunzel Syndrome actually published in literature, pointing out the importance of a correct clinical suspicion to avoid the delay in a lifesaving surgical intervention.

This syndrome generally affect young women between 13 and 20 years of age with mental retardation or psychiatric illness, such as trichotillomania and trichophagia.[2]

Usually, the clinical complaint is unclear up to it reaches a significant size. The most common features are abdominal pain, abdominal mass, gastrointestinal obstruction, weight loss, poor appetite, and anemia, like in our case, that we would briefly present.

A 14-year-old girl was admitted to our emergency department with a 3 months' medical history characterized by epigastric pain, vomiting, and weight loss. The abdominal examination revealed a mobile mass in the epigastrium and left hypochondrium. Investigations such as complete blood count, liver function test, and serology showed a microcytic normochromic anemia (hemoglobin 9.7 g/dL). Abdominal ultrasonography and computed tomography revealed an abnormal gastric dilatation and a luminal mass extending up to the proximal jejunum [Figure 1]. The diagnosis was done by upper gastrointestinal endoscopy and consensually we perform a “Y-shaped” subumbilical exploratory laparotomy with gastrostomy. A large trichobezoar peeping to the proximal jejunum was removed. The strategy against recurrence was carried out by a psychiatric counseling and at 1-year follow-up the girl feed regularly, not presenting similar episodes.

Figure 1

Computed Tomography: The arrow keys show the abnormal gastric dilatation and the luminal mass

Both cases have in common an important delay in the diagnosis (3 months). Despite the case presented by Kumar et al.,[1] the patient was an adolescent affected by an ascertained trichotillomania disorder, was not dehydrated and presented only a mild microcytic anemia.

The diagnostic procedures were started owing to acute abdominal symptoms. A differential diagnosis in pediatric age should comprehend pancreatic pseudocyst, gastrointestinal stromal tumors, and phytobezoar.[3]

Regarding the correct treatment, the classical therapy includes endoscopy, often with subsequent laparotomy, and associated psychiatric intervention and in some selected cases laparoscopy.[4] Small trichobezoars can be removed by endoscopy, anyway the procedure is difficult and at risk of esophageal perforation.

Although success is greater in laparotomy, especially in emergency with a compromised very young child, as pediatric surgeons faced with this rare entity, it is important to take into consideration the benefit of laparoscopy mostly in partially obstructing bezoar.

In 2013, Javed and Agarwal described a very interesting modified laparoscopy-assisted technique with temporary gastropexy for the effective removal of a large trichobezoar using a camera port and a 4–5 cm incision, taking advantage of the presence of digestive endoscopy, like a percutaneous endoscopic gastrostomy procedure.[5]

From our case, we learned an important lesson. Due to the patient's age, the absence of severity of the gastric outlet obstruction, the lack of complications such as intussusception or perforation and the extension of the bezoar, the laparoscopy could be a good option that we discarded too quickly, probably given the exceptional nature of the case.

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Conflicts of interest

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