“A rare presentation of Rapunzel syndrome manifesting in the immediate post-appendicectomy period”

For my second academic annotation I chose the topic of “Rapunzel Syndrome” (trichobezoar; the human hairball), resulting from a disorder known as “trichophagia,” which goes hand-in-hand with trichotillomania. Trichophagia is a disorder where the individual removes a hair (trichotillomania), and either chews off the root, or chews on the entire strand, followed by spitting it out or consuming it. Individuals with Rapunzel syndrome happen to be the ones that swallow it, which results in a “human hairball” trapped in the stomach, “small bowel,” and/or the right colon. This “human hairball” causes bowel obstruction, which can lead to death if not surgically removed. “Rapunzel syndrome is a rare presentation of a trichobezoar, with 27 cases having been reported in literature so far. The cause of appendicitis in this case was due to luminal obstruction by hair concretions. If appendicitis is due to luminal obstruction by hair concretions, the patient should be investigated for the presence of a gastric or intestinal trichobezoar by endoscopy, ultrasonography or CT scan”(Nair 1).

I thought it would be important to do a little research on trichophagia since it’s the second part of the trich disorder I also suffer from. And although it’s not a main theme of my paper I feel it might be important when putting together the pieces of my “new” trich definition as “addiction.” Take this as a bit of humor, but when comparing trich with substance dependency as “addictions,” their similarities fall in line to the “dead end.” (Pun intended)

Although it may seem petty, or an insignificant observation, the truth is you can die from a drug problem if it goes on untreated, and you can die from trich, or trichophagia, just the same. I admit, this is a far-out correlation I’m trying to make, but is it really? Of the wide range of chemical substances available to become addicted to, some will possibly kill you on their first or second use, other’s will leave you dead in a few short years, and still some of the most dangerous addictive substances may take 20, 40, or 60 years to kill you. For instance, cigarettes and alcohol, two of the most widely used, and overused, drugs in the world, may take a lifetime (pun intended) to leave you dead.

So although cases of death related to trich and trichophagia, resulting from Rapunzel syndrome, are rare, they are only rare in origin to begin with. The number of drug addicts compared to the number of, not only trich, but also trichophagia, AND Rapunzel syndrome cases, are so drastic it creates this humorous response to their comparison. Even as an individual with trichotillomania and trichophagia I stand a rare chance of dieing from Rapunzel syndrome since I spit out the hair after I chew it (sorry that sounds sick). Still, the reality is if I suffered from sever trichotillomania, trichophagia, and consumed the strands of hair, leading to Rapunzel syndrome (which went on untreated, just like most drug addicts), there’s a good chance I would die. The uncontrollable urge to pluck, chew, and swallow would literally kill me since the human stomach can’t digest hair. Death from a disorder that is simply less known, doesn’t make it any less plausible.

“A 16-year-old, healthy girl was admitted with right lower abdominal pain, vomiting and fever of 2 days duration. On examination, she was febrile, with a pulse rate of 110/min. Abdominal examination revealed tenderness at McBurney's point with rebound tenderness and guarding at the right iliac fossa. Her investigations revealed a white cell count of 18,600/mm3 with 96% polymorphonuclear forms. Ultrasound imaging of the abdomen reported minimal free fluid in right iliac fossa. She underwent an appendicectomy through a Lanz incision. The appendix was inflamed and gangrenous. Terminal ileum and caecum were normal on inspection and palpation. On the 4th postoperative day she developed massive upper and central abdominal distension with severe vomiting. Her haematological and biochemical parameters were within normal limits. X-ray of the abdomen revealed intestinal obstruction. She underwent an exploratory laparotomy. There was a firm tubular mass tightly occluding the distal jejunum. The proximal extend of this was occupying the stomach. Through an anterior gastrotomy a black hard block of trichobezoar and its tail-like extension to the jejunum were retrieved”(Nair 1).

Now if that’s not an incentive to seek treatment, or “cure” her disorder, I don’t know what is. In a funny (not so funny) way, Rapunzel syndrome is like an overdose: HAIR KILLS!

Important Noted Information Regarding: Trichotillomania, Trichophagia, Rapunzel Syndrome, and Trichobezoars-

“Trichotillomania in children is a habit disorder and thus has a better prognosis in most cases. However, in adults the psychopathology is usually deeper and thus entails a poor prognosis. Trichotillomania can have a biological basis as two mutant versions of the gene SLITKR1 were found to be more common in trichotillomania patients. These mutations were found to account for only a small percentage of cases. Treatment modalities vary in childhood and adult varieties. Apart from psychotherapy, the drug treatment involves several agents including selective serotonin reuptake inhibitors (SSRIs) and domipramine. Recurrence of Rapunzel Syndrome is very uncommon, and does occur only if the patients are left untreated for their underlying problem. Intensive psychiatric follow-up is mandatory for preventing relapses”(Nair 1).

Nair, Manojkumar S., and Balachandran M. Nair. "A rare presentation of Rapunzel syndrome manifesting in the immediate post-appendicectomy period." Internet Journal of Surgery 14 Issue 2 (2008): 13