The theme for the DFTB18 conference was Science and Story. We recognize that stories, both those of patients and practitioners, are a wonderful way of passing knowledge. Last year we showcased stories as a means of promoting mental illness in healthcare practitioners and the challenges of neonatal retrieval at DFTB17, amongst others. The patient experience will, once again, be a key component of the conference. But here is a grim patient that you won’t see – the patient with Rapunzel syndrome.
Consider the story of Persinette or Petrosinella. You might know her as Rapunzel. Whatever version you are aware of – that by Giambattista Basile, the Brothers Grimm or the Mandy Moore-voiced version that seems to be on constant repeat in my house, the story is the same…
A young woman, in the first trimester of pregnancy, gets a craving for rampion. Her husband, eager to please, finds some in the local market and brings it back for her to nibble on (perhaps with some cottage cheese and bread). The market stores soon run low, but luckily, he spies some growing in the grounds of the rundown cottage next door. In the middle of the night, shoes bound with hessian strips to dampen the noise, he sneaks into the garden and begins to forage.
Unfortunately for the young man, the owner of the cottage, Dame Gothel, is a light sleeper and confronts him. Desperate to please his wife, he promises to give her the child when she is born in exchange for a regular supply of rampion (or rapunzel as it is also known). The pregnancy continues without issue, and when the baby girl is born, the heartbroken couple reluctantly hands over their child.
When she reaches the age of twelve, the witch (because that is what Mother Gothel clearly is) locks her up in a tower, away from the world. She bricks up the door so the child cannot escape and uses an alternative means of access and egress – the window.
“Rapunzel, Rapunzel, let down your hair – so I might climb without a care.”
There are some problems with having such long hair, though.
Rapunzel syndrome
Vaughan JE, Sawyers JL, Scott JH. The Rapunzel syndrome. An unusual complication of intestinal bezoar. Surgery. 1968 Feb;63(2):339-43.
Trichophagia and trichotillomania are uncommon. Unlike the old wives tale of what happens when you swallow chewing gum, it does have a tendency to get stuck and form a hairball, or bezoar, in the stomach. This paper by Vaughan et al. is the first documented case of what is known as Rapunzel syndrome. As human hair is relatively smooth, it tends to resist normal peristaltic expulsion through the pylorus, so it bundles up in the stomach. Like a wayward fisherman’s net, it accumulates mucus and partially digested food until an impacted bezoar forms. Rapunzel syndrome occurs when the tail of the bezoar extends through the pylorus into the small intestine. Of all of the reported cases to date, only one has taken place in a male patient, but perhaps, with the rise of man-buns, we might see more.
Trichobezoars have been linked with cases of intussusception, bowel obstruction and even death due to mucosal perforation. Patients may also present more innocuously with iron deficiency anaemia and weight loss on a background of psychiatric illness. These glistening balls of keratin are not visible on plain x-ray though may be picked up by ultrasound.
Day after day, Dame Gothel calls out to fair Rapunzel to let down her golden braids for her to climb, little knowing the harm she was doing to her poor little head.
One day a handsome prince (because they are always handsome) espies the lovely Rapunzel at the windows. As he peeps out of the bushes, he spots the elderly Dame approaching, calling up to fair Rapunzel and ascending. He waits so patiently for her to climb back down again, and once she has gone on her way, he calls up to her.
“Rapunzel, Rapunzel, let down your hair – so I might climb without a care.”
Little did he know that it would not support his weight.
Traction alopecia
Zimmerman B, Ivars M, Cordoro KM. Bibbidi bobbidi bald: Two “hairowing” tales of Princess Package hairstyles. Pediatric dermatology. 2018 Apr 15.
The weight of a wrinkly witch is enough to detach growing follicles from the scalp. The article cited above describes cases where young girls, in their attempt to look like a Disney princess, have had their hair scraped and chignoned, then finished off with some extensions. Unfortunately, the tension on the scalp probably leads to localized ischaemia, then erythema, followed by a grey necrotic crust and a patch of alopecia a fortnight or so later. Longer-term follow-up showed a lack of hair growth in the area of the necrotic patch.
There is great power in storytelling and sharing.
Selected references
Dalshaug GB, Wainer S, Hollaar GL. The Rapunzel syndrome (trichobezoar) causing atypical intussusception in a child: a case report. Journal of Pediatric Surgery. 1999 Mar 1;34(3):479-80.
Frey AS, McKee M, King RA, Martin A. Hair apparent: Rapunzel syndrome. American Journal of Psychiatry. 2005 Feb 1;162(2):242-8.
Naik S, Gupta V, Naik S, Rangole A, Chaudhary AK, Jain P, Sharma AK. Rapunzel syndrome reviewed and redefined. Digestive surgery. 2007;24(3):157-61.
Pul N, Pul M. The Rapunzel syndrome (trichobezoar) causing gastric perforation in a child: a case report. European Journal of Pediatrics. 1996 Jan 1;155(1):18-9.
Slepyan AH. Traction alopecia. AMA Archives of Dermatology. 1958 Sep 1;78(3):395-8.
Ventura DE, Herbella FA, Schettini ST, Delmonte C. Rapunzel syndrome with a fatal outcome in a neglected child. Journal of Pediatric Surgery. 2005 Oct 1;40(10):1665-7.
