See “Human and Doll's Hair in a Gastric Trichobezoar, Endoscopic Retrieval Hazards” by Niţă et al on page 163.
In the 1980's action adventure television series, the title character, Angus “Mac” MacGyver, is known for using simple, readily available objects to elegantly innovate his way out of trouble. In the Grimm Brother's fairy tale, the princess Rapunzel uses her incredibly long and strong hair to plot her escape from a tower. Although these 2 heroes have seemingly little in common, all pediatric gastroenterologists should be aware of their rare but true potential to cross paths in the endoscopy suite, as well as various mishaps that might ensue.
In this issue of the journal, Niţă et al (1) describe their use of electric and argon plasma cautery (APC) to effect the endoscopic removal of a 30 cm trichobezoar from a 9-year old girl who meets criteria for “Rapunzel Syndrome” by its extension into the distal duodenum. The authors, who staff an internationally renowned center of excellence for pediatric therapeutic endoscopy, display their propensity for MacGyvering technologies that are generally available to many pediatric endoscopists. They also provide our close-knit pediatric gastrointestinal (GI) community with an honest and gripping case report that is appropriately cautionary. In addition, Niţă and colleagues have performed a useful literature review that suggests endoscopic removal of trichobezoars should currently be understood to have a 30% success rate.
On their part, bezoars have a long and storied history in clinical medicine. There are many colorful reports dating from the 18th century of their oft-surprising discovery; their varied, but always magnificently gross composition; as well as numerous surgical and nonsurgical techniques for extraction. Trichobezoars are generally constituted of human hair, but have been described previously to include pet and doll hair, as well as plastic bristles from brushes (2). They can present with anemia, weight loss, and hypoproteinemia, and are well associated with severe complications of mucosal ulceration, perforation, and obstruction (3). The presence of a trichobezoar is generally first revealed in standard radiological imaging. Nevertheless, the diagnostic gold standard is upper endoscopy, which can be used both to visualize esophageal and gastric components, as well as to obtain biopsies for compositional analysis.
Although endoscopic therapy is considered effective for small bezoars composed of vegetable matter (phytobezoars) and milk curd (lactobezoars), its role in managing trichobezoars continues to be explored (4). Endoscopy is limited in its utility for gauging length, volume, depth, and complexity of a trichobezoar, as well as the full extent of damage it may have inflicted on the intestinal wall before diagnosis (2). Pre-procedural diagnosis of Rapunzel syndrome with extension of a trichobezoar into and even beyond the small bowel should ideally involve both endoscopy and a CT scan (4). Postprocedural monitoring for late complications because of perforation that may not have been apparent to the endoscopy team may also be appropriate.
Treatment of a trichobezoar once identified is critical. In 1938, the legendary American surgeon and medical MacGyver, Dr Michael DeBakey, was the lead author on a review of more than 300 phyto, lacto and trichobezoars that confirmed nonoperative mortality to be very high at 73%, while operative mortality was 5% (5). In 2010, Gorter et al (6) sought to review all case reports of trichobezoars and found 100 of 108 patients (92.5%) had been treated by surgical laparotomy, with a 100% success rate and a 12% complication rate. Gorter also reported that laparoscopic extraction had been performed in 3 patients, whereas endoscopic success was very low at 5%.
Fast-forward to 2020 and you will find that surgeons have continued to pursue laparoscopic approaches to Rapunzel syndrome (7). Similar to their surgical colleagues, there are few challenges, advanced endoscopists enjoy tackling more than those posed by conventions that proclaim laparotomy as “treatment of choice,” especially for intraluminal conditions. In their article, Niţă and colleagues note that a decade has passed since Gorter's publication, during which endoscopic techniques and endoscopist abilities have improved. By providing an updated review of the literature, they are to be commended for continuing to examine whether and when endoscopic removal of trichobezoars, rather than surgical removal via laparotomy or laparoscopy, should be considered.
Of course, another clear pleasure inherent in the practice of advanced endoscopy often comes from successfully problem solving unique scenarios with existing endoscopic instruments that were not necessarily designed for the exact situation at hand. Starting with a case report by “Mac” Mckechnie in 1972, fragmentation of esophageal and gastric bezoars by gastroenterologists has been attempted using biopsy forceps, water jets, snares and baskets – all with variable success (4,8). In 1998, Wang et al (9) presented several MacGyvered endoscopic instruments, including a modified monopolar 15 mm needle-knife (which they called a “bezotome”) and a modified mechanical lithotriptor (a “bezotriptor”) for “pulverizing” bezoars either mechanically or with acoustic waves. Needless to say, there is no great market for any specific device given the rarity of the diagnosis and none have been rigorously tested. Instead, endoscopists today who wish to attack a trichobezoar must pick from their available modern day arsenal of technologies, using their best clinical judgment regarding feasibility versus potential pitfalls.
In their case report, Niţă and colleagues describe their use of APC in combination with electrocautery with the goal of shrinking the trichobezoar, and then fragmenting it. According to the authors, there are 4 cases of small intragastric trichobezoar removal using APC/electrocautery in the literature, although none involved such a long Rapunzel tail. They also explain that at least 1 of their team had prior endoscopic experience removing human hair this way. APC is a noncontact method of thermal coagulation that has been associated rarely with perforation, and more commonly with gas insufflation leading to significant intraluminal distention. Electrocautery with snare is designed to apply high-frequency electrical current through a wire to cut and coagulate tissue. Obvious but rare complications of electrocautery include ulceration and perforation. Not only more common but also more subtle complications of typical tissue electrocautery involve aerosolization of fine particles released by the coagulated cells that have been long known to have detectable toxic components (10). Hazards associated with these normal “diathermy plumes” are likely underappreciated by medical personnel. In the details of their case report, Niţă et al (1) describe encountering unexpected smoke during their endoscopic “bezectomy.” They later understandably lament in their discussion that they were unaware of the vinyl plastic doll hair contributing to the large trichobezoar they were treating, and their writing conveys amazement at the toxic fumes that were likely produced by applying electrocatery and argon gas in their patient. It is important for all pediatric gastroenterologists who might be tempted “to try this at home” to recognize the many unknown factors that may affect outcomes when using endoscopic instruments for tasks they were not designed for.
In the end, advanced pediatric endoscopists, like all heroes, are prepared to do what is best for their patients, particularly if it can save a child from pain and trauma. What is best for a child may not always be what is least invasive, however. To date, almost all case reports of endoscopic attempts to treat Rapunzel Syndrome, including the one in this issue of the journal, have delineated prolonged procedures, multiple passes of instruments and the scope, and even serial endoscopies, requiring multiple anesthetics (3,4). Meanwhile, surgical removal of these extensive trichobezoars can be efficiently and safely accomplished today—often in 1 piece—by general pediatric surgeons performing a gastrotomy or enterotomy through an upper midline laparotomy, with the potential for direct visualization and confirmation of no mucosal perforation before closing the patient.
Throughout their report, Niţă et al grapple with the definition of procedural success. Without giving away the plot, suffice it to say the authors show how initial high-fives in the endoscopy suite can be premature, as simply extracting a large bezoar does not ensure a patient is in the clear. The authors reflect in their “lessons learned” that achieving procedural success in 1 scenario using a MacGyvered technique does not assure the next will have the same outcome. They also recommend that endoscopic attempts at removal be limited to trichobezoars occupying less than two-thirds of the stomach, with limited or no extension into the small bowel. The moral of their story may be that balancing efficacy, safety, and what is best for a child requires careful investigation before entering the endoscopy suite of all possible known facts that might influence outcomes, as well as experienced clinical judgment and prudence. These characteristics of great therapeutic endoscopists are always to be cherished, especially when comparing the benefits and risks of cut and dry surgery to endoscopic ingenuity.
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