Beyond Inflammatory Bowel Disease: Calprotectin as a Marker for Trichobezoar? A Case Report Open Access

Bezoar is defined as an aggregation of indigestible foreign materials that cannot be cleared by peristalsis [1]. Many types have been described based on their composition ranging from phytobezoars (plant fibers) to pharmacobezoars (medications) [1]. Trichobezoar is formed from clumping of hair strands that get entangled over a period of time eventually organizing into a large gastric mass [1]. Occasionally, trichobezoars may extend from the stomach into the small bowels, a condition known as “Rapunzel syndrome” [2, 3].

Typical presentation includes vague abdominal pain, vomiting, diarrhea, and rarely small bowel obstruction or perforation [3, 4]. Treatment is typically with surgical removal but reports of successful endoscopic retrieval have been published [3‒5].

The diagnostic workup includes imaging such as computerized tomography and ultrasonography and eventually endoscopy [4]. Since the mucosa in this condition is typically spared, inflammatory markers such as calprotectin should not – at least in theory – show any abnormality. Herein we present a case of a patient that has had elevated calprotectin solely from a massive trichobezoar and early Rapunzel syndrome.

The CARE Checklist has been completed by the author for this case report, attached as online supplementary material (for all online suppl. material, see https://doi.org/10.1159/000546623).

A 7-year-old girl presented to an outpatient gastroenterology clinic with a complaint of chronic abdominal pain that lasted for about 6 months. The pain was described as colicky in nature and was located in the periumbilical region with no radiation. The pain was episodic, lasting for a few hours per episode, with several episodes per month. It was not related to food intake or bowel movements and was associated with occasional vomiting that was non-bilious and non-bloody, as well as occasional low-grade (38.5°C) fevers lasting for less than 48 h. The patient also has had occasional bouts of loose stools (type 6 on Bristol scale), about 8 times a day lasting 3–4 days per episode, along with fecal urgency, tenesmus, and rectal pain, but there was no blood seen in her stools. She denied having eye symptoms, skin rashes, joint pains, weight loss, or loss of appetite. Family history was significant for ulcerative colitis in her mother. On physical examination, her vitals were within normal limits, her weight was on the 50th centile for age, and her height was on the 85th centile for age, her examination was otherwise unremarkable including abdominal and rectal examinations. Diagnostic workup at the time of presentation revealed a low mean corpuscular volume at 56 fL, a high red cell distribution width at 17%, and more importantly, fecal calprotectin value of 433.9 mg/kg. Otherwise, her routine blood and stool tests were all within normal limits including liver function panel, erythrocyte sedimentation rate, high-sensitivity C-reactive protein, tissue transglutaminase antibodies IgA, Helicobacter pylori stool antigen, stool ova, and parasites.

Due to the family history of ulcerative colitis and elevated calprotectin value, a decision was made to proceed with upper and lower gastrointestinal (GI) endoscopies. Endoscopically, the mucosa of the upper and lower tracts looked normal; however, there was a massive gastric trichobezoar mass (Fig. 1) with early formation of a long tail (i.e., Rapunzel syndrome) extending into the first part of the duodenum but without a complete bowel obstruction. Histologically, the mucosal samples from the entire tract were normal, except in areas where there was direct mechanical irritation of the mucosa at the incisura in the gastric mucosa by hair strands that showed reactive gastropathy.

During the initial endoscopy screening, removal of the massive trichobezoar was attempted by different tools including snares, forceps, nets, but unfortunately, all attempts failed to pull the mass through the lower esophageal sphincter, so individual locks of hair were being piecemeal retrieved by forceps and nets which was time-consuming (Fig. 2). Eventually and after discussing with the family, it was decided to abort the endoscopy procedure and proceed with a scheduled surgical removal via upper laparotomy and gastrotomy to retrieve the mass (Fig. 3).

On follow-up, the patient’s symptoms had resolved before they came back on postoperative day 40 when she presented with vomiting, acute abdominal pain, and constipation to the emergency department. A plain film at the time showed dilated bowel loops with air-fluid levels. A contrast-enhanced CT scan of the abdomen confirmed an intraluminal lesion with air foci with dilated proximal jejunal loops measuring up to 3.5 cm down to the lesion. The scan otherwise showed no other abnormalities including the rest of bowels. The patient was then taken back to the operating room for another laparotomy with a small enterostomy to retrieve the remaining trichobezoar. The postoperative recovery was uneventful. Eventually, the patient had complete resolution of symptoms and normalization of calprotectin on follow-up a year later.

Calprotectin is a cytosolic protein that is naturally present in neutrophils, monocytes, dendritic cells, as well as keratinocytes and squamous cells of the mucosal linings. It is only excreted in tiny amounts in a healthy state; however, it can be induced during inflammation [6].

It is a well-established marker of inflammatory activity in inflammatory bowel disease but has been also reported with intestinal bacterial and viral infections as well as malignancies [6]. Fecal calprotectin is not an exclusive marker to lower GI pathologies, albeit its levels in upper GI pathologies are not as high [7‒9].

The cutoff value is not well established and seems to vary from one study population to another [6]. But most cohorts seem to agree on a cutoff value of less than 50 µg/g for healthy mucosa compared to IBD [6, 10, 11]. As such, it does have a high sensitivity but a much lower specificity, as it can be elevated in many non-IBD pathologies as well [6].

Trichobezoars are not typically associated with frank GI inflammation but rather present a clinical picture of mechanical obstruction when advanced [1, 3]. To our knowledge, it has never been reported in association with elevated calprotectin as a sole etiology. Salada et al. [12] reported a calprotectin level of above 2,000 in a patient who presented with watermelon-seed bezoar. That patient, however, had multiple pathologies simultaneously, including volvulus, anal fissure, stercoral colitis, as well as a frank rectal ulceration that was attributed to mechanical injury from the seeds and fecal impaction [12]. This case by comparison did not have any form of frank ulceration or any inflammatory changes histologically besides reactive gastropathy in small areas that had direct mechanical rubbing with hair strands. It can be theorized that the moderate elevation in calprotectin in this case can be explained by that finding alone. Moreover, keratinocytes (including hair cells) do express calprotectin in their cytosol in healthy state as well as in inflammatory dermatoses [13‒15]. One could speculate that keratin denaturation by gastric hydrochloric acid may release some of the keratinocyte-specific calprotectin. A limitation of the present study is the fact that it is a single case; thus, more robust evidence from larger scale observational studies is needed before establishing a direct causal relationship between trichobezoars and calprotectin.

In conclusion, fecal calprotectin is an excellent biomarker for IBD; however, it can be elevated in other non-inflammatory conditions as well, such as trichobezoar-induced mechanical injury. The values seen in upper GI pathologies are typically much lower than in lower GI inflammatory conditions. One must always take the entire clinical context into account when interpreting calprotectin values and keep an open mind approach when deciding on further invasive testing including endoscopies.

Written informed consent was obtained from the legal guardian of the individual for publication of the details of their medical case and any accompanying images. This study was performed in accordance with the Declaration of Helsinki of 1975, as revised in 2008 [5]. All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national). This study did not require ethical approval in accordance with local/national guidelines.

The author has no conflicts of interest to declare.

No funding was received for this research.

A.K. contributed to drafting, data collection, and final approval of the manuscript.

The data that support the findings of this study are not publicly available due to privacy reasons but are available from the corresponding author upon reasonable request.