Clinical Case Summary

Aim: Abdominal Cocoon syndrome is the complete or partial wrapping of the abdominal organs with a membrane structure. Diagnosis can be made by laparoscopy. Patients mostly present with acute abdomen symptoms due to intestinal obstruction.
Case: 27-year-old male patient had recurrent abdominal pain for 12 years and was diagnosed with Behçet's disease and started on treatment. The patient’s abdominal pain increased in the last 6 months. He was followed up 4 months ago with suspected ileus, recovered with symptomatic treatments. A colonoscopy was performed and aphthous ulcers were detected in the terminal ileum and linear ulcers in the sigmoid colon. Tuberculosis,vasculitis and amyloid was excluded in the biopsies .With the preliminary diagnosis of Crohn's disease, 5-ASA and Azathioprine were started, and when no clinical response was obtained, Adalimumab was switched. The patient, who had no history of alcohol, cigarette use or any surgery, stopped all of her medications because she had not had symptomatic improvement for a long time, and was relieved by vomiting voluntarily. Her family history included rheumatoid arthritis in her sister and ulcerative colitis in her cousin. In the physical examination, there was no pathology other than widespread tenderness in the abdomen. In the ultrasonography; the small intestine walls were seem to be slightly edematous and retracted. Enteroscopy was completely normal. In the CT-Enterography, it was thought that it could be an "abdominal cocoon", so a laparotomy was performed and the diagnosis. The small intestines were freed from the fibrotic bands. The patient did not develop any post-operative complications, and her abdominal pain completely resolved after the operation.
Conclusion: Abdominal cocoon syndrome should be kept in mind as a very rare disease that can cause diagnostic confusion, remain undiagnosed for a long time, and cause unnecessary treatments, and can be fixed totally with surgical treatment.

Introduction

Epithelial injury is a hallmark of chronic mucosal damage in Crohn’s disease (CD), which can lead to fibrostenotic strictures. Type VII collagen, a critical component of the epithelial basement membrane, forms U-shaped anchoring fibrils that link the basement membrane to the interstitial matrix by binding fibrillar collagen. Genetic mutations in the COL7A1 gene, which encodes type VII collagen, are known to impair fibril formation and reduce tissue tensile strength, resulting in epithelial dysfunction, as seen in disorders such as dystrophic epidermolysis bullosa. This highlights the essential structural role of type VII collagen in maintaining matrix stability. In CD, the importance of this protein is underscored by the presence of autoantibodies targeting type VII collagen in up to 50% of patients with CD. Loss of tissue integrity, because of loss of the interaction between the basement and interstitial matrix, leads to tissue dysfunction and fibrosis.

Aims & Methods

We investigated C7M, a biomarker of type VII collagen degradation, to assess its specificity for intestinal mucosal damage. We analyzed serum samples from CD patients enrolled in a prospective cohort study. The cohort included 62 CD patients undergoing surgical resection for fibrostenotic strictures and 49 patients with luminal disease receiving treatment adjustments without surgery. Serum was collected at baseline, post-operative day 1 (POD1), and one-month post-resection. C7M levels were quantified using a competitive ELISA.

Results

At baseline, C7M levels were significantly elevated in fibrostenotic CD patients (median: 9.79ng/mL) compared to those with luminal CD patients (median: 6.03ng/mL) (p<0.01, AUC: 0.69). In the resected group, C7M levels rose sharply on POD1 (p<0.0001, AUC: 0.81), suggesting acute release in response to surgical injury. By one-month post-surgery, C7M levels had returned to baseline, closely resembling those observed in luminal CD patients (p<0.0001, AUC: 0.80). C7M also demonstrated mild-moderate correlations to biochemical markers of inflammation, including CRP (rho=0.62, p<0.0001), hemoglobin (rho=-0.28, p=0.0024), thrombocytes (rho=0.24, p=0.0105), albumin (rho=-0.52, p<0.0001), and fecal calprotectin (rho=0.25, p=0.019).

Conclusion

C7M was elevated in fibrostenotic CD patients relative to luminal CD, indicating its potential as a biomarker of more severe or complex mucosal pathology. The pronounced but transient increase following surgery, with normalization within one month, further supports C7M as a dynamic marker of intestinal epithelial damage. These findings suggest C7M may serve as a novel, disease-specific biomarker for assessing mucosal integrity and inflammatory burden in CD.

Disclosure

Full time employee at Nordic Bioscience