Clinical Case Summary

29-year-old male with bloating, early satiety and frequent stools for last 8 years. He reported loss of around 30 kg weight in the past 6-8 years. His physical examination showed gross emaciation and BMI of 11.75 kg/m². He had pallor, oedema and bradycardia with heart rate of 46–54 per minutes.
Blood investigations showed Hemoglobin 8 gm%, total protein 4.8 gms with albumin 2.1 gms. Serum tissue transglutaminase antibody was negative. Contrast enhanced CT showed multifocal symmetric mural thickening involving short segment of small bowel with proximal dilatation and mild thickening of ileocecal junction. Barium meal-follow through and enteroclysis corroborated these findings. Colonoscopy and upper GI endoscopy were normal.
Diagnosis of Crohn’s Disease was considered and oral prednisolone and mesalamine were started. He continued to be symptomatic over next two years. Laparotomy showed multiple dilated small bowel loops and no stricture. Ileal biopsy showed lipofuscinosis.
The final diagnosis was confirmed to be Brown Bowel Syndrome. Patient was then started on Vitamin E, multivitamins, anti-oxidants and mesalamine.
Intestinal lipofuscinosis is a rare disorder that presents as brown pigmentation, occasionally with bowel dilatation and pseudo-obstruction. It co-exists with malabsorption of fat soluble vitamins, especially Vitamin-E. Pathologically, mitochondrial malfunction and degeneration lead to lipofuscin deposition followed by smooth muscle mitochondrial myopathy. Clinical features show motility disorders along with malabsorption features. Treatment involves treating the underlying cause and early initiation of Vitamin-E supplementation.
Conclusion
We present a rare intestinal disorder, Brown bowel syndrome, characterised by the deposition of lipofuscin in the intestinal cells following chronic malabsorption syndrome and vitamin E deficiency.

Introduction

Endoscopic retrograde cholangiopancreatography (ERCP) is a cornerstone in the diagnosis and treatment of pancreatobiliary diseases. However, it carries the risk of complications including potentially fatal post-ERCP pancreatitis (PEP). Despite ongoing efforts, effective strategies for managing PEP remain unestablished. Current guidelines define PEP based on symptoms and findings occurring within 24 hours after ERCP; however, early diagnosis and intervention may prevent its incidence and severity. Recently, urinary trypsinogen-2 (UT2) has emerged as a potential biomarker for early acute pancreatitis, but its role and the optimal timing for PEP remain unclear. Therefore, we aimed to evaluate the diagnostic performance of UT2 at multiple time points following ERCP.

Aims & Methods

This is a single-center prospective observational study conducted at The University of Tokyo Hospital. Patients who underwent their initial ERCP at our institution between February 2022 and June 2024 were enrolled. Patients with choledochojejunostomy, biliary pancreatitis, or those undergoing hemodialysis were excluded. UT2 levels were measured using a rapid dipstick test before ERCP, immediately after, and at 1 and 3 hours post-procedure. Serum amylase (AMY) levels were measured 3 hours after ERCP. We evaluated the diagnostic performance (sensitivity, specificity, and accuracy) of UT2 at multiple time points, for the diagnosis of PEP. PEP was defined as an elevation in pancreatic enzyme >3x upper limit of normal with abdominal pain at 24 hours post-pocedure.

Results

A total of 521 patients underwent initial ERCP during the study period, and 337 were included in the final analysis. The median age was 74 years, and 64% were male. A naïve papilla was present in 89% of patients. The indications for ERCP included bile duct stones (39%), malignant biliary obstruction due to pancreatic cancer (13%) or non-pancreatic cancer (18%), benign biliary stricture (5%), diagnostic pancreatogram (24%), and others (1.2%). EST, EPBD, and EPLBD were performed in 37%, 6.5%, and 4.5%, respectively. Prophylactic pancreatic stent placement and rectal NSAIDs administration were performed in 29% and 20%, respectively. PEP occurred in 10% (mild 8.6%, moderate 1.2%, and severe 0.3%) of patients. UT2 demonstrated a trend toward higher sensitivity compared to AMY (AMY: 52.9% vs. UT2 at 0 h: 52.9%, 1 h: 64.7%, 3 h: 64.7%), while AMY exhibited significantly higher specificity (AMY: 92.7% vs. UT2 at 0 h: 65.3%, 1 h: 64.7%, 3 h: 65.0%) and accuracy (AMY: 88.7% vs. UT2 at 0 h: 64.1%, 1 h: 64.7%, 3 h: 65.0%). Notably, 28.5% of patients were positive for UT2 prior to undergoing ERCP. These patients were significantly older (79 vs. 71 years, P < 0.01), more likely to have pancreatic cancer (19.8% vs. 9.5%, P = 0.02), and less likely to have chronic pancreatitis (3.1% vs. 12.9%, P = 0.01). After excluding 96 cases (28.5%) who were UT2-positive before ERCP, we further evaluated the diagnostic performance of UT2 and AMY. UT2 at 0 h had lower sensitivity compared to AMY (AMY: 54.2% vs. UT2 at 0 h: 37.5%, 1 h: 54.2%, 3 h: 54.2%). In contrast, specificity was similarly high across all markers (AMY: 91.7% vs. UT2 at 0 h: 88.9%, 1 h: 87.1%, 3 h: 87.1%). Diagnostic accuracy was comparable between AMY and UT2 (AMY: 88.0% vs. UT2 at 0 h: 83.8%, 1 h: 83.8%, 3 h: 83.8%).

Conclusion

UT2 showed slightly lower predictive performance for PEP compared to AMY. However, excluding UT2-positive pre-ERCP improved the diagnostic accuracy. The diagnostic performance plateaued at 1 hour after ERCP, suggesting its potential utility in predicting PEP earlier.

Disclosure

The author is affiliated with Nipro Corporation through a labor-related arrangement, but has received no financial support or remuneration related to this study.

Introduction

In contrast to mucosal T1 rectal cancers with an R0 rate of up to 92% at centers, R0 resection may drop to 30% in case of deep sm infiltration due to thermal artefacts at the basal resection margin. TME often follows as completion surgery. Deep sm infiltration as isolated risk factor excluding L+, V+, G3 and Bd2-3 seems associated with a low risk of LN metastases of only 2.6%. Endoscopic Intermuscular Dissection (EID) is a new flexible endoscopic dissection technique with separation of the circumferential and longitudinal muscular layer. EID ideally provides a specimen incl. the entire submucosa and inner circumferential mp layer. 12 sequential EIDs were compared to 53 historical rectal ESDs with HGIEN or rectal cancer as hp. result.

Aims & Methods

Lesions with biopsy proven ADC or HGIEN of the rectum without endoscopic, EUS or clinical signs of deep local infiltration. 2 pat. included after neo-adjuvant RT/Ch th. 24 and 26 mo before with complete response and refusing definitive surgery. Both with local flat polyps (SSA (1) hyperplastic polyp / LGIEN adenoma (2). Endoscopy, EUS, MRI, CT and rectal digital examination without suspicion of local malignant infiltration. Pts were offered to prospectively undergo EID after extensive informed consent within a prospective protocol. All procedures were carried out under gen. anesthesia by an exp. interv. endoscopist with >1000 ESDs. Results of EID were compared to a historical collective of 53 cases with rectal ESD and a histological result of rectal cancer or HGIEN (08-2018 to 12-2021).

Results

From 11-2023 to 09-2024, 12 EIDs were carried out in 12 pts (6 f/ 6 m; mean 63 y; 34-83y). 09/12 lower rectum, 1/12 middle, 1/12 lower and middle, 1/12 upper rectum. 'En bloc' resection rate was 100%, R0 resection rate 89% for neoplasia / HGIEN. Histopathology: 3x ADC G2, 6x HGIEN, 3x LGIEN including both cases with neodajuvant RT/Ch. R0 resection was achieved in 2 of the 3 ADCs. Both with deep sm infiltration without any other risk factors. Tumor board: close clin. & imaging FU. In the 3rd ADC: TU infiltration of inner muscular layer (T2). Pat. underwent uneventful TME 10 d after EID. Clinical FU in all others. Length of hosp. stay 7.1 days. No serious major complications. All pts. closely followed with uneventful FU.
In the historical ESD group R0 resection was achieved in 100% of the HGIEN cases (45/45) but only in 3/8 lesions (37.5%) of cases with adenocarcinoma.

Conclusion

EID seems a new perspective for the endosc. resection of T1 rectal cancers and suspicious polyps in the rectum. More experience and a comparison to surgical local procedures (TAMIS etc.) is necessary for the final assessment of EID as new promising endoscopic technique.

References