Clinical Case Summary

Background: AL (amyloid light chain) amyloidosis is a rare systemic disorder affecting the heart, kidneys, nervous system, and gastrointestinal (GI) tract. GI involvement typically presents with malabsorption or dysmotility from mucosal amyloid deposition. We describe an unusual case presenting as small bowel obstruction (SBO), initially attributed to adhesions.
Case presentation: An 84-year-old man with a history of radical cystoprostatectomy and ileal conduit for urothelial carcinoma presented with recurrent SBO over three months. The initial suspicion was that of adhesive obstruction due to the surgical history. He underwent laparotomy with adhesiolysis and small bowel resection, but the symptoms persisted with high nasogastric output and ongoing obstruction. Histology from the first resected segment did not reveal any specific pathology.
A contrast follow-through study showed dilated small bowel loops with a transition point. Computed Tomography (CT) of the abdomen and pelvis revealed proximal jejunal dilation and segmental circumferential wall thickening.
Antegrade double-balloon enteroscopy (DBE) was performed and it revealed a tumor-like nodular stricture in the jejunum, 68 cm from the incisors. Biopsies showed Congo red-positive amyloid with birefringence and lambda-restricted plasma cells. Repeat surgical resection confirmed amyloid at both margins.
Bone marrow aspirate showed a lambda-restricted plasma cell population (0.5%). Mass spectrometry confirmed AL amyloidosis (lambda type).Positron Emission Tomography-CT (PET-CT) revealed Fluorodeoxyglucose uptake in the right paracolic peritoneum. Cardiac MRI ruled out cardiac involvement.
The patient received 8 cycles of bortezomib-cyclophosphamide-dexamethasone (VCD). Interval PET-CTs showed resolution and he remained well on maintenance bortezomib 16 months post-treatment initiation.
Conclusion: This case illustrates an unusual AL amyloidosis presentation, with a focal tumor-like jejunal stricture, causing SBO.

References

We plan to include endoscopic, radiological, and pathological visuals in the final poster. This includes:

1. Double-balloon enteroscopy (DBE) image showing a jejunal nodular lesion;
2. Congo red-stained histology demonstrating apple-green birefringence under polarized light;
3. Computed tomography (CT) showing proximal jejunal dilation and segmental mural thickening;
4. Initial and follow-up PET-CT scans showing fluorodeoxyglucose (FDG) uptake and treatment response;
5. Bone marrow aspirate results with flow cytometry and immunofixation confirming a lambda-restricted plasma cell population.

• Kwon JH et al. An Unusual Cause of Acute Intestinal Pseudo-Obstruction. Case Rep Gastroenterol. 2019;13(3):507–512. [PMC6873005]
• González-Suárez B et al. A Rare Cause of Diarrhea Detected by Balloon-Assisted Enteroscopy. Case Rep Gastroenterol. 2022;16(3):577–582. [PMC9638858]
• Siddiqui A et al. A Rare Cause of Intestinal Pseudo-obstruction: Gastrointestinal Amyloidosis. Cureus. 2020;12(4):e7583. [PMC7199901]

Clinical Case Summary

Diseases damaging the enteric mucosa cause malabsorption that may trigger neurologic symptoms, both by immune-related processes and impaired absorption of essential vitamins.
Celiac disease (CD) is found in patients presented with nonhereditary ataxia. In these patients, abnormalities referred to as “gluten ataxia”, are probably the result of immunologic damage to the CNS and peripheral nerves.
B1 is an essential vitamin which its deficiency has broad clinical spectrum; thus, its diagnosis is challenging, and a sensitized clinician is needed to prompt identification. The encephalopathic form of adult thiamine deficiency is called Wernicke-Korsakoff syndrome.
A 40-y/o/w, G4P2, suffered from severe hyperemesis gravidarum resistant to antiemetics during trimester 1.
On 13^th week of pregnancy, she went to the ER, d/t confusion & extensive weakness. Her lab tests showed severe electrolytes deficiencies with transaminitis and elevated CPK levels. She was diagnosed with rhabdomyolysis secondary to her continuous vomiting. On her neurological examination psychomotor slowness and ataxia were found. Thus, Wernicke encephalopathy was suspected and her B1 level was tested and found low: 12 ug/L. She was hospitalized in the ICU to get electrolytes replacement and intravenous thiamine. To confirm the diagnosis, brain MRI was performed and demonstrated a classic picture of Wernicke encephalopathy (1).
6m later she gave birth, but her neurological deficits got worse. TTG level was tested and found positive. Gastroscopy showed duodenal scalloping (2) and Marsh 3C on biopsy (3). She started GFD and neurological rehabilitation. On repeated anamnesis she had abdominal pain before the pregnancy, but her investigation was negative.
This case illustrates a rare herald of CD and increases the awareness of possible manifestation of B1 def.
Points to discuss:

1. Two-hit theory of B1 def. (hyperemesis gravidarum and CD)
2. Neurologic manifestation of CD & B1 def.
3. Vitamins’ malabsorption in CD

References

1. Brain MRI with classic picture of Wernicke encephalopathy
2. Endoscopic picture of duodenal scalloping
3. H&E staining that demonstrates Marsh 3C in duodenal biopsy

Clinical Case Summary

Case report: A 47-year-old woman presented with persistent abdominal bloating, cytolysis and hyperalkaline phosphatase. The patient was underweight. Over 10 years, pancreatic hyperamylasemia (>900 U/l), thrombocytopenia and anemia with low iron and folate levels are observed. Medical history - one spontaneous abortion, 2 children conceived by in vitro fertilization. Viral, autoimmune and genetic liver diseases, autoimmune thrombocytopenia, macroamylasemia, Helicobacter pylori and Giardia lamblia infection were excluded. Immunoglobulins and calprotectin were normal. Serological tests for Celiac disease (CD) detected a positive result only for antigliadin Ig G. Abdominal tomography scan did not detect pathological changes. Duodenoscopy revealed flattening of the intestinal villi suggestive of CD. Duodenal biopsy determined a morphological picture characteristic of CD: type 3c/B2 according to the Marsh-Oberhuber criteria. It was recommended a gluten-free diet, iron and folate therapy. At repeated check-up after 3 months, the patient's condition showed significant improvement, and no changes were detected in the laboratory tests.
Discussion: CD can be diagnosed at any age and can present with a wide range of associated or isolated clinical manifestations. Evaluation of the causes of various biological abnormalities may lead to the diagnosis of CD. The presented case highlights atypical clinical presentations that can be associated with CD. Despite the evolution of serological test performances, there are significant problems regarding the diagnosis in atypical conditions, especially in the patient with intestinal atrophy. In such situations, the intestinal biopsy remains the gold standard diagnosis for CD.
Conclusion: The non-classical clinical presentation of CD is becoming increasingly common in the daily practice of the physician, which requires an awareness of its many clinical faces with atypical forms.

References

- 2 illustrative materials with fragments of biopsy from duodenal mucosa (A - complete villous atrophy, stage 3c according to Marsh-Oberhuber, cryptic hyperplasia by numeral growth and depth of the glandular layer. B - m​arked lymphocytic infiltrate within the lamina propria, minor eosinophilia, hyperplastic crypts, slightly distorted architecture, complete villous atrophy).
- 4 illustrative materials from duodenoscopy

Disclosure

None to declare.

Introduction

The peripherally inserted central catheter (PICC-Line) is an essential vascular access method used across various clinical settings. It is particularly indicated in patients requiring prolonged antibiotic therapy, mid-term parenteral nutrition, extended hospitalization with intravenous treatments, or who have poor peripheral venous access. PICC lines are typically delivered in standard lengths (approx. 60 cm) and must be individually trimmed to the correct length prior to insertion. Various approaches exist to estimate the optimal insertion length. While traditional practice relies on clinician experience and anatomical landmarks, this study aims to compare the precision of clinician predictions against those made by a predictive formula developed in a previous retrospective study.

Aims & Methods

To evaluate the accuracy of a previously derived formula for estimating ideal PICC-line length and compare it with estimates made by experienced clinicians.
Patient data on a total of 60 PICC lines was previously retrospectively analyzed to develop a multivariate regression formula. The data of that data set was summarized in 60 individual cases, each to be shown to participants in this study. Participants included both clinicians of varying level of experience in the placement of PICC lines. The participants were asked to estimate the ideal PICC length for each anonimized clinical case. Their estimates were compared to the formula-based ideal lengths using absolute deviation and proximity categories (±2 cm, ±3 cm, ±4 cm).

Results

A total of 264 estimates were recorded from 10 participants. The average deviation from the ideal PICC length was 6.3 cm (range: 2.8–12.5 cm). The proportion of estimates falling within the predefined accuracy margins were:

• ±2 cm: 65 estimates (24.6%) [participant range: 1.7%–45.0%]
• ±3 cm: 97 estimates (36.7%) [range: 3.3%–61.7%]
• ±4 cm: 114 estimates (43.2%) [range: 3.3%–73.3%]

• ±2 cm: 30 out of 60 (50.0%)
• ±3 cm: 49 out of 60 (81.7%), p<0.05
• ±4 cm: 57 out of 60 (95.0%), p<0.05

Conclusion

Clinician estimations of PICC-line length based on experience and anatomical reasoning show considerable variability and are frequently inaccurate. The findings of this study demonstrate that the formula significantly outperforms the average clinician estimate in terms of accuracy. A formula-driven approach, developed from empirical data and integrated into a web-based calculator, provides superior precision and reproducibility. This suggests that routine use of the calculator could improve patient outcomes by minimizing malposition-related complications and unnecessary repositioning. Further validation in larger and more diverse clinical settings is warranted.