Clinical Case Summary

Littoral Cell Angioma (LCA) is a rare splenic vascular tumor, first described in 1991. Although it is usually benign and clinically
non-specific, the outcome is not predictable, and some cases with malignant behavior have been described.
The pathogenesis of this lesion remains unknown, although a correlation with important immune disorders such as Crohn's disease (CD) is established.
We report the 5th described case of LCA in a patient affected by CD.
We describe the case of a 32-year-old patient diagnosed with CD stenosing dijuno-ileal and colic associated with splenomegaly (Dec 2011). He was treated with Adalimumab (Feb-Sep 2012, discontinued due to ineffectiveness), Infliximab (Oct 2012-Dec 2014, discontinued due to healing mucosa) and AZA since Dec 2013. For subsequent finding of splenic parenchymal lesions of difficult clinical-instrumental definition, in Jan 2023 splenectomy, digiunal resection (20cm) and digiuno-digiunal Michelassi strictureplasty (30cm) were performed.
Histological examination showed CD in a severe phase of activity with reactive lymphadenitis. In the splenic parenchyma there were circumscribed and demarcated areas consisting of blood exudates and an exudative/productive inflammatory infiltrate with histiocytic elements. Immunohistochemistry: CAM5.2-, CK7-, CDX-2-, CD68-PMG1+, CD1a-, CD207-. In light of the clinical- instrumental picture, histological preparations and immunohistochemical investigations, the morphological and immunophenotypical findings were compatible with splenic LCA.
LCA is often associated with immune diseases. Its diagnosis should be evoked in case of splenomegaly contextual to CD. The tumor may be related to the natural history of CD, and splenomegaly may be present at the time of diagnosis.
The entero- RM to establish the degree of CD activity could represent an opportunity in the study of the prevalence and natural history of this rare splenic neoplasia.

References

We will present the clinical case with RM, CT scan and PET immagines.

Clinical Case Summary

Splenic granulomas are a rare extraintestinal manifestation of Crohn’s disease (CD). In previously reported cases, the diagnosis was confirmed by biopsy. However, due to the increased risk of complications associated with biopsy, confirmation by imaging is desirable.
52-year-old male patient presented with a previously diagnosed, currently untreated CD.
Symptoms:

• Abdominal pain in the right lower quadrant for several weeks, postprandial
• Bowel movements: 4-6 times/day for a few weeks, liquid consistency
• No fever or weight loss
• Palpation: tenderness in the lower right abdomen

Findings:

• C-reactive protein and leukocytes: not elevated
• Calprotectin: 184ug/g. Stool culture and intestinal cytomegaly virus PCR biopsy negative
• Tuberculosis interferon-g release assay: negative
• Colonoscopy and magnetic resonance enterography (MRE): ileitis of terminal Ileum (Fig. 1)
• MRE: multiple T2w-hyperintense splenic lesions, increased in number since last imaging 2024 (Fig. 2)
• B-mode ultrasound: splenomegaly, round nodules of mixed echogenicity (Fig. 3)
• Contrast-enhanced ultrasound (CEUS): 1,5ml contrasting agent (sulphur hexafluoride microbubbles): arterial isoenhancement and late phase hypoenhancement of nodules (Fig. 3), atypical for haemangiomas
• 18-Fluorodeoxyglucose positron emission tomography with computed tomography: no uptake in the spleen (Fig. 4)

After excluding malignancy, abscesses, sarcoidosis, and infectious causes, the splenic granulomatous lesions represented an extraintestinal manifestation of CD. The patient was started on prednisolone, 60mg/day, resulting in marked clinical improvement in 4 days; with reduced abdominal pain and decreased stool frequency. Patient declined recommended maintenance therapy with ustekinumab.
This CEUS pattern is novel to CD and resembles previously reported enhancement patterns of inflammatory lesions. This is a rare reported case of splenic granulomas as an extraintestinal manifestation of CD diagnosed non-invasively by CEUS.

References

Figure 1: Colonoscopy shows 2-4mm aphthous ulcers in the terminal Ileum
Figure 2: (A)T2-Haste and (B)T2-STIR MRE depicting multiple hyperintense splenic lesions
Figure 3: B-mode (left) and CEUS (right) of the splenic lesions. (A) Nodules appear mixed in echogenicity, nodules show isoenhancement during the arterial phase. (B) Nodules exhibit hypoenhancement in the late phase
Figure 4: 18F-Fluorodeoxyglucose (FDG) positron emission tomography with computed tomography shows no uptake of FDG in the spleen
1. Castellani L, Sartini A, Marocchi M, Balbi T, Belluzzi A. Splenic granulomas in a patient with severe Crohn's disease associated with multiple extraintestinal manifestations. Inflamm Bowel Dis. 2011 Jun;17(6):E35-7. doi: 10.1002/ibd.21665. Epub 2011 Feb 11. PMID: 21319276.
2. Schulz C, von Arnim U, Kuester D, Fischbach F, Malfertheiner P. Milzbefall - seltene extraintestinale Manifestation bei Morbus Crohn [Splenic granulomas - rare extraintestinal manifestation of Crohn's disease]. Z Gastroenterol. 2010 Dec;48(12):1367-70. German. doi: 10.1055/s-0029-1245562. Epub 2010 Dec 1. PMID: 21125512
3. Safai Zadeh E, Prosch H, Ba-Ssalamah A, Findeisen H, Alhyari A, Raab N, Görg C. Contrast-enhanced ultrasound of the liver: basics and interpretation of common focal lesions. Rofo. 2024 Aug;196(8):807-818. English, German. doi: 10.1055/a-2219-4726. Epub 2024 Jan 4. PMID: 38176437

Disclosure

Travel grant from Johnson & Johnson

Introduction

Restorative proctocolectomy with ileal pouch–anal anastomosis (IPAA) performed in two or three stages has become the standard surgical treatment in patients with ulcerative colitis (UC) in which the medical management fails. It is a technically complex operation associated with significant morbidity that can be performed with a laparoscopic or robotic approach. Robotic colorectal surgery is gaining popularity because it overcomes many of the mechanical and optical disadvantages of laparoscopy, although limited data are available regarding its safety, efficacy, and costs.

Aims & Methods

This study compares the postoperative short-terms surgical outcomes of the two surgical approaches. Robotic and laparoscopic restorative proctocolectomy procedures with IPAA consecutively performed between June 2015 and May 2023 were retrospectively collected. Inclusion criteria was UC not responders to medical therapy. Exclusion criteria was conversion to open surgery.
Primary analyzed outcome were complications rate in the first month (30 days) according to Clavien Dindo Score and length of hospital stay (days). Statistical analysis was performed with a dedicated software (SPSS).

Results

A total of 54 patients with UC refractory to medical therapy were included, with 31 laparoscopic procedure and 23 robotic procedures.
Demographic data were comparable between two groups in terms of age (48.7 vs 45.7 years; p= 0.62), BMI (22,0 vs 23,7 Kg/m2; p= 1.311) and ASA score (p=0,72).Short-term complications defined as Clavien Dindo scores 2-3, (compared with score 1) were 13% in the robotic group and 31% in the laparoscopic group (RR= 0.27, 95% conf. interval. 0.11-1.06).
In particular, no high-grade Clavien-Dindo complications occurred in the robotic group while 3 events (grade 3) occurred in the laparoscopic group. No surgical intervention was needed in both groups.
Length of hospital stay was a mean of 7.3 days in lap-group and 6.3 days in rob-group with no statistical significativity (p=0,09).

Conclusion

This preliminary study concludes that robot-assisted surgery allows a reduction of short-term complications compared to laparoscopic approach. Moreover, robotic surgery allows a reduction of length of hospital stay although not statistically significant. Limits of the study were the retrospective design and the low numerosity of the sample, although comparable with the experiences already published.

Clinical Case Summary

Crohn’s disease (CD) is a multi-factorial immune-mediated inflammatory condition of the gastrointestinal tract (GIT). While CD-related granulomas typically occur within the GIT, they can appear in other areas such as lymph nodes, mesentery, peritoneum, liver, and lungs. This case highlights a rare instance of renal granuloma secondary to CD and discusses an effective treatment approach. An 18-year-old male presented to a GP with a one-year history of abdominal pain. There was no significant medical or family history. Physical examination was unremarkable. Laboratory findings showed raised white cell count, elevated erythrocyte sediment rate, and renal dysfunction. He was referred to a nephrologist who noted additional symptoms of diarrhoea and occasional per-rectal bleeding with no significant improvement in symptoms or renal function after complete cessation of non-steroidal anti-inflammatory drugs. Renal biopsy confirmed acute interstitial nephritis and granuloma, therefore he was referred to a gastroenterologist to rule out CD or sarcoid. Colonoscopy showed pancolitis with rectal sparing and segmental biopsies revealed chronic colitis with granulomas confirming CD. Fecal calprotectin was raised. Further investigations ruled out autoimmune disease, tuberculosis and sarcoidosis as causes for renal impairment. Treatment involved steroid tapering followed by intravenous infliximab induction and maintenance therapy with subcutaneous injections fortnightly. Renal function and GIT symptoms improved with infliximab monotherapy, though dose escalation to weekly infliximab injections was required at seven months due to loss of response from subcutaneous infliximab. At one year, colonoscopy showed endoscopic remission, with ongoing improvement in renal function (creatinine 194 to 111 umol/L) and resolution of pyuria. In conclusion, we present a rare case of CD-associated interstitial nephritis, showing a positive clinical response to infliximab monotherapy.

References

1. Lamb CA, Kennedy NA, Raine T, et al. British Society of Gastroenterology consensus guidelines on the management of inflammatory bowel disease in adults. Gut. 2019;68:s1-s106.
2. Eade MN, Cooke WT, Williams JA. Liver disease in Crohn's disease. Scand J Gastroenterol. 1971;6:199-204.
3. Whitehead R. Pathology of Crohn's disease. In: Kirsner JB, Shorter RG, eds. Inflammatory bowel disease. 2nd ed. Philadelphia: Lean and Febiger; 1980:296-310.

Disclosure

Nil