Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
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Case Report

Volume 12, Number 8, August 2021, pages 332-337

Neuromuscular and Vascular Hamartoma: Starting Point in Crohn’s Disease?

Figures

Figure 1.
Figure 1. CT abdomen and pelvis with intravenous contrast showing multiple dilated small bowel loops with fecalization, interloop fluid and transition point in the right lower quadrant (colon not completely collapsed, and findings likely consistent with early small bowel obstruction). CT: computed tomography.
Figure 2.
Figure 2. MRI enterography with and without intravenous contrast showing resolution of small bowel obstruction, focal, short segment luminal narrowing of an ileal loop in the lower mid abdomen in the region of previously seen transition point, and unremarkable appendix. MRI: Magnetic resonance imaging.
Figure 3.
Figure 3. Ectatic small bowel on the laparoscopic image taken during the resection (black square showing entire ectatic segment).
Figure 4.
Figure 4. (a) Stricture area showing surface mucosal ulceration (H&E, × 40). (b) Dense mixed inflammatory infiltrate in the lamina propria (H&E, × 100). (c, d) Thickened and disorganized muscularis mucosa and submucosa, prominent vasculature, prominent nerve bundles and clusters of ganglion cells. H&E: hematoxylin and eosin stain.
Figure 5.
Figure 5. (a) Desmin immunostain highlights the disorganized bundle of smooth muscle. (b) S-100 highlights prominent ganglion cells and nerve fibers within the wall of intestine.

Tables

Table 1. Biomarkers for Inflammatory Bowel Disease (IBD)
 
AntibodyResult
ASCA: anti-Saccharomyces cerevisiae antibody; Ig: immunoglobulin; Anti-Ompc: anti outer membrane porin C; Anti-CBir: anti-bacterial flagellin antibody; pANCA: perinuclear anti-neutrophil cytoplasmic antibody; ICAM: intracellular adhesion molecule; VCAM: vascular cell adhesion molecule; VEGF: vascular endothelial growth factor; CRP: C-reactive protein.
ASCA IgANegative
ASCA IgGNegative
Anti-OmpC IgANegative
Anti-CBir 1 IgGNegative
Anti-FlaX IgGNegative
IBD-specific pANCANegative
ICAM-1Negative
VCAM-1Negative
VEGFNegative
CRP13.2 mg/L

 

Table 2. Twenty-Eight Cases of NMVH so far in the Literature Describing the Authors, Year of Publication With Patient Characteristics and Comorbidities [1, 2, 3, 5-9]
 
Author and yearAgeSexSymptomOriginComorbidity
NMVH: neuromuscular and vascular hamartoma; F: female; M: male; ND: not detected.
1Fernando et al, 1982 [1]30FAnemiaSmall intestine
2Fernando et al, 1982 [1]36FAbdominal pain, vomitingSmall intestine
3Smith et al, 1986 [4]51FAbdominal pain, vomitingSmall intestine
4Shepherd et al, 1987 [7]34FAbdominal pain, vomitingSmall intestineCrohn’s disease
5Shepherd et al, 1987 [7]58MAbdominal pain, diarrheaSmall intestineCrohn’s disease
6Shepherd et al, 1987 [7]73FAbdominal painSmall intestine
7Shepherd et al, 1987 [7]63FAbdominal painSmall intestine
8Kwasnik et al, 1989 [10]91MNDSmall intestine
9Salas et al, 1990 [11]NDNDNDSmall intestine
10Cortina et al, 1999 [12]73MBowel obstructionSmall intestine
11Cortina et al, 1999 [12]76MBowel obstructionSmall intestine
12De Sanctis et al, 2001 [13]76FAnemiaSmall intestine
13Scintu et al, 2001 [14]50MBowel obstructionSmall intestine
14Shiomi et al, 2002 [15]76FAsymptomaticCecum
15Company et al, 2005 [16]76FNDSmall intestine
16Theodosiou et al, 2009 [17]60MAbdominal painSmall intestine
17Krishnamurthy et al, 2010 [6]32MAbdominal painSmall intestine
18Kaplan et al, 2013 [18]12FAbdominal pain, vomitingSmall intestine
19Ren et al, 2014 [19]NDFAbdominal pain, anemiaSmall intestine
20Ren et al, 2014 [19]NDNDNDSmall intestine
21Setaffy et al, 2015 [20]NDNDNDSmall intestine
22Crothers et al, 2014 [2]73FNDSmall intestine
23Liu et al, 2015 [21]27FAbdominal pain, diarrheaSmall intestine
24Elstner et al, 2016 [8]59FAbdominal pain, vomitingSmall intestineCrohn’s disease
25Caruso et al, 2018 [9]58MAbdominal pain, anemiaSmall intestine
26Pattnaik et al, 2019 [5]45MNDSmall intestine
27Sasaki et al, 2020 [3]60MAbdominal painAppendix
28Dhruv et al, 2021 (our case)44FAbdominal painSmall intestine