Danielle Alkov, Harvard Medical School Year III Gillian Lieberman, MD

 HPI

• 61 year old man with 2 months of worsening

nausea, vomiting, diarrhea and crampy abdominal pain precipitated by PO intake • He lost 35 pounds over this period

2

 Celiac

disease

• Diagnosed 5 years prior to admission based on

elevated tTG levels and an abnormal duodenal biopsy  Hypertension

3

The patient underwent abdominal CT with contrast to evaluate his symptoms

4

Axial CT image with contrast

Bowel wall thickened to 4 mm

Image from BIDMC PACS 5

Coronal CT image with contrast

Image from BIDMC PACS 6

Coronal CT image with contrast

Lymph node measuring 13.2 mm

Image from BIDMC PACS 7

Coronal CT image with contrast

Misty mesentery has a hazy appearance and is an indication of mesenteric edema

Image from BIDMC PACS 8

Please continue for a general review of celiac disease

9



Most common among Caucasians of northern European descent



As prevalent as 1:300 to 1:500

Not T, Horvath K, Hill ID, Partanen J. Celiac disease risk in the USA: high prevalence of antiendomysium antibodies in healthy blood donors. Scandinavian Journal Gastroenterology 1998; 33(5):494-8

10

 Autoimmune

disorder of the small intestine that occurs in response to gliadin, a protein in gluten-containing foods

 Improves

with cessation of gluten consumption

11

 Chronic

or recurrent diarrhea  Malabsorption • Vitamin A, D, E, K, B12, folic acid, iron deficiency  Weight

loss  Abdominal distension or bloating

12

     

Dermatitis herpetiformis Down syndrome Selective IgA deficiency Type 1 diabetes mellitus Thyroid disease Atrophic glossitis

Images: Kumar, V, Abbas, A, et al. Robbins and Cotran Pathologic Basis of Disease, 7th Edition. Saunders 2004. and Scully C. Oral Medicine Practice- Update for the dental practitioner. British Dental Journal 2005; 199, 639 - 645

13

 Serologic tests: • IgA anti tissue transglutaminase (tTG) • IgA endomysial antibody • Antigliadin antibody (no longer used routinely due to

low sensitivity and specificity)  Test while on a gluten-containing diet  Small bowel biopsy of the second or third

portion of the duodenum  May consider HLA haplotype testing for HLA DQ2 or HLA DQ8 which are present in 99% of celiac disease patients Hadithi M, von Blomberg BM, Crusius JB. Accuracy of serologic tests and HLA-DQ typing for diagnosing celiac disease. Annals of Internal Medicine 2007;147(5):294-302

14

Tortora, GJ, Grabowski, SR, Principles of Anatomy and Physiology, 8th Edition. John Wiley & Sons Inc. 1998

15

 mucosal

inflammation  villous atrophy  crypt hyperplasia Celiac disease:

Normal:

Images: Kumar, V, Abbas, A, et al. Robbins and Cotran Pathologic th

16

 mucosal

inflammation

Celiac disease:

Normal:

Images Kumar, V, Abbas, A, et al. Robbins and Cotran Pathologic th

17

 mucosal

inflammation  villous atrophy Celiac disease:

Normal:

Images Kumar, V, Abbas, A, et al. Robbins and Cotran Pathologic th

18

 mucosal

inflammation  villous atrophy  crypt hyperplasia Celiac disease:

Normal:

Images: Kumar, V, Abbas, A, et al. Robbins and Cotran Pathologic th

19

 Barium

studies

 Abdominal

CT

 Abdominal

MRI

20

Reversed

jejunoileal fold pattern Small bowel wall thickening Jejunal and Ileal fold thickening Enlarged lymph nodes Vascular engorgement

21

Let’s continue to a menu of examples that demonstrate the radiologic findings in celiac disease

22



As inflammation occurs in the jejunum, mucosal folds are lost and absorptive surface area is decreased



Normally, the jejunum displays above 5 folds per inch, but in celiac disease, this number is decreased to below 5 folds per inch



In order to compensate for the loss of absorptive surface area, the ileum increases the number of mucosal folds



Therefore, the ileum will display above 5 folds per inch



This phenomenon is known as reversed jujunoileal fold pattern and can be 100% specific for the diagnosis of celiac disease

Soyer, P, Boudiaf, M, Dray, X, et al. CT Enteroclysis Features of Uncomplicated Celiac Disease. Radiology: 2009; 253: (2): 416424

23

Reversed jejunoileal fold pattern

24

Decrease in jejunal folds per inch

Increase in ileal folds per inch

Barium enema Image: Rubesin, S, Herlinger, H et al. Adult Celiac Disease and its Complications. RadioGraphics 1989; 9(6): 1045-1066

25

Coronal MR Enterography

Decreased jejunal folds

Image from 26 BIDMC PACS

Bowel wall thickening

27

Axial Ct image with contrast

Wall thickened above 3 mm

Image from 28 BIDMC PACS

Small bowel fold thickening

29

 Normal

fold thickness is 3 mm or less

A

2009 CT study demonstrated 66% of celiac patients have jejunal fold thickening and 64% have ileal fold thickening

Soyer, P, Boudiaf, M, Dray, X, et al. CT Enteroclysis Features of Uncomplicated Celiac Disease. Radiology: 2009; 253: (2): 416-424

30

Mucosal folds thicken when they are infiltrated. Below is a list of substances that can infiltrate the walls of small bowel:

Edema

Lymph

Blood

Cells (lymphoma, carcinoid)

Pus

Stuff (amyloid, glycogen) 31

Barium followthrough examination

Small bowel folds over 3 mm thick Image: Masterson, JB, Sweeney EC. The role of small bowel follow-through examination in the diagnosis of coeliac disease. British Journal of Radiology 1976; 49, 660-664

32

Coronal CT Enteroclysis

Jejunal folds greater than 3 mm thick Image: Lomoschitz, F, Schima, W, Schober, E et al. Enteroclysis in adult celiac disease: diagnostic value of specific radiographic features. European Radiology 2003; 13:890–896

33

Enlarged lymph nodes

34

lymph nodes are considered enlarged when the short axis measures above 10 mm

Lymph node measuring 13.2 mm Coronal CT image with contrast

Although lymph node enlargement is generally due to benign mesenteric lymphadenopathy in celiac patient, because of the risk of intestinal lymphoma, it is important to closely follow this finding

Image from 35 BIDMC PACS

Vascular engorgement

36

Coronal TrueFISP MRI

Enlarged mesenteric vessels along mid-length of the mesentery, above 3 mm in diameter Image: Tomei, E, Richard, L, Semelka, C. Adult Celiac Disease: What Is the Role of MRI?

37

 Splenic

atrophy  Small bowel luminal dilatation  Intussusception  Submucosal fat  Fluid effusion

38

 Celiac

disease is not a radiologic diagnosis

 Celiac

symptomatolgy is often nonspecific leading to imaging before diagnosis

 Radiographic

studies can lead to a consideration of the diagnosis or provide alternate diagnoses 39

Soyer, P, Boudiaf, M, Dray, X, et al. CT Enteroclysis Features of Uncomplicated Celiac Disease. Radiology: 2009; 253: (2): 416-424

40

 After

a two week hospitalization, he was discharged home on TPN with minor improvement in symptoms

 He

continued to decline and returned to the hospital a few weeks later where endoscopy with biopsies was performed

 Shortly

after the endoscopy, he began experiencing severe abdominal pain 41

Abdominal plain film

Please evaluate the image and then continue on to the next slide for the answer

Image from 42 BIDMC PACS

Abdominal plain film

Pneumoperitoneum, free air under the diaphragm

Image from BIDMC PACS

43

Axial CT image with contrast

Please evaluate the image and then continue on to the next slide for the answer

Image from 44 BIDMC PACS

Axial CT image with contrast

Pneumoperitoneum, free air under the diaphragm

Image from 45 BIDMC PACS

Axial CT image with contrast

Can you identify the boxed ligament on this axial CT image?

Image from 46 BIDMC PACS

Axial CT image with contrast

The falciform ligament

Image from 47 BIDMC PACS

 He

was taken to surgery for an exploratory laparotomy with small bowel resection

 In

the proceeding days, he was found to have fascial dehiscence and underwent a second exploratory laparotomy

 He

later developed psuedomonas pneumonia and passed away in ICU 48

  

 

    

Hadithi M, von Blomberg BM, Crusius JB. Accuracy of serologic tests and HLA-DQ typing for diagnosing celiac disease. Annals of Internal Medicine 2007;147(5):294-302 Kumar, V, Abbas, A, et al. Robbins and Cotran Pathologic Basis of Disease, 7th Edition. Saunders 2004. Lomoschitz, F, Schima, W, Schober, E et al. Enteroclysis in adult celiac disease: diagnostic value of specific radiographic features. European Radiology 2003; 13:890–896 Masterson, JB, Sweeney EC. The role of small bowel follow-through examination in the diagnosis of coeliac disease. British Journal of Radiology 1976; 49, 660-664 Not T, Horvath K, Hill ID, Partanen J. Celiac disease risk in the USA: high prevalence of antiendomysium antibodies in healthy blood donors. Scandinavian Journal Gastroenterology 1998; 33(5):494-8 Rubesin, S, Herlinger, H et al. Adult Celiac Disease and its Complications. RadioGraphics 1989; 9(6): 1045-1066 Scully C. Oral Medicine Practice- Update for the dental practitioner. British Dental Journal 2005; 199, 639 - 645 Soyer, P, Boudiaf, M, Dray, X, et al. CT Enteroclysis Features of Uncomplicated Celiac Disease. Radiology: 2009; 253: (2): 416-424 Tomei, E, Richard, L, Semelka, C. Adult Celiac Disease: What Is the Role of MRI? Journal of Magnetic Resonance Imaging 2006; 24:625– 629 Tortora, GJ, Grabowski, SR, Principles of Anatomy and Physiology, 8th Edition. John Wiley & Sons Inc. 1998.

49

 Monica

Agarwal, MD  Martin Smith, MD  Gillian Lieberman, MD  Emily Hanson  Deborah Levy, MD

50