CASE 14439 Published on 04.02.2017

Angioneurotic oedema of the small bowel

Section

Abdominal imaging

Case Type

Clinical Cases

Authors

Fatmah Al Zeyoudi, Tamer Elholiby radiology Specialist, Anurag Jain Radiology Specialist, Inas Fouad Mikhail tropical medicine consultant

SKMC, radiology department, Abu Dhabi , United Arab Emirates;
Email:falzeyoudi@SEHA.AE

Patient

32 years, male

Categories

Area of Interest Abdomen ; Imaging Technique CT

Procedure Diagnostic procedure, Radiobiology ; Special Focus Oedema, Congenital ;

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Clinical History

A 32-year-old man presented to the emergency department with a 3-day history of severe epigastric pain associated with vomiting and constipation. A blood test at presentation showed elevated CRP of 20.49 mg/L, high WBC of 13.9 x 10^9/L. Further investigations revealed that C1 Esterase inhibitor was 53 mg/L.

Imaging Findings

A CT abdomen with iodine showed oedema of the bowel wall, stomach antrum and duodenum, with surrounding free fluid. There was enhancement of the mucosa and serosa as well as fluid accumulation in the bowel lumen. The mesenteric vessels were prominent.
No bowel loop obstruction or pneumoperitoneum was observed.

Discussion

Angioedema is a noninflammatory disease characterized by episodes of increased capillary permeability resulting in extravasation of intravascular fluid and subsequent angioedematous swelling. Angioedema was first described by J.L. Milton in 1876 and first named angioneurotic oedema by Quinckein in 1882 [1, 2].

There are various cases of angioedema, including hereditary angioedema, acquired C1 esterase inhibitor deficiency (like in our case), drugs and food. Some cases are idiopathic [3, 4].
It is characterized by localized and transient edematous swelling of superficial regions such as the face, upper airways, genitals and limbs [5]. Angioedema, however, may affect any part of the body, and intra-abdominal involvement may occur with or without cutaneous or respiratory involvement [3, 4].

Angioedema of the upper airways may result in acute respiratory distress, airway obstruction, and asphyxia [5, 6]. Gastrointestinal tract involvement sometimes mimics acute abdomen resulting in unnecessary laparotomy [2] or rarely causes potentially life threatening hypovolemic shock [5, 7]. Gastrointestinal complaints may be the initial symptoms in patients with angioedema, as in our case. Angioedema is seen during infancy or early adolescence, although it may occur until the sixth decade [7]. Clinical diagnosis of angioedema of the gastrointestinal tract is rarely considered until repeated attacks occur, like in our case.

Radiographic features during the acute phase may show multiple dilated small bowel loops with regularly thickened mucosal folds, wall thickening. Air-fluid level may also be seen [8, 9].
Ultrasound usually reveals mucosal thickening, edematous bowel wall with increased intraluminal fluid and decreased motility, and it may demonstrate peritoneal fluid in dependent areas of the abdomen [10, 11].
Enhanced CT usually demonstrates segmental or diffuse thickening of the small bowel wall, low attenuation of the edematous submucosa, and markedly enhanced thickened mucosa and serosa [12].

Diagnosis of angioedema is mainly clinical, nevertheless imaging findings can help greatly, warranting further clinical workup and avoiding unnecessary surgical laparotomy.

Differential Diagnosis List

Angioedema of the small bowel secondary to C1 esterase inhibitor deficiency

Ischemia

Shock bowel

Henoch Schonlein purpura (vasculitis)

Final Diagnosis

Angioedema of the small bowel secondary to C1 esterase inhibitor deficiency

References

[1] Quinke HE (1882) Über akutes umschriebenes Hautödem. Monatsh Prakt Dermatol 1:129–131

[2] de Graaff LC, van Essen M, Schipper EM, Boom H, Duschek EJ (2012) Unnecessary surgery for acute abdomen secondary to angiotensin-converting enzyme inhibitor use. Am J Emerg Med 30(8):1607–1612 (PMID: 22867837)

[3] Weinstock LB, Kothari T, Sharma RN, Rosenfeld S (1987) Recurrent abdominal pain as the sole manifestation of hereditary angioedema in multiple family members. Gastroenterology 93:1116–1118 (PMID: 3653633)

[4] Polger M, Kuhlman JE, Hanssen FC III, Fishman EK (1988) Computed tomography of angioedema of small bowel due to reaction to radiographic contrast medium. J Comput Assist Tomogr 12: 1044–1046 (PMID: 3183111)

[5] Cohen N, Sharon A, Golik A, Zaidenstein R, Modai D (1993) Hereditary angioneurotic edema with severe hypovolemic shock. J Clin Gastroenterol 16:237–239 (PMID: 8505498)

[6] Ebo DG, Stevens WJ, Bosmans JL (1997) An adverse reaction to angiotensin-converting enzyme inhibitors in a patient with neglected C1 esterase inhibitor deficiency. J Allergy Clin Immunol 99:425–426 (PMID: 9058701)

[7] Talavera A, Larraona JL, Ramos JL, et al (1995) Hereditary angioedema: an infrequent cause of abdominal pain with ascites. Am J Gastroenterol 90:471–474 (PMID: 7872288)

[8] Eck SL, Morse JH, Janssen DA, Emerson SG,Markovitz DM (1993) Angioedema presenting aschronic gastrointestinal symptoms. Am J Gastroenterol 88:436–439 (PMID: 8438855)

[9] Ciaccia D, Brazer SR, Baker ME (1993) Acquired C1 esterase inhibitor deficiency causing intestinal angioedema: CT appearance. AJR Am J Roentgenol 161: 1215–1216 (PMID: 8249728)

[10] Sofia S, Casali A, Bolondi L (1999) Sonographic findings in abdominal hereditary angioedema. J Clin Ultrasound 27:537-40 (PMID: 10525217)

[11] Burghardt W, Wernze H. (1987) Abdominal sonography in hereditary angioneurotic edema. A contribution to the early diagnosis of a disease of interdisciplinary significance. Burghardt W, et al. Ultraschall Med 8(6):278-82 (PMID: 3324342)

[12] Wakisaka M, Shuto M, Abe H, Tajima M, Shiroshita H, Bandoh T,Arita T, Kobayashi M, Nakayama T, Okada F, Mori H, Uemura N (2008) Computed tomography of the gastrointestinal manifestation of hereditary angioedema. Radiat Med 26:618-21 (PMID: 19132494)

Case information

URL:	https://eurorad.org/case/14439
DOI:	10.1594/EURORAD/CASE.14439
ISSN:	1563-4086

License

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Figure 1

CT abdomen and pelvis with IV contrast

Enhanced CT - Coronal reformat - soft tissue window reveals mural thickening and submucosal oedema of the gastric antrum with surrounding free fluid and prominent mesenteric vessels

Figure 2

CT abdomen

Enhanced CT abdomen - axial view - soft tissue window

Figure 3

CT abdomen

Enhanced CT abdomen - coronal reformat - soft tissue window - clear delineation of different layers of duodenal wall and fluid accumulation within dilated duodenum. There is surrounding free fluid.

Figure 4

Free fluid