J Clin Med Res

Journal of Clinical Medicine Research, ISSN 1918-3003 print, 1918-3011 online, Open Access

Article copyright, the authors; Journal compilation copyright, J Clin Med Res and Elmer Press Inc

Journal website http://www.jocmr.org

Case Report

Volume 9, Number 7, July 2017, pages 654-658

Gastrointestinal Tract Amyloidosis Presenting With Pneumatosis Intestinalis

Figure 1. Non-contrast abdominal CT demonstrating widespread colonic distension and air-fluid levels along with numerous areas of submucosal gas in the colonic wall.

Figure 3. Congo red stain of colon tissue showing amyloid deposits within the submucosa (top) and illustrating apple-green birefringence under polarized light (bottom).

**Table 1.** Characteristics of Patients With Pneumatosis Intestinalis From Amyloidosis
	Yanamoto et al [8]	Khalid et al [5]	Pearson et al [7]	Current study
Age, gender	63-year-old male	46-year-old male	76-year-old male	86-year-old male
Clinical presentation	Nausea, vomiting, diarrhea, and 10-year history of rheumatoid arthritis	Diffuse abdominal pain, melena, weight loss, dyspepsia	Postprandial bloating, periumbilical abdominal pain, weight loss	Abdominal pain, distention, and constipation
Radiographic studies	CT abdomen/pelvis: gas pockets in portal venous system, pancreas, gut wall, and free peritoneal air	KUB: air fluid levels, free air under right hemidiaphragm. CT abdomen/pelvis: extensive pneumoperitoneum with focal dilation of small bowel loop.	KUB: pneumoperitoneum and pneumatosis intestinalis of small bowel. CT abdomen: pneumoperitoneum, pneumatosis intestinalis of small bowel, and gas in portal venous system.	CT scan showed diffuse colonic distension with submucosal gas pockets and numerous polypoid mucosal lesions with bowel wall thickening but no portal venous air or free intra-abdominal air. There was no whirl sign.
Diagnosis	Secondary AA amyloidosis of GIT associated with rheumatoid arthritis. Hepatic portal vein gas and pneumatosis intestinalis.	Amyloidosis of GIT, sub-type unknown	IgG kappa multiple myeloma. Amyloidosis of GIT sub-type unknown.	Lambda light chain multiple myeloma. AA amyloidosis and ATTR amyloidosis of GIT.
Outcome	Pneumatosis intestinalis resolved with conservative management. Long-term outcome unknown.	Exploratory laparotomy showed no bowel perforation. Patient recovered with conservative management. Long-term outcome unknown.	Exploratory laparotomy with 4 cm small bowel resection. Died 15 months after surgery from recurrent small bowel obstruction.	Symptoms resolved with conservative management

Table 1. Characteristics of Patients With Pneumatosis Intestinalis From Amyloidosis

Yanamoto et al [8]

Khalid et al [5]

Pearson et al [7]

Current study

Age, gender

63-year-old male

46-year-old male

76-year-old male

86-year-old male

Clinical presentation

Nausea, vomiting, diarrhea, and 10-year history of rheumatoid arthritis

Diffuse abdominal pain, melena, weight loss, dyspepsia

Postprandial bloating, periumbilical abdominal pain, weight loss

Abdominal pain, distention, and constipation

Radiographic studies

CT abdomen/pelvis: gas pockets in portal venous system, pancreas, gut wall, and free peritoneal air

KUB: air fluid levels, free air under right hemidiaphragm.
CT abdomen/pelvis: extensive pneumoperitoneum with focal dilation of small bowel loop.

KUB: pneumoperitoneum and pneumatosis intestinalis of small bowel.
CT abdomen: pneumoperitoneum, pneumatosis intestinalis of small bowel, and gas in portal venous system.

CT scan showed diffuse colonic distension with submucosal gas pockets and numerous polypoid mucosal lesions with bowel wall thickening but no portal venous air or free intra-abdominal air. There was no whirl sign.

Diagnosis

Secondary AA amyloidosis of GIT associated with rheumatoid arthritis.
Hepatic portal vein gas and pneumatosis intestinalis.

Amyloidosis of GIT, sub-type unknown

IgG kappa multiple myeloma.
Amyloidosis of GIT sub-type unknown.

Lambda light chain multiple myeloma.
AA amyloidosis and ATTR amyloidosis of GIT.

Outcome

Pneumatosis intestinalis resolved with conservative management.
Long-term outcome unknown.

Exploratory laparotomy showed no bowel perforation. Patient recovered with conservative management.
Long-term outcome unknown.

Exploratory laparotomy with 4 cm small bowel resection.
Died 15 months after surgery from recurrent small bowel obstruction.

Symptoms resolved with conservative management