Systemic amyloidosis frequently involves the small intestine. However, its association with diverticular disease has been seldom reported to date. To draw attention to this rare but potentially harmful association, we herein present an additional case of small bowel diverticular disease associated with amyloidosis.
Diverticular disease is seldom observed in the small bowel [
Jejunoileal diverticula result from mucosal and submucosal herniation through the muscular layer of the bowel wall in places of minor resistance to the intraluminal pressure. In most cases the etiology is unclear. The pathogenesis is probably related to abnormalities of the muscular layers or the myenteric plexus that cause intestinal dyskinesia resulting in intestinal contractions which generate increased intraluminal pressure, favoring diverticula formation through the weakest point of the bowel [
Amyloidosis (derived from immunoglobulin light chain [AL, formerly termed “primary”] or derived from amyloid serum A protein [SAA, formerly termed “secondary”]) commonly affects the small intestine and it is often associated with dysmotility disorders or dyskinesia of the small and large bowel. However, small bowel amyloidosis is only very rarely associated with small bowel diverticular formation with only two cases described so far [
A 91-year-old man with a medical history of multiple myeloma and respiratory insufficiency was admitted with a 24-hour history of nausea and colicky abdominal pain located in the epigastrium. He also reported constipation for the past 3 days with only one bowel movement and a recent episode of enterorrhagia. On admission, clinical examination revealed generalized mild abdominal tenderness, with local guarding to the left abdomen and rebound tenderness. Mild bowel sounds were present with high frequency. Rectal examination revealed an empty rectum. The blood count revealed leukocytosis (18,500/mm3) with left shift (90% polymorphonuclears) and elevated C-reactive protein of 11.1 mg/dL. After 3 hours, the patient became febrile, tachycardic (110 beats per minute), and hypotensive (80/50 mmHg) with diffuse abdominal tenderness, rebound, and guarding. An ultrasound examination of the abdomen revealed dilated small bowel loops with scattered diverticula and free fluid in the abdominal cavity. He underwent an emergency laparotomy at which 197 cm of ischemic small bowel was identified and excised. Despite postoperative therapy in the intensive care unit, the patient developed severe coagulopathy and acidosis and finally died 3 days following surgery. Permission for autopsy was not granted.
Gross examination showed a segment, 197 cm in length, of small intestine with a grey, smooth, and focally hemorrhagic serosal surface. The mucosa was dark and purple as a result of extensive hemorrhages and revealed superficial ulceration. Sections of the bowel wall displayed numerous diverticular structures.
Histologic sections of the small intestinal wall displayed ischemia-related changes, mainly mucosal, numerous diverticula and deposits of a hyaline, eosinophilic, and homogeneous material on blood vessel walls and in the muscularis propria, submucosa and muscularis mucosa layers (Figures
(a) Whole mount section of the small intestine showing diverticular disease (hematoxylin and eosin stained section, magnification
Amyloidosis is characterized by the extracellular deposition of diverse types of amyloid proteins. These proteins have a typical fibrillary appearance under electron microscopy. Their arrangement in a beta-pleated sheet formation provides the binding sites for the Congo red dye used for the diagnosis as it conveys typical apple-green birefringence when examined under polarized light. Yet, despite the morphological and histological uniformity of the amyloid protein, it is clear that several biochemical types of the amyloid protein exist. The most common form of amyloidosis is amyloidosis AL (amyloidosis derived from monoclonal immunoglobulin light chain, formerly termed “primary”), which is associated with plasma cell dyscrasias, including multiple myeloma. The second most common form is systemic (AA) amyloidosis, caused by deposition of fragments of the acute phase reactant, serum amyloid A protein. This type is associated with chronic inflammatory diseases and infections, including tuberculosis, familial Mediterranean fever, rheumatoid arthritis, and Crohn’s disease [
Gastrointestinal (GI) involvement is common in all types of amyloidosis (primary and secondary), ranging from 85% to 100% [
In our case, the extensive deposition of amyloid in the small intestine caused ischemic changes due to blood vessel stenosis. However, it also led to diverticula formation due to amyloid deposition in the muscular layers. Patel et al. [
Even though amyloidosis is frequently seen in the small bowel, it represents an extremely rare cause of diverticular disease with only two cases reported to date [
In conclusion, we have presented a rare case of diverticulosis and ischemia of the small bowel caused by massive AL amyloid deposition to draw attention to the possibility of these associations. It should be stressed that both ischemia and diverticular disease are serious events. They are of special concern because of their potential risk for infection, perforation, bleeding, and obstruction, all of which are severe complications that can compromise patient lives.
The authors declare that they have no conflict of interests regarding to the publication of this paper.