Amyloidosis presenting as postcricoid esophageal stricture

AT THE FOCAL POINT Lawrence J. Brandt, MD, Associate Editor for Focal Points

Amyloidosis presenting as postcricoid esophageal stricture

A 44-year-old woman presented with 3 months of progressive dysphagia to both solids and liquids with significant loss of weight. A peripheral blood smear showed a dimorphic (normocytic normochromic and microcytic hypochromic) anemia. Laboratory data included a hemoglobin level of 9.0 g/dL, hematocrit of 34%, and normal renal and liver biochemical test results except for a serum albumin level of 1.7 g/dL. EGD showed diffuse nodularity in the hypopharyngeal and postcricoid region with stricture (A). The vocal cord movements were normal. She underwent esophageal bougienage. Postdilation endoscopy showed similar nodular lesions in the upper esophagus, antrum of stomach,

and the second part of the duodenum. Biopsy specimens from all these affected areas of the upper GI tract were taken. Histology with hematoxylin and eosin stain showed extracellular pink hyaline material deposition (B), which was strongly positive with Congo red stain, confirming amyloidosis (C). No obvious cause for amyloidosis could be established.

180 GASTROINTESTINAL ENDOSCOPY Volume 71, No. 1 : 2010

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DISCLOSURE All authors disclosed no financial relationships relevant to this publication.

At the Focal Point

Raju S. S. Bhavani, MD, Sandeep Lakhtakia, MD, DM, Gastroenterology, Anuradha Sekaran, MD, DNB, Pathology, Manu Tandan, MD, DM, Nageshwar D. Reddy,

MD, DM, Gastroenterology, Asian Institute of Gastroenterology, Hyderabad, India doi:10.1016/j.gie.2009.08.013

Commentary First described by Rokitansky in 1842, amyloidosis is now known to result from the deposition of insoluble proteinacious material that is produced in association with a variety of diseases and that may involve one or multiple organs. In the GI tract, amyloid may appear as ulcers, nodules, or polypoid masses and may have ischemic, malabsorptive, hemorrhagic, or dysmotility presentations. Indeed, it joins syphilis, cytomegalovirus, and lupus as another great imitator. The disease often is diagnosed by biopsy when Congo red staining demonstrates amyloid fibrillar protein. The term Congo red was introduced at the 1885 Berlin West Africa Conference as the name for the first direct textile dye. At that time, the Congo River basin was an exotic new geopolitical and potentially profitable area; hence, the name had marketing cache´, just like another textile dye with an African name used in medicine: Sudan black. The inventor, Paul Bottiger, subsequently sold his patent rights for Congo red to a major German textile dyestuff company (AGFA) that ultimately merged with others to form the I.G. Farben Company. Congo red rarely is used today as a textile dye because it stains the fabrics of other garments when they are washed together. Unfortunately, there is no specific therapy for the disease, and median survival is only about a year. The take-home message here is to be aware of amyloid as a possibility even when you see what seems to be a familiar disease pattern, and especially when a patient has associated chronic inflammatory disease, multiple myeloma, or monoclonal gammopathy or is on hemodialysis. We often think of amyloid whenever a large tongue prevents the endoscope from being easily advanced into the posterior pharynx. Now when we enter the posterior pharynx and see nodules or upon seeing an esophageal stricture, we again should be reminded of Rokitansky’s contribution. Lawrence J. Brandt, MD Associate Editor for Focal Points

Balloon-assisted peroral cholangioscopy by using an 8.8-mm gastroscope for the diagnosis of Mirizzi syndrome A 50-year-old woman was referred for treatment of an obstructing gallstone in the common hepatic duct, found on MRCP (A). An ERCP was performed, revealing a 2.5-cm stone obstructing the common hepatic duct (B), with no opacification of the gallbladder. Attempts using mechanical lithotripsy were unsuccessful, so a biliary sphincterotomy

was performed, and a 10F plastic stent was placed for temporary decompression. The patient returned 1 week later for cholangioscopy. So that the duct could optimally be visualized, a 0.035-inch guidewire was advanced above the obstructing stone by using a therapeutic duodenoscope (TJF-160VF; Olympus America, Inc, Center Valley, Penn).

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