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Lymphangiomas (LA) of the gastrointestinal tract: three case studies of lesions involving stomach, small bowel, and colon
S172
Abstracts
Microscopic examination of biopsy specimen showed necrotic material with numerous neutrophils consistent with brain abscess. Culture of the aspirate was negative. After stereotactic brain biopsy was done and while the patient was on intravenous antibiotics, there was improvement of her neurologic status. Serial magnetic resonance imaging of the brain showed resolution of the brain lesion. Two months after the initial presentation, colonoscopy was done showing scattered diverticuli in the sigmoid with prominent hyperemic folds and narrowing of the distal sigmoid. After the patient completed the course of antibiotics she was discharged home without any sequelae. This case illustrates that serious complications can occur even in a mild case of diverticulitis. Aggressive and early management of diverticulitis may prevent such complications from occuring.
522 LIVER TRANSPLANTATION FOR CHOLESTEROL ESTER STORAGE DISEASE Joseph E. Cassara, M.D., Neeraj K. Sachdeva, M.D., Michael W. Fried, M.D., Steven L. Zacks, M.D. and Roshan Shrestha, M.D.*. Department of Medicine, University of North Carolina, Chapel Hill, NC. Purpose: Cholesterol Ester Storage Disease(CESD) is a rare autosomal recessive disorder leading to lysosomal storage of triacylglycerols and cholesterol esters(CE) in hepatocytes and other tissues. This is due to a deficiency in lysosomal acid lipase, an enzyme necessary for cellular degradation of plasma low density lipoproteins. Orthotopic liver transplantation(OLT) offers a potential cure for CESD as the new liver replenishes the lysosomal acid lipase stores required for normal liver function. We describe a case of orthotopic liver transplantation for CESD. A 25 year– old white female diagnosed with CESD and started on treatment with lovastatin 15 years prior, presented with a massive upper gastrointestinal bleed. The patient reported an unremarkable course over the previous 15 years. An esophagogastroduodenoscopy revealed esophageal varices and signs of portal hypertensive gastropathy. She subsequently underwent a transjugular intrahepatic portosystemic shunt. The patient underwent an OLT. The grossly cirrhotic explanted liver weighed 1400 grams. Histopathology revealed massive accumulation of CE in lysosomes. Subsequently, she did remarkably well and was discharged two weeks post surgery. One year post– operatively the patient is well with stable graft function and a normal cholesterol level. Patients with CESD often have a relatively benign course and survive well into adulthood. Extrahepatic organ involvement has been reported but generally does not appear to cause significant tissue injury. However a subset of CESD patients develop cirrhosis early in the course of their disease and manifest portal hypertension and its complications. OLT offers a potential cure for CESD as the new liver replenishes the lysosomal acid lipase stores required for normal functioning. This case is an example of the progressive nature of the disease and sudden decline in condition seen in patients with CESD from liver failure after many preceding years of an uneventful course. OLT has been shown to improve hepatocellular metabolism and reduce complications from cirrhosis. In summary, we report the fourth case, in the english literature, of end stage liver disease with a life–threatening complication due to CESD successfully treated with OLT. Liver transplantation provides the best option for long term solution in patients with decompensated liver disease due to Cholesterol Ester Storage Disease.
523 LYMPHANGIOMAS (LA) OF THE GASTROINTESTINAL TRACT: THREE CASE STUDIES OF LESIONS INVOLVING STOMACH, SMALL BOWEL, AND COLON Mahmoud M. Yousfi, M.D., Kevin O. Leslie, M.D., Jacques Heppell, M.D. and Coung Nguyen, M.D.*. Gastroenterology & Hepatology, Mayo Clinic, Scottsdale, AZ; Pathology, Mayo Clinic, Scottsdale, AZ and Surgery, Mayo Clinic, Scottsdale, AZ.
AJG – Vol. 97, No. 9, Suppl., 2002
Purpose: LA are localized collections of benign lymphatic channels that rarely involve the GI tract. When visualized endoscopically, they appear as submucosal cystic lesions and often do not produce symptoms. Symptomatic patients may present with GI bleeding, abdominal pain, or obstruction. Advances in endoscopic tools have improved our ability to detect these lesions. We report three cases of cystic LA involving the stomach, small intestine, and colon diagnosed with endoscopic ultrasound (EUS) and video capsule endoscopy (VCE). Case reports. Case 1: A 78 YOM referred for evaluation of a submucosal mass in the transverse colon seen on previous colonoscopies and abdominal CT scan. The patient had no GI symptoms but was concerned because the lesion grew in size since his initial colonoscopy. EUS revealed a 3– cm soft compressible submucosal mass with normal overlying mucosa in the midtransverse colon. The lesion was anechoic containing fluid with internal septation. Mucosal biopsies were nondiagnostic and the patient elected to proceed with surgery. Histology revealed characteristic features of LA. Case 2: A 93 YOM underwent laproscopic–assisted cecal resection of a large cecal polyp found on colonoscopy. He presented 2 months later with obscure GI bleeding with nondiagnostic routine upper and lower endoscopies. VCE revealed mid small bowel mass. This was confirmed with intra– operative endoscopy and partial small bowel resection was performed. Histology revealed a benign LA. Case 3: A 26 YOM referred for evaluation of a fundic cystic mass seen on an upper endoscopy performed for evaluation of severe anemia (hemoglobin of 5 g/dL). EUS revealed a 1– cm polypoid lesion in fundus, which appeared to emanate from the mucosal layer with anechoic features and clear separation from the deep mucosa. Using snare polypectomy technique and normal saline injection submucosally, the lesion was resected. Histology revealed a benign cystic LA. Conclusions: LA of the GI tract should be part of the differential of cystic submucosal lesions. EUS is useful for diagnosis and typically reveals anechoic septated lesions confined to the submucosa with intact underlying muscularis propria. No treatment is necessary in asymptomatic patients. However, endoscopic or surgical resection may be performed for symptomatic lesions
524 WALDENSTROM’S MACROGLOBULINEMIA PRESENTING AS NEW–ONSET ASCITES Elena Stoffel, M.D., David Spigel, M.D. and Norman Grace, M.D., FACG*. Division of Gastroenterology, Brigham and Women’s and Faulkner Hospitals, Boston, MA and Division of Adult Oncology, Dana– Farber Cancer Institute, Boston, MA. Purpose: Myeloproliferative and other hematologic diseases can occasionally involve the liver and present diagnostic challenges. We describe the case of a 57 year– old woman referred because of increasing abdominal girth over 4 days. She had previously been healthy and her only complaints were related to her enlarging abdomen. Physical exam was notable for tense ascites, without jugular venous distension or stigmata of chronic liver disease. Laboratory tests revealed a normal CBC and liver panel, with the exception of an elevated alk phos of 259mg/dl. Of note her albumin was preserved at 3.7gm/dl and total protein was elevated at 10.4 g/dl. ESR was increased at 124mm/hr. The patient underwent paracentesis of 4 liters of straw– colored ascitic fluid which revealed a SAAG ⬎1.1 and no evidence of infection or malignancy. Abdominal ultrasound revealed a normal– appearing liver with normal doppler flow in portal, hepatic, and splenic veins. Liver biopsy revealed portal lymphoplasmacytic inflammation and occasional granulomas with mild portal and sinusoidal fibrosis, but no cirrhosis. Further laboratory testing revealed a positive AMA 1:640. ANA and ASMA were non–reactive. Serum protein electropheresis revealed presence of an IgM kappa chain monoclonal gammopathy. Ascites persisted despite treatment with oral diuretics. Venography revealed patent hepatic veins and inferior vena cava with an elevated hepatic venous pressure gradient of 13mmHg. Because of the finding of monoclo-
Abstracts
Microscopic examination of biopsy specimen showed necrotic material with numerous neutrophils consistent with brain abscess. Culture of the aspirate was negative. After stereotactic brain biopsy was done and while the patient was on intravenous antibiotics, there was improvement of her neurologic status. Serial magnetic resonance imaging of the brain showed resolution of the brain lesion. Two months after the initial presentation, colonoscopy was done showing scattered diverticuli in the sigmoid with prominent hyperemic folds and narrowing of the distal sigmoid. After the patient completed the course of antibiotics she was discharged home without any sequelae. This case illustrates that serious complications can occur even in a mild case of diverticulitis. Aggressive and early management of diverticulitis may prevent such complications from occuring.
522 LIVER TRANSPLANTATION FOR CHOLESTEROL ESTER STORAGE DISEASE Joseph E. Cassara, M.D., Neeraj K. Sachdeva, M.D., Michael W. Fried, M.D., Steven L. Zacks, M.D. and Roshan Shrestha, M.D.*. Department of Medicine, University of North Carolina, Chapel Hill, NC. Purpose: Cholesterol Ester Storage Disease(CESD) is a rare autosomal recessive disorder leading to lysosomal storage of triacylglycerols and cholesterol esters(CE) in hepatocytes and other tissues. This is due to a deficiency in lysosomal acid lipase, an enzyme necessary for cellular degradation of plasma low density lipoproteins. Orthotopic liver transplantation(OLT) offers a potential cure for CESD as the new liver replenishes the lysosomal acid lipase stores required for normal liver function. We describe a case of orthotopic liver transplantation for CESD. A 25 year– old white female diagnosed with CESD and started on treatment with lovastatin 15 years prior, presented with a massive upper gastrointestinal bleed. The patient reported an unremarkable course over the previous 15 years. An esophagogastroduodenoscopy revealed esophageal varices and signs of portal hypertensive gastropathy. She subsequently underwent a transjugular intrahepatic portosystemic shunt. The patient underwent an OLT. The grossly cirrhotic explanted liver weighed 1400 grams. Histopathology revealed massive accumulation of CE in lysosomes. Subsequently, she did remarkably well and was discharged two weeks post surgery. One year post– operatively the patient is well with stable graft function and a normal cholesterol level. Patients with CESD often have a relatively benign course and survive well into adulthood. Extrahepatic organ involvement has been reported but generally does not appear to cause significant tissue injury. However a subset of CESD patients develop cirrhosis early in the course of their disease and manifest portal hypertension and its complications. OLT offers a potential cure for CESD as the new liver replenishes the lysosomal acid lipase stores required for normal functioning. This case is an example of the progressive nature of the disease and sudden decline in condition seen in patients with CESD from liver failure after many preceding years of an uneventful course. OLT has been shown to improve hepatocellular metabolism and reduce complications from cirrhosis. In summary, we report the fourth case, in the english literature, of end stage liver disease with a life–threatening complication due to CESD successfully treated with OLT. Liver transplantation provides the best option for long term solution in patients with decompensated liver disease due to Cholesterol Ester Storage Disease.
523 LYMPHANGIOMAS (LA) OF THE GASTROINTESTINAL TRACT: THREE CASE STUDIES OF LESIONS INVOLVING STOMACH, SMALL BOWEL, AND COLON Mahmoud M. Yousfi, M.D., Kevin O. Leslie, M.D., Jacques Heppell, M.D. and Coung Nguyen, M.D.*. Gastroenterology & Hepatology, Mayo Clinic, Scottsdale, AZ; Pathology, Mayo Clinic, Scottsdale, AZ and Surgery, Mayo Clinic, Scottsdale, AZ.
AJG – Vol. 97, No. 9, Suppl., 2002
Purpose: LA are localized collections of benign lymphatic channels that rarely involve the GI tract. When visualized endoscopically, they appear as submucosal cystic lesions and often do not produce symptoms. Symptomatic patients may present with GI bleeding, abdominal pain, or obstruction. Advances in endoscopic tools have improved our ability to detect these lesions. We report three cases of cystic LA involving the stomach, small intestine, and colon diagnosed with endoscopic ultrasound (EUS) and video capsule endoscopy (VCE). Case reports. Case 1: A 78 YOM referred for evaluation of a submucosal mass in the transverse colon seen on previous colonoscopies and abdominal CT scan. The patient had no GI symptoms but was concerned because the lesion grew in size since his initial colonoscopy. EUS revealed a 3– cm soft compressible submucosal mass with normal overlying mucosa in the midtransverse colon. The lesion was anechoic containing fluid with internal septation. Mucosal biopsies were nondiagnostic and the patient elected to proceed with surgery. Histology revealed characteristic features of LA. Case 2: A 93 YOM underwent laproscopic–assisted cecal resection of a large cecal polyp found on colonoscopy. He presented 2 months later with obscure GI bleeding with nondiagnostic routine upper and lower endoscopies. VCE revealed mid small bowel mass. This was confirmed with intra– operative endoscopy and partial small bowel resection was performed. Histology revealed a benign LA. Case 3: A 26 YOM referred for evaluation of a fundic cystic mass seen on an upper endoscopy performed for evaluation of severe anemia (hemoglobin of 5 g/dL). EUS revealed a 1– cm polypoid lesion in fundus, which appeared to emanate from the mucosal layer with anechoic features and clear separation from the deep mucosa. Using snare polypectomy technique and normal saline injection submucosally, the lesion was resected. Histology revealed a benign cystic LA. Conclusions: LA of the GI tract should be part of the differential of cystic submucosal lesions. EUS is useful for diagnosis and typically reveals anechoic septated lesions confined to the submucosa with intact underlying muscularis propria. No treatment is necessary in asymptomatic patients. However, endoscopic or surgical resection may be performed for symptomatic lesions
524 WALDENSTROM’S MACROGLOBULINEMIA PRESENTING AS NEW–ONSET ASCITES Elena Stoffel, M.D., David Spigel, M.D. and Norman Grace, M.D., FACG*. Division of Gastroenterology, Brigham and Women’s and Faulkner Hospitals, Boston, MA and Division of Adult Oncology, Dana– Farber Cancer Institute, Boston, MA. Purpose: Myeloproliferative and other hematologic diseases can occasionally involve the liver and present diagnostic challenges. We describe the case of a 57 year– old woman referred because of increasing abdominal girth over 4 days. She had previously been healthy and her only complaints were related to her enlarging abdomen. Physical exam was notable for tense ascites, without jugular venous distension or stigmata of chronic liver disease. Laboratory tests revealed a normal CBC and liver panel, with the exception of an elevated alk phos of 259mg/dl. Of note her albumin was preserved at 3.7gm/dl and total protein was elevated at 10.4 g/dl. ESR was increased at 124mm/hr. The patient underwent paracentesis of 4 liters of straw– colored ascitic fluid which revealed a SAAG ⬎1.1 and no evidence of infection or malignancy. Abdominal ultrasound revealed a normal– appearing liver with normal doppler flow in portal, hepatic, and splenic veins. Liver biopsy revealed portal lymphoplasmacytic inflammation and occasional granulomas with mild portal and sinusoidal fibrosis, but no cirrhosis. Further laboratory testing revealed a positive AMA 1:640. ANA and ASMA were non–reactive. Serum protein electropheresis revealed presence of an IgM kappa chain monoclonal gammopathy. Ascites persisted despite treatment with oral diuretics. Venography revealed patent hepatic veins and inferior vena cava with an elevated hepatic venous pressure gradient of 13mmHg. Because of the finding of monoclo-