intestinal lymphangiectasia is a relatively rare disorder characterized by dilated intestinal submucosal lymphatics with a loss of lymph into the bowel lumen ( 1 ) . it has a clinical importance in that hypoproteinemia , edema , lymphopenia , and hypogammagloblinemia can be frequently associated . it is classified into two groups according to its causes ; primary type ( idiopathic ) , which has no particular causes , and secondary type , which has underlying causes ( 2 ) . in korea , only two cases of intestinal lymphangiectasia have been reported in korean literatures ( 3 , 4 ) . so far , to our knowledge , there are no reports of intestinal lymphangiectasia associated with cirrhotic ascites . until now however , bac et al . ( 5 ) reported that octreotide was effective in one patient with the condition . here we report a case of protein - losing enteropathy induced by intestinal lymphangiectasia in liver cirrhosis patient who was successfully treated with octreotide with a review of the literature . a 47-yr - old male patient was admitted to our hospital with complaints of abdominal pain and diarrhea . nine years before the admission he had been diagnosed as chronic hepatitis b virus infection , and eight months before admission , he had been diagnosed as liver cirrhosis , but he received no specific treatment . on admission , he complained of diffuse abdominal cramping pain and watery diarrhea . his blood pressure was 100/50 mmhg ; heart rate 72 beats / min ; respiratory rate 24 breaths / min ; and body temperature 38.4. he showed an acutely - ill appearance . physical examination showed mild abdominal distention , diffuse abdominal tenderness , but no rebound tenderness . laboratory findings were as follows : wbc 2,700/l ( lymphocyte 810/l ) , hb 9.1 g / dl , platelet 53,000/l , esr 24 mm / hr , total protein 3.9 g / dl , albumin 2.3 g / dl , total cholesterol 94 mg / dl , alt / ast 16/57 iu / dl , total bilirubin 1.2 mg / dl , creatinine 1.1 mg / dl , prothrombin time 65% , hbsag positive , hbsab negative , hbeag negative , hbeab positive , and normal urine analysis . serum immunoglobulin levels included a normal iga level of 246 mg / dl ( 82 - 453 mg / dl ) , a normal igm level of 107 mg / dl ( 46 - 304 mg / dl ) , and a low igg level of 537 mg / dl ( 751 - 1,560 mg / dl ) . ascites showed yellowish color and analysis showed wbc 1,300/l ( neutrophil 75% ) , albumin 0.2 g / dl , triglyceride 8 mg / dl and ldh 36 u / l . therefore , we diagnosed the patient as having a spontaneous bacterial peritonitis associated with liver cirrhosis and started antibiotics therapy , diuretics , and albumin supply . so we suspected that another causes of hypoalbuminemia might be present and started relevant investigation.with a suspicion of protein - losing enteropathy , albumin scan was performed . it showed protein - losing at jejunum after 2 hr of injection and subsequently moved distal jejunum , ileum , and colon ( fig . , 1 anti - trypsin clearance was increased at 50.03 ml / day ( nomal < 30 ml / day ) . duodenoscopy showed serosanguinous exudates in the duodenal mucosa , mucosal erosion , snow flake appearances , and erythema in the duodenal third portion ( fig . histological examination of a biopsy specimen from the duodenum showed markedly dilated lymphatics in the lamina propria ( fig . , we could make a diagnosis of protein - losing enteropathy induced by intestinal lymphangiectasia . since clinical and chemical abnormalities did not respond to low - fat diet , on the 24th day of hospitalization , we started subcutaneous octreotide therapy on the patient ( 0.1 mg three times daily ) . after two weeks of octreotide therapy , follow - up albumin scan showed no albumin leakage for three hours after injection . after octreotide therapy , the serum albumin level increased from 2.2 g / dl to 2.7 g / dl after 50 g albumin infusion . it was consistent with the albumin scan finding . during octreotide therapy , the serum albumin level was maintained at 2.8 g / dl without albumin infusion . during follow - up , we stopped octreotide therapy for economic problem of the patient . after one month , the serum albumin level decreased to 1.9 g / dl with continuous albumin infusion and the patient 's symptoms recurred with exacerbation of laboratory findings . after initiation of octreotide therapy , the serum albumin level increased with time . until now his serum albumin level has maintained , and there have been no apparent adverse effects from octreotide treatment . intestinal lymphangiectasia is characterized by obstruction of the intestinal lymphatics , and the increased lymphatic pressure can cause protein - losing enteropathy and malabsorption . intestinal lymphangiectasia is a primary disorder in cases of malformation of lymphatic vessels at the intestinal level or in other areas of the body . it can also occur secondary to diseases that may cause intestinal lymphatic obstruction , for example , abdominal or retroperitoneal fibrosis ( 6 ) . the most common causes of secondary intestinal lymphangiectasia are cardiac diseases and chemotherapeutic , infectious , or toxic substances that are associated with inflammatory processes that may cause retroperitoneal lymph node enlargement . therefore , all of above condition should be included as a differential diagnosis in intestinal lymphangiectasia . however , ct or lymphangiogram may be needed in our case to exclude other possible conditions . it usually presents with features of a protein - losing enteropathy ; diarrhea , edema , and hypoalbuminemia . chyluria , chylothorax , chylous ascites , lymphocytopenia and hypocalcemia have also been recognized ( 7 , 11 ) . endoscopic findings of intestinal lymphangiectasia include a scattered snowflake - like lesion in the mucosa ( 8) . consumption of a high - fat meal during the evening before endoscopic evaluation may make these findings more apparent . the diagnosis of intestinal lymphangiectasia is generally established by a biopsy of the intestinal mucosa , which demonstrates dilated mucosal and submucosal lymphatic channels . because the lesions are frequently patchy , multiple biopsies may be necessary to demonstrate the lymphatic lesion . a low - fat diet with medium - chain triglycerides has been recommended as the initial management of intestinal lymphagiectasia ( 7 ) . however , the patient 's compliance to low - fat diets is not always good , and refractory cases are occasionally observed with low - fat diet . so several intervention strategies have been reported to date , such as corticosteroid , antiplasmin therapy , cytotoxic drug , and even surgery , but the results have not been satisfactory ( 5 , 9 ) . and there are some reports that protein - losing enteropathy due to portal hypertension may be improved by placement of a transjugular intrahepatic portosystemic shunt ( 11 , 13 ) . octreotide is a long - acting somatostatin analog that has been shown to suppress gastrointestinal motility and hormone secretion in the pituitary gland , pancreas , and intestine . it also reduces the splanchnic blood flow , decreases endogenous fluid secretion in the jejunum , reduces lymph fluid excretion , and inhibits the absorption of triglycerides ( 10 ) . there have been only three patients who were treated with octreotide for protein - losing enteropathy from intestinal lymphangiectasia ( 2 , 5 , 7 ) . the mechanism of action of octreotide in intestinal lymphangiectasia involves reduction of intestinal blood flow , inhibition of triglyceride absorption , and reduction of lymph flow . the true impact of somatostatin and octreotide on portal hemodynamics remains controversial , and variable changes in portal pressure and intravariceal pressure in response to these drugs have been reported in different studies ( 14 , 15 ) . so , in our case such as intestinal lymphagiectasia combined with portal hypertension , octreotide may effect on portal pressure and therefore improve protein losing . all of three cases including our case show the efficacy of octreotide in intestinal lymphangiectasia , however , long - term administration of octreotide seems to be required . we think that the lesion in our patient had been acquired from impaired lymph flow , considering the late onset of disease and the fact that the lymphangiectasia appeared to be associated with liver cirrhosis , a disease known to alter lymph flow . thus we think that intestinal lymphangiectasia should be suspected as a differential diagnosis in refractory hypoalbuminemia in liver cirrhosis patients . first , it is the first case of intestinal lymphangiectasia developing in liver cirrhosis patient . we speculate that liver cirrhosis can be a cause of intestinal lymphangiectasia though the previous mentioned mechanism . second , through it is a relatively rare disease , we should consider intestinal lymphangiectasia as a differential diagnosis in a patient with protein - losing enteropathy . third , when we experience continuous hypoalbuminemia despite albumin infusion in a liver cirrhosis patient , we should suspect that presence of another cause of hypo - albuminemia . although there can be an economic problem and side effects of long - term therapy of octreotide , the administration of octreotide should be considered for patients with intestinal lymphangiectasia whose clinical and biochemical abnormalities do not respond to a low - fat diet . the mechanism of action is not entirely clear but appears to involve reduction in gut protein loss .
a 47-yr - old man with hepatitis b virus associated liver cirrhosis was admitted to our hospital with diarrhea and generalized edema and diagnosed as protein - losing enteropathy due to intestinal lymphangiectasia by intestinal biopsy and 99mtc albumin scan . during hospitalization 
 , he received subcutaneous octreotide therapy . 
 after 2 weeks of octreotide therapy , follow - up albumin scan showed no albumin leakage , and the serum albumin level was sustained . 
 we speculate that liver cirrhosis can be a cause of intestinal lymphangiectasia and administration of octreotide should be considered for patients with intestinal lymphangiectasia whose clinical and biochemical abnormalities do not respond to a low - fat diet .