Duodenum - Endoscopic Ablation Therapy of Primary Intestinal Lymphangiectasia Using Argon Plasma Coagulation
Comments: Primary intestinal lymphangiectasia is a rare congenital disorder of lymphatic system.
This is characterized by abnormally dilated lymphatic and excessive enteric loss of lymph fluid rich in plasma protein, chylomicron and lymphocytes.
So far, suggested mechanisms of lymphatic fluid loss are exudation from the epithelium and rupture of dilated lymphatics due to blocked flow.
The disorder can be partially controlled with dietary modification, surgical resection if localized, lymphatic decompression by lymphovenous anastomosis, administration of octreotide and antiplasmin therapy.
A 17 year-old female presented with progressive edema of both lower legs over several years. She also complained of frequent loose stools, generalized weakness and lower leg edema beginning since childhood. There was no steatorrhea or body weight loss.
Both past medical history and family history were unremarkable.
The patient had puffy face, mild pitting edema in abdominal wall and severe pitting edema in both lower legs.
Laboatory findings showed lymphcytopenia, hypoproteinemia and hypoalbuminemia.
Total cholesterol and triglyceride showed decreases.
However, renal and liver function tests were within normal.
There was no urinary loss of albumin.
Stool exam had ruled out steatorrhea.
Plasma clearance of alpha1-antitrypsin showed an increase to 760 ml per day.
Technetium 99m-labeled human serum albumin scan revealed leak of radioisotope from ascending and transverse colons beginning six hours after injection.
Initial gastroduodenoscopic findings consisted of numerous tiny white spots scattered all over from duodenal bulb portion to third portion. Distal part of the third portion was not involved.
This is lower power view of duodenal biopsy showing dilated lymphatics in lamina propria.
CT scan of the abdomen in this patient showed symmetrical wall thickening in the duodenal third portion, small amount of ascites in the pelvic cavity and no retroperitoneal mass or enlarged lymph nodes in coronal view.
Then, how can the followings be explained?
1. Limited clinical improvement after lymphatics decompression by lymphovenous anastomosis.
2. Dilated lymphatics vessels found only in the tip of villi in many cases.
3. Scattered white spots in the mucosa, not subepithelial lesion as typical endoscopic finding.
After a day of high-fat meal, repeat gastroduodenoscopy was done.
In the antrum, reflux of milky fluid from duodenum is seen.
Numerous white spots have become more prominent and increased in numbers.
Also, pool of milky fluid is noted in the bulb portion.
Argon plasma coagulation set at 40 watt was applied to fulgurate the involved duodenal mucosa. Pinpoint coagulation technique was used.
APC was not applied on the major duodenal papilla.
Three sessions of APC, each two months apart, were done without significant complications.
At 6 months after three sessions of APC, follow-up endoscopy after fatty meal showed disappeared oozing, marked decrease in number of white spots. Although fulguration was not done on major duodenal papilla or duodenal distal third portion, no new lesion appeared.
Endoscopic ultrasonography also demonstrated no abnormally dilated mural or extramural tubular structures.
Lower power view of follow-up duodenal biopsy demonstrated disappearance of dilated lymphatics in lamina propria.
Technetium 99m-labeled human serum albumin lymphoscintigraphy demonstrated no enteric loss or abnormally dilated lymphatic vessel.
At 6 months after therapy with APC, serum protein, albumin and blood lymphocyte count have normalized and remained normal afterwards.
Also, alpha 1-antitrypsin clearance rate has decreased to 29 ml per day.
In conclusion, lymphatic fluid seems to ooze from ruptured lymphatics in this patient.
This finding may be attributed to abnormally fragile lymphatics susceptible even to a physiologic increase in lymphatic pressure.
Endoscopic ablation therapy using argon plasma coagulation seems safe and efficacious in treating protein-losing enteropathy and lymphocytopenia associated with primary intestinal lymphangiectasia. Its effect is also durable.
| Contributed by: |
Chang Keun Park, M.D. Physician Daegu Fatima Hospital, Korea Jimin Han, MD Daegu Fatima Hospital, Korea Ho Gak Kim, MD Daegu Fatima Hospital, Korea |
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Citation: Park, CK & Han, J. & Kim, HG (May 21 2007). Duodenum - Endoscopic Ablation Therapy of Primary Intestinal Lymphangiectasia Using Argon Plasma Coagulation. The DAVE Project. Retrieved Sep, 8, 2010, from http://daveproject.org/viewfilms.cfm?film_id=599 Times viewed since Feb 2006: 9221 |
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