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Partial enterectomy: treatment for primary intestinal lymphangiectasia in four cases

ZHU, Ling-hua; CAI, Xiu-jun; MOU, Yi-ping; ZHU, Yi-ping; WANG, Song-biao; WU, Jia-guo

Editor(s): HAO, Xiu-yuan; PAN, Cheng

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doi: 10.3760/cma.j.issn.0366-6999.2010.06.023
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Intestinal lymphangiectasia (IL) is a rare disorder characterized by protein-losing enteropathy and fat malabsorption. The underlying cause of this disorder has been revealed to be impaired intestinal lymphatic drainage. According to the etiologies, IL can be classified into primary (idiopathic) form and secondary form. Diagnosis of primary IL is usually established by characteristic endoscopic findings and biopsy of intestinal mucosa. Various medical treatments for primary IL have been reported, including dietary manipulation, somatostatin, antiplasmin therapy, and even cytotoxic drugs. However, the results of these treatments were not satisfying. Here we presented four cases of primary IL successfully treated by partial enterectomy to analyze the indication as well as the long term effect of surgical intervention.


Case 1

A 22-year-old man was admitted in July 1997 with lower extremity edema lasting for one year, diarrhea (three to five times per day, yellow and pasty stool) and abdominal distension lasting for six months. He was healthy in the past and denied any family history of systemic disorders. His body weight was 46.5 kg and the body mass index (BMI) was 17.5 kg/m2. Obvious edema of both legs was visible. The abdomen was slightly distended with shifting dullness positive. A complete blood count (CBC) revealed a decreased number of lymphocyte (0.69×109 /L) which accounted for 9.5% of the total number of white blood cells (WBCs). WBC count was 7.3×109 /L, hemoglobin (Hgb) was 132 g/L. The total serum protein was only 24 g/L, and albumin was 14 g/L. His serum IgG and IgM were below normal level (IgG 4.0 g/L, IgM 0.45 g/L; normal range: IgG 6.94-16.20 g/L, IgM 0.6-2.63 g/L). A stool examination showed a yellow, soft stool with positive occult blood (+), no lipid droplet, no mucous or pus cells. The analysis of ascites was as follows: glucose 107 mg/dl, chloride 4 mmol/L, and the Pandy's test was positive. The small intestine series showed disorder mucosae at the upper part of jejunum. Some of the jejunal plicae were thickened like cerebral gyri. Tiny filling defects with protuberant margins were also found (Figure 1A). Based on the patient's clinical manifestations and radiological examination findings, an initial diagnosis of protein-losing enteropathy induced by primary IL was considered. Laparotomy was performed for a definite diagnosis in August 1997. During operation, the upper part of jejunum was discovered to be hyperaemic and edematous with multiple rough protruding plicae inside the bowel lumen. Moderate ascites was also seen. About 90 cm of the diseased jejunum were removed. Pathological examination confirmed it to be primary IL (Figure 4A). The patient's symptoms (abdominal distension, diarrhea and edema) were obviously relieved after operation. A CBC showed an increase of lymphocyte count (1.3×109 /L) and serum albumin level returned to normal (37 g/L) 53 months postoperatively, while abdomen shifting dullness was negative. Until now (138 months postoperatively), the patient has been on a normal diet without the experience of discomfort and does not need medication and his body weight has increased to 62.5 kg (BMI 23.5 kg/m2).

Figure 1.
Figure 1.:
Small intestine series displayed rough and disorderly mucosae with small filling defects in primary IL patients of cases 1 (A) and 4 (B). CT scan revealed thickened intestinal mucosa (case 4, C).
Figure 3.
Figure 3.:
Gross appearance of surgical specimen showed hyperaemia and edema of small intestine (A) and creamy yellow villi in the bowel lumen were seen in case 2 (B). Dilated lymphatics were observed under laparoscopy (C), part of the ileum was hyperemic and edematous in case 4 (D).Figure 4. Histological examinations showed dilated mucosal and submucosal lymphatic vessels of case 1 (A), case 2 (B), case 3 (C) and case 4 (D) (HE staining, original manuscription ×100).

Case 2

A 44-year-old woman was admitted in July 2001. She complained of fatigue and continuous weight loss (11 kg totally) for half a year, edema of bilateral lower extremities, abdominal distention and oliguria for one month. The patient had quite frequent occurrences of diarrhea (three to five times per day, pasty and yellow stool), and sometimes passed tarry stool. Eighteen years before, she was diagnosed with pulmonary tuberculosis and received a standard course of anti-tubercle bacillus (TB) treatment. The patient had a chronically ill appearance. Her body weight was 45 kg and the BMI was 17.57 kg/m2. No superficial lymph node was found. Her abdomen was soft and distended without obvious tenderness, but with a positive shifting dullness. Edema was seen in the lumbosacral region and bilateral lower extremities. The results of CBC were WBC 4.2×109 L (neutrophil 78.9%), lymphocyte 0.3×109 /L, and Hgb 73 g/L. Her total protein was 38.4 g/L with albumin only 17.7 g/L. Serum levels of IgG and IgM were decreased to 3.0 g/L and 0.4 g/L, respectively. Her erythrocyte sedimentation rate (ESR) was normal (4 mm/h). A stool examination revealed positive occult blood (2+), no lipid droplet, and no mucous or pus cells. The ascites analysis showed: glucose 37 mg/dl, chloride 99 mmol/L, total protein 24 g/L and albumin 11 g/L. The purified protein derivatives (PPD) test and serum TB antibody were negative. A chest X-ray indicated left lower pleurisy. An abdominal ultrasonography disclosed moderate ascites. A cardiovascular assessment did not find any clue of pericarditis or valvular abnormality. A small intestine series displayed rough and disorderly mucosae of jejunum and ileum with small filling defects inside. A coloscopy discovered multiple polypoid fluffy elevations at the end portion of ileum (40 cm away from the ileocecal valve). The biopsy of ileum mucosa indicated primary IL. Conservative therapy (food manipulation and intravenous albumin infusion) was prescribed for one month with no obvious improvement. A laparotomy was performed in August 2001. Moderate fluid collection (about 300 ml) in the abdominal cavity was seen. Almost the whole length of small intestine was discovered to be hyperaemic and edematous with the intestinal wall rigid and thickened (Figure 3A). Creamy yellow villi were seen in the bowel lumen (Figure 3B). About 190 cm of the most obviously diseased intestine (initiated from the end of jejunum and ended at ileum 20 cm from ileocecal valve) was removed. A pathological examination confirmed primary IL (Figure 4B). The patient recovered well after operation and her symptoms disappeared. Her body weight increased by 14 kg (BMI returned to 23 kg/m2) over the 36 months postoperative. Her abdomen was soft with negative shifting dullness. A CBC showed normal erythrocyte and lymphocyte counts. Serum albumin level was 36 g/L, stool occult blood was negative. Until now (90 months postoperatively), there has been no recurrence of her previous symptoms.

Case 3

A 71-year-old man suffering from five-year recurrent melena was admitted in August 2001. The patient had a history of chronic hepatitis B for over twenty years. His body weight was 65 kg and BMI was 22.76 kg/m2. A subsequent physical examination found slight edema of bilateral lower extremities. The abdomen was flat and soft without tenderness. Shifting dullness was negative. His CBC revealed a decrease of Hgb (76 g/L). The count of lymphocyte was only 0.6×109 /L, which accounted for 5.2% of the total number of WBCs. The serum total protein level was 52.5 g/L with albumin of 27.1 g/L. A hepatitis B screening test revealed HBs-Ab (+) and stool examination showed positive occult blood (3+) with no lipid droplet, no mucous or pus cells. An abdominal CT scan and ultrasonography showed no obvious abnormalities. Gastroscopy and coloscopy did not disclose any lesion either. An angiography of superior mesenteric artery detected abnormal dyeing at the 6th group of the small intestine. For treating refractory GI bleeding and excluding underlying diseases, laparotomy was performed to make the diagnosis clear 19 days after admission. During the operation, obvious edema was found in the end portion of the ileum (about 50 cm in length) with sub-serosal elevations filled with milky fluid. The last fifty centimeters of ileum and part of the ascending colon were removed. A pathological examination confirmed it to be primary IL (Figure 4C). The patient recovered smoothly after the operation and a stool occult blood showed negative three months after discharge. In the recent follow-up (87 months postoperatively), the patient had no obvious discomfort or edema. His stool was soft and yellow, three to four times per day with no melena and his body weight has increased to 72 kg (BMI 25.2 kg/m2). His lymphocyte count was 1.1×109IL, while Hgb was 125 g/L and albumin was 31 g/L.

Case 4

A 55-year-old woman complaining of recurring peripheral edema for over twenty years was admitted in March 2005. She had quite obvious abdominal distension and oligury (less than 600 ml per day) before admission. She also suffered from diarrhea after eating greasy food. Her body weight was 57 kg and BMI was 21.45 kg/m2. A subsequent physical examination found moderate edema of bilateral lower extremities. Her abdomen was flat and soft, without tenderness. Shifting dullness was negative. A CBC did not show anything abnormal. The serum total protein level was 42.4 g/L with albumin of 24.5 g/L. A decrease of serum IgG and IgM was observed (IgG 4.07 g/L, IgM 0.40 g/L). Examinations of urea and stool were normal. A gastroscopy and coloscopy did not disclose any obvious lesion. A small intestine series displayed rough and disorderly mucosa with small filling defects in the small intestine (Figure 1B). An abdominal CT scan revealed thickening of bowel mucosa in the small intestine (Figure 1C). A capsule endoscopy found multiple polypoid fluffy elevations in the bowel lumen, especially in the middle and lower part of the small intestine (Figure 2). The patient underwent laparoscopic exploration for a definite diagnosis in April 2005. During operation, part of the ileum was discovered to be hyperemic and edematous with dilated lymphatics seen on its surface (Figure 3). A total length of 150 cm of the diseased bowel segment (ended at 20 cm to the ileocecal valve) was removed in a subsequent small incisional open procedure. The pathological examination reported primary IL (Figure 4). No underlying etiologies could be identified. The patient's symptoms including peripheral edema and abdominal distension resolved after the operation. She expelled more than 1000 ml of urine per day without use of diuretics. The CBC was normal and serum albumin level elevated to 35 g/L three months postoperatively. Up to now (46 months postoperatively), the patient has been on a normal diet without medication, and free of previous symptoms with body weight of 52 kg (BMI 19.6 kg/m2).

Figure 2.
Figure 2.:
Capsule endoscopy findings of primary IL (case 4): multiple polypoid fluffy elevations in the intestinal lumen.


Intestinal lymphangiectasia, which was first described by Waldmann et al1 in 1961, is a rare disorder caused by the obstruction of intestinal lymphatic drainage. Increased lymphatic pressure leads to dilatation and ruptures of lymphatic vessels and thus results in a loss of lymph fluid into the bowel lumen. This would subsequently cause lymphocytopenia, hypoproteinemia, and chronic diarrhea. According to the etiology, IL can be classified into two types: primary IL and secondary IL. Secondary IL could be the result of a variety of disorders that impede intestinal lymphatic drainage, such as constrictive pericarditis, retroperitoneal fibrosis, systemic lupus erythematosus, lymphoma, intestinal tuberculosis, inflammatory bowel disease, and malignancy2. The exact cause of primary IL is not clear. However, it is thought to be congenital and is occasionally associated with some genetic syndromes3. Primary IL generally affects children and young adults but may also occur in older people4.

Clinical manifestations are similar in both types of IL. Typical symptoms of IL include peripheral edema, ascites, plural effusion, weight loss, and growth retardation in children5. Some of these common manifestations were found in cases 1, 2 and 4. Gastrointestinal tract bleeding, which was the main complaint of case 3, had also been reported in literature as a rare symptom of primary IL. It was thought to be associated with the rupture of dilated lacteals that have potential communications with blood vessels6. Indicative laboratory findings for IL include hypoproteinemia, hypoalbuminemia, lymphocytopenia (especially absence of CD4+ and CD8+ lymphocytes), decrease of immunoglobulin (IgG, IgM and IgA) levels, and decrease of serum transferring and fibrinogen levels.5,7 Radiological examinations can find that characteristic features of IL and sometimes are able to indicate the location and extent of the lesion. Small intestine series usually find rough and disorderly plicae of mucosa accompanied with dilatation of intestine lumen as shown in cases 1, 2 and 4. CT findings of IL have been described as diffused thickening of the small intestine wall (as in case 4) without adenopathy or hepatosplenomegaly 8 and linear hypodense streaking densities in the small intestine caused by dilated lymphatic channels.9 Specific endoscopic findings, including white tipped villi, scattered white spots, white nodules and submucosal elevations, contribute greatly to the diagnosis of IL. As IL often occurs at jejunoileum where traditional gastroendoscopy can not reach, capsule endoscopy is a novel and non-invasive technique in evaluating such lesions10, but it has not been widely applied partly because of its high cost. The diagnosis of primary IL is established by pathological examinations, which demonstrate dilated mucosal, submucosal or serosa lymphatic channels,11-13 after excluding the attribute of secondary IL.


Three patients in our series (cases 1, 2 and 4) were admitted for peripheral edema, ascites, weight loss, or diarrhea, which are the symptoms of malnutrition. Laboratory findings disclosed hypoproteinemia, but did not show any evidence of liver dysfunction or protein losing nephropathy. As these patients had a normal food intake, excessive protein loss from the digestive tract (protein-losing gastroenteropathy) was considered. Measurement of α-1-antitrypsin clearance from plasma has been suggested to be a reliable way to detect enteric protein loss,14 but it was not applied in the present study. Protein-losing gastroenteropathy encompasses a variety of disorders, such as lymphoma, Crohn's disease, intestine tuberculosis, constrictive pericarditis, giant hypertrophic gastritis, ulcerative colitis, and IL, which accounts for about 25% of total cases.5 A series of workups including laboratory evaluation, imaging studies and endoscopic approaches are needed to distinguish IL from other protein-losing gastroenteropathies. Once the other possibilities are excluded, IL should be suspected. Some authors have proposed the diagnostic criteria for IL as: 1. typical clinical manifestations, 2. lymphocytopenia, 3. simultaneous decrease level of serum albumin and IgG, 4. pathological confirmation of IL by endoscopy biopsy, or surgery and 5. experimental findings of protein loss from digestive tract. The first three items serve as suggestions, while the latter two are essential. According to these criteria, the diagnosis of IL was proposed for cases 1, 2 and 4.

The situation in case 3, presented with recurrent melena, which is not a common symptom of IL, is quite special. Laboratory findings disclosed hypoalbuminemia, lymphopenia and positive stool occult blood. A superior mesenteric arteriography revealed abnormal dyeing at the 6th group of small intestine. A laparotomy was performed for a definite diagnosis and eventually determined it to be IL. To our knowledge, this is a relatively early report that an IL lesion found by angiography. One possible reason could be that IL might associate with some congenital vascular deformity that could be detected by angiography.

As had neither a past history nor any ongoing diseases that might relate to secondary IL, cases 1, 3 and 4 were finally diagnosed as primary IL. Case 2 had had lung tuberculosis history eighteen years before the admission presented here. However, laboratory examinations (normal ESR, TB antibody negative, and purified protein derivative test negative) as well as the postoperative pathology did not support the existence of active intestinal tuberculosis which could be a possible etiology for secondary IL. Therefore, diagnosis of primary IL was preferred for this patient.

Currently, treatment for primary IL still remains difficult. Various treatments for primary IL have been reported, including dietary manipulation (a low-fat diet supplemented with medium-chain triglycerides), somatostatin, antiplasmin, and corticosteroids therapy, and even cytotoxic drugs. However, the results of these medical treatments were not satisfying. Dietary mani-pulation is the most commonly used medical therapy. Medium-chain triglycerides (MCT) are absorbed rapidly by the intestinal mucosa and can be metabolized and excreted directly into the portal venous system rather than via intestinal lacteals so that the load on the impaired lymphatic system could be reduced. However, patients may have to maintain dietary manipulation for the duration of their lives, as the symptoms may soon recur if treatment is withdrawn.

In case the lesion of primary IL is considered local or segmental, small bowel resection could be chosen. Several primary IL patients with localized involvements have been reported to be successfully treated by partial enterectomy.15-17 Imaging studies and endoscopy are common ways to judge the location and extension of an IL lesion. Other methods, such as 99mtechnetium-dextran scintigraphy, had also been performed.18 In the current report, IL lesions were determined to be localized according to the radiological and endoscopic findings. Laparotomies were then performed for further assessment. Cases 1, 3 and 4 showed quite clear margins of the affected intestine, which presented serosal and mucosal lymphangiectasia and vascular congestion. These three patients had the affected bowel segments completely excised and experienced long term relief of symptoms without the use of medication. In case 2, however, the whole length of the small intestine seemed to be affected by hyperaemic and edematous appearance and thickening of the intestinal wall. The most seriously diseased bowel segment, about 190 cm in length, was removed. According to follow-up results, the long term effect of bowel resection in this case was decent.


Surgery is proposed for primary IL patient with pre-operative assessment of segmental or localized involvement. When the affected intestine has clear margins, it is easy to decide the resection. However, when the entire small intestine is found to be edematous (as in case 2), it is important, yet difficult, to ascertain the main or the most serious lesion of lymphangiectasia for excision. The decision of resection must weigh the potential benefits of surgery against the risks of complications (such as short bowel syndrome) and the possibility of persistence or recurrence of IL. Preoperative ingestion of lipophilic dyes or intra-operative lymphangiography has been used to identify the main site of lymphangiectasia. In case 2, we determined the most seriously diseased bowel segment for excision by observation, which has also been proved to be feasible by other researches.16,17,19 Anyway this approach has only been used in a limited number of cases, more clinical experiences are needed for verification.


Primary IL is a rare disorder. In this study, we reported 4 cases of primary IL with localized involvement treated by small bowel resection. A long-time follow-up demonstrates the definitive cure of surgical resection. We suggested that when a diagnosis of primary IL is considered and the disease extent is indicated to be segmental or localized by radiological and/or endoscopic examinations, laparotomy is recommended to disclose the extent of lymphangiectasia and decide whether the lesion could be resected or not. Laparoscopic exploration, an effective and minimally invasive procedure, could be used as a better option for traditional open approach.

Acknowledgement: We are grateful to Ms. Debra Marovitch, for proof reading this article and giving many valuable suggestions.


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intestine; small; lymphangiectasis; surgery

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