Dynamic Contrast Magnetic Resonance Lymphangiography Localizes Lymphatic Leak to the Duodenum in Protein-Losing Enteropathy.

OBJECTIVES: Protein-losing enteropathy (PLE) is a disorder of intestinal lymphatic flow resulting in leakage of protein-rich lymph into the gut lumen. Our primary aim was to report the imaging findings of dynamic contrast magnetic resonance lymphangiography (DCMRL) in patients with PLE. Our secondary objective was to use these imaging findings to characterize lymphatic phenotypes. METHODS: Single-center retrospective cohort study of patients with PLE unrelated to single-ventricle circulation who underwent DCMRL. We report imaging findings of intranodal (IN), intrahepatic (IH), and intramesenteric (IM) access points for DCMRL. RESULTS: Nineteen patients 0.3-58 years of age (median 1.2 years) underwent 29 DCMRL studies. Primary intestinal lymphangiectasia (PIL) was the most common referring diagnosis (42%). Other etiologies included constrictive pericarditis, thoracic insufficiency syndrome, and genetic disorders. IN-DCMRL demonstrated a normal central lymphatic system in all patients with an intact thoracic duct and localized duodenal leak in one patient (1/19, 5%). IH-DCMRL detected a duodenal leak in 12 of 17 (71%), and IM-DCMRL detected duodenal leak in 5 of 6 (83%). Independent of etiology, lymphatic leak was only visualized in the duodenum. CONCLUSIONS: In patients with PLE, imaging via DCMRL reveals that leak is localized to the duodenum regardless of etiology. Comprehensive imaging evaluation with three access points can provide detailed information about the site of duodenal leak.

Echocardiographic and Ultrasonographic Parameters Associated with Protein-losing Enteropathy in Patients with Fontan Physiology: a Systematic Review with Meta-analysis

Abstract Background: Fontan circulation can be associated with significant morbidity, especially Protein-Losing Enteropathy (PLE). Echocardiographic parameters can provide valuable diagnostic information about a patient's risk of developing PLE after Fontan surgery. Objectives: To describe echocardiographic/ultrasonographic parameters associated with PLE in patients after Fontan surgery through a systematic review with meta-analysis. Methods: A literature search was performed in electronic databases to identify relevant studies about echocardiographic parameters and PLE prediction in children after Fontan surgery. The search terms used were: "echocardiography", "ultrasonography", "Fontan," and "protein-losing enteropathy". A p < 0.05 was considered statistically significant. Results: A total of 653 abstracts were obtained from electronic databases and bibliographic references. From these, six articles met criteria to be included in the qualitative analysis and three in the quantitative (meta-analysis). The resistance index in the superior mesenteric artery was described in three studies, and the quantitative analysis showed statistical significance (p < 0.001). Other echocardiographic and ultrasonographic parameters were also described, albeit in single studies not allowing a meta-analysis. Conclusion: This systematic review with meta-analysis identified echocardiographic and ultrasonographic parameters related to PLE in patients with Fontan physiology. Vascular ultrasonography seems to play a prominent role in this aspect, but additional studies are needed to increase the degree of evidence.

Intrahepatic Dynamic Contrast-Enhanced Magnetic Resonance Lymphangiography: Potential Imaging Signature for Protein-Losing Enteropathy in Congenital Heart Disease.

Background Protein-losing enteropathy (PLE) is a significant cause of morbidity and mortality in congenital heart disease patients with single ventricle physiology. Intrahepatic dynamic contrast-enhanced magnetic resonance lymphangiography (IH-DCMRL) is a novel diagnostic technique that may be useful in characterizing pathologic abdominal lymphatic flow in the congenital heart disease population and in diagnosing PLE. The objective of this study was to characterize differences in IH-DCMRL findings in patients with single ventricle congenital heart disease with and without PLE. Methods and Results This was a single-center retrospective study of IH-DCMRL findings and clinical data in 41 consecutive patients, 20 with PLE and 21 without PLE, with single ventricle physiology referred for lymphatic evaluation. There were 3 distinct duodenal imaging patterns by IH-DCMRL: (1) enhancement of the duodenal wall with leakage into the lumen, (2) enhancement of the duodenal wall without leakage into the lumen, and (3) no duodenal involvement. Patients with PLE were more likely to have duodenal involvement on IH-DCMRL than patients without PLE (P<0.001). Conclusions IH-DCMRL findings of lymphatic enhancement of the duodenal wall and leakage of lymph into the duodenal lumen are associated with PLE. IH-DCMRL is a useful new modality for characterizing pathologic abdominal lymphatic flow in PLE and might be useful as a risk-assessment tool for PLE in at-risk patients.

Successful Treatment of Protein-Losing Enteropathy After Superior Mesenteric Artery Occlusion without Surgery.

BACKGROUND Protein-losing enteropathy as a complication of superior mesenteric artery occlusion is extremely rare and severe, and sometimes requires intestinal resection. However, the ideal treatment strategy has not yet been determined. CASE REPORT A 77-year-old man with underlying hypertension and diabetes was admitted to the Emergency Department with acute abdominal pain after eating. Contrast-enhanced computed tomography revealed complete occlusion of the superior mesenteric artery with thrombosis, and superior mesenteric artery occlusion was diagnosed. It was successfully treated with interventional therapy, followed by continuous intra-arterial prostaglandin E1 infusion and continuous intravenous heparin infusion. However, the patient developed hypoproteinemia and diarrhea about 10 days after the interventional therapy. Colonoscopy and X-ray studies did not reveal any abnormal findings; however, technetium-99m-labeled human serum albumin scintigraphy indicated protein-losing enteropathy. With total parenteral nutrition and protein-rich oral nutrition, with protein intake at twice the amount in a standard diet, serum albumin improved from 15 g/L to 32 g/L after treatment. Additionally, we administered diuretics to avoiding edema related to the hypoproteinemia. The patient recovered from the hypoproteinemia and diarrhea without complications. CONCLUSIONS Protein-losing enteropathy is an extremely rare but critical complication of superior mesenteric artery occlusion. Treating the underlying pathology is the mainstay of protein-losing enteropathy and dietary modifications also play a critical role. Our patient was successfully treated with strict nutritional therapy, combined oral protein-rich nutrition and total parenteral nutrition, which avoided surgery.

Therapeutic Lymphatic Embolization in Pediatric Primary Intestinal Lymphangiectasia.

Primary intestinal lymphangiectasia (IL) can cause leakage of lymphatic fluids into the gastrointestinal tract, eventually leading to protein-losing enteropathy. A 15-year-old male patient, whose disease began at the age of 8 years, recently felt worsening general weakness. After diagnosing abnormal lymphatic lesions in the duodenum through endoscopy with biopsy and contrast-enhanced magnetic resonance lymphangiography, glue embolization of the leaking duodenal lymphatic channel was successfully performed. This procedure is typically reserved for adult patients, although as shown in this case, it can be properly performed in children. His serum albumin level was initially 1.5 g/dL, but elevated to 5.0 g/dL after two sessions of lymphatic embolization. Accordingly, we suggest that embolization could potentially be considered a first-line treatment for focal lesions of primary intestinal IL.

Abdominal Imaging of Children and Young Adults With Fontan Circulation: Pathophysiology and Surveillance.

OBJECTIVE. The Fontan procedure has significantly improved the survival in children with a functional single ventricle, but it is associated with chronically elevated systemic venous pressure that leads to multisystemic complications. Imaging plays an important role in assessing these complications and guiding management. The pathophysiology, imaging modalities, and current surveillance recommendations are discussed and illustrated. CONCLUSION. Significant improvement in survival of patients with Fontan circulation is associated with ongoing cardiac and extracardiac comorbidities and multisystemic complications. The liver and intestines are particularly vulnerable to damage. In addition, this patient population has been shown to be at increased risk of certain malignancies such as hepatocellular carcinoma and neuroendocrine tumors. Familiarity with imaging findings of Fontan-associated liver disease and other abdominal complications of the Fontan circulation is essential for radiologists because we are likely to encounter these patients in our general practice.

The diagnostic value of capsule endoscopy in children with intestinal lymphangiectasia.

BACKGROUND: intestinal lymphangiectasia is an unusual cause of protein-losing enteropathy due to either congenital malformation or obstruction of the intestinal lymphatics. However, few reports have investigated the use of video capsule endoscopy in children with intestinal lymphangiectasia. This study was performed to evaluate the diagnostic value of video capsule endoscopy for pediatric intestinal lymphangiectasia. METHODS: in this retrospective study, all patients who underwent video capsule endoscopy between January 2014 and July 2020 were included. Clinical information and video capsule endoscopy data were analyzed. RESULTS: twelve children were enrolled, 7 males and 5 females, with an age at disease onset of 4.5 (range: 3.2-9.3) years and a disease duration of 12.0 (range: 1.3-30.0) months. The most common symptoms were hypoproteinemia (10, 83.3 %), diarrhea (7, 58.3 %), edema (6, 50.0 %), and abdominal pain (3, 25.0 %). Eight patients had low lymphocyte counts, whereas 10 had reduced serum albumin levels (23.2 ± 5.8 g/L). Video capsule endoscopy revealed an overall white snowy appearance due to the presence of whitish, swollen villi in all patients. Regarding the macroscopic lesions of lymphangiectasia, 7 cases involved the entire small bowel from the duodenum to the ileocecal valve, while 5 cases involved part of the small bowel. All patients were treated with medium-chain triglyceride diets, and albumin infusions were administered to 10 patients; sirolimus treatment was administered to 3 patients. At the last follow-up, 5 patients still had hypoalbuminemia and one patient had died of intestinal lymphoma. CONCLUSION: video capsule endoscopy is useful for the diagnosis of intestinal lymphangiectasia and should be applied as a valuable and less invasive examination to confirm or establish a diagnosis.

Revascularization of Isolated Superior Mesenteric Artery Dissection for Post-Mesenteric Infarction Exudative Enteropathy: The Contribution of a Multidisciplinary Care Teamwork.

BACKGROUND: Isolated dissection of the superior mesenteric artery (SMA) is increasingly diagnosed; the conservative treatment appears to be the best option when blood supply is maintained. However, some patients require a more invasive attitude, due to acute or chronic ischemic complication. Multidisciplinary care within a health-care network improves medical and surgical support. We present a case of chronic complication of isolated SMA dissection requiring a revascularization, followed by bowel resection. CASE PRESENTATION: A 49-year-old man presented abdominal pain secondary to necrotizing enterocolitis of unknown cause. An isolated small bowel resection with immediate restoration of continuity was first performed to remove the necrotic tissue. The patient developed post-ischemic exudative enteropathy and small intestinal bacterial overgrowth (SIBO). Re-examination of the first computed tomography (CT) scan revealed an isolated dissection of the SMA that had not been diagnosed. The patient was then successfully treated by superior mesenteric revascularization, and after recovering, he underwent small bowel resection for chronic ischemia. CONCLUSIONS: SMA revascularization has to be performed in case of SMA dissection complicated of bowel ischemia. Enteropathy is a rare complication of chronic mesenteric ischemia requiring digestive and vascular management in a dedicated health-care team.

Detection of protein-losing enteropathy by 99m Tc-UBI scintigraphy.

nd localizing site of protein loss.

Egested Feces Revealed Massive Protein Loss in 99mTc-HSA-D Scintigraphy.

A 66-year-old man with severe diarrhea and severe hypoproteinemia was referred for Tc-DTPA-human serum albumin (HSA-D) scintigraphy. The scintigraphy at 24 hours postinjection showed strong accumulation of Tc-HSA-D in not only the colon but also all his diapers containing his egested feces. The ratio of the diapers counts to the total counts was 17.0%, and a considerable amount of protein loss was confirmed. Tc-HSA-D scintigraphy has an important role in diagnosing protein-losing enteropathy, but the abdomen is the only routinely scanned area. Additional scanning of egested radiotracer and the whole body can be useful for evaluating the disease severity.

Protein losing gastroenteropathy due to Helicobacter pylori infection without giant rugal folds, erosion or polyposis.

An 85-year-old woman was admitted to our hospital because of progressive hypoproteinemia and generalised oedema. Technetium-99m human albumin scintigraphy revealed protein leakage in the gastrointestinal tract. Upper gastrointestinal endoscopy revealed small whitish nodules from the gastric body up to the duodenal bulb. The urease test for Helicobacter pylori infection was positive. We diagnosed her as having protein-losing gastroenteropathy (PLGE) caused by H. pylori infection. The patient's hypoproteinemia and clinical symptoms promptly resolved after H. pylori eradication. Our results suggest that a trial of H. pylori eradication is warranted in patients with PLGE, even if endoscopy reveals neither giant rugal folds, erosion of the mucosa, nor polyposis, which are previously reported characteristic endoscopic findings of PLGE.

Enteropatía pierde-proteínas resuelta tras la reparación de una eventración abdominal / Protein-losing enteropathy resolved after ventral abdominal hernia repair

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Evaluation by MR Enterocolonography of Lansoprazole-induced Collagenous Colitis Accompanied with Protein-losing Enteropathy.

We herein describe a 69-year-old man suffering from chronic diarrhea caused by lansoprazole (LPZ)-induced collagenous colitis (CC) accompanied with protein-losing enteropathy (PLE), diagnosed by increased fecal alpha-1 antitrypsin clearance and the findings of leakage from the descending colon to the sigmoid colon on scintigraphy. MR enterocolonography (MREC) was also performed for differentiating digestive diseases, and inflamed findings were observed around the same portion as those on scintigraphy, suggesting that this region was responsible for protein loss in this case. The MREC findings improved after the cessation of LPZ, and hypoalbuminemia also improved simultaneously. This case suggests that MREC may be a new and useful diagnostic tool for CC with PLE.

Multiple small intestinal ulcers associated with protein-losing enteropathy secondary to cholesterol crystal embolism:a case report.

A 78-year-old man with hypertension, nephrosclerosis, and angina pectoris visited his family doctor with a history of fatigue and leg edema. He had a history of percutaneous coronary intervention 5 years prior, and was taking low-dose aspirin. Blood tests revealed hypoalbuminemia, gastrointestinal 99mTc-HSA scintigraphy was positive, and alpha-1 antitrypsin clearance was high;therefore, the hypoalbuminemia was thought to be secondary to a protein-losing enteropathy. A small bowel series revealed multiple, ring-shaped, longitudinal ulcers in the ileum. Balloon-assisted enteroscopy from the anus showed severe stenosis with an ileal ulcer. Since we were not able to diagnose the ulcers, mesalazine and supplemental nutritional care were provided. Four years after the hypoalbuminemia had been diagnosed, the patient died because of pulmonary congestion secondary to renal failure. An autopsy revealed severe atherosclerosis in his aorta and multiple cholesterol embolisms in his small intestine, kidney, stomach, colon, liver, and spleen. The multiple ulcers in the small intestine were thought to be caused by cholesterol crystal embolism, which should be considered in the differential diagnosis of small intestinal ulcers in elderly men or patients after cardiovascular intervention.

Protein-losing Gastroenteropathy Related to Mixed Connective Tissue Disease: A Case Report of a Successful Outcome and Literature Review.

We herein report the case of a 44-year-old woman who developed protein-losing gastroenteropathy (PLGE) with hypoalbuminemia as the first manifestation of mixed connective tissue disease (MCTD). Albumin leakage from the stomach and intestinal tract was demonstrated by 99mTc-labeled human serum albumin scintigraphy. The patient's response to prednisolone therapy was insufficient; therefore, additional cyclosporin A (CsA) treatment was administered, and clinical remission was achieved. We concluded that although PLGE is a rare complication of MCTD, it may manifest as an initial clinical episode of MCTD. Furthermore, CsA can be a useful treatment option for refractory PLGE related to MCTD.

Applicability of 99m Tc-Labeled Human Serum Albumin Scintigraphy in Dogs With Protein-Losing Enteropathy.

BACKGROUND: Diagnosis of protein loss into the gastrointestinal tract using noninvasive techniques is challenging. In people, scintigraphy not only is a sensitive tool to confirm protein-losing enteropathy (PLE), but it also allows for localization of protein loss. HYPOTHESIS/OBJECTIVES: To investigate the feasibility of 99m Tc-labeled human serum albumin (HSA) scintigraphy in dogs with PLE in comparison with control dogs. ANIMALS: A total of 8 clinically healthy control research dogs and 7 client-owned dogs with gastrointestinal clinical signs and hypoalbuminemia (serum albumin concentration <2.0 g/dL). METHODS: Prospective case-control study. After IV injection of 400 MBq freshly prepared 99m Tc HSA (30 mg/dog), images of the abdomen were obtained 10, 60, 120, and 240 minutes postinjection. Additional images of the salivary and thyroid glands were obtained to rule out free 99m Tc. A scan was considered positive for PLE when radiopharmaceutical exudation was detectable in the intestinal tract. RESULTS: Only 1 control dog showed exudation of the radiopharmaceutical into the intestinal tract. No free 99m Tc was detected in any dog. In dogs with PLE, focal small intestinal and diffuse small intestinal radiopharmaceutical exudation into the bowel was detected in 2 and 3 dogs, respectively, whereas in 2 dogs, there was disagreement about whether radiopharmaceutical exudation was focal or diffuse. CONCLUSION AND CLINICAL IMPORTANCE: 99m Tc-labeled HSA scintigraphy was feasible to diagnose PLE in dogs.

Protein-losing enteropathy in a patient with familial adenomatous polyposis and advanced colon cancer.

A 29-year-old female visited a hospital because of increasingly severe lower leg edema. She was diagnosed as having multiple polyps in the stomach and colon by gastroscopy and sigmoidoscopy as well as multiple liver tumors by abdominal CT. She was referred to our hospital for further examination. Total colonoscopy revealed a type 2 tumor in the transverse colon and more than 200 polyps distributed throughout the colorectum. Biopsies of the tumor and polyps showed histological characteristics of adenocarcinoma and tubulovillous adenoma, respectively. Thus, she was diagnosed as having metastatic colon cancer derived from familial adenomatous polyposis (FAP). Laboratory tests showed a marked hypoalbuminemia of 1.1 g/dl. The fecal alpha-1 anti-trypsin test showed abnormal clearance (62.1 ml/day), and scintigraphy using 99mTc-human serum albumin revealed protein loss in the whole colon. Multiple ligation probe amplification analysis of the APC gene identified a germline duplication of exons 11-13. Direct sequencing of the reverse transcription PCR products of APC mRNA revealed a deletion of 25 base pairs and a tandem duplication of exons 11-13. This case was considered to be protein-losing enteropathy resulting from numerous colonic tubulovillous adenomas and advanced colon cancer in a FAP patient with unusual mutational events in APC.

Early Impact of Fontan Operation on Enteric Protein Loss.

BACKGROUND: Protein-losing enteropathy (PLE) is a challenging complication after a Fontan operation. Subclinical enteric protein loss may precede development of overt PLE. We evaluated the acute effects of Fontan circulation on enteric protein loss and mesenteric vascular resistance. METHODS: A prospective cohort study was performed evaluating enteric protein loss in children undergoing Fontan operations. Stool alpha-1 antitrypsin (A1AT) concentration was measured in the preoperative, early postoperative, and intermediate postoperative (3-9 months) periods. The intestinal circulation was characterized by Doppler-derived resistance indices of the superior mesenteric artery (SMA), and serum albumin and protein levels were obtained. RESULTS: We enrolled 33 participants at a median age at operation of 3.0 years (interquartile range [IQR], 2.5-3.3 years). No clinical PLE was observed. Six of the 93 stool samples obtained had elevated A1AT levels (>54 mg/dL), with 2 abnormal samples at each of the 3 time points. Two of the 5 participants with elevated stool A1AT values had significant hemodynamic disturbances requiring intervention (junctional bradycardia or tricuspid stenosis). There was no difference in SMA resistance in the preoperative versus early postoperative periods (p = 0.9). Serum albumin levels were lower in the early postoperative period compared with the preoperative period (3.2 mg/dL [{IQR}, 2.9-3.5] versus 4.1 mg/dL; IQR, 3.4-4.5; p = 0.01) but did not correlate with abnormal stool A1AT concentration or SMA resistance indices. CONCLUSIONS: The Fontan operation does not commonly result in acute development of increased enteric protein loss. However, increased enteric protein loss may occur in children before or after a Fontan operation, particularly when hemodynamic disturbances are present.