Isolated Intestinal Lymphatic Anomaly Underwater Endoscopic Identification and Therapeutic Intervention Disclosures are stated below. We present a 12-month-old with a past medical history of protein-losing enteropathy secondary to presumed primary intestinal lymphangictasia. This diagnosis was based off of outside hospital endoscopic biopsies showing dilated villus lymphatics. PLE involves excessive leakage of protein-rich lymphatic fluid into the intestinal lumen. It is more of a symptom of an underlying condition, including those with erosive and non-erosive gastrointestinal disease as well as lymphatic abnormalities. Clinical features of this diagnosis include peripheral edema, diarrhea, and ascites, as our patients showed chronic evidence of, as well as biochemical features including hypoalbuminemia, hypogammaglobulinemia, lymphopenia, and malabsorption. This diagnosis is typically confirmed with an elevated alpha-1 antitrypsin in the stool, such as our patient. Endoscopically, primary intestinal lymphangictasia often presents as scattered diffuse white spots or plaques on the intestinal mucosa representing those dilated lymphatic channels. Treatment of this disease process is to address the underlying cause of secondary dietary modifications, including high-protein, low-fat diet with medium-chain triglycerides, supportive care including albumin infusions and diuresis, as well as potential interventional procedure to address lymphatic abnormalities. Our patient presented with acute onchronic watery diarrhea and peripheral edema. On arrival to the ED, he was hypovolemic, requiring intravenous resuscitation. His labs were pertinent for a metabolic acidosis, hypokalemia, and hypoalbuminemia. A GIPCR did detect norovirus infection. He was initiated on Actreotide with a dose titrated to 75 mics twice a day and was continued on his home low-fat formula. Although his diarrhea improved with clearance of his norovirus infection, he continued to require frequent infusions of 25% albumin and diuresis for clinical edema. He ultimately remained hospitalized for this edema and fluctuations in albumin and electrolytes. After extensive coordination, he underwent a combined procedure of a dynamic contrast enhanced magnetic resonance lymphangiogram, cardiac catheterization, and an upper endoscopy. The procedure was performed in the cardiac catheterization lab in the supine position under general anesthesia. The patient first underwent Gatavist injection into the inguinal lymph nodes with the help of interventional radiology and was then transferred to MRI for the lymphangiogram. After this was completed, he was transported to the cardiac catheterization lab with right-sided cardiac catheterization showing normal central venous pressure without evidence of obstruction. Endoscopy was then pursued. Figure A shows the MRI lymphangiogram showing the bilateral lumbar lymphatic ducts and thoracic duct being well visualized with contrast, which appear to be normal and patent. There is visualized lymphatic leakage in the abdomen into a pouch that is rightward of the spine and extends posteriorly, as evidenced by the red arrow, which is suspected to be the duodenum. Figure B re-demonstrates these findings on the delayed IR steady-state free procession sequence as well as the contrast moving into other portions of the small intestine. This is an ultrasound-guided image showing increased duodenal wall thickening. The upper endoscopy included a 27 French endoscope being introduced through the mouth and advanced to the second portion of the duodenum. The esophagus and stomach appeared grossly normal. Lymphangiangtasia was present in the first and second portion of the duodenum with multiple white punctate lesions. Underwater submersion revealed white lymph aggressively flowing from a singular location separate from the orifice of the bile duct at the major papilla. After IR injected methylene blue and a lipid mixture into the lymphatic system, this mixture was then seen to drain from the singular location. Coagulation for tissue destruction was performed with silver probe bipolar cautery and argon beam coagulation. After treatment and with continued underwater submersion, there was no visible drainage from that site. Total procedure time was deemed to be 5 hours with total time under anesthesia to be 6 hours. This long procedure time may have likely been decreased if endoscopy was pursued first. Total fluoroscopy time was deemed to be about 16 minutes. After the procedure, patient did well with no further clinical evidence of edema. He was tolerating his home Toler-X feedings and was discharged home with close outpatient follow-up. The patient continued to show clinical and biochemical improvement far beyond the time of the endoscopic procedure. As you can see in the graphs below, he had normalization of his albumin, IgG, as well as his lymphopenia. His stool alpha-1 antitrypsin also normalized after the procedure, again demonstrating the durability of the endoscopic ablation of his lymphatic abnormality. Although primary intestinal lymphangictasia is generally characterized by diffuse endoscopic findings, our case demonstrates a unique presentation with a singular enlarged vessel responsible for lymphatic leakage and subsequent protein-losing enteropathy. Underwater, endoscopic visualization and verification of treatment success was utilized in this case and should be considered in the future to further characterize intestinal lymphatic leaks. Endoscopic findings can be correlated with other imaging modalities like dynamic contrast magnetic resonance lymphangiography to provide a comprehensive understanding of lymphatic abnormalities. Underwater endoscopic visualization of intestinal lymphatic leak contributes to the understanding of the pathophysiology of lymphatic disorders and protein-losing enteropathy, leading to improved diagnostic and treatment strategies.

underwater endoscopy

protein-losing enteropathy

intestinal lymphangiectasia

lymphatic leakage

endoscopic treatment