Enteral Nutrition as a Primary Therapy for Intestinal Lymphangiectasia: Value of Elemental Diet and Polymeric Diet Compared with Total Parenteral Nutrition

Abstract

Intestinal lymphangiectasia (IL) is a rare disease requiring oral fat restriction. The aim of this study was to evaluate the efficacy of enteral nutrition compared to that of total parenteral nutrition (TPN). We retrospectively reviewed nine patients with IL presenting with protein-losing enteropathy. Of these, seven patients not responding to a low-fat diet were treated with elemental diet (ED), polymeric diet (PD) containing medium-chain triglycerides, or TPN. Improvement in serum total protein was observed in two of three on ED and in one of two on PD, compared with three of three on TPN. Enteric protein loss was improved in two of two on ED, one of two on PD, and two of two on TPN. Outpatients who continued to receive enteral nutrition maintained a total protein level. Enteral nutirition appears to be as effective as TPN for patients with IL, and it may provide a valid and safe alternative therapy.

Editor's quiz: Protein, protein everywhere…but none to keep

“…Endoluminal examination of the small bowel with histological correlation is usually necessary for diagnosis2; however, in our patient, the initial upper gastrointestinal endoscopy series was normal, hence evaluation of more distal small bowel was necessary. Treatment of the underlying disease can improve outcome,3 and dietary modification with a low fat diet and substitution of long-chain fatty acids with medium-chain triglycerides may prevent further lacteal dilatation 4 5. Despite the aforementioned dietary measures, as well as supplementary parenteral nutrition, our patient continued to deteriorate.…”

Waldmann’s disease: a rare cause of protein losing enteropathy in an adult patient

“…In patients not responding to a low fat diet, enteral nutritional therapy (elemental, semi-elemental and polymeric diets) may be required. In a few severe cases, total parenteral nutrition is warranted (8). Alternative treatment options with variable efficacy are discussed in several publications.…”

How to treat an extensive form of primary intestinal lymphangiectasia?

“…In addition, gamma globulin infusions should be considered in the presence of recurrent infections, preceded by low serum IgG. We did not apply either therapy, as Aoyagi et al [6] have shown that enteral nutrition is an effective substitute for TPN in patients with PIL. Gamma globulin therapy was not used because there was no obvious infection.…”