Individual approach for treatment of primary intestinal lymphangiectasia in children: single-center experience and review of the literature

Abstract Background Intestinal lymphangiectasia is a rare disease. Thus, prospective studies are impossible, and therapy still controversial. Several medicines suggested for treatment but there no existing indications drug choice guidelines. We aimed to introduce the action mechanism of each overview in single-center experience review literature on second-line primary intestinal lymphangiectasia. Method Children under 18 years old diagnosed with from June 2000 2020 were included retrospectively reviewed study. Capsule endoscopy, MR lymphangiography, or whole-body MRI investigating extent abnormal lymphatic vessels addition endoscopy biopsy conducted. The individual approaches depended upon lymphangiectasis locations involved. Results Only one patient showed response dietary therapy. One was successfully cured after two therapeutic embolization. Octreotide tried patients who had extensive lymphangiectasis. Lymphangiectasis recurred when octreotide used 3 months patient, effect other patient. Sirolimus four patients. Two them lesions only intestine, others former group clinical improvement 3–4 sirolimus treatment, whereas latter 1 month treatment. Conclusion Surgery embolization potential option focal lesions. not an optimal effective safe can be first