ABSTRACT
Objective To improve the diagnosis and treatment level of primary intestinal lymphangiectasia (PIL) in infants.Methods Clinical,laboratory,gastroscopy imaging,lymph radionuclide imagining,and therapeutic intervention data in eight patients admitted with PIL in Beijing Children's Hospital from Jan.2007 to Feb.2012 were reviewed.Results Their ages ranged from 4 to 8 months old.The common complaints were edema(8/8 cases),diarrhea (8/8 cases),infection (8/8 cases),and ascites (7/8 cases).Other symptoms included vomiting (5/8 cases),low body weight (2/8 cases),and convulsions (2/8 cases).Infections involving respiratory tract,blood,and gastrointestinal system were prominent.The causal pathogens for the infections were bacteria,virus,fungi,and ectosarc.The laboratory abnormalities included lymphocytopenia (8/8 cases),hypoalbuminemia (8/8 cases),and hypogammaglobulinemia (8/8 cases).On immunologic evaluation,the CD4 cell counts and serum IgG levels were significantly decreased(7/7 cases) while B cell and NK cell counts were normal.The gastroscopy revealed nodular lesions in duodenal that appeared white opaque spots(8/8 cases).Further pathological examinations indicated dilated lymphatic channel in mucosal and submucosal(5/8 cases).Lymph radionuclide imaging discovered abnormalities consistent with PIL in 6 out of 8 patients,including 3 cases that were negative for pathological examination.A multidisciplinary approach was taken to treat each patient.The low-fat and medium chain triglycerides-rich diet was introduced with supplements of albumin and globulin.Infection control and edema alleviation were well managed through medication.However,diarrhea remained a problem.Only 2 out of 8 patients had recovered from hypoalbuminemia and lymphocytopenia.One case had a complete remission after surgical therapy withno relapse.Conclusions PIL in infant has common clinical presentations with an exception of more severe infection.Lymph radionuclide imaging is a sensitive diagnostic method.Medication treatment for infant PIL is not satisfactory.Surgical management is recommended if the locus of pathology is confined.