Clinical Profile, Response to Therapy, and Outcome of Children with Primary Intestinal Lymphangiectasia

Abstract

Objective: Intestinal lymphangiectasia (IL; primary or secondary) is an important cause of protein-losing enteropathy. We evaluated the clinicolaboratory profile, response to therapy, complications, and outcome of children with primary IL (PIL). Methods: Consecutive children (≤18 years) diagnosed with PIL (clinical setting, typical small bowel histopathology, and exclusion of secondary causes) from 2007 to 2017 were evaluated. Results: Twenty-eight children with PIL (16 boys, age at symptom onset-12 [1–192] months and at diagnosis 8 [1–18] years) were studied. Pedal edema (93%), chronic diarrhea (78.6%), and recurrent anasarca (64%) were the common presentations. Ascites, pleural, and pericardial effusion were seen in 64 (n-18; chylous-5, non-chylous-13), 18, and 18% cases, respectively. Hypoproteinemia, hypoalbuminemia, hypocalcemia, and lymphopenia were present in 82, 82, 75 and 39% cases, respectively. Duodenal biopsy established the diagnosis in 86% cases, while 14% required distal small bowel biopsies. Dietary therapy was given in all and 6 cases required additional therapy (octreotide-6, tranexamic acid-3, and total parenteral nutrition-1). Lymphedema (3/5 vs. 1/23), pleural effusion (4/5 vs. 1/23), and the need for additional therapy (4/5 vs. 2/23) were significantly more in patients with chylous ascites (n = 5) than those without chylous ascites (n = 23). Twenty-four cases in follow-up (39 [6–120] months) showed improvement; however, 8 required readmission (symptom recurrence-6 [25%], complication-2 [8.3%], Budd Chiari Syndrome-1, and abdominal B cell lymphoma-1). Conclusion: Presence of chylous ascites suggests severe disease in children with PIL. Majority of PIL children respond to dietary therapy; only 20% need additional therapy. Long-term follow-up is essential to monitor for symptoms relapse and complications.

Individual Approach for Treatment of Primary Intestinal Lymphangiectasia in Children: Single-center Experience and Review of the Literature

“…(2010) [30], Prasad et al (2019) [29], and some other reports described clinical improvement after octreotide treatment but there is no information on the long-term e cacy of octreotide (Table 2) [37,38]. We analyzed patient characteristics in the aspects of location and extent of lymphangiectasis in reports of clinical outcomes after using octreotide.…”

Individual Approach for Treatment of Primary Intestinal Lymphangiectasia in Children: Single-center Experience and Systemic Review

“…There are also reports that describe the recurrence of lymphangiectasis after the discontinuation of octreotide. Sari et al (2010) [30], Prasad et al (2019) [29], and some other reports described clinical improvement after octreotide treatment but there is no information on the long-term e cacy of octreotide (Table 2).…”

“…Only two case reports presented treatment experience with tranexamic acid [27,29]. Tranexamic acid 25 mg/kg/dose three times a day was used orally (maximum 1,000 mg) for ve days [29], and patients showed clinical improvement after one month of treatment [27]. The mechanism of antiplasmin therapy is the normalization of tissue brinolytic activity [36].…”

Individual Approach for Treatment of Primary Intestinal Lymphangiectasia in Children: Single-center Experience and Systemic Review

“…There are also reports that describe the recurrence of lymphangiectasis after the discontinuation of octreotide. Sari et al (2010) [30], Prasad et al (2019) [29], and some other reports described clinical improvement after octreotide treatment but there is no information on the long-term e cacy of octreotide ( Table 2). We analyzed patient characteristics in the aspects of location and extent of lymphangiectasis in reports of clinical outcomes after using octreotide.…”

“…Only two case reports presented treatment experience with tranexamic acid [27,29]. Tranexamic acid 25 mg/kg/dose three times a day was used orally (maximum 1,000 mg) for ve days [29], and patients showed clinical improvement after one month of treatment [27]. The mechanism of antiplasmin therapy is the normalization of tissue brinolytic activity [37].…”