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Table 2 Summary of articles/case reports of intestinal lymphangiectasia treated with second-line therapy. wks; week-old, yr.; year-old, mo.; month-old

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

Author

Age

Group

Type of lymphangiectasia

Intestinal involve

3rd space involve

Response to diet therapy

Type of 2nd-line therapy

Results of 2nd-line therapy

CP Chen et al. (2003) [24]

49 yr.

1

Focal intestine

Focal

No

No

Surgical resection

Remission

L Zhu et al. (2010) [32]

22 yr.

1

Focal intestine

Focal

No

Limited data

Surgical resection

Remission

44 yr.

1

Focal intestine

Focal

No

Limited data

Surgical resection

Remission

71 yr.

1

Focal intestine

Focal

No

Limited data

Surgical resection

Remission

55 yr.

1

Focal intestine

Focal

No

Limited data

Surgical resection

Remission

W Kneist et al. (2013) [25]

58 yr.

1

Focal intestine

Focal

No

No

Surgical resection

Remission

J Mari et al. (2019) [28]

10 yr.

1

Focal intestine

Focal

No

Limited data

Surgical resection

Remission

G Kuroiwa et al. (2001) [26]

21 yr.

1

Focal intestine

Focal

No

No

Octreotide

Recurred after discontinuation

L Filk et al. (2004) [34]

25 yr

Limited data

Limited data

Limited data

Limited data

No

Octreotide

Clinical improvement

S Sari et al. (2010) [30]

N = 6, age 2–24 mo.

Limited data

Limited data

Limited data

Limited data

No

Octreotide

Clinical improvement

Suehiro et al. (2012) [22]

63 yr.

1

Focal intestine

Focal

No

No

Octreotide

Clinical improvement

MJ Alshikho et al.(2016) [35]

24 yr

Limited data

Limited data

Limited data

Limited data

No

Coconut oil + Octreotide

Clinical improvement

Troskot et al. (2015) [31]

42 yr.

2

Diffuse intestine

Diffuse

No

No

Octreotide

Clinical improvement

Z Altın et al. (2018) [2]

34 yr.

3

Extensive

limited

Yes

No

Octreotide

Recurred after discontinuation

Acer-Demir et al. (2020) [23]

3 yr.

3

Extensive

Diffuse

Yes

No

Octreotide âž”

Surgical resection

Died d/t uncontrolled symptoms

MacLean et al. (2002) [27]

14 yr.

3

Extensive

Diffuse

Yes

No

Octreotideâž”

Tranexamic acid

Clinical improvement

D Prasad et al. (2019) [29]

N = 2, age 2–18 yr.

2

Diffuse intestine

Diffuse

No

No

Octreotide

Clinical improvement

N = 4, age 1–10 yr.

3

Extensive

Diffuse

Yes

No

Octreotide + Tranexamic acid

Clinical improvement

Ozeki et al. (2016) [20]

12 yr.

3

Extensive

Diffuse

Yes

No

Propranolol➔Everolimus

Remission for 12 months