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

Table 1 Clinical characteristics of 7 patients diagnosed with primary intestinal lymphangiectasia. wks; week-old, yr.; year-old

Patient number	Age range at diagnosis^a	Time interval of 2nd-line therapy^b	Group	Type of lymphangiectasia	Intestinal involve	3rd space involved	Response to diet therapy	Type of 2nd-line therapy
1	Infant	–	1	Focal intestine	Focal	No	Yes	None
2	Child	0.5 yr	1	Focal intestine	Focal	No	No	Surgery
3	Child	8 yr	1	Focal intestine	Focal	No	No	Embolization
4	Adolescent	0.7 yr	3	Extensive	Diffuse	Yes	No	Octreotide➔Sirolimus
5	Neonate	3 yr	3	Extensive	Diffuse	Yes	No	Octreotide➔Sirolimus
6	Neonate	8.9 yr	2	Diffuse intestine	Diffuse	No	No	Sirolimus
7	Child	0.7 yr	2	Diffuse intestine	Diffuse	No	No	Sirolimus

^aThere is an accurate diagnosis age, but age is expressed as a range for anonymity
^bThe time when the second line therapy started from the diagnosis

ISSN: 1471-2431