Pediatric localized intestinal lymphangiectasia treated with resection

Figures & data

Figure 1 Band-like cystic mass seen on the follow-up ultrasound.

Figure 2 MRI showed multilocular cystic mass involving the mesenteries.

Figure 3 Microscopic picture showing typical changes for PIL: dilated lymphatics in subserosa, submucosa and lamina propria.

Note: Scale bars represent 500μm.
Abbreviation: PIL, primary intestinal lymphangiectasia.

Table 1 Pediatric localised PIL cases treated successfully with surgical resection.

Year of publication	Age	Sex	Ethnicity	Length of history	Presenting symptoms	Investigations/findings	Length/localization of bowel resected	Follow-up period—outcome
1998 Persić et al^Citation8	14 years	Male	Caucasian	13 years	Diarrhea, edema, low albumin/protein, left leg edema	18 Cr labeled albumin study: 18% recovery, lymphangiography: significant lymphatic obliteration in both legs	305 cm small bowel	6 years no diet normal growth, no symptoms
2001 Uğuralp et al^Citation9	7 years	Male	Caucasian	4 years	Recurrent abdominal pain, diarrhea, vomiting, neck swelling (asymmetric)	CT: diffuse bowel wall thickening, blind biopsy: normal, laparotomy + biopsy: PIL	70 cm jejunoileal	1 year with diet—growth percentile increased, no symptoms
2008 Katoch et al^Citation10	6 months	Male	Indian	1 week	Abdominal pain, palpable mass, intussusception	None	7 cm	None
2009 Kim et al^Citation11	8 years	Male	Korean	3 months	Acute abdomen, vomiting, diarrhea, generalized edema	Upper/lower endoscopy + biopsy: petechiae in small bowel, otherwise normal	150 cm (107+26+17) jejunoileal	10 years no diet—normal growth normal lab values
2017 Mitsiakos et al^Citation12	2 days	Male	Caucasian	IU diagnosed	None	US: multilocular cystic mass, MRI: multiple cystic lesion of the mesentery	~10 cm jejunoileal	1 month no diet—no symptoms
2018	10 years	Male	Caucasian	3 months	Recurrent abdominal pain, diarrhea	US: band like cystic mass, MRI: multilocular cystic mass involving the mesenteries	30 cm ileum	4 years no diet—normal growth, normal lab values

Note: Result of the literature search: pediatric localized PIL treated with resection.

Abbreviations: CT, computed tomography; IU, intrauterine; MRI, magnetic resonance imaging; US, ultrasound; PIL, primary intestinal lymphangiectasia.

PersićMBrowseNLPrpićIIntestinal lymphangiectasia and protein losing enteropathy responding to small bowel restrictionArch Dis Child1998782194

 Web of Science ®Google Scholar

UğuralpSMutusMKutluOCetinSBaysalTMizrakBPrimary intestinal lymphangiectasia: a rare disease in the differential diagnosis of acute abdomenJ Pediatr Gastroenterol Nutr200133450851011698774

 PubMed Web of Science ®Google Scholar

KatochPBhardwajSLymphangiectasia of small intestine presenting as intussusceptionIndian J Pathol Microbiol200851341141218723975

 PubMed Web of Science ®Google Scholar

KimNRLeeSKSuhY-LPrimary intestinal lymphangiectasia successfully treated by segmental resections of small bowelJ Pediatr Surg20094410131719159713

 PubMed Web of Science ®Google Scholar

MitsiakosGDrogoutiEDrogoutiMDoitsidisCPazarliESpyridakisICongenital intestinal lymphangiectasia. A case reportJ Pediatr Neonatal Individ Med201871070106

 Web of Science ®Google Scholar