LYMPHANGIOMATOSIS OF SMALL INTESTINE
WITH MULTIPLE CHYLOLYMPHATIC
MESENTERIC CYSTS: A RARE CASE REPORT
WITH REVIEW OF LITERATURE
Shashi Kant Kumar , Punam Prasad Bhadani, Ruchi Sinha ,
Kaushal Kumar, Shuchismita, Nishi
Department of Pathology & Laboratory Medicine
All India Institute of Medical Sciences, Patna
INTRODUCTION
PATHOLOGIC FINDINGS
 Lymphangioma is a benign,
slow growing tumour
characterized by proliferation of
the thin-walled lymphatic spaces.
Most lymphangiomas are found
in the head and neck; intra
abdominal locations particularly
small intestine are very unusual.
a
They result from an
embryological failure of the
lymphatic system: lack of
communication between small
bowel lymphatic tissue and the
main lymphatic vessels result in
blind cystic lymphatic spaces
lined by endothelial layers.
c
Clinical presentation is variable
from asymptomatic lump to
complications like intestinal
obstruction, volvulus or even
torsion of intestine.
b
d
e
a,b - Gross picture shows resected segment of terminal ileum with attached part
of mesentery. Multiple varying sized cystic structure present in the intestinal wall
and mesentery.
c,d- histological sections show variable sized, markedly dilated lymphatic
channels throughout the bowel wall as well as in the mesentery, lined by
flattened endothelial cells.
e- Mesenteric cyst wall shows fibro-connective tissue lined by flattened cuboidal
epithelium
CASE REPORT
A 2 year 6 months old boy was admitted with complaints of abdominal pain, vomiting,
and abdominal distension of 6 month duration.
USG of abdomen show multiple cystic lesion in the wall of intestine with dilated
proximal bowel loop.
Excision of around 12 cm ileum with cyst was done en bloc and sent for HPE
GROSS- Resected segment of terminal
ileum received with attached part of
mesentery. Multiple varying sized cystic
nodules present more towards serosa and
mucosa too. Thin milky fluid was drained
out from the small cystic nodules.
Attached multiple mesenteric cyst was
also seen towards serosa , size varies
from around 2-5 cm.
MICROSCOPY- The histological
sections show variable sized, markedly
dilated lymphatic channels throughout
the bowel wall as well as in the
mesentery, lined by flat tened endothelial
cells. Few of them contain proteinaceous
fluid.
Mesentric cyst show presence of flattened
cuboidal epithelium with presence of
fibroconnective tissue.
Few sub endothelial lymphoid follicles
also seen.
The final diagnosis made was cystic
lymphangioma with chylolymphatic
mesenteric cyst.
CONCLUSION
 Lymphangiomas are exceedingly rare
malformation of lymphatics in GIT with
involvement of terminal ileum along with
multiple mesenteric cyst.
Definitive preoperative diagnosis may not
be possible. However optimal surgical
management can result in remarkably good
prognosis.
REFERENCES
1-Ratan SK, Ratan KN, Kapoor S, Sehgal T: Giant chylolymphatic cyst of the jejunal mesentery in a child: report of a case. Surg Today 2003, 33(2):120.
2-K. N. Rattan, V. J. Nair, M. Pathak, and S. Kumar, “Pediatric chylolymphatic mesenteric cyst—a separate entity from cystic lymphangioma: a case series,” Journal of Medical Case Reports, vol. 3, article 111, 2009.
3-Rieker RJ, Quentmeier A, Weiss C, Kretzschmar U, Amann K, Mechtersheimer G, Bläker H, Herwart OF. Cystic lymphangioma of the small-bowel mesentery: case report and a review of the literature. Pathol Oncol Res 2000; 6: 146-148.
4-Chang SC, Tu SH, Shi MY, Huang SH, Chen KM. Mesenteric lymphangioma causing bowel obstruction: report of one case. Acta Paediatr Taiwan 2002 Jan-Feb; 43(1):43-45.