IJCRR - vol 05 issue 21, November
Date of Publication: 21-Nov-2013
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A RARE CAUSE FOR HUGE ABDOMEN
Author: Govindarajalu Ganesan, Ganesh babu
Abstract:Both intraabdominal cystic lymphangioma and chylolymphatic mesenteric cyst present as huge abdominal mass and both have chyle as their content. Hence the only way to differentiate between these two conditions is by histopathological examination which is extremely important as lymphangiomas are more invasive and relapsing than mesenteric cyst. Here we report a rare case of huge abdomen due to cystic lymphangioma arising from lesser omentum that did not have recurrence for more than six years after surgical excision.
Keywords: intraabdominal cystic lymphangioma, lesser omentum, chyle, recurrence
Lymphangiomas is of three types- capillary, cavernous and cystic lymphangiomas (1-3). Cystic lymphangiomas are most commonly found in head and neck and axilla of young children (1-8).
But intraabdominal lymphangiomas are extremely rare and the incidence is only in 1 per 100000 individuals (2, 9) While occurring intraabdominally, lymphangiomas occur most frequently in the mesentery (2 - 6, 9). But it is extremely rare to find intraabdominal lymphangioma arising from lesser omentum (10, 11). Hence an extremely rare patient of cystic lymphangioma arising from lesser omentum and presenting as huge abdominal mass is reported here.
A 29 year old female presented with a huge painless soft, smooth, nontender, mobile mass occupying umbilical and hypogastric regions extending on either sides to both lumbar region and both iliac fossa for a period of two months. Ultrasound abdomen showed a large thin walled cyst occupying abdomino-pelvic region with septations suggestive of lymphatic cyst. Intravenous pyelogram showed normal function and drainage of both kidneys.
On 11th July 2007, the patient underwent laparotomy by midine incision. A huge cyst was arising from lesser omentum extending upto pelvic region. (fig.1).Cyst was excised in total and sent for biopsy.Patient had an uneventful post-operative period and discharged on 8th post-operative day.
The cyst was found to contain more than 1 litre of chyle as its content. Biopsy report showed cyst wall lined by flattened endothelium. The cyst wall showed fibro-collagenous tissue, smooth muscle bundles and aggregates of lymphocytes suggestive of cystic lymphangioma. (fig.2).Hence this patient is a case of huge intraabdominal chylous cystic lymphangioma arising from lesser omentum.
Pathologically, lymphangiomas consist of three groups :1) capillary lymphangiomas composed of small lymphatics, 2) cavernous lymphangiomas
composed of larger lymphatics and 3) cystic lymphangiomas composed of large macroscopic lymphatic spaces containing collagen and smooth muscle (1). Cystic lymphangiomas contain serous fluid or chyle as its content (2,4,5) Cystic lymphangiomas having chyle as its content can be called as chylous cystic lymphangiomas. Histopathologically cystic lymphangiomas are lined by a single row of flat endothelial cells and contain fibrous tissue, aggregates of lymphoid tissue and smooth muscles in the cyst wall (7).
But mesenteric cysts which are often confused with intraabdominal cystic lymphangiomas (12-14) do not contain smooth muscles in the cyst wall in histology (14). Chylolymphatic mesenteric cyst is a type of mesenteric cyst which has chyle as its content and can present as huge abdominal mass (14). Intraabdominal cystic lymphangiomas commonly has chyle as its content and can also present as huge abdominal mass (4). Hence it is extremely important to differentiate between these two conditions.
There are no blood tests to confirm the diagnosis of these two lesion (4). Radiological investigations like ultra sound or computed tomography are very sensitive in diagnosing intraabdominal cysts but are not very specific and cannot differentiate between these two conditions (4, 6, 15). Hence the only way to differentiate between these two conditions is by histopathological examination (12-14). The differentiating histologic feature is the presence of smooth muscles in the cyst wall of intraabdominal cystic lymphangiomas (1-9,12)which are absent in the cyst wall of chylolymphatic mesenteric cyst (14).
An exact histological diagnosis is extremely important as lymphangiomas are more invasive, aggressive and relapsing than mesenteric cyst (3,6,12,13,15,16).
Our patient is on regular follow up for 6 years and she did not have any evidence of recurrence for more than six years after surgical excision.
Though intraabdominal chylous cystic lymphangioma and chylolymphatic mesenteric cyst are exactly similar to one another clinically, radiologically and intraoperatively, they are two entirey different lesions histologically. Histologically smooth muscles are found in the cyst wall of chylous cystic lymphangioma, but smooth muscles are absent in the cyst wall of chylolymphatic mesenteric cyst. This histological differentiation is extremely important as lymphangiomas are more invasive and relapsing than mesenteric cyst. But our patient with cystic lymphangioma arising from lesser omentum did not have any evidence of recurrence for more than six years after surgical excision and hence is reported here.
We are extremely thankful to Professor Dr. N. Anantha Krishnan for his valuable guidance which has helped us greatly to prepare this article. We are also extremely thankful to Dr. Nilotpal Chowdhury, the pathologist, who has helped us greatly in preparing the photomicrograph of the slide of the patient.
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