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ABDOMINAL IMAGING Table of Contents   
Year : 2002  |  Volume : 12  |  Issue : 3  |  Page : 347-348
Congenital cystic lymphangioma with an uncommon presentation


Dept of Radiodiagnosis, Mother and child Health Institute of SMS Medical College, Rajasthan, India

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Keywords: Lymphangioma, Multiculated retroperitoneal mass

How to cite this article:
Parakh P, Shah V, Udawat M, Lalwani N. Congenital cystic lymphangioma with an uncommon presentation. Indian J Radiol Imaging 2002;12:347-8

How to cite this URL:
Parakh P, Shah V, Udawat M, Lalwani N. Congenital cystic lymphangioma with an uncommon presentation. Indian J Radiol Imaging [serial online] 2002 [cited 2020 Aug 8];12:347-8. Available from: http://www.ijri.org/text.asp?2002/12/3/347/28478

   Introduction Top


Lymphangiomas are benign congenital malformation of lymphatic system and are common in pediatric practice specially involving neck and axillary soft tissue. However it may present at few uncommon and rare sites including spleen, mediastinum, retroperitoneum, ovary and mesentery [1].

Clinical presentation is variable and may be misleading; therefore complex imaging studies are required in evaluation. We present a case of rare retroperitoneal lymphangioma with an uncommon presentation.


   Methods and Materials Top


An eight month old male child presented in our pediatric hospital with the complaint of gradually increasing abdominal mass since last four to five months. On clinical examination the lesion was huge extending from epigastrium to hypogastrium and laterally to flanks. On close interrogation parents revealed that the small hump was present since birth, which remained unnoticed because it was asymptomatic.

Plain film of abdomen showed a huge soft tissue mass lesion occupying almost whole abdomen, displacing the stomach bubble and bowel loops on the left side. Both hemi diaphragms were pushed upwards.

Sonographic evaluation performed with five MHz linear transducer, which showed large multiseptate cystic, fluid filled lesion having enhanced through transmission. The Multiloculated cystic lesion had cysts of varying sizes (measuring maximally upto six cm) almost completely filling the abdomen. Septae measured about 2 mm in thickness [Figure - 1]. The mass showed clear cleavage from surrounding structures like liver, gall bladder, pancreas, kidneys and urinary bladder. No pressure effect was seen over ureter or kidney. No evidence of ascitis was seen. On the basis of sonographic findings a provisional diagnosis of cystic lymphangioma was given. CT abdomen confirmed sonographic findings and in addition it showed the cyst was displacing bowel loops anterolaterally on left side. The lesion showed attenuation values within loculi ranging from - 10 to + 10 HU [Figure - 2].

The routine investigations including blood, urine and biochemistry were within normal limits.

On surgical exploration a large retroperitoneal cystic, soft and fluctuating lesion was found, which was separate from mesentery and showed clear cleavage from all retroperitoneal organs. It was removed in toto and an incision over the specimen showed milky white chyle in the cyst, which confirmed the radiological diagnosis [Figure - 3]. The histopathology also confirmed lymphangioma


   Discussion Top


There are very few reports of retroperitoneal cystic lymphangiomas in radiology literature, probably because of it's rare location. The only relevant study done on nineteen patients is reported from "Armed force institute of Pathology, Washington DC" [2]. This study has shown that 68% of the lesions were confined to one side of retroperitoneum, especially more on left side. Out of nineteen only one showed midline crossing and two showed hemorrhage within.

In our case, the lesion was not only at a rare site that is the retroperitoneum but also crossed mid line to involve whole abdomen hence it is one of the very uncommon presentations.

The postulated mechanism of formation of lymphangioma is early developmental sequestration of lymphatic vessels that fail to establish connection with normal draining vessels at about 14-20 weeks of intrauterine life. They become markedly dilated under the pressure effect of accumulating lymph. In addition, active growth of endothelium results in a slow dissection through normal tissue and pressure atrophy of adjacent structures.

It has been divided into three histological subtypes viz simple, cavernous and cystic types. Simple lymphangioma is an ill-defined mass of thin walled dilated lymphatics in a rich cellular connective tissue stroma. Cavernous type is spongy, compressible tumor of dilated lymphatics with fibrous adventitia. Cystic type is poorly circumscribed multilocular cystic mass containing clear, watery or chylous fluid.

Clinically cystic lymphangioma in retroperitoneum presents in infancy, fifty to sixty percent of which occur during first year of life and up to ninety percent in second year. They mostly present as palpable soft abdominal mass, which may appear suddenly and grow rapidly. Pressure on adjacent structures like bowel may cause obstruction. Preoperative radiological diagnosis is possible only in 25% cases [3]. Plain radiograph shows soft tissue mass displacing bowel loops. 10% may show thick rim calcification [2]. On IVU study, mass may displace urinary tract structures without any evidence of invasion. Sono shows cystic mass, (of which 61% are multiloculated with thick wall septa, while 39% are uniloculate). Septation, debris and fluid-fluid level may also be seen [2]. Progressive enlargement, multiplication, increase in echogenicity (due to hemorrhage) and thickening of septae are signs of complication and signifies immediate treatment [4]. On CT, attenuation values of loculi range in between the fat and fluid suggesting variable nature of cystic contents [2].

Management of choice is complete surgical enucleation. Latest development in management is 'Sclerotherapy' with doxycycline [5], alcoholic solution of zein [6] or with OK432[7], which is a lyophilized biological preparation containing the cells of group A streptococci. A. Differential diagnosis in Indian context should always include multiloculated ascitis due to high prevalence of tubercular abdomen, in addition to mesenteric cyst. Other bubbly lesions encountered in the abdomen include the enteric duplication cyst, the enteric cyst, the mesothelial cyst and the non pancreatic pseudocyst. Other cystic lesions that may be located in the mesentery or omentum include cystic mesothelioma, cystic spindle cell tumor and cystic teratoma [8].

 
   References Top

1.Sun CCJ, Tang CK, Hill JL, Mesenteric lymphangioma. Arch Pathol Lab Med 1980; 104: 316-8.  Back to cited text no. 1    
2.AJ Davidson and DS Hartman, Lymphangioma of the retroperitoneum: CT and sonographic characteristic, Radiology, 1990; 175(2): 507-510  Back to cited text no. 2    
3.Patel AP, Kothari JM, Shukla SN et al. Retroperitoneal lymphangioma. Ind J Pediatrics 1994; 61: 194-7.  Back to cited text no. 3    
4.Osnat Konen, Valeria Rathaus, Elena Dlugy et al 'Childhood abdominal cystic lymphangioma Ped Radiol 2002; 32: 88-94.  Back to cited text no. 4    
5.HI Molitch, EC Unger, CL Witte and E vanSonnenberg, Percutaneous sclerotherapy of lymphangiomas, Radiology, 1995; 194 (2): 343-347  Back to cited text no. 5    
6.J Dubois, L Garel, A Abela, L Laberge and S Yazbeck, Lymphangiomas in children: percutaneous sclerotherapy with an alcoholic solution of zein, Radiology, 1997; 204 (3), 651-654  Back to cited text no. 6    
7.Ogita S, Tsunoda A, Okabe I, et al: Phase II study on OK-43 therapy for lymphangioma in children. - The ChildrenOs Lymphangioma Study Grup - Therapeutic Research, 1994; 15: 3285-3297  Back to cited text no. 7    
8.C Stoupis, PR Ros, PL Abbitt, SS Burton and J Gauger, Bubbles in the belly: imaging of cystic mesenteric or omental masses, RadioGraphics, 1994; 14(4): 729-737  Back to cited text no. 8    

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Correspondence Address:
P Parakh
B-109, Sethi Colony, Jaipur 302004
India
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Source of Support: None, Conflict of Interest: None


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    Figures

[Figure - 1], [Figure - 2], [Figure - 3]

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