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Abstract:
Lymphangiomas are benign tumors that can involve the gastrointestinal tract and mesentery with a potential to grow to considerable size. They usually present in childhood and have characteristic gross, histological, and radiographic features. Here we report a case of a pediatric patient with a gastrointestinal lymphangioma.
History:
A 2-year-old African American male presented with a history of chronic abdominal distension and new onset abdominal pain (several days duration). He also had a history of asthma and recurrent otitis media.
Physical Examination:
On physical examination, the abdomen was noted to distended and protuberant. The abdomen was non- tender and non-tympanic. A discrete mass was not palpable.
Radiographic Studies:
A contrast enhanced CT scan of the abdomen and pelvis showed a multiloculated cystic mass displacing abdominal and pelvic organs. It was considered radiographically to be consistent with lymphangioma, most likely associated with sigmoid colon and mesocolon.
Operative Findings:
Upon laparotomy, a large, fluid filled, intraperitoneal mass with multiple thin septations causing a significant mass effect on the abdominal organs was noted. The bulk of bowel was displaced superiorly and centrally. The mass was arising in the omentum and the vessels were tethered adjacent to the greater curvature of the stomach, colon, and spleen. Therefore, the mass was resected with omentectomy.
Pathology:
The gross specimen was a 2165gm, 32 x 10 x 5cm red-tan multicystic and encapsulated mass. The outer surface was smooth and glistening. Upon opening, the mass revealed 1600 cc of a red-brown cloudy fluid. Most of the interior lining was red but at several foci, the cysts were white. Microscopically, multiple vascular spaces of varying sizes were noted. These were lined by a single layer of flat to cuboidal cells resting on fibromuscular stroma. Several lymphoid nodules were present. Clear, pink staining lymph was in some of the spaces.
Discussion:
Lymphangiomas are lymphatic hamartomas that usually present in childhood and are more commonly seen in boys. They were first described by Wernher in 1843. According to the classification of Watson and McCarthy three different types of lymphangiomas may be found in the gastrointestinal tract: simple, cavernous, and cellular. These lesions have the potential to grow to considerable size. Clinical presentation is variable and may be misleading. They present as either an acute abdomen or, more commonly, a silent abdominal mass. Chronic non-specific abdominal signs include progressive abdominal distension and chronic abdominal pain, while the most frequent acute signs are acute abdominal pain and distension, vomiting, fever, and peritonitis. When they are omental cysts they usually occur singly and are easily resected, but multiple cysts predominate in the mesentery and retroperitoneum. Extra-abdominal lesions, mainly cutaneous lymphangiomas, may develop in some patients. A total surgical excision, if feasible without sacrifice of adjacent organs, seems to be the best therapeutic option to minimize the risk of recurrence. Prognosis is good after surgical excision, however long-term follow-up is advisable because of the possibility of recurrence, even during adulthood. In a few cases the malformation is diffuse, and extensive bowel resection is necessary. Because it has been suggested that these entities are lymphatic malformations, a more specific term like cystic lymphatic malformations has been suggested by some authors to describe these lesions.
Radiographic Discussion:
Lymphangiomas appear as multilocular cystic lesions with thin walls and septations on imaging studies. On ultrasound, the cyst contents are anechoic, but may contain echogenic debris as a result of infection or hemorrhage. Intracyst hemorrhage is common and may produce a characteristic fluid-debris level. Lymphangiomas are of fluid attenuation on CT. A few small calcifications may be seen in the walls or septations. Occasionally, the attenuation of the cyst contents is less than that of fluid (zero Hounsfield units) due to the presence of chyle. On MR imaging, the cyst contents usually demonstrate signal characteristics of fluid; that is, they are low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. The presence of infection or hemorrhage may alter the signal characteristics. Fluid-debris levels may be appreciated on axial and sagittal images of the abdomen. With administration of intravenous contrast material, the cyst contents do not enhance. Only the walls and septa enhance. Imaging differential diagnosis includes other fluid-containing lesions such as cysts, abscesses, and loculated ascites.
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The opinions and assertions contained herein are the private views of the authors, and are not to be construed as official, or as reflecting the views of the Departments of the Army and Defense.
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