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Lymphangioma is an uncommon malformation of lymphatic system. Multiple colonic lymphangioma named as lymphangiomatosis is considered an extremely rare disease.
We report a case of colonic lymphangiomatosis presenting as recurrent right iliac fossa pain in a young female treated surgically.
Lymphangiomatosis is a rare but benign disease with a variety of presentations. There are few reported cases in the literature at present and no guidelines regarding the diagnosis or treatment of the same.
Our case demonstrates an unusual presentation of lymphangiomatosis treated surgically. Debate will remain on this rare condition.
Lymphangioma is a lymphatic malformation which shows benign proliferation of lymph vessels, with the characteristics of submucosal tumors covered with normal mucosa.1 Multiple colonic lymphangiomas are called ‘colonic lymphagiomatosis’, it is a rare condition with paucity of data in the literature. Although lymphangioma is a benign tumor and most colonic lymphangiomas do not cause symptoms and do not require treatment, resection of lymphangioma is necessary in the presence of symptoms such as abdominal pain, bleeding, intussusceptions.2
We report a patient with lymphangiomatosis of the colon diagnosed at laparoscopy. A.K. is a 25 year old female, who presented to the Emergency Department with a four day history of lower abdominal pain, localized to the right iliac fossa (RIF) associated with a low grade pyrexia and raised inflammatory markers (WCC 18.3, CRP 8.2). A diagnostic laparoscopy revealed an appendiceal mass, a mesenteric cyst and some free fluid in the pelvis. An appendicectomy was not performed. Subsequent CT scan revealed a fluid collection in the RIF measuring 4 cm × 3 cm. She was treated conservatively with intravenous co-amoxiclav and metronidazole and was discharged once clinically well. A decision was made for an elective appendicectomy.
She represented to the Emergency Department seven weeks later with ongoing lower abdominal pain with raised inflammatory markers (WCC 14.6). CT scan revealed multiple fluid collections, 4.5 cm × 2.6 cm adjacent to the cecum, 1.9 cm × 1.6 cm in the LIF and a right adnexal cyst (Fig. 1). MRI pelvis confirmed these collections. A diagnostic laparoscopy revealed multiple gelatinous cysts on the omentum and in the RIF. She was reviewed by the gynecology team and trans-vaginal ultra sound was unremarkable. Tumor markers (CEA, CA 125, CA 19-9, B-HCG, A-FP) were all within normal limits.
The case was discussed at the institutional multi disciplinary conference and decision was made for surgical management. An exploratory laparotomy revealed large gelatinous cysts adherent to the cecum, omentum and a small cyst at the sigmoid colon. No other pathology was seen. An open right hemi-colectomy, omentectomy and wedge excision of the sigmoid colon was performed (Fig. 2). She had an uncomplicated post operative recovery and was discharged seven days post operatively.
The case was subsequently discussed at the MDT conference and histology confirmed similar lesions in all three samples consistent with lymphangioma/lymphangiomatosis. No evidence of atypia or malignant cells (Figs. 3 and 4).
In most reported cases of colonic lymphangioma, the condition is solitary. Only a few cases of multiple lymphangiomas of the colon, the so-called “colonic lymphangiomatosis”, have been reported so far.1,3 Many lymphangiomas are asymptomatic and require no treatment, because the condition is considered absolutely benign. No cases, in which lymphangioma underwent malignant transformation, have been reported. However when they present with bleeding, pain or intussusceptions, resection may be necessary.4,5
There is ongoing discrepancy in the limited available literature, ranging from spontaneous resolution and surveillance6 to complete surgical resection due to potential to grow, invade surrounding structures and developing complications. Losanoff and Kjossev reported a young man presenting with an acute abdomen who had an incidental finding of pedicled cystic mass of the mid-ileal mesentery, causing a volvulus.7
Our case demonstrates an unusual presentation of colonic lymphangiomatosis. The rarity of the condition makes diagnosis difficult. In this case, our initial diagnosis from detailed history, clinical examination and biochemical markers was a classic case of appendicitis. The second presentation and subsequent investigations proved otherwise. A decision to proceed to definitive surgery was made at the MDT conference without tissue diagnosis. A biopsy of the gelatinous cystic material was not offered due to the risk of spread of potential malignant cells. The diagnosis was clarified from the specimen of the right hemicolectomy. There is one further similar case reported in the literature of lymphangiomatosis mimicking appendicitis.8
Developing trends in the diagnosis of lymphangiomatosis of colon have been changing since the development of EUS, and a recently Cha et al., reported the first case that lymphangiomatosis of the colon was diagnosed without invasive procedures using EUS. The characteristic EUS features of the lymphangioma are localization to the submucosal layer, a homogenous echo pattern and multilocular cystic features that are echo-free or contain low-echo septal structures. They elected not to treat the asymptomatic patient as a result.9
Lymphangioma and lymphangiomatosis is a rare condition with varied presentations. Historically surgical resection was often offered due to the unclear nature of the lesion, as was the case in our experience. Debate will remain on this rare yet benign condition and the clinical picture of an incidental finding or an acute presentation will dictate treatment options.
There is no conflict of interest.
No source of funding.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.
GJ Nason, author involved in data collection; BD Barry involved in data collection and editing; R Shatwan involved in histology analysis and preparing report; and P Neary who is the cheif editor of the manuscript.