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 (). 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.
CT demonstrating simple fluid collection in right iliac fossa.
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 (). She had an uncomplicated post operative recovery and was discharged seven days post operatively.
Gross histology image of omentum with gelatinous cyst.
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 ().
Histology image showing high power with dilated vascular channels field with lymphocytes.
D2-40 stains highlight the lining for lymphatic channels.