Intestinal lymphangiectasia: a rare cause of intussusception in an adolescent

Abstract

Intussusception in adolescents is usually idiopathic in nature. A 17-year-old woman with diffuse large B cell lymphoma presented with signs of intestinal obstruction after initiation of induction chemotherapy. On evaluation, the patient was diagnosed to have ileoileal intussusception with intestinal lymphangiectasia as the lead point. Intestinal lymphangiectasia as a rare cause for intussusception and its relationship with lymphoma is discussed in this case report.

Introduction

Intestinal lymphangiectasia is a rare malformation characterised by abnormal dilatation and proliferation of lymphatic duct.^1,2 These abnormal lymphatics may be located in the mucosa, submucosa or subserosa of the gastrointestinal tract.² Intestinal lymphangiectasias are classified into primary and secondary types based on their aetiology.¹ Secondary intestinal lymphangiectasia due to lymphatic obstruction has been associated with malignant conditions such as lymphoma.^3–5 As intestinal lymphangiectasia is a rare entity whether its association with the malignant condition is causative or an incidental finding is difficult to interpret. Intussusception in adolescents is usually idiopathic and lead points, if there are any, are usually benign in nature.⁶ Secondary intestinal lymphangiectasia, a rare cause of intussusception in an adolescent with lymphoma, is described in this report.

Case history

A 17-year-old woman presented as easily fatigued with a low-grade fever of one month’s duration. She was diagnosed as having a large diffuse B cell lymphoma and was started on induction therapy with L-asparaginase, vincristine and daunorubicin, together with steroids. After initiation of induction chemotherapy, the patient developed vomiting and colicky abdominal pain for two weeks. She had no melaena, haematochezia or abdominal distension. Abdominal examination revealed an ill-defined non-tender lump in the right iliac fossa. Contrast enhanced computed tomography was suggestive of ileoileal intussusception (Fig 1). She underwent emergency laparotomy. Intraoperatively, the intussusception was found to have spontaneously reduced. A soft eccentric intraluminal lesion measuring approximately 2 × 2 cm was palpated in the proximal ileum 110 cm from the ileocecal junction. No other lesions were identified. The patient underwent segmental ileal resection and anastomosis. The gross examination of the specimen revealed a bulge with loss of mucosal folds measuring approximately 2.5 × 2.5 × 0.5 cm (Fig 2). Microscopic examination revealed ectatic lymphatic channels with the widening of the submucosal region with unremarkable adjacent mucosa and muscularis propria. The patient had an uneventful postoperative course and discharged on the fifth postoperative day.

Figure 1.

Contrast enhanced computed tomography shows ileoileal intussusception (arrow)

Figure 2.

Cut section shows intraluminal polypoidal mass

Discussion

Intestinal lymphangiectasia was first described by Waldmann et al in 1961.^2,7 The primary condition is a congenital abnormality is predominantly diagnosed in children and the secondary condition is increasingly recognised in adults. The most common sites of occurrence of lymphangiectasia are head, neck and axilla. Only 1.6% of the reported patients with lymphangiectasia had involvement of the intra-abdominal organs. The predominant intra-abdominal location is the mesentery followed by the omentum, mesocolon and retroperitoneum. The small intestine is a rare site for intra-abdominal lymphangiectasia.¹⁰ While some suggest that intestinal lymphangiectasia is an incidental finding in patients with lymphoma others propose an etiological relationship.⁹ One of the proposed hypotheses is that lymphoma can cause secondary intestinal lymphangiectasia by means of lymphatic obstruction. Lymphoma can also occur in intestinal lymphangiectasia secondary to immune deficiency which occurs because of the loss of T cell and immunoglobulins in the gut.^3,9 The review of the literature revealed that intestinal lymphangiectasia can cause small intestinal obstruction either by mechanical obstruction, secondary haemorrhage or volvulus.^5,8,10 To date, only one case of small bowel intussusception in a neonate secondary to lymphangiectasia has been reported in the literature.⁷ Although intestinal lymphangiectasia can resolve with chemotherapy given for lymphoma, resection should be the first line of treatment when they cause mechanical small bowel obstruction.^3,9,10

The clinical importance of this case report is that when a patient with lymphoma presents with abdominal pain, vomiting and radiological imaging shows features of intestinal obstruction secondary to intussusception, lymphangiectasia should be considered in the differential diagnosis of the organic lesions that can cause intussusception. When the clinical presentation is with intestinal obstruction, surgical approach with resection of the involved segment is the preferred treatment as it treats the obstruction in addition to facilitating histopathological diagnosis of intestinal lymphangiectasia.