Abstract

Abstract Background Kikuchi disease is a rare, self-limiting condition that is characterized by histiocytic necrotizing lymphadenitis with unknown pathogenesis. Common presentation is cervical lymphadenopathy and fever in a previously well young female. Mediastinal lymph node involvement is uncommon. Methods We report a case of spontaneous esophagocutaneous fistula secondary to necrotizing mediastinal lymphadenopathy in a patient with Kikuchi disease. Results A 38-year-old lady presented with fever and right back pain of two weeks duration. She had background history of Kikuchi disease which was diagnosed 1 year ago. Examination revealed multiple enlarged left cervical lymph nodes and right back tenderness. CT scan showed an esophagocutaneous fistula with right paraspinal abscess and multiple enlarged cervical and mediastinal lymph nodes. OGDS revealed a 5mm fistula opening at right posterior wall of distal esophageal, 3cm proximal to esophagogastric junction. Rest of esophagus and stomach were normal. Tuberculosis and SLE workup were negative. Cervical lymph node biopsy was consistent with Kikuchi disease. She was treated as spontaneous esophagocutaneous fistula secondary to necrotizing mediastinal lymph node with underlying Kikuchi disease. Percutaneous drainage of paraspinal abscess was done and antibiotics were commenced. Laparoscopic feeding jejunostomy was done for feeding. Esophageal stenting and endoscopic clipping of fistula were attempted but the fistula tract persisted. She then underwent laparoscopic excision of esophageal fistula tract (LEEFT). Intraoperative findings were dense inflamed paraesophageal tissue with thickened fistula tract at right posterolateral wall of distal esophagus with multiple enlarged mediastinal lymph nodes. Fistula tract and the lymph nodes were excised via laparoscopic transhiatal approach. She had an uneventful recovery and was discharged on post-operative day 7. Conclusion Spontaneous esophageal fistula is rare and necrotizing mediastinal lymphadenopathy needs to be considered. Laparoscopic excision of esophageal fistula tract (LEEFT) is a novel transhiatal approach and it is feasible in distally located fistula. Disclosure All authors have declared no conflicts of interest.

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