A large superior mesenteric artery aneurysm and ileal obstruction: a rare presentation of polyarteritis nodosa in an infant

Akihiko Shimizu; Kensuke Tanaka; Shinya Takazawa; Akira Nishi; Shinya Shimoyama; Tomio Kobayashi; Tomoyuki Imagawa; Junko Hirato; Yoshiyuki Yamada

doi:10.1093/omcr/omz092

A large superior mesenteric artery aneurysm and ileal obstruction: a rare presentation of polyarteritis nodosa in an infant

Oxf Med Case Reports. 2019 Sep 28;2019(9):omz092. doi: 10.1093/omcr/omz092. eCollection 2019 Sep.

Authors

Akihiko Shimizu¹, Kensuke Tanaka², Shinya Takazawa³, Akira Nishi³, Shinya Shimoyama², Tomio Kobayashi², Tomoyuki Imagawa⁴, Junko Hirato^{5

6}, Yoshiyuki Yamada¹

Affiliations

¹ Department of Allergy, Infectious Diseases and Immunology, Gunma Children's Medical Center, Shibukawa 377-8577, Japan.
² Department of Cardiology, Gunma Children's Medical Center, Shibukawa 377-8577, Japan.
³ Department of Surgery, Gunma Children's Medical Center, Shibukawa 377-8577, Japan.
⁴ Department of Infectious Diseases and Immunology, Kanagawa Children's Medical Center, Yokohama 232-8555, Japan.
⁵ Department of Laboratory, Gunma Children's Medical Center, Shibukawa 377-8577, Japan.
⁶ Department of Pathology, Public Tomioka General Hospital, Tomioka 370-2393, Japan.

Abstract

Polyarteritis nodosa (PAN) is a rare form of vasculitis that occurs in childhood and affects small- and medium-sized arteries. Large aneurysms due to PAN can induce fatal complications like rupturing or occlusion of the affected arteries. Here, we report a case of a 4-month-old girl with PAN complicated by a large superior mesenteric artery aneurysm and ileal obstruction. We controlled her blood pressure to prevent the artery from rupturing. A combination of prednisolone, intravenous cyclophosphamide, and plasma exchange reduced the inflammation. She developed mechanical ileus due to ileum stricture and underwent bowel resection. Histopathological examinations revealed reparative arteritis around the healed ulcer. Her postoperative course was uneventful without further dilatation of the aneurysm. This case highlights the importance of intensive immunosuppressive therapy and appropriate blood pressure control in pediatric patients with PAN complicated by large aneurysms. Mechanical ileus can develop and may require surgical management even after remission of vasculitis.