Loeys-Dietz症候群に合併した臍ヘルニアに対しenhanced-view totally extraperitoneal法にて修復した1例

Enhanced-view totally extraperitoneal repair of umbilical hernia in Loeys-Dietz syndrome : A case report

◆要旨：症例は62歳，男性．背景に常染色体顕性（優性）遺伝の全身結合組織疾患であるLoeys-Dietz症候群があり，臍ヘルニアの診断で当科に紹介となった．脆弱な結合組織を有することから，メッシュを使用した修復が好ましいと考えられた．しかし腹腔内メッシュ留置による消化管との癒着は，大動脈解離・瘤破裂などの緊急手術時に消化管損傷のリスクになると考え，enhanced-view totally extraperitoneal（eTEP）法による修復方法を選択した．術後9か月，再発なく経過している．全身結合組織疾患に合併した臍ヘルニアに対し，メッシュを用いた内視鏡下修復術は有用と考えられ，文献的考察を含め報告する．

［Background］Umbilical hernia in an adult is a relatively well-known disease. However, what type of repair surgeries are appropriate is still poorly understood for the systemic connective tissue disease patient. ［Case presentation］A 62-year-old Japanese male presented with umbilical swelling. His past medical history was significant for replacement of ascending aorta, aortic arch, descending aorta and aortic valve due to Loeys-Dietz syndrome previously. An umbilical hernia was suspected, and CT scan showed a defect of the linea alba at the umbilicus. Umbilical swelling gradually increased and caused discomfort. Enhanced-view totally extraperitoneal repair with polyester mesh was performed successfully, following hernia orifice closure using non-absorbable sutures. The patient has no evidence of recurrence at 9-months follow up. ［Conclusion］An umbilical hernia patient with a Loeys-Dietz syndrome was repaired endoscopically. This method can reduce the risk of adhesion between mesh and intra-abdominal organs. Endoscopic repair is considered an excellent technique with a low recurrence rate.