脊髄損傷患者に合併した巨大副腎骨髄脂肪腫の1例

Abstract

38歳男。右側腹部痛を主訴とした。脊髄損傷による両下肢麻痺のため, 13歳時より車椅子を使用している。腹部CTにて17×16×12cmの巨大腫瘍を認め, 入院時検査, 副腎髄質シンチ各所見より右副腎脂肪腫を強く疑った。経腹的アプローチにて右副腎・腎合併切除を行った。摘出腫瘍は1320gでGerota筋膜内で右腎と接し, 一部に正常右側腎組織を認めた。病理組織学的所見より, 副腎骨髄脂肪腫と診断した。なお, 脊髄損傷患者であるため, 術前よりの呼吸リハビリテーションによる肺合併症予防, 手術時の除圧マット使用による褥瘡予防, 弾性ストッキング着用, 間歇的空気圧迫装置の装着, フラグミン投与による血栓予防などの厳重な周術期管理を行った。術後経過は良好で, 術後10日に退院した。

A 38-year-old man in a wheelchair for spinal cord injury from a traffic accident was referred to our hospital with complaint of dull right flank pain. The patient had a history of skin ulcer formation on both toes due to poor circulation. Abdominal ultrasonography and computed tomographic scan revealed a huge retroperitoneal tumor compressing the right kidney. Serum levels of cortisol, adrenocorticotropic hormone, aldosterone, adrenalin, noradrenalin, urinary levels of vanillylmandelic acid, homovanillic acid, 17-ketosteroid and 17-hydroxycorticosteroid were all within normal limits. Combining all preoperative diagnostic information, the possibility of retroperitoneal liposarcoma could not be thoroughly ruled out. The patient consented to undergo resection of the tumor with the right kidney. Prior to the scheduled operation simulation of the positioning on the operation table was done to evaluate the compressing pressure. During operation decompressing pads were used to minimize the compressing pressure and intermittent pneumatic compression was also used to prevent pressure ulcer and facilitate circulation to the lower extremities. Five thousands units of heparin (FragminR, Pfizer, USA) was used daily to prevent deep vein thrombosis until the patient could use his wheelchair. Perioperative rehabilitation was conducted by a team of physical therapists. The excised tumor weighed 1, 320 g. Pathologic diagnosis was an adrenal myelolipoma. Convalescence was uneventful and the patient was discharged on the 10th post operative day. Between 1992 and 2006, 80 cases of adrenal myelolipoma were reported in Japan. Our case was found to be the second largest one. We reviewed these 80 cases and discussed the diagnosis and treatment strategy of the adrenal myelolipoma. We also discussed the perioperative management for patients with spinal cord injury.