神経線維腫症1型（レックリングハウゼン病）患者の皮膚伸展性を利用した広背筋皮弁による乳房再建の1例

A Case of Breast Reconstruction Using a Latissimus Dorsi Myocutaneous Flap in a Patient with Neurofibromatosis Type 1 and Hyperextensible Skin

はじめに

　神経線維腫症1型（neurofibromatosis type 1：以下，NF1）は，カフェオレ斑や神経線維腫をはじめ，各種臓器に多彩な病変を生じる遺伝性疾患である 1）。報告は少ないが，以前よりNF1の患者の皮膚では伸展性が高く，瘢痕が目立ちにくいことが知られている 2）3）。今回NF1で乳癌を発症した患者に対して，皮膚の伸展性を利用したデザインによる広背筋皮弁を用いて乳房再建を行い，ほぼ満足する結果を得られたので報告する。

　It is known from clinical experience that scars heal unobtrusively in patients with neurofibromatosis type 1 (von Recklinghausenʼs disease) because of their hyperextensible skin. We report the case of a patient with neurofibromatosis type 1 in whom breast reconstruction was performed using a latissimus dorsi myocutaneous flap that was designed to take advantage of the hyperextensible skin in such patients. The patient was a 46-year-old woman with neurofibromatosis type 1 who underwent a nipple-sparing mastectomy for breast cancer and breast reconstruction with a latissimus dorsi myocutaneous flap. Anticipating skin extensibility, a bi-lobed flap was used. Although the skin defect was large and would not normally have been amenable to sutures, we were able to suture the skin defect in this patientʼs case. We were also able to reproduce a breast mount similar to that on the patientʼs unaffected side by folding the bi-lobed flap. The patientʼs hyperextensible skin made it possible to reproduce ptosis of the breast. The wounds healed unobtrusively without hypertrophic scars or keloids despite the high tension on the wound edges. This case demonstrates that the outcome of breast reconstruction may be satisfactory in patients with neurofibromatosis type 1 because of their hyperextensible skin.