Novel operative technique of advancement urethral meatoplasty utilizing buccal mucosa for Vulvar Paget\u2019s disease with urethral invasion: two\xa0case reports

Masaki Nakamura,Taketo Kawai,Haruki Kume,Kazuto Hoshi,Motofumi Suzuki,Yasuhiko Igawa,Jun Omatsu,Yusuke Sato,Katsuhiro Makino,Masanobu Abe,Shohei Iwata,Yuji Hakozaki,Yuta Yamada,Daisuke Yamada

doi:10.1186/s13256-021-02729-1

Abstract

BackgroundVulvar Paget’s disease (VPD) is a rare malignant disorder originating in the external genitalia. It occasionally invades into urethral or vaginal mucosa of female, making surgical treatment more complicating. In case of urethral invasion of Paget’s cells, systematic mapping biopsy of urethral mucosa is the standard of care to determine the range of surgical resection. Resection of urethral mucosa and simple skin grafting often result in urethral stricture after surgery, which severely deteriorates patient’s quality of life.Case presentationWe applied a new technique of advancement urethral meatoplasty using buccal mucosa, in two Japanese cases of VPD with urethral invasion. After broad resection of vulvar skin together with the urethral mucosa, buccal mucosa was implanted between advanced urethral mucosa and skin graft. In both cases, we could prevent urethral stricture one year and two years after surgery, respectively.ConclusionThis technique prevented urethral stricture after surgery and could be a useful technique as part of urethroplasty for VPD.

Highlights

Vulvar Paget’s disease (VPD) is a rare malignant skin disease which originates in vulvar apocrine gland bearing cells
We present a novel operative technique of advancement urethral meatoplasty utilizing buccal mucosa for Vulvar Paget’s disease that prevented urethral stricture after surgery
Case 1 An 87-year old Japanese female presented with erythema and papules on her external genitalia, after diagnosis of VPD at another hospital

Summary

Introduction

Vulvar Paget’s disease (VPD) is a rare malignant skin disease which originates in vulvar apocrine gland bearing cells. Case 1 An 87-year old Japanese female presented with erythema and papules on her external genitalia, after diagnosis of VPD at another hospital. She underwent broad mapping biopsy at the Department of Dermatology and Paget’s cells were detected at vaginal opening and external urethral meatus (Fig. 2a). She was referred to us, and systematic mapping biopsy of the urethral mucosa was performed under anesthesia. Clinical examination revealed palm sized erythematous lesion with ulcer at vulva and bilateral inguinal lymphadenopathy (Fig. 3a) She was diagnosed with VPD by skin biopsy, and Paget’s cells were present at the external opening of urethra. Lower urinary tract function is completely preserved as before operation, and she is free from any signs of urethral stricture or incontinence

Discussion

Conclusion