尿道上列合併陰莖彎曲之手術案例分享及文獻探討

黃維倫 陳冠廷 莊德彥 董聖雍 曾啟新 張奕凱 黃國皓 張宏江

國立台灣大學附設醫院泌尿部

Epispadias with Chordee: A single surgical experience

Wei-Lun Huang, Guan-Ting Chen, Te-yen Chuang, Sheng-Yung Tung, Chi-Shin Tseng, Yi-Kai Chang, Kuo-How Huang, Hong-Chiang Chang

Department of Urology, National Taiwan University Hospital

A 23-year-old man has a medical history of epispadias. He has received surgical reconstruction for epispadias at 2-3 years old, but the epispadias persisted after the operation. Chordee during erection has been noted since adolescenthood. He denied voiding difficulty or history of urinary tract infection. He came to our OPD for help. PE showed epispadias and adhesion of the prepuce that could not be fully withdrawn to expose the entire glans. The fissure of the epispadias could be partially exposed also (Fig. 1 to Fig. 3). MRI on 2019/01/21 found epispadias with torturous course of penis. Sexual hormones (E2, LH, FSH, Testosterone) were within normal range. The operation was performed in Feb. 2019, which was composed of three parts, the repair of epispadias, the urethroplasty, and the correction of chordee. The detail of the surgery was described below. After the operation, we placed Foley catheter for 2 weeks. The epispadias has been completely reconstructed. The chordee was partially corrected with mild deviation to the left side and rotation. Phimosis with a scar ring, meatus stricture, and deviation of urine stream to the right side were also noted (Fig. 9). He received circumcision and meatus dilation with Sounding to Fr.26 one month after the first reconstruction (Fig. 10).

Surgical reconstruction

Degloving, de-adhesion, and relief of the Chordee

We inserted a 16Fr. Foley catheter into the urethra for identification and guiding. Degloving of the prepuce followed by circumcision was performed, which revealed severe adhesion and fibrosis of the subcutaneous tissue. The adhesion and fibrotic tissue was dissected. The dartos fascia was opened and the Bucks fascia was exposed. The epispadias fissure, which extended to the bas  e of the glans was completely uncovered after de-adhesion and degloving (Fig. 4a and 4b). We performed artificial erection with normal saline instillation to check the orientation of the penis. However, there was no communication between two cavernosa, so the artificial erection should be obtained by separately instillation of normal saline into two cavernosa. Resolution of chordee was found after de-adhesion and artificial erection (Fig. 5).

Repair of the epispadias, glandular reconstruction, and the urethroplasty

We incised the edge of urethral plate, from 0.5cm proximal to the tip of the glans to the original meatus, for tubularization (Fig. 6). After dissected the connective tissue below bilateral parts of the urethral plate. The plate was tubularized and approximated with 5-0 Mexon (Fig. 7). The dartos fascia and subcutaneous tissue was closed with 5-0 Mexon and 4-0 Vicryl. The prepuce was sutured with 3-0 Chromic (Fig. 8).

Discussion

Epispadias with concurrent bladder exstrophy which is called Exstrophy-Epispadias Complex is a rare anomaly, with incidence of 2.2 in 100,000 live births. Isolated epispadias is rarely with a reported incidence of 1 in 117, 000 males. It could be classified into penopubic type, penile type, and glandular type, according to the extension of defect. All types of epispadias are associated with varying degrees of dorsal chordee. Furthermore, complete epispadias is also associated with deformities of the external genitalia, diastasis of the pubic symphysis, deficiency of the urinary continence mechanism, and UVJ deficiency. Surgeons should take bladder storage function, anti-reflux mechanism, urinary continence, and genital reconstruction in consideration for managements. Fortunately, our case had only genital defect, which was relatively uncomplicated. For the genital reconstruction, four issues should be addressed, including correction of dorsal chordee, urethral reconstruction for micturition and semen transport, glandular reconstruction, and penile skin closure. The chordee correction of our patient was partial, which might be due to uncommunicated cavernosa that made the artificial erection during the operation unequally. The two cavernosa might be also different in size naturally. Further correction of the chordee is needed. The patient suffered from deviated voiding stream, so urethroscopy is necessary to evaluated the urethral reconstruction. Ejaculation evaluation and semen analysis are also needed for the purpose of fertility. The glandular reconstruction is successful, but phimosis due to scaring of the foreskin and meatus stricture were found. Circumcision and meatus dilation have been complete. The following course and clinical condition will be reported in 2019 TUA annual meeting.