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Urethroperineal Fistula

A urethroperineal fistula (UPF) is an epithelialized communication between the urethra and the perineal skin. It exists in two operationally distinct forms: a rare congenital posterior urethroperineal fistula (CUPF) — a urothelium-lined tract from the posterior urethra to the perineum, with ~30 reported cases — and a far more common acquired UPF that follows Fournier's gangrene, pelvic-fracture urethral injury, periurethral abscess, urethral stricture surgery, lichen sclerosus, or chronic catheterization.[1][2][3][4][5] CUPF is curative with simple ventral-tract excision; acquired UPF requires multilayer urethral closure with vascularized tissue interposition (gracilis is the workhorse), and definitive perineal urethrostomy is increasingly recognized as a legitimate primary option in the most complex cases rather than a salvage.

For the male-shaft equivalent and the broader interposition-flap framework, see Urethrocutaneous Fistula. For the operative principles of perineal-tissue reconstruction, see The Perineum and Fournier's Gangrene.

Congenital Posterior Urethroperineal Fistula (CUPF)​

Definition​

A urothelium-lined tract between the posterior urethra and the perineum in a male — one of the rarest urogenital anomalies, with ~30 reported cases as of 2021.[1][3]

Embryology​

Two competing models:[2][3][7]

  • Variant of Effmann Type IIA2 Y-duplication — but with a critical inversion: the dorsal (orthotopic) urethra is the functional channel and the ventral perineal tract is hypoplastic. Bello proposed designating CUPF as "Type IIA2, Y-hypoplastic ventral urethra."[3]
  • A distinct entity from urethral duplication, since the dorsal urethra is anatomically and functionally normal and the ventral channel is non-functional — meaning excision of the ventral channel is curative, in contrast to hypospadiac urethral duplication where ventral excision can be catastrophic.[2]

Clinical presentation​

  • Perineal urinary leakage during or after voiding
  • Normal voiding through the penile meatus — the dorsal urethra is functionally intact[2]
  • Visible perineal opening between the scrotum and anus
  • Recurrent UTI[6]
  • No anorectal malformation — distinguishing CUPF from H-type rectourethral fistula[1]

Diagnostic discriminators — CUPF vs urethral duplication vs H-type RUF​

FeatureCUPFY-type urethral duplicationH-type rectourethral fistula
Dorsal urethraNormal, functionalHypoplastic / absentNormal
Ventral channelHypoplastic, non-functionalFunctional (main channel)Connects to rectum
Perineal openingSkinSkinRectal mucosa
VoidingNormal per meatusAbnormal (perineal)Normal per meatus
Rectal involvementNoneNoneYes
TreatmentSimple excision / fulgurationComplex reconstructionFistula excision + interposition

Source: synthesized from Cheng et al.[1]

Workup​

  • VCUG — opacifies both the normal dorsal urethra and the ventral perineal tract[2][6]
  • MRI pelvis — confirms the fluid-filled tract, increases in size during micturition; useful for surgical planning[6]
  • Cystourethroscopy — visualizes the internal opening of the fistula and confirms a normal dorsal urethra[2]
  • Fistulography — through the perineal opening[8]

Treatment and outcomes​

  • Excision of the ventral (accessory) channel through a perineal incision — the standard approach[2][3][8]
  • Endoscopic fulguration of the tract is an alternative[3]
  • Cure rate ≈ 100% in reported cases[2][3]
  • The single most important point: misdiagnosis as urethral duplication or rectourethral fistula leads to inappropriate and potentially catastrophic surgery — recognize CUPF before operating[1]

Acquired Urethroperineal Fistula​

The far more common form — and the operatively challenging one.

Etiology​

SettingNotes
Fournier's gangreneNecrotizing fasciitis with urethral / periurethral debridement; suprapubic cystostomy in the acute phase; reconstruction delayed until wound is clean and granulating[9][10][11][21][22]
Pelvic fracture urethral injury (PFUI)Fistula forms when urethral continuity is not restored; often clusters with concurrent urethrorectal fistula[12][20][24][28][29]
Periurethral abscessStricture- or instrumentation-related abscess eroding through urethra and perineal skin
Urethral stricture / post-urethroplastyUCF / UPF in 3–5% of urethroplasties; higher (21–23% revision) in staged procedures and in failed-hypospadias-related strictures[17][18][19]
Lichen sclerosus (BXO)Progressive panurethral stricture with tissue destruction; can present as a perineal/scrotal mass with a draining fistula; biopsy required to rule out SCC[13][14][15][25]
Urethral calculiChronic stone impaction eroding to the perineum; usually with stricture or long-term catheterization[4]
Neurogenic bladderDecubitus ulcers (33%), wound infection (24%), condom-catheter complications (19%), traumatic catheterization (19%) — 81% ultimately need permanent diversion[16]
Penetrating traumaGSW, stab, straddle injury directly disrupting urethra and perineum

Pathophysiology​

A common final pathway: urethral wall compromise (ischemia, necrosis, infection, inflammation, trauma) → loss of supporting spongiosal and fascial layers → cutaneous communication → epithelialization of the tract preventing spontaneous closure.

  • In Fournier's gangrene, debridement of necrotic tissue creates large defects that often expose or sacrifice urethra; suprapubic cystostomy is the acute urinary diversion of choice when debridement involves the urethra or periurethral tissues[9][10][21]
  • In PFUI, the membranous urethra is distracted from the bulbar urethra by pelvic ring disruption; if continuity is not re-established, urinary extravasation finds its way to a perineal wound or the surgical incision[24][29]
  • In lichen sclerosus, progressive inflammatory destruction produces dense fibrosis, panurethral stricture, and eventual fistulization through the perineum[13][14][15]

Clinical presentation​

  • Urinary leakage from a perineal opening
  • Perineal wetness, skin maceration, recurrent local infection
  • Obstructive voiding symptoms if a concurrent stricture is present (almost universal in acquired UPF)
  • Recurrent UTI
  • A perineal mass or induration in lichen sclerosus

Workup​

  1. Physical exam — opening(s), skin quality, scarring, available tissue for reconstruction
  2. RUG and VCUG — fistula and concurrent stricture; both must be defined before any operative plan[2][6]
  3. Cystourethroscopy — internal opening, urethral mucosa, distal obstruction
  4. MRI pelvis — best soft-tissue characterization of complex tracts; particularly helpful in LS and post-radiation[6]
  5. Fistulography — through the perineal opening
  6. Biopsy — mandatory in LS-associated UPF to rule out squamous cell carcinoma[13][15]

Management​

General principles (any acquired UPF)​

  1. Wait ≥ 3–6 months from the inciting event or last surgery for tissue maturation
  2. Exclude and correct distal obstruction before or at the time of fistula repair — meatal stenosis or stricture is the dominant driver of recurrence
  3. Excise the fistula tract completely
  4. Watertight, tension-free urethral closure
  5. Vascularized tissue interposition between urethral and skin suture lines
  6. Urinary diversion (suprapubic tube) during healing
  7. The first repair offers the best chance of success — consider referral to a high-volume reconstructive center[26]

Conservative management​

Catheter drainage (urethral or suprapubic) closes a small minority of small, early UPFs in non-infected, non-irradiated tissue; spontaneous closure occurs in up to ~15% of urogenital fistulas with prolonged drainage alone but is rare in chronic, epithelialized, or stricture-associated UPF.[26]

Etiology-specific management​

A. Post-Fournier's-gangrene UPF​

The most challenging soft-tissue scenario.[9][10][11][21][22]

  • Acute phase: aggressive debridement, broad-spectrum antibiotics, suprapubic cystostomy for urinary diversion when urethra / periurethral tissue is involved, negative-pressure wound therapy
  • Reconstructive phase (delayed):
    • Split-thickness skin grafts for wound coverage[22]
    • Gracilis or VRAM flap for complex defects requiring bulk — 100% success in one series at mean 6.3 yr[27]
    • Perineal urethrostomy when urethral tissue loss is extensive — often the most practical definitive option[21]
    • Permanent urinary diversion (suprapubic tube, ileal conduit) in the most severe cases

B. PFUI-associated UPF (often clustered with urethrorectal fistula)​

  • Transperineal anastomotic urethroplasty with tissue interposition is the standard approach[20][24][28]
  • When PFUI is associated with concurrent urethrorectal fistula, transperineal urethroplasty + gracilis interposition achieves 91% success (100% primary, 70% redo)[20]
  • Posterior urethroplasty for PFUI alone: 84% retreatment-free survival at 10 years[29]
  • Length-gaining maneuvers: bulbar mobilization, corporal separation, inferior pubectomy, urethral rerouting[24][29]
  • Functional sequelae: erectile dysfunction in 75–98% (mostly trauma-driven, not the operation); de novo urinary incontinence ~6.6%[12][29]

C. Post-urethroplasty / stricture-associated UPF​

  • Small fistulas — multilayer closure with dartos or tunica vaginalis flap interposition[5]
  • Complex with concurrent stricture — staged urethroplasty with buccal mucosa graft (BMG); the fistula is addressed as part of the urethral reconstruction[18][19]
  • Recurrent or refractory — perineal urethrostomy (see below) is endorsed by the AUA Urethral Stricture Disease Guideline as a long-term option for high-risk reconstruction patients[23]

D. Lichen-sclerosus-associated UPF​

  • Genital skin must not be used for reconstruction — it remains susceptible to LS recurrence[13][15]
  • Buccal mucosa is the graft of choice[13][14]
  • Staged urethroplasty — first stage perineal urethrostomy with BMG inlay; second stage tubularization[14]
  • Definitive perineal urethrostomy is a reasonable durable option — 72% success at mean 56 months in one multicenter LS series, with many patients preferring this simpler endpoint[13][14]
  • Biopsy and long-term surveillance mandatory[13][15]

E. UPF in neurogenic bladder​

  • Repair durability is poor — 81% ultimately require permanent urinary diversion (suprapubic tube, ileal conduit, or perineal urethrostomy). Counsel for diversion early.[16]

Tissue interposition options​

FlapSourceBest fitNotes
Gracilis muscle flapMedial thighComplex, irradiated, Fournier's defectsReliable medial-circumflex pedicle; long reach; workhorse[20][27]
VRAM flapRectus abdominisLarge pelvic / perineal defectsExcellent bulk and vascularity[27]
Dartos pedicled flapPerineal subcutaneous tissueModerate-complexity fistulasLocal, technically simple[30]
Bulbospongiosus muscle flapPerineal bulbospongiosusPFUI-associated, urethrorectal septumLocal, anatomically natural[28]
Rectus fascia graftLower abdominal wallRecurrent UPF (e.g., post-metoidioplasty)Autologous; separates suture lines[32]
Perivesical fat flapBladder domeWhen omentum unavailableNovel alternative[31]

Perineal urethrostomy as definitive management​

For complex, recurrent, or refractory acquired UPF — particularly with panurethral stricture, lichen sclerosus, failed hypospadias repair, or neurogenic bladder — permanent perineal urethrostomy is increasingly used as a primary definitive option rather than a salvage:[23][33][34][35][36][37]

  • AUA Urethral Stricture Disease Guideline endorses perineal urethrostomy as a long-term option for high-risk patients[23]
  • Retreatment-free survival 84% at median 55-month follow-up; patient satisfaction high (median 21/24)[34]
  • Comparable success to complex urethroplasty for long strictures (RR 0.93, 95% CI 0.84–1.03 in meta-analysis)[35]
  • Use has risen from 4.3% of complex reconstructions in 2008 to 38.7% in 2017 — with 94.8% success vs 78.5% for BMG and 78.2% for skin flaps in that contemporary cohort[37]
  • Particularly appropriate for older patients with cardiovascular comorbidity, panurethral disease, or longer strictures; nearly half of patients undergoing first-stage Johanson refuse closure of the urethrostomy, suggesting it should be offered up front[36]

Outcomes​

EtiologyApproachSuccess
Congenital (CUPF)Simple excision or fulguration~100% — curative; correct diagnosis is the key[1][2][3]
PFUI-associatedTransperineal urethroplasty + gracilis90–100% primary; 70% redo[20][29]
Post-urethroplastyMultilayer closure ± flap71–96% (must correct distal obstruction)[5][18][19]
Lichen sclerosusStaged BMG urethroplasty or perineal urethrostomy72–91%; biopsy for SCC surveillance[13][14]
Fournier's gangreneGracilis/VRAM flap or perineal urethrostomyVariable; permanent diversion in severe cases[22][27]
Neurogenic bladderRepair attempted; most progress to diversionPoor; 81% need permanent diversion[16]

Operative Principles​

  • Recognize CUPF before operating — the dorsal urethra is normal; ventral excision is curative; misdiagnosis as urethral duplication or rectourethral fistula leads to inappropriate surgery[1][2]
  • Acquired UPF is almost always associated with concurrent stricture — treat the obstruction simultaneously or first[5][28][13]
  • Vascularized tissue interposition (gracilis, VRAM, dartos) is essential in any complex repair[20][27][30]
  • Perineal urethrostomy is a legitimate primary option — not failure — for older patients, panurethral disease, LS, neurogenic bladder, or after multiple failed reconstructions[23][34][36][37]
  • Biopsy in any LS-associated UPF to rule out SCC[13][15]
  • First repair is the best repair — refer to an experienced reconstructive center[26]

References​

1. Cheng JW, Ahn JJ, Cain MP, et al. "Misdiagnosis of congenital posterior urethroperineal fistula and comparison with urethral duplications and rectourethral fistula." Urology. 2021;158:193–196. doi:10.1016/j.urology.2021.09.013

2. Bates DG, Lebowitz RL. "Congenital urethroperineal fistula." Radiology. 1995;194(2):501–504. doi:10.1148/radiology.194.2.7824732

3. Bello JO. "Congenital posterior urethroperineal fistula: a review and report of the 25th case in literature." Urology. 2014;84(6):1492–1495. doi:10.1016/j.urology.2014.09.002

4. Zeng M, Zeng F, Wang Z, et al. "Urethral calculi with a urethral fistula: a case report and review of the literature." BMC Res Notes. 2017;10(1):444. doi:10.1186/s13104-017-2798-z

5. Horton CE, Devine CJ, Graham JK. "Fistulas of the penile urethra." Plast Reconstr Surg. 1980;66(3):407–418.

6. Ghadimi-Mahani M, Dillman JR, Pai D, Park J, DiPietro M. "MRI of congenital urethroperineal fistula." Pediatr Radiol. 2010;40(Suppl 1):S1–S5. doi:10.1007/s00247-010-1852-y

7. Wagner JR, Carr MC, Bauer SB, et al. "Congenital posterior urethral perineal fistulae: a unique form of urethral duplication." Urology. 1996;48(2):277–280. doi:10.1016/s0090-4295(96)00171-9

8. Brown WC, Dillon PW, Hensle TW. "Congenital urethral-perineal fistula: diagnosis and new surgical management." Urology. 1990;36(2):157–159. doi:10.1016/0090-4295(90)80216-a

9. Tarasconi A, Perrone G, Davies J, et al. "Anorectal emergencies: WSES-AAST guidelines." World J Emerg Surg. 2021;16(1):48. doi:10.1186/s13017-021-00384-x

10. Kuzaka B, Wróblewska MM, Borkowski T, et al. "Fournier's gangrene: clinical presentation of 13 cases." Med Sci Monit. 2018;24:548–555. doi:10.12659/msm.905836

11. Hejase MJ, Simonin JE, Bihrle R, Coogan CL. "Genital Fournier's gangrene: experience with 38 patients." Urology. 1996;47(5):734–739. doi:10.1016/s0090-4295(96)80017-3

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13. Stewart L, McCammon K, Metro M, Virasoro R. "SIU/ICUD consultation on urethral strictures: anterior urethra — lichen sclerosus." Urology. 2014;83(3 Suppl):S27–S30. doi:10.1016/j.urology.2013.09.013

14. Kulkarni S, Barbagli G, Kirpekar D, Mirri F, Lazzeri M. "Lichen sclerosus of the male genitalia and urethra: surgical options and results in a multicenter international experience with 215 patients." Eur Urol. 2009;55(4):945–954. doi:10.1016/j.eururo.2008.07.046

15. Chung ASJ, Suarez OA. "Current treatment of lichen sclerosus and stricture." World J Urol. 2020;38(12):3061–3067. doi:10.1007/s00345-019-03030-z

16. Raup VT, Eswara JR, Weese JR, Potretzke AM, Brandes SB. "Urinary-cutaneous fistulae in patients with neurogenic bladder." Urology. 2015;86(6):1222–1226. doi:10.1016/j.urology.2015.07.057

17. Granieri MA, Webster GD, Peterson AC. "Critical analysis of patient-reported complaints and complications after urethroplasty for bulbar urethral stricture disease." Urology. 2015;85(6):1489–1493. doi:10.1016/j.urology.2015.03.002

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19. Verla W, Van Nieuwenhuyse F, Hoebeke P, et al. "Urethroplasty for failed hypospadias repair related strictures in adults: a retrospective analysis with long-term follow-up." Urology. 2020;143:248–254. doi:10.1016/j.urology.2020.05.070

20. Guo H, Sa Y, Fu Q, Jin C, Wang L. "Experience with 32 pelvic fracture urethral defects associated with urethrorectal fistulas: transperineal urethroplasty with gracilis muscle interposition." J Urol. 2017;198(1):141–147. doi:10.1016/j.juro.2017.01.071

21. Erickson BA, Flynn KJ. "Management of necrotizing soft tissue infections (Fournier's gangrene) and surgical reconstruction of debridement wound defects." Urol Clin North Am. 2022;49(3):467–478. doi:10.1016/j.ucl.2022.04.008

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23. Wessells H, Morey A, Souter L, Rahimi L, Vanni A. "Urethral stricture disease guideline amendment (2023)." J Urol. 2023;210(1):64–71. doi:10.1097/JU.0000000000003482

24. Horiguchi A. "Management of male pelvic fracture urethral injuries: review and current topics." Int J Urol. 2019;26(6):596–607. doi:10.1111/iju.13947

25. Kumar S, Nagappa B, Ganesamoniv R. "Extensive balanitis xerotica obliterans of urethrocutaneous fistula presenting as mass in scrotum." Urology. 2010;76(2):332–333. doi:10.1016/j.urology.2009.09.005

26. Hillary CJ, Osman NI, Hilton P, Chapple CR. "The aetiology, treatment, and outcome of urogenital fistulae managed in well- and low-resourced countries: a systematic review." Eur Urol. 2016;70(3):478–492. doi:10.1016/j.eururo.2016.02.015

27. Paprottka FJ, Krezdorn N, Lohmeyer JA, et al. "Plastic reconstructive surgery techniques using VRAM or gracilis flaps in order to successfully treat complex urogenital fistulas." J Plast Reconstr Aesthet Surg. 2016;69(1):128–137. doi:10.1016/j.bjps.2015.08.026

28. Xu YM, Sa YL, Fu Q, Zhang J, Jin SB. "Surgical treatment of 31 complex traumatic posterior urethral strictures associated with urethrorectal fistulas." Eur Urol. 2010;57(3):514–520. doi:10.1016/j.eururo.2009.02.035

29. Plamadeala N, Waterloos M, Waterschoot M, Lumen N. "Posterior urethroplasty for pelvic fracture urethral injuries: risk factors for recurrence and complications." World J Urol. 2025;43(1):469. doi:10.1007/s00345-025-05839-3

30. Youssef AH, Fath-Alla M, El-Kassaby AW. "Perineal subcutaneous dartos pedicled flap as a new technique for repairing urethrorectal fistula." J Urol. 1999;161(5):1498–1500.

31. Hwang A, Watson M, Talluri S, Okafor H, Singh A. "A novel perivesical fat rotational flap as an alternative to omental interposition in challenging urological reconstruction." Urology. 2023;182:e262–e263. doi:10.1016/j.urology.2023.08.023

32. Johnsen NV, Voelzke BB. "Autologous rectus fascia graft interposition repair of urethrocutaneous fistulae in female-to-male metoidioplasty patients." Urology. 2018;116:208–212. doi:10.1016/j.urology.2018.03.013

33. Murphy GP, Fergus KB, Gaither TW, et al. "Urinary and sexual function after perineal urethrostomy for urethral stricture disease: an analysis from the TURNS." J Urol. 2019;201(5):956–961. doi:10.1097/JU.0000000000000027

34. Klemm J, Dahlem R, Schulz RJ, et al. "Perineal urethrostomy for complex urethral strictures: long-term patient-reported outcomes from a reconstructive referral center and a scoping literature review." J Urol. 2024;212(5):738–748. doi:10.1097/JU.0000000000004169

35. Zhao X, Li X, Song Y, Guo Q, Wang J. "Comparison of success rates between urethroplasty and perineal urethrostomy in the treatment of complex urethral strictures: a meta-analysis." World J Urol. 2025;43(1):327. doi:10.1007/s00345-025-05679-1

36. Verla W, Oosterlinck W, Waterloos M, Spinoit AF, Lumen N. "Perineal urethrostomy for complicated anterior urethral strictures: indications and patient's choice — an analysis at a single institution." Urology. 2020;138:160–165. doi:10.1016/j.urology.2019.11.064

37. Fuchs JS, Shakir N, McKibben MJ, et al. "Changing trends in reconstruction of complex anterior urethral strictures: from skin flap to perineal urethrostomy." Urology. 2018;122:169–173. doi:10.1016/j.urology.2018.08.009