Workstream 2

Workstream 2: Functional urogenital conditions requiring highly specialised surgery

Workstream 2 focuses on functional urogenital conditions requiring highly specialised surgery. The Expertise Areas (EAs) covered by Workstream 2 are below.

These EAs demand require specific diagnoses and surgical correction. Expertise is crucial to prevent costly and harmful secondary surgeries. Multi-disciplinary teams (MDTs) offer life-long care, enhancing post-operative well-being. ERN eUROGEN bridges paediatric and adult care gaps, enhancing outcomes through crossover and workstream transfer from WS1 to WS2. Improved transition ensures lifelong positive outcomes and disease self-management, ensuring continuous care across life stages.

Leads

  • WS Lead: Margit Fisch, DE Universitätsklinikum Hamburg-Eppendorf
  • Clinical Lead: Michel Wyndaele, NL Utrecht UMC
  • Education Lead: Frank van der Aa, BE Leuven UZ
  • Research Lead: John Heesakkers, NL Maastricht UMC

EA 2.1 Complicated & complex pelvic floor disorders

Definition Complicated and complex pelvic floor disorders refer to a group of conditions affecting the muscles, ligaments, and nerves that support the pelvic organs, such as the bladder, uterus, and rectum. These conditions can cause a variety of symptoms, such as pelvic pain, urinary and fecal incontinence, and sexual dysfunction. Complicated pelvic floor disorders typically involve additional medical complications, such as a history of pelvic surgery or radiation therapy, pelvic trauma, or comorbid medical conditions, which can complicate diagnosis and treatment. Complex pelvic floor disorders refer to cases that are particularly difficult to diagnose or treat, often due to the presence of multiple coexisting conditions or a lack of clear understanding of the underlying causes. Treatment for complicated and complex pelvic floor disorders may involve a multidisciplinary approach, including medication, physical therapy, behavioral therapy, and surgery, tailored to the individual’s specific condition and needs. Whilst the majority of primary conditions associated with pelvic floor dysfunction will be managed by gynaecologists and urologists with an interest in female urology and urogynaecology more complex cases should be managed by a core multidisciplinary team should consist of a urogynaecologist, female urologist, colorectal surgeon, nurse specialist and pelvic floor physiotherapist. Additional members may include neurologists, geriatricians, pharmacists and medical physicists.
Conditions & Codes
  • Vesicointestinal fistula: ICD-10 Code N32.1
  • Vesical fistula, not elsewhere classified: ICD-10 Code N32.2
  • Fistulae involving female genital tract: ICD-10 Code N82
  • Stress incontinence: ICD-10 Code N39.3
  • Other specified urinary incontinence: ICD-10 Code N39.4
  • Female genital prolapse: ICD-10 Code N81
EAC & Vice-EAC
  • Expertise Area Coordinator: John Heesakkers, NL Maastricht UMC
  • Vice-EAC: Michel Wyndaele, NL Utrecht UMC
Healthcare Providers BE Antwerp UZ, BE Leuven UZ, DE Hamburg-Eppendorf UK, ES Madrid 12 de Octubre, ES Barcelona Fundació Puigvert, ES Santander Valdecilla, FR Paris Necker, IT Padua AOU, IT Rome Gemelli, NL Nijmegen Radboudumc, NL Utrecht UMC, SE Stockholm Karolinska
Additional Resources

EA 2.2 Rare diseases & conditions affecting the female urethra

Definition Rare diseases and conditions affecting the female urethra refer to a group of uncommon and often poorly understood medical conditions that affect the structure or function of the female urethra, the tube that carries urine from the bladder to the outside of the body. These conditions can range from congenital anomalies, such as urethral duplication or urethral diverticulum, to acquired conditions, such as urethral stricture or urethral cancer. Symptoms of rare diseases and conditions affecting the female urethra can include urinary incontinence, pain or discomfort during urination, recurrent urinary tract infections, or blood in the urine. Diagnosis may involve a combination of physical exams, imaging studies, and diagnostic procedures, such as cystoscopy or urodynamic testing. Treatment for rare diseases and conditions affecting the female urethra may involve medical management, such as antibiotics or hormone therapy, or surgical interventions, such as urethral reconstruction, depending on the specific condition and the individual’s needs.
Conditions & Codes
  • Urethral stricture, urethral fistula: ICD-10 Codes N35, N36
EAC & Vice-EAC
  • Expertise Area Coordinator: Malte Vetterlein, DE Universitätsklinikum Hamburg-Eppendorf
  • Vice-EAC: vacant
Healthcare Providers BE Antwerp UZ, BE Leuven UZ, DE Hamburg-Eppendorf UK, ES Madrid 12 de Octubre, IT Padua AOU, IT Rome Gemelli, NL Nijmegen Radboudumc, NL Utrecht UMC, SE Stockholm Karolinska
Additional Resources

EA 2.3 Urethral reconstruction in rare diseases & conditions

Definition Urethral reconstruction in rare diseases and conditions refers to surgical procedures that aim to repair or reconstruct the urethra in cases of congenital anomalies or acquired conditions that affect its structure or function. These conditions can include rare diseases such as urethral duplication or cloacal anomalies, or acquired conditions such as urethral stricture or urethral cancer. Urethral reconstruction may involve various techniques, such as urethral dilation, urethral stent placement, or open surgical procedures such as urethroplasty, depending on the location and severity of the urethral abnormality. In some cases, tissue grafts or flaps may be used to reconstruct the urethra, and in other cases, the urethra may need to be completely rerouted or replaced.
Conditions & Codes
  • Urethral stricture, urethral fistula: ICD-10 Codes N35, N36
EAC & Vice-EAC
  • Expertise Area Coordinator: Gunter de Win, BE Antwerp UZ
  • Vice-EAC: vacant
Healthcare Providers BE Antwerp UZ, BE Leuven UZ, DE Hamburg-Eppendorf UK, ES Madrid 12 de Octubre, ES Barakaldo Cruces, ES Barcelona Fundació Puigvert, ES Santander Valdecilla, IT Padua AOU, IT Rome Gemelli, NL Nijmegen Radboudumc, NL Utrecht UMC, SE Stockholm Karolinska, SE Lund SUS, SE Uppsala Akademiska
Additional Resources

EA 2.4 Rare retroperitoneal diseases & conditions

Definition Retroperitoneal diseases and conditions refer to those that affect the organs and structures located behind the peritoneum, which is the membrane that lines the abdominal cavity. Rare retroperitoneal diseases and conditions are those that are not commonly found in this area and may affect various structures, such as blood vessels, nerves, lymph nodes, or connective tissues.
Conditions & Codes
  • Obstructive and reflex uropathy: ICD-10 Code N13
  • IgG4-related retroperitoneal fibrosis: ORPHAcode 49041
  • Familial vesicoureteral reflux: ORPHAcode 289365
EAC & Vice-EAC
  • Expertise Area Coordinator: vacant
  • Vice-EAC: vacant
Healthcare Providers BE Antwerp UZ, BE Leuven UZ, DE Hamburg-Eppendorf UK, ES Madrid La Paz, ES Barcelona Fundació Puigvert, IT Padua AOU, IT Rome Gemelli
Additional Resources

EA 2.5 Interstitial Cystitis

Definition Interstitial cystitis (IC) (also known as Bladder Pain Syndrome or Painful Bladder Syndrome) is a chronic condition that causes pain and discomfort in the bladder and pelvic region. The exact cause of IC is unknown, but it is characterised by symptoms such as frequent and urgent urination, pressure or pain in the bladder, and discomfort in the pelvic region. It can also lead to bladder dysfunction and decreased quality of life. There is no cure for IC, but various treatments, such as medication, bladder training, and physical therapy, can help manage its symptoms.
Conditions & Codes
EAC, Vice-EAC & ePAGs
  • Expertise Area Coordinator: Mariangela Mancini, IT
  • Vice-EAC: Alessandro Giammò, IT Turin AOU
  • Patient Representatives: Anna De Santis, AICI, Serena Bartezzati, independent
Healthcare Providers BE Antwerp UZ, BE Leuven UZ, DE Hamburg-Eppendorf UK, ES Madrid 12 de Octubre, ES Madrid La Paz, ES Barcelona Fundació Puigvert, IT Padua AOU, IT Turin AOU, IT Rome Gemelli, NL Nijmegen Radboudumc
Additional Resources
Patient Journey (Click here to download as a PDF) IC-Patient-Journey

EA 2.6 Adult urogenital reconstructive surgery (transition from WS1)

Definition This Expertise Area (EA) relates to patients transitioning from paediatric care under Workstream 1: Rare congenital uro-recto-genital anomalies once they reach adulthood. Adult urogenital reconstructive surgery is a specialised surgical subspecialty that focuses on restoring function and normal anatomy to the urinary tract and reproductive system of adult patients who have congenital abnormalities, acquired conditions, or injuries that affect these systems. This type of surgery involves complex procedures to repair, reconstruct, or replace parts of the urinary and genital organs, including the kidneys, ureters, bladder, urethra, prostate, and genitalia. The goal of adult urogenital reconstructive surgery is to improve patients’ quality of life by addressing problems such as urinary incontinence, bladder dysfunction, urethral strictures, genital malformations, and sexual dysfunction.
Conditions & Codes
  • From congenital malformations: See Workstream 1 Expertise Areas
  • Rare tumours with highly specialized surgery:
    • Nephroblastoma (Wilms’ tumor): ORPHAcode 654
    • Neuroblastoma: ORPHAcodes 635
    • Rhabdomyosarcoma: ORPHAcode 780,
    • Vulvovaginal rhabdomyosarcoma: ORPHAcode 206492,
    • Rhabdomyosarcoma of the cervix uteri: ORPHAcode 213802,
    • Rhabdomyosarcoma of the corpus uteri: ORPHAcode 213615
    • Malignant neoplasm of other connective and soft tissue, unspecified: ICD-10 Code C49.9
    • Malignant neoplasm of vagina: ICD-10 Code C52
    • Malignant neoplasm of cervix uteri: ICD-10 Codes C53, C53.1, C53.8
    • Malignant neoplasm of corpus uteri: ICD-10 Code C54.2
    • Malignant neoplasm of kidney, except renal pelvis: ICD-10 Code C64
    • Malignant neoplasm of adrenal gland: ICD-10 Code C74.9
EAC, Vice-EAC & ePAG
  • Expertise Area Coordinator: Laetitia de Kort, NL Utrecht UMC
  • Vice-EAC: vacant
  • ePAG: Kate Tyler, TOFS
Healthcare Providers BE Antwerp UZ, DE Mannheim UK, DE Hamburg-Eppendorf UK, ES Barcelona Fundació Puigvert, NL Utrecht UMC
Additional Resources

EA 2.7 Surgery for transgender patients

Definition Urinary incontinence may occur seen in transgender individuals after gender-affirming surgery. Urogenital/urological surgery for transgender patients is a specialized surgical subspecialty that focuses on addressing urinary and reproductive system-related concerns of transgender individuals. This type of surgery can include various procedures such as vaginoplasty, phalloplasty, metoidioplasty, orchiectomy, penectomy, and urethroplasty. The goal of urogenital/urological surgery for transgender patients is to alleviate gender dysphoria, a condition where individuals experience distress due to a mismatch between their gender identity and their assigned sex at birth.
Conditions & Codes
  • Transgender: Male-to-female and female-to-male surgery and surgery for complications: ICD-10 Code F64
EAC & Vice-EAC
  • Expertise Area Coordinator: Armin Soave, DE Universitätsklinikum Hamburg-Eppendorf
  • Vice-EAC: Timo Ole Nieder, DE Universitätsklinikum Hamburg-Eppendorf
Healthcare Providers DE Mannheim UK, DE Hamburg-Eppendorf UK