Categories
Female Reproductive System ORGAN SYSTEMS

Youssef’s syndrome

Cyclic hematuria, absence of vaginal bleeding (amenorrhoea), menouria, and urinary continence due to vesicouterine fistula (VUF).

Cyclic hematuria, absence of vaginal bleeding (amenorrhoea), menouria, and urinary continence due to vesicouterine fistula (VUF).

  • VUF is the least common urogynecological fistulae (1–4%)

History:

A vesicouterine fistula is an abnormal pathway between the bladder and the uterus. The first case was reported by Knipe and colleagues in 1908. In 1957, Youssef described a syndrome comprising of cyclic hematuria, amenorrhea, menouria, and complete urinary incontinence in a patient who had lower segment Cesarean section (LSCS). In previous years, it used to be regarded as a complication of assisted delivery applications like vacuum and forceps techniques. Today, 83–93% of VUFs are observed after caesarean delivery

Classification

VUF clinical classification:

Based on the routes of menstrual flow
  • Type 1: Triad of amenorrhea, menouria (cyclic hematuria) and complete continence of urine “Youssef’s syndrome” (M/C, 90% cases)
  • Type 2: Dual menstrual flow through both the bladder and vagina)
  • Type 3: Normal vaginal menses and lack of menouria)

Aetiology

  • LSCS (M/C, 83–93% cases)
  • High vaginal forceps-aided delivery
  • External cephalic version
  • Curettage/manual removal of placenta
  • Placenta percreta
  • Myomectomy
  • Uterine rupture due to obstructed labor
  • Uterine artery embolization (UAE)
  • Perforation of intrauterine device
  • Brachytherapy for carcinoma of cervix

Clinical features

Triad:

Amenorrhea, cyclic hematuria without urinary incontinence in combination with a history of LSCS has been described as pathognomonic of VUF
  1. Cyclical hematuria
  2. Amenorrhea
  3. Urinary continence (urinary incontinence occurs if the level of the VUF is at or below the internal os or if the os is incompetent)

Diagnosis

Diagnosis is mainly established by clinical detection of urine or dye passing through the external cervical os or by means of a hysterosalpingogram or micturating cystourethrogram, which will demonstrate the fistulous communication.

Ultrasonography:

  • Double echogenic line between the uterus anterior wall and the posterior wall of the bladder

Cystoscopy

Determination and localization of the presence of a fistula and the determination of its position with the trigone.
Cystoscopic view of the fistula. | Bhattacharjee, S., Kohli, U. A., Sood, A., Tripathy, S., & Gupta, M. (2015). Vesicouterine fistula: Youssef’s syndrome. Medical journal, Armed Forces India, 71(Suppl 1), S175–S177. https://doi.org/10.1016/j.mjafi.2013.11.006

MRI:

Coronal T2-weighted HR/SENSE MR image depicting the abnormal hypointense out pouching and fistulous tract (arrow) from posterior wall of bladder to anterolateral wall of uterus above the isthmus. Endometrial cavity is being opacified (hyperintense) with urine | Shanmugasundaram, R., Gopalakrishnan, G., & Kekre, N. S. (2008). Youssef’s syndrome: Is there a better way to diagnose?. Indian journal of urology : IJU : journal of the Urological Society of India, 24(2), 269–270. https://doi.org/10.4103/0970-1591.40631

Hysterosalpingography (HSG):

Gold standard investigation in demonstrating the fistulous track.
HSG depicting the fistulous tract | Taingson, M., Adze, J., Bature, S., Amina, D., Caleb, M., Amina, A., … Silas, B. (2017). Post-caesarean vesicouterine fistula: Youssef’s syndrome – A case report. Archives of International Surgery, 7(4), 132–134. https://doi.org/10.4103/ais.ais_40_17

Management

Treatment methods include expectant management with long-term bladder catheterization, medical treatment, and surgery.

Medical management:

Involves induction of amenorrhea to aid in fistula healing
  • Oral contraceptives
  • Progestational agents
  • Gonadotropin releasing hormone analogs

Surgical management:

Definitive method of treatment. It can be performed transabdominally, endoscopically, and robotically.
  • Transabdominal repair: Extraperitoneal or retrovesical (O’Connor) technique
  • Laparoscopic approach

Leave a Reply

%d bloggers like this: