Youssef's syndrome, a rare complication of caesarean section: A case report

Abstract

Introduction and importance

Youssef's syndrome or vesico-uterine fistula is an abnormal communication between the bladder and the uterus. It complicating of dystocic delivery, caesarean section and uterine rupture. The prevalence is globally 1–4 % of all obstetric fistulas. In Niger, it accounts for 6.4 % of iatrogenic urogenital fistulas. The best treatment is transvesical or transperitoneal surgery to close the bladder and uterus separately, with or without retaining the uterus.

Case presentation

We report a case of Youssef's syndrome secondary to caesarean section for uterine rupture in a 32-year-old woman. She consulted in our department for urinary incontinence occurred in the postoperative period. The diagnosis was made on clinical examination using the methylene blue test that is the only diagnosis method available in our setting. Treatment was initially medical with a bladder catheter until uterine involution (six weeks), followed by surgical cure using the Chassar-Moir technique.

Discussion

Youssef's syndrome accounts 1–4 % in general population, 6.4 % in west Africa and 5,2 % in Niger. Various management were reported: Medical treatment, surgical treatment via transvesical, retroperitoneal and transperitoneal routes that remains the most indicated method and, cystoscopic fulguration and laparoscopic treatment that have recently been proposed.

Conclusion

This study reminds practitioners to be more vigilant in preventing this complication during any pelvic surgery by ensuring: careful release of vesico-uterine adhesions, bladder leakage test and careful repair of any bladder wounds followed by postoperative bladder drainage.

Highlights

• •Youssef's syndrome is a rare form of urogenital fistula caused by obstetrics complications or complicating pelvic surgery.
• •Treatment of Youssef syndrome is bladder catheter and surgical repair.
• •In this case report, the authors discuss the aetiopathogenic, diagnostic and therapeutic aspects of this rare pathology.

1. Introduction

Youssef's syndrome or vesico-uterine fistula is a rare form of urogenital fistula characterised by an abnormal communication between the bladder and the uterus [1]. Fluid flow is classically one-way, with menstrual flow into the bladder, giving the classic cyclic haematuria. [1,2] The main causes of Youssef's syndrome are lower segment Caesarean section with an undiagnosed bladder injury (83–93 %), induced abortion, dilatation and curettage, vaginal birth after previous Caesarean section, obstructed labor, forceps delivery, placenta percreta, migrated intrauterine contraceptive device, brachytherapy, advanced uro-gynecological malignancies such as cervical cancer and, radiation induced therapies [[2], [3], [4], [5]]. It was first described in 1908 by Knipe et al. From 1974 to date, there have been less than 55 cases reported in the literature [3]. In 1957, the Egyptian Abdel Fattah Youssef was the first to propose the term “menouria” for vesico-uterine fistula, that gave his name to this condition, which became known as Youssef's syndrome. This syndrome is characterised by the triad of cyclic haematuria or menouria, amenorrhoea and complete continence of urine. Management must be performed with a multidisciplinary approach comprising of gynaecologist and urologists. The best treatment is transvesical or transperitoneal surgery to close the bladder and uterus separately, with or without retaining the uterus [6,7]. Medical treatment with continuous bladder drainage has a low success rate [6]. We report a case of vesico-uterine fistula complicating a caesarean section in a 32-year-old patient treated in our department. We discuss the aetiopathogenic, diagnostic and therapeutic aspects of this rare condition. This work has been reported in line with the SCARE criteria [8].

2. Case presentation

This was a 32 years old woman, gravida 7, para 6 and mother of six live children. Her history included an undocumented caesarean section prior in her 6th pregnancy. She was admitted to our department with uterine contractions in a 38 weeks pregnancy. On general examination, she was anxious. She was not pale. Her vitals were within normal range, and she was apyrexic. On obstetrical examination, the uterus was 32 cm high and the fetal heart rate was 138 beats/min. There were four uterine contractions in 10 min, with persistent pelvic pain between contractions. There was minimal vaginal bleeding. The cervix was dilated to 4 cm, the membranes had ruptured and the foetus was in cephalic position. The fingernail brought back blood‑tinged amniotic fluid. The bladder catheter returned haematic urine. Emergency obstetric ultrasound revealed a pregnancy at 38 weeks' gestation with no abnormalities of the foetus or adnexa. No uterine breach was observed on ultrasound. The biological assessment showed a haemoglobin level of 11 g/dl; her renal and liver function tests were within normal range. These clinical signs were suggestive of disunion of the uterine scar from the previous caesarean section. We performed a caesarean section. Intraoperatively, there were numerous parieto-uterine and vesico-uterine adhesions, which were released with difficulty. We extracted a healthy newborn weighing 2.7 kg. We proceeded with an incision of the margins and then a hysterorraphy using Vicryl 1 overjet. Post operatively there was no any complications reported. The patient was discharged from hospital on post-operative days 7, in good health. Fourteen days later, the patient was admitted again with urinary incontinence. Speculum examination coupled with intravesical injection of methylene blue showed leakage of methylene blue through the cervix. The diagnosis of a vesico-uterine fistula was made. Urine culture and sensitivity revealed no organism growth. Management consisted of insertion of an indwelling bladder catheter for six weeks, followed by transperitoneal surgical cure of the fistula. Adhesionalysis was achieved with difficult. After isolation of the fistulous path. We closed the bladder and uterine orifices after gullying the edges. Post-operative management was straightforward (bladder catheter, Paracetamol 1 g for infusion twice a day, antibiotic prophylaxis with amoxicillin and metronidazole for 5 days). Surgery was performed in rural area with high risk of post-operative infectious complication. So we gave antibiotic for five days according to the protocol of service. The bladder catheter was left in place for three weeks. After three months follow up catheter was removed with resolved initial symptoms.

3. Discussion

Vesico-uterine fistula is a rare condition, accounting for only 1 to 4 % of all obstetric fistulas, and particularly affects young women of childbearing age [1]. The frequency varies from one country to another. In Niger, it accounts for 6.4 % of iatrogenic urogenital fistulas [6]. This frequency is increasing all over the world because of the more frequent use of Caesarean section. [3,4] In the aetiopathogenesis point of view, it is caused by obstetric events or secondary to caesarean section or uterine rupture, as in this case report. According to the literature, one in three fistulas occurs after repeated caesarean sections due to difficult vesico-uterine detachment [4,6]. In this case, the release of parieto-uterine and vesico-uterine adhesions was laborious. Cases of Youssef's syndrome have been described after insertion of an intrauterine device, manual removal of the placenta, uterine curettage or biopsy and d treatment of cervical carcinoma by brachytherapy [4]. The contiguity of the genital and urinary systems in the pelvis exposes both to obstetrical trauma. Physiopathologically, the vesico-uterine fistula (VUF) is due to the existence of a cervico-isthmic uterine sphincter that only allows the fistula to function in the uterus-bladder direction, which is sometimes difficult to understand. Logically, the fistula should function in both directions, since it allows the bladder and uterus to communicate [6,7]. Numerous cases of utero-vesical “one-way” fistulas with perfect urinary continence have been reported in the literature [6]. The mechanism of fistula function remains controversial. For most authors, the location of the fistula in relation to the uterine isthmus determines the direction in which the fistula functions [6]. Thus, fistulas located below the isthmus would function in both directions with urinary leakage and haematuria; those opening into the isthmus would be one-way with catamenial haematuria, amenorrhoea and perfect urinary continence. The uterine isthmus therefore plays a fundamental role in diverting menstruation towards the bladder and also retaining urine in the bladder. [4,7] Isthmic tone after the return from childbirth appears to be the determining factor in the clinical symptomatology of Youssef's syndrome. When its tone has recovered well, menstrual blood finds its way more easily to the bladder where the pressure is lower, explaining the absence of urinary leakage through the vagina, menouria and amenorrhoea. This suggests that the fistula functions in both directions before uterine involution [1,2]. This was the case with our patient in whom the fistula was revealed by urinary incontinence three weeks after the caesarean section. The clinical symptoms are varied. Youssef's syndrome is typically characterised by association of menouria with amenorrhoea and urinary continence. However, this typical picture is rare. Urinary continence is rarely observed, and urinary urgency is sometimes noted. Finally, symptoms may vary over time in the same patient, resulting in successive “two-way” forms, with initial urinary leakage followed by catamenial haematuria with perfect urinary continence [2,9]. In our case, the clinical picture was dominated by urinary leakage. The diagnosis was primarily clinical. Gynecological examination under valve before and after filling the bladder with methylene blue allows diagnosis by observing the leakage of the product through the uterine cervix, which indicates the presence of the fistula. However, this examination is not always positive, hence the need for further investigations, in particular intravenous urography (IVUS) and a urinary common rejection module (UCRM) [4,9]. The methylene blue test can also be used to rule out a vesico-vaginal fistula or true urinary incontinence. The punctured tyre technique is a simple method of diagnosing vesico-uterine fistulas: it involves filling the bladder with air and the vagina with warm distilled water in order to observe bubbles [6]. The diagnosis can also be made in the immediate post-operative period on the basis of the bladder leakage test. From a paraclinical point of view, IVUS is the imaging test most frequently used in the literature. It can establish the diagnosis by showing uterine opacification during the cystogaphic phase, and helps to rule out an associated ureteral lesion. UCRM is generally performed when IVUS is inconclusive. Cystoscopy in dry forms with associated menorrhagia and vaginal menstruation enables the bladder orifice of the fistula to be visualised, its size and location to be determined and a bladder endometriosis to be ruled out. [9] Magnetic resonance imaging has the specificity of showing the hypodense aspect of the fistulous path and the hyperdense aspect of the endometrial cavity due to the stagnation of urine [6]. CT urography is helpful for the confirmation of diagnosis and should give a differential diagnosis like vesical endometriosis [7]. But any of these exams were available in our setting. In our setting, the methylene blue test is the only diagnosis method available and was sufficient to make the diagnosis in this case report.

Surgical treatment via transvesical, retroperitoneal and transperitoneal routes remains the most indicated method for vesico-uterine fistulas. The aim of surgery is to close the bladder and uterine orifices separately, with or without preserving the uterus [6,9,10]. In this case, despite the difficulties of vesico-uterine detachment, we were able to preserve the uterus. The surgery is inspired by the Chassoir Moir technique. This technique comprises three essential steps: The first step consists of exposing the operating field using an auto-static retractor between the right muscles to keep them separated. An additional digital exploration is performed. Once the fistula has been located, a bladder catheterisation and methylene blue test are carried out. A Foley catheter is then inserted through the fistula and traction is applied to the FUV using the Foley catheter or 4 threads. The second step is dissection: Incision around the fistula 2–3 mm from the edge with the number 11 scalpel. This may be the uterine or cervical wall, with the uterine cavity opening into the bladder. The bladder is separated from the uterus by 2 cm all round. This dissection is performed using angled dissecting scissors. The third step consists of repairing the fistula: each plane is repaired through the fistula and closure is generally done in three planes, starting with the deepest:

• -The uterus, the deepest plane, is repaired with interrupted absorbable sutures.
• -Interposition plane: The rectus muscle may be inserted, especially if there is an increased risk of failure, for example if the tissue is friable or if the urine is infected.
• -Bladder: The bladder wall is exposed. A plane of interrupted or continuous 2/0 or 3/0 Vicryl sub-mucosal sutures is inserted. Finally, the mucosal plane is closed with interrupted or, more often, continuous sutures using 3/0 Vicryl, which provides better haemostasis [11].

Cystoscopic fulguration and laparoscopic treatment have recently been proposed. According to their authors, they offer the same quality of repair as laparotomy. A urinary catheter is essential, and a radiographic check three to four weeks after the cure is desirable [1,2,9]. Some authors suggest medical treatment for fistulas diagnosed before three months post-partum [4,9]. Indeed, several cases of spontaneous regression by bladder drainage, sometimes with suction, for two to eight weeks have been reported in the literature [2,9]. This medical management, with a success rate of less than 5 %, requires two conditions: Early discovery of the fistula (less than three months) and the possibility of stopping fluid leakage through the fistulous orifice by bladder drainage [4,7]. It should be noted that these two conditions were met in our patient. However, the fistula persisted after six weeks of medical treatment. Definitive cure was obtained after surgical treatment.

4. Conclusion

Vesico-uterine fistula is a rare but increasingly common obstetric complication in developing countries. It is mainly secondary to caesarean section, uterine rupture, hysterectomy and instrumental manipulation. The gold standard treatment is surgery. Emphasis should be placed on prevention, with certain precautions taken during caesarean section: careful release of vesico-uterine adhesions, bladder leak test and repair of any bladder wounds under cover of postoperative bladder drainage.

Ethical approval

The publication of this case report has been authorized by the quality service of our Institution because case reports are exempted from ethical approval in our institute.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Author contribution

HSD and IS managed the patient and wrote the first draft. M.C.Y, R.M.G, M.S.O.M, M.N helped in editing and reviewing the paper. All authors read and approved the final version to be published.

Guarantor

Hamidou Soumana Diaouga

Research registration number

None.

Patient consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Conflict of interest statement

The authors declare having no conflicts of interest for this article.