Cyclic Hematuria Research Articles

The prevailing social stigma around disorders of sexual development in developing countries

Disorders of sexual development (DSD), apart from medical disease, is a psychosocial stigma to not only the patient but also their whole family. We present the case of a 24-year-old 46XX, raised as male, with ambiguous genitalia and cyclical hematuria with normal female internal genitalia and secondary sexual characteristics. This case underscores the influence of social stigma and the psychosocial vulnerability associated with DSD on individuals’ lives in developing countries.

Atypical presentation of vesicouterine fistula (Youssef’s syndrome) post-caesarean gossypiboma: a case report

Vesicouterine fistula as described by Youssef manifests with amenorrhea, and cyclic haematuria (menouria) without urinary incontinence commonly following caesarean section. Other aetiologies have been reported for this disease. The post-caesarean section gossypiboma, a dual aetiology causing this condition is rare and can make its presentation atypical, thus posing a diagnostic dilemma. The clinical evaluation alone in the atypical presentation of the vesicouterine fistula is insufficient except complemented by imaging investigations and endoscopic examination of the related organs. We report a case of vesicouterine fistula that was managed initially as chronic pelvic inflammatory disease despite urinary incontinence until further evaluation including a transvaginal ultrasound scan and urethrocystoscopy raised an index of suspicion of a vesical mass suggestive of gossypiboma. The mass was confirmed to be a foreign body, an abdominal gauze pack, or gossypiboma. Its removal and repair of the vesicouterine fistula were associated with a satisfactory outcome.

Fertility Preserving Robotic Surgery for Infiltrating Bladder Endometriosis

<h3>Study Objective</h3> The uterus is normally lined by endometrium, when any other site has endometriotic tissue it is known as pathological endometriosis. If this endometroitic tissue is found in the gynaecologist most feared organ the bladder it is bladder endometriosis which is a rare entity almost to the tune of 1-2%. <h3>Design</h3> Mrs V, nulligravida with primary infertility presented to Gyane OPD with complaints of dysmenorrhea, dyspareunia and heavy menstrual bleeding for last one and a half years. She had cyclical hematuria. On history a strong clinical suspicion of bladder endometriosis was confirmed by transvaginal ultrasound (TVS) and MRI <h3>Setting</h3> Cystoscopy performed by Urologist reaffirmed the diagnosis of bladder endometriosis. A 3.5 × 2cms nodule was visualized in bladder mucosa on posterior wall away from the uteric orifice. Using operative cystoscopy transurethral coring of the endometroitic nodule in the bladder was uptil the muscularis. <h3>Patients or Participants</h3> 1 <h3>Interventions</h3> Robotic Fertility Sparing Surgery for Infiltrating Bladder Endometriosis <h3>Measurements and Main Results</h3> Multidisciplinary approach towards bladder endometriosis and complete disease resection <h3>Conclusion</h3> Bladder endometriosis can be diagnosed both clinically & radiologically. With the advent of robotic surgery, it has now become easier to perform such complex surgeries with better postoperative

Diagnosis and Management of Vesicouterine Fistula (Youssef’s Syndrome): A Case Report

Background. Vesicouterine fistula (VUF) is a rare pathological communication between the uterus or cervix and the bladder. Youssef's syndrome is an atypical presentation of a patient with a vesicouterine fistula, characterized by a triad of cyclic hematuria, amenorrhea, and absence of urinary incontinence. Because of this atypical presentation, the patient may go undiagnosed and patient management will be delayed.&#x0D; Case presentation. A 39-year-old woman complained of hematuria. The patient underwent a second caesarean section in 2008 and a few weeks after the procedure the patient complained of leakage of urine from her vagina. Six months later the patient complained hematuria on her menstrual cycle and amenorrhea, with absence of urinary incontinence. Ultrasound examination found adhesions between the uterus and vagina, and irregular bladder mucosa. Patient underwent cystoscopy with endometriosis were suspected on vesicovaginal repair scars and a bladder biopsy was also performed with the results of no endometrial stroma and glands then followed by hysteroscopy. Hysteroscopy result showed a 20 mm diameter hole with irregular margins. Subsequently, the patient was treated with hysterorrhaphy laparotomy and fistula repair.&#x0D; Conclusion. One type of vesicouterine fistula is Youssef's syndrome, which is a complication following a lower segment caesarean section with undiagnosed bladder injury. The diagnosis can be confirmed by cystoscopy, intravenous pyelography, hysterosalpingogram, sonography, and other types of imaging tests. Management of patients with VUF can be conservative, medical, or surgical.

Surgical Evaluation and Management of Concomitant Anterior and Posterior Deep Infiltrating Endometriosis

Study Objective The objective of this video is to present the evaluation and management of a patient who presents with concomitant anterior and posterior compartment deep infiltrating endometriosis. Design Surgical Video. Setting The operating room. Patients or Participants We present the case of a 34-year-old G0 female who was referred for evaluation and management of biopsy proven bladder endometriosis. The patient reported a long-standing history of chronic pelvic pain, dyspareunia, dysmenorrhea, cyclic hematuria and cyclic dyschezia as well. Of note, she had failed 2 IVF cycles. On pelvic examination, the patient was noted to have a fixed uterus with a scarred posterior cul-de-sac with significant nodularity. Although the patient had known bladder endometriosis, her history and physical exam findings were suggestive of possible rectosigmoid involvement as well. The patient then underwent further evaluation with imaging including a transvaginal ultrasound and pelvic MRI. The transvaginal ultrasound demonstrated hypoechoic nodules in the anterior and posterior compartments, highly suggestive of deep infiltrating endometriosis. The MRI demonstrated anterior compartment disease transmurally invading the urinary bladder as well as posterior disease as well. Given these findings, the patient underwent preoperative planning with a multidisciplinary team including Gynecologic surgery, Urology, and Colorectal surgery. Interventions Definitive surgical management. Measurements and Main Results The patient underwent a cystoscopy, bilateral ureteric stenting, cystectomy, and excision of endometriosis. In the post operative period, the patient underwent a retrograde cystogram which demonstrated no leakage at the site of repair. Her symptoms improved significantly and she is now attempting to conceive. Conclusion A thorough pre-operative evaluation of patients with deep infiltrating endometriosis is of utmost importance. Although the patient was referred for bladder endometriosis, she was incidentally found to have significant posterior compartment disease. As a result, she underwent surgical planning with a multidisciplinary team, which ultimately allowed for the best patient outcome. The objective of this video is to present the evaluation and management of a patient who presents with concomitant anterior and posterior compartment deep infiltrating endometriosis. Surgical Video. The operating room. We present the case of a 34-year-old G0 female who was referred for evaluation and management of biopsy proven bladder endometriosis. The patient reported a long-standing history of chronic pelvic pain, dyspareunia, dysmenorrhea, cyclic hematuria and cyclic dyschezia as well. Of note, she had failed 2 IVF cycles. On pelvic examination, the patient was noted to have a fixed uterus with a scarred posterior cul-de-sac with significant nodularity. Although the patient had known bladder endometriosis, her history and physical exam findings were suggestive of possible rectosigmoid involvement as well. The patient then underwent further evaluation with imaging including a transvaginal ultrasound and pelvic MRI. The transvaginal ultrasound demonstrated hypoechoic nodules in the anterior and posterior compartments, highly suggestive of deep infiltrating endometriosis. The MRI demonstrated anterior compartment disease transmurally invading the urinary bladder as well as posterior disease as well. Given these findings, the patient underwent preoperative planning with a multidisciplinary team including Gynecologic surgery, Urology, and Colorectal surgery. Definitive surgical management. The patient underwent a cystoscopy, bilateral ureteric stenting, cystectomy, and excision of endometriosis. In the post operative period, the patient underwent a retrograde cystogram which demonstrated no leakage at the site of repair. Her symptoms improved significantly and she is now attempting to conceive. A thorough pre-operative evaluation of patients with deep infiltrating endometriosis is of utmost importance. Although the patient was referred for bladder endometriosis, she was incidentally found to have significant posterior compartment disease. As a result, she underwent surgical planning with a multidisciplinary team, which ultimately allowed for the best patient outcome.

Vesicouterine Fistula: A Robotic Approach.

This video demonstrates surgical repair of a vesicouterine fistula via a robotic, uterine-sparing approach. In this video, we present a vesicouterine fistula, which occurred after cesarean delivery. The patient presented with cyclical hematuria 4 years following delivery. She underwent uterine-conserving robotic repair via excision of the fistula tract through an intentional cystotomy. The uterus and bladder were closed in multiple layers. The patient tolerated the procedure well, and CT cystogram 6weeks following surgery demonstrated no concern for defect or recurrent fistulization. The patient was asymptomatic 9months following her procedure. Repair of a vesicouterine fistula may be safely completed via a minimally invasive approach without need for routine hysterectomy.

SEMINOMA ARISING FROM TESTICULAR AND OVARIAN REMNANTS HERALDS THE EMERGENCE OF A RARE MALE OVOTESTICULAR DISORDER OF SEXUAL DEVELOPMENT

INTRODUCTIONOvotesticular Disorder of Sex Development (OT DSD) or true hermaphroditism is a very rare subset of DSD and accounts for only 5% of cases. It has great phenotypic variability and poses diagnostic challenge to clinicians. It usually presents in childhood with ambiguous genitalia, characterized by histologic demonstration of ovarian and testicular tissues within the same individual. RESULTSWe describe a 22-year-old man with OT DSD complicated by seminoma. He was delivered preterm with ambiguous genitalia and was lost to follow-up. He was raised as a female, at 14 years old, his teachers referred him due to concerns of male phenotype while participating in competitive sports. Phenotypically, he was a developed male with a micropenis, hypospadia, left scrotal cystic structure and empty right scrotum. Chromosomal analysis revealed 46XY and presence of SRY gene. Radiological imaging at age 16 showed fully developed Mullerian structures, with a single cervix and incomplete septate uterus, and an oval structure suggestive of testes at the left hemipelvis. Cysto-genitoscopy demonstrated normal urethra without prostatic urethra, opening at posterodistal bladder neck with blood clots likely representing the vagina. Laparoscopy identified tubulo-nodular structure inside the pelvis suggestive of vas deferens with suspicious early malignant changes. Wolffian remnant and bicornuate uterus were present with a right Fallopian tube with suspicious hydro-corpus. He was advised surgery, however, he defaulted again. He consulted again at age 22 due to pyuria, suprapubic pain and painless cyclical haematuria. Imaging studies demonstrated pyometra, bulky left ovary and bilateral undescended testes suspicious of malignant transformation. Exploratory laparotomy, gonadectomy, subtotal hysterectomy and left orchidectomy were performed. Histopathological examination revealed seminomas arising from testicular and ovarian remnants with suppurative inflammation in the uterus. He was provided testosterone replacement post operatively, and received chemotherapy (etoposide, bleomycin and platinum). CONCLUSIONThe complexity of the case exemplified the pivotal role of multidisciplinary input from various specialties.

Spectrum of external genital anomalies in disorders of Sex Development at Children Hospital & Institute of Child Health, Lahore, Pakistan.

Objective:To describe the mode of presentation and frequency of external genital anomalies in disorder of sex development (DSD)Methods:This cross-sectional study was conducted at Children Hospital & Institute of Child Health, Lahore from January to December, 2016 on Children with DSD above 10 years of age. A detailed history and physical examination were done. Positive findings were recorded on a predesigned proforma and analyzed by SPSS 21. Karyotyping on blood samples was done to determine their genetic sex.Results:Out of 83 DSD children, 67% (n=56) were assigned a female sex at birth of which 9% (n=5) had ambiguous genitalia. Male sex at birth was given to 33% (n=27) of which 96% (n=26) had genital ambiguity. Mode of presentation other than ambiguous genitalia were delayed puberty, amenorrhea, hirsuitism, gynaecomastia, cyclic hematuria etc. Clitoromegaly was the main finding in 62.5% (n=5) and micropenis in 45% (n=9). Karyotypic sex of 56 female sex of rearing was 46XX 80% (n=45), 45X0 13% (n=7), XXX 2% (n=1) and 46 XY in 5% (n=3). Karyotypic sex of 27 male sex of rearing was 46XY in 78% (n=21), 46XX in 15% (n=4) and 47XXY in 7% (n=2).Conclusion:Disorders of sex development presented with a wide spectrum of external genital anomalies ranging from clitoromegaly in females to micropenis and hypospadias in males. There was also an extreme diversity in mode of presentation of these cases including pubertal delay, amenorrhea in females and gender confusion disorders.

A Rare Complication After Cesarean Section: Vesicouterine Fistula

Vesicouterine fistulas are rarely seen (1-4%) genitourinary fistulas which occur mostly due to iatrogenic reasons related to gynecologic and obstetric surgeries. They are mostly caused by iatrogenic reasons. Vesicouterine fistulas which are seen after cesarean section, which are not accompanied by urinary incontinence but cause amenorrhea and cyclic hematuria are called Youssef syndrome. In this article, we aim to present a case of vesicouterine fistula which developed after the fifth cesarean section, in the light of the literature.

Vesicouterine fistula – a rare delayed complication of cesarean section

A vesicouterine fistula is an abnormal pathway between the urinary bladder and the uterus. It is a rare urogynaecological fistula. In vesicouterine fistula, clinical presentations are cyclic haematuria (menouria), amenorrhoea, urinary continence which mimics Youssef’s syndrome. This case presents, a 37 year old multiparous woman with vesicouterine fistula which she developed 8 years after cesarean section. Diagnosis was confirmed by cystoscopy and fistula was repaired through trans abdominal surgery. She regained normal vaginal menses 4 months after surgery.&#x0D; J Bangladesh Coll Phys Surg 2020; 38(4): 209-212

Klinefelter Syndrome Mosaicism 46,XX/47,XXY: A New Case and Literature Review.

Most cases of Klinefelter syndrome (KS) have 47,XXY karyotype. We reported the first case of 46,XX/47,XXY KS whose genital ambiguity was detected prenatally with postnatal confirmation of the mosaicism and ovotesticular disorder of sex development (OT-DSD). The paternal origin of the extra X chromosome was identified using trio cytogenomic single-nucleotide polymorphism array. Additional 18 cases were also reviewed. The clinical presentation of 46,XX/47,XXY is age-dependent with two age peaks, including ambiguous genitalia during infancy and gynecomastia with or without cyclical hematuria and left scrotal pain and mass in adolescence. The 46,XX is the predominant karyotype both in peripheral blood and gonadal tissue. The risk of germ cell tumor is very high throughout life in these individuals. Individuals with 46,XX/47,XXY mosaicism should be treated more as OT-DSD other than a simple mosaic KS. A multidisciplinary approach and long-term monitoring are necessary.

MRI in evaluation of cyclical hematuria: A rare case of persistent cloaca with Mullerian duct anomaly.

A rare case of persistent cloaca with associated Mullerian anomaly, emphasizing the role of pre‐operative MRI for planning appropriate surgical management and obtaining acceptable outcomes.

2934 Minimally Invasive Treatment of Bladder Deep Endometriosis and Isthmocele

Video Objective To demonstrate the endoscopic management of bladder endometriosis and isthmocele. Setting Bladder endometriosis, defined by the presence of detrusor muscle layer invasion, affect around 1%-2% of womens. In nowadays, laparoscopic partial cystectomy is the gold standard surgical treatment. By other hand, the isthmocele is a defect of the anterior wall of the cervical canal at the site of the previous C-section, and affect around 0.6% of women's. It will be symptomatic in about 30% of the cases with AUB as the main presentation. Laparoscopic treatment report between 59%-90% of symptom control, and will be choose when overlying myometrial mantle is less than 3mm and/or 50% of total myometrial thickness. Due the invasive nature of endometriosis, added to the inflammatory response following the c-section heal, it is not surprising that both pathologies could be anatomically closer. A 37 years old patient, with clinical history of dysuria, cyclic hematuria and post-menstrual spotting. Ultrasound evidence a 29 millimeter hypo-echogenic nodule at the bladder dome plus a 12mm isthmocele, with overlying miometrial mantle of 7mm. Interventions Hysteroscopic isthmoplasty was done using a mini-resectoscope including the treatment of both caudal and cranial edges and resection of all the diverticular mucosal hyperplasia. Posteriorly, laparoscopic partial cystectomy was done. Under general anesthesia, the patient was placed in 0 degrees dorsal decubitus with her arms alongside her body. Operative set-up include 15mmhg pneumoperitoneum and four trocars: a 10mm trocar at the umbilicus for a zero-degree laparoscope; a 5mm trocar in the right and left iliac fossa; and a 12mm trocar in the suprapubic area. Using mechanical, electrical and ultrasonic energy, partial cystectomy followed by two layer closure was done. During final chromotubation, massive leakage was noted coming from the isthmic area. Complementary laparoscopic resection and closure was done. Histopathological result show no cleavage plane between isthmus area and bladder endometriosis tissue. Conclusion Full endoscopic treatment of bladder endometriosis and Isthmocele is feasible, safe and effective in experienced hands. In this patient, procedure was uneventful, without any intra or pos-operative complications.

THE VALUE OF MAGNETIC RESONANCE IMAGING AS COMPARED TO LAPAROSCOPY FOR THE DETECTION OF ENDOMETRIOSIS

The objective of the study is to determine the value of MRI for detecting Endometriosis by considering the main determinants of diagnostic accuracy which are essential to minimize false positive and false negative diagnoses. The present study aims to assess the diagnostic value of MRI as a standard reference to detect endometriosis as compared to the laparoscopic findings. According to the opinion given by a panel of experts, forty consecutive patients with complaints of chronic pelvic pain, infertility, dysmenorrhea, dyspareunia, cyclic hematuria, pelvic pain between menstrual cycles and corresponding gastrointestinal symptoms, were referred to the Radiology department for further evaluation. The MRI examinations were performed with a 1.5- T unit. After MRI, the patients underwent laparoscopy within a maximum interval of 10 days as the reference standard. Data was analysis by SPSS. Sixteen patients underwent normal laparoscopy and normal MRI without any endometriotic lesions. In the other 16 patients, laparoscopy could identify 24 sites of endometriosis (20 endometriomas, 3 in posterior cul-desac, 1 in trigon of bladder). 20 out of these 24 sites (83.3%) were reported in their MRI. It is evident that the MRI had a sensitivity, specificity, PPV, NPV, and accuracy of 93.7%, 100%, 100%, 94.1%, and 96.8%, respectively for the diagnosis of Endometriosis. MRI with its high sensitivity and specificity can be considered as a high reproducible tool to explore Endometriosis.

Successful Repair of Iatrogenic Cyclical Menouria

Vesicouterine fistula (VUF) is a very rare occurrence and is estimated to occur in only (1-4%) of all genitourinary fistulas. It is an abnormal pathway between the bladder and the uterus. The most common cause is lower segment caesarean section. Patients usually present in the early post operative period with the problem of continuous urinary incontinence. On the rare occasion, recurrent urinary tract infection, recurrent gross painless haematuria, or secondary infertility associated with secondary amenorrhoea would be the presenting complaint. Among all vesicouterine fistula 90% are Youseef’s Syndrome the least common of the urogynaecological fistulas. Youseef’s syndrome is characterized by cyclic haematuria (menouria), absence of vaginal bleeding (amenorrhea) and urinary incontinence due to vesicouterine fistula (VUF).TAJ 2017; 30(1): 70-72

Vesicouterine fistula (Youssef's Syndrome): A case report and review of literature

Vesicouterine fistula is a rare urogynecological fistula, usually iatrogenic following lower uterine segment cesarean section. The clinical features of cyclical hematuria, amenorrhea, and urinary continence, Youssef's syndrome, were first reported in the literature in 1957, among patients with vesicouterine fistula. The case reported was a 36-year-old multipara who had two previous lower segment cesarean sections and presented with amenorrhea and menouria. Hysterosalpingography confirmed the diagnosis. The fistula was repaired following the transabdominal surgery. She regained normal vaginal menses.

A very rare complication of vesico-uterine fistula following lower segment caesarean section

Introduction Vesico-uterine fistula (VUF) is a recognised but rare complication following lower segment caesarean section with an incidence of less than 4% of all urogenital fistulae. Patients commonly present with urinary incontinence, cyclical haematuria, amenorrhoea, infertility and spontaneous first trimester abortions. Presentation can be immediate or delayed. Case description A 34-year-old lady presents with an eight-day history of continuous urinary leakage, following LSCS. She had the operation due to failure to progress during labour. She was discharged home after 2 days only to re-present. Speculum examination revealed drainage of clear fluid from the vagina. A vesico-vaginal fistula (VVF) was suspected and she underwent intravenous urogram (IVU) and CT abdomen and pelvis. Radiological findings revealed a suspected fistula but its precise anatomy was uncertain, hence a diagnosis was not made. She underwent examination under anaesthesia, cystoscopy, colposcopy and hysteroscopy, which revealed a small defect in the dome of the bladder and further advancement of the scope led to an entry into a second confined space – the uterus. Clearly there was communication between these spaces. A hysteroscopy revealed missing anterior aspect of the cervix, and proximal to this was the fistula. A urinary catheter was inserted and elective laparotomy planned in six weeks to allow for uterine involution. On the day of her operation, the patient reported cessation of urinary leakage and felt there was no significant vaginal discharge. The assumption was that her fistula had closed spontaneously. A formal cystogram was performed showing the presence of contrast within two cavities. Methylene blue dye was instilled into the bladder via the urinary catheter. Blue staining on the cervix end of the tampon confirmed a patent fistula tract. She underwent laparotomy where the bladder was bivalved to separate it from the anterior aspect of the uterus and cervix, the fistulous tract was excised, and the bladder repaired. An omental ‘tongue’ was place as interposition between the uterus and the bladder. Her post-operative recovery was uneventful and she was discharged home. On review 6 weeks post-operatively, she had made a full recovery with no further urinary leakage. Results and Conclusions VUF is an unusual complication of LCSC, and presentation is very variable, from frank urinary leakage to vaginal discharge. What makes it unique is a competent cervix, which closes and allows urine to be trapped in to the uterus. This creates the variability of the intensity of discharge - if at all and other non-specific presentation as previously alluded to. A high index of suspicion is therefore required to make this diagnosis, as cystograms may not necessarily fill the uterus, especially if already involuted. For the same reasons, VUF may present early following caesarean section or as a delayed presentation several years later. Patients may not necessarily present with urinary leakage. Take home message A double ring overlap should be looked for, in cystograms, and lateral views must be obtained. A methylene blue test can be helpful if carefully conducted. A pelvic ‘scopy’ – cystoscopy, hysteroscopy and colposcopy are part of required armamentarium in making a diagnosis. CT and MRI scans could also be employed. Once a diagnosis is made, repairs of VUF is similar to VVF.

After Cesarean vesicouterine fistula (Youssef Syndrome) with successful conservative management

Vesicouterine fistula without vaginal leakage of urine, cyclic hematuria and amenorrhea is a rare condition, identified as Youssef’s syndrome. The most common cause of this syndrome is trauma during a cesarean section. Different therapeutical approaches that include conservative treatment, fulguration, hormonal therapy and open, laparoscopic or robotic surgeries have been mentioned in the literature. Herein a case in a 37-year-old patient is reported. Conservative treatment with urinary drainage was successful because the fistula was recognized in the immediate postoperative period.

Post-caesarean vesicouterine fistula: Youssef's syndrome – A case report

Vesicouterine fistula (VUF) with cyclical hematuria, amenorrhea, and urinary continence is a rare condition referred to as Youssef's syndrome. It is most commonly observed after caesarean section (C/S) due to iatrogenic reasons. Open surgical management (transabdominal) has good results as it involves excision of the tract. We report a case of a VUF which developed after a repeat C/S and was successfully repaired with interposed vesico-peritoneal fold. Strict adherence to obstetric and surgical principles during Caesarean section can prevent formation of these fistulae.

Laparoscopic Repair of Vesicouterine Fistula: A Case Report and Mini-Review of the Literature

Vesicouterine fistula (VUF) is the rarest form of genitourinary fistulas. Despite the advantages of laparoscopy, there are few case reports showing its feasibility in the management of this rare entity. A 40-year-old woman presented to our department with urinary incontinence associated to cyclic hematuria and amenorrhea. After diagnosis of cervicovesical fistula, the laparoscopic approach was chosen. Catheterization of the fistula tract during cystoscopy at the time of laparoscopy was beneficial to localize the fistula tract and allowed meticulous dissection in the retrovesical space between the bladder and the uterus. Operating time was approximately 165 min. The woman had no signs of recurrence after 12 months of follow-up. Laparoscopic VUF repair is an effective and safe technique with successful outcome. J Curr Surg. 2016;6(3-4):75-77 doi: https://doi.org/10.14740/jcs309w