Urethral keratosis caused by vitamin A deficiency: two case reports.

BACKGROUND: Dysuria in children can have various etiologies, including ureteric stones, phimosis, congenital obstructive posterior urethral membrane, and neurogenic bladders. However, there have been no reports of vitamin A deficiency (VAD) causing dysuria due to urethral keratosis. Here, we report two cases of urethral keratosis caused by a VAD. CASE PRESENTATION: An 8-year-old boy (Patient 1) and a 6-year-old boy (Patient 2) presented with multiple episodes of dysuria and epididymitis. Both patients had intellectual disabilities and autism, which prevented voiding cystourethrography. Therefore, the patient was admitted for cystoscopy under general anesthesia. Cystoscopy revealed urethral diastolic dysfunction and a large amount of desquamated epithelium obstructing the urethra in both patients, causing urinary obstruction and dysuria. Catheterization was repeated; however, the symptoms recurred after catheter removal. Although the cause of recurrent urinary obstruction could not be initially identified, an ophthalmologist found Bitot's spots and suggested the possibility of a VAD. Serum vitamin A levels were extremely low in both patients, leading to the diagnosis of urethral keratosis due to VAD. VAD can be attributed to an unbalanced diet resulting from intellectual disabilities or autism. Vitamin A replacement therapy improved both the urethral symptoms and cystoscopic findings. CONCLUSION: Dysuria due to VAD is extremely rare, and urethral keratosis as a cause of dysuria is likely the first report of its kind worldwide. VAD may develop due to an unbalanced diet in patients with intellectual disabilities or autism. Therefore, it is essential to consider VAD as a potential cause of dysuria in patients with intellectual disabilities and autism.

Urethral diverticulum in pregnancy: Rare case report and brief literature review.

OBJECTIVE: Female urethral diverticulum (UD), an evagination of the urethral mucosa into the surrounding connective tissue, is extremely rare in pregnancy. No clear guidelines on the optimal management of UD have been established, except for a common conservative approach. Here, we discuss how to manage UD with pregnancy. CASE REPORT: A 39-year-old gravida 4, para 0, abortion 3 (G4P0A3) woman at 34+0 gestational weeks (GW) visited our outpatient department with a 6-cm septate vaginal mass. Transvaginal ultrasound sonography (TVUS) revealed a 5.5 x 4.9-cm multicystic mass, which was confirmed as UD with pelvic MRI. She was admitted because of preterm labor. A cesarean section was performed at 36+5 GW due to a previous myomectomy, and a healthy male baby was born. UD was still observed in the patient two months after delivery. Periurethral diverticulectomy was performed, and pathological analysis revealed UD with chronic inflammation and edema. CONCLUSION: Previous reports and our case report show that UD can develop during pregnancy and that pelvic MRI is suitable for its accurate diagnosis. Vaginal delivery is possible in pregnant women with the small size of the UD. UD aspiration can permit vaginal delivery in a few cases; however, pus can occur at the aspirated site after the operation. If UD is still observed after delivery, urethral diverticulectomy is recommended.

Current practice of diagnosis and treatment for rectourethral fistula in male patients with anorectal malformation: a multicenter questionnaire survey in Japan.

PURPOSE: Surgical procedures for anorectoplasty for anorectal malformations (ARMs), particularly rectourethral fistula (RUF), depend on the institution. We investigated the diagnosis and treatment of RUF in male patients with ARMs in Japan using a questionnaire survey. METHODS: An online survey inquiring about the diagnosis and treatment (diagnostic modalities, surgical approaches, fistula dissection devices, and fistula closure techniques) of each type of ARM in male patients was conducted among institutional members of the Japanese Study Group of Anorectal Anomalies. Fisher's exact test was used to compare surgical methods between posterior sagittal anorectoplasty (PSARP) and laparoscopy-assisted anorectoplasty (LAARP). RESULTS: Sixty-one institutions (100%) completed the survey. LAARP was the preferred approach for high-type ARM (75.4%). PSARP was preferred for intermediate-type ARM (59.0%). Monopolar devices were most commonly used (72.1%) for RUF dissection. Blunt dissection was more frequent in the PSARP group (PSARP vs. LAARP: 55.6 vs. 20.0%, p < 0.005). Cystoscopy/urethroscopy to confirm the extent of dissection was used more frequently in the LAARP group (70.0% vs. 25.0%, p < 0.005). Clips and staplers were used more frequently in the LAARP group (p < 0.05). CONCLUSION: Distinct fistula management strategies for PSARP and LAARP were revealed. Further studies are needed to investigate the postoperative outcomes associated with these practices.

Evaluation and management of urethral and periurethral masses in women.

PURPOSE OF REVIEW: Female periurethral masses are an uncommon occurrence. The purpose of this review is to describe etiologies of female urethral and periurethral masses and to provide an update on diagnosis and management. RECENT FINDINGS: The most common causes of periurethral and urethral masses in women are urethral caruncles, urethral diverticula, and Skene's gland cysts. Urethral meatal lesions such as urethral caruncles and prolapse can be managed conservatively with topical estrogen therapy and close follow-up or should be excised in the setting of thrombosis, significant or recurrent bleeding, acute urinary retention, or persistent pain. Benign periurethral gland masses, such as Skene's gland cysts, Gartner's duct cysts, and Mullerian duct cysts, remain rare. Recent case series reveal a high rate of surgical management of these lesions with few complications. Urethral malignancy or malignant transformation of benign etiologies are even rarer but can be aggressive in nature and should be treated promptly. SUMMARY: Nonspecific urinary and vaginal symptoms as well as similar physical presentations make diagnosis of urethral and periurethral lesions in females difficult. Magnetic resonance imaging is useful for differentiation of periurethral masses. The decision for conservative or surgical management is typically guided by patient symptom bother, as well as concern for urethral malignancy.

Urethrorectal fistula ligation and transection in a dog with a history of unilateral abdominal cryptorchidism and misdiagnosed anal gland disease.

A 10-month-old unilaterally cryptorchid male labradoodle dog was presented to a specialty surgery referral center with a reported history of persistent anal sac leakage that was unresponsive to conservative management. The dog underwent castration and left anal sacculectomy but was witnessed urinating from the anus postoperatively. A contrast urethrogram was performed, and a urethrorectal fistula located between the distal portion of the pelvic urethra and the caudal aspect of the rectum was diagnosed. One week later, the dog underwent ligation and transection of the fistula. Surgery was successful and the dog recovered unremarkably with complete resolution of clinical signs. A contrast urethrogram 1 mo after surgery confirmed the successful ligation and transection of the fistula. Key clinical message: To the authors' knowledge, this is the first clinical report of urethrorectal fistula ligation and transection in a dog with unilateral abdominal cryptorchidism and misdiagnosed anal sac disease. Our findings underscore the importance of a thorough physical examination, history taking, and clinical workup for appropriate diagnosis and treatment of multiple congenital abnormalities.

Ligature et transection de la fistule urétrorectale chez un chien ayant des antécédents de cryptorchidie abdominale unilatérale et de maladie des glandes anales mal diagnostiquée. Un chien Labradoodle mâle unilatéralement cryptorchide âgé de 10 mois a été présenté à un centre de référence en chirurgie spécialisée avec des antécédents signalés de fuite persistante du sac anal qui ne répondait pas à une prise en charge conservatrice. Le chien a subi une castration et une sacculectomie anale gauche, mais a été vu en train d'uriner par l'anus après l'opération. Un urétrogramme de contraste a été réalisé et une fistule urétrorectale située entre la partie distale de l'urètre pelvien et la face caudale du rectum a été diagnostiquée. Une semaine plus tard, le chien a subi une ligature et une section de la fistule. La chirurgie a été un succès et le chien s'est rétabli sans particularité avec une résolution complète des signes cliniques. Un urétrogramme de contraste 1 mois après la chirurgie a confirmé la réussite de la ligature et de la section de la fistule.Message clinique clé :À la connaissance des auteurs, il s'agit du premier rapport clinique de ligature et de transection de fistule urétrorectale chez un chien atteint de cryptorchidie abdominale unilatérale et d'une maladie du sac anal mal diagnostiquée. Nos résultats soulignent l'importance d'un examen physique approfondi, d'une anamnèse et d'un bilan clinique pour un diagnostic et un traitement appropriés des anomalies congénitales multiples.(Traduit par Dr Serge Messier).

Urethral Diverticulum Marsupialization With Modified Spence-Duckett Procedure.

OBJECTIVE: To demonstrate a modified approach to the Spence-Duckett procedure for treatment of a distal urethral diverticulum. A urethral diverticulum is an outpouching of urethral mucosa occurring in 2-5% of the population.1 They are thought to commonly arise due to chronic inflammation or infection of the peri-urethral glands.2,3 MATERIALS AND METHODS: We present a 37-year-old female with vaginal bulge, dyspareunia, and dysuria. On examination, she had a 2-centimeter tender mass abutting the distal urethra. Imaging such as ultrasound or magnetic resonance imaging is critical to map the location of the diverticula along the urethra and extent of urethral involvement as it can inform surgical technique. Diverticula are typically located postero-laterally at the mid- or distal urethra; however, they can be found at any location along the urethra.2,3 Care must be taken to avoid disruption of the continence mechanism at the mid-urethra to prevent incontinence after surgery. Magnetic resonance imaging revealed a 1.7 × 1.7 × 1.8 centimeter unilocular cystic structure at the left posteromedial distal urethra consistent with a urethral diverticulum. The patient desired surgical management. RESULTS: Spence and Duckett traditionally described insertion of one blade of the Metzenbaum scissors in the urethra with incision into the diverticulum and anterior vaginal wall followed by marsupialization.4 Given the small size of the diverticular ostium identified, we opted to make an incision using a scalpel from the ostium down the posterior aspect of the urethra and proximally to the anterior vaginal wall. We then excised the diverticular sac prior to marsupialization. At 6 weeks after surgery, she had full resolution of her symptoms without development of urinary incontinence. Pathologic examination is important because while rare, cancers can originate from urethral diverticula, with a prevalence of 6-9%.5 Pathology was consistent with urethral diverticulum and negative for dysplasia. CONCLUSION: While effective, the Spence-Duckett technique is described as a "generous meatotomy" with risks of urethral shortening. Our modified approach reduces these risks, resolves bothersome symptomatology, improves cosmesis, and minimizes risk of anatomic or functional urethral compromise.

Congenital anterior urethral valves and diverticula.

AIMS OF STUDY: Anterior urethral valves (AUVs) are rare congenital anomalies causing lower urinary tract obstruction in children. The present study highlights the clinical approach in identifying the condition and its treatment options. METHOD: Single centre, retrospective study of children. RESULT: Over a 16 year duration, 14 children were diagnosed with AUVs and diverticula with a mean age of 15 months. Clinical features included straining at micturation and a palpable penile swelling. Diagnosis was confirmed with voiding cystourethrogram and cystoscopy. An open surgical procedure was performed in 12 cases and endoscopic intervention done in one. On follow up 13 patients showed restoration of a normal voiding stream and normal renal function. One child died of chronic renal failure due to a delayed presentation, severe back-pressure changes, urinary ascites, and urosepsis. One patient developed a small urethrocutaneous fistula that healed spontaneously. CONCLUSION: AUV, though rare, should be recognizable due to the combination of a poor urinary stream and visible swelling in the ventral aspect of penis. Open surgical excision is the method of choice, and the outcome is excellent. LEVEL OF EVIDENCE: Level IV.

Congenital Anterior Urethral Diverticulum in Identical Twins.

Congenital anterior urethral diverticulum (CAUD) is an uncommon abnormality of the male urethra. In the literature, cases of CAUD affecting both children within a set of identical twins or presenting concomitantly with another urethral condition are exceedingly rare. We describe 2 cases of CAUD in identical twins: a pair of newborns in which Twin 1A and Twin 1B both present with CAUD, and a second pair of newborns in which only Twin 2A presents with CAUD along with a partial collateral urethral duplication. In doing so, we aim to add to the incomplete literature on the embryological development of CAUD.

[Thirty-nine cases of suburethral diverticula in women: 30 years single center experience]. / A propos de 39 cas de diverticules sous-urétraux de la femme: expérience monocentrique sur 30 ans.

We here report a retrospective analysis of 30 years´ experience with 39 female patients suffering from suburethral diverticula. The average age of patients was 37 years of age (24-56 years). The average parity was 2 (1-7); 65% of deliveries were complicated by dystocia, with forceps used in 43% of cases. All patients had a history of urological or gynaecological infections. The revealing symptoms were heterogeneous but recurrent urinary tract infections (26 cases), pollakiuria (23 cases), post-micturition urethral discharge (21 cases), vaginal pain (17 cases) and a sensation of vaginal bulge (15 cases) were mostly reported. Radiological assessment were performed, including intravenous urography, retrograde and micturition urethrography, ultrasound, or MRI. Transvaginal diverticulectomy was the treatment of choice for all patients, with no reported intraoperative complications. At 4 years of follow up outcome was satisfactory. Four patients developed recurrence of diverticulitis. These data provide important information about clinical features, diagnostic results and long-term outcomes of transvaginal diverticulectomy, enabling better management of this rare condition.

A urethral diverticulum presenting with pure stress urinary incontinence.

OBJECTIVE: To report the management for a urethral diverticulum presenting with pure stress urinary incontinence (SUI). CASE REPORT: A 67-year-old postmenopausal woman resorted to urogynecological outpatient department for the treatment of bothersome SUI. She denied other lower urinary tract symptoms and previous pelvic surgeries. On examination, there was stage I anterior vaginal wall prolapse. Urinalysis showed negative findings. Urodynamic studies revealed negative findings. An ultrasound disclosed a complex paraurethral lesion and no urethral hypermobility. A magnetic resonance image of the pelvis revealed a 4-cm circumferential urethral diverticulum. A urethral diverticulectomy was performed. Histopathological examination confirmed the diagnosis of urethral diverticulum. The patient recovered uneventfully and reported freedom from SUI postoperatively. CONCLUSION: In women deemed uncomplicated stress urinary incontinence after undertaking a holistic urogynecological evaluation including detailed clinical history, physical examination, and urodynamic studies, further image studies investigating lower urinary tract is required for disclosing other rare conditions that necessitate different management from anti-incontinence surgery.

Traumatic Rectovesicular Fistula in a Dog.

A 7 mo old intact male Australian cattle dog presented for evaluation of a suspected urethrorectal fistula after being examined by the primary veterinarian for leaking of urine from the rectum at rest and when posturing to urinate. Advanced imaging identified a caudally retroflexed urinary bladder, several healing pelvic fractures, and a rectovesicular fistula. Primary surgical repair of the fistula was performed. The dog recovered well from surgery and was urinating and defecating normally as of 5 mo after the operation. This is the first report of a traumatic rectovesicular fistula in the veterinary literature.

Congenital anterior urethral valves and diverticulum: A case series and association with posterior urethral valves.

INTRODUCTION: There is limited data regarding long-term results and associated complications in patients with anterior urethral valve (AUV) and diverticulum (AUD). We retrospectively reviewed AUV/AUD cases managed by us between the year 2002-2020. MATERIAL AND METHODS: Presentation, investigations, management, concomitant posterior urethral valves (PUV) and pre-operative characteristics predisposing to long-term poor renal outcome were assessed. RESULTS: There were 27 patients [AUV (n = 11); AUD (n = 16)] with 5 having concomitant PUV. All presented with poor urinary stream and dribbling at a median age of 1-year (5 days-12 years). More patients with AUD (9 of 16, 56.3%) especially with concomitant PUV presented at ≤1-year-age as compared to those with AUV (4 of 11, 36.4%). Concomitant PUV and AUD cases (n = 3) had characteristic micturating cystourethrography (MCUG) features. (Fig. 1A) Retrograde urethrography delineated the valve and distal urethra dimensions better (Fig. 1B, C). Urethral hypoplasia distal to the valve (n = 4) urethral duplication (n = 1) were associated (Fig. 1D). Syringocele was ruled out in proximal AUD by absence of filling defect on MCUG and appearance of urethral walls on urethrocystoscopy. Serum creatinine > 1 mg/dL (n = 5), trabeculated bladder (n = 12), and vesicoureteral reflux (VUR) (n = 12) was noted at presentation. Diverticulum/valve excision (n = 13, 48.2%), fulguration alone (n = 12, 44.4%) and primary urinary diversion (n = 2, 7.4%) were performed. Follow up (range:3 mo-19 years), showed deranged renal function tests (n = 6), VUR (n = 4), impaired renal function on scans (n = 8), and lower urinary tract dysfunction (n = 7). Outcome with and without associated PUV was similar. Long term results were better in AUV compared to AUD. Pre-operative raised serum creatinine (>1 mg/dL), trabeculated bladder, non-dilated posterior urethra on MCUG and bilateral impaired renal function on scans had significant association with follow up eGFR less than 60 ml/kg/min. CONCLUSIONS: Co-existing PUV and AUD present earlier and have specific imaging findings. In proximal AUD, possibility of syringocele should be kept in mind, as they have similar presentation and imaging. Concomitant PUV did not alter prognosis. Secondary effects on bladder and renal function were more with AUD. Follow up eGFR less than 60 ml/kg/min was associated with pre-operative elevated serum creatinine, trabeculated bladder, non-dilated posterior urethra, and bilateral impaired renal function on scans.

Urethral Pathology in Women.

PURPOSE OF REVIEW: This paper describes the differences in benign, malignant, and iatrogenic urethral pathology in women and reviews common presenting symptoms and management strategies. RECENT FINDINGS: The advancement of magnetic resonance imaging has led to MRI becoming the imaging modality of choice for urethral pathology. Urethral pathology is rare, and there remains a paucity of evidence-based literature for management. Urologists and gynecologists must be familiar with common benign urethral pathology, the most common of which includes urethral diverticula, Skene's gland cyst, urethral prolapse, and caruncle. Further case series are providing further insights to the management of these rare entities. Urologists and gynecologists must understand the most common urethral pathologies and be able to identify the most appropriate treatment approach. It is essential to be able to differentiate these entities from malignancy and obtain a thorough surgical history to identify possible iatrogenic causes.

Characterization of urethral diverticula in males.

INTRODUCTION: Urethral diverticulum in a male is a rare entity and the literature is limited to case reports and small case series. The aim of our study is to characterize this disease in patients from three Mayo Clinic locations. MATERIALS AND METHODS: Chart analysis was performed of patients across all three Mayo Clinic sites that had International Classification of Diseases (ICD) codes corresponding to urethral diverticulum or urethral diverticulectomy via CPT code. Data were available for patients that were seen from 6/1/2003 through 10/5/2018. Patients were classified by age, etiology, presenting symptomatology, location, treatment, pathology, and postoperative outcomes. RESULTS: A total of 87 men met the initial search criteria with 52 having documented urethral diverticula. The most common presenting complaint in these men was incontinence (37%). The majority of diverticula (83%) were within the anterior urethra. The most common diagnostic modalities were retrograde urethrogram (46%) and cystoscopy (50%). Most diverticula were iatrogenic (77%). Of the men that were diagnosed, 42% went on to have diverticulectomy. Median follow up was 1.5 years. Eighteen percent of patients had persistent urinary symptoms following diverticulectomy with incontinence being the most common finding. Postoperative complications were experienced by 26% patients with the most prevalent complication being urethrocutaneous fistula. The patients who did not undergo diverticulectomy either had other surgical procedure to manage their coexisting conditions or were managed medically. CONCLUSION: Urethral diverticulum in males is a rare yet important entity that requires special consideration, especially in those who have had prior surgery within the lower urinary tract.

The ultrasonographic characteristics of female periurethral solid masses.

INTRODUCTION AND HYPOTHESIS: Studies on the imaging of female periurethral masses are sparse, and most are focused on cystic lesions. In this article, we studied female periurethral solid masses and reported their ultrasonographic features. METHODS: Fifteen women with periurethral solid masses pathologically diagnosed between January 2008 and April 2021 were assessed. RESULTS: Each patient had only one mass. The pathological types included urethral caruncle (5 patients), urethral leiomyoma (3 patients), urethral malignant tumor (MT) (3 patients), periurethral spindle tumor (3 patients) and cartilage necrosis of pubic symphysis (PS) (1 patient). On ultrasound, all urethral caruncles were located at the urethral meatus. They were hypoechoic/isoechoic and rich in blood flow signal. Each leiomyoma presented as a well-defined hypoechoic mass with an oval shape. The urethral MT had inhomogeneous/isoechoic echoes, with medium to abundant blood flow signal. The spindle cell tumors had regular/irregular shapes, moderate/high density echogenicity and little/rich blood flow signals. The articular cartilage necrosis of PS was regular in shape, with mixed echogenicity and no blood flow. CONCLUSIONS: Ultrasound imaging is a convenient and useful method to evaluate the morphological characteristics of female periurethral solid masses.