Absence Of Bladder Research Articles

Long-term outcomes in sacral agenesis.

Sacral agenesis (SA) includes a range of clinical presentations of varying severity, with implications for function and quality of life (QoL). Diagnosis is often made perinatally, and prognostic discussions become an important aspect of parental counselling. This study engaged SA sufferers and their caregivers to obtain objective, long-term patient reported outcome data. Patients with radiologically confirmed SA from a single tertiary spinal unit underwent retrospective medical record review. Patients were then contacted by telephone to complete QoL questionnaires including EQ-ED-5L for adults and EQ-ED-Y for < 16-year-olds. Additional information including Renshaw grade, employment, living situation and bladder function was also collected. Twenty-six patients with SA were identified. Mean age is 23.35years (range 0.92-63.53), 13M:17F. Renshaw grade ranged from 1 to 4. Sixty-eight percent had associated kyphoscoliotic deformities. The majority (70%) had either impaired or absent bladder control, and 80% need walking aids to mobilise. Twenty patients completed the questionnaire (10 adults and 10 < 16-year-olds). Mean EQ-ED-5L index for adults was +0.474 (range -0.1 to +0.089, 1 = best), with a lower mean value of +0.287 (range -0.54 to +1) for the < 16-year cohort. Those undergoing spinal fusion procedures had significantly lower scores (-0.08 v +0.44, p = 0.022). This study provides an objective record of the QoL of individuals with SA, illustrating a wide variety of outcomes, with differences between younger and older individuals which may reflect the results of a long-term adaptive process. The implications for individuals should be carefully tailored to the specific deformity and the likely underlying neurological deficits.

Optimizing prenatal diagnosis and referral of classic bladder exstrophy: Lessons from a single-institution experience

Classic bladder exstrophy (CBE) is a malformation of the genitourinary system that occurs due to failure of abdominal wall closure. Unlike other malformations of similar incidence, prenatal diagnosis of CBE relies on suggested, rather than formal, diagnostic criteria. This report describes prenatal diagnosis of CBE in the largest single-institutional cohort to date and delineates key sonographic findings and protocols for specialist referral. A single-institutional database was reviewed for CBE patients born since 2000. Data on screening ultrasound use, gestational age at ultrasound, and abnormal findings were extracted. Where possible, time of prenatal diagnosis (pre- or postnatal and gestational age), ultrasound findings and other imaging data, specialist referral, institution of birth and closure, and outcome of primary closure attempt were compared. Of 557 patients born with CBE between 2000 and 2022, 284 met inclusion criteria and complete data were available for 280 (229 born domestically and 51 born internationally) who were included for analysis. Abnormal sonography suggestive of CBE was present for 48% (n=134) of patients, for whom absent bladder was the most common abnormal finding (76% [102/134]). Of domestic patients, 46% (n=106) were diagnosed prenatally at a median gestational age of 22 weeks (inter-quartile range [IQR]: 20-24), and 14% (n=32) underwent confirmatory fetal magnetic resonance imaging. Of domestic patients with abnormal prenatal findings, 75% (n=80/106) consulted with maternal-fetal medicine and 58% (n=62/106) consulted with pediatric urology. On univariate analysis, prenatal diagnosis was positively associated with primary repair at Association for the Bladder Exstrophy Community-recognized centers of excellence (54% vs. 38%, p=0.02) and negatively associated with osteotomy at primary closure (41% vs 59%, p=0.003) but not success of primary closure (74% vs. 82%, p=0.07). Rates of prenatal diagnosis in this cohort were similar to previous reports of smaller cohorts. Diagnosis allows for comprehensive pre- and postnatal follow-up with a pediatric urologist, with implications on birth planning and decisions on termination of pregnancy. Because of the previously-reported association between exstrophy and invitro fertilization, these pregnancies should undergo detailed sonography. Any nonvisualization of the fetal bladder should prompt a detailed exam, and any finding characteristic of bladder exstrophy warrants referral to pediatric urology. Although CBE is a rare disorder, it is underdiagnosed during pregnancy. Sonographers and obstetricians should be aware of characteristic findings and best practices following diagnosis. Early referral to pediatric urology and maternal-fetal medicine is important for counseling and postnatal planning.

Novel variant of TMEM231 in Meckel syndrome: a case report

A 34-year-old woman underwent routine antenatal ultrasound scanning at 17 weeks gestation which identified multiple fetal abnormalities including severe oligohydramnios, growth restriction, occipital encephalocele, short and bowed long bones, bilateral enlarged multicystic kidneys and an absent bladder. As the findings were highly suspicious for a lethal fetal ciliopathy, the patient received counselling and elected for a medical termination of pregnancy at 19 weeks. Autopsy confirmed the imaging findings, additionally demonstrating low set ears, micrognathia, a broad nasal bridge, polysyndactyly and bilateral hypoplastic lungs.

MRI as an aid to ultrasound in detecting bladder or parametrial invasion in accreta spectrum

Ultrasonography (US) is used for screening and diagnosis of placenta accreta spectrum (PAS) . Magnetic resonance imaging (MRI) has become an aid in confirmation. Our objective was to determine whether there is utility in MRI to detect findings such as parametrial invasion and bladder invasion in women with suspected PAS by ultrasound. We performed a retrospective cohort study of 62 people who underwent a hysterectomy for the management of PAS in a tertiary hospital system. MRI was used only after PAS was suspected by ultrasound. US, MRI, operative and pathology reports were reviewed for the presence or absence of bladder and/or parametrial invasion. Parametrial invasion was defined as extension of the placenta into the lateral pelvic side walls. Data were analyzed, with the reference gold standard being operative findings, using ROC area under the curve, sensitivity, specificity, positive predictive value and negative predictive value. From the study population, 8 people had suspected bladder invasion by US and none with suspected parametrial invasion. Whereas MRI suspected bladder invasion in 12 people and 8 had suspected parametrial invasion. At the time of surgery, bladder involvement was identified in 8 people and parametrial invasion was identified in 2 people. The performance of both MRI and US were similar for bladder involvement with a fair ROC-AUC (Table 1). The imaging modalities together had similar sensitivities except that of US in detecting parametrial involvement. The performance of MRI was better than US at identification for parametrial involvement due to the difficulty in identifying parametrial involvement on US alone. Table 2 compares the agreement of MRI vs. US on bladder and parametrial invasion. In a tertiary care center, among those with suspected PAS by US, the overall addition of MRI to detect the severity, as well as bladder invasion and/or parametrial invasion, has a low detection rate with a high false positive rate.View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Distal Ureteric Dilatations Functioning as Urinary Reservoir in a Case of Ectopic Vesicae; A Case Report

Background: Bladder exstrophy is a rare congenital malformation of the genitourinary system, with an estimated incidence of approximately 1 per 50,000 live births. Clinically, patients do not have capacity to accumulate urine and urine continously leak. We present patient with partial storing capacity from the dialated distal ureters. A case of dialated distal ureters from an 8-year-old female patient with ectopic vesicae is described. The dialated ureters act as reservoir of urine where the patient is partially continent in the night time. These dialated ureters are the compensation for the literally absent bladder. During reconstruction, we observed that they can be used as an additional bladder volume reducing risk of reconstruction failure from tension.

Treatment outcomes of detrusor underactivity in women based on clinical and videourodynamic characteristics.

Treatment of voiding dysfunction due to detrusor underactivity (DU) remains challenging. This study sought to determine the characteristics of video urodynamic study (VUDS) in female DU and outcomes after active treatment. A total of 409 female patients with VUDS-proven DU were recruited. All patients received conservative bladder management, with others receiving active treatment, such as oral medication, transurethral incision of bladder neck (TUI-BN), surgery for prolapse, and urethral botulinum toxin A (BoNT-A) injection, according to their VUDS characteristics. Treatment outcomes were then analyzed by different VUDS characteristics. Satisfactory treatment outcomes were noted in 123 patients (30.0%), among whom 35 and 88 had a VE of > 90% and > 66.7%, respectively. A total of 165 patients (40.3%) had voiding efficiency (VE) of 33.3-66.7%, whereas 121 (29.6%) remained unable to void (12.5%) or had a VE of < 33.3% (17.1%). Conservative treatment had the worst outcome. Patients with normal or slightly reduced bladder sensation (49.3%) and those with a post-void residual (PVR) of < 250mL (59.4%) displayed better satisfactory outcome after treatment; while TUI-BN and urethral BoNT-A injection promoted better treatment results. Patients with detrusor acontractility and a PVR of ≥ 500mL still achieved high VE rates (> 66.7%) after TUI-BN. A 56.3% satisfactory outcome was achieved after TUI-BN, whereas 58.1% satisfactory outcome was noted in those with a tight external sphincter after medical treatment. Female patients with DU exhibited VE improvement after active treatment. Patients with very low detrusor contractility and absent bladder sensation generally exhibited poor treatment outcomes.

Natterin-like depletion by CRISPR/Cas9 impairs zebrafish (Danio rerio) embryonic development

BackgroundThe Natterin protein family was first discovered in the venom of the medically significant fish Thalassophryne nattereri, and over the last decade natterin-like genes have been identified in various organisms, notably performing immune-related functions. Previous findings support natterin-like genes as effector defense molecules able to activate multiprotein complexes driving the host innate immune response, notably due to the pore-forming function of the aerolysin superfamily members. Herein, employing a combination of the CRISPR/Cas9 depletion system, phenotype-based screening, and morphometric methods, we evaluated the role of one family member, LOC795232, in the embryonic development of zebrafish since it might be implicated in multiple roles and characterization of the null mutant is central for analysis of gene activity.ResultsMultiple sequence alignment revealed that the candidate natterin-like has the highest similarity to zebrafish aep1, a putative and better characterized fish-specific defense molecule from the same family. Compared to other species, zebrafish have many natterin-like copies. Whole-mount in situ hybridization confirmed the knockout and mutant embryos exhibited epiboly delay, growth retardation, yolk sac and heart edema, absent or diminished swim bladder, spinal defects, small eyes and head, heart dysfunction, and behavioral impairment. As previously demonstrated, ribonucleoproteins composed of Cas9 and duplex guide RNAs are effective at inducing mutations in the F0 zebrafish.ConclusionsThe considerably high natterin-like copies in zebrafish compared to other species might be due to the teleost-specific whole genome duplication and followed by subfunctionalization or neofunctionalization. In the present work, we described some of the natterin-like features in the zebrafish development and infer that natterin-like proteins potentially contribute to the embryonary development and immune response.

Therapeutic outcome of active management in male patients with detrusor underactivity based on clinical diagnosis and videourodynamic classification

Detrusor underactivity (DU) could be resulted from many different etiologies. Patients with DU might have reduced bladder sensation, low detrusor contractility, and large post-void residual volume. This study analyzed therapeutic outcome of active management for male DU patients, based on clinical and urodynamic characteristics. Male DU patients aged > 18 years old were retrospectively reviewed from the videourodynamic study (VUDS) records in recent 10 years. The patients’ demographics, VUDS results, treatment modalities, and treatment outcome were analyzed. The treatment outcomes were compared among patients with different DU subgroups, clinical diagnosis and treatment modalities. Patients with voiding efficiency of > 66.7% were considered having a successful treatment outcome. For comparison, 30 men with normal VUDS finding served as the control arm. Most of the DU patients had reduced bladder sensation. The reduced bladder sensation is closely associated with low detrusor contractility. After active treatment, a successful outcome was achieved in 68.4% of patients after bladder outlet surgery, 59.1% after urethral botulinum toxin A injection, and 57.6% after medical treatment, but only 18.2% after conservative treatment. A successful treatment outcome was achieved in patients with an intact detrusor contractility, either low (69.2%) or normal voiding pressure (81.8%), and in patients with a normal or increased bladder sensation (78.1%). However, patients with detrusor acontractile (41.3%) or absent bladder sensation (17.9%) had less favorable treatment outcome after any kind of urological management. This study revealed that active management can effectively improve voiding efficiency in patients with DU. The normal bladder sensation, presence of adequate detrusor contractility, and bladder outlet narrowing during VUDS provide effective treatment strategy for DU patients. Among all management, BOO surgery provides the best treatment outcome.

Evaluation of PSMA-PET Biology-Guided Radiotherapy Sequential Boost to the PSMA-avid Subvolume in the Prostate Region in Low-Volume Advanced Prostate Cancer

Biology-guided radiation therapy (BgRT) is a new technology that aims to deliver radiation based on detecting positron emissions in real time. BgRT combined with prostate specific membrane antigen (PSMA) PET/CT imaging may be an attractive option for prostate cancer patients with low-volume pelvic nodes and distant metastases since all lesions may be treated during the same session. This study investigates the feasibility of BgRT sequential boost to the PSMA-avid subvolume (PAS) in the prostate region. The ratio of standard uptake value (SUV) to background SUV is quantified, with specific focus on adjacent bladder uptake.Patients enrolled in the ProPSMA prospective single-institution clinical trial underwent Ga-68-PSMA-11 PET scan at the time of prostate cancer diagnosis. Those with pelvic node or distant metastases were selected for analysis. PAS gross target volume (GTV) was delineated on the PET component of a PET/CT acquisition by using a PET edge tool in commercially available software. Three dimensional shells of thickness 5 mm, 10 mm and 20 mm were created by expanding the GTV to characterize the uptake background and to determine suitability of BgRT based on these tracking zones. Normalized SUV (nSUV) was calculated as the ratio of SUVmax in the GTV to SUVmean of the adjacent shell. Lesions with nSUV larger than 3 were defined to be suitable for BgRT. An increase in nSUV with increasing shell thickness indicates absence of uptake surrounding the GTV.From the ProPSMA patient cohort of 89 patients, 24 patients had at least one pelvic nodal or distant metastases. PAS GTV volume ranged from 1 cc to 147 cc (median = 25 cc, IQR = 12 cc - 62 cc). GTV SUVmax varied from 6.4 to 80 (median = 21.5; IQR = 14 - 33). nSUV ranged from 3.4 to 21.2 (median = 6.4; IQR = 4.9 - 8.5), from 4.1 to 20.9 (median = 8.6; IQR = 6 - 10.5), and from 2.8 to 31.4 (median = 8.2; IQR = 5.4 - 13.8) in 5 mm, 10 mm, and 20 mm shell, respectively. Moreover, 100%, 100%, and 96% of lesions had nSUV greater than 3 in a shell thickness of 5 mm, 10 mm, and 20 mm, respectively. nSUV increased from the 5 mm shell to the 10 mm shell in 75% of patients and from the 10 mm shell to the 20 mm in 46% patients, indicating the absence of bladder uptake.An nSUV greater than 3 was achieved in more than 95% of all GTVs for all shell thicknesses investigated in this study, which suggests that PSMA-guided BgRT is a feasible modality for sequential boost directed at the dPSMA-avid subvolume in the prostate region. If bladder proximity impacts geometric specificity of BgRT delivery or if greater tumor-to-background contrast is desired, additional refinements may be required. These may include modifications to the BgRT algorithm that enable masking of the bladder. Clinical trial number ACTRN12617000005358.

OEIS Syndrome: Omphalocele, Exstrophy of the Cloaca, Imperforate Anus, and Spinal Defects

OEIS Syndrome: Omphalocele, Exstrophy of the Cloaca, Imperforate Anus, and Spinal Defects

Prenatale echografische diagnose van een cloacale malformatie

Prenatal ultrasound diagnosis of a cloacal malformation Cloacal malformations are a spectrum of rare congenital disorders caused by the arrest of the cloacal division during different stages in early embryogenesis. They necessitate critical intervention at birth, posing important surgical challenges. The majority unfortunately remains undetected during prenatal screening. Knowledge about sonographic red flags, however, may improve antenatal detection and therefore postnatal outcome as a result of a timely referral to multidisciplinary tertiary centers. A 29-year-old patient presents at the hospital due to decreased fetal movements in the third trimester of pregnancy. The ultrasound reveals an anechoic bilobed cystic mass with a thick midline septum within the pelvis of a female fetus. The diagnosis of a cloacal anomaly with associated duplicated vaginas and uteri is made. Severe accumulation of urine lies at the base of bilateral hydrocolpos, peritoneal ascites and a urinary tract obstruction with an adverse influence on both pulmonary and renal development. The baby is prematurely delivered by an emergency caesarean section. Her condition is poor, requiring immediate respiratory resuscitation. An explorative laparotomy on the following day confirms the diagnosis. The first steps towards anatomical correction are taken. It may be concluded that the triad of bilateral hydronephrosis, a pelvic cystic mass and an absent normal bladder within a female fetus should always raise suspicion of a cloaca.

A huge bladder diverticulum in an elderly: A case report

An 81-year-old male patient presented to the department of gastroenterology with increasing lower abdominal pain for 2 years, aggravated with bloody stool for 1 month. Computed tomographic examination revealed a huge cyst (207 × 93 × 208 mm3) in the abdominal cavity, absence of bladder, thickening and strengthening of the rectal wall, and benign prostatic hyperplasia. Colonoscopy showed colon cancer and surgery was planned. Interestingly, after magnetic resonance imaging and cystography, we found colon cancer and a large bladder diverticulum rather than tumor metastasis or others. Severe bacteremia occurred in the elderly chronic obstructive pulmonary disease patient before operation. After careful consideration, we decided to take a large risk and combined urology and gastrointestinal surgery professionals to carry out bladder diverticulectomy, cystostomy, radical resection of rectal carcinoma, and so on. Fortunately, the patient recovered well after the operation. In addition to the common tumor metastasis and cystadenoma, the abdominal mass should also be alert to the rare bladder diverticulum, which eventually leads to diagnostic confusion. Multidisciplinary diagnosis and treatment has become an important treatment for complex diseases.

A rare case of congenital pyloric atresia with situs inversus abdominalis

Congenital pyloric atresia and situs inversus are rare conditions. The combination of congenital pyloric atresia and situs inversus is extremely rare, only two such cases reported in the literature so far. We present third such case of a term, male baby born to a primigravida mother. All antenatal ultrasound up to 31 weeks were unremarkable. Follow-up antenatal ultrasound was suggestive of duodenal atresia. The baby was noted to have respiratory distress at birth requiring respiratory support in the form of nasal continuous positive air pressure. X-ray abdomen revealed a single bubble sign, but toward the right side of the abdomen, cardiac shadow being on the left side, suggestive of congenital pyloric atresia along with situs inversus abdominalis. The baby was operated, laparotomy done, findings confirmed, and retrograde side-to-side gastroduodenostomy was done. Postoperatively, feeds were started and baby discharged on day 17 of life. Isolated congenital pyloric atresia has a good prognosis and all attempts should be made to identify other possible associations (since there are different anomalies associated with congenital pyloric atresia such as junctional epidermolysis bullosa, renal anomalies [hydroureteronephrosis, multicystic kidney, absent bladder], aplasia cutis congenita, and colonic atresia).

Extraordinary Bulging Mass in the Foetus - A Case Report of Bladder Exstrophy

Abstract INTRODUCTION: Prenatal diagnosis of bladder exstrophy is extremley rare and difficult. BACKGROUND: Due to abnormal development of the cloacal membrane there is an incomplete closure of the lower abdominal wall, absence of the anterior wall of the bladder and external exposition of the posterior wall. The pubic bones are usually separated, the umbilical cord low inserted and there is abnormal external genitalia development. CASE REPORT: At 21st week of gestation of 39-year-old multigravida multipara referred by a primary care obstetrician to high-specialised centre for a detailed ultrasound examination with a suspicion of bladder absence and inferior umbilical localisation. At 29 weeks of gestation presence of bulging mass of 2 cm, between the umbilical outlet and labia was detected. At 31 weeks of gestation previously detected structure among thighs had 3 cm diameter with lateral umbilical outlet. Major labia were prominent and minor labia were within normal limits. Between two umbilical arteries with an appropriate intraabdominal course there were no transsonic area corresponding to the urinary bladder. The newborn baby was born at term in a good condition, but with an exposed bladder of 4 cm in diameter. The urethral outlet was not visualised and the female genitals were abnormal. After a month the girl underwent primary bladder exstrophy closure. Although she suffers from recurring urinary tract infections, she is in a good general condition. CONCLUSIONS: Due to prenatal diagnostics it was possible to detect and make an initial diagnosis of severe malformation. Early diagnosis allowed to prepare parents for a newborn with a defect and teach them how to take care of the baby.

EP11.01: Prenatal ultrasound‐based diagnosis of fetal OEIS complex associated with lower limb polymelia and cardiac, hepatic dysplasia

Omphalocele exstrophy imperforate anus spinal defects (OEIS) complex is a type of congenital malformation syndrome involving multiple fetal systems. Its characteristic ultrasonographic features include omphalocele, spinal defects, absence of bladder, and ambiguous anus and genitalia, and it is often accompanied with limb malformations. Here, we report a case of fetal OEIS complex combined with lower limb polymelia and cardiac, hepatic dysplasia at the 24th gestational week diagnosed on prenatal ultrasonography. The ultrasonogram showed an omphalocele on the right side of the umbilical cord entry, spinal meningocele at the lumbosacral portion, bladder absence and ambiguous genitalia. Concurrent multiple malformations included lower extremity multi-limb deformity, feet abnormity, congenital ventricular septal defect, three-lobe liver malformation, ectopic kidneys, and single umbilical artery. Besides characteristic ultrasonographic features of OEIS complex, this case was combined with severe and rare lower limb polymelia and multi-lobe liver deformity. It was easily missed diagnosis when oligoamnios and the liver bulged. This case will strengthen our understanding of such rare deformities and provide reference information for early diagnosis of similar cases. Supporting information can be found in the online version of this abstract Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

EP12.13: A case of partial urorectal septum malformation sequence with a very small lower abdominal cyst and review of literature

Background: Partial urorectal septum malformation sequence is a rare congenital malformation of urorectal septum dysplasia and failure of the breakdown of th cloacal membrane, is characterised by abnormalities of the ambiguous genitalia, urogenital organs, a single perineal/anal opening that drains a common Cloaca, and absent anus. We report a case of partial urorectal septum malformation sequence, who was suspected on pregnancy ultrasound scan at 24+6 weeks' gestation in our department and confirmed upon delivery. Case presentation: A 19-year-old married woman, Gravida 1 Para 0, presented to antenatal clinic of Cheng Du Women and Children's Central Hospital, Si Chuan province, China, at 24+6 weeks' gestation by dates, when ultrasound examination was performed, showing a very small common cloaca in the lower abdomen, absence of bladder, ambiguous genitalia and imperforate anus. Both mother and clinicians agreed on termination of pregnancy. The mother spontaneously delivered a dead female fetus weighing 1085 grams at 25+4 weeks' gestation after amniotic cavity injection rivanol, who had multiple congenital anomalies including imperforate anus, smooth perineum, absence of normal urethral and vaginal openings, absent bladder, uterus, vagina, and urethra, in combination with a very small common cloaca opening in the perinea and myelomeningocele in the sacral vertebrae. Conclusion: The characteristic features of the URSMS in ultrasonography are relate to the type of the URSMS, not all URSMS along with very dilated cystic regions in the abdomen.

Male and female aphallia associated with severe urinary tract dysplasia

Aphallia is exceedingly rare (1/30 million births). Previous reports have provided limited detail on associated urinary tract findings. We reviewed urinary tract anomalies in two boys with aphallia (patients 1 and 2) and a girl with urinary tract dysplasia, a similar external appearance and lack of corporal tissue (patient 3), also consistent with aphallia. Patients 1 and 2 both had a 46XY karyotype, bilateral descended testes in well-formed scrotums, and posterior skin tags containing rudimentary urethras. Patient 1 had a focal area of urethral narrowing; a posterior bladder diverticulum, which drained a ureter; bilateral grade 5 vesicoureteral reflux, with a right partial renal duplication; and hydronephrosis of all moieties. Patient 2 had posterior urethral valves and a bladder diverticulum. Right ureterovesical junction obstruction required a tapered reimplant and later conversion to right-to-left transureteroureterostomy. Patient 3 had a 46XX karyotype and fused, well-formed labia majora. A posterior skin tag was associated with a stenotic urogenital sinus, beyond which were a vagina posteriorly and a right refluxing ureter anteriorly. The left ureter was absent, and a miniscule pouch represented a maldeveloped or absent bladder. Laparoscopy revealed ovaries and normal Müllerian structures. Bilateral renal dysplasia necessitated renal transplant and the creation of an ileocecal neobladder and Mitrofanoff channel. Corporal tissue was diminutive or absent in all. We see from these three patients that corporal tissue absence can occur in both male and female patients. We propose that the term aphallia can apply to both sexes, as it is the absence of corporal tissue that defines this condition. This is the only report to include and characterize findings in both male and female aphallia patients. Labioscrotal folds develop with a smooth appearance, and, posteriorly, a urethral orifice or Urogenital (UG) sinus with skin tag may be seen. Obstruction at the level of the urethra was common. Severe urinary tract dysplasia was seen in all, a finding not consistently seen or characterized in previous reports. In girls with severe urinary tract dysplasia and characteristic genital ambiguity, aphallia should be considered. Co-occurrence of aphallia and severe urinary tract dysplasia warrants further urinary tract imaging in all aphallia patients, including voiding cystourethrography, renal bladder ultrasound, and serum creatinine level. Urinary tract reconstruction may be performed without hampering future penile reconstruction, due to modern phallic reconstructive techniques.

Updates of underactive bladder: a review of the recent literature.

Underactive bladder (UAB) is a major component of lower urinary tract symptoms. The physiopathology mechanisms underlying UAB include detrusor underactivity, acontractile detrusor, and reduced or absent bladder sensation. UAB can influence lower and upper urinary tract function and lead to serious consequences; however, the symptom syndrome is poorly understood and incompletely researched at present. Moreover, there is no agreement among authorities regarding the terminology, definition, epidemiology, classification, risk factors, animal model, pathogenesis, diagnosis, and treatment of UAB. In this review, we discuss the current terminology, epidemiology, physiopathology, evaluation, and management of UAB.

An incidental finding of absent gall bladder in laparoscopic exploration - a rare occurrence in extra hepatic biliary anatomy

An incidental finding of absent gall bladder in laparoscopic exploration - a rare occurrence in extra hepatic biliary anatomy

Diagnostic and treatment of retrograde ejaculation as a manifestation of urogenital form of autonomic diabetic polyneuropathy

Background. Retrograde ejaculation in patients with type 1 diabetes mellitus is a complication of autonomic neuropathy that causes excretory infertility. It can be partial (reduction of ejaculate) or total (absence of ejaculate) and occurs in 10%–20% of men with type 1 diabetes mellitus.Aim. The aim of the study was to assess the effectiveness of a new endoscopic method for retrograde ejaculation correction and antegrade ejaculation restoration.Materials and methods. We included 30 patients with type 1 diabetes mellitus who had spermatozoa present in their post-orgasmic urine and ultrasonographic evidence of impaired or absent bladder neck closure. The mean age of participants was 32 (30–35) years, mean duration of diabetes was 17 (12–22) years and mean preoperative glycated haemoglobin level was 7.4% (6.9%–8.0%). All participants had total retrograde ejaculation. We used conventional irrigated urethrocystoscopy under local anaesthesia. During urethroscopy, bladder neck gaping was observed in all cases. Biocompatible material was injected at three points under the mucous layer of the posterior urethra, reaching the closing of the opposite edges of the urethra. A spermiogram was examined 1 week after the operation.Results. Restoration of antegrade ejaculation was achieved for 22 patients (73%), and the effects persisted for a mean of 7 (2–12) months. The spouses of four men became pregnant after surgery. In one case, the pregnancy resulted in a spontaneous abortion at gestational week 8, but the other three cases continued normally.Conclusion.This new method provides a highly effective means of restoring the physiological passage of the ejaculate. The operation is a low-invasive endoscopic procedure that does not disrupt urination, and it is possible to receive ejaculate of sufficient.