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A case is described of urinary ascites and a perinephric urinoma due to obstruction of the urinary tract by posterior urethral valves.

Background The commonest cause of obstructive uropathy in males is PUV, with well-recognised ultrasonographic features and a mortality of up to 95% if associated with oligohydramnios. Palliative treatment (vesicoamniotic shunting) has increased survival, but with a high complication rate and significant incidence of long-term renal impairment. We evaluated the diagnostic and therapeutic role of thin gauge embryofetoscopy in suspected PUV. Method A joint fetal medicine and paediatric urological team performed the procedures under local anaesthetic. Nine fetuses were assessed, mean gestation 22 weeks (7 subsequently confirmed PUV, 1 unconfirmed, 1 suspected urethral atresia). After confirmation of urinary electrolyte normality, a 1.3-mm semirigid fetoscope (Karl Storz Ltd) was inserted under ultrasound guidance into the dilated fetal bladder. Fetoscopic features were documented and an attempt made to enter the dilated upper posterior urethra. Two therapeutic procedures were assessed: valvular flushing under pressure and guidewire passage. Results The hypertrophied bladder neck was seen in 7 cases, the posterior urethra entered in 4 and the obstruction visualised in 2. Five therapeutic procedures were attempted (2 flushing, 3 guidewire). One case flushed therapeutically was confirmed after delivery to have a patent urethra. Conclusions Some cases of PUV may be treated in utero. Further studies are indicated to explore fetoscopy as a therapeutic tool. Acknowledgements Alec Welsh is supported by a Research Training Fellowship from RCOG/WellBeing.

Background Vesicoamniotic shunting may be difficult or impossible in selected cases of fetal lower obstructive uropathy (LOU). The purpose of this paper is to describe the performance of fetal hydrolaparoscopy (FHL) and endoscopic fetal cystotomy (EFC) in fetuses with complicated LOU. Method FHL-EFC was performed in one patient with a markedly thickened bladder that could not be entered percutaneously. A peritoneoamniotic shunt was also placed. FHL-EFC was performed in a second patient with a collapsed bladder from a previous vesicocentesis, as vesicoinfusion resulted in further ascites. Fetal cystoscopy was performed after EFC, and posterior urethral valves were ablated with YAG-laser. A vesicoamniotic shunt was inserted. Results Adequate bladder drainage was obtained in both cases. The first baby required bilateral nephrostomies and a permanent cystotomy at birth, and is scheduled for a bladder expansion procedure at one year of age. The second patient had premature rupture of membranes and fetal demise from treatment of this complication 5 days after the original procedure. Conclusion FHL-EFC can be performed in complicated LOU cases. The procedure involves the creation of a defect in the bladder dome under direct endoscopic visualization within a hydroperitoneum. Peritoneo or vesicoamniotic shunting, or ablation of posterior urethral valves may then be performed. FHL-EFC should be reserved only for complicated cases of LOU where conventional vesicoamniotic shunting is not possible.

Summary— A series of 46 children treated by the author since January 1972 for congenital posterior urethral valves is presented: 22% were diagnosed at birth, 28% as neonates and 52% in the first 3 months of life. Ninety-three per cent had unilateral or bilateral dilatation of the upper urinary tract at the time the valves were diagnosed and 72% had ureteric reflux. Unilateral reflux occurred into the left ureter twice as often as the right. Renal failure was present at the time of diagnosis in 72% of all of the children but in 83% of those aged less than 3 months. Surface urinary diversion was used minimally during post-operative management and contributed little to the recovery of renal function. Reflux disappeared spontaneously in one-third of the refluxing ureters. Ureteric dilatation subsided spontaneously in 57% of dilated ureters. Surgery was performed mostly for reflux. Non-refluxing ureteric dilatation was made worse by surgery in a few instances and in others the dilatation improved with time rather than as a result of surgery. Renal function returned to normal in over 60% of the children who were in renal failure at diagnosis. Measurement of glomerular filtration rate was the most accurate method of predicting recovery of renal function: a value of less than 50% of normal for age at the time of diagnosis forecast persistent chronic renal failure with all its attendant complications.

A 15-month-old infant's peripheral blood chromosome analysis showed the following defects: 46,XY,r(10)(p15.3q26.1) in 84 cells, 45,XY,-r(10) in 13 cells, and 47,XY,r(10),+r(10) in one cell. Clinical abnormalities included growth retardation, microcephaly, prominent nasal bridge, macular hypoplasia, persistent pulmonary hypertension, and posterior urethral valves with hydronephrosis. Comparison of the phenotype of five other patients with a ring chromosome 10 with the present case showed the following common manifestations: growth retardation, microcephaly, undescended testes, hydronephrosis, and, in males, posterior urethral valves. To date, this last anomaly has not been seen in patients with either a del(10p) or a del(10q) abnormality.

SUMMARY The symptoms and signs of children with congenital posterior urethral valves have been discussed in detail. The radiological findings, cystoscopic and urethroscopic appearances of thirty-five children with this condition have been described. Six of the children were under 3 years of age and the remainder between 3 and 16 years of age. Of the six children under 3, one died before operation, two are cured, and three have occasional urinary symptoms. Of the twenty-nine children over 3, fourteen are cured, two improved, eight unchanged. Five were operated on during the last six months and are not included in the results. We wish to thank the consultants at the Royal Belfast Hospital for Sick Children and the Ulster Hospital for Children and Women for referring their cases to us.

Summary— Twenty-seven patients with posterior urethral valves with varying degrees of renal function underwent both intravenous urography (IVU) and DTPA scan during their follow-up. Although the IVU was successful in excluding obstruction in 31 kidneys, the DTPA scan was successful in 43 kidneys. In addition, the DTPA scan provides quantifiable renal function, exposes the child to a lower radiation dose and has no morbidity compared with an IVU. It is recommended that the DTPA scan should replace the IVU for long-term follow-up and that the latter should be reserved for certain problems.

The first case of congenital ureteric valve was presented by Wolffer in 1887. Since then only 29 cases have been reported (Sant et al., 1985). Twelve were associated with congenital abnormalities such as complete and incomplete duplication of the kidney, horseshoe kidney and posterior urethral valves. We report a patient with a congenital ureteric valve and renal dysgenesis.

Bladder outflow obstruction caused by congenital posterior urethral valves is a well defined surgical condition which presents in early life. Presentation after the age of 10 years is rare.

To review new challenges of fetal therapy in Japan after the establishment of four existing fetal therapies as standard prenatal care with National Health Insurance coverage over the past 20 years.Reported studies and our current research activities related to four fetal therapies newly performed in Japan were reviewed.Fetoscopic endoluminal tracheal occlusion (FETO) for congenital diaphragmatic hernia (CDH) aims to occlude the trachea using a detachable balloon to promote lung growth. Following the recent successful completion of an international randomized controlled trial for CDH, in which we participated, FETO is offered for severe left CDH to perform balloon insertion at 27-29 weeks and removal at 34 weeks of gestation. Fetal cystoscopy (FC) for low urinary tract obstruction was introduced to overcome the demerits of vesicoamniotic shunting. FC may provide a proper diagnosis by visual observation of the urethra and physiological treatment of the posterior urethral valve. The effectiveness of open fetal surgery for myelomeningocele (MMC), direct surgery with laparotomy and hysterotomy, for ameliorating hindbrain herniation and the motor function was demonstrated, but it was also associated with substantial maternal and fetal risks. Fetal aortic valvuloplasty (FAV), ultrasound-guided fetal aortic balloon dilation for critical aortic stenosis with evolving hypoplastic left heart syndrome may improve left heart development and maintain biventricular circulation. Feasibility and safety studies for FC, MMC open fetal surgery, and FAV are currently ongoing.Clinical research on FETO, FC, MMC open fetal surgery, and FAV has proceeded with careful preparations in Japan.

Posterior urethral valve (PUV) is the most common congenital cause of bladder outflow obstruction in male infants. Despite timely treatment, renal damage can still occur in the long-term leading to chronic kidney disease (CKD).A retrospective review of all PUV patients in a single tertiary institution between April 1998 and July 2019 was conducted to analyze their presentations, management and outcomes. Long-term renal function, radiologic scans and somatic growth were evaluated.A total of 16 patients were included in this study. Two patients who defaulted all follow-ups were excluded. Seven patients (43.7%) presented in the antenatal period; four patients (25%) presented in the neonatal period and five patients (31.3%) presented in the post-neonatal period. Primary transurethral fulguration of valves was done in 13 patients, while three had vesicostomies as the primary procedure. Three patients had associated anterior urethral valves (AUV), which were treated endoscopically. Nine boys had additional procedures for diversion and undiversion, VUR, non-functioning kidney and clean intermittent catheterization. Ten patients had urodynamic studies performed, of which eight patients received anticholinergic therapy. Eleven patients had DMSA scans, of which three patients had a normal study and eight patients showed unilateral reduced function. Four patients were diagnosed with CKD on long-term follow-up duration over 5 years. All patients were shown to have good somatic growth.Patients with PUV can suffer from complications despite primary treatment. In our small cohort, a quarter of our patients developed CKD on follow-up. Thus, patients need long-term follow-up to optimize bladder and renal function.

Le traitement au laser par foetoscopie est utilise pour la coagulation d'anastomoses arterio-veineuses dans le cadre de syndrome transfuseur-transfuse. Actuellement, certaines malformations peuvent etre une indication a ce traitement comme le syndrome des bandes amniotiques, le choriangiome, l'obstruction de l'uretre, le kyste sacro-coccygien et les masses pulmonaires. Ces pathologies peuvent etre letales sans intervention et ce traitement, encore experimental, pourrait etre propose dans ces cas. Il s'agit d'une meta-analyse et revue systematique de la litterature a l'aide de « PubMed », « Medline » et « Web of Science » dans laquelle nous avons recense tous les cas publies de traitement par laser durant la periode foetale depuis 1980. Cinq groupes de pathologie peuvent beneficier de ce traitement et sont decrits separement. Le syndrome des bandes amniotiques peut engendrer une amputation du membre atteint par compression induisant une ischemie ou un deces foetal si cette bande atteint le cordon ombilical. De larges choriangiomes, teratomes sacrococcygiens ou masses pulmonaires peuvent mener a un hydrops foetal par compression ou « vol vasculaire » menant dans les cas les plus severes a la perte foetale. Des valves de l'uretre posterieur creent une obstruction induisant une megavessie avec des repercussions renales ainsi qu'une hypoplasie pulmonaire. Le pronostic de ces differentes pathologies peut etre fatal et les options therapeutiques sont limitees. Dans certains cas, la therapie au laser par foetoscopie peut changer ce pronostic. Encore experimentale, cette technique montre des resultats prometteurs. Le taux de reussite et le taux de survie dans les differentes categories est encore perfectible. L'amelioration devra se faire aussi bien au niveau de l'indication operatoire et de la selection des cas, de la technique et du materiel que de l'experience des operateurs. Cette technique peut offrir un espoir de survie pour des foetus tres certainement condamnes. Cette etude est basee essentiellement sur des petites series de cas ou de cas unique, les resultats doivent donc etre analyses avec prudence car des biais de report ou au niveau des investigateurs ne peuvent pas etre exclus. La prise en charge de tels cas doit se faire dans un centre de reference, la de ision d'intervenir devrait etre multidisciplinaire et les parents bien informes du pronostic. -- Fetoscopic coagulation of placental anastomoses is the treatment of choice for severe twin-to-twin transfusion syndrome. In the present day, fetal laser therapy is also used to treat amniotic bands, chorioangiomas, sacrococcygeal teratomas, lower urinary tract obstructions and chest masses, all of which will be reviewed in this article. Amniotic band syndrome can cause limb amputation by impairing downstream blood flow. Large chorioangiomas (>4 cm), sacrococcygeal teratomas or fetal hyperechoic lung lesions can lead to fetal compromise and hydrops by vascular steal phenomenon or compression. Renal damage, bladder dysfunction and lastly death because of pulmonary hypolasia may be the result of megacystis caused by a posterior urethral valve. The prognosis of these pathologies can be dismal, and therapy options are limited, which has brought fetal laser therapy to the forefront. Management options discussed here are laser release of amniotic bands, laser coagulation of the placental or fetal tumor feeding vessels and laser therapy by fetal cystoscopy. This review, largely based on case reports, does not intend to provide a level of evidence supporting laser therapy over other treatment options. Centralized evaluation by specialists using strict selection criteria and long-term follow-up of these rare cases are now needed to prove the value of endoscopic or ultrasound-guided laser therapy.

The aim of the current study was to evaluate long-term outcomes of pediatric live kidney transplantation in patients with genitourinary anomalies relative to those with primary kidney diseases. The study included 35 pediatric patients who received a live kidney transplantation during the last 25 yr (28 males, six females). Median age at the time of transplantation was nine yr (range 1–15 yr), and the median follow-up period was 151 months (range 6–239 month). The patients were divided into two groups. The urological group (n = 14) included patients with primary obstructive/reflux nephropathy. The renal group (n = 20) included patients with primary renal disorders. Differences between groups in graft survival, clinical course, and final graft function were evaluated. Original diseases represented in the urological group included five cases with primary VUR and eight cases with secondary VUR. Diseases in the renal group included eight cases with bilateral hypo-dysplastic kidney, three cases with focal/segmental glomerular sclerosis, two cases with membranous proliferative glomerulonephritis, two cases with congenital nephrotic syndrome and five cases with other forms of chronic nephritis. Ten of 14 cases in the urological group, relative to six of 20 in the renal group, were preemptive. Median age at transplantation was 7.5 or 10 yr old, respectively, in the urological or renal group. Twelve kidney recipients in the urological group had also undergone other urinary surgeries, including upper urinary tract drainage, ureteroneocystostomy, augmentation cystoplasty, endoscopic incision of posterior-urethral valve, urethroplasty, etc. Cumulative post-operative complications occurred in nine or 16, respectively, in the urological or renal group. The acute rejection free and overall graft survival were similar in both groups. One patient in the urological group lost his graft while six patients in the renal group lost their grafts. Thus, the post-transplant clinical outcome of pediatric transplantation in patients with urological anomalies is comparable to that of recipients with primary renal disease. Appropriate urinary tract reconstruction and management is essential to reduce the risk of graft dysfunction because of urinary problems.

Objective: 1) To detect fetal anomalies in unselected pregnant population. 2) To study the accuracy of ultrasound in the detection of fetal anomalies. Material and Methods: This study was conducted in the Department of Radiology, Ultrasound division with Toshiba Color Doppler machine with linear and convex sector probes of 3.5 MHz frequency. Study of population: – routine ultrasound examination was performed in 11 116 unselected pregnant population at first and second level obstetric unit to detect fetal anomalies. Various anomalies detected: – Anencephaly, Hydrocephalus, Holoprosencephaly, dandy-Walker malformation and variant, acrania, encephalocele, choriod plexus cyst, PUJ, Multiple cystic dysplastic kidney, Polycystic renal disease, Posterior urethral valve, Omphalocele, Esophagealduodenal atresia, Twin–Twin transfusion syndrome, Tetralogy of Fallot, Ebsteins anomaly, hypoplastic right ventricle, diaphragmatic hernia, club foot, osteogenesis imperfecta, dacrocystocele, ovarian cyst, VSD, Holt-Oram syndrome etc. Results: Over the two year study, 11 116 pregnant women were routinely scanned. We found 84 malformations of various systems, of which we detected 74 anomalies, 8 missed. In our study the ovar-all accuracy of ultrasound for the detection of fetal anomalies was approximately 91%. Conclusion: Ultrasonography has a tremendous role in the evulation of fetal anomalies. The accuracy is high. Ultrasonography is boon to obstetrics. It is safe, simple, non-invasive, easily available, acceptable to all. The Ultrasonography is the imaging modality of choice for the detection of fetal anomalies.

Objective: 1) To detect fetal anomalies in unselected pregnant population. 2) To study the accuracy of ultrasound in the detection of fetal anomalies. Material and Methods: This study was conducted in the Department of Radiology, Ultrasound division with Toshiba Color Doppler machine with linear and convex sector probes of 3.5 MHz frequency. Study of population: – routine ultrasound examination was performed in 11 116 unselected pregnant population at first and second level obstetric unit to detect fetal anomalies. Various anomalies detected: – Anencephaly, Hydrocephalus, Holoprosencephaly, dandy-Walker malformation and variant, acrania, encephalocele, choriod plexus cyst, PUJ, Multiple cystic dysplastic kidney, Polycystic renal disease, Posterior urethral valve, Omphalocele, Esophagealduodenal atresia, Twin–Twin transfusion syndrome, Tetralogy of Fallot, Ebsteins anomaly, hypoplastic right ventricle, diaphragmatic hernia, club foot, osteogenesis imperfecta, dacrocystocele, ovarian cyst, VSD, Holt-Oram syndrome etc. Results: Over the two year study, 11 116 pregnant women were routinely scanned. We found 84 malformations of various systems, of which we detected 74 anomalies, 8 missed. In our study the ovar-all accuracy of ultrasound for the detection of fetal anomalies was approximately 91%. Conclusion: Ultrasonography has a tremendous role in the evulation of fetal anomalies. The accuracy is high. Ultrasonography is boon to obstetrics. It is safe, simple, non-invasive, easily available, acceptable to all. The Ultrasonography is the imaging modality of choice for the detection of fetal anomalies.