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Fetal megacystis (MC) can be severe and is mainly caused by fetal lower urinary tract obstruction (LUTO). Mortality of fetal LUTO can be high as a result of pulmonary hypoplasia and/or (chronic) renal insufficiency. Several technical procedures for vesicoamniotic shunting (VAS) were developed to improve fetal MC outcomes.We present the outcome of nine fetuses with MC who received VAS in the prenatal period (14 + 6 to 27 + 6 weeks GA) using the SomatexEight Fetuses had uncomplicated VAS intervention. One case developed PPROM 24 h after VAS leading to abortion. Pregnancy was later terminated in further two cases. All six live-born infants received intensive care treatment. Invasive-mechanical ventilation was necessary in one case who died 24 h post-partum of severe cardiac depression. Five infants who survived the follow-up time developed chronic renal insufficiency (CRI), with one infant developing end-stage renal failure requiring peritoneal dialysis.Overall, 5 of 9 LUTO fetuses (55%) undergoing VAS with the Somatex

Introduction A case of spontaneous fetal bladder rupture occurring in a woman with SARS-CoV-2 pneumonia receiving invasive ventilatory support is reported. Case A 33-year-old woman was admitted at 30.6 weeks' gestation with the diagnosis of severe pneumonia due to COVID-19. The patient required invasive mechanical ventilation on day 2. Propofol, fentanyl, midazolam, and dexmedetomidine were administered for sedation, pain relief, and to improve patient-ventilator interaction. A bedside ultrasound on day 3 revealed fetal megacystis. Follow-up scan two days later showed urinary ascites and a collapsed bladder. The diagnosis of fetal bladder rupture was confirmed postpartum. Bladder repair was performed on day 5, with an uneventful recovery. Discussion Transplacental transfer of opioids during invasive ventilatory support in pregnancy may cause acute fetal bladder atony leading to severe urine retention and, potentially, bladder rupture. This can be a serious complication of adjunctive therapy in women with severe SARS-CoV-2 pneumonia.

Objectives: Fetal megacystis is very rare; however this problem may be associated with other structural or chromosomal abnormalities leading to morbidity and mortality. Due to renal injury and pulmonary hypoplasia, prognosis is poor especially in fetuses with early onset oligohydramnios. In this study, we evaluated the management strategies and outcomes of fetal megacystis in our hospital.Study Design: The data of cases who were prenatally diagnosed with megacystis between 1 January 2017 and 31 December 2019 were analyzed. Ultrasonography findings and antennal interventions were withdrawn from computerized database. Information about postpartum status was also received. The data were analyzed in terms of diagnostic methods, fetal interventions (vesicosynthesis (VS), vesicoamniotic shunt (VAS)), potential prognostic markers and short/long-term prognostic results.Results: A total of 15 megacystis patients were detected. One of the fetuses was female and the remaining cases were male. Six of the patients were isolated. Ten patients underwent invasive procedures for prenatal aneuploidy diagnosis and one of them diagnosed with trisomy 18. Totally, 4 patients underwent VS; however no VAS was recorded. Four fetuses were terminated and intrauterine fetal demise occurred in 2 fetuses. Four patients were lost during follow up. Survival rate was 33.3%.Conclusion: Fetal megacystis is an important ultrasonographic finding which may be a component of chromosomal / genetic anomalies. This problem may also be isolated in some cases. Because of the poor outcome, parents should be well informed and all interventions should be offered to families.

OBJECTIVES To evaluate aneuploidy rate, prognostic factors, and perinatal outcomes following a diagnosis of fetal megacystis at 11-14 week's gestation. METHODS A retrospective study of first trimester fetal megacystis from 2010 to 2020 was performed, including ultrasound finding, perinatal outcomes, pathology reports, genetic tests, and neonatal investigations. RESULTS A total of 98 cases of first trimester fetal megacystis was identified with an overall aneuploidy rate of 12%. There were 54% live births and 46% fetal losses including spontaneous fetal demise and elective termination. Among the 45 fetal losses, 64% had additional structural abnormalities at index ultrasound and final diagnoses were achievable in 64% cases. Among the 53 livebirths, additional ultrasound abnormalities were detected in only 1 fetus and spontaneous resolution of megacystis was detected in 96% of cases. The two cases where fetal megacystis persisted had major postnatal diagnoses: cloacal malformation and megacystis-microcolon-intestinal hypoperistalsis syndrome, respectively. Our data showed LBD ≥ 12 mm was the best individual predictor of adverse perinatal outcome and all 11 cases of lower urinary tract obstruction (LUTO) were diagnosed in fetuses with LBD ≥ 12 mm. CONCLUSIONS First trimester ultrasound provides important prognostic factors and isolated megacystis

Megacystis-microcolon-intestinal-hypoperistalsis syndrome (MMIHS) is a severe congenital visceral myopathy characterized by an abdominal distension due to a large non-obstructed urinary bladder, a microcolon and intestinal hypo- or aperistalsis. Most of the patients described to date carry a sporadic heterozygous variant in ACTG2. More recently, recessive forms have been reported and mutations in MYH11, LMOD1, MYLK and MYL9 have been described at the molecular level. In the present report, we describe five patients carrying a recurrent heterozygous variant in ACTG2. Exome sequencing performed in four families allowed us to identify the genetic cause in three. In two families, we identified variants in MMIHS causal genes, respectively a nonsense homozygous variant in MYH11 and a previously described homozygous deletion in MYL9. Finally, we identified compound heterozygous variants in a novel candidate gene, PDCL3, c.[143_144del];[380G>A], p.[(Tyr48Ter)];[(Cys127Tyr)]. After cDNA analysis, a complete absence of PDLC3 expression was observed in affected individuals, indicating that both mutated transcripts were unstable and prone to mediated mRNA decay. PDCL3 encodes a protein involved in the folding of actin, a key step in thin filament formation. Presumably, loss-of-function of this protein affects the contractility of smooth muscle tissues, making PDCL3 an excellent candidate gene for autosomal recessive forms of MMIHS.

Los primeros procedimientos fetales considerados quirúrgicos fueron realizados en 1961, por Liley, al transfundir sangre a fetos con anemia por isoinmunización Rh. En 1974, se realizó la primera fetoscopia diagnóstica, en la Universidad de Yale, EE. UU. Un hito histórico sucede en 1981, cuando Harrison en la Universidad de California realizó por primera vez drenaje en un caso de mega vejiga causado por valva uretral; colocó, bajo control ecográfico, un catéter desde la vejiga a la cavidad amniótica. Por este hecho, se le reconoce como el ‘padre de la cirugía fetal’. En 1982, se reunieron especialistas de 5 países, vinculados a la cirugía fetal, y liderados por Harrison publicaron las primeras normas a regir en este campo nuevo de la medicina. Posteriormente, grupos colaborativos europeos revisaron las aplicaciones de esta cirugía, así como las normas éticas y de seguridad que debían ser prioritarias en estas técnicas. En el 2011, instituciones académicas y de ética de los EE. UU. reafirmaron los principios, ya reconocidos, en los que debían basarse los nuevos centros de alta calidad en cirugía fetal. En 2015, se establecieron las reglas para la innovación tecnológica y la investigación en este campo, remarcando la necesidad de crear los comités de ética. En el 2017, los especialistas involucrados, nuevamente convocados por organizaciones académicas de los EE. UU., emitieron opinión sobre el contexto global de esta cirugía, las obligaciones éticas, de infraestructura, implementación tecnológica, investigación conjunta y capacitación integral del equipo; remarcaron la necesidad de crear los comités de supervisión en estos centros. En América Latina, el desarrollo de la cirugía fetal se encuentra en una etapa intermedia, de acuerdo a los reportes actuales. En el Perú, se ha publicado artículos sobre los primeros casos de cirugía prenatal.

Objective To explore the outcomes and prognostic factors associated with fetal megacystis (enlarged bladder). Study design The MEDLINE and EMBASE databases were searched for studies reporting on outcomes of fetal megacystis. The outcomes observed were chromosomal abnormalities, associated structural anomalies, spontaneous resolution, and survival rates. We also evaluated the potential role of fetal gender, oligohydramnios, gestational age at diagnosis, and intrauterine intervention as prenatal prognostic factors. Results The search identified 558 articles in total, and 13 studies (1675 fetuses) were included in this systematic review. The overall incidences of chromosomal abnormalities and associated structural anomalies in fetal megacystis were 10% and 24%, respectively. Spontaneous resolution of megacystis occurred in 32% of fetuses, and 44% of fetuses were born alive and survived until the follow-up. The odds ratio of survival with oligohydramnios was 0.14, and the mean difference in gestational age at diagnosis between survival and non-survival was 3.43 weeks. No significant difference in survival rate was observed between the genders, and an intrauterine intervention did not significantly improve the prognosis. Conclusions A considerable proportion of fetuses with megacystis are born with a good prognosis. Oligohydramnios and lower gestational age at diagnosis are associated with worse outcomes.

OBJECTIVE To construct reference values for fetal urinary bladder distension in pregnancy and use Z-scores as a diagnostic tool to differentiate posterior urethral valves (PUV) from urethral atresia (UA). METHODS This was a prospective cross-sectional study in healthy singleton pregnancies aimed at constructing nomograms of fetal urinary bladder diameter and volume between 15 and 35 weeks' gestation. Z-scores of longitudinal bladder diameter (LBD) were calculated and validated in a cohort of fetuses with megacystis with ascertained postnatal or postmortem diagnosis, collected from a retrospective, multicenter study. Correlations between anatomopathological findings, based on medical examination of the infant or postmortem examination, and fetal megacystis were established. The accuracy of the Z-scores was evaluated by receiver-operating-characteristics (ROC)-curve analysis. RESULTS Nomograms of fetal urinary bladder diameter and volume were produced from three-dimensional ultrasound volumes in 225 pregnant women between 15 and 35 weeks of gestation. A total of 1238 urinary bladder measurements were obtained. Z-scores, derived from the fetal nomograms, were calculated in 106 cases with suspected lower urinary tract obstruction (LUTO), including 76 (72%) cases with PUV, 22 (21%) cases with UA, four (4%) cases with urethral stenosis and four (4%) cases with megacystis-microcolon-intestinal hypoperistalsis syndrome. Fetuses with PUV showed a significantly lower LBD Z-score compared to those with UA (3.95 vs 8.83, P

Objective: To evaluate the best management of fetal megacystis diagnosed in the first trimester and define the prognosis and the most appropriate follow-up as early as possible. Methods: This is a retrospective study of first-trimester fetal megacystis diagnosed in pregnant women who performed a combined screening for fetal aneuploidy. Megacystis was defined as a longitudinal bladder diameter (LBD) greater than 7 mm. All fetuses were divided into two groups according to the LBD: Group A with LBD > 15 mm and Group B with LBD Results: Twenty-six cases were identified between 2011 and 2016; three cases of aneuploidy were excluded from the study. Of the remaining 23 cases, 11 were included in Group A and 12 in Group B. All Group A fetuses had an adverse outcome. In Group B: five (41.7%) cases had an adverse outcome and seven (58.3%) had a spontaneous resolution of megacystis. The ultrasound findings of both the ultrasound scans, the second and the third, were 100% concordant. Conclusion: An ultrasound scan performed 2 weeks after the megacystis diagnosis can predict the outcome in fetuses with an LBD

To assess prenatal prognostic criteria for fetuses with megacystis in order to counsel parents. In a retrospective observational study at a single tertiary referral center, we assessed the clinical course of 53 fetuses with megacystis cared for at the Department of Obstetrics of the University Hospital Zurich between 1995 and 2008 and followed them up for 2 to 12 years. We determined fetal karyotype, amniotic fluid volume and fetal urinary biochemistry as prenatal prognostic factors. The renal function of survivors was grouped according to age-related creatinine values. Using logistic regression analysis, gestational age-dependent discrimination curves and corresponding ROC curves for fetal urine, beta-2 microglobulin, osmolarity and chloride were calculated. 43 out of 53 fetuses underwent vesicocentesis, and spontaneous remission occurred in 3 fetuses. 15 fetuses survived, termination of pregnancy was requested in 23 cases, and 12 neonatal and 3 intrauterine deaths were observed. Reduced amniotic fluid volume showed a significant (p = 0.0027) increase of impaired renal function or perinatal death. Discrimination between survivors and non-survivors was complete for fetal urine beta-2 microglobulin with an area under the curve (AUC) of 1.0. For fetal urine osmolarity and fetal urinary chloride, the AUC was 0.81 and 0.76, respectively. The assessment of prognosis for fetal megacystis should include fetal k aryotyping, determination of amniotic fluid and assessment of fetal urine biochemistry. Gestational age-dependent regression lines disclose clinically relevant discrimination and can be used as selection criteria for fetal interventions and parental counselling.ZIEL: Zur Beratung der Eltern, deren Feten eine Megavesika aufweisen, sollen prognostische Kriterien evaluiert werden. In einer retrospektiven Beobachtungsstudie am Perinatalzentrum des Universitätsspitals Zürich wurden zwischen 1995 und 2008 53 Feten mit Megavesika betreut und für 2 bis 12 Jahre nachuntersucht. Als Prognosefaktoren wurden der fetale Karyotyp, die Fruchtwassermenge und die Biochemie des fetalen Urins bestimmt. Die Nierenfunktion der Überlebenden wurde aufgrund der altersabhängigen Kreatininwerte klassiert. Mittels logistischer Regression wurden schwangerschaftsalterabhängige Regressionskurven und die zugehörigen ROC-Kurven für das fetale beta-2 Mikroglobulin im Urin, die Urin-Osmolarität und das Urin-Chlorid bestimmt. 43 von 53 Feten wurden einer Vesicozentese unterzogen und in 3 Fällen kam es zu einer Spontanremission. 15 Feten überlebten, ein Schwangerschaftsabbruch wurde in 23 Fällen durchgeführt und 12 neonatale und 3 intrauterine Todesfälle wurden beobachtet. Eine verminderte Fruchtwassermenge war signifikant (p = 0.0027) mit einer verminderten Nierenfunktion oder einem perinatalen Tod assoziiert. Die Trennung von Überlebenden und Nicht-Überlebenden war mittels fetalem Urin beta-2 Mikrogloblulin bei einer Fläche unter der Kurve (AUC) von 1,0 komplett möglich. Für die fetale Urin-Osmolarität wurde eine AUC von 0.81 und das fetale Urin-Chlorid von 0.76 kalkuliert. Zur Prognoseeinschätzung bei fetaler Megavesika sollten eine fetale Karyotypisierung, die Bestimmung der Fruchtwassermenge und die Analyse der fetalen Urinbiochemie erfolgen. Schwangerschaftsalterabhängige Regressionskurven ermöglichen eine klinisch hilfreiche Trennung zwischen Überlebenden und Nicht-Überlebenden und sind somit wertvolle Kriterien für die Indikationsstellung vor fetalen Interventionen und zur Beratung der Eltern.

Objectives: To investigate the natural history of fetal megacystis from diagnosis in utero to postnatal outcome, and to identify prognostic indicators of spontaneous resolution and postnatal outcome after resolution. Methods: This was a national retrospective cohort study. Fetal megacystis was defined in the first trimester as a longitudinal bladder diameter (LBD) ≥ 7 mm, and in the second and third trimesters as an enlarged bladder failing to empty during the entire extended ultrasound examination. LBD and gestational age (GA) at resolution were investigated with respect to likelihood of resolution and postnatal outcome, respectively. Sensitivity, specificity and area under the receiver–operating characteristics curve (AUC) were calculated. Results: In total, 284 cases of fetal megacystis (93 early megacystis, identified before the 18th week, and 191 late megacystis, identified at or after the 18th week) were available for analysis. Spontaneous resolution occurred before birth in 58 (20%) cases. In cases with early megacystis, LBD was predictive of the likelihood of spontaneous resolution (sensitivity, 80%; specificity, 79%; AUC, 0.84), and, in the whole population, GA at regression was predictive of postnatal outcome, with an optimal cut-off at 23 weeks (sensitivity, 100%; specificity, 82%; AUC, 0.91). In the group with early megacystis, the outcome was invariably good when resolution occurred before the 23rd week of gestation, whereas urological sequelae requiring postnatal surgery were diagnosed in 3/8 (38%) cases with resolution after 23 weeks. In the group with late megacystis, spontaneous resolution was associated with urological complications after birth, ranging from mild postnatal hydronephrosis in infants with resolution before 23 weeks, to more severe urological anomalies requiring postnatal surgery in those with resolution later in pregnancy. This supports the hypothesis that an early resolution of megacystis is often related to a paraphysiological bladder enlargement that resolves early in pregnancy without consequences, while antenatal resolution occurring later in pregnancy (after the 23rd week of gestation) should suggest a pathological condition with urological sequelae. Conclusions: In fetal megacystis, LBD and GA at regression can be used as predictors of resolution and outcome, respectively. These parameters could help in fine-tuning the prognosis and optimizing the frequency of follow-up scans.

Summary Fetal megacystis is variably defined and understood. The literature on fetal megacystis was systematically reviewed, focusing on prenatal diagnosis, associations and outcomes. This yielded a total of 18 primary references and eight secondary references. Fetal megacystis has an estimated first-trimester prevalence of between 1:330 and 1:1670, with a male to female ratio of 8:1. In the first trimester, megacystis is most commonly defined as a longitudinal bladder dimension of ≥7 mm. Later in pregnancy, a sagittal dimension (in mm) greater than gestational age (in weeks) + 12 is often accepted. Megacystis can be associated with a thickened bladder wall, which has been objectively defined as >3 mm. Oligohydramnios is present in approximately half of all cases. The most common underlying diagnosis is posterior urethral valves (57%), followed by urethral atresia/stenosis (7%), prune belly syndrome (4%), megacystis-microcolon-intestinal-hypoperistalsis syndrome (MMIHS) (1%), and cloacal anomalies (0.7%). Karyotype anomalies are found in 15%, and include trisomy 18, trisomy 13 and trisomy 21. Ultrasound imaging alone is often insufficient to enable a definitive diagnosis, although it may indicate that a specific diagnosis is more likely. Overall, about 50% of reported fetuses with megacystis are terminated, but this proportion varies considerably between countries and over time. Prognostic stratification is evolving, with the most important factors being oligohydramnios, gestational age at diagnosis, degree of bladder enlargement, renal hyperechogenicity, karyotype, and sex. Conclusions This review demonstrated some consensus on the ultrasound criteria for defining fetal megacystis, and illustrated the spectrum of pathologies and their relative frequencies that can cause this condition. It also underlined important associated karyotype anomalies. To progress understanding of the natural history of enlarged fetal bladders, more accurate diagnostics are required, and risk stratification needs to be refined to facilitate prenatal counseling.

Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital disorder, acknowledged as the most debilitating form of chronic intestinal pseudoobstruction disorders. Because the disease is often fatal in the first years of life and the surviving individuals are dependent on supportive medical or surgical treatment, definite antenatal diagnosis is desirable. We report a case of prenatally diagnosed MMIHS and discuss the suspicious findings and diagnostic criteria that allow a rapid and definite prenatal diagnosis.

Monozygote Zwillinge gelten gemeinhin als „identische Klone“. Es gibt jedoch eine zunehmende Anzahl an Fallberichten uber diskordante eineiige Zwillinge und Ursachen fur Diskordanz werden vermehrt untersucht. Wir berichten uber monozygote Zwillingsfruhgeborene, diskordant fur die seltene fetale Megazystis bei Kloakenfehlbildung. Durch Konzeption mittels intrazytoplasmatischer Spermieninjektion entstand nach Transfer von 2 Embryonen ein monochoriales, diamniales Zwillingspaar. Im ersten Trimenon wurde eine diskordante fetale Megazystis mit konsekutivem Oligohydramnion und Lungenhypoplasie bei unauffalligem Karyotyp diagnostiziert. Das betroffene Kind wurde bei 25+3 SSW geboren, palliativ behandelt und verstarb rasch. Post mortem wurde eine Kloakenfehlbildung festgestellt. Typische Ursache fur die Megazystis beim mannlichen Geschlecht ist eine obstruktive Uropathie. Bei den seltener betroffenen weiblichen Feten liegt meist ein komplexes urogenitales Fehlbildungssyndrom vor, wie die Kloakenfehlbildung, deren Entstehung auf eine Storung der Gastrulation zuruckgeht. Wir fanden 10 Fallberichte/-serien uber mono- oder dizygote Zwillinge mit Diskordanz entweder fur eine Megazystis oder fur eine Kloakenfehlbildung. Die Vergleichbarkeit mit unserem Fall war eingeschrankt, da Aspekte wie Konzeptionsmechanismus, Geschlecht, Karyotyp oder Atiologie der Fehlbildung nicht in allen Fallen aufgefuhrt wurden. Diskordanz monozygoter Zwillinge fur strukturelle Fehlbildungen hangt nach bisherigem Verstandnis eng mit den Vorgangen der Zwillingsentstehung selbst zusammen. Bei kunstlicher Befruchtung wird eine erhohte Inzidenz von (monozygoten) Zwillingsschwangerschaften und Fehlbildungen diskutiert. Im Hinblick auf Konzeptionsmechanismus, Zygotie, Geschlecht und Fehlbildungskonstellation ist unser Fall einzigartig.

Objective: To propose a clinical score for the optimal antenatal diagnosis of fetal lower urinary tract obstruction (LUTO) in the second and third trimesters of pregnancy, as an alternative to the commonly used ultrasound triad of megacystis, keyhole sign and hydronephrosis. Methods: This was a national retrospective study carried out at the eight tertiary fetal medicine units (FMUs) in The Netherlands. Only cases referred for megacystis from the second trimester onwards and with a clear postnatal diagnosis were included in the study. At referral, data were collected on amniotic fluid volume, renal cortical appearance, bladder volume, hydronephrosis, fetal ascites, ureteral size, keyhole sign, fetal sex and gestational age. Multivariate analysis was performed, starting by including all antenatal variables, and then excluding the weakest predictors using the backward stepwise strategy. Results: Over a 7-year period, 312 fetuses with a diagnosis of megacystis were referred to the eight Dutch tertiary FMUs. A final diagnosis was achieved in 143 cases, including 124 of LUTO and 19 reclassified after birth as non-obstructive megacystis. The optimal bladder volume cut-off for prediction of LUTO was 35 cm3 (area under the curve (AUC) = 0.7, P = 0.03). The clinical score formulated on the basis of the multivariate analysis included fetal sex, degree of bladder distension, ureteral size, oligo- or anhydramnios and gestational age at referral. The combination of these five variables demonstrated good accuracy in discriminating LUTO from non-obstructive megacystis (AUC = 0.84, P < 0.001), compared with the poor performance of the ultrasound triad (AUC = 0.63, P = 0.07). Conclusions: We propose a clinical score that combines five antenatal variables for the prospective diagnosis of congenital LUTO. This score showed good discriminative capacity in predicting LUTO, and better diagnostic accuracy compared with that of the classic ultrasound triad. Future studies to validate these results should be carried out in order to refine antenatal management of LUTO and prevent inappropriate fetal interventions. © 2017 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology.

Evaluation of the kidneys, bladder, and amniotic fluid volume forms part of any standard obstetrical ultrasound. When a fetal genitourinary anomaly is suspected, a more detailed evaluation is necessary. This detailed imaging can be challenging in the setting of decreased or absent amniotic fluid or large maternal body habitus, and in complex malformations. In these situations, magnetic resonance imaging can help to better define the fetal anatomy and provide a more confident and specific prenatal diagnosis.

Objective: To identify the molecular basis for prenatally suspected cases of megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) (MIM 249210) in 3 independent families with clinical and radiographic evidence of MMIHS. Methods: Whole-exome sequencing (WES) and Sanger sequencing of the ACTG2 gene. Results: We identified a novel heterozygous de novo missense variant in ACTG2 c.770G>A (p.Arg257His) encoding γ-2 smooth muscle actin (ACTG2) in 2 siblings with MMIHS, suggesting gonadal mosaicism of one of the parents. Two additional de novo missense variants (p.Arg257Cys and p.Arg178His) in ACTG2 were identified in 2 additional MMHIS patients. All of our patients had evidence of fetal megacystis and a normal or slightly increased amniotic fluid volume. Additional findings included bilateral renal hydronephrosis, an enlarged fetal stomach, and transient dilated bowel loops. ACTG2 immunostaining of the intestinal tissue showed an altered muscularis propria, a markedly thinned longitudinal muscle layer, and a reduced amount and abnormal distribution of ACTG2. Conclusion: Our study demonstrates that de novo mutations in ACTG2 are a cause of fetal megacystis in MMIHS and that gonadal mosaicism may be present in a subset of cases. These findings have implications for the counseling of families with a diagnosis of fetal megacystis with a preserved amniotic fluid volume and associated gastrointestinal findings.

Objectives: The aim of the study was to assess the outcome of vesico-amniotic shunting performed before 16 weeks of pregnancy in fetuses with severe megacystis diagnosed in the first trimester of pregnancy. Material and methods: Between January 2008 and October 2012 severe megacystis with the bladder length > 15 mm was diagnosed in 17 fetuses. The procedure of early vesico-amniotic shunting (VAS) was offered to 8 patients with presumably isolated LUTO. The procedure of VAS was performed in 6 fetuses. Before the intervention one or two procedures of vesicocentesis and urine analysis were performed. Results: In all treated cases shunts provided urinary tract decompression. All babies were born prematurely, 2 of them died due to premaurity, 3 of them survived and have normal renal function at the age of 5–6 years. In 4/5 children accompanying malformations were later diagnosed, in 1 born prematurely neonate necropsy was not performed. Conclusions: Our results suggest that early vesico-amniotic shunting in fetal LUTO is feasible and may potentially prevent not only pulmonary hypoplasia but also renal insufficiency. However, the rationale of the procedure needs further investigation due to a high risk of long-term morbidity and co-existing malformations in children Before offering the therapy detailed counseling of the parents about the possible pros and cons of the therapy is necessary.

Background Fetal megacystis is a sonographic feature that may be indicative of several underlying pathologies. Despite advances in diagnosis and management, the overall prognosis of affected fetuses remains poor and about 50% of such pregnancies are terminated. Aims To define the frequency, management, survival and renal outcomes of fetal megacystis over nine years at Wellington Hospital, New Zealand. Materials and Methods A nine-year retrospective review of fetuses with an antenatal diagnosis of megacystis was undertaken. Results Sixteen cases were identified (nine live births, five terminations and two perinatal deaths). This gives an observed frequency of one per 940 fetuses scanned. Two-thirds of the live births have survived and been followed for a mean of 5.3 years. None have required renal dialysis or transplantation to date. Conclusions The current series contributes to our knowledge of fetal megacystis and helps to inform antenatal counselling. Improved prognostic criteria are urgently required to accurately differentiate between fetuses with favourable versus poor outcomes.

Objective To describe the diagnostic criteria and outcome of fetal megacystis according to the gestational age at diagnosis. Methods A 7-year retrospective study was carried out from 2004 to 2011, including cases of megacystis referred to 2 prenatal fetal medicine units. The following data were collected and analyzed: maternal age, term at diagnosis (gestational weeks), ultrasonographic and magnetic resonance imaging data, karyotype, decision of the multidisciplinary prenatal team, fetopathology in cases of termination of pregnancy or fetal death, final diagnosis at birth after ultrasonography and voiding cystourethrography, and medical and surgical follow-up. Results Of the 69 fetuses included in this study, 82.6% were males; 26 were diagnosed during the first trimester, 21 during the second, and 22 during the third. During the first trimester, the main etiologies were urethral occlusions and prune-belly syndrome with poor fetal prognosis. Nineteen pregnancies (69%) were terminated for medical reasons including the association with other malformations, poor evolution, or miscarriage. Only 4 children were born alive. The main etiologies of megacystis discovered during the second and third trimesters were vesicoureteral reflux and urethral occlusion. Twenty of 22 fetuses (91%) were born alive when the fetal megacystis was discovered after 27 weeks of gestation. Conclusion Antenatal discovery of megacystis is a complex and challenging prognostic situation. The prognosis depends on the gestational age at discovery. Megacystis is not always associated with obstruction. In a newborn with megacystis, bladder outlet obstruction has to be excluded. Optimal counseling of the involved parents requires a multidisciplinary approach.

Objective: To investigate prenatal ultrasonographic findings associated with megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS). Methods: A PubMed search was performed using the terms ‘MMIHS', ‘MMIH' and ‘prenatal diagnosis'. Results: A total of 50 cases were analyzed. Prenatal diagnosis was achieved in 26% of cases. In 54% of patients with a correct antenatal diagnosis there was a previously affected sibling. Fetal megacystis with or without hydroureteronephrosis was the most common initial ultrasonographic finding (88%). While megacystis eventually complicated all fetal presentations, isolated bilateral hydronephrosis and isolated dilated stomach were noted (in 10 and 2% of cases, respectively) prior to megacystis. The initial sonographic abnormality was most commonly detected (in 70% of patients) in the second trimester. Amniotic fluid was normal in 69% and increased in 27% of cases. Gastrointestinal abnormalities were noted in 24% of pregnancies. Conclusion: MMIHS should be prenatally suspected when fetal megacystis is associated with a normal or increased amount of amniotic fluid and normal external genitalia, especially in the setting of a suggestive family history. Associated gastrointestinal findings support this diagnosis. Isolated bilateral hydronephrosis may precede the development of megacystis. Due to preserved renal function and a general absence of oligohydramnios, no rationale exists for vesicoamniotic shunt placement.

Objective To investigate the gestational age-specific outcomes and the different etiologies of megacystis diagnosed at screening ultrasound. Methods A retrospective single-center study was conducted between 1989 and 2009. We identified all consecutive cases of megacystis prenatally diagnosed during routine ultrasound screening. Outcome, final diagnosis, and renal function were recorded. Results Eighty-four patients were included. An isolated lower urinary tract obstruction was observed in 38/84 (45.2%), ureterovesical reflux in 9/84 (10.7%), an associated congenital abnormality in 32/84 (38.1%) and a normal bladder in 5/84 (6%). Increased gestational age at diagnosis was correlated with an increased rate of live born children (P

The urofacial, or Ochoa, syndrome is characterised by congenital urinary bladder dysfunction together with an abnormal grimace upon smiling, laughing and crying. It can present as fetal megacystis. Postnatal features include urinary incontinence and incomplete bladder emptying due to simultaneous detrusor muscle and bladder outlet contractions. Vesicoureteric reflux is often present, and the condition can be complicated by urosepsis and end-stage renal disease. The syndrome has long been postulated to have neural basis, and it can be familial when it is inherited in an autosomal recessive manner. Most individuals with urofacial syndrome genetically studied to date carry biallelic, postulated functionally null mutations of HPSE2 or, less commonly, of LRIG2. Little is known about the biology of the respective encoded proteins, heparanase 2 and leucine-rich repeats and immunoglobulin-like domains 2. Nevertheless, the observations that heparanase 2 can bind heparan sulphate proteolgycans and inhibit heparanase 1 enzymatic activity and that LRIG2 can modulate receptor tyrosine kinase growth factor signalling each point to biological roles relevant to tissue differentiation. Moreover, both heparanase 2 and LRIG2 proteins are detected in autonomic nerves growing into fetal bladders. The collective evidence is consistent with the hypothesis that urofacial syndrome genes code for proteins which work in a common pathway to facilitate neural growth into, and/or function within, the bladder. This molecular pathway may also have relevance to our understanding of the pathogenesis of other lower tract diseases, including Hinman-Allen syndrome, or non-neurogenic neurogenic bladder, and of the subset of individuals who have primary vesicoureteric reflux accompanied by bladder dysfunction.

Objective: Fetal megacystis has a poor prognosis. During the first trimester, it is frequently associated with chromosomal abnormalities or multiple malformations, but can also be isolated and resolve spontaneously. In this study, our main objective was to determine the fetal and pediatric prognosis in this particular situation. Methods: This was a retrospective multicenter study. We describe the cases referred to our fetal medicine centers and also cases previously reported in the international literature. Results: Five cases were referred to our fetal medicine centers. After spontaneous resolution of megacystis, close ultrasound follow-up revealed urinary tract abnormalities in all cases. These abnormalities were all transient. After birth, 1 of the children developed a mild ureteropelvic junction obstruction. Pediatric follow-up was normal for the other children. Our 5 cases plus 79 in the literature mean that 84 cases of isolated first-trimester fetal megacystis with spontaneous resolution have been reported to date. The risk of chromosomal abnormality was 2.4% (2/84) and pediatric follow-up was normal in 96.4% of cases (81/84). Conclusion: Even when isolated first-trimester fetal megacystis resolves spontaneously, fetal karyotype analysis and close prenatal ultrasound follow-up should be performed. When there is no chromosomal abnormality, the renal pediatric prognosis seems to be good.

To examine the outcome of fetuses with megacystis treated with vesicoamniotic shunting (VAS) from 14 weeks onward.Retrospective review of all fetuses that received VAS at two centres from 2004 to 2012.53 fetuses with megacystis were included in the study. Mean gestational age at diagnosis was 16.4 weeks. Mean gestational age at first shunt placement was 17.8 weeks. The first shunt placement was performed before 16 weeks in 18 (34 %) cases. The mean number of shunts was 1.38. Dislocation occurred in 35 (66 %). TOP was performed in 21 (39.6 %), intrauterine death occurred in two (3.8 %) and spontaneous abortion in three cases (5.7 %). Of the 27 (50.9 %) live births, 17 (32.1 %) infants survived. Normal renal function was present in 10 cases, 4 have compensated renal failure and 3 infants had renal transplantation. Oligohydramnios was significantly associated with non-survival and renal insufficiency. The gestational age at VAS was neither correlated with renal function after birth nor with the survival in our cohort. Conversely, the interval between first shunt placement and delivery was positively correlated with survival and normal renal function. The gestational age at delivery was significantly higher in survivors and those born with normal renal function.Despite intervention, the morbidity and mortality of megacystis is still high. We failed to demonstrate that early intervention is associated with an improved rate of normal renal function after birth. Oligohydramnios was the only parameter identifying fetuses with unfavourable outcome, while all other parameters were inconclusive.

Objective: In this study, we reported a case of a temporary isolated fetal megacystis presenting in the first trimester which resulted in intrauterine death of the fetus at term. Case: A 26-year-old pregnant patient was referred to our clinic at her 12th gestational week for double test. The initial prenatal ultrasonography revealed an isolated megacystis measuring 11.2 mm longitudinally. Chorionic villus sampling was offered for fetal karyotyping but the patient did not accept. Afterwards, the megacystis regressed spontaneously at 14th gestational week. No other fetal anomaly was observed throughout gestation and the pregnancy went without any adverse problems. Sudden intrauterine fetal death was observed at 37th gestational week. The fetus was observed as normal macroscopically on postpartum examination and again fetal karyotyping could not be done due to rejection of the procedure by the family. Conclusion: Contrary to our case, fetal karyotyping should be carried out in order to diagnose the etiology and to fulfill followup on fetus accurately whenever fetal megacystis is diagnosed during the first trimester. Birinci trimesterde gorulen gecici ve izole fetal megasistis ucuncu trimesterde ac›klanamayan intrauterin fetal olume neden olabilir mi? Olgu sunumu ve literatur ozeti

Fetal megacystis, an abnormally enlarged bladder identified at any gestational age, may be transient and resolve spontaneously or may be an early manifestation of bladder outlet obstruction. Anterior urethral valves are the most common cause of the rare condition of congenital obstruction of the anterior urethra. Herein is reported the prenatal observation and postnatal management of a case of megacystis with anterior urethral valves. The etiology is reviewed and strategies recommended for patients with these rare entities.

Objective. The purpose of this series was to report the first-trimester sonographic findings, antenatal course, and outcome in fetuses with a patent urachus. Methods. We conducted a review of ultrasound reports and medical charts from 3 pregnancies complicated by a congenital patent urachus detected in the first trimester. Results. All 3 fetuses had megacystis and an umbilical cord cyst close to the fetal abdominal insertion that was detected in the first trimester. In 2 cases, the megacystis resolved spontaneously while the umbilical cord cyst worsened in appearance; among them, massive edema of the umbilical cord was documented in 1, and evidence of partial bladder exstrophy was detected in the third trimester in the other. Both cases required neonatal surgery with no complications. In the remaining case, the fetus died in the early second trimester. Postmortem examination confirmed the diagnosis of a patent urachus, an allantoic cyst, and thrombosis of the umbilical vessels. Conclusions. Megacystis is a warning sign of a patent urachus in the first trimester. The prognosis of this condition is generally good; however, fetal death can occur secondary to compression of umbilical vessels due to the expanding allantoic cyst. Key words: allantoic cyst; fetal megacystis; fetal sonography; patent urachus; prenatal diagnosis; umbilical cord.

Anterior urethral valves are a rare congenital anomaly associated with distal urethral obstruction, which can result in a poor prognosis. We report on the endoscopic creation of a fetal urethrotomy for obstructive uropathy resulting from anterior urethral valves. A 33-year-old woman was evaluated at 17 weeks gestation due to fetal megacystis. The diagnosis of anterior urethral valves was confirmed by the characteristic sonographic feature of a dilated membranous penile urethra. Oligohydramnios with normal-appearing kidneys and favorable urinary electrolytes led to fetal intervention. Ablation on the ventral site of the fetal penis for a cutaneous urethrotomy was performed using a YAG laser under a 1-mm fetoscope at 19 weeks gestation. Urine was drained from the incision and the dilated penis and the distended bladder shrunk with an increase in amniotic fluid. However, the fetus died unexpectedly on postoperative day 3, and chorioamnionitis was suspected as the etiology. While the outcome was unfavorable, our preliminary experience shows that fetal urethrotomy for obstructive uropathy can be achieved in utero using an endoscopic laser approach. Further experience will be required to evaluate the therapeutic value of this new procedure in the management of fetal anterior urethral valves.

Aims To examine the prevalence of chromosomal defects and outcome of fetuses with megacystis at 10-14 weeks of gestation.Methods At the 10-14-week scan fetal megacystis was defined by a lontitudinal bladder diameter of 7 mm or more. In 145 such fetuses the fetal karyotype and pregnancy outcome were examined in relation to the longitudinal diameter of the fetal bladder.Results Chromosomal defects, mainly trisomies 13 and 18, were present in 30 cases. In the group with longitudinal bladder diameter of 7-15 mm the incidence of chromosomal defects was 23.6% (26/110), whereas in those with bladder diameter >15 mm the incidence was 11.4% (4/35). The fetal nuchal translucency (NT) was above the 95th centile of the normal range for crown-rump length in a higher proportion of cases with abnormal rather than normal karyotype (76.7% compared to 31.3%; Chi-square P 15 mm demonstrated spontaneous resolution of the megacystis.Conclusions In fetal megacystis with longitudinal bladder diameter of 7-15 mm there is a risk of about 25% that the fetus will have a chromosomal defect but in the chromosomally normal group there is spontaneous resolution of the megacystis in about 90% of cases. If the bladder diameter is > 15 mm the risk of chromosomal defects is about 10% and in the chromosomally normal group the condition is invariably associated with progressive obstructive uropathy. Copyright (C) 2003 ISUOG. Published by John Wiley Sons, Ltd.

OBJECTIVE: To present the outcome of a consecutive series of 19 fetuses referred to our center for megacystis in the first half of the pregnancy. STUDY DESIGN: Retrospective analysis. METHODS: 19 cases of early fetal megacystis were reviewed. Inclusion criteria were the visualization of an enlarged bladder with a maximum longitudinal diameter >10 mm and a gestational age of no more than 19 weeks. RESULTS: The median gestational age at diagnosis was 16 weeks (range 12-19 weeks). The median maximum longitudinal diameter of the fetal bladder at the time of diagnosis was 21 mm (range 11-35 mm). The fetal megacystis was associated with another extra renal anomaly in six cases (30%). There was no fetus with abnormal karyotype. Termination of pregnancy was performed in 11 cases because of the severity of the renal disease. One spontaneous intrauterine death occurred. A vesicoamniotic shunt was inserted in three cases, two of which died in utero. The surviving fetus that was shunted died in the neonatal period from acute renal failure. In the remaining four fetuses, resolution of the megacystis occurred spontaneously (n = 2) or following serial single-needle aspiration (n = 2). One of these had renal insufficiency requiring kidney transplantation. CONCLUSION: The outcome of fetuses with early obstructive uropathy is poor, with or without in utero therapy. Pathophysiology, clinical presentation and outcome of fetuses with early uropathy may differ from those previously described in the latter part of pregnancy.

RATIONALE The pathogenesis of fetal megacystis is divided into obstructive and nonobstructive. Megacystis combined with chromosomal abnormalities is rare and most of the cases are nonobstructive. PATIENT CONCERNS The fetus showed posterior urethral obstructive megacystis with features of bladder enlargement, "keyhole" feature, and thick bladder wall. DIAGNOSES Here, we present a case of fetal megacystis diagnosed by ultrasound at pregnancy week 15+2 and with multisystem abnormalities. OUTCOMES Moreover, the fetus showed edema, umbilical cord cyst, cardiac dysplasia, hook-shaped hand, and strephenopodia. These abnormalities strongly suggested chromosomal abnormalities. The fetus was diagnosed with trisomy 18 by amniocentesis. Posterior urethral obstructive megacystis was confirmed by pathology. LESSONS In conclusion, this case suggests that in the presence of fetal megacystis and multisystem abnormalities, causes should be investigated and the possibility of chromosomal abnormalities should be considered in the presence of multisystem developmental abnormalities.

This report of four cases shows that fetal vesicocentesis at 10-14 weeks of gestation is a useful treatment option in some cases with megacystis. Although megacystis at this gestation has been reported, there are few studies examining the role of early vesicocentesis. The natural history of the condition and outcome following treatment is reviewed with reference to the literature.

Objective The purpose of this study was to evaluate the prognostic criteria of early fetal megacystis. Design A prospective, transvaginal ultrasound, crosssectional study at 11–15 weeks of gestation at a tertiary referral fetal medicine unit. Subjects and methods Sixteen pregnancies out of a total of 5240 were identified with early fetal megacystis. Fetal biometry, morphology, amniotic fluid, bladder size and volume were also evaluated. The karyotype was available in 15 cases. Vesicocentesis was performed in six fetuses and three had concomitant cystoscopies. Results In six fetuses, the megacystis was isolated. In the remaining ten, we detected associated hygroma (n = 5), nuchal translucency (n = 3), omphalocele (n = 1), mild pyelectasis (n = 1) and bilateral talipes (n = 1). In three cases the fetuses demonstrated renal hyperechogenicity with cysts, and in two cases oligohydramnios was found; four cases (25%) had chromosomal abnormalities; 47,XY + 13 (two cases), 47,XY + 18 and 47,XY + 21. Only one fetus from this study survived. In the remaining 13 cases, termination was proposed after counselling of the patients on the poor prognosis. The mean gestational age at termination was 15.5 ± 2.4 weeks (range 12–20). Three fetal transabdominal cystoscopies did not allow us to view the valves; one urethral atresia was suspected, and confirmed postnatally. Conclusions We found a high rate of associated malformations, especially intestinal malformations. The systematic evaluation of the intestinal enzymes in the amniotic fluid and urine samples might be an important aid in the diagnosis of multiple malformations, such as cloacal dysgenesis.

A 2,510 g female newborn was delivered by Cesarean section at 33 weeks gestation due to increasing volume of her huge bladder and bilateral hydronephrosis. Just after birth 190 ml of urine was drainaged by catheterization without difficulty. Voiding cystourethrography showed no VUR. She had no ureteral dilation, either. Urodynamic study revealed large bladder capacity and detrusor hypocontractility. Neither neurological nor gastrointestinal abnormality was detected neonatally. Clean intermitted catheterization was performed every 4 hours. At 12 months of her age she had no history of urinary tract infection. However, as she grew up with normal diet, severe constipation became apparent. This is a case of idiopathic intestinal pseudo-obstruction syndrome whose gastrointestinal symptom was masked in the newborn period. Attention should be paid for gastrointestinal tract as well as urinary tract when the body has congenital megacystis with unknown etiology.

Objectives: Megacystis (MC) is rare and often associated with other structural and chromosomal anomalies. In euploid cases with early oligohydramnios, prognosis is poor mainly due to pulmonary hypoplasia and renal damage. We report our experience of the past 20 years. Methods: A retrospective review of cases with prenatally diagnosed MC was performed. Complete prenatal as well as postnatal medical records from 1989 to 2009 were reviewed focusing on diagnostic precision, fetal interventions [vesicocentesis (VC), vesicoamniotic shunt (VAS)], short- and long-term outcome, and potential prognostic factors. Results: 68 cases were included. Follow-up was available in 54 cases (9 girls and 45 boys including 3 cases with aneuploidy). We found 39 isolated MC at sonography (5 girls and 34 boys). 24 fetuses with isolated MC underwent VC and VAS at 19.6 ± 6.3 and 20 ± 4.9 weeks of gestation, respectively. Survival rate was higher in male than in female fetuses (51 vs. 33%). Renal problems occurred in 4/14 prenatally treated fetuses and in 1/10 when cases with prune belly syndrome (PBS) were excluded from the analysis. Conclusions: Our study shows that a careful selection of cases with MC excluding fetuses with PBS and early treatment has still the potential to improve outcome.

During the study period, 24,492 pregnant women attended the Harris Birthright Research Centre at 10-14 weeks of gestation, at which time, in addition to the measurements of nuchal translucency thickness and crown-rump length (CRL), data on fetal abnormalities were recorded onto a computer database. Cases of megacystis were identified and the records were reviewed. Additionally, the relationship of the longitudinal bladder diameter with the CRL and the bladder diameter/CRL ratio (expressed as a percentage) were examined with the use of data from 300 normal fetuses at 10-14 weeks. Megacystis was present in 15 of the 24,492 pregnancies (1 in 1,633) and in these cases the minimum longitudinal bladder diameter was 8 mm and the minimum bladder diameter/CRL ratio was 13%. In the 300 control fetuses the bladder was visualized in 278 (92.7%) of the cases and the longitudinal bladder diameter increased with the CRL (bladder diameter = 0.065 x CRL - 0.69; r = 0.47, p < 0.001), none of the measurements was more than 6 mm and the median bladder diameter/CRL ratio was 5.4% (range 0-10.4%) which did not change significantly with gestation (r = 0.1, p = 0.09). The bladder was visible in all cases with a minimum CRL of 67 mm. In three of the 15 cases with megacystis, there were chromosomal abnormalities. In the chromosomally normal group, there were seven cases with spontaneous resolution, whereas in four cases there was progression to severe obstructive uropathy. The bladder diameter was 8-12 mm and the bladder diameter/CRL ratio 13-22% in all cases with resolution and in one case with progressive megacystis; in the other three cases with progressive obstruction, the bladder length was more than 16 mm and the bladder diameter/CRL ratio was more than 28%.

Isolated congenital megacystis represents a rare variant of fetal megacystis without other associated anomalies. The etiology is unclear, and various management strategies have been proposed. We report a case of isolated congenital megacystis that resolved over the first year of life with observation alone. Considerations for the evaluation and management of this rare entity are discussed.

The purpose of our retrospective observational series was to determine whether the sonographic characteristics of fetal megacystic bladders can be used to reliably establish the most likely diagnosis in fetuses with this condition. The sonographic records of pregnant patients referred to our institutions over a 10-year period who were found on initial 2-dimensional sonography to be carrying fetuses with megacystis were examined for evidence of a keyhole sign, bladder thickness, amniotic fluid index, and fetal sex. When available, 3-/4-dimensional sonography, Doppler angiography, tomographic ultrasound imaging, virtual organ computer-aided analysis, and automatic volume calculation were used as part of the detailed fetal anatomic survey. Twenty fetuses with megacystis were identified. Seventeen were male; 2 were female; and 1 had ambiguous genitalia. All male fetuses with megacystis originally had a diagnosis of prune belly syndrome. The diagnosis for 10 male fetuses with a keyhole sign was changed to megacystis secondary to posterior urethral valves. The fetus with ambiguous genitalia had prune belly syndrome. One of the female fetuses had a diagnosis of urethral atresia, and the diagnosis for the other female fetus was megacystis-microcolon-intestinal hypoperistalsis syndrome. In conclusion, in fetuses with megacystic bladders, it is possible to distinguish between cases with prune belly syndrome, posterior urethral valves, urethral atresia, and megacystis-microcolon-intestinal hypoperistalsis syndrome by a detailed anatomic survey using 2- and 3-/4-dimensioinal sonographic techniques.

Wereport on an innovative therapeutic attempt in a case with obstructive uropathia in a fetus. Placement of an an terograd urethral stent was performed in the 26 th gestational week. Transurethral catheterization via the fetal megacystis, the urethra and the orificium externum of the penis was feasible during fetal endoscopy (Fetendo). During serial ultrasound scans from the 27 th to the 36 th gestational week a normalization of the amniotic fluid volume was observed. The postpartal evaluation demonstrated that the kidney structure, values of kreatinin serum levels and renal clearance were in the normal range. The boy's development after the seventh month is completely normal. Fetendo with urethral stent application for obstructive uropathy should be performed for the carefully selected patient with oligohydramnion and normal kidney appearance.

Ultrasound routinely identifies the fetal bladder from 10 weeks of gestation. Understanding the normal embryology of the fetal bladder forms the basis of understanding the mechanisms for pathology. Early onset megacystis

Background: Fetal obstructive uropathies complicated by severe oligohydramnios can cause neonatal death due to renal dysplasia and pulmonary hypoplasia. Case: A 31-year-old multigravida was referred at 19.6 weeks with sonographic evidence of fetal megacystis and bilateral hydroureteronephrosis, and severe oligohydramnios. A vesicoamniotic shunt was placed for urinary diversion. Complications included shunt migration, intestinal evisceration, amniorrhexis, preterm labor, and preterm delivery. Bowel characteristics were assessed by measuring the intestinal lumen diameter for evidence of obstruction. The lumen diameter remained stable at 3–5 mm, and the luminal wall remained stable at 2.5 mm. After birth at 31 weeks, the infant underwent exploratory laparotomy and extraction of malpositioned shunts, vesicostomy, and closure of abdominal wall defect. He was discharged at 61 days of life after resolution of neonatal pulmonary and infectious complications. Conclusion: Operative management of complete fetal obstructive uropathy is beneficial despite potential complications.