Fetal lower urinary tract obstruction: international Delphi consensus on management and core outcome set.

OBJECTIVES: To reach an international expert consensus on the diagnosis, prognosis and management of fetal lower urinary tract obstruction (LUTO) by means of a Delphi procedure, and to use this to define a core outcome set (COS). METHODS: A three-round Delphi procedure was conducted among an international panel of experts in fetal LUTO. The panel was provided with a list of literature-based parameters to consider for the diagnosis, prognosis, management and outcomes of LUTO. A parallel procedure was conducted with patient groups during the development of the COS. RESULTS: A total of 168 experts were approached, of whom 99 completed the first round and 80/99 (80.8%) completed all three rounds of the study questionnaires. Consensus was reached that, in the first trimester, an objective measurement of longitudinal bladder diameter of ≥ 7 mm should be used to suspect LUTO. In the second trimester, imaging parameters suggestive of LUTO could include enlarged bladder, keyhole sign, bladder wall thickening, bilateral hydronephrosis, bilateral hydroureteronephrosis and male sex. There was 79% agreement that the current prognostic scoring systems in the literature should not be used clinically. However, experts agreed on the value of amniotic fluid volume (at < 24 weeks) to predict survival and that the value of fetal intervention is to improve the chance of neonatal survival. Experts endorsed sonographic parameters suggestive of renal dysplasia, at least one vesicocentesis, and renal biochemistry for prognosis and counseling, but these items did not reach a consensus for determining candidacy for fetal intervention. On the other hand, imaging parameters suggestive of LUTO, absence of life-limiting structural or genetic anomalies, gestational age of ≥ 16 weeks and oligohydramnios (defined as deepest vertical pocket < 2 cm) should be used as candidacy criteria for fetal intervention based on expert consensus. If bladder refill was evaluated, it should be assessed subjectively. Vesicoamniotic shunt should be the first line of fetal intervention. In the presence of suspected fetal renal failure, serial amnioinfusion should be offered only as an experimental procedure under research protocols. A COS for future LUTO studies was agreed upon. CONCLUSION: International consensus on the diagnosis, prognosis and management of fetal LUTO, as well as the COS, should inform clinical care and research to optimize perinatal outcomes. © 2024 The Author(s). Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Percutaneous fetoscopic spina bifida repair: effect on ambulation and need for postnatal cerebrospinal fluid diversion and bladder catheterization.

OBJECTIVE: A trial comparing prenatal with postnatal open spina bifida (OSB) repair established that prenatal surgery was associated with better postnatal outcome. However, in the trial, fetal surgery was carried out through hysterotomy. Minimally invasive approaches are being developed to mitigate the risks of open maternal-fetal surgery. The objective of this study was to investigate the impact of a novel neurosurgical technique for percutaneous fetoscopic repair of fetal OSB, the skin-over-biocellulose for antenatal fetoscopic repair (SAFER) technique, on long-term postnatal outcome. METHODS: This study examined descriptive data for all patients undergoing fetoscopic OSB repair who had available 12- and 30-month follow-up data for assessment of need for cerebrospinal fluid (CSF) diversion and need for bladder catheterization and ambulation, respectively, from eight centers that perform prenatal OSB repair via percutaneous fetoscopy using a biocellulose patch between the neural placode and skin/myofascial flap, without suture of the dura mater (SAFER technique). Univariate and multivariate logistic regression analyses were used to examine the effect of different factors on need for CSF diversion at 12 months and ambulation and need for bladder catheterization at 30 months. Potential cofactors included gestational age at fetal surgery and delivery, preoperative ultrasound findings of anatomical level of the lesion, cerebral lateral ventricular diameter, lesion type and presence of bilateral talipes, as well as postnatal findings of CSF leakage at birth, motor level, presence of bilateral talipes and reversal of hindbrain herniation. RESULTS: A total of 170 consecutive patients with fetal OSB were treated prenatally using the SAFER technique. Among these, 103 babies had follow-up at 12 months of age and 59 had follow-up at 30 months of age. At 12 months of age, 53.4% (55/103) of babies did not require ventriculoperitoneal shunt or third ventriculostomy. At 30 months of age, 54.2% (32/59) of children were ambulating independently and 61.0% (36/59) did not require chronic intermittent catheterization of the bladder. Multivariate logistic regression analysis demonstrated that significant prediction of need for CSF diversion was provided by lateral ventricular size and type of lesion (myeloschisis). Significant predictors of ambulatory status were prenatal bilateral talipes and anatomical and functional motor levels of the lesion. There were no significant predictors of need for bladder catheterization. CONCLUSION: Children who underwent prenatal OSB repair via the percutaneous fetoscopic SAFER technique achieved long-term neurological outcomes similar to those reported in the literature after hysterotomy-assisted OSB repair. © 2021 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Percutaneous fetoscopic closure of large open spina bifida using a bilaminar skin substitute.

OBJECTIVE: We have described previously our percutaneous fetoscopic technique for the treatment of open spina bifida (OSB). However, approximately 20-30% of OSB defects are too large to allow primary skin closure. Here we describe a modification of our standard technique using a bilaminar skin substitute to allow closure of large spinal defects. The aim of this study was to report our clinical experience with the use of a bilaminar skin substitute and a percutaneous fetoscopic technique for the prenatal closure of large OSB defects. METHODS: Surgery was performed between 24.0 and 28.9 gestational weeks with the woman under general anesthesia, using an entirely percutaneous fetoscopic approach with partial carbon dioxide insufflation of the uterine cavity, as described previously. If there was enough skin to be sutured in the midline, only a biocellulose patch was placed over the placode (single-patch group). In cases in which skin approximation was not possible, a bilaminar skin substitute (two layers: one silicone and one dermal matrix) was placed over the biocellulose patch and sutured to the skin edges (two-patch group). The surgical site was assessed at birth, and long-term follow-up was carried out. RESULTS: Percutaneous fetoscopic OSB repair was attempted in 47 consecutive fetuses, but surgery could not be completed in two. Preterm prelabor rupture of membranes (PPROM) occurred in 36 of the 45 (80%) cases which formed the study group, and the mean gestational age at delivery was 32.8 ± 2.5 weeks. A bilaminar skin substitute was required in 13/45 (29%) cases; in the remaining 32 cases, direct skin-to-skin suture was feasible. There were 12 cases of myeloschisis, of which 10 were in the two-patch group. In all cases, the skin substitute was located at the surgical site at birth. In five of the 13 (38.5%) cases in the two-patch group, additional postnatal repair was needed. In the remaining cases, the silicone layer detached spontaneously from the dermal matrix (on average, 25 days after birth), and the lesion healed by secondary intention. The mean operating time was 193 (range, 83-450) min; it was significantly longer in cases requiring the bilaminar skin substitute (additional 42 min on average), although the two-patch group had similar PPROM rate and gestational age at delivery compared with the single-patch group. Complete reversal of hindbrain herniation occurred in 68% of the 28 single-patch cases and 33% of the 12 two-patch cases with this information available (P < 0.05). In four cases there was no reversal; half of these occurred in myeloschisis cases. CONCLUSIONS: Large OSB defects may be treated successfully in utero using a bilaminar skin substitute over a biocellulose patch through an entirely percutaneous approach. Although the operating time is longer, surgical outcome is similar to that in cases closed primarily. Cases with myeloschisis seem to have a worse prognosis than do those with myelomeningocele. PPROM and preterm birth continue to be a challenge. Further experience is needed to assess the risks and benefits of this technique for the management of large OSB defects. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Management of twin-twin transfusion syndrome in pregnancies with iatrogenic detachment of membranes following therapeutic amniocentesis and the role of interim amniopatch.

OBJECTIVE: Detachment of membranes may occur after therapeutic amniocentesis for twin-twin transfusion syndrome (TTTS). Subsequent amniocenteses or endoscopic fetal therapy may be hindered or made altogether impossible by this complication. The purpose of this study was to describe our experience in the assessment and management of TTTS patients with iatrogenic detached membranes (IDM). METHODS: Patients with IDM referred for fetal surgery for TTTS were considered ineligible for standard surgery and were offered different alternatives, including expectant management, serial amniocentesis, or an attempt at surgery with or without prior amniopatch. Pregnancy outcomes were compared between surgical and non-surgical patients. RESULTS: Nine hundred and forty-four patients with a diagnosis of TTTS were referred between July 1997 and December 2004, of whom 322 (34.1%) had a prior therapeutic amniocentesis. Twenty-six of the 322 patients (8%) had IDM. Ten patients opted to be managed with subsequent amniocenteses, two of which had an amniopatch. One patient had voluntary interruption of pregnancy. Fifteen patients underwent surgery, 10 of whom underwent an amniopatch. Overall, resealing of membranes occurred in 8/12 (66%) patients treated with an amniopatch. Survival of at least one fetus was greater in patients treated surgically with or without an amniopatch (12/15, 80% vs. 4/11, 36%, P = 0.04). CONCLUSION: Membrane detachment is an important complication of therapeutic amniocentesis in the treatment of TTTS. Although successful treatment of IDM can be achieved with an interim amniopatch, this alternative is not without risks. Therapeutic amniocenteses should be discouraged in patients considering endoscopic fetal surgery for TTTS.

Ultrasound assessment of venous blood flow before and after laser therapy: approach to understanding the pathophysiology of twin-twin transfusion syndrome.

OBJECTIVE: To estimate direction and volume of blood exchange between the donor twin and recipient twin by ultrasound assessment of blood flow in the umbilical vein before and after selective laser photocoagulation of communicating vessels (SLPCV) for twin-twin transfusion syndrome (TTTS). METHODS: Forty-one TTTS patients underwent Doppler examination of the umbilical vein before and 24 h after SLPCV. The diameter and mean time-averaged velocity of the umbilical vein were estimated. Total umbilical venous flow (TUVF) was calculated as follows: TUVF (mL/min) = mean time-averaged velocity (cm/s) x mean cross-sectional area (cm2) x 60 (s). RESULTS: TUVF was significantly higher in the recipient (111.2 mL/min) than in the donor twin (44.8 mL/min) before SLPCV (P < 0.0001). However, TUVF was no different between the recipient and the donor twin after SLPCV (93.1 vs. 70.7 mL/min, recipient and donor twin, respectively, P = 0.11). The donor twin's TUVF increased after surgery (P < 0.0001), while the recipient twin's TUVF decreased (P = 0.041). The median postoperative increase in the donor twin's TUVF of 25.9 mL/min had a corresponding decrease of TUVF in the recipient twin of 18.1 mL/min (P = 0.27). CONCLUSIONS: Our data suggest that untreated TTTS is characterized by excessive umbilical venous blood flow in the recipient twin relative to the donor twin. Laser surgery results in concordant changes in umbilical venous flow in opposite directions between the donor and recipient twins, eliminating the initial imbalance. Our results lend support to the fundamental hypothesis of unbalanced blood flow exchange (net flow from donor to recipient) between monochorionic twins as the cause for TTTS and that laser surgery eliminates the pathophysiological cause.

The role of Doppler studies in predicting individual intrauterine fetal demise after laser therapy for twin-twin transfusion syndrome.

OBJECTIVE: To investigate the role of Doppler studies in predicting individual fetal demise in patients scheduled for selective laser photocoagulation of communicating vessels (SLPCV) for twin-twin transfusion syndrome (TTTS). METHODS: Doppler studies of the umbilical artery, umbilical vein, ductus venosus, tricuspid valve regurgitation and middle cerebral artery were performed in the donor and recipient twins before and 24 hours after SLPCV. Results were analyzed cross-sectionally and longitudinally. As multiple comparisons were made, an a priori alpha rejection was set at P < 0.001. RESULTS: One hundred and ten consecutive patients were available for analysis. Overall fetal survival was 68.6% (151/220) with at least one survivor in 88.2% (97/110) of cases. Absent or reversed end-diastolic velocity in the umbilical artery of the donor twin was the only preoperative Doppler result predictive of intrauterine fetal demise (IUFD) (10/15, 66.7%, P < 0.001). Postoperatively, reversed flow during atrial contraction in the ductus venosus of the donor twin showed a trend towards prediction of IUFD of this fetus (4/5, 80%, P = 0.007). No other Doppler studies, including the longitudinal analyses, were predictive of IUFD. CONCLUSIONS: Our data suggest that preoperative absent or reversed end-diastolic velocity in the umbilical artery may be useful in predicting individual fetal demise of the donor twin in TTTS patients scheduled for SLPCV. This may reflect the role of decreased individual placental mass that may be associated with some donor twins. The inability of other Doppler studies to predict individual IUFD may be explained preoperatively by the effect of the interfetal vascular connections on the individual Doppler signals and postoperatively by the effect of surgery or the timing of the assessment. Our findings may be important in patient counseling, in furthering understanding of the disease, and perhaps in improving surgical technique.

In vivo endoscopic assessment of arterioarterial anastomoses: insight into their hemodynamic function.

OBJECTIVE: To assess endoscopically the hemodynamic function of arterioarterial (AA) anastomoses in twin-twin transfusion syndrome (TTTS) and monochorionic selective intrauterine growth restriction (IUGR). MATERIALS AND METHODS: The videotapes of TTTS and IUGR patients undergoing laser surgery between July 1997 and December 2001 were reviewed for the presence of AA anastomoses. The hemodynamic equator was defined as the site within the AA anastomosis with color flashing. AA anastomoses were classified as having unidirectional flow, having bi-directional flow, or being non-functional, depending on whether the hemodynamic equator reached a returning vein to one, both, or neither twin, respectively. TTTS was classified in stages as previously described. RESULTS: AA anastomoses were present in 35/183 (19.1%) of TTTS and in 12/24 (50%) IUGR patients. Of these, the hemodynamic equator was visible in 8/35 (22.8%) TTTS patients (all in stage III, and mostly in atypical stage III) and in 6/12 (50%) IUGR patients (overall 14/47, 29.8%). Of the 14 patients with a visible hemodynamic equator, 13 (92.8%) AA anastomoses showed unidirectional (9/13, 69.2% from the smaller to the larger twin) flow, and only 1/14 (7.1%) showed bi-directional flow. CONCLUSION: The hemodynamic equator is visible in approximately 30% of patients with AA anastomoses. Within this group, most AA anastomoses behave as functional arteriovenous anastomoses, and the direction of flow can be from the smaller to the larger twin or vice versa. The data suggest a correlation between sonographic findings and placental vascular design, also implying possible interfetal oxygenation differences. Further assessment of the functional behavior of AA anastomoses is warranted to understand the pathophysiology of TTTS and selective IUGR.

Operative fetoscopy via telesurgery.

We describe a case in which telesurgical consultation from Tampa, Florida, USA was used to accomplish operative fetoscopy in Santiago, Chile for the treatment of a twin pregnancy involving an acardiac twin. The procedure was successful and a healthy infant was delivered at 37.5 weeks. Operative fetoscopy, a surgical approach to correct birth defects in utero via combined ultrasound and endoscopy, is only available in a handful of centers worldwide. Telesurgery makes use of telecommunication to allow a surgeon at a primary operating site to consult with another experienced surgeon for complex surgical cases. This case illustrates the potential for ultrasound and telesurgery to expand the horizons of operative fetoscopy.

Selective photocoagulation of communicating vessels in the treatment of monochorionic twins with selective growth retardation.

OBJECTIVE: Current treatment of patients with selective intrauterine growth retardation in monochorionic twins includes expectant management, termination of pregnancy, or umbilical-cord occlusion. The purpose of this study was to assess the outcome of monochorionic twins with selective intrauterine growth retardation who were treated with selective laser photocoagulation of the communicating vessels. STUDY DESIGN: Monochorionic twin pregnancies with selective intrauterine growth retardation at less than 26 weeks were eligible for the study. Selective intrauterine growth retardation was defined as <10th percentile for gestational age. Absent or reverse end-diastolic velocity in the umbilical artery of the twin with selective intrauterine growth retardation was required for eligibility after January 2000. RESULTS: Thirty patients met the criteria for the study: 17 patients were treated expectantly (group I); 2 patients underwent umbilical-cord ligation of the twin with selective intrauterine growth retardation, and 11 patients underwent selective laser photocoagulation of the communicating vessels (group II). Survival rates for at least 1 fetus were no different between groups I and II (14/17 [82.3%] vs 8/11 [72.3%]; P = .4). However, concomitant demise of the co-twin occurred in 4 of 7 patients, and iatrogenic premature delivery for deterioration of the twin with selective intrauterine growth retardation was necessary in 2 patients in group I, which resulted in significant neonatal morbidity. Of the live-born babies, neurologic handicap was present in 3 of 22 babies (13.6%) versus 0 of 12 in groups I and II, respectively (P < .0001). CONCLUSION: Selective intrauterine growth retardation in monochorionic twins can be effectively treated with selective laser photocoagulation of the communicating vessels. By unlinking the circulations between the fetuses, the pregnancy is rendered "functionally" dichorionic, which improves pregnancy treatment and results in decreased neonatal morbidity. This approach constitutes a new valuable alternative in the treatment of monochorionic twin pregnancies with selective intrauterine growth retardation. A randomized clinical trial of expectant treatment versus selective laser photocoagulation of the communicating vessels for monochorionic selective intrauterine growth retardation can be considered.

In-utero treatment of fetal bladder-outlet obstruction by a ureterocele.

Fetal bladder-outlet obstruction by ureteroceles is usually treated at birth. However, such obstruction may be detrimental to the health of the fetus and so in-utero correction is preferable. We describe the successful cytoscopy guided laser incision, with no complications, of a uterocele that was causing bladder-outlet obstruction in a fetus of 19 weeks and 6 days gestation.

Selective laser photocoagulation of communicating vessels in severe twin-twin transfusion syndrome in women with an anterior placenta.

BACKGROUND: We describe two techniques for the laser treatment of twin-twin transfusion syndrome in women with anterior placentas. TECHNIQUE: In the first technique, anastomoses were photocoagulated using a flexible endoscope through a single port. The second technique used a side-firing laser fiber with a rigid angled-view endoscope (two ports). EXPERIENCE: Seventy-two women had surgery between July 1997 and December 1999, 35 (48.6%) of whom had anterior placentas. Survival was similar for fetuses with anterior (80%) and posterior (75.6%) placentas, but operating time was significantly longer for those with anterior placentas (81.1 compared with 64.4 minutes for the anterior and posterior placentas, respectively; P = .02, Student t test). At least one fetus survived in 76% (16 of 21) of women treated with flexible endoscopes and 86% (12 of 14) of those treated with the side-firing lasers. Six of 72 women (8.3%) had patent vascular anastomoses on placental examination, and five of them had anterior placentas (P = .08, Fisher exact test). CONCLUSION: Although anterior placentas are surgically more challenging than posterior placentas, both techniques allow an effective percutaneous approach to the laser treatment of twin-twin transfusion syndrome.

New horizons in the treatment of preterm premature rupture of membranes.

In the last 5 years, significant experience has been gained in the clinical understanding and treatment of PPROM. From this work, one can conclude that iatrogenic PPROM is not only a different entity etiologically, but also in its clinical behavior and response to therapeutic measures. The current success rate with the amniopatch allows the author [figure: see text] to suggest that iatrogenic PPROm should no longer be viewed as a devastating complication of pregnancy. Instead, it should be viewed as a readily treatable condition with the aminopatch. Regarding spontaneous PPROM, work suggests that perhaps, in a select group of patients in which infection has not set in and in which a smaller and better defined membrane defect may exist, there is the potential for developing a surgical technique that may successfully graft the defect and allow the integrity of the amniotic membrane to be restored. The actual surgical technique, the materials to be used, and the approach (transabdominal versus transcervical) are all areas of research for the future. Meanwhile, continued efforts are warranted to decipher whether this obstetric complication can one day be conquered.

Fetal hydrolaparoscopy and endoscopic cystotomy in complicated cases of lower urinary tract obstruction.

OBJECTIVE: Vesicoamniotic shunting may be difficult or impossible in selected cases of fetal lower obstructive uropathy. The purpose of this article is to describe the performance of fetal hydrolaparoscopy and endoscopic fetal cystotomy in two fetuses with complicated lower obstructive uropathy. STUDY DESIGN: Fetal hydrolaparoscopy-endoscopic fetal cystotomy was performed in a patient with a markedly thickened bladder that could not be entered percutaneously. A peritoneoamniotic (bridge) shunt was also placed. Fetal hydrolaparoscopy-endoscopic fetal cystotomy was performed in a second patient with a collapsed bladder from a previous vesicocentesis, because vesicoinfusion resulted in further ascites. Fetal cystoscopy was performed after endoscopic fetal cystotomy, and posterior urethral valves were ablated with neodymium:yttrium-aluminum-garnet laser energy. A vesicoamniotic shunt was left in place. RESULTS: Adequate bladder drainage was obtained in both cases. The first baby required bilateral nephrotomy and a permanent cystotomy at birth and is scheduled for a bladder expansion procedure at the age of year. The second patient had premature rupture of membranes and fetal death from treatment of this complication 5 days after the original procedure. CONCLUSION: Fetal hydrolaparoscopy-endoscopic fetal cystotomy can be performed in complicated cases of lower obstructive uropathy. The procedure involves the creation of a defect in the bladder dome under direct endoscopic visualization within a spontaneous or intentional hydroperitoneum. Peritoneoamniotic shunting, vesicoamniotic shunting, or ablation of posterior urethral valves may then be performed. Fetal hydrolaparoscopy-endoscopic fetal cystotomy should be reserved only for complicated cases of lower obstructive uropathy in which conventional vesicoamniotic shunting is not safely possible. Further experience with fetal hydrolaparoscopy-endoscopic fetal cystotomy is necessary to establish its risks and benefits.

Successful in utero endoscopic ablation of posterior urethral valves: a new dimension in fetal urology.

Fetal lower urinary tract obstructive uropathy, when associated with oligohydramnios, is usually associated with a poor outcome. We present a case of successful in utero endoscopic ablation of posterior urethral valves in which the infant survived the neonatal period without evidence of renal dysplasia. The role, indications, and potential benefits of this novel technique are discussed.

Minimally invasive treatment of twin-to-twin transfusion syndrome.

Selective laser photocoagulation of communicating vessels (SLPCV) is an application of minimally invasive endoscopic fetal surgery (i.e., surgical fetoscopy) performed for severe cases of twin-to-twin transfusion syndrome (TTTS), a complication of monochorionic twin pregnancies. Advances in ultrasound and endoscopy have aided the identification and treatment of this potentially lethal or fatal condition. Surgical fetoscopy, an innovation in the field of surgery and maternal-fetal medicine, benefits patients by reducing the morbidity and mortality associated with TTTS, with minimal risks to the mother.

In utero diagnosis of trichothiodystrophy by endoscopically-guided fetal eyebrow biopsy.

OBJECTIVE: To describe the prenatal diagnosis of trichothiodystrophy (TTD) through endoscopically-guided fetal eyebrow biopsy. MATERIALS AND METHODS: A 32-year-old patient, gravida 4, para 3, with a history of 2 previous infants affected with TTD was referred at 17(5)/(7) weeks for fetal hair biopsy. DNA repair studies had been normal in the previous children. Four 1-mm biopsies were obtained from the external aspect of the fetal eyebrows under direct endoscopic guidance. Fetal hair samples were assessed with polarized microscopy, electron microscopy, hematoxylin and eosin staining, and were also sent for analysis of sulfur content (cystine levels). RESULTS: The fetal eyebrows were the only adequate source of hair in the early second trimester. The biopsy samples yielded adequate material for all tests. Polarized microscopy showed characteristic banding patterns, but trichoschisis was not apparent. Cystine levels (19 micromol/l) in the biopsy sample were significantly lower than an age-matched (fresh spontaneous abortion) control (368 micromol/l). CONCLUSION: Prenatal diagnosis of TTD is possible in the second trimester through endoscopically-guided eyebrow biopsy. An adequate amount of hair is present in the eyebrows by then, and the disease is already manifest. Analysis of sulfur content of the hair samples is preferred over polarized or electron microscopy, as many classic microscopic findings of TTD may not be present in the early second trimester.