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Ultrasound was used in three cases to assist the physician in performing fetal puncture for decompression of abnormal fluid collections in the fetus. The use of real-time ultrasound guidance can help prevent trauma to the mother and fetus due to such procedures.

There are compelling physiologic arguments for correcting certain malformations before birth. Although fetal surgery has been successful in sheep and lower animals, it has proven difficult in primates because the gravid uterus is exquisitely sensitive to induction of preterm labor and abortion. Because the feasibility of fetal intervention can be determined only in a primate model, we have investigated the variables affecting fetal-neonatal survival after fetal surgery in 25 monkeys. As we improved our anesthetic and surgical techniques and refined our tocolytic therapy, mortality fell from 73.3% (11/15) to 20% (2/10). Since spontaneous perinatal loss in 56 controls was 21.4% we can now operate on the late second and early third trimester fetal monkey without increasing maternal or fetal-neonatal mortality. Success in this rigorous model is a requisite for attempted correction of human malformations in utero.

The records were reviewed of the 25 neonates found to have hydronephrosis due to primary vesicoureteral reflux at the authors' hospital in the 6 1/2-year period from January 1981 to June 1987. Modes of discovery and investigation, detection of associated lesions, treatment, and errors in both diagnosis and management were evaluated. Twenty-one of the neonates were boys. Fourteen cases were found at fetal screening, and the infants were asymptomatic. Inappropriate maternal/fetal intervention occurred in four cases: Three were incorrectly thought to have posterior urethral valves. The increased number of neonates found to have reflux is attributable to the widespread availability of obstetric ultrasonography and early screening of infants at risk for urinary tract abnormalities. Since the pathophysiologic characteristics of reflux are well understood, appropriate postnatal therapy can be instituted without delay.

Specific diagnoses of fetal genitourinary anomalies can be made with current high resolution ultrasound. Accurate diagnosis is essential if fetal intervention is contemplated.

The ability to diagnose genitourinary abnormalities in the fetus frequently poses management dilemmas for the urologist. Our experience with 13 cases of abnormal fetal ultrasonography examinations thought to be genitourinary in nature underscores difficulties posed by this new technology. In 3 cases the prenatal diagnosis was eventually found to be incorrect. In 1 case, vesicoureteric reflux gave the appearance of hydronephrosis that resolved after birth. In 3 cases in which intervention was deemed necessary, the eventual outcome was unaffected. Prenatal ultrasound is most useful when detecting occult hydronephrosis that would have gone unnoticed in the routine newborn physical examination. However, our patients received no benefit from fetal intervention.

Dilatation of the fetal urinary tract can now be detected by ultrasound imaging in pregnancy. Forty-six cases of pre-natally diagnosed dilatation were analysed to assess the contribution of this information to subsequent urological management. Positive pre-natal scans proved accurate. Dilatation was confirmed by neonatal ultrasound imaging or by autopsy in 24 of 25 consecutive inborn neonates and two aborted fetuses. Forty-four live births were studied. Twelve neonates had physical signs at birth which rendered the pre-natal diagnosis unnecessary. In 32 neonates (73%) the urinary tract anomaly was identified solely as a result of pre-natal imaging and would otherwise have remained undetected. However, in seven neonates the information was of doubtful value (mild unilateral dilatation), in 16 it was of probable value (confirmed unilateral PUJ obstruction, multicystic kidney, etc.) and in only nine neonates (20%) was it of definite value (treatable conditions affecting both kidneys or a solitary kidney, e.g. urethral valves, PUJ obstruction in a solitary kidney, etc.). Pre-natally detected bilateral dilatation (18 infants) carried a poor prognosis--33% mortality and 56% incidence of coexistent abnormalities. No infants in our series had undergone intra-uterine drainage, but the numbers of survivors and their renal function was comparable to the published results for fetal intervention.

Maternal ultrasonography makes it possible to easily detect hydronephrosis in the fetus, and therefore fetal “screening” should be part of every obstetrical ultrasonogram. However, the mere presence of fetal hydronephrosis virtually never means that either fetal intervention or early delivery is warranted. The importance of fetal case-finding is that it enables us to promptly (and electively) evaluate and treat the asymptomatic neonate before infection or other complications occur.

Real-time ultrasonic imaging during pregnancy has made it possible to diagnose accurately fetal hydronephrosis as early as at 17 weeks of gestation. The ultrasonic finding of oligohydramnios in conjunction with megacystis and bilateral hydronephrosis is the most reliable indicator of marked permanent urinary tract obstruction and, therefore, is an absolute requirement for fetal urological intervention. Fetal intervention by means of percutaneous catheter drainage of the bladder into the amniotic space is undertaken to preserve renal and pulmonary function. It appears that urinary tract decompression at 17 weeks of gestation is unable to prevent renal dysplasia. The degree to which renal cortical function is preserved by fetal drainage of the hydronephrotic kidney is unknown. Experimental evidence has suggested that early urinary tract decompression promotes improved pulmonary development, which may allow for normal pulmonary function postnatally. We report 6 cases that illustrate important considerations with regard to urological intervention for fetal hydronephrosis.

During a 6-year period (1979-1985), 142 neonates with significant hydronephrosis were seen. Seventy-eight percent of the cases were discovered on fetal screening during obstetric sonography. Maternal/fetal intervention was virtually never indicated and most babies were asymptomatic. The most common conditions found were obstruction of the ureteropelvic junction (41%), obstruction of the distal ureter (usually primary megaureter) (23%), upper-pole hydronephrosis associated with duplex anomalies (13%), and posterior urethral valves (10%). Seventeen neonates with multicystic dysplastic kidney were seen (three per year or one for every eight with hydronephrosis). In comparison, during the 30-year period, 1947-1977, 146 neonates with significant hydronephrosis were seen. Most cases were discovered because the patients had signs and/or symptoms--either an abdominal mass (an enlarged kidney or bladder) or urosepsis. The three most common conditions were obstruction of the ureteropelvic junction (22%), posterior urethral valves (19%), and ectopic ureterocele (14%). During this period, 53 neonates with multicystic dysplastic kidney were discovered (two per year or one for every three with hydronephrosis). The dramatic increase in the number of neonates found to have hydronephrosis is primarily due to the widespread use of obstetric sonography and concomitant fetal screening. The pattern of causes before 1979 represented the incidence of symptomatic lesions. The current pattern more accurately reflects the true incidence of congenital anomalies of the urinary tract.

Thirteen cases of fetal hydronephrosis associated initially with normal volumes of amniotic fluid were diagnosed by maternal sonography. No invasive procedures were done in utero. Postnatal evaluation demonstrated 8 neonates with urinary tract obstruction and 5 normal neonates. Long-term followup of these 13 patients revealed that 12 are healthy and have normal renal function. The exception is a child whose amniotic fluid decreased during pregnancy.Our data suggest that fetuses with normal volumes of amniotic fluid do well. Treatment should be limited to frequent prenatal sonography and subsequent early postnatal evaluation. Caution should be exercised with the diagnosis of hydronephrosis, since 5 of our patients had false positive findings. In the rare instances when oligohydramnios develops during pregnancy fetal intervention may be warranted.

This article is a summary of the prenatal and perinatal management of a variety of congenital lesions as practiced by the fetal treatment program at the University of California in San Francisco. Emphasis is placed on those lesions that are amenable to fetal intervention.

Our aim was to determine the postnatal effects of single and repeated glucocorticoid injections during late gestation. Repeated (104, 111, 118, 125 days) or single (104 days) injections of betamethasone or saline were given to the ewe or by ultrasound guided injection to the fetus (term 150 days). Lambs were born spontaneously and studied at 3 and 6 mo and 1 yr of age. Arterial pressure was measured at each age, and we performed intravenous glucose tolerance tests at 6 mo and 1 yr. Repeated maternal, but not single maternal or fetal, betamethasone injections prolonged gestation, reduced weight at birth and 3 mo, and was associated with low arterial pressure at 3 mo but not at 6 mo and 1 yr. Glucose metabolism was altered in all betamethasone treatment groups, regardless of the number or route of injections. Our data demonstrate that glucocorticoid-induced fetal growth restriction is associated with a transient reduction in postnatal arterial pressure, but glucocorticoid exposure with or without growth restriction alters glucose metabolism.

The past decade has produced profound changes in pediatric urology. The advent of prenatal ultrasound has led to antenatal diagnosis and early treatment of many lesions. As so often occurs, pediatric urologists are faced with solving clinical problems without a complete database that will outline the natural history of the lesion and thereby guide therapy. This volume attempts to highlight areas of controversy produced by these incomplete databases and to provide further information for the practitioner. Cook and Stephens have provided a review of normal embryology as well as pathoembryology. Additionally, they expanded upon the role of deformation in the production of many of the abnormalities associated with urologic problems. The next two chapters deal with in utero ultrasonic detection of urologic abnormalities. In some ways, one might interpret these chapters as viewing the glass as either half empty or half full, respectively, with regard to fetal intervention. It is

HHS Public Access Author manuscript Author Manuscript Ultrasound Obstet Gynecol. Author manuscript; available in PMC 2015 July 15. Published in final edited form as: Ultrasound Obstet Gynecol. 2015 April ; 45(4): 447–451. doi:10.1002/uog.14650. Twin-reversed arterial perfusion sequence associated with decreased fetal cerebral vascular impedance S. Peyvandi *,† , V. A. Feldstein †,‡,§ , S. Hirose †,¶ , L. Rand †,§ , M. M. Brook *,† , and A. J. Moon- Grady *,† * Division of Cardiology, Department of Pediatrics, University of California San Francisco Benioff Children’s Hospital, San Francisco, CA, USA Author Manuscript † The Fetal Treatment Center, University of California San Francisco Benioff Children’s Hospital, San Francisco, CA, USA ‡ Department of Radiology, University of California San Francisco, San Francisco, CA, USA § Department of Obstetrics and Gynecology, University of California San Francisco, San Francisco, CA, USA ¶ Division of Pediatric Surgery, Department of Surgery, University of California San Francisco Benioff Children’s Hospital, San Francisco, CA, USA Abstract Author Manuscript Objectives—Twin-reversed arterial perfusion (TRAP) sequence affects 1% of monochorionic twin pregnancies and is caused by abnormal vascular connections between a pump twin and an acardiac mass. The effects of abnormal vascular connections on cerebral vasculature in the pump twin are unknown. We hypothesize that abnormal cerebral vascular impedance, as assessed by the pulsatility index (PI), is present in pump twins and that fetal intervention alters cerebral impedance. Methods—Fetal echocardiograms performed between 2010 and 2013 in pregnancies diagnosed with TRAP (n = 19), recorded at presentation, and uncomplicated monochorionic twin pregnancies (controls, n = 18; 36 fetuses) were analyzed. In all subjects, the middle cerebral artery (MCA)-PI, combined cardiac output (CCO) and cardiothoracic ratio were calculated, and the values for cases and controls were compared. Author Manuscript Results—The mean gestational age at the time of echocardiography was 20 weeks in both groups. MCA-PI was lower in TRAP cases than in controls (1.55 (95%CI, 1.47–1.64) vs 1.74 (95% CI, 1.65–1.82), respectively; P = 0.004). CCO in TRAP cases was mildly elevated for gestational age (199.7 (95% CI, 138.4–261.1) mL/min) compared with that of controls (131.4 (95% CI, 102.2–160.7) mL/min). In six TRAP cases with a second echocardiogram available, the mean MCA-PI increased after intervention, from 1.5 (95%CI, 1.3–1.7) to 1.8 (95% CI, 1.4–2.2). Conclusions—TRAP pump twins have lower cerebral vascular impedance than do controls, suggestive of a brain-sparing effect. MCA-PI appeared to increase in a small group of pump twins Correspondence to: Dr S. Peyvandi, Department of Pediatrics, University of California San Francisco Benioff Children’s Hospital, 505 Parnassus Ave Box 0214, San Francisco, CA 94143, USA (shabnam.peyvandi@ucsf.edu).