Natural history of fetal ventriculomegaly.

The natural history of in utero ventriculomegaly was defined by a retrospective review of the outcome of 47 fetuses evaluated during a 5-year period by the Fetal Treatment Program at the University of California. In 20 fetuses, a diagnosis of ventriculomegaly associated with other severe abnormalities was made early in pregnancy. Termination of pregnancy was elected in 19 of 20 cases, and no fetus survived. In five fetuses, the diagnosis was made late in pregnancy and was associated with severe abnormalities. Fetuses were handled in a routine obstetric fashion and none survived. Of the other 22 fetuses 19 had stable and two had progressive ventriculomegaly; in one case, ventriculomegaly resolved in utero. Nineteen of these fetuses have survived, 13 with normal intellectual development and six with moderately to severely delayed development. Associated abnormalities were detected with ultrasonography in 74% of fetuses; there was a 20% false-negative rate of detection. Ventriculomegaly was isolated and progressive in two fetuses. In both cases, fetuses were delivered at term, and postnatally a shunting procedure was performed. Both children are neurologically normal. From our results and a review of the literature, which supports our findings, we were unable to define a group of fetuses with in utero ventriculomegaly that would benefit from in utero shunting.

Rapid B-scan (real-time) ultrasonography in the identification and evaluation of twin pregnancies.

Real-time ultrasonography utilizing a hand-held linear array transducer provides a highly accurate method of identifying and evaluating the twin gestation. The rapidity of real-time scanning eliminates interpretational problems caused by fetal movements. Additionally, the versatility of transducer positioning facilitates recognition of appropriate fetal planes for assessment of viability and growth. A prospective study of 595 consecutive pregnancies yielded no misdiagnoses of the number of fetuses present and only one false positive diagnosis of fetal viability.

Accuracy of real-time sonography in the determination of fetal viability.

After 8 weeks of fetal age, real-time ultrasonography is a simple, rapid, and highly reliable technic for differentiating a viable from a dead fetus. Fetal movements are invariably detected after 8 weeks of fetal life, and a discrete, fluid-filled, intrathoracic pulsating heart can be seen after 11-12 weeks of fetal life. Absence of these findings, as observed by two independent examiners each viewing the fetus for 3 minutes with real-time sonography, provides reliable evidence of fetal demise. A prospective evaluation of 390 consecutive fetuses yielded only one false positive diagnosis of fetal viability. No living fetus was diagnosed as being dead.

First trimester molar pregnancy: nonspecific ultrasonographic appearance.

Ultrasonographers and gynecologists have come to anticipate not only a high diagnostic sensitivity of ultrasonic detection of hydatidiform mole, but also that the examination will suggest specifically the correct pathologic diagnosis. Although this appears true for molar gestations examined in the second trimester, molar pregnancies detected in the first trimester do not appear to demonstrate the characteristic features necessary to diagnose this abnormality specifically. Three cases of early molar pregnancy examined in the past year with good quality gray-scale ultrasonographic units failed to demonstrate specific diagnostic findings.

Ultrasonography in the evaluation of intrauterine transfusion.

Numerous techniques have been described in the literature to aid the obstetrician in safely and easily performing an intrauterine transfusion. None, however, have addressed themselves to the question of whether the transfused cells actually enter the fetal peritoneal cavity. We have used gray-scale ultrasonography to evaluate this question in 4 patients who had undergone a total of 8 intrauterine transfusions. By scanning the fetal abdomen both before and after the transfusion, we were able to identify the transfused fluid within the fetal abdomen in all cases.

The role of velamentous cord insertion in the etiology of twin-twin transfusion syndrome.

OBJECTIVE: To evaluate the prevalence of velamentous cord insertion in twin-twin transfusion using the hypothesis that such insertions may contribute to the etiology of the condition. METHODS: All cases of placentas referred for pathologic evaluation at the University of California at San Francisco from 1984-1992 were reviewed for the citation of diamniotic-monochorionic placentation, including the presence of velamentous cord insertions. Maternal and infant records were studied for findings consistent with twin-twin transfusion syndrome. RESULTS: Thirty-eight cases of monochorionic-diamniotic twins were identified, 11 of which showed twin-twin transfusion syndrome. The prevalence of velamentous cord insertion in the transfusion syndrome subset was 63.6%, compared with 18.5% in those without (significant difference at P < .01). Twin-twin transfusion syndrome pregnancies with velamentous insertions were delivered at a significantly earlier gestational age; they also had fewer surviving infants and were more likely to have been treated prenatally than transfusion syndrome pregnancies without velamentous insertion, although these latter two findings were not significantly different. CONCLUSIONS: Velamentous cord insertions are more common in twin-twin transfusion syndrome pregnancies and may contribute to the development of profound disparity in fluid volume because the membranously inserted cord can be easily compressed, reducing blood flow to one twin. Large-volume amniocentesis may reduce this compressive force on the cord insertion, thus explaining the success of this mode of intervention.

Sonographic identification of autosomal recessive polycystic kidney disease associated with increased maternal serum/amniotic fluid alpha-fetoprotein.

A patient with no family history of renal disease was referred for obstetric sonography because of elevated maternal serum and amniotic fluid alpha-fetoprotein (AFP), and was proved to be carrying a fetus with autosomal recessive polycystic kidney disease. Review of records of previous cases with a sonographic diagnosis of this disease revealed one other case with elevated and one case with normal amniotic fluid AFP. Sonographers should be aware of the potential diagnosis of this condition in patients with elevated AFP, even in gestations not known to be at risk. Because the sonographic diagnosis of this disease can be difficult in gestations known to be at risk, AFP determination may prove to be a useful diagnostic adjunct in these pregnancies as well.

Gastrointestinal tract obstruction in the fetus.

Surgical advice is often sought when a prenatal diagnosis of gastrointestinal tract obstruction is made. We reviewed our experience with 17 such cases during a 4-year period. Eight fetuses had complete proximal obstruction. Seven of the 8 did well after maternal transport to a perinatal center and prompt neonatal surgery. Six fetuses had distal obstruction with dilated bowel and increased peristalsis. Two died after birth (1 with severe associated anomalies and 1 with short-bowel syndrome), and the other 4 did well. Three fetuses had a false-positive diagnosis of in utero meconium peritonitis. Two died and the other had no postnatal evidence of obstruction. Our data suggest (1) polyhydramnios may not be present early in gestation or with distal obstruction; (2) other anomalies, including a family history of cystic fibrosis, should be sought; (3) dilated bowel with increased peristalsis is diagnostic of fetal gastrointestinal tract obstruction, whereas intra-abdominal calcification and ascites are nonspecific findings; (4) late development of ascites in a fetus with documented obstruction may be an indication for early delivery; and (5) prenatal diagnosis permits appropriate counseling, planned delivery, and prompt postnatal resuscitation and surgery with a good prognosis in most cases.

Management of children with urinary tract infections: the Stanford experience.

Two hundred seventy-eight female children with urinary tract infections have been evaluated at Stanford division of urology. All children were followed up for a period of not less than twelve months. Age of onset of infection, clinical presentation, and nature of infecting organisms were observed. The group consisted of 144 children without ureteral reflux and 134 children with ureteral reflux. Sixty-one of the female children with ureteral reflux had ureteral reimplantation, while 73 received medical treatment alone. A study of infection rates in each of the three groups of children indicated a similar infection rate, although those children with reflux experienced a higher incidence of clinical pyelonephritis. Correction of ureteral reflux did not alter the infection rate; however, infections after surgical correction were generally of a type usually associated with children without reflux. Twenty-nine children had urethral dilatation, and the infection rate prior to and following urethral dilatation indicated a similar rate of infection pre- and posturethral dilatation. One hundred nonrefluxing kidneys were observed radiologically: 97 were normal and 3 showed clubbing and scarring. Of 110 refluxing renal units observed, 62 were clubbed and scarred and 48 were normal. Following surgical correction of reflux, renal clubbing and scarring were not observed in previously normal renal units. Of those renal units found to be abnormal at time of surgery, 66 per cent showed progression of clubbing and scarring after surgical correction of reflux. It was observed that the greater the degree of reflux present, the higher the incidence of renal damage. This study suggests that children who experience recurrent urinary tract infections who do not have ureteral reflux are seldom at renal risk; similar children who do have ureteral reflux are at risk unless the infections are controlled or the reflux either disappears or is corrected surgically.

Antenatal diagnosis of subependymal heterotopia.

Subependymal heterotopia consist of gray matter nodules along the lateral ventricular walls and are associated with epilepsy and other cerebral malformations. Some cases have an X-linked inheritance, and early antenatal diagnosis of affected fetuses is important for appropriate management. We present a case of heterotopia diagnosed by sonography and MR imaging at 23 weeks' gestation and discuss the differential diagnosis, reviewing the evolution and imaging appearances of the germinal matrix and its implications for detection of heterotopia.

Fast MR imaging of fetal CNS anomalies in utero.

BACKGROUND AND PURPOSE: Although sonography is the primary imaging technique for evaluating the developing fetus, significant limitations exist in the sonographic prenatal diagnosis of many brain disorders. Fast MR imaging is increasingly being used to determine the underlying cause of nonspecific fetal CNS abnormalities detected sonographically and to confirm or provide further support for such anomalies. Our goal was to determine the value of MR imaging in establishing the diagnosis of fetal CNS anomalies, to ascertain how this information might be used for patient counseling, and to assess its impact on pregnancy management. METHODS: We prospectively performed MR examinations of 73 fetuses (66 pregnancies) with suspected CNS abnormalities and compared these with available fetal sonograms, postnatal images, and clinical examinations. Retrospectively, the impact on patient counseling and pregnancy management was analyzed. RESULTS: Images of diagnostic quality were routinely obtained with in utero MR imaging, which was particularly valuable in detecting heterotopia, callosal anomalies, and posterior fossa malformations, and for providing excellent anatomic information. We believe that 24 (46%) of 52 clinical cases were managed differently from the way they would have been on the basis of sonographic findings alone. In every case, the referring physicians thought that MR imaging provided a measure of confidence that was not previously available and that was valuable for counseling patients and for making more informed decisions. CONCLUSION: Sonography is the leading technique for fetal assessment and provides reliable, inexpensive diagnostic images. Fast MR imaging is an important adjunctive tool for prenatal imaging in those instances in which a complex anomaly is suspected by sonography, when fetal surgery is contemplated, or when a definitive diagnosis cannot be determined.

Ultrasonography in the evaluation of nontraumatic abdominopelvic emergencies.

Ultrasonography can provide prompt diagnostic information in many non-traumatic abdominal and pelvic emergencies. The safety of of diagnostic ultrasonography enables its use in many emergency situations that relate to pregnancy. The ability to demonstrate normal fluid-filled organs with clarity makes ultrasonography valuable in the emergency assessment of the gallbladder and aorta. Similary, ultrasound is sensitive to the detection of pathologic collections of fluid that can be applied in the search for abscesses or hematomas. Finally, ultrasonography is independent of organ function. In this regard, it is a useful ancillary test when radiographic examinations of the biliary or urinary tracts fail because of poor contract excretion.

Sonographic predictors of survival in fetal diaphragmatic hernia.

The authors studied the predictive value of detailed fetal sonographic parameters on outcome for 55 patients with prenatally diagnosed congenital diaphragmatic hernia managed at an ECMO center. Their sonographic assessment included gestational age at time of diagnosis, polyhydramnios (largest amniotic fluid pocket diameter), presence of liver and/or stomach herniation, and abdominal circumference at the level of the umbilical cord. They measured the right lung two-dimensional area at the level of the atria as an estimate of lung size and mediastinal shift. The ratio of right lung area to head circumference (LHR) was calculated to minimize lung size differences owing to gestational age. The principal outcome variable was survival. The overall survival rate was 65%. If the diagnosis was made after 25 weeks' gestation, the survival rate was 100% (12 of 12); the rate was 56% if the diagnosis was made at or before 25 weeks (P < .005). All five neonates with an LHR of less than 0.6 died; the survival rate was 100% for those whose LHR was greater than 1.35; and those with an LHR between 0.6 and 1.35 had a 61% survival rate (P < .001). The survival rate for those whose liver was not herniated was 100% (10 of 10); herniation of the liver decreased the survival rate to 56% (P < .05). Stomach position, polyhydramos, and abdominal circumference were not found to be useful survival predictors. No prenatal sonographic parameter was absolutely predictive of postnatal death except very small right lung size, which was present in only 5 of the 55 patients. Survival is highly likely if the liver is not herniated into the thorax and/or the right lung is large.

Fetendo-clip: a fetal endoscopic tracheal clip procedure in a human fetus.

Fetal tracheal occlusion accelerates lung growth and corrects the often fatal pulmonary hypoplasia seen in fetuses with congenital diaphragmatic hernia. Fetoscopy presents a unique opportunity to glimpse into the world of the fetus, but its use, until recently, has been limited to diagnostic and simple procedures. Using fetoscopic techniques ("Fetendo"), we now report successful tracheal occlusion in a 30-week-old fetus for the treatment of congenital diaphragmatic hernia.

Fetal surgery for cystic adenomatoid malformation of the lung.

We reviewed our experience with fetal therapy for congenital cystic adenomatoid malformation of the lung (CCAM) at the University of California, San Francisco Fetal Treatment Center. Fetuses with life-threatening CCAM were selected for prenatal treatment according to predetermined guidelines, including the gestational age of the fetus, the size of the intrathoracic lesion, maternal health, and the development of fetal hydrops. The knowledge that fetuses with hydrops are at high risk for fetal or neonatal death led to fetal surgical resection of the massively enlarged pulmonary lobe (fetal lobectomy) in six cases. In the first case, resection was too late, since preoperative labor and maternal preeclampsia could not be reversed, leading to premature delivery of a nonviable infant. In the next four cases, CCAM resection led to resolution of the hydrops, impressive in utero lung growth, and neonatal survival. Right middle and lower lobe resection in the sixth fetus at 21 weeks was successful, but subsequent inexplicable fetal death highlights the need for better postoperative fetal monitoring and treatment. Three other fetuses with a single predominant cyst underwent thoracoamniotic shunt placement alone; two survived after delivery and prompt neonatal surgery with the assistance of high-frequency ventilation or extracorporeal membrane oxygenation. Fetal therapy can now be considered for otherwise fatal space-occupying intrathoracic lesions in the fetus.

Prenatal diagnosis of esophageal atresia.

The prenatal sonographic detection of esophageal atresia (EA) has been possible for more than a decade and relies on the finding of a small or absent fetal stomach bubble associated with maternal polyhydramnios. The aims of this study were to assess the accuracy of this technique and to determine whether the outcome of prenatally diagnosed EA differs from its postnatal counterpart. All fetal sonograms performed between January 1989 and October 1993 demonstrating a small or absent fetal gastric bubble were reviewed together with all neonates with EA treated during the same period. Eighty-seven fetuses with a small (n = 53) or absent stomach bubble (n = 34) were identified, representing 1.4% of all fetal sonographic surveys. Esophageal atresia was present in 15; in 13 of these, the maternal amniotic fluid volume was increased. The positive predictive value of an absent stomach bubble and polyhydramnios was 56%, and the sensitivity of prenatal sonography in the diagnosis of EA was 42%. One neonate with EA had the prenatal diagnosis established at another institution, yielding a total of 16 cases of prenatally diagnosed EA for analysis. Seven (44%) of these had trisomy 18. Of the remaining nine, two had isolated EA, two had laryngeal atresia and EA, and there were two late-gestational unexplained fetal deaths. Only four (25%) survived through the neonatal period. The prognosis of the fetus with EA is radically different from that of the neonate with EA.

Fetal diaphragmatic hernia without visceral herniation.

Many cases of congenital diaphragmatic hernia (CDH) are currently detected before birth. The authors hypothesized that there is a subgroup of patients with CDH who have no evidence of visceral herniation in utero and who would be expected to have less pulmonary hypoplasia and a good prognosis. Among 41 neonates with left-sided CDH treated between January 1990 and October 1993, 17 cases were diagnosed after birth. Ten of the 17 had undergone detailed fetal sonographic imaging at or after 20 weeks' gestation. After independent review of the prenatal scans of these 10 patients, one was found to have evidence of a diaphragmatic hernia and was excluded from further analysis. The other nine fetuses survived, and prosthetic repair of the diaphragmatic defect and extracorporeal membrane oxygenation (ECMO) were each required in only one patient. This contrasted with the outcome for 18 control patients with prenatally diagnosed CDH: 4 (22%) died, 13 (72%) required prosthetic repair, 9 (50%) were supported with ECMO and the duration of ventilatory support and hospital stay were significantly longer. There is a spectrum of severity among both pre and postnatally diagnosed cases of CDH. In the neonate with an isolated left-sided diaphragmatic hernia, a good prognosis is to be expected if the condition was not detectable by detailed prenatal sonography in the second half of pregnancy.

The incidence and spectrum of neurological injury after open fetal surgery.

A preterm infant's immature brain is susceptible to both anoxic and hemorrhagic injury during periods of physiological stress. The advent of in utero surgery has created a new population of premature patients at risk for central nervous system (CNS) injury. The aim of this study was to evaluate the frequency and nature of CNS injuries in fetal surgical patients. Of 33 fetuses with known neurological outcome after fetal surgery, CNS injuries were identified in seven (21%). Of the seven, four had significant episodes of fetal bradycardia (3) or neonatal hypotension (1), which suggests that asphyxia contributed to the neurological injury. The CNS injuries in the other three patients occurred unexpectedly and without associated signs of fetal distress. The authors speculate that these injuries may have been caused by sudden fluxes in cerebral blood flow, induced by maternal hypoxia (1) or by maternally administered tocolytic drugs (2) used to treat postoperative preterm labor.

Congenital high airway obstruction syndrome (CHAOS): a potential for perinatal intervention.

Congenital high airway obstruction syndrome (CHAOS) results in a predictable constellation of findings: large echogenic lungs, flattened or inverted diaphragms, dilated airways distal to the obstruction, and fetal ascites and/or hydrops. The authors report on four fetuses referred for evaluation. None of them survived. Postmortem evaluation showed that three fetuses had laryngeal atresia, and one had tracheal stenosis. Coexistent fetal anomalies were accurately diagnosed by ultrasound in three of the four patients. The finding of CHAOS on prenatal ultrasound examination is diagnostic of complete or near-complete obstruction of the fetal upper airway, most likely caused by laryngeal atresia. A greater understanding of the natural history of CHAOS may permit improved prenatal and perinatal management.

Cervical cystic hygroma in the fetus: clinical spectrum and outcome.

Cervical cystic hygroma is thought by most pediatric surgeons to be an isolated, usually resectable lesion with an excellent prognosis. However, prenatal sonography has revealed a high "hidden mortality" among fetuses with this condition, and most perinatologists consider it to be uniformly fatal. In an attempt to resolve these two differing perspectives, we analyzed 29 cases seen at two centers over 4 years. Of 27 fetuses diagnosed before 30 weeks' gestation, only one survived. Twenty-five of the 27 were aborted; severe hydrops was present in 21 of these 25. Two of the 27, both with stigmata of Noonan's syndrome, underwent spontaneous regression during the second trimester: one died at 2 weeks of age, and the other survived. Successful karyotypes were obtained on 17 fetuses: nine were normal, seven were 45X, and one was trisomy 21. Fetuses with abnormal karyotypes had a lower incidence of polyhydramnios (0% v 67%), additional anomalies (12% v 67%), and consanguinity or a history of abnormal pregnancies (0% v 89%). Two fetuses were diagnosed after 30 weeks' gestation. Neither had hydrops, polyhydramnios, associated anomalies, or an abnormal karyotype. One had a completely normal sonogram at 17 weeks' gestation. Both were operated on within the first 4 days of life; one did well without complications, and the other required a permanent tracheostomy because of extensive hypopharyngeal involvement. A cystic hygroma presenting in the fetus has a different natural history and prognosis from one presenting postnatally. The vast majority of fetal cases are diagnosed before 30 weeks' gestation, and present with hydrops or diffuse lymphangiomatosis. The dismal outlook in this group justifies elective termination in most cases.(ABSTRACT TRUNCATED AT 250 WORDS)