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Biomedical subjects

R A Filly

Publications and source records attributed to R A Filly.

At least 73 records · Page 4Linked to original sources

Gastroschisis: can sonography of the fetal bowel accurately predict postnatal outcome?

To determine whether prenatal sonographic features of the small bowel can accurately predict postnatal outcome in fetuses with gastroschisis, the sonograms of 24 fetuses with prenatally detected gastroschisis were retrospectively reviewed for fetal bowel features including small bowel dilatation and bowel wall thickening. To identify a relationship between the sonographic features and neonatal outcome, each feature was analyzed against eight adverse clinical outcome measures including bowel obstruction or atresia, necrosis, and need for bowel resection, using chi-square analysis and Kendall's taub correlation. When a relationship was identified, the sensitivity and specificity of the sonographic feature for predicting adverse outcome were determined. Only maximum small bowel diameter was related to postnatal bowel complications. Significantly more fetuses with a maximum small bowel diameter of greater than 11 mm (7/12) had bowel complications than did fetuses with MBD 11 mm or less (2/12) P < 0.05). Using a cutoff point of greater than 11 mm for maximum small bowel diameter, the sensitivity was 78%, specificity 66%, and positive predictive value 71% for predicting postnatal bowel complications. However, in only seven of 24 cases did the two observers make the same maximum small bowel diameter measurement, and in 14 of 24 cases their measurements differed by 2 mm or more. The observers varied sufficiently in their measurements to shift three fetuses (13%) between categories (< or = 11 mm or > 11 mm). Although a maximum small bowel diameter of 11 mm stratified our fetuses, this measurement may not be clinically meaningful.

Abdominal Muscles↗

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.

Adult↗

Correction of congenital diaphragmatic hernia in utero: VI. Hard-earned lessons.

Extensive experimental work suggests that repair of congenital diaphragmatic hernia (CDH) in utero may salvage severely affected fetuses who otherwise have a high expected mortality despite optimal postnatal care including extracorporeal membrane oxygenation (ECMO). We have reported that repair of CDH in utero is physiologically sound and safe for the mother, but technically difficult especially when the liver is herniated into the fetal chest. In the 3 years since our last report (1989 to 1991), 61 additional patients were referred for consideration of in utero repair. Fetal repair was attempted in 14 with severe isolated left CDH diagnosed before 24 weeks gestation. Five fetuses died intraoperatively, from technical problems related to reduction of incarcerated liver and uterine contractions--problems which have subsequently been surmounted. Nine patients were successfully repaired. Four babies survived, two delivered prematurely and died, and three died in utero within 48 hours of repair. Intraoperative technical problems have been overcome; the factors limiting successful outcome are postoperative physiologic management of the maternal-fetal unit and effective tocolysis to control preterm labor.

Fetal Death↗

Color flow ultrasound for delineating microsurgical vessels: a clinical and experimental study.

There is a need in plastic and reconstructive surgery for a method of monitoring flaps and replants pre- and postoperatively. This study evaluates color flow Doppler done as a monitoring technique for microvascular vessels. The capability of color flow ultrasound to monitor venous and arterial insufficiency and occlusion was studied by means of an experimental system in the Yucatan miniature swine model. A circulatory circuit in line with a magnetic flow probe is created. Measurements are made on the isolated hind limb of the animal. Color flow ultrasound is demonstrated to be a technique that is sensitive to venous and arterial insufficiency at flow rates as low as 3.0 ml/min. Characteristic waveform patterns are shown to predict arterial and venous insufficiency before they actually progress to occlusion. A clinical series of 25 postoperative patients is presented, consisting of volunteer studies as well as pre- and postoperative examinations of free flaps and replantations. Color flow ultrasound is shown to visualize a spectrum of pre- and postoperative microvascular conduits encountered in plastic and reconstructive surgery.

Anastomosis, Surgical↗

Elevated levels of amniotic fluid alpha-fetoprotein: sonographic evaluation.

From 1978 to 1990, 263 fetuses with an elevated level of amniotic fluid alpha-fetoprotein (AF-AFP) (> 2.0 multiples of the median) were examined with targeted fetal sonography. All cases of AF-AFP elevation among 22,355 genetic amniocenteses were represented. Sonography correctly showed 32 open neural-tube defects, including 20 myelomeningoceles, and depicted 94% (63 of 67) of the anomalous fetuses. Two of five anomalous fetuses with normal sonograms, however, had extremely high AF-AFP levels leading to prospectively correct diagnoses of congenital nephrosis. Therefore, programmatically, 97% (65 of 67) of the anomalous fetuses were recognized. The three programmatic misdiagnoses were all detected in the neonatal period and surgically corrected; subsequent development was normal. The combination of an elevated AF-AFP level and a detailed sonogram allowed distinction between a normal and an anomalous fetus in 99% of cases. When elevated levels are noted, AF-AFP analysis followed by detailed sonography is highly successful for the detection and characterization of anomalous fetuses and the recognition of normal fetuses with physiologic increases of this protein in the amniotic fluid.

Acetylcholinesterase↗

Frequency of fetal anomalies in sonographically detected polyhydramnios.

One hundred and five patients who met the criterion of having an amniotic fluid pocket depth greater than 8.0 cm were categorized as polyhydramniotic. Of these cases, 82% were singleton pregnancies and 18% were twin pregnancies. The degree of polyhydramnios correlated directly with the probability that an anomaly would be detected. For the lowest rank group (pocket depth of 8 to 9.5 cm), 50% of fetuses manifested an anomaly, whereas the highest rank (16.0 cm or greater pocket depth) carried an 88% risk for an anomaly. Overall, 63% of pregnancies with polyhydramnios revealed anomalies. Sonography found all to be anomalous. There were three false diagnoses of potential anomalies. Monochorionic anomalies of twinning (twin transfusion syndrome and acardiac twin) and gastrointestinal anomalies among singletons were the most commonly seen abnormalities. However, a broad spectrum of anomalies was represented in the study group. Only five diabetic mothers were seen in our study cohort, and in each of them the pregnancy was further complicated by an associated fetal anomaly.

Brain↗

Growth retardation in prenatally diagnosed cases of gastroschisis.

Gastroschisis is a rare congenital anomaly characterized by the herniation of fetal intestines directly through an abdominal wall defect. It is associated infrequently with chromosomal or other nonbowel defects and can be treated surgically after delivery, with survival rates reported to be between 87 and 100%. We reviewed 21 cases of prenatally diagnosed gastroschisis to ascertain the effect of fetal growth retardation on perinatal outcome. Ten of the 21 fetuses (48%) were identified prenatally as growth retarded, although only seven of these ten truly had birth weights less than the 10th percentile. Three additional fetuses that had not been identified prenatally as growth retarded did, in fact, have birth weights less than the 10th percentile, for a total frequency of growth retardation at birth of 48% (10/21 fetuses). When compared to non-growth-retarded fetuses with gastroschisis, fetuses who were growth retarded, although more likely to have been delivered by emergency cesarean section, had shorter hospitalization times, were more likely to have undergone primary closure on the first day of life, and had fewer major complications. We conclude that growth retardation is common in fetuses with gastroschisis and the postnatal outcome in gastroschisis is not poorer for fetuses who are growth retarded.

Abdominal Muscles↗

The lemon sign: not a specific indicator of meningomyelocele.

The association of the "lemon sign" with myelomeningocele has been well documented. This study was performed to determine if this sign is exclusive to myelomeningocele. The study population included all abnormal fetuses in our data base with this sonographically observed finding, from 1988 to 1991 (total of 23 cases). Twelve fetuses were found to have a myelomeningocele and six an encephalocele. Five fetuses with the lemon sign demonstrated a variety of structural anomalies not involving the neural tube. This sonographic finding appears not to be limited to cases of myelomeningocele.

Female↗

Sonographic diagnosis of ectopic pregnancy with endovaginal probes: what really has changed?

To evaluate the impact of endovaginal (EV) sonography in the diagnosis of ectopic pregnancy, a 2 year retrospective study was performed identifying 123 at-risk patients. Of these 123 women, 19 (15.4%) had a surgically proved ectopic pregnancy, only three (15.8%) of which were visualized directly at sonography. A confident diagnosis of an intrauterine pregnancy (IUP) was made at the initial scan in 74%, which contrasts with 58% diagnosed at the first transabdominal (TA) scan in an earlier study from this laboratory, thus confirming an improvement in diagnostic ability with EV transducers. This study has failed to confirm some findings of other workers, particularly that adnexal ring-like structures are visualized frequently in the presence of an ectopic pregnancy. No adnexal rings were observed in our 19 cases. The combination of an adnexal mass and free pelvic fluid was found to correlate best with the presence of an ectopic pregnancy. This study further emphasizes that a significant proportion (26.3%) of ectopic pregnancies have a normal EV sonogram at presentation. The group failing to demonstrate an IUP and showing no evidence of an adnexal mass or pelvic fluid (i.e., a normal pelvic sonogram) carried a 1:3 risk for the presence of an ectopic pregnancy, a result that is very similar to our data published before the introduction of EV technology. We conclude that, although it provides a significant improvement and refinement in the recognition of intrauterine pregnancies, EV scanning does not permit a confident diagnosis of ectopic pregnancy in many cases.

Decidua↗

The AIUM/ACR antepartum obstetrical sonographic guidelines: expectations for detection of anomalies.

A survey was sent to 52 physicians considered experts in obstetrical sonography asking them to comment on the probability of observing various fetal abnormalities when the AIUM/ACR guidelines for a level one obstetrical sonogram were followed. Although definite trends were noted in several areas, there was much diversity in opinion concerning what should or should not be seen when the guidelines were followed. Given the fairly specific nature of the AIUM/ACR guidelines, we were uncertain why the responses were not in more uniform agreement.

Congenital Abnormalities↗

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.

Female↗

Outcome of antenatally diagnosed cystic adenomatoid malformations.

OBJECTIVE: Twenty-two cases of antenatally diagnosed congenital cystic adenomatoid malformations are reported. STUDY DESIGN: Case management is reviewed. RESULTS: Eighteen women continued pregnancy after diagnosis. In nine cases nonimmune hydrops fetalis did not develop and all infants survived. Nonimmune hydrops fetalis developed in the other nine; fetal intervention was performed in eight cases. In the single case of nonimmune hydrops fetalis without intervention, the neonate died. In four cases aspiration of macrocystic lesions was performed. In two cases cystoamniotic shunts were placed. Neither aspiration or shunting provided long-term benefit. In six cases fetal lobectomy was ultimately performed and four survived. Two fetuses did not undergo in utero surgery; one was delivered prematurely after cyst aspiration and lived, and the other previable fetus was delivered soon after shunting. CONCLUSIONS: Fetal survival is best related to development of nonimmune hydrops fetalis. Aspiration of cystic lesions and cystoamniotic shunts generally provide short-term benefit. Early experience with fetal surgery for congenital cystic adenomatoid malformations has been encouraging.

Cystic Adenomatoid Malformation of Lung, Congenita↗

Prenatally detected myelomeningoceles: sonographic accuracy in estimation of the spinal level.

The sonograms and case records of 53 patients with prenatally detected myelomeningoceles were retrospectively reviewed. Sonographic findings were correlated with pathologic, surgical, and/or radiographic findings in 28 cases. Fourteen mothers chose to continue their pregnancies, and the level of neuromotor deficit was available for 11 children of this group. The sonographic and pathologic levels were in agreement in 18 of 28 cases (64%) and were within one spinal level in 22 of 28 cases (79%). The pathologic level of the lesion was underestimated (sonographic level lower than pathologic) in three fetuses and overestimated (sonographic level higher than pathologic) in another three. In 10 of 11 living children (91%) the neuromotor level was equal to or better than the anatomic level. The authors conclude that ultrasonography can, in most cases, allow accurate prediction of the level of the spina bifida lesion and the severity of neuromotor handicap in children with prenatal diagnoses of myelomeningocele.

Female↗

Correlation between omphalocele contents and karyotypic abnormalities: sonographic study in 37 cases.

To evaluate the observation that fetuses with omphaloceles containing only bowel have an especially high prevalence of karyotypic abnormalities, we retrospectively reviewed the sonograms and case records of 37 fetuses with omphaloceles detected sonographically between 1984 and 1990. Nine fetuses had concomitant morphologic abnormalities characteristic of the amniotic band syndrome. Of the remaining 28 fetuses, karyotypic correlation was available in 22, and the karyotype was abnormal in five of these (23%). The omphaloceles contained liver in 22 fetuses and only bowel in six fetuses. Among fetuses with exteriorized liver, karyotypes were abnormal in one (6%) of 16 tested. In contrast, four (67%) of the six fetuses whose omphaloceles contained only bowel had abnormal karyotypes; for each of these four, sonograms showed morphologic abnormalities in addition to the omphalocele. In the two fetuses with bowel-only omphaloceles and normal karyotypes, the omphalocele was the only abnormality seen on sonograms, and these children are well after surgical repair. When fetuses with the amniotic band syndrome were excluded, sonograms showed concomitant anomalies in 15 fetuses with liver-containing omphaloceles, and the karyotype was abnormal in only one of these 15. The results of this study support previous observations that karyotypic abnormalities are more common in association with omphaloceles that contain only bowel compared with those that contain only liver. If we combine our data with data from three other studies that address this issue, 87% of fetuses with omphaloceles containing only bowel had an abnormal karyotype, a significantly higher rate than in those fetuses whose omphaloceles contained liver also (9%).

Amniotic Band Syndrome↗

The "lying down" adrenal sign: a sonographic indicator of renal agenesis or ectopia in fetuses and neonates.

An inability to image the kidneys, severe oligohydramnios, and an empty (nonvisualized) urinary bladder are the most common sonographic findings in fetuses with renal agenesis. The same features, except for the oligohydramnios, are seen in neonates with renal agenesis. Failure to visualize a kidney in the usual flank location with or without demonstration of the kidney in an ectopic location may also be a feature in fetuses or neonates with renal ectopia. The adrenal gland has an unusual but distinctive appearance on longitudinal sonograms in these patients. Sonograms of 23 fetuses and six neonates referred for presumed bilateral or unilateral renal agenesis or ectopia were reviewed retrospectively for the presence of a flattened ("lying down") adrenal gland. At the time of the examination, the adrenal gland was not actively sought as a part of the fetal survey or postnatal examination but was found retrospectively in 48% of these subjects. Although this finding has been described previously, the emphasis was on this observation as a potentially confusing sonographic feature. This manuscript emphasizes the characteristic appearance of the adrenal gland which instead serves as a simple means of confirming that the kidney did not develop in the flank. Detection of a flattened ("lying down") adrenal gland is an observation that should be actively sought in all fetuses and neonates with a presumed diagnosis of renal agenesis or ectopia.

Adrenal Glands↗