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Biomedical subjects

J A Estroff

Publications and source records attributed to J A Estroff.

32 records · Page 2Linked to original sources
PubMed1992

Prenatal diagnosis of the megacystis-megaureter association.

A total of 11 male neonates with hydronephrosis and a large bladder in utero had the megacystis-megaureter association. Prenatal ultrasound findings included bilateral hydroureteronephrosis; a large, smooth, thin-walled bladder, and normal volume of amniotic fluid. Postnatal studies demonstrated grades 4 to 5 bilateral reflux, a large bladder without obstruction and in 2 infants nonfunction of a kidney or renal moiety. Of 7 infants initially managed nonoperatively 5 have undergone surgery due to persistent reflux or breakthrough urinary infections. The prenatal diagnosis of the megacystis-megaureter association can be suspected with reasonable accuracy. Prompt postnatal continuous antibiotic prophylaxis and uroradiological confirmation allow for nonemergency management of this condition with excellent results.

Female↗
PubMed1992

Prevalence of cystic fibrosis in fetuses with dilated bowel.

The authors reviewed the ultrasonographic images and medical records of 15 consecutive fetuses with dilated loops of bowel distal to the duodenum and determined the prevalence of cystic fibrosis among them. The criteria for dilated bowel loops included both subjective criteria and luminal measurements. Five of the fetuses (33%) had cystic fibrosis. Eleven had bowel obstruction at birth, and four of those 11 (36%) also had cystic fibrosis. One of the four fetuses without bowel obstruction at birth had cystic fibrosis. There were no differences in sonographic findings between fetuses with and without cystic fibrosis, except that one third-trimester fetus with cystic fibrosis had echogenic small bowel with shadowing. All of the fetuses with dilated bowel loops persisting at birth required surgery. Further studies are indicated to determine the exact risk of cystic fibrosis in this population.

Cystic Fibrosis↗
PubMed1992

Isolated choroid plexus cysts in the second-trimester fetus: is amniocentesis really indicated?

Choroid plexus (CP) cysts have been associated with trisomy 18, although most fetuses with CP cysts are normal. Since many fetuses with trisomy 18 have other sonographic abnormalities, the necessity of obtaining a karyotype for all fetuses with isolated CP cysts remains controversial. The authors prospectively studied 234 second-trimester fetuses with sonographically discovered CP cysts. Two hundred twenty of them had no other sonographic findings. None of these 220 normal fetuses had evidence of aneuploidy at amniocentesis or an anomaly at birth. Fourteen fetuses had major anomalies detected in utero: 11 had trisomy 18, one had triploidy, and two had normal karyotypes but were structurally abnormal. While size and bilaterality of the CP cysts were not helpful in predicting aneuploidy, the meticulous anatomic survey of fetuses with CP cysts allowed successful identification of all aneuploid fetuses. These data show that the yield of abnormal karyotypes in fetuses with isolated CP cysts is low and may not justify the risk of amniocentesis.

Amniocentesis↗
PubMed1992

Dandy-Walker variant: prenatal sonographic features and clinical outcome.

The Dandy-Walker variant is a less severe posterior fossa anomaly than the classic Dandy-Walker malformation. In 17 consecutive fetuses, the Dandy-Walker variant was diagnosed at sonography, and associated defects, karyotypic anomalies, and outcomes were evaluated. Four of the 17 fetuses (24%) had mild ventriculomegaly. Eight of the 17 (47%) had concurrent non-central nervous system (CNS) anomalies. Five fetuses (29%) had an abnormal karyotype (two with trisomy 18, one each with trisomy 13, 21, and 11q+) and associated sonographic anomalies. Six of the 17 fetuses (35%) died in utero or during the neonatal period, two are severely handicapped, and the other nine are developing normally at ages 4 months to 4 years. Six of the nine normally developing infants (53%) lacked non-CNS sonographic findings. Because the prognosis is uncertain for an infant born with the prenatal diagnosis of Dandy-Walker variant, prenatal recognition of the anomaly allows for the option of fetal karyotyping and for arrangement for postnatal follow-up.

Abnormalities, Multiple↗
PubMed1992

Fetal umbilical vein varix: sonographic appearance and postnatal outcome.

We describe five cases of dilation or varix of the umbilical portion of the left portal vein appearing as a fetal intra-abdominal cyst. Doppler or color flow examination, or both, indicated umbilical venous flow within the cystic area in each case. Only one fetus exhibited transient cardiomegaly, which resolved before birth. This fetus was the only one discovered to have the varix as early as the second trimester. All five infants were delivered at term without sequelae.

Dilatation, Pathologic↗
PubMed1991

Structural genitourinary defects detected in utero.

Fetal genitourinary anomalies are detected with increasing frequency due to the large numbers of fetuses that undergo screening ultrasonography (US) for nonspecific indications. One hundred seventy-seven patients were evaluated for fetal urinary abnormalities over a 2-year period. Fetal hydronephrosis accounted for 154 (87%) of the cases, with the remaining diagnoses including multicystic dysplastic kidney, autosomal recessive polycystic kidney disease, and renal agenesis or hypodysplasia. Ureteropelvic junction obstruction was the most common postnatal anatomic abnormality (29%), with a large number of cases of prenatally diagnosed hydronephrosis resolving either prenatally (33%) or postnatally (24%). Prenatal US findings were retrospectively analyzed with regard to the degree of hydronephrosis at different stages of gestation. Data showed that there is a gradual rise in anteroposterior renal pelvic diameter during gestation and that there is a correlation between this diameter and the ultimate renal outcome in regard to surgical repair. This information can provide guidance in making recommendations to parents and physicians.

Congenital Abnormalities↗
PubMed1991

Increased renal parenchymal echogenicity in the fetus: importance and clinical outcome.

Pre- and postnatal ultrasound (US) findings and clinical course in 19 fetuses (16-40 menstrual weeks) with hyperechoic kidneys (renal echogenicity greater than that of liver) and no other abnormalities detected with US were evaluated to determine whether increased renal parenchymal echogenicity in the fetus indicates renal disease. Four infants (21%) were healthy at birth and had normal postnatal sonograms. Another 10 infants (53%) survived, but abnormalities were found at neonatal US. Postnatal diagnoses in these 10 neonates included unilateral renal dysplasia (n = 3), unilateral multicystic dysplastic kidney and a contralateral hyperechoic kidney (n = 2), hydronephrosis (n = 2), and renal abnormalities of unknown type (n = 3). Five fetuses with either infantile polycystic kidney disease (n = 4) or bilateral multicystic dysplasia (n = 1) did not survive. Oligohydramnios was predictive of a poor prognosis. Hyperechoic renal parenchyma in the fetus was associated with sonographic or functional abnormalities in 15 of 19 cases (79%) and a 74% survival rate.

Female↗
PubMed1990

Fetal pyelectasis: a possible association with Down syndrome.

Two hundred ten consecutive fetuses were identified as having renal pyelectasis among 7400 patients scanned during 1 year. Seven of these 210 fetuses had Down syndrome. We reviewed images of the kidneys of 44 fetuses with Down syndrome collected over 5 years and found that 25% of these affected fetuses had pyelectasis. The incidence of Down syndrome was 3.3% when fetal pyelectasis was present.

Amniocentesis↗
PubMed1990

Abnormal karyotype of fetuses with omphalocele: prediction based on omphalocele contents.

The majority of fetuses with omphalocele have other congenital abnormalities and, in many cases, an abnormal karyotype. We retrospectively studied 22 consecutive fetuses with sonographically diagnosed omphalocele and available karyotype to determine whether the contents of the omphalocele could predict the karyotype. Of these 22 fetuses, 18 had normal and four had abnormal karyotypes. Sixteen of the karyotypically normal fetuses had liver herniated into the omphalocele and two had only bowel present in the defect. All four of the fetuses with abnormal chromosomes had only bowel in the defect.

Abnormalities, Multiple↗
PubMed1989

Neurological complications of hemolytic-uremic syndrome.

Of 78 children identified with hemolytic-uremic syndrome at the Children's Hospital, Boston, from 1976 to 1986, 16 patients (20.5%) had neurological manifestations during their hospitalization. The most common manifestations were significant alterations in consciousness (coma, stupor) in 12 patients, and either generalized or partial seizures in ten patients. Others included hemiplegia (4 patients), decerebrate posturing (3), cortical blindness (2), hallucinations (1), and dystonic posturing (1). Cranial computed tomographic scans were abnormal in eight of 11 patients scanned. The abnormalities included diffuse cerebral edema (4 patients), large vessel infarctions (3), diffuse multiple small infarcts (4), and multiple hemorrhages (1). Five patients died as a result of their central nervous system complications, and six had neurological sequelae at discharge. Five patients recovered and at discharge had no evidence of neurological dysfunction.

Brain↗
PubMed1989

First trimester bradycardia. A sign of impending fetal loss.

Normal first trimester fetal heart rates rise from an average of 100 beats per minute (bpm) at 5 to 6 weeks to 140 bpm at 8 to 9 weeks. The heart rates of 65 consecutive first trimester fetuses between 5+ and 8+ weeks were measured to determine whether unusually slow first trimester fetal heartbeats are associated with a poor outcome. All five pregnancies in which heart rates were below 85 ended in spontaneous miscarriage. This study suggests that first trimester bradycardia may be associated with a poor prognosis for the pregnancy.

Abortion, Spontaneous↗
PubMedDate not supplied

Cystic neuroblastoma: emphasis on gene expression, morphology, and pathogenesis.

Cystic neuroblastoma (CN) is an unusual variant of neuroblastoma characterized by a grossly visible cyst(s) and almost always distinctive microcysts on light microscopy. Rarely, CN will appear solid grossly, but microcystification will be present. We examined the clinical, pathologic, and biologic features of 17 cases of CN. The majority of CN had been detected by prenatal ultrasound. The tumors were favorable stage, stroma-poor, but with low or intermediate mitotic-karyorhectic indices and had favorable biologic markers reflected by aneuploidy and by an absence of N-myc amplification and chromosome 1p deletions. However, the high trk expression typically identified in good risk tumors was absent. Although the complete natural history of CN is not fully defined, our experience suggests that some tumors progress in size, whereas others may spontaneously regress or mature. The clinical outcome is excellent, as is expected in localized and stage 4S neuroblastoma in infancy.

Adrenal Gland Neoplasms↗