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Biomedical subjects

P W Callen

Publications and source records attributed to P W Callen.

At least 19 recordsLinked to original sources

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

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

The choroid plexus pseudocyst: sonographic identification and characterization.

Amniocentesis has been advocated by some authors when fetal choroid plexus cysts are discovered, because of their reported association with karyotype abnormalities. We have noted an oval hypoechoic structure projecting into the adjacent choroid plexus, which simulates a cyst when imaged across its short axis, in 39 of 50 consecutive fetuses. This presumably normal structure or pseudocyst may represent the corpus striatum. Knowledge of its existence and benign nature will help avoid diagnostic errors and unnecessary amniocenteses.

Amniocentesis

Ultrasound-guided aspiration of a neonatal brain abscess.

The authors successfully treated a Serratia marcescens brain abscess in the right parieto-occipital region of a newborn infant born at 27 weeks' gestation and weighing 800 g. Bedside ultrasound-guided aspiration techniques and local anesthesia were used to treat the abscess.

Brain Abscess

Central nervous system damage and other anomalies in surviving fetus following second trimester antenatal death of co-twin. Report of four cases and literature review.

Four cases of multiple gestation with second trimester death of one fetus and subsequent damage to a survivor are reported. Monochorionic placentation was documented in three of the cases. Central nervous system lesions occurred in all cases, and bowel injury was noted in two of the damaged fetuses. Although rare maternal clotting problems have been reported in similar situations, none was noted in any of these four cases. Prior reports have indicated that losses in the first half of gestation were of no consequence to the surviving fetuses. These four cases contradict this suggestion, and indicate that close sonographic observation of the survivors is important in any multiple gestation where on fetus has died.

Abnormalities, Multiple

Fetal intervention in obstructive uropathy: prognostic indicators and efficacy of intervention.

Management of the fetus with bilateral hydronephrosis is controversial; ability to predict outcome and efficacy of prenatal intervention are unknown. We studied 40 fetuses referred for ultrasonography, examination of fetal urine, and possible therapy. We retrospectively assigned fetuses to a good prognosis group if fetal urine was hypotonic (sodium less than 100 mEq/L, chloride less than 90 mEq/L, osmolarity less than 210 mOsm/L) and there was no ultrasonographic evidence of dysplasia; we assigned fetuses to a poor prognosis group if even one criterion was abnormal. Survival was greater in the good prognosis group than in the poor prognosis group (81% vs 12.5%; 87% vs 30%, excluding abortions) (p less than 0.005). We then attempted to assess the efficacy of prenatal urinary decompression by comparing outcome within the good and poor prognosis groups. Survival with intervention was greater in both the good prognosis group and the poor prognosis group (89% vs 70% and 30% vs 0%). In 6 of the 8 survivors in the good prognosis group, severe oligohydramnios was reversed by decompression. We conclude the fetal urine electrolyte levels and ultrasonographic appear helpful in predicting residual fetal renal function and neonatal outcome and that prenatal decompression may prevent the development of fatal pulmonary hypoplasia.

Amniotic Fluid

Correction of congenital diaphragmatic hernia in utero, V. Initial clinical experience.

Review of our experience with 45 cases of prenatally diagnosed congenital diaphragmatic hernia (CDH) confirms that most fetuses (77%) will not survive despite optimal pre- and postnatal care. Polyhydramnios, associated anomalies, early diagnosis, and a large volume of herniated viscera (including liver) are associated with a particularly dismal prognosis. After extensive experimental work demonstrated the efficacy, feasibility, and safety of repair in utero, we attempted to salvage six highly selected fetuses with severe CDH by open fetal surgery. Five had liver incarcerated in the chest: three died at operation because attempts to reduce the liver compromised umbilical venous return. In one, a Goretex diaphragm was constructed around the liver, but the baby died after birth. The last two fetuses, one with incarcerated liver, were successfully repaired. Both demonstrated rapid growth of the lung in utero, had surprisingly good lung function after birth despite prematurity, had the abdominal patch removed at 2 weeks, and subsequently died of nonpulmonary problems (an unrelated nursery accident in one and intestinal complications in the other). The only maternal complication was amniotic fluid leak and preterm labor. All six women are well and four have had subsequent normal children. From this phase I experience, we conclude that fetal surgery appears safe for the mother and her reproductive potential, that fetal CDH repair is feasible in selected cases, and that the fetal lung responds quickly after decompression. However, fetal repair remains a formidable technical challenge.

Adolescent

Primary fetal hydrothorax: natural history and management.

Primary fetal hydrothorax presents a wide spectrum of severity ranging from small, harmless effusions, to life-threatening thoracic compression. To define natural history and management, we reviewed 32 cases seen at two large perinatal centers from 1980 to 1987. Spontaneous resolution of the effusions was seen in three fetuses, all of whom survived. Three fetuses were electively terminated. The overall mortality was 53%. In the 24 untreated fetuses, sex and the presence of polyhydramnios did not influence mortality, but hydrops, gestational age less than 35 weeks at delivery, and bilateral effusions were associated with a poor prognosis. Five fetuses underwent in utero decompression. In four, thoracentesis was performed, with rapid reaccumulation of the effusion. All four died from pulmonary insufficiency. In the fifth fetus, a thoracoamniotic shunt permanently decompressed the effusion, with resolution of the hydrops, and delivery of a normal viable infant. We conclude that (1) primary fetal hydrothorax may resolve or progress to hydrops, necessitating close follow-up with ultrasound; (2) pulmonary hypoplasia as a result of undrained large pleural effusions may result in neonatal mortality; (3) the gestational age at both diagnosis and delivery, the development of hydrops, and bilaterality of effusions are important prognostic predictors; and (4) the fetus with large effusions and hydrops has a poor prognosis, and thoracic decompression with a thoracoamniotic shunt may prove life saving.

Female

High-output cardiac failure in fetuses with large sacrococcygeal teratoma: diagnosis by echocardiography and Doppler ultrasound.

With two-dimensional echocardiography and Doppler ultrasound, we demonstrated high-output cardiac failure in three fetuses with large sacrococcygeal teratomas. All fetuses had normal cardiac structure, dilated ventricles maintaining a normal fractional shortening index, a dilated inferior vena cava reflecting the increased venous return from the lower body, pericardial and pleural effusions as a manifestation of fetal hydrops, and a markedly thickened placenta. When fetal hydrops was present, the combined ventricular output was very high (mean 1280 ml/min/kg; normal 553 +/- 153 (SD)). Descending aortic flow was also sharply increased (mean 930 ml/min/kg; normal 184 +/- 20), as was placental flow (mean 480 ml/min/kg, normal 110 +/- 26). High-velocity arterial flow signals were also found within the tumor. In one fetus studied serially, placental thickness and tumor diameter increased rapidly; placental flow as a percentage of descending aortic flow decreased, indicating a further increase of flow to the tumor. These abnormal hemodynamic changes were reversed after the fetus's teratoma was surgically removed. We conclude that the sacrococcygeal teratoma acts as a large arteriovenous fistula, which causes high-output cardiac failure. Surgical removal of the teratoma in a previable fetus with such hemodynamic findings may prove to be the most effective treatment.

Echocardiography

Effacement of the fetal cisterna magna in association with myelomeningocele.

The cisterna magna is effaced in association with myelomeningocele. The authors retrospectively investigated the size of the fetal cisterna magna as a predictor of fetal myelomeningocele in 67 pregnant women (17-38 menstrual weeks) referred for prenatal sonography because of an elevated serum alpha-fetoprotein level (n = 61) or a suspicion of fetal ventriculomegaly on previously obtained sonograms (n = 6). Twenty fetuses had myelomeningocele, 14 had isolated ventriculomegaly, and 33 were normal. A normal-sized cisterna magna (range, 4-9 mm in depth) was present in all normal fetuses. In 19 of 20 fetuses with myelomeningocele, the views of the posterior fossa were adequate, and in each of these the cisterna magna was effaced (n = 18) or very small (n = 1). The cisterna magna was effaced in five of 13 (38%) fetuses with isolated ventriculomegaly in whom the posterior fossa was adequately imaged. Although effacement of the cisterna magna is a nonspecific finding, the high negative predictive value of this sign is useful during routine screening of the fetal neural axis.

Cisterna Magna

Fetal tissue sampling. The San Francisco experience with 190 pregnancies.

Prenatal diagnosis of genetic defects was done using fetal blood sampling in 167 at-risk pregnancies, by fetal skin biopsy in 15 pregnancies, and by fetal liver biopsy in 8 pregnancies. Fetal blood sampling was done by fetoscopy through January 1985 and by sonographically directed percutaneous umbilical blood sampling since then. In our series, cytogenetics has become the major indication for fetal blood sampling, increasing from 6% of the cases with fetoscopy to 48% with umbilical blood sampling. Fetoscopy provided pure fetal blood in 61% of cases while umbilical blood sampling provided pure fetal blood 97% of the time. The corrected risk of fetal demise after percutaneous umbilical fetal blood sampling was 2% and after fetoscopy was 4%.

Biopsy

Early experience with open fetal surgery for congenital hydronephrosis.

The fetus with severe bilateral hydronephrosis and associated oligohydramnios in the second trimester is doomed at birth by ongoing pulmonary and renal damage. Since decompression with percutaneously placed catheters anesthetic, surgical, and tocolytic techniques for open fetal anesthetic, surgical, and tocolytic techniques for open fetal urinary tract decompression in animals, and have now applied those techniques to a small group of five patients. One had bilateral ureterostomies and the subsequent four had marsupialization of the bladder. All pregnancies proceeded to cesarean delivery at 32 to 35 weeks' gestation. There was no long-term maternal morbidity, and two mothers have since experienced normal pregnancies. Three fetuses had return of normal amniotic fluid dynamics, and all three had adequate pulmonary function at birth, suggesting that fatal pulmonary hypoplasia associated with early severe oligohydramnios had been reversed. Two neonates died at birth with pulmonary hypoplasia. One had no amniotic fluid even after decompression, and the other had some amniotic fluid after decompression but a tiny chest cavity due to the long period of severe oligohydramnios before decompression. Of the three surviving infants, one had normal renal function when she died of unrelated causes at 9 months of age. One has normal renal function at 23 months and the third had failing renal function at 2 1/2 years and has grown and developed normally, but will require renal transplantation. We have now developed selection criteria that would exclude from treatment the two fetuses who died of pulmonary hypoplasia and the one who developed renal failure.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Severity of intestinal damage in gastroschisis: correlation with prenatal sonographic findings.

From 1982 to 1986, 26 abdominal wall defects were detected prenatally. Seventeen pregnancies culminated in live births with ultrasound correctly distinguishing between omphalocele and gastroschisis in all cases. In the 11 cases of gastroschisis diagnosed before birth, we attempted to correlate the clinical outcome with the size of the abdominal wall defect, sonographic appearance of the eviscerated bowel, and known time of exposure to amniotic fluid (gestational age at birth to gestational age at diagnosis). Neither the time of exposure to amniotic fluid (median duration of 14 weeks) nor the defect size could be correlated with eventual clinical outcome. The presence of small bowel dilatation and mural thickening on prenatal sonography (four patients) had a high correlation with severe intestinal damage and poor clinical outcome. The absence of these two sonographic findings (seven patients) was associated with mild intestinal changes and benign clinical course with no morbidity or mortality. We conclude that obstetric ultrasound cannot only accurately detect the presence and type of abdominal wall defect, but it also gives an indication of the severity of intestinal damage and subsequent clinical course in prenatally diagnosed cases of gastroschisis. Early delivery of the fetus with prenatally diagnosed gastroschisis should no longer be performed to limit exposure to amniotic fluid. Now that reliable sonographic criteria of severe intestinal damage have been defined, the decision to deliver early can be restricted to those fetuses with bowel dilatation and mural thickening.

Abdominal Muscles

Amniotic sheets.

Seventeen cases of an aberrant sheet of tissue in the amniotic cavity are described to expand and clarify previous observations of this entity. The sheet of tissue demonstrates a thickened base and a free edge that undulates. The fetus moves freely about the sheet of tissue. There are no associated fetal deformities, and infants have no manifestations of the amniotic-band syndrome. Evidence suggests that these sheets may originate from "wrapping" of the amniochorionic membrane over a uterine synechia. These benign sheets of tissue should not be confused with the amniotic-band syndrome.

Adult

Factors affecting prenatal sonographic estimation of weight in extremely low birthweight infants.

Because critical management decisions are based on sonographic estimation of fetal weight in fetuses less than 1000 g, we sought to evaluate the accuracy of birthweight prediction in this range and to identify factors affecting this accuracy. Fetal weight was estimated using several published methods in 53 fetuses with birthweights less than 1000 g. Standard deviations greater than 12.3% indicate more random error in the sonographic weight prediction than has been reported in higher weight groups. No statistically significant differences were found between patient groups with decreased, normal, or increased amniotic fluid volume or portable examination. There was a trend toward lower mean deviation (2.9 vs 6.0%) and standard deviation (8.9 vs. 15.0%) in studies with scan quality judged "good" compared with "poor" based on ability to visualize anatomic landmarks.

Amniotic Fluid

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.

Adult