In utero diagnosis of obstructed supracardiac total anomalous pulmonary venous connection in a patient with right atrial isomerism and asplenia.

We describe a rare case of right atrial isomerism, levocardia, right-side stomach, obstructed supracardiac total anomalous pulmonary venous connection, double outlet right ventricle with complete atrioventricular septal defect and absent spleen. From the pulmonary venous confluence behind the atrium an ascending as well as a descending vertical vein communicated with the systemic venous system in the supracardiac as well as the infracardiac position. The pulsed and color Doppler examination of the individual pulmonary veins as well as of the vertical vein helped in making the diagnosis of obstructed total anomalous pulmonary venous connection. The diagnosis was made by fetal echocardiographic examination at 22 weeks of gestation and confirmed on postnatal echocardiography, cardiac catheterization, and at surgery.

Double-outlet right ventricle with intact ventricular septum in a foetus with trisomy-18.

A rare case of double-outlet right ventricle with intact ventricular septum diagnosed by foetal echocardiography at 21 weeks of gestation is described. Amniocentesis revealed trisomy-18. The cardiac diagnosis was confirmed at autopsy.

Indirect prediction of fetal lung maturity. Value of ultrasonographic colonic and placental grading.

OBJECTIVE: To correlate fetal morphometrics with studies of fetal lung maturity. STUDY DESIGN: One hundred six patients undergoing amniocentesis for fetal lung maturity studies were examined prospectively. Eighty-four patients were normal (79%), and 22 were diabetic (21%). Fetal morphometrics were obtained prior to amniocentesis. The fetal colon and placenta were graded. Discriminant analysis was used to identify variables that were predictive of a mature lecithin/sphingomyelin ratio and the presence of phosphatidylglycerol (PG). All patients delivered within 48 hours of amniocentesis. RESULTS: In the normal group, 28 (33%) fetuses had a grade 3 colon, which was 68% sensitive and 98% specific for a mature amniocentesis. A grade 3 colon was the single best predictor of a mature amniocentesis (P < .001). Twenty-five (29%) fetuses had a grade 3 placenta, which was 64% sensitive and 96% specific for a mature amniocentesis (P < .005). Diabetes did not influence colonic grading since a grade 3 colon was present in seven (32%) patients (47% sensitivity and 100% specificity for PG) (P < .02). Interexaminer and intraexaminer variability for the study was excellent, kappa = 1.0 (P < .001). CONCLUSION: Colonic and placental stage 3 grading are reliable and reproducible ultrasonographic scales that can help predict the findings of fetal lung maturity studies.

Syntelencephaly in an infant of a diabetic mother.

Here we report on an infant of a diabetic mother (IDM) with midline interhemispheric "fusion" (MIF), or syntelencephaly. This is a rare anomaly characterized by segmental failure of cleavage of the cerebral hemispheres and other brain structures in the posterior frontal and parietal regions, with a normal interhemispheric fissure anterior and posterior to the "fused" region. While there is obvious overlap with holoprosencephaly (HPE), this condition differs from HPE in that the midline "fusion" in MIF is complete but segmental, while the structural brain anomalies seen in the HPE spectrum progress smoothly in severity in a posterior to anterior "fusion." However, while it is apparent that there are key distinctions between MIF and HPE, in all likelihood they arise from a similar pathogenetic mechanisms. We therefore suggest that MIF is a distinct variant of the HPE spectrum of midline brain anomalies. Given the known increased incidence of HPE in IDMs, MIF is likely a maternal diabetes-associated malformation.

Prenatal myocardial infarction suspected by fetal echocardiography.

Myocardial infarction is rare in the newborn period. We describe a case in which myocardial infarction was suspected at 25 weeks of gestation by fetal echocardiography. There was an aneurysm of the left ventricular apex with paradoxical motion and bulging of the left ventricular free wall during systole. The diagnosis was confirmed by postnatal electrocardiogram, vectorcardiogram, and thallium myocardial perfusion imaging.

Porphyria cutanea tarda and pregnancy.

Porphyria cutanea tarda is the most common disorder of porphyrin metabolism in Europe and North America. The disorder is characterized by specific cutaneous lesions, associated systemic findings, and excessive accumulation and excretion of uroporphyrin and coproporphyrins. Reports of this condition associated with pregnancy are scarce in the literature. In this review, we present the case of a patient with porphyria cutanea tarda to illustrate the natural progression and complications of the disorder during pregnancy. Based in this report and a review of the published cases, pregnancy may exacerbate the cutaneous lesions of porphyria cutanea tarda during the first trimester. The incidence of diabetes, antinuclear antibodies, and hepatitis B and C are increased among them, making glucose tolerance and antibody testing mandatory. Newborns should be tested for the disorder during the neonatal period. Genetic counseling is advisable as well as teaching avoidance of provocative factors in affected children.

Vacuum sources in obstetrics.

OBJECTIVE: To characterize the performance of vacuum equipment used in operative vaginal deliveries. STUDY METHOD: We measured wall suction in eight Cleveland area labor-and-delivery units. Additionally, we tested samples of hand-operated and electric vacuum pumps. For each apparatus we recorded vacuum in millimeters of mercury using a calibrated, diaphragm-type gauge. We calculated mean maximal vacuum. Work required to produce 500 mm Hg of vacuum was determined for the hand-operated pumps. At increments of vacuum, we compared the vacuum displayed by the gauge head included with the device to our standard. RESULTS: Mean wall suction was 494 mm Hg (range, 248-655). As compared to the Mity-vac, the CMI hand-operated pump needed significantly more work to generate 500 mm Hg of vacuum. Electric pumps produced a reliable and consistent vacuum. Gauges included with the pump had an average error of 4% over 400-600 mm Hg. CONCLUSION: Wall suction was an unreliable source of vacuum. The two hand-operated pumps tested required significantly different amounts of work to achieve an adequate vacuum. Addition of a fluid trap does not affect the vacuum, and head gauges supplied with vacuum pumps are accurate.

The effect of artificial caput on performance of vacuum extractors.

OBJECTIVE: To isolate and describe the effect of chignon formation (vacuum-induced caput) on the performance of six vacuum extractors. METHODS: Six vacuum extractors were examined in the laboratory: the 6-cm Malmstrom, Mityvac, M-Type, Silc, Silastic, and Tender-Touch. The devices were tested in two model systems that duplicated the fetal scalp with and without chignon. A fetal cephalic model mimicked the head without chignon. The second model used the hind quarters of anesthetized dogs. Chignon was formed by slowly increasing vacuum until the target vacuum was reached. At increments of vacuum, traction was applied in the axis of the cup and maximal tractive force (pop-off) was measured. RESULTS: A statistically unique regression line described each device. There was no significant difference in the regression lines for the Malmstrom and M-Type cups with or without chignon. At equal vacuum, there was a significant decrease (P < .05) in maximal tractive force with chignon formation in the bell-shaped cups. CONCLUSIONS: There is a demonstrable deterioration in the performance of bell-shaped cups with the formation of chignon. These data suggest that when using a bell-shaped extractor, efforts should be directed toward minimizing chignon formation by applying vacuum only with traction.

Effect of angular traction on the performance of modern vacuum extractors.

OBJECTIVE: Our objective was to describe the effect of off-axis traction on the performance of modern vacuum extractors. STUDY DESIGN: Eight vacuum extractors were examined in the laboratory with a force indicator and fetal cephalic model. Devices evaluated included the 6 cm Malmstrom, Mity-Vac, M-Type, O'Neil, Posterior, Silc, Tender-Touch, and silicone elastomer. Maximal tractive force (pop-off) was measured for each device at 10-degrees increments from the vertical. RESULTS: Multivariate regression analysis described the best model as follows: Maximal tractive force = Constant + Angle + Vacuum (p < 0.05). The partial regression coefficients for angle were negative in all devices except the Posterior cup. At increasing angles of off-axis traction, maximal tractive force decreased in the following order: Silc, silicone elastomer, Tender-Touch, M-Type, Mity-Vac, O'Neill, Malmstrom, and Posterior. CONCLUSIONS: Application of oblique traction resulted in a linear decline in maximal tractive force. An understanding of in vitro performance may allow tailoring of cup selection to the clinical situation.