Prenatal diagnosis of cleidocranial dysostosis.

BACKGROUND: Cleidocranial dysostosis is an autosomal dominant disorder characterized by absent or hypoplastic clavicles and dysplasia of the osseous tissue in the cranium. CASE: We describe the prenatal diagnosis and neonatal evaluation of cleidocranial dysostosis in the pregnancy of a woman also affected with this disorder. CONCLUSION: The sonographic appearance of absent or hypoplastic clavicles, in the absence of other findings suggesting a skeletal dysplasia, strongly suggests the diagnosis of cleidocranial dysostosis. Nomograms are available to follow the growth and development of normal clavicles. Assessment of normal long-bone growth in relation to clavicular size may aid in the diagnosis.

Sonographic fetal growth curves from an indigent population in Atlanta, Georgia: I. Singleton pregnancies.

The sonographic estimation of gestational age relies on standards of normal fetal growth established in highly selective patient populations. The majority have used middle-class, Caucasian gravidas with excellent menstrual dating criteria. These reference populations differ considerably from the indigent patient population in most southern medical centers: menstrual dating is often imprecise and there is a high rate of low birthweight infants delivered. Therefore, we established models of fetal growth in a southern, indigent, predominantly black patient population. We also compare our study to other sonographic studies.

Congenital complete heart block associated with maternal anti-Ro antibody: a case report.

The presence of anti-Ro antibody in maternal serum is a serologic marker for increased risk of the development of congenital complete heart block in the fetus. Current etiology and evaluation of these mothers and their fetuses are examined. A case report is presented.

Effects of gestational weight gain in morbidly obese women: I. Maternal morbidity.

Current recommendations for appropriate weight gain in pregnancy suggest an optimum of 120% of ideal body weight (IBW) at delivery. This represents an increase of approximately 24 pounds in the normal weight woman and even the obese patient (more than 135% IBW) is told to gain 16 pounds. Information concerning gestational weight gain in the morbidly obese woman (more than 160% IBW) has not been reported. We evaluated 40 morbidly obese pregnant women for maternal morbidity relative to gestational weight gain. No correlation was found between maternal weight gain and the development of gestational diabetes, pregnancy-induced hypertension, preeclampsia, preterm labor, premature rupture of membranes, incompetent cervix, or intrauterine growth retardation. The incidence of primary cesarean delivery was statistically greater in those women gaining more than 24 pounds (p less than 0.05). It appears that current recommendations for gestational weight gain in the morbidly obese are excessive and may result in increased maternal risk.

Effects of gestational weight gain in morbidly obese women: II: Fetal morbidity.

Recommended gestational weight gain is based on supplying adequate nutrition to the conceptus, ensuring normal growth and development. Since morbidly obese women have an overabundance of nutrients for fetal transfer, we examined the effects of weight gain on fetal outcome in women greater than 160% of ideal body weight. Gestational weight gains varied from -18 to +64 pounds, allowing analysis of fetal outcome based on maternal weight gain. No correlation between maternal weight gain and ketonuria, birthweight or length, placental weight, or Apgar scores was noted. Gestational age at delivery was inversely related to weight gain. When grouped by maternal weight gain, fetal outcome was no different if mothers gained less than or greater than 10 pounds. We conclude that limited weight gain in the morbidly obese women does not adversely affect fetal outcome, and prospective trials of limited weight gain diets in morbidly obese women are warranted.

Difficulty of fetal monitoring in a fetus with intracardiac tumors.

Fetal intracardiac tumors are rare but, when present, are a common cause of poor or uninterpretable fetal heart tracings due to fetal arrhythmias. The most frequently encountered tumor, the rhabdomyoma, is associated with tuberous sclerosis. We present a case demonstrating this difficulty of fetal monitoring in a fetus with an arrhythmia later found to be due to multicentric intracardiac rhabdomyomas. The appropriate evaluation and management of such fetal heart tracings are discussed.

Hemodynamic consequences of severe pancreatitis.

Fifteen patients with severe pancreatitis underwent cardiovascular monitoring in an intensive care unit. The principal findings were a high cardiac index and a decrease in systemic vascular resistance. A significant negative correlation was found to exist between these two parameters (p less than 0.001). Severe pancreatitis apparently results in hemodynamic changes similar to those observed in sepsis. The mechanisms responsible for these observations are not known, although circulating vasoactive compounds resulting from pancreatic necrosis remain a strong possibility. Despite demonstrating a significant decrease in left ventricular stroke work index and an abnormal elevation in pulmonary capillary wedge pressure, the existence of a myocardial depressant factor could not be conclusively proved from these data.