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Smith-Lemli-Opitz syndrome (SLOS) is caused by an inborn error of cholesterol metabolism that results in deficiency of cholesterol and accumulation of the cholesterol precursor, 7-dehydrocholesterol (DHC) and its isomer, 8-DHC. Affected patients present with dysmorphic facial features, congenital anomalies, and growth and mental retardation. Postnatal treatment with cholesterol supplementation has been shown to improve plasma sterol levels in most patients. The most significant clinical improvement is seen in patients who began treatment at the youngest ages.

The objective of this article is to describe maternal morbidity in a large cohort of triplet pregnancies managed by a single Maternal-Fetal Medicine group over a short period of time. Records from all triplet pregnancies managed and delivered from 1992 to 1996 were reviewed for maternal outcome data. Pregnancies delivered prior to 20 weeks were excluded. During the 4-year study period, 55 triplet pregnancies were managed and delivered at this center. The most common maternal complication was preterm labor, which occurred in 42 cases (76%). Preterm premature rupture of membranes occurred in 11 cases (20%). Pregnancy-induced hypertensive complications occurred in 15 cases (27%), which included severe preeclampsia 13 (24%), hemolysis, elevated liver function tests, and low platelets (HELLP) syndrome 5 (9%), and eclampsia 1 (2%). Other maternal antenatal complications included anemia 15 (27%), acute fatty liver of pregnancy 4 (7%), gestational diabetes 4 (7%), supraventricular tachyarrhythmias 2 (4%), dermatoses 2 (4%), urinary tract infection 2 (4%), and acute disc prolapse requiring surgery in 1 case (2%). Postnatal complications occurred in 18 cases (33%), including endometritis 13 (24%), postpartum hemorrhage 5 (9%), pneumonia 2 (4%), urinary tract infection 2 (4%), and diastasis of rectus muscles requiring surgery in 1 (2%). There were no maternal deaths. Antenatal and postnatal maternal complications occur in almost all triplet gestations, suggesting that such pregnancies be managed at centers that have appropriate multidisciplinary expertise available.

SIR—The shared placental circulation of monochorionic twins predisposes a surviving twin to devastating neurological injury in the event of co-twin demise. We report use of fetoscopic cord ligation specifically to prevent neurological injury in monozygous twins in which severe congenital heart disease incompatible with life in one twin threatened the normal co-twin. A 32-year-old woman with one previous child was referred for evaluation of a 24-week twin gestation. Ultrasound revealed a single posteriorly placed placenta, same-sex twins, and a two-layered membrane consistent with monochorionic twins. Twin A was structurally normal, but fetal echocardiography of twin B revealed hypoplastic left heart syndrome associated with the unusual finding of severe right ventricular dysfunction, marked left ventricular endocardial fibroelastosis, severe tricuspid regurgitation, and early signs of non-immune hydrops indicating imminent fetal demise. Under combined epidural and general anaesthesia (providing maternal and fetal anaesthesia and uterine relaxation for fetal surgery), two 3 mm ports were placed under ultrasound guidance through 2-0 silk pledgeted purse-string sutures in the myometrium incorporating the amnion to prevent postoperative amniotic fluid leakage. A 2-0 vicryl suture was used for umbilical cord ligation under fetoscopic and ultrasonographic control, after which twin B became bradycardic and died. Trocars were removed and the pursestring sutures were tied, and the abdomen closed in layers. Follow-up sonograms revealed no central nervous system changes in the surviving twin. 8 weeks after the procedure the mother spontaneously ruptured her membranes and vaginally delivered a 1535 g neurologically intact baby girl with Apgars of 9 at 1 min and 9 at 5 min. Postnatal ultrasound revealed only an old unilateral grade I intraventricular haemorrhage. At 6 months computed tomography of the brain was normal and at 1 year of age she is neurologically intact and has met all developmental milestones. Necropsy on twin B confirmed the echocardiographic findings. Neurological injury in a surviving co-twin of a monochorionic gestation complicated by intrauterine demise has been estimated to occur in up to 46% of fetuses. The nature of the neurological injury is devastating with multicystic leucoencephalomalacia the most common pathology. The pathophysiology of the neurological injury in the surviving co-twin is thought to be acute hypotension and ischaemia occurring at the time of co-twin demise. If co-twin demise can be anticipated then fetoscopic cord ligation may prevent such injury. Although uterine exposure by laparotomy was necessary, it allowed precise mapping of the amniotic sacs and purse-string closure of the trocar sites. Although an entirely percutaneous technique has been reported, closure of the trocar sites is not possible and amniotic fluid leakage occurs in up to 30% of patients. Unlike previous cases, the indication for fetoscopic cord ligation in our case was specifically to prevent neurological injury and death of the normal co-twin.

Objective. —To develop a consensus on the use of antenatal corticosteroids for fetal maturation in preterm infants. Participants. —A nonfederal, nonadvocate, 16-member consensus panel including representatives from neonatology, obstetrics, family medicine, behavioral medicine, psychology, biostatistics, and the public; 19 experts in neonatology, obstetrics, and pharmacology presented data to the consensus panel and a conference audience of approximately 500. Evidence. —An extensive bibliography of references was produced for the consensus panel and the conference audience using a variety of on-line databases including MEDLINE. The consensus panel met several times prior to the conference to review the literature. It also commissioned an updated meta-analysis, a neonatal registry review, and an economic analysis that were presented at the conference. The experts prepared abstracts for distribution at the conference, presented data, and answered questions from the panel and audience. The panel evaluated the strength of the scientific evidence using the grading system developed by the Canadian Task Force on the Periodic Health Examination and adapted by the US Preventive Services Task Force. Consensus. —The consensus panel, answering predefined consensus questions, developed their conclusions based on the scientific evidence presented in open forum and the scientific literature. Consensus Statement. —The consensus panel composed a draft statement that was read in its entirety at the conference for comment. The panel released a revised statement at the end of the conference and finalized the revisions a few weeks after the conference. Conclusions. —Antenatal corticosteroid therapy is indicated for women at risk of premature delivery with few exceptions and will result in a substantial decrease in neonatal morbidity and mortality, as well as substantial savings in health care costs. The use of antenatal corticosteroids for fetal maturation is a rare example of a technology that yields substantial cost savings in addition to improving health. (JAMA. 1995;273:413-418)

A patient was referred at 21 weeks' gestation for evaluation of an echogenic mass in the fetal right chest. Color-doppler sonography facilitated the diagnosis of a bronchopulmonary sequestration. Clinicopathologic correlation was possible after termination at 2.3 weeks' gestation. Pathologic examination confirmed the prenatal diagnosis and demonstrated the systemic vascular supply seen on color-doppler sonography. Color doppler is a useful adjunct in the sonographic evaluation of the fetus with an echogenic chest mass.

Ultrasonographic measurement of fetal foot length is useful in the assessment of gestational age. Two hundred twenty-three postpartum and 224 ultrasonographic measurements were performed between 11 and 43 weeks' gestation; 207 postpartum and 160 ultrasonographic measurements met our requirements of secure dates and no physical anomaly or maternal disease. Mean foot length at each week of gestation compared favorably with Streeter's data, based on pathologic specimens, described in 1920. Comparison of curvilinear regression of foot length versus gestational age demonstrated a strong correlation with an R2 value of 0.981; 95% confidence intervals at each week compared favorably with both biparietal diameter and femur length data. Fetal foot length is a reliable parameter for use in the assessment of gestational age and is particularly useful when other parameters do not accurately predict gestational age, for example, hydrocephalus, anencephaly, short limb dysplasia.

The in utero sonographic detection of some of the more subtle anomalies of the hand and foot is now possible with the improved resolution of the newer ultrasound equipment. Nine case reports of fetal hand and/or foot deformities are presented, with 12 total lesions. The deformities include clinodactyly, symbrachydactyly, talipomanus, talipes, and "rockerbottom" foot. The sonographic appearance of each of these is discussed.

A case of congenital neuroblastoma is presented. The interesting features are maternal symptomatology consistent with fetal production of catecholamines and the dilemmas of differential diagnosis. Observations are made on the frequency and diagnosis of fetal neoplasia. Antepartum detection of catecholamine metabolites may be the basis for the diagnosis of fetal neuroblastoma.

In a prospective study of 69 consecutive twin gestations, chorionicity was assessed ultrasonographically, by the new technique of counting the number of layers visualized in the dividing membrane. For purposes of the study the antenatal categorization of chorionic type was based only on the number of layers observed. The pregnancy was classified as monochorionic when only two layers were identified and as dichorionic if three or four layers were seen. Confirmation of chorionic type was obtained after delivery by histopathologic examination of the placenta. The ultrasonographic technique used correctly determined chorionicity in 68 of 69 twin pregnancies. The predictive accuracy was 100% for 51 pregnancies designated by ultrasonography as dichorionic and 94.4% for 18 pregnancies considered to be monochorionic. These results suggest that counting the number of layers seen in fetal membranes by ultrasonography examination is an accurate way of determining chorionicity in twin gestation.