Prospective study of infantile haemangiomas: incidence, clinical characteristics and association with placental anomalies.

BACKGROUND: The aetiology and exact incidence of infantile haemangiomas (IHs) are unknown. Prior studies have noted immunohistochemical and biological characteristics shared by IHs and placental tissue. OBJECTIVES: We investigated the possible association between placental anomalies and the development of IHs, as well as the demographic characteristics and other risk factors for IHs. PATIENTS AND METHODS: Pregnant women (n = 578) were prospectively enrolled and their offspring followed for 9 months. Placental evaluations were performed and demographic data collected on all mother-infant pairs. RESULTS: We evaluated 594 infants: 34 haemangiomas [either IH or congenital (CH)] were identified in 29 infants, yielding an incidence of 4·5% for IH (27 infants) and 0·3% for CH (two infants). Placental anomalies were noted in almost 35% of haemangioma-related pregnancies, approximately twice the incidence noted in pregnancies with unaffected infants (P = 0·025). Other risk factors for IH included prematurity (P = 0·016) and low birth weight (P = 0·028). All IHs were present by 3 months of age, and cessation of growth had occurred in all by 9 months of age. Most occurred on the trunk. Of note, 20% of identified IHs were abortive or telangiectatic in nature, small focal lesions that did not proliferate beyond 3 months of age. Only one IH required intervention. CONCLUSIONS: This is the first prospective American study to document the incidence of IHs in infants followed from birth to early infancy. The association with placental anomalies was statistically significant. The overall incidence mirrors prior estimates, but the need for treatment was lower than previously reported.

Anesthetic management of a pregnant woman with Gorham-Stout disease.

Gorham-Stout disease is a rare disorder of bone loss and proliferation of lymphatic and vascular tissue (lymphangiomatosis). A 30-year-old nulliparous woman with Gorham-Stout disease presented at 8weeks of gestation with a fused cervical spine. At 31weeks she developed basilar invagination and neurological symptoms that were managed with a neck brace. Anesthetic considerations were those of airway compromise, development of severe preeclampsia and Kasabach-Merritt coagulopathy. Elective tracheostomy was declined. She presented two days before a planned cesarean delivery at 35weeks in preterm labor. A semi-urgent cesarean delivery under spinal anesthetic proceeded uneventfully, with an otolaryngologist present in case a surgical airway was required. Mother and baby were discharged home after three days. Maternal postpartum recovery was complicated by episodes of respiratory compromise and critical bone loss in the cervical spine, necessitating further surgical reinforcement.

Targeted mid-trimester ultrasound examination: how does fetal anatomic visualization depend upon the duration of the scan?

OBJECTIVE: To determine the relationship between visualization of key fetal anatomic structures during mid-trimester ultrasound examination with gestational age and duration of examination. METHODS: One hundred ultrasound examinations at 16-22 weeks' gestation were reviewed to determine the times at which key fetal anatomic features were seen. Scans were terminated at 30 min or when a comprehensive anatomic survey was complete. Exclusion criteria included multiple gestation, maternal weight>77 kg, abdominal wall scarring, and suspected fetal anomalies. RESULTS: Visualization of cranial anatomy including lips, face, midline, ventricles, choroid plexus, and cerebellum was achieved in 98% of patients within 30 min. The corresponding figures for spine, cardiac screening (four-chamber, aortic, and pulmonary outflow views) and for abdominal anatomy (stomach, kidneys, bladder, ventral wall, and three-vessel cord) were 91%, 91%, and 99%, respectively. A complete anatomic survey including each of the above elements was obtained by 10, 15, 20, 25, and 30 min in 8%, 31%, 53%, 72% and 81% of the subjects. Rates of complete anatomic surveys within 30 min improved by gestational age interval, from 20/30 (67%) at 16-18 weeks, to 36/44 (82%) at 18-20 weeks, and 25/26 (96%) at 20-22 weeks; this rise was primarily due to improvements in visualization of the spine and heart. CONCLUSIONS: A comprehensive anatomical survey can be completed in 10 min or less in a minority of patients. For each 5-min time increment up to 30 min, the rate of complete surveys improves. Rates of completed anatomic surveys rise with gestational age.

Choroid plexus cysts in the mid-trimester fetus--practical application suggests superiority of an individualized risk method of counseling for trisomy 18.

OBJECTIVES: Analysis of a referral population of patients with choroid plexus cysts (CPCs) was performed to compare an average risk method of counseling to an individualized risk method. METHODS: A total of 395 patients referred to a Prenatal Diagnosis Center were included, of whom 341 had isolated CPCs and 54 had associated ultrasound abnormalities. For isolated CPCs, an average risk of 1/150 for aneuploidy was compared to an individualized risk assessment [prior risk as determined by maternal age or serum screening multiplied by the likelihood ratio established by Gupta et al. (1997)]. Accuracy, cost, and procedure-related losses were assessed. RESULTS: Both methods resulted in 100% sensitivity. The individualized method resulted in greater specificity, decreased costs, and (theoretically) fewer procedure-related pregnancy losses. CONCLUSIONS: An individualized risk method of counseling utilizing the likelihood ratios established by Gupta et al. (1997) was superior to an average risk method for assessing trisomy 18 risk in the setting of CPC detected in mid-trimester.

Prenatal sonographic diagnosis of vasa previa: ultrasound findings and obstetric outcome in ten cases.

OBJECTIVE: To assess the specificity of sonographic diagnosis of vasa previa and pregnancy outcome in sonographically diagnosed cases. METHODS: We prospectively collected cases of vasa previa diagnosed by color Doppler sonography. Delivery by elective Cesarean section after demonstration of fetal pulmonary maturity and prior to the onset of labor was recommended unless obstetric complications supervened. Data regarding maternal obstetric courses and newborn status were collected. RESULTS: Eleven cases of vasa previa without placenta previa were diagnosed among 33 208 women over an 8-year period. Ten patients had confirmation of the diagnosis by the delivering obstetrician and/or placental examination, giving a specificity of diagnosis of 91%. Among the 10 patients with confirmed vasa previa, two were delivered at 31.5 and 35.5 weeks' gestation prior to demonstration of lung maturity, and the remainder were delivered at 34-37.5 weeks, after lung maturity. All infants had normal Apgar scores and survived. One baby had Scimitar syndrome, which was not suspected from sonography. CONCLUSIONS: The specificity of sonographic diagnosis of vasa previa at our center was 91%. Antenatal diagnosis permitted us to prevent the catastrophic outcomes commonly associated with vasa previa.

Acute respiratory distress syndrome in pregnancy and the puerperium: causes, courses, and outcomes.

OBJECTIVE: To describe causes, courses, complications, and outcomes of patients with pregnancy-associated acute respiratory distress syndrome (RDS). METHODS: Twenty-eight women with ARDS during pregnancy or within a week postpartum formed the study population. Eight cases had been reported previously. Charts were abstracted for maternal demographics, etiology, and treatment of acute RDS, and maternal outcomes. For antepartum acute RDS, newborn charts were also reviewed. RESULTS: The incidence of acute RDS, excluding maternal transports, was one per 6277 deliveries or 0.016% (95% confidence interval [CI] 0, 0.027%). Leading causes were infection (12 cases), preeclampsia or eclampsia (seven cases), and aspiration (three cases). Eleven mothers died, a maternal mortality rate of 39.3% (CI 21.5%, 59.4%). Six of eight women who were ventilated for over 14 days survived. Nine of the acute RDS cases might have been preventable. Ten mothers with living fetuses were ventilated during the third trimester; nine delivered within 4 days. Among six infants delivered because of fetal heart rate abnormalities, one died and at least three had evidence of asphyxia. CONCLUSIONS: Acute RDS occurs more frequently in pregnancy than the 1.5 cases per 100,000 per year reported for the general population. Prolonged ventilator support is warranted. The high rate of perinatal asphyxia in infants who have fetal heart rate abnormalities supports a strategy of expeditious delivery during the third trimester.

External cephalic version: predictors of success.

OBJECTIVE: To evaluate predictive variables for successful external cephalic version. METHODS: During 1987-1996, 128 women had external cephalic version attempts. Uterine tone, fetal spine position, breech location, breech type, gestational age, placental location, parity, maternal weight, amniotic fluid index, and estimated fetal weight were evaluated as predictors of success. RESULTS: Seventy-eight (64%) women were successfully converted from breech to vertex presentation. All subjects with low uterine tone had successful version. In women with high uterine tone, the combination of anterior or lateral fetal spine, noncornual placental location, and breech location out of the pelvis predicted success. Other independent variables associated with successful version included non-frank breech presentation, gestational age under 38 weeks, and parity of at least 1. CONCLUSION: Uterine tone may be the most important predictor of success when selecting candidates for external cephalic version.

Pre-term and particularly pre-labor cesarean section to avoid complications of gastroschisis.

The marked advantages and merit of pre-term and particularly pre-labor (PTPL) cesarean section (C-section) in the avoidance, and indeed, virtual elimination of severely disabling gastroschisis (GS) complications in infants diagnosed prior to birth by ultrasound has unfortunately remained controversial in the 10 to 12 years since it was first reported and strongly recommended by numerous authors. During this period, GS has remained one of the four major causes of the short-gut syndrome (SGS) in infancy and childhood and a major cause of prolonged, costly, complicated, and hazardous neonatal intensive care unit stays with requirements for total parenteral nutrition (TPN). The most serious and frequent complications of GS in infants born without PTPL C-section are the occurrence of the "peel", which greatly enlarges and rigidifies the eviscerated gut, and of "complicated GS" (intestinal atresia/s, stenosis, necrosis, perforations) (CGS). The "peel" occurs in 100% of these cases and CGS in approximately 20%. "Peel" enlargement and rigidification of eviscerated intestine in the presence of a reduced peritoneal cavity causes great difficulty in covering the eviscerated, enlarged, and rigidified gut with abdominal wall, skin, a prosthesis, etc., and frequently produces gut ischemia from excessive pressure, which may lead to necrotizing enterocolitis (NEC) and SGS as well as prolonged hospital stays. The presence of a "peel" greatly complicates the hazards of dealing with cases of CGS, as resection and anastomosis are virtually impossible in the presence of a "peel." The authors report personal experience with 77 cases of GS dating as far back as 1951; 44 of the infants were born after the onset of labor by vaginal or C-section delivery and all had some degree of "peel" formation. Of 320 cases from the literature (including some of the cases reported here), 61 (19.1%) involved CGS. Of the 33 cases born PT, and especially PL, there were no cases of "peel" and only 1 case of CGS (3.0%). This infant had a single atresia associated with a very small (1 cm) defect in the abdominal wall and no labor-induced "peel," which was easily and successfully repaired by resection and anastomosis. The 6.4-fold reduction in the occurrence of CGS by PTPL C-section (3.0% vs 19.1%) was statistically significant by the chi-square test (P < 0.05), as was the 100% elimination of the disabling "peel." If the single case of CGS associated with a very small defect and no labor or labor-associated "peel" is eliminated, the incidence of CGS in the remaining PTPL group of 32 cases falls to 0 (0% versus 19.1%, P < 0.007). PT and especially PL C-section may be expected to virtually eliminate "peel" formation and CGS and to remove GS as one of the four major causes of SGS. The findings of this report that PT labor prior to PT C-section may result in both "peel" formation and CGS further solidifies the role of labor in the production of both the "peel" and the equally disabling CGS. Failure to appreciate the central role of labor in GS complications has doubtless contributed to the persistent controversy concerning the value and importance of PTPL C-section for gastroschisis diagnosed in utero. The pediatric surgeon has an important responsibility with the obstetrician to monitor the possible occurrence of occult labor in the waning weeks of pregnancy and be prepared to do a prompt C-section if it occurs and there is adequate lung maturity. The achievement of "peel"- and CGS-free gut would greatly facilitate the use of the new Bianchi technique of gut reduction without anesthesia. The combination of the use of epidural anesthesia for the elective PTPL C-section with the Bianchi approach would spare both mother and baby any untoward effects of general anesthesia and present the potential for massive reductions in hospital costs with minimal patient manipulation and disturbance. For infants born with labor-associated "peel," re-evaluation of the suitability and effectiv

Hydroceles identified prenatally: common physiologic phenomenon?

Though the reported frequency of infant hydrocele has varied, the frequency of hydrocele in the fetus has not been studied. Our purpose was to determine the frequency of fetal hydrocele in the third trimester and subsequent outcome. Of the 123 fetuses studied, 19 fetuses had hydroceles. Of these, 14 babies were normal at follow-up, one baby had a persistent hydrocele, and four babies were lost to follow-up. Our study suggests that hydrocele is a relatively common finding in the third trimester in utero. Moreover, it suggests that parents can be reassured that, in the absence of other abnormalities, a hydrocele is usually a physiologic finding which resolves spontaneously.

Brain death during pregnancy: tocolytic therapy and aggressive maternal support on behalf of the fetus.

We report a case of maternal brain death at 25 weeks gestation in which aggressive maternal hemodynamic, respiratory, and metabolic support and tocolytic drug therapy resulted in prolongation of pregnancy for 25 days. The indication for delivery was torulopsis giabrata amnionitis, which may have occurred due to transmembrane or transplacental route. The baby was treated for fungal sepsis, and did well. Premature labor may occur spontaneously after maternal brain death, and may be precipitated by infection or by maternal drug therapy. The myriad of hemodynamic and endocrine issues associated with maternal brain death complicate the choice of tocolytic drugs, but this case illustrates that uterine activity can be successfully blocked, potentially diminishing risks to the newborn, following the tragedy of maternal brain death during pregnancy.

Adult respiratory distress syndrome in pregnancy: report of three cases and review of the literature.

Adult respiratory distress syndrome (ARDS) is rarely encountered in association with pregnancy, but with the decline in other causes of maternal death, is an increasingly important cause of mortality in obstetric patients. ARDS may result from a variety of different types of pulmonary injury; uniquely obstetric causes include preeclampsia, amnionitis-endometritis, obstetric hemorrhage, and tocolytic therapy. Crucial management issues include support of maternal oxygenation and cardiac output, myriad interactions between the pulmonary process and its treatment, with maternal and fetal physiology, and decision making regarding delivery. Our review of the literature suggests that, for the patient requiring antepartum intubation for ARDS, except at a very early gestational age or when pyelonephritis or varicella pneumonia is a cause of respiratory compromise, delivery will likely be required for maternal and/or fetal indications, and an early decision for delivery may be beneficial. Postpartum management is similar to treatment of the nonpregnant patient with ARDS, with aggressive attention to potential surgically correctable causes for infection. Maternal mortality rates are affected little by duration of intubation, and therefore prolonged mechanical ventilation is justified and appropriate for mothers with ARDS.

Ovarian vein thrombosis during cesarean section. A report of two cases.

BACKGROUND: Puerperal ovarian vein thrombosis occurs in 0.2-0.5% of deliveries. It is usually thought to result from infection, but it has been hypothesized that thrombosis may occur as a primary event, and radiologic studies raise the possibility that ovarian vein thrombosis may occur quite frequently. CASES: Case 1 had right ovarian vein thrombosis diagnosed at the time of nonemergency cesarean section for placenta previa. This was treated with ligation of the infundibulopelvic ligament above the level of the clot. Subsequent magnetic resonance imaging showed contralateral ovarian vein thrombosis, and therefore anticoagulant therapy was begun. The patient was asymptomatic. Case 2 had right ovarian vein thrombosis extending to the inferior vena cava diagnosed at elective repeat cesarean section. She was heparinized in the recovery room; subsequent ventilation/perfusion scan showed a probable pulmonary embolism. Both patients recovered uneventfully. CONCLUSION: These two cases demonstrate that ovarian vein thrombosis may occur as a primary event, in the absence of infection, and result in pulmonary embolism. Individualized management based on operative findings is recommended.

Pregnancies in a patient with congenital absence of prothrombin activity: case report.

Congenital hypoprothrombinemias are very rare, inherited disorders in which factor II (prothrombin) levels and/or activity are extremely low or absent. We report eight pregnancies in a patient with this disorder. Obstetric complications attributed to the coagulation disturbance included first-trimester bleeding in each pregnancy, miscarriage in four of the pregnancies, spontaneous maternal subarachnoid hemorrhage in one, and postpartum hemorrhage in one of four term pregnancies despite administration of clotting factor concentrate. The management of pregnancy in congenital hypoprothrombinemia, and issues of coagulation factor replacement, are discussed.

Placental cord insertion visualization with prenatal ultrasonography.

Color Doppler and gray scale sonography can be used prenatally to identify the location of the cord insertion into the placenta. The purposes of this paper were to (1) relate sonographic identification of placental cord insertion with placental pathology; (2) evaluate the possibility that a marginal cord insertion may evolve into a velamentous cord insertion; and (3) determine the frequency and factors affecting sonographic visualization of cord insertion. Our results show that the sonographic assessment of cord insertion correlated with the pathologic outcome in 83% (106 of 128) of singleton pregnancies and at least one of the fetuses in 72% (8 of 11) of twin or triplet pregnancies. Although the sensitivity for identification of an abnormal cord insertion was low (42%), the specificity was high (95%). Our data suggest that marginal cord insertion evolved into velamentous cord insertion in one singleton and one twin. Our results showed that cord insertion was visualized in 54% of fetuses scanned in a routine clinical practice. Cord insertion visualization was possible at all gestational ages, although it was more difficult at later gestational ages. In conclusion, this study provides evidence that (1) ultrasonography (either gray scale or color Doppler) is useful in identifying normal, marginal, and velamentous cord insertion; (2) marginal cord insertion may evolve into velamentous cord insertion as pregnancy progresses; (3) in clinical practice the cord insertion site was visualized in just over half of the cases, and (4) prenatal identification of marginal and velamentous cord insertion potentially may be useful for planning obstetrical management.

Sonographic diagnosis of traumatic and later recurrent uterine rupture.

We report a case of traumatic transverse fundal uterine rupture with fetal death, followed by recurrent rupture during the subsequent (twin) pregnancy. Rupture was sonographically diagnosed within minutes of arrival at hospital following a motor vehicle accident in the first pregnancy and defined operative intervention, which was lifesaving for the mother. Rupture was again diagnosed sonographically in the second pregnancy based on extrusion of the bag of waters through the uterine incision without fetal distress. Emergency cesarean section was performed with good outcomes for both babies. Causes and clinical/sonographic diagnosis of uterine rupture are reviewed and obstetric management discussed. A high level of suspicion, early diagnosis, and expeditious management are keys to favorable outcome.

Severe preeclampsia with fulminant and extreme elevation of aspartate aminotransferase and lactate dehydrogenase levels: high risk for maternal death.

We report a subgroup of patients with fulminant hemolysis, elevated liver enzymes, low platelet count (HELLP) syndrome, manifesting extreme elevation of aspartate aminotransferase (AST; SGOT) and lactate dehydrogenase (LDH) levels and abnormal mental status. These gravidas are at high risk for mortality. Only four patients treated by the authors over a 10-year period have had AST more than 2000 IU/L and LDH more than 3000 IU/L in the HELLP syndrome. This report is based on retrospective chart review. All patients manifested disordered mental status, jaundice, intense hemolysis, and extreme hypertension. One patient had developed multiple organ system failure, was moribund at initial perinatal consultation, and died. The three others were treated with aggressive afterload reduction and plasma infusion or plasmapheresis; two survived. Fulminant HELLP syndrome occurs rarely, but marks a group of patients at high risk for mortality. Optimal therapy is unclear; early intervention, including afterload reduction, volume expansion, and consideration of plasma infusions or plasmapheresis, is recommended.

Monoamniotic and pseudomonoamniotic twins: sonographic diagnosis, detection of cord entanglement, and obstetric management.

OBJECTIVE: To assess accuracy of detecting cord entanglement in monoamniotic twins, and to describe perinatal outcomes with aggressive obstetric management. METHODS: Seven nonconjoined monoamniotic twin pregnancies and one pseudomonoamniotic twin pregnancy were diagnosed sonographically and evaluated with serial scans and cardiotocography. In the absence of other indications, patients were delivered by elective cesarean on demonstration of lung maturity at or beyond 32 weeks' gestation. RESULTS: Cord entanglement was diagnosed correctly in four pregnancies, missed in one, and excluded correctly in three. Four pregnancies were delivered after demonstration of pulmonary maturity, three because of premature rupture of membranes or uncontrollable preterm labor, and one because of fetal heart rate abnormality during tocolysis for preterm labor. The mean gestational age at delivery was 33.2 +/- 1.6 weeks, with birth weight 2011 +/- 262 g; all neonates were live-born. Newborn stays averaged 12.0 +/- 5.8 days for the eight neonates delivered electively. CONCLUSION: Monoamniotic twin pregnancies and cord entanglement in such twins were diagnosed reliably by ultrasound. Abnormal tracings prompting cesarean delivery occurred in two of the five pregnancies with cord entanglement. Amniocentesis reflected pulmonary maturity of both twins in all pregnancies so assessed, and delivery after 32 weeks' gestation, with lung maturity, resulted in good perinatal outcomes. Statistical validity of these findings is limited by our small sample size.

Prenatal sonographic diagnosis of intracranial haemorrhage: report of a case with a sinusoidal fetal heart rate tracing, and review of the literature.

The sinusoidal fetal heart rate pattern has been described in association with severe fetal anaemia, with fetal hypoxaemia, and with the administration of parenteral narcotics. Here, we report a case of decreased fetal movement in which a sinusoidal tracing was recorded. The sonographic diagnosis of a massive fetal intracranial haemorrhage was made. A non-interventive approach was taken and the fetus died soon after in utero. We review 28 previous cases in which the prenatal sonographic diagnosis of fetal intracranial haemorrhage was made, including the underlying maternal and fetal factors and neonatal outcomes. We propose that the sinusoidal tracing in this case was due to the intracranial bleed and suggest that fetal intracranial haemorrhage be considered in the sonographic evaluation of the fetus with a sinusoidal pattern.