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[Doppler ultrasound study of a twin pregnancy with feto-fetal transfusion syndrome].

A report is made on a typical case of twin-twin transfusion syndrome of monozygotic twins with severe polyhydramnios. By treatment with decompression amniocentesis, we achieved prolongation of the pregnancy by 10 weeks. Daily performed Doppler velocimetry showed, that the resistance values did not differ before and after amniocentesis. We found the greatest differences between the pulsatility indices of the umbilical cords and the aortae. The arteriovenous anastomosis was visualized by colour flow mapping.

Adult↗

[Prenatal management of fetofetal transfusion syndrome].

The twin-twin transfusion syndrome is a rare but severe complication in monozygotic twins. A total of 172 twin pregnancies were investigated in our hospital between January 1990 and August 1993. The patients were divided into 4 groups: Group I: Normal twin pregnancy, treatment only in our hospital. Group II: Twin-twin transfusion syndrome, treatment only in our hospital. Group III: Normal twin pregnancy, delivery in another hospital. Group IV: Twin-twin transfusion syndrome, after treatment and therapy delivery in another hospital. These cases were analysed concerning prenatal management and perinatal outcome. The perinatal mortality rate in group I-IV was 17%, 58%, 18% and 60%, respectively. A benefit of aggressive amniocentesis in case of polyhydramnios, administration of digoxin and timing of labour could be demonstrated.

Adult↗

Nonimmune hydrops fetalis due to congenital syphilis associated with negative intrapartum maternal serology screening.

We present an unusual case, in which a woman presenting with markedly decreased fetal movements at 29 weeks gestation following a recent increase in fundal height was noted sonographically to have fetal hydrops consisting of scalp edema, marked hepatomegaly, ascites, and polyhydramnios. No lethal structural congenital anomaly was noted. Admission laboratory examinations revealed a negative antibody screen and a negative RPR. Emergent cesarean section was performed due to prolonged fetal bradycardia during biophysical profile testing. The acidotic hydropic neonate weighing 1825 g was resuscitated yet succumbed at 3 hr of life following intravenous administration of antibiotics. Neonatal blood was RPR positive at 1:16. Postmortem pathology examination demonstrated severe multiorgan system failure secondary to overwhelming congenital syphilis. Extensive extramedullary hematopoiesis was noted and histopathology with Dieterle stains revealed numerous hepatic spirochetes. Postpartum reexamination of the maternal blood with serial dilutions revealed a positive RPR at 1:1024. This case emphasizes that initial negative screening for syphilis may be seen despite overwhelming infection, a condition that has been termed the "prozone effect."

Adult↗

Prenatal ultrasonographic diagnosis of fetal intracranial tumors: a review.

Our objective was to review current literature pertaining to prenatal ultrasonography of various fetal intracranial neoplastic and non-neoplastic tumors. To this goal, all manuscripts published in the English language regarding this topic obtained from a MEDLINE search from 1966 through January 1998 were selected and reviewed. Additional sources were identified through cross-referencing. Intracranial fetal tumors are extremely rare and precise diagnosis is dependent on histology examination of tissue obtained at subsequent surgery or autopsy. Currently, prenatal ultrasonographic findings associated with the following fetal intracranial tumors have been described: teratomas; neuroepithelial tumors including: glioblastoma, astrocytoma, gangliocytoma, medulloblastoma, choroid plexus, and papilloma; and mesenchymal tumors. Non-neoplastic fetal intracranial tumors are even less frequent and include: unilateral megalencephaly, heterotopia, and lipoma of the corpus callosum. Cardinal ultrasonographic findings associated with fetal intracranial tumors include: echogenic and semicystic space occupying lesions with or without distortion of normal symmetrical intracranial (usually midline) structures, calcifications, craniomegaly, polyhydramnios, obstructive hydrocephaly, high-output cardiac failure (hydrops fetalis), the presence of other associated structural anomalies, and infrequently abnormal cerebral Doppler flow velocimetry.

Astrocytoma↗

Prenatal diagnosis of asphyxiating thoracic dysplasia (Jeune syndrome).

Asphyxiating thoracic dysplasia (ATD), or Jeune syndrome, is an uncommon autosomal recessive skeletal disorder characterized by a small thorax, varying degrees of rhizomelic brachymelia, polydactyly, pelvic abnormalities, and renal anomalies. We describe prenatal sonographic examinations in the third trimester of a fetus with abnormal small thorax, short limbs, polyhydramnios, and absence of fetal respiratory movements. At 36 weeks gestation, the fetal biparietal diameter was 93 mm, compatible with 37 weeks; the long-bone lengths measured < 5th percentile; the thoracic circumference (TC) measured 230 mm (< 5th percentile); the abdominal circumference (AC) measured 286 mm (25th percentile), and the TC/AC ratio was 0.80 (lower limit of normal). Our case shows that, although a discrepancy between gestational age and TC is prominent, TC/AC ratio may reach lower limit of normal in cases of fetal ATD, when fetal growth is compromised.

Adult↗

Prenatal sonographic diagnosis of Treacher Collins syndrome: a case and review of the literature.

We report the prenatal ultrasonographic diagnosis of a fetus with mandibulofacial dysostosis (Treacher Collins syndrome). Sonographic findings included: polyhydramnios, microcephaly, abnormal fetal facial features (slanting forehead, microphthalmos, micrognathia), and abnormal fetal swallowing. A review of the current literature pertaining to prenatal diagnosis of this condition is presented.

Adolescent↗

Ultrasound prediction of fetal pulmonary hypoplasia in pregnancies complicated by oligohydramnios and in cases of congenital diaphragmatic hernia: a review.

The objective of this review is to discuss the current knowledge of fetal pulmonary hypoplasia and to summarize the clinical significance of the many ultrasound methods for predicting pulmonary hypoplasia in pregnancies complicated by oligohydramnios due to spontaneous rupture of the fetal membranes and in cases complicated by fetal congenital diaphragmatic hernia. We concluded that the presence or absence of polyhydramnios, fetal breathing movements, mediastinal shift, thoracic position of the stomach, fetal breathing-related nasal and oropharyngeal fluid flow, ductal flow velocity modulation, and gestational age at onset and severity of ventricular disproportion as useful markers for predicting fetal pulmonary hypoplasia is a productive area for continued research. All studies show that there is a clear association between most of these markers and pulmonary hypoplasia. However, these markers have not been studied together in a large number of cases, and comparisons between each of the markers is unknown.

Animals↗

Right-sided diaphragmatic hernia associated with superior vena cava syndrome.

The term "hydrops fetalis" denotes generalized fetal edema, a condition that differs from localized edema confined to head, extremities, or body cavities. This report illustrates a case of right-sided diaphragmatic hernia associated with severe edema of the head, polyhydramnios, small hydrothorax, and ascites with an antenatal diagnosis of hydrops fetalis. At autopsy, edema was confined to the head and neck and the peritoneal sac. The mechanisms responsible for these forms of localized edema were obstruction of the superior vena cava and hepatic veins, respectively. Localized fetal edema may be confused with hydrops fetalis. Fetal edema of the head and neck may result from obstruction of the superior vena cava by abnormal mediastinal structures.

Adult↗

Prognostic factors and management in pregnancies complicated with severe kell alloimmunization: experiences of the last 13 years.

Because of the recent referral of an anti-Kell sensitized pregnant woman, whose fetus became severely anemic despite intensive antepartum surveillance, the prevalence and characteristics of fetal Kell isoimmunization were reviewed and analyzed. Cases with Kell and RhD alloimmunization requiring intrauterine intravascular transfusions (IUT) at the Mount Sinai Medical Center during the 13-year period ending March 1998 were compared. Thirty-six fetuses with RhD and 5 with Kell isoimmunization required IUTs. Lower fetal and neonatal hematocrit levels were observed in the RhD group. A significantly higher incidence of polyhydramnios was found among fetuses with Kell isoimmunization and the maternal serum titers were much lower than those in the RhD group. DeltaOD450 values did not reliably reflect the Kell sensitized fetus's condition. There were no intrauterine deaths or neonatal direct hyperbilirubinemia in the Kell group, and the hemolytic disease of the newborn was more severe in the RhD group. Although the course of the hemolytic disease in our cases of Kell isoimmunization showed a better prognosis than that in the RhD group, the importance of this condition should not be underestimated, and differences in the pathophysiology of Kell and RhD alloimmunization should be taken into consideration during the management of these cases.

Adult↗

Effect of type of anesthesia on blood loss at elective repeat cesarean section.

It has recently been reported that the use of halogenated agents during balanced general anesthesia may result in an increase in blood loss associated with cesarean section. This report has been criticized for failure to control for a variety of other factors that may have contributed to the increased blood loss, particularly the indication for and type of cesarean section. The present study was designed in an attempt to resolve this criticism. Blood loss was evaluated in uncomplicated patients undergoing elective repeat cesarean section under either general anesthesia using a halogenated agent (isoflurane) or regional anesthesia (spinal/epidural). All 117 singleton term, nonlaboring women underwent repeat low transverse cesarean section performed through a midline abdominal incision. Exclusion criteria included maternal medical complications, abnormal placentation, polyhydramnios, presence of uterine leiomyomas, and intraoperative complications. Seventy-five patients (64%) received regional and 42 (36%) received general anesthesia. A greater proportion of women undergoing general anesthesia experienced a postoperative decrease in hematocrit of 5 vol% or more compared with patients receiving regional anesthesia (10 of 42 versus 5 of 75, p = 0.018). Thus, we conclude that women undergoing uncomplicated elective repeat cesarean section under general anesthesia supplemented with a halogenated agent are at risk for increased blood loss compared with those women receiving regional anesthesia. However, the increased blood loss was not clinically significant in this study, since none of the patients required transfusion.

Anesthesia, Conduction↗

Fetal echocardiography: indications for referral, prenatal diagnoses, and outcomes.

Three hundred three fetuses were studied with fetal echocardiography and 21 were diagnosed with congenital heart disease (CHD). Indications for study, prenatal findings, and postnatal outcomes were examined in all fetuses studied. The most common indications for referral were previous family history and fetal dysrhythmia. Of 189 fetuses studied for these indications, six had CHD. Indications with the highest proportion of abnormal studies included an abnormal four-chamber view of the heart (50% abnormal), fetal hydrops (30.8% abnormal), and polyhydramnios (25% abnormal). Five of 21 with CHD had chromosomal defects and eight had extracardiac anomalies. Two of the 21 fetuses with CHD survived. Present referral indications led to the diagnosis of the most severe lesions, yet reassurance is provided to many couples with normal fetuses. Fetal echocardiography is important in the care of the fetus at risk for CHD.

Echocardiography↗

Anesthesia for perinatal surgery.

A 30 year gravida 3 para 2002 woman presented at 28 weeks' gestation with polyhydramnios. Ultrasound revealed a single fetus with a large anterior neck mass. With halothane general anesthesia providing uterine relaxation and maintaining placental and umbilical blood flow, an otolaryngologist attempted to secure the airway. We discuss the rationale for our anesthetic management and suggest its use in similar situations in which surgical intervention may be required in the immediate perinatal period.

Anesthesia, General↗

Prenatal ultrasound diagnosis of intestinal obstruction: a case report.

A case of intrauterine intestinal obstruction diagnosed at 31 weeks' gestation by sonographic examination is presented. Unlike other reported cases, the diagnosis was made early in the third trimester and before the development of polyhydramnios. The patient was managed conservatively until delivery at 33.5 weeks. At exploratory laparotomy, immediately after birth, ileal atresia was found and resected. The infant recovered uneventfully.

Adolescent↗

[Iniencephaly: prenatal and postnatal findings].

A 33-year-old para-3 was admitted in the 33rd week of gestation because of a suspected foetal anomaly. Ultrasound examination showed polyhydramnios, exaggerated cervico-thoracic lordosis and significant shortening of the spine because of a reduced number of vertebrae. The facial profile was flat, the foetal movements were rare and slow, and the extremities normal. A biopsy of the placenta revealed a normal female karyotype. Based on ultrasound examination, the diagnosis of iniencephaly was made. Because of the fatal prognosis of this malformation, labour was induced at 35 weeks of gestation. The patient delivered spontaneously. The infant died after 90 minutes. The postmortem examination confirmed the diagnosis of iniencephaly. Iniencephaly is a very rare malformation comprising a bone defect at the occiput, malformation of the cervical and thoracic vertebrae, spina bifida, and retroflexion of the head. The aetiology is not clear. 90% of the probands are female. The malformation is incompatible with survival after birth.

Abnormalities, Multiple↗

A case of mosaic tetrasomy 12p (Pallister-Killian Syndrome) diagnosed prenatally: comparison of chromosome analyses of various cells obtained from the patient.

A pregnant woman was referred to our hospital at the 29th week of gestation with the symptom of polyhydramnios; diaphragmatic herniation of the fetus was suspected. Fetal chromosome anlaysis was performed using fibroblasts obtained by aminocentesis, and mosaicism of 46,XX and isochromosome of 12p were diagnosed. Out of 50 karyotyped cells, 19 (38.0%) showed the tetrasomy of the isochromosome of 12p. The mother vaginally delivered a baby girl who died just after delivery. The analysis of cord blood lymphocytes revealed only 0.5% incidence of tetrasomy of 12p. The incidence of tetrasomy was 8.0% for the placental chorionic villi, 48.0% for the fibroblasts obtained from the umbilical cord, and 70.0% for the skin fibroblasts. Thus, the diagnosis of Pallister-Killian Syndrome (PKS) is confirmed by mosaicism of i(12p), that is, the affected patients exhibit tissue-specific mosaicism, with the abnormal karyotype expression in limited lymphocytes, but marked in fibroblasts.

Adult↗

Liver tumors in neonates and very young infants: diagnostic pitfalls and therapeutic problems.

We report on 26 infants under 3 months of age with various liver tumors, who were treated in our hospital since 1977 and/or registered in the German Cooperative Pediatric Liver Tumor Study HB-89. 17 of these had an infantile hemangioendothelioma (inf HE), 7 a hepatoblastoma (HB), one a mesenchymal hamartoma (mes H) and one a neuroblastoma stage IV-S (Nbl). Polyhydramnios occurred in 5 cases of inf HE, in one associated with hydrops fetalis. This led to a preterm delivery of 4 infants. 7 infants with a large inf HE suffered from high output congestive heart insufficiency, 2 additionally from a Kasabach-Merritt-syndrome. Serum alpha-fetoprotein was within normal range of age in all infants except in one HB patient. Neuron-specific enolase was clearly elevated in the Nbl patient and slightly above normal range in one inf HE and 3 HB patients. Other tumor markers and platelet counts were not indicative of diagnosis. Also imaging techniques as ultrasonography, CT and angiography were not always reliable in differentiating the tumors. In 2 cases the initial histological diagnosis of an HB had to be corrected for an inf HE. In 6 children the tumors were initially treated under a wrong assumption: 4 tumors as an HB instead of an inf HE, one as an inf HE instead of a Nbl IV-S and one as a focal nodular hyperplasia of the liver instead of an HB. 7 not resected inf HEs (Dehner type I) showed spontaneous regression after 6 months to 3 years. One inf HE type II relapsed twice after resection.(ABSTRACT TRUNCATED AT 250 WORDS)

Biomarkers, Tumor↗

Effect of esophageal ligation on amniotic fluid volume and urinary flow rate in fetal sheep.

OBJECTIVE: Although the fetus normally swallows large volumes of amniotic fluid each day, it is unclear whether amniotic fluid volume increases after fetal esophageal obstruction or whether fetal urine production changes. Our objective was to determine the effects of fetal esophageal ligation on amniotic fluid volume and urinary flow rate over time. STUDY DESIGN: Seven late-gestation fetal sheep underwent esophageal ligation, and 7 served as time control animals. The urachus was ligated to eliminate urine flow to the allantoic cavity. On days 1, 3, 5, 7, and 9 after surgery, we measured the composition of amniotic fluid, fetal urine, and fetal and maternal blood, as well as amniotic fluid volume and fetal urinary flow rate. A 3-factor analysis of variance was used for statistical analysis. RESULTS: Amniotic fluid volume did not change with time in the control group, averaging 876 +/- 142 mL (mean +/- SEM), and it decreased in the esophageal ligation group (P =.020), averaging 309 +/- 75 mL on day 9. Fetal urinary flow rate was lower (P =.0063) in the esophageal ligation group (431 +/- 27 mL/d) than in the control group (631 +/- 54 mL/d). There were no differences in fetal or maternal blood compositions between the two groups. Amniotic fluid sodium and chloride increased in the ligated animals. CONCLUSION: Polyhydramnios did not occur after esophageal ligation, even though the fetuses excreted approximately 4000 mL of urine over the 9-day study period. This suggests that intramembranous absorption is substantially increased. With only small changes in amniotic solute concentrations, intramembranous solute absorption must occur simultaneously with water, suggesting a near-zero reflection coefficient for solutes. We speculate that fetal urine, lung secretions, or both contain a factor that increases intramembranous permeability.

Amniotic Fluid↗