R. D. Wilson

Thoracoamniotic Shunts: Fetal Treatment of Pleural Effusions and Congenital Cystic Adenomatoid Malformations

Objective: To determine whether fetuses that underwent thoracoamniotic shunt placement for treatment of pleural effusion (PE) or macrocystic congenital cystic adenomatoid malformation (CCAM) have an improved outcome as compared with an untreated population. Methods: A retrospective review from a single tertiary center was performed using thoracoamniotic shunt placement to treat PE or macrocystic CCAM between 1998 and 2001. Thoracoamniotic shunts were used on 26 occasions in 19 pregnancies. Results: The average gestational age at the diagnosis of PE and CCAM was 22 + 4 and 20 + 0 weeks, respectively. Shunts were offered in pregnancies complicated by hydrops or at significant risk for pulmonary hypoplasia. Shunts were placed at 26 + 2 weeks (average) and 23 + 1 weeks (average) in the PE and CCAM groups, respectively. In CCAM patients, the mean pre- and postshunting mass volumes were 50.5 and 25.7 cm3, representing a 51% reduction in mass volume following shunt placement. In the PE group, the average delivery age was 33 + 5 weeks, with an average shunt placement to delivery time of 7 + 3 weeks. In the CCAM group, the average delivery was 33 + 3 weeks, with an average shunt placement to delivery time of 10 + 2 weeks. The postnatal survival rates were 67% (6/9) and 70% (7/10) in the PE and CCAM groups, respectively. Conclusions: (1) Thoracoamniotic shunts should be considered as a treatment option for selected PE or macrocystic CCAM fetuses with hydrops or a significant risk for pulmonary hypoplasia; (2) the neonatal survival with shunting was similar for PE and CCAM groups and was improved as compared with literature reports, and (3) fetuses with CCAM presented earlier with hydrops than those with PE. Successful shunting resulted in a prolongation of pregnancy into the 3rd trimester in both groups.

Outcomes in a cohort of patients with Stage I twin-to-twin transfusion syndrome.

To determine pregnancy outcomes of patients who present with Stage I twin‐to‐twin transfusion syndrome (TTTS).

Prenatal Diagnosis and Management of Mainstem Bronchial Atresia

The prenatal diagnosis, natural history and management of mainstem bronchial atresia have not been described previously. We report two cases of prenatally diagnosed proximal bronchial atresia. The first patient presented at 18 weeks with sonographic and MRI findings consistent with bronchial atresia with fetal hydrops. The mother developed the mirror syndrome and labor was induced. A non-viable fetus was delivered at 25 weeks. The second patient presented at 16 weeks gestation with evidence of an intrathoracic mass that was subsequently prenatally diagnosed as a right mainstem bronchial atresia. The right lung increased rapidly in size and was associated with the onset of fetal hydrops. At 24 weeks, fetal pneumonectomy was performed but the fetus expired intraoperatively due to cardiovascular collapse. Post-mortem findings in both cases confirmed the presence of an atretic mainstem bronchus with massive enlargement of the lung. Bronchial atresia involving the mainstem bronchus is associated with a poor prognosis.

Amniocentesis for twin pregnancies : Is alpha-fetoprotein useful in confirming that the two sacs were sampled?

Objective: To assess if amniotic fluid alpha-fetoprotein (AFAFP) could be useful to determine if both sacs are sampled during an amniocentesis for twin pregnancies. Method: We reviewed all amniocenteses performed on twin pregnancies over a 5-year period. Inclusion criteria were restricted to pregnancies where both karyotypes and AFAFP were available on each fetus. Pregnancies complicated by fetal anomalies were excluded. The following information was obtained: maternal age, gestational age at the procedure, karyotypes, AFAFP values, pregnancy and neonatal outcome. Placental pathology reports were used to confirm chorionicity. Analysis was performed to evaluate the impact of the fetal gender and chorionicity on the AFAFP values. Results: 260 pregnancies were reviewed. Mean maternal age was 36.9 years (33.6, 40.1). Gestational age at the time of the procedure was 16.2 weeks (14.5, 17.9). Complications included 1.8% of misdiagnosis (discrepancy between karyotype and gender). The difference of AFAFP values between the two fetuses was statistically larger in dichorionic pregnancies than in monochorionic gestations. Fetal gender had no influence on the AFAFP. Conclusion: Amniocentesis in twin pregnancies is associated with a 1.8% risk of misdiagnosis. AFAFP can help to assess the chorionicity of a twin pregnancy. When the difference between the two values is <0.2 MoM and the chorionicity was thought to be dichorionic and the two karyotypes are similar, then failure to sample both sacs should be suspected.

Survival in Primary Congenital Pulmonary Lymphangiectasia with Hydrops Fetalis

The recent advances in neonatal and pediatric intensive care have changed the outcome of patients with congenital pulmonary lymphangiectasia of different types, including those cases with early neonatal symptoms. However, the patients who present the most severe form of the disease, manifested by in utero hydrops (including those treated by in utero thoracoamniotic shunting to relieve the mediastinal compression), have had an unvaryingly fatal ending in all published reports, with most cases dying before birth, and the few born alive dying during the first days of life. We present a patient with primary congenital pulmonary lymphangiectasia complicated by hydrops fetalis, who was treated in utero, survived the neonatal period after intensive medical and surgical support, and was discharged home at the age of 2 months. She is currently 6 months old, and has minimal signs or symptoms of chronic lung disease. The different aspects of the management of congenital pulmonary lymphangiectasia are discussed in this report, together with a review of the literature.

OP10.04: Outcomes in a cohort of patients with stage 1 twin–twin transfusion syndrome

SD); 1.10 (0.46) vs. 0.86 (0.34) (P = 0.019), and 0.58 (0.15) vs. 0.47 (0.095) (P = 0.001), respectively. When data were analyzed according to the stages of severity, recipients and donors from TTTS Grade 2 showed a significantly higher DVPI than TTTS Grade 1 (1.30 (0.49) vs. 0.86 (0.28) (P = 0.003), and 0.99 (0.38) vs. 0.71 (0.22) (P = 0.011), respectively). Despite the fact that differences did not reach statistical significance, Mod-MPI showed the same trend only in recipients (0.61 (0.16) in Grade 2 vs. 0.54 (0.13) in Grade 1, P = 0.1). Conclusions: Fetal cardiac function, assessed by increased DVPI and Mod-MPI, is impaired in TTTS, especially in recipient fetuses. These alterations seem to be related to the degree of severity according to Quintero stage.

OP10.10: The CHOP cardiovascular score for twin–twin transfusion: the effect of treatment with selective laser ablation

Objectives: To study the impact of treatment with endoscopic laser ablation for twin–twin transfusion syndrome (TTTS) on the CHOP cardiovascular score. Methods: Retrospective review of patients undergoing laser ablation for TTTS with both preoperative and postoperative fetal echocardiograms. Results: Between June 2005 and February 2007, 41 patients underwent laser ablation therapy for TTTS. Quintero staging was Stage 2 for 19, Stage 3 for 19 and Stage 4 for three. The preoperative CHOP scoring was Grade I (0–5) = 22; Grade II (6–10) = 7; Grade III (11–15) = 8; Grade IV (16–20) = 4. The mean gestational age at treatment was 20.6 ± 2.07 weeks. Fifteen patients underwent both preoperative and postoperative fetal echocardiography with an average of 3 days between the preoperative evaluation and treatment and an average of 10 days between treatment and post-op evaluation. The mean preoperative CHOP score was 9.9 ± 1.4 and the mean postoperative CHOP score was 4.8 ± 0.9 (P = 0.0002). Conclusions: The CHOP cardiovascular score measures physiologic perturbations that underlie the clinical findings in TTTS. Following treatment with laser ablation, clinical resolution is associated with a significant decrease in the CHOP cardiovascular score. This provides further validation of the CHOP cardiovascular score as a tool for the evaluation of pregnancies complicated by TTTS.

OP16.07: Antenatal skeletal evaluation—the use of low dose CT in the diagnosis of skeletal dysplasia

correlated with the angle of progressive head deflection (so called Angle of Progression) on sagittal ultrasound planes. Results: (1) There was a strong correlation between the angle of progression on ultrasound and fetal head station on MRI (r2 = 0.8, p < 0.001). (2) From our study engagement would be associated with an angle of progression of 100◦. Conclusion: Our in vivo study confirms what has been previously derived from CT-scans of non pregnant women using geometrical model.

OP22.03: Fetoscopic surgery in triplet pregnancies with TRAP sequence

defect simultaneously with removal of the operative cannula to create a gelatin seal of membrane defect. Iatrogenic premature prelabor rupture membranes (PPROM) was defined as gross rupture with vaginal pooling, a positive nitrazine test and confirmation by ultrasound of reduced amniotic fluid volume in the recipient twin’s sac. A complicated procedure was defined as development of intraoperative hemorrhage, septostomy, multiple puncture or preoperative reduce cervical length (< 25 mm). Results: The median gestational age of intervention was 20.1 wks (16–26.4 wks). In 39 (95%) the place of the gelatin plug was successful. PPROM < 32 weeks occurred in 6 (15%). Complicated procedures occurred in 3/6 (50%) PPROM and 9/33 (27%) nonPPROM cases. This difference was not statistically significant, P = 0.19. (Fisher Exact test). There were no adverse maternal, fetal or neonatal outcomes attributable to the placement of the gelatin seal. Conclusions: The rate PPROM < 32 wks reported here, 15%, was similar to that reported without gelatin seal, 17%. (Robyr et al2006). The PPROM cases in our study had a tendency to be associated with complicated procedures. Before gelatin seal is considered a routine clinical part of fetal intervention, a randomized controlled trial is necessary to demonstrate safety and efficacy.

P33.18: Co‐occurrence of multiple placental surface cysts and massive perivillous fibrin deposition in a fetus with left CDH: A case report

VATER association was firsts described as a combination of three or more of these defects: vertebral defects (V), anal atresia (A), esophageal atresia and/or tracheo-esophageal fistula (TE), and radial and renal anomalies (R standing for both of them). No specific cause has been identified and causal heterogeneity seems to be most acceptable conclusion. By the other hand, placental chorioangioma is a benign vascular tumor found in about 1% of pregnancies at routine histological examination of the placenta. Tumors that are large enough to produce clinical symptoms and that can be visualized on sonography are uncommon. Potential fetal complications include polyhydramnios, fetal hydrops, fetal growth restriction and fetal demise. No association has been described between chorioangioma and VATER association. Patient, 20 years old, followed in Fetal Medicine Service of UNIFESP/EPM, due to fetal malformation. The following findings were observed in ultrasound, using Sonoace 80 000 LIVE (Medison, Seoul, Korea): large intestine dilatation, horseshoe kidney, genital anomaly and ventricular septal defect. The fetal Magnetic Resonance confirmed these findings. No placental neoplasms were diagnosed on sonography or fetal Magnetic Resonance. During gestation also occurred oligoamnio and fetal growing restriction. Patient went to vaginal delivery by 37 weeks of gestation, newborn 2.260 g of weight, clinically with atresia anal and genital anomalies (micropenis and hypospadia). Colostomy was realized with 24 hours of life. After exams, horseshoe kidney, lombar hemivertebra L3, ventricular septal defect, pulmonary stenosis, patent foramen ovale were described. Kariotype 46 XY. At 39th day of life, infant developed intestinal volvulus and infection. At 59th day of life, infant died due to septic shock. Necropsy confirmed all alterations described. Histologic examination of placenta has found the presence of chorioangioma.