Stephanie Mann

Sacrococcygeal Teratomas: Prenatal Surveillance, Growth and Pregnancy Outcome

Objective: Prenatal surveillance and growth characteristics are evaluated in a cohort of fetuses with sacrococcygeal teratomas (SCT) as part of risk assessment. Methods: Retrospective review of 23 fetuses with SCT: prenatal diagnosis, surveillance, delivery, and early postnatal outcome are reported. Results: Cardiac output failure physiology requires serial evaluation. The size of the SCT determines obstetrical risks and mode of delivery. An SCT growth rate approaching >150 cm3 per week may be associated with increased perinatal mortality risks. Maternal morbidity is related mainly to polyhydramnios and preterm labor. Conclusions: Perinatal mortality is approximately 43%. Maternal-fetal surgery for fetal physiologic deterioration is not frequent, but serial surveillance is required to minimize fetal morbidity/mortality and maternal morbidity. Rapid SCT growth rates may be associated with increased risk of perinatal mortality.

Prenatal and postnatal management of omphalocele

Omphalocele is one of the most common abdominal wall defects seen in the prenatal period. Once this diagnosis is confirmed, it is important to check the fetal karyotype and thoroughly assess the fetus for other malformations. Prenatal management involves serial assessment of fetal growth and prenatal testing to ensure fetal well‐being. Closure of the abdominal wall and replacement of organs into the abdominal cavity can be done directly if the omphalocele is small or in a staged manner if the omphalocele is large. Successful outcomes for these neonates can be optimized with a multidisciplinary team approach to prenatal and postnatal management. Copyright

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).

Fetal thoracic and bladder shunts.

Congenital obstructive lesions involving the bladder and the lung can lead to serious complications for the newborn. The in-utero placement of a diverting shunt in the fetal bladder or thoracic cavity can decrease the morbidity and mortality associated with these obstructive conditions. This review focuses on the indications for prenatal evaluation, technique, and outcomes for those fetuses with a lower urinary tract obstruction, congenital pleural effusion or macrocystic congenital cystic adenomatoid malformation after placement of a vesicoamniotic or thoracoamniotic shunt.

OEIS sequence - A rare congenital anomaly with prenatal evaluation and postnatal outcome in six cases

To describe the outcome of children prenatally diagnosed with omphalocele, exstrophy of the cloaca, imperforate anus and spinal defects (OEIS).

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.

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.

OP10.02: The CHOP cardiovascular score: a method for quantification of cardiovascular abnormality in the twin–twin transfusion syndrome

Objectives: Umbilical vein volume flow (UVVF), calculated as velocity (cm/s) times transversal section area at the straight portion of the intra-abdominal umbilical vein (cm2), is an interesting parameter to study in twin–twin transfusion syndrome (TTTS). Recipients were found to have 2 to 3 times higher UVVF than donors, but this parameter was not studied in normal monochorionic pregnancies. Methods: Comparison of recipient to donor (rec/don) UVVF ratio and bigger-to-smaller UVVF ratio in non-TTTS monochorionic twin pregnancies (control group). The method of dividing the same parameter from each pair of twins aimed to emulate other factors such as gestational age and maternal condition, which change between pregnancies. The control group consisted of monochorionic twin pregnancies with normal umbilical artery Doppler and normal amniotic fluid (2 to 8 cm of MVP), between 18 and 26 weeks. Results: Eight-four TTTS and 24 normal monochorionic twin pregnancies were compared. The median weight discordance was 1.3 (rec/don) and 1.2 respectively (big/small, Wilcoxon P < 0.7). The UVVF ratio between recipients and donors (UVVF R/D) was greater than that of the bigger to smaller UVVF ratio from normal monochorionic twins (medians 2.2 and 1.3, Wilcoxon P < 0.001). Conclusions: This study shows that UVVF R/D imbalance occurs in cases with the polyhydramnios and oligohydramnios sequence found in TTTS, and that normal monchorionic pregnancies lack this imbalance. The difference is not due to fetal weight discordance as it was shown that normal monochorionic twins had similar discordance. The parameter has still not proven to be useful in screening for early diagnosis by this methodology, but it describes that UVVF imbalance between twins does not exist in normal monochorionic pregnancies. OP10.02 The CHOP cardiovascular score: a method for quantification of cardiovascular abnormality in the twin–twin transfusion syndrome