Placental Mesenchymal Dysplasia Associated with Transient Neonatal Diabetes Mellitus and Paternal UPD6

18th World Congress on Ultrasound in Obstetrics and Gynecology

Poster abstracts

All fetuses underwent serial monitoring sessions including especially computerized cardiotocographic (cCTG) short term variability (STV) analysis, Doppler assessment of middle cerebral artery (MCA) and ductus venosus (DV) at least twice weekly. Timing of delivery was independently decided by the clinical staff based on a short term STV or cCTG anomalies. All growth-restricted fetuses were free from structural and chromosomal abnormalities. Individual data were normalized by converting the results in Z-scores. Results: When the mean values of the last session were compared with those obtained from previous recordings, we found that MCA RI shows significant changes only more than 2 weeks before cCTG anomalies. Also, for DV PVI the values in the last recording were not different from values obtained in the last 5 days, but significantly increased compared with earlier recordings. It appears that the STV has a constant and significant decrease with gestational age during last three weeks, being under 2 SD only for the last session. Conclusions: IUGR fetuses show a specific temporal sequence in antenatal tests. Doppler velocimetry alterations are the first to appear abnormal at the level of UA and then of MCA, followed by DV anomalies. The last test alterations to appear are represented by STV on cCTG analysis. For the clinician, the combination of venous Doppler and cCTG, on a daily monitoring in last week can provide an optimal fetal assessment.

IUGR and fetal or neonatal death are common in PMD but it also may be associated with normal fetus. Neonatal diabetes mellitus is a rare disorder with reporting incidence of 1/400,000–500,000 live births. Transient neonatal diabetes mellitus (TNDM) is characterized by neonatal hyperglycemia that resolves within the first few months and may relapse in adolescence. In some of these cases defects in the paternal chromosome 6 are involved. To the best of our knowledge, placentomegaly and PMD were not reported in association with TNDM. Herein we report a case of TNDM with PMD and pat(UPD6), and discuss the possible connection between these two entities.

P36.06 Congenital heart disease confounding the diagnosis of severe intrauterine growth restriction: A case report A. V. Carmo, C. M. R. Campos, M. A. M. Carmo Obstetrics and Gynecology, Federal University of Mato Grosso, Cuiaba, Brazil A 29-year-old was referred to our unit at 29 weeks’ gestation with the fetus presenting severe intrauterine growth restriction. The ultrasound examination demonstrated estimated birth weight of 641 g. The head to abdominal circumference ratio was elevated. The amniotic fluid index was normal (13.4 cm). There were bilateral uterine artery notches and the mean pulsatility index was in the upper limit of normal. The umbilical artery Doppler was normal and the resistance in the middle cerebral artery was around the lower limit of normal. The ductus venosus Doppler demonstrated elevated resistance with positive flow during atrial contraction. During the ultrasound assessment was detected a large aortic root, a small pulmonary artery, a ventricular septal defect in the outlet portion of the septum and an overriding aorta. The computerized cardiotocography was normal with a short term variation of 8.2 ms. The male neonate was weighting 620 g and was delivered by emergency cesarean section. The Apgar scores were 2 and 7 at 1 and 5 minutes, respectively. The tetralogy of Fallot and the assimetrical intrauterine growth restriction were confirmed by postnatal echocardiography and clinical examination. After delivery signs of cardiopulmonary decompensation were observed and prostaglandin and dobutamine were administrated, however the neonate died after fifteen days of life. The surgical correction was not possible due to the small infant weight. The karyotype is being processed.

P36.07 Placental mesenchymal dysplasia associated with transient neonatal diabetes mellitus and paternal UPD6 R. Aviram1 , D. Kidron1 , D. Abeliovich2 , R. Tepper1 , O. Markovitch1 , R. Aviram1 Meir Medical Center, Kfar Sava, Israel, 2 Hadassah Medical Center, Jerusalem, Israel

1

Placental mesenchymal dysplasia (PMD) is a distinct placental phenotype that has often been misdiagnosed as partial hydatiform mole. PMD placentas are typically larger than average (placentomegaly) and show cystic areas with absence of trophoblast hyperplasia.

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P37: MULTIPLE PREGNANCY P37.01 Change in hCG in twin gestations during sequential independent screening D. A. Kahn1 , A. Platt2 , N. S. Silverman1 , L. D. Platt1 1

Obstetrics and Gynecology, UCLA Medical Center, Los Angeles, United States, 2 Center for Fetal Medicine and Women’s Ultrasound, Los Angeles, United States

Objective: To determine the effect of twin gestation and assisted reproduction on the increase in MoMs of free b hCG to total hCG observed between the 1st and 2nd trimester during biochemical screening for aneuploidy. Study Design: This is a retrospective cohort study involving 130 twin pregnancies between December 2002 and May 2007 in a single center. Results: Of these twins 74 were conceived with IVF, 41 from ICSI and 25 from donor ovum. There were 77 pregnancies that had both 1st and 2nd trimester screening. A mathematical model was applied to create a distribution of the change in MoMs. A breakpoint was identified at an increase in 1 MoMs (19/77).

Increase in hCG (MoM) < 1 (n = 58) > 1 (n = 19)

Age

Age + NT

Age + NT + Biochemistry

Absent Nasal Bone

Pregnancy Complications

39.6% 21.0%

13.8% 21.0%

6.9% 21.0%

0.9% 5.5%

12.1% 26.3%

Despite the increase incidence of a positive screen, all of the invasive testing (7/58) occurred in the group with increase less than 1. The increase in hCG was unrelated to method of conception. Conclusions: Data is lacking for non-invasive risk assessment in twin gestations. In our cohort, there was a subgroup with an exaggerated increase in hCG that was more likely to be screen positive. This sub group is at higher risk to have a pregnancy complication. However, this increase was unrelated to the use of ART.

P37.03 Clinical, sonographic and psychosomatic aspects of delayed-interval delivery in twin pregnancy J. P. Siedentopf, N. S. Salomon, J. W. Dudenhausen Department of Perinatal Medicine, Charite Universitatsmedizin Berlin, Berlin, Germany Early second trimester rupture of the membranes in twin gestations often results in the loss of both fetusses. Some case reports and small series of delayed-interval twin births with survival of one fetus have been published. The general public gains knowledge of these publications via the internet. In cases of premature rupture of membranes patients suggest ‘‘sacrificing’’ one twin in order to

Ultrasound in Obstetrics & Gynecology 2008; 32: 398–466