A Case of Prenatally Diagnosed Klippel-Trenaunay-Weber Syndrome / 대한주산의학회잡지

Klippel-Trenaunay-Weber syndrome is a rare sporadically occurring congenital soft tissue anomaly characterized by cutaneous hemangiomata, hemihypertrophy and occasionally arteriovenous malformations(AVMs). No definite genetic defect has been identified. The appearance is a soft tissue mass of an extremity, usually affecting the adjacent trunk, hydrops fetalis, ascites, abdominal hemangiomatous masses, and hepatomegaly. When diagnosed prenatally, the disorder may be severe. Thrombocytopenia due to platelet consumption within the hemangioma and high output cardiac failure may complicate the outcome. Termination of pregnancy can be offered in severe forms, otherwise no alteration of management in expected. The management of newborns is primarily nonoperative, but some may benefit from surgical intervention. We report a case of Klippel-Trenaunay-Weber syndrome diagnosed prenatally by ultrasonogram in the second trimester and subsequently was terminated, with a brief review of literature.

Two Cases of Term Pregnancy Associated with Thrombophilia / 대한산부인과학회잡지

These are natural inhibitors of coagulation, and deficiencies of any of these factors is referred to as thrombophilia. The identified main causes of thrombophilia are deficiencies of antithrombin III, protein C, or protein S, resistance to actived protein C associated with Factor V Leiden mutation, and inherited hyperhomocystinemia. Inherited and acquired thrombophilias may also contribute to pathophysiological processes involved in recurrent pregnancy loss, fetal death, intrauterine growth restriction, placental abruption, placental infarction, and pre-eclampsia. Various therapeutic protocols with low-molecular-weight heparin (LMWH) were used. because it is associated with a low incidence of osteoporosis and thrombocytopenia. We experienced the two cases of successful deliveries by Cesarean section following a successful pregnancy maintenance in thrombophilia. we administered LMWH to prevent thromboembolism. one patient was the primi-gravidarum, with inherited thrombophilia, who has the familial history of pulmonary embolism and deep vein thrombosis. the other was the multi-gravidarum, with acquired thrombophilia, who has the past medical history of pulmonary embolism.

A Case of Anencephaly Associated with Twin Pregnancy Conceived by In Vitro Fertilization and Embryo Transfer / 대한산부인과학회잡지

Twin pregnancies are increasing these days due to recent development of the technology in treating infertility and twin pregnancies tend to cause more congenital anomaly than singleton pregnancies do. Although anencephaly is not uncommon, occurring in about one in every 1,000 births, anencephaly developed in a twin pregnancy is very rare. We experienced a twin pregnancy which consisted of an anencephalic fetus and a normal one. This pregnancy was conceived by IVF and ET. This pregnancy was continued without having special problems and was delivered at 36 weeks of gestation by cesarean section due to SROM (spontaneous rupture of membrane). The anencephalic fetus weighed 1,430 gm and was already dead in the uterus. The other weighed 2,660 gm and showed no external anomaly. Its Apgar score was 8 in 1 minute and 9 in 5 minute. The pregnancy resulted in a cesarean section delivery of one dead anencephalic fetus and one normal healthy fetus. We report with a brief review of literature a case of a twin pregnancy, conceived by IVF and ET, in which anencephaly was associated with a normal fetus.

Genetic amniocentesis after multifetal pregnancy reduction / 대한산부인과학회잡지

OBJECTIVE: This study is directed to evaluate the pregnancy loss rate resulting from genetic amniocentesis after multifetal pregnancy reduction. METHODS: From March 1998 to April 1999, total 145 patients with multifetal pregnancy were included in this study. Pregnancy loss in a study population of 44 patients who underwent genetic amniocentesis after multifetal pregnancy reduction were compared with a control group of 99 patients who did not have genetic amniocentesis after multifetal pregnancy reduction. RESULTS: The pregnancy loss rate in patients who underwent genetic amniocentesis after multifetal pregnancy reduction was 2.2% (1/44) compared with 4% (4/99) in the controls (P>.05). In the study group, one woman lost her pregnancy at 19 weeks' gestation, 3 weeks after the genetic amniocentesis, and the predisposing factor was spontaneous rupture of membranes. CONCLUSION: Genetic amniocentesis following multifetal pregnancy reduction does not increase the risk of pregnancy loss.

A Case of Prenatally Diagnosed Apert syndrome / 대한산부인과학회잡지

Apert syndrome or acrocephalosyndactyly is a rare developmental deformity with a sporadic or autosomal dominant trait characterized by coronal craniosynostosis, midface hypoplasia, exorbitism, typical symmetrical syndactyly of both hands and feet with varying degrees of mental retardation. It results from a mutation of the fibroblast growth factor receptor type-2 (FGFT2) gene. In the absence of family history, prenatal diagnosis may be difficult based on ultrasonographic findings alone. The original description was presented by Apert in 1906 with nine cases. Since then more than 200 cases have been reported in the world. We report a case of Apert syndrome diagnosed prenatally by ultrasonogram in the third trimester and subsequently was terminated, with a brief review of prenatal sonographic findings in 11cases reported in literature.

The comparison of the defecation physiology between postpartum and postoperative women by defecogram and pudendal nerve terminal motor latency / 대한산부인과학회잡지

OBJECTIVE: To understand the difference of defecation physiology between postpartum and postoperative women. METHODS: Between July 1998 to April 1999, we performed defecogram and pudendal nerve motor latency to 31 women, who were 8 postoperative women, 9 post cesarean-section state women, and 14 normal vaginal delivery-state women. RESULTS: According to the defecogram results, only squeezing angles of the anorectal angle were significantly increased(96.0 vs 72.3, 74.9 degree) in normal vaginal delivery-state women compared to post cesarean-section state and postoperative women, but rest and evacuation angles were not. And to pudendal nerve latency, there were no statistically significant difference. CONCLUSION: We concluded that the pudendal plexus was damaged during labor, therefore its ability to control puborectalis muscle was damaged. So, the anorectal angles of squeezing of postpartum women were significantly increased, compared to those of post cesarean section women or postoperative women.

Screening of high risk pregnancy using maternal serum triple markers / 대한산부인과학회잡지

OBJECTIVE: Our purpose was to determine whether abnormal triple marker in the second trimester may be associated with adverse pregnancy outcomes. METHODS: Between November 1996 and April 1998, we evaluated 1,158 pregnant women undergoing second trimester triple marker screening tests who delivered at our hospital. The pregnancy outcomes of 48 women with false positive screens were compared with 1,158 screen negative controls. The pregnancy outcomes were obtained from hospital delivery records. RESULTS: Women with abnormal triple marker showed increased risks for low birth weight(p<0.01). But there was no significant differences between study and control groups with respect to preterm labor, pregnancy induced hypertension, oligohydroamnios, premature rupture of membrane, placenta previa, abruptio placenta, fetal death in utero. CONCLUSION: Abnormal triple marker in the second trimester was associated with low birth weight.