Clinical challenges in the management of a prenatally diagnosed cloacal malformation.

Cloacal dysgenesis sequence is a severe malformation of the primitive cloaca and is characterized by a phallus-like structure, smooth perineum and the absence of genitourinary and anal orifices. It is usually accompanied by oligohydramnios, kidney dysplasia, and pulmonary hypoplasia. We present a case of a 29-year-old woman who was referred at 26 weeks of gestation due to an enlarged fetal abdominal circumference. Investigations revealed the presence of fetal ascites, intrapelvic cysts, calcified meconium, severe oligohydramnios and a 46XX karyotype. Fetal abdominal parecentesis performed on several occasions failed to reduce intra-abdominal pressure. To our knowledge this case represents the first variation of cloacal dysgenesis sequence to contain three dysmorphic structures along with the common findings of this anomaly.

[Comparison of Doppler flow in selected maternal and fetal vessels in pregnancies with fetal intrauterine growth restriction]. / Porównanie przeplywów mierzonych metoda Dopplera w wybranych naczyniach matczynych i plodowych u ciezarnych z opóznieniem wzrostu wewnatrzmacicznego plodu.

OBJECTIVES: The aim of the study was to compare Doppler flows in materno-fetal circulation in pregnancies with IUGR and small for gestational age (SGA) neonatal weight or normal weight, as well as in uncomplicated pregnancies. MATERIAL AND METHODS: The study population comprised 216 pregnant women from Lodz and Kalisz between 2003-2006. In the group I we included 68 women with IUGR observed in prenatal ultrasound examination and SGA neonates, in the group II--29 women with IUGR observed in US but normal neonatal weight and in the group III--110 women with normal fetal and neonatal weight. RESULTS: Mean fetal weight in the group II was (2035 g +/- 400 g) and in the group I (1947 g +/- 414 g) and it was lower than in the group III (2765 g +/- 543 g) (p<0.0005). Notch in UTA was found more often in the group I (39.7%), than in the groups III (8.4%) and II (10.3%), (p< 0.0005). The mean PI in the right UTA in the group I (0.957 +/- 0.440) was higher than in the groups II (0.718 +/- 0.259) and III (0.730 +/- 0.215), (p<0.001). Abnormal Doppler flows in MCA in the groups I (39.7%) and II (34.5%) were observed more often than in the group III (6.4%), (p<0.0005). Similarly abnormal Doppler examination in DV in the groups I (45.5%) and II (46.4%) were observed more often than in the group III (17.8%), (p<0.0005). PI in UA in the groups I (1.061+/- 0.325) and II (0.965 +/- 0.184) was higher than in the group III (0.848 +/- 0.166), (p<0.0005).

Meigs' syndrome and virilizing ovarian fibrothecoma complicating pregnancy. A case report and review of the literature.

BACKGROUND: A half of virilizing ovarian tumors in pregnancy are malignant. The risk of cancer increases with ascites and hydrothorax. Our case shows that such tumor can be benign with good outcome. CASE: Primigravida in 30 weeks with advanced hirsutism (from 15 weeks), vomiting, epigastric pain, dyspnoea, ascites, hydrothorax, ovarian mass and high level of serum testosterone was operated. After the tumorectomy (fibrothecoma) we performed cesarean section (placental abruption suspected). The female fetus had no signs of virilization. In mother all symptoms and effusions resolved. CONCLUSION: Meigs' syndrome in pregnancy leads to acute clinical symptoms and needs the surgical intervention with the risk of placental abruption. Virilization of mother was the first symptom of ovarian tumor but the female fetus was protected of hyperandrogenism.

Maternal obesity as a perinatal risk factor.

OBJECTIVE: The aim of the study was to estimate the effect of maternal obesity on pregnancy course, delivery and newborn well-being. MATERIAL AND METHODS: Data about women who delivered in PMMH was analyzed and obese pregnant women (pre-pregnancy BMI > or =30) were included in the study group; the rest of the mothers constituted the control group. The pregnancy course, labor and delivery, and newborn well-being were taken into consideration. RESULTS: 4648 women were found in our delivery database, among them 208 (4.48%) were classified as obese. In this group, pregnancy-associated hypertension was common, either non-proteinuric one (8.65% vs 2.39%, p=0.001) or preeclampsia (4.81% vs 1.58 %, p<0.05). There were also more cases of gestational diabetes mellitus requiring insulin therapy (9.62% vs 1.48%, p<0.001) and polyhydramniosis (4.81% vs 2.11%, p<0.05) than in case of controls. The mean gestational age at delivery and newborn general health (estimated by Apgar score, mean umbilical cord pH and the incidence of cases with pH < or =7.10) were similar in both groups. The mean birthweight (3266 g vs 3100 g, p<0.05) and the incidence of macrosomia (20.19% vs 5.69%, p<0.001) were significantly higher in the study group. The delivery mode was comparable in both groups, with the marked tendency towards higher incidence of elective cesarean sections in case of obese mothers (27.88% vs 19.90%, p=0.01). CONCLUSION: Maternal obesity is a significant perinatal risk factor; with pregnancy-associated hypertension and gestational diabetes requiring insulin therapy in obese mothers and macrosomia in newborns as most common complications.

[Massive fetomaternal hemorrhage--case report]. / Masywne przetoczenie plodowo-matczyne--opis przypadku.

Massive fetomaternal hemorrhage is defined as a loss of more than 30-50ml of fetal blood, transferred into maternal circulation. It can lead to severe anemization of the fetus and its consequences such as fetal hydrops or stillbirth. We present a case in which the diagnosis was based on the decresased fetal movement and pathological cardiotocography record. The Kleihauer-Betke test revealed a high percentage of fetal cells and allowed to state a proper diagnosis. An immediate cesarean section was performed and the lab tests confirmed severe anemization of the newborn.

[Occurrence of plasmic and platelet prothrombotic polymorphisms in women in childbirth]. / Wystepowanie osoczowych i plytkowych polimorfizmów prozakrzepowych u poloznic.

DESIGN: Plasmic pro-thrombotic factors as well as pro-thrombotic platelet glycoprotein mutations have been shown to play an important role in the mechanism of the thrombo-embolic disease. However, there is no published study evaluating the role of above mentioned genetic factors in the thrombo-embolic episodes in women in childbirth. OBJECTIVE: The aim of the study was to evaluate the role of selected genetic factors in appearance of thrombo-embolic complications in the women in childbirth, and to determine if there is the coexistence of selected platelet glycoprotein polymorphisms and factor V Leiden mutations. MATERIAL AND METHODS: 71 cases of women in child birth with thrombo-embolic disease were analyzed. Selected demographic characteristics, and genetic pro-thrombotic factors like factor V Leiden mutation, and pro-thrombotic platelet glycoprotein GP Ia, and GP IIIa polymorphisms were examined. RESULTS: Amongst pro-thrombotic platelet glycoprotein polymorphisms moderately pro-thrombotic heterozygous A1/A2, and heterozygous C/T were most prevalent. The least common were strongly pro-thrombotic homozygous A2/A2, and T/T. Analysis of the factor V Leiden mutation revealed statistically significant difference in the presence of allele A, which determines the prothrombotic tendencies in its carrier. CONCLUSIONS: Our study shows that factor V Leiden mutation, and investigated platelet GP Ia, and GP IIIa polymorphisms frequently coexist. Moreover, presence of factor V Leiden mutation is a risk factor for thrombo-embolic disease in the women in childbirth.

[Pregnancy and delivery after bariatric surgery]. / Przebieg ciazy i porodu u pacjentek po chirurgii bariatrycznej.

The obesity in Europe is established for 15-20% of population. About 20% of women in reproductive age have accrued body weight. Obesity in this group leads to increased obstetrical risk. Preconceptional normalization of body mass is crucial. Bariatric surgery is one of the most effective methods of obesity treatment with Roux en Y gastric by pass (RYGB) as the one of its kind. In this report we present tree cases of pregnancy and delivery after RYGB.

[Unconjugated serum estriol in the prediction of preterm delivery]. / Zastosowanie oznaczen estriolu w surowicy ciezarnych w diagnostyce porodów przedwczesnych.

OBJECTIVE: The objective was to analyze the serum estriol levels among patients with sings and symptoms of preterm labor and/or preterm rupture of membranes. STUDY DESIGN: A prospective study included pregnant women with sings and symptoms of preterm labor. The main end point of the study was the delivery <28 days from testing. RESULTS: 196 patients were included. 116 patients were included in group I (idiopathic preterm labor), 37 patients in group II (PROM) and the control group (group III) consisted of 43 patients. The incidence of preterm delivery was 31% in group I; in the PROM group all the patients delivered preterm. The mean serum estriol levels in all groups were compared regarding delivery <28 days from testing. In group I patients that delivered <28 days had statistically higher serum level of E3 (14.5 ng/ml vs. 11.1 ng/ml, p = 0.03); in group II the mean E3 serum level did not differ significantly (12.5 ng/ml, p = 0.168). The detailed analysis revealed that significant difference was observed in patients tested after completed 30 wk of gestation (15.4 ng/ml vs 12,8 ng/ml, p = 0.043), but not in patients <30 wk of gestation (9,5 ng/ml vs. 10.0 ng/ml, p = 0.842). CONCLUSIONS: The serum level of E3 seems to have prognostic value in the diagnosis of preterm delivery among symptomatic patients after 30 week of gestation.

[The pregnancy and delivery course in patients with reproductive tract anomalies]. / Przebieg ciazy i porodu u pacjentek z wadami rozwojowymi narzadu rodnego.

OBJECTIVES: The goal of our study was to analyse the course of pregnancy and delivery in women with reproductive tract anomalies. MATERIAL AND METHODS: We retrospectively analyzed the pregnancy and delivery course of 16 patients with previously diagnosed reproductive tract anomalies. The control group constisted of 200 healthy pregnant women. All patients were hospitalized in our department between January 1st 2001 and 31st May 2005. RESULTS: In the study group preterm delivery and abnornal fetal lie were more common. The incidence of cesarean sections (81%) was also statistically higher in the study group. CONCLUSIONS: The course of pregnancy and delivery in women with previous diagnosis of specific reproductive tract anomalies is more likely complicated by several obstetrical pathologies. The pregnant women with this a diagnosis should be considered as a group of high risk pregnancy and perinatal intensive care procedures should be carried out.

[Cervical ectopic pregnancy--a case report]. / Ciaza szyjkowa--opis przypadku.

Authors present a case of a viable cervical pregnancy in a 31 year old woman. Conservative management with intramuscular methotrexate and local injection of KCl was applied. We describe the ultrasound and biochemical findings in this case and discuss those reported in the literature along with the available treatment options.

Polymorphism of the glycoprotein Ia and IIIa in the group of women in childbirth does not correlate with an increased risk of developing thrombosis.

OBJECTIVE: The aim of the study was to evaluate the prevalence of the GP Ia and GP IIIa polymorphisms in the group of women in labor, and to assess the risk of thrombosis associated with their occurrence. DESIGN AND SETTINGS: 245 women in labor hospitalized between 1.01.2001 and 31.12.2003 r. were enrolled in the study. Patients were qualified for the study if detailed physical exam and past medical history excluded existence of known risk factors predisposing to thrombosis. Study group was composed of 72 women in childbirth, which at some point during current pregnancy or in early labor were diagnosed with thrombosis, and control group included 173 women in labor randomly picked from the group of patients with uncomplicated pregnancies. Polymorphic regions of platelets glycoprotein were detected using genotyping methods based on polymerase chain reactions (PCR). RESULTS: 1.72% of patients were found to have thrombosis. The thrombosis was located in the venous system in 97.2% of cases. Arteries were affected in two patients (2.7%). Prevalence of individual platelets glycoprotein mutations did not differ between controls and study group. In both groups platelets glycoprotein polymorphisms moderately pro-thrombotic A1/A2 and C/T dominated, and the least numerous were strongly pro-thrombotic A2/A2 and T/T. CONCLUSIONS: Our results did not show the causative relationship between the existence of platelets GP IIIa/GP Ia mutations and venous system thrombosis in the women in labor. Probably presence of pro-thrombotic mutations of platelets glycoprotein in this group of patients does not represent the increased risk of thrombosis.

Pregnancy complicated by Myasthenia gravis - twelve years experience.

OBJECTIVE: The aim of the study was to evaluate the effect of Myasthenia gravis (MG) on the course of pregnancy, labor and delivery, and the condition of neonate. DESIGN AND SETTINGS: Retrospective chart review of pregnant patients with MG hospitalized in tertiary care center over 12 year period was performed. Course of MG before and during pregnancy, mode of delivery, and condition of the newborn were analyzed. RESULTS: There was 42 913 deliveries in the Polish Mother's Research Institute in Lodz during analyzed period. Thirteen patients with MG were identified, giving the incidence of 3/10 000 live births. Mean age of the patients was 28 years, range 18 - 36 years. Average time since diagnosis was 9 years. Eleven women with MG delivered at term (one twin pregnancy), and two babies were born prematurely. In seven patients pregnancy was ended via normal spontaneous vaginal delivery (NSVD), one was forceps extraction, four patients had elective Cesarean Section (CS), and two had emergency CS. Indications for CS were purely obstetrical. Spontaneous vaginal deliveries were speedy. None of the patients had exacerbation of MG during current pregnancy. Mean birth weight of the newborns was 3014,3 g, range 1730 g to 3700 g. Mean Apgar score was 8,7 at 1 minute and 8,9 at 5 minutes. Only one neonate (second twin) developed Transient Neonatal Myasthenia Gravis (TNMG). CONCLUSIONS: Myasthenia gravis did not have significant effect on the course of pregnancy or mode of delivery. In the twin pregnancies it is possible that only one twin will present with the symptoms of TNMG.

Orthotopic liver transplantation for acute liver failure resulting from "acute fatty liver of pregnancy".

Steatosis of the liver, demonstrating itself as the acute liver failure during the third trimester of pregnancy (Acute Fatty Liver of Pregnancy--AFLP) is a rarely observed liver pathology. Herewith we describe the case of a 19-years old pregnant patient with this rare disease. The authors present the clinical course, dynamics, diagnostics, and outcome of treatment in this unique case. In this patient the ultimate treatment chosen was the liver transplantation. The opinions, concerning liver transplantation in AFLP (expressed in the world literature) are somewhat controversial. This paper presents the first case of AFLP treatment with orthotopic liver transplantation (OLTx) in Poland.

[Central nervous system tumors in pregnancy]. / Guzy osrodkowego ukladu nerwowego w ciazy.

Central nervous system tumour in pregnancy constitutes a serious complication. Considering frequent difficulties in diagnostics and therapy, the aim of the study was to present our experience in management with pregnant women with brain and spinal cord tumours. Between 1988-2000, in The Research Institute Polish Mother's Memorial Hospital in Lodzi, 4 pregnant women had been diagnosed with brain and spinal cord tumours. The incidence of tumours complicating pregnancy was 1/11460. Two patients diagnosed at 29 weeks' gestation, underwent craniotomy and tumour resection during pregnancy. Two other women with central nervous system tumours diagnosed at 39 weeks' gestation, were operated in the postpartum period. The analysis of the postoperative period, gestation and/or postpartum period in all women and well-being of their new-borns confirm undertaken medical decisions.

[The number and histopathologic type of ovarian tumors operated during cesarean section at the Polish Mother's Health Institute between 1990-2000]. / Liczba i rodzaj guzów jajnika operowanych w czasie ciecia cesarskiego w Instytucie Centrum Zdrowia Matki Polki w Lodzi w latach 1990-2000.

OBJECTIVES: The differences exist in concerning the estimation of number and histopathological type of ovarian tumors in pregnancy. MATERIAL AND METHODS: From all 41,661 labours which took place in our Institute between 1990-2000 retrospective analysis of medical documentation of 11,050 caesarean sections (CS) as well as histopathological protocols were performed. RESULTS: The prevalence of ovarian tumors removed during CS performed between 28-41 weeks of gestation was 0.19%. The prevalence of malignancy was low (0.005% of all labours). Unilateral cystectomy was found to be the most frequent kind of surgery and adult teratoma, serous cyst and paraovarian cyst as most common histopathological tumor types. CONCLUSION: We conclude that ovarian tumors, especially of malignant type are rarely the complication of pregnancy. The most are benign, small, unilocular and smooth-wall cystic tumors.