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Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht.

In 1909 the neurologist Dr. Hans Steinert was the first to describe the most common of all neural-muscular diseases: Mytotonic Dystrophy Curschmann Steinert. Up to today this disease is seldom published particularly in obstetrics. This is a case report of a 23-year-old patient. Following extensive interdisciplinary diagnostic a successful caesarian delivery of a healthy boy was performed in the 39(th) week of pregnancy without any complications., (© Georg Thieme Verlag KG Stuttgart · New York.)

Introduction: Foetal megacystis (incidence 1:1 500) occurring in the first trimester may already be a sign of congenital malformation. Often, urethral valves are causally responsible in male foetuses far more frequently than urethral atresia. As a further differential diagnosis, the "prune-belly syndrome" needs to be distinguished. Far more difficult to classify prenatally is the rare MMIHS which, in contrast to the diagnosis of urethral valves, is associated with an unfavourable prognosis., Case Report: This is a report on a 28-year-old IG/0P, whose foetus of 26+4 gestational weeks was found on ultrasonography for the first time to have a megacystis of 48 mm diameter and bilateral hydronephrosis. The female foetus was surrounded by a normal amount of amniotic fluid which, during the further course of the pregnancy, developed into polyhydramnios. The serial puncture of the urinary bladder showed a normal karyotype and no impairment of the renal concentrating capacity or of protein loss. By reason of an immense enlargement of the abdomen due to the 100 mm large urinary bladder, a Caesarean section was conducted at 36+0 gestational weeks. A catheter could be inserted into the urinary bladder postpartum easily. However, nourishment was not possible and after radiological examination, MMIHS was diagnosed., Conclusion: In the case of a foetal megacystis detected by ultrasonography, especially associated with polyhydramnios and female gender, the rare MMIHS, which is infaust, should be taken into consideration., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: The group of the so-called late preterms (infants born at 34 0/7-36 6/7 weeks gestational age) has been underestimated with respect to their neonatal outcome. Among infants born before the 29th week of pregnancy, a gender-specific difference in favour of females regarding morbidity became evident. The aim of this study is to investigate whether these findings are transferable to the group of late preterms., Methods: The neonatal outcome of 528 consecutive singletons, born at 34 0/7-36 6/7 weeks gestational age and requiring intensive care, was examined., Results: Neonatal complications have been particularly analysed with regard to gender-specific differences. Boys (n=292) were significantly more frequently affected by sepsis (3.8 vs. 0.9%; p=0,0314, x²-test). Girls had significantly longer stays in the neonatal intensive care unit (median 12 (Q1:8; Q3:17) vs. 11 (6;16) days; p=0.0149, t-test). In a multiple logistic regression model, male gender and premature rupture of membranes were borderline significant with respect to the occurrence of sepsis - boys had a 4.4-fold risk (OR=0.228 [95% CI: 0.050-1.041]; p=0.0564) and premature rupture of membranes had a 3.5-fold risk (OR=3.462 [0.938-12.779]; p=0.0623). Strong cause variables for the length of stay in the neonatal intensive care unit were birth weight, gestational age and premature rupture of membranes after adjustment., Conclusion: The influence of foetal gender on the neonatal outcome in the late preterm group (34 0/7-36 6/7 gestational age) has been relativised., (© Georg Thieme Verlag KG Stuttgart · New York.)

Introduction: Ultrasound diagnostics enable the prenatal diagnosis of extensive lymphatic malformations. The diagnosis is made prenatally or directly after birth in 50-60% of the cases, in about 80% of the afflicted in the first year of life and up to 90% of the cases in the second year of life. PRENATAL DIAGNOSTICS AND POSSIBILITIES OF PRE- AND PERINATAL INTERVENTIONS: The so called hygroma colli is a primary symptom, which is detected during the routine ultrasound examination. Lymphatic malformations, which are diagnosed antenatally are often associated with chromosome abnormalities. The EXIT procedure allows invasive therapeutic or diagnostic interventions. Hereby the airway safety of Newborns with extended cervical or zerviko-facial lymphangiomas can be warranted. The survival chance and the prognosis may be significantly improved. MANIFESTATION AND ENT-CLINICS: 72 patients (mean age 12.3 yrs) with lymphatic malformations of the Head and Neck presented for the first time during a 5-years period until 31.12.2008 at the Department of Otolaryngology of the University of Marburg. Therapeutic interventions consisted in conventional surgery, laser assisted therapy, sclerosing therapy or a combined therapeutic approach., Conclusion: Extensive lymphatic malformations of the Head and Neck challenge treating physicians and affected families. Advancements of prenatal diagnostics and opportunities of peri- and postnatal interventions contribute to an improved survival chance and prognosis. Intensive interdisciplinary cooperation in specialised centers is of outstanding importance for an optimal care of these patients., (Georg Thieme Verlag KG Stuttgart.New York.)

Background: Pre-eclampsia affects 2% of pregnancies. This multisystem disorder is a major cause of maternal, foetal and neonatal mortality and morbidity. Neurological manifestations of eclampsia are headache, nausea, vomiting, cortical blindness and recurrent seizures., Objective: The purpose of this study was to determine whether the neurological symptoms correlate with MR imaging findings., Results: In a patient with eclamptic seizure and another one with blindness due to pre-eclampsia, the white matter hyperintensities on T (2)-weighted MR and FLAIRsequence images could be demonstrated in the occipital region and in the basal ganglia. Within 3-5 days all neurological symptoms and radiological abnormalities had resolved., Conclusion: These cerebral lesions could be classified as posterior reversible encephalopathy syndrome (PRES) or as reversible leukoencephalopathy syndrome (PLES). Thus, MRI supports differential diagnosis regarding non pregnancy-related cerebral disease and can be helpful for therapy planning in cases of pre-eclampsia.

Introduction: Leptin and neuropeptide Y (NPY) play important roles in the regulation of food intake, energy expenditure, hematopoesis and reproduction. The biological functions of leptin and NPY in fetal development, and their regulation during pregnancy by fetal and maternal factors remain poorly understood., Patients and Methods: From 55 women undergoing diagnostic amniocentesis between the 14th and 18th weeks of gestation samples of amniotic fluid were collected. In accord with the circadian rhythm of leptin secretion all amniocenteses were performed between 8.00 and 12.00 a. m. The concentrations of leptin and NPY in amniotic fluid were analysed using commercially available RIA's., Results: The amniotic fluid samples of 32 male and 23 female fetuses were determined and demonstrated no gender-dependent differences in leptin and NPY levels. No correlation was found between leptin/NPY and the maternal body mass index. NPY concentrations are lower in advanced gestational age pregnancies. Leptin levels revealed no differences with respect to gestational age., Conclusion: Leptin and NPY levels were independent of fetal gender and maternal BMI. This provides clues for alternative regulatory mechanisms in leptin and NPY secretion. Compared to the data of adults, our findings show high leptin concentrations in the amniotic fluid in the presence of only minor amounts of white adipose tissues which might include the placenta. Our data including the observation of lower levels of NPY in more advanced gestational age support the idea of specific factors regulating leptin and NPY secretion into the amniotic fluid.

Objective: During pregnancy about 30 grams of calcium are transferred to a full-term neonate. The enormous demand for calcium can be regulated by compensatory mechanisms in the maternal metabolism such as elevated steroid hormone levels, increased intestinal absorption of dietary calcium, and renal conservation, but also by mobilization of calcium from the maternal skeleton. Therefore we have investigated whether a decrease in maternal bone mineral density (BMD) can be observed during pregnancy and whether it differs between singleton and twin pregnancies., Methods: Quantitative ultrasonometry (QUS) was performed at the distal metaphysis of the phalanges (digits II - V). 54 singleton pregnancies and 6 twin pregnancies were longitudinally followed throughout pregnancy. In each trimester the amplitude-dependent speed of sound (AD-SOS) and the bone transmission time (BTT) were measured., Results: In 54 singleton pregnancies significant decreases in AD-SOS of 1.9 % (p

Persistent fetal supraventricular tachycardia (SVT) with more than 210 bpm frequently leads to congestive heart failure. We report on a case with SVT and congestive heart failure that converted into sinus rhythm within 19 days of therapy with flecainide and beta-acetyldigoxin. A 32-year-old II gravida I para (25 + 1 weeks of gestation) presented with fetal SVT of 267 bpm. A non-immunologic hydrops fetalis was diagnosed by ultrasound showing ascites, pleural and pericardial effusion and tricuspid regurgitation. Within 19 days of combination therapy with flecainide and digoxin, cardioversion was achieved. After 36 days of therapy no more signs of cardiac failure could be detected. A healthy boy was born at 38 + 6 weeks of gestation. Although cardioversion is expected after 72 h of therapy according to the literature, this fetus converted into sinus rhythm on day 19 of therapy. This indicates that patients should not be considered resistant to treatment within the first 3 - 4 days.

Introduction: A polyhydramnion is diagnosed in 0.4 to 3.3 % of all pregnancies. The most common causes of increased amniotic fluid include maternal diabetes mellitus, fetal malformations and chromosomal aberrations, twin-to-twin transfusion syndrome, rhesus incompatibility syndrome, and congenital infections. After exclusion of other etiologies for polyhydramnion, the very rare, autosomal-recessive transferred hyperprostaglandin E syndrome (HPS) has to be considered., Patients and Methods: We report on a 31-year-old women who visited our obstetrical outpatient clinics at 22 + 4 weeks of gestation for prenatal ultrasound scanning and amniocentesis. This, the patient's third pregnancy had been without complications so far. She had delivered two children before, one of them bearing the HPS. The women herself suffered from epilepsy. At 26 + 0 weeks of gestation the pregnancy was complicated by a polyhydramnion requiring serial amniocentesis for reducing amniotic fluid load. Among others, her amniotic fluid was analyzed for chloride concentration and for genetic aberrations regarding HPS. Serological investigations and an oral glucose tolerance test (oGTT) were performed., Results: Amniocentesis revealed a normal chromosomal pattern. The oGTT demonstrated values in the normal range. Serological investigations regarding TORCH infections were without pathological findings. The chloride concentration was highly increased in the amniotic fluid, which is suspicious for HPS. Finally, molecular analysis proved an NKCC2-mutation responsible for HPS. A cesarean section was performed at 33 + 3 weeks of gestation., Conclusion: If HPS is suspected to be the cause of polyhydramnions, the chloride concentrations in the amniotic fluid and molecular analysis for HPS should be performed. Interdisciplinary care, diagnostics and therapy in an experienced perinatal center are essential for an optimal outcome of the pregnancy and the newborn infant.

Objective: The aim of this study was to determine if aprotinin could affect postpartal fibrinolysis when given at the latest 15 min before delivery and if there is a difference between normal delivery and caesarean section. Furthermore we wanted to examine if the thrombin-antithrombin-III-complex (TAT-III) and prothrombin fragments F1 + 2 changed in the peripartal period and if prethrombotic stages could be recognized., Patients and Methods: 84 patients (15 - 44 years of age) have been examined (41 normal deliveries, 43 cesarean sections). After giving informed consent and randomization, 30 of these patients were administered 1 Mio KIE aprotinin (Trasylol(R)) at the latest 15 min before delivery (15 normal deliveries, 15 cesarean sections). The results of TAT-III, prothrombin fragments F1 + 2, factor VIII and partial thromboplastin-time (PTT) were collected shortly before and after delivery and 30 and 120 min after detachment of the placenta., Results: Normal deliveries without aprotinin showed a significant increase of TAT-III and an evident increase of prothrombin fragments F1 + 2. After administration of aprotinin this increase was significantly lower. The increase of TAT-III and prothrombin fragments F1 + 2 in patients with caesarean sections was evidently lower than in normal deliveries and was not influenced significantly by aprotinin. Factor VIII and partial thromboplastin time (PTT) showed no relevant changes in all study groups., Discussion and Conclusion: The consumption of coagulation and fibrinolysis factors induced by delivery of the child and detachment of the placenta can be reduced after administration of aprotinin given at the latest 10 - 18 min before partus. This could be used in therapy and prophylactic treatment in high-risk patients (e. g., pre-eclampsia, HELLP syndrome, etc.).

Background: Are obstetric doppler ultrasonographic measurements of the fetal and maternal flow parameters reproducible?, Patients and Methods: For internal quality management, doppler ultrasonographic measurements were performed on 81 patients (random screening sample) with an Acuson Sequoia Ultrasound at the Universitätsfrauenklinik Marburg. Successively two experienced investigators measured the umbilical artery, medial cerebral artery, and the uterine arteries. The correlation between the measurements of the two investigators was presented in a spread chart. In order to exclude systematic differences between the measurements, linear regression was analyzed and the distance to the abscissa was calculated. A relative divergence of more than 20% was determined as a non corresponding measurement., Results: The distance of the linear regression to the abscissa was calculated for the four vessels: umbilical artery 0.4 (95% CI; 0.124 - 0.486), medial cerebral artery 0.9 (95% CI; 0.534 - 1.264), right uterine artery 0.2 (95% CI; 0.124 - 0.305), and left uterine artery 0.2 (95% CI; 0.121 - 0.317). Concerning the four arteries, a divergence of more than 20% between the two investigators was found: umbilical artery 16%, medial cerebral artery 42%, right uterine artery 28%, and left uterine artery 37%., Conclusions: Because the interobserver variability was surprisingly high and acceptable correlation could be stated only for the umbilical artery, internal quality standards are essential. Routine use of different devices should be made after careful consideration only, especially if clinical decisions are to be based on them.

Objective: The aim of this study was to determine (a) if alpha-2-antiplasmin, plasminogen and plasminogen-activator-inhibitors (PAI 1 - 4) change in the peripartal period, (b) if these changes are explainable by the detachment of the placenta and the release of coagulation factors, and (c) if aprotinin could affect postpartal fibrinolysis when given shortly before delivery., Patients and Methods: 84 patients have been examined (41 normal deliveries, 43 cesarean sections). After informed consent and randomization, 30 of these patients were administered 1 million KIU aprotinin (Trasylol(R)) at the latest 15 minutes before delivery (15 normal deliveries, 15 cesarean sections). The results of PAI (1 - 4), alpha-2-antiplasmin and plasminogen were collected shortly before and after delivery and 30 and 120 min after detachment of the placenta., Results: Normal deliveries and cesarean sections without aprotinin showed an evident decrease of PAI (1 - 4) after delivery while alpha-2-antiplasmin and plasminogen values did not change. After administration of aprotinin the activity of PAI (1 - 4) increased slightly after labour in normal deliveries and cesarean sections and then slowly decreased later. alpha-2-Antiplasmin levels increased post partum and then decreased to the original values. Plasminogen did not change. There were no significant differences between normal deliveries and cesarean sections with regard to the three parameters studied in this work., Discussion and Conclusion: Changes in PAI (1 - 4) and alpha-2-antiplasmin after administration of aprotinin could be the result of a minimal demand on the fibrinolytic system. Finally, aprotinin can reduce the consumption of coagulation and fibrinolysis factors induced by detachment of the placenta. This could be used in therapy and prophylactic treatment in high-risk patients (e.g., pre-eclampsia, HELLP syndrome, etc.).

Endemic occurrence of Q fever among persons in close contact with domestic animals is well known in some rural regions of Germany. The prevalence of antibodies indicating acute Q fever in pregnancy reported in the literature varies between 0.2 % and 4.7 % of the screened population. Q fever in pregnancy initially manifests as placentitis and often leads to premature birth (30 %), growth restriction (46 %), spontaneous abortion (22 %) or fetal death in utero (7 %). Some impairment of pregnancy is observed in over 70 % of cases with seroconversion during pregnancy. Thus Q fever serology should be tested in all pregnant women presenting with atypical pneumonia and/or prolonged fever of unknown etiology. It is of interest that medical staff members in contact with Cociella burnetii infected pregnant women are also at risk of acquiring an acute Q fever infection. We report about a patient presenting with confirmed acute and later chronic Q fever during pregnancy in whom antibiotic treatment with rifampicin and clarithromycin proved to be effective and led to the vaginal delivery of a premature but healthy infant. We believe that maternal serum screening for transmissible infections should also include Q fever serology in certain rural regions.

Aim: This retrospective study aims at determining the accuracy of sonographic estimation of birth weight based on ultrasound examinations performed at the department of gynaecology of Philipps University at Marburg, Germany., Method: 630 children were born at the university department of gynaecology during the first six months of 1998. 519 babies had been examined sonographically and their birth weight estimated within ten days prior to delivery. 176 (33,91 %) of these examinations were carried out by experienced sonographers complying to the level II standard of performance set out by DEGUM. 343 (66.09 %) of examinations were performed by less experienced junior doctors at the time of the mothers' admission to the delivery room. Two reference tables published by Hansmann und Ferrero were used to estimate birth weight., Results: The lower and upper quartile of deviation between estimated weight and actual weight came to -200 g and + 200 g using the Hansmann method and -180 g and + 220 g respectively based on the method of Ferrero. It has to be noted that even greater differences occurred: the 10 % least exact estimates deviated by 500 g and more. The difference between estimated and actual weight increased with the length of pregnancy. The fully trained physician (DEGUM II) tended to estimate the birth weight more accurately. The average birth weight estimated sonographically was lower in "DEGUM II-babies" than that of children whose weight was estimated at the point of admission to the delivery room (Mann-Whitney p < 0,0001). Junior doctors showed a tendency to over-estimate the birth weight of babies actually being underweight. The opposite happened with babies who presented a relatively higher birth weight: their projected birth weight was underestimated. On the other hand, the regression line of the sonographic estimations of birth weight performed by the expert (level II DEGUM) fit to the expected bisector of the angle (Passing-Bablock p > 0,05)., Conclusion: When clinical decisions are based on estimated values of birth weight, the possible deviation of this value from the actual weight has to be taken into account. In our study this difference came up to 500 g independent of the level of the examiner's ultrasonographic training.