Stabilisation of cardiopulmonary function in newborns with congenital diaphragmatic hernia using lung function parameters and hemodynamic management.

OBJECTIVE: Evaluation of lung function parameters and additional use of prostaglandin E1 (PGE1) for the stabilisation of cardiopulmonary function in patients with congenital diaphragmatic hernia (CDH) and pulmonary hypertension (PHT). DESIGN: Observational study. PATIENTS: Between 2007 and 2009 8 patients with CDH have been treated in our pediatric intensive care unit (gestational age 34 + 0 - 40 + 4 weeks, birth weight 2 160-3 840 g). All patients required respiratory support. Gentle mechanical ventilation adapted to the degree of pulmonary hypoplasia based on serially measurements of lung function parameters to find appropriate ventilator settings has been performed. MAIN RESULTS: Functional residual capacity (FRC) and compliance of the respiratory system in all patients were markedly reduced. A FRC between 9.3-10.6 ml/kg and compliance between 1.1-1.8 ml/kPa/kg indicated pronounced hypoplasia of the lungs. Doppler flow patterns through the arterial duct were classified into left-to-right, right-to-left and bidirectional shunting and correlated to the degree of PHT. The additional use of PGE1 to reopen the arterial duct and to stabilize right ventricular function led to an amelioration of severe PHT and preoperative stabilisation in 2 newborns with pronounced pulmonary hypoplasia. All patients underwent successful surgery, and did not show any complications after 2 years follow-up. CONCLUSION: Measurements of lung function parameters and adaptation of mechanical ventilation to the degree of pulmonary hypoplasia and additional therapy with PGE1 may help to improve the outcome in CDH patients.

[Fetal juvenile granulosa cell tumor with hermaphroditism verus - prenatal diagnosis, management and outcome]. / Fetaler juveniler Granulosazelltumor mit Hermaphroditismus verus - pränatale Diagnostik, Management und postnatales Outcome.

Fetal ovarian cysts are common during pregnancy and after delivery. Most of these cysts are simple cysts that involute during pregnancy or in the first months of life. However, complicated cyst with a heterogeneous structure and also possible and can result in various complications: rupture, hemorrhage, ascites, edema of the labia, compression of other viscera, and ovarian torsion. In this case report we describe rare diagnosis of a complicated fetal ovarian cyst with edema of the labia and moderate ascites. The neonate had ambiguous genitalia with clitoromegaly. The newborn underwent surgery with oophorectomy. During the operation a uterus with fallopian tubes was found. The pathological findings showed a juvenile granulosa cell tumor FIGO Ia. Karyotyping revealed a mosaic of 45, X/ 46, X mar (Y) in the peripheral blood as well as in the granulosa cell tumor. Because of a right side inguinal hernia, the child underwent a second surgery. Specimen taken from the abdominal gonad and the inguinal region showed testicular and dysplastic ovarian tissue. There were elevated levels of androgens in the child's peripheral blood due to the granulosa cell tumor. In summary, this case report describes a fetus with true hermaphroditism and a juvenile granulosa cell tumor diagnosed as a complicated ovarian cyst in the 32 (nd )week of pregnancy.

Preliminary report on elective preterm delivery at 34 weeks and primary abdominal closure for the management of gastroschisis.

INTRODUCTION: We aimed to critically evaluate elective preterm delivery and immediate abdominal wall closure and other techniques for the management of gastroschisis, hypothesizing that the advantages of an elective preterm delivery outweigh possible complications related to prematurity at birth. PATIENTS AND METHODS: 13 gastroschisis patients were enrolled in the elective preterm delivery program (Group 1) since 1999. Patients were delivered by cesarean section in the 34th gestational week, with immediate primary closure of the defect. Data regarding parameters at and after birth were compared with a historical control group of 10 patients conventionally managed for gastroschisis in a similar period (1994 - 1999) (Group 2). The primary endpoints of this study were the initiation of oral feeding and the length of hospital stay. RESULTS: There was a significantly faster initiation of oral feeding (p = 0.0012) and a shorter hospital stay (p = 0.0160) in Group 1. The postoperative outcome was excellent in all patients. Acute and late complications were fewer and less severe in Group 1 and none were related to prematurity. CONCLUSIONS: Elective preterm delivery appears to be an effective method for the management of gastroschisis, and a method whose advantages thus far have outweighed the possible complications due to prematurity.

Screening for trisomy 21 by maternal age, fetal nuchal translucency and maternal serum biochemistry at 11-14 weeks: a German multicenter study.

OBJECTIVE: To examine the effectiveness of screening for trisomy 21 by a combination of maternal age, fetal nuchal translucency (NT) thickness and maternal serum biochemistry using free beta-human chorionic gonadotropin (hCG) and pregnancy-associated plasma protein-A (PAPP-A) at 11-14 weeks of gestation. METHODS: This was a multicenter study of screening for trisomy 21 by a combination of maternal age, fetal NT and maternal serum free beta-hCG and PAPP-A at 11-14 weeks of gestation, using the methodology developed by the Fetal Medicine Foundation. The distribution of estimated risks for trisomy 21 was determined and the sensitivity and false-positive rate for a risk cut-off of 1 in 300 were calculated. RESULTS: In total, 3864 singleton pregnancies with live fetuses at 11-14 weeks were examined and the fetal NT and maternal serum free beta-hCG and PAPP-A were successfully measured in all cases. The median maternal age was 33 (range 15-46) years and, in 1271 (35.8%), the age was 35 years or more, the median gestation at screening was 12 (11-14) weeks and the median fetal crown-rump length was 64 (range 45-84) mm. The fetal NT was above the 95th centile in 73.7% (14 of 19) of trisomy 21 and in 4.8% (169 of 3505) of normal pregnancies. The estimated risk for trisomy 21 based on maternal age, fetal NT and maternal serum free beta-hCG and PAPP-A was 1 in 300 or greater in 6.6% (233 of 3505) of normal pregnancies, in 84.2% (16 of 19) of those with trisomy 21 and 88.9% (24 of 27) of those with other chromosomal defects. CONCLUSIONS: In Germany, the results of screening for chromosomal defects by measurement of fetal NT and maternal serum biochemistry, in centers with appropriately qualified sonographers, are similar to those reported in the UK using the same methodology.

Fryns syndrome: two further cases without lateral diaphragmatic defects.

We report on two unrelated infants with Fryns syndrome but without lateral diaphragmatic defects. In case 1, a female stillborn, pertinent findings included a coarse facies, cleft palate, short neck with a broad pterygium, heart defect, lung hypoplasia, omphalocele, urogenital malformations, anal atresia, and distal digital hypoplasia. Case 2 showed a coarse facies with cleft lip and palate, para-oesophageal hernia with herniation of the stomach into the thoracic cavity, malrotation of the intestine, hydronephrosis, and anal atresia. At age 14 months she was developmentally severely retarded. These observations represent the 11th and 12th reports of probable Fryns syndrome without lateral diaphragmatic defect.

[Lung edema as a complication of tocolysis in twin pregnancy]. / Lungenödem als Tokolysekomplikation bei Geminischwangerschaft.

It was reported about a case of acute pulmonary oedema during tocolytic therapy with beta 2-stimulator Fenoterol. The aetiologic factors of the isotonic hyperhydration by beta-mimetic drugs, medicamentous influences and favoured factors have been discussed. Diagnostic and therapeutic consequences have been concluded.

[Sonographically evident changes in placental structures in pregnancy. 1]. / Sonographisch nachweisbare Veränderungen plazentarer Strukturen während der Schwangerschaft. 1. Mitteilung.

During the routine prenatal care a sonographical valuation of the placental tissue structure is performed. It could be proved at 657 gravids (1436 examinations) that the placental tissue structure is changing the course of pregnancy. The results were graduated in three stages from 0 to 3. Stage 0 represents the placenta in the first and second trimester and Stage 3 is used for the ripe placenta. The practical use of the placental tissue structure diagnosis is discussed.

[Ehlers-Danlos syndrome and pregnancy]. / Ehlers-Danlos-Syndrom und Schwangerschaft.

There is reported about pregnancy and delivery of a 31 year old woman with Ehlers-Danlos syndrome type VIII. In the present case there were no complications. Occurrence of perilous injuries in other subtypes is discussed.

[Sonographically detectable changes in placental structures in pregnancy. 4. Statistical comparison of the frequency distribution of placental stages 0-3 in newborn infants in a pregnancy of 37-42 weeks duration]. / Sonographisch nachweisbare Veränderungen plazentarer Strukturen während der Schwangerschaft. 4. Mitteilung: Statistischer Vergleich der Häufigkeitsverteilung der Plazentastadien 0-3 bei Neugeborenen mit einer Schwangerschaftsdauer von 37-42 Wochen.

Among women with a duration of pregnancy between 37 and 42 gestational weeks procentual frequency, confidence intervals of O. Bunke, pounts of separability and areas of unsharpners were analysed. 1412 sonographical examinations were done in 645 pregnant women. Stage 0 was found statistically significant more frequent with error of probability alpha = 0.05 until the 30. week of pregnancy, stage 1 between the 21. and 36. week of pregnancy, stage 2 between the 33. and 36. gestational week and stage 3 in 37. and 38. week. Transformation (areas of unsharpness) from stage 0 to stage 1 occurs between the 24. and 32. gestational week, from stage 1 to stage 2 between the 31. and 34. week, from stage 2 to stage 3 between the 36. and 42. gestational week.

[Sonographically detectable changes in placental structures in pregnancy. 5. Statistical comparison of the frequency distribution of placental stages 0-3 in newborn infants in a pregnancy of less than 37 weeks duration]. / Sonographisch nachweisbare Veränderungen plazentarer Strukturen während der Schwangerschaft. 5. Mitteilung: Statistischer Vergleich der Häufigkeitsverteilung der Plazentastadien 0-3 bei Neugeborenen mit einer Schwangerschaftsdauer von weniger als 37 vollendeten Wochen.

The analysis of sonographically provable changes of placental structures in 97 pregnant women (202 examination) shows in cases with a duration of pregnancy less than 37 gestational weeks that stage 0 could be found statistically significant more frequent until the 32nd week of pregnancy, stage 1 in the whole pregnancy, stage 2 and 3 between the 29. and 36. gestational week. Stage 0 and 1 don't effect prematurity; however stages 2 and 3 could be proved before the 32nd or 34th week of pregnancy in 41 or 100% of examination respectively and effect a premature birth. Stage 2 could be proved 2,8 times more frequent and stage 3 4 times more frequent in premature babies than in newborns with a normal duration of pregnancy.

[Sonographically detectable changes in placental structures in pregnancy. 6. Effect of sonographic placental morphology on premature labor and intrauterine growth retardation]. / Sonographisch nachweisbare Veränderungen plazentarer Strukturen während der Schwangerschaft. 6. Mitteilung: Der Einfluss der sonographischen Plazentamorphologie auf Frühgeburtlichkeit und intrauterine Retardierung.

Influence of sonographically demonstrable changes of placental structure on prematurity and intrauterine retardation was analysed and concerning its statistical rehability in 807 gravids. While the stages 0 and 1 don't effect prematurity and intrauterine retardation, the stage 2 is accompanied premature infant of normal birthweight if proved before the 32nd week of pregnancy. If stage 3 appears before the 34. week of pregnancy we found in 30.6% a small for date newborn, in 64% a normal weight and in 80% a small for date premature newborn. In comparison with the normal weight newborn the difference is statistically significant with an error probability of alpha = 0.05.

[Correlation of sonomorphologic placental maturity with the L/S ratio and creatinine level in the amniotic fluid]. / Zur Korrelation der sonomorphologischen Plazentareife mit der L/S-Ratio und dem Kreatininspiegel im Fruchtwasser.

With 184 L/S-ratio determinations and 132 creatinine determinations in amniotic fluid we tried to show a connection between these parameters and sonographically provable changes of placental structures. We found no relationship between placental grading and fetal lung and renal maturity by statistical processing of the results, considering gestational age. There is between L/S-ratio and creatinine determinations no statistical relationship, too.

[Retroperitoneal ganglioneuroma as a cause of obstructed labor]. / Retroperitoneales Ganglioneurom als Geburtshindernis.

The case of a primigravida is reported who was operated on for a retroperitoneal ganglioneuroma at the age of 12. At that time the tumor surgically could not be completely removed because of its size and anatomical location in relation to the backbone and the large blood vessels. A renewed tumour growth was observed in the course of the years. At the end of pregnancy the tumour proved to be an obstruction to the labour process.

[Sonographically detectable changes in placental structures in pregnancy. 2. Statistical comparison of the frequency distribution of placenta stages 0-3 newborn infants with a birth weight of 2,500-3,999 grams]. / Sonographisch nachweisbare Veränderungen plazentarer Strukturen während der Schwangerschaft. 2. Mitteilung: Statistischer Vergleich der Häufigkeitsverteilung der Plazentastadien 0-3 bei Neugeborenen mit einem Geburtsgewicht von 2 500-3 999 Gramm.

1200 examinations of sonographical demonstrable placental ripeness were done in 552 pregnant women. The frequency of stages 0 to 3 as compared with aid of O. Bunke's confidence intervals. Stage 0 were found frequently until the 30. week of pregnancy, stage 1 between the 21. and 36. gestational week, stage 2 between the 33. and 38. week of pregnancy and stage 3 between the 35. and 40. week. The comparison of the areas of unsharpness several stages possibly may give informations about the period of pregnancy when the stages coincide.

[Sonographically detectable changes in placental structures in pregnancy. 3. Statistical, comparison of the frequency distribution of placenta stages 0-3 in newborn infants with a birth weight of less than 2,500 grams]. / Sonographisch nachweisbare Veränderungen plazentarer Strukturen während der Schwangerschaft. 3. Mitteilung: Statistischer Vergleich der Häufigkeitsverteilung der Plazentastadien 0-3 bei Neugeborenen mit einem Geburtsgewicht von weniger als 2 500 Gramm.

The utilisation of sonographically provable changes of placental structures in 131 pregnant woman (277 examinations) shows in cases with newborn infants with a weight of birth under 2500 gram than the stage 0 is found significant frequent to the 32. week of pregnancy, the stage 1 to the 40. week of pregnancy, the stage 2 between the 29. and 40. week of pregnancy and the stage 3 between the 31. and 40. week of pregnancy. The stages 0, 1 and 2 influence in comparison with the newborn infants with a weight of birth between 2500 gram and 3999 gram not the weight in newborn infants under 2500 gram. The stage 3 will prove frequent in 13,5 times before the 34. week of pregnancy as in the comparison group with normal weight of birth.

[Diagnostic and therapeutic aspects of pelvic vein thrombosis in pregnancy]. / Diagnostische und therapeutische Aspekte der Beckenvenenthrombose in der Schwangerschaft.

Deep pelvic venous thrombosis is a severe complication during pregnancy and requires an early diagnosis and an adequate well-timed therapy. 12 pregnant women with thrombosis of deep pelvic veins have been registered ++ over a period of 10 years. Diagnosis was secured with phlebography in 5 of these cases. It is reported about the necessity and the x-ray exposure of the examination. Treatment of choice is the heparin-longtime-therapy 90 per cent of our cases have been cured successfully.

[Hyperthyroidism and acute renal failure in partial bladder mole]. / Hyperthyreose und akutes Nierenversagen bei partieller Blasenmole.

We report on a 31-year-old II gravida I para treated because of a hydatidiform mole, hyperthyroidism and acute renal failure in the 16th week of pregnancy. The pathomechanism of trophoblast-induced hyperthyroidism will be discussed. To our knowledge this is the first report on acute renal failure in association with trophoblast-induced hyperthyroidism.

[Immune thrombocytopenia, anemia and leukopenia during pregnancy. Successful therapy with extracorporeal immunoadsorption]. / Immunogene Thrombozytopenie, Anämie und Leukopenie während Schwangerschaft. Erfolgreiche Extrakorporaltherapie mit immunadsorption.

HISTORY AND CLINICAL FINDINGS: A 28-year-old woman in her fourth pregnancy had a positive direct Race-Coombs test in her 14th week of pregnancy. About 6 weeks later she developed marked anaemia, severe thrombocytopenia and leukopenia with nose-bleeds as well as petechiae over the lower abdomen and the inguinal regions. INVESTIGATIONS: Platelet count was 7000/microliter, haemoglobin concentration 4.8 mmol/l (7.73 g/dl) and WBC count 2600/microliter). Bleeding time was markedly prolonged. Erythrocyte-attached immunoglobulin G and complement components as well as antithrombocyte and antigranulocyte antibodies were demonstrated. Haemoblastosis or aplastic anaemia was excluded by bone marrow examination. TREATMENT AND COURSE: The pancytopenia with immune thrombocytopenia, autohaemolytic anaemia and associated leukopenia were classified as Evans syndrome. Administration of glucocorticoids and of immunoglobulins intravenously for 5 days failed to improve the blood picture significantly. But platelet count rose significantly on immunoadsorption (Ig Therasorb columns), allowing the pregnancy to proceed. CONCLUSION: In life-threatening states Ig immunoadsorption can achieve well tolerated, effective and specific elimination of pathogenetically relevant antibodies against blood cells and can also be applied in pregnant women.

Ophthalmomyiasis externa due to the sheep botfly Oestrus ovis (Diptera: Oestridae) in southwest Germany.

We present a case of ophthalmomyiasis externa in a 54-year-old man in southwest Germany (Baden-Württemberg). After the patient had been attacked by a fly, he complained of a foreign-body sensation and itching in his right eye and developed purulent conjunctivitis. Slit-lamp examination revealed the presence of translucent, rapidly moving insect larvae in the conjunctival sac and on the corneal surface. Following removal of all larvae and instillation of gentamicin, the symptoms completely resolved within 2 days. Two of the nine larvae removed were examined by light microscopy and taxonomically classified as first-instar larvae of the sheep botfly Oestrus ovis L. 1758. Although this fly is the most common causative agent of external ophthalmomyiasis in the world, infestations in central Europe have only rarely been reported. Besides the case report and a detailed description of the larvae, an overview of the literature on European cases of ophthalmomyiasis externa caused by Oestrus ovis is given.