Differentiation between TTTS Stages I vs II and III vs IV does not affect probability of double survival after laser therapy.

OBJECTIVE: To compare the perinatal outcome of monochorionic twin pregnancies with twin-twin transfusion syndrome (TTTS), according to the disease severity, defined using Quintero staging, after treatment with fetoscopic laser surgery. METHODS: This was a single-center study of 1020 consecutive cases with severe TTTS, which were treated with fetoscopic laser surgery. During the study period from January 1995 to March 2013, the participants were included at a mean ± SD gestational age of 20.8 ± 2.2 weeks. Perinatal survival analysis, including the rates of double survival and survival of at least one fetus, was undertaken according to the Quintero staging system. For blockwise comparisons of data, the whole population was divided into five chronologically consecutive study subgroups of 200 patients in each of the first four subgroups and 220 in the last one. RESULTS: For the entire study population with known outcome (n = 1019), the rate of pregnancy with double fetal survival was 69.0% (127/184) in Stage-I, 71.4% (257/360) in Stage-II, 55.4% (236/426) in Stage-III and 51.0% (25/49) in Stage-IV TTTS cases. At least one twin survived in 91.3% (168/184) of pregnancies with Stage-I, 89.7% (323/360) of those with Stage-II, 83.1% (354/426) of those with Stage-III and 77.6% (38/49) of those with Stage-IV TTTS. The rates of double survival and survival of at least one fetus were both significantly higher in Stage-II TTTS compared with those in Stage-III TTTS cases (P < 0.001 and P = 0.011, respectively). Survival rates between pregnancies with Stage-I vs Stage-II TTTS and between those with Stage-III vs Stage-IV TTTS were not significantly different. Therefore, we combined pregnancies with Stage-I or Stage-II TTTS, and those with Stage-III or Stage-IV TTTS. The double survival rate was 70.6% (384/544) in combined Stage-I and Stage-II vs 54.9% (261/475) in combined Stage-III and Stage-IV TTTS cases (P < 0.001). At least one twin survived in 90.3% (491/544) of pregnancies with Stage-I or Stage-II TTTS vs 82.5% (392/475) in those with Stage-III or Stage-IV TTTS (P < 0.001). The double survival rate increased between the first and the last consecutive study subgroups from 59.8% (55/92) to 75.0% (96/128) (adjusted odds ratio (aOR)linear trend , 1.26 (95% CI, 1.01-1.56); P = 0.037) in pregnancies with Stage-I or Stage-II TTTS and from 41.7% (45/108) to 62.0% (57/92) (aORlinear trend , 1.21 (95% CI, 0.98-1.50); P = 0.082) in those with Stage-III or Stage-IV TTTS. Double survival rate was the lowest for Stage-III cases in which the donor twin was affected by severely abnormal Doppler findings (45.4% (64/141)). CONCLUSIONS: Double survival and survival of at least one fetus in monochorionic twin pregnancies with TTTS were related significantly to Quintero stage. However, our data show that the differentiation between Stages I vs II and Stages III vs IV does not have any significant prognostic implication for perinatal survival. © 2020 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

First-trimester intervention in twin reversed arterial perfusion sequence.

OBJECTIVE: To report the outcome of monochorionic twins with twin reversed arterial perfusion (TRAP) sequence following interstitial laser therapy in the first trimester. METHODS: This was a retrospective cohort study of all consecutive cases of TRAP that underwent interstitial laser therapy at ≤ 14 + 3 weeks' gestation between January 2014 and April 2016. Interstitial laser treatment was performed under ultrasound guidance using a 400-nm Nd:YAG laser fiber. Hospital records were reviewed to ascertain perinatal survival and morbidity. RESULTS: Twelve monochorionic twin pregnancies underwent interstitial laser treatment of the umbilical artery of the acardiac fetus, at a median gestational age of 13 + 5 (interquartile range (IQR), 13 + 4 to 14 + 0) weeks. In all cases, one treatment was sufficient to achieve complete interruption of the perfusion of the acardiac twin. There were no procedure-related complications during or within 48 h after the procedure. In one (8.3%) case, intrauterine death of the pump twin occurred 2 weeks after the intervention. All other cases (91.7%) resulted in a live birth at a median gestational age of 39 + 6 (IQR, 37 + 1 to 41 + 2) weeks and with a median birth weight of 3370 (IQR, 2980-3480) g. No neonatal mortality or serious morbidity occurred. CONCLUSIONS: Our results support the use of interstitial laser therapy in the first trimester of pregnancy complicated by TRAP sequence, showing a live birth rate of 92%. The results of a randomized controlled trial, evaluating early vs late intervention in pregnancy with TRAP sequence, are awaited. © 2019 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology.

Quantified discordant placental echogenicity in twin anemia-polycythemia sequence (TAPS) and middle cerebral artery peak systolic velocity.

OBJECTIVE: To quantify sonographic placental echogenicity in twin anemia-polycythemia sequence (TAPS) and to correlate it with middle cerebral artery peak systolic velocity (MCA-PSV) measurements. METHODS: We performed a retrospective search for consecutive TAPS cases between 16 and 36 weeks of gestation (MCA-PSV > 1.5 multiples of the median (MoM) in the anemic donor and < 1.0 MoM in the polycythemic recipient) in our database of monochorionic twin gestations from January 2007 until December 2016. In cases for which ultrasound images showing the donor's and the recipient's part of the placenta were available, echogenicity for both twins was quantified by image processing. MCA-PSV Doppler values of both fetuses were correlated to their respective placental echogenicity. Placental thickness of both twins was also measured. RESULTS: Of 756 cases with MCA-PSV measurements identified from the database, 36 (4.8%) had TAPS; of these, 23 had TAPS combined with twin-twin transfusion syndrome and 13 showed isolated TAPS. Placental echogenicity could be quantified in 28 pregnancies. Mean ± SD placental echogenicity of donor twins was significantly higher than that of recipients (138.7 ± 22.8 vs 77.9 ± 37.0; P < 0.0001). Furthermore, a significant positive correlation was found between placental echogenicity and MCA-PSV MoM (R = 0.67, P < 0.0001). Mean placental thickness of donor twins (n = 20) was significantly higher than that of recipients (49.3 mm ± 13.4 vs 25.4 mm ± 10.1; P < 0.0001). CONCLUSIONS: Echogenicity of the placental share in recipient and donor twins with TAPS correlates with MCA-PSV values. Quantification of sonographic placental echogenicity may help to determine the severity of TAPS in monochorionic twins. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Use of intrapartum ultrasound in the prediction of complicated operative forceps delivery of fetuses in non-occiput posterior position.

OBJECTIVE: To evaluate the hypothesis that intrapartum ultrasound (ITU) measurements, including the angle of progression (AOP), progression distance (PD) and head direction (HD), can predict complicated forceps delivery in non-occiput posterior deliveries. METHODS: In this prospective observational study, a single operator performed ITU on 30 patients with an indication for operative forceps delivery. Managing obstetricians were blinded to the results. ITU was performed just before blade application, between contractions and concurrently with contractions and active pushing. Forceps delivery was classified as complicated when one or more of the following situations occurred: three or more tractions; a subjective impression of a difficult or failed application; a third-degree or higher perineal tear; significant bleeding during the episiotomy repair; major tear; significant traumatic neonatal lesion. RESULTS: Twenty-one forceps deliveries were classified as uncomplicated and nine were complicated. The strongest predictor of a complicated forceps delivery, calculated using the area under the receiver-operating characteristics curve (AUC), was the AOP between contractions (AOP1) (AUC = 98.9%). The best cut-off for predicting a difficult forceps delivery was an AOP1 of 138° (sensitivity = 85.7%, specificity = 100%). The best predictive model included both the AOP1 and the HD during a contraction with active pushing (HD2). CONCLUSION: The sonographic parameters AOP and HD can be used to predict complicated operative forceps delivery in fetuses in non-occiput posterior position.

Vasa praevia: risk-adapted modification of the conventional management--a retrospective study.

PURPOSE: Undiagnosed vasa praevia carries an imminent risk of fetal death and increases with IVF. When diagnosed, the question arises as to whether the conventional prenatal management of routine steroid administration for fetal lung maturation and elective caesarean section in week 35 is generally justified in face of the risks involved. We present a retrospective study of a risk-adapted modification of the conventional management of vasa praevia. MATERIAL AND METHODS: We analysed 11 years of records involving 18 cases of antenatally diagnosed vasa praevia at our perinatal centre. Each case was managed by a risk-adapted modification of the conventional treatment where both, the steroid administration and the timing of delivery, were dependent on the patient history and clinical signs for preterm birth. RESULTS: There were no lethal fetal, neonatal, or maternal complications. The earliest caesarean section took place at 34 weeks 1 day, the latest at 37 weeks 1 day, and in more than half of the cases at ≥ 36 weeks. CONCLUSION: Steroid application is generally recommended for pregnancies before 34 weeks carrying a risk for preterm birth. Thus, retrospectively, none of our cases required steroid administration. This supports our protocol of not obligatorily administering steroids. Delaying the caesarean section up to two weeks beyond the conventionally recommended date of 35 weeks in 78% of our cases resulted in no complications. This justifies the suitability of determining the timing of delivery based on our individual patient assessment. In conclusion, the following recommendations for a risk-adapted management of vasa praevia can be made: 1. weekly evaluation of risk factors for preterm delivery; 2. steroid administration only at risk for preterm birth; 3. admission to hospital with full obstetric and neonatal care facilities between 32 and 34 weeks; 4. elective caesarean section between 35 and 37 weeks, risk-adapted.

Magnetic resonance imaging of the active second stage of labour: proof of principle.

OBJECTIVE: To prove that magnetic resonance imaging of foetal anatomy during the active second stage of vaginal delivery is feasible. MATERIALS AND METHODS: Initially, five pregnant volunteers around the 30th week of gestation were examined in an open MRI. Based on the findings, one vaginal delivery was acquired under real-time imaging. To monitor the birth status during image acquisition, an MR-compatible wireless cardiotocography (CTG) system was built. Single-shot sequence parameters were optimised to compensate motion artefacts during labour. RESULTS: Safety requirements to monitor the birth process under real-time MR imaging were met. High-resolution MR images were acquired immediately before and after delivery. In one patient, TSE single-shot cinematic sequences of the active second stage of labour were obtained. All sequences were adapted to tolerate movement of the mother and infant, as well as residual noise from the CTG. Furthermore, the MR imaging during labour showed only minor image artefacts. CONCLUSION: CTG-monitored acquisition of MRI series during the active second stage of delivery is feasible. Image quality should allow various further studies to improve models for birth simulation as well as potential investigation of obstructed labour and obstetric complications.

Impact of intrapartal ultrasound to assess fetal head position and station on the type of obstetrical interventions at full cervical dilatation.

OBJECTIVES: Recent ultrasound studies have shown that it is feasible to objectively and reproducibly assess fetal head position and station within the pelvis. We sought to evaluate the impact of this new approach on decision making by physicians in a cohort of women with a prolonged second stage of labor. METHODS: This was a retrospective cohort study that included all women with fetuses in cephalic presentation, who were diagnosed with a prolonged second stage of labor, and who delivered in a 1-year period. We compared a group of women (n = 121) with a prolonged second stage of labor who underwent intrapartal ultrasound prior to obstetrical intervention (Group A, n = 43) with a group of women for whom the delivery modus was decided upon after clinical digital examination alone (Group B, n = 78). RESULTS: There were no significant differences in maternal and neonatal morbidity between both groups. The rate of second-stage cesarean section was significantly higher (p < 0.50) in Group B without ultrasound compared to Group A with ultrasound prior to operative delivery (20/78 vs. 7/43). Seven patients in Group A delivered spontaneously, but none of the patients in Group B had spontaneous deliveries. CONCLUSIONS: Intrapartal ultrasound in patients with a prolonged second stage of labor may change obstetrical practice by reducing the number of second stage cesarean section without increasing maternal and neonatal morbidity.

Relationship between fetal head station established using an open magnetic resonance imaging scanner and the angle of progression determined by transperineal ultrasound.

OBJECTIVE: We investigated the correlation between the angle of progression measured by transperineal ultrasound and fetal head station measured by open magnetic resonance imaging (MRI), the gold standard, in pregnant women at full term. METHODS: Thirty-one pregnant women at full term with a fetus in the occipitoanterior position were enrolled. First, the distance between the leading part of the skull and the interspinal plane was obtained using an open MRI system with the patient in a supine position. Immediately after MRI, the angle of progression was obtained by transperineal ultrasound without changing the woman's posture. RESULTS: There was a significant correlation between the angle of progression determined by transperineal sonography and the distance between the presenting fetal part and the level of the maternal ischial spines (y = - 0.51x + 60.8, r(2) = 0.38, P < 0.001). None of the fetal heads was engaged at the time of MRI and ultrasound examinations. CONCLUSIONS: The present study demonstrated a predictable relationship between the angle of progression obtained by transperineal ultrasound and the traditional scale used to quantify fetal head descent. Based on our results, station 0 would correspond to a 120° angle of progression. However, this correlation is based on statistical assumptions only and has to be proven in future studies.

In utero development of symmetric thalamic and brainstem necrosis in a preterm hydropic stillborn.

Focal and symmetric necrotic lesions of the brainstem are thought to result from fetal hypotension or cardiac arrest in the perinatal period and thus occur in the course of postnatal intensive care rather than in utero. Here, we report for the first time on brainstem necrosis in a preterm stillborn demonstrating that brainstem necrosis occurs already in utero. The preterm stillborn of 28 weeks gestation of a mother that suffered from HELLP-syndrome was severely affected by a fetal hydrops with bilateral pleural effusions and lung hypoplasia. Bilateral tegmental brainstem necrosis and thalamic lesions were detected.

Measurement of fetal head descent using the 'angle of progression' on transperineal ultrasound imaging is reliable regardless of fetal head station or ultrasound expertise.

OBJECTIVES: To assess whether ultrasound experience or fetal head station affects the reliability of measurement of fetal head descent using the angle of progression on intrapartum ultrasound images obtained by a single experienced operator, and to determine reliability of measurements when images were acquired by different operators with variable ultrasound experience. METHODS: One experienced obstetrician performed 44 transperineal ultrasound examinations of women at term and in prolonged second stage of labor with the fetus in the occipitoanterior position. Three midwives without ultrasound experience, three obstetricians with < 5 years' experience and three obstetricians with > 10 years' experience measured fetal head descent based on the angle of progression in the images obtained. The angle of progression was measured by two obstetricians in independent ultrasound examinations of 24 laboring women at term with the fetus in the cephalic position to allow assessment of the reliability of image acquisition. Intraclass correlation coefficients (ICCs) with 95% confidence interval (CI) were used to evaluate interobserver reliability and Bland-Altman analysis was used to assess interobserver agreement. RESULTS: In total, 444 measurements were performed and compared. Interobserver reliability with respect to offline image analysis was substantial (overall ICC, 0.72; 95% CI, 0.63-0.81). ICCs were 0.82 (95% CI, 0.70-0.89), 0.81 (95% CI, 0.71-0.88) and 0.61 (95% CI, 0.43-074) for observers with > 10 years', < 5 years' and no ultrasound experience, respectively. There were no significant differences between ICCs among observer groups according to ultrasound experience. Fetal head station did not affect reliability. Bland-Altman analysis indicated reasonable agreement between measurements obtained by two different operators with > 10 years' and < 5 years' ultrasound experience (bias, -1.09 degrees ; 95% limits of agreement, -8.76 to 6.58). The reliability of measurement of the angle of progression following separate image acquisition by two experienced operators was similar to the reliability of offline image analysis (ICC, 0.86; 95% CI, 0.70-0.93). CONCLUSIONS: Measurement of the angle of progression on transperineal ultrasound imaging is reliable regardless of fetal head station or the clinician's level of ultrasound experience.

The effect of surgery on lung volume and conventional monitoring parameters in ventilated newborn infants.

In newborn infants, thoraco-abdominal surgery is a serious intervention with respect to gas exchange and lung mechanics. This prospective clinical study compared surgery-induced changes in functional residual capacity (FRC) and ventilation inhomogeneity (VI) indices with changes in conventional monitoring parameters. Of 29 ventilated newborns (mean weight 2,770+/-864 g at surgery), 13, nine and seven underwent thoracic, abdominal or congenital diaphragmatic hernia (CDH) surgery, respectively. The multiple breath washout (MBWO) technique using heptafluoropropane as tracer gas (Babylog 8000; Dräger, Lübeck, Germany) was performed <6 h before surgery, 22-24 h after surgery and <6 h before extubation. Gas exchange, respiratory mechanics, FRC and VI index data were recorded. Thoraco-abdominal surgery resulted in changes to FRC and VI indices in a procedure-specific manner; however, these changes were not reflected in conventional mechanical or ventilatory monitoring parameters. FRC decreased in non-CDH infants, while FRC increased and VI indices decreased in CDH infants. Despite improvements, the differences in FRC and VI between CDH and non-CDH infants indicated persistent impaired lung function in CHD infants. MBWO can be advantageously used to measure the effect of surgery on the lung. While FRC and VI indices changed following surgery, conventional monitoring parameters did not.

[Cutaneous xanthomas associated with minocycline-induced cholestatic jaundice]. / Xanthomatose der Haut bei cholestatischem Ikterus nach Minocyclin-Langzeittherapie.

Minocycline is an effective treatment of acne vulgaris, especially for inflammatory forms. Prescription rates have increased in recent years accompanied by a number of reports concerning drug-induced side effects. An otherwise healthy woman developed an erythema multiform-like rash and and toxic hepatic damage causing cholestatic jaundice following long-term minocycline use. Unusual cutaneous lipid deposition also developed. Minocycline-induced side effects are reviewed.

Late-onset encephalopathy associated with a C11777A mutation of mitochondrial DNA.

A 67-year-old man presented with cognitive deficits, status epilepticus, left hemiparesis, and severe lactic acidosis. Respiratory chain enzyme analysis of skeletal muscle revealed a defect in complex I activity, associated with a heteroplasmic C11777A mutation in the mitochondrial ND4 gene. This case is remarkable not only because of the late onset of symptoms, but because this mutation affects the identical ND4 codon as the G11778A mutation that causes Leber hereditary optic neuropathy.

Hashimoto's encephalitis as a differential diagnosis of Creutzfeldt-Jakob disease.

OBJECTIVES: During an epidemiological study of Creutzfeldt-Jakob disease in Germany, Hashimoto's encephalitis was encountered as a differential diagnosis, which has not yet been described in this context. METHODS: The symptoms and findings of seven patients who fulfilled the criteria for "possible" Creutzfeldt-Jakob disease are presented. RESULTS: A Hashimoto's thyroiditis with antibodies against thyroglobulin or thyroid peroxidase, or both and a hypoechoic thyroid ultrasonogram were found in all cases. Analysis of CSF disclosed an increased leucocyte count in three patients, and a raised CSF:serum concentration ratio of albumin (QA1b) in four patients. The 14-3-3 protein, typical of Creutzfeldt-Jakob disease, could not be detected in any of our patients. No periodic sharp wave complexes, which are typical of Creutzfeldt-Jakob disease, were detected on EEG in any of the cases. By contrast with Creutzfeldt-Jakob disease, which leads to death within a few months, the patients with Hashimoto's encephalitis often recover quickly when treated adequately. All the patients improved after administration of corticosteroids. CONCLUSION: The clinical symptomatology of both diseases may be very similar: dementia, myoclonus, ataxia, and personality change or psychotic phenomena are characteristic symptoms.

[Long-term treatment with 7S immunoglobulins in myasthenia gravis. Preliminary clinical results]. / Zur Langzeitbehandlung mit 7S-Immunglobulinen bei Myasthenia gravis. Vorläufige klinische Ergebnisse.

After initial high-dose intravenous 7S immunoglobulin therapy, six patients with seropositive myasthenia gravis received intermittent low-dose 7S immunoglobulins for at least 4-12 months. This treatment was started in five cases following an acute exacerbation of myasthenic symptoms (Oosterhuis class 3-4) and in one case because of marked clinical fluctuations (Oosterhuis class 3). In five of the six patients, there was a clinical response to the immunoglobulin therapy within 2 weeks, followed by marked long-standing improvement and stability of the clinical outcome. In four cases a decrease in the titer of acetylcholine receptor antibodies was noted in parallel. Our observations suggest an additional positive therapeutic effect of long-term, low-dose intravenous immunoglobulin therapy following the acute management of myasthenic exacerbations.