Three pregnancies in a Marfan syndrome patient after a mitral and tricuspid valve surgery

Marfan syndrome is an autosomal dominant disorder of connective tissue with up to 25% of cases related to a spontaneous mutation. It has been associated with perinatal loss, preterm labor, and, potentially, a rupture of the maternal aortic arch. We present a case of a woman diagnosed with Marfan syndrome after a miscarriage of her first pregnancy. At the time of diagnosis she had mild aortic bulb dilation and insufficiency of the mitral and tricuspid valves. She underwent cardiosurgical correction, after which she had two uneventful pregnancies. This case suggests that preconceptional correction of valve defects in women with Marfan syndrome may decrease the risk of cardiac decompensation during future pregnancies. Additionally, close clinical follow up and the appropriate use of beta-adrenergic blockade may decrease the risk of aortic rupture, a significant risk factor for mortality in pregnant women

Current views on fetal surgical treatment of myelomeningocele — the Management of Myelomeningocele Study (MOMS) trial and Polish clinical experience

Abstract Myelomeningocele (MMC) is the most frequent congenital defect of the central nervous system for which there is no satisfactory alternative to postnatal treatment. On the contrary prenatal MMC surgery is conducting before birth and is aimed at protecting from Chiari II malformation. The main goal of fetal MMC repair is to improve development and life quality of children with Chiari II malformation. Management of Myelomeningocele Study (MOMS) which was published in 2011 clearly confirmed effectiveness of prenatal surgery. In this paper we compare MOMS results with our own clinical experience. Thanks to high effectiveness and significant improvement in safety of maternal-fetal surgery prenatal MMC surgery become a new standard of treatment.  Myelomeningocele (MMC) constitutes the most common congenital defect of the central nervous system, with no satisfactory alternative to the postnatal treatment. Prenatal repair of MMC is aimed at protecting from a Chiari type II malformation. The main goal of fetal MMC repair is to improve the development and quality of life in children with a Chiari type II malformation. The Management of Myelomeningocele Study (MOMS), which was published in 2011, confirmed the effectiveness of prenatal surgery. In this paper, we compared the MOMS results with our own clinical experience. Owing to high effectiveness and significant improvement in the safety of the maternal-fetal surgery, prenatal MMC repair has become a new standard of treatment

Open fetal surgery for myelomeningocele — is there the learning curve at reduction mother and fetal morbidity?

Objectives: We aimed to show how increased experience of a surgery team in fMMC repair influences maternal andfetal/neonatal outcomes.Material and methods: We compare perinatal results of fMMC repair in our Fetal Surgery Center (FSC) in cohort groups forthe early period (2005–2011 year; previous — PFSC, n = 46) and current period (2012–2015 year; current — CFSC, n = 74)to results of the randomized Management of Myelomeningocele Study (MOMS, 78 patients).Results: The maternal morbidity due to fMMC repair was low and there was no difference comparing CFSC to PFSC andMOMS. The frequency of iatrogenic preterm labor (iPTL) ≤ 30 weeks of gestation decreased from 34.1% in PFSC to 23.9%in CFSC. Iatrogenic preterm premature rupture of membranes (iPPROM) was a common complication after fMMC repair inall cohorts. The total reduction rate of hindbrain hernation (HH) was similar in CFSC — 90.3% and PFSC — 82.1%.Conclusions: The increasing experience of our surgery team in fMMC repair majorly decreased the risk of iPTL

Alternative technique of intrauterine myelomeningocele repair to decrease the incidence of unfavorable maternal and fetal outcomes

Objectives: The aim of the study was to determine the effectiveness of an alternative method of open fetal surgery to prevent severe unfavorable prenatal events, both for the mother and the fetus. Material and methods: In this study, the previously published results for a cohort of 46 patients, who had undergone intrauterine myelomeningocele repair (IUMR) at our Center by 2014, constituted the retrospective control group (CG). The MOMS protocol had been applied for hysterotomy, with an automatic uterine stapling device. The study group (SG) n = 57 was assembled during a prospective observation. IUMR was performed using an alternative method of hysterotomy, with the typical opening and closure of the uterus, without automatic stapling device, as described by Moron et al. Additionally, our single-center results were compared with the post-MOMS findings of other centers: CHOP (Children’s Hospital of Philadelphia) and VUMC (Vanderbilt University Medical Center). Results: No cases of delivery before 30 weeks of gestation (0%, 0/55) were observed in the study group, which is a statistically significant difference (p < 0.05) as compared to controls (15/44). Statistically significantly lower incidence of chorioamniotic separation (5.4% (3/55) vs CHOP 22.9% (22/96), p < 0.001) and contractile activity resulting in preterm labor (16.3% (9/55) vs CHOP 37.5% (36/96), p < 0.05) was found in the study group. Premature rupture of the membranes was statistically significantly less common in the study group as compared to controls, CHOP and VUMC (SG 12.7% (7/55) vs CG 52.2% (24/46), p < 0.001; vs CHOP 32.3% (31/96), p < 0.001; vs VUMC 22% (9/43), p < 0.01, respectively). Conclusions: The presented IUMR method is associated with improved perinatal outcomes, i.e., lower rates of preterm delivery at < 30 weeks of gestation, preterm premature rupture of membranes, and uterine contractility resulting in preterm delivery. That, in turn, results in lower prematurity rates and, consequently, more favorable neonatal outcomes

Venous thromboembolism as an essential clinical problem in the group of patients with gynecologic cancer

Malignancies are a high and a very high risk factor for thrombotic complications. The risk of venous thromboembolism is 4–7 fold increased in patients with cancer, and is particularly high in the first months after diagnosis. Venous thromboembolism most often occurs as deep vein thrombosis and pulmonary embolism. It is the most common complication and, at the same time, the second leading cause of death in cancer patients, after cancer itself. At present, low molecular weight heparins are recommended for both, prophylaxis and treatment of venous thromboembolism. Pharmacological antithrombotic prophylaxis is recommended for cancer patients undergoing abdominal or pelvic surgical procedures, and should be extended to at least 4 weeks after the procedure. Prophylaxis of up to 6 months or even for an indefinite period of time can be considered. Risk assessment for thromboembolic complications as well as the inclusion of appropriate antithrombotic prevention/treatment are essential components of care for cancer patients. At the same time, there is a need to educate cancer patients on their increased risk of venous thromboembolism. The growing awareness of patients will help them accept medications they use.Nowotwory złośliwe stanowią czynnik dużego i bardzo dużego ryzyka powikłań zakrzepowych. Ryzyko zakrzepicy żylnej u pacjentów z chorobą nowotworową wzrasta 4–7-krotnie i jest ono szczególnie wysokie w pierwszych miesiącach po ustaleniu rozpoznania. Żylna choroba zakrzepowo-zatorowa najczęściej występuje w postaci zakrzepicy żył głębokich oraz zatoru tętnicy płucnej. Stanowi najczęstsze powikłanie, a jednocześnie jest drugą po nowotworze przyczyną śmierci pacjentów onkologicznych. Obecnie stosowanie heparyn drobnocząsteczkowych jest postępowaniem rekomendowanym zarówno w profilaktyce, jak i leczeniu żylnej choroby zakrzepowo-zatorowej. Farmakologiczną profilaktykę przeciwzakrzepową rekomenduje się w przypadku pacjentów onkologicznych poddawanych operacjom w obrębie jamy brzusznej lub miednicy mniejszej, a jej stosowanie powinno zostać wydłużone do co najmniej 4 tygodni po zabiegu, z rozważeniem kontynuacji do 6 miesięcy, a nawet bezterminowo. Ocena ryzyka wystąpienia powikłań zakrzepowo-zatorowych oraz włączenie odpowiedniej profilaktyki/leczenia przeciwzakrzepowego jest nieodzownym elementem opieki nad pacjentami onkologicznymi. Jednocześnie istnieje potrzeba edukacji pacjentek z chorobą nowotworową w zakresie podwyższonego ryzyka wystąpienia u nich żylnej choroby zakrzepowo-zatorowej. Rosnąca świadomość pacjentek przyczyni się do łatwiejszej akceptacji stosowanych leków

Absence of a Hernia Sack in Patients Undergoing Prenatal Repair of Spina Bifida Increases the Risk of Developing Shunt-Dependent Hydrocephalus

Spina bifida aperta (SBA), with (myelomeningocele) or without (myeloschisis) a hernia sack, is the most common congenital defect of the central nervous system. Prenatal surgical closure of SBA lowers the risk for developing shunt-dependent hydrocephalus, which offers a chance at improved motor, urinary, and gastrointestinal function. A total of 96 patients who had undergone open surgery prenatal repair for SBA were analyzed. The patients were divided into two groups: Group I—12 patients (12.5%)—without a hernia sack (myeloschisis) and Group II—84 patients (87.5%)—with a hernia sack (myelomeningocele). In this study, we demonstrated that prenatal SBA repair was statistically significantly less often associated with the need for ventriculoperitoneal shunting (p > 0.00001). The shunting was statistically significantly more often required in patients from Group I (p > 0.004). The absence of a hernia sack increases the risk for developing shunt-dependent hydrocephalus in patients after prenatal SBA repair. However, as prenatal SBA repair is associated with better motor, urinary, and gastrointestinal function, increased risk of developing shunt-dependent hydrocephalus in fetuses without a hernia sack should not be treated as a contraindication to prenatal intervention