Volume 6, Issue 2

Genital herpes in pregnancy is associated with increased perinatal morbidity and mortality. Maternal primary infection with herpes simplex virus (HSV) prior to labor usually does not affect the fetus (1/200,000 deliveries) The greatest risk associated with intrapartum HSV exposure is neonatal herpes infection. Neonatal risk occurs in the case of a primary or reccurent HSV infection. The risk of neonatal HSV infection in case of non-genital herpes is low. The American Congress of Obstetricians and Gynecologists (ACOG) recommends elective cesarean delivery for women with demonstrable genital herpes or prodromal symptoms in labor.

Introduction Postpartum or peripartum hemorrhage (PPH) is a major cause of maternal death in Western industrialized countries. Fertility preserving second stage interventions following uterotonic drugs include embolization or ligation of relevant arteries, uterine tamponade or compression sutures. Little is known about the complications due to uterine compression sutures. We describe a case report in association with uterine compression sutures and provide a systematic review on necrosis due to compression sutures (CSU). Data sources A PubMed database search was done up to October 1, 2016 without any restrictions of publication date or journal, using the following key words: “compression suture” and “postpartum hemorrhage” or “peripartum hemorrhage”. Reported cases were considered eligible when reason for postpartum hemorrhage (PPH), type of compression suture, suture material and type of complication were described. Results Among 199 publications a total of 11 reported on uterus necrosis after CSU applied for PPH. B-Lynch and modifications were applied in seven cases, Cho compression sutures in three cases and in one case B-Lynch and Cho techniques were combined. In six cases no additional measures were applied, in two cases vessel ligation, in one case embolization and in one case intrauterine balloon application were applied. In one case of partial necrosis it is not reported if additional measures were applied. Discussion and conclusion Uterine compression sutures are a useful method for fertility preserving management of postpartum hemorrhage. The risk of serious complications demands the careful consideration of its use. More research is necessary to improve the technique.

Porphyrias are very rare and easily overlooked diseases in pregnancy. Among these eight known metabolic disorders, acute intermittent porphyria (AIP) is the most common and most severe type. An enzymatic alteration in the haem biosynthesis pathway causes liver overproduction of neurotoxic toxic metabolites, which cause attacks of acute neurovisceral symptoms, severe abdominal pain and/or skin lesions. Women with AIP can sometimes develop their first acute attack during pregnancy, and because the symptoms are unspecific, the diagnosis is difficult to obtain and often missed. However, prompt diagnosis of AIP during pregnancy is crucial as treatment can significantly improve the pregnancy outcome. The backbone of the therapy is food rich with carbohydrates, complemented by 20% glucose infusion and adequate pain control. We present a case of previously undiagnosed AIP in a 34-year primigravida, whose treatment was especially challenging due to co-existing gestational diabetes mellitus and problems with the central venous catheter.

Background Cardiac disease is the leading cause of maternal mortality during pregnancy in high-resource countries. Case A 31-year-old woman had an ST-elevated myocardial infarction (STEMI) at 16 gestational weeks. The patient received three coronary drug-eluting stents followed by dual antiplatelet therapy (DAPT) with aspirin and clopidogrel. An elective caesarean delivery was performed under general anesthesia at 37+1 gestational weeks. Due to subnormal response to clopidogrel, administration was paused only 24 h perioperatively without bridging with tirofiban because of the slightly increased risk of stent thrombosis in clopidogrel nonresponders. There was no postoperative bleeding. Conclusions There is a lack of evidence-based guidelines regarding the management of acute myocardial infarction (AMI) during pregnancy; thus, delivery should be performed in a tertiary center with a multidisciplinary approach.

Cervical ectopic pregnancy is defined as implantation of the embryo in the endocervical canal below the level of the internal os. It is a rare condition that accounts for 0.15% of all ectopic pregnancies. As it can lead to complications such as uncontrolled hemorrhage, it is associated with high morbidity and mortality. This paper illustrates a rare case of a 2 nd trimester cervical pregnancy with failed conservative management due to the limitations of ultrasonography. Successful conservative management requires not only early diagnosis but also usage of other sophisticated techniques such as magnetic resonance imaging (MRI) that can help in detecting adherent placenta.

A 35-year-old woman, gravida 3, para 2, spontaneously delivered an infant without any major complications. On the 38 th day after delivery, she returned to the hospital due to irregular bleeding. Transvaginal ultrasound showed a mass in the cervix; therefore, dilatation and curettage was performed, using placental forceps, to remove the retained placenta. During the procedure, a uterine perforation was suspected. Sonohysterography was performed in order to confirm the uterine perforation. The sonohysterogram revealed that the high echogenic mass that was suspected to be retained placenta was adhered on the posterior uterine myometrium. Saline that had been injected into the uterine cavity escaped into the Douglas pouch via a small hole in the posterior uterine wall. An emergency laparotomy was performed. Pathological examination of the removed uterus revealed placenta increta in the posterior wall, as well as an adjacent perforated fistula. Sonohysterographic diagnosis of uterine perforation in the present case was not only validated with diagnosis, but also the residual placenta was clearly visible. The use of sonohysterography for detection of a suspected case of uterine perforation after dilatation and curettage was accurate and provided a safe procedure for fast evaluation.

We present the use of epidural anesthesia for the management of transfusion associated circulatory overload. After a vaginal delivery, a patient with preeclampsia and postpartum hemorrhage developed respiratory failure following rapid transfusion and fluid resuscitation. Her dyspnea and clinical status markedly improved after receiving an epidural bolus of 2-chlroprocaine. This unusual application of epidural anesthesia induces similar physiological changes to standard medical therapy for pulmonary edema and volume overload.

Allantoic cysts of the umbilical cord are embryological remnants of either the allantois or the omphalomesenteric duct. These cysts represent a rare ultrasound finding especially in the first trimester. Allantoic cysts can be associated with fetal malformations and chromosomal aberrations or are characterised by spontaneous resolution and good pregnancy outcome. Here we present a rare case of umbilical cord cyst detected by ultrasound in the first trimester with later resolution and favourable pregnancy outcome.

Background Thrombotic microangiopathy (TMA) is one of the most difficult thrombotic complications, characterized by damage of microvessels of various organs and accompanied by thrombocytopenia and hemolytic anemia. Today, TMA includes thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome, heparin-induced thrombocytopenia and hemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome. A key characteristic in the development of TMA is endothelial dysfunction. Recent discoveries suggest that this pathology is more widespread than it was previously. One of the most important triggers for TMA is pregnancy. Methods The clinical observation of a patient with an inhibitory form of a disintegrin and metalloproteinase with a thrombospondrin type 1 motif, member 13 (ADAMTS-13) deficiency, recurrent thrombotic and “typical obstetric” severe complications is presented. Results We diagnosed a reduction in the activity of ADAMTS-13, an increase in the level of antibodies to ADAMTS-13 and disturbances in functioning of the protein C system along with symptoms of disorders of blood flow in the mother-placenta-fetus system as a poor prognosis in regard to both thrombotic and placenta-mediated combinations. Conclusions An analysis of cases such as this patient immediately pushes the clinician to the idea of antiphospholipid syndrome (APS). It is questionable whether this patient could be regarded as an APS patient if her symptoms do not correspond with the classic diagnostic criteria for the syndrome. At the same time, the discovery of molecular mechanisms of TMA warrants a fresh look into the pathogenesis of thrombotic complications associated with pregnancy, as well as the pathogenesis of placental obstetric complications, including severe forms of preeclampsia, premature detachment of normally situated placenta and septic shock.

Among the three delays that play a pivotal role in increasing maternal mortality, delay in transportation is a major challenge for the uptake of services for pregnant women in remote areas of Nepal. Due to difficult terrain and the lack of adequate public transportation, women face difficulties in reaching health facilities and end up giving birth on the way unsafely. Here, I describe the struggle of a pregnant woman who started her journey to deliver at health facility, but never reached it. She remained helpless in the middle of the highway, where our team intervened and conducted her delivery right over there.

Introduction Benign cardiac rhabdomyomas are the most common cardiac tumors in fetuses and children. They are most often located in the ventricles and may disturb myocardial function, the severity correlating with location and size of the tumor. Rhabdomyomas are commonly associated with the tuberous sclerosis complex (TSC) and are the first clinical manifestation in 50–80% of the cases [Isaacs H Jr. Fetal and neonatal cardiac tumors. Pediatr Cardiol. 2004;25:252–73, Colosi E, Russo C, Macaluso G, Musone R, Catalano C. Sonographic diagnosis of fetal cardiac rhabdomyomas and cerebral tubers: a case report of prenatal tuberous sclerosis. J Prenat Med. 2013;7:51–5]. Several authors have documented the sensitivity of TSC-associated rhabdomyomas to everolimus treatment [Hoshal SG, Samuel BP, Schneider JR, Mammen L, Vettukattil JJ. Regression of massive cardiac rhabdomyoma on everolimus therapy. Pediatr Int. 2016;58:397–9, Mlczoch E, Hanslik A, Luckner D, Kitzmüller E, Prayer D, Michel-Behnke I. Prenatal diagnosis of giant cardiac rhabdomyoma in tuberous sclerosis complex: a new therapeutic option with everolimus. Ultrasound Obstet Gynecol. 2015;45:618–21, Tiberio D, Franz DN, Phillips JR. Regression of a cardiac rhabdomyoma in a patient receiving everolimus. Pediatrics. 2011;127:e1335–7]. The present study provides convincing evidence of successful everolimus therapy in a newborn without the TSC complex. Case presentation A cardiac rhabdomyoma measuring 35 × 28 × 24 mm was seen in a fetus in pre- and postnatal echocardiography. There was no family history for TSC and amniocentesis showed no mutations in the TSC1 / TSC2 genes. Off-label treatment with everolimus began when the neonate was 11 days old and was discontinued when the infant was 11 months old after echocardiography showed marked regression of tumor size and improvement of the tricuspid valve insufficiency. Echocardiography 3 months later showed an increase in size to 13.2 × 9 mm, so that everolimus therapy was re-instated. The next echocardiography, 10 weeks later, showed renewed regression of tumor size and a residual moderate tricuspid valve insufficiency under everolimus therapy. Discussion The present report of a rhabdomyoma in a newborn without an association with TSC is of interest because it identifies a treatment effect of everolimus. A medical approach in patients with cardiac decompensation due to intracardiac rhabdomyomas offers an attractive alternative to surgery.

Introduction Iniencephaly is characterized by a defect in the occipital bone in the inion, occipital encephalocele, spinal dysraphism and fixed hyperextension of the fetal head. The associated anomalies are observed in many systems including the central nervous system and the gastrointestinal system. Prenatal diagnosis is rare because early and careful ultrasonographic evaluation is necessary. Fetuses with iniencephaly are almost always stillborn. We aimed to present a fetus with iniencephaly reaching term in utero . Case A 25-year-old healthy primigravida Syrian woman consulted with labor pain at 39 weeks of gestation. She had no laboratory tests or ultrasonographic examination during the pregnancy. Ultrasound evaluation demonstrated a single live intrauterine fetus with a retroflexed fetal head and excess amniotic fluid. A girl was delivered by cesarean section weighing 3350 g with a length of 48 cm. The fetal head was retroflexed with an occipital bone defect. Associated anomalies such as club foot were also observed (Figures 1 and 2). Discussion Iniencephaly is an extremely rare neural tube defect first described by Saint-Hilare in 1836. Its incidence varies from 0.1 to 10 : 10,000. The incidence is higher in families with a history of neural tube defect. Maternal drug use, smoking, alcohol use, low parity and low socioeconomic status are risk factors. Prenatal diagnosis is possible by careful and early ultrasonographic examination. Termination of pregnancy is the generally accepted approach in these pregnancies because of poor prognosis and severe mortality. The most important precaution is to prevent obstructed labor and maternal trauma due to corporal distortion of the fetus.

Acephalous fetus in a singleton pregnancy is an extremely rare case. In twin pregnancy, it could be presumed as one type of twin reverse arterial perfusion sequence (TRAPS). In this particular case report, the situation was different. An acephalous fetus developed in a singleton pregnancy and may have been a complication of an amniotic band in the very early weeks of gestation. Nevertheless proving it is still a constraint. Despite that, motor findings in utero by using four-dimensional (4D) ultrasound were very interesting to study. Movement of the acephalous fetus is challenging thought on fetal behavior theory, as brain development and function play the central role. The Kurjak antenatal neurodevelopmental tests (KANET) was used to measure the fetal behavior of this acephalous fetus. A comparison with post natal movement findings was also done to provide a better understanding.

Aim To propose a diagnostic algorithm for non-immune fetal hydrops (NIFH) of metabolic origin based on a review of the literature and on the workup of a clinical case. Background The etiology of NIFH is complex and remains unexplained in 15%–25% of patients. The appropriate work up beyond an initial approach is still not well defined but it should include screening for metabolic conditions. Inborn errors of metabolism comprise a heterogeneous group of autosomal recessive rare inherited disorders, among which lysosomal storage disorder is the most common subtype. Case description We report a case of a 30-year-old pregnant, primiparous woman, referred to a tertiary hospital at 22 weeks of gestation because of a fetal hydrops. The second trimester obstetric ultrasound showed a hydrothorax and a large subcutaneous edema. At 30 weeks of gestation, the fetal health status deteriorated and a massive hepatomegaly was detected. The metabolic study of the amniotic fluid supernatant suggested a lysosomal disease. The ominous prognosis of the condition motivated the parents to opt for a termination of pregnancy. The autopsy study confirmed the existence of a metabolic disease. Conclusion The incidence of inborn errors of metabolism may be significantly higher in NIHF than reported previously. Consequently, an extensive investigation for the etiology of NIHF including the screening for metabolic disorders seems to be crucial for a definitive diagnosis. Clinical relevance Despite the lack of treatment options for the majority of these disorders, it is of great importance to follow an established workup, in order to identify the index case as soon as possible, as pregnancy management decisions and prenatal counselling in future pregnancies will depend on a more precise diagnosis.

Monochorionic (MC) twin pregnancy is a distinct entity and has certain differences from dichorionic twins due to the unique placental angioarchitecture. It is characterized by twin-to-twin transfusion syndromes (twin oligohydramnios-polyhydramnios sequence and twin anemia-polycythemia sequence), acardiac twinning, selective fetal growth restriction and congenital anomalies. Selective termination is an option in MC twins complicated by selective fetal growth restriction and discordant fetal anomaly. Fetal demise of the co-twin can occur even after uncomplicated surgery. A selective fetal termination using an intrafetal laser was performed in the case of an MC twin pregnancy complicated by twin oligohydramnios-polyhydramnios sequence and hydrocephalus in the donor twin. Fetal demise of the co-twin was observed after surgery. The placenta was examined with dye injections after abortion and showed vascular anastomoses causing unexpected fetal demise.

Acute mastoiditis (AM) is rarely seen in newborns. It is characterized by retroauricular pain, swelling, tenderness and protrusion of the auricle. This is the first report of the neonatal mastoiditis in a 17-day-old term neonate with no obvious clinical manifestation of mastoiditis and no associated malformation of the ears and mastoids. A computed tomography (CT) scan of the temporal bones revealed right mastoiditis without osteitis, destruction of the mastoid bone or abscess formation. Discharge culture revealed streptococcus A colonies sensitive to ampicillin, ceftriaxone, vancomycin and chloramphenicol. She was successfully treated with intravenous ampicillin and ceftizoxime. No complication or recurrence was reported.

Hepatic hemangioendothelioma is a rare and benign vascular tumor, usually asymptomatic at birth. However, it can present with severe cardio-respiratory symptoms and consumptive coagulopathy. Here we present a severe case of hepatic hemangioendothelioma presenting as hydrops fetalis, suggesting that it can have significant prenatal and postnatal morbidity.

Respiratory distress in the premature neonate has a variety of causes, some more common than others. Sometimes it may not be apparent that rare underlying conditions may be associated with common clinical presentations. In this case report, we describe an extremely premature neonate presenting with severe respiratory distress who was subsequently diagnosed with both myotonic dystrophy and Turner syndrome (monosomy X; 45, X0). A literature review correlating this neonate’s particular presentation with these diagnoses has been included.