Volume 3, Issue 2

Pulmonary arteriovenous malformations in a pregnant patient are rare and can cause deleterious, life-threatening complications. We report a patient with multiple pulmonary arteriovenous malformations, with the subsequent diagnosis of hereditary hemorrhagic telangiectasia, requiring multiple embolizations during pregnancy. Pulmonary arteriovenous malformations can carry a high risk of morbidity in the pregnant woman; however, they can be safely treated in pregnancy.

Background: Vasa previa is a condition fraught with severe morbidity when undiagnosed at time of delivery. Case: A 25-year-old G4P2012 presented with preterm labor at 27 weeks. She was fully dilated and with the first push two independent vessels were coursing over the membranes; vasa previa was diagnosed. The patient pushed through two contractions to deliver a vigorous fetus en caul. The neonate did well, not requiring any blood transfusions during its neonatal stay for prematurity, and was seen for a well-child examination 4 months later, doing well. Conclusion: The mode of delivery for a known vasa previa is a planned Cesarean section (C-section), however if vasa previa is encountered in the labor room with quick progression of the second stage an en caul delivery could be considered.

Severe bleeding from placenta previa usually leads to immediate delivery. We report a case of a 23 weeks twin pregnancy who presented profuse bleeding while she was in the operating room for cervical cerclage. Cervical compression with a pack was applied in an attempt to gain time whilst preparing for cesarean section. The attempt was successful in halting bleeding and helped to extend the pregnancy until 33 weeks. We did not find a similar case in the literature and our conclusion was that in carefully selected cases, vaginal packing could be an immediate option to stop bleeding in placenta previa.

Amniotic fluid embolism (AFE) is an obstetrical catastrophe characterized by acute hypotension, hypoxia, and coagulopathy occurring during labor, cesarean section, post-delivery, or during amniocentesis. Thromboelastography (TEG) is a bedside test of global homeostasis and has seldom been used to study coagulation in AFE despite increasing popularity in other diseases. We report TEG usage as part of a treatment for patients with AFE.

A woman carrying monochorionic twins underwent sudden cardiopulmonary arrest at 31 weeks of gestation due to spontaneous coronary artery dissection in the hospital. The resuscitation techniques, with her uterus manually displaced to her left, were initiated immediately and maternal spontaneous circulation was resumed 18 min later. A cesarean section was performed 2 h after the revival and the patient was kept hypothermic for 24 h. Finally, full recovery of both mother and twins was achieved. As in this case, the resuscitation techniques for pregnant women recommended by the American Heart Association, and therapeutic hypothermia, might be effective and contribute much to the survival and recovery of patients.

Background: Large uterine fibroids and placenta percreta are often associated with a hysterectomy. The case: A 31-year-old nulligravida had a transcervical resection of a large fibroid (158 g) in the anterior wall of the uterus. A subsequent spontaneous pregnancy was complicated by placenta percreta. In late pregnancy, she had an elective cesarean section, the percrete area was resected, and she had an abdominal cerclage for hemostasis and support. Subsequently, she had two term pregnancies with normal placentation. Conclusion: The woman avoided hysterectomy twice, first for gynecological and second for obstetrical reasons – and had three healthy infants. We conclude that the uterus should be preserved whenever possible in young fertile women.

We present a case in which a 28-year-old pregnant woman (para 1) was suspected to have inherited factor VII (FVII) deficiency following investigation for recurrent mild easy bruising. However, this was ruled out by identification of a rare FVII variant not associated with an increased bleeding risk. This allowed usual obstetric care to proceed without any restrictions, facilitating an uncomplicated spontaneous vaginal delivery at term.

Objective: To assess the effectiveness of using an 18-gauge needle inserted through the perineal surface of the fetus to solve magacystis and posterior urethral valve (PUV). Methods: From February 2008 to January 2012, 15 cases of magacystis were referred to our tertiary referral center, and in ten, PUV was suspected. In the cases where PUV was suspected after a detailed ultrasound scan, women gave consent to proceed with fetal therapy under sonographic guidance using an 18-gauge needle. The procedures lasted for a median time of 30 min (range 15–50 min). The needle was inserted through the perineal surface of the fetus to perforate the dilated posterior ureteral valve, as well as to perform a perineal vescicocentesis. Results: Median gestational age at time of referral was 17 weeks of gestation (range 16–19 weeks). Five fetuses were female and ten male. Nine cases opted for pregnancy termination, as the kidneys were dysplastic showing hyperechoic structure, while six cases were treated, as the kidneys showed a normal functionality as well as cortico-medullar differentiation. Five fetuses were male and one female. After fetal therapy, there were no stillbirths. All fetuses were delivered at term and the infants were followed up by routine kidney ultrasonography and functionality after a median follow-up of 16 months. Conclusions: Despite the most commonly used fetal therapy being vesico-amiotic shunting, percutaneous fetal cystoscopy that allows laser valve ablation and mechanical disruption was the procedure used in the present study. This allowed the direct treatment of the urethral obstruction that caused megacystis, without need to repeat the procedure and without the need of any postnatal therapy.

Owing to the improvement in obstetric ultrasound imaging, prenatal diagnosis of ovarian masses has increased considerably. Fetal ovarian cysts can be suspected when an ultrasound scan shows intra-abdominal structures in female fetuses in the presence of normal bowel and urinary structures. The most common complication is the adnexal torsion, causing partial or complete strangulation of blood supply via ovarian vessels, leading ovarian ischemia, or necrosis. Current information regarding the treatment of fetal ovarian cysts is based on personal experiences and some case series. The management is controversial, characterized by dissimilar approaches, such as “wait and see”, prenatal or postnatal aspiration, or neonatal surgery. In more than half of the cases, spontaneous regression occurs in the prenatal or postnatal period, probably due to the small size and simple aspect. Large cysts may cause both local effects (adnexal torsion, ovarian autoamputation), and distant effects (intestinal and urinary obstruction, adhesion with adjacent organs, abdominal and thoracic mass effect, pulmonary hypoplasia, hemoperitoneum, ascites, polyhyramniosis). In the absence of accurate guidelines for management, we must start with the development of more accurate methods for diagnosing associated complications such as torsion. This case report describes the role of three-dimensional (3-D) ultrasonography as potential diagnostic method for ruling out adnexal torsion when an ovarian cyst is present.

Congenital chylous ascites is a rare condition. We describe a case in which fetal ascites was found on a routine antenatal ultrasound, with all abnormalities resolving by 36 weeks’ gestation. No investigations or treatment for the ascites was undertaken after the baby´s birth. At the age of 3 months, when the baby underwent surgery for bilateral inguinal hernias and hydrocele, milky fluid was drained from the hernia sac. Laboratory and ultrasound investigations confirmed the presence of a significant amount of chyle in the peritoneal cavity. Congenital chylous ascites was treated by means of a diet based on medium chain triglycerides. By the age of 6 months, the fluid in the abdomen had resolved. However, it partly reappeared once a normal mixed diet was allowed. A minimal amount of fluid in the lower abdominal cavity was consistently found up to the age of 22 months. The fluid had finally disappeared by the age of 30 months when the child was generally in good health and developing normally. We conclude that congenital chylous ascites may be diagnosed prior to birth but may become more clinically significant only after birth, when its severity is enhanced by feedings of milk containing long chain fats. We recommend a thorough postnatal evaluation and follow-up in all infants diagnosed with fetal ascites, so that appropriate and timely management decisions can be made, if the chylous origin of the ascites is confirmed.

Extreme Doppler abnormalities of the umbilical artery such as absent or reversed end diastolic velocity are associated with adverse perinatal outcomes. We present a case of a triphasic umbilical artery waveform identified at 24 weeks. The fetus was severely growth restricted with an estimated fetal weight of 314 g. A week later, fetal demise occurred. Placental pathology revealed a placental weight of 83 g, an extensive maternal floor infarction, and an extreme velamentous cord insertion 7 cm from the edge of the placental disc, with vessels entering at opposite poles of the placental disc and a single anastomotic bridging vessel on the chorionic plate connecting these two vascular poles. A triphasic umbilical artery waveform may be associated with a premorbid state and severe placental vascular abnormality. We hypothesize that the third and positive component in late diastole is present due to forward flow across the communicating bridging vessel into the contralateral entering vessel.

Radiofrequency ablation (RFA) has become widely accepted as first-line management for twin reversed arterial perfusion (TRAP) sequence. Most RFA procedures have been performed using RFA needles of 14–17 gauge (G) focusing on an acardiac mass at an average age of 21 weeks of gestation (17–24 weeks). In this case report, we describe treatment of TRAP sequence using RFA with a 20 G needle focusing a feeding artery on the placental surface at gestational age of 11+6.

Diastrophic dysplasia is a rare genetic disorder characterized by short limbs and deformities of several joints occurring in conjunction with xyphoscoliosis, distinctive abduction of the first metacarpals (hitchhiker thumbs). A 28-year-old pregnant patient was referred due to detection of increased nuchal translucency at the first-trimester scan. We describe a case of diastrophic dysplasia diagnosed by two- and three-dimensional ultrasound and termination of pregnancy at 13 weeks of gestation. This case is the first report in the literature in which 3D ultrasound was used in diagnosis of diastrophic dysplasia as early as the 13th week of pregnancy. We think that prenatal diagnosis of diastrophic dysplasia can be possible even at first trimester.

Twin anemia-polycythemia sequence (TAPS), which was first described in 2007, is an atypical chronic form of twin-twin transfusion syndrome (TTTS). It presents as a large intertwin hemoglobin difference, with one twin developing anemia and the other polycythemia, without the traditional “polyhydramnios-oligohydramnios sequence” that is usually seen in TTTS. So far, ideal management of TAPS is not clear. We report here the case of a monochorionic biamniotic gestation, complicated by TAPS, detected at 30 weeks’ gestation that led to an emergent cesarean section. The recipient twin had an uneventful evolution. The donor twin was severely anemic, with a hemoglobin level of 2.5 g/dL that required immediate blood transfusion and intensive care management measures. Clinical evolution was favorable with good outcome in both twins. This case report intends to emphasize the association between hydrops fetalis and severe anemia, as well as the relevance of antenatal Doppler ultrasound investigations and the importance of an accurate management decision.

Umbilical cord teratomas are rare tumoral lesions of umbilical cord which have challenging antenatal diagnosis. The cord teratomas contain tissue from all three germ layers and have both cystic and solid components. This ultrasonographic appearance may help the clinician to clarify the correct diagnosis. We report a case of cord teratoma diagnosed prenatally by ultrasonography and magnetic resonance imaging. Since cord teratomas may lead to adverse fetal outcomes, close follow-up of the fetus is recommended.

Fetal seizures are relatively rare and most often associated with anomalies or adverse neonatal outcome. We describe a patient who presented in both her G1 and G2 pregnancies with fetal seizures. The second pregnancy was a twin gestation in which only one twin was affected. The fetal seizures were noted by the patient as “extreme rhythmic movement” and were observed on ultrasound. Both neonates were diagnosed with a seizure disorder within 1 day of life. Currently, the seizures are controlled by medication; however, both children have some developmental delay. Additionally, the patient and her partner are consanguineous, suggesting a likely genetic etiology. In utero diagnosis of fetal seizures warrants a multidisciplinary approach to attempt to further define prognosis and provide appropriate treatment and counseling.

Spontaneous intestinal perforation (SIP), a rare complication in neonates, was diagnosed in a preterm newborn. After surgery, small bowel histology showed acute ischemic changes and one area of perforation. Fibrin thrombus in the submucosal capillary vessels suggested a thrombotic mechanism in the pathogenesis. Subsequently, two severe asynchronous thrombotic events developed: cerebral sinovenous thrombosis and renal vein and inferior vena cava thrombosis. The neonate presented with hypercoagulability and factor V Leiden heterozygosity. This is the first association of SIP with inherited thrombophilia and thrombotic events in a neonate, suggesting hypercoagulability as a new research focus for pathogenesis of this enigmatic disease.

Infections of the central nervous system (CNS) in neonates with very low birth weight (VLBW) may have major clinical consequences due to their immunocompromised status. Bacillus cereus is a rare pathogen that can cause serious infection in these patients and is associated with a high mortality rate. We report the case of an extremely preterm neonate who developed severe infection of the CNS caused by B. cereus with progressive neurological deterioration despite broad spectrum antibiotic treatment. She died at the age of 16 months. In conclusion, we wish to increase the awareness among health care practitioner about the possibility of infection due to B. cereus in sick neonates and its devastating course in this population.

High frequency oscillatory ventilation through nasal prongs (nHFOV) has been utilised in an 11-day-old infant suffering from a severe form of respiratory syncytial virus (RSV) bronchiolitis with severe hypoxia (A-aDO 2 374 mm Hg) and deep hypercapnic acidosis (pH 6.97, pCO 2 148 mm Hg). Mean airway pressure (P aw ) was set at 12 cmH 2 O, amplitude (ΔP) at 38 cmH 2 O (level set according to the perception of vibration of the chest wall), frequency (FR) at 10 Hz, inspiratory time (Ti) 33%, FiO 2 0.95. pCO 2 dropped to 90 mm Hg in 6 h and to 60 mm Hg in the following 12 h. FiO 2 was progressively tapered to 0.75 in 3 h. The patient was weaned from nHFOV after 65 h. We speculate that nHFOV may be useful in the treatment of bronchiolitis-induced hypercapnia avoiding intubation.