We report two cases of pentalogy of Cantrell diagnosed in utero using a combination of fetal echocardiography and magnetic resonance imaging. The cardiac component consisted of tetralogy of Fallot in the first fetus and ventricular septal defect in the second fetus. Whereas fetal echocardiography allowed accurate delineation of the cardiac anatomy, prenatal magnetic resonance imaging allowed clearer delineation of the extent of the thoracic and abdominal wall defects. Fetal magnetic resonance imaging in conjunction with prenatal echocardiography allows optimal assessment of the fetus with ectopia cordis, which has significant implications from the standpoint of preoperative planning and providing prognostic information. This report represents the first description of applying magnetic resonance imaging in combination with echocardiography toward a better understanding of this clinical entity in the fetus.

Fetal ultrasonography is an important tool used to prenatally diagnose many craniofacial conditions. Pierre Robin sequence (PRS) is a rare congenital deformation characterized by micrognathia, glossoptosis, and airway obstruction. PRS can present as a perinatal emergency when the retropositioned tongue obstructs the airway leading to respiratory compromise. More predictable and reliable diagnostic studies could help the treating medical team as well as families prepare for these early airway emergencies. The medical literature was reviewed for different techniques used to prenatally diagnose PRS radiologically. We have reviewed these techniques and suggested a possible diagnostic pathway to consistently identify patients with PRS prenatally.

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Needle core biopsy appears to have a low rate of associated complications, and its sensitivity for diagnosing PC and follicular neoplasm is comparable to what has been reported for fine needle aspiration biopsy in a similar patient population.

Our study identified specific characteristics on fetal MR imaging that can be used as predictors of the diagnosis of congenital aqueductal stenosis. Most of these findings are secondary to the obstructive nature of the resulting hydrocephalus. Common associated malformations such as rhombencephalosynapsis and dystroglycanopathies should also increase the suspicion of congenital aqueductal stenosis when present with ventriculomegaly.

Introduction: This retrospective study aims to describe systematically the fetal cerebral MR morphology in cases with occipital meningoencephaloceles using standard and advanced fetal MRI techniques. Material and Methods: The 1.5-tesla MR examinations (T1- and T2-weighted imaging, echo planar imaging, EPI, diffusion-weighted imaging, DWI) of 14 fetuses with occipital/parietal meningoencephaloceles were retrospectively analyzed for the classification of anatomic characteristics. A diffusion tensor sequence was performed in 5 cases. Results: In 9/14 cases the occipital lobes were entirely or partially included in the encephalocele sac. Typical features of Chiari III malformation were seen in 6/14 cases. The displaced brain appeared grossly disorganized in 6/14. The brainstem displayed abnormal ‘kinking'/rotation (3/14), a z-shape (1/14) and/or a molar tooth-like configuration of the midbrain (3/14). Tractography revealed the presence and position of sensorimotor tracts in 5/5 and the corpus callosum in 3/5. DWI was helpful in the identification of a displaced brain (in 8/9). EPI visualized the anatomy of draining cerebral veins in 7/9 cases. Clinical (9/14) and MRI (7/14) follow-up data are presented. Discussion: Encephaloceles show a wide range of morphological heterogeneity. Fetal MRI serves as an accurate tool in the visualization of brainstem, white matter pathway and cerebral venous involvement and facilitates the detection of specific underlying syndromes such as ciliopathies.

Objectives: Hysterotomy scar disruption, ranging from myometrial thinning to complete dehiscence, is a well-established complication of open-hysterotomy fetal myelomeningocele (MMC) repair. This study sought to (a) determine the feasibility of postoperative magnetic resonance imaging (MRI) in detecting signs of hysterotomy scar disruption and (b) identify the sonographic and clinical signs suggestive of subacute scar dehiscence, including decreasing amniotic fluid index (AFI) and uterine contractions, respectively. Methos: A unique index case of suspected hysterotomy dehiscence following MMC repair prompted a retrospective review of 31 total open-hysterotomy fetalMMC repairs performed at our center, including 21 cases found to have intact hysterotomy scarring and 10 cases of non intact scarring detected at subsequent cesarean delivery. In each case, routine post operative MRI, performed 6 weeks after the MMC repair, was reviewed to evaluate the thickness of the hysterotomy site. Cases were also reviewed for sonographic and clinical patterns preceding delivery, including changes in AFI and the presence or absence of uterine contractions. Results:Of the 31 total reviewed cases, 21 cases were found to have intact hysterotomy scar sites at the time of cesarean delivery. Among the intact cases, the net change in AFI from the time of MRI to delivery ranged from −45% to 47%, with a mean increase in fluid levels of 8% over an average of 5.6 weeks. The other 11 cases, including the index case, were found to have signs of scar disruption at delivery, including seven with thinned scar sites and four with grossly dehiscent sites. Amongst non-intact cases, AFI predominately decreased, with a net change ranging from −56% to 9% for a mean change of −24% over an average of 5.4 weeks. Regular uterine contractions close to the time of delivery occurred in 82% of the non intact cases. Conclusion:Hysterotomy scar disruption can rarely be detected by MRI following MMC repair. Decreasing AFI and contractions may serve as early warning signs of scar dehiscence and should be taken into consideration for obstetric management.

Introduction: Without fetal or perinatal intervention, congenital high airway obstruction syndrome (CHAOS) is a fatal anomaly. The ex utero intrapartum treatment (EXIT) procedure has been used to secure the fetal airway and minimize neonatal hypoxia, but is associated with increased maternal morbidity. Case Presentation: A 16-year-old woman (gravida 1, para 0) was referred to our hospital at 31 weeks gestation with fetal anomalies, including echogenic lungs, tracheobronchial dilation and flattened diaphragms. At 32 weeks, fetoscopic evaluation identified laryngeal stenosis, which was subsequently treated with balloon dilation and stent placement. The patient developed symptomatic and regular preterm contractions at post-operative day 7 with persistent sonographic signs of CHAOS, which prompted a repeat fetoscopy with confirmation of a patent fetal airway followed by Cesarean delivery under neuraxial anesthesia. Attempts to intubate through the tracheal stent were limited and resulted in removal of the stent. A neonatal airway was successfully established with rigid bronchoscopy. Direct laryngoscopy and bronchoscopy confirmed laryngeal stenosis with a small tracheoesophageal fistula immediately inferior to the laryngeal stenosis and significant tracheomalacia. A tracheostomy was then immediately performed for anticipated long term airway and pulmonary management. The procedures were well tolerated by both mom and baby. The baby demonstrated spontaneous healing of the tracheoesophageal fistula by day of life 7 with discharge home with ventilator support at three months of life. Conclusion: Use of repeated fetoscopy in order to relieve fetal upper airway obstruction offers the potential to minimize neonatal hypoxia, while concurrently decreasing maternal morbidity by avoiding an EXIT procedure. Use of the tracheal stent in CHAOS requires further investigation. The long-term reconstruction and respiratory support of children with CHAOS remain challenging