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Permanent junctional reciprocating tachycardia (PJRT) is a rare form of congenital arrhythmia occurring predominantly in infants and children. Prenatal presentation is frequently characterized by incessant tachycardia leading to dilated cardiomyopathy (DCM). Some patients can have a normal heart rate which leads to a delayed diagnosis. We report a case of a neonate who was presented prenatally with DCM, fetal hydrops, and no signs of fetal arrhythmia. Diagnosis of PJRT was established after delivery with characteristic electrocardiographic patterns. Successful conversion to sinus rhythm with digoxin and amiodarone was achieved three months later. At 16 months of age, both echocardiography and electrocardiography were normal.

Resume Objectif Decrire les moyens actuels de proceder a l'exploration et a la prise en charge de l'anasarque fœtoplacentaire non immune, en mettant l'accent sur les etiologies traitables ou recurrentes. issues Offrir de meilleurs services de counseling et de prise en charge en presence de cas d'anasarque non immune identifies par diagnostic prenatal. Resultats La litterature publiee a ete recuperee par l'intermediaire de recherches menees dans PubMed, CINAHL et The Cochrane Library en 2011 au moyen de mots cles (« non-immune hydrops fetalis », « fetal hydrops », « fetal therapy », « fetal metabolism »). Les resultats ont ete restreints aux analyses systematiques, aux essais comparatifs randomises / essais cliniques comparatifs, aux etudes observationnelles et aux exposes de cas significatifs. D'autres publications ont ete reperees a partir des bibliographies de ces articles. Aucune restriction n'a ete appliquee en matiere de date ou de langue. Les recherches ont ete mises a jour de facon reguliere et integrees a la directive clinique jusqu'en mai 2012. La litterature grise (non publiee) a ete identifiee par l'intermediaire de recherches menees dans les sites Web d'organismes s'interessant a l'evaluation des technologies dans le domaine de la sante et d'organismes connexes, dans des collections de directives cliniques, dans des registres d'essais cliniques et aupres de societes de specialite medicale nationales et internationales. Avantages, desavantages et couts La presente directive clinique sensibilise les lecteurs aux causes de l'anasarque fœtoplacentaire non immune, ainsi qu'au diagnostic prenatal et a la prise en charge de celle-ci. Elle offre egalement une approche standardisee envers l'anasarque fœtoplacentaire non immune, en mettant l'accent sur la recherche de troubles pouvant etre pris en charge pendant la periode prenatale et d'etiologies genetiques recurrentes. valeurs La qualite des resultats est evaluee au moyen des criteres decrits dans le rapport du Groupe d'etude canadien sur les soins de sante preventifs (Tableau 1). Recommandations 1.Toutes les patientes qui presentent une anasarque fœtoplacentaire devraient etre orientees sans delai vers un centre de soins tertiaires a des fins d'evaluation. Certains troubles pouvant faire l'objet d'un traitement prenatal constituent une urgence therapeutique apres 18 semaines. (II-2A) 2.L'analyse chromosomique fœtale et le depistage moleculaire sur microreseau d'ADN devraient etre offerts (la ou cela s'avere possible) dans tous les cas d'anasarque fœtoplacentaire non immune. (II-2A) 3.Les etudes d'imagerie devraient comprendre une echographie obstetricale exhaustive (dont des etudes Doppler arterielles et veineuses fœtales) et une echocardiographie fœtale. (II-2A) 4.Chez les femmes exposees a des risques en raison de leur origine ethnique, des explorations visant des infections fœto-maternelles et l'alpha-thalassemie devraient etre menees dans tous les cas d'anasarque fœtoplacentaire inexpliquee. (II-2A) 5.Pour evaluer le risque d'anemie fœtale, la mesure du pic de vitesse systolique de l'artere cerebrale moyenne par etude Doppler devrait etre menee chez tous les fœtus hydropiques apres 16 semaines de gestation. Lorsque l'on soupconne la presence d'une anemie fœtale, le prelevement de sang fœtal et la transfusion intra-uterine devraient etre offerts rapidement. (II-2A) 6.Tous les cas d'anasarque fœtoplacentaire inexpliquee devraient etre orientes vers des services de genetique medicale, lorsque de tels services sont disponibles. Une evaluation postnatale detaillee devrait etre menee par un geneticien medical pour tous les nouveau-nes presentant une anasarque non immune inexpliquee. (II-2A) 7.La tenue d'une autopsie devrait etre recommandee dans tous les cas d'interruption de grossesse ou de deces fœtal ou neonatal. (II-2A) Du liquide amniotique et/ou des cellules fœtales devraient etre conserves aux fins de la tenue d'un futur depistage genetique. (II-2B)

Yellow nail syndrome (YNS) is a rare disorder characterized by a triad of yellow dystrophic nails, lymphedema, and chronic respiratory manifestations.1 YNS often occurs in adults older than 40 years, with no sex predominance. However, few pediatric cases have been reported.1 We describe a child having congenital YNS with several features, including congenital nail dystrophy, pleural effusion, and eyelid and lower-limb lymphedema.

PurposeFetal hydrops arises from multiple disease processes and can portend a grim prognosis. We reviewed our experience with hydropic fetuses to understand relevant antenatal anatomic and physiologic predictors of survival.MethodsWe reviewed fetal ultrasounds and echocardiograms of hydropic fetuses evaluated from 1996 to 2013.ResultsOverall neonatal survival in 167 fetuses was 44% (range, 0-75%) and was influenced by the underlying disease process. The anatomic distribution of fluid varied and was not significantly different between survivors and nonsurvivors. Univariate analysis indicated that resolution of hydrops and delivery at a later gestational age were predictive of survival (OR: 5.7 (95% CI: 2.5-13.2) and OR: 1.3 (95% CI: 1.1-1.4), respectively). Fetal intervention also improved survival in some diseases. Echocardiograms were reviewed to group fetuses with similar cardiac physiology and defined categories with high or low/normal cardiothoracic ratio (CTR). Among patients with a high CTR, the cardiovascular profile score was predictive of survival (p=0.009).ConclusionSurvival in hydrops depends on the underlying disease, available fetal therapies to resolve hydrops, and the gestational age of delivery and not on the specific anatomic manifestations of hydrops. In hydropic fetuses with high CTRs, the cardiovascular profile score may be a useful prognostic indicator.

Mirror syndrome, also called Ballantyne syndrome, is a rare condition in pregnancy, defined by the presence of the clinical triad of fetal hydrops, placentomegaly and maternal oedema. Any aetiology of fetal hydrops, including rhesus iso-immunization, congenital infection, twin-to-twin transfusion, structural anomalies and fetal malignancies, can lead to the syndrome. The pathogenesis, although not well established, mimics trophoblastic damage and maternal vascular endothelial dysfunction, as is also seen in pre-eclampsia, and, hence, the two conditions may have a similar clinical presentation. They may even co-exist, where a patient with maternal mirror syndrome develops features of pre-eclampsia. A timely, accurate diagnosis and prompt interventions are needed to prevent fetal mortality and maternal morbidity., Highlights • Mirror syndrome is a rare condition in pregnancy defined by the clinical triad of fetal, placental and maternal hydrops • The correction of fetal hydrops (and, hence placental hydrops) resolves Mirror syndrome • The trophoblastic damage caused by placental oedema causes endothelial dysfunction leading to Mirror syndrome • Although Mirror syndrome and pre-eclampsia have different aetiologies they may have similar presentations and co-exist

Resume Les blocs auriculoventriculaires congenitaux d’origine immunologique sont lies au passage transplacentaire d’anticorps anti-SS-A/Ro et/ou anti-SS-B/La maternels pouvant s’integrer dans le cadre d’une connectivite. L’echographie avec Doppler est essentielle pour le depistage de ces blocs auriculoventriculaires au cours des grossesses a risque. Elle permet le diagnostic des blocs du premier ou deuxieme degre qui constituent probablement des etapes preliminaires dans l’atteinte des voies de conduction. Un traitement maternel par corticoides peut dans cette situation etre propose au couple, en raison d’un possible effet de cette therapeutique sur les blocs du premier ou deuxieme degre. En tant que signes precoces d’une lesion nodale, ils peuvent toutefois manquer : certains fœtus developpent un bloc complet sans qu’aucun bloc de degre inferieur n’ait ete identifie prealablement. De meme, la valeur semiologique du bloc du premier degre n’est pas totalement claire puisqu’il semble pouvoir etre spontanement reversible. D’autres marqueurs de lesion nodale seraient donc precieux. En cas de bloc complet, l’echographie est utile pour identifier des signes de decompensation cardiaque et guider la conduite obstetricale et l’extraction fœtale sur la base de signes pronostiques defavorables.

Before the 20th week of gestation, the most common cause of nonimmune hydrops fetalis is chromosomal abnormalities. Herein, we report a case of fetal hydrops, anemia, and intrauterine growth retardation that presented at 27 weeks of gestation with a negative chromosomal abnormality screening. Cordocentesis and karyotype analysis revealed fetal pancytopenia and Down syndrome. Down syndrome rarely presents with fetal hydrops and anemia. Therefore, when hydrops and anemia are diagnosed, especially in the second trimester of gestation, the possibility of Down syndrome should be kept in mind. In addition, if the pregnancy results in a live birth, the baby should be examined for transient abnormal myelopoiesis.

The objective of this paper was to evaluavate a case of non-immune fetal hydrops associated with generalized lymphangiectasia. We studied the ultrasound examinations, and clinical and biochemical data of a patient with this syndrome. A bilateral pleural effusion detected by ultrasound in the third trimester of gestation was the first manifestation of the disease. Though rare, generalized lymphangiectasia should be considered in patients with pleural effusion and a progressive increase of the fluids in the fetal cavities and of the amniotic fluid.

As the incidence of Rh-isoimmunization has been decreasing with the availability of Rh immune globulin, the proportion of cases of fetal hydrops from nonimmune causes has increased. Evaluation of the fetus with hydrops requires an integrated approach, beginning with targeted ultrasound evaluation and potentially including maternal and fetal blood testing and other invasive testing. Because the list of conditions that may cause hydrops is long and continues to grow, it is often difficult to make a precise diagnosis; however, through a systematic approach, one may frequently narrow the etiology to a category of disorders and determine whether any interventions are available that are likely to be helpful in improving the outlook for the fetus.

Ballantyne syndrome or Mirror syndrome or triple edema syndrome is a rare and dangerous disorder affecting pregnant women. It describes unusual association of fetal and placental hydrops with maternal edema. It was first described in 1882 by John William Ballantyne. Awareness of the syndrome is important due to associated fetal and maternal risks [1]. We present a case of Ballantyne syndrome in Rhesus isoimmunised pregnancy with severe fetal hydrops.

Fetal hydrops is a known complication of sacrococcygeal teratoma (SCT) due to arteriovenous shunts within the tumor, which is clinically important and influence patients' prognosis. We present unique MRI morphological findings of a marked fetal anasarca in a case of an end-second trimester fetus presenting with SCT.

The technique of intrauterine fetal visualization is described and 53 cases are reported. No side effects were observed. Indications for employing fetography are proposed: 1. 1. hydramnios and other conditions associated with a high incidence of fetal malformation; 2. 2. Rh iso-immunization and α-thalassemia: (a) as a diagnostic method for detection of hydropic edema, (b) as an aid in intrauterine transfusion; 3. 3. as an adjunct in the estimation of fetal maturity; 4. 4. as a diagnostic method in suspected monoamniotic twin pregnancies.

The presence of large bilateral theca lutein cysts in pregnancy not associated with hydatidiform mole is apparently very rare, only 2 cases having been previously reported in the medical literature. A case of our own occurring at the Deaconess Hospital in Buffalo is added. In 2 of these 3 cases, fetal hydrops (before the Rh era) was reported; in our case fetal hydrops together with proved erythroblastosis due to Rh incompatibility was present. A theory is proposed correlating the anatomic findings in hydatidiform moles and in cases of erythroblastosis (persistence of the cytotrophoblastic layer which may be the site of gonadotropic hormone production).

Nonimmure hydrops fetalis is a serious perinatal complication with diverse causes but few successful treatment modalities. The first reported case of hydrops fetalis caused by a massive fetomaternal hemorrhage treated successfully prenatally is presented. A modification of the standard intrauterine transfusion technique was used for therapy. Implications of future treatment are discussed.

The experience of the authors to January 1971, includes 46 fetal transfusions in 28 patients with Zone III amniotic fluid. Nine of these 28 babies represented the second pregnancy for the mother. For half of these patients (14 of 28), the baby requiring transfusion represented the first affected pregnancy, and more than two thirds (22 of 28) had not experienced a previous hemolytic perinatal death. Most perinatal losses (all 6 neonatal deaths and 9 of 13 fetal deaths) resulted from the inability to reverse fetal hydrops.