Non Immune Hydrops Fetalis; Two Premature Infants with Favorable Outcome

Kayıran, Petek; Gürsoy, Tuğba; Gürakan, Berkan

doi:10.5472/mmj.2013.02956.0

Cited by 3 publications

(7 citation statements)

References 13 publications

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“…Fetal atrial flutter is the more common tachyarrhythmia and is responsible for up to 25 % of fetal tachyarrhythmias [21]. The incidence of NIHF in fetal SVT is bet ween 35-60 % [7]. In the retro spective analysis of 127 cases of fetal tachyarrhythmia by Simpson and Sharland [22], SVT was found in 105 patients and atrial flutter in 22 cases, and 52 (40.9 %) cases were hydropic.…”

Section: Cardiac Dysrhythmiasmentioning

confidence: 99%

“…The baby required adenosine treat ment after birth and was on regular digoxin and flecainide. Kayiran et al [7] reported a fetus developed Wolff-Parkinson-White syn drome with recurrent paroxysmal tachycardia (300 beats/min) and with hydrops fetalis after 30 weeks' gestation. Postnatal adenosi ne followed by oral digoxin (6 μg/kg/day) and propranolol (2 mg/ kg/day) ensured a favorable recovery.…”

Section: Cardiac Dysrhythmiasmentioning

confidence: 99%

“…The aim of in utero in tervention for hydrops fetalis is to avoid adverse fetal events and to prolong gestation time for expected fetal lung maturation. Hy drops fetalis is a grave condition and hydropic babies may need prompt interventions such as intubation and surfactant replace ment, umbilical catheterization, thoracentesis or paracentesis in the delivery room [7]. Successful treatment of hydrops fetalis by intrauterine albumin and blood transfusion has been described [44,45].…”

Section: Managementmentioning

confidence: 99%

“…Non-immune hydrops fetalis (NIHF) presenting before 24 weeks' gestation is usually due to chromosomal anomalies (aneu ploidy, deletion, duplication, or genetic mutation); while that pre senting after 24 weeks' gestation is usually caused by structural anomalies that interfere with the fetoplacental circulation [6]. Up to now, there have been no guidelines available for the optimal tim ing and mode of delivery for hydropic babies [7]. However, recently the Society for Maternal-Fetal Medicine published a clinical guide line on NIHF that indicated 34 weeks' gestation seemed to be a re asonable time for delivery and that vaginal delivery was the prefer red delivery mode if there were no contraindications [8].…”

Section: Introductionmentioning

confidence: 99%

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Cardiac Etiologies of Hydrops Fetalis

Yuan

2017

Z Geburtshilfe Neonatol

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Cardiac etiologies of hydrops fetalis have been a topic of concern due to challenging perinatal management. The common cardiac etiologies leading to hydrops fetalis include structural cardiac anomalies, cardiac dysrhythmias, cardiac tumors, cardiomyopathy and myocarditis. The mechanisms of cardiogenic hydrops fetalis may be: 1) elevation of atrial pressure and volume overload, 2) decrease of cardiac output, and 3) development of congestive heart failure. The diagnosis of hydrops fetalis was usually made at 19-36 gestational weeks, when ultrasound is a highly effective diagnostic method. Intrauterine interventions for certain congenital heart defects, maternal transplacental or direct fetal medications and fetal pacing placement for cardiac arrhythmias, and fetal or postnatal tumor resections are important progressions of etiologic treatment for hydrops fetalis. Treatment strategies for hydrops fetalis are usually ultrasound-guided pericardiocentesis and feto-amniotic shunting, whereas reaccumulation may require further interventions or postnatally. Hydrops fetalis often carries a poor prognosis, and mortality remains high. Current developments in the management of hydrops fetalis should encourage physicians to attempt further fetal interventions.

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Section: Cardiac Dysrhythmiasmentioning

confidence: 99%

Section: Cardiac Dysrhythmiasmentioning

confidence: 99%

Section: Managementmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Cardiac Etiologies of Hydrops Fetalis

Yuan

2017

Z Geburtshilfe Neonatol

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“…The incidence of NIHF in fetal supraventricular tachycardia is between 35-60%. (10) Antenatal ultrasound scan permits the detection, monitoring and often helps to determine the cause of hydrops foetalis. (11) Advances in ultrasonography (pulsed Doppler, colour Doppler, power Doppler) have made the diagnosis of these defects possible early in gestation.…”

Section: Introductionmentioning

confidence: 99%

Non - Immune Hydrops Fetalis: A Case Report

Colney¹,

Singh²,

Devi³

et al. 2015

jemds

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Hydrops fetalis is a serious fetal condition characterized by abnormal accumulation of fluid in fetal soft tissues and serous cavities. There are two types of hydrop fetalis:Immune hydrops fetalis (IHF). Non-immune hydrops fetalis (NIHF). We report a case of NIHF delivered by a 18 year old primi gravida. A single female fetus 27 weeks 5 days of gestational age was delivered by caesarean section. The mother was blood group A positive. She went for regular antenatal check-up. Ultrasound at 24 weeks revealed cystic hygroma with hydrops fetalis, severe oligohydramnios and intra-uterine fetal death. On examination, the neonate was a female and grossly oedematous. She showed scalp and body wall edema including limbs edema and ascites. Radiograph of the fetus was taken and fetus was dissected to rule out anomalies in internal organs.

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Lymphatic Abnormalities in Noonan Syndrome Spectrum Disorders: A Systematic Review

Sleutjes¹,

Kleimeier²,

Leenders

et al. 2021

Mol Syndromol

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Noonan syndrome spectrum disorders are a group of phenotypically related conditions, resembling Noonan syndrome, caused by germline pathogenic variants in genes within the Ras/mitogen-activated protein kinase (Ras/MAPK) signalling pathway. Lymphatic dysplasia with a clinical lymphatic abnormality is one of the major features. We performed a systematic review to get more insight in (1) the prevalence of clinically lymphatic abnormalities in patients with a genetically proven Noonan syndrome spectrum disorder, (2) if a genotype-lymphatic phenotype relation can be found and describe the clinical presentation and course of the lymphatic abnormality. Most studies report patients with Noonan syndrome. Prenatally, the prevalence of increased nuchal translucency differs from 7% in patients with pathogenic PTPN11 variants to 38% in patients with pathogenic RIT1 variants, and the prevalence of pleural effusions differed from 7% in patients with pathogenic SOS1 to 29% in patients with pathogenic RIT1 variants. Postnatally, the prevalence of lymphedema differs from 16% in patients with pathogenic PTPN11 variants to 44% in patients with pathogenic SOS1 variants, and the prevalence of acquired chylothorax is 4% in patients with pathogenic RIT1 variants. Lymphatic abnormalities do occur in patients with cardiofaciocutaneous syndrome and Costello syndrome. In conclusion, Noonan syndrome spectrum disorders, Noonan syndrome in particular, are associated with lymphatic abnormalities. Combining the available published literature about genetically proven Noonan syndrome spectrum disorders, it appears likely that the lifetime prevalence of these abnormalities in Noonan syndrome is higher than the 20% that were generally accepted so far. This is increasingly important, because the activation of the RAS/MAPK pathway can be inhibited by RAS/MAPK inhibitors, and clinically severe lymphatic abnormalities may improve.

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Non Immune Hydrops Fetalis; Two Premature Infants with Favorable Outcome

Cited by 3 publications

References 13 publications

Cardiac Etiologies of Hydrops Fetalis

Cardiac Etiologies of Hydrops Fetalis

Non - Immune Hydrops Fetalis: A Case Report

Lymphatic Abnormalities in Noonan Syndrome Spectrum Disorders: A Systematic Review

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