Nonimmune Hydrops Fetalis.

Nonimmune hydrops fetalis (NIHF) historically has been considered a lethal fetal condition. Understanding NIHF to be a symptom or an end-stage status of a variety of fetal conditions, along with improved fetal diagnostics and interventions, has changed the landscape for at least some fetuses. Understanding the pathophysiologic mechanisms has led to the development of diagnostic algorithms, improved understanding of cause, and therefore fetal or neonatal treatments. Multidisciplinary counseling and shared decision making are critical to supporting families through pregnancy decisions, potential fetal therapeutic interventions, neonatal management decisions, and at times accepting or transitioning to palliative care.

Obstetric complications in pregnancies with life-limiting malformations.

PURPOSE OF REVIEW: The implementation of palliative care at birth has led to a significant rise in the number of couples who choose to continue with pregnancies complicated by life-limiting malformations (LLMs). Prenatal counselling and appropriate antenatal/perinatal management in these cases are poorly studied and may pose significant challenges. The purpose of this review is to outline specific obstetric risks and to suggest management for mothers who choose to continue with pregnancies with the most common LLMs. RECENT FINDINGS: In pregnancies complicated by LLMs where parents opt for expectant management, clinicians should respect parental wishes, whilst openly sharing potential serious maternal medical risks specific for the identified abnormalities. The focus of both antenatal and perinatal care should be maternal wellbeing rather than foetal survival. Follow-up ultrasound examinations and maternal surveillance should be aimed at achieving timely diagnosis and effective management of obstetric complications. A clear perinatal plan, agreed with the couples by a multi-disciplinary team including a foetal medicine specialist, a neonatologist and a geneticist, is crucial to reduce maternal morbidity. SUMMARY: This review provides a useful framework for clinicians who face the challenges of counselling and managing cases complicated by LLMs where parents opt for pregnancy continuation.

Long-Term Neurodevelopmental and Respiratory Outcome after Intrauterine Therapy for Fetal Thoracic Abnormalities.

INTRODUCTION: The aim of this study is to evaluate long-term neurodevelopmental and respiratory outcome after fetal therapy for fetal pleural effusion, congenital cystic adenomatoid malformation, and bronchopulmonary sequestration. METHODS: Children ≥18 months of age underwent an assessment of neurologic, motor, and cognitive development. Medical records were reviewed to determine respiratory outcome. Behavioral outcome was assessed using the Child Behavioral Checklist. RESULTS: Between 2001 and 2016, 63 fetuses with fetal hydrops secondary to thoracic abnormalities were treated at our center. Overall perinatal survival was 64% (40/63). Twenty-six children were included for follow-up (median age 55 months). Severe neurodevelopmental impairment (NDI) was detected in 15% (4/26). Three out of 4 children with severe NDI had associated causes contributing to the impairment. Overall adverse outcome, including perinatal mortality or NDI, was 55% (27/49). Fifteen percent (4/26) had severe respiratory sequelae. Parents did not report more behavioral problems than Dutch norms. DISCUSSION: Our results suggest that severe NDI in this specific high-risk cohort occurs in 15%, which is above the range of the incidence of NDI reported in case series treated with other fetal therapies (5-10%). Large multicenter studies and an international web-based registry are warranted to prospectively gather outcome data at fixed time points.

Long-Term Outcomes After Thoracoamniotic Shunt for Pleural Effusions With Secondary Hydrops.

BACKGROUND: Congenital pleural effusion is a rare condition with an incidence of approximately one per 15,000 pregnancies. The development of secondary hydrops is a poor prognostic indicator and such cases can be managed with a thoracoamniotic shunt (TAS). Our objective is to describe postnatal outcomes in survivors after TAS placement for congenital pleural effusions. MATERIALS AND METHODS: A retrospective study of all cases with fetal pleural effusions treated between 2006 and 2016. Patients with dominant unilateral or bilateral pleural effusions complicated by secondary hydrops fetalis received TAS placement. The results are reported as median (range). RESULTS: A total of 29 patients with pleural effusion with secondary hydrops underwent TAS placement. The gestational age at the initial TAS placement was 27.6 (20.3-36.9) wk. Before delivery, hydrops resolved in 17 (58.6%) patients. The delivery gestational age was 35.7 (25.4-41.0) wk and the overall survival rate was 72.4%. Among the 21 survivors, 19 (90.5%) required admission to the neonatal intensive care unit for 15 (5-64) d. All 21 survivors had postnatal resolution of the pleural effusions. All 21 children were long-term survivors, with a median age of survivorship of 3 y 3 mo (9 mo-7 y 6 mo) at the time of last reported follow-up. CONCLUSIONS: Thoracoamniotic shunting in fetuses with a dominant pleural effusion(s) and secondary hydrops resulted in a 72% survival rate. Nearly all survivors required admission to the neonatal intensive care unit. However, a majority did not have significant long-term morbidity.

Outcomes and morbidities of patients who survive haemoglobin Bart's hydrops fetalis syndrome: 20-year retrospective review.

INTRODUCTION: Haemoglobin Bart's hydrops fetalis syndrome was once considered a fatal condition. However, advances over the past two decades have enabled survival of affected patients. Data relating to their morbidities and outcomes will help medical specialists formulate a management plan and parental counselling. METHODS: All babies with the syndrome who survived beyond the neonatal period and were subsequently managed long-term in eight public hospitals in Hong Kong from 1 January 1996 to 31 December 2015 were included. Patient and parent characteristics, antenatal care, reasons for continuation of pregnancy, intrauterine interventions, perinatal course, presence of congenital malformations, stem-cell transplantation details, and long-term neurodevelopmental outcomes were reviewed. RESULTS: A total of nine patients were identified, of whom five were female and four male. The median follow-up duration was 7 years. All were Chinese and were homozygous for the Southeast Asian α-thalassaemia deletion. Five of the nine mothers received antenatal care at a public hospital and opted to continue the pregnancy after antenatal diagnosis and counselling. Despite intrauterine transfusions, all babies were born with respiratory depression and required intubation and mechanical ventilation during the neonatal period. Hypospadias was identified in all four male infants. Growth retardation, global developmental delay, and residual neurological deficits were noted in two-thirds of the patients. Haematopoietic stem-cell transplantation was performed in two patients, who became transfusion-independent. CONCLUSIONS: Survival of patients with Bart's hydrops fetalis syndrome is possible but not without short- and long-term complications; local epidemiology is comparable to that documented for an international registry. Detailed antenatal counselling of parents with a non-judgemental attitude and cautious optimism are imperative.

Fetal cystic hygroma: the importance of natural history.

OBJECTIVES: To evaluate the natural history of cystic hygroma (CH) in fetal and neonatal periods to enrich parental counselling. Ultrasonographic characteristics, associated syndromes, chromosomal anomalies, fetal cardiac pathology and life after birth were considered. STUDY DESIGN: From May 1985 to September 2010, 207 pregnancies were seen the authors' centre with suspected vascular-lymphatic fetal malformation: 156 of them had CH. Cases were followed up by telephone interview to determine fetal and neonatal outcomes. Chi-squared test was used for statistical analysis. RESULTS: Among the 156 cases of CH, the condition was septated in 75% of cases, associated with other pathologies in 74%, and retronuchal in 88%. Intrauterine regression was seen in 36% of cases, with complete disappearance in 77%. The karyotype was normal in 55% of cases. Follow-up was completed in 85 cases and revealed 54 spontaneous abortions (63%) and 31 live births (37%). Amongst these, 21 out of 31 children had a favourable outcome (68%). A negative embryo-fetal outcome was significantly associated with CH being associated with other pathologies, such as hydrops, retronuchal position and altered karyotype. Spontaneous regression or resolution of CH was associated with live births. CONCLUSIONS: The management of pregnancies with a diagnosis of fetal CH requires knowledge of natural history of the malformation for appropriate parental counselling.

Two cases of prenatally diagnosed sacrococcygeal teratoma type I with different clinical features.

Sacrococcygeal teratoma (SCT) is a rare congenital disease and prognostic factors have not been entirely established. We report two cases of fetal SCT with different clinical courses. Case 1 was a cystic, slow growing tumor with mild vascularity. The tumor was removed one week after delivery at 35 weeks, and there was no recurrence at 1.5-year follow-up. Case 2 was a solid, rapid growing tumor with rich vascularity. Cesarean section was performed due to severe fetal hydrops and mirror syndrome in the mother at 27 weeks. The tumor had ruptured and was removed soon after delivery to control bleeding, but the baby died the next day. Our cases suggest that solid component and rich vascularity might correlate with poor prognosis.

Mirror syndrome in a Chinese hospital: diverse causes and maternal fetal features.

OBJECTIVE: To investigate the clinical characteristics of mirror syndrome. METHODS: Retrospective analysis of cases with mirror syndrome. Data of clinical manifestations, laboratory examinations, placental morphology, treatment and prognosis of these patients were obtained and studied. RESULTS: Five cases satisfying the inclusion criteria for mirror syndrome were identified from our hospital database. The incidence of the condition was 0.0154% in China. Mirror syndrome was associated with Rhesus isoimmunization, intrauterine parvovirus B19 infection, fetal neuroblastoma, fetal heart malformation and unknown cause respectively. Fetal symptoms were multi-hydrocele and fetal heart failure complicating fetal hydrops. All of the cases manifested maternal hydrops and hemodilution, the other most common symptoms included hypertension, proteinuria, hypoalbuminemia, anemia, thrombocytopenia and elevated uric acid levels. Fetal outcomes in this study were poor with a perinatal mortality rate of 100%. Placentomegaly was observed in most cases and placental morphology showed villous edema, increased intervillous fibrin deposition and one rare case of fetal adrenal neuroblastoma. Resolution of maternal symptoms was noted within 3-30 days after delivery. CONCLUSION: Mirror syndrome is associated with a substantially increased risk of fetal death and severe maternal complications. Early diagnosis of this condition during pregnancy is crucial for providing proper treatments and achieving better clinical outcomes.

First-trimester cystic hygroma: relationship of nuchal translucency thickness and outcomes.

OBJECTIVE: To estimate the relationship between nuchal translucency thickness and abnormal karyotype, major congenital anomaly, perinatal loss, and composite abnormal outcome in fetuses with first-trimester nuchal cystic hygroma. METHODS: We performed a retrospective cohort study of first-trimester fetuses with ultrasound-diagnosed nuchal cystic hygroma collected over a 10-year period. RESULTS: There were 944 first-trimester fetuses with nuchal cystic hygroma. A karyotype abnormality occurred in 54.9% (400 of 729) of fetuses. A major congenital anomaly occurred in 28.8% (61 of 212) of fetuses with a normal karyotype. Perinatal loss occurred in 39% (115 of 295) of fetuses not electively terminated. Overall, an abnormal outcome occurred in 86.6% (543 of 627) of fetuses. After adjusting for potential confounders, every 1-mm increase in nuchal translucency thickness increased the odds of an abnormal karyotype by 44% (adjusted odds ratio [OR] 1.44, 95% confidence interval [CI] 1.29-1.60, P<.001), the odds of major congenital anomaly by 26% (adjusted OR 1.26, 95% CI, 1.08-1.47, P=.003), the odds of perinatal loss by 47% (adjusted OR 1.47, 95% CI 1.07-2.02, P=.019), and the odds of a composite abnormal outcome by 77% (adjusted OR 1.77, 95% CI 1.15-2.74, P=.01). CONCLUSION: First-trimester nuchal cystic hygroma is associated with high rates of karyotype abnormality, major congenital anomaly, perinatal loss, and abnormal outcome. As the thickness of the nuchal translucency increases, the odds of abnormal karyotype, major congenital anomaly, perinatal loss, and abnormal outcome increase.

Short-term and long-term outcomes of 214 cases of non-immune hydrops fetalis.

Despite advances in diagnosis and management, non-immune hydrops fetalis (NIHF) has a high mortality rate. Perinatal survival depends on the underlying disorder and the gestational age at diagnosis. As prognostic information is limited, this study acquired data regarding the neurological development of perinatal survivors. We performed a retrospective chart review of 214 cases in which NIHF was diagnosed antenatally. We recorded maternal demographic characteristics and interventions and their effectiveness, as well as the short-term outcome (survival) and long-term outcome including developmental quotients. Among the affected fetuses, 91 (42.5%) survived the perinatal period. Fetuses with chylothorax, chyloascites, or meconium peritonitis, and those in whom therapy was effective, had high survival rates irrespective of the type of intrauterine intervention. The subsequent intact survival rate was 28/56 (50.0%), with intact defined as ratio of the number of infants with normal development to the number of all infants followed. In contrast to the perinatal survival rate, the intact survival rate decreased as gestational age at diagnosis advanced. These findings suggest that the long-term intact survival rate depends on the underlying cause of NIHF. Additionally, while survival was improved with intensive perinatal care during the perinatal period, aggressive perinatal intervention was not a prognostic factor for neurological outcome.

S-adenosylhomocysteine hydrolase deficiency: two siblings with fetal hydrops and fatal outcomes.

This paper reports the clinical and metabolic findings in two sibling sisters born with fetal hydrops and eventually found to have deficient S-adenosylhomocysteine hydrolase (AHCY) activity due to compound heterozygosity for two novel mutations, c.145C>T; p.Arg49Cys and c.257A>G; p.Asp86Gly. Clinically, the major abnormalities in addition to fetal hydrops (very likely due to impaired synthetic liver function) were severe hypotonia/myopathy, feeding problems, and respiratory failure. Metabolic abnormalities included elevated plasma S-adenosylhomocysteine, S-adenosylmethionine, and methionine, with hypoalbuminemia, coagulopathies, and serum transaminase elevation. The older sister died at age 25 days, but the definitive diagnosis was made only retrospectively. The underlying genetic abnormality was diagnosed in the second sister, but treatment by means of dietary methionine restriction and supplementation with phosphatidylcholine and creatine did not prevent her death at age 122 days. These cases extend the experience with AHCY deficiency in humans, based until now on only the four patients previously identified, and suggest that the deficiency in question may be a cause of fetal hydrops and developmental abnormalities of the brain.

Outcome and treatment in an antenatally diagnosed congenital cystic adenomatoid malformation of the lung.

BACKGROUND: The natural history of patients with antenatally diagnosed congenital cystic adenomatoid malformation of the lung (CCAM) is still fully unknown. In symptomatic patients with respiratory distress, an operation is performed during the neonatal period. However, in asymptomatic patients, the optimal timing of the operation remains controversial. During the period from 1977 to 2007, we experienced 14 CCAM patients diagnosed antenatally. Therefore, we investigated the outcome of antenatally diagnosed CCAM patients to clarify the optimal treatment for such patients. METHODS: Fourteen patients were reviewed regarding the antenatal ultrasonography findings and postnatal clinical course. They were then classified into three groups according to the clinical manifestations. Group A was associated with hydrops fetalis (n = 2), group B had respiratory symptoms just after birth (n = 6), and group C was asymptomatic at birth (n = 6). The postnatal clinical courses in three groups were reviewed. RESULTS: In group A, all two patients with hydrops fetalis died just after birth. In group B, six patients had a severe respiratory distress and underwent operation during the neonatal period. In group C, five out of six patients were asymptomatic and received elective operation during the early infant period. In the remaining one patient, the lesion spontaneously disappeared over time after birth. The mean age at the time of operation in group B and group C was 4.5 days and 4.5 months of age, respectively. In almost all patients in group C, we performed an operation within the first 6 months. During this observation period, we did not experience any complications associated with CCAM. CONCLUSION: In patients with hydrops fetalis, fetal intervention is thought to be needed. In patients with asymptomatic CCAM, an elective operation during the early infant period is recommended to prevent the risk of complications associated with CCAM before 6 months of age. In addition, we recommend the performance of a partial lung resection using an axillary skin crease incision in order to obtain a good postoperative quality of life.

Incidence, causes and pregnancy outcomes of hydrops fetalis at Srinagarind Hospital, 1996-2005: a 10-year review.

OBJECTIVE: To identify the incidence and determine causes and pregnancy outcomes of hydrops fetalis at Srinagarind Hospital. STUDY DESIGN: A retrospective descriptive study. SETTING: Department of Obstetrics and Gynecology, Faculty of Medicine, Srinagarind Hospital, Khon Kaen University. MATERIAL AND METHOD: A retrospective medical record review of all pregnant women and newborns who were diagnosed with hydrops fetalis at all gestational ages and delivered at Srinagarind Hospital between 1996 and 2005. RESULTS: During the period of study, the incidence of hydrops fetalis was 1.80 per 1,000 total births (82 cases of 45,499 total births). Thirty-nine cases (47.56%) were idiopathic, 30 cases (36.58%) were Hb Bart's hydrops fetalis, and three (3.66%) cases were caused by congenital infection. The others 10 cases (12.19%) were achondrogenesis, Turner syndrome, twin-to-twin transfusion syndrome, severe anemia with unknown primary cause, cystic hygroma, multiple congenital anomalies, and Rh isoimmunization. The mortality rate of hydrops fetalis in the present series was 98.78%. One case, caused by Rh isoimmunization, survived. Maternal complications were 30 cases (36.59%) consisting of preeclampsia, preterm labor, disseminated intravascular coagulopathy, placenta previa, and postpartum hemorrhage. CONCLUSION: The incidence of hydrops fetalis was 1.80 per 1,000 total births. The common known cause was Hb Bart's hydrops fetalis. The mortality rate of hydrops fetalis in the present study was very high.

Congenital cystic adenomatoid malformation: monitoring the antenatal and short-term neonatal outcome.

OBJECTIVE: To determine the antenatal and short-term neonatal outcome of antenatally detected congenital cystic adenomatoid malformation (CCAM). METHODS: A retrospective review was conducted on all women with an antenatal diagnosis of CCAM who attended the Royal Women's Hospital, Melbourne, between January 1995 and December 2005. RESULTS: An antenatal diagnosis of CCAM was made in 38 singleton pregnancies. Serial ultrasounds were performed in 34 cases. Thirteen lesions (38%) appeared to resolve, ten lesions (29%) appeared to decrease, four lesions (12%) appeared to remain unchanged and seven lesions (21%) appeared to increase in size. Four pregnancies (10.5%) were complicated by hydrops fetalis which was associated with a poor outcome. Thirty-seven babies were liveborn. Seven babies (18.9%) developed respiratory distress. Two of these babies died within two days of birth resulting in three deaths in total. Two babies were lost to follow up (n=33). Sixty per cent of babies in whom the CCAM appeared to resolve on antenatal ultrasound had an abnormal chest X-ray (CXR). All computed tomography (CT) scans (eight of eight) in this group were abnormal. Of the surviving babies, 27.3% (nine of 33) have had surgery to date. CONCLUSIONS: The antenatal diagnosis of CCAM is associated with a good short-term prognosis. The pregnancy should initially be managed at a tertiary centre with serial ultrasound. Asymptomatic babies should have a postnatal CT even if the CCAM appears to have resolved or decreased on antenatal ultrasound.

Implementation of routine screening for Kell antibodies: does it improve perinatal survival?

BACKGROUND: In 1998 a national program for first-trimester screening for red cell (RBC) antibodies in all pregnant women was implemented. The aim of our study was to assess the impact on perinatal mortality caused by Kell alloimmunization STUDY DESIGN AND METHODS: Prospectively collected data on all pregnant women referred to our center from 1988 until 2005 for intrauterine transfusion (IUT) for fetal anemia due to Kell alloantibodies were analyzed. The cohort was divided into two groups, those treated before 1998 and those treated after 1998. The primary outcome was fetal and neonatal survival. Secondary outcome variables were gestational age, fetal hemoglobin (Hb) levels at first IUT, severity of hydrops, and total number of IUTs per pregnancy. Causes for mortality were analyzed in detail. RESULTS: A total of 43 pregnancies were included, 18 before introduction of screening and 25 thereafter. Perinatal survival increased from 61 percent in the first period to 100 percent after introduction of screening. After 1998, fetal hydrops was generally less severe at first IUT, while gestational age and fetal Hb levels at first IUT were similar. CONCLUSION: Implementation of routine screening for Kell antibodies in pregnancy was associated with an increased referral rate for suspected fetal anemia, more timely referrals, and a higher perinatal survival rate after intrauterine treatment.

Isolated fetal hydrothorax with hydrops: a systematic review of prenatal treatment options.

OBJECTIVE: To evaluate the effect of prenatal therapeutic interventions on perinatal outcome in pregnancies complicated by isolated fetal hydrothorax with hydrops. METHODS: A systematic review of the literature from January 1982 to January 2006 of perinatal outcome in pregnancies with isolated fetal hydrothorax with hydrops with any form of prenatal treatment was conducted. RESULTS: Forty-four articles met our selection criteria, reporting a total of 172 fetuses treated prenatally. Reported treatment options were single (n = 13) or serial thoracocentesis (n = 18), thoraco-amniotic shunt placement (n = 100) or a combination of thoracocentesis and shunting (n = 36). Four case-reports described pleurodesis with OK-432, (n = 3) and intrapleural injection of autologous blood (n = 2). Overall survival rate was 63%, ranging from 54% for single thoracocentesis to 80% in the 5 cases treated with pleurodesis, without statistically significant differences between the treatment modalities. Shunt-placement with or without prior thoracocentesis was most often described, with survival rates of 67 and 61% respectively. DISCUSSION: The available literature consists exclusively of case reports and case series. This systematic review suggests that with prenatal intervention, perinatal survival rates around 63% are possible. There is a need for prospective, adequately controlled studies with long-term follow-up to determine the best treatment and more reliable outcome data in pregnancies complicated by fetal hydrothorax with hydrops.

[Hydrops fetalis--a retrospective study]. / Hydrops foetus universalis, visszatekinto tanulmány.

In a retrospective study, the authors examined the occurrence of hydrops in foetal and neonatal cases in a five-year period. During this time, the clinical and pathologic diagnosis of hydrops was established in 28 cases. In three cases, the hydrops was caused by Rh incompatibility, and in 25 cases non-immune hydrops was discovered by clinical and pathologic examination. The cause of hydrops was recognised in 25 cases and no underlying disease was discernible in three. In 12 cases, the pregnancy was spontaneously terminated. Artificial abortion was performed in four cases. In this series, four hydropic newborns died on the first week of their life. The average age in artificial abortion was 16-20 gestational week and 24 in spontaneous abortion or stillbirth. Pathologic examination revealed increased weight, oedema, ascites, and hydrothorax in all cases. In half of the cases, there was also hydropericardium. Hepatosplenomegaly, cardiomegaly, pulmonary hypoplasia, increased extramedullary hemopoiesis, and placenta oedema were seen in all the cases. Causes of the non-immune hydrops were cardiac malformation in 4 cases, chromosome anomaly in 3, cystic hygroma in 2, skeletal anomaly in 1, foeto-foetal transfusion in 3, infection in 7 and sacrococcygeal teratoma in 1 case. In two cases, the underlying disease was reported to be of maternal origin. Comparing the clinical and pathologic findings there were only three cases with absolute agreement. The underlying disease revealed by pathologic examination was regarded to be significant from respect of inheritance, therapeutic approach, and further management in 10 cases. The authors emphasise the importance of pathologic examinations in foetal hydrops and point out the significance of clinico-pathological collaboration in the decision about further management of hydropic foetuses.

Large fetal congenital cystic adenomatoid malformations: growth trends and patient survival.

PURPOSE: The prognosis for fetuses with large congenital cystic adenomatoid malformations (CCAMs) remains uncertain. This study examined the natural history of large fetal CCAMs managed expectantly at a major referral center. METHODS: A 5-year retrospective review was conducted on fetuses diagnosed with a thoracic lesion (n = 59). Large CCAMs were identified on prenatal imaging and followed longitudinally. Perinatal outcomes were assessed. RESULTS: Twelve (20.3%) fetuses had large CCAMs in the absence of other congenital anomalies. Peak CCAM size occurred at 25.3 +/- 3.6 weeks' gestation. Serial magnetic resonance volumetry demonstrated a trend toward decreasing CCAM mass volume relative to thoracic cavity volume over time. Overall, 6 patients, including 3 with signs of early hydrops, showed a marked regression of their lesions relative to estimated fetal weight. Five fetuses required an emergent intervention postnatally, including extracorporeal membrane oxygenation support (n = 1), cyst aspiration (n = 1), and lung resection (n = 5). Overall survival was 75%, with severe hydrops before 30 weeks seen in all 3 deaths. CONCLUSION: Large fetal CCAMs tend to peak in size at 25 weeks' gestation and are characterized by in utero diminution relative to overall fetal growth. The prognosis for most fetuses with large CCAMs remains quite favorable under careful perinatal management.

Hidrops fetal no inmune: presentación de un caso y revisión de la literatura / No immune fetal hydrops: a case report and literature review

El hidrops fetal no inmune (HFNI) es una importante causa de pérdida perinatal, con mortalidad que varía entre 50-100 por ciento. El HFNI es una condición causada por un grupo heterogéneo de patologías. La fisiopatología del desorden que lo produce se conoce en muchos casos. Sin embargo, existen muchos casos en que la causa no se puede detérminar. Presentar un caso de hidrops fetal no inmune, el estudio realizado y la revisión de la literatura. Un caso de hidrops fetal no inmune, fue diagnósticado por ultrasonido antenatal a las 298 semanas de gestación. El feto murió al nacer, el cariotipo de muestra de sangre obtenida del cordón umbilical fue anormal. El examen postmorten fue compatible con Síndrome de Tuner e Higroma Quístico. En el presente caso el HFNI fue causado por la cromosomopatía tipo monosomía X y la anatomía linfática denominada higroma quístico. Todos los casos de HFNI deben ser evaluados prenatalmente para un adecuado diagnóstico y tratamiento cuando la mortalidad es prevenible.

Non-immune hydrops fetalis: prenatal diagnosis and perinatal outcomes.

PURPOSE: To describe the perinatal outcomes after a prenatal diagnosis of non-immune hydrops fetalis (NIHF). METHODS: Retrospective case series. University of Mississippi ultrasound database from July 2000 to January 2003 was reviewed for cases of NIHF. Records abstracted for maternal demographics and neonatal outcomes. RESULTS: Thirty-one cases of NIHF were identified and full data was available for 29 cases. Median gestational age at diagnosis was 17.3 weeks. Structural fetal malformations were noted in 22 cases (76%); most common were cystic hygroma (N = 17, 59%), followed by cardiac (N = 10, 34%). Amniocentesis was performed in 20 cases and 10 were aneuploid. Only 10 fetuses (34%) were liveborn and only five (17%) survived the neonatal period. CONCLUSIONS: Antepartum diagnosis of NIHF conveys a grave perinatal prognosis. Associated anomalies can be expected in approximately 75% of cases. Even for those fetuses achieving viability, survival beyond the neonatal period is rare.