Etiology and Outcome of Isolated Fetal Ascites: A Systematic Review.

OBJECTIVE: To describe the etiology of isolated fetal ascites and associated perinatal outcomes, and to assess the progression of isolated fetal ascites to fetal hydrops. DATA SOURCES: PubMed, Cochrane Library, Scopus, and ClinicalTrials.gov databases were searched using the following keywords: "fetus" OR "foetal" OR "fetal" OR "foetus" AND "ascites" from inception to February 2020. The search was limited to the English language. METHODS OF STUDY SELECTION: A total of 1,983 articles were identified through the search strategy. All studies containing five or more cases of isolated fetal ascites were included. TABULATION, INTEGRATION, AND RESULTS: Eleven studies, involving 315 cases of isolated fetal ascites, were eligible for inclusion in this systematic review. All included studies were evaluated using the tool for evaluating the methodologic quality of case reports and case series described by Murad et al. Data were summarized using narrative review and descriptive statistics. Two-tailed Fisher exact P values calculated from hypergeometric distribution were used to compare outcome by etiology. CIs were calculated with Clopper-Pearson exact binomial interval. The etiologies of isolated fetal ascites are genitourinary (24%), gastrointestinal (20%), viral or bacterial infections (9%), cardiac (9%), genetic disorders not otherwise categorized (8%), chylous ascites (6%), metabolic storage disorders (3%), other structural disorders (4%), other causes (4%) and idiopathic (13%). Survival is most favorable for cases of isolated fetal ascites as a result of chylous (100%), idiopathic (90%), gastrointestinal (77%) and genitourinary (77%) etiologies. Survival is least favorable for fetuses with isolated fetal ascites as a result of structural disorders (25%), cardiac etiology (32%) and metabolic storage disorders (33.3%). When pregnancy terminations were excluded, survival rates were similar between fetuses diagnosed at or after 24 weeks of gestation compared with those diagnosed at less than 24 weeks (74% vs 61%, P=.06). Progression of fetal ascites to fetal hydrops occurred in 6.6% (95% CI 3.6-9.6%) (17/259) of cases when pregnancies that were terminated were excluded. CONCLUSION: Isolated fetal ascites has a diverse etiology. Outcome is related to the etiology of isolated fetal ascites. In the majority of cases, fetal ascites does not progress to fetal hydrops. SYSTEMATIC REVIEW REGISTRATION: PROSPERO, CRD42020213930.

Prenatal sonographic diagnosis of isolated fetal ascites in cri-du-chat (5p-) syndrome: A case report.

Isolated fetal ascites and cri-du-chat syndrome (CdCS; OMIM #123450) are two very rare conditions that, to our best knowledge, have never been reported together. Here, we describe a case of isolated fetal ascites detected in the first trimester ultrasound, with no other remarkable signs. After an extensive work-up (fetal ultrasound, serologies, Coombs test, and NIPT), an amniocentesis was performed and revealed an abnormal karyotype of 46,XX,del(5)(p15.2), characteristic of CdCS. We hypothesize that isolated fetal ascites has to be considered an antenatal ultrasonographic marker for CdCS, a finding that should be confirmed in further cases.

Ascitis fetal aislada idiopática / Idiopathic isolated foetal ascites

La ascitis fetal aislada es un cuadro poco frecuente, ya que en la mayoría de ocasiones se diagnostica formando parte del cuadro de hidrops fetal. Presentamos el caso de una secundigesta de 31 años y 32 semanas y 5 días, que acude a urgencias por el hallazgo de polihidramnios y ascitis fetal masiva aislada en una ecografía rutinaria. La gestación había tenido una evolución normal hasta el momento. Como dato relevante presentó una translucencia nucal (TN) en el primer trimestre por encima del percentil 99, con un estudio de aneuploidías y ecocardiografía precoz normales, y una ecografía a las 20 semanas sin hallazgos patológicos. Una vez ingresada la paciente se indica tocólisis, maduración pulmonar fetal, se completa el estudio ecográfico morfológico fetal, Doppler fetal incluyendo pico de velocidad sistólica de la arteria cerebral media, anticuerpos irregulares, serologías TORCH y parvovirus B19, amniocentesis diagnóstica (estudio de infecciones congénitas) y evacuadora. Las pruebas realizadas resultaron negativas, por lo que se diagnosticó de ascitis fetal aislada, finalizándose la gestación mediante cesárea a las 33 semanas y 3 días por un registro cardiotocográfico (RCTG) patológico. La ascitis no tuvo repercusión en el desarrollo neonatal

Isolated foetal ascites is uncommon since, in most instances, it is diagnosed as being part of a clinical condition known as hydrops fetalis. We report the case of a 31-year-old secundigravida of 32 weeks and 5 days who came to A&E due to the finding of isolated massive foetal ascites and polyhydramnios in a routine ultrasound. The pregnancy had had a normal course until this point. Relevant data include a nuchal translucency (NT) in the first trimester above the 99th percentile with aneuploidy screening, normal early echocardiography and ultrasound at 20 weeks without pathological findings. Once the patient was admitted to hospital, tocolysis and foetal lung maturation were indicated as well as completion of a foetal morphological ultrasound study, foetal Doppler including middle cerebral artery peak systolic velocity, irregular antibodies, TORCH and parvovirus B19 serological tests, diagnostic (study of congenital infections) and reduction amniocentesis. The tests were negative; therefore the diagnosis was isolated foetal ascites. The pregnancy was terminated by caesarean section at 33 weeks and 3 days due to pathological cardiotocography (CTG) findings. The ascites had no effect on neonatal developmenT

Isolated ascites in a newborn with 'apple peel' jejunal atresia.

Isolated fetal ascites was diagnosed at 20 weeks in a primiparous woman with no significant medical history. Progressive fetal ascites worsened after 28 weeks and resulted in fetal hydroceles. Delivery was by caesarian section at 33 weeks, preceded by reduction of fetal ascites under ultrasound guidance. Following delivery, the baby required further reduction of abdominal fluid and endotracheal intubation to provide respiratory support. An extensive set of investigations, including metabolic and genetic screening, was performed; all results were negative. On day two of life, the baby developed bilious aspirates and an abdominal radiograph suggested intestinal obstruction. At laparotomy, an 'apple peel' jejunal atresia, abnormal mesentery with precarious blood supply and a proximal perforation were identified and the perforation 'sewn over'. The postoperative course was unremarkable, with Monogen feeds tolerated three weeks later. The baby continued to thrive at one year, tolerating increasing amount of long-chain fatty acids in diet.

Meconium Peritonitis: Correlation of Antenatal Diagnosis and Postnatal Outcome - An Institutional Experience over 10 Years.

OBJECTIVE: To identify the fetal and neonatal imaging characteristics of meconium peritonitis (MP) and their clinical outcome. We also studied the role of prenatal ultrasound (US) in antenatal diagnosis and its use in predicting the need for surgical intervention postnatally. MATERIAL AND METHODS: We conducted a retrospective analysis of a cohort of 18 infants with MP from April 2004 to March 2014. RESULTS: Prenatal US detected MP-related abnormalities in 15/18 (83.3%) fetuses. The median gestational age at initial diagnosis of MP was 24 weeks (range 19-31). Fetal ascites (93.3%) was the most common prenatal US finding. Of the 18 infants, 12 (66.7%) required surgical intervention. The overall survival rate was 94.4%. All infants with a prenatal US scan showing meconium pseudocyst or bowel dilatation required surgical intervention postnatally. DISCUSSION: A combination of ascites, intraperitoneal calcification, and echogenic bowel on fetal US raises a high suspicion of MP. Surgical intervention is indicated in the presence of meconium pseudocyst on fetal or postnatal US scan. Antenatal US has high specificity (100%) but low sensitivity (22.2%) in detecting meconium pseudocyst. A favorable outcome can be expected with early antenatal diagnosis and timely surgical intervention in a tertiary hospital.

Can we select fetuses with intra-abdominal calcification for delivery in neonatal surgical centres?

BACKGROUND: Prenatal ultrasound (US) diagnosis of fetal intra-abdominal calcification (iAC) is frequently caused by an in utero perforation causing meconium peritonitis. Our ability to predict which fetuses will require postnatal surgery is limited. The aim of our study is to correlate iAC and associated US findings with postnatal outcome. METHODS: A single centre retrospective review of all cases of fetal iAC diagnosed between 2004 and 2010 was performed. Maternal demographics, fetal US findings, and outcomes (need for surgery and mortality) were collected. Descriptive and comparative statistical analyses were performed. RESULTS: Twenty-three cases of iAC were identified. There were no cases of fetal demise or postnatal deaths. Three liveborns (13%) required abdominal surgery at a median of 2 days (0-3) for intestinal atresia. US findings of iAC and dilated bowel with (p=0.008) or without (p=0.005) polyhydramnios predicted a need for postnatal surgery as did the combination of iAC, polyhydramnios, and ascites (p=0.008). Conversely, iAC alone or associated with oligohydramnios, polyhydramnios, ascites, or growth restriction did not predict need for postnatal surgery. CONCLUSION: The majority of fetuses with iAC on prenatal US do not require surgery. Associated US findings (bowel dilation) can be used to select fetuses for delivery in neonatal surgical centres.

Fetal obstructive uropathy complicated by urinary ascites: outcome and prognostic value of fetal serum ß-2-microglobulin.

OBJECTIVES: To determine whether the prognostic value of fetal serum ß-2-microglobulin is altered and whether the occurrence of fetal urinary ascites prevents kidney damage in cases of fetal obstructive uropathy with urinary ascites. METHODS: This was a retrospective study of cases of fetal bilateral obstructive uropathy that occurred between 2006 and 2010, for which both fetal serum and ascites samples were sent to our laboratory for analysis. ß-2-microglobulin was assayed in both fetal serum and the corresponding ascites. Renal outcome was analyzed. Histological features of the kidney in cases of termination of pregnancy and renal function of liveborn infants were recorded. RESULTS: Fourteen cases with analysis of fetal serum and fetal ascites in a context of urinary obstruction were included. Renal outcome was unfavorable in eight cases (57%) and favorable in six (43%). When fetal serum ß-2-microglobulin was < 5 mg/L, renal outcome was favorable in all cases (4/4). When fetal serum ß-2-microglobulin was ≥ 5 mg/L, 8/10 cases (80%) had an unfavorable renal outcome (sensitivity, 100%; specificity, 66%). CONCLUSION: Fetal serum ß-2-microglobulin reliably predicts postnatal renal outcome in obstructive uropathy complicated by urinary ascites. Moreover, urine extravasation does not seem to protect fetal renal function.

Hepatitis E virus infection causing isolated fetal ascites: a case report.

Maternal hepatitis infection, excepting hepatitis E, causing isolated fetal ascites with variable outcome has been reported previously. We present a case of maternal hepatitis E virus (HEV) infection causing isolated fetal ascites which resolved spontaneously during pregnancy and resulted in a term live-born baby with anti-HEV seropositivity. A 39-year-old primigravida woman was diagnosed with acute HEV infection at 15 weeks of gestation. Ultrasound at 19 weeks showed significant fetal ascites with abdominal calcifications. Fetal karyotype did not show any abnormality. Cord blood was positive for anti-HEV IgM and negative for other intrauterine infections. Ultrasound at 25 weeks showed partial resolution of fetal ascites with complete resolution at 30 weeks. She delivered a healthy baby at 38 completed weeks, with normal liver enzymes at birth and 1-month follow-up.

In utero incarceration of congenital diaphragmatic hernia.

In utero diagnosis of incarcerated congenital diaphragmatic hernia has never been reported. In our case, congenital diaphragmatic hernia presented at 34 weeks of gestation with dilated bowel loops, pleural effusion, and ascites on fetal ultrasound. Preterm delivery and emergency exploration revealed a tight posterolateral diaphragmatic defect with extensive bowel infarction.

The prognostic factors and the outcome of primary isolated fetal ascites.

PURPOSE: The purpose of the present study was to evaluate the prognostic factors and review the outcome of primary isolated fetal ascites. METHODS: A retrospective cohort study was conducted for fetuses with primary isolated ascites with a prenatal diagnosis between 1994 and 2009. The patients were divided into the favorable group (Group I) whose ascites were resolved by medical treatment alone and an unfavorable group (Group II) who required surgical intervention after birth due to refractory ascites. RESULTS: There were seven patients in Group I and five patients in Group II. Six of seven patients who developed ascites after 30 weeks' gestation were categorized in Group I, and four of five infants who developed ascites before 30 weeks' gestation were categorized in Group II. There was a negative correlation between the gestational age at diagnosis and the severity of the fetal abdominal distention. In Group II, the ascites resolved in two cases and was reaccommodated in another two cases after surgery. An infant with trisomy 21 received continuous drainage and eventually died of infection. CONCLUSIONS: The prognosis of primary isolated fetal ascites can be predicted based on the gestational age at diagnosis and the severity of the fetal abdominal distention.

Successful management of a large fetal mediastinal teratoma complicated by hydrops fetalis.

This report describes a case of fetal mediastinal teratoma complicated by hydrops fetalis managed successfully by aspiration of the tumor cyst fluid. Fetal mediastinal teratomas are rare tumors that cause hydrops fetalis or fetal demise in the prenatal period and respiratory distress in the neonatal period. The patient presented with a large cystic mass in the thoracic cavity complicated by hydrops fetalis. The hydrops resolved after fetal aspiration of the tumor cyst fluid. The infant was born without respiratory distress, and tumor resection was performed at the age of 30 days. The postoperative course was uneventful, and the patient was in good health 6 months postoperatively.

[Impact of fetal urinary ascites on serum beta2 microglobuline in obstructive uropathies: a case report]. / Impact de l'ascite urinaire foetale sur le taux de beta2 microglobuline sérique dans les uropathies obstructives : à propos d'un cas.

We report a posterior urethral valves case diagnosed at 33 week's gestation on a fetus presenting with anamnios and urinary ascites. In this fetus, the serum beta2 microglobuline rate was high, suggesting a very poor renal prognosis. At 1-year-old, the creatinine rate is nearly normal. In case of urinary ascites, the serum beta2 microglobuline rate could be improved in relation with the transperitoneal reabsorption of this protein.

Umbilical arterial necrotic vasculopathy associated with fetal ascites.

Immune and nonimmune neonatal ascites may be part of hydrops fetalis or may be an isolated finding. However, a significant percentage of nonimmune ascites do not have an identifiable pathogenesis and are considered idiopathic. We report a case of fetal ascites and umbilical arterial necrotic vasculopathy, an association not previously described.

A rare case of congenital pulmonary lymphangiectasia, hydrothorax and ascites in a male embryo aborted at 20 weeks of gestation.

A case of a male embryo aborted at the 20th week of gestation with extensive ascites, hydrothorax, pulmonary lymphangiectasia and pulmonary hypoplasia is presented together with the pathological findings, the etiology, differential diagnosis, course and therapy of this pathologic entity. Also a short review of the literature is discussed.

Repeated paracentesis in a fetus with meconium peritonitis with massive ascites: a case report.

Meconium peritonitis (MP) is defined as a sterile inflammatory reaction in the fetal abdomen that is seen in cases of intrauterine bowel perforation. Recently, there have been increasing numbers of fetuses with MP prenatally diagnosed by ultrasonography. Massive fetal ascites in MP may cause hydrops and hypoplastic lungs. However, antepartum management of MP has not yet been established. We encountered a fetus with MP and massive ascites. Repeated paracentesis between 29 weeks and 4 days and 31 weeks and 6 days of gestation prevented the progression to fetal hydrops and hypoplastic lungs, which may occur due to massive meconium ascites with an increased preload index. Amniocentesis was also performed in patients with polyhydramnios for treatment of preterm labor. These observations suggest that aggressive therapy can prolong the gestation period and improve MP treatment outcomes.

Fetal urinary bladder rupture and urinary ascites secondary to posterior urethral valves. A case report.

Megacystis is a typical prenatal sonographic finding in cases of lower urinary tract obstruction. Urinary bladder perforation represents a rare complication in this condition. We report on a boy with in utero bladder perforation and urinary ascites secondary to posterior urethral valves. The pre- and postnatal therapy is described and the current literature is reviewed.

Prenatal DNA diagnosis of Noonan syndrome in a fetus with massive hygroma colli, pleural effusion and ascites.

Prenatal molecular genetic diagnosis for Noonan syndrome I is reported. Noonan syndrome was suspected because of large cystic hygroma colli, massive pleural effusion and ascites at 23 weeks of gestation and normal karyotype (46,XX). DNA was prepared from amnion cells and screened for mutations in the PTPN11 gene. In exon 8, a missense mutation (S285F) was found. Delivery was induced at 33 weeks of gestation because of silent cardiotocography (CTG). Despite immediate drainage of the hydrothorax, mechanical ventilation was insufficient and the child died 9 h after birth due to severe pulmonary hypoplasia. Pleural punctate was enriched for small lymphocytes and thus was characterized as chylus. Prenatal ultrasound findings in Noonan syndrome usually are unspecific and rarely lead to a diagnosis. However, with the combination of cystic hygroma, pleural effusion, ascites and normal karyotype Noonan syndrome should be considered and DNA testing for PTPN11 mutations may be appropriate. Malformations of lymphatic vessels and/or chylothorax in Noonan syndrome seem to be more frequent than usually anticipated.

Effect of chronic hypoxia during embryonic development on physiological functioning and on hatching and post-hatching parameters related to ascites syndrome in broiler chickens.

The present study was designed to investigate the effect of different atmospheric pressure on the endogenous functions of broiler chickens during embryonic, hatching and growing periods related to ascites. Eggs from a commercial broiler line were incubated in two similar commercial incubators at high and low altitudes. The effect on embryonic development and physiological functions including hatching parameters, incidence of ascites and growth performance were examined. Embryos incubated at high altitude had higher plasma tri-iodothyronine, thyroxine, corticosteroid and lactic acid levels, and hatched earlier than those incubated at low altitude. Embryonic mortality was higher at high altitude. Chickens that had been incubated at high altitude showed less right ventricular hypertrophy and ascites mortality than those incubated at low altitude. It was concluded that different atmospheric pressure during incubation interacts with the endocrine functions of the embryo and hence affects hatching parameters, thereby influencing ascites susceptibility.

Multi-system cytomegalovirus fetopathy by recurrent infection in a pregnant woman with hepatitis B.

A pregnant woman with acute hepatitis B virus (HBV) infection had her second pregnancy terminated at 25 weeks' gestation because of fetal ascites and ventriculitis. Meconium peritonitis was also found at autopsy. No HBV DNA but cytomegalovirus (CMV) DNA was detected in the fetal liver and ascitic fluid. Recurrent maternal CMV infection was demonstrated by pre-existing CMV IgG antibodies, high IgG avidity and low IgM levels. After abortion, the patient developed chronic active hepatitis. Nevertheless, having become pregnant again with a new partner, she had an uneventful third pregnancy and gave birth to a healthy boy.

Bilateral renal agenesis and fetal ascites in association with partial trisomy 13 and partial trisomy 16 due to a 3:1 segregation of maternal reciprocal translocation t(13;16)(q12.3; p13.2).

A female fetus with bilateral renal agenesis and fetal ascites was found to have partial trisomy 13 (pter-q12.3) and partial trisomy 16 (p13.2-pter), 47,XX,+der(13)t(13;16)(q12.3; p13.2)mat. The chromosomal aberration was due to a 3:1 segregation with tertiary trisomy transmitted from a maternal reciprocal translocation 13;16. Prenatal ultrasound of a 29-year-old, gravida 2, para 0 woman at 22 gestational weeks showed fetal ascites, severe oligohydramnios and non-visualization of fetal urinary bladder and kidneys. The pregnancy was terminated. At delivery, the proband displayed dysmorphic features of hypertelorism, a prominent glabella, epicanthic fold, a stubby nose with a depressed nasal bridge, anteverted nares, thin lips, micrognathia, low-set ears, a short neck and a distended abdomen. Necropsy confirmed bilateral renal agenesis and ascites. A cytogenetic study performed on fibroblasts obtained from the proband's skin revealed an extra supernumerary chromosome. The mother was later found to have a reciprocal translocation. Fluorescence in situ hybridization for a submicroscopic deletion in chromosome 22q11 in the proband was negative. The parents had no urological anomalies. Our observation further extends the clinical spectrum associated with proximal trisomy 13q and distal trisomy 16p. We suggest prenatal cytogenetic analysis in fetuses with urological anomalies, including renal agenesis, to uncover underlying genetic disorders.