Diagnostic approach to fetal ventriculomegaly.

Ventriculomegaly (VM) is defined as an enlargement of the lateral ventricles of the developing fetal brain. The diagnosis is easily made by measuring the lateral ventricle width at the level of the atrium, which is normally <10 mm. VM is defined as mild when the atrial width is 10-12 mm, moderate 12-15 mm, severe >15 mm. VM is a non-specific sonographic sign which is common to different pathological entities and genetic conditions. When no associated anomaly can be found VM is defined as isolated. Since the prognosis of fetal VM mainly depends on the presence of associated anomalies, a careful diagnostic approach is necessary to rule out CNS and extra- CNS fetal anomalies. Magnetic Resonance Imaging can be a useful diagnostic tool complementary to ultrasound in order to recognize subtle brain anomalies, particularly cortical disorders. In this review the diagnostic approach to fetal VM will be discussed starting from ultrasound screening, moving to neurosonographic and MRI examination and genetic evaluation, in order to recognize the cause of VM and offer the appropriate counselling to the parents.

Diagnosis of chorionicity: the role of ultrasound / Diagnóstico de corionicidad: papel de la ecografía

La corionicidad es el principal determinante del desenlace perinatal en los embarazos gemelares: la morbimortalidad perinatal es significativamente superior en los gemelos monocoriónicos que en los bicoriónicos. Esto se debe principalmente a las complicaciones asociadas con la corionicidad, a saber, el síndrome de transfusión fetal-fetal (STFF), la restricción del crecimiento fetal selectivo (CIR) y la perfusión arterial reversa (secuencia TRAP), que son debidas a anastomosis vasculares intergemelares en la placenta común. Por esta razón, el diagnóstico de corionicidad en gemelos es de importancia clínica, ya que así se podrá planificar una mayor vigilancia en gestaciones monocoriónicas y detectar la aparición de complicaciones en las primeras etapas. Distintos signos ecográficos sirven para evaluar la corionicidad: número de masas placentarias, sexo de los fetos, características de la membrana intergemelar. Esto último es, sin duda, la herramienta más útil y valiosa: la separación de la membrana de la superficie placentaria muestra una típica forma «lambda» en el embarazo bicoriónico y un típico signo en «T» en el monocoriónico. En este artículo se revisan las características ecográficas que ayudan a la descripción exacta de la corionicidad (AU)

Chorionicity is the main determinant of the perinatal outcome in twin pregnancies: perinatal mortality and morbidity are significantly higher in monochorionic versus dichorionic twins. This is mainly due to complications associated specifically with monochorionicity, such as twin to twin transfusion syndrome (TTTS), selective fetal growth restriction (FGR) and twin reverse arterial perfusion syndrome (TRAP), consequences of the presence of inter-twin vascular anastomoses in the common placenta. For this reason the diagnosis of chorionicity in twins is of clinical importance in order to plan an increased surveillance in monochorionic gestations and to recognize the appearance of complications in their early stages. Different sonographic signs may be used to evaluate chorionicity: number of placental masses, sex of the fetuses, characteristics of the intertwine membrane. The last one is surely the most useful and valuable tool: the take-off of the membrane from the placental surface shows the typical "lambda" appearance in dichorionic pregnancy and the typical "T" appearance in the monochorionic ones. In this article the sonographic features that help in the accurate depiction of chorionicity are reviewed (AU)

Survival outcomes of twin-twin transfusion syndrome stage I: a systematic review of literature.

OBJECTIVE: To review literature about treatment of twin-twin transfusion syndrome (TTTS) stage I. MATERIALS: A search in PubMed, EMBASE, Medline, and reference lists was performed. Inclusion criteria were TTTS diagnosed with standard criteria and classified with Quintero staging and survival rates (SR) stratified for stage and first-choice treatment. Outcomes were SR and progression to advanced stages. Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines were followed. RESULTS: Seven articles pooled 262 twin sets treated with amnioreduction (16%), conservative management (22%), and laser therapy (62%). The overall SR was 77% after amnioreduction, 86% in the conservative management group, and 85% in the laser therapy group. Progression rate occurred in 30%, 15%, and 0%, respectively. Outcomes were similar between laser therapy as first- versus second-choice treatment. Because most studies were observational and not comparative, a meta-analysis was not performed. CONCLUSION: Conservative management of TTTS stage I is a reasonable option until randomized clinical trials are presented.

Neonatal outcomes of assisted and naturally conceived twins: systematic review and meta-analysis.

OBJECTIVE: To review the neonatal outcomes of assisted reproductive technique (ART) vs. naturally conceived (NC) twins. METHODS: A search in PubMed, Medline, EMBASE, and Cochrane library was performed (January 2000-September 2010). Articles reporting outcomes of ART vs. NC twins were classified in controlled and non-controlled studies. A sub-group was limited to unlike sex twins (UST). The MOOSE guidelines were followed. A randomized model was generated if inter-studies heterogeneity was >25%. Inter-group comparison was significant if P<0.05. RESULTS: Thirteen articles were reviewed. In non-controlled studies, outcomes were equal between groups, except for cesarean section rates (ART: 71.8%; NC: 49.6%; P<0.0001; OR: 2.34; 95% CI: 1.55-3.54). Similar results were obtained in controlled studies, except for higher rates of preterm delivery in ART (55%) than in NC (53%) twins (P=0.03; OR: 1.30; 95% CI: 1.03-1.65). UST differed with regard to preterm delivery (ART: 58.6%; NC: 49.5%; P<0.0001; OR: 1.51; 95% CI: 1.25-1.83), and perinatal death (ART: 3.6%; NC: 1.8%; P<0.0001; OR: 1.95; 95% CI: 1.41-2.71). Birth weight differed between groups. CONCLUSION: ART twins carry increased risk of adverse outcomes. UST are at higher risk of perinatal death, although further studies are needed to assess whether chorionicity impacts on neonatal outcomes after ART.

Laparoscopic diagnosis and treatment of pelvic benign multicystic mesothelioma associated with high CA19.9 serum concentration.

We report a case of benign multicystic mesothelioma in a 20-year-old woman referred because of amenorrhea. She underwent pelvic transabdominal ultrasound, which disclosed a micropolycystic appearance of the ovaries and a fluid collection in the pouch of Douglas. Tumor serum markers revealed an increase in CA19.9. Abdominal and pelvic computed tomography scans confirmed the presence of ascites. Laparoscopy disclosed small, thin-walled, translucent cysts in the Douglas cavity. The cysts were free-floating in a yellowish, sticky, gelatinous material. Microscopically, cystic lesions showed mesothelium-lined cystic spaces surrounded by a delicate thin fibrovascular wall. With immunohistochemistry, the tumor cells were strongly positive for cytokeratin and calretinin. These aspects were suggestive of benign multicystic mesothelioma. Electron microscopy confirmed the mesothelial nature of this tumor. Serial evaluation of the CA19.9 concentration showed a progressive decrease in the serum marker in the normal range. The patient is now well and symptom-free with no recurrence 24 months after surgery. The association between benign multicystic mesothelioma and increased CA19.9 serum concentration has been described only once, in a man. To our knowledge, this is the second case of benign multicystic mesothelioma associated with increased CA19.9 serum concentration and the first diagnosed in a woman. In the present case, a minimally invasive laparoscopic approach enabled not only histologic diagnosis of benign multicystic mesothelioma but also its surgical treatment. Although benign multicystic mesothelioma is a rare pathologic entity, it is important that sonologists include it in the differential diagnosis of diseases that manifest with ascites. Furthermore, all surgeons should be aware of the macroscopic and laparoscopic appearance of the lesion, and its generally benign course.

The efficacy of Quintero staging system to assess severity of twin-twin transfusion syndrome treated with laser therapy: a systematic review with meta-analysis.

Severity of twin-twin transfusion syndrome (TTTS) is classified in five stages according to Quintero staging. However, the efficacy of such staging was recently debated. We reviewed the efficacy of Quintero staging to predict survival rate in TTTS treated with laser therapy. Articles reporting survival rate for each stage in TTTS treated with laser therapy were reviewed. Number of twins alive per pregnancy (NAP) was compared between early (I + II) and advanced (III + IV) stages and within stages. Meta-analysis was performed according to Meta-analysis Of Observational Studies in Epidemiology guidelines. Heterogeneity was tested with chi-square for heterogeneity at a significance level of P < 0.10, and random or fixed models were generated as appropriate. A P value < 0.05 was considered statistically significant. NAP was similar between early (zero survivors: 34/228, 15%; one survivor: 49/228, 21%; two survivors: 145/228, 63%) and advanced stages (zero survivors: 38/214, 18%; one survivor: 64/214, 30%; two survivors: 112/214, 52%; P > 0.05) except for one survivor ( P < 0.05). A trend for increased NAP was observed in all stages. Because clinically relevant differences were not observed, laser therapy is the optimal treatment for all stages. As Quintero staging does not provide information about prognosis, a new staging system is proposed.

Umbilical cord occlusion for selective feticide in complicated monochorionic twins: a systematic review of literature.

The aim of this study was to review literature concerning selective feticide (SF) in monochorionic pregnancies complicated with twin-twin transfusion syndrome, twin reversed arterial perfusion, severe malformation, and discordant growth. The remaining twins' outcomes were stratified for indication and surgical technique. Three hundred forty-five cases of SF were reviewed. Premature rupture of membranes was described in all the procedures and complicated 76/345 (22%) pregnancies within 4 postoperative weeks (59%) or later (41%; P = .52). Fetal demise accounted for 51/345 (15%), more frequently within 2 postoperative weeks (79%) than thereafter (21%; P = .004; odds ratio [OR], 6.12; 95% confidence interval [CI], 1.81-20.70). Improved survival rate was achieved in surgeries after 18 weeks (89%) than earlier (69%; P = .02; OR, 0.28; 95% CI, 0.10-0.80) without differences for indication. Survivors were 86% after radiofrequency ablation, 82% after bipolar cord coagulation, 72% after laser cord coagulation, 70% after cord ligation. In spite of favorable outcomes, the optimal surgical approach remains undetermined.

Comparison of donor and recipient outcomes following laser therapy performed for twin-twin transfusion syndrome: a meta-analysis and review of literature.

We reviewed current literature about donor and recipient outcomes after laser therapy performed for twin-twin transfusion syndrome (TTTS). Study inclusion criteria were monochorionic diamniotic pregnancies, TTTS diagnosed with standard criteria, survival, and cerebral anomalies rates defined individually for donors and recipients. Exclusion criteria were studies including triplets and monoamniotic pregnancies, therapeutic techniques different from laser therapy, data reported in graphs or percentage, and non-English language publications. Meta-analysis was performed by random effects model whenever heterogeneity across studies was > 25%. Fifteen articles were reviewed. Survival rate was 60% in donors and 70% in recipients ( P = 0.0002; odds ratio: 0.66; 95% confidence interval: 0.53 to 0.82). Neurological morbidity did not differ between twins (donors: 9% versus recipients: 10%; P = 0.66). This review shows that recipients are more likely to survive as compared with donors, whereas no differences are noted with regard to neurological morbidity. It may be hypothesized that a lethal injury (to which donors appear to be more sensitive) or a cerebral damage (with which donors and recipients are equally affected) occurs before the timing of surgery. Therefore, a study protocol to assess whether diagnosis of TTTS may be anticipated in the first trimester is proposed.

Comparison of six sonographic signs in the prenatal diagnosis of spina bifida.

AIMS: To compare the diagnostic accuracy of sonographic signs that may be looked for in fetuses with spina bifida. METHODS: Forty-nine fetuses affected by spina bifida were enrolled, at a gestational age of 18-28 weeks. The following sonographic signs were looked for: "lemon" sign, small cerebellum, effaced cisterna magna, small posterior fossa, ventriculomegaly and direct visualization of a spinal defect. RESULTS: The "lemon" sign was present in 53%, a small cerebellum in 96%, an effaced cisterna magna in 93%, a small posterior fossa in 96%. Ventriculomegaly was present in 40/49 (81%) cases and was severe in 20 fetuses and borderline in the remaining 20. The spinal defect was missed in one fetus presenting the cerebellar and posterior fossa signs. In two fetuses, the myelomeningocele was present without cranial signs of Chiari II malformation and in both cases the defect was covered by intact skin. CONCLUSIONS: Our results confirm the usefulness of evaluation of the posterior fossa in the diagnosis of spina bifida, particularly in cases of small spinal defects that may be missed at ultrasound. Conversely, myelomeningocele covered by intact skin was not associated with the cranial signs of Chiari II malformation.

Maternal morbidity following a trial of labor after cesarean section vs elective repeat cesarean delivery: a systematic review with metaanalysis.

This study reviewed maternal morbidity following trial of labor (TOL) after cesarean section, compared with elective repeat cesarean delivery (ERCS). Articles were pooled to compare women planning vaginal birth after cesarean (VBAC) with those undergoing ERCS with regard to maternal morbidity (MM), uterine rupture/dehiscence (UR/D), blood transfusion (BT), and hysterectomy. The former group was subdivided into successful VBAC (S-VBAC) and failed TOL (F-TOL). VBAC was successful in 17,905 of 24,349 patients (73%). MM, BT, and hysterectomy were similar in women planning VBAC or ERCS, whereas UR/D was different (1.3%; 0,4%). MM, UR/D, BT and hysterectomy were more common after F-TOL (17%, 4.4%, 3%; 0.5%) than after S-VBAC (3.1%, 0.2%, 1.1%; 0.1%) or ERCS (4.3%, 0.4%, 1%; 0.3%). Outcomes were more favorable in S-VBAC than ERCS. These findings show that a higher risk of UR/D in women planning VBAC than ERCS is counterbalanced by reduction of MM, UR/D. and hysterectomy when VBAC is successful.

Laser therapy and serial amnioreduction as treatment for twin-twin transfusion syndrome: a metaanalysis and review of literature.

OBJECTIVE: The objective of the study was to review current controversy on laser therapy (LT) vs serial amnioreduction (SA) performed for twin-twin transfusion syndrome (TTTS). STUDY DESIGN: A search in PubMed from 1997-2007 was performed. Inclusion criteria were diamniotic monochorionic pregnancy, TTTS diagnosed with standard parameters, and peri- and neonatal outcomes well defined. Triplets and investigations on other topics of TTTS rather than perinatal outcomes were excluded. A metaanalysis was performed by fixed-effect model (heterogeneity <25%). RESULTS: Ten articles provided 611 cases of TTTS (LT: 70%; SA: 30%) and included 4 studies comparing the 2 treatments (395 cases: LT, 58%; SA, 42%). Fetuses undergoing LT were more likely to survive than fetuses undergoing SA (overall survival rate: P < .0001; odds ratio [OR], 2.04; 95% confidence interval [CI], 1.52-2.76; neonatal death: P < .0001; OR, 0.24; 95% CI, 0.15-0.40; neurologic morbidity: P < .0001; OR, 0.20; 95% CI, 0.12-0.33). CONCLUSION: This metaanalysis shows that LT is associated with better outcomes than SA and proposes new topics for future research.

Sonographic diagnosis of fetal cerebral ventriculomegaly: an update.

Dilatation of the fetal cerebral ventricles (ventriculomegaly) is a generic sonographic sign that is common to several pathological entities carrying different prognoses. The main causes of fetal ventriculomegaly are aqueductal stenosis, Chiari II malformation, Dandy-Walker complex, and agenesis of the corpus callosum. Ventriculomegaly is easily recognized by ultrasound by measuring the atrial width. This simple measure allows the recognition of mild forms of ventricular dilatation and is used in screening for ventriculomegaly. However, although the diagnosis of ventriculomegaly is easy, the prenatal identification of the cause of ventricular dilatation is a more difficult task. For this purpose the evaluation of the posterior fossa in association with the visualization of the corpus callosum is useful. Research into the causes of ventriculomegaly is clinically useful, since the prognosis mainly depends on the etiology and on the presence of associated abnormalities. In this article the role of prenatal sonography in determining the cause of the ventriculomegaly is reviewed, as well as the prognostic value of the prenatal sonographic findings.

Fetal thymic involution: a sonographic marker of the fetal inflammatory response syndrome.

OBJECTIVE: The purpose of this study was to evaluate whether there is a relationship between the sonographic fetal thymus size and the presence of an intrauterine infection in patients with preterm labor. STUDY DESIGN: Thirty-one women who had been admitted with preterm labor and intact membranes between 24 and 32 weeks of gestation were included. Fetal thymus perimeter was measured sonographically, and amniocentesis for the microbiologic assessment of the amniotic cavity was performed. Placentas and umbilical cords were examined for the presence of chorioamnionitis/funisitis. RESULTS: The prevalence of preterm delivery and intra-amniotic infection was 51.6% (16/31 women) and 32.3% (10/31 women), respectively. In all cases with intrauterine infection and in 23.8% of cases without intrauterine infection, the fetal thymus perimeter was below the 5th percentile for gestational age (10/10 women vs 5/21 women; P < .01). Isolated histologic chorioamnionitis and funisitis were found in 22.6% and 25.8% of fetuses, respectively. The fetal thymus was below the 5th percentile for gestational age in 100%, 71.4%, and 12.5% of patients with histologic signs of funisitis and isolated chorioamnionitis and without histologic signs of infection, respectively. CONCLUSION: Fetal thymus involution in preterm labor patients is strongly associated with funisitis, which is the histologic manifestation of the fetal inflammatory response syndrome.

The midsagittal view of the fetal brain: a useful landmark in recognizing the cause of fetal cerebral ventriculomegaly.

AIM: To evaluate the positive predictive value of the midsagittal view of the fetal brain in recognizing the cause of ventriculomegaly diagnosed with traditional axial scan. METHODS: Fifty-eight pregnant women, referred to our Center following a generic diagnosis of ventriculomegaly have been evaluated: 38 had marked and 20 had borderline ventriculomegaly. The fetal brain was scanned by the midsagittal view using a transabdominal probe in fetuses in breech presentation or transverse lie and a transvaginal probe in fetuses in cephalic presentation. The possible cause of ventriculomegaly was postulated by combining the findings of the corpus callosum/cavum septi pellucidi complex with those of the posterior fossa. The prenatal diagnoses were compared with the anatomical specimens of aborted fetuses or with postnatal neuroimaging. RESULTS: The prenatal diagnoses were confirmed in 54/58 cases (PPV 93.1%). In the marked ventriculomegaly group, one case of partial agenesis of the corpus callosum was mistaken for a complete agenesis. In the group of borderline ventriculomegaly, two cases of partial agenesis of the corpus callosum were confused with a complete agenesis, while one case of suspected isolated ventriculomegaly was diagnosed after birth as partial agenesis of the corpus callosum. CONCLUSIONS: The sagittal scan of the fetal brain is a useful source of information and allows the contemporary view of both corpus callosum and posterior fossa, where various typical sonographic findings are present in ventriculomegaly.

The role of ultrasonography in recognizing the cause of fetal cerebral ventriculomegaly.

Dilatation of the fetal cerebral ventricles (ventriculomegaly) is a generic sonographic sign common to several pathological entities carrying different prognoses. The main causes of fetal ventriculomegaly are aqueductal stenosis, Chiari II malformation, Dandy-Walker complex and agenesis of the corpus callosum. Ventriculomegaly is easily recognized by ultrasound by measuring the atrial width. This simple measure allows the recognition of mild forms of ventricular dilatation and is used as a screening method for ventriculomegaly. However, although the diagnosis of ventriculomegaly is easy, the prenatal identification of the cause of ventricular dilatation is a more difficult task. To this end, the evaluation of the posterior fossa in association with the visualization of the corpus callosum is a useful landmark. Research into the cause of ventriculomegaly is clinically useful, since the prognosis mainly depends on the etiology and on the presence of associated anomalies. In this article the role of prenatal sonography in recognizing the cause of ventriculomegaly and the prognostic value of the prenatal sonographic findings are discussed.