Antenatally Detected Choledochal Malformation: What Are We Waiting For?

INTRODUCTION: An increasing proportion of congenital choledochal malformation (CCM) are being detected on antenatal ultrasound. However, the actual timing of its surgical correction remains controversial with some series showing an excess of complications the earlier the operation. The aim of this study was to characterize the pathophysiological aspects of this cohort from the perspective of age at surgery in order to inform a more rational basis for clinical decision-making. METHODS: We analysed a prospective database of CCM acquired over a 26-year period (Jan. 1997 to Dec. 2022) for patient demography; details of pre- and post-natal imaging; age at surgical intervention; operative complications (Clavien-Dindo classification) and outcome. Data are quoted as median (IQR). All comparisons are non-parametric. A P value of 0.05 was accepted as significant. RESULTS: There were 58 (72% female) children with an antenatally-detected CCM from a total of 265 (21.8%) in the series. These were classified as Type 1C (n = 47; 81%), type 4C (n = 3; 5%) and Type 5 (n = 8; 14%). There were no Type 1F lesions in this cohort. Median age at surgery was 113 (IQR 57-198) days. Postnatal cyst (US) size varied from 12 to 130 mm but there was little evidence of a relationship between this and post-natal liver biochemistry (e.g. bilirubin rS = 0.01, P = 0.44; AST rS = 0.14, P = 0.19). Choledochal pressure measurements (n = 46) showed resting pressures of 12 (9-21) mmHg with no significant correlation with age (P = 0.4) or aspartate aminotransferase (P = 0.2) or γ-glutamyl transferase (P = 0.06). The cohort was divided into 2 groups (Early and Late) based on the median age at surgery (all open) (113 days). Biliary obstruction was more common in the Early group (10 vs. 2; P = 0.01). Two perforations occurred, both in the Early group. With a deliberate policy of regular ultrasound-based follow-up we have seen no anastomotic complications (leak, stenosis, persistent intrahepatic biliary dilatation or stones) or post-operative cholangitis in any child [median follow-up 3.42 (1.30-8.05) years]. CONCLUSIONS: This is one of the largest series documenting the outcomes of antenatally-detected CCMs, certainly in Europe and North America. Such lesions are invariably cystic in nature, and either Types 1C, 4C or 5. The absence of complications using a policy of early intervention (where possible) in experienced hepatobiliary units was shown. EVIDENCE LEVEL: II.

Mature Cystic Oral Teratoma in a Neonate- A Case Report.

BACKGROUND: Neonatal oral cavity masses present complex diagnostic and management challenges, often involving respiratory distress. We present a case of a 1-day-old male neonate with an oral cavity mass and respiratory distress, whose antenatal scan revealed polyhydramnios and a nasopalatine cyst. CASE REPORT: Clinical examination revealed a 5 * 5 cm cystic swelling arising from the right hard palate. CT scans confirmed an intraoral cystic lesion with a complete cleft palate and excluded oropharyngeal/neck extension. Aspiration of cystic fluid facilitated surgical excision, leading to a histopathological diagnosis of mature cystic teratoma. Primary closure of the soft palate defect was performed. Histopathological examination revealed it to be mature cystic teratoma. CONCLUSION: This case underscores the intricate interplay between antenatal imaging, clinical assessment, and surgical intervention in managing neonatal oral cavity masses. The successful excision and histopathological confirmation of a mature cystic teratoma highlights the significance of accurate diagnosis and timely intervention. The complexities surrounding neonatal oral cavity masses necessitate a comprehensive approach to optimize patient outcomes.

Our experience with management of congenital urological pathologies in adulthood: What pediatric urologists should know and adult urologists adopt in pediatric practice experience.

Purpose: To summarize our experience in the management of congenital anomalies in the kidney and urinary tract (CAKUT) in adults. Materials and methods: We conducted a retrospective chart review of all adult patients who underwent primary surgical intervention for CAKUT between 1998 and 2021. Results: The study included 102 patients with a median age of 25 (interquartile range, 23-36.5). Of these, 85 (83.3%) patients reported normal prenatal ultrasound, and the remaining 17 (16.7%) patients were diagnosed with antenatal hydronephrosis. These patients were followed-up conservatively postnatally and were discharged from follow-up because of the absence of indications for surgical intervention or because they decided to leave medical care. All studied adult patients presented with the following pathologies: 67 ureteropelvic junction obstructions, 14 ectopic ureters, 9 ureteroceles, and 6 primary obstructive megaureters, and the remaining 6 patients were diagnosed with vesicoureteral reflux. Forty-three percent of the patients had poorly functioning moieties associated with ectopic ureters or ureteroceles. Notably, 67% of patients underwent pyeloplasty, 9% underwent endoscopic puncture of ureterocele, 3% underwent ureteral reimplantation, 6% underwent endoscopic correction of reflux, 7% underwent partial nephrectomy of non-functioning moiety, and the remaining 9% underwent robotic-assisted laparoscopic ureteroureterostomy. The median follow-up period after surgery was 33 months (interquartile range, 12-54). Post-operative complications occurred in 5 patients (Clavien-Dindo 1-2). Conclusions: Patients with CAKUT present clinical symptoms later in life. Parents of patients diagnosed during fetal screening and treated conservatively should be aware of this possibility, and children should be appropriately counseled when they enter adolescence. Similar surgical skills and operative techniques used in the pediatric population may be applied to adults.

T2-weighted Imaging Features of the Fetal Thymus in the Middle and Late Pregnancy: A Post-mortem Study based on Magnetic Resonance Imaging.

BACKGROUND: Fat-suppressed (FS) T2-weighed turbo spin-echo (TSE) sequence was used to detect the signal of the thymus and the characteristics of the thymus location, measure the two-dimensional diameter at specific levels, and analyze the association with gestational weeks. METHODS: This study involved 51 fetal specimens. Post-mortem MRI scanning was implemented with a 3.0-T MRI system. T2-weighted imaging (T2WI) features of the thymus in fetuses were quantitatively investigated with DICOM images. Statistical analysis was done with the Chi-Square test, oneway ANOVA, and Student's t-test. RESULTS: There was heterogeneity in the morphology of the fetal thymus. FS T2-weighted TSE sequence clearly exhibited the microstructure of the fetal thymus. The thymus extensively showed a lobulated appearance. The central signal is much higher than the peripheral signal in each lobule. In addition, FS-T2WI images can clearly show the interlobular septum, which is filled with fluid and presents a linear high signal. The signal intensity of fetal thymus increased with gestational weeks. The diameter measured in a particular plane was highly correlated with gestational week. CONCLUSION: FS T2-weighted TSE sequence provides high-resolution images of the fetal thymus. The change in signal intensity, location, and two-dimensional diameter in a specific plane can be used as a research direction for the fetal thymus.

Radiological characteristics of the posterior fossa of the fetal skull and presentation of a rare case of antenatal screening for Dandy-Walker malformation using antenatal fetal ultrasound and MRI.

INTRODUCTION AND IMPORTANCE: Dandy-Walker malformation is a rare congenital anomaly of the brain that mainly affects the cerebellum region. It is characterised by abnormal dilatation of the fourth ventricle of the brain and partial or total absence of the cerebellar vermis. This malformation may also be accompanied by other anomalies of the brain. Ante-natal diagnosis is becoming increasingly frequent given the performance of medical imaging, in particular ante-natal ultrasound and MRI. The object of this article is to clarify the possible causes of rare cystic malformations of the posterior cerebral fossa, which are very rare congenital malformations. CASE PRESENTATION: a 30 year old patient, second gesture, mother of a live child by caesarean section, referred to us at 32 weeks of amenorrhoea at the university hospital centre for management of a cystic malformation of the posterior cerebral malformation detected on 2nd trimester ultrasound and confirmed as a Dandy Walker malformation on 3rd trimester fetal MRI. CLINICAL DISCUSSION: The Dandy-Walker malformation can be described on prenatal MRI as vermian hypoplasia and can be detected as early as the 1st trimester of pregnancy using ultrasound, This cystic malformation poses a problem of differential diagnosis with other pathologies which also result in a cystic image of the posterior cerebral fossa, in particular Black's pouch cyst, arachnoid cyst and mega magna cistern, which requires careful interpretation of cerebral MRI of the foetus. CONCLUSION: Imaging techniques play a fundamental role in diagnosis. Prenatal ultrasound and MRI can reveal a Dandy-Walker malformation as early as the 2nd month of pregnancy. MRI is ideal for differentiating differential diagnoses.

Optimal timing of surgery for prenatally diagnosed choledochal cysts.

Objective: Choledochal cysts are increasingly being diagnosed antenatally. The appropriate time of surgical treatment has the greatest impact on the prognosis of choledochal cyst treatment. The purpose of this study was to compare the clinical outcomes of prenatally diagnosed choledochal cysts in infants according to the surgical treatment timing. Methods: We retrospectively reviewed the medical records of infants who underwent surgery for choledochal cysts with antenatal diagnoses. We investigated each patient's demographic information, type of choledochal cyst, serum liver enzyme levels, and surgical outcomes according to the surgical intervention timing. Results: Between May 2006 and December 2020, 93 infants underwent surgery to treat choledochal cysts; among them, 68 had antenatally suspected choledochal cysts. Of the 68 patients, 21 developed symptoms directly after birth. While 38 patients remained asymptomatic, 9 developed symptoms before operation. To compare surgical outcomes, asymptomatic patients were divided into early (13 cases) and late (25 cases) operation groups based on an age benchmark of 30 days. The early surgical group experienced longer times to resume a full diet (6.0 ± 1.6 vs. 4.5 ± 0.7, p < 0.001) and longer postoperative hospital stays (11 ± 3.9 vs. 7.5 ± 0.8, p < 0.001). Surgical complications occurred in two patients in the early operation group. Minimally invasive surgery was performed in 12 patients in the late operation group. In both groups, postoperative liver function recovered at 6 months, with no significant difference. Conclusion: The results of this study showed longer hospital stays, increased diet durations, and postoperative complications in early surgery patients. However, liver function recovery was not different between the early and late operation groups. Thus, asymptomatic patients should be closely monitored, and we recommend that definitive surgical intervention be postponed until 4 months of age or until weight reaches 7 kg.

Isolated choroid plexus cysts and health and developmental outcomes in childhood and adolescence - A systematic review.

OBJECTIVES: Choroid plexus cysts (CPCs) are incidental findings on ultrasound examination of the fetal brain. It is not known if isolated CPCs are associated with any adverse health or neurodevelopmental outcomes during the life course. This systematic review aimed to collate and synthesize the evidence on whether or not isolated choroid plexus cysts are associated with an increased risk of adverse health or developmental outcomes during childhood and adolescence. METHODS: A search strategy was developed specifically for this study and applied to four electronic databases Medline (Ovid), Embase (Ovid), Web of Science, and Google Scholar. Studies were assessed and selected for inclusion if there was a measurement of CPC (including single or multiple; unilateral or bilateral; isolated or presenting alongside other markers) during the antenatal or early neonatal period (<7 days) with follow-up of children and adolescents for health and developmental outcomes measured at any time from age 1 month onwards. Study quality was assessed using the Newcastle-Ottawa Quality Assessment Scale. Due to heterogeneity in the types of outcome measures included and the timing of measurement of outcomes across the studies, it was not possible to pool data across studies and a narrative description of findings was presented. RESULTS: Eight studies (three cohorts and five case series) met the inclusion criteria. Different methods were used for outcome assessment, such as in-person assessment, parent questionnaires, medical records, and telephone interviews with parents. Six studies measured outcomes only once during the specified duration of follow-up; two studies carried out paediatric reviews of the children several times during follow-up. There were no differences in developmental outcomes or physical health between babies with CPCs reported in the three cohort studies, and no abnormalities were detected in the children that were followed up in four of the five case series studies. Most of the included studies were graded as low quality due to the small sample size, high risk of selection bias, unclear definitions of CPC or lack of a comparison group. CONCLUSIONS: The studies conducted to date do not provide evidence of adverse physical health outcomes or neurodevelopmental delays in babies with CPCs. However, most of these studies were small and included a narrow range of outcomes. Further research is needed to explore the relative incidence of outcomes such as ASD, ADHD, epilepsy and educational attainment in children with CPCs.

Fetal Klippel-Trenaunay-Weber Syndrome: Antenatal Diagnosis and Postnatal Management.

Context: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare disease characterized by a triad of venous malformations, vascular skin nevus and asymmetric hypertrophy of bone and soft tissue. The spectrum of disease in utero varies from asymptomatic nevus flammeus to life threatening complications like Kasabach-Merritt phenomena. Aim: The aim of this study was to review our experience of antenatal diagnosis of KTWS and it's postnatal management. Settings and Design: This was a retrospective observational study of all pregnant women who were antenatally diagnosed with KTWS and postnatally confirmed at a tertiary care center in north India between 2012 and 2021. Subjects and Methods: The electronic medical records were reviewed and data were collected regarding demographic information, obstetric history, clinical presentation, sonographic findings, mode of delivery, fetal outcome, and follow-up. Results: During the study period, four fetuses were diagnosed with KTWS on sonography. Three women were multigravida whereas one was a primigravida. Two women opted for medical termination of pregnancy and one each had liveborn child and an intra-uterine fetal death. KTWS was confirmed in all cases. The liveborn child underwent treatment for the vascular malformation and is alive at 4 years of age. Conclusions: This study attempts to add onto the available literature regarding the spectrum of prenatal presentations of KTWS. It emphasizes the importance of prenatal diagnosis and follow-up of the fetus/neonate.

(New) antenatal ultrasound signs of fetal junctional epidermolysis bullosa: A case report and systematic review of literature.

Epidermolysis bullosa is a rare hereditary autosomal disease that is included in the heterogeneous group of genodermatosis. It is characterized by skin and mucous membranes fragility and denudation, and it can be associated with pyloric atresia. Prognosis is often poor, and death can occur in neonatal period due to severe sepsis. We present a case of fetal junctional epidermolysis bullosa in a consanguineous couple, highly suggested by previous obstetric history and several antenatal ultrasound signs, such as polyhydramnios, gastric enlargment, the "snowflake sign", abnormal external ears, signs of skin desquamation, lower limbs anomalies and chorioamniotic membrane separation. We describe a marked perioral hipoecogenicity as a novel sign of skin-mucous denudation, which could be helpful for future diagnosis. A review of literature, focused specifically on the antenatal sonography role, is also reported. Prenatal ultrasound-based diagnosis of epidermolysis bullosa is difficult, especially in apparently low risk contexts, but may be possible.

Manejo de embarazos únicos con diagnóstico antenatal de vasa previa / Management of singleton pregnancies with prenatal diagnosis of vasa previa

Introducción: vasa previa (VP) corresponde al paso de los vasos umbilicales por las membranas amnióticas, sin protección de gelatina de Wharton o placenta, antes de la presentación fetal, sobre el orificio cervical interno. Pese a su baja incidencia, el diagnóstico prenatal es relevante por las graves consecuencias que puede tener esta patología en caso de no ser diagnosticada. El objetivo de esta revisión es presentar la evidencia disponible para el manejo de embarazadas con diagnóstico antenatal de VP. Materiales y métodos: analizamos todos los estudios publicados (prospectivos, retrospectivos y reporte de casos) entre los años 1999 y 2023, con diagnóstico VP en embarazo único, reportando la edad gestacional de interrupción y el resultado neonatal. Resultados: incluimos 19 investigaciones (18 en la búsqueda primera y una adicional por relevancia). Las pacientes con manejo intrahospitalario desde las 34 semanas tuvieron mayor latencia al parto, mejores resultados neonatales y menor tasa de cesárea de urgencia que las pacientes con manejo ambulatorio. La edad gestacional de interrupción es variable entre los estudios, sin embargo, no se evidenció beneficio de interrupción a las 34 semanas comparado con manejo expectante hasta las 37 semanas de edad gestacional. Conclusión: existiría beneficio de hospitalización entre las 32-34 semanas en mujeres con diagnóstico de VP, siendo razonable la interrupción cercana a las 37 semanas por cesárea electiva.

Introduction: vasa previa (VP) corresponds to the passage of the umbilical vessels through the amniotic membranes, without the protection of Wharton's gelatin or placenta, in front of the fetal presentation, over the internal cervical os. Despite its low incidence, prenatal diagnosis is relevant due to the severe consequences of this pathology if the diagnosis is missed. This review presents the available evidence for pregnant women's management with an antenatal diagnosis of VP. Materials and methods: we analyzed all the studies published (prospective, retrospective, and case reports) between 1999 and 2023, with a diagnosis of VP in a single pregnancy, reporting gestational age at delivery and neonatal outcome. Results: We included 19 investigations (18 in the first search and another for relevance). Patients with in-hospital management from 34 weeks had a more extended latency period until delivery, better neonatal outcomes, and a lower rate of emergency cesarean section than patients with outpatient management. The gestational age at birth is variable between the studies; however, no benefit of delivery at 34 weeks was evidenced compared with expectant management until 37 weeks of gestational age. Conclusion: there would be a benefit of hospitalization between 32-34 weeks in women diagnosed with VP, being reasonable to schedule the delivery close to 37 weeks by elective cesarean section.

Thrombosed Arteriovenous Malformation of Umbilical Cord.

Background: Arteriovenous malformation of umbilical cord is an extremely rare congenital malformation. Causes of this condition are unknown. AVM of umbilical cord can cause significant complications in the developing fetus. Methods: We report our management of the case with accurate ultrasound study that could improve and facilitate the approach to this pathology due to the lack of literature and with an overview of the available literature. Results: There are only two cases of umbilical AVM diagnosed in the prenatal period with associated pathology. The mainstay of prenatal detection is the accurate study of umbilical cord also even if it is not requested from the actual guide lines in a way to improve the perinatal morbidity and mortality.

Prenatal diagnosis of an adrenal mature teratoma mimicking a neuroblastoma.

Teratomas are defined by the presence of cell types from different germ layers, they typically involve the gonads or the sacrococcygeal region and are rarely retroperitoneal. Prenatally detected adrenal teratomas are extremely uncommon. Aim of this paper is to share our experience with an adrenal antenatal mass initially diagnosed as a left adrenal neuroblastoma that turned out to be a mature teratoma after microscopical examination. We present the case of a male fetus with antenatal diagnosis of a left adrenal cystic image at the 22nd week of amenorrhea. The fetal magnetic resonance imaging showed a non-calcified cystic mass of the left adrenal gland, compatible with a neuroblastoma. At birth an ultrasound confirmed the presence of an anechogenic lesion of the left adrenal gland. The infant was closely monitored during his first year and in the absence of significant regression of the adrenal mass, it was decided to perform a laparoscopic left adrenalectomy. Unexpectedly, the final pathological diagnosis was mature cystic adrenal teratoma. In conclusion, an adrenal mass diagnosed antenatally is generally a hemorrhage or a neuroblastoma. Adrenal teratomas are very rare and those diagnosed antenatally even more. At present, we have no clinical, biological, or radiological evidence to suspect them before surgical removal. There are only two other cases of unexpected adrenal teratoma in infants described in Literature.

Validation of a sonographic checklist for the detection of histologic placenta accreta spectrum.

BACKGROUND: To standardize research terminology and to reduce unanticipated placenta accreta spectrum, the European Working Group for Abnormally Invasive Placenta developed a consensus checklist for reporting suspected placenta accreta spectrum observed during an antenatal ultrasound. The diagnostic accuracy of the European Working Group for Abnormally Invasive Placenta checklist has not been assessed. OBJECTIVE: This study aimed to test the performance of the European Working Group for Abnormally Invasive Placenta sonographic checklist in predicting histologic placenta accreta spectrum. STUDY DESIGN: This was a multisite, blinded, retrospective review of transabdominal ultrasound studies performed between 26 to 32 weeks' gestation for subjects with histologic placenta accreta spectrum between 2016 and 2020. We matched a control cohort of subjects without histologic placenta accreta spectrum in a 1:1 ratio. To reduce reader bias, we matched the control cohort for known risk factors including previa, number of previous cesarean deliveries, previous dilation and curettage, in vitro fertilization, and clinical factors affecting image quality including multiple gestation, body mass index, and gestational age at the ultrasound. Nine sonologists from 5 referral centers, blinded to the histologic outcomes, interpreted the randomized ultrasound studies using the European Working Group for Abnormally Invasive Placenta checklist. The primary outcome was the sensitivity and specificity of the checklist to predict placenta accreta spectrum. Two separate sensitivity analyses were performed. First, we excluded subjects with mild disease (ie, only assessed subjects with histologic increta and percreta). Second, we excluded interpretations from the 2 most junior sonologists. RESULTS: A total of 78 subjects were included (39 placenta accreta spectrum, 39 matched control). Clinical risk factors and image quality markers were statistically similar between the cohorts. The checklist sensitivity (95% confidence interval) was 76.6% (63.4-90.6) and the specificity (95% confidence interval) was 92.0% (63.4-99.9) with a positive and negative likelihood ratio of 9.6 and 0.3, respectively. When we excluded subjects with mild placenta accreta spectrum disease, the sensitivity (95% confidence interval) increased to 84.7% (73.6-96.4) and the specificity was unchanged at 92.0% (83.2-99.9). Sensitivity and specificity were unchanged when the interpretations from the 2 most junior sonologists were excluded. CONCLUSION: The 2016 European Working Group for Abnormally Invasive Placenta checklist for interpreting placenta accreta spectrum has a reasonable performance in detecting histologic placenta accreta spectrum and excluding cases without placenta accreta spectum.

'You have a little human being kicking inside you and an unbearable pain of knowing there will be a void at the end': A meta-ethnography exploring the experience of parents whose baby is diagnosed antenatally with a life limiting or life-threatening condition.

BACKGROUND: Parents of babies diagnosed with life limiting conditions in the perinatal period face numerous challenges. Considerations include the remainder of the pregnancy, delivery of the baby and decisions around care in the neonatal period. AIM: To increase understanding of how parents experience the diagnosis of a life-limiting or life-threatening condition, during pregnancy and following the birth of their baby, by answering the question: 'what is known about the perinatal experiences of parents of babies with a life-limiting or life-threatening diagnosis?' DESIGN: A meta-ethnography was conducted to synthesise findings from existing qualitative evidence. DATA SOURCES: British Nursing Database, CINAHL, Medline, PsycINFO and Embase databases were searched in January 2023. FINDINGS: Relationships between parents and their families and friends, and with professionals influence the needs and experiences of parents, which oscillate between positive and negative experiences, throughout parents' perinatal palliative care journey. Parents highlighted the need for control and a sense of normality relating to their parenting experience. Validation was central to the experience of parents at all stages of parenthood. Relationships between the parent and the baby were unwavering, underpinned with unconditional love. CONCLUSION: Professionals, family members and friendship groups influence the experience, validating parents and their baby's identity and supporting parents in having a sense of control and normality by demonstrating empathy, and providing time and clear communication.

Use, misuse, and overuse of antenatal corticosteroids. A retrospective cohort study.

OBJECTIVES: To evaluate the timing of antenatal corticosteroids (ACS) administration in relation to the delivery timing based on indications and risk factors for preterm delivery. METHODS: We conducted a retrospective cohort study to understand what factors predict the optimal timing of ACS administration (ACS administration within seven days). We reviewed consecutive charts of adult pregnant women receiving ACS from January 1, 2011, to December 31, 2019. We excluded pregnancies under 23 weeks, incomplete and duplicate records, and patients delivered outside our health system. The timing of ACS administration was categorized as optimal or suboptimal. These groups were analyzed regarding demographics, indications for ACS administration, risk factors for preterm delivery, and signs and symptoms of preterm labor. RESULTS: We identified 25,776 deliveries. ACS were administered to 531 pregnancies, of which 478 met the inclusion criteria. Of the 478 pregnancies included in the study, 266 (55.6 %) were delivered in the optimal timeframe. There was a higher proportion of patients receiving ACS for the indication of threatened preterm labor in the suboptimal group as compared to the optimal group (85.4 % vs. 63.5 %, p<0.001). In addition, patients who delivered in the suboptimal timeframe had a higher proportion of short cervix (33 % vs. 6.4 %, p<0.001) and positive fetal fibronectin (19.8 % vs. 1.1 %, p<0.001) compared to those who delivered in the optimal timeframe. CONCLUSIONS: More emphasis should be placed on the judicious use of ACS. Emphasis should be placed on clinical assessment rather than relying solely on imaging and laboratory tests. Re-appraisal of institutional practices and thoughtful ACS administration based on the risk-benefit ratio is warranted.

The Impact of Prenatal Diagnosis on Clinical Outcomes of Isolated Vascular Rings From a Statewide Paediatric Cardiology Tertiary Service.

BACKGROUND: Vascular rings, including right aortic arch with aberrant left subclavian artery (RAA-ALSCA), double aortic arch (DAA) and pulmonary artery sling (PAS), are congenital anomalies that may cause airway and oesophageal compression. As prenatal detection has improved, literature comparing clinical outcomes of antenatally versus postnatally diagnosed cases continues to emerge. The aim is to define a statewide tertiary paediatric institution's clinical profile and outcomes of prenatal versus postnatally diagnosed isolated vascular rings. METHOD: A retrospective single-centre review of isolated RAA-ALSCA, DAA and PAS between 1 January 1999 and 31 December 2020 was conducted. Clinical characteristics, surgical and follow-up information were collected. Antenatal and postnatally diagnosed groups were compared. RESULTS: Out of 123 cases diagnosed with isolated vascular rings, 98 (79.7%) cases had RAA-ALSCA, 21 (17.1%) with DAA, 4 (3.3%) with PAS. The antenatal detection rate was 73.6% in the past decade; 20.3% had a genetic disorder, of which 48% had 22q11.21 microdeletion. Of prenatally diagnosed cases, 31.3% developed symptoms, commonly stridor and dysphagia, at a median age of 2.0 months (IQR 0.0-3.0), compared to a median age of diagnosis for the postnatal cohort of 9 months (IQR 1.0-40.7). Postnatally diagnosed cases were more likely to present with symptoms, primarily respiratory distress, than prenatally diagnosed cases (p=0.006). Fifty-nine (59) cases (50% antenatally diagnosed) required vascular ring division; 6.8% had residual symptoms following surgery. DISCUSSION: Antenatal diagnosis has improved and leads to better parental awareness and more timely, appropriate intervention. Postnatally diagnosed patients were older, more likely to be symptomatic, underwent more investigations and were commenced on more medications for symptom management prior to diagnosis. One in five cases of isolated vascular ring anomalies carried a genetic diagnosis, which has important implications on prenatal counselling and genetic testing.

Effective training program for antenatal diagnosis of placenta accreta spectrum disorder.

OBJECTIVE: To develop and evaluate a training program for diagnosing placenta accreta spectrum (PAS) disorder in obstetrics/gynecology and radiology residents. METHODS: This single-center prospective study was based on 177 ultrasound images of pathologically confirmed PAS extracted from 534 cases of placenta previa suspicious for PAS. First- to third-year residents were evaluated before training to assess experience and ability to diagnose PAS. They attended a principal lecture followed by weekly self-study exercises over 5 weeks. Post-course tests evaluated the efficacy of the training program for improving diagnosis of PAS after the program. RESULTS: Twenty-three (38.3%) obstetrics/gynecology and 37 (61.7%) radiology residents were trained. Before the training program, 98.3% reported minimal experience and 100% low confidence to correctly diagnose PAS. During the program, the overall accuracy of all participants in diagnosing PAS increased from 71.3% before to 95.2% after training (P < 0.001). Regression analyses showed that ability to diagnose PAS increased 2.52-fold (P < 0.001) following the program. The retention of knowledge at 1, 3, and 6 months posttest was 84.7%, 87.5%, and 87.7%, respectively. CONCLUSION: An antenatal diagnosis of PAS training program can be effective as a residency training program, considering the globally rising cesarean delivery rates.

Fetus-in-Fetu: A Differential Diagnosis of Neonatal Fetiform Encysted Abdominal Mass.

Fetus-in-fetu (FIF) is a rare embryological anomaly in which an encysted fetiform mass develops within the infant or adult host body. It mainly occurs intraabdominal. There are embryo-pathogenetic debates over whether it belongs to the spectrum of highly differentiated teratomas or is a parasitic twinning from a monozygotic monochorionic diamniotic pregnancy. The presence of vertebral segments and an encapsulating cyst can reliably distinguish FIF from teratoma. The diagnosis may be initially made by imaging modalities such as computed tomography (CT) and magnetic resonance imaging (MRI), and a confirmatory diagnosis made by histopathology of the excised mass. Our center experienced a case of a male neonate presented after emergency cesarean delivery at 40-week gestation with the suspicion of an intraabdominal mass identified antenatally. Antenatal ultrasonography at 34 weeks gestation suggested the presence of an intraabdominal cystic mass measuring 6.5 cm with a hyperechoic focus. A follow-up MRI performed after the delivery showed a well-defined mass with the cystic formation in the left abdominal region with a centrally located fetiform structure. Vertebral bodies and long limb bones were visualized. The diagnosis of FIF was initially made preoperatively by the characteristic findings of imaging studies. Laparotomy was scheduled on day 6, revealing a large encysted mass with fetiform content. FIF should be considered a possible differential diagnosis of neonatal encysted fetiform mass. Routine antenatal imaging permits more frequent antenatal detection with earlier workup and management.

Fetal Lung Volume Appears to Predict Respiratory Morbidity in Congenital Diaphragmatic Hernia.

Congenital diaphragmatic hernia (CDH) is associated with pulmonary hypoplasia and respiratory morbidity. To assess whether respiratory morbidity during the first 2 years of life in infants with left-sided CDH is associated with fetal lung volume (FLV) evaluated by the observed-to-expected FLV ratio (o/e FLV) on antenatal magnetic resonance imaging (MRI). In this retrospective study, o/e FLV measures were collected. Respiratory morbidity in the first 2 years of life was studied according to two endpoints: treatment with inhaled corticosteroids for >3 consecutive months and hospitalization for any acute respiratory disease. The primary outcome was a favorable progression defined by the absence of either endpoint. Forty-seven patients were included. The median o/e FLV was 39% (interquartile range, 33-49). Sixteen (34%) infants were treated with inhaled corticosteroids and 13 (28%) were hospitalized. The most efficient threshold for a favorable outcome was an o/e FLV ≥ 44% with a sensitivity of 57%, specificity of 79%, negative predictive value of 56%, and positive predictive value of 80%. An o/e FLV ≥ 44% was associated with a favorable outcome in 80% of cases. These data suggest that lung volume measurement on fetal MRI may help to identify children at lower respiratory risk and improve information during pregnancy, patient characterization, decisions about treatment strategy and research, and personalized follow-up.