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Prenatal Diagnosis Jun 2024To describe and compare maternal and fetal comorbidities and obstetrical outcomes in pregnancies with hypoplastic left and right heart (HLHS and HRH) single ventricle...
Maternal-fetal comorbidities and obstetrical outcomes of fetal single ventricle cardiac defects: 10 years' experience with a multidisciplinary management protocol at a single center.
OBJECTIVES
To describe and compare maternal and fetal comorbidities and obstetrical outcomes in pregnancies with hypoplastic left and right heart (HLHS and HRH) single ventricle cardiac defects (SVCD) from a single center under a multidisciplinary protocol.
METHOD
A single center retrospective review of fetal SVCD from 2013 to 2022. Maternal-fetal comorbidities, delivery, and postnatal outcomes were compared between HLHS and HRH using descriptive statistics and univariate and multivariate analyses.
RESULTS
Of 181 SVCD pregnancies (131 HLHS; 50 HRH), 9% underwent termination, 4% elected comfort care, 5 died in utero and 147/152 liveborns survived to the first cardiac intervention. Cesarean delivery occurred in 57 cases (37%), planned in 36 and unplanned in 21. Comorbidities, which did not differ between HLHS and HRH, included fetal growth restriction (FGR, 17%), prematurity (14%), maternal hypertension (9%), maternal obesity (50%), fetal extracardiac anomalies and chromosome anomalies (12%, 13%). In multivariate analysis, only earlier gestational age at delivery and oligohydramnios predicted decreased odds of survival at one year.
CONCLUSION
Maternal-fetal comorbidities are common in both HLHS and HRH. Earlier gestational age at delivery and oligohydramnios predict lower postnatal survival. FGR, even with severe early onset, did not significantly impact short- or long-term neonatal survival in single ventricle conditions.
Topics: Humans; Female; Pregnancy; Retrospective Studies; Adult; Comorbidity; Pregnancy Outcome; Hypoplastic Left Heart Syndrome; Infant, Newborn; Heart Defects, Congenital; Pregnancy Complications; Univentricular Heart
PubMed: 38782600
DOI: 10.1002/pd.6591 -
Hip International : the Journal of... May 2024DDH is 1 of the most important causes of childhood disability. A diagnosis of instability can be made in the neonatal period via the Ortolani and Barlow manoeuvres....
BACKGROUND
DDH is 1 of the most important causes of childhood disability. A diagnosis of instability can be made in the neonatal period via the Ortolani and Barlow manoeuvres. However, clinical examination, although highly specific, has poor sensitivity as compared to ultrasound. There is controversy between the necessity of universal screening for dysplasia of all newborns or selective screening reserved for those with clinical signs of instability or known risk factors of DDH.
AIM
To analyse the risk factors of congenital hip dysplasia in a consecutive case series of children referred for diagnosis and treatment of DDH.
MATERIALS AND METHODS
This was a cross-sectional study on infants consecutively examined between January 2000 and December 2019 at the Marino Ortolani Centre in Ferrara, Italy. The first 3 types on Graf's classification were considered physiological (1A, 1B, 2A+), while the last 6 pathological (2A-, 2B, 2C, 2D, 3, 4).
RESULTS
18,954 infants met the inclusion criteria and were therefore considered eligible for the study. Of these 18,954 infants, 56% ( = 10,629) were females and 44% ( = 8325) were males. According to Graf classification, 34.9% ( = 6621) were 1A, 52.7% ( = 9999) were 1B, 4.0% ( = 753) were 2A+, 2.5% ( = 478) were 2A-, 1.5% ( = 284) were 2B, 1% ( = 196) were 2C, 1.3% ( = 243) were 2D, 1% ( = 193) were 3 and 1.0% ( = 187) were 4. The most significant risk factor was the female gender (OR 5.36; 95% CI, 4.63-6.20) followed by a family history (OR 2.35; 95% CI, 2.08-2.65), then skeletal pathologies (OR 2.04; 95% CI, 1.21-3.42), oligohydramnios (OR 1.75; 95% CI, 1.44-2.13), and finally breech presentation (OR 1.42: 95% CI, 1.27-1.60).
CONCLUSIONS
Based on our data, family history, musculoskeletal disease, oligohydramnios and breech presentation are the main risk factors for DDH development, as is the female sex.
PubMed: 38767233
DOI: 10.1177/11207000241248416 -
Journal of Perinatal Medicine May 2024To identify factors associated with poor prognoses in newborns with a prenatal diagnosis of gastroschisis in eight hospitals in Bogota, Colombia, from 2011 to 2022.
OBJECTIVES
To identify factors associated with poor prognoses in newborns with a prenatal diagnosis of gastroschisis in eight hospitals in Bogota, Colombia, from 2011 to 2022.
METHODS
A multi-center retrospective case-control study was conducted on newborns with gastroschisis in eight hospitals in Bogota, Colombia. Poor prognosis was defined as the presence of sepsis, intestinal complications, or death.
RESULTS
The study included 101 patients. Preterm newborns under 32 weeks had a poor neonatal prognosis (OR 6.78 95 % CI 0.75-319). Oligohydramnios (OR 4.95 95 % CI 1.15-21.32) and staged closure with silo (OR 3.48; 95 % CI 1.10-10.96) were risk factors for neonatal death, and intra-abdominal bowel dilation of 20-25 mm was a factor for the development of intestinal complications (OR 3.22 95 % CI 1.26-8.23).
CONCLUSIONS
Intra-abdominal bowel dilation between 20 and 25 mm was associated with intestinal complications, while oligohydramnios was associated with the risk of perinatal death, requiring increased antenatal surveillance of fetal wellbeing. Management with primary reduction when technically feasible is recommended in these infants, considering that the use of silos was associated with higher mortality.
PubMed: 38758017
DOI: 10.1515/jpm-2023-0520 -
Ultrasound in Obstetrics & Gynecology :... May 2024To reach a Delphi-generated international expert consensus on the diagnosis, prognostic, management, and core outcome set (COS) of fetal Lower Urinary Tract Obstruction...
OBJECTIVE
To reach a Delphi-generated international expert consensus on the diagnosis, prognostic, management, and core outcome set (COS) of fetal Lower Urinary Tract Obstruction (LUTO).
METHODS
A three-round Delphi procedure was conducted among an international panel of LUTO experts. The panel was provided with a list of literature review-generated parameters for the diagnosis, prognostic, management, and outcomes. A parallel procedure was conducted along with patient groups during the development of COS.
RESULTS
A total of 160 experts were approached, of whom 99 completed the first round and 80 (80/99, 80.8%) completed all three rounds. In the first trimester, an objective measurement of longitudinal bladder diameter (with ≥7 mm being abnormal) should be used to suspect LUTO. In the second trimester, imaging parameters of LUTO could include: a) an enlarged bladder, b) a keyhole sign, c) bladder wall thickening, d) bilateral hydro (uretero) nephrosis, and e) male sex. There was a lack of consensus on the current prognostic scoring literature. However, experts agreed on the value of amniotic fluid volume (< 24 weeks) to predict survival and that the value of fetal intervention is to improve neonatal survival. While experts endorsed the role of sonographic parameters of renal dysplasia, at least one vesicocentesis, and urine biochemistry for prognosis and counseling, these items did not reach a consensus for determining fetal intervention candidacy. On the other hand, imaging parameters suggestive of LUTO, absence of life-limiting structural or genetic anomalies, gestational age of ≥16 weeks, and oligohydramnios defined as deepest vertical pocket (DVP) <2 cm should be used as candidacy criteria for fetal intervention based on experts' consensus. If a bladder refill was evaluated, it should be assessed subjectively. Vesicoamniotic shunt should be the first line of fetal intervention. In the presence of suspected fetal renal failure, serial amnioinfusion should only be offered as an experimental procedure under research protocols. The core outcome set for future studies was agreed upon.
CONCLUSION
International consensus on the diagnosis, prognosis, and management of fetal LUTO, as well as the Core Outcome Set, should inform clinical care and research to optimize perinatal outcomes. This article is protected by copyright. All rights reserved.
PubMed: 38748971
DOI: 10.1002/uog.27684 -
Acta Radiologica Open May 2024Fetal ultrasound has limitations, especially if the patient is obese or in cases with oligohydramnios. Magnetic resonance imaging (MRI) can then be used as a complement,...
BACKGROUND
Fetal ultrasound has limitations, especially if the patient is obese or in cases with oligohydramnios. Magnetic resonance imaging (MRI) can then be used as a complement, but only few studies have focused on examinations in the second trimester.
PURPOSE
To validate MRI as a complement to diagnose fetal anomalies in the second trimester.
MATERIAL AND METHODS
This retrospective study retrieved data from January 2008 to July 2012 from the Fetal Medicine Unit and Department of Radiology at Uppsala University Hospital. Ultrasound and MRI findings were reviewed in 121 fetuses in relation to the final diagnosis, including postpartum follow-up and autopsy results.
RESULTS
Of the 121 fetuses, 51 (42%) had a CNS anomaly and 70 (58%) a non-CNS anomaly diagnosed or suspected. MRI provided additional information in 21% of all cases without changing the management and revealed information that changed the management of the pregnancy in 13%. When a CNS anomaly was detected or suspected, the MRI provided additional information in 22% and changed the management in 10%. The corresponding figures for non-CNS cases were 21% and 16%, respectively. The proportion of cases with additional information that changed the management was especially high in patients with a BMI >30 kg/m (25%) and in patients with oligohydramnios (38%). In five cases in category III, false-positive ultrasound findings were identified.
CONCLUSIONS
MRI in the second trimester complements ultrasound and improves diagnosis of fetal CNS- and non-CNS anomalies especially when oligohydramnios or maternal obesity is present.
PubMed: 38737558
DOI: 10.1177/20584601241248820 -
Pediatric Research May 2024To investigate association of prenatal risk factors and neonatal outcomes of preterm infants with pulmonary hypertension (PH).
BACKGROUND
To investigate association of prenatal risk factors and neonatal outcomes of preterm infants with pulmonary hypertension (PH).
METHODS
A prospective cohort study of very-low-birth-weight infants born at 22-29 weeks' gestation who received PH-specific treatment during hospitalization. Infants were classified using a two-step cluster analysis based on gestational age (GA), small-for-gestational-age (SGA), exposure to antenatal corticosteroids (ACS), histologic chorioamnionitis (HCA), and oligohydramnios.
RESULTS
Among 910 infants, six clusters were identified: cluster A (HCA, n = 240), cluster B (oligohydramnios, n = 79), cluster C (SGA, n = 74), cluster D (no-ACS, n = 109), cluster E (no dominant parameter, n = 287), and cluster F (HCA and oligohydroamnios, n = 121). Cluster A was used as a reference group for comparisons among clusters. Compared to cluster A, cluster C (aHR: 1.63 [95% CI: 1.17-2.26]) had higher risk of overall in-hospital mortality. Clusters B (aHR: 1.52 [95% CI: 1.09-2.11]), D (aHR: 1.71 [95% CI: 1.28-2.30]), and F (aHR: 1.51 [95% CI: 1.12-2.03]) had higher risks of receiving PH-specific treatment within the first week of birth compared to cluster A.
CONCLUSION
These findings may provide a better understanding of prenatal risk factors contributing to the development of PH.
IMPACT
Pulmonary hypertension (PH), presenting as hypoxic respiratory failure, has complex etiologies in preterm infants. Although multifactorial risks for the development of PH in preterm infants are known, few studies have classified infants with similar etiologies for PH. Each cluster has distinct patterns of prenatal condition and neonatal outcome.
PubMed: 38734814
DOI: 10.1038/s41390-024-03232-1 -
Diagnostics (Basel, Switzerland) Apr 2024Diagnosis of developmental dysplasia of the hip (DDH) mostly relies on physical examination and ultrasound, and both methods are operator-dependent. Late detection can...
Diagnosis of developmental dysplasia of the hip (DDH) mostly relies on physical examination and ultrasound, and both methods are operator-dependent. Late detection can lead to complications in young adults. Current evidence supports the involvement of environmental and genetic factors, such as single nucleotide variants (SNVs). Incorporating genetic factors into diagnostic methods would be useful for implementing early detection and management of affected individuals. Our aim was to analyze environmental factors and SNVs in DDH patients. We included 287 DDH cases and 284 controls. Logistic regression demonstrated an association for sex (OR 9.85, 95% CI 5.55-17.46, = 0.0001), family history (OR 2.4, 95% CI 1.2-4.5, = 0.006), fetal presentation (OR 3.19, 95% CI 1.55-6.54, = 0.002), and oligohydramnios (OR 2.74, 95%CI 1.12-6.70, = 0.026). A model predicting the risk of DDH including these variables showed sensitivity, specificity, PPV, and NPV of 0.91, 0.53, 0.74, and 0.80 respectively. The SNV rs1800470 in showed an association when adjusted for covariables, OR 0.49 (95% CI 0.27-0.90), = 0.02. When rs1800470 was included in the equation, sensitivity, specificity, PPV and NPV were 0.90, 0.61, 0.84, and 0.73, respectively. Incorporating no-operator dependent variables and SNVs in detection methods could be useful for establishing uniform clinical guidelines and optimizing health resources.
PubMed: 38732313
DOI: 10.3390/diagnostics14090898 -
Journal of the College of Physicians... May 2024To investigate whether there is a relationship between the 2nd finger and 4th finger length measurement ratios and developmental dysplasia of the Hip (DDH). (Observational Study)
Observational Study
OBJECTIVE
To investigate whether there is a relationship between the 2nd finger and 4th finger length measurement ratios and developmental dysplasia of the Hip (DDH).
STUDY DESIGN
Cross-sectional observational study. Place and Duration of the Study: Department of Orthopaedics and Traumatology, Meram Faculty of Medicine Hospital, Konya, Turkiye, from January 2020 to May 2023.
METHODOLOGY
Infants were screened for DDH with Graff method for the ultrasounds of both hips. Lengths of the 2nd and 4th fingers of both hands were measured and recorded. Patients with additional risk factors for developmental dysplasia of the hip (breech birth, family history, oligohydramnios, swaddling) were excluded.
RESULTS
Two hundred and fifty-six babies were screened including 55.1% (n = 141) girls and 44.9% (n = 115) boys. Their mean age was 2.51 ± 0.80 months. The average lengths were 31.73 ± 3.05 mm, for the left 2nd finger and 34.26 ± 3.48 mm for the left 4th finger. In the hip USG measurements, the mean alpha angles were 62.91 ± 3.12° for the right hip and, 63.20 ± 3.55° for the left hip. Eighteen (7%) of babies who underwent hip ultrasound (USG) had unilateral or bilateral DDH. Among these cases, 2.7% (n = 7) had right, 2.3% (n = 6) had left, and 2% (n = 5) had bilateral DDH. There was no statistically significant correlation between the ratios of right 2/4 finger lengths and the right alpha angle (rs = 0.051; p = 0.421). There was a statistically positive and statistically significant correlation between the ratios of left 2/4 finger lengths and the left alpha angle (rs = 0.154; p = 0.013).
CONCLUSION
Only the left-hand finger ratio among the parameters in the model had a statistically significant effect on DDH. Therefore, the left hand 2D/4D finger length may be of value in screening for DDH.
KEY WORDS
Developmental dysplasia of the hip, Second to fourth finger digit ratio, Ring finger, Digit ratios.
Topics: Humans; Female; Male; Cross-Sectional Studies; Developmental Dysplasia of the Hip; Fingers; Ultrasonography; Infant; Neonatal Screening; Infant, Newborn; Hip Dislocation, Congenital; Mass Screening
PubMed: 38720223
DOI: 10.29271/jcpsp.2024.05.600 -
Journal of Ultrasound in Medicine :... May 2024Prior studies show conflicting evidence as to whether obesity in the absence of other medical or pregnancy-related conditions contributes to amniotic fluid disorders....
OBJECTIVES
Prior studies show conflicting evidence as to whether obesity in the absence of other medical or pregnancy-related conditions contributes to amniotic fluid disorders. The purpose of this study is to determine the association between late-pregnancy obesity with oligohydramnios (amniotic fluid index [AFI] ≤5 cm or maximum vertical pocket [MVP] <2 cm) and/or polyhydramnios (AFI ≥24 cm or MVP ≥8 cm).
METHODS
This is a retrospective cohort study of 961 women with singleton gestations who had one or more obstetrical ultrasounds at a single institution at 36 0/7 weeks gestation or beyond between August 1, 2015, and May 1, 2020. Patients were included if they had valid pregnancy dating and a documented AFI and/or MVP. Patients were categorized based on body mass index or BMI (eg, normal, overweight, Class I Obesity, Class II Obesity, or Class III Obesity).
RESULTS
A total of 6.2% of patients met criteria for oligohydramnios based on AFI, MVP or both (n = 60). There was no significant association between oligohydramnios and increasing BMI, regardless of obesity class (P = .21). In terms of polyhydramnios, 5.6% of patients met criteria based on AFI, MVP, or both (n = 54). Similarly, there was also no significant association between polyhydramnios and increasing BMI, regardless of obesity class (P = .66).
CONCLUSIONS
Elevated maternal BMI was not significantly associated with disorders of amniotic fluid, regardless of the severity of obesity.
PubMed: 38712537
DOI: 10.1002/jum.16476 -
Children (Basel, Switzerland) Mar 2024Pediatric cystic kidney disease (CyKD) includes conditions characterized by renal cysts. Despite extensive research in this field, there are no reliable genetics or...
INTRODUCTION
Pediatric cystic kidney disease (CyKD) includes conditions characterized by renal cysts. Despite extensive research in this field, there are no reliable genetics or other biomarkers to estimate the phenotypic consequences. Therefore, CyKD in children heavily relies on clinical and diagnostic testing to predict the long-term outcomes.
AIM
A retrospective study aimed to provide a concise overview of this condition and analyze real-life data from a single-center pediatric CyKD cohort followed during a 12-year period.
METHODS AND MATERIALS
Medical records were reviewed for extensive clinical, laboratory, and radiological data, treatment approaches, and long-term outcomes.
RESULTS
During the study period, 112 patients received a diagnosis of pediatric CyKD. Male patients were more involved than female (1:0.93). Fifty-six patients had a multicystic dysplastic kidney; twenty-one of them had an autosomal dominant disorder; fifteen had an isolated renal cyst; ten had been diagnosed with autosomal recessive polycystic kidney disease; three had the tuberous sclerosis complex; two patients each had Bardet-Biedl, Joubert syndrome, and nephronophthisis; and one had been diagnosed with the trisomy 13 condition. Genetic testing was performed in 17.9% of the patients, revealing disease-causing mutations in three-quarters (75.0%) of the tested patients. The most commonly presenting symptoms were abdominal distension (21.4%), abdominal pain (15.2%), and oligohydramnios (12.5%). Recurrent urinary tract infections (UTI) were documented in one-quarter of the patients, while 20.5% of them developed hypertension during the long-term follow-up. Antibiotic prophylaxis and antihypertensive treatment were the most employed therapeutic modalities. Seventeen patients progressed to chronic kidney disease (CKD), with thirteen of them eventually reaching end-stage renal disease (ESRD). The time from the initial detection of cysts on an ultrasound (US) to the onset of CKD across the entire cohort was 59.0 (7.0-31124.0) months, whereas the duration from the detection of cysts on an US to the onset of ESRD across the whole cohort was 127.0 (33.0-141.0) months. The median follow-up duration in the cohort was 3.0 (1.0-7.0) years. The patients who progressed to ESRD had clinical symptoms at the time of initial clinical presentation.
CONCLUSION
This study is the first large cohort of patients reported from Croatia. The most common CyKD was the multicystic dysplastic kidney disease. The most common clinical presentation was abdominal distention, abdominal pain, and oliguria. The most common long-term complications were recurrent UTIs, hypertension, CKD, and ESRD.
PubMed: 38671609
DOI: 10.3390/children11040392