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Clinics in Perinatology Mar 2024Preterm infants with bronchopulmonary dysplasia (BPD) are prone to develop pulmonary hypertension (PH). Strong laboratory and clinical data suggest that antenatal... (Review)
Review
Preterm infants with bronchopulmonary dysplasia (BPD) are prone to develop pulmonary hypertension (PH). Strong laboratory and clinical data suggest that antenatal factors, such as preeclampsia, chorioamnionitis, oligohydramnios, and placental dysfunction leading to fetal growth restriction, increase susceptibility for BPD-PH after premature birth. Echocardiogram metrics and serial assessments of NT-proBNP provide useful tools to diagnose and monitor clinical course during the management of BPD-PH, as well as monitoring for such complicating conditions as left ventricular diastolic dysfunction, shunt lesions, and pulmonary vein stenosis. Therapeutic strategies should include careful assessment and management of underlying airways and lung disease, cardiac performance, and systemic hemodynamics, prior to initiation of PH-targeted drug therapies.
Topics: Infant; Infant, Newborn; Female; Humans; Pregnancy; Bronchopulmonary Dysplasia; Hypertension, Pulmonary; Infant, Premature; Placenta; Premature Birth
PubMed: 38325941
DOI: 10.1016/j.clp.2023.12.002 -
Arkhiv Patologii 2024A rare clinical case of a newborn boy with a diagnosed Potter sequence is presented. The diagnosis was made based on polycystic dysplasia of the kidneys, cysts in the...
A rare clinical case of a newborn boy with a diagnosed Potter sequence is presented. The diagnosis was made based on polycystic dysplasia of the kidneys, cysts in the liver, hypoplasia of the lungs and characteristic external signs due to critical oligohydramnios. The child's parents were closely related, which suggested an autosomal recessive form of the disease. The newborn lived for 15 hours, after which the death, developed as a result of respiratory failure, was ascertained.
Topics: Male; Child; Infant, Newborn; Female; Pregnancy; Humans; Polycystic Kidney Diseases; Kidney; Hyperplasia; Liver
PubMed: 38319272
DOI: 10.17116/patol20248601149 -
Fertility and Sterility May 2024To demonstrate the surgical techniques for improving safety in robotic-assisted abdominal cerclage in patients with bicornuate uteri complicated by recurrent pregnancy...
OBJECTIVE
To demonstrate the surgical techniques for improving safety in robotic-assisted abdominal cerclage in patients with bicornuate uteri complicated by recurrent pregnancy loss and cervical insufficiency.
DESIGN
Stepwise demonstration with narrated video footage.
SETTING
An academic tertiary care hospital.
PATIENTS
Our patient is a 22-year-old G2P0020 with a history of recurrent pregnancy loss. During her first pregnancy, she was asymptomatic until 19 weeks and delivered because of a preterm premature rupture of membranes. A transvaginal cerclage was performed for her second pregnancy at 14 weeks, which ended at 16 weeks because of preterm premature rupture of membranes. The final magnetic resonance imaging report noted a "bicorporeal uterus with duplication of the uterine body, resulting in two markedly divergent uterine horns that are fused at the isthmus... unlike a typical didelphic uterus, a single, non septated cervix is noted, which shows normal appearances, measuring 3.8 cm in length." Given her history of a uterine anomaly and recurrent pregnancy loss in the absence of other biochemical factors, her maternal-fetal medicine specialist referred her to us as the patient strongly desired future viable pregnancies. The patient was counseled on multiple alternatives, including different methods of performing the cerclage, and ultimately decided on the robotic-assisted (Da Vinci Xi) prophylactic abdominal cerclage.
INTERVENTIONS
The bicornuate uterus is a rare class IV mullerian duct anomaly caused by the impaired fusion of the mullerian ducts in the uterus, classically appearing in imaging studies as a heart-shaped uterus. This patient demographic reports a high incidence of obstetric complications. Pregnancy in such a uterus causes complications like first- and second-trimester pregnancy loss, preterm labor, low-birthweight infants, and malpresentation at delivery. Researchers have postulated that there is an abnormal ratio of muscle fibers to connective tissue in a congenitally abnormal cervix. During pregnancy, an inadequate uterine volume may lead to increased intrauterine pressure and stress on the lower uterine segment, which can lead to cervical incompetence. To address cervical incompetence, cervical cerclages are a commonly utilized procedure, as recent studies demonstrate that the incidence of term pregnancies in the group with documented cervical incompetence treated with cerclage placement increased from 26% to 63%. One observational study noted improved obstetrical outcomes occurred with interval placement, a cerclage placed in between pregnancies in the nongravid uterus, compared with cerclage placement between 9 and 10 weeks gestation, with the mean gestational age for delivery at 32.9 weeks and 34.5 weeks when a cerclage was placed in gravid and nongravid women, respectively. In addition, another retrospective study was done, which demonstrated a lower incidence of neonatal death with prophylactic cerclages. Operating on a nonpregnant uterus offers several benefits, including its reduced size, fewer and smaller blood vessels, and simplified handling. Moreover, there are clearly no concerns regarding the fetus. In the decision to use a robotic-assisted platform vs. laparoscopic, a systematic review showed the rates of third-trimester delivery and live birth (LB) using laparoscopy during pregnancy were found to be 70% and 70%-100%, respectively. The same review demonstrated slightly improved outcomes via the robotic route regarding gestational age at delivery (median, 37 weeks), rates of LB (90%), and third-trimester delivery (90%). Additional factors contributing to the preference for robotics in surgical procedures include incorporating advanced tools, which can enhance the robotic system's advantages compared with traditional laparoscopy. An invaluable tool in this context is the simultaneous utilization of Firefly mode, which employs a near-infrared camera system, achieved through injecting indocyanine green dye or integrating other light sources concurrently. The intravenous administration of indocyanine green is acknowledged widely for its safety and efficacy as a contrast agent in the evaluation of microvascular circulation and organ vascularization. This property equips surgeons with heightened precision when guiding the needle, proving especially advantageous when faced with challenges in visualizing vascular anatomy. In our specific case, we harnessed the capabilities of Firefly mode in conjunction with hysteroscopic light, enabling us to vividly illustrate the contours of a bicornuate uterus from both external and internal perspectives. We demonstrate a simplified technique of the abdominal cerclage, one cerclage around the internal cervical os of the uterus, using a robotic-assisted platform in a nongravid patient. The surgery began with the eversion of the umbilicus, and a 15-mm skin incision was made in the umbilicus. A Gelpoint mini advanced access site laparoscopy device was inserted into the incision, and CO was allowed to insufflate the abdominal cavity with careful attention given to intraabdominal pressure. Once the DaVinci was docked, the surgeon began the creation of a bladder flap. The bladder was carefully dissected from the lower uterine segment and both uteri using monopolar scissors. The anatomical differences of a bicornuate uterus prompted the surgeon to dissect a wider circumference for safety reasons, where a wider dissection offers a better view of the uterine vessels and ease of introducing the Mersilene tape later on. Bilateral uterine vessels were further skeletonized and exposed anteriorly using blunt dissection and monopolar scissors. After further dissection and lateralization, the final result creates a landmark medial to the right uterine vessels at the level of the internal cervical os with which the needle of the Mersilene tape will be able to pass through. The Mersilene tape was guided from anterior to posterior via a previously straightened needle. Similarly, a landmark was created on the left, and the Mersilene tape was directed from anterior to posterior. The Mersilene tape was placed circumferentially around the internal cervical os of the bicornuate uterus, medial to the uterine vessels. Both ends of the Mersilene tape were then gently pulled, ensuring that the tape was lying flat on the anterior of the internal cervical os with no bowels or uterine vessels within it. The tape was then tied posteriorly at the 6 o'clock position with appropriate tension. A 2-0 silk was then sutured to the tails of the tape using the purse-string technique to ensure that it would remain securely tied and in the correct position. Hemostasis was assured. Both a hysteroscopy and a cystoscopy were done after the completion of the cerclage to ensure that no tape or sutures were seen within the cervical canal or the uterine cavity. None were observed.
MAIN OUTCOMES MEASURES
The success criteria for the surgery were identified as the patient's ability to attain a viable pregnancy after the cerclage placement, along with achieving LB.
RESULTS
Subsequently, a spontaneous pregnancy was achieved. An infant weighing 3 pounds and 16 ounces was delivered by cesarean section at 36 weeks because of an oligohydramnios. The infant is currently healthy at 13 pounds.
CONCLUSION
Robotic-assisted abdominal cerclage around the internal cervical os in a bicornuate uterus offers a possibly feasible and straightforward technique for surgeons seeking to reduce risks, although further research is needed.
Topics: Female; Humans; Pregnancy; Young Adult; Abortion, Habitual; Bicornuate Uterus; Cerclage, Cervical; Robotic Surgical Procedures; Treatment Outcome; Urogenital Abnormalities; Uterine Cervical Incompetence; Uterus
PubMed: 38316208
DOI: 10.1016/j.fertnstert.2024.01.036 -
JAMA Pediatrics Apr 2024
Topics: Pregnancy; Infant, Newborn; Female; Humans; Oligohydramnios; Delivery, Obstetric; Surveys and Questionnaires
PubMed: 38315476
DOI: 10.1001/jamapediatrics.2023.6403 -
Annals of Saudi Medicine 2024Adolescent pregnancies are more likely to be complicated with adverse perinatal outcomes. (Review)
Review
BACKGROUND
Adolescent pregnancies are more likely to be complicated with adverse perinatal outcomes.
OBJECTIVE
Assess the sociodemographic and clinical characteristics of adolescents who have delivered singleton newborns.
DESIGN
Retrospective cohort.
SETTINGS
A tertiary training and research hospital in Turkey.
PATIENTS AND METHODS
This was a review of adolescents and adults who delivered singleton newborns at a tertiary health center between January 2018 and June 2022. Pregnant adolescents were aged <20 years.
MAIN OUTCOME MEASURES
Adverse maternal and perinatal outcomes.
SAMPLE SIZE
2233 pregnant women (754 adolescents and 1479 adults).
RESULTS
Turkish nationality was significantly less prevalent in pregnant adolescents than pregnant adults (=.001). Oligohydramnios, fetal growth restriction, perineal injury and postpartum intravenous iron treatment were significantly more prevalent in pregnant adolescents than pregnant adults (<.05 for all). The neonates born to adolescent mothers had significantly lower birth weight and first minute Apgar score than the neonates born to adult mothers (=.001 for both). Small for gestational age, need for intensive care and death were significantly more prevalent in neonates born to adolescent mothers than those born to adult mothers (=.001 for all). Compared with pregnant adults, pregnant adolescents had a significantly higher risk of oligohydramnios (=.001), preterm delivery (=.024), intravenous iron treatment (=.001), and small for gestational age (=.001).
CONCLUSION
Due to the refugee population received by Turkey, it would be prudent to expect more frequent adolescent pregnancies. Adolescent pregnancies are more likely to be complicated with low birth weight, oligohydramnios, preterm delivery, postpartum iron treatment, lower Apgar scores, need for neonatal intensive care and neonatal death.
LIMITATION
Retrospective.
Topics: Adult; Adolescent; Pregnancy; Infant, Newborn; Female; Humans; Pregnancy in Adolescence; Premature Birth; Retrospective Studies; Oligohydramnios; Turkey; Iron; Pregnancy Outcome
PubMed: 38311869
DOI: 10.5144/0256-4947.2024.11 -
Zhonghua Yi Xue Yi Chuan Xue Za Zhi =... Feb 2024To carry out prenatal diagnosis for a fetus with Meckel syndrome (MKS) and explore its genetic basis.
OBJECTIVE
To carry out prenatal diagnosis for a fetus with Meckel syndrome (MKS) and explore its genetic basis.
METHODS
A pregnant woman presented at Suzhou Municipal Hospital in February 2018 was selected as the study subject. Clinical data was collected. Muscle tissue sample from the abortus and peripheral blood samples from the couple were collected. Genomic DNA was extracted and subjected to chromosomal microarray analysis (CMA) and whole exome sequencing. Candidate variant was verified by Sanger sequencing.
RESULTS
The fetus was found to have microcephaly, oligohydramnios, polycystic kidneys and banana-shaped cerebellum at 18 weeks of gestation. After induction of labor, it was found to have encephalocele, renal cysts and polydactyly. CMA has found no abnormality. Whole exome sequencing revealed novel compound heterozygous variants c.296delA (p.Lys99SerfsTer6) and c.1243G>A (p.Val415Met) in the TMEM67 gene. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the c.296delA variant was predicted to be pathogenic (PVS1+PM2_Supporting+PP4), whilst the c.1243G>A variant was predicted to be likely pathogenic (PM2_Supporting+PM3+PP3_Moderate+PP4).
CONCLUSION
The c.296delA and c.1243G>A compound heterozygous variants of the TMEM67 gene probably underlay the MKS in this fetus.
Topics: Female; Pregnancy; Humans; Encephalocele; Polycystic Kidney Diseases; Fetus; Ciliary Motility Disorders; Mutation; Membrane Proteins; Retinitis Pigmentosa
PubMed: 38311563
DOI: 10.3760/cma.j.cn511374-20230201-00045 -
Zhonghua Yi Xue Yi Chuan Xue Za Zhi =... Feb 2024To explore the pregnancy outcome and postpartum clinical phenotype of LCR22B/C~D central 22q11.2 deletion syndrome.
OBJECTIVE
To explore the pregnancy outcome and postpartum clinical phenotype of LCR22B/C~D central 22q11.2 deletion syndrome.
METHODS
For fetuses diagnosed with central 22q11.2 deletion by chromosomal microarray analysis (CMA) at the Prenatal Diagnosis Center of the Third Affiliated Hospital of Zhengzhou University from January 2019 to April 2022, their prenatal imaging, parental CMA verification, pregnancy outcomes and postpartum clinical phenotype were analyzed.
RESULTS
Eight cases of central 22q11.2 deletion syndrome were included, including six cases with LCR22B~D 22q11.2 deletions and two with LCR22C~D 22q11.2 deletions. Among the six cases with LCR22B~D type 22q11.2 deletions, three had shown cardiovascular malformations (right aortic arch, ventricular septal defect, mild tricuspid regurgitation), one had shown urinary defect (right kidney heterotopia). Two cases with LCR22C~D 22q11.2 deletions showed nonspecific ultrasonographic findings, including oligohydramnios with growth restriction and nuchal skin thickening. The CMA verification showed that six cases were inherited from their parents, and five couples had chosen to continue with the pregnancy. Postpartum follow-up showed that the physical and intellectual development of all children were normal. One couple had opted to terminate the pregnancy considering the ectopic fetal right kidney. Two remaining cases had decided to terminate their pregnancies without parental verification.
CONCLUSION
The central 22q11.2 deletion syndrome of the LCR22B/C~D type is different from the classical types. Its genetic information mainly comes from parents. Prenatal imaging has mainly shown cardiovascular and urinary abnormalities. Postnatal growth and intellectual development have been normal. Therefore, the couples should be provided with suffice prenatal genetic counseling.
Topics: Child; Female; Pregnancy; Humans; DiGeorge Syndrome; Prenatal Diagnosis; Fetal Diseases; Pregnancy Outcome; Heart Septal Defects, Ventricular
PubMed: 38311551
DOI: 10.3760/cma.j.cn511374-20221125-00812 -
Surgical and Radiologic Anatomy : SRA Apr 2024Fraser syndrome, named after George Fraser, is an autosomal recessive disorder showing a highly variable interfamilial phenotypic variation, with malformations ranging...
INTRODUCTION
Fraser syndrome, named after George Fraser, is an autosomal recessive disorder showing a highly variable interfamilial phenotypic variation, with malformations ranging from minor symptoms to lethal anomalies like renal agenesis, incompatible with survival. Limb reduction defects have not been reported to be associated with it.
CASE PRESENTATION
A 21-year-old primigravida presented to the antenatal outpatient department with a level two targeted anomaly scan report suggestive of severe oligohydramnios with suspected renal agenesis. The cranial vault bones were compressed, and orbital globes and lenses could not be visualized. Renal agenesis was confirmed due to sleeping adrenals sign, non-visualization of the urinary bladder, and Doppler of renal arteries. A detailed examination of the fetal head in the sagittal section showed the absence of an eye globe and lens, arousing suspicion of Fraser syndrome. After pregnancy termination, a complete fetal autopsy was done to look for any additional findings.
CONCLUSION
Patients who have a syndromic mix of acrofacial and urogenital abnormalities with or without cryptophthalmos should be evaluated for Fraser syndrome, which can be diagnosed by clinical examination and perinatal autopsy.
Topics: Humans; Female; Pregnancy; Young Adult; Adult; Fraser Syndrome; Syndactyly; Abnormalities, Multiple; Anatomic Variation; Congenital Abnormalities; Kidney; Kidney Diseases; Urogenital Abnormalities
PubMed: 38310170
DOI: 10.1007/s00276-024-03299-9 -
Zhonghua Er Ke Za Zhi = Chinese Journal... Feb 2024To develop a risk prediction model for identifying bronchopulmonary dysplasia (BPD) associated pulmonary hypertension (PH) in very premature infants. This was a...
To develop a risk prediction model for identifying bronchopulmonary dysplasia (BPD) associated pulmonary hypertension (PH) in very premature infants. This was a retrospective cohort study. The clinical data of 626 very premature infants whose gestational age <32 weeks and who suffered from BPD were collected from October 1, 2015 to December 31, 2021 of the Seventh Medical Center of the People's Liberation Army General Hospital as a modeling set. The clinical data of 229 very premature infants with BPD of Hunan Children's Hospital from January 1 , 2020 to December 31, 2021 were collected as a validation set for external verification. The very premature infants with BPD were divided into PH group and non PH group based on the echocardiogram after 36 weeks' corrected age in the modeling set and validation set, respectively. Univariate analysis was used to compare the basic clinical characteristics between groups, and collinearity exclusion was carried out between variables. The risk factors of BPD associated PH were further screened out by multivariate Logistic regression, and the risk assessment model was established based on these variables. The receiver operating characteristic (ROC) area under curve (AUC) and Hosmer-Lemeshow goodness-of-fit test were used to evaluate the model's discrimination and calibration power, respectively. And the calibration curve was used to evaluate the accuracy of the model and draw the nomogram. The bootstrap repeated sampling method was used for internal verification. Finally, decision curve analysis (DCA) to evaluate the clinical practicability of the model was used. A total of 626 very premature infants with BPD were included for modeling set, including 85 very premature infants in the PH group and 541 very premature infants in the non PH group. A total of 229 very premature infants with BPD were included for validation set, including 24 very premature infants in the PH group and 205 very premature infants in the non PH group. Univariate analysis of the modeling set found that 22 variables, such as artificial conception, fetal distress, gestational age, birth weight, small for gestational age, 1 minute Apgar score ≤7, antenatal corticosteroids, placental abruption, oligohydramnios, multiple pulmonary surfactant, neonatal respiratory distress syndrome (NRDS)>stage Ⅱ, early pulmonary hypertension, moderate-severe BPD, and hemodynamically significant patent ductus arteriosus (hsPDA) all had statistically significant influence between the PH group and the non PH group (all <0.05). Antenatal corticosteroids, fetal distress, NRDS >stage Ⅱ, hsPDA, pneumonia and days of invasive mechanical ventilation were identified as predictive variables and finally included to establish the Logistic regression model. The AUC of this model was 0.86 (95% 0.82-0.90), the cut-off value was 0.17, the sensitivity was 0.77, and the specificity was 0.84. Hosmer-Lemeshow goodness-of-fit test showed that 0.05. The AUC for external validation was 0.88, and the Hosmer-Lemeshow goodness-of-fit test suggested 0.05. A high sensitivity and specificity risk prediction model of PBD associated PH in very premature infants was established. This predictive model is useful for early clinical identification of infants at high risk of BPD associated PH.
Topics: Infant, Newborn; Infant; Child; Humans; Female; Pregnancy; Bronchopulmonary Dysplasia; Infant, Premature; Retrospective Studies; Hypertension, Pulmonary; Fetal Distress; Models, Statistical; Prognosis; Placenta; Infant, Premature, Diseases; Gestational Age; Respiratory Distress Syndrome, Newborn; Adrenal Cortex Hormones
PubMed: 38264812
DOI: 10.3760/cma.j.cn112140-20230912-00178 -
Frontiers in Medicine 2023The aim was to investigate gestational age at birth of women after induction of fetal lung maturation (antenatal corticosteroids = ACS) because of imminent preterm...
BACKGROUND
The aim was to investigate gestational age at birth of women after induction of fetal lung maturation (antenatal corticosteroids = ACS) because of imminent preterm birth (PTB), and to quantify incidence of late PTB (gestational age < 260 days) and extreme immaturity (gestational age < 196 days) in relation to several diagnoses (PPROM, placental bleeding, premature labor, preeclampsia, oligohydramnios, amniotic infection syndrome (AIS), cervical insufficiency) and risk factors (age > 35, history of preterm delivery, multifetal gestation, gestational diabetes, hypertension, nicotine abuse).
METHODS
The study was designed as a retrospective cohort trial, in which the data of all births taking place in 2016 in the German federal state Rhineland-Palatinate were evaluated. Frequency analyses, subgroup analysis (Chi-square tests and Friedman's tests), as well as multinomial logistic regressions and linear regressions were used to determine odds ratios (OR).
RESULTS
In total, = 1,544 patients were included who had been hospitalized due to an imminent PTB and had received ACS, of whom 52% had a late PTB and 8% a PTB with extreme immaturity. Regarding the gestational age at birth, there were only minor differences between the individual risk factors and diagnoses, only AIS patients showed a significantly lower gestational age (mean: 207 days). A significantly increased risk of PTB with extreme immaturity was found in patients with AIS (OR = 5.57) or placental bleeding (OR = 2.10).
CONCLUSION
There is a need for further research in order to be able to apply therapeutic measures more accurately in relation to risk factors and diagnoses.
PubMed: 38264055
DOI: 10.3389/fmed.2023.1285306