Objective To discuss the value of intrapartum operation in management of birth defects and the prognosis. Methods From August 2008 to November 2009, 11 fetuses were identified with birth defects through 3D color Doppler ultrasound and confirmed by MRI and fetal karyotype in the Maternal Fetal Medicine Center, Affiliated Shengjing Hospital, China Medical University including three lymphangiomas,two congenital diaphragmatic hernias (CDH), one sacrococcygeal teratoma, three omphalocele and two gastroschisi. All the above identified birth defects were indications for surgery. All fetuses were born abdominally and received intrapartum operations, including three intrapartum fetal operations with placental infusion (two repairs of CDH, one sacrococcygeal teratoma resection), six ex-utero intrapartum treatment (EXIT; two repairs of omphalocele, two repairs of gastroschisi, two lymphangioma resection) and two surgeries in house (one omphalocele repair and one lymphangioma resection). Both the mothers and fetuses were regularly followed up. Results ( 1 ) Operations: the average operating time for the three intrapartum fetal operations was 89 minites, 5.5 minites for the six EXIT, during which EXIT was performed first,followed by blocking the umbilical circulation and neonatal surgery, and 37 minites for the two surgeries in house. All neonates survived except for one death from severe CDH at 3.5 hours after the operation. The average blood loss for cesarean section and fetal operation was 275 ml. All mothers recovered soon without fever or infection and were discharged three to five days after the operation. (2) Follow-ups: the ten survived neonates were followed up at 1 - 18 mouths at the pediatric clinics and all were growing and developing normally except for one baby with gastroschisi suffered from enteral torsion and feeding intolerance showed lower weight than babies at the same age, but catched up to normal at four months old after posture therapy. One baby with mild CDH developed pulmonary infection at two months after operation with 1/4 pneumothorax on chest X-ray, and were hospitalized for two weeks. At six months old, patent ductus arteriosus was diagnosed in the same baby and chest X-ray was normal. The baby with omphalocele was complicated with ventricular septal defect before operation and the cardiac function was normal during followups for one year. The baby with sacrococcygeal teratoma was reported to have no automatic micturition, but recovered to normal at one month of age. Conclusion Babies with certain birth defects can be managed through intrapartum operation with better outcomes.

Objective:To meet the individualized needs of residents in standardized residency training of cardiac intervention specialty for cardiac ultrasound teaching, to construct a bionic 3D printing model of ultrasound source, and to discuss its application value in ultrasound teaching and simulation exercises.Methods:Sixty residents majored in cardiovascular medicine receiving standardized residency training of ultrasound in ultrasound imaging department of Renmin Hospital of Wuhan University were randomized into experimental group and control group in average. The experimental group adopted ultrasound bionic 3D printing heart model combined with problem-based learning (PBL) teaching method, and the control group adopted traditional multimedia PPT combined with PBL teaching method. At the end of teaching activities, theoretical examination, operation assessment and questionnaire survey were conducted to evaluate and compare the teaching effect between the two groups. SPSS 21.0 software was performed for t test. Results:The theoretical examination scores of the experimental group and the control group were (81.4±8.2) points and (74.8±9.4) points, respectively, P=0.002, and the operation assessment scores were (89.1±5.6) points and (71.5±8.8) points, respectively, P<0.001. The questionnaire survey showed that the experimental group had better feedbacks than the control group in the aspects of mastery of heart anatomy knowledge, learning experience, learning interest, learning efficiency, understanding of interventional heart disease, clinical skills of interventional operation and overall satisfaction (all P<0.05). Conclusions:Ultrasound bionic 3D printing combined with PBL teaching method can help strengthen the mastery of cardiac anatomy knowledge, promote the learning experience of cardiac ultrasound and improve the skills of interventional operation. The teaching effect is significant and the students' satisfaction is high.

Objective To investigate the feasibility,security,indication and prophylactic measures of postpartum hemorrhage during the fetalintrapartum operation by analyze the maternal prognosis after intrapartum fetal operation(IFO) performed.Methods We performed a retrospective evaluation of 10 cases that has been performed intrapartum fetal operation(IFO group,n=10) successfully between August 2008 and October 2009 at Department of Obstetrics and Gynecology,Shengjing Hospital,China Medical University.Healthy pregnant women(n=10) were chosen as control group,who received Caesarean section for refusing to vaginal delivery.Between the two groups,we compared the indexes including blood lose during the operation and 24 hours post-operation,the hemoglobin level pre-operation and 24 hours post-operation,the involution of uterus,the puerperal infection,and the follow-up at 42 days post-operation.Results Good involution,no primary and secondary postpartum hemorrhage and no puerperal infection were found in both of the 2 groups.Intra-operation,24 hours post-operation and the total volumes of blood lose,the hemoglobin level before operation,24 hours post-operation and 42 days post-operation had no statistic significant difference between the IFO group and control group(P>0.05).No significant difference was found in the operation time between IFO group and the control group(66.40±53.40 minutes and 34.50±4.97 minutes,respectively.Intra-operation,24 hours post-operation and the total volume of blood lose had no relation to the maternal age and gestational weeks(P>0.05).The blood lose of intra-operation in IFO group had positive linear correlation to the operation time.(Pearson relation coefficient R=0.458,.P=0.021).The linear recurrence equation was:the volumes of blood lose intra-operation(ml)=172.68+1.342x(operation time).Conclusion IFO was safe to the mother.It did not affect the involution of uterus and could not increase the blood lose of postpartum and the puerperal infection morbidity.Through the effective measures of preventing postpartum hemorrhage,the postpartum blood lose of the patients that performed intrapartum fetal operation could be no more than that of the patients that had normal Caesarean section.Improving the technique of the intrapartum fetus operation and shortening the operation time are important to prevent postpartum hemorrhage.

OBJECTIVE: To explore the prenatal ultrasonographic features and genetic basis for an abortus suspected for type II Cornelia de Lange syndrome (CdLS2). METHODS: A fetus diagnosed with CdLS2 at the Shengjing Hospital Affiliated to China Medical University on September 3, 2019 was selected as the study subject. Clinical data of the fetus and family history was collected. Following induced labor, whole exome sequencing was carried out on the abortus. Candidate variant was verified by Sanger sequencing and bioinformatic analysis. RESULTS: Prenatal ultrasonography (33 weeks of pregnancy) has revealed multiple anomalies in the fetus, which included slightly widened cavity of septum pellucidum, blurred corpus callosum, slightly reduced frontal lobe volume, thin cortex, fusion of lateral ventricles, polyhydramnios, small stomach bubble, and digestive tract atresia. Whole exome sequencing has revealed a heterozygous c.2076delA (p.Lys692Asnfs*27) frameshifting variant in the SMC1A gene, which was found in neither parent and was rated as pathogenic based on the guidelines of American College of Medical Genetics and Genomics (ACMG). CONCLUSION The CdLS2 in this fetus may be attributed to the c.2076delA variant of the SMC1A gene. Above finding has provided a basis for genetic counseling and assessment of reproductive risk for this family.
Pregnancy ; Female ; Humans ; Cell Cycle Proteins/genetics* ; De Lange Syndrome/diagnosis* ; Phenotype ; Ultrasonography, Prenatal ; Fetus/diagnostic imaging* ; Mutation

Objective:To evaluate the effect of preoperative cognitive behavioral therapy (CBT) on pain catastrophizing in the patients with orthopedic trauma.Methods:A total of 120 patients with lower extremity bone trauma, aged 18-64 yr, of American Society of Anesthesiologists physical status Ⅰor Ⅱ, with body mass index of 18-28 kg/m 2, with Pain Catastrophic Scale (PCS) score on admission >16, scheduled for surgical treatment, were enrolled.The patients were divided into 2 groups ( n=60 each) by the stratified randomization method based on the type of fracture: CBT group and routine group (group R). Group CBT received CBT for pain through the internet on the day of admission and one day before operation.The patients in both groups underwent reduction and internal fixation of lower extremity fractures under combined spinal-epidural anesthesia.The PCS scores were recorded immediately after admission and on the morning of the operation day.The effective pressing times of the patient-controlled analgesia pump, consumption of analgesics for rescue analgesia, and occurrence of nausea and vomiting within 48 h after operation were recorded.The visual analogue scale score of the surgical site during activity and occurrence of the score >3 at 3 months after operation and use of opioids within 3 months after operation were recorded. Results:Compared with group R, the PCS score was significantly decreased on the morning of the operation day, the pressing times of the patient-controlled analgesia pump, consumption of analgesics for rescue analgesia and incidence of nausea and vomiting within 48 h after operation were decreased, the requirement for opioids within 3 months after operation was decreased ( P<0.05), and no significant change was found in VAS score during activity and occurrence of the score >3 at 3 months after operation in group CBT ( P>0.05). Conclusions:Preoperative CBT can reduce the degree of pain catastrophizing and is helpful in increasing the quality of postoperative analgesia in the patients with orthopedic trauma.