Congenital deformities of the diaphragm include (1) Hiatus hernia; (2) Congenital diaphragmatic hernia; (3) Eventration of deaphragm. Fifty-one cases were seen by the authors in the past 30 years. Each type of the pathologic feature and the experiences in the surgical repair were suggested in this paper. Choice of an appropriate surgical procedure on the basis of its pathoanatomic and pathophysiologic features was emphasized in order to enhance the efficacy of treatment.
【Abstract】Objective To evaluate the distribution of nerve growth factor receptor( P75 NGFR) in congenital choledochal cyst(CCC) and its clinical implication. Methods Specimens from 18 children with CCC and normal choledochal specimens from 9 controls were immuno-stained with P75 NGFR antibody. Results Extensive P75 NGFR staining was found in the nerve fibres of normal comnon bile duct，bly staining of ganglion cells were observed on the normal specimens. There was very little immunoreactive fibre in the CCC. Conclusion The abnormal distribution of P75 NGFR in the aganglionic choledochal suggests that abnormal P75 NGFR is related to the occurrance of the CCC.
The diagnosis and management of congenital heart disease (CHD), the most common inborn defect, has been a tremendous success of modern medicine. With the development of diagnostic techniques, surgical procedures and interventional techniques, more than 90% of CHD children can survive to adulthood. Consequently, the prevalence of patients with CHD has shifted away from infancy and childhood towards adulthood. Adult CHD cardiology is now encompassing not only young or middle-aged adults but also patients aged above 60 years. Standardized guidelines can provide good theoretical support for the comprehensive management of adult CHD. Ten years after the European Society of Cardiology guidelines for the management of grown-up CHD released in 2010, the new version was officially released in August 2020. The new version of guidelines updated the classification and stratification of diseases, comprehensive intervention methods and intervention timing, and put forward some new concepts, new intervention standards and methods. For adult CHD that has not been repaired or needs to be repaired again, the indication and mode of surgical intervention and perioperative management have a great impact on the prognosis. The new version of the guidelines provides a detailed description of the surgical and intervention indications and methods for different diseases, and clarifies the management methods for high-risk groups. This article attempts to interpret this newly updated guideline from the perspective of a surgeon, sort out several key diseases introduced by the guideline, and strives to provide a concise and actionable guideline for domestic counterparts.
Objective To explore the mechanism of pulmonary hypoplasia in case of congenital diaphragmatic hernia (CDH), and study the ultramicrostructural features of lung tissue of CDH fetal rat models at different developmental stages. Methods Seven SpragueDawley (SD) pregnant rats were randomly divided into CDH group (n=4) and control group (n=3). For the rats in the CDH group, Nitrofen was used to fill in the stomach once at day 9.5 of pregnancy (125 mg of Nitrofen dissolved in 2 ml of olive oil each), and 3, 10, 17 fetal rats were collected at day 16, 18 and 21 of pregnancy respectively. For the rats in the control group, 2 ml of olive oil was used to fill in the stomach, and 10 fetal rats were collected at day 16, 18, and 21 of pregnancy respectively. The lung tissue sections of the fetal rats collected on day 16 were observed under transmission electron microscope (TEM). For the lung tissue of the fetal rats collected on day 18, hematoxylineosin (HE) staining and TEM observation were performed and the incident of CDH was detected. Besides the procedures carried out for the rats collected on day 18, the ratio of fetal lung to body weight was observed for the lung tissue of the fetal rats collected on day 21. Results (1) The ratio of fetal lung to body weight of fetal rats in the CDH group was significantly lower than that of fetal rats in the control group (0.0238 vs. 0.0430, Plt;0.01). The incidences of CDH in the 18thday and 21stday fetal rats in the CDH group were 90.00% and 82.35%respectively, while no CDH was observed in the corresponding fetal rats in the control group, suggesting pulmonary hypoplasia in the CDH group. (2) The ultramicrostructural observation showed that compared with the control group, pulmonary hypoplasia appeared in 16thday fetal lungs in the CDH group, i.e., broad breathing barrier substrate, little contents, predominant euchromatin and rich ribosomes in the alveolar epithelial cells, and no microvilli in the bronchial lumen. The observation on the 18thday and 21stday samples suggested that, with the progressing of pregnancy, the abovementioned features became more obvious. (3) Typical lamellated body was observed in fetal lung type Ⅱ alveolar epithelial cells from the 21stday fetuses in both the CDH group and the control group, suggesting that some late subcellular structures were normal. Conclusion Lung hypoplasia develops in the early period of fetal rats with CDH rather than in the late period, implying that the treatment of pulmonary hypoplasia of diaphragmatic hernia should be performed in the early stage of lung development.
Objective To investigate the value of systemic-normothermic/cardiac-hypothermic cardiopulmonary bypass（CPB）on operation of congenital heart disease. Methods Thirty patients of congenital heart disease were randomly divided into two groups, the normothermia group（n=15）and hypothermia group（n=15）. The changes of CPB time, aortic cross-clamp time,operation time and postoperative drainage and the value of blood cell were observed. Results The duration of CPB （37. 5 ±11. 6rain vs. 51. 6± 12. 0 min, P〈0. 05） and operation time （2.2± 0.6h vs. 2. 7±0. 5h, P〈0. 01） in normothermia group were shorter than those of hypothermia group statistically, the differences of postoperative drainage and the value of blood cells between two groups were not statistically significant. Conclusion The use of systemic-normothermic/cardiac-hypothermic CPB on operation of congenital heart disease shows that the time of operation is shorter remarkly , and it could be clinically used safely.
Since October 1975, 35 patients with congenital constriction bands were treated. Follow-up for 10 months to 14 years showed that the results of correction were satisfactory except 1 patient died from other disease. The etiolology, the concurrent deformities and the method of treatment were discussed.
Objective To introduce a method of the surgicalcorrectionof Poland’s syndrome in children. Methods From May 1990 to May 2002, 3 female children with Poland’s syndrome were treated. One child of 12 years old with defects of the right second and third costal cartilages, pectoralis major and pectoralis minor underwent repair of chest wall with graft of autologous costal cartilage from left sixth costal cartilage and transfer of the latissimus dorsi flap. The other 2 children, 3 and 16 years old, with defects of theleft second, third and fourth costal cartilages, pectoralis major and pectoralis minor underwent graft of autogenous costal cartilage from the right sixth costal cartilage and implant of Dexon mesh. Results Three patients were followed up 1, 7 and 10 years postoperatively, respectively.The contour of chest wall in 3 patients were improved. The functions of the chest, back and upper limbs in the deformitis side were good and the growth and development of thechildren were normal during follow-up postoperatively.Conclusion The congenital deformities of multiple systems and parts in the body are involved in Poland’s syndrome so that it is necessary to make a proper surgical plan according to the extent of lesions for a satisfactory result.
ObjectiveTo explore the midterm therapeutic effect of modified Blalock-Taussing shunts (MBTs) in the treatment of tetralogy of Fallot.
MethodsWe retrospectively analyzed the clinical data of 69 children with tetralogy of Fallot undergoing MBTs in Shanghai Xinhua Hospital between July 2006 and January 2013. There were 44 males and 25 females with mean age of 17.97±24.73 months (ranged from 2 months to 10 years). The patients weighted from 4 to 24 (9.00±4.03) kg. All the MBTs between subclavian artery and pulmonary artery were performed through right or left posterior lateral incision.
ResultsThe patients were followed up for 6-36 months including 57 patients with 6 months following-up, 33 patients with 6 months and 12 months following-up, 16 patients with 12 months and 24 months following-up, and 11 patients with 24 months and 36 months following-up. There was significant growth in McGoon ratio during the first 12 months follow-up (preoperative vs. 6 months:1.09 ±0.33 vs. 1.40 ±0.40, P=0.00; 6 months vs. 12 months:1.29±0.31 vs. 1.36±0.33, P=0.00). There was no obvious growth in McGoon ratio after 12 months (12 months vs. 24 month:1.22±0.31 vs. 1.19±0.32, P=0.14; 24 months vs. 36 months:1.22±0.23 vs. 1.23±0.20, P=0.45). The left ventricular end diastolic volume index (LVEDVI) increased significantly in 6 months after MBTs (preoperative vs. 6 months:29.60±10.12 ml/m2 vs. 49.18±11.57 ml/m2, P=0.00), but there was no significant growth after 6 months. There was no significant decline in left ventricular ejection fraction (LVEF) after MBTs.
ConclusionThe MBTs can significantly promote the growth of McGoon ratio in 12 months of patients with tetralogy of Fallot, but there is no obvious growth of McGoon ratio after 12 months. MBTs can significantly improve left ventricular development within 6 months, and it won't lead to excessive expansion of the left ventricle when we extend follow-up time. The MBTs affects little on cardiac function of patients with tetralogy of Fallot.
ObjectiveTo summarize the research progress of one-stage posterior hemivertebra resection for congenital kyphoscoliosis.
MethodsThe domestic and foreign related literature about spinal hemivertebra deformity,and many aspects of its operation mode,operation timing,the fixed segment,and operation complications were summarized and analysed.
ResultsThe hemivertebra resection can remove teratogenic factors directly,and is favor by the majority of domestic and foreign physicians,but the procedure,indications,long-term effectiveness,and postoperative complications are still unconcern,and the operation timing and fixed-fused segment is still controversial.
ConclusionThe operation timing and the fixed segment of one-stage posterior hemivertebra resection for congenital kyphoscoliosis need further research.
Objective
To investigate the early motor development and the risk factors affecting motor development in children with congenital muscular torticollis (CMT) aged 0–3 months.
Methods
CMT infants admitting to the Department of Rehabilitation Medicine, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine between January 1st, 2016 and April 30th, 2018 were enrolled as CMT group, and contemporaneous age-matched healthy infants were enrolled as the control group. Motor development was assessed with the Alberta Infant Motor scale (AIMS). We collected the birth weight, birth length, mode of birth, sleep position, and prone time when awake as dependent variables, and used multiple linear regression to find the variables that had significant effect on AIMS scores.
Results
There were 97 CMT infants (62 males and 35 females) with the mean age of (46.8±17.3) days, mean birth weight of (3.34±0.38) kg and mean birth length of (49.56±0.93) cm in the CMT group, while there were 97 healthy infants (60 males and 37 females) with the mean age of (45.1±19.4) days, mean birth weight of (3.38±0.35) kg and mean birth length of (49.84±1.03) cm in the control group, and the differences in sex, age, birth weight, birth length between the two groups were not statistically significant (P>0.05). AIMS centiles showed that 36 infants (37.1%) in CMT group had suspicious or abnormal motor development, while only 12 infants (12.4%) in the control group had; there was a significant statistical difference between the two groups (χ2=15.945, P<0.001). Multiple linear regression analysis showed that the time of prone position when awake and CMT had significant influence on the AIMS scores (F=64.851, P<0.001). Infants who had a long prone position when awake had significantly higher AIMS scores and CMT had a significantly lower AIMS scores (P<0.001).
Conclusions
The risk of early motor retardation in infants with CMT aged 0–3 months is higher than that in healthy infants of the same age. The decrease in prone position when awake and CMT may be the causes of delayed motor development. Clinical medical personnel and family caregivers should pay more attention to motor development and provide reasonable intervention to CMT infants.
