Objective To explore the factors which are related to the reoperation and relapse hiatus hernia in children.Methods Between Jan.2002 and May 2013,64 patients who suffered from hiatus hernia in Children's Hospital of Fudan University were divided into uneventful group (U group,n =49) and reoperation group (R group,n =15).The ages at operation,barium swallow examinations,operative procedures,findings during the operation and the followup of these two groups were reviewed.Results The average ages at operation in U group and R group were (14.70 ± 0.79) months and (13.60 ± 0.59) months,respectively.The major symptoms before the second operation in R group was vomiting(6 cases),pneumonia (4 cases) and dysphagia (2 cases).Asymptomatic recurrence was found in 3 patients.Through barium swallow examination,short esophagus was found in 10 cases in U group,while 3 cases were reported in R group.However,during the operation,the length of intra-abdominal esophagus without tension could be obtained with average (3.33 ± 0.86) cm in these short esophagus patients,which was only (3.18 ± 1.14) cm in those normal esophagus patients.There was no significant difference between the U group and R group in the width of the hiatus and the ratio of stomach above the hiatus.The length of esophagus which mobilized during operation was shorter in R group than that in U group(P =0.003).The difference of operative methods and the ratio of large hernia between these two groups could not be found.The common cause of failure in R group was herniated fundoplication (9 cases),which was followed by disputed fundoplication (4 cases),twisted fundoplication (1 case) and rupture of esophagus (1 case).The average follow up time was (8.3 ± 4.2) months in R group.Vomiting was found in 3 patients,and pneumonia was found in one case in R group after the second operation.The symptoms of these four patients were relieved half year later postoperatively.Conclusions The age,symptoms,size of the hiatus,short esophagus,and the operative methods are not related to the reoperation in hiatus hernia.But if the length of intra-abdominal esophagus without tension was not obtained enough,it may contribute to the recurrence of hiatus hernia.Wrap herniation has now become the most common mechanism of failure requiring reoperation.

Objective To summarize the experience of multidisciplinary consultation for prenatal fetal deformity, and to explore the mode suitable for China. Methods The Obstetrics and Gynecology Hospital of Fudan University and Children's Hospital of Fudan University established a joint multidisciplinary consultation center, including obstetrics, pediatrics, pediatric surgery, ultrasound and other departments. A total of 3 378 pregnant women visited the consultation center from July 31, 2003 to August 1, 2013. After consultation, treatment was divided into three classes:pregnancy termination, pregnancy continuation and perinatal treatment. Follow-up was made through correspondence and telephone communication. Retrospective analysis on reasons for consultation, fetal structural abnormalities of the classification system, chronological order of abnormalities, gestational weeks of diagnosis, maternal-related factors, treatment and prognosis was performed. Results (1) Reasons for consultation:Among 3 378 women undertaking prenatal multidisciplinary consultation, 3 243 (96.00%) were due to fetal factors, and 135 (4.00%) were due to maternal factors. (2) Classification of fetal structural abnormalities:Among the 3 243 cases undertaking consultation with fetal factors, fetal abnormality was found in 80.85%(2 622/3 243). The most common were neurological abnormalities(23.19%, 608/2 622), followed by urinary tract malformation (20.25%, 531/2 622) and cardiovascular malformation (15.48%, 406/2 622). These were followed by digestive system malformation, limb deformities and space-occupying lesions. There were 156 cases of multiple malformations. (3) Average gestational weeks for diagnosis of fetal deformity:The 2 622 cases of fetal deformity were diagnosed at a mean (26.7± 2.1) of gestational weeks (21.1–30.4 weeks). Urinary tract malformations were detected at (24.0±0.7) weeks, whereas digestive system malformations were detected at (28.3±2.6) weeks. (4) Induced labor:Induced labor cases accounted for 35.66% (935/2 622), among which, 92 cases were fetal intrauterine death and 843 cases were active choice. The several highest induced labor rates resulted from multiple malformations (75.64%, 118/156), abdominal wall defects (62.22%, 28/45), diaphragmatic hernia (61.54%, 24/39), cleft lip and palate (55.32%, 26/47) and cardiovascular malformations (49.51%, 201/406). For nervous system (27.80%, 169/608), urinary tract (25.80%, 137/531) and digestive system malformations (26.94%, 66/245), induced labor rates were <30%. For abdominal lesions (14.04%, 25/178) and sacrococcygeal teratoma (13.64%, 3/22), induced labor rates were<15%. (5) Continuation of pregnancy in 1 687 cases:Cesarean section was conducted in 1 046(61.94%). Neonatal death occurred in 117(6.94%).(6) Perinatal treatment:Twenty-one cases were treated during pregnancy, including thirteen cases with fetal ascites and hydrothorax treated by drainage, five cases with fetal anemia treated by intrauterine transfusion and three cases with fetal tachycardia treated by digoxin. Ten cases were treated by ex-utero intrapartum treatment. After birth, 297 newborns immediately underwent neonatal surgery. Among these, 259 cases underwent radical surgery, eleven palliative surgery, and sixteen elective surgery after follow-up. Conclusions Prenatal multidisciplinary consultation can make comprehensive multidisciplinary assessment of fetal prognosis and improve the diagnosis and treatment of structural malformations.

Objective Radiological grade of splenic injury was seldomly used in China trauma center now,though it had been established in 1994 by American Association for Surgery of Trauma (AAST) and widely used.The present study is aimed to analyze the imaging grade and clinical characteristics of traumatic splenic rupture in children,discuss the feasibility of conservative treatment,and the role of radiographic grading during clinical treatment.Methods Information (including age,gender,severity based on radiological findings,treatment strategies,and clinical outcome) regarding 59 hospitalized splenic injury patients whose injuries occurred between 2008 and 2014 was retrospectively analyzed.Results Between 2008 and 2014,59 pediatric patients with splenic injury were treated in our institution.Median age was 9.5 years (range,3 months to 16 years).Of all patients,41 (69.5%) were male.The injuries were primarily caused by traffic crash (45.7%),stumbling/falling from a height (38.9%).According to AAST,5 cases were grade Ⅰ,26 patients grade Ⅱ (44.1%),and 21 cases grade Ⅲ (35.6%),6 over grade Ⅳ,and only one was unclear.Of all patients,25 cases were with the other organs complications.All patients underwent fasting,bed rest,and antibiotics.Only 1 case was transferred to operation during the conservative treatment.Forty-nine patients underwent with CT scan over 2 times.Conclusion Imaging classification helps guide clinical treatment.Conservative treatment is feasible for traumatic splenic injury in children.Early imaging classification of splenic injury may be helpful in clinical judgment,and reduce children radiation exposure.

OBJECTIVE: To explore the genetic basis of a child with Very early onset inflammatory bowel disease (VEOIBD). METHODS: A female child who had presented at the Children's Hospital of Fudan University on May 23, 2018 due to occurrence of diarrhea and fever 6 days after birth was selected as the study subject. Clinical data of the child was collected. Family-based whole-exome sequencing (WES) was carried out. Candidate variant was verified by Sanger sequencing and PCR of the patient and her parents. RESULTS: The child had developed the symptoms 6 days after birth, with main manifestations including diarrhea, fever, failure to thrive, rectovestibular fistula and hypothyroidism. An enterostomy was performed at the age of 3.5 months due to severe intestinal adhesion and obstruction. Based on her clinical manifestations, colonoscopic finding, and results of biopsies, she was diagnosed with VEOIBD in conjunct with congenital hypothyroidism. Replacement treatment of levothyroxine was given since one month of age. Family-based WES revealed that the child has harbored compound heterozygous variants of the DUOX2 gene, namely c.2654G>T (p.R885L) and c.505C>T (p.R169W), in addition with a heterozygous c.301C>T (p.R101W) variant of the IL10RA gene. Re-analysis of the WES data revealed that the patient also had a 333 bp deletion spanning exon 1 of the IL10RA gene (Chr11: 117857034_117857366). CONCLUSION For patients with VEOIBD, genetic testing is recommended. Presence of additional DUOX2 gene variants might have exacerbated the clinical symptoms in this patient. Above finding has facilitated genetic counseling and prenatal diagnosis for this family, and raised clinicians' awareness of this rare disease.
Female ; Humans ; Infant ; Pregnancy ; Diarrhea ; Dual Oxidases/genetics* ; Exons ; Failure to Thrive ; Inflammatory Bowel Diseases/genetics*

Objective: To study the feasibility of ¹⁸F-fluoro-L-dihydroxyphenylalanine positron emission tomography/Computed tomography (¹⁸F-DOPA PET/CT) scanning in the localization and differential diagnosing of focal versus diffuse form of pancreas lesions in patients with hyperinsulinemic hypoglycemia (HH). Method: Twenty-four patients were diagnosed with HH between January, 2016 and February, 2017 in the Department of Pediatric Endocrinology and Inherited Metabolic Diseases, Children′s Hospital of Fudan University using an integrated clinical and biochemical diagnostic protocol, domestic ¹⁸F-DOPA PET/CT imaging technique were applied after MRI and ultrasound failed to detect pancreas lesions. Pancreas ¹⁸F-DOPA standardized uptake values (SUV) were measured, and pancreas′ lesions were dually analyzed via visual method and pancreas percentage SUV method. Among these patients, 9 patients received surgical pancreatic lesion resections, the correlations among surgical outcomes, histopathological findings and ¹⁸F-DOPA PET/CT scan results were analyzed. Result: Seven patients were detected with focal form of pancreas lesions, the mean peak of SUV was 4.7±1.7(2.6-7.1), and 17 patients were found to have diffuse form lesions after ¹⁸F-DOPA-PET/CT scanning. Among the 24 cases, 9 patients (7 showed focal and 2 showed diffuse ¹⁸F-DOPA PET/CT pancreatic uptake)were euglycemic without any medical support after surgery; the resected pancreatic tissue histopathological results were consistent with that of PET/CT imaging. Only one patient, who responded to medical treatment before surgery, had temporary hyperglycemia after operation. Conclusion Domestic ¹⁸F-DOPA PET/CT could successfully locate and differentiate the pancreatic lesions and thus improve the success of surgery.

Objective:To investigate the clinical outcomes and prognosis of children who were prenatally diagnosed with solid space-occupying abdominal lesions by ultrasound.Methods:This study retrospectively enrolled 30 children with solid space-occupying abdominal lesions that were indicated by prenatal ultrasound at Children's Hospital of Fudan University from March 2015 to March 2021. Prenatal ultrasound findings, postnatal treatment, clinical outcomes, and prognosis were analyzed.Results:These subjects included 18 male and 12 female infants, with the median gestational age at the first sonographic diagnosis of 36 weeks (28 to 39 weeks). The intra-abdominal solid masses were postnatally confirmed to be located in liver, retroperitoneum, and gastrointestinal tract, which were hepatic hemangioma ( n=14), hepatoblastoma ( n=2), neuroblastoma ( n=6), abdominal teratoma ( n=4), adrenal hematoma ( n=1), adrenocortical adenoma ( n=1), hyperplasia of renal capillary ( n=1), and gastrointestinal teratoma ( n=1). The accuracy of prenatal ultrasound in detecting the location of masses was 73% (22/30) and which was 13/16 in detecting masses from liver and 9/14 in that outside the liver. Among the 30 cases, 73% (22/30) were benign tumors, and 27% (8/30) were malignant tumors (hepatoblastoma in two cases and neuroblastoma in six cases). Among 15 patients with benign tumors (hepatic hemangioma and adrenal hematoma) who received close follow-up or drug therapy, tumor/lesion regression occurred in 13 cases and the other two were observed with stable hepatic hemangiomas. Fourteen patients, including six with neuroblastoma, two with hepatoblastoma, five with teratoma, one with adrenocortical adenoma, and one with hyperplasia of renal capillary, had good prognosis after primary tumor resection or combined with postoperative chemotherapy. One hepatoblastoma case died after withdrawing treatment. The overall survival rate was 97% (29/30) with a median follow-up time of 24 months (4 to 60 months). Conclusions:Prenatal ultrasound has high accuracy in identifying the anatomic region of fetal solid space-occupying abdominal lesions. With close postnatal follow-up and proper treatment, most of the affected fetuses will have a good outcome and prognosis.

Objective:To investigate the effects of improving the prenatal multidisciplinary consultation mode on the outcomes of fetuses with structural malformations.Methods:Clinical data of pregnant women attending the Prenatal Multidisciplinary Consultation Center, jointly established by the Obstetrics & Gynecology Hospital of Fudan University and the Children's Hospital of Fudan University from January 2004 to December 2019, were retrospectively collected and analyzed. In 2014, the center optimized the multidisciplinary consultation mode to achieve a more individualized approach to genetic testing based on more accurate imaging diagnosis and deeper cooperation between the obstetrics and pediatrics teams. Differences in the number of cases, structure of the diseases, genetic testing results, outcomes, and prognosis between the improved group (enrolled from January 2014 to December 2019) and the baseline group (enrolled from January 2004 to December 2013) were compared. The Chi-square test was used for statistical analysis. Results:(1) This study recruited 5 977 pregnant women, including 3 424 in the baseline group and 2 553 in the improved group. The main indications for consultation were fetal factors [97.2% (5 812/5 977)], among which congenital structural malformations accounted for 77.5% (4 503/5 812). There was a significant difference in the systematic distribution of congenital structural malformations between the two groups ( χ2=141.31, P<0.001). The proportion of malformations involving the central nervous, cardiovascular, and urinary systems ranked in the top three in both groups. (2) The percentage of women receiving genetic testing was higher in the improved group than in the baseline group [26.7% (682/2 553) vs. 15.9% (546/3 424), χ2=103.87, P<0.001] and the positive rate of genetic testing was also higher in the improved group [19.9% (136/682) vs. 9.9% (54/546). χ2=23.42, P<0.001]. (3) Among the 5 977 cases, 418 (7.0%) were lost to follow-up; 1 741 (29.1%) opted for pregnancy termination; 123 (2.2%) had intrauterine fetal death; and 3 695 (61.8%) were live births. The rate of pregnancy termination in the improved group was lower than that of the baseline group [27.7% (707/2 553) vs. 30.2% (1 034/3 424), χ2=4.45, P=0.035]. (4) In the 1 741 cases with pregnancy termination, fatal cardiovascular system malformations ( n=413, 23.7%), central nervous system malformations ( n=377, 21.7%), multiple malformations ( n=258, 14.8%), and chromosomal abnormalities ( n=162, 9.3%) were the main causes. The top five diseases leading to pregnancy termination were cleft lip and palate [59.0% (46/78)], meningocele (5/9), gastroschisis/omphalocele [49.3% (33/67)], diaphragmatic hernia [46.5% (33/71)], and skeletal malformations [40.9% (83/203)]. The rates of pregnancy termination due to gastroschisis/omphalocele, diaphragmatic hernia, and skeletal malformations in the improved group were all lower than those in the baseline group [57.4% (27/47) vs. 30.0% (6/20), χ2=4.23; 59.0% (23/39) vs. 31.3% (10/32), χ2=5.43; 51.8% (72/139) vs. 17.2% (11/64), χ2=21.72; all P<0.05]. (5) Among the 3 695 live births, 1 979 (53.6%) were delivered by cesarean section and 1 716 (46.4%) by vaginal delivery; 3 633 (98.3%) survived and 62 (1.7%) died in the neonatal period. The survival rate of newborns in the improved group was higher than that in the baseline group [98.8% (1 648/1 668) vs. 97.9% (1 985/2 027), χ2=4.23, P=0.040]. Among the 62 dead newborns, 51 (82.3%) had fatal structural or chromosomal abnormalities. (6) In the surviving neonates, 372 (10.2%) showed no abnormalities in a postnatal reexamination, and 468 (12.9%) received surgical treatment in the neonatal period. The other 2 793 cases received no treatment in the neonatal period but were followed up regularly. Conclusion:The optimized prenatal multidisciplinary consultation mode effectively reduces the rate of pregnancy termination due to non-fatal single structural malformations and increases the survival rate of newborns.

Objective: To study the segment of liver according to the large amount of three-dimensional(3D) reconstructive images of normal human livers and the vascular system, and to recognize the basic functional liver unit based on the anatomic features of the intrahepatic portal veins. Methods: The enhanced CT primitive DICOM files of 1 260 normal human livers from different age groups who treated from October 2013 to February 2017 provided by 16 hospitals were analyzed using the computer-aided surgery system.The 3D liver and liver vascular system were reconstructed, and the digital liver 3D model was established.The vascular morphology, anatomical features, and anatomical distributions of intrahepatic portal veins were statistically analyzed. Results: The digital liver model obtained from the 3D reconstruction of CAS displayed clear intrahepatic portal vein vessels of level four.Perform a digital liver segments study based on the analysis of level four vascular distribution areas.As the less anatomical variation of left hepatic portal vein, the liver was classified into four types of liver segmentation mainly based on right hepatic portal vein.Type A was similar to Couinaud or Cho′s segmentation, containing 8 segments(537 cases, 42.62%). Type B contained 9 segments as there are three ramifications of right-anterior portal vein(464 cases, 36.82%). The main difference for Type C was the variation of right-posterior portal vein which was sector shape(102 cases, 8.10%). Type D contained the cases with special portal vein variations, which needs three-dimensional simulation to design individualized liver resection plan(157 cases, 12.46%). These results showed that there was no significant difference in liver segmental typing between genders(χ²=2.179, P=0.536) and did not reveal any significant difference in liver segmental typing among the different age groups(χ²=0.357, P=0.949). Conclusions The 3D digital liver model can demonstrate the true 3D anatomical structures, and its spatial vascular variations.The observation of anatomic features, distribution areas of intrahepatic portal veins and individualized liver segmentation achieved via digital medical 3D visualization technology is of great value for understand the complexity of liver anatomy and to guide the precise hepatectomy.

OBJECTIVETo summarize the experience of the chemotherapy regimen cisplatin + fluorouracil + vincristine (C5V) for hepatoblastoma, and analyze the factors associated the outcome.

METHODA retrospective analysis was conducted for the outcome of hepatoblastoma. Sixty-three patients who received the regimen of C5V as the first choice of chemotherapy were reviewed, including 37 males and 26 females. The age at diagnosis ranged from 2 days after birth to 124 months, median 15 months. Four patients with stage I, 16 patients with stage II, 28 patients with stage III, 15 patients with stage IV disease were enrolled in the study. Nine patients had primary tumor resection while the remain by 54 received neoadjuvant chemotherapy. The median follow-up time was 30 months.

RESULTForty patients had delayed surgery, including 35 patients with regimen C5V alone, the others were treated with regimen C5V and cisplatin + adriamycin (CITA). The mean time of neojuvant chemotherapy was (3.4 ± 1.7) cycles. The mean time of chemotherapy after surgery was (5.3 ± 2.0) cycles. In 12 cases the (24.5%) tumor recurred after surgery. The margin of resection less than 0.5 cm , vascular invasion, stage III or IV disease were all the high risks of relapse (P = 0.049,0.001,0.022, respectively). Two-year overall survival (OS) and 5-year OS of the study was 61.1% and 58.7%, respectively. The 2-year OS and 5-year OS of stage I to III were 75.0% and 75.0%, 100.0% and 100.0%, 65.8% and 61.4%. The 1-year OS and 3-year OS of stage IV was 20.0%, 13.3%, respectively. Univariate analysis showed that age at diagnosis less than 60 months, vascular invasion, thrombocythemia at diagnosis, stage III or IV, tumor resection was the prognostic factor (P = 0.019, <0.001,0.011, <0.001, respectively). Multivariate analysis showed that tumor resection and age at diagnosis less than 60 months were both the prognostic factor (P < 0.001, 0.004, respectively ).

CONCLUSIONThe regimen of C5V is useful for hepatoblastoma. Tumor resection is the key factor of treatment. Prognostic factor is composed of age, stage, and clinical sign at diagnosis.

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Child ; Child, Preschool ; Cisplatin ; administration & dosage ; Doxorubicin ; administration & dosage ; Female ; Fluorouracil ; administration & dosage ; Hepatoblastoma ; drug therapy ; Humans ; Infant ; Infant, Newborn ; Liver Neoplasms ; drug therapy ; Male ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Retrospective Studies ; Vincristine ; administration & dosage