Objective:To evaluate the predictive value of postnatal esophageal deviation index for clinical outcomes of fetuses with left-sided congenital diaphragmatic hernia (L-CDH).Methods:This retrospective study analyzed the clinical data of 103 neonates with prenatally diagnosed L-CDH who were admitted to Guangzhou Women and Children's Medical Center from January 2016 to February 2023. These patients were divided into the survival group ( n=82) and the death group ( n=21) according to the outcomes, and the extracorporeal membrane oxygenation (ECMO) group ( n=25) and the non-ECMO group ( n=78) according to whether ECMO support was required. Thoracoabdominal X-ray screening was performed on all neonates within 24 h after admission and the esophageal deviation index and cardiac deviation index were calculated. Independent sample t-test or Fisher's exact probability test were used to analyze the differences in general condition and postnatal imaging features between different groups. Receiver operating characteristic (ROC) curve was used to evaluate the value of postnatal imaging features in predicting the prognosis of L-CDH. Results:The esophageal deviation index and the cardiac deviation index of neonates in the survival group were lower than those in the death group [(10.5±5.3)% vs. (18.0±4.5)%, t=－5.47; (37.7±7.1)% vs. (42.8±8.2)%, t=－2.62; both P<0.05], while that were both higher in the ECMO group compared with the non-ECMO group [(18.0±4.3)% vs. (10.1±5.2)%, t=6.34; (42.4±7.9)% vs. (37.6±7.1)%, t=2.63; both P<0.05]. ROC curve showed that the area under the curve (AUC) for predicting the need for ECMO support was 0.879 (95% CI: 0.805-0.953) for esophageal deviation index and 0.712 (95% CI: 0.570-0.854) for cardiac deviation index, with the optimal cut-off values of 11.7% and 41.7%, respectively. The AUC for predicting the survival rate in patients with L-CDH by esophageal deviation index and cardiac deviation index were 0.854 (95% CI: 0.761-0.947) and 0.735 (95% CI: 0.582-0.887), respectively, with the corresponding optimal cut-off values of 15.8% and 41.7%. Conclusion:Postnatal esophageal deviation index is of value in predicting the need for ECMO support and survival rate in patients with L-CDH.

This article reported a case of kaposiform lymphangiomatosis (KLA) identified in the fetal stage and diagnosed at the neonatal stage. A routine ultrasound examination at 19 weeks of gestation showed multiple masses in the whole body of the fetus (involving neck, chest wall and armpit) complicated by pleural and peritoneal effusion. Shunting was performed to drain pleural effusion from the right chest in another hospital at 26 +5 weeks of gestation. The patient was born at 34 +3 weeks of gestation by cesarean section due to "intrauterine distress" and required invasive ventilator assisted ventilation support after birth because of respiratory distress. A large amount of hemorrhagic effusion was drained out during the shunting. Coagulation dysfunction and thrombocytopenia occurred on the 3rd day after birth and KLA was suspected. Empirical treatment with sirolimus turned out to be ineffective. Biopsy was taken on postnatal day 7. However, the patient died on the 12th day after birth due to respiratory and circulatory failure. Pathological findings obtained the day after death were consistent with the features of KLA. The diagnosis of KLA was confirmed based on the clinical manifestations and pathological results.

Objective:To investigate the clinical features and management of right-sided congenital diaphragmatic hernia (RCDH) with hepatopulmonary fusion (HPF).Methods:This retrospective study analyzed the clinical characteristics of three cases of RCDH complicated by HPF that were treated in Guangzhou Women and Children's Medical Center from June to December 2022. Diaphragm defects in the three cases were classified according to the international standard of diaphragm defect classification. Besides, an extensive search of publications was performed including domestic and foreign databases, including CNKI, Wanfang Database, Yiigle, VIP Chinese journals, PubMed, Embase and UpToDate databases from January 1997 to April 2023 using terms including "congenital diaphragmatic hernia" and "hepatopulmonary fusion". Clinical features and prognosis of RCDH complicated by HPF were summarized.Results:(1) Cases in the present study: RCDH was found in case 1 and case 2 during routine prenatal ultrasound examination; antenatal fetal MRI showed partial displacement of the hepatocele into the right hemithorax, right lung hypoplasia, a normal-sized left lung and without left shift of the mediastinum in both cases. Postnatal chest radiographs of case 1 and case 3 showed dense shadow in the left lung and mediastinum shifted to the right. Case 2 had a D-type defect and a slight shift of the mediastinum to the left was observed on the postnatal chest radiograph. Preoperative imaging findings indicated highly suspected HPF in the three cases. Case 1 and case 2 had complete separation of liver and lung and underwent diaphragmatic herniorrhaphy with patch. Partial lung resection was performed in case 2. Both case 1 and case 2 survived (length of hospital stay was 22 d and 23 d, respectively). Case 3 did not undergo hepatopulmonary separation or herniorrhaphy after exploratory operation and died of persistent pulmonary hypertension. (2) Literature review: Only 40 cases of CDH with HPF were retrieved from PubMed. Among the 43 cases including the above three cases, 27 (62.8%) had a right shift or no deviation of the mediastinum before surgery and nine (20.9%) had a left shift of the mediastinum, while the condition of seven patients (16.3%) were not described. There were 26 patients undergoing complete separation of liver and lung and 19 (73.1%) of them survived. Thirteen patients underwent partial separation of liver and lung and six of them survived. Four patients died without receiving separation.Conclusions:HPF should be considered in patients with RCDH, especially in cases with no left shift in the mediastinum in the imaging. Preoperative evaluation for surgery in such cases needs to be managed as if it were a major operation that may require hepatectomy or partial pneumonectomy.

Objective:To explore the complications and nutritional outcomes of Home Enteral Nutritional (HEN) in newborn surgical patients.Method:The medical records of neonates with HEN after surgery between 2017 and 2020 were retrospectively reviewed and complications of HEN and the nutritional status before and after HEN were analyzed.Results:A total of 66 neonates were included. The average gestational age at delivery and birth weight were (35.7 ± 3.0) weeks and (2426 ± 709) g, respectively. Diagnoses were mainly congenital esophageal atresia and intestinal diseases, such as intestinal atresia, intestinal torsion and necrotizing enterocolitis. The median age at HEN initiation was 92 (50, 112) days and HEN duration was 64 (41,95) days. HEN was conducted with tube feeding, with 14 patients (21.2%) through gastrostomy, 52 (78.8%) through nasal feeding tube, 20 (30.3%) through intermittent bolus infusion and 46 (69.7%) through continuous infusion. As for the formulas, 19 patients (28.8%) were given whole protein formula, 33 (50%) extensively hydrolyzed formula and 14 (21.2%) free amino acid-based formula. During the follow-up, 10 patients (71.4%) in gastrostomy group experienced 18 cases of catheter-related complications, including accidental removal (6 patients, 42.8%), catheter displacement (4 patients, 18.6%) and excessive granulation tissue at the gastrostomy site (4 patients, 18.6%). In nasal tube feeding group, 14 patients (26.9%) experienced 21 cases of catheter-related complications, including accidental tube removal (19 cases in 12 patients, 23.1%) and tube breakage (2 patients, 3.8%). Both the weight for age Z score and the height for age Z score were improved after HEN.Conclusions:HEN can help to improve the nutrition status in postoperative neonates. Management of catheter-related complications is challenging and warrants team work to improve the outcome of HEN.

This article reported a case of neonatal CHARGE syndrome complicated by congenital esophageal atresia. A prenatal ultrasound examination at 30 weeks of gestation revealed polyhydramnios and a small magenblase of the fetus, then fetal MRI suggested congenital esophageal atresia. The infant was born with severe asphyxia at 37 +5 gestational weeks by cesarean section due to placental abruption with a birth weight of 2 310 g. Gastric tube could not be placed after resuscitation. Congenital esophageal atresia complicated by tracheoesophageal fistula was diagnosed by esophageal imaging. Bilateral choanal atresia was detected by electronic nasopharyngoscopy and MRI. Moreover, skull defect, suspected meningocele were also observed. CHARGE syndrome was confirmed by whole exome sequencing, revealing a frameshift deletion of c.2155delA (p.Thr719GlnfsTer9) in the CHD7 gene. The infant died after withdrawing treatment.

Objective:To study the risk factors of necrotizing enterocolitis (NEC) after surgery for intestinal atresia.Method:From August 2013 to June 2020, children with intestinal atresia receiving surgery in our hospital were retrospectively reviewed. The patients were assigned into NEC group and non-NEC group according to the occurrence of postoperative NEC. Demographic data and clinical characteristics were summarized and the risk factors for postoperative NEC were analyzed using Logistic regression analysis method.Result:A total of 96 infants were enrolled and NEC occurred in 13 patients (13.5%) after surgery for intestinal atresia. Compared with the non-NEC group, the NEC group were diagnosed of intestinal atresia [4.0(1.5,6.0)d vs. 1.4(0,2.0)d, P<0.001] and received surgery [4.8(2.0,7.0)d vs. 3.1(1.0,4.0)d, P=0.034] at later ages. The incidences of complex intestinal atresia [76.9%(10/13) vs. 44.6%(37/83), P=0.030] and blood transfusion [46.2%(6/13) vs. 13.3%(11/83), P=0.007] in the NEC group were higher than the non-NEC group. Logistic regression analysis showed that the age of initial diagnosis of intestinal atresia ( OR=3.346, 95% CI 1.493~7.500, P=0.003), complex intestinal atresia ( OR=9.052, 95% CI 1.119~73.209, P=0.039) and blood transfusion ( OR=6.835, 95% CI 1.399~33.380, P=0.018) were independent risk factors for postoperative NEC. Conclusion:Patients with delayed diagnosis of intestinal atresia, complex intestinal atresia and blood transfusion within 48 hours after surgery should be monitored for the occurrence of postoperative NEC.

Objective:To study the incidences of postoperative complications and nutritional status following different timings of stoma reversal in necrotizing enterocolitis (NEC).Method:From January 2017 to December 2019, NEC patients receiving enterostomy surgery and later stoma reversal in our hospital were retrospectively analyzed. They were assigned into three groups according to the timing of stoma reversal: early group (reversal within 8 weeks of stoma formation), middle group (reversal at 8~12 weeks from stoma formation) and late group (reversal after 12 weeks from stoma formation). Weight-for-age-Z-score (WAZ) was used to evaluate nutritional status.Result:A total of 56 infants were enrolled, including 6 cases in the early group, 11 cases in the middle group, and 39 cases in the late group. The gestational age and birth weight were (33.4±3.4) weeks and (1 894±640) g, respectively. The median age of stoma formation and the interval between stoma formation and reversal were 16.5 (8.0, 28.2) days and 94.0 (76.5, 126.5) days. No significant differences existed on gestational age, birth weight, age of stoma formation and complications of stoma reversal among the three groups ( P>0.05). The incidence of growth retardation (WAZ<-2) was 14.3% at stoma formation, and significantly increased to 62.5% at stoma reversal ( P<0.05). The WAZ at stoma reversal in early, middle and late groups were (-3.2±1.9), (-3.0±1.6) and (-2.3±1.5), without significant differences( P>0.05). The WAZ gradually increased to (-0.7±1.2), (-0.1±2.0) and (-0.1±0.8) at 42~48 weeks after reversal, respectively. Conclusion:The timing of stoma reversal may not influence the complications of reversal. Growth retardation are common in NEC infants with stoma formation and stoma reversal may improve the nutritional status of the infants. Early reversal of stoma is suggested to improve the nutritional status of patients with poor weight gain after stoma formation.

We report the clinical characteristics of congenital malignant rhabdoid tumor (MRT) of the neck in a fetus. Prenatal ultrasound and MRI at 33 +4 and 34 weeks gestation revealed a round solid mass on the right side of the fetus' neck. An initial differential diagnosis was between neuroblastoma and vascular malformation. Re-examination with ultrasound at 36 gestational weeks revealed an enlarged fetal neck mass, with concomitant multiple subcutaneous solid masses all over his body, right-side hydrothorax, and abnormal liver echo, which were highly suspicious of metastasis of a malignant tumor. The baby boy was delivered by cesarean section at 37 weeks of gestation with a normal Apgar score and slight shortness of breath. Physical examination showed scattered lesions in the neck, armpits, and limbs, etc. The condition of the infant deteriorated rapidly with the increasing number and volume of the masses after admission. The boy was confirmed as MRT (stage Ⅳ) by pathological biopsy on the left upper arm and died on postnatal day 10 after treatment was withdrawn.

Objective To study the effect of multi-disciplinary team (MDT) management on the outcome in neonates with omphalocele.Method A retrospective non-randomized controlled clinical study was conducted.Neonates who were diagnosed as omphalocele and admitted to the surgical neonatal intensive care unit of the Guangzhou Women and Children Medical Center from December 2010 to December 2017 were collected.Because MDT was established in December 2014,infants were assigned into non-MDT group and MDT group according to their dates of admission.The characteristics and outcomes between non-MDT group and MDT group were compared using x2,t-test or rank-sum test.Multivariate analysis was performed by Logistic regression.Result A total of 91 neonates were included in the study,50 were in non-MDT group and 41 were in MDT group.The mortality in MDT group (2.4％,1/41) was lower than that in non-MDT group (18.0％,9/50),the difference was statistically significant (P ＜ 0.05).The median time of mechanical ventilation of giant omphalocele in non-MDT group (18.3 hours) was longer than that in MDT group (41.7 hours),the difference was also statistically significant (P ＜ 0.05).After adjusting for the associated confounding risk factors,the risk of death in non-MDT group was 54 times higher than that in MDTgroup (OR=54.19,95％CI2.64 ～1 113.49,P＜0.05).Conclusion There was significant association between the MDT management and the decreased risk of death of omphalocele.

Objective To summarize the experience of perioperative management for repair of congenital diaphragmatic hernia (CDH) supported by extracorporeal membrane oxygenation (ECMO). Method Retrospective review was conducted for the clinical data of CDH patients who received surgical repair on ECMO from December 2016 to June 2018 in Guangzhou Women and Children's Medical Center. Result Four fetus with prenatal diagnosis of left-side CDH were transferred to our Center and received standardized perinatal management. Moderate-severe pulmonary hypoplasia was recognized after evaluation by fetal imaging. Four cases were initiated with veno-arterial ECMO at 3, 35, 41, 11 h of life, respectively. Repair of the diaphragmatic defect was performed within two weeks after cannulation of ECMO. Furthermore, activated clotting time goals were adjusted to 180～220 s, activated partial thromboplastin time were stabilized between 50～80 s, platelets count were maintained>100×109/L and hematocrit was kept>30％before the surgery. The surgeries of four patients were completed on the 0.9th, 0.5th, 3.6th, 5.1th day of life on ECMO, respectively. The defect was repaired by parachute patch. The operative time was 85～210 min. According to CDH Staging System defect size (A to D), there were two with defects at grade C and other two at grade D. Postoperative total volume of drainage was 215～1301 ml and ECMO duration was 3.0～39.3 d. Three of them survived during neonatal period, while one died. Conclusion Repair of CDH on ECMO is feasible and help to improve neonatal survival, especially for those with moderate-severe pulmonary hypoplasia.