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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.
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Objective:To study the risk factors of surgical therapy in neonates with necrotizing enterocolitis (NEC).Methods:From January 2016 to July 2020, neonates with a confirmed diagnosis of NEC (Bell's Stage Ⅱ and above) admitted to our hospital were retrospectively enrolled. They were assigned into surgical group and conservative group according to whether surgeries were performed. The conditions during perinatal period, clinical characteristics and laboratory examinations at the onset of NEC were compared between the two groups. Multivariate Logistic regression analysis was used to determine the risk factors of surgical therapy.Results:A total of 177 neonates with NEC were identified, including 62 cases (35.0%) in the surgical group and 115 cases (65.0%) in the conservative group. Multivariate Logistic regression analysis showed that male gender ( OR=3.178,95% CI 1.457~6.929, P=0.004), comorbidity with shock ( OR=3.434, 95% CI 1.112~10.607, P=0.032), mechanical ventilation>7 d before NEC onset ( OR=3.663, 95% CI 1.098~12.223, P=0.035) and lymphocytes <2.0×10 9/L ( OR=4.121, 95% CI 1.801~9.430, P=0.001) at the onset of NEC were independent risk factors for surgical therapy. Conclusions:Male gender, comorbidity with shock, mechanical ventilation >7 d before NEC and lymphocytopenia at the onset are independent risk factors for surgical therapy in neonates with NEC (Stage Ⅱ and above).
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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.
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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.
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Objective:To study the clinical characteristics of double aortic arch (DAA) combined with aortoesophageal fistula (AEF), and summarize the diagnosis and treatment experience.Methods:Retrospective analysis was performed on the diagnosis and treatment of a newborn with hemorrhagic shock caused by DAA combined with AEF in the Guangzhou Women and Children's Medical Center. The key searching words included "double aortic arch", "aortoesophageal fistula", "vascular ring", "newborn or neonate", and "infant, newborn". The relevant reports were retrieved from databases of CNKI, Wanfang, VIP, PubMed, Springer Link, Google Scholar, Web of Science, Embase, Cochrane Library and OVID, to summarize the clinical features, diagnosis and treatment experience of neonates with DAA and AEF. The retrieval deadline was December 31, 2020.Results:A full-term female newborn was hospitalized for dyspnea immediately after birth, and failed to evacuate from the ventilator for several times. The patient was fed with nasogastric tube and transferred to our hospital because of hemorrhagic shock occurring in 32 days after birth, and gastrointestinal bleeding occurred repeatedly with the maximum bleeding volume reaching 200 ml/time. DAA was diagnosed by cardiac ultrasound and CT, AEF hemorrhage was finally confirmed by gastroscopy, aortography and operation. DAA correction and esophagus repair were successfully performed, and the infant recovered well after the operation. At 9-month old, the infant grew and developed well. At present, no reports of DAA combined with AEF neonates have yet to be published in medical literatures in China. Seven English language literatures included 7 cases of AEF complicated with DAA in neonatal period, 5 cases survived and 2 cases died have so far been reported. All patients have a long history of gastric tube indwelling.Conclusions:The incidence of DAA combined with AEF is rare in the newborn with respiratory and swallowing difficulties as the first manifestation. The disease symptoms progressed rapidly, and life-threatening digestive tract hemorrhage may occur, which often requires surgical treatment. Prolonged gastric tube retention should be avoided in DAA children to prevent the occurrence of AEF.
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Objective:To study the characteristics and related factors of neonatal intestinal necrosis caused by midgut volvulus.Methods:We retrospectively analyzed the clinical data of neonates with midgut volvulus who were admitted to Guangzhou Women and Children's Medical Center, from January 2009 to December 2019 and confirmed by surgery. The cases with intestinal necrosis belong to the intestinal necrosis group, and those without intestinal necrosis, the non-intestinal necrosis group which was randomly sampled at a ratio of about 4∶1 to the number of cases in the intestinal necrosis group. The two groups were compared in terms of personal history, age of onset, initial symptoms, vital signs within 2 h after admission, time from symptom onset to operation, clinical outcome, laboratory indicators within 2 h after admission, etc. Multivariate Logistic regression analysis was used to screen the related factors of intestinal necrosis in midgut volvulus. The effective warning indexes are screened by receiver operating characteristic (ROC) curve.Results:(1) Among 231 cases of midgut volvulus, 21 cases (9.1%, 21/231) had intestinal necrosis at the time of operation, 87 cases were included in the non-intestinal necrosis group. (2) The levels of heart rate within 2 h after admission, mean arterial pressure, WBC, C reactive protein (CRP), blood glucose and potassium in intestinal necrosis group were significantly higher than those in non-intestinal necrosis group ( P<0.05). Admission days of age, hemoglobin, serum albumin, serum sodium, pH and BE levels were significantly lower than those in the group without intestinal necrosis ( P<0.05). (3) In the multivariate analysis, increased heart rate, mean arterial pressure, serum CRP, and decreased serum sodium, serum albumin, and pH levels were predictors related to intestinal necrosis in patients with midgut volvulus. (4) The area under the ROC curve (AUC) of CRP was 0.883, the cutoff value was 9.88 mg/L, the sensitivity was 76.2%, and the specificity was 94.3%. The ROC curve of serum albumin was 0.792, the cut-off value was 36.65 g/L, the sensitivity was 70.1%, and the specificity was 94.3%. Conclusions:Heart rate, mean arterial pressure, increased CRP, decreased serum sodium, serum albumin and pH are helpful to predict whether intestinal necrosis occurs in midgut volvulus, and CRP > 9.88 mg/L and serum albumin < 36.65 g/L are likely warning indicators.
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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.
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Objective:To investigate the prediction value of observed to expected lung area to head circumference ratio (o/e LHR), measured at different gestational age with various methods, on indication for extracorporeal membrane oxygenation (ECMO) in fetuses with isolated left-sided congenital diaphragmatic hernia (CDH).Methods:Clinical data of 40 neonates who were diagnosed with left-sided CDH and treated in Guangzhou Women and Children's Medical Center were retrospectively collected from January 2017 to May 2021. The o/e LHRs were prenatally calculated using maximum diameter and tracing method at 22-24 and 31-33 weeks of gestation. According to whether the neonates had indications for ECMO after birth or not, they were divided into ECMO ( n=12) or non-ECMO group ( n=28). Differences in the o/e LHR and general situations between the two groups were analyzed using C hi-square test, independent sample t-test, and non-parametric Mann-Whitney U test. Binary logistic regression was used to analyze the influencing factors for ECMO requirement and receiver operating characteristic (ROC) curve was used to evaluate the value of o/e LHR in predicting the indication for ECMO. Results:Both maximum diameter and tracing method suggested that the o/e LHR at 31-33 gestational weeks was lower than that at 22-24 gestational weeks [maximun diameter method: 40.4 (32.9-51.5) vs 45.1 (36.3-53.4), Z=－2.48, P=0.013; tracing method: 38.6 (33.2-47.6) vs 44.1 (35.9-51.7), Z=－3.29, P=0.001]. There was no statistical difference in o/e LHR detected at the same gestational weeks between the two methods (both P>0.05). Binary logistic regression showed that o/e LHR measured at 31-33 gestational weeks using maximum diameter method was an independent protective factor for ECMO requirement ( OR=0.873, 95% CI: 0.790-0.965, P=0.008). ROC curve analysis showed that the area under the curve for evaluating the predictive value of o/e LHR for ECMO requirement was 0.830 with the sensitivity of 83.3% and the specificity of 71.4% when the cut-off value of o/e LHR at 31-33 gestational weeks was 38.195 measured by maximum diameter method. Conclusions:The o/e LHR measured at 31-33 weeks is lower than that at 22-24 weeks of gestation by both methods. The o/e LHR measured by maximum diameter method at 31-33 weeks of gestation may be useful for predicting the ECMO indication after birth but requiring comprehensive evaluation of clinical conditions due to its insufficient predicting power.
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We report the clinical features of a case of congenital extralobar pulmonary sequestration in the left upper lobe supplied by the pulmonary artery. Prenatal ultrasound examination at 24 weeks of gestation revealed a high echogenic and uniform density mass in the fetal left thoracic cavity with the congenital pulmonary airway malformation volume vatio (CVR) of 1.16, which was supplied by pulmonary arterial vessels. MRI examination at 27 weeks indicated that the left lung volume increased to about 48.52 ml, while the right lung volume was about 8.56 ml giving the total lung volume of 57.08 ml. The congenital pulmonary airway malformation in the left upper lobe was suspected to be congenital bronchial atresia (CBA) or congenital lobar overinflation (CLO). The baby boy was born through vaginal delivery assisted by forceps at 38 +1 weeks without neonatal asphyxia. Postnatal CT and MRI were both indicated suspicious bronchial atresia in the left upper lobe. Bronchofibroscopy on postnatal day 2 excluded CBA or CLO and extralobar pulmonary sequestration was considered. Thoracoscopic surgery was performed due to continuous shortness of breath after birth, despite two-week conservative treatment including oxygenation, invasive and non-invasive mechanical ventilation,etc, and congenital extralobar sequestration was diagnosed. Blood supply from the left pulmonary artery was observed at the base of abnormal lung tissue. Resection of the pathogenic tissue of the left lung was performed thoracoscopically. The boy recovered and was discharged after the operation. Pulmonary sequestration was confirmed by histopathology.
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Objective:To investigate the clinical features and outcomes of giant fetal hepatic hemangioma (GFHH).Methods:In this retrospective study, patients with GFHH from Guangzhou Women and Children's Medical Centre who received conservative management or medical intervention with a minimum of one year's follow-up after birth from August 2012 until March 2020 were enrolled. Prenatal and postnatal data were collected to analyze the pathological (size, location, and lesion type) and clinical manifestations, treatment, and prognosis of GFHH using independent sample t-test or Chi-square (or Fisher's exact) test. Results:(1) A total of 22 subjects were enrolled, including one preterm infant (4.5%), with an average gestational age of (38.9±1.5) weeks at birth, ranging from 33 +4 to 40 +3 weeks. Among them, 17 were male (77.3%), and five were female (22.7%). The gestational age at the first diagnosis was (35.0±2.8) weeks, ranging from 30 to 40 +1 weeks. Fifteen babies (68.2%) were born vaginally and seven (31.8%) by cesarean section due to fetal distress. Focal type accounted for 21 of the 22 (95.5%) patients, and multifocal type in one patient (4.5%). All the patients received B-ultrasonography on an average of 2.5 times (2-4 times) prenatally, which showed that the diameter of hepatic hemangioma increased with gestational age and reached its maximum at birth [56 mm (42-99 mm)]. Nine fetuses (40.9%) were diagnosed with GFHH with prenatal ultrasonography, and 12 with MRI. Among them, six fetuses were diagnosed with both ultrasonography and MRI. The other cases showed space-occupying lesions. (2) Ten babies received conservative management (conservative group), and 12 received drug therapy (treatment group). There was no significant difference between the two groups in gestational age at diagnosis or birth, birth weight, the maximum diameter of the tumor before birth, and location and classification of the lesions (all P>0.05). The incidence of thrombocytopenia in the treatment group was significantly lower than that in the conservative group (7/12 vs 0/10, Fisher's exact test, P=0.014). No abnormality of heart function or pulmonary hypertension was found in the conservative group, while two patients were complicated with pulmonary hypertension in the treatment group. The outcomes between the two groups were similar ( P>0.05). (3) During a median followed-up of 3.3 years (1.2-7.0 years), all patients survived, and none of the children in the conservative group received drug therapy. One of the ten patients in the conservative group had a complete tumor involution at two years old without recurrence, and the other nine had the tumor regressed in size. Besides oral propranolol, three of 12 cases in the treatment group also had dexamethasone at the early stage, two had rapamycin, and two received invasive therapy due to progressive enlargement of lesions at the early stage, with the lesions completely involuted in two cases at 1.7 and 5.5 years of age and regressed in the other ten cases. Conclusions:The diameter of GFHH increases with gestational age. Conservative management conservative is recommended for asymptomatic GFHH cases. For those affected with dyspnea and abdominal distention resulting in repeated vomiting, drugs therapy can be considered regardless of the test results, and invasive therapy is an option when drugs are ineffective. Some fetuses may have unsignificant imaging manifestations. Most children prenatally detected with giant hepatic hemangiomas can survive, and the tumor begins to regress after birth with a good long-term prognosis.
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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.
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Objective:To analyze the characteristics of prenatal MRI and postnatal CT images of fetal congenital mesoblastic nephroma (CMN).Methods:We retrospectively recruited three women with a singleton pregnancy suspected of having fetal CMN by prenatal imaging examination and confirmed by postpartum surgical resection and pathological examination at Guangzhou Women and Children Medical Center from August 2017 to April 2019. The prenatal MRI, postnatal CT, and pathological examination data were analyzed to summarize the image features of fetal CMN using descriptive statistical analysis.Results:(1) The total number of deliveries in the setting during the same period was 57 716, and the incidence of CMN was 0.005% (3/57 716). All the cases were male and born through cesarean section due to polyhydramnios for case 1 and 2 and sudden fetal distress, fetal pericardial effusion with enlarged mass in the third trimester for case 3. (2) Prenatal MRI examination found that the lesions in case 1 and 3 were located in the left kidney, and in the right kidney in case 2. The tumors in the three cases were all around with clear borders and hyperintense signal on T2-weighed imaging (T2WI). Iso-signal on T1WI was noted for case 1 and 2, a slightly hypointense signal on T1-weighed imaging (T1WI), and a hyperintense signal on diffused weighed imaging were noted for case 3. (3) Postnatal CT plain scan showed uniform density tumor in cases 1 and 2 with "packing sign" in CT enhancement. Non-uniform density, internal, larger and high-density bleeding tumor was found in case 3, and the CT enhancement manifested as nodular, non-uniform, and intratumoral bleeding. (4) Postoperative pathological examination revealed that cases 1 and 2 were classic types, and case 3 was cellular type.Conclusions:In this study, the prenatal MRI characteristic of CMN is a uniform signal, iso-signal on T1WI for classic type and non-uniform signal, and reduced signal on T1WI for cellular type. In classic type cases, postnatal CT enhancement showed a "packing sign." In contrast, in cellular type cases, the CT plain scan is manifested as non-uniform, intratumoral bleeding, and the enhancement pattern showed nodular and non-uniform enhancement. However, the results of this study is limited due to the small sample size and studies with larger sample size is needed in the future.
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Objective:To evaluate the clinical effect of applying the programmed management procedure in the prenatal diagnosis of pyriform sinus fistula(PSF).Methods:This study retrospectively enrolled eight fetuses with PSF who were managed according to the programmed management procedure for prenatal diagnosis of PSF, which was established in January 2016, in Guangzhou Women's and Children's Medical Center from January 2016 to October 2020. The procedure consisted of the detection of fetal neck cysts by prenatal ultrasound followed by further confirmation by MRI, evaluation of the degree of airway compression, indwelling gastric tube after birth, no oral feeding, complement of CT/MRI, and surgical treatment within a limited time after necessary preoperative examination. The prenatal diagnosis, postnatal treatment, and follow-up were summarized using descriptive analysis.Results:(1) Prenatal: The gestational age at the first detection of cervical cysts by prenatal ultrasound was (27.1±4.1) weeks and all the cysts were located on the left side. Prenatal MRI indicated that the largest cysts was (32.0±12.2) mm in diameter, and the tracheal transit index was (10.9±2.8) mm. (2) After birth: Among the eight children, five were males and three were females, with the gestational age of (38.0±0.9) weeks and birth weight of (3 020±459) g. One case was intubated during labor due to a intrauterine tracheal transposition index of 17.4 mm. All infants were not allowed for oral feeding. The median age at CT/MRI examination was 2.5 d (1-8 d), which revealed that the maximum diameter of the cysts was (40.6±6.9) mm and visible air bubbles in all cysts. The infection index before operation was not high and the age at operation was (8.6±2.3) d. All cysts were completely removed and the PSFs were ligated at a higher position, with the average operative duration of (95.0±19.6) min, and the postoperative duration of mechanical ventilation and hospitalization of 5 h (3-71 h) and (8.8±1.0) d, respectively. No complications such as hoarseness were reported. During the follow-up of 4 to 58 months through outpatient clinic and telephone, no recurrence were observed.Conclusions:The programmed management procedure can provide guidance for postnatal treatment of patients with a prenatal diagnosis of PSF, and help to achieve a successful treatment result.
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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.
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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.
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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.
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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.
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Objective To compare the prognosis of different surgical procedures and to find the relatively safe and effective treatment for severe jejunoileal atresia(sJA).Method From January 2007 to June 2018,children with sJA receiving different surgical procedures in our hospital were retrospectively reviewed.Their clinical data were analyzed,including the survival rate,complication rate,unplanned re-operation rate and postoperative nutritional status.Result A total of 130 patients were enrolled in this study.According to the different types of surgical procedures,the patients were assigned into primary anastomosis group (58 cases,44.6％),Mikulicz double barrel ileostomy group (17 cases,13.1％) and Bishop-Koop anastomosis group (55 cases,42.3％).The overall mortality rate was 6.2％ (8/130).No significant differences existed in mortality rates among the three groups (P＞0.05).The incidences of gastrointestinal complications in primary anastomosis group (70.6％,12/17) and Mikulicz group (70.6％,12/17) were both higher Bishop-Koop group (34.5％,19/55),the differences were statistically significant (P＜0.05).The unplanned re-operation rates were 34.5％ (20/58) in the primary anastomosis group and 17.6％ (3/17) in the Mikulicz group,both higher than the Bishop-Koop group (3.6％,2/55),the differences were also statistically significant (P＜0.05).Multivariate analysis showed that the risk of complications in the primary anastomosis group (OR=3.434,95％CI 1.392～8.471) and Mikulicz group (OR=5.933,95％CI 1.467～23.991) were higher than the Bishop-Koop group.The risk of unplanned re-operation in the primary anastomosis group was 12.422 times as the Bishop-Koop group (95％CI 2.535～60.877).No significant differences existed between the Mikulicz group and the Bishop-Koop group in the risk of unplanned re-operation (P＞0.05).The weight for age (Z-score) in the Bishop-Koop group (-1.4,95％CI-2.0～-0.8) at the stoma closure time was better than the Mikulicz group (-3.2,95％CI-4.4～-2.0),the difference was statistically significant (P＜0.01).Conclusion Bishop-Koop anastomosis has lower complication rate and lower unplanned re-operation rate in the treatment of sJA.The nutritional status of children who received Bishop-Koop anastomosis is better than Mikulicz double barrel ileostomy at the stoma closure time.Bishop-Koop anastomosis is relatively safe and effective for sJA patients.
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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.
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Objective To summarize the experience of cannulation for extracorporeal membrane oxygenation (ECMO) in infants less than 5 kg. Methods Eleven infants with ECMO support who weighed less than 5 kg were admitted to critical care medicine of Guangzhou Women and Children's Medical Center from June 2016 to June 2018 were enrolled. Retrospective analysis of support type, configuration, site of cannula and complication of ECMO was performed. Results The 11 infants consisted of 9 males and 2 females. The weight on ECMO of 1.96-4.60 kg, with an average of (3.14±0.65) kg; age 0.1-30.0 days, with a median of 5.6 (1.5, 8.3) days. Four cases were given ECMO because of congenital diaphragmatic hernia with severe pulmonary hypertension and other 7 cases were cannulated due to complication of congenital cardiac surgery. All infants were received veno-arterial (V-A) ECMO. In 4 cases, the cannulas were placed in the right internal jugular vein for drainage (8-10 French catheter with 6.0-7.5 cm depth) and the right carotid artery for infusion (6-8 French catheter with 2.5-3.5 cm depth); the average time of cannulation in right carotid and jugular vessels was (73±20) minutes (range 55-100 minutes). In other 7 cases, the cannulas were inserted into the right atrium (12-14 French catheter with 2.8-3.0 cm depth) for draining blood and returning it to the ascending aorta (6-8 French catheter with 1.0-2.0 cm depth); the average time of cannulation in central vessels was (64±31) minutes (range 35-110 minutes) with exclusion of 2 cases intraoperative cannulation. There were three infants with complications. One episode was shown in vascular rupture, one in catheter site hemorrhage and one in cannula malposition with later repositioning. There was no case of insertion site infection, cannula-related bloodstream infection and accident detached cannula. Conclusion Cannulation for ECMO can be performed in infants less than 5 kg with a high rate of success and a low rate of complication owing to appropriate catheter and skillful cannulation.