We Reduced the Incidence of Postoperative Complications in Neonatal Ostomy Patients by Using Simple Devices.

Background: Complications frequently occur after neonatal enterostomy. Enterostomy formation is a common outcome following an emergency neonatal laparotomy. This study investigated whether the incidence of complications after enterostomy could be decreased with a drainage device (composed of foreskin cerclage staple, a condom, and a 0-Mersilk braided nonabsorbable suture) fixed in the proximal ostomy bowel tube to improve proximal enterostomy in newborns. Methods: This study was a retrospective case note review of the incidence of emergency neonatal enterostomy incidence over a 3-year period (2/2016-2/2019) at the authors' center. A single surgeon conducted all surgeries. The incidence of intraoperative and postoperative complications was compared between modified and traditional surgery groups. Results: All 47 surgeries were successfully completed (32 boys and 15 girls; sex ratio: 2.13:1). The mean (±SD) birth weight, gestational period, and daily age were 2.64 ± 0.81 kg 35.62 ± 3.76 weeks and 3.49 ± 5.61 days, respectively. The patients were divided into modified surgery groups (20 cases) and traditional surgery groups (27 cases). The modified surgery group had significantly lower rates of total complications, unplanned reoperations, wound-related complications, and stoma-related complications than the traditional group (p <0.05). Conclusions: The preliminary observations suggested that the simple drainage device was a safe and effective operation device that reduced the risk of stoma-related complications.

New Device of Neonatal Ostomy: To Effectively Reduce the Postoperative Complications of Neonatal Ostomy.

Background need. Complications frequently occur after neonatal enterostomy. Enterostomy formation is a common outcome following emergency neonatal laparotomy. We introduce a new method for improving proximal enterostomy in newborns.Methodlogy and device description. We added a simple drainage device on the proximal enterostomy.The simple drainage device consists of several materials: a foreskin cerclage staple (Chong Qing BORN Biological Technology Co. Ltd., Sichuan, China), a condom,and 0- Mersilk Silk braided nonabsorbable suture (ETHICON 15 × 60 cm).Preliminary results. A total of 20 cases participated in the study. All surgeries were performed by a single surgeon. The cases only occurred one case of prolapse of the intestinal, one case of premature surgery due to excessive orifice flow, and one case of periostomy dermatitis, Other patients not experienced intraoperative or postoperative complications.Current status. Based on our preliminary observations, the simple drainage device is a safe and effective operation device that can reduce the risk of stoma-related complications.

Modified single-port versus multiport laparoscopic choledochal cysts excision and Roux-en-Y hepaticojejunostomy: a retrospective comparative cohort study.

Background: The feasibility, benefit, and safety of multiport laparoscopic choledochal cyst (CDC) excision and Roux-en-Y hepaticoenterostomy (MPCH) have been consistently confirmed. Single-port laparoscopic CDC excision and Roux-en-Y hepaticoenterostomy (SPCH) has advantages of less traumatic and more cosmetic beneficial, it has been reported in some case series, but it is technically challenging. We propose a modified technique to reduce technical difficulty in performing SPCH. The safety and feasibility of modified SPCH were compared with those of conventional multiport laparoscopic CDC excision. Methods: A total of 43 consecutive patients who diagnosed with CDC by preoperative magnetic resonance cholangiopancreatography (MRCP) and underwent SPCH (n=24) and MPCH (n=19) for choledochal cyst (CDC) by a single surgeon between January 1, 2018, and January 1, 2021, were enrolled. The baseline clinical characteristics, efficacy and safety outcomes of short-term were compared. Results: The baseline clinical characteristics of the MPCH and SPCH groups are comparable. Average postoperative length of hospital stay was shorter in the SPCH group than in the MPCH group, but the difference was not statistically significant (7.00 vs. 7.58 days; P>0.99). The operation time (281.75 vs. 277.3 min; P=0.58) and the amount of blood loss (9.33 vs. 16.68 mL; P=0.57) were similar in both groups. A significantly greater number of drainage tubes were placed in the MPCH group compared to the SPCH group (11 vs. 5; P=0.01). One patient suffered from hepaticoenterostomy anastomosis stricture in the SPCH group. Conclusions: The short-term outcome of modified SPCH is comparable with MPCH according to our study. It is easily adaptable treatment of CDC.

Long-term prophylactic intravenous antibiotics after Kasai portoenterostomy for biliary atresia do not reduce the risks of post-operative cholangitis, a retrospective study.

BACKGROUND: Postoperative cholangitis (PC) is the most common and serious complication of biliary atresia (BA) patients post-Kasai portoenterostomy (KPE). The duration of prophylactic intravenous antibiotics (IVA) after KPE varies with no clear consensus. We conducted a retrospective cohort study to explore the effects of IVA duration on preventing post-operative cholangitis and analyze the risk factors for cholangitis and short-term prognosis. METHODS: All patients diagnosed with BA and received KPE in Guangzhou Women and Children's Hospital in 2018, were included in this study. The patients received prophylactic IVA after KPE. Firstly, the patients were divided into two groups based on the presence or absence of PC (PC and NPC group). The correlation between PC and the IVA duration was analyzed, followed by a comparison of short-term prognosis, outcome, and other risk factors between the groups. Next, the patients were divided based on the median IVA duration of 11 days (long IVA and short IVA group), followed by a comparison of the incidence of PC, short-term prognosis, outcome, and other risk factors between the two groups. RESULTS: Totally 89 patients were included in this study. Amount them, eleven patients who were lost during follow-up, were excluded from the study. The prophylactic IVA duration of the PC (n=52) and NPC (n=25) groups was 12.6±8.5 and 13.0±4.5 days, respectively (P=0.79). Further, the jaundice clearance rate of the two groups was similar (PC: 31/52, NPC: 13/25, P=0.53). There was no difference in the incidence and frequency of cholangitis between the short (n=42) and long (n=35) IVA groups (27/42, 25/35, P=0.51), and the duration of IVA had no effect on jaundice clearance (24/42, 20/35, P=1.00). The short IVA group had a significantly shorter hospital stay than the long IVA group (16.2±5.1, 25.3±8.3, P=8.95×10-8). Patients undergoing KPE at an older age were at a higher risk of cholangitis (NPC: 60.6±19.7, PC: 72.3±17.8, P=0.01). CONCLUSIONS: A long duration of IVA after KPE for BA may not be necessary. Early diagnosed patients had timely surgery had a lower incidence of PC. Our findings may help in promoting the scientific use of antibiotics and reducing the LHS.

Comparison of outcomes following three surgical techniques for patients with severe jejunoileal atresia.

BACKGROUND: Severe jejunoileal atresia is associated with prolonged parenteral nutrition, higher mortality and secondary surgery. However, the ideal surgical management of this condition remains controversial. This study aimed to compare the outcomes of patients with severe jejunoileal atresia treated by three different procedures. METHODS: From January 2007 to December 2016, 105 neonates with severe jejunoileal atresia were retrospectively reviewed. Of these, 42 patients (40.0%) underwent the Bishop-Koop procedure (BK group), 49 (46.7%) underwent primary anastomosis (PA group) and 14 (13.3%) underwent Mikulicz double-barreled ileostomy (DB group). Demographics, treatment and outcomes including mortality, morbidity and nutrition status were reviewed and were compared among the three groups. RESULTS: The total mortality rate was 6.7%, showing no statistical difference among the three groups (P = 0.164). The BK group had the lowest post-operative complication rate (33.3% vs 65.3% for the PA group and 71.4% for the DB group, P = 0.003) and re-operation rate (4.8% vs 38.8% for the PA group and 14.3% for the DB group, P < 0.001). Compared with the BK group, the PA group showed a positive correlation with the complication rate and re-operation rate, with an odds ratio of 4.15 [95% confidence interval (CI): 1.57, 10.96] and 12.78 (95% CI: 2.58, 63.29), respectively. The DB group showed a positive correlation with the complication rate when compared with the BK group, with an odds ratio of 7.73 (95% CI: 1.67, 35.72). The weight-for-age Z-score at stoma closure was -1.22 (95% CI: -1.91, -0.54) in the BK group and -2.84 (95% CI: -4.28, -1.40) in the DB group (P = 0.039). CONCLUSIONS: The Bishop-Koop procedure for severe jejunoileal atresia had a low complication rate and re-operation rate, and the nutrition status at stoma closure was superior to double-barreled enterostomy. The Bishop-Koop procedure seems to be an appropriate choice for severe jejunoileal atresia.

Autoimmune liver disease-related autoantibodies in patients with biliary atresia.

AIM: To investigate the prevalence and clinical significance of autoimmune liver disease (ALD)-related autoantibodies in patients with biliary atresia (BA). METHODS: Sera of 124 BA patients and 140 age-matched non-BA controls were assayed for detection of the following autoantibodies: ALD profile and specific anti-nuclear antibodies (ANAs), by line-blot assay; ANA and anti-neutrophil cytoplasmic antibody (ANCA), by indirect immunofluorescence assay; specific ANCAs and anti-M2-3E, by enzyme linked immunosorbent assay. Associations of these autoantibodies with the clinical features of BA (i.e., cytomegalovirus infection, degree of liver fibrosis, and short-term prognosis of Kasai procedure) were evaluated by Spearman's correlation coefficient. RESULTS: The overall positive rate of serum autoantibodies in preoperative BA patients was 56.5%. ALD profile assay showed that the positive reaction to primary biliary cholangitis-related autoantibodies in BA patients was higher than that to autoimmune hepatitis-related autoantibodies. Among these autoantibodies, anti-BPO was detected more frequently in the BA patients than in the controls (14.8% vs 2.2%, P < 0.05). Accordingly, 32 (25.8%) of the 124 BA patients also showed a high positive reaction for anti-M2-3E. By comparison, the controls had a remarkably lower frequency of anti-M2-3E (P < 0.05), with 6/92 (8.6%) of patients with other liver diseases and 2/48 (4.2%) of healthy controls. The prevalence of ANA in BA patients was 11.3%, which was higher than that in disease controls (3.3%, P < 0.05), but the reactivity to specific ANAs was only 8.2%. The prevalence of ANCAs (ANCA or specific ANCAs) in BA patients was also remarkably higher than that in the healthy controls (37.9% vs 6.3%, P < 0.05), but showed no difference from that in patients with other cholestasis. ANCA positivity was closely associated with the occurrence of postoperative cholangitis (r = 0.61, P < 0.05), whereas none of the autoantibodies showed a correlation to cytomegalovirus infection or the stages of liver fibrosis. CONCLUSION: High prevalence of autoantibodies in the BA developmental process strongly reveals the autoimmune-mediated pathogenesis. Serological ANCA positivity may be a useful predictive biomarker of postoperative cholangitis.

Differentiation between cystic biliary atresia and choledochal cyst: A retrospective analysis.

AIM: Cystic biliary atresia (CBA) can be easily misdiagnosed as choledochal cyst (CC). Some patients have already progressed to severe liver fibrosis and missed the optimal surgical time, when the differential diagnosis is made. We aim to determine the differentiation between CBA and CC, and to validate the value of aspartate aminotransferase-to-platelet ratio index (APRI) in the assessment of liver fibrosis and prediction of post-operative outcome for infants with biliary cystic malformations (BCMs). METHODS: Clinical data of children (categorised into CBA and CC groups) with BCMs were analysed retrospectively. Biochemical indicators together with B-ultrasound examinations and the degree of liver fibrosis were analysed, and those with statistical difference between the two groups were selected for diagnostic receiver operating characteristic curve analysis. RESULTS: The parameter that showed the highest accuracy with a significant diagnostic performance for differentiating CBA from CC was cyst size. Liver assessment at operation was categorised into mild fibrosis and moderate-to-severe fibrosis. The APRI values were much lower in the mild fibrosis groups than in the moderate-to-severe fibrosis group (0.4 ± 0.2 vs. 1.4 ± 0.8, P < 0.001). A cut-off value of 0.96 (area under the curve 0.92, P < 0.001) showed a sensitivity of 81.3% and a specificity of 100% for moderate-to-severe fibrosis. Lower APRI value was correlated with short-term post-operative bilirubin clearance. CONCLUSION: There is still certain difficulty in the early identification of CBA and CC clinically. Liver fibrosis could occur as early as infantile period in both CBA and CC. In infants with BCMs, APRI can be used as a non-invasive method to detect liver fibrosis in early stages.

Prenatal diagnosis and early postnatal management of pyriform sinus cyst: experience at a single medical center in mainland China.

OBJECTIVE: The aim of this study was to determine whether prenatal diagnosis of pyriform sinus cyst can improve the prognosis of this disorder. METHODS: A retrospective review was performed in 15 neonates with a pyriform sinus cyst seen at a single center between 2010 and 2014. Among the 15 cases, the diagnosis was made prenatally in eight cases (PreD), while the diagnosis was made postnatally in seven cases (PostD). Neonatal outcome was compared in the two subgroups. RESULTS: The mean gestational age at diagnosis of PreD was 27 ± 6.8 weeks, while the mean age at admission of PostD was 10.1 ± 8.8 days. Cervical mass, fever, respiratory distress, and hoarseness were common symptoms. The mean duration of postoperative mechanical ventilation was 11.5 ± 13.9 and 100.71 ± 80.0 h, respectively, in PreD and PostD (p < 0.01). The average postoperative length of stay and the length of hospital stay were 11.3 ± 3.34 and 19.6 ± 4.41 days in PreD, and 15.14 ± 8.28 and 24.14 ± 8.51 days in PostD, respectively. CONCLUSION: Prenatal diagnosis and timely postnatal sequential intervention were associated with less complications and shortened duration of postoperative mechanical ventilation. © 2016 John Wiley & Sons, Ltd.

Role of CD56-expressing immature biliary epithelial cells in biliary atresia.

AIM: To analyze the clinical and pathological parameters and expression of the neural cell adhesion molecule (CD56) in patients with biliary atresia (BA). METHODS: Established clinical laboratory markers of hepatic function, including enzyme activity, protein synthesis, and bilirubin metabolism, were evaluated in patients with BA and compared with those in patients with choledochal cysts and neonatal hepatitis. Pathological changes in tissue morphology and fibrosis were examined by histological and tissue collagen staining. Immunohistochemical staining for the biliary epithelial cell markers CD56 and CK19 together with the Notch signaling related molecules Notch1 and Notch2 was performed in the context of alterations in the structure of intrahepatic biliary ducts. RESULTS: Differences in some clinical laboratory parameters among the three diseases examined were observed, but they did not correlate with the pathological classification of fibrosis in BA. Immunohistochemical staining showed the presence of CD56-positive immature bile ducts in most patients (74.5%) with BA but not in patients with choledochal cysts or neonatal hepatitis. The number of CD56-expressing cells correlated with disease severity, with more positive cells present in the later stages of liver damage (81.8% vs 18.2%). Furthermore, bile plugs were mainly found in CD56-positive immature biliary ducts. Notch signaling was a key regulatory pathway in biliary duct formation and played a role in tissue fibrosis. Notch1 was co-expressed in CD56-positive cells, whereas Notch2 was found exclusively in blood vessels in the portal area of patients with BA. CONCLUSION: The maturation of biliary epithelial cells and the expression of Notch may play a role in the pathogenesis of BA.

Intussusception patients older than 1 year tend to have early recurrence after pneumatic enema reduction.

PURPOSE: To identify age risk factors of early recurrent intussusception after pneumatic enema reduction. Management opinions are proposed. METHODS: Two thousand two hundred and ninety-five intussusception patients' medical records from January 2009 to December 2011 were retrospectively reviewed and analyzed. RESULTS: Of the 2295 patients, the intussusception of 1917 of them was initially reduced by pneumatic enema, with 127 cases recurring within 72 h. The early recurrence rate is 6.62%. The early recurrence rate of patients younger than 1 year old is 2.1% (22/1032), while the rate for those older than 1 year is 11.9% (105/885). The difference is significant (P = 0.0001). There were no significant differences between age groups older than 1 year. One hundred and seventeen cases of recurrence happened within 48 h, which accounted for 92.1% of all early recurrence. Recurrence patients were treated again with pneumatic enema, with a successful reduction in 93.7%. They were followed up for 2-4 years; the long-term recurrent rate was 11.8% (14/119). No patient had poor prognosis because of delayed treatment. CONCLUSION: Intussusception patients older than 1 year tend to have greater early recurrence rate after pneumatic enema reduction; 92.1% of the early recurrent cases happened in 48 h. There is no need to hospitalize patients after pneumatic enema reduction. A repeat pneumatic enema is a good choice before surgical approach.

Validation of aspartate aminotransferase to platelet ratio for diagnosis of liver fibrosis and prediction of postoperative prognosis in infants with biliary atresia.

AIM: To validate the value of aspartate aminotransferase to platelet ratio index (APRI) in assessment of liver fibrosis and prediction of postoperative prognosis of biliary atresia (BA) infants from Mainland China. METHODS: Medical records of 153 BA infants who were hospitalized from January 2010 to June 2013 were reviewed. The efficacy of APRI for diagnosis of liver fibrosis was assessed using the receiver operator characteristic (ROC) curve compared to the pathological Metavir fibrosis score of the liver wedge specimens of 91 BA infants. The prognostic value of preoperative APRI for jaundice persistence, liver injury, and occurrence of cholangitis within 6 mo after KP was studied based on the follow-up data of 48 BA infants. RESULTS: APRI was significantly correlated with Metavir scores (rs = 0.433; P < 0.05). The mean APRI value was 0.76 in no/mild fibrosis group (Metavir score F0-F1), 1.29 in significant fibrosis group (F2-F3), and 2.51 in cirrhosis group (F4) (P < 0.001). The area under the ROC curve (AUC) of APRI for diagnosing significant fibrosis and cirrhosis was 0.75 (P < 0.001) and 0.81 (P = 0.001), respectively. The APRI cut-off of 0.95 was 60.6% sensitive and 76.0% specific for significant fibrosis diagnosis, and a threshold of 1.66 was 70.6% sensitive and 82.7% specific for cirrhosis. The preoperative APRI in infants who maintained jaundice around 6 mo after KP was higher than that in those who did not (1.86 ± 2.13 vs 0.87 ± 0.48, P < 0.05). The AUC of APRI for prediction of postoperative jaundice occurrence was 0.67. A cut-off value of 0.60 showed a sensitivity of 66.7% and a specificity of 83.3% for the prediction of jaundice persistence. Preoperative APRI had no significant association with later liver injury or occurrence of cholangitis. CONCLUSION: Our study demonstrated that APRI could diagnose significant liver fibrosis, especially cirrhosis in BA infants, and the elevated preoperative APRI predicts jaundice persistence after KP.

[Treatment strategy and prognosis analysis in children with type I esophageal atresia].

OBJECTIVE: To analyze the postoperative short-term and long-term outcomes in the management of type I esophageal atresia, and to explore the ideal operative strategy. METHODS: Clinical data of 22 patients with type I esophageal atresia treated from January 2005 to September 2012 were retrospectively reviewed. Of 22 patients, 6 patients gave up the treatment. Two underwent primary repair after birth. Of 14 patients undergoing cervical esophagostomy and gastrostomy, 8 patients received esophageal replacement. Postoperative short-term and long-term complications, nutritional state and neurodevelopment were studied on above 10 children with radical operations. RESULTS: Of 10 patients with radical operation, the short-term complications were hydrothorax in 1 case, anastomotic leakage in 4, dumping syndrome in 1, anastomotic stricture in 1. The long-term complications were esophageal stricture in 2 cases, and repeated respiratory infection in 3. These complications could be managed successfully. The postoperative follow-up duration ranged from 2 to 62 months. Two cases were lost during follow-up after 2 years. Weight-for-age was normal in 2 patients, mild malnutrition in 5 patients, and moderate malnutrition in 1 patients. Neurodevelopment is significantly delayed as compared to normal children. CONCLUSIONS: Operative strategy should be chosen according to the distance between proximal and distal esophagus in the treatment of type I esophageal atresia. The efficacy of radical operation is relative satisfactory in terms of short-term and long-term complications and the quality of life.

[Wolman disease with novel mutation of LIPA gene in a Chinese infant].

OBJECTIVE: To explore the clinical characteristics of Wolman disease and diagnostic methods using enzymatic and molecular analysis. METHOD: Lysosomal acid lipase activity was measured using 4-methylumbelliferyl oleate in the leukocytes of an infant suspected of Wolman disease and LIPA gene mutational analysis was performed by PCR and direct sequencing in the proband and his parents. After the diagnosis was confirmed, the clinical, biochemical, radiological and histopathological findings in this case of Wolman disease were retrospectively reviewed. RESULT: The sixteen-day-old boy was failing to thrive with progressive vomiting, abdominal distention and hepatosplenomegaly. Abdominal X-ray revealed adrenal calcifications which were confirmed on abdominal CT scan. Xanthomatosis were observed on enlarged liver, spleen and lymph nodes during abdominal surgery. Liver and lymph node biopsy showed foamy histiocytes. The lysosomal acid lipase activity in leukocytes was 3.5 nmol/(mg·h) [control 35.5 - 105.8 nmol/(mg·h)]. Serum chitotriosidase activity was 315.8 nmol/(ml·h) [control 0 - 53 nmol/(ml·h)]. The patient was homozygote for a novel insert mutation allele c.318 ins T, p. Phe106fsX4 in exon 4 on LIPA gene. His both parents were carriers of the mutation. CONCLUSION: The clinical features of Wolman disease include early onset of vomiting, abdominal distention, growth failure, hepatosplenomegaly and bilateral adrenal calcification after birth. A plain abdominal X-ray film should be taken to check for the typical pattern of adrenal calcification in suspected cases of Wolman disease. The enzymatic and molecular analyses of lysosomal acid lipase can confirm the diagnosis of Wolman disease.

[Long-term outcomes and nutrition evaluation in children with total colonic aganglionosis].

OBJECTIVE: To examine the long-term outcomes of total colonic aganglionosis (TCA) and to evaluate their nutritional status. METHODS: Eleven pediatric patients treated for TCA between January 1999 and December 2010 were included in the study and followed up. Physical measurements including height, weight and laboratory tests were assessed. Anorectal functions were evaluated with Kelly score and quality of life(QOL) using questionnaire. RESULTS: The length of follow-up ranged from 8 to 147 months. The children had satisfactory anorectal function (Kelly score, 5-6). One child had a Kelly score of 3. The children who were followed up less than 48 months had significant higher Kelly scores compared with those with more than 48 months follow-up(P<0.05). QOL was good in nine patients (QOL score, 9-10) and moderate (score, 7-8) in 2 patients. Weight-for-age was normal in 2 patients, mild malnutrition in 6 patients, and moderate malnutrition in 3 patients. Height-for-age was normal in 6 patients, mild malnutrition in 3 patients, and moderate malnutrition in 2 patients. The serum albumin was(49.0±2.7) g/L in children with well-educated parents, significantly higher than those with poorly-educated parents(44.3±1.9) g/L(P<0.05). CONCLUSIONS: Long-term outcomes of children with TCA are satisfactory with good anorectal function and quality of life. Low body weight is more common than low height. Children with well-educated parents have better nutrition status.