Objective To review the authors′ experience in the diagnosis and management of Hirschsprung′s disease (HD) in newborn during a 7 year′s period (1995～2002),and evaluate a new therapeutic regime. Methods Clinical data of all 155 newborn HD cases were analyzed. Protocols were compared before and after May 2000. Results Barium enemas, anorectic manometry and rectal biopsies confirmed the diagnosis of HD in 155 newborn cases. Two stage radical procedure was performed in 107 cases , and one stage in 48 cases. One hundred and forty-seven cases were cured. Postoperative pneumonia occurred in 25 cases, wound dishences in 8 cases with 8 deaths. There was substantial difference in the incidence of complications, hospital stay and mortality between the two periods. Conclusions (1) Gastrointestinal perforation in neonatal HD cases should be managed by rectal biopsies during the exploration. (2)Different surgical treatment should be adopted in HD cases less than one month of age.

OBJECTIVETo examine the effect of post-discharge nutrition management on the growth speed of neonates after bowel surgery.

METHODSNutrition feeding guidance was carried out in 133 post-discharge infants after surgery through nutrition clinic. The growth speed was detected every month and compared with the normal standards, then the time to accelerate growth speed was evaluated.

RESULTSThe growth speed of neonates in the first postoperative month was lower than the normal standards, especially in males(weight P=0.000; length P=0.041; circumference P=0.010). While during two to three months, male infants showed acceleration in length growth speed [(4.53±1.22) cm vs. (3.1±0.4) cm, P=0.013], and female infants showed acceleration in weight [(1.51±0.76) kg vs. (0.83±0.39) kg, P=0.028] and circumference growth speed [(2.50±0.93) cm vs. (1.2±0.7) cm, P=0.021].

CONCLUSIONSScientific post-discharge nutrition management helps neonates grow faster after bowl surgery. The two to three months after operation is the key period of growth speed acceleration.

Body Weight ; Digestive System Surgical Procedures ; Female ; Humans ; Infant ; Infant, Newborn ; Male ; Nutritional Status

OBJECTIVETo investigate a new bowel management program for children patients with fecal incontinence.

METHODSClinical data of 19 children with fecal incontinence undergoing bowel management program in our center between January 2012 and January 2013 were retrospectively analyzed. The main outcome measure was clinical efficacy of this program.

RESULTSFifteen out of 19 cases were genuine fecal incontinence and required continuous treatment by enema. The other 4 cases were false fecal incontinence. After treatment with this program, stool dirty and constipation were improved in genuine incontinence. Two cases of false continence could control defecation independently by oral administration of antispasmodic drug. Two cases of false continence were cured and did not need medical interference.

CONCLUSIONSBowel management program is an effective treatment for pediatric patients with fecal incontinence. The key of success is maintenance of perianal hygiene for 24 hours by continual adjustment of the elements and volumes of enemas.

Child ; Constipation ; Enema ; Fecal Incontinence ; therapy ; Humans ; Intestines ; physiopathology ; Retrospective Studies

OBJECTIVETo summarize the experience of minimally invasive treatment in 520 patients with intracranial aneurysms on a retrospective study.

METHODSThe measures used in the treatment of 520 patients were reviewed in terms of timing of surgery, induced-hypotensive anesthesia, brain protection combined with temporal occlusion of the feeding artery, external drainage of CSF, dynamic monitoring of intracranial pressure, blood flow velocity, serum osmolality and CT scanning, anti-vasospasm therapy as well as selected interventional endovascular embolization of aneurysms.

RESULTSOf the 520 patients, 485 were treated with either direct clipping or endovascular embolization and 35 patients were treated non-surgically. In 449 patients undergoing direct clipping and 36 undergoing endovascular embolization, intraoperative rupture of aneurysm occurred in 27 (6.0%) and 0%, respectively. Death occurred in 13 (2.6%), hemiplegia in 8 (1.6%), and vegetative state in 2 (0.4%). The operative mortality of direct clipping was 3.8% in 210 patients before 1990 and 1.8% in 275 patients after 1990 (36 patients undergoing endovascular embolization, the operative mortality was 0%).

CONCLUSIONThe outcome of patients with intacranial aneurysms can be markedly improved and the operative mortality can be lowered by minimally invasive treatment.

Adult ; Aneurysm, Ruptured ; mortality ; therapy ; Embolization, Therapeutic ; Female ; Follow-Up Studies ; Humans ; Intracranial Aneurysm ; mortality ; surgery ; Intraoperative Complications ; mortality ; Male ; Microsurgery ; Middle Aged ; Minimally Invasive Surgical Procedures ; Retrospective Studies ; Survival Rate ; Treatment Outcome

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.

Objective To study the nutritional status and risk factors in neonates receiving enterostomy.Method From January 2015 to July 2017,patients who had enterostomy during neonatal period and had the stoma closed in our hospital were retrospectively studied.Z score (weight-for-age) was used to evaluate their nutritional status.The patients were divided into two groups according to the nutritional status when the stoma closed:the malnutrition group and the normal nutrition group.The differences in gender,premature birth,low birth weight,primary disease,ostomy methods,length of proximal small intestine and high output diarrhea through stoma were compared between the two groups.Multivariate Logistic regression analysis was used to determine the risk factors of malnutrition at the closure of the stoma.Result A total of 75 infants with various primary diseases were included.Among them,23 patients were diagnosed with neonatal necrotizing enterocolitis,27 patients jejunoileal atresia,5 patients meconium ileus,15 patients meconium peritonitis and 5 patients Hirschsprung's disease.The median age of enterostomy was 3 (2,8) days,and the median hospital stay after enterostomy was 26 (20,40) days.The median age of stoma closure was 6.0 (5.0,8.5) months.The median Z score at discharge and stoma closure were -1.6 (-2.9,-0.9) and-1.5 (-2.6,-0.5) respectively.No statistically significant differences existed between the two groups (P ＞ 0.05).28 infants (37.3％) were malnutrition when the stoma was closed.The incidence of low birth weight and high output diarrhea through stoma in malnutrition group were significantly higher than the normal nutrition group (35.7％ vs.10.6％,32.1％ vs.10.6％,P ＜0.05).No significant differences in gender,premature birth rate,primary disease,ostomy method and length of proximal intestine between the two groups (P ＞ 0.05).Multivariate Logistic regression analysis indicated that low birth weight,high output diarrhea and jejunoileal atresia were risk factors for malnutrition.Conclusion Malnutrition is common in neonates after enterostomy,and the risk factors for malnutrition are low birth weight,high output diarrhea through stoma and the primary disease jejunoileal atresia.

Objective To evaluate the diagnostic value of MRI-measured fetal percent predicted lung volume (PPLV) for the prognosis of left congenital diaphragmatic hernia (CDH) in fetus. Methods Clinical data of 32 children who were admitted to Women and Children's Medical Center of Guangzhou from September 2012 to December 2017 for prenatally diagnosed left CDH were retrospectively analyzed. These children were divided into two groups, the survival group (n=24) and the death group (n=8), according to the postoperative outcomes at 30 days after CDH repair. Moreover, they were also divided into non-pulmonary hypertension (non-PH) group (n=20) and PH group (n=12), based on whether they suffered from PH or not. Clinical data such as gestational age, birth weight, Apgar score and PPLV values in different groups were compared with t- or Fisher's exact test. The receiver operating characteristic (ROC) curve of the MRI-measured fetal PPLV values of the 32 children was plotted. Results (1) Comparing with the death group, fetal PPLV was significantly higher [(39.5±2.5)% vs (20.4±2.1)%, t=4.27], the gestations on initial diagnosis of CDH was later [(31.6±4.2) vs (25.4±4.6) gestational weeks, t=3.40], Apgar score of the neonates at 5 min was higher (8.7±1.5 vs 5.7±3.8, t=3.26), and fewer cases of PH were reported in the survival group [16.7% (4/24) and 8/8], all P<0.01. The area under the ROC curve of PPLV values for mortality prediction was 0.930 (95%CI: 0.843-1.016, P<0.01). When the PPLV value was 28.55%, its sensitivity and specificity for death prediction in children with left CDH were 100% and 79%, respectively. (2) Comparing with the PH group, fetal PPLV was significantly higher [(41.7±2.6)% vs (23.0±2.0)%, t=4.98], the gestations on initial diagnosis of CDH was later [(32.3±3.4) vs (26.3±5.2) gestational weeks, t=3.81], neonatal Apgar score at 5 min was higher (8.6±1.4 vs 6.8±2.5, t=2.62) and death rate was lower [0(0/20) vs 8/12] in the non-PH group (all P<0.01). The area under the ROC curve of the PPLV values for predicting PH was 0.902 1 (95%CI : 0.800-1.004, P＜0.01). When the PPLV value was 33.67%, its sensitivity and specificity for PH prediction was 100% and 75%, respectively. Conclusions Prenatal MRI measurement of PPLV can be used to predict death or as a warning sign of PH in children with left CDH, which may provide evidence for prenatal evaluation and rational clinical decision-making.

Objective To investigate the prenatal imaging features of fetal congenital esophageal atresia and to further evaluate the value of MRI and ultrasound scan in the same condition.Methods This study recruited 12 singleton gravidas whose fetuses were initially suspected with congenital esophageal atresia by prenatal ultrasound scan and then confirmed by surgery and/or upper gastrointestinal angiography after birth at Guangzhou Women and Children's Medical Center from May 2011 to May 2017.Imaging features of prenatal MRI and ultrasonography of the 12 fetuses were retrospectively analyzed.Differences in imaging findings of these two methods were analyzed by Chi-square test.Results All 12 women received prenatal ultrasound examination and eight of them underwent MRI scan when fetal congenital esophageal atresia was suggested by ultrasound.Both ultrasound and MRI were capable of identifing polyhydramnios and absent or small stomach bubble (12/12 and 8/8,respectively).However,MRI was superior to ultrasound in detecting "pouch sign "/"oral filling sign" or poor filling of small intestine (7/8 vs 3/12 and 8/8 vs 0/12,x2 were 7.500 and 20.000,both P＜0.01).While,no statistical difference was shown in detecting curved tracheal between MRI and ultrasound (2/8 vs 0/12,x2=3.333,P=0.067).For Gross Ⅰ or Gross Ⅲ congenital esophageal atresia fetuses,no statistically significant difference was found in their imaging features (all P＞0.05).The total detection rates after 32 weeks of gestation of Gross Ⅰ and Gross Ⅲ cases were both 3/6.Conclusions Prenatal MRI is a vital supplement to ultrasound due to its high display rate of characterized features of congenital esophageal atresia.Thus,the combined use of ultrasound and MRI is of great importance for prenatal diagnosis of this fetal abnormality.

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.

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.