ObjectiveTo summarize the clinical features, diagnosis, treatment, and prognosis of congenital gluteal dermal sinus tracts in children, providing insights for better clinical management.MethodsA retrospective analysis was conducted on 20 patients diagnosed with congenital gluteal dermal sinus tracts at the Children's Hospital of Fudan University over 15 years. The analysis focused on age, gender, diagnostic delay, diagnostic methods, course and opening of the sinus tract, and postoperative follow-up.ResultsThe study included 6 males and 14 females, with symptom onset ranging from birth to 12 years (median age of 9.5 months). Initial symptoms included abnormal gluteal depression with recurrent infections (n = 8), unexplained recurrent infections (n = 6), and asymptomatic gluteal depression or small holes (n = 6). The time from symptom onset to diagnosis ranged from 1 to 102 months (median: 13.5 months). MRI was positive in 87.5%, identifying the tract in 81.25%. CT scans were positive in all cases, but only 50% identified the tract. Sinus tractography successfully identified the tract in 7 of 10 patients. The sinus tracts were classified into three types based on the opening location. All sinus tracts were excised during surgery, with an average length of 4.95 cm. Two patients experienced recurrence and underwent reoperation, while the remaining 15 had no recurrence.ConclusionCongenital gluteal dermal sinus tracts are more common in females and often misdiagnosed. CT is more sensitive but less specific than MRI. Sinus tractography helps determine the tract's path. Prognosis is generally good after complete excision, with recurrence being the main postoperative complication.

Purpose: There is no standard surgical approach for pancreaticobiliary maljunction (PBM) without congenital biliary dilatation (CBD). This study aimed to compare outcomes between therapeutic endoscopic retrograde cholangiopancreatography (ERCP) and laparoscopic hepaticojejunostomy (LH) for pediatric patients of PBM without obvious biliary dilatation (PBM-nonOBD). Methods: We retrospectively reviewed demographic and clinical data of pediatric patients with PBMnonOBD from 2015 to 2021. There were 33 patients in ERCP group and 35 patients in LH group. Primary outcomes included treatment efficiency, postoperative recovery, and postoperative complications. Univariate analysis was further used to explore prognostic factors for ERCP. Results: The mean diameter of the common bile duct in LH group was larger than that in ERCP group (8.6 +/- 1.3 mm vs. 6.9 +/- 2.1 mm, p 1 / 4 0.003), while there were no significant differences between the two groups in age, gender, clinical manifestations, complications, and other imaging findings. Compared with LH group, ERCP group had a shorter operation time and postoperative recovery time. The treatment effective rate of ERCP was inferior to that of LH (45.4 % vs. 85.7 %, p <0.001). For postoperative adverse events, post-ERCP pancreatitis (15.1 %) was most common in the ERCP group. 30.3 % of patients eventually required LH. Intestinal obstruction (5.7 %), recurrent cholangitis (5.7 %), gastrointestinal bleeding (2.8 %), and anastomotic stenosis (2.8 %) were observed in LH group and 8.6 % of patients required a reoperation. A long common channel may be associated with poor prognosis after ERCP. Conclusions: ERCP is associated with less surgical trauma, shorter recovery time, and fewer serious complications than LH, while the treatment effective rate of ERCP is inferior to LH. The indications for endoscopic sphincterotomy and the timing of radical surgery need to be further explored. Level of evidence: III Study type: Retrospective Comparative Study. (c) 2023 Elsevier Inc. All rights reserved.

OBJECTIVE:To evaluate the efficacy and side effects of additional postoperative steroid therapy for type 3 BA versus the current routine care.SUMMARY BACKGROUND DATA:Whether steroid therapy post-Kasai portoen-terostomy improves the outcomes of BA remains controversial. Clinical evidence from 2 randomized trials in the UK and USA do not support the routine use of steroid in the treatment of BA.METHODS:In this open-label randomized controlled trial, patients with type 3 BA were randomized to routine postoperative treatment with or without 10 to 12 weeks of adjuvant steroid treatment. The primary outcome was the postoperative jaundice clearance rate with native liver at 6 months. The secondary outcomes included postoperative jaundice clearance rate at 3, 12, and 24 months, survival with native liver at 12 and 24 months, and SAEs within 3 months.RESULTS:Overall, 200 participants were randomized and allocated into either steroid or control group (n = 100/group). The proportion of participants that are jaundice free without liver transplantation was significantly higher in the steroid group than in the control group at 6 months (54.1% vs 31.0%, P = 0.0015). The native liver survival rate was higher postoperatively in the steroid group than in the control group at 12 (66.3% vs 50.0%, P = 0.02) and 24 (57.1% vs 40.0%, P = 0.02) months. The survival time with native liver was significantly longer in the steroid group than in the control group (median survival, steroid vs control: not reached vs 1.21 years, P = 0.02). There were no significant differences between the 2 groups in the mean occurrence of SAEs within 3 months (steroid vs control: 0.63 vs 0.45, P = 0.20).CONCLUSIONS:Postoperative adjuvant steroid intervention improved bile drainage and survival with native liver in type 3 BA patients, without increasing early-stage SAEs.

Introduction The objective of this study is to summarize the clinical characteristics and management of rare diseases of colorectal vascular malformation (CRVM) in children. Methods We retrospectively analyzed the clinical data of CRVM patients admitted to the Children's Hospital of Fudan University from 2004 to 2019. Results A total of 23 cases (16 males, 7 females) were enrolled. The median age of symptom onset was 1.4 years. Hematochezia and anemia were cardinal symptoms. Fourteen patients (60.9%) were misdiagnosed as anal fissures ( n = 4), internal hemorrhoids ( n = 3), rectal polyps ( n = 2), inflammatory bowel disease ( n = 2), portal hypertension ( n = 2), and Meckel's diverticulum ( n = 1), respectively. The average time from symptom onset to diagnosis was 4.5 +/- 4.4 years. Other vascular malformations were detected in eight patients (34.8%). All patients showed a positive anomalous vascular image on contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI). The sensitivity of colonoscopy in the diagnosis of CRVM was 82.6% (19/23). A total of 21 patients underwent a modified Soave procedure. The lesions were mostly restricted to the colorectum and showed transmural diffuse distribution, with an average length of 20 +/- 5.4 cm. Two patients (9.5%) experienced surgical complications. Bloody stools reappeared in two patients (9.5%), and colonoscopy showed abnormal angiogenesis at the anastomotic site, which were cured by sclerotherapy and/or electrocautery. The median follow-up time was 78 months. Bloody stools were absent at the last time of follow-up, and hemoglobin was in the normal range for all patients. Conclusion The identification of CRVM in children often is delayed. Colonoscopy, CT, and MRI are essential in making the correct diagnosis. The modified Soave procedure is safe and feasible to treat CRVM in children. Endoscopic sclerotherapy and/or electrocautery can be used for residual lesions.

Objective:To summarize the clinical efficacy of Frey's operation for chronic pancreatitis in children with pancreatic duct dilation.Methods:From January 2015 to May 2020, 16 children with chronic pancreatitis and pancreatic duct dilatation underwent Frey's operation.Clinical data and surgical approaches were retrospectively reviewed along with perioperative complications, surgical outcomes, long-term pancreatic morphology and function.Results:There were 5 boys and 11 girls with an average age of 7.8 years.Abdominal pain was an initial symptom ( n=14). One case was emaciated and one case had hypoglycemia.Serum amylase ranged from 143.5 to 678.8 U/L.The diameter of pancreatic duct dilatation was (2.6-12.2) mm; color Doppler ultrasound, magnetic resonance cholangiopancreatography (MRCP) and enhanced computed tomography (CT) indicated pancreatic duct dilatation and distortion, pancreatic atrophy with peripheral fibrous tissue hyperplasia.The average surgical duration was 132 min.During perioperative period, one patient developed pancreatic fistula and drainage tube was implanted.It healed after 2 weeks; one child of obstructive jaundice faded after endoscopic retrograde cholangiopancreatography (ERCP) duodenal papillotomy; one case of pancreatic tail pseudocyst was cured after re-operation and internal drainage.The average follow-up period was 42(11-60) months.Serum amylase of 10 cases returned to normal at Day 7 post-operation and abdominal pain disappeared; 5 cases of postoperative abdominal pain gradually relieved and disappeared 1 month later.Serum amylase gradually normalized and both growth and development were satisfactory.One case of severe preoperative pancreatic atrophy and hypoglycemia had recurrent postoperative abdominal pain, blood amylase spiked occasionally, growth and development were slightly poor and yet blood glucose was well-controlled.There was no deterioration of pancreatic exocrine function, postoperative bleeding or intestinal obstruction. Conclusion:Frey's operation is both safe and efficacious for children with chronic pancreatitis complicated with pancreatic duct dilation.Wider popularization is recommended.

1 前言 胆道闭锁是新生儿严重、致死性疾病,以进行性炎症、纤维化为特征,肝内外胆管出现部分或完全闭塞.若不及时诊治,常于1～2岁内因肝功能衰竭而死亡[1].文献报道[2] ,胆道闭锁发病率为0.55 ～1.3 / 10000 名活婴,中国上海地区胆道闭锁发病率约为10.86/10万活婴,为欧美国家的2～3倍[3].

Objective: To evaluate the efficacy and side effects of additional postoperative steroid therapy for type 3 BA versus the current routine care. Summary Background Data: Whether steroid therapy post-Kasai portoen-terostomy improves the outcomes of BA remains controversial. Clinical evidence from 2 randomized trials in the UK and USA do not support the routine use of steroid in the treatment of BA. Methods: In this open-label randomized controlled trial, patients with type 3 BA were randomized to routine postoperative treatment with or without 10 to 12 weeks of adjuvant steroid treatment. The primary outcome was the postoperative jaundice clearance rate with native liver at 6 months. The secondary outcomes included postoperative jaundice clearance rate at 3, 12, and 24 months, survival with native liver at 12 and 24 months, and SAEs within 3 months. Results: Overall, 200 participants were randomized and allocated into either steroid or control group (n = 100/group). The proportion of participants that are jaundice free without liver transplantation was significantly higher in the steroid group than in the control group at 6 months (54.1% vs 31.0%, P = 0.0015). The native liver survival rate was higher postoperatively in the steroid group than in the control group at 12 (66.3% vs 50.0%, P = 0.02) and 24 (57.1% vs 40.0%, P = 0.02) months. The survival time with native liver was significantly longer in the steroid group than in the control group (median survival, steroid vs control: not reached vs 1.21 years, P = 0.02). There were no significant differences between the 2 groups in the mean occurrence of SAEs within 3 months (steroid vs control: 0.63 vs 0.45, P = 0.20). Conclusions: Postoperative adjuvant steroid intervention improved bile drainage and survival with native liver in type 3 BA patients, without increasing early-stage SAEs.

Piriform sinus fistula (PSF) is commonly managed by surgery in quiescent inflammation, while medical conditions are hard to be stabilized in patients suffering from recurrent infections. This research attempts to discuss the diagnostic and therapeutic strategies for PSF in acute inflammation. We retrospectively analyzed the medical records of 113 patients with PSF. Presence of infection within 4 weeks before the operation was taken as a reference to divide the cases into the operation group without infection (Group A) and with infection (Group B). There were 75 cases in Group A and 38 cases in Group B, with an average onset age of 8.16 (5.25-11.58) and 5.63 (4.00-9.54) years, respectively. Esophageal barium swallow identified a positive predictive value of 98.2% in Group A and 100% in Group B. Endoscopic argon gas cauterization was performed in 19 cases (19/75) in Group A, a combination with peripheral doxycycline injection in 42 cases (42/75) and endoscopic- assisted fistulectomy in 14 cases (14/75), compared to 14 cases (14/38), 16 cases (16/38) and 8 cases (8/38) respectively in Group B. The overall recurrence rate was 29.3% in Group A, while those in Group B was 36.8%. Bacterial culture of pus mainly grew Streptococcus in both groups. There was no significant difference in the recurrence rate by EAGC in patients with or without acute infections, and the addition of local doxycycline injection may lead to a decrease of the recurrence rate. Additionally, patients who were complicated with thyroid abscess suffered from a relatively high recurrence rate, requiring specific treatment.

To investigate the diagnostic utility of immunohistochemistry for paired-like homeobox 2B (PHOX2B) expression in neuroblastomas (NBs) and tumors that mimic them, tissue samples (n = 229) from 157 cases of NB, 210 central nervous system tumors, and 170 extracranial non-NB solid tumors (n = 170) were immunostained for PHOX2B. Additionally, PHOX2B expression in 67 body fluid cytology specimens was analyzed. In tissue specimens, PHOX2B expression was positive in NBs, pheochromocytomas, and paragangliomas but negative in all of the other tumors evaluated. PHOX2B was detected by immunohistochemistry in 5 NB cytology specimens; all of the others were negative. These results suggest that PHOX2B may be a sensitive and specific immunohistochemical marker for the pathological diagnosis and differential diagnosis of NB in both tissue and cytology specimens.

Objectives : To summarize the clinical features, treatment methods, and outcomes of the rare diseases of colorectal vascular malformation (CRVM). Methods : We retrospectively analyzed clinical data of CRVM patients admitted to the Department of Pediatric Surgery of Children's Hospital of Fudan University from 2004 to 2019. Results : A total of 23 cases (16 males, 7 females) were enrolled. The median age of symptom onset was 1.4 years. Eleven patients had been misdiagnosed, and the average time from symptom onset to diagnosis was 4.5±4.4 years. Associated vascular malformations in the skin and other parts were found in eight patients. All patients showed a positive anomalous vascular image in the rectum and surrounding areas with contrast-enhanced computed tomography (CT) / magnetic resonance imaging (MRI). The sensitivity of endoscopy in the diagnosis of CRVM was 82.6%. A total of 21 patients underwent a modified Soave procedure. The lesions were mostly restricted to the rectum and sigmoid, with an average length of 20±5.4cm. The median follow-up time was 78 months. Bloody stools reappeared within 6 months in three patients, but were cured by sclerotherapy or fulguration. At the last time of follow-up, bloody stool symptoms were absent and hemoglobin was in the normal range for all patients. Conclusions : CRVM occurs more commonly in male children than in female children. Combined with enhanced MRI, CT, and colonoscopy, CRVM diagnosis can be confirmed. The lesioned intestinal segment can be safely removed through a modified transabdominal Soave procedure, effectively curing or relieving hematochezia.

目的评估改良Pe?a术一期根治肛门闭锁伴直肠前庭瘘的临床疗效。方法选取2003年1月至2017年12月复旦大学附属儿科医院109例肛门闭锁伴直肠前庭瘘患儿作为研究对象,其中行改良Pe?a术59例（改良Pe?a术组）,经会阴肛门移位术50例（经会阴肛门移位术组）。对两种术式进行评估（包括术前准备时间、手术时间、术中输血量、术后禁食天数、抗生素应用时间、住院天数、费用及术后并发症）,采用Krickenbeck分类和直肠肛管测压比较患儿术后排便控制能力。结果改良Pe?a术组手术年龄为（2.3±0.4）个月,经会阴肛门移位术组为（6.4±1.2）个月,差异有统计学意义（t=13.93,P ＜0.001）。改良Pe?a术组术前均未伴发直肠扩张,经会阴肛门移位术组有31例伴发直肠扩张,差异有统计学意义（χ2=51.118,P ＜0.001）。两组在伴发畸形和术前准备时间、手术时间、术中输血量、术后禁食天数、应用抗生素时间、住院天数及费用、近期并发症方面无统计学差异（P＞ 0.05）。经会阴肛门移位术组再手术率（5/50,10.0%）高于改良Pe?a术组（1/59,1.7%）,差异有统计学意义（χ2=105.644,P=0.043）。两组患儿术后污粪和便秘总发生率无统计学差别（P＞ 0.05）。经会阴肛门移位术组术后2级便秘和3级便秘发生率分别为22.0%和6.0%,改良Pe?a组分别为10.3%和2.6%,差异有统计学意义（P ＜0.05）。两组患儿术后12个月行直肠肛管测压检查均未引出直肠肛管抑制反射,术后直肠静息压、主动收缩压、直肠感觉阈值差异无统计学意义（P＞ 0.05）。虽然经会阴肛门移位术组患儿术后的直肠顺应性[（3.5±1.1） mL/mmH g]低于改良Pe?a术组[（5.3±1.4） mL/mmH g],但差异无统计学意义（P＞ 0.05）。结论改良Pe?a术一期根治先天性肛门闭锁伴直肠前庭瘘患儿,术后远期排便功能优于经会阴肛门移位手术。

Objective To explore the pathological types and clinical values of intraoperative frozen pathology for surgical children with persistent hyperinsulinemic hypoglycemia of infancy (PHHI).Methods A total of 25 PHHI infants were operated from April 2011 to October 2016.And retrospective analyses were performed for clinical data,surgical approaches and pathological characteristics.Results There were 17 boys and 8 girls with an age range from 16 days to 12 months.The fasting levels of blood glucose and insulin were (0.6-5.5) mmol/L and (3.1-50.1) mU/L respectively.Based upon the preoperative examinations and intraoperative frozen pathology,they underwent focal type and subtotal pancreatectomy respectively.During a postoperative follow-up period of (2-38) months,fasting blood glucose had a range of (3.0-12.6) mmol/L.Fifteen children recovered well without any complication.One case of hypoglycemia required glucagon.And a low fasting level of glucose normalized after ingesting (n =3) and postoperative hyperglycemia required medications for controlling blood glucose (n =6).One case with suspected focal pancreatic lesion underwent a focal resection of 50％ and relapsed after 2 weeks.According to the pathological classification criteria,their clinical types were focal (n =6),diffuse (n =18) and atypical (n =1).Conclusions Clinically it is important to confirm the classification of pathological types.Preoperative examinations and intraoperative frozen pathology may help to find effective treatments and improve the outcomes.The most common pathological type is diffuse Further studies are required for atypical type.

Objective:To further classify liver hemangiomas to examine whether or not a continuum existed between multifocal versus diffuse liver hemangioma.Methods:Between January 2000 and January 2020, a total of 43 children with a diagnosis of multifocal or diffuse hepatic hemangioma at Children's Hospital of Fudan University were analyzed retrospectively. They were divided into multifocal-type (n=34) and diffuse-type (n=9) groups according to radiological features. And multifocal-type group was further divided into countable (MC)(n=6) or uncountable (MU)(n=28) subgroups according to whether lesions were countable or not. Clinical features between each group were compared.Results:For MC, MU and diffuse-type groups, the incidence of cutaneous hemangioma was 66.7%(4/6), 50.0%(14/28) and 44.4%(4/9); the incidence of hepatomegaly 0, 46.4%(13/28) and 100%(9/9); the incidence of heart failure 0, 28.6%(8/28) and 44.4%(4/9); the incidence of dyspnea 0, 25.0%(7/28) and 66.7%(6/9); the incidence of hypothyroidism 0, 21.4%(6/28) and 77.8%(7/9). The incidence of hepatomegaly, dyspnea and hypothyroidism was higher in diffuse-type group with statistical significance than that in MC group ( P<0.001; P=0.017; P=0.006) and that in MU group ( P=0.004; P=0.032; P=0.004). And the incidence of hepatomegaly was higher in MU group than that in MC group ( P=0.040). Among 34 cases in multifocal-type group, 16 cases (47.1%) were observed and 18 cases (52.9%) received medical treatment or medical treatment plus interventional measures. Particularly, 6 cases (100%) in MC group were observed; 10/28 cases (35.7%) in MU group were observed and 18 cases (64.3%) received medical treatment or medical treatment plus interventional measures. Nine cases (100%) in diffuse-type group received medical treatment and 3 cases (33.3%) received medical treatment plus interventional measures. The proportion of children receiving medical treatment or medical treatment plus interventional measures in diffuse-type group was higher than those in MC/MU/multifocal-type group with statistical significance ( P=0.027; P=0.038; P<0.001). And the proportion of those receiving medical treatment or medical treatment plus interventional measures was also higher in MU group than that in MC group with statistical significance ( P=0.006). During a follow-up period of over 6 months, all children in multifocal-type group finally survived with stable conditions. However, 2 cases (22.2%) in diffuse-type group died of severe heart failure after medical treatment plus hepatic arteriovenous fistula embolization. In contrast, the rate of complete remission was 83.3%(5/6) in MC group, 50.0%(14/28) in MU group and 33.3%(3/9) in diffuse-type group. It decreased gradually. Conclusions:MU group is a subgroup of multifocal hepatic hemangioma with too-numerous-to-count lesions on transverse sections of CT/MRI. With similar clinical features to diffuse hepatic hemangioma, there may be a continuum between multifocal and diffuse hepatic hemangiomas. Timely treatment is needed.

目的探讨肝肿瘤切除术治疗POST-TEXT（post-treatment extent of disease）Ⅲ期和Ⅳ期肝母细胞瘤的中长期治疗效果。 方法回顾性分析2009年1月至2019年6月复旦大学附属儿科医院肿瘤外科收治的POST-TEXT Ⅲ期和Ⅳ期肝母细胞瘤患儿的临床资料（包括PRETEXT分期、甲胎蛋白、CT或MRI影像学资料、肿瘤切除术式及预后结果）。采用SPSS18.0统计软件包中的Kaplan-Meier法进行生存分析。 结果本研究共纳入POST-TEXT Ⅲ期和Ⅳ期肝母细胞瘤患儿37例，其中男25例，女12例，发病年龄2~91个月，平均发病年龄（23.92±22.45）个月。POST-TEXTⅢ期和Ⅳ期分别为34例和3例，其中1例PRETEXT Ⅱ期病例治疗过程中升级为POST-TEXT Ⅳ期，3例PRETEXT Ⅳ期病例治疗过程中降级为POST-TEXT Ⅲ期。新辅助化疗2个、4个和6个疗程内手术人数分别为8例（21.6%）、22例（59.4%）和7例（18.9%）。除2例被建议行肝移植手术后放弃治疗外，其余35例均进行肝肿瘤切除术，其中肝三叶切除术7例，扩大半肝切除术8例，不规则肝切除术4例，肝中叶切除术12例，ALPPS（associating liver partition and portal vein ligation for staged hepatectomy）术4例。平均手术时间（262.29±107.16）min，术中平均出血量（236.86±212.41）mL。采用Glisson蒂横断技术22例，平均出血量（147.73±137.46）mL，胆漏发生率27.3%，与未采用该技术的病例比较，出血量明显减少[（147.73±137.46）mL vs.（387.69±235.69）mL，P=0.001]，胆漏发生率相似（27.3%vs. 23.1%，P=0.784）。术中切缘>1 cm者7例（20.0%），切缘0.5~1 cm者15例（42.9%），切缘 0.05）。5年总体生存率为72.3%，5年无瘤生存率为67.4%。根据切缘距离分类，切缘>1 cm、切缘0.5~1 cm、切缘 结论对于POST-TEXT Ⅲ期和Ⅳ期的肝母细胞瘤，根据肿瘤累及的不同部位选择相应的术式，可以取得较好的治疗效果。熟练掌握Glisson鞘分离技术可以减少术中出血和胆道损伤的发生。手术中只需确保一定的切缘距离，就可以减少肿瘤复发，提高远期生存率。

Objective: To investigate the long-term outcomes of extended hepatectomy for POST-TEXT III and IV hepatoblastoma. Methods: The clinical data of 35 patients with POST-TEXT III and IV hepatoblastoma from January 2009 to June 2019 were analyzed retrospectively. Results: There were 34 cases of POST-TEXT III and 1 cases of POST-TEXT IV, respectively. All 35 cases underwent hepatic resection, including 7 cases of hepatic trisectionectomy, 8 cases of extended hemihepatectomy, 4 cases of irregular hepatectomy, 12 cases of mesohepatectomy and 4 cases of ALPPS procedure. Pringle maneuver and Glissonean approach was used in 4 and 22 patients, respectively. The average blood loss of patients with Glissonean approach (147.73 ± 137.46ml) was significantly less than that of patients without the approach (387.69 ± 235.69ml; P = 0.001). The 5-year overall survival rate and event-free survival rate was 79.2% and 73.7%, respectively. According to the classification of tumor margin distance, the 5-year overall survival rates of the tumors with margin > 1cm, 0.5-1cm, < 0.5cm and close to the tumor margin were 100%, 78.0%, 83.3% and 53.3%, respectively (P=0.371). Seven patients developed recurrence which occurred within 1 year after tumor resection. Conclusion: Non-transplant extended hepatic resection is a feasible approach for POST-TEXT III and IV hepatoblastomas. On the basis of mastering the Glisson approach and ensuring a certain resection margin, it is possible to achieve a similar oncological outcome to liver transplantation.

Neuroblastoma (NB), which is a subtype of neural-crest-derived malignancy, is the most common extracranial solid tumor occurring in childhood. Despite extensive research, the underlying developmental origin of NB remains unclear. Using single-cell RNA sequencing, we generate transcriptomes of adrenal NB from 160,910 cells of 16 patients and transcriptomes of putative developmental cells of origin of NB from 12,103 cells of early human embryos and fetal adrenal glands at relatively late development stages. We find that most adrenal NB tumor cells transcriptionally mirror noradrenergic chromaffin cells. Malignant states also recapitulate the proliferation/differentiation status of chromaffin cells in the process of normal development. Our findings provide insight into developmental trajectories and cellular states underlying human initiation and progression of NB.

Objective To analyze the clinical data of separation surgeries for conjoined twins to compare the different kinds of surgeries and to summarize the prognosis to discuss the optimal treatment strategy.Methods The clinical data were collected for all conjoined twins treated from November 2002 to November 2017.Then retrospective analysis was performed for conjoined types,operative duration,separating methods,postoperative complications and prognosis.Results Among 8 couples of symmetrical twins,there were 5 couples of female twins,3 couples of male twins and 6 couples of omphalopagus,1 couple of pygopagus and 1 couple of ischiopagus respectively.During separations,3 couples of twins underwent enterostomy and 1 couple enterostomy and cystostomy.There were also 2 infants in 2 different couples undergoing extracorporeal circulation because of heart disease.Seven couples of twins (14 infants) survived for a long term.As for other couple of twins,one died within 1 month and another one died of some other factors within 6 months.Three-dimensional (3D) printing was used for evaluating the characteristics,ranges and relations of conjoined organs for 4 couples and then separated skin was repaired by Z-plasty transposition flap design.All 4 couple twins recovered well after operations.Conclusions The overall survival rate of conjoined twins is relatively high after separation operations.And 3D printing offers system evaluations and preoperative simulations.Z-plasty transposition flap design is ideal for repairing skin defect and beautifying injury to raise living quality.

Purpose: The purpose of this study was to investigate (i) postoperative course of apple-peel atresia (APA), (ii) long-term follow-up of APA children, and (iii) risk factors for poor prognosis. Methods: We conducted a retrospective review of 39 APA neonates treated at our institution between 2008 and 2017. Patient characteristics, operative details, postoperative course, long-term outcomes, and prognostic factors were analyzed. Results: Of the 39 APA neonates, 30 (76.9%) were born preterm, and 20 (51.3%) were diagnosed prenatally. All patients underwent primary anastomosis within the first week after birth: 10 laparoscopic-assisted (25.6%) and 29 open (74.4%). Postoperative complications occurred in 28 patients (71.8%), of which 20 (71.4%) developed cholestasis. Survival at hospital discharge was 94.9%. Median parenteral nutrition period was 59 days. Reoperation was required in 7 children (17.9%) owing to anastomotic obstruction (n = 3) and adhesive intestinal obstruction (n = 4). 32 children (82.1%) were followed up for an average of 5.7 years, of which 23 children (71.9%) showed normal growth and development. APA patients with low birth weight and associated anomalies had significantly worse outcomes. Conclusion: Most of the patients with apple-peel atresia have excellent long-term outcomes, though initial postoperative complications are common. Low birth weight and the presence of associated anomalies are independent prognostic factors in APA. (C) 2019 Elsevier Inc. All rights reserved.

Objective To explore the clinical characteristics of kaposiform lymphangiomatosis (KLA) and to enhance its awareness and management skills of sirolimus therapy.Methods Four hospitalized cases of KLA were recruited from February 2014 to January 2017.Age,gender,location of pathologic change,clinical symptoms,pathological characteristics,imaging features,therapeutic regimens and prognosis were analyzed retrospectively.Results There were 3 boys and 1 girl with a median age of 60 (10-108) months.The major clinical symptoms were dyspnea and shortness of breath.There were skin lesions or ecchymosis (n=3),bilateral pleural effusion (n =2) and unilateral pleural effusion (n =2).Computed tomography (CT) indicated the involvement of multiple bones.And magnetic resonance imaging (MRI) revealed multiple soft tissue lesions with high signal on T2-weighted images.Some invaded as far as mediastinum and posterior peritoneum.Laboratory examinations suggested coagulation dysfunctions:fibrin (0.82 ± 0.1) g/L,prothrombin time (16.6 ± 1.5) seconds and activated partial thromboplastin time (22.7±2.2) seconds.Blood routine examinations revealed platelets (16-139) × 109/L,with a median of 105 × 109/L,hemoglobin 69-138 g/L with a median of 89 g/L.Thoracentesis showed bloody pleural effusion with positive chyle test.Puncture and drainage could relieve symptoms which recurred.Two cases of skin biopsy yielded a pathological diagnosis of generalized lymphatic anomaly (GLA).Two cases undergoing thoracotomy showed multiple chylous exudating in dorsal pleura and suture was ineffective.Subsequently 4 patients received sirolimus (0.8 mg/m2,bid).One case achieved a complete response,2 cases had a partial response and 1 died.Three patients stayed in treatment.No significant side effect was observed during treatment.Conclusions Though as a benign vascular malformation,KLA has aggressive clinical presentations with coagulation dysfunction,bloody chylothorax and a high mortality.Sirolimus is an effective and safe treatment.Further studies are needed to evaluate its long-term efficacy in KLA patients.