OBJECTIVETo summarize the experience of applying gastric tube esophagoplasty for complicated diseases of esophagus in children and the short-middle-term efficacy.

METHODSA retrospective and observational case series study was performed.

INCLUSION CRITERIA(1) burn length of esophagus > 2 cm, multisegmental or extensive esophageal scar stenosis, and about 6 months after burn; (2) longitudinal diameter of esophageal tumor > 2 cm, or esophagus considered as impossible to reserve;(3) Severe esophageal fistula with diameter > 2 cm, or relapse again after ≥3 times of repair; (4) Tracheal cartilaginous esophageal heterotopia with a length of >2 cm or no end to end anastomosis after removal of the esophageal lesion.

EXCLUSION CRITERIApatients with severe cardiopulmonary insufficiency, or poor prognosis of gastric primary disease; the gastric volume did not allow long enough gastric tube; the parents did not accept the surgery. According to above criteria, 36 children with complicated diseases of esophagus who underwent gastric tube esophagoplasty at Department of Thoracic Surgery, Guangzhou Women and Children's Medical Center from March 2010 to June 2017 were enrolled into this study. Among 36 children, 27 were with corrosive strictures of esophagus, 5 with esophageal tumor, 3 with severe esophageal fistula, and 1 with tracheal cartilaginous esophageal heterotopia. Above-mentioned 27 cases with corrosive strictures of esophagus underwent gastric tube esophagoplasty via retrosternal route with preservation of the original esophagus. The other 9 cases underwent resection for esophageal lesion and gastric tube esophagoplasty via prevertebral route. The construction of gastric tube was as follows: the stomach was cut along the lesser curvature from pylorus to cardia and fundus of stomach with stapler, making the diameter of the gastric tube equal to pylorus. Operative time, intra-operative bleeding, time of mechanical ventilation, anastomotic leakage, anastomotic stricture were observed. The postoperative short-middle-term growth presentation of children was evaluated according to CDC 2000 children growth evaluation table(2 to 20 years).

RESULTSAll the 36 children survived their operations successfully. Nine cases underwent esophagectomy for lesion esophagus and the other 27 cases received preservation of original esophagus. Average time of postoperative mechanical ventilation was 8 (4-20) hours. Three cases developed anastomotic leakage and were healed after one week. Eight cases developed anastomotic stricture and resumed normal diet after balloon expansion. The patients were followed up from 6 months to 7 years. Five cases were found to have esophageal cyst 4-8 months after the operation, and received resection. One children with infantile esophageal fibrosarcoma recurred 3 weeks after the operation and died 2 weeks later because the family abandoned the treatment. The quality of life of 35 cases was improved significantly. Short-middle-term body height and weight in 85.7%(30/35) children met basically the criteria of CDC 2000 children growth evaluation table.

CONCLUSIONGastric tube esophagoplasty can effectively treat the children with complicated esophagus diseases with good short-middle-term efficacy, and is a recommended esophageal replacement surgery.

Burns ; surgery ; Child ; Esophageal Diseases ; surgery ; Esophageal Stenosis ; surgery ; Esophagoplasty ; Female ; Humans ; Quality of Life ; Retrospective Studies ; Stomach

Objective To investigate the significance of albumin-bilirubin (ALBI) score in predicting prognosis of hepatocellular carcinoma (HCC).Methods The clinical data of 644 HCC patients were retrospectively analyzed from May 2010 to May 2013 in the First People's Hospital of Changde City and the Affiliated Tumor Hospital of Guangxi Medical University.Peripheral blood test results were used to calculate ALBI score,and ALBI score was categorized into the following 3 groups:grade 1,grade 2,grade 3.Overall survival(OS) and disease free survival(DFS) were evaluated by the Kaplan-Meier method.And independent prognostic predictors were determined by the Cox proportional hazard model.Results Multivariate analysis showed that ALBI grade 2 was an independent risk factor for OS(P＜0.05).The ALBI grade stratified patients into at least two distinct overall survival cohorts (P＜0.05),whereas the CP grade did not.The ALBI grade also classified patients with CP grade A patients into two distinct overall survival cohorts (P＜0.05).In the subgroup analysis,the ALBI grade seemed to be an independent factor in terms of Barcelona Clinical Liver Cancer staging system(BCLC) 0-C.Conclusion For patients with HCC who underwent R0 resection,the ALBI grade is a good predictor of OS in HCC patients,and the above conclusions is true in patients with BCLC stage 0-C.

Objective To investigate the application of 3-D scanning in the diagnosis and evaluation of pectus excava-tum.Methods From July 2016 to June 2017, chest CT concomitant 3-D scanning were performed in 90 pectus excavatum pa-tients before Nuss procudure.Another 30 cases underwent chest CT scanning for non-chest deformity causes were chosen as a control group.The transverse and anterior-posterior maximum diameter through the deepest point of chest wall deformity were measured.The surface topography index and Haller index were calculated respectively .Results There was a positive correla-tion between CT and 3-D scanning diameters of the transverse and anterior-posterior maximum diameters, and consistency rates were 94% and 82%, respectively.There was significant difference between pectus excavatum group 1.82 ±0.21 and control group 1.41 ±0.07 in the STI(P<0.001).There was a positive correlation between Haller index 5.12 ±3.36 and STI 1.82 ± 0.21, and consistency rate was 89%.The Delong's test showed no significant difference between ROC of HI and STI(Z =1.18, P=0.28).Conclusion 3-D scanning and STI of pectus excavatum is a validated alternative for CT and Haller index, especially in the infant and non-surgical treatment PE cases.

Objective: To investigate the pathologic features of gonadal tissues of disorders of sexual development (DSD) in children. Methods: Fifty-three cases of gonadal developmental disorders were collected from July 2015 to August 2017 at Guangzhou Women and Children′s Medical Center. Clinical manifestations, karyotypes, sex hormone levels, ultrasound imaging, histology and immunophenotype of gonadal tissues were analyzed. Results: The age of patients ranged from 7 months to 17 years with an average of (50.7 ± 47.1) months. Social genders of the patients included 32 males and 21 females. Forty-eight patients had abnormal sex hormone levels. Clinical presentations included: toward female genitalia in 25 cases, male genitalia tendency in 17 cases and ambiguous external genitalia in 11 cases. Hypospadias was seen in 31 cases and short stature was seen in 8 cases. Chromosomal karyotyping of peripheral blood revealed 23 cases of sex chromosome disorders, 22 cases of 46 XY disorders, of which 3 cases were 5α-reductase deficiency and 8 cases of 46 XX disorders. Ultrasound examination showed cryptorchidism in 30 cases, including 16 cases of unilateral, 14 cases of bilateral and 1 case presenting a huge pelvic tumor. A total of 97 gonadal tissues from 53 cases of DSD were examined, including 9 cases of unilateral and 44 cases of bilateral gonads. Microscopically, 55 gonads (56.7%) showed dysplastic testes including 17 unilateral and 19 bilateral gonads. Fourteen were streak gonads (14.4%) including 8 unilateral and 3 bilateral gonadal tissues. Nine streak gonad with epithelial cord-like structures (9.3%) were found, of which 5 were unilateral and 2 were bilateral lesions. Seven gonads were ovotestis (7.2%), unilateral in 5 cases (the other side of the gonads of ovary in 4 cases, 1 case of dysplastic testes) and bilateral in 1 case. Seven gonads showed follicular-rich ovarian tissue (7.2%). One case showed bilateral dysplastic testes with gonadoblastoma and ectopic adrenal cortex. One case of streak gonad showed epithelial cord-like structures and undifferentiated glandular tissue embedded in malignant mixed germ cell tumors (mixed gonadoblastoma, dysgerminoma, mature teratoma and yolk sac tumor). One case had testicular microlithiasis. Uterus and fallopian tube structures were found in 11 cases. Immunohistochemical stains were performed in 15 cases. D2-40, PLAP and CKIT were expressed in germ cells and Calretinin, WT1 and inhibin were positive in Setoli cells. SALL4 and OCT3/4 were positive in 3 cases. Inhibin highlighted interstitial Leydig cells in 2 cases. GPC3 was positive in yolk sac tumor component. Conclusions Gonadal dysgenesis presents a broad spectrum of gonadal phenotypes with variable degrees of differentiation. The development of bilateral gonadal tissues has certain variability. Chromosomal karyotypes have no correlation with gonadal phenotypes. Accurate histopathologic diagnosis of gonadal dysgenesis plays an important role in the treatment and prognosis of the patient.

Objective To compare the clinical significance of peripheral blood neutrophil to lymphocyte ratio (NLR) and platelet to lymphocyte ratio (PLR) in predicting prognosis of patients with hepatocellular carcinoma (HCC).Methods The clinical data of 661 patients with HCC were retrospectively analyzed.Routine peripheral blood test results were used to calculate the NLR and PLR,and the receiver operating characteristic (ROC) curves were drawn.Using the thresholds of NLR and PLR,the patients were divided into the low NLR group and the high NLR group,and the low PLR group and the high PLR group.Overall survival (OS) and disease free survival (DFS) were evaluated by the Kaplan-Meier method.Independent prognostic predictors were determined by the Cox proportional hazard model.Results The NLR and PLR thresholds were 2.790 and 99,respectively.Analysis of the ROC curves showed higher NLR and PLR were significantly associated with poorer OS and DFS (all P ＜ 0.05).Multivariate analysis showed that NLR was an independent risk factor of OS and DFS (both P ＜ 0.05).The results remained unchanged when the NLR was further analyzed by applying different cut-off values of 2.810 and 3.In subgroup analysis,NLR remained an independent factor of Barcelona Clinical Liver Cancer staging system (BCLC) 0/A/B (P ＜ 0.05 for all measurements).Conclusion An elevated preoperative NLR could be a better prognostic predictor for HCC patients in comparison with PLR,especially for BCLC 0/A/B patients.

Objective:To evaluate the effect of surgery on 47 patients with moyamoya disease by retrospective analysis.Methods:A total of 47 patients with moyamoya disease were enrolled from August,2010 to According to the improved treatment in August,2013,all cases were divided into two groups:a pre-improved group and a post-improved group.According to different surgical methods,they were divided into two subgroups:an indirect revascularization subgroup and a combined revascularization subgroup.Results:The cerebral ischemia in 77.4％ of patients was relieved after the surgery.There was significant difference in outcomes of patients between the pre-improved group and the post-improved group (P＜0.05),while there was no significant difference between the pre-improved indirect revascularization subgroup and the pre-improved combined revascularization subgroup.There was also no significant difference between the post-improved indirect revascularization subgroup and the post-improved combined revascularization subgroups (P＞0.05).Conclusion:Surgical treatment can improve the outcomes of patients with moyamoya disease,but there is no significant difference in surgical effects between indirect and combined revascularization.

Purpose To investigate the histopathological features of cystic lung diseases ( CLD) , and to discuss the timing of clinical interventions. Methods HE and immunohistochemical staining were performed and reviewed in 125 cases of CLD. Results 125 ca-ses of CLD aged from birth to 11 years and 6 month, with an average age of 23. 0 months, median age 15 months, of which 60 cases were less than 1 year (48. 0%). 75 cases were male and 50 cases female, with male to female ratio of 1. 5 ∶ 1. Grossly, 50 cases showed single or multiple cysts with the size 0. 5 ~8. 0 cm in diameter, which did not communicate with bronchial cavity. 18 cases showed honeycomb cysts with the diameter of 0. 1~2. 0 cm. 26 cases were solid lesions without visible cysts. 21 cases were observed lung abscess with thick and rough wall and pus inside. 7 cases of emphysema showed microcysts with crepitation. 2 cases were identi-fied cystic and solid masses, with fish-fresh like cut surface. Histopathologically, 94 cases (75. 2%) were related to congenital bron-chopulmonary dysplasia in 125 cases of CLD, in which there were 59 patients (47. 2%) of congenial pulmonary airway malformation (CPAM), including 29 cases of type 1 (49. 2%), 18 cases of type 2 (30. 5%), and 12 cases of type 4 (20. 3%), there were 26 ca-ses (20. 8%) of pulmonary sequestration, including 15 cases of intralobar type (57. 7%) and 11 of extralobar cases (42. 3%), 5 ca-ses were complicated with CPAM type 2, 8 cases were bronchial cyst (6. 4%) and 1 case of enteric cyst (0. 8%). Acquired lesions were detected in 31 cases (24. 8%), including 21 cases of infected lung abscess, 1 case of fungal abscess. 7 cases of emphysema, and 3 cases of pleuralpulmonary blastoma (typeⅠ1 case and typeⅡ2 cases). Conclusion Pediatric CLD is characterized as com-plexed categories. The prognosis depends on correct pathological diagnosis, combined with imaging evaluation and appropriate timing of surgery.

OBJECTIVETo investigate clinical and pathological features of lung lesions in children.

METHODSClinical manifestations, radiologic imaging, histopathological features and immunohistochemical results were analyzed in 215 cases of lung lesions in children.

RESULTSA total of 215 cases of lung lesions in children aged 0 day to 13 years (average age of 27.2 months and the median age of 18.0 months) were selected, including 137 male and 78 female patients with a male to female ratio of 1.76:1.00. The incidence of congenital lung disease was higher in patients of less than 1 year old than those of over 1 year old age, and the difference of the two groups was statistically significant (P = 0.004). 142 cases had acquired lung diseases, and 73 cases had congenital bronchopulmonary dysplasia. Lung abscess was the most common lesion seen in 86 cases (40.0%), including 1 case of fungal abscess. Congenital pulmonary airway malformation (CPAM) was the second most common, seen in 44 patients (20.5%), including 20 cases of type 1, 18 cases of type 2 and 6 cases of type 4 CPAM. Pulmonary sequestration was found in 25 cases (11.6%) including 14 cases of intralobar type and 11 cases of extralobar type. Two cases of extralobar pulmonary sequestration showed simultaneous CPAM2 type 2 lesion. Other lesions included tuberculosis (13 cases, 6.0%), emphysema (12 cases, 5.6%), interstitial pneumonia (7 cases, 3.2%), pulmonary hemorrhage (6 cases, 2.8%), bronchogenic cyst (4 cases, 1.9%), bronchiolitis obliterans (2 cases, 0.9%), idiopathic pulmonary hemosiderin deposition disease (2 cases, 0.9%) and 1 cases of lung non-specific changes. 13 cases of neoplastic lesions (6.0%) were found, of which 11 cases were primary tumors (5.1%), including inflammatory myofibroblastic tumor in 5 patients (2.3%), pleuropulmonary blastoma in 5 cases (1 case of type I, 2 type II and 2 type III) and 1 case of mucoepidermoid carcinoma (0.5%) and 2 cases of metastatic tumors (hepatoblastoma and Wilm's tumor, 0.9%).

CONCLUSIONSInfectious diseases are the most common lung diseases in children. Congenital bronchopulmonary dysplasia is the most common in children of less than 1 year old. Malignant lesions are rare.

Abscess ; pathology ; Adolescent ; Bronchopulmonary Sequestration ; pathology ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Infant, Newborn ; Lung ; pathology ; Lung Diseases ; pathology ; Male ; Pulmonary Blastoma ; pathology

Objective To investigate the clinical and pathological characteristics,diagnosis,differential diagnosis,treatment and prognosis of giant neurofibroma of penis in the child.Methods The clinical data including general data,imaging data,treatment methods,pathological characteristics of a case with giant neurofibroma of penis in a child were analyzed retrospectively and the relevant literature was reviewed.Results Gross appearance of the penile shaft neurofibroma was about 9 cm × 11 cm × 15 cm,with local ulceration.Computerized tomography scan revealed a giant mass in the penile shaft,about 9.0 cm × 10.0 cm × 13.4 cm.Partial excision of the penis was performed.Postoperative appearance of the residual penile shaft was about 2 cm long.The HE staining showed spindle cells with the red dye cytoplasm,spindle or elliptic nuclei and arranged in wavy partly.Positive immunostaining was presented with S-100 protein and Vimentin.The pathologic examination revealed a neurofibroma.There was no evidence of recurrence and the penis of the boy had normal sensation and erection by follow-up in 2 years.Conclusions Neurofibroma of penis in the child is extremely rare and the differential diagnosis of soft-tissue tumors of penis should be considered.The operative method should be individualized,the treatment goal is the complete resection;however,this goal must be weighed against detriment to functioning and the cosmetics of the involved organ.

OBJECTIVETo analyze chromosome aberration in a child with mental retardation and abnormalities and its parents.

METHODSChromosome G banding, multiplex ligation-dependent probe amplification, fluorescence in situ hybridization and single nucleotide polymorphisms array were employed for analysis.

RESULTSKaryotype analysis revealed that the child was 46,XX and the father was 46,XY, while the mother was 46,XX, add (12)(p13). Subtelomeric region analysis with MLPA displayed that the child has reduced ACP1 gene copy number in 2p25 region and increased SLC6A12,KDM5A gene copy numbers in 12p11 region. SNP-array has fine mapped the duplication to 12p13.33-p12.3, a 15.142 Mb region, and a deletion to 2p25.3 for 3.194 Mb, which resulted in duplication of 9 genes including SLC6A12 as well as deletion of 11 genes including SNTG2, respectively. FISH analysis revealed that the child was 46,XX,ish,der(2),t(2;12)(p25;p13)mat, or partial monosomy 2p25 and partial trisomy 12p13. In addition,the mother was a carrier with cryptic balanced translocation chromosome, 46,XX,isht(2;12) (p25;p13). Mental abnormalities and retardation of the child may be attributed to heterozygous deletion of SNTG2, MYT1L genes and duplication of SLC6A12 gene.

CONCLUSIONCombined use of MLPA, FISH and SNP-array can facilitate accurate diagnosis of cryptic rearrangement at genomic level.

Adolescent ; Adult ; Carrier Proteins ; genetics ; Child ; Child, Preschool ; Chromosome Banding ; Chromosome Deletion ; Chromosomes, Human, Pair 12 ; genetics ; Chromosomes, Human, Pair 2 ; genetics ; Female ; Gene Rearrangement ; Humans ; Intellectual Disability ; diagnosis ; genetics ; Male ; Pedigree ; Protein Tyrosine Phosphatases ; genetics ; Proto-Oncogene Proteins ; genetics ; Translocation, Genetic ; Trisomy ; Young Adult