Objective:To discuss the diagnosis and treatment 0f WAGR syndrome.Methods:The clinical data of 10 cases of WAGR syndrome children admitted to our hospital from January 2008 to November 2019 were respectively analyzed including the clinical features, diagnosis, and surgical treatments. There were 6 males and 4 females, aged from 13 to 36 months, with an average of 23.6 months. 9 cases were diagnosed as iris absence due to ocular abnormalities in infancy, and 1 case was diagnosed as iris absence due to ocular abnormalities by physical examination because of renal mass. There were 2 boys with cryptorchidism, and 2 boys with hypospadias, 1 of which did not received operation because of mild hypospadias, and another undergoing surgery. There were no abnormality of genitourinary system in the remaining 5 cases. There were 7 cases of unilateral nephroblastoma, with 1 case at the left and 6 cases at the right, and there were 3 cases of bilateral nephroblastoma. Abdominal doppler ultrasound and enhanced abdominal CT were performed for all patients. Abdominal doppler ultrasound indicated solid mass in renal parenchyma or non-uniform echo zone. Abdominal enhanced CT indicated renal tumor with diameter of 1.8 cm-12.7 cm and locally non-uniform enhanced echo. Among the 7 cases of unilateral nephroblastoma, 4 underwent nephrectomy, 1 underwent tumor enucleation, and 2 underwent tumor enucleation for unilateral tumor complicated with nephrogenic rests. There were 3 cases of bilateral nephroblastoma, 2 cases undergoing unilateral tumor enucleation firstly and contralateral tumor enucleation following chemotherapy. One case underwent unilateral tumor nephrectomy followed by contralateral tumor enucleation. One case of unilateral nephrogenic rests did not undergo renal tumor surgery. Preoperative chemotherapy was performed in 7 patients, including 3 bilateral nephroblastoma, 1 unilateral nephroblastoma combined with contralateral nephroblastoma, and 3 unilateral tumors larger enough to pass the midline. The chemotherapy regimen was VCR+ ACTD in 5 cases, VCR+ ACTD+ CTX+ DOX/CDDP+ VP16 and VCR+ CTX+ DOX in another 2 cases respectively.Results:All 10 cases were diagnosed as nephroblastoma. There were 3 patients without preoperative chemotherapy which belongs to COG stageⅠ(1 case) and STAGEⅢ(2 cases); Preoperative chemotherapy was performed in 2 patients with SIOP stage Ⅱ, 2 patients with SIOP stage Ⅲ, and 3 patients with SIOP stageⅤ. Nine children received regular chemotherapy after surgery, among which 1 child in stage Ⅰ received DD4A chemotherapy regimens, 2 children in stage Ⅱ received DD4A and EE4A regimen respectively, and 3 of the 4 children in stage Ⅲ received regular chemotherapy after surgery, including EE4A(1 case)and DD4A(2 cases). EE4A(1 case)and DD4A(2 cases) chemotherapy were performed in 3 patients with stage Ⅴ according to their unilateral tumor stage. Ten cases were followed up, with 9 of the 10 cases having no tumor recurrence or metastasis, and death in 1 case. At present, abdominal doppler ultrasound of 1 child with nephrogenic rests showed no obvious progress. The renal function of 9 children was not significantly abnormal during the regular follow-up. The results of intelligence screening showed that 6 of the 10 patients were significantly behind their peers, and 4 had no obvious abnormality compared with their peers. Gene tests were performed 3 times after surgery, and the results showed the deletion of 11p13 and adjacent distal genes.Conclusions:WAGR syndrome is rare in clinical practice, and renal ultrasound should be monitored after diagnosis to detect renal tumors in early stage. For bilateral cases, renal function should be preserved as long as possible in order to reduce the probability of renal failure. Long-term follow-up of nephroblastoma with this syndrome is particularly important.

Objective:To investigate the clinical characteristics and therapeutic efficacy of spontaneous intracranial hypotension (SIH) complicated with cerebral venous thrombosis (CVT).Methods:The clinical data of 4 patients with SIH complicated with CVT admitted to our hospital from March 2014 to April 2020 were retrospectively analyzed. And the clinical data of 35 patients with SIH complicated with CVT were included for summary analysis through literature retrieval (the databases included PubMed, CNKI and Wanfang; retrieval period was from database construction to December 31, 2020).Results:These 4 patients were with onset of orthostatic headache; one was with recurred orthostatic headache after relief, and the other 3 developed persistent headache and epileptic seizure; case 1 was with superior sagittal sinus and cortical vein thrombosis, case 3 was with superior sagittal sinus thrombosis, and other 2 patients were with isolated cortical vein thrombosis. Twenty-six documented cases demonstrated headache changes: 12 patients (46.15%) developed persistent headache, 12 patients (46.15%) showed orthostatic headache persistently, and 2 patients (7.69%) had disappeared headache. The most common new symptoms were epilepsy in 17 patients (48.57%) and limb weakness in 10 patients (28.57%). Totally, these 31 patients (4 patients from our hospital+27 patients from literature retrieval) had hemorrhage after treatment; the percentage of patients having hemorrhage changes in the 17 patients accepted anticoagulant therapy was significantly increased as compared with that in 14 patients accepted other treatments (7/17 vs. 1/14, P<0.05); there were no bleeding changes in 5 patients accepted epidural blood patch and anticoagulant therapy. Conclusions:The clinical features of SIH complicated with CVT are various, and the change of headache is not a reliable marker. In the course of SIH, it is necessary to be alert to the occurrence of CVT if there are new symptoms such as epileptic attack or limb weakness. The etiological treatment of SIH is essential and the hemorrhage risk after anticoagulant therapy should be concerned in patients with SIH complicated with CVT.

Objective:To analyze the clinical and imaging features of patients with spontaneous intracranial hypotension (SIH) combined with vestibulocochlear nerve symptoms, and explore the mechanism of vestibulocochlear nerve damage.Methods:From January 2014 to September 2019, 72 patients with SIH were chosen in our hospital; their clinical data were retrospectively analyzed; all patients underwent brain MR imaging. Based on vestibulocochlear nerve symptoms including dizziness, vertigo, tinnitus, aural fullness, and hearing loss, these patients were divided into two groups: 27 patients with vestibulocochlear nerve symptoms and 45 patients without vestibulocochlear nerve symptoms. The clinical and brain MR imaging features were compared between the two groups. The quantitative indexes of brain MR imaging were measured including the height of the pituitary gland, distances of the suprasellar cistern and prepontine cistern, distance from optic chiasm to pituitary fossa, mamillopontine distance, pontomesencephalic angle, and angle of transverse sinus. The qualitative signs of brain MR imaging were evaluated including pachymeningeal enhancement, subdural effusion or hematoma, effaced suprasellar cistern and prepontine cistern, decreased mamillopontine distance and pontomesencephalic angle, and transverse sinus distention.Results:In 27 SIH patients with vestibulocochlear nerve symptoms, the mean CSF pressure was (37.50±27.54) mmH 2O, and 22 patients (91.7%) presented with orthostatic headache. In 45 SIH patients without vestibulocochlear nerve symptoms, the mean CSF pressure was (39.00±26.91) mmH 2O, and 39 patients (95.1%) presented with orthostatic headache. As compared with SIH patients without vestibulocochlear nerve symptoms, the patients with vestibulocochlear nerve symptoms showed significantly lower height of the pituitary gland ( P<0.05). The positive rate of effaced suprasellar cistern in SIH patients with vestibulocochlear nerve symptoms was significantly decreased as compared with those without vestibulocochlear nerve symptoms ( P<0.05); there were no significant differences in other quantitative indexes and positive rates of qualitative signs between the two groups ( P>0.05). Conclusion:The decrease of pituitary height and effaced suprasellar cistern may be closely related to the pathogenesis of vestibulocochlear nerve symptoms in SIH patients.

Objective:To investigate the characteristics of magnetic resonance myelography (MRM) and its application in the treatment of spontaneous intracranial hypotension (SIH).Methods:The clinical data, MRM characteristics, and treatment of 15 patients with SIH who underwent MRM examination in the First Affiliated Hospital of Zhengzhou University from August 2014 to August 2019 were retrospectively analyzed. According to treatment methods, nine patients were divided into conservative treatment group and six patients were divided into combined epidural blood patch treatment group. The gender, age, time interval from onset to MRM examination, cerebrospinal fluid pressure and MRM characteristics between the two groups were compared. SPSS 20.0 software was used for statistical description, and independent sample t-test was applied to compare the differences between groups. Results:All of the 15 cases reported orthostatic headache. Their cerebrospinal fluid pressure was (29.67±19.77, range 0-55) mmH 2O (1 mmH 2O=0.009 8 kPa), and onset-MRM interval was (33.07±24.22, range 7-90) days. The MRM characteristics were observed, including all 15 cases with periradicular leaks, four cases with anterior epidural fluid collections, six cases with posterior epidural fluid collections, and eight cases with high cervical (C 1-2 to C 2-3) retrospinal cerebrospinal fluid collections. There were 2 to 32 leak sites with an average of (10.20±7.87) sites. Among the 153 leak sites, 58(37.9%) sites were located at cervical vertebra, 77(50.3%) sites at thoracic vertebra, 18(11.8%) sites at lumbar vertebra, and 61(39.9%) sites at either the cervicothoracic junction (C 7-T 1 to T 1-2) or the upper thoracic region (T 2-3to T 6-7). Five patients responded well to one-time targeted autologous epidural blood patch on the basis of the location of the cerebrospinal fluid leakage. Besides, one patient improved with targeted epidural blood patch twice. There were no statistically significant differences in gender, age, onset-MRM interval, cerebrospinal fluid pressure, number and location of leak sites between the conservative treatment group and combined treatment group. Conclusions:The periradicular leaks of cerebrospinal fluid at cervical vertebra and thoracic vertebra are the most common feature of MRM in patients with SIH. MRM can identify the existence and location of cerebrospinal fluid leakage, assist in the diagnosis of SIH, and guide targeted epidural blood patch.

Objective The purpose of this study was to evaluate whether whole-tumor histogram-based analysis of readout-segmented echo-planar diffusion-weighted imaging (RESOLVE-DWI) ADC map can help in the discrimination of parotid gland tumors(pleomorphic adenoma, Warthin tumor, malignant parotid gland tumor). Methods The MR images(pre / post-contrast enhanced MRI, RESOLVE-DWI, ADC map) of 47 patients with a biopsy-or surgery-proven pleomorphic adenomas, 25 patients with Warthin tumors and 36 patients with malignant parotid gland tumors were retrospectively analyzed. Histogram-based analysis was performed with the software MaZda. ROIs were drawn on every section of the ADC map containing the tumor, then 12 Parameters(Area, MinNorm, MaxNorm, Mean,Variance, Skewness, Kurtosis, Perc.1％, Perc.10％, Perc.50％, Perc.90％, Perc.99％) derived from histogram were calculated. Statistical analysis among the three groups (One-way ANOVA or Kruskal-Wallis test) were performed to find out the statistical significance of each histogram parameter. Then LSD test or Mann-Whitney U test was used for pairwise comparison between groups. And the differential efficiency of each parameter was determined using ROC analysis. Results Overall, 9 parameters (MinNorm, MaxNorm, Mean, Variance, Skewness, Perc. 10％, Perc. 50％, Perc. 90％, Perc. 99％) among three groups were shown to be statistically significant (P<0.05). Between the pleomorphic adenomas and Warthin tumors, these 9 parameters were of statistical significance. Perc.50％revealed the highest diagnostic efficiency, followed by Mean and Perc.10％. Between the pleomorphic adenomas and malignant parotid gland tumors, also all these 9 parameters were of statistical significance. Mean was revealed to have the highest diagnostic efficiency, followed by Perc. 10％ and Perc. 90％. However, between the Warthin tumors and malignant parotid gland tumors, only 5 parameters (MinNorm, Mean, Skewness, Perc. 10％, Perc. 50％) were statistically significant. MinNorm was revealed to have the highest diagnostic efficiency, followed by Perc. 50％ and Perc. 10％. Generally, Mean, Perc. 10％ and Perc. 50％ were more effective in the differential diagnosis of these three types common parotid neoplasms. Conclusion Whole-tumor histogram analysis of ADC maps are effective in differentiating common parotid neoplasms.

Objective To discuss the diagnosis and treatment of unilateral ectopic ureter in children.Methods The clinical data of 41 cases of ureteral ectopic children admitted to our hospital from January 2014 to June 2018 were retrospectively analyzed including the clinical features,diagnosis,surgical treatments.There were 4 males and 37 females patients,aged from 0.4 to 12.7 years,with an average of 3.5 years old.Urinary incontinence was the main manifestation in 35 cases,including 14 cases with urinary tract infection.Preoperative ultrasonography and IVP examination were performed in all the 41 children.The dynamic radionuclide renal imaging was performed in the children who showed no renal inhencement with IVP.There were 27 cases of ipsilateral renal duplication and 9 cases of ipsilateral renal dysplasia.Ectopic fusion of kidney with ipsilateral ectopic ureter has one case on each side,and bilateral renal duplication with ectopic fusion of kidney with left ureteral ectopic in 1 case.The ipsilateral kidneys were normal in 2 cases.There were 27 cases with renal duplication,24 cases with upper renal dysplasia due to upper heminephrectomy,3 cases with well upper renal function,2 cases with lower superior ureteral pelvis anastomosis,and 1 case with ureterocystostomy.Laparoscopic dysplasia nephrectomy was performed on 9 patients with renal dysplasia,and nephrectomy was performed on 3 patients with renal dysplasia with ectopic renal fusion.Ureterocystostomy was performed in 2 cases with normal kidney.Results All 41 patients were followed up for 4-57 months,with an average of 25.3 months.Among the 35 children with urinary incontinence before the operation,33 cases had complete disappearance of urinary incontinence symptoms,and 2 cases had urgent urinary incontinence after the operation,presenting as frequent and small amount of urine discharge,with a strong sense of urination urgency.The micturition interval was shortened,ranging from 30 to 40min in the daytime,and 2 to 3 hours at night.The parents of the children were required to remind them to micturate regularly.Of the 41 cases,3 developed urinary tract infection 6-10 months after operation,and cured by antibiotics without recurrence.Conclusions Ectopic ureter is relatively rare,but urinary incontinence is the most common clinical manifestation.Ultrasound examination could be the preferred examination method.IVP further identified the patients with ectopic ureter who had kidney combined with malformation and renal function.The surgical treatments are mainly based on the corresponding renal function,and the prognosis is good.

Objective To investigate the timing and scheme of surgical treatment for the concomitant ureteropelvic junction obstruction(UPJO) and congenital abnormalities of the kidney.Methods The clinical data of 155 patients with concomitant UPJO and congenital abnormalities of the kidney from January 2006 to January 2016 was retrospectively analyzed.There were 107 males and 48 females,who aged 6 months to 16 years and 6 months.The average time was 5 years and 9 months old when they received operation.There were 8 cases less than 1 year old.There were 93 cases of UPJO on the left side,54 cases on the right side,and 8 cases on both sides.There were 33 cases with duplication of kidney,19 cases with solitary kidney,and 6 cases with renal dysplasia,6 cases with renal ectopia,12 cases with polycystic kidney disease,and 41 cases with dysplasia;2 cases with renal malrotation.There were 100 cases with symptoms such as fever,abdominal pain,vomiting.5 cases had received Anderson-Hynes pyeloplasty in other hospitals,2 cases received nephrectomy with symptoms did not relieve.4 cases were treated with nephrostomy in other hospital.Children with the repeated clinical symptoms,or renal function decreased significantly,or hydronephrosis progressive to the anteroposterior diameter of more than 30 mm received surgical treatment.Results There were 140 cases received Anderson-Hynes pyeloplasty,and 8 cases received nephrectomy with 5 cases were UPJO side and 3 cases were only abnormalities of the kidney without UPJO.All patients received IVP or ultrasonography postoperative 3-6 months,which showed hydronephrosis improved or no obvious change,and 4 cases were improved obviously.The IVP results showed that 5 patients with renal dysplasia together with UPJO had the renal function improved.There were 128 cases followed up for 12 to 106 months,with an average of 64.5 months.All patients had no clinical symptoms.83 cases were reexamined by IVP or ultrasonography,and hydronephrosis was getting better or no change.Conclusions The patients with concomitant UPJO and congenital abnormalities of the kidney don't need surgery in advance.The best choice for those patients is Anderson-Hynes pyeloplasty.The indication of nephrectomy should be considered carefully.

Objective To determine the efficacy and long-term outcome of pyeloureteroplasty in the ureteropelvic junction obstruction (UPJO) patients with poor kidney function(＜ 10％).Methods The data of UPJO patients with poor kidney function treated from January 2006 to September 2016,was retrospectively analyzed.The renal function ＜ 10％ undergoing pyeloureteroplasty were included.Ipsilateral vesicoureteric reflux,ureterovesical junction obstruction and renal dysplasia were excluded.39 patients was enrolled.There were 31 boys and 8 girls.The mean age at surgery was 3.1 years old (range 7 months-14 years).There were 36 primary UPJO and 3 extrinsic vessel cases.Twenty-seven cases of UPJO in left side,9 cases in right side,and 1 case in bilateral side.The preoperative examination included ultrasound,intravenous pyelography (IVU) and 99 mTc DTPA renography.Mean anteroposterior diameter of pelvis was (5.5 ± 2.4) cm before operation (range 3.4-7.4cm);IVU showed non visualized kidney or rim sign.The ill kidneys' mean renal function was (3.25 ± 2.78) ％ (range 0-9％).Results Open pyeloureteroplasty were performed in the 38 patients,additional nephrectomy was required in 1 because of repeated urinary infection,while the other patients had good prognosis,nephrostomy was kept until 2-3 days' successful clipping.Laparoscope pyeloplasty were performed in one patient with double-J.The patient had urinary infection post operation and was cured.The postoperative examination included ultrasound,intravenous pyelography and 99mTc DTPA renography were performed after 3-6 months.Mean anteroposterior diameter of pelvis was (3.2 ± 1.9) cm (range 2.1-4.5 cm);Intravenous urography showed visualized kidney;Mean renal function was (18.16 ± 13.17)％ (9％-27％).There was significant difference between preoperational and postoperational evaluation of renography and pelvis (P ＜ 0.05).There was no correlation between the changed renal function and age (P ＞ 0.05).The mean follow-up time was 10.2 months (range 6-25 months).Conclusions Pyeloureteroplasty could be safe and feasible for the UPJO patients with renal function less than 10％,and could improve the renal function.

Brain ; blood supply ; pathology ; surgery ; Diagnosis, Differential ; Humans ; Middle Aged ; Pituitary Neoplasms ; cerebrospinal fluid ; complications ; diagnosis ; Stroke ; cerebrospinal fluid ; complications ; diagnosis ; Tuberculosis ; cerebrospinal fluid ; complications ; diagnosis

Objective To investigate the experience of diagnosis and management of coexisting ureteropelvic junction obstruction (UPJO) and nonreflux megaureter (NRM).Methods The retrospective study of UPJO with NRM was based on 10 years information retrieved from January 2005 to December 2015.The data of 13 patients (8 males and 5 females) were available and recorded.Mean age at surgery was 3.7 years old (range 1.8 to 14 years).The diagnosis and mangement were summarized.Coexisting ureterovesical junction obstruction (UVJO) and vesicoureteral reflux,iatrogenic stricture and vesicoureteral reflux were excluded.Intravenous pyelography,voiding cystourethrography,ultrasound and CT reconstruction were performed before operation.Only six patients had an accurate diagnosis as UPJO with UVJO before surgery.Pyeloplasty was the initial surgical management choice for 10 patients,and ureteroneocystostomy in 3 patients.Results UVJO were diagnosed with pyelography techniques in 3 patients after pyeloplasty,while 4 were diagnosed as nonreflux and nonobstruction megaureter.Of the 10 patients who underwent initial pyeloplasty,additional ureteroneocystostomy was required in 3 and the prognosis was good.Additional pyeloplasty was required in 2 of the 3 patients who initially underwent ureteroneoeystostomy.Mean follow-up time from last operation was 23.3 months (6-53 months),the overall prognosis was good.Conclusions It is often difficult to correctly diagnose coexisting UPJO and NRM.In patients with UPJO,it is highly recommended nephrostomy radiography after pyeloplasty to evaluate the distal ureterovesical junction.Initial pyeloplasty is always recommended as first-line therapy.Additional ureteroneocystostomy was required when hydroureteropelvic was aggravated.