0.05).Conclusion Febrile seizures seldomly cause severe neurological damage.

OBJECTIVETo investigate the effect and safety of imaging-guided percutaneous catheterization drainage and alcohol sclerosis for treatment of renal cysts.

METHODSThirty-six patients with primary renal cysts, including 22 men and 14 women aged 18-65 years (mean 42.5 years), were treated with imaging-guided percutaneous puncture catheterization drainage and alcohol sclerosis treatment. The location of the renal cysts and puncture route, angle and depth were determined by ultrasound or CT scan. Paracentesis and catheterization external drainage were carried out under fluoroscope. Absolute alcohol was used as the sclerosis agent.

RESULTSThirty-eight cysts were detected in the 36 patients, locating at the upper pole (n=21), subtus pole (n=10) and intermediate pole (n=7). The length of renal cysts was 4.5-8.5 cm (mean 5.5 cm). Puncture was performed through the lumbar back and the success rate was 100%. Thirty-eight multi-lateral holes 5-7F drainage catheters were placed in the 38 cysts. Alcohol was injected for 169 times through the drainage tube and the average volume was 25 ml, with an average injection of 4.45 times. During the follow-up for 1 to 6 years (mean 3.5 years), 37 renal cysts disappeared and 1 cyst was reduced in a patient with polycystic kidney. The total cure rate was 97% in this series, and no serious complications occurred after the operation.

CONCLUSIONImaging-guided percutaneous puncture catheterization drainage and alcohol sclerosis is effective and safe for treatment of renal cysts.

Adolescent ; Adult ; Aged ; Catheterization ; Drainage ; methods ; Ethanol ; administration & dosage ; Female ; Humans ; Kidney Diseases, Cystic ; diagnostic imaging ; therapy ; Male ; Middle Aged ; Sclerosing Solutions ; administration & dosage ; therapeutic use ; Sclerotherapy ; methods ; Ultrasonography ; Young Adult

OBJECTIVETo study the clinicopathological, immunohistochemical and electron microscopic characteristics of pediatric rhabdomyosarcomas (RMS).

METHODSOne hundred and forty-five cases of pediatric rhabdomyosarcomas were studied by routine histological, immunohistochemical and electron microscopic studies.

RESULTSThere were 97 male and 48 female patients with ages ranging from 4 months to 13 years and a mean of 4.2 years. The follow-up period of 100 patients was from 1 year to 20 years with a mean of 5 years after diagnosis. All cases were subtyped into the following histological categories: embryonal RMS, botryoid RMS, spindle cell RMS, alveolar RMS and solid RMS. Histopathological subtypes, tumor site and tumor stage correlated significantly with the patients' 5 years survival. The best prognosis was observed in spindle cell and botryoid RMS. Embryonal RMS carried an intermediate prognosis. Patients with alveolar RMS and solid RMS had the worst prognosis. Tumors involving bladder, head and neck carried a favorable clinical outcome. Patients with tumors involving trunk extremities retroperitoneum and pelvis did poorly. Immunohistochemically, all cases were positive for Vimentin. The positive staining rates for desmin, SMA and myoglobin were 78%, 75% and 37%, respectively. All tumors were negative for NSE, CD99 and LCA. Electron microscopy study showed features of myofilament and sarcomere in 10 of 15 cases.

CONCLUSIONSRMS is the most common soft tissue sarcoma of childhood. Immunohistochemistry and electron microscopy are helpful in diagnosis and classification of RMS.

Adolescent ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Head and Neck Neoplasms ; pathology ; ultrastructure ; Humans ; Immunohistochemistry ; Infant ; Male ; Retrospective Studies ; Rhabdomyosarcoma ; classification ; pathology ; ultrastructure ; Soft Tissue Neoplasms ; classification ; pathology ; ultrastructure ; Urogenital Neoplasms ; metabolism ; pathology ; ultrastructure

Objective To establish an easy culture method of successively getting high purity and yield of microglia. Methods Cortices of neonatal Wistar rats (1-3 days old) were employed in this experiment. The first-generation microglial cells were isolated from the mixed glial culture by mechanical means (gently shaking and blowing with pipette). After the mixed glial cells being passaged at a density third generations ofmicroglial cells were harvested. CD1 lb/c, CD45, CD80, CD86 and GFAP were employed as the identification markers in detecting the phenotypes and purity of different generation of microglial cells by scanning electron microscope and flow cytometry. Immunofluorescence staining and CCk8 vitality measurement were used to judge the expression of CD11b/c and detect the proliferation of microglia cells. Microglial phagocytotic function was evaluated by phagocytosis of fluorescent microspheres. Results High yield and purity of microglial cells were stably obtained in this experiment. CD11b/c, CD45, CD80 and CD86 positive expressions were noted in the first and third generations of microglial cells by flow cytometry; CD1 1b/c positive expression was noted in the first,second and third generations of microglial cells by immunofluorescence staining. No obvious differences in the 3 different generations of microglia cells were found on proliferation ability by CCk8 vitality measurement, and on morphology and phenotypes by scanning electron microscope; no obvious differences in the first and third generations of microglia cells were found on phagocytic ability (P＞0.05).Conclusion High yield and purity of microglial cells can successively obtain through the above method;no significant differences are noted among different generations of microglia cells on purity, morphology,phenotypes, proliferation activity and phagocytic ability.

BACKGROUNDCongenital quadricuspid aortic valve is rarely seen during aortic valve replacement (AVR). The diagnosis and treatment of the disease were reported in 11 cases.

METHODSEleven patients (nine men and two women, mean age 33.4 years) with quadricuspid aortic valve were retrospectively evaluated. Medical records, echocardiograms and surgical treatment were reviewed.

RESULTSIn accordance with the Hurwitz and Roberts classification, the patients were classified as type A (n = 2), type B (n = 7), type F (n = 1) and type G (n = 1). Three patients were associated with other heart diseases, including infective endocarditis and mitral prolaps, left superior vena cava, aortic aneurysm. All had aortic regurgitation (AR) except two with aortic stenosis (AS), detected by color-flow Doppler echocardiography. The congenital quadricuspid aortic valve deformity in seven patients was diagnosed by echocardiography. All patients underwent successful aortic valve replacement.

CONCLUSIONQuadricuspid aortic valve is a rare cause of aortic insufficiency, while echocardiography plays an important role in diagnosing the disease. Aortic valve replacement is the major therapy for the disease.

Adolescent ; Adult ; Aortic Valve ; abnormalities ; Aortic Valve Insufficiency ; diagnosis ; surgery ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Young Adult

Objective:To explore the genetic etiology and the value of early diagnosis of early onset epileptic encephalopathy (EOEE) with unknown etiology.Methods:A total of 60 children with EOEE of unknown etiology were prospectively enrolled in the outpatient and inpatient departments of Fujian Provincial Hospital from January 2018 to January 2021.Peripheral blood was collected prospectively for whole-exome sequencing and copy number variation (CNV) detection to analyze the clinical characteristics and genetic sequencing results of the children.Results:Twenty-four patients with EOEE-related pathogenic or suspected pathogenic mutations were detected, including infantile spasms (10 cases), Dravet syndrome (3 cases), pyridoxine-dependent epilepsy (1 case) and ohtahara syndrome (1 case), and unknown epileptic encephalopathy (9 cases). The onset age of EOEE-related patients ranged from 1 day to 11 months (median age was 4.2 months), the treatment age ranged from 2 days to 4 years (median age was 10 months), and the age of diagnosis was controlled within 1 month after treatment.There were 20 cases (33.3%) single gene variants and 4 cases (6.7%) CNV variants.A total of 13 genes were involved: KCNQ2, SCN1A, SCN8A, CACNA1E, CDKL5, PPP3CA, PCDH19, TSC1, TSC2, ZEB2, ALDH7A1, DCX and HNRNPU.The 4 CNV abnormalities were 17p13.3 deletion, 11q23.3q25 deletion, 1q36.31-p36.33 deletion, 1q43-1q44 deletion and Xp22.33 duplication, respectively.Totally, 20 mutations were new loci reported for the first time at home and abroad; 11q23.3q25 deletion that resulted in infantile spasm was first reported at home and abroad.Infantile spasm caused by ZEB2 mutation and epileptic encephalopathy caused by PPP3CA gene were both reported for the first time in China. Conclusions:Gene and CNV are important potential causes of children suffering from EOEE.When the etiology is unclear, the combination of whole-exome sequencing and CNV sequencing technology can improve the diagnosis level of genetic etiology of children with EOEE.The early genetic detection of these children can early diagnose and accurately treat epilepsy.

OBJECTIVETo investigate the effectiveness of surgical approaches, outcomes and prognosis of aortic root pathology due to Stanford A aortic dissection.

METHODSRetrospective analysis the clinical data of 161 patients (122 male and 39 female, mean age of (44 ± 21) years) underwent surgical treatment for Stanford A aortic dissection between January 2001 and June 2011. There were 146 patients of acute aortic dissection and 15 patients of chronic aortic dissection. All the patients had aortic root pathologies that included commissural prolapsed in 140 cases, more than moderate aortic insufficiency in 75 cases, aortic sinus intima rupture in 15 cases, right and/or left coronary artery tearing in 8 cases, right and/or left coronary artery dissection in 16 cases, aortic root aneurysm in 31 cases.

RESULTSAortic root replacement (Bentall procedures) were used in 72 cases, aortic root remodeling (including aortic valve replacement) in 80 cases, aortic root reimplantation (David procedure) in 9 cases. The cardiopulmonary bypass time was shorter in aortic root remodeling group ((193 ± 42) minutes) than the other two groups ((210 ± 61) minutes, (197 ± 34) minutes, F = 3.22, P = 0.04). The in-hospital mortality was 8.1% (13 cases), 5 cases (6.9%) in aortic root replacement group, 7 cases (8.8%) in aortic root remodeling group, 1 case in aortic root reimplantation. The cause of death included respiratory failure (4 cases), permanent neurological deficits (3 cases), multiple organ failure (4 cases), acute renal failure (2 cases). The survivors were followed up for 6 months to 6 years. There was no patient required reoperation for aortic root pathologies. There was no statistically significant difference between aortic root remodeling group and reimplantation group (P > 0.05).

CONCLUSIONSThe surgical treatment for aortic root pathology due to Stanford A aortic dissection is challenging. Appropriate procedures according to the specialty of aortic root pathology can be performed with favorable functional results.

Adolescent ; Adult ; Aged ; Aneurysm, Dissecting ; surgery ; Aorta ; pathology ; surgery ; Aortic Aneurysm ; surgery ; Aortic Valve ; surgery ; Blood Vessel Prosthesis Implantation ; methods ; Female ; Heart Valve Prosthesis Implantation ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Treatment Outcome ; Young Adult

OBJECTIVETo evaluate the effect of axial rotation of lumbar vertebrae on the accuracy of pedicle screw placement using the traditional method, as well as to assess the value of intraoperative three-dimensional (3D) navigation in improving the accuracy.

METHODSSixteen lumbar simulation models at different degrees of axial rotation (0°, 5°, 10° and 20°), with every four assigned with the same degree, were equally divided into two groups (traditional method group and intraoperative 3D navigation group). Random placement of pedicle screws was carried out, followed by CT scan postoperatively. Then the outer pedicle cortex contours were depicted from reconstructed sectional pedicle images using Photoshop. The accuracy of pedicle screw placement was evaluated by determining the interrelationship between screw trajectory and pedicle cortex (qualitative) and measuring the shortest distance from pedicle screw axis to outer cortex of the pedicle (quantitative).

RESULTSEighty pedicle screws were implanted respectively in each group. In traditional method group, statistical difference existed in the accuracy of pedicle screw placement at different axial rotational degrees (P < 0.05). With degrees increasing, the accuracy declined. The accuracy of intraoperative 3D navigation group was higher than traditional method group in vertebrae with axial rotation (P < 0.01). In qualitative evaluation, the accuracy of the two methods had statistical difference when the degree was 20°, and in quantitative evaluation, statistical difference existed in 5°, 10° and 20° of vertebral axial rotation.

CONCLUSIONSScrew malposition can be caused by vertebral axial rotation in lumbar spine using traditional method. Accuracy of pedicle screw placement declines with the increase of axial rotational degrees. However, the accuracy can be improved by using intraoperative 3D navigation.

Bone Screws ; Humans ; Imaging, Three-Dimensional ; Lumbar Vertebrae ; diagnostic imaging ; surgery ; Models, Anatomic ; Rotation ; Spinal Fusion ; methods ; Surgery, Computer-Assisted ; Tomography, X-Ray Computed

OBJECTIVETo analyze the clinical, imaging and pathological findings of congenital intrapulmonary lymphangioma and hemangioma in 5 infants and young children.

METHODData of 3 cases with congenital intrapulmonary lymphangioma and 2 cases with haemangioma were analyzed.

RESULTAll the 5 cases had cough, difficulty in breathing, cyanosis of lips, and shortness of breath. Imaging study indicated cystic and space occupying changes of partial lung. In the two cases of hemangioma, the blood vessels passing through the hemangioma were observed on CT films. Histochemical studies showed that the cystic pockets from removed tissues were different in size and there was a line of flat endothelial tissue around these pockets. Immunochemical studies indicated D2-40 positive, factor VIII weak positive for lymphangioma cases; while in hemangioma cases, factor VIII was positive, D2-40 was negative or weakly positive, and both of cytokeratin was negative.

CONCLUSIONIt is very important to consider and identify congenital intrapulmonary lymphangioma or hemangioma when a patient has cystic pockets and space occupying change in their lung by imaging and pathological studies.

Female ; Hemangioma ; congenital ; pathology ; Humans ; Infant ; Lung Neoplasms ; congenital ; pathology ; Lymphangioma ; congenital ; pathology ; Male

OBJECTIVEIntra-ventricular hemorrhage (IVH) is one of the most serious complications of preterm infants. Significant numbers of the surviving infants with severe IVH go on to develop post-hemorrhagic hydrocephalus (PHH). The management of PHH remains a very challenging problem for both neonatologists and pediatric neurosurgeons. This study aimed to evaluate the efficacy and safety of the use of Ommaya reservoirs and serial cerebrospinal fluid (CSF) drainage in the management of a series of neonates with PHH.

METHODBetween January 1, 2003 and December 30, 2005, 15 consecutive newborn infants with IVH grades III to IV, complicated with progressive ventricular dilatation, underwent placement of an Ommaya reservoir. CSF was intermittently aspirated percutaneously from the reservoir. The amount and frequency of CSF aspiration were based on the clinical presentation and the follow-up results of serial cranial ultrasonograms or CT scans. The changes of CSF cell counts and chemistries were also followed. Patients whose progressive ventricular dilatation persisted despite serial CSF aspiration through Ommaya reservoir eventually had ventriculo-peritoneal shunts (V-P shunt) placed. All the patients were followed up in the outpatient clinic after discharge from the hospital and the neurodevelopmental outcomes were evaluated through 18-36 months of age.

RESULTA total of 15 infants were included in this series. Of them, 11 were preterm infants who were at gestational ages of 29 to 34 weeks and 4 infants were full-term. All of the 4 full term infants presented with progressive ventricular dilatation after suffering from the intra-cranial hemorrhage (3 infants were due to vitamin K deficiency and 1 was due to birth trauma). Thirteen infants had grade III IVH, and 2 had grade IV IVH based on initial cranial ultrasonographic and CT scans. The mean age when IVH was diagnosed was (9 +/- 1) days in preterm infants and (22 +/- 7) days in full-term infants; the mean age when Ommaya reservoir was placed was (18 +/- 11) days in preterm infants and (31 +/- 7) days in full-term infants. All the infants tolerated the surgical procedure well. The Ommaya reservoir was tapped for an average of (21.5 +/- 4.6) times per patient. The mean CSF volume per tap was (10.2 +/- 1.3) ml/kg. The values of CSF protein, glucose and cell counts slowly reached normal levels at approximately 3 - 5 weeks after the placement of the reservoir. The velocity of head circumference increase per week was less than 1 cm in 13 patients in 1 - 4 weeks after the placement of the reservoir and the size of ventricles decreased gradually. By 12 - 18 months, 12 infants had normal size ventricles, and 1 patient still had mild ventricular dilation at 36 months. Two infants developed progressive hydrocephalus after serial CSF aspiration through Ommaya reservoir. One infant had a V-P shunt placed at 2 months of age and another infant died at 3 months of age at home after parents refused further therapy. Complications consisted of reservoir leaking and CSF infection at 16th day of placement in one patient after repeated tapping. By the end of 18 - 36 months of follow-up, 11 of 14 infants were considered normal, two patients had mild impairment in neurodevelopmental outcome (both had spastic bilateral lower limbs paresis, and one of whom also had amblyopia) and the other had seizure disorder.

CONCLUSIONThe results from this series indicate that the placement of an Ommaya reservoir is relatively safe in newborn infants and is useful in the initial management of neonates with PHH and may be beneficial in improving their neurodevelopmental outcomes. A multicenter randomized trial may be needed to further validate the results of this report.

Cerebral Hemorrhage ; complications ; therapy ; Cerebral Ventricles ; Drainage ; methods ; Female ; Humans ; Hydrocephalus ; etiology ; therapy ; Infant, Newborn ; Male ; Subdural Effusion ; etiology ; therapy