Tension pneumocephalus following suboccipital sitting craniotomy in the pediatric population.

BACKGROUND: Sitting craniotomy often results in entrapment of air in fluid-filled intracranial cavities. Gas under pressure exerts a deleterious effect on adjacent nervous tissue, resulting in clinical deterioration. AIM OF STUDY: To assess the incidence of tension pneumocephalus (TP) and to define risk factors associated therewith. MATERIAL AND METHOD: Analysis included 100 consecutive patients (57 boys, 43 girls, mean age 9.7 y) undergoing suboccipital sitting craniotomy since 2012 to 2014. RESULTS: In our material (n=100) TP was seen in 7 cases, asymptomatic pneumocephalus (AP) in 77 and no pneumocephalus (NP) in 16. Tumor types encountered were typical for pediatric population. In the TP group (n=7) the ratio of low-grade to high-grade tumors was 5:2, in the AP group (n=77) 2:1 and in the NP group (n=16) 1:1. Preoperative hydrocephalus was present in 21 cases (21%, mean incidence), thereof 3 in the TP group (3/7; 42.8%), 12 in AP group (12/77; 15.5%) and 6 in the NP group (6/16; 37.5%). All TP patients received an emergency external drainage, thereof 4 required a permanent ventriculo-peritoneal shunt (57.1%), while AP and NP patients combined (n=93) required a permanent shunt in 4 cases only (4.3%). TP-associated morbidity (n=2) consisted in a significant deterioration of neurological condition. CONCLUSIONS: TP is a relatively rare but potentially serious complication of suboccipital sitting craniotomy. Risk factors for TP are low-grade tumor and pre-existing long-standing hydrocephalus. TP requires emergency decompression by temporary external drainage. TP patients significantly more often require a permanent CSF shunt.

Prognostic value of proliferating cell nuclear antigen in Wilms' tumour in children.

AIMS: To evaluate the prognostic value of index Proliferating Cell Nuclear Antigen (PCNA) in Wilms' tumour in children. METHODS: The study comprised 64 children aged from 2 days to 13 years treated according to the SIOP (Society International of Oncology Paediatric) and accepted by the PPGGL (Polish Paediatric Group for the Treatment of Solid Tumours). The studies were conducted on tumour tissue removed during surgery, fixed in formalin and embedded in paraffin blocks. Sections (4 microns) were evaluated by immunohistochemistry, using the peroxidase method to determine the expression of PCNA in Wilms' tumour cells by primary monoclonal antibody NCL-PCNA from Novocastra. RESULTS: The percentage of immunopositive cells in particular fragments of the tumour ranged from 0--93%, mean 30.5%, median 25.5%. Mean and median values enabled division of children into two groups: Group A, where the percentage of cells staining with anti-PCNA was <30% and Group B, where this percentage was >30%. The expression of PCNA was evaluated in various stages of advancement, various histological types and depending on the course of disease. The studies revealed the correlation between index PCNA and stage of advancement P<0.01, index PCNA and histological type of Wilms' tumour P<0.025. Moreover we observed that deaths were found more frequently in tumours with index PCNA >30%, P<0.001. CONCLUSIONS: PCNA is a useful prognostic factor in Wilms' tumour in children.

Prognostic value of p53 expression in Wilms' tumor in children.

BACKGROUND: The aim of this report was to evaluate the prognostic value and clinical correlations of p53 expression in children with Wilms' tumor. MATERIAL AND METHODS: The study comprised 61 children aged from 2 days to 13 years (median 39 months), diagnosed and treated according to SIOP and PPGGL criteria in three centers co-operating with the PPGGL. The studies were conducted on tumor tissue removed during surgery, fixed in formalin and embedded in paraffin blocks. Then 4-micron sections were evaluated by immunohistochemistry, using the peroxidase method to determine the expression of p53 in Wilms' tumor cells by means of primary monoclonal antibody NCL-p53 from Novocastra. RESULTS: The percentage of immunopositive cells in particular fragments of the tumor ranged from 0% to 70% (mean 20.4%, median 16.0%). The mean and median values enabled the children to be divided into two groups: Group A, where the percentage of cells staining with anti-p53 antibody was >20% (23 cases), and Group B, where this percentage did not exceed 20%. The expression of p53 was then evaluated in various stages of advancement and various histological types, depending on the course of the disease. In Group A, tumors at higher stages of advancement stages were more frequent (p<0.05), and showed a higher degree of malignancy (p<0.06; EFS=56.53%). In Group B, lower stages of advancement were more frequent (p<0.05), the degree of malignancy was lower, and the EFS was 81.58%. A discrimination test, however, showed that the determination of p53 expression in Wilms' tumor cells has moderate sensitivity (58.825%), positive prediction (43.47%), and relatively high specificity (70.45%) and negative prediction (81.57%), which means that low indexes of p53 expression have higher prognostic value. CONCLUSIONS: The index of p53 expression is not an independent prognostic factor in Wilms' tumor in children, but this determination may be helpful in identifying high-risk and low-risk patients.

[Technical and diagnostic difficulties in stereotactic biopsy in children]. / Trudnosci techniczne i diagnostyczne podczas biopsji stereotaktycznych u dzieci.

The paper presents technical and diagnostic problems associated with stereotactic biopsies in children. Between the years 1992 and 2000, in the Dept. of Neurosurgery, Child's Health Center, we performed 132 stereotactic procedures, including 110 biopsies. In 15 cases bleeding developed from the biopsy site; in this group we performed 3 craniotomies for open haematoma evacuation, 1 child died (mesencephalic glioblastoma multiforme). The remaining 2 children recovered with no additional neurologic sequelae related to this complication. Since 1997 we use the Nashold needle to obtain tissue samples. This enables us to take larger and fewer samples with less risk of bleeding. The biopsy was non-diagnostic in 3 cases. In 1 case we observed a slight movement of the child's head in relation to the stereotactic frame when the patient was moved from the CT-scanner to the operating theater. In order to avoid such problems in the future and to facilitate transposition of heavy and paretic or unconscious patients, we devised a hard board with side handles. Methods designed to overcome these and other difficulties are described.

16q heterozygosity loss in Wilms' tumour in children and its clinical importance.

INTRODUCTION: The loss of heterozygosity (LOH) of 16q is a structural change detected in about 20-30% of Wilms' tumour cases. Aberrations which result in deletion of 16q are also found in breast cancer, prostate cancer and liver cancer, where they are connected with a worse prognosis. The hypothesis of a bad prognosis in nephroblastomas with LOH 16q was first formulated by scientists from NWTS (National Wilms Tumor Study) on the basis of 232 cases of Wilms' tumour. However, SIOP studies (International Society of Paediatric Oncology) which included 28 cases of Wilms' tumour, did not show any clinico-pathological correlations with LOH 16q. Therefore, we aimed to evaluate the importance of LOH 16q in relation to clinico-pathological factors in a group of children, treated according to the SIOP criteria. AIMS: The aim of this work was to evaluate the frequency of LOH 16q in sporadic unilateral Wilms' tumour and to study the relationship between LOH 16q and selected patho-clinical parameters. The study comprised 66 children (31 girls and 35 boys) aged from 2 days to 13 years. METHODS: LOH 16q was studied by the examination of polymorphism of marker sequences in the region 16q24. DNA was isolated from paraffin sections of tissue for routine microscopic examination by the microdissection method. The method of study involved the amplification of polymorphic sequences from the 16q24 region by polymerase chain reaction (PCR) and separation of the products of amplification by polyacrylamide gel electrophoresis. The results were the subject of statistical analysis in relation to gender, age of child at first diagnosis, stage of clinical advancement and histological type of tumour. The connection between LOH 16q and recurrences, metastases and death, and failure free survival and absolute survival of children followed-up for over 24 months after nephrectomy were studied. RESULTS: The study revealed a lack of correlation between LOH 16q and gender, however LOH 16q was more frequent in children with Wilms' tumour aged >24 months, P<0.05. Also, LOH 16q was more frequent in tumours classified as clinical stage (CS) II or III than in CS I, P<0.05, but there were no differences in the occurrence of LOH 16q in tumours classified as CS II and CS III. We have found no correlation between LOH 16q and the histological type of tumour. However, LOH 16q has been found three times as frequently in tumours from children who died than in tumours of children who survived, P<0.0024.

[Rasmussen syndrome--indications for surgical treatment of refractory epilepsy: report of two cases]. / Zespól rasmussena--wskazanie do operacyjnego leczenia lekoopornej padaczki. Opis dwóch przypadków.

AIM: Presentation of two cases of children with Rasmussen's encephalitis, treated by functional hemispherectomy. Two boys aged 4 and 6, with a typical clinical course, typical findings in CT and MRI scans and characteristic pathologic changes in brain specimens. In both cases was performed functional hemispherectomy. RESULTS: Immediate cessation of seizures in the immediate postoperative period (Engel class I and II). Later, in a 4-months' follow-up period, a worthwhile improvement in psychomotor development and social functioning was noted. Up-to date opinions published in available literature, related to pathogenesis and treatment modalities of Rasmussen's encephalitis are presented. CONCLUSIONS: 1) Rasmussen's encephalitis is a definite nosologic entity, leading to drug-resistant epilepsy and a progressive psychomotor deterioration; 2) functional hemispherectomy is a viable alternative, which should be considered in the treatment of Rasmussen's encephalitis; 3) Functional; hemispherectomy, in spite of it's aggressiveness, is a relatively safe procedure; 4) in the follow-up time of 4 months, the results are promising.

[Benign tectal tumors: clinical and neuroradiological correlations]. / Lagodne guzy pokrywy sródmózgowia w materiale wlasnym.

Benign tectal tumours in children constitute a distinct group of brainstem gliomas, characterised by a usually benign clinical course. The aim of this paper was a retrospective analysis of 1) results of conservative treatment, 2) diagnostic value of CT and MRI and 3) correlation of the MR image with the clinical course of the disease. Our material includes 15 patients aged from 6 to 16. The treatment consisted in the implantation of a CSF-shunting device (6 children), endoscopic ventriculostomy (6 children) or ventriculostomy in a child with malfunction of a previously implanted shunt (3 cases). Follow-up periods range from 3 to 219 months (mean 46.8 mo.). A slight progression of tumour in imaging studies was noted in 3 cases, while in the remaining patients neither clinical nor radiologic progression of the disease was observed. There was no correlation between tumour size and focal contrast enhancement in MRI and the natural course of the disease. The method of choice in the diagnosis of benign tectal tumours is MRI and in the treatment of associated hydrocephalus-endoscopic third ventriculostomy. An in-depth diagnostic work-up and a more aggressive cause-oriented treatment is used only in cases of a documented clinical and radiological progression.

[Callosotomy in the treatment of "catastrophic" drug-resistant epilepsy in children]. / Kallosotomia w leczeniu ciezkich postaci lekoopornej padaczki u dzieci.

UNLABELLED: The aim of this paper was to assess the preliminary results of callosotomy in children with "catastrophic" drug-resistant epilepsy. During the last 5 years, we performed callosotomy in 8 cases. Indications were: "catastrophic" drug-resistant epilepsy with frequent seizures, progressive psychomotor and intellectual deterioration, generalised structural and functional abnormalities in neuroimaging and neurophysiological studies. These children were of the mean age of 6 years, with the mean duration of illness 58 months. In the mean follow-up time of 27 months the following results were obtained: Engel class II--3 cases; class III--4 cases and class IV--1 case. Any intellectual improvement was noted in 2 cases only. CONCLUSIONS: (1) callosotomy effectively reduces the frequency and severity of seizures in children with drug-resistant epilepsy; (2) in spite of reduced seizures, in the majority of cases no significant psychomotor and/or intellectual improvement was achieved; (3) callosotomy did not produce any permanent morbidity.

Loss of heterozygosity of WT1 gene in the prognosis of sporadic Wilms' tumour in children.

The aim of this report was to evaluate the prognostic value of allele loss of the WT1 gene in children with sporadic Wilms' tumour. Allele loss of the WT1 gene was evaluated using microsatellite polymorphisms in the 3' untranslated region of WT1 in a radioactive PCR assay. The study comprised 66 children (30 girls and 36 boys), aged from 2 days to 13 years, treated for Wilms' tumour according to the SIOP-09 and PGGL scheme. We have used DNA isolated from the neoplastic versus normal kidney tissue from the paraffin embedded sections using microdissection procedure. Loss of heterozygosity (LOH) of the WT1 gene was found in 12 children (19.6%), 5 cases were non-informative. No significant correlation could be found between the LOH of WT1 gene and sex and age. Significantly more frequent occurrence of LOH in tumor in low stage of advancement and low degree of malignancy was found. However, no significant effect of LOH of WT1 gene was observed on frequency of recurrences, metastasis and deaths. Study of allele loss of the WT1 gene may be recommended in difficult cases as an additional factor useful for the diagnosis and in the assignment of the tumour to the appropriate risk group.

[The role of chemotherapy in treatment of nephroblastoma in children. Report of the Polish Pediatric Group re. treatments of solid tumors]. / Udzial chemioterapii w leczeniu nerczaka zarodkowego u dzieci. Raport Zespolu Polskiej Grupy Pediatrycznej ds. Leczenia Guzów Litych.

Retrospective analysis of chemotherapy results of children with nephroblastoma was performed in 220 patients aged from 1 yr to 14 yrs of live in 12 centers. Stage I nephroblastoma was documented in 24.5% but stage II--in 55.3%. Histologically 74.6% cases were diagnosed as medium malignant and 12.7%--high malignant. Therapy results were similar to observed in other centers.

[A case of gigantic orbital meningoencephalocele in a newborn]. / Przypadek olbrzymiej przepukliny oponowo-mózgowej w obrebie oczodolu u noworodka.

A case of a gigantic orbital meningoencephalocele in a neonate treated successfully by staged correction is presented. Correction of this congenital malformation enabled a more normal further development of brain and face, and also resulted in a much more acceptable cosmetic appearance. All this had a positive influence on psychomotor development of this child. An overview of pertinent literature is included.

[A case of Galen's vein aneurysm in an infant treated by combined transtorcular/transfemoral embolization]. / Przypadek tetniaka zyly Galena u niemowlecia, leczony embolizacja transtorkularna i transfemoralna.

We present a case of an aneurysm of Galen vein in an infant with concomitant large hydrocephalus, treated by ventriculoperitoneal shunting and combined transtorcular-transfemoral embolization with a good clinical outcome. The technical aspect of the procedure is described in detail; current concepts concerning the pathophysiology and data in the available literature are reviewed.

[Multiple pleomorphic xanthoastrocytoma tumors in a 12-year-old girl]. / Przypadek mnogich guzów xanthoastrocytoma pleomorphicum u 12-letniej dziewczynki.

This report presents a case of multiple bilateral hemispheric tumours (pleomorphic xanthoastrocytoma) in a practically asymptomatic 12-years-old girl. The tumours were removed radically in a staged procedure, with a favorable clinical outcome. Clinical history and neuropathologic findings are described. A review of pertinent literature is included.

[A case of Wilm's tumor with full symptomatic WAGR syndrome]. / Przypadek guza Wilmsa z pelnoobjawowym zespolem WAGR.

The authors of this paper presented a case of a baby with full-symptomatic WAGR syndrome (Wilms tumor, aniridia, genital tract malformation and mental retardation) treated in the I Department of Pediatrics, Institute of Pediatrics, Medical Academy Poznan. The etiology of this syndrome was discussed (deletion of the 13th band of the 11th chromosome short arm). The reason for treatment failure was analysed.

Can busulfan replace fractionated total body irradiation as conditioning regimen for allogeneic bone marrow transplantation in children with acute lymphoblastic leukemia.

The results of allogenic bone marrow transplantation (allo-BMT) in 26 children with ALL treated with the same initial- and relapse-BFM-protocols, but transplanted in different centers (Poznan, Wroclaw, Hannover) after conditioning with two different regimens have been compared. Ten children (6 in Poznan, 4 in Wroclaw) were conditioned for BMT with busulfan and cyclophosphamide when fractionated TBI (FTBI) was not available there. Sixteen children obtained FTBI and etoposide (11 in Hannover, where in children with ALL exclusively chemoradioconditioning regimen has been employed, and 5 in Poznan). It has been found, that the chemoconditioning procedure was probably less effective (5-year EFS 18%) than the regimen with FTBI (7-year EFS 60%). Therefore, TBI seems to be at this point still mandatory in pediatric ALL patients, unless combinations of chemotherapeutic drugs might be able to substitute the radiation in the future.

Multifocal central nervous system glioma--a case history.

A case history of the multifocal brain glioma in 13-year-old girl is reported. Numerous neoplasmatic foci were found using MRI within the vermis and cerebellar hemisphere and, later, also within the brain stem, cervical spinal cord and both brain hemispheres. Bioptical examination of the tumors revealed the structure of anaplastic astrocytoma with oligodendromatous component. The authors suggest that the foci may be considered as multiple metastases from the primary cerebellar astrocytoma and the neoplastic cells might have been transported within CNS through cerebrospinal fluid.