Biomedical subjects
Publications and source records attributed to L Genitori.
We report the clinical features and neurosurgical management of a 9-month-old girl with cranioectodermal dysplasia and scaphocephaly. The technique of surgical correction is discussed together with details of the pre-operative investigations. The importance of early referral to an experienced geneticist is emphasized.
Shunt infection remains the foremost problem of shunt implantation after mechanical malfunctions. Diversionary cerebrospinal fluid shunt implantation has a high complication rate, with 5% to 15% of such shunts becoming infected. Of these infections, 70% are diagnosed within 1 month after surgery and more than 90% within 6 months. Shunt infection in the vast majority of cases is therefore a complication of shunt surgery. The authors review their experience with shunt implantation during two time periods. From January, 1978, to December, 1982, 302 children with hydrocephalus underwent 606 operations. Among these children, 47 (15.56%) developed a proven shunt infection, with an incidence of infection per procedure of 7.75%. As a result of this study, a new protocol for shunt procedures involving modifications in the immediate pre-, intra-, and postoperative management of children undergoing shunt implantation was initiated. With this new protocol, 600 children underwent a total of 1197 procedures between January, 1983, and December, 1990. The incidence of shunt infection decreased dramatically, with two infections (0.33%) in 600 patients and a per-procedure rate of 0.17%. The overall annual risk of a shunt infection in the pediatric neurosurgical unit is currently 1.04%.
The authors review their experience of 14 children with syringomyelia and associated Chiari I malformation observed during the last 21 years. Initial symptoms were motor weakness (57%), sensory signs (50%), scoliosis (50%) and bladder dysfunction (21.5%), while signs of admission were pyramidal signs (78.5%) with motor deficit (43%), spinal deformities (64.2%), syringomyelic syndrome (36%) and sensory deficit (21.5%). Four children were investigated with myelography, three with myelography and spinal CT Scan and the last seven cases with MRI. Ten children (71.4%) underwent a decompression of the Chiari malformation with or without drainage of the cyst and shunting of an associated hydrocephalus. Syringo-peritoneal shunts were used in 3 children and laminectomy with syringo-subarachnoid shunt in 1 case. The neurological symptoms improved in 57% of the cases, 2 patients deteriorated and 4 patients were lost to follow-up. The authors discuss the clinical symptomatology, the MRI study, the controversies concerning the treatment and concluded with their attitude concerning this pathology in children.
We have examined pathological criteria in 16 cases of infratentorial ependymomas of childhood using a conventional histological approach, with immunohistochemistry and silver nucleolar organizer region staining (AgNORs). We have found that some of these criteria are of prognostic value. The following histological features were evaluated in each case: cellular density, cellular or nuclear pleiomorphism, mitosis, focal necrosis, endothelial proliferation and complete loss of differentiation. The expression of the following antigens was also studied: epithelial membrane antigen (EMA), human natural killer (HNK1), glial fibrillary acidic protein (GFAP) and vimentin. Only three histological criteria have been retained as indicative of bad prognosis, i.e., high mitotic index, a large amount of necrosis and complete loss of differentiation. These criteria distinguish ependymomas from anaplastic ependymomas. GFAP was expressed in all tumors while other antigens were more variable. In addition tumors expressing large amounts of GFAP were statistically associated with a better prognosis. Increased vimentin expression associated with a decrease of GFAP immunoreactivity correlated with anaplasia and short survival. EMA was not directly correlated with postoperative survival but may be considered as a further prognostic factor. Finally AgNORs values were not statistically correlated with postoperative survival.
The various treatments currently available for medulloblastoma are reviewed and modifications which have occurred over the last ten years are analyzed. Surgery is the first step of the treatment of a medulloblastoma. Recent advances in neurosurgical anesthesia and intensive care have substantially improved the quality and extent of exeresis and markedly reduced operative mortality (currently under 2%). Radiotherapy of the entire central nervous system should follow surgery in every case. Use of high energy radiations (together with improvements in surgical treatment) have increased survival rates to 50-60%. However, side effects are frequent. The modalities and timing of chemotherapy have also changed considerably over the last ten years; the different modalities currently used are reviewed, as well as the strategies available for reducing toxic effects and hematologic complications. Currently, chemotherapy is "sandwiched" between surgery and radiotherapy. Treatments under evaluation (monoclonal antibodies, immunotoxins, and immunomodulation) are discussed. The purpose of this therapeutic strategy for medulloblastomas is not only to increase survival rates but, above all, to improve the quality of life in survivors. Close cooperation between health care professionals of the different specialties involved and long term multicenter studies are indispensable if this goal is to be achieved.
The case of a 14-year-old girl who had severe subarachnoid bleeding as the first manifestation of a benign astrocytic tumor of the lateral aspect of the third ventricle is reported. Tumors that cause bleeding are usually neuroectodermal tumors in children and malignant tumors in adults. A review of the literature serves as the basis for a discussion of the frequency of bleeding as the first manifestation of brain tumors (1 to 10%) and of the pathogenesis of these hemorragies for which three theories (vascular, mechanical, and metabolic) have been put forward.
24 cases of choroid plexus tumours (16 papillomas and 8 carcinomas) were observed in the Department of Paediatric Neurosurgery, Hôpital des Enfants de la Timone, Marseille France between 1975 and 1989. The case records were reviewed and the clinical, surgical, and pathological results are presented here. 14 (58%) were infants and 10 (42%) of these were aged less than 1 year at presentation. In 11 (46%) the tumour arose in the lateral ventricle, in 7 (29%) it was located in the IIId ventricle, and in 6 (25%) the tumour was found in the IVth ventricle. 5 of the 8 carcinomas had their origin in the IVth ventricle. 17 cases (75%) required ventriculo-peritoneal shunt procedures before surgery of the tumour and in all these it was left in place post-operatively. One child required shunting de novo after removal of the tumour. A total excision was achieved in 20 children (83%) out of 23 operated upon. The operative mortality (mortality within one month of surgery) was 8%, the overall mortality was 25%. One child with a IIId ventricle papilloma died before surgery from a large gastro-intestinal haemorrhage. 5 children under the age of 3 years with carcinomas on the IVth ventricle died. The 3 children with supratentorial carcinomas are alive respectively 2 months, 8 years, and 13 years after treatment. Among 15 children with papillomas alive, 10 (67%) are neurologically intact and 5 (33%) have sequelae.
A series of 61 cases of cerebral arteriovenous malformations(AVM) was observed in children under 15 years of age during the last 18 years; 7 concern infants under 12 months and more than 50% are children between 10 and 15 years old. Intra-cerebral hemorrhage was revealing in 45 cases (7.7%) and 12 patients were in comatose state at presentation; 8 children (1.1%) only presented epilepsy alone; 53 cases (86.8%) were operated upon and only one has had a partial removal; 2 children were cured by stereotactic radiosurgery; one child must be embolize and 5 children were not treated; 50 cases (82%) have a good or an excellent result with normal life; 7 cases (11.5%) have sequellae and only 4 children (6.5%) died.
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The clinical and operative findings of 40 infants treated for Extradural Haematomas (EDH) between 1960 and 1988 are presented. This series represents 19% of the total number of children with EDH during this period. Twenty-five (63%) were male, fifteen (37%) female. They were divided into three groups according to age for comparison. Group A, less than 6 months (11 cases); group B, 7-12 months (16 cases); and group C, 13-24 months (13 cases). Sixteen (40%) resulted from falls less than 1 m. Seven (17.5%) fell whilst walking. Twelve (30%) fell more than 1 m. Two EDH followed obstetric trauma, three occurred as a result of a road traffic accident. A lucid interval was identified in 30 cases, and in 15 it was longer than 24 h. Drowsiness (60%), and delayed vomiting (45%), were the most important symptoms. Anaemia occurred in 19 (47.5%). Thirty-six (90%) had abnormal skull X-rays. Thirty (75%) EDH were parietal, temporal, or temporo-parietal. Two were located in the posterior fossa. There were no frontal EDH in this series in contrast to that found in older children. Twenty-seven (67.5%) EDH were larger than 75 cc in volume. The source of bleeding was identified in 31; in 17 (42.5%) it was from the middle meningeal artery; in 11 (27.5%) from the bone; and in three (7.5%) from the dural surface. The mortality was 12.5% with a 15% morbidity rate, three infants (7.5%), suffering motor deficits, and three requiring medical treatment for epilepsy.
The authors present aspects of the skull base in trigonocephalic children. Different patterns on clinical anthropometric investigation, bidimensional computer tomography (CT) scan and three-dimensional (3D) CT scan were studied. We present a series of 27 cases of trigonocephaly operated on in the department of pediatric neurosurgery at the Hôpital des enfants La Timone in Marseille since 1975. The skull base has been studied with CT scan in 12 patients and in 5 a 3D CT reconstruction was performed. A control series of 27 children without synostosis has been studied with the same patterns. We have analyzed in 3D, the volume of the anterior fossa related to the orbital volume, the permeability of basal sutures and the spatial orientation of petrous, sphenoid and zygomatic bones. An analysis of nasoethmoidal complex was performed in relation with hypotelorism. CT scan allows the study of the opening of orbital, nasion-pterional angles and the clinopterional angles, as well as nasion-clinoidal and bipterional distances. A computerized analysis of these data compared with normal skull base permits a new approach to these malformations and the necessity for subdivision of trigonocephaly.
Brain tumors are the most frequent childhood tumors. There have been few cytogenetic studies published on these tumors in children compared to the numerous studies on adult brain tumors. We examined chromosomes from 45 primary pediatric brain neoplasms including 14 medulloblastomas, 12 astrocytomas, 4 glioblastomas, 7 ependymomas, 5 craniopharyngiomas, 2 meningiomas, and 1 ganglioglioma. Chromosomal abnormalities were found in 10 medulloblastomas out of the 14 analyzed. The most frequently observed abnormalities were the total or partial loss of one chromosome 17: monosomy 17, i(17q), and a monosomy 22 in 4 cases of desmoplastic medulloblastoma. In glioblastoma, we observed the gain of chromosome 7, chromosome 3, a monosomy 10, and hyperdiploidy. The loss of chromosome X was observed in 2 cases of ependymoma as was a monosomy 22. Our observations show that from the cytogenetic point of view childhood brain tumors differ from adult brain tumors.