Biomedical subjects
Publications and source records attributed to G Lena.
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.
The association of hydrocephalus and intramedullary spinal-cord tumor with leptomeningeal spread is described in a 20 month-old boy. The pathogenesis of increased intracranial pressure in spinal cord tumor is discussed. Meningeal gliomatosis is rare and is more often observed in the follow-up of cerebral gliomas. It exceptionally occurs in patients without known cerebral or spinal tumor. Such a tumor often occurs in young patients and has a poor prognosis.
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 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.
Spontaneous cerebellar hematomas account for 10 p. 100 of all spontaneous intra-cranial hemorrhages. In this series of 44 cases, 3 clinico-pathological groups are described on the basis of clinical and pathological data (small, middle and massive hematomas). The mortality rate was high (50 p. 100). Nevertheless, early and direct draining of the hematomas provides the best chances of survival.
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.
Cerebral aneurysms at the bifurcation of the internal carotid artery are uncommon and represent less than 10% of intracranial aneurysms. A recent series of 10 cases (out of a total of 221 aneurysms over a period of five years, from 1983 to 1988, i.e. 4.5%) treated via a microsurgical approach is presented. Anatomical and operative aspects are discussed. These malformations are located at the point of union of the carotid artery, sylvian and lamina terminalis cisterns. They are close to perforating vessels which usually lie on their deep aspect. Surgery is performed via the standard pterional approach and, as Yasargil points out, two specific dissecting times are required for the opening of the sylvian and lamina terminalis cisterns, in order to avoid dangerous traction on the brain. In our experience, deep hypotension was used in the four cases of large malformations, and there was no need for a temporary dip. All aneurysms were clipped at the neck and the sac then punctured or removed. Post-operative evolution was completely asymptomatic in 9 cases (grade I-II--Hess and Hunt), one patient (grade IV) died from an apparent vascular spasm, confirmed by CT scan.
138 cervical spine fractures are reviewed during a recent period of 5 years (1979-1983). Their frequency stay high. The serial concern 67% of men and 33% of women. We observe a peak age between 20 and 30 (23%). 65% of lesions are caused by a traffic injury, 27% by a falling down, 10% by a diving. 45% of cases interest the upper cervical spine (C1-C2) with a high proportion of odontoid process fractures (60%) and Hangman's fractures (30%); 54% of cases concern the lower cervical spine (C3-C7) with an important part of fracture-luxation (72%), specially C5-C6 (35%). Clinically, we note almost a same part of fractures without neurological disturbances (54%) and with neurological abnormalities (46%). In this situation, the sensitive and motor loss are often severe (78%). In 40%, the injuries are polytraumatism and association spine lesion, cranio-cerebral lesion is the most frequent (61%). In upper cervical spine fractures, after Crutchfield or Gardner traction, posterior fixation was performed (62%). The treatment was conservative in 30%. In lower cervical spine lesions, a secondary surgical approach by an antero-lateral way was made (53%). The treatment was initially surgical in 13% and conservative in 28%. The mortality rate of this pathology is important (22%; i.e. 30 deaths on 138 cases).
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Our study is based on the analysis of traumatic I.C.H. 38 cases observed since we have had a C.T. Scan at our disposal (1975). Those cases (recorded in the departments of Paillas, Vigouroux, Sedan, Combalbert) represent 2,3% of the brain traumas, 6,5% of the traumatic intra-parenchymatous lesions and 9,5% of the traumatic intra-cranial hematomas. Most of the cases reported deal with male patients, with on average age of 46, who are often victims of traffic accidents. In half the cases of skull fractures, the hematoma is located in the opposite side of the fracture. In almost all cases, initial loss of consciousness is observed, followed by consciousness alterations, with few free intervals and with neurological focal signs depending on the site of the lesion. The C.T. Scan data of the those traumatic hematoma have been studied.
Therapeutic indications vary in patients with a malignant glioma of the brain and must be envisaged differently according to whether the patients is operable or not. These are the following possibilities according to previously defined criteria: