Intracranial hemorrhages in children with hemophilia: medical and surgical treatment.
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Adolescent↗
Biomedical subjects
D Renier
Publications and source records attributed to D Renier.
[Intracranial ependymoma in children. Prognosis and therapeutic perspectives].
The authors report 47 cases of intracranial ependymomas occurring in children less than 15 years of age, who were operated on from 1969 to 1979. The aim of the study was to study the incidence of intraspinal metastases and to suggest a logical protocol for postoperative radiotherapy. The operative mortality rate was 17%. The 5 year survival rate, after exclusion of postoperative mortality, was 51%. Recurrences developed in 41% of cases and metastases in 20%. Among these, intraspinal metastases occurred in 14.5% and were found only in infratentorial ependymomas, whether benign or malignant. The results and those in the literature suggest that a protocol of irradiation adapted to the site and the histological grading of these tumors should be discussed with the teams of radiotherapy; in infratentorial ependymomas, the irradiation should include the whole craniospinal axis.
Adolescent↗
Brain abscess in childhood. A study of 34 cases treated by puncture and antibiotics.
During the last 15 years, 34 children were treated for a brain abscess. 13 (38%) had cyanotic heart disease; 12 (35%) had an ENT infection. The standardized treatment protocol included puncture of the abscess and administration of antibiotic and antiedematous drugs. Postoperative permanent epidural monitoring of intracranial pressure and, in the last 5 years, repeated CT scans indicated if retapping the abscess or reinforcing the antiedematous treatment was necessary. In 7 out of these 34 cases the abscess had to be excised. Postoperative mortality rate was 6%. Overall mortality rate was 12%. Neurological sequelae were slight. Epilepsy frequency was 10%. Mental retardation (18%) was found only in cyanotic congenital heart disease to which it is related. Repeated CT scans demonstrated the progressive disappearance of the capsule within a few months. This study shows an improvement in the results when brain abscesses in children are punctured rather than excised.
Adolescent↗
[Early treatment of orbital asymmetry in plagiocephaly].
Plagiocephaly results from premature unilateral closure of the coronal suture with subsequent frontal and orbital asymmetry and inequality of ocular levels requiring complex osteotomies if treatment is delayed. From 1976, early treatment from the age of approximately 6 months has been instituted by means of bilateral frontal remodelling. Results in 25 neonates in whom the operation was performed demonstrated that not only was the frontal correction maintained with growth, but that principally the facial asymmetry was reduced and later orbital surgery was never necessary. More complicated problems were raised in 15 infants with plagiocephaly operated upon at a later stage.
Child↗
Effect of hypothalamic and pituitary irradiation on pubertal development in children with cranial tumors.
The purpose of the present study was to report on gonadotropin function and puberty of a large group of children treated by cranial irradiation for cranial and neck tumors and medulloblastoma. Forty-five children of pubertal age were investigated. The mean interval time since radiation was 5 2/12 yr. Gonadotropin and gonadal function were evaluated by clinical criteria, plasma sex steroids, and plasma LH and FSH responses to LRH. Puberty was complete or progressing normally in 31 cases and was abnormal in 14 cases. Severe gonadotropin deficiency, with lack of or slow progression of puberty and decreased LH and FSH responsiveness to LRH, was observed in 5 cases; 2 of these had moderately elevated plasma PRL levels. Secondary amenorrhea or lack of pubertal progression was found in 5 other cases. GH deficiency was associated with gonadotropin deficiency in 9 of these 10 cases. Adrenal function, estimated by basal dehydroisoepiandrosterone, dehydroisoepiandrosterone sulfate, and estrone, was normal according to pubic hair stages. In conclusion, complete or partial gonadotropin deficiency can be the consequence of cranial irradiation in children receiving 6000 rads or less. It is usually associated with GH deficiency. The site of the damage on the pituitary gland or the hypothalamus remains to be demonstrated.
Adolescent↗
Intracranial pressure in craniostenosis.
In this study, intracranial pressure (ICP) was recorded with an epidural sensor for periods of 12 to 24 hours in 92 cases of craniosynostosis. Pre- and postoperative recordings were performed in 23 patients, and 55 children underwent preoperative psychometric testing. The ICP was found to be normal in one-third of the cases, was obviously elevated in one-third, and was borderline in one-third. Waves of increased ICP were recorded during rapid eye movement (REM) sleep. After surgery, ICP decreased progressively and returned to normal in several weeks. A significant statistical relationship was found between the patients' ICP and their mental level: the higher the ICP the lower the mental level. The regression curve of ICP as a function of age shows that ICP is maximal at the age of 6 years and decreases later. The significance of these results is discussed. The authors recommend that ICP be recorded in cases of craniosynostosis since it is of some help in deciding whether patients should undergo surgery.
Adolescent↗
Children's cervical spine instability after posterior fossa surgery.
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Astrocytoma↗
[Intra-cranial abscesses and congenital heart disease in children (author's transl)].
The authors report on 13 cerebral abscesses developed in children with cyanogenic heart disease. The abscesses have been treated by one or several punctures and by a general antibiotherapy adjusted to the germ when it was isolated. C.T. Scan and epidural Intra-cranial pressure monitoring have defined the Therapeutic tactics. The mortality rate is 7.7%. 60% of the survivors lead a normal life; 40% are mentally retarded; however this mental retardation is not directly related to the abscess; it appears to be a consequence of the cyanogenic cardiopathy. The neurological sequelae are one epilepsy and two visual defects compatible with normal life. Successive C.T. Scans in the months following treatment have shown the progressive disappearance of the abscess membranes. The conclusions of this study are under antibiotherapy, it is better to tap abscesses than to remove them and that their prophylaxy is the suppression of the shunt responsible of the cyanosis.
Adolescent↗
[Cerebral abscesses in children treated by puncture. A 16 year-experience].
In the last 16 years, 33 children have been treated for brain abscesses. Fifteen (45.5%) presented with cyanogenic congenital heart disease; eleven (33.5%) with E.N.T. infection. The standardized treatment protocol included puncture of the abscess, antibiotherapy and anti-edematous drugs. Postoperative permanent epidural ICP monitoring and, in the last 6 years, repeated CT scans indicated when it was necessary to tap the abscess again or to reinforce the anti-edematous treatment. However, in 3 of these 33 cases, the abscess had to be removed. Postoperative mortality rate was 3%. Overall mortality rate was 6%. Neurological sequelae were slight. Epilepsy occurred in 10%. Mental retardation (16%) was only found in children with cyanogenic congenital heart diseases. Repeated CT scans showed the progressive disappearance, within a few months, of the abscess membranes. This study shows an improvement in the results when brain abscesses in children are punctured rather than excised.
Brain Abscess↗
[Congenital intraspinal neuroblastomas. A propos of 7 cases treated surgically].
Neuroblastomas appear to be one of the most frequent congenital solid tumors. Nevertheless, intraspinal congenital neuroblastomas are uncommon. The authors report seven cases of congenital intraspinal neuroblastomas operated on in the "Service de Neurochirurgie des Enfants-Malades" from 1970 to 1980. Their study and that of 16 cases published in the literature allows to stress several points (7, 12). The dumb-bell intraspinal neuroblastomas present a large extension. They usually determine a severe and definitive paraplegia. They probably have a long prenatal evolution. The tumoral prognosis of these congenital neuroblastomas is good. Success should be expected in nearly 80% of the cases. Surgery, in two steps, intra and extra spinal, remains compulsory even though the neurological prognosis is poor. It is useless to operate upon the neuroblastomas as emergencies, when neurological involvement is total at birth. The complementary treatment, X ray-therapy or chemotherapy is not without any danger on newborns. It necessary is not proved.
Female↗
Aneurysms of the vein of Galen in infants aged 2 to 15 months. Diagnosis and natural evolution.
This is a report of seven cases of aneurysm of the vein of Galen (AVG) with a review of the clinical and radiological aspects of 48 cases in the literature. The natural evolution and pathophysiology of this vascular malformation are discussed. The clinical signs of this condition are often misleading. The appearance of AVG on CT is pathognomic. Surveillance of the vascular malformation and associated cerebral lesions can be maintained with subsequent CT. Spontaneous thrombosis of the aneurysm was observed in three cases. The diagnosis of thrombosis of the AVG was always made at operation or by histological examination. The radiological appearance of a thrombosed aneurysm is typical.
Cerebral Angiography↗
Cranio-facial surgery for cranio-synostosis.
Cranio-facial principles have been applied to the treatment of craniosynostosis by the senior author since 1973, and original techniques have been developed for the treatment of oxycephaly, trigonocephaly, plagiocephaly brachycephaly and scaphocephaly; 156 patients have been operated upon. There is now enough follow-up material to establish that the good morphological results obtained initially are maintained through growth, and that there is a definite improvement in affected facial structures when early correction has been performed. The functional results have also been very satisfactory and reoperation for insufficient release has been rare. A multidisciplinary approach is highly recommended, and this type of surgery should be performed in a pediatric neuro-surgical environment.
Adolescent↗
[Instability and kyphosis following cervical laminectomy and occipital craniectomy in children (author's transl)].
The authors analyse the various factors of spinal instability and their respective importance in a series of 113 patients in which, either the occipital cerebellar pits or the cervical spine had been approached surgically. The essential cause was mechanical failure consecutive and related to the number of levels of laminectomies performed, even when the articular processes and their capsule were left intact. While craniectomy and ablation of the posterior arch of the Atlas was rarely, if ever, responsible for instability, the frequency rises to 25% once the Axis, the spinous processes and laminae have been removed. Instability was present in 80% of the cases in which three or more levels are involved. Moreover, cervical laminectomy was nearly always followed by instability, above all when the cervico-dorsal transitional zone was involved and even when only a small number of vertebral levels had been operated on. Location of the instability was always at the limit between the lowest level of laminectomy and the lover lying intact vertebra. Diagnosis has gone unrecognized until presently: the severity of the possible complications have led the authors to propose preventive surgical management. It this serie, an anterior vertebral bone graft was prefered. Consciousness of the risks involved should lead to better prevention, be it at the moment of neurosurgical surgery or in the follow-up period. However, preventive treatment creates several problems, which may only be solved by a currently undertaken prospective study.
Adolescent↗
[Meningioma of infants and children (author's transl)].
The authors report 11 meningiomas observed on infants and children. They point out four items: --In children the diagnosis of intraspinal meningiomas is impossible before surgery while diagnosis of intracranial meningiomas should be possible since in this case the neuroradiological investigations are most often characteristic. --The total removal of intraspinal meningiomas less than (en plaque) is not always possible. --In intracranial meningiomas detected on children less than five years-old, asymmetric macrocrania is very frequent. --Since infants' intracranial meningiomas are sometimes very large, it is likely that the tumor is congenital.
Adolescent↗
Hydrocephalus and achondroplasia. A study of 25 observations.
Two series of achondroplastic patients were studied. The first series included 5 patients referred to our neurosurgical department for macrocranium and/or mental retardation. The second series was composed of 20 patients arbitrarily chosen from a medical group. Macrocranium was observed in 60% of the patients and was related to hydrocephalus in all but 2 cases. Radiological studies demonstrated that the posterior fossa was deformed and narrowed in its 3 planes, while its volume was conserved because of an abnormal ascension of the tentorium. The conflict between normal brain development and the craniostenosis at the base of the skull is responsible for an upward tilt of the petrous pyramids, a lowering of the mastoid process, and the illusion of a basilar invagination. Ventriculographies, pneumoencephalographies and isotopic transits demonstrated normal CSF circulation. The study suggests that hydrocephalus is related to the stenosis of the sigmoid sinus at the level of narrowed jugular foramina with a resulting raise in intracranial venous pressure. The majority of patients with macrocranium stabilizes spontaneously. Thus, a surgical indication should be extremely limited.
Achondroplasia↗
[Neurological complications of hemophilia in children (author's transl)].
Although the treatment of hemophilia has greatly improved, the disease is still sometimes responsible for neurological or neurosurgical disorders. Such disorders were observed in the department of Neurosurgery at the "Hopital des ENFANTS MALADES' in twelve hemophiliacs, five of them having antifactor antibodies. Four main points results from this study: 1) A traumatic etiology was found only in one third of the cases. 2) CT Scan is the main investigation since it allows to find out what cases should be operated upon. The ratio of neurosurgical cases varies from one statistic to another from 40% to 70%. 3) The frequency of neurological disorders is correlated with the importance of the deficit in antihemophilic factors, which is usually less than 1%. 4) The death rate in this series was nil. Hemophiliacs without antifactor antibodies should receive a substitutive treatment as soon as possible. However, in hemophiliacs with antifactor antibodies, this treatment should only be given when surgery is required.
Brain Diseases↗
[Early treatment of facial-craniostenosis (Crouzon-Apert) (author's transl)].
The possibilities of treatment of the facial deformities related to Apert and Crouzon syndromes were transformed by the total facial advancement osteotomies described by Paul Tessier. These facial osteotomies are possible in children, and there is enough follow-up nom to assure that facial advancement osteotomies in children do not impair significantly the remaining growth possibilities. There are however severe psychological sequalaes in patients operated as children or youngsters, resulting from a deformed appearance during early childhood. We therefore try to give these children an acceptable appearance before entering school, around 3 or 4 years of age. Now in fact, we are trying not only to correct, but to prevent these deformities by early age release of the synostoses. The synostoses are usually involving the coronal suture and the cranial base. The cranial base being out of safe reach, we have started in 1976 to perform a 2 cm advancement of the forehead, including the supra-orbital rim. At first, we were using a lateral tenon-mortise fixation. We have tried the extended coronal craniectomy advocated by McCarthy and Coll., but since July 1977, we are using the original floating forehead principle: the advanced forehead is wired back only to the face, malar bones and root of the nose, leaving behind and extended craniectomy. Thus, the rapidly expanding brain of the infants is able to project anteriorly the forehead and upper part of the face. We have operated on 26 infants with faciocraniostenosis and severe brachycephaly according to these early release principle, the last 11 with the floating forehead approach. The results are very encouraging, the appearance being normal or subnormal after a few months. Even if an upper maxillary osteotomy is necessary later, we feel that an early improvement of these children is of high benefit to them. The improvement can also be functional, the psychological tests and the intracranial pressure measures showing that an early release is benefitial even in the absence of obvious intracranial hypertension.
Child, Preschool↗
Medulloblastoma in childhood. Survival and functional results.
The authors report on a series of 57 medulloblastomas in children operated upon -etween 1964 and 1976. Among these children, 44 completed the treatment with radiotherapy to the whole central nervous system. The postoperative mortality rate in 10.5%. Postoperative deaths occurred mainly in infants or when a tumour involved the brain stem. The five-year survival rate is 54% in the whole series. It rose to 71% in the patients who completed the treatment with radiotherapy. The study shows that the life of survivals is frequently impaired by mental or behavioural disturbances. IQ varies from 70 to 90 in 58% of the children; it is below 70 in 31%. Behavioural disturbances are found in 93% of cases. 82% have defective spatial orientation, dysphasia, or dysgraphia. In order to evaluate the responsibility of X-ray therapy for the development of these sequelae, the results have been compared to those of a series of cerebellar astrocytomas surgically removed, but not irradiated. The comparison shows that radiotherapy is at least partially responsible for the mental and behavioural disturbances. No relation was found between these disturbances and a persistent ventricular dilatation or an endocrine dysfunction. However the endocrine assessment showed growth hromone deficiency in 65% of the children, short stature in 55%, and compensated hypothyroidism in 58%.
Adolescent↗