Hendrick Eb

Arteriovenous malformations of the brain in children : a forty year experience

Despite the great capacity for the pediatric brain to recover from stroke, the morbidity and mortality in children who harbor an arteriovenous malformation (AVM) remains high. This study examines the clinical data and management experience with 132 patients with brain AVM from 1949 to 1989. Although the high tendency for a childhood AVM to present with hemorrhage (79%) remained constant for the forty year study period, the associated morbidity and mortality of hemorrhage changed. The mortality rate from hemorrhage for the entire series was 25%, which was reduced from 39% to 16% after the introduction of computed tomography. The mortality from AVM hemorrhage since 1975 was dependent on location; 8 of 14 patients (57%) with a cerebellar AVM died from hemorrhage while only 2 of 44 patients (4.5%) with a cerebral hemisphere AVM died (p less than 0.0001). Sixteen children (12%) presented with a chronic seizure disorder. Surgical excision of the malformation resulted in complete seizure control off anti-convulsant medication in 73% of patients. Although 21% of patients were treated non-operatively (many with terminal poor-grade hemorrhage), 79% had a surgical procedure with total AVM excision achieved in 70 patients (53.1%). Complete AVM resection was followed by a normal neurological outcome in 47 children (67%). Most partial excisions (n = 9) and clipping of feeding arteries (n = 7) were performed in the early years of this study, and did not provide protection from rehemorrhage. Although conservative management has been advocated for selected non-hemorrhagic AVMs, we conclude that essentially all children with an AVM should be treated in order to eliminate the risk of hemorrhage.(ABSTRACT TRUNCATED AT 250 WORDS)

Experience with Surgical Decompression of the Arnold-Chiari Malformation in Young Infants with Myelomeningocele

Forty-five infants with myelomeningocele in whom hydrocephalus was absent or adequately controlled developed signs and symptoms of the Arnold-Chiari malformation before the age of 3 months. All of them underwent laminectomy and opening of the dura mater for hindbrain decompression. The clinical presentation included swallowing difficulty, apneic episodes, stridor, bronchial aspiration, arm weakness, and opisthotonos. Within 2 weeks of the initial clinical presentation, the neurological status of 14 patients (31%) deteriorated dramatically and culminated in irreversible neurological deficits. In all patients, compression of the brain stem occurred in the spinal canal. A transverse dural band constricting the dural sac at the C-1 level was noted in 41% of the patients, and a mild degree of arachnoidal adhesion was noted in 23%. The lowermost level of the cerebellar tongue or medullary kink was located at C-1 to C-4 in 28 cases and at C-5 to T-1 in 17 cases. Twenty-eight (62%) of the patients were alive and 17 (38%) had died at the last follow-up assessment. All survivors showed improvement of their overall neurological function. Twenty-four made a complete recovery. The majority of deaths were attributed to respiratory failure. Early recognition of symptoms and prompt decompressive laminectomy are essential for successful management of the Arnold-Chiari malformation in infants.

Hydrosyringomyelia and Its Management in Childhood

Syringomyelia, once regarded as a degenerative disease of adults, is now recognized to be a disorder usually associated with the Chiari malformation and occurring in patients of all ages. We have reviewed 47 patients with syringomyelia treated on the Neurosurgical Service at the Hospital for Sick Children during the years 1977 to 1985. Twelve of these patients had a Chiari I malformation, 30 had a Chiari II malformation, and 5 had an acquired Chiari malformation. Thirty-one of these patients were treated by decompression of the Chiari malformation and plugging of the obex, 5 were treated by a simple posterior fossa decompression, 9 were treated by shunting of the syringomyelic cavity, and 2 were treated by a combined decompression of the posterior fossa and shunting of the syrinx. The Gardner procedure (decompression of the Chiari malformation and plugging of the obex) was the procedure most commonly used in managing our group of patients and resulted in improvement in over 70% of patients.

Transependymal Benign Dorsally Exophytic Brain Stem Gliomas in Childhood: Diagnosis and Treatment Recommendations

Sixteen children with dorsally exophytic transependymal benign brain stem gliomas were treated at the Hospital for Sick Children, Toronto, between 1949 and 1985. The diagnosis of these benign brain stem gliomas was based on neuroradiographic and operative observations. Five children were diagnosed in the pre-computed tomography (CT) era using ventriculography and pneumoencephalography, and 11 children were diagnosed using CT with and without contrast enhancement. Histological diagnosis was obtained in all 16 cases. In 1980, we first described this distinct group of benign brain stem gliomas, which accounted for 8% (total, 121 cases) of all brain stem gliomas diagnosed at our institution. However, with the use of high resolution CT in recent years, earlier and better definition of this particular type of brain stem tumor can be made. During the 10 years (1976 to 1985) in which CT has been used for preoperative evaluation in our institution, we have treated 49 patients with primary brain stem tumors. Eleven of these cases (22%) fell into the category of dorsally exophytic transependymal benign brain stem tumors. These tumors characteristically protrude into and largely fill the 4th ventricle, breaking through the overlying ependyma and frequently extending into the cisterna magna. On CT, they are isodense and enhance brightly with contrast agent. Clinically, these patients have a longer history of symptoms in comparison with patients with the more malignant, intrinsic types of brain stem tumor. All of our patients were treated by subtotal resection. Radiotherapy was utilized in 7 of the 16 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Dandy-Walker Syndrome: Experience at the Hospital for Sick Children, Toronto

Thirty-five patients with the Dandy-Walker syndrome (DWS) treated over the years 1964-1987 at the Hospital for Sick Children, Toronto, were reviewed. Thirty-three patients (94%) had hydrocephalus at the time of diagnosis. The primary association of aqueduct stenosis was excluded by radiological investigation and clinical course in all patients. Associated central nervous system (CNS) anomalies were present in 10 patients. Seven had occipital encephalocele and in 1 of these there was an associated Klippel-Feil syndrome. Four patients had agenesis of the corpus callosum and 1 patient had polyporencephaly. The treatment of these patients has changed over the years in our institution so that during an earlier period, 3 patients were treated by resection of the cyst membranes. We then went through a period of shunting the lateral ventricle so that 21 patients were treated with either a lateral ventricle to peritoneal or lateral ventricle to atrial shunt. More recently, patients with the DWS have been treated with a cyst-peritoneal shunt and 10 patients were thus treated. Only 1 patient was treated with a simultaneous lateral ventricle and cyst-peritoneal shunt. Nine of the twenty-one patients (43%) with a primary lateral ventricle to peritoneum or lateral ventricle to atrial shunt developed a secondary aqueduct stenosis and an isolated fourth ventricle which required additional cyst-peritoneal shunting. Only 1 of the 10 patients treated with a primary cyst-peritoneal shunt required an additional lateral ventricle peritoneal shunt. Two patients initially treated with a lateral ventricle peritoneal shunt and with a primary cyst-peritoneal shunt subsequently had a percutaneous stereotactic third ventriculostomy.(ABSTRACT TRUNCATED AT 250 WORDS)

Myxopapillary Ependymoma of the Filum Terminale and Cauda Equina in Childhood: Report of Seven Cases and Review of the Literature

Seven of fourteen children with spinal cord ependymoma had myxopapillary tumors of the filum terminale. These tumors made up 15.9% of all primary spinal neuroectodermal tumors in children (44 cases) seen during a 62-year period (1919 to 1981). Their clinical presentation, radiological features, pathological findings, treatment, and outcome are reported. Six of the seven patients were known to be alive at the time of writing. The seventh patient was lost to follow-up after 3 years without tumor recurrence. Of 5 patients whose primary mode of treatment was operation alone. 3 had intraspinal or intracranial recurrences. Despite tumor recurrences, 2 patients were long term survivors after further operation and irradiation, whereas the third patient recently received craniospinal irradiation for intracranial tumor recurrence. The 2 patients who did not have tumor recurrence after operation alone had been followed for 3 and 7 years, respectively. Two children with subtotal tumor resection and spinal irradiation had no recurrences at 1 and 17 years, respectively. Our data suggest that this unusual subtype of spinal ependymoma is not uncommon during childhood and has a good prognosis. All patients with this tumor require prolonged follow-up for tumor recurrence after operation and irradiation.

Dermoid Cysts of the Anterior Fontanelle

A review of the medical records at The Hospital for Sick Children in Toronto, Ontario, from 1967 through 1980 showed that, of the 94 cases of dermoid cyst of the skull treated at this institution by the neurosurgical staff, 25 were located over the anterior fontanelle. This is the largest single group of cases reported to date. The children were 2 months to 13 years of age, but two-thirds were treated before their 1st birthday. Females outnumbered males 2:1, and all of the patients presented with a nontender swelling over the anterior fontanelle, which had been present from birth. The lesions were treated by a variety of surgical approaches, all of which were curative. None of the lesions has recurred. The most interesting finding was that all but 2 of the children were white, in contrast to all previously published series. This confirms our belief that the racial incidence of dermoid cysts of the anterior fontanelle is a reflection of the general population. A review of previously published cases and the pathology and embryology of the lesion is included.

Surgical management of unilateral and bilateral coronal craniosynostosis: 21 years of experience.

From 1955 to 1975, 116 patients with unilateral and bilateral coronal synostosis, including 39 with craniofacial dysmorphism, were treated surgically. Five techniques were used: multiple linear craniectomies, linear craniectomies with supraorbital grafting, morcellation craniotomies, lateral canthal advancement of the supraorbital margin, and radical cranio-orbitofacial reconstruction. Analysis of clinical and radiological indices of the synostotic process, coupled with evaluation of the surgical results, indicates that the anterior skull base is the site of origin of the bony dysplasia. Therefore, early creation of artificial sutures in the skull base is recommended to provide adequate expansion of the frontal bones and consequently of the entire facial skeleton.

Primary Intracranial Choriocarcinoma: A Report of Two Cases and a Review of the Literature

Among 848 cases of primary intracranial malignancy seen during a 63-year period at a pediatric hospital, there were 2 cases of primary pineal choriocarcinoma. The clinical and laboratory findings of these 2 cases were similar to those of 33 cases of intracranial choriocarcinoma reported in the literature. In 1 patient with precocious puberty, the diagnosis was confirmed by labeling human chorionic gonadotropin (HCG) within the tumor, which had been stored for 25 years. In the other patient, who is alive and well 18 months after diagnosis, the response to cranial irradiation and intensive chemotherapy was monitored with serial measurements of serum, cerebrospinal fluid, and urinary HCG and with computed tomography. We conclude that intensive chemotherapy, radiation, and tumor resection, if feasible, offer the best chance of curing this otherwise fatal disease.

Lumbar disc disease in children.

The clinical experience with lumbar disc disease in 38 patients at a large childrens hospital is reviewed. Thirty children underwent surgical treatment; eight patients were not operated upon. The surgical results were excellent, with 93% of the children cured or markedly improved on follow-up. The main differential aspects of lumbar disc disease between children and adults are emphasized.