Lateral ventricle choroid plexus papilloma in childhood: management and complications.

BACKGROUND: A review of the choroid plexus papilloma of the lateral ventricle in the pediatric age group is presented. The characteristic clinical features, imaging studies, preoperative, and operative approaches, as well as complications, will be included. METHODS: Among a group of 24 patients with ventricular choroid plexus papilloma treated by the authors over a 12-year period, seven patients were selected for this study. The selection included patients under the age of 8 years at the time of their presentation with choroid plexus papilloma of the lateral ventricle. Adult patients and children with choroid plexus papilloma at other sites or choroid plexus carcinoma were excluded. At least a 30-month follow-up period was available for all patients except one. Preoperative and postoperative imaging studies confirming total tumor resection were available for all patients. RESULTS: Developmental delays and regression were the most consistent clinical features. Magnetic resonance imaging (MRI) proved to be the diagnostic test of choice. The addition of magnetic resonance angiogram (MRA) obviates the need for angiography. Our attempts at tumor embolization failed. CONCLUSION: An intersulcal splitting approach to the lateral ventricular trigone, combined with a perioperative external ventricular drainage, may be of value in the avoidance of symptomatic subdural effusions.

Myxopapillary ependymoma of the conus medullaris and filum terminale in the pediatric age group.

Myxopapillary ependymoma of the conus medullaris and filum terminale is a relatively common spinal intradural neoplasm in adulthood. However, only a reported 8-12% of such tumors affect this site in children, and the ideal management remains controversial. Three children with myxopapillary ependymomas of the conus medullaris and filum terminale were treated by the author over a 2-year period with an at least 24-month follow-up. These children, ages 7, 8 and 13 years, included 1 male and 2 females. Their salient presentation was an acute exacerbation of chronic lower back pain. The duration of symptoms prior to diagnosis ranged from 16 to 18 months. A preoperative MRI, with and without contrast, was available for every case. All children underwent replacement laminoplasty with gross total tumor resection. Somatosensory evoked potentials were used in all surgeries. An early postoperative MRI of the entire neural axis was available for all cases. No permanent complications were noted. Self-catheterization for 6 weeks was required in 1 child with preoperative urinary incontinence. One child received radiation therapy following a recurrence. Clinical and surgical results were compared to the only 2 other reported series (11 patients) addressing this type of tumor in children. Based on this review, the authors propose that: (1) unexplained and intractable lumbar pain in childhood should be thoroughly investigated with an MRI scan: (2) the gross feature of myxopapillary ependymoma allowing for complete resectability appears to be the key prognostic factor; (3) radiotherapy appeared to have no proven value in completely resected tumors in children; (4) postoperative baseline MRI and regular sequential imaging studies are essential for long-term follow-up, and (5) replacement laminoplasties may be of value in preventing future spinal deformities, musculoskeletal pain and allowing for an 'easier' resection in the event of a recurrence.

Posterior fossa lateral ependymoma in childhood.

Over a period extending from 1984 to 1993, 16 children ranging in ages from 2 months to 12 years with posterior fossa ependymoma were treated. Four of these tumors were classified as lateral ependymomas because of their configuration and suspected site of origin. These 4 patients' ages ranged from 2 months to 5 years. Signs and symptoms of increased intracranial pressure and cranial nerve dysfunction were the hallmark of their presentation. Their imaging evaluation included a preoperative and postoperative CT san and/or MRI. A gross total surgical resection' was completed in the 4 children. At least a 1-year follow-up was available for all the patients. Intraoperative brain stem evoked potentials and facial and glossopharyngeal nerve monitoring were used in all the surgeries. Three children required a ventriculoperitoneal shunt postoperatively. Chemotherapy was used postoperatively in 3 children. The 4th child did not receive chemotherapy due to parental refusal and succumbed to a recurrence 1 years postoperatively. Radiation therapy was given to 2 children. Tumor recurrences developed in all children at 12 months, 18 months, 3 years and 4 years postoperatively in proximity to the original tumor site. Histologic sections in 3 patients, including those at the time of recurrence, were consistent with a low mitotic index. Only 1 child's histologic diagnosis was consistent with a high-grade ependymoma. The lower cranial nerve dysfunction transiently worsened in all the patients and was permanent in 2. The child's age, tumor histology and extent of resection play an important role in the prognosis of posterior fossa ependymoma. The lateral posterior fossa ependymoma has a particularly poor prognosis due to its location and its significant postoperative morbidity. The authors propose a combined midline and lateral suboccipital approach in order to expose the fourth ventricular floor, upper cervical spine, lateral recess, cerebellomedullary fissure, cerebellopontine and the cerebellomedullary cistern in an attempt at a "total' resection with a focus on the site of origin. The usage of neurophysiological monitoring appears to be useful in limiting and predicting the extent of postoperative complications. However, prolonged morbidity is likely and is commonly related to lower cranial nerve deficits. The author proposes a proactive approach in order to limit the sequelae of these complications.

An approach to symptomatic children (ages 4-14 years) with Chiari type I malformation.

A review of 16 pediatric patients examined and treated from 1987 to 1991 is presented. The purpose for this review was to examine the different initial clinical presentation in the younger age group versus the older child and to identify the factors impacting on the patients' surgical outcome, including the patients' age, clinical presentation and MRI findings. Patients' ages ranged from 4 to 14 years. Ten were under 6 years of age. Seven males and 9 females were included. The diagnosis was confirmed by MRI in all patients and all were followed by clinical and MRI evaluations for at least 1 year postoperatively. The patients were grouped into three subgroups: (a) group IA patients with a Chiari I malformation, (b) group IB patients with a Chiari I malformation and syrinx, and (c) group IC patients with a Chiari I malformation and hydrocephalus with or without an associated syringomyelic cavity. A uniform surgical approach was used and tailored to the degree of anomalies seen on MRI. Patients with a Chiari IA and IB were treated by a suboccipital craniectomy, upper cervical laminectomy, arachnoidal retraction and duraplasty. Only 1 patient in group B required a syringosubarachnoid shunt initially. Patients with a Chiari IC malformation were treated by a ventriculoperitoneal shunt initially. The clinical presentation of younger children (less than 6 years old) appeared similar to older children (over 6 years of age) except for sleep apnea, which was limited to the younger age group. Patients with pronounced motor and sensory deficits had the worst prognosis for recovery.(ABSTRACT TRUNCATED AT 250 WORDS)

Transoral labiomandibular approach to basiocciput chordomas in childhood.

Many excellent reports have dealt with the various aspects of cranial chordoma. It remains a relatively rare neoplasm, particularly in younger children. The authors have had the opportunity to treat a 5-year-old child harboring a basiocciput chordoma. It extended from the mid-clivus to C3. A transoral labiomandibular approach was used, allowing its resection. No evidence of recurrence was noted 3 years later. A literature search confirmed the rarity of basiocciput chordoma in young children. The perioperative difficulties encountered prompted this report.

Analysis of management in thirty-three closed head injury patients who "talked and deteriorated".

Of 215 patients with severe head injuries, 33 (15%) closed head injury patients who talked before their conditions deteriorated to a Glasgow coma scale score of 8 or less were identified. Of this select group, 15 died (45%), but none of the remaining were left in a vegetative state and 14 patients had a "favorable" outcome (42%). Twenty-five patients (76%) underwent surgical decompression. In these 25 patients, 14 subdural hematomas, 4 epidural hematomas, and 7 intracerebral contusions and hematomas were the initial surgical lesions. Twenty of the 25 patients were operated on within 4 hours (16 within 2 hours) of their neurological deterioration. Eleven of the 25 surgically treated died, for a mortality rate of 44%. All 15 deaths were studied further. Autopsies with examination of the brain were performed in 13 patients. Five patients died with severe brain injuries not complicated by iatrogenic factors, and 4 patients died of severe associated injuries. Iatrogenic factors significantly complicated the deaths of 6 patients (40%). It is concluded that most patients who "talk and deteriorate" have sustained very serious life-threatening injuries. Intracranial hematomas are the most frequent cause of this situation, and rapid diagnosis and decompression is the most important factor in salvaging these patients.

Neuroschisis of the cervical spinal cord in a patient with Klippel-Feil syndrome.

During the neurological work-up of a young patient with Klippel-Feil syndrome, the presence of a neuroschisis of the cervical spinal cord was detected. The patient presented with a transient and acute hemisensory loss and a Horner's syndrome of the opposite side. The unusual presentation and radiological findings in a patient with Klippel-Feil syndrome prompted this report.

Civilian gunshot wounds to the brain: prognosis and management.

The extent of treatment for the victims of gunshot wounds to the brain remains quite controversial, particularly when these patients present with extensive neurological dysfunction. We propose guidelines regarding the degree and aggressiveness of therapy. The factors that seem to have a significant impact on the patient's final outcome are the neurological examination at the time of admission, the radiological findings, and the motivation for the shooting. Thus, the authors propose a nonsurgical line of therapy for comatose patients with unilateral or bilateral cerebral gunshot wounds where bone or metal fragments are visualized away from the bullet path on computed tomography scan, particularly when these individuals are suicide victims.

Klippel-Feil syndrome in children: clinical features and management.

A retrospective analysis of 11 children with the diagnosis of Klippel-Feil syndrome treated at the University of Minnesota Hospital over a period of 20 years is presented. The salient features of the syndrome and its associated anomalies are reviewed. Emphasis is placed on its neurological aspects, particularly the potential risks of injury to the craniocervical junction and cervical spine. Guidelines for the management of these patients are suggested.

Identification and management of high-risk patients with Klippel-Feil syndrome.

Patients with Klippel-Feil syndrome are often at high risk for neurological injury. The cervicomedullary junction and cervical spinal cord are especially vulnerable. Twenty-one patients examined and treated over a 20-year period are reviewed. The salient features of the syndrome are identified, and an approach to management is proposed.

Tuberous sclerosis: a review for the neurosurgeon.

The clinical, radiological, and pathological characteristics of tuberous sclerosis are reviewed. Neurosurgical intervention in the syndrome is discussed in light of two recently treated cases and a literature review.