Arachnoid cysts in children.

Twenty-seven cases of intracranial arachnoid cysts in children aged 1 to 16 years have been studied with attention to diagnosis, pathologic findings, and surgical treatment. In nine patients, the cyst was located in the posterior fossa. In eight patients, there were other CNS or other organ system abnormalities. No specific symptom complex can be ascribed to arachnoid cysts in that the clinical manifestations are the result of their size and location, secondary complications, and remote effects. This is illustrated in the ten cases reported.

Combined anti-fungal therapy and surgical resection as treatment of disseminated aspergillosis of the lung and brain following BMT.

This report describes a 6-year-old girl with pre-B ALL who developed systemic aspergillosis following BMT. She was successfully treated with a combination of amphotericin B, itraconazole and surgery. This report emphasizes the importance of surgical resection in the management of disseminated aspergillosis.

Suprasellar arachnoid cysts: 1. CT recognition.

Suprasellar arachnoid cysts are basal midline masses that represent a rare but surgically remediable cause of hydrocephalus and neurologic deficits. These cysts represent a diagnostic challenge and often go unrecognized for many years. The authors review the computed tomographic (CT) findings in seven patients with documented suprasellar arachnoid cysts and define previously undescribed diagnostic criteria. These cysts usually can be differentiated from cystic midline neoplasms by their CT density, homogeneity, and location as well as by their lack of fat, lack of calcification, and absence of contrast enhancement. Accurate distinction from marked third-ventricular enlargement secondary to obstructive hydrocephalus and from third-ventricular ependymal cysts can be made on the basis of basal cisternal expansion, distinctive mass effect and displacement, the characteristic shape and contour of the apparent "third ventricle," and the appearance of structures at the foramen of Monro. Although metrizamide CT ventriculography and cisternography allow confirmation of the diagnosis and evaluation of cerebrospinal fluid dynamics, these definitive studies will not be obtained unless the cysts are first suspected by their conventional CT appearance.

Suprasellar arachnoid cysts: 2. Evaluation of CSF dynamics.

Suprasellar arachnoid cysts are an infrequent but surgically remediable cause of hydrocephalus and neurologic deficits. The optimal method of treatment varies according to whether the cyst communicates with the subarachnoid space; the presence or absence of associated hydrocephalus; and the exact site(s) and severity of intraventricular and extraventricular cerebrospinal fluid (CSF) obstruction. Eight patients with suprasellar arachnoid cysts are described to emphasize the importance of pretherapeutic investigation of CSF dynamics. The variability of the ventriculocisternal-cyst CSF dynamics and the value of metrizamide computed tomographic cisternography and ventriculography in investigation of the CSF pathways are stressed. A diagnostic approach to the evaluation of the CSF dynamics is outlined, based on whether hydrocephalic or compressive symptoms predominate.

Presentation and management of pediatric Chiari malformations without myelodysplasia.

Chiari malformations without myelodysplasia are rarely diagnosed in the pediatric age group. With current neurodiagnostic techniques, however, they are being seen more frequently. Unfortunately, the prognosis is not clear because publications have included a number of different entities, used a variety of surgical approaches, and lacked long term follow-up. Sixteen patients younger than 20 years were treated for Chiari malformations (without myelodysplasia) between 1975 and 1985. The average age was 11 years, and the average duration of symptoms was 20 months. The common symptoms were isolated motor weakness (56%), pain (37.5%), and sensory loss (25%). Frequently seen signs were motor deficit (81%), sensory loss (50%), scoliosis (50%), and cranial nerve palsy (50%). The surgical procedures used were foramen magnum decompression (3 transoral clivus odontoid resections and 15 posterior fossa decompressions with dural grafting), alteration of cerebrospinal fluid (CSF) pathways at the cervicomedullary junction (plugging the foramen cecum and a 4th ventricle to subarachnoid shunt with posterior fossa decompression), and ventriculoperitoneal shunting (2 cases). In follow-up, 37.5% of the patients are asymptomatic, 50% are improved, and 12.5% are stable after an average follow-up period of 43 months. The asymptomatic group was younger (9.3 years) and had a shorter symptom duration (7.2 months) than both the improved (11.9 years, 16.4 months) and the stable groups (15 years, 20 months). Optimal outcome depends on complete evaluation of the abnormal CSF pathways and bony abnormalities at the craniovertebral junction. Operation is then directed toward correction of these abnormalities as delineated radiographically. Of our patients, 87.5% have at least shown improvement, which has been long term in all cases.

Pediatric spinal cord and vertebral column injury.

Between January 1, 1970, and December 31, 1988, 179 children (birth to age 16) were treated for spinal cord and/or vertebral column injury by the Neurosurgical Service at the University of Iowa Hospitals and Clinics. Pediatric injuries accounted for 9% of all spinal trauma seen during this period. The mean age was 10.2 years. Sixty-two children were between birth and 8 years of age and 117 were between ages 9 and 16. The cause, distribution, type of injury, and severity of neurological injury varied with age. Neurological outcome was dependent on the severity of the initial neurological injury. Children with complete or severe incomplete myelopathy uniformly remained with severe neurological dysfunction; children with mild to moderate injuries recovered normal or nearly normal neurological function. Surgical versus nonoperative management had no bearing on neurological outcome. Twelve percent of the children with severe spinal cord injuries developed posttraumatic spinal deformity. We conclude that spinal injury patterns differ between preadolescent and older children. Most injuries can be successfully managed with nonoperative therapy. Prognosis is primarily correlated with the severity of the initial neurological insult. Finally, children with severe spinal cord injury must have close, long-term follow-up to monitor the development of posttraumatic spinal deformity.

Hemorrhagic complications in association with silastic dural substitute: pediatric and adult case reports with a review of the literature.

OBJECTIVE AND IMPORTANCE: Delayed intracranial hemorrhage is an unusual complication associated with the use of silastic dural substitute. CLINICAL PRESENTATION: We present three patients with this complication. Two adult patients developed subdural and epidural hemorrhages 9 months and 10 years after posterior fossa surgery for Chiari malformations. The remaining patient, a 13-month-old child, is the youngest reported patient to develop an epidural hematoma 8 months after a craniosynostosis repair. INTERVENTION: The hematomas were removed with the silastic dural substitute from all three patients, and the dural defect was repaired with autologous paracervical fascia. CONCLUSION: Silastic dural grafts have an increased incidence of hemorrhage associated with their use. A slight increase in the prevalence of hemorrhagic complications with silastic dural substitute was observed in women. We report our radiographic and surgical findings, including an extensive review of the literature.

Pediatric pituitary tumors.

This retrospective review of pediatric patients with pituitary tumors causing onset of symptoms by 17 years of age was done to define their pathological distribution, clinical presentation, treatment, and prognosis. Eighteen patients were evaluated and treated from 1979 to 1989. Five had Cushing's disease and 13 had prolactin-secreting tumors. The mean age at the onset of symptoms was 14.7 years, with a range of 7 to 17 years. The mean follow-up period was 4.6 years, and the series consisted of 15 girls and 3 boys. Four of the 5 patients with tumors secreting adrenocorticotropic hormone were girls. The five patients exhibited obesity, hypertension, and growth retardation. The mean age of this group of patients at diagnosis was 12.2 years, and all had intrasellar lesions removed by the transsphenoidal approach. Adenoma was documented in 4 cases by histopathology. There was complete resolution of the endocrinological and clinical abnormalities in each case. The group of patients with prolactinomas comprised 11 girls and 2 boys, and their mean age at diagnosis was 15.7 years. The girls exhibited either primary or secondary amenorrhea. Seven had macroadenomas and 4 had microadenomas. Nine of the 11 girls underwent transsphenoidal resection, and surgery failed in 6, based on hormonal or radiological data. The two boys had suprasellar tumor extension and required multiple surgical procedures plus radiation therapy for control of the tumor mass.

Posterior fossa hemorrhage in the term neonate.

Posterior fossa hemorrhage (PFH) in the newborn term infant has been infrequently reported, and before computed tomographic (CT) scanning most were identified at postmortem examination. The origin and causes of PFH in the term neonate differ from those in adults. The main possible causes are: (a) tentorial and falx disruption with avulsion of bridging veins due to mechanical compression and distortion of the head during the birth process, resulting in posterior fossa subdural collections and vermis hematoma, and (b) occipital osteodiastasis associated with cerebellar hemisphere laceration, with intracerebellar hematoma and subdural collections. Three full-term neonates with PFH were operated on with good outcome. Both of the possible causes contributed to the hemorrhage. Review of the literature to date has led to the following conclusions: (a) PFH in the term infant is frequently associated with breech delivery, forceps instrumentation, and prolonged labor with cranial molding. (b) Massive PFH is associated with dural sinus laceration, rupture of the vein of Galen, or rupture of hematoma into the subdural space. Symptoms may be delayed up to 96 hours in cases where there is a small continuing hemorrhage. (c) CT scanning with reconstructions is essential to provide precise localization of the hemorrhage and thus avoid errors in interpretation. (d) Prompt removal of PFH improves survival and decreases sequelae. (e) Shunting procedures are necessary in 36% of the cases.

Idiopathic spinal epidural lipomatosis.

OBJECTIVE: Spinal epidural lipomatosis (SEDL) is a rare disorder often associated with the administration of exogenous steroids or the elevation of endogenous steroids. Spinal epidural lipomatosis develops in some patients in the absence of elevated steroid levels. The limited information known about idiopathic SEDL comes predominantly from isolated case reports. We proposed to study our experience with idiopathic SEDL and to review the literature. METHODS: We identified eight symptomatic patients with idiopathic SEDL treated at our institution, which is the largest series reported. All patients were male and obese by body mass index (> 27.5 kg/m2). The mean age of the patients was 35.4 years. Idiopathic SEDL was equally distributed between the thoracic and lumbar spine. Six patients underwent laminectomy and fat debulking with good postoperative results; two patients were treated with a weight loss diet, which resulted in the relief of symptoms after losing > 15 kg each. RESULTS AND CONCLUSION: A review of our patients in conjunction with other reported cases reveals the following: 1) idiopathic SEDL occurs almost exclusively in the obese population; 2) idiopathic SEDL seems to occur with equal frequency between the thoracic and lumber spine; 3) a strong male predominance exists; 4) thoracic SEDL presents at an earlier age compared with lumbar SEDL; 5) surgical decompression remains the treatment of choice for the immediate relief of symptoms. Our experience suggests that idiopathic epidural lipomatosis may be a pathological entity that has been underdiagnosed.

Primary intracranial rhabdomyosarcoma.

Primary intracranial rhabdomyosarcoma is rare. Twenty-one cases with dismal outcomes have been reported. We add five children with this disease treated between 1977 and 1982. Their therapy consisted of surgical resection, craniospinal irradiation, and intravenous-intrathecal chemotherapy. Two children have recovered, 21 and 67 months after diagnosis; the last is the longest survival reported in the literature. Two succumbed to tumor recurrence, and one died due to pulmonary embolism. The posterior fossa was invariably a site of tumor at presentation. Diagnosis with light microscopy can be elusive; electron microscopic and immunohistochemical evaluation are necessary to confirm the pathological condition. This is essential so that early, aggressive therapy can be instituted. These diagnostic and therapeutic techniques have led to a reevaluation of this malignancy and its prognosis.

Ganglioglioma: 13 years of experience.

A 13-year retrospective review of 17 patients with gangliogliomas treated at the University of Iowa was conducted to investigate the association between tumor location, extent of resection, pathological findings, and patient prognosis. Thirteen were in the cerebral hemispheres and 4 in the midline. The mean ages at diagnosis and symptom onset were 16 and 8.8 years, respectively. The most common presenting symptom was seizures (11 patients); focal neurological deficit was seen in 5 patients and headache in 1. Patients with hemispheric tumors had an older age at time of diagnosis and a longer duration of symptoms. Pathologically, they had more microcalcifications, lymphocytic infiltration, microcystic degeneration, and eosinophilic bodies. Cerebral hemispheric tumors were more amenable to total resection than midline neoplasms, 77% versus 25%, respectively. In the patients with hemispheric tumors, 10 patients were tumor-free after total resection. Two of the 3 patients with partial resection had stable residual tumors. The third died of tumor progression. In the 4 patients with midline neoplasms, one was tumor-free after total resection. The other 3 had subtotal resection and radiation therapy. Two died within 2 years; the third is still alive with progressive tumor. Five of 9 patients who had seizure disorders and who underwent total tumor removal were seizure-free postoperatively. The other 4 patients and the 2 with partial surgical resection continued to have seizures.

Cervical epidural hematoma secondary to an extradural vascular malformation in an infant: case report.

We present a report of a 22-month-old infant with a spontaneous spinal epidural hematoma arising from a purely epidural vascular malformation. Although often suspected as a cause of spontaneous epidural hemorrhage, vascular malformations have rarely been demonstrated. The important aspects of the presenting symptoms in this young age group are highlighted. We discuss the entity of spontaneous epidural hemorrhage and the characteristics that distinguish purely epidural from dural arteriovenous malformations.

Comparative evaluation of intracranial epidermoid tumors with computed tomography and magnetic resonance imaging.

The diagnosis of intracranial epidermoid tumors with computed tomography (CT) is often difficult because of indistinct margins, close proximity to the skull base, and a density similar to that of cerebrospinal fluid (CSF). Recent experience with six histologically confirmed epidermoid tumors served to emphasize the value of magnetic resonance (MR) imaging in studying these lesions. MR images were obtained using varying spin echo and inversion recovery techniques with a 0.5-tesla superconducting magnet. CT with and without enhancement had been performed in each case. In Case 1, CT showed an ill-defined left cerebellopontine angle hypodensity. MR imaging clearly showed the presence of abnormal tissue at that location. Case 2 showed a CSF density mass in the right upper posterior fossa. MR imaging of that area showed a variegated signal of a mass extending supratentorially. CT of Case 3 showed a left medial middle fossa hypodensity with an enhancing rim. MR imaging showed a clearly extraaxial mass in that location. In Case 4, a diffuse cerebellar hemispheric hypodensity was observed on CT and was clearly demarcated by MR studies. A huge lesion, thought initially to be an arachnoid cyst on CT of Case 5, was seen on MR imaging to be a large, extraventricular mass displacing the temporal lobe. Finally, CT in Case 6 was suggestive of a poorly demarcated right cerebellopontine angle lesion, which was seen on MR images to be extraaxial, displacing the brain stem. Various MR images more clearly demonstrate the extent of abnormal tissue than CT of epidermoid tumors.(ABSTRACT TRUNCATED AT 250 WORDS)

Increased height in patients with medulloblastomas.

OBJECTIVE: Medulloblastomas demonstrate histological features similar to neuroendocrine tumors. Expression of various receptors for growth factors and production of growth hormones have been identified to occur with medulloblastomas. We studied the preoperative height of patients with medulloblastomas. METHODS: We studied 85 patients (64 children and 21 adults) with medulloblastomas and 42 patients (27 children and 15 adults) with cerebellar astrocytomas who served as a control group. All of the patients had their height and weight documented on standardized growth charts. In addition, age, sex, symptoms, radiographic findings, treatment, and survival were examined. RESULTS: Preoperatively, 22.4% of the patients with medulloblastomas were above the 95% curve in height and 80.0% were above the 50% curve for height. Compared with patients with cerebellar astrocytomas, 7.1% were above the 95% curve for height and 54.8% were above the 50% curve for height. The distribution of patients along the weight curves for both tumor types demonstrated a slight prevalence for lower weights but was not significantly different from the national average. A significant number of patients presenting with medulloblastomas attained increased height, which was disproportionate to the weight loss generally observed with neoplasms. To our knowledge, the disproportionate number of patients with medulloblastomas and increased height has not been reported before. A similar deviation in height distribution from the normal population could not be identified in patients with cerebellar astrocytomas. CONCLUSION: This study suggests that medulloblastomas may be influenced by growth hormone production or may produce growth factors in vivo.

Brain tumors occurring before 1 year of age: a retrospective reviews of 22 cases in an 11-year period (1977-1987).

Congenital brain tumors have been reported infrequently and their management remains ill defined. An 11-year review (1977-1987) of all children with brain tumors with the onset of symptoms before 1 year of age was completed. Twenty-two children with the following histological diagnoses were treated: astrocytoma (7 patients), primitive neuroectodermal tumor (6 patients), papilloma or carcinoma of the choroid plexus (3 patients), malignant teratoma (2 patients), dermoid tumor (2 patients), embryonal rhabdomyosarcoma (1 patient), and chloroma (1 patient). Fifteen tumors were supratentorial in location, and 7 were infratentorial. Initial symptoms were hydrocephalus (32%), focal neurological deficit (23%), asymptomatic increase in head circumference (18%), failure to thrive (14%), and seizures (4.5%). The goal of treatment was a radical excision when possible, with primary chemotherapy in the last 6 years of the review period. Radiation therapy was the adjunct to surgery in the initial 5-year period. All patients with papillomas of the choroid plexus and dermoid lesions underwent a total resection with no recurrence. All 7 astrocytomas were supratentorial, with 6 occurring in the diencephalon. Five of the seven patients with astrocytomas survived more than 5 years. The 6 primitive neuroectodermal tumors were located equally between the supra- and infratentorial spaces. Four of the 6 infants with these tumors received chemotherapy (2 received chemotherapy alone; 2 received chemotherapy and radiation therapy) and are tumor free 2 to 9 years later. A fifth child received radiation therapy alone early in the series and survived only 4 months. The family of the other child refused adjunctive treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Transoral-transpharyngeal approach to the anterior craniocervical junction. Ten-year experience with 72 patients.

The anterior transoral-transpharyngeal operation to correct ventral irreducible compression of the cervicomedullary junction was utilized in 72 individuals. The patients' ages ranged from 6 to 82 years, and 29 were children. The pathology encountered was primary basilar invagination, rheumatoid irreducible cranial settling, secondary basilar invagination due to migration of odontoid fracture fragments, dystopic os odontoideum, granulation masses, clivus chordoma, osteoblastoma, and chondroma of the atlas. Fifteen patients had associated Chiari malformation with basilar invagination. Fifty-two patients required subsequent atlantoaxial or occipitocervical fusion. Neurological improvement was the rule. There were two deaths within 30 days of surgery: one from myocardial infarction 4 weeks after surgery and one from Gram-negative septicemia of urinary tract origin. There was one pharyngeal wound infection. The ventral transoral approach provides a safe, rapid, and effective means for decompression of the abnormal craniovertebral junction.

Basilar invagination in osteogenesis imperfecta and related osteochondrodysplasias: medical and surgical management.

Osteogenesis imperfecta (OI) is a heritable disorder of bone development caused by defective collagen synthesis. Basilar invagination is an uncommon but devastating complication of this disease. The authors present a comprehensive strategy for management of craniovertebral anomalies associated with OI and related osteochondrodysplasias. Twenty-five patients with congenital osteochondrodysplasias (18 OI, four Hajdu-Cheney syndrome, and three spondyloepiphyseal dysplasia) and basilar invagination were evaluated between 1985 and 1995. The male/female ratio in this cohort was 1:1. The mean age at presentation was 11.9 years (range 13 months-20 years). Fourteen patients (56%) presented during adolescence (11-15 years of age). Symptoms and signs included headache (76%), lower cranial nerve dysfunction (68%), hyperreflexia (56%), quadriparesis (48%), ataxia (32%), nystagmus (28%), and scoliosis (20%). Four patients (16%) were asymptomatic. Seven (28%) had undergone previous posterior fossa decompression; one had also undergone ventral decompression. Imaging findings included basilar invagination (100%), ventral brainstem compression (84%), hydrocephalus (32%), hindbrain herniation (28%), and syringomyelia/syringobulbia (16%). Patients with hydrocephalus underwent ventricular shunt placement. Reducible basilar invagination (40%) was treated with posterior fossa decompression and occipitocervical fusion. Those with irreducible ventral compression (60%) underwent transoral-transpalatopharyngeal decompression followed by occipitocervical fusion. All patients improved initially. However, basilar invagination progressed radiographically in 80% (symptomatic in 24%) despite successful fusion. Prolonged external orthotic immobilization with the modified Minerva brace afforded symptomatic improvement and arrested progression of the deformity. The mean follow-up period was 5.9 years (range 1.1-10.5 years). Ventral brainstem compression in OI should be treated with ventral decompression, followed by occipitocervical fusion with contoured loop instrumentation to prevent further squamooccipital infolding. Despite fusion, however, basilar invagination tends to progress. Prolonged immobilization (particularly during adolescence) may stabilize symptoms and halt further invagination. This study represents the largest series to date addressing craniovertebral anomalies in OI and related congenital bone softening disorders.

Lesions in Meckel's cave: variable presentation and pathology.

A series of 12 patients with mass lesions arising from Meckel's cave is presented. Patients' age on presentation ranged from 13 months to 71 years. Nine of the 12 patients had symptoms referable to the fifth cranial nerve, but only three complained of facial pain. The 12 patients presented eight different pathological entities, including meningioma, lipoma, schwannoma, malignant melanotic schwannoma, arachnoid cyst, neurofibroma, epidermoid tumor, and chordoma. Computerized tomography and magnetic resonance imaging were most useful in localizing the lesion to Meckel's cave. All 12 patients underwent a subtemporal approach to the lesion, and gross total removal was achieved in 11. Postoperative results were excellent with no increased neurological deficits seen 3 months postoperatively. Most patients had resolution of the cranial nerve deficits except for fifth nerve function, which was impaired in nine patients postoperatively. This series demonstrates that lesions in Meckel's cave can have a varied and unusual presentation, as well as an assortment of pathology. Total removal of lesions in this area resulted in relief of symptoms in most patients, with minimum morbidity.

Cervicomedullary compression in achondroplasia.

Six patients with achondroplasia and symptoms suggestive of cervicomedullary junction compression are reviewed; these included three females and three males, with an average age of 8 years (range 7 months to 30 years). The mean duration of symptoms prior to intervention was 1.9 years. Symptoms included occipitocervical pain, ataxia, incontinence, apnea, and respiratory arrest. Radiological investigations consisted of plain films with flexion and extension views, pluridirectional tomography, thin-section computerized tomography, and magnetic resonance imaging. Typical findings included marked foramen magnum stenosis, ventrolateral cervicomedullary junction compression secondary to central and paramesial basilar invagination, and dorsal cervicomedullary junction compression secondary to ligamentous hypertrophy and invagination of the posterior atlantal arch. All patients underwent posterior fossa decompression and atlantal laminectomy. Surgery consistently revealed marked dorsal and paramesial overgrowth of the rim of the rim of the foramen magnum, with thickening and invagination of the atlantal posterior arch and a dense fibrotic epidural band resulting in dorsal cervicomedullary compression. Intraoperative ultrasonography was used to determine the extent of decompression required. Three patients required duraplasty. Three patients had concurrent hydrocephalus, two of whom had undergone ventriculoperitoneal shunting prior to surgical decompression of the posterior fossa. One patient developed a pseudomeningocele postoperatively, requiring serial lumbar punctures before it resolved. No patient developed craniovertebral instability following decompression. Improvement or resolution of symptoms was noted in all patients, with an average follow-up period of 4.8 years. Thus, cervicomedullary compression in patients with achondroplasia can be successfully treated with dorsal decompression of the craniovertebral junction. Dense epidural fibrotic bands are frequently noted in these cases and must be aggressively released to ensure satisfactory decompression.