Frederick H. Sklar

Posterior fossa syndrome: Identifiable risk factors and irreversible complications

Cerebellar mutism was first described by Rekate et al. in 1985 as a transient condition which occurs after posterior fossa operations in children. Posterior fossa syndrome (PFS) and cerebellar mutism are often used interchangeably in the literature. In our experience, we found cerebellar mutism to be a reversible component of a persistent neurologic syndrome. The cause and identifiable risk factors have not been clearly elucidated in the literature. To further characterize PFS, we reviewed 253 children with posterior fossa tumors who underwent surgical resection. We documented 20 cases of PFS (8%), 12 males and 8 females. Age ranged from 1.5 to 13 years (mean = 6.5). Of the 20, 16 were medulloblastoma, 3 ependymoma and 1 astrocytoma. There was a 21% incidence (16/76) of PFS in medulloblastoma of the posterior fossa. The incidence for ependymoma was 13% (3/24) and 1% (1/102) for astrocytoma. All 20 cases (100%) had brainstem involvement by the tumor. The most frequent postoperative findings included mutism, ataxia, 6th and 7th nerve palsies and hemiparesis. Mutism had a latency range of 1–7 days (mean = 1.7) and a duration of 6–365 days (mean = 69.2, median = 35). Although mutism resolved in all cases, the remaining neurologic complications which characterized our findings of PFS were rarely reversible. We describe potential risk factors for developing PFS after surgery with hopes of making neurosurgeons more aware of potential problems following the removal of lesions in this area. Early recognition of PFS would further promote patient and family understanding and coping with this syndrome.

Cerebrospinal fluid dynamics in patients with pseudotumor cerebri

CSF dynamics were determined as a function of intracranial pressure in patients with pseudotumor cerebri. Servocontrolled variable rate lumbar infusions were used to determine net CSF-absorptive capacities and resting pressures in 10 patients; serial studies were done in 5 of the patients. Nearly all of the patients had abnormally low CSF-absorptive capacities. On the other hand, marked elevations in resting pressure were not a constant feature of the disease. Concurrent changes in the cerebrovascular bed could introduce errors into this manometric determination of CSF dynamics; the significance of this potential artifact is examined. The results of this study suggest that the CSF compartment may be of etiological importance in the pathophysiology of pseudotumor cerebri.

Surgical outcomes using bioabsorbable plating systems in pediatric craniofacial surgery

The purpose of this study was to evaluate the surgical outcomes of the 1.5-mm LactoSorb plating system (Walter Lorenz Surgical, Inc., Jacksonville, FL, U. S. A.) used to stabilize the osteotomized calvarial bone in pediatric patients who have undergone craniofacial surgery. The records of 33 consecutive pediatric patients who underwent craniofacial surgery from January 1997 through December 1997 were reviewed. There were 18 male and 15 female patients, and the age ranged from 4 months to 12 years. Patients were followed-up at 1 week, 1 month, 3 months, 6 months, and 12 months after surgery. For those patients reviewed, the following information is included: age, sex, diagnosis, surgical procedures, number and size of LactoSorb plates and screws used in each patient, operative difficulty of the screws and the heat pack, and postoperative complications, including wound healing, palpability, and infection. The LactoSorb plating system was used to stabilize the osteotomized calvarial bones in 33 patients who were diagnosed with: 1) craniosynostosis, 2) hydrocephalus, 3) fibrous dysplasia, or 4) cranial deformation. Orbital rim advancement and anterior cranial vault reshaping were performed in 17 patients. Posterior cranial vault reshaping, orbital rim advancement, and anterior cranial vault reshaping were performed in eight patients. Posterior cranial vault reshaping only was performed in seven patients. Excision of fibrous dysplasia from temporal bone was performed in one patient. One patient had a postoperative wound infection, and LactoSorb plates were palpable postoperatively in four patients. The LactoSorb plating system provided adequate rigidity for stabilizing the osteotomized calvarial bone during surgery and maintained adequate rigidity after surgery during the bone healing period before absorption. This plating system showed satisfactory results in pediatric craniofacial surgery patients.

Robotically guided radiosurgery for children

A robotically guided linear accelerator has recently been developed which provides frameless radiosurgery with high precision. Potential advantages for the pediatric population include the avoidance of the cognitive decline associated with whole brain radiotherapy, the ability to treat young children with thin skulls unsuitable for frame‐based methods, and the possible avoidance of general anesthesia. We report our experience with this system (the “Cyberknife”) in the treatment of 21 children.

Virruid evoked potentials in infants with hydrocephalus

Visual evoked potentials to flash stimuli were recorded in 15 infants with hydrocephalus. All demonstrated increased latencies for the prominent positive component (P2) of the response, compared to the mean values for age-matched controls. In nine infants studied prior to and 1 week after a shunt procedure, the P2 latency decreased.

The influence of thin-section tomography on the treatment of cervical spine injuries

A study was performed to determine the influence of routine thin-section tomography on subsequent treatment of the patient with acute trauma to the cervical spine. Of 79 such patients who underwent tomography over a 14-month period, 55 had suspicious indirect findings on the radiograph and 25% of these were shown to have fractures, while 67% of the patients without radiographic abnormalities who presented with a neurological deficit also demonstrated fractures on tomography. Of the patients who were shown to have fractures prior to tomography, 54% had more extensive injury than originally suspected. In nearly 18% of patients, the mode of therapy was changed significantly after tomographic evaluation. Indications for tomography are discussed and specific recommendations for systematic radiographic evaluation of cervical spine injuries are presented.

Subtemporal decompressions for shunt-dependent ventricles: Mechanism of action

The literature suggests that subtemporal decompression is an effective treatment for the syndrome of shunt-dependent ventricles because it allows the ventricle to enlarge around the shunt catheter tip. Pre- and post craniectomy ventricular areas were measured from computed tomography scans with a computer digitizing technique in three patients with this syndrome who had undergone four surgical procedures. All patients improved symptomatically following craniectomy. A significant decrease in total ventricular area was noted in all instances. The results suggest that subtemporal craniectomy causes the ventricles to become smaller, not larger. Other possible explanations for the effectiveness of the procedure are discussed.

Recirculatory spinal subarachnoid perfusions in dogs: a method for determining CSF dynamics under non-steady state conditions

Open-ended ventriculocisternal perfusion techniques for determining cerebrospinal fluid production and absorption rates are severely restricted by the absolute requirement that steady state conditions be present. A new closed recirculatory spinal perfusion technique is described. Because steady state equilibrium is not necessary, numerous determinations at multiple intracranial pressures or under varied experimental conditions are possible within relatively brief perfusion periods. Cerebrospinal fluid (CSF) and nondiffusible albumin tracer are rapidly recirculated through the spinal subarachnoid space in a cephalad direction. The concentration of fluorescein-tagged albumin is continuously monitored as the CSF is circulated through a fluorometric flow cell. Measured continuously, intracranial pressure (ICP) is regulated by changing the volume of the external perfusion circuit with a syringe pump connected in series to the recirculatory spinal perfusion. CSF formation and absorption rates are calculated from measurements of albumin concentration, concentration changes with time, ICP, syringe pump infusion rate, and the external perfusion circuit volume. In dogs, data can be collected after only 45 minutes for mixing; perfusions at four or five intracranial pressures in addition to normal resting pressure can be completed within 2 to 3 hours. The data from 15 perfusions in 14 dogs are presented. The method provides normal resting pressure values of CSF production and absorption consistent with those values in the literature determined by traditional ventriculocisternal perfusion techniques. Determinations at multiple intracranial pressures suggest a proportional relationship between absorption and ICP. No consistent acute change in CSF formation with pressures to 50 mm Hg can be inferred from these data.

Servo-controlled lumbar infusions: a clinical tool for the determination of CSF dynamics as a function of pressure.

The variable rate lumbar subarachnoid infusion technique allows rapid quantitative study of the cerebrospinal fluid (CSF) compartment. Numerous pressure plateaus are studied in a brief time period with a servo-controlled system. The test determines the difference between rates of CSF absorption and formation as a function of pressure. On-line computer data analysis increases experimental efficiency to guarantee statistical significance. Animal and patient data are presented in the report.

Thermoregulatory disorders after removal of a craniopharyngioma from the third cerebral ventricle

Aspiration and dissection of a cystic tumor from the third ventricle of a 3-year-old child resulted in several hypothalamic symptoms. One of the most persistent and troubling symptoms was marked hyperthermia which could not be traced to an infectious origin. The elevated temperature was not reduced by acetaminophen and there were abnormalities of circadian temperature cycles. In thermal stimulation tests the high body temperature was defended against induced cooling but the body temperature was raised above 40 degrees C without evoking physiological heat-loss responses and thermal discomfort. The unusual thermoregulatory status of this patient is similar to that seen in laboratory animals with hypothalamic lesions and to that observed with certain naturally occurring hypothalamic lesions in man. This dysthermia has been successfully treated with chlorpromazine.