Arthur E. Marlin

Assessment of quality of survival in children with medulloblastoma and cerebellar astrocytoma

To determine the quality of survival for children with posterior fossa tumors, comprehensive neuropsychological, behavioral, and academic assessment and physician ratings of functional status were obtained on 15 brain tumor patients (ages 6–19 years) at a median of 20 months postdiagnosis. More than 50% of the children (whether irradiated or not) experienced major problems in academic, motor, sensory, cognitive, and emotional function. All but two children were reported by teachers to be “slow workers,” and four of 15 patients were able to maintain their school work in regular classes. Although 80% of the patients were rated by physicians as having “excellent” or “good” functional status, no relationship was found between these global ratings and psychometric measures. Although the affected site was the posterior fossa, deficits also involved higher cortical function. These findings indicate the need for further evaluation of treatment effects and the provision of intervention for survivors.

Pseudocysts of the Abdomen Associated with Ventriculoperitoneal Shunts: A Report of Twelve Cases and a Review of the Literature

Shunt complications are reported to occur at a rate of approximately 26%. One of the less frequent but important complications is that of the pseudocyst. Since Harshs first mention of a periumbilical cyst associated with a shunt in 1954, 44 cases have been reported in the literature. These are reviewed in addition to 12 cases of our own. From the collected series several features about the etiology and management become apparent. The most common presentation is that of abdominal distension and/or pain rather than shunt malfunction. Diagnosis is then readily made with ultrasonography. Etiologically, it is evident that an inflammatory process is a frequent predisposing factor. In our series 16% had acute infection, 41.6% had a past history of CSF infection (6 months to 6.2 years), and 16% had CNS tumor although tumor cells were not isolated from the peritoneal cysts. Our management of the cyst itself was different from that reported in other series; it was found that the cyst reabsorbed spontaneously without excision or aspiration once the CSF was diverted. The peritoneal cavity could then be used for shunting once the cyst had reabsorbed. This sometimes required conversion to an atrial or pleural shunt before reutilization of the peritoneal cavity. There were no problems with cyst recurrence despite the conversion of 58% of the shunts to ventriculoperitoneal shunts with follow-up ranging from 3 months to 4 years. The mode of management of both the cyst and the hydrocephalus is reviewed.

Glioblastoma multiforme masquerading as a pleomorphic xanthoastrocytoma

Since its first description by Kepes in 1979, pleomorphic xanthoastrocytoma (PXA) has been considered a tumor with a benign course. Two cases are presented here that support the concept that PXA may be more accurately considered part of a spectrum of astrocytomas that occasionally may act aggressively. These cases represent astrocytomas with PXA components and are characterized by meningeal proximity, a high number of mitoses, and subsequently aggressive clinical behavior. The importance of recognizing the potential of a “benign” PXA to transform into a malignant entity has obvious implications for the therapeutic management of these tumors.

Changes in Regional Blood-flow and Water Content of Brain and Spinal Cord in Acute and Chronic Experimental Hydrocephalus

The effects of kaolin‐induced hydrocephalus on regional blood‐flow and water content of cat brain and spinal cord were measured. The rôle of the central canal of the spinal cord as an alternative pathway for cerebrospinal fluid in experimental hydrocephalus was also studied by positive contrast ventriculography.

Chronic headache in the shunt-dependent adolescent with nearly normal ventricular volume: diagnosis and treatment.

The shunt-dependent adolescent will occasionally manifest shunt malfunction by intermittent or chronic headache without any obvious signs of increased intracranial pressure (ICP). A small percentage of these patients will have nearly normal ventricular volume, making the diagnosis and treatment difficult. The authors present a comprehensive approach to this problem that uses computerized axial tomography (CT), ICP monitoring, positive contrast or isotope shunt scan, shunt revision, subtemporal craniectomy, or medical management as the individual situation dictates. The CT scan identifies patients with nearly normal ventricular volume. ICP monitoring then determines whether there is shunt malfunction. The shunt scan aids in localization of the malfunction. For distal obstruction, a simple revision is performed. When proximal shunt malfunction occurs, either subtemporal craniectomy or revision is carried out. It has been documented that occasionally ICP is episodically increased in the absence of shunt malfunction. This is secondary to abnormal brain compliance, and subtemporal craniectomy alone or in conjunction with corticosteroids is curative. This comprehensive approach is designed to provide a method for recognizing and treating intermittently increased ICP in the shunt-dependent child.

Choroid plexus tumors in childhood : response to chemotherapy, and immunophenotypic profile using a panel of monoclonal antibodies

Clinical and immunophenotypic (IP) data are presented on three children with choroid plexus (CP) tumors. Two children ages 0.2 and 2 years old with histologically proven malignant tumors had subtotal tumor resections and were treated with ten monthly cycles of eight‐drugs‐in‐1‐day chemotherapy without radiation therapy (XRT). Both are free of tumor 4 and 7 years later. The literature on survival of children with CP carcinomas after chemotherapy and XRT is reviewed. Monoclonal antibodies to 17 neuroectodermal, neuronal, glial, and leukocytic markers on frozen sections were used to IP the two malignant tumors and a CP papilloma. All tumors expressed two neuroectodermal markers (PI‐153/3 and UJ 223.8), cytokeratin 18, and a neural and leukocyte marker (Thy‐1). Two of three expressed neurofilament protein (NF‐H) and glial fibrillary acidic protein (GFAP) and one expressed NF‐M and common leukocyte antigen. None had strong expression for the panneuroectodermal antigen UJ13/A. There was variable expression of the other markers. The most common IP profile for CP tumors (cytokeratin 18+, PI‐153/3+, Thy‐1+, UJ 223.8+, and GFAP+ and UJ13A‐, UJ 127.11‐, and NF‐L‐) is discussed in the context of the current knowledge of the ontogenetic origin of the CP. It was concluded that chemotherapy for malignant CP tumors can be associated with long‐term survival in young children and that the unique IP profile of CP tumors with coexpression of three intermediate filaments suggests new and provocative evidence of their cellular complexity and heterogeneity.

The subcutaneous ventricular reservoir: an effective treatment for posthemorrhagic hydrocephalus

Use of the subcutaneous ventricular reservoir in the treatment of posthemorrhagic hydrocephalus was studied in a series of 38 patients. All of the patients were considered to be medically labile. Additionally, all had failed conservative modes of therapy consisting of lumbar punctures with or without furosemide or acetazolamide. Management of the hydrocephalus consisted of reservoir placement. Subsequently, taps were performed at various intervals and amounts, depending upon the degree of ventricular dilatation as determined by sonography and signs of increased intracranial pressure. The majority of reservoirs were left in place for 1–2 months. There were no reservoir infections. Once the patients were medically stable, the reservoir was removed and a shunt placed. Eight patients died before shunt placement and 2 patients died after shunting, reflecting a 29% mortality. In no case was a death related to the shunt, but rather reflected the medical lability of the patient population. Four patients (15% of surviving patients) did not require shunting. The total shunt infection rate was 6.9% (among survivors with a shunt in place, 7.7%). These results support the use of the reservoir as an easy and effective means of protecting the cortical mantle while decreasing morbidity related to future shunt placement.

A prospective evaluation of leak point pressure, bladder compliance and clinical status in myelodysplasia patients with tethered spinal cords.

We evaluated prospectively 26 patients with myelodysplasia and a tethered spinal cord to determine whether surgical release of the tethered cord positively influenced leak point pressure, bladder compliance, upper tract status and/or clinical management. Urodynamics were performed immediately before and after the neurosurgical procedure, and at 3 and 6 months postoperatively. Mean patient age was 7.8 years (range 2 days to 34 years) and median interval from onset of symptoms to surgery was 60 days (range 2 days to 4 years). Patient presentation included a combination of orthopedic, neurological and urological symptoms. Of 26 patients 9 (35%) had new hydronephrosis, urinary tract infections or urinary incontinence. Leak point pressure and bladder compliance did not change significantly by 6 months postoperatively. Of the 4 patients who presented with hydronephrosis 1 worsened in status, 2 stabilized and 1 improved. Clinical status was unchanged in 16 patients, improved in 4 and worsened in 6. There was no significant relationship between patient age and urodynamic or clinical outcome. Among patients followed for at least 6 months radiographic and clinical improvement occurred in 25% and 15%, respectively. Urodynamic improvements were transient. Surgical release of a tethered cord improved the urological status in less than a quarter of the patients in this series.

The Currarino Triad: Its Importance in Pediatric Neurosurgery

Currarinos triad is a hereditary condition diagnosed when three abnormalities are noted: (1) an anorectal malformation; (2) an anterior sacral defect, and (3) a presacral mass. This condition often presents with symptoms related to the presacral mass or with an incidental finding of a sacral anomaly on plain radiographs. Presented here are 2 cases of Currarinos triad noted in siblings, both of whom underwent surgery for anorectal malformations in infancy. The importance of early diagnosis and treatment of this condition and the need for genetic counseling are emphasized.

Subclavian steal syndrome

Three patients with central nervous system symptoms due to subclavian steal syndrome were treated with proximal vertebral to common carotid artery transposition. Neurological symptoms were relieved or improved in all three, with no decrease in blood pressure or pulse in the ipsilateral upper extremity. The colorful history of this syndrome is reviewed, and the various surgical approaches to its treatment are discussed. Although the literature suggests that the commonly used carotid to subclavian artery bypass graft and other similar extrathoracic procedures are generally safe and effective for relief of symptoms of the steal, there is also evidence that these bypasses may fail to restore antegrade flow in the vertebral artery, and, in fact, may steal from the carotid artery. Thus, the blood flow provided to the brain by these procedure may be hardly more than that provided by vertebral artery ligation, whereas the principal effect is to restore blood flow into the upper extremity. Vertebral artery ligation alone has been used in 20 patients, with neurological improvement in all cases and production of persistent intermittent brachial claudication in only one. These considerations and our patient experience suggest that a relatively simple operation, proximal vertebral to common carotid artery transposition, which emphasizes restoration of flow to the brain rather than to the upper extremity, may be preferable for most patients with neurological symptoms of subclavian steal syndrome.