Mark S. O'Brien

Abdominal Metastases of Primary Intracranial Yolk Sac Tumors through Ventriculoperitoneal Shunts: Report of Three Cases

Three cases of rare primary intracranial yolk sac tumor are reported. Two cases had a pineal location, whereas the third presented as a suprasellar mass. After the placement of ventriculoperitoneal shunts for relief of hydrocephalus, all of the patients developed metastases restricted to the peritoneum, as demonstrated by autopsy in one patient (Case 1) and clinical and radiographic evidence in two patients (Cases 2 and 3). The peritoneal metastases were directly associated with the death of one patient, but were successfully treated with chemotherapy in another patient, who is still alive more than 2.5 years after initial presentation. The value of ascitic fluid cytology and alpha-fetoprotein determination in the diagnosis of this complication was demonstrated in one patient. These cases emphasize the need for awareness of this mode of metastasis and its potentially lethal effect.

Recurrent intramedullary enterogenous cyst of the cervical spinal cord

Intramedullary enterogenous cysts of the cervical spinal cord are rare. We report a case of symptomatic recurrence of this type of cyst 8 years following its original surgical treatment.

Benign Hemangioendothelioma Involving the Central Nervous System“Strawberry Nevus” of the Neuraxis

Benign hemangioendothelioma is rarely included in discussions of vascular neoplasms involving the central nervous system, whereas it is a well-defined entity outside the neuraxis. We present four cases of benign hemangioendothelioma, including an infantile hemangioendothelioma with venous drainage into the transverse dural sinus, an intracranial hemangioendothelioma adherent to the falx cerebri, a spinal epidural hemangioendothelioma causing spinal cord compression, and a parasellar hemangioendothelioma. Although hemangioendothelioma resembles hemangioblastoma in some ways, it is a distinct entity differing from the other vascular lesions involving the central nervous system.

Intracranial extracerebral glioneural heterotopia

A newborn baby girl with intracranial extracerebral glioneural (brain) heterotopia in the right frontoparietal area is described. The heterotopic brain was predominantly composed of neuronal and glial elements, with partial cerebellar differentiation. It also contained well-differentiated adipose tissue. The possible mechanism for its origin and the causes of central nervous system heterotopia are discussed.

Predictors of the need for cerebrospinal fluid diversion in patients with myelomeningocele

OBJECT Many patients with myelomeningocele (MMC) develop hydrocephalus, and most will undergo CSF diversion. The goal of this retrospective study was to determine whether there was a change in the shunt rate over the 7 consecutive years of the study. The authors will also identify the criteria used to determine the need for shunt placement. METHODS During a 7-year period, 73 patients underwent MMC closure at Arkansas Childrens Hospital. The shunt rate for each year was calculated. Clinical characteristics were evaluated, including apneic and bradycardic spells, CSF leak, level of the MMC, head circumference, and rate of head growth. In addition, radiological images were reviewed, and the frontooccipital horn ratio (FOHR), ventricular index (VI), and thalamooccipital distance (TOD) were calculated. Comparisons were made between those patients who underwent shunt placement and those who did not. RESULTS One patient was excluded due to death in the perinatal period. Of the 72 remaining patients, 54 (75%) underwent placement of a ventriculoperitoneal shunt. This rate did not change significantly over time. Between the cohorts with and without a shunt there was no significant difference in age, sex, or race. There was no significant difference in apneic episodes or bradycardic episodes. There was a statistically significant difference in fontanelle characteristics, head circumference at birth, and rate of head growth. Patients who required CSF diversion had a mean head growth of 0.32 cm/day compared with those who did not receive a shunt (0.13 cm/day; p < 0.05). All radiological parameters were found to be statistically significant. CONCLUSIONS In this study, several classic indicators of hydrocephalus in the neonate were not found to be significantly associated with the need for CSF diversion. Fontanelle characteristics, head circumference at birth, and head growth velocity were associated with the need for shunt placement. Imaging information including the VI, TOD, and FOHR are statistically significant measures to evaluate prior to placement of a ventriculoperitoneal shunt. The optimal patient with MMC for CSF diversion will have full to tense fontanelle, increasing head circumference of more than 3 mm/day, and radiological evidence of an elevated VI, TOD, and/or FOHR.

Systematic approach for detection of endocrine disorders in children treated for brain tumors

Endocrine dysfunction can be challenging to diagnose in children treated for brain tumors. Treatments are available for hormonal replacement and when necessary, hormonal suppression. Without these endocrine treatment regimens, life can be unnecessarily difficult or unpleasant. An endocrine survey can be used to screen at-risk neuro-oncology patients once or twice a year to facilitate the recognition of endocrine dysfunction. It is hoped that through the use of a routine screening program, physicians will be able to diagnose and begin treatment of endocrine problems in a time-efficient manner.

Metrizamide cisternography and the management of the chiari II malformation

A case of Arnold-Chiari malformation (Chiari type II) is reported to describe the usefulness of metrizamide cisternography in the diagnosis and management of this condition.