PURPOSE: To determine if the degree of tumor cell apoptosis at diagnosis predicts outcome, tissue sections of medulloblastoma were examined and the amount of apoptosis and progression-free survival were correlated. PATIENTS AND METHODS: The study cohort consisted of 43 children in whom medulloblastoma was diagnosed between 1984 and 1995: 29 patients at high risk (HR) treated with radiation and chemotherapy, and 14 children at low risk (LR) treated with radiation alone. A terminal deoxynucleotidyl transferase (TdT) end-labeling assay was used to detect apoptosis in paraffin-embedded tissue sections prepared at diagnosis. RESULTS: Progression-free survival was examined in cohorts of children whose tumors were divided into quartiles based on the apoptotic index (AI) of their pretreatment tumor specimens. A comparison of these four groups of children revealed an association between AI and outcome (p = 0.03); patients with tumors in the highest AI quartile had substantially improved outcome compared to all other patients combined (p = 0.02). In this cohort of patients treated with different therapies, assignment at the time of diagnosis to LR and HR groups based on widely-accepted clinical criteria was not closely associated with outcome (p = 0.47). CONCLUSION: AI is a strong indicator of treatment outcome for children with medulloblastoma after treatment with cytotoxic therapy, independent of risk group. Because HR and LR patients included in this study received different modalities of cytotoxic therapy, it is possible that AI predicts outcome independent of the precise antineoplastic therapy a patient receives.

Adolescent↗

PubMedDate not supplied

Cervical neuroschisis and meningocoele manque in type I (no neck) Klippel-Feil syndrome.

A male child with type I (no neck) Klippel-Feil syndrome presented at birth with a transient and partial cord injury. Investigations failed to reveal spinal instability or foramen magnum compression. Subsequent deterioration led to investigations that demonstrated neuroschisis. A cervical meningocoele manque with cord tethering was found at exploration and untethering reversed the deficit. The surgical pathology of the cervical cord is described.

Cervical Vertebrae↗

PubMedDate not supplied

The application of i.v. digital subtraction angiography to cranial disease in children.

All intracranial IV digital subtraction angiographic examinations performed over the past 2 years were reviewed retrospectively to ascertain the uses and limitations of this technique for the evaluation of pediatric intracranial disease. Of the various abnormalities studied, this imaging technique was particularly useful in diagnosing venous and dural sinus abnormalities; in screening for suspected large aneurysms, vascular malformations, and major arterial occlusive disease; and in preoperative vascular mapping. IV digital subtraction angiography has selected usefulness in confirming brain death, in evaluating cerebral ischemia, in identifying vascular abnormalities underlying intracranial hemorrhage, and in evaluating vascularity and sinus extension of masses. The IV route for digital subtraction angiography is not useful in diagnosing segmental arterial occlusive or small-vessel disease, nor is it useful in preoperative localization of specific arterial supply to arterial venous malformations, aneurysms, or neoplasms. IV digital subtraction angiography can be performed successfully in children of all ages with minimal patient morbidity. For most patients, the diagnostic information obtained was adequate without the need for standard cerebral arteriography.

Adolescent↗