Isolated cervical juvenile xanthogranuloma in childhood.

STUDY DESIGN: This is a report of an exceptional case of isolated cervical juvenile xanthogranuloma in a child. OBJECTIVES: This case report draws attention to the fact that isolated xanthogranuloma of the central nervous system should be considered among possible diagnosis of subdural extramedullary spinal masses in children and young adults. SUMMARY AND BACKGROUND DATA: Isolated juvenile xanthogranuloma of the central nervous system is extremely rare. When located in the spinal canal it behaves like any extramedullary mass-occupying lesion. MRI depicts the tumor's association with adjacent structures. In cases in which a subtotal surgical removal was possible, radiotherapy has been indicated. METHODS: A three-year-old girl presented severe pain in the right shoulder and spastic tetraparesis. The MRI showed an intradural extramedullary mass homogeneously enhancing after DTPA-gadolinium infusion. Complete surgical removal of the tumor was performed through open-door laminoplasty. RESULTS: The child was pain free immediately after the surgical removal of the tumor. A gradual complete recovery of the neurologic deficits followed. Open-door laminoplasty provided sufficient operative space, and it minimized the impact on the growing spinal column. CONCLUSIONS: Isolated juvenile xanthogranuloma does not show any predilections of localization inside the central nervous system. Both intracranial and spinal juvenile xanthogranulomas appear isointense in MRI and enhance homogeneously with gadolinium. Whenever possible, total surgical removal alone seems to be curative. Otherwise, a subtotal removal of the tumor might be followed by radiotherapy. Immunohistochemical tests ensure the diagnosis.

Low-cost self-irrigating bipolar forceps.

A low cost method for providing continuous saline drip in bipolar diathermy is described, which use is adaptable to any type of standard bipolar forceps. The advantages of this system versus commercially available self-irrigating forceps are reported.

[Neuroradiology in cerebral gliomas]. / Neuroradiologia dei gliomi cerebrali.

CT Scan has greatly helped preoperative diagnosis in brain tumors. CT Scan alterations are found in about 95% of brain tumors and the most modern instruments can pick-up even small alterations, locate them precisely and, in 80% to 90% of the cases can give their exact extension. But precise CT-scan diagnosis in brain tumors can be done in only 80% of the cases when in a supratentorial site; about 90% in infratentorial localizations. Differential diagnosis difficulties occur with metastasis, abscesses, infarction, meningiomas, and vessel malformations. Angiography does not always solve these diagnostic problems left uncovered by CT Scan and not infrequently surgical proof is necessary.

[Radiotherapy of primary and secondary spinal tumors]. / La radioterapia dei tumori spinali primitivi e metastatici.

Radiotherapy in primary and metastatic spinal cord tumors following surgical treatment, or even without surgery, is very effective. The Authors analyse the different types of spinal tumors giving the survival time for every one. The results are related to the possible medullary damage before radiotherapy and to the sensibility of the tumor itself. The Authors emphasize the importance of two factors: 1) early diagnosis (myelography); 2) immediate therapy (high dose steroids, surgical decompression and/or radiotherapy).

[Radiotherapy of brain tumors in childhood]. / Radioterapia dei tumori cerebrali nell'infanzia.

External radiotherapy with high energy photons in children's brain tumors improves results of surgical treatment according to their histology. Radiotherapy treatment is a decisive factor in survival time and quality of life. Undifferentiated tumors with high radiosensitivity are treated when radiotherapy is the only means for recovery; tumors presenting histologically low malignancy in which effective radiotherapy treatment is uncertain and the risk of radiotherapy damage is the predominant factor. Radiotherapists who treat children's brains which in many cases are not yet totally myelinized , must have good technical ability. Literature and personal experience show that in medulloblastomas irradiation of the entire central nervous system associated with chemotherapy gives a survival time of 5 years in 50% of the cases; in glioblastomas and anaplastic astrocytomas, fortunately rare in children, results are poor; in brainstem tumors the association of the extracranial shunt to radiotherapy has improved results of survival time at 5 years to 50%. A good quality of life is frequent and radiological effects are rare. It is clear that radiotherapy has reached its maximum. Progress is still to be made thanks to discoveries of more effective radiosensitizer or radioprotector compounds.

Skull changes and intellectual status in hydrocephalic children following CSF shunting.

A total of 76 shunt-treated hydrocephalic children were examined at least once between two and 12-years after surgery to determine what skull changes had occurred and to assess the children's intellectual status. The high incidence of skull changes in shunt-treated, non-tumoral, hydrocephalic children was confirmed. A strict relationship existed between skull changes and low intracranial pressure. The results indicate that impairment of mental development parallels the degree of skull changes, suggesting a close relationship between impaired mental development and cerebrospinal fluid hypotension.

Vertebral artery dissection in a child. Is "spontaneous" still an appropriate definition?

Though a rare cause of stroke in the general population, in almost one quarter of young patients affected by stroke cervical artery dissection (CAD) is the underlying cause. Among these cases "spontaneous" dissections, intended as non-traumatic, represent about 34% of posterior circulation arterial dissection in patients aged less than 18 years. We here describe the case of a seven-year-old boy who developed a spontaneous vertebral artery dissection (VAD) leading to occipito-mesial, thalamo-capsular and cerebellar infarction. Once a traumatic origin was excluded, clinical history and laboratory findings were further analysed: fever associated with tonsillitis during the previous week, raised inflammatory indices, a throat culture positive for beta haemolysing Streptococcus group A and high titres of streptococcal antibodies were found. This case suggests that patients with CAD referred as spontaneous deserve extensive analysis. Subjects presenting with a dissection and an underlying infection are likely to have a hyperinflammatory response (although further experience is needed). In these patients immediate start of antibiotic therapy, treatment with anti-inflammatory drugs and further a lifelong prophylaxis with antibiotics before any invasive procedure are strongly recommended.