Michael J. Fritsch

Endoscopic Aqueductoplasty and Stenting

OBJECTIVE This paper discusses indications for and the technique of endoscopic aqueductoplasty with stenting. METHODS We review and summarize the clinical experience with endoscopic aqueductoplasty gained over the last 15 years on pediatric and adult patients. RESULTS Endoscopic aqueductoplasty with stenting presents a safe procedure. In well selected patients, it provides a long-term, stable clinical course. Aqueductoplasty alone has a high reclosure rate and should be avoided. CONCLUSIONS Aqueductoplasty with stenting is the procedure of choice for the treatment of isolated fourth ventricle. Membranous and tumor-related aqueductal stenosis should be treated by endoscopic third ventriculostomy.

A History of Ventricular Neuroendoscopy

OBJECTIVE To describe the history of neuroendoscopy through the history of the major neurosurgeons who worked and published in the field. METHODS All relevant data described in publications before 1980 about the history of neuroendoscopy and found through OVID MEDLINE searches and related references are reported. RESULTS Contributions of 14 neurosurgeons who were pioneers in neuroendoscopy are described in chronologic order: Lespinasse, Dandy, Payr, Mixter, Fay, Grant, Volkmann, Putnam, Dereymacker, Scarff, Feld, Guiot, Fukushima, and Griffith. CONCLUSIONS An historical review of ventricular neuroendoscopy remains by essence incomplete. Medical technical progress proceeds by leaps and bounds, related to the ingenuity of surgeons able to understand rapidly the value of a technical change to improve their surgical procedure. The ability to remain attentive to patients and evolving pathologies as well as the evolution of modern technology is required to make further progress in neuroendoscopy.

Primary diffuse leptomeningeal gliomatosis in a 2-year-old girl

BACKGROUND Primary diffuse leptomeningeal gliomatosis is a rare tumor disease affecting the leptomeninx of the CNS. It is thought to originate from subarachnoidal glial cell nests. The symptoms are often rather unspecific. Surgery is not an option and despite the use of chemotherapy and radiotherapy patients rarely survive for more than 12 months. CASE DESCRIPTION We present a case of PDLG in a 2-year-old girl, the youngest patient reported on so far. She presented with increasing somnolence, intermittent strabism, and vomiting. Magnetic resonance imaging of the cranium showed enlarged ventricles and contrast enhancement of the basal leptomeninx. Cerebrospinal fluid diagnostic studies showed a mild pleocytosis and elevated protein levels but no tumor cells or evidence of infection. A ventriculoperitoneal shunt was placed and a biopsy of the leptomeninx was taken in the right Sylvian fissure. The histopathology findings suggested a nonspecific meningeal inflammation. Because the girl developed spinal symptoms with paresthesia and hyperalgesia, a spinal MRI was performed which showed a similar contrast enhancement of the spinal leptomeninx. A spinal biopsy was taken and subsequently a paucicellular astrocytic tumor was diagnosed corresponding to a WHO I diffuse leptomeningeal gliomatosis. Chemotherapy with vincristine, carboplatin, and etoposide was initiated (Protocol SIOP-LGG 2004) but was stopped by the parents when the child was in partial remission after 50 weeks because of a neurologic deterioration. The girl has so far survived for more than 29 months. CONCLUSION Primary diffuse leptomeningeal gliomatosis must be included in the differential diagnosis of diffuse leptomeningeal contrast enhancement in young children. There are promising treatment options that need to be carefully evaluated.

Unusual mesencephalic developmental venous anomaly causing obstructive hydrocephalus due to aqueductal stenosis

Developmental venous anomalies (DVAs) are benign vascular malformations that rarely become symptomatic. They are anatomical variations of the venous drainage system and most are incidentally discovered. Mechanical (obstruction and compression of cerebral and neural structures) and flow-related pathological mechanisms have been described in rare cases of symptomatic DVAs. The authors present the case of a 10-month-old boy with a mesencephalic DVA compressing the aqueduct and causing occlusive hydrocephalus. Endoscopic inspection confirmed the venous malformation causing aqueductal stenosis. The authors successfully performed endoscopic third ventriculostomy, resulting in decrease in the size of the ventricles. At the 6-month follow-up after surgery, the patient had significantly progressed in his psychomotor development. One year postsurgery the patient is doing fine, with no neurological or developmental deficits.