Reversible intracranial hypertension following treatment of an extracranial vascular malformation: case report.

Pediatric hydrocephalus is a well-studied and still incompletely understood entity. One of the physiological means by which hydrocephalus and intracranial hypertension evolve is through perturbations to normal vascular dynamics. Here the authors report a unique case of an extracranial vascular anomaly resulting in persistently elevated intracranial pressures (ICPs) independent of CSF diversion in a patient with a Joubert syndrome-related disorder. The patient developed worsening intracranial hypertension after successful CSF diversion of Dandy-Walker malformation-associated hydrocephalus via endoscopic third ventriculostomy-choroid plexus cauterization (ETV/CPC). Vascular workup and imaging revealed an extracranial arteriovenous fistula of the superficial temporal artery at the site of a prior scalp intravenous catheter. Following microsurgical obliteration of the lesion, ICP normalized from > 30 cm H2O preoperatively to 11 cm H2O postoperatively. A repeat lumbar puncture at 4 months postoperatively again demonstrated normal pressure, and the patient remained asymptomatic for 9 months. Recurrent symptoms at 9 months were attributed to inadequate CSF diversion, and the patient underwent ventriculoperitoneal shunt placement. This is the first report of an extracranial-to-extracranial vascular anastomosis resulting in intracranial hypertension. This case report demonstrates the need to consider extracranial vascular anomalies as potential sources of persistently elevated ICP in the syndromic pediatric population.

Using urinary bFGF and TIMP3 levels to predict the presence of juvenile pilocytic astrocytoma and establish a distinct biomarker signature.

OBJECTIVE The authors report the use of urinary biomarkers as a novel, noninvasive technique to detect juvenile pilocytic astrocytomas (JPAs), capable of distinguishing JPAs from other CNS diseases, including other brain tumors. Preliminary screening of an array of tumors implicated proteases (including matrix metalloproteinases [MMPs]) and their inhibitors (tissue inhibitors of metalloproteinase [TIMPs]) as well as growth factors (including basic fibroblast growth factor [bFGF]) as candidate biomarkers. These data led the authors to hypothesize that tissue inhibitor of metalloproteinase 3 (TIMP3) and bFGF would represent high-probability candidates as JPA-specific biomarkers. METHODS Urine was collected from 107 patients, which included children with JPA (n = 21), medulloblastoma (n = 17), glioblastoma (n = 9), arteriovenous malformations (n = 25), moyamoya (n = 14), and age- and sex-matched controls (n = 21). Biomarker levels were quantified with enzyme-linked immunosorbent assay, tumor tissue expression was confirmed with immunohistochemical analysis, and longitudinal biomarker expression was correlated with imaging. Results were subjected to univariate and multivariate statistical analyses. RESULTS Using optimal urinary cutoff values of bFGF > 1.0 pg/µg and TIMP3 > 3.5 pg/µg, multiplexing bFGF and TIMP3 predicts JPA presence with 98% accuracy. Multiplexing bFGF and MMP13 distinguishes JPA from other brain tumor subtypes with up to 98% accuracy. Urinary biomarker expression correlated with both tumor immunohistochemistry and in vitro tumor levels. Urinary bFGF and TIMP3 decrease following successful tumor treatment and correlate with changes in tumor size. CONCLUSIONS This study identifies 2 urinary biomarkers-bFGF and TIMP3-that successfully detect one of the most common pediatric brain tumors with high accuracy. These data highlight potential benefits of urinary biomarkers and support their utility as diagnostic tools in the treatment of children with JPA.

Posterior thoracic laminoplasty with dorsal, intradural identification of ventral defect and transdural discectomy for a spontaneous cerebrospinal fluid leak: case report.

Spontaneous intracranial hypotension (SIH) has been increasingly reported in the literature concomitant with the improved sensitivity of imaging modalities. Although typically associated with meningeal weakening, a handful of cases of SIH secondary to thoracic disc osteophytes have been reported. Five of 7 reported cases were treated with epidural blood patch (EBP) alone while 2 required surgical management. There is no standard operative approach; both anterior and posterolateral approaches can be cumbersome and associated with morbidity, particularly in young, healthy patients. The authors report a case of SIH in which a ventral dural tear secondary to a calcified thoracic disc was repaired via posterior thoracic laminoplasty with dorsal durotomy and intradural exposure of the ventral defect with transdural discectomy followed by primary closure. A 34-year-old man presented with low-pressure headaches following axial load injury from a ski accident 5 years earlier. The patient's symptoms were refractory to a trial of conservative treatment and EBP, and he developed bilateral upper-extremity paresthesias. MRI of the spine demonstrated an extrathecal collection spanning the thoracic spine, and dynamic CT myelography identified contrast extravasation adjacent to a calcified paramedian disc at T9-10. The patient underwent posterior laminoplasty with neuromonitoring. A ventral dural defect was visualized via a dorsal durotomy, the penetrating disc osteophyte was removed transdurally, and the ventral and dorsal dura maters were closed primarily. Both somatosensory and motor evoked potentials were unchanged during surgery. The patient has remained asymptomatic more than 10 months postoperatively and he has resumed work as a surgeon. Cases of SIH secondary to a calcified thoracic disc are rare with little precedent as to optimal surgical intervention. This case illustrates the potential usefulness of posterior laminectomy in nonmyelopathic patients in whom there is no evidence of canal compromise and for whom neuromonitoring is available. Additionally, surgeon experience and patient preference may guide surgical planning.