Cormac O. Maher and Liliana Goumnerova
The aim of this study was to report the long-term outcomes of patients receiving endoscopic ventriculocystocisternostomy (VCC) for suprasellar arachnoid cysts (SACs), and to analyze all published reports on outcomes of ventriculocystostomy (VC) versus VCC to compare the effectiveness of the 2 techniques.
Eleven consecutive patients with previously untreated SACs were surgically treated using endoscopic VCC. Another 2 patients were treated with VCC following ventriculoperitoneal shunt placement. Clinical imaging data were recorded. An analysis was performed of all published patient outcomes following endoscopic VC or VCC for an SAC.
Developmental delay and progressive macrocephaly were the most common preoperative symptoms. At a mean clinical follow-up interval of 63 months, 10 of 11 patients undergoing primary VCC did not require reoperation. An analysis of the literature suggests that VCC may be more effective than VC. Of the 44 reported patients that underwent VC as a first treatment, 7 (16%) required reoperation, and 7 (8%) of 86 patients who underwent VCC as a first treatment required reoperation. When VC or VCC was performed following a prior surgical procedure, 4 of 11 patients undergoing VC had a treatment failure requiring reoperation. In contrast, only 2 of the 17 reported cases of VCC following a prior procedure required further treatment. The difference in reoperation rates following either primary or secondary VC was significantly higher than following primary or secondary VCC (p = 0.04).
The authors conclude that VCC is an effective and durable treatment for symptomatic SACs in most cases.
Edward S. Ahn and Liliana Goumnerova
Endoscopic biopsy is an important minimally invasive method of diagnosis in the initial management of lesions in children with intraventricular and periventricular tumors. The procedure can be performed in conjunction with CSF-diverting procedures for obstructive hydrocephalus. The authors present their single-institution experience in a predominantly pediatric series with respect to diagnostic efficacy, utility in guiding treatment plans, and safety.
A retrospective review was conducted in a consecutive series of patients who underwent endoscopic biopsy of brain tumors during a 13-year period.
There were 33 endoscopic biopsies in 31 patients (16 males and 15 females). The average age of the patients was 11.3 years, with a mean follow-up duration of 2.4 years. The majority of biopsies were performed in conjunction with CSF-diverting procedures, such as endoscopic third ventriculostomy or fenestration of the septum pellucidum. Overall, 23 (70%) of 33 biopsies were diagnostic, with results that directed subsequent treatment. When stratified by tumor location, biopsy samples obtained in the lateral ventricle or pineal region were more favorable toward a successful diagnosis than those in the thalamus or tectal region. In 4 cases, elevated CSF tumor marker levels led to modification of the diagnosis and appropriate adjustment of treatment. The endoscopic third ventriculostomy success rate was 82.4%. There were 2 major complications (6.1%), which resulted in neurological deficits. There were no procedure-related deaths.
Endoscopic biopsy is an effective means of diagnosis of brain tumors in children. The diagnostic power may be more favorable with tumors in the lateral ventricle or pineal region. Collection of CSF during the procedure for tumor marker analysis is an integral component of diagnosis.
Paul Klimo Jr. and Liliana C. Goumnerova
The authors retrospectively reviewed the charts of all patients harboring brainstem tumors treated at their institution, excluding those with tectal gliomas, who underwent an endoscopic third ventriculocisternostomy.
Endoscopic third ventriculocisternostomy was performed in 13 patients with tumors involving the brainstem: nine patients with diffuse pontine gliomas, two with posterior fossa ependymomas, one with a cervicomedullary tumor, and one with a pontine primitive neuroectodermal tumor. No technical difficulties attributable to the location of the tumors or surgery-related complications were encountered. Immediate symptomatic relief of hydrocephalus was achieved in all patients, and there was an associated decrease in steroid and analgesic agent requirements. Only one patient eventually required a shunt.
Endoscopic third ventriculocisternostomy can be used in the terminal treatment of patients with brainstem tumors, yielding good results without significant surgical morbidity.
Mark W. Kieran, Liliana C. Goumnerova, Michael Prados and Nalin Gupta
Satoru Takeuchi and Yoshio Takasato
Craig D. McClain, Sulpicio G. Soriano, Liliana C. Goumnerova, Peter M. Black and Mark A. Rockoff
✓The authors report unanticipated intraoperative intracranial hemorrhaging in two pediatric neurosurgical patients. Both children were undergoing elective craniotomies with the aid of intraoperative magnetic resonance (iMR) imaging. In both cases, the ability of iMR imaging to aid in diagnosis allowed prompt and definitive treatment of potentially life-threatening complications. These cases illustrate the ability of iMR imaging to aid in differentiating unexpected and/or unexplained intraoperative events in pediatric neurosurgery.
Emily B. Ridgway, Alexander E. Ropper, John B. Mulliken, Bonnie L. Padwa and Liliana C. Goumnerova
Complications of Le Fort III midfacial advancement include CSF rhinorrhea, meningitis, and ocular and cerebral injury. This report reviews the anatomy of the Le Fort III osteotomies and their relevance to the unusual complication of meningoencephalocele.
In this report, a young male patient with Crouzon syndrome underwent subcranial midfacial advancement at the age of 10 years for obstructive sleep apnea and ocular exposure. He presented 4 years later complaining of nasal obstruction. On physical examination, a mucous-covered mass was noted in the left upper nasal vault medial to the turbinates. Computed tomography scanning and MR imaging confirmed the diagnosis of frontoethmoidal meningoencephalocele. Repair of the meningoencephalocele was accomplished using a combined neurosurgery and plastic surgery approach.
Meningoencephalocele is a rare complication of subcranial midfacial advancement. The abnormal anatomy of the anterior cranial base in patients with syndromic craniosynostosis places them at greater risk for fracture of the cribriform plate and dural tears during this procedure. Unrecognized dural injury is the etiology of this complication in this young patient; however, elevated intracranial pressure may have been a confounding factor. Attention to the anatomy of the anterior cranial base, as seen on sagittal CT images, will aid in preventing this complication.
Sarah C. Jernigan, Jay G. Berry, Dionne A. Graham and Liliana Goumnerova
The purpose of this study was to compare the effectiveness of CSF diversion with endoscopic third ventriculostomy (ETV) versus shunt therapy in infants with hydrocephalus.
The authors conducted a retrospective analysis of 5416 infants 1 year of age or younger with hydrocephalus (congenital or acquired) in whom CSF diversion was performed using either ETV or shunt placement at 41 children's hospitals between 2004 and 2009. Data were obtained from the Pediatric Health Information Systems database. Surgical failure was defined as the need for a repeat diversion operation within 1 year of initial surgery. The authors compared failure rates of ETV and shunt, as well as patient demographics and clinical characteristics, using hierarchical regression according to treatment group.
During the period examined, 872 infants (16.1%) initially underwent ETV and 4544 (83.9%) underwent ventricular shunt placement. The median infant age was 37 days (IQR 11–122 days) for both ETV and shunt placement. More infants who underwent ETV rather than shunt placement were born prematurely (41.6% vs 23.9%, respectively; p < 0.01) and had intraventricular hemorrhage (45.4% vs 17.5%, respectively; p < 0.01). Higher operative failure rates at 1 year were observed in infants who underwent ETV as opposed to shunt surgery (64.5% vs 39.6%, respectively; OR 2.9 [95% CI 2.3–3.5], p < 0.01). After controlling for prematurity, intraventricular hemorrhage, and spina bifida, ETV remained associated with a higher risk of failure (OR 2.6 [95% CI 2.1–3.2]).
In infants with hydrocephalus, a greater 1-year CSF diversion failure rate may occur after ETV compared with shunt placement. This risk is most significant for procedures performed within the first 90 days of life. Further investigation of the need for multiple reoperations, cost, and impact of surgeon and hospital experience is necessary to distinguish which treatment is more effective in the long term.
Berndt P. Schmit, Patricia E. Burrows, Karl Kuban, Liliana Goumnerova and R. Michael Scott
✓ The authors report a unique case involving a 2-year-old child with idiopathic moyamoya disease who presented with cerebral infarctions and seizures. On initial evaluation, computerized tomography (CT) showed a left parietal infarct and angiograms demonstrated early moyamoya disease with no evidence of arteriovenous malformation (AVM). Approximately 9 years later, angiography and magnetic resonance (MR) imaging revealed an AVM centered on the same region of the left parietal lobe. Angiographic, CT, and MR images are presented that demonstrate the progression of moyamoya disease and de novo development of the AVM in the infarct site. The possible role of angiogenesis in the etiology of acquired AVMs and moyamoya disease is discussed.