Sonja Vulcu, Leonie Eickele, Giuseppe Cinalli, Wolfgang Wagner, and Joachim Oertel
Endoscopic third ventriculostomy (ETV) is the procedure of choice in the treatment of obstructive hydrocephalus. The excellent clinical and radiological success rates are well known. Nevertheless, very few papers have addressed the very long term outcomes of the procedure in very large series. The authors present a large case series of 113 patients who underwent 126 ETVs, and they highlight the initial postoperative outcome after 3 months and long-term follow-up with an average of 7 years.
All patients who underwent ETV at the Department of Neurosurgery, Mainz University Hospital, between 1993 and 1999 were evaluated. Obstructive hydrocephalus was the causative pathology in all cases.
The initial clinical success rate was 82% and decreased slightly to 78% during long-term follow-up. Long-term success was analyzed using Kaplan-Meier curves. Overall, ETV failed in 31 patients. These patients underwent a second ETV or shunt treatment. A positive impact on long-term success was seen for age older than 6 months, and for obstruction due to cysts or benign aqueductal stenosis. The complication rate was 9% with 5 intraoperative and 5 postoperative events.
The high clinical success rate in short-term and long-term follow-up confirms ETV’s status as the gold standard for the treatment of obstructive hydrocephalus, especially for distinct pathologies. The patient’s age and underlying pathology may influence the outcome. These factors should be considered carefully preoperatively by the surgeon.
Giuseppe Cinalli, Alessia Imperato, Giuseppe Mirone, Giuliana Di Martino, Giancarlo Nicosia, Claudio Ruggiero, Ferdinando Aliberti, and Pietro Spennato
Neuroendoscopic removal of intraventricular tumors is difficult and time consuming because of the lack of an effective decompression system that can be used through the working channel of the endoscope. The authors report on the utilization of an endoscopic ultrasonic aspirator in the resection of intraventricular tumors.
Twelve pediatric patients (10 male, 2 female), ages 1–15 years old, underwent surgery via a purely endoscopic approach using a Gaab rigid endoscope and endoscopic ultrasonic aspirator. Two patients presented with intraventricular metastases from high-grade tumors (medulloblastoma, atypical teratoid rhabdoid tumor), 2 with subependymal giant cell astrocytomas (associated with tuberous sclerosis), 2 with low-grade intraparaventricular tumors, 4 with suprasellar tumors (2 craniopharyngiomas and 2 optic pathway gliomas), and 2 with pineal tumors (1 immature teratoma, 1 pineal anlage tumor). Hydrocephalus was present in 5 cases. In all patients, the endoscopic trajectory and ventricular access were guided by electromagnetic neuronavigation. Nine patients underwent surgery via a precoronal bur hole while supine. In 2 cases, surgery was performed through a frontal bur hole at the level of the hairline. One patient underwent surgery via a posterior parietal approach to the trigone while in a lateral position. The endoscopic technique consisted of visualization of the tumor, ventricular washing to dilate the ventricles and to control bleeding, obtaining a tumor specimen with biopsy forceps, and ultrasonic aspiration of the tumor. Bleeding was controlled with irrigation, monopolar coagulation, and a thulium laser.
In 7 cases, the resection was total or near total (more than 90% of lesion removed). In 5 cases, the resection was partial. Histological evaluation of the collected material (withdrawn using biopsy forceps and aspirated with an ultrasonic aspirator) was diagnostic in all cases. The duration of surgery ranged from 30 to 120 minutes. One case was complicated by subdural hygroma requiring a subduro-peritoneal shunt implant.
In this preliminary series, endoscopic ultrasonic aspiration proved to be a safe and reliable method for achieving extensive decompression or complete removal in the management of intra- and/or paraventricular lesions in pediatric patients.
Giuseppe Cinalli, Christian Sainte-Rose, Isabelle Simon, Guillaume Lot, and Spiros Sgouros
This study is a retrospective analysis of clinical data obtained in 28 patients affected by obstructive hydrocephalus who presented with signs of midbrain dysfunction during episodes of shunt malfunction.
All patients presented with an upward gaze palsy, sometimes associated with other signs of oculomotor dysfunction. In seven cases the ocular signs remained isolated and resolved rapidly after shunt revision. In 21 cases the ocular signs were variably associated with other clinical manifestations such as pyramidal and extrapyramidal deficits, memory disturbances, mutism, or alterations in consciousness. Resolution of these symptoms after shunt revision was usually slow. In four cases a transient paradoxical aggravation was observed at the time of shunt revision. In 11 cases ventriculocisternostomy allowed resolution of the symptoms and withdrawal of the shunt.
Simultaneous supratentorial and infratentorial intracranial pressure recordings performed in seven of the patients showed a pressure gradient between the supratentorial and infratentorial compartments with a higher supratentorial pressure before shunt revision. Inversion of this pressure gradient was observed after shunt revision and resolution of the gradient was observed in one case after third ventriculostomy. In six recent cases, a focal midbrain hyperintensity was evidenced on T2-weighted magnetic resonance imaging sequences at the time of shunt malfunction. This rapidly resolved after the patient underwent third ventriculostomy.
It is probable that in obstructive hydrocephalus at the time of shunt malfunction, the development of a transtentorial pressure gradient could initially induce a functional impairment of the upper midbrain, inducing upward gaze palsy. The persistence of the gradient could lead to a global dysfunction of the upper midbrain.
Third ventriculostomy contributes to equalization of cerebrospinal fluid pressure across the tentorium by restoring free communication between the infratentorial and supratentorial compartments, resulting in resolution of the patient's clinical symptoms.
Carmine Mottolese, Alexandru Szathmari, Carole Ginguene, Emile Simon, and Anne Claire Ricci-Franchi
In this study the authors conducted a retrospective evaluation of the effectiveness of endoscopic aqueductoplasty, performed alone or accompanied by placement of a stent, in the treatment of an isolated fourth ventricle (IFV) in seven patients afflicted with loculated hydrocephalus after a hemorrhage or infection.
Seven children with symptomatic IFV and membranous aqueductal stenosis underwent endoscopic aqueductoplasty alone or combined with placement of a stent in the cerebral aqueduct. The mean age of the patients at the time of surgery was 10 months. The mean duration of follow up was 26 months. In all patients a supratentorial shunt had already been implanted, and in five patients neuroendoscopy had already been performed because other isolated compartments had been present inside the ventricular system. Aqueductoplasty alone was performed in three patients and aqueductoplasty and aqueductal stent placement in four. A precoronal approach was performed in five patients and a suboccipital approach in two. Signs and symptoms of intracranial hypertension resolved in all cases. Stent placement was successful in all five cases, resulting in clinical and neuroimaging-confirmed improvements in the IFV. Restenosis of the aqueduct occurred in two patients in whom stents had not been placed. In one of these patients restenosis was managed by an endoscopic procedure, during which the aqueduct was reopened and a stent implanted; in the other patient a shunt was placed in the fourth ventricle. Hydrocephalus was controlled by a single shunt in six cases (86%) and by a double shunt in one case.
Endoscopic placement of a stent in the aqueduct is more effective in preventing the repeated occlusion of the aqueduct than aqueductoplasty alone and should be indicated as the initial treatment in each case of compatible anatomy.
Paul D. Chumas, Giuseppe Cinalli, Eric Arnaud, Daniel Marchac, and Dominique Renier
✓ Cases of craniosynostosis usually fall into well-demarcated categories: those related to a syndrome or those identified by a combination of suture involvement and morphological appearance. Between 1976 and 1995, 53 (3.6%) of 1474 cases in the craniofacial databank were assessed and designated as nonsyndromic but unclassifiable. The records and radiological studies obtained in these patients were retrospectively analyzed and comparisons were made with patients classified in the databank as having simple craniosynostoses.
It proved possible to divide the formerly unclassifiable cases into two groups: those with “two-suture disease” (Group A) and a “complex” group (Group B) in which more than two sutures were affected. Group A consisted of 36 cases (68%) of patients presenting with clear evidence of simultaneous involvement of two sutures but with no progression over time to suggest a more diffuse pansynostosis. Suture involvement was as follows: 17 of 36 sagittal plus one coronal; seven of 36 sagittal and metopic; six of 36 sagittal plus one lambdoid; and six of 36 metopic plus one coronal. The only significant difference between the Group A cases and the cases of simple craniosynostoses was in the percentage requiring a second operation (24% vs. 5%, p < 0.0001).
Group B consisted of 17 cases in which the patients presented at a slightly earlier age (mean 1 year) with severe morphological changes and multiple suture involvement. At the time of surgery, six of 17 patients showed large areas of lacunae within the cranial vault, making craniectomy the only option. In Group B, 10 of 17 patients displayed bilateral lambdoid plus sagittal suture involvement resulting in marked occipital recession posteriorly, whereas anteriorly in six of these 10 patients there was a massive frontal bone associated with posteriorly located coronal sutures. In contrast, there were also four patients in Group B with bilateral coronal plus metopic involvement resulting in a small frontal bone. There was a trend toward a lower intelligence quotient and a worse morphological outcome in the patients in Group B, but again the only result attaining statistical significance when compared to the databank was the rate of second operation (37.5 vs. 5%, p < 0.0001).
“Two-suture synostosis” is a relatively straightforward condition and is treatable with standard craniosynostosis techniques. However, possibly as a result of surgical compromise when two sutures are involved, the rate of reoperation is far higher than in simple suture cases. In contrast, patients in the “complex” group presenting with severe multisuture involvement require a more tailor-made approach to their management that often entails a second procedure.
Illustrative cases and review of the literature
Giuseppe Cinalli, Christian Sainte-Rose, Arielle Lellouch-Tubiana, Guy Sebag, Dominique Renier, and Alain Pierre-Kahn
✓ Over the past 15 years, eight children affected by intramedullary low-grade gliomas associated with hydrocephalus were treated at l'Hôpital des Enfants Malades. In all cases the diagnosis of hydrocephalus was made prior to that of the spinal tumor. Neuroradiological examination of all patients revealed contrast enhancement of the intracranial subarachnoid spaces. In six cases this was progressive, suggesting subarachnoid spread of the tumor, which was confirmed in two cases by histological examination.
The authors analyzed 38 cases of intramedullary low-grade glioma associated with hydrocephalus that were reported in the literature. Fifteen of the cases had intracranial leptomeningeal seeding. Several hypotheses have been proposed to explain this unusual association, such as 1) increase in cerebrospinal fluid (CSF) viscosity because of elevated fluid protein content; 2) obliteration of the cisterna magna due to a rostral extension of the tumor; and 3) blockage of the spinal subarachnoid pathways of CSF resorption. Two other theories seem of particular interest. Bamford and Labadie suggested that the abnormal presence of fibrinogen in the CSF and its transformation into fibrin at the level of the basal cisterns and Pacchioni's granulation may alter CSF hydrodynamics. This mechanism alone is sufficient to induce hydrocephalus of the communicating type. In addition, as suggested by Maurice-Williams and Lucey, the resulting leptomeningeal fibrosis might predispose secondary implantation of neoplastic elements in the subarachnoid spaces of the intracranial compartment.