Multilocular hydrocephalus usually requires placement of multiple ventricular catheters for the treatment of secondary cysts and intraventricular septation. The formation of strong adhesions can embed the catheters so that they cannot be removed without a higher risk of intraventricular hemorrhage. Moreover, the devices could represent a nidus for infection and a risk for formation and enlargement of intraventricular secondary cysts. Neuroendoscopy allows the surgeon safely to reach the ventricular catheters that have been left or lost in the ventricular cavity, and to remove them via a minimally invasive approach. In this paper, the authors document another application of ventricular endoscopy in the management of this kind of hydrocephalus. The removal of an unnecessary and no longer useful prosthetic device that is a possible focus of infection justifies endoscopic treatment, which, in this experience, is not associated with morbidity.
Benedetta Ludovica Pettorini, Paolo Frassanito, Gianpiero Tamburrini, Luca Massimi, Massimo Caldarelli and Concezio Di Rocco
Federica Novegno, Massimo Caldarelli, Antonio Massa, Daniela Chieffo, Luca Massimi, Benedetta Pettorini, Gianpiero Tamburrini and Concezio Di Rocco
Since the advent of MR imaging, an increasing number of asymptomatic or oligosymptomatic patients have been diagnosed with Chiari malformation Type I (CM-I). The decision of whether or not to operate is more difficult in these patients than in those with clear symptoms because of the lack of information about the natural course of this disease.
The authors report on their experience in a series of 22 patients with CM-I who were evaluated at the authors' institution, and for whom a conservative approach to treatment was adopted. The patients ranged in age from 1 to 16 years (mean 6.3 years) at diagnosis. Neuroradiological and complete clinical examinations were performed in all patients 6 months after the first observation and every year thereafter. The follow-up period ranged from 3 to 19 years (mean 5.9 years).
Chiari malformation Type I was incidentally detected on MR images in 11 of 22 patients. The remaining 11 patients had minimal clinical signs at presentation that were not regarded as necessitating immediate surgical treatment. Seventeen patients (77.3%) showed progressive improvement in their symptoms or remained asymptomatic at the last follow-up whereas 5 patients (22.7%) experienced worsening, which was mild in 2 cases and required surgical correction in the remaining 3 cases. On MR imaging a mild reduction in tonsillar herniation was appreciated in 4 patients (18.18%), with complete spontaneous resolution in 1 of these. In 16 patients, tonsillar herniation remained stable during follow-up.
The authors' data confirm the common impression that in both asymptomatic and slightly symptomatic patients with CM-I, a conservative approach to treatment should be adopted with periodic clinical and radiological examinations.
Luca Massimi, Pasquale De Bonis, Giuseppe Esposito, Federica Novegno, Benedetta Pettorini, Gianpiero Tamburrini, Massimo Caldarelli and Concezio Di Rocco
Scalp masses are not infrequently encountered in daily clinical practice. They are represented by a wide spectrum of different clinical entities and are usually managed by an excision or by simple observation. Although it happens rarely, head lumps may hide an underlying cranioencephalic malformation that has to be preoperatively diagnosed to perform an appropriate treatment.
Cerebral arteriovenous malformations (AVMs) are not included among the intracranial malformations connected with a scalp mass. The authors report on the unusual case of a child harboring a complex intracranial AVM that initially presented as a small scalp mass. Actually, this young boy came to the authors' attention just for a small, soft, pulsatile, and reducible mass of the vertex that produced a circumscribed bone erosion. The presence of macrocranium and venous engorgement of the face, however, suggested the presence of an intracranial “mass.” The neuroimaging investigations pointed out a temporal AVM causing dilation of the intracranial sinuses and ectasia of the vein of the scalp; one of the veins was appreciable as a lump on the vertex.
Benedetta L. Pettorini, Gianpiero Tamburrini, Luca Massimi, Massimo Caldarelli and Concezio Di Rocco
The intracystic injection of chemo- and radiotherapeutic agents was introduced for the treatment of craniopharyngioma to control tumor growth and to delay the potentially harmful effects of surgery or radiation therapy. The positioning of cyst catheters has been performed by means of direct vision, stereotactically guided insertion, and ultrasonographic and ventriculoscopic guidance. The insertion of a catheter into the cyst is not devoid of complications, with an incidence ranging up to 16%, independent of the surgical technique used.
Eight patients (mean age 25.8 years) with symptomatic cystic craniopharyngioma were treated by means of an endoscopic transventricular approach for the insertion of an intracystic catheter for intratumoral therapy with interferon-α.
A single right precoronal bur hole is made, and the frontal horn of the lateral ventricle is accessed under neuronavigation guidance. A ventricular catheter with an inserted stylet was advanced anterior to the endoscope sheath through the same cortical access as the endoscope and was guided under endoscopic view down to the cyst dome wall. The coagulated surface of the craniopharyngioma cyst was punctured and the tip of the ventricular catheter was advanced; the depth was established preoperatively on MR scans and confirmed by neuronavigation guidance. The proximal end of the cystic catheter was connected to an access chamber to be left in the subcutaneous space, and the endoscope was slowly retracted.
The authors' experience favors the use of neuroendoscopic positioning of intracystic catheters as safer than open and stereotactic approaches.
Sergio Cavalheiro, Concezzio Di Rocco, Sergio Valenzuela, Patricia A. Dastoli, Gianpiero Tamburrini, Lucca Massimi, Jardel M. Nicacio, Igor V. Faquini, Daniela F. Ierardi, Nasjla S. Silva, Benedetta Ludovica Pettorini and Silvia R. C. Toledo
The authors assessed the efficacy of intratumoral interferon-α (IFNα)–based chemotherapy in pediatric patients with cystic craniopharyngiomas.
In a prospective multicenter study of 60 pediatric patients, the authors assessed the efficacy of intratumoral INFα2A-based chemotherapy. The study was conducted between 2000 and 2009 at 3 locations: the Medical School of the Federal University of São Paulo, Catholic University of Rome, and the Neurosurgery Institute of Santiago, Chile. The assessment included clinical and radiological control examinations, side effects observed, and total dose used.
Sixty cases of cystic craniopharyngioma were analyzed. The cohort consisted of 35 male and 25 female children (mean age 11 years). Clinical and radiological improvement was achieved in 76% of the cases. New endocrinological deficits were observed in 13% of the cases. In approximately 30% of the patients, the evolution included some light side effects, the most common being headache (33%) and eyelid edema (28%). The number of cycles varied from 1 to 9 (mean 5 cycles), and the total dose applied per cycle was 36,000,000 IU.
This has been the largest documented series of intratumoral chemotherapy using INFα for the control of cystic craniopharyngiomas. The treatment has proved efficacious; there was no mortality, and morbidity rates were low.
Benedetta Ludovica Pettorini, Paolo Frassanito, Massimo Caldarelli, Gianpiero Tamburrini, Luca Massimi and Concezio Di Rocco
Craniopharyngioma has long been considered a benign tumor because of its pathological aspect. This primordial view of craniopharyngioma fit with the primitive treatment attempts based on blind resection of the tumor each time it recurred. The limits of this management strategy were proven early by the high morbidity related to the resection and recurrence risk despite radical lesion removal. Nowadays, craniopharyngioma must be considered a complex molecular disease, and a detailed explanation of the mechanisms underlying its aggressive biological and clinical behavior, despite some benign pathological features, would be the first step toward defining the best management of craniopharyngioma. Indeed, advances in the knowledge of the molecular mechanisms at the base of craniopharyngioma oncogenesis will lead to comprehension of the critical checkpoints involved in neoplastic transformation. The final research target will be the definition of new biological agents able to reverse the neoplastic process by acting on these critical checkpoints. This biological approach will lead to a refined therapy combining higher efficacy and safety with lower morbidity. In this paper the authors reveal state-of-the-art comprehension of the molecular biology of craniopharyngioma and the consequent therapeutic implications.