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Benedetta Ludovica Pettorini, Luca Massimi, Alessandro Cianfoni, Giovanna Paternoster, Gianpiero Tamburini and Concezio Di Rocco

✓ Accessory human tails are uncommon malformations consisting of meningeal and neural structures and possibly occult spinal dysraphism (pseudotails). The thoracic location of a lipomeningocele constituting a pseudotail is very rare. The authors report the case of a young boy harboring a thoracic dorsal appendage consisting of a lipomeningocele and a dermoid cyst that was complicated by the presence of diastematomyelia, tethered spinal cord, syringomyelia, and mild hydrocephalus. The preoperative moderate ventricular dilation was decompressed by the surgical repair of the spinal malformation and required a specific treatment. The authors discuss the rare association of these clinical entities and conclude that complete spine and brain neuroimaging studies are necessary for the correct surgical planning of this kind of malformation.

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Benedetta Ludovica Pettorini, Paolo Frassanito, Gianpiero Tamburrini, Luca Massimi, Massimo Caldarelli and Concezio Di Rocco

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.

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Federica Novegno, Massimo Caldarelli, Antonio Massa, Daniela Chieffo, Luca Massimi, Benedetta Pettorini, Gianpiero Tamburrini and Concezio Di Rocco

Object

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.

Methods

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).

Results

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.

Conclusions

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.

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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.

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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.

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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

Object

The authors assessed the efficacy of intratumoral interferon-α (IFNα)–based chemotherapy in pediatric patients with cystic craniopharyngiomas.

Methods

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.

Results

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.

Conclusions

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.

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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.

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John Goodden, Barry Pizer, Benedetta Pettorini, Dawn Williams, Jo Blair, Mohammed Didi, Nicky Thorp and Conor Mallucci

Object

Optic pathway/hypothalamic gliomas (OPHGs) are generally benign tumors situated in an exquisitely sensitive brain region. The location and natural history of OPHGs has led to much debate about optimal treatment. This paper revisits the role of and optimal timing of debulking surgery in OPHG.

Methods

This paper presents a series of cases managed by the neuro-oncology team at Alder Hey Children's Hospital and a single surgeon. Data were collected retrospectively for periods prior to 2009 and prospectively thereafter. Tailored treatment strategies were used, including observation and combinations of surgery, chemotherapy, and radiotherapy. Tumor control rates and outcomes are reviewed.

Results

Forty-two patients were treated between 1998 and 2011. Their median age at diagnosis was 5 years 7 months. Nineteen patients were positive for neurofibromatosis Type 1 (NF1) and 23 patients were negative for NF1. The median duration of follow-up was 77 months (range 21.8–142.3 months). Presenting symptoms included visual impairment (in 50% of cases), headache (in 24%), and hypothalamic/pituitary dysfunction (in 29%).

Twenty-two debulking procedures were performed in 21 patients. Four biopsies (3 open, 1 endoscopic) were also performed. The histological diagnosis was pilocytic astrocytoma in 21 patients and pilomyxoid astrocytoma in 2 patients. Ten patients (Group 1) had primary surgical debulking alone and were then observed. Four patients (Group 2) had surgical debulking, plus planned chemotherapy within 3 months. Seven patients (Group 3) required surgical debulking for progressive disease following a variety of treatments. Patient age had the greatest impact on subsequent tumor progression.

In total, 13 patients received chemotherapy, 4 on initial presentation, 4 in combination with surgery, and 5 for further tumor progression. Five patients were treated with radiotherapy, 3 prior to referral to Alder Hey.

Eleven patients required shunt insertion for hydrocephalus. Vision was stabilized for 74% of patients. The number of patients with hypothalamic/pituitary dysfunction increased from 12 at presentation to 16 by the end of treatment. The overall survival rate was 93%. Three patients died—1 from tumor progression, 1 from infective complications from tumor biopsy, and 1 from a spontaneous posterior fossa hemorrhage. NF1 was associated with improved outcome—fewer patients required active intervention and rates of visual impairment and/or or hypothalamic/pituitary dysfunction were lower.

Conclusions

Good long-term survival and functional outcomes can be achieved in children with OPHG. Tumor control was achieved through an individualized approach using surgery, chemotherapy, or radiotherapy in varied combinations. The authors aim to limit radiotherapy to cases involving older children in whom other therapies have failed, due to the well-described and often devastating late effects associated with midline cranial irradiation. Surgery has a clear role for diagnosis, tumor control, and relief of mass effect. In particular, primary surgical debulking of tumor (without adjuvant therapy) is safe and effective. Recent advances in intraoperative MRI may add value and need further assessment.

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Mueez Waqar, Jonathan R. Ellenbogen, Ram Kumar, Christine Sneade, Bassel Zebian, Dawn Williams and Benedetta L. Pettorini

Intrathecal baclofen (ITB) is a reversible treatment that reduces muscle tone to ameliorate spasticity and dystonia in patients with cerebral palsy (CP). The resulting decrease in energy expenditure allows patients to gain much-needed weight, albeit temporarily. Modern techniques require sufficient abdominal musculature and subcutaneous fat to permit the implantation of an indwelling pump. In patients with extremely low muscle bulk, visceral pumps may be impractical or impossible, with increased risks of dehiscence and infection. The authors describe a variation of the classical procedure in a young patient with severe cachexia.

A 10-year-old boy with spastic-dystonic quadriplegic CP was admitted to the neuromedical unit. Numerous drug trials had failed, and surgical intervention was deemed necessary but was complicated by his cachectic body habitus. The authors inserted a lumbar intrathecal catheter and subcutaneously tunneled it to the anterolateral abdomen, where it was connected to a subcutaneous injection port. Baclofen was continuously infused into the subcutaneous port using a noncoring needle connected to an external pump. The needle and line were changed every 5 days to minimize the risk of sepsis.

Although other techniques, such as intraventricular baclofen delivery, have been described, these are largely dependent upon sufficient musculature to support a visceral pump. A subcutaneous injection port system represents an alternative approach that reduces the risk of sepsis and may be better tolerated in cachectic patients.

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Ros Whelan, Eric Prince, David M. Mirsky, Robert Naftel, Aashim Bhatia, Benedetta Pettorini, Shivaram Avula, Susan Staulcup, Allyson L. Alexander, Maxene Meier and Todd C. Hankinson

OBJECTIVE

Pediatric adamantinomatous craniopharyngiomas (ACPs) are histologically benign brain tumors that confer significant neuroendocrine morbidity. Previous studies have demonstrated that injury to the hypothalamus is associated with worsened quality of life and a shorter lifespan. This insight helps many surgeons define the goals of surgery for patients with ACP. Puget and colleagues proposed a 3-tiered preoperative and postoperative grading system based on the degree of hypothalamic involvement identified on MRI. In a prospective cohort from their institution, the authors found that use of the system to guide operative goals was associated with decreased morbidity. To date, however, the Puget system has not been externally validated. Here, the authors present an interrater reliability study that assesses the generalizability of this system for surgeons planning initial operative intervention for children with craniopharyngiomas.

METHODS

A panel of 6 experts, consisting of pediatric neurosurgeons and pediatric neuroradiologists, graded 30 preoperative and postoperative MRI scans according to the Puget system. Interrater reliability was calculated using Fleiss’ κ and Krippendorff’s α statistics.

RESULTS

Interrater reliability in the preoperative context demonstrated moderate agreement (κ = 0.50, α = 0.51). Interrater reliability in the postoperative context was 0.27 for both methods of statistical evaluation.

CONCLUSIONS

Interrater reliability for the system as defined is moderate. Slight refinements of the Puget MRI grading system, such as collapsing the 3 grades into 2, may improve its reliability, making the system more generalizable.