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Objective: Infradiaphragmatic craniopharyngiomas (ICs) represent a distinct subtype, harboring a sellar-suprasellar origin and generally growing in the extra-arachnoidal space contained by the diaphragma sellae. They have been considered ideal for surgical removal through the transsphenoidal approach since the 1960s. The authors present a multicentric national study, intending to selectively analyze IC behavior and the impact of the transsphenoidal endoscopic endonasal approach (EEA) on surgical outcomes., Methods: Craniopharyngiomas that were intraoperatively recognized as infradiaphragmatic and removed with standard EEA between 2000 and 2021 at 6 Italian neurosurgical departments were included in the study. Clinical, radiological, and surgical findings and outcomes were evaluated and reviewed., Results: In total, 84 patients were included, with 45.23% identified as pediatric cases and 39.28% as having recurrent tumors. The most common presenting symptoms were endocrine (75%), visual (59.52%), and hypothalamic (26.19%) disorders. ICs were classified as extending below (6 intrasellar and 41 occupying the suprasellar cistern) or above (26 obliterating the anterior recesses of the third ventricle and 11 extending up to the foramina of Monro) the chiasmatic cistern. Gross-total resection (GTR) was achieved in 54 cases (64.28%). Tumor extension above the chiasmatic cistern and calcifications were associated with lower likelihood of GTR. The cumulative rate of postoperative complications was 34.53%, with CSF leak being the most common (14.28%). Endocrine, visual, and hypothalamic functions deteriorated postoperatively in 41/78 patients (52.56%), 5/84 (5.95%), and 14/84 (16.67%), respectively. Twenty-eight patients (33.33%) had recurrence during follow-up (mean 63.51 months), with a mean 5-year progression-free survival (PFS) rate of 58%. PFS was greater in patients who achieved GTR than patients with other extent of resection., Conclusions: This is the largest series in the literature to describe ICs removed with standard EEA, without the need for additional bone and dural opening over the planum sphenoidale. EEA provides a direct route to ICs, the opportunity to manage lesions extending up to the third ventricle without breaching the diaphragma, and high rates of GTR and satisfactory clinical outcomes. Increased surgical complexity and morbidity should be expected in patients with extensive suprasellar extension and involvement of the surrounding vital neurovascular structures.

Cephalohematoma, one of the most common neonatal head injuries, generally undergoes spontaneous resorption. When calcified, it may cause cranial vault distortion and depression of the inner skull layer, although it remains asymptomatic. Surgery, indeed, is usually performed for cosmetic purposes. For these reasons, the long-term effects of calcified cephalohematoma (CC) are widely unknown. The authors report the case of an 11-year-old girl with a persistent calcified CC causing skull deformity and delayed electroencephalography (EEG) anomalies. These anomalies were detected during routine control EEG and were not clinically evident. The young girl underwent surgical removal of the CC for cosmetic purpose. The EEG abnormalities disappeared after surgery, thus reinforcing the hypothesis of a correlation with the brain "compression" resulting from the CC. To the best of the authors' knowledge this is the first time that CC-associated EEG anomalies have been described: even though these anomalies cannot be considered an indication for surgery, they merit late follow-up in case of skull deformity.

Descending transtentorial herniation (DTH) is a complication of raised pressure in the supratentorial compartment, usually resulting from mass lesion of several etiologies. The authors report an exceptional case of DTH complicating the implant of a CSF shunting device in the trapped fourth ventricle of a 17-year-old boy in whom a second CSF shunting device had been implanted for neonatal posthemorrhagic and postinfectious hydrocephalus. The insidious clinical and radiological presentation of DTH, mimicking a malfunction of the supratentorial shunt, is documented. Ultimately, the treatment consisted of removal of the infratentorial shunt and endoscopic acqueductoplasty with stenting. The absence of supratentorial mass lesion and other described etiologies of DTH prompted the authors to speculate on the hydrodynamic pathogenesis of DTH in the present case.

Palsy of the abducens nerve is a neurological sign that has a wide range of causes due to the nerve's extreme vulnerability. Need of immediate neuroimaging is a matter of debate in the literature, despite the risks of delaying the diagnosis of a skull base tumor. The authors present 2 cases of skull base tumors in which the patients presented with recurrent and self-remitting episodes of sixth cranial nerve palsy (SCNP). In both cases the clinical history exceeded 1 year. In a 17-year-old boy the diagnosis was made because of the onset of headache when the tumor reached a very large size. In a 12-year-old boy the tumor was incidentally diagnosed when it was still small. In both patients surgery was performed and the postoperative course was uneventful. Pathological diagnosis of the tumor was consistent with that of a chondrosarcoma in both cases. Recurrent self-remitting episodes of SCNP, resembling transitory ischemic attacks, may be the presenting sign of a skull base tumor due to the anatomical relationships of these lesions with the petroclival segment of the sixth cranial nerve. Physicians should promptly recommend neuroimaging studies if SCNP presents with this peculiar course.

Chiari malformation Type I (CM-I) is usually suspected in patients with slowly progressing neurological symptoms. However, in some instances, especially if syringomyelia is associated, an abrupt clinical onset is reported and is accompanied by an acknowledged risk of potentially severe clinical signs or even sudden death. Little is known about such a critical course in CM-I/syringomyelia complex. The authors describe 3 challenging cases of the abrupt onset of CM-I/syringomyelia to reveal more information on the clinical presentation and pathogenetic mechanisms of this sudden and potentially severe clinical phenomenon: a 38-year-old man experienced acute respiratory failure requiring intubation following acute decompensation of hydrocephalus associated with Noonan syndrome, a 1-year-old boy had sudden hemiparesis and Horner syndrome after a minor head/neck injury, and a 2.5-year-old boy presented with quickly progressing tetraplegia and dyspnea after a mild flexion and extension neck injury a few hours before. All 3 patients showed a CM-I/syringomyelia complex at diagnosis, and all of them had a good neurological outcome after surgery despite the ominous clinical presentation.

Chiari malformation (CM) is the downward herniation of the caudal part of the cerebellum and/or medulla oblongata into the spinal canal. It can alter several neurological functions, including respiratory control and upper airway motility, and can be the cause of sleep-disordered breathing (SDB). The authors describe a 6-year-old boy affected by CM Type II associated with myelomeningocele who showed symptoms indicative of severe airway obstruction during sleep. Polysomnography revealed severe obstructive sleep apnea syndrome (OSAS). Magnetic resonance imaging demonstrated herniation of the cerebellar tonsils and diffuse ventricular dilation with a large pseudocystic formation in the third ventricle. Surgical marsupialization of the cystic wall was performed, associated with ventriculocystostomy and endoscopic replacement of the ventricular catheter. Polysomnography repeated 2 months after surgery revealed a striking improvement in the sleep-related respiratory pattern. The pathogenesis of OSAS was probably referable to a combination of CM and elevated intracranial pressure. However, the striking improvement of symptoms after ventriculoatrial shunt placement suggested that hydrocephalus plays a major role in this condition. Assessment and effective treatment of SDB is crucial in the care of patients with CM.

Object: The authors assessed the efficacy of intratumoral interferon-alpha (IFNalpha)-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 INFalpha2A-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 INFalpha for the control of cystic craniopharyngiomas. The treatment has proved efficacious; there was no mortality, and morbidity rates were low.

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.

Object: The authors evaluated the results of complete cognitive function examinations in a series of 65 adolescents who had undergone surgery for sagittal or unicoronal craniosynostosis when they were younger than 1 year old., Methods: Each of the 65 study participants was evaluated individually. The neuropsychological assessment, consisting of a battery of tests tailored to the patient's age, cognitive level, and level of cooperation, was conducted in 2 separate sessions on the same day. The main outcome measures included fine motor skills, language, visual motor spatial and visual perceptual skills, working and visual memory, attention, executive function, and verbal fluency., Results: Thirty-five children (mean age 13.4 years) were affected by sagittal synostosis and 30 (mean age 14.9 years) by unicoronal synostosis (16 right-sided, 14 left-sided). The mean age at surgery was 7.2 months. All of the children had begun school at a normal age and attended regular classes. Seven percent of those with sagittal craniosynostosis demonstrated visuospatial and constructional ability defects with associated visual memory recall deficits; 17% also exhibited selective and sustained attention deficits. Approximately one-third (30%) of the children with anterior plagiocephaly had processing and planning speech deficits., Conclusions: Data in this study support the hypothesis that children with sagittal or unicoronal craniosynostosis, although they undergo early surgical treatment, may still manifest lower than average results at long-term selective neuropsychological evaluations.

The authors report a case of minimal prenatal trauma producing a large subdural hematoma in the fetus, which was diagnosed in utero by MR imaging. The occurrence of such a complication is extremely rare in the absence of significant maternal trauma. Prenatally diagnosed intracranial hemorrhages, particularly those that are subdural in origin, have a poor prognosis in most cases. After birth, brain compression required a complex neurosurgical intervention because simple hematoma evacuation was not possible. The clinical and neurological outcome at 6 months was excellent, as confirmed by the neuroimaging findings.

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-alpha. 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.

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.

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.

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.

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.

The late failure of endoscopic third ventriculostomies (ETVs) has been described as a consequence of scarring phenomena involving the third ventricular floor. Although the possibility of closure of the stoma by intraventricular tumor seeding has been hypothesized, no case of ETV failure caused by tumor diffusion has been reported in the literature. The authors report on a 19-year-old patient, affected by obstructive hydrocephalus associated with a periaqueductal pilocytic astrocytoma, who underwent late secondary lesional obstruction of an ETV performed at diagnosis. The authors suggest tumor extension along the diencephalic neural pathways as the possible cause of ETV failure in this patient.

Object: Dermoid and epidermoid cysts are rare space-occupying lesions of the central nervous system. Although characterized by a slow growth rate, they are often associated with serious complications. Surgery is the only effective treatment, and radical resection of the entire cyst, whenever possible, generally succeeds in achieving a cure. Authors of large series have only occasionally reported on pediatric cases, and these reports often lack a specific analysis of those cerebral midline lesions., Methods: The authors report on the treatment of 19 patients (16 with intracranial intradural dermoid and three with epidermoid cysts located along the cerebral midline). All patients underwent surgery at the Catholic University Medical School in Rome. The patients ranged in age from 3 months to 16 years. Nine cysts were located in the posterior cranial fossa, six in the frontobasal subarachnoid spaces, two in the third ventricle, and two in the quadrigeminal plate cistern. In the cases presenting with dermal sinus tracts, attempts at resecting the dermoid cyst and the associated dermal sinus were made in a single stage to achieve an en bloc removal. In the cases without dermal sinus tracts, and in the three with epidermoid cysts, a standard craniotomy was performed to reach the lesion. There were no surgery-related deaths, and the morbidity rate was low. Total and subtotal resections were achieved in 15 and four cases, respectively; however, regrowth of the residual tumor was observed only in two of them. After repeated resection, tumor progression has not been observed. At a mean follow-up period of 92.7 months, excellent clinical results were achieved in 18 patients., Conclusions: Because surgery is the only effective treatment modality for these lesions, radical resection should be performed in all cases to avoid tumor recurrence; however, because the cyst capsule can adhere firmly to vital structures and attempts at its radical removal can be dangerous, subtotal resection may be a wise option in selected cases.

An 18-year-old man presented with a spontaneously occurring radial nerve palsy that spared the triceps muscle. At surgery, the portion of the radial nerve located at the midarm level had an hourglass-like appearance. Under magnification, an external-internal neurolysis of the narrowed portion of the hourglass-shaped portion revealed nerve torsion. Straightening of the twisted nerve and fixation accomplished using epiperineurium-fascia stitches to avoid a new torsion resulted in complete functional recovery of the radial nerve.