Biobank for craniosynostosis and faciocraniosynostosis, rare pediatric congenital craniofacial disorders: a study protocol.

PURPOSE: Craniosynostosis (CRS) is a rare congenital cranial malformation in which 1 or more cranial or facial sutures are fused in utero or rapidly fused in early infancy. The cranial sutures separate the skull bone plates and enable rapid growth of the skull in the first 2 years of life, in which growth is largely dictated by growth of the brain. CRS is a rare disease that occurs in 1 in 2100 to 1 in 2500 births and may be either nonsyndromic (also referred to as isolated) or syndromic. In syndromic CRS, other birth defects are present next to the CRS. The distinction between nonsyndromic and syndromic manifestations is made on the basis of dysmorphologic evaluation and genetic evaluation. Owing to advances in genetic diagnostics, nonsyndromic patients are increasingly recognized as syndromic patients. CRS treatment is almost entirely surgical and is sometimes paired with postoperative helmet therapy for maintenance. Corrective procedures are complex, long, and associated with the risk of numerous complications, including heavy blood loss and its sequelae. Although surgery may restore a normal appearance, even in nonsyndromic patients, patients may experience persistent deficits in intellectual ability and cognitive function. The European Commission (EC) has prioritized rare diseases in recent horizon European research programs; indeed, collections or even individual samples may be extremely valuable for research. METHODS AND RESULTS: Here, we present a study protocol in which the combined expertise of clinicians and researchers will be exploited to generate a biobank dedicated to CRS. The generation of the CRS biobank presented in this study will include the collection of different types of biological materials as well as advanced radiological images available to the scientific community. CONCLUSION: The activation of a CRS biobank will provide an opportunity to improve translational research on CRS and to share its benefits with the scientific community and patients and their families.

Low-grade epilepsy-associated tumors: Epilepsy outcome and antiseizure medication discontinuation after lesionectomies as first-line surgical approach in pediatric population.

OBJECTIVE: This study aimed to evaluate epilepsy outcome and antiseizure medication (ASM) discontinuation after lesionectomies as first surgical approach in pediatric population diagnosed with low-grade epilepsy-associated neuroepithelial tumors (LEATs). METHODS: We conducted a retrospective study. Thirty-six consecutive patients with histological diagnoses of LEATs who underwent surgery between 2018 and 2021 at our institution were included. The clinical and surgical data were retrospectively analyzed. RESULTS: Thirty (83.3%) of 36 patients are free of disabling seizures (Engel class I) and 19 (63,4%) of them are classified as Engel Ia. In 17 (47.2%) patients, ASM could be discontinued. The mean age at surgery was 8.6 years (±4.04) and the mean age at onset of epilepsy was 7.2 years (±3.8), whereas the mean duration of epilepsy in months at the time of surgery was 21.3 months (±23.7). The epileptogenic tumor was in the temporal lobe in 20 (55.5%) patients. Because of seizure persistence, a second or a third surgery was necessary for six patients (16.7%) and four of them had residual lesions (three in temporal and one in extratemporal site). No perioperative complications were recorded, including acute seizures, with a median hospitalization time of 7 days. Shorter epilepsy duration at time of surgery as long as a single ASM was significantly correlated with an Engel class I outcome (p-value = .01 and p-value = .016, respectively). Focal seizure semeiology was associated with an increased probability of antiseizure medication discontinuation (p-value = .042). SIGNIFICANCE: Our findings confirm that shorter epilepsy disease duration, monotherapy before surgery, and seizure semeiology are determinant factors for a positive seizure outcome and medication discontinuation, also with less invasive surgical approaches such as lesionectomies. However, considering the intrinsic multifactorial epileptogenic nature of LEATs, a tailored surgical approach should be considered to optimize clinical and seizure outcome, especially for lesions located in the temporal lobe.

Pineocytoma in a child with Pallister-Killian syndrome: a case report and review of the literature.

Pallister-Killian syndrome (PKS; OMIM #601803) is a rare genetic disorder typically characterized by developmental delay, seizures, sparse temporal hair, and facial dysmorphisms. PKS is most frequently caused by mosaic supernumerary isochromosome 12p. Here, we report a 27-month-old girl with a prenatal diagnosis of PKS and a histopathological diagnosis of pineocytoma.

Jugular foramen stenosis in external hydrocephalus in infants.

OBJECTIVE: To measure the size of jugular foramina in infants affected by external hydrocephalus (EH) and in a control group, to support the hypothesis that a jugular foramen (JF) stenosis may determine dural venous sinus alterations and increased venous outflow resistance as main pathophysiological factor. METHODS: Minimum, maximum, and mean values of JF areas were measured in a series of phase-contrast magnetic resonance venous angiography (angio MRV PCA3D) performed on 81 infants affected by EH. Results were compared with a group of 54 controls. RESULTS: Smaller JF area was significantly smaller in patients versus controls (43.1 ± 14.6 vs. 52.7 ± 17.8; p < 0.001) resulting in a significantly smaller mean JF areas in patients vs. controls (51.6 ± 15.8 vs. 57.0 ± 18.3; p = 0.043). In patients, smaller JF areas were significantly associated with higher venous obstruction grading score (VOGS) both on the right (p = 0.018) and on the left side (p = 0.005). Positional plagiocephaly (cranial vault asymmetry index > 3.5%) was more frequent among EH patients than controls (38/17) but the difference was not significant (p = 0.07). In the 38 plagiocephalic patients, JF area was smaller on the flattened side than the contralateral in a significant number of cases both in right (21/7) and left (9/1) plagiocephaly (p < 0.0005) as well as the mean area (48.2 + 16.4 mm2 vs. 57.5 + 20.7 mm2, p = 0.002) and VOGS was significantly higher on the plagiocephalic side than on the contralateral side (1.6 ± 1.1 vs. 1.1 ± 0.9, p = 0.019). CONCLUSION: In this series of infants affected by EH, the mean size of the ostium of both JF resulted significantly smaller than controls. JF stenosis was significantly associated with higher degrees of venous obstruction on both sides, suggesting a direct extrinsic effect of JF size on dural sinus lumen and possible consequent effect on venous outflow resistance. Positional plagiocephaly, when present, was associated with a decreased JF area and increased VOGS on the flattened side.

Image-guided biopsy of intracranial lesions in children, with a small robotic device: a case series.

BACKGROUND AND OBJECTIVES: Robot-assisted biopsies have gained popularity in the last years. Most robotic procedures are performed with a floor-based robotic arm. Recently, Medtronic Stealth Autoguide, a miniaturized robotic arm that work together with an optical neuronavigation system, was launched. Its application in pediatric cases is relatively unexplored. In this study, we retrospectively report our experience using the Stealth Autoguide, for frameless stereotactic biopsies in pediatric patients. METHODS: Pediatric patients who underwent stereotactic biopsy using the Stealth Autoguide cranial robotic platform from July 2020 to May 2023 were included in this study. Clinical, neuroradiological, surgical, and histological data were collected and analyzed. RESULTS: Nineteen patients underwent 20 procedures (mean age was 9-year-old, range 1-17). In four patients, biopsy was part of a more complex surgical procedure (laser interstitial thermal therapy - LITT). The most common indication was diffuse intrinsic brain stem tumor, followed by diffuse supratentorial tumor. Nine procedures were performed in prone position, eight in supine position, and three in lateral position. Facial surface registration was adopted in six procedures, skull-fixed fiducials in 14. The biopsy diagnostic tissue acquisition rate was 100% in the patients who underwent only biopsy, while in the biopsy/LITT group, one case was not diagnostic. No patients developed clinically relevant postoperative complications. CONCLUSION: The Stealth Autoguide system has proven to be safe, diagnostic, and highly accurate in performing stereotactic biopsies for both supratentorial and infratentorial lesions in the pediatric population.

Endoscopic Ultrasonic Aspiration of Brain Abscess.

Brain abscess is a focal infection occurring within the brain parenchyma consisting of a pus collection surrounded by a vascularized capsule and a fibrinous-caseous layer between the pus and capsule. Surgery is indicated for lesions with a diameter >25 mm. Different surgical approaches have been described, including puncturing of the abscess (under neuronavigation, stereotactic or echographic guidance) with aspiration of the purulent fluid through a catheter and craniotomy with microsurgical removal of the purulent material and surrounding capsule. In recent years, the endoscopic approach has become more frequently used to treat brain abscesses. The theoretical advantages are visual awareness of the completeness of pus removal and the possibility of also removing the more solid fibrinoid component, which could be the source of recurrence. Craniotomy is likewise avoided. We present the case of an 11-year-old boy affected by a parieto-occipital brain abscess and initial ventriculitis who underwent endoscopic surgery. An ultrasonic aspirator was used to wash and suck the purulent material and fragment and remove the more solid fibrinoid component. The occipital horn of the lateral ventricle was also entered, and pus was removed. At the end of the procedure, a ventricular drainage tube was left in the surgical cavity. It was removed 1 day later, because the cavity had completely collapsed. The key surgical steps are presented in Video 1.1-9 The procedure was uneventful, with very good clinical and radiological results. The endoscopic technique has been shown to be a safe and effective treatment option for intracranial abscess. In the case of large superficial lesions, the surgical risks appear similar to those of simple drainage through catheters, with a possible reduction of the 20% reported recurrence rate. The use of an ultrasonic aspirator could facilitate complete and faster pus removal, increasing the efficacy of the procedure. The patient and his parents consented to the procedure and the report of the patient's case details and imaging studies. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.

Removal of a thoracic intramedullary epidermoid tumor in a child.

Epidermoid cysts are rare, benign neoplasms that account for less than 1% of all intraspinal tumors. The most common localization is in the lumbar area, and one-third of the tumors are intramedullary. In this video, the authors present removal of a thoracic intramedullary epidermoid tumor in a 6-year-old boy, carrier of a 22q11 gene duplication and affected by psychomotor retardation. He presented a 1-year history of progressive gait impairment. No history of lumbar puncture or trauma was reported. The procedure was performed under neurophysiological monitoring, and it was uneventful with complete recovery of neurological function. Technical nuances are illustrated.

Surgical Approaches to the Third Ventricle: An Update.

The third ventricle is located in the deepest part of the brain and is delimited by both telencephalic and diencephalic structures. Its location makes every surgical procedure inside or around it quite challenging, due to the distance from the surface to the fragility of the neurovascular structures that is necessary to dissect before entering its cavity and to the narrow surgical corridors through which it is necessary to work. Its geometric localization inside the cranial cavity and the anatomical relationship with the interhemispheric fissure offers nevertheless to the surgeon an impressive variety of surgical approaches, which allow to reach every millimeter of the third ventricle lumen. Mastering properly all these approaches requires an impressive anatomical knowledge, the best available technology, and most refined technical skills, making the surgery of the third ventricle a point of excellence in the evolution of each neurosurgeon. The development of neuronavigation and neuroendoscopy has been a revolution in neurosurgery in the last 20 years and offered special advantages for the surgery of the third ventricle. In fact, the narrow corridors of approach make the precision of the neuronavigation and the enlightenment and magnification of the neuroendoscopy especially useful to reach the third ventricle cavity and working inside or around it. This chapter reviews the history of the surgery of the third ventricle and offers an update of the variety of surgical corridors identified and of the technology now available to properly work through them and inside the third ventricle cavity.

Neuroendoscopy: intraventricular and skull base tumor resection in children.

During the last 30 years, the neurosurgeons have witnessed a revolution in the practice of interventricular surgery. The advent of neuroendoscopy at the end of the 1980s has allowed a minimally invasive management of a very large series of pathologies in pediatric neurosurgery ranging from hydrocephalus to arachnoid cyst to intraventricular tumors. The progresses in the management of hydrocephalus, intracranial cyst, and the fluid filled collection nevertheless has been more rapid and radical due to the simpler equipment that is necessary to perform this kind of surgery. The intraventricular tumors instead have been addressed in a slower way, and for many years, the only endoscopic procedure that was allowed on interventricular tumors was a biopsy associated with the management of hydrocephalus. Only very small tumors have been considered operable for complete removal during many years due to the limitations of the neuroendoscopic equipment and to the small calibers of the working channel. More recently, the advent of new devices and new surgical techniques are offering new perspectives on the possibility of intraventricular tumor surgery in children. In this review, we describe the historical perspective of the learning curve of intraventricular tumor surgery under neuroendoscopic control and try to offer a view of the future perspective in the removal of larger intraventricular tumors, analyzing the main indications for intraventricular endoscopic tumor surgery. We offer as well an historical perspective of the evolution of skull base surgery and endonasal transsphenoidal approach for skull-based tumors in children. This kind of surgery that has acquired widespread acceptance for many pathologies in adult age has diffused more slowly in pediatric neurosurgery due to the anatomical limitation observed in these age range. Also in this field, the slow evolution of the technique and of the technology available to neurosurgeons has allowed a very significant expansion of indication for the minimally invasive removal of skull base tumors in children.

Extra-neural metastases in pediatric diffuse midline gliomas, H3 K27-altered: presentation of two cases and literature review.

Introduction: Pediatric diffuse midline gliomas (DMG), H3 K27- altered, are the most aggressive pediatric central nervous system (CNS) malignancies. Disease outcome is dismal with a median survival of less than one year. Extra-neural metastases are an unusual occurrence in DMG and have been rarely described. Methods and results: Here, we report on two pediatric patients affected by DMG with extra-neural dissemination. Their clinical, imaging, and molecular characteristics are reported here. An 11-year-old male 5 months after the diagnosis of diffuse intrinsic pontine glioma (DIPG) developed metastatic osseous lesions confirmed with computed tomography (CT) guided biopsy of the left iliac bone. The patient died one month after the evidence of metastatic progression. Another 11-year-old female was diagnosed with a cerebellar H3K27- altered DMG. After six months, she developed diffuse sclerotic osseous lesions. A CT-guided biopsy of the right iliac bone was non-diagnostic. She further developed multifocal chest and abdominal lymphadenopathy and pleural effusions. Droplet digital polymerase chain reaction (ddPCR) on pleural effusion revealed the presence of H3.3A mutation (c.83A>T, p.K28M). The patient died 24 months after the diagnosis of DMG and 3 months after the evidence of metastatic pleural effusion. Discussion: Extra-neural metastasis of DMG is a rare event and no standard therapy exists. An accurate and early diagnosis is necessary in order to develop a personalized plan of treatment. Further research is needed to gain further insights into the molecular pathology of DMG, H3K27- altered and improve the quality of life and the final outcome of patients with this deadly disease.

Dry Field Technique and Ultrasonic Aspirator in Endoscopic Removal of a Hemorrhagic Intraventricular Tumor in a 2-Year-Old Girl.

Neuroendoscopic procedures inside the ventricular system always bear the risk for an unexpected intraoperative hemorrhage. Most hemorrhages can be managed by constant irrigation with low- and high-pressure washes. In the other rare cases, the dry field technique may be necessary.1-5 It requires the aspiration of the entire intraventricular cerebrospinal fluid with the aim of establishing a proper environment for hemostasis. Video 1 illustrates a step-by-step removal of an intraventricular tumor in a 2-year-old girl through an endoscopic technique where the dry field technique was undertaken because of its hemorrhagic nature. Postoperative magnetic resonance imaging showed complete removal of the left frontal tumor infiltration at the level of the left frontal ependyma. The small residual tumor on the left frontal horn was removed using microsurgical technique with another procedure and after achieving complete removal of all visible tumor, the patient was referred to radiotherapy.

Tumors of Choroid Plexus and Other Ventricular Tumors.

Tumors arising inside the ventricular system are rare but represent a difficult diagnostic and therapeutic challenge. They usually are diagnosed when reaching a big volume and tend to affect young children. There is a wide broad of differential diagnoses with significant variability in anatomical aspects and tumor type. Differential diagnosis in tumor type includes choroid plexus tumors (papillomas and carcinomas), ependymomas, subependymomas, subependymal giant cell astrocytomas (SEGAs), central neurocytomas, meningiomas, and metastases. Choroid plexus tumors, ependymomas of the posterior fossa, and SEGAs are more likely to appear in childhood, whereas subependymomas, central neurocytomas, intraventricular meningiomas, and metastases are more frequent in adults. This chapter is predominantly focused on choroid plexus tumors and radiological and histological differential diagnosis. Treatment is discussed in the light of the modern acquisition in genetics and epigenetics of brain tumors.

Hemostasis in a giant intraventricular tumor using a saline-cooled radiofrequency bipolar coagulator: technical note.

BACKGROUND: Meningiomas are relatively rare in children and tend to be intraventricular and cystic, with often malignant behavior. Complete excision is associated with the most favorable outcome; moreover, the size and extent of these lesions often make complete excision in one step impossible because of the risk of intraoperative death from uncontrollable hemorrhage. CASE PRESENTATION: A 10-year-old girl was admitted for headache in the last 3 months and was found to have a giant left intraventricular lesion with a volume of 166.63 cm3, which caused hydrocephalus and significant mass effect. Very large draining veins were evident within the tumor, draining into the thalamostriates and internal cerebral veins. Cerebral angiography showed multiple feeders originating mainly from branches of the posterior left choroidal artery with distal afferents that could not be embolized. Therefore, a left parietal transcortical approach was chosen. Given the vascularity of the tumor, saline-cooled radiofrequency coagulation (Aquamantys®) was used to reduce blood loss intraoperatively. Gross total resection (GTR) was achieved with an estimated blood loss of 640 mL. Pathology analysis was consistent with WHO grade 1 transitional meningioma. Postoperatively, the patient was neurologically intact, and MRI confirmed complete resection. CONCLUSION: Aquamantys® is a novel bipolar coagulation device that employs a new bipolar coagulation technique combining radiofrequency energy and saline to achieve hemostatic sealing by denaturing collagen fibers. This offers the possibility of achieving adequate hemostasis even in giant intraventricular tumors in infants to obtain GTR resection with minimal blood loss.

Endoscopic ultrasonic aspirator-assisted removal of a third ventricular colloid cyst.

BACKGROUND: Colloid cysts are benign tumors usually located on the roof of the third ventricle. Cyst removal is the treatment of choice. It can be accomplished microsurgically through a transcortical- or transcallosal approach, or endoscopically. There is a lack of consensus regarding the best strategy for cyst removal. One of the challenges of the traditional endoscopic technique is dealing with the cyst content density. Hyperdensity on computed tomography scan and low signal on T2-weighted magnetic resonance imaging (MRI) cyst are correlated with high viscosity cystic content. CASE REPORTS: We present a case of a colloid cyst of the third ventricle in a 15-year-old boy removed through a pure endoscopic transventricular approach. The cyst presented a low signal on T2 MRI; nevertheless, it was easily removed with the help of an endoscopic ultrasonic aspirator. DISCUSSION AND CONCLUSION: The colloid cyst of the third ventricle can be safely treated by a purely endoscopic approach. The rationale of the use of the ultrasonic aspirator relies on the facilitation of aspiration of the content even when the consistency is extremely firm.

Pure endoscopic ultrasonic removal of choroid plexus papillomas of the third ventricle: technical report of two cases.

BACKGROUND: Tumors of the choroid plexus of the third ventricle are uncommon. Surgical excision is technically challenging because of the rich vascularisation, central location, and high incidence in young children. Open microsurgical resection is considered the standard treatment. However, attempts at purely endoscopic removal of choroid plexus tumors of the third ventricle have also been made in the past, with encouraging results. CASE REPORTS: We report our experience with endoscopic ultrasonic removal of two cases of tumors of the third ventricular choroid plexus. The first case was a large atypical choroid plexus papilloma (WHO grade 2) in the anterior third ventricle associated with hydrocephalus; the second case was a smaller choroid plexus papilloma (WHO grade 1) in the middle/posterior third ventricle without overt hydrocephalus requiring a more anterior neuronavigation guided approach. DISCUSSION AND CONCLUSION: Choroid plexus papillomas of the third ventricle can be safely treated by a purely endoscopic approach because they are usually smaller than their counterparts in the lateral ventricle and often have a recognizable vascular pedicle. Early detection and control of the vascular pedicle at the choroidal border is key to success. The use of ultrasonic aspirator facilitates and expedites endoscopic access. By alternating surface coagulation with fragmentation and aspiration with the ultrasonic aspirator, the tumor can be removed without difficult dissection maneuvers.

Endoscopic ultrasonic resection of calcified tumor of the third ventricle.

In this video, the authors present ultrasonic resection of calcified tumor of the third ventricle in a 12-year-old boy. He presented to the emergency department with a 1-week history of headache, drowsiness, and bilateral papilledema. Despite extensive calcification visible on a CT scan, a minimally invasive pure endoscopic approach was chosen. The use of an ultrasonic aspirator allows fast and safe removal of the tumor. The histological diagnosis was a low-grade glioneuronal tumor. In conclusion, the endoscopic ultrasonic aspirator is a useful tool to resect tumors in the ventricular system. The presence of calcifications within the tumor does not contraindicate an endoscopic approach. The video can be found here: https://stream.cadmore.media/r10.3171/2023.1.FOCVID22143.

Fourth ventricle to spinal subarachnoid space stenting in pediatric patients with refractory syringomyelia: case series and systematic review.

A series of 5 patients treated with the fourth ventricle to spinal subarachnoid space stent (FVSSS) is presented. Indication for surgery, surgical technique, pre-operative and post-operative images, and outcome are analyzed. A systematic review of the pertinent literature has also been performed. This is a retrospective cohort review of a series of 5 consecutive patients with refractory syringomyelia who underwent a fourth ventricle to spinal subarachnoid space shunt surgery. The surgical indication was based on the presence of refractory syringomyelia in patients already treated for Chiari malformation or in patients who developed scarring at the level of the outlets of the fourth ventricle following posterior fossa tumor surgery. The mean age at FVSSS was 11.30 ± 5.88 years. Cerebral MRI revealed crowded posterior fossa, with a membrane at the level of the foramen of Magendie. Spinal MRI showed syringomyelia in all patients. Before surgery, the averages of the craniocaudal and the anteroposterior diameter were 22.66 and 1.01 cm, respectively, whereas the volume was 28.16 cm3. The post-operative period was uneventful in 4 out of 5 patients; one child died on the 1st post-operative day due to complications unrelated to surgery. In remaining cases, syrinx marked improvement. The post-operative volume was 1.47 cm3 with an overall reduction of 97.61%. With regard to literature, 7 articles with a total of 43 patients were analyzed. After FVSSS, syringomyelia reduction was observed in 86.04% of cases. Three patients underwent reoperation due to syrinx recurrence. Four patients presented a catheter displacement, one a wound infection and meningitis and one CSF leak requiring placement of a lumbar drain. FVSSS is highly effective in restoring CSF dynamics, with dramatic improvement of syringomyelia. In all our cases, the volume of the syrinx was reduced by at least 90%, with improvement/resolution of accompanying symptomatology. This procedure should be reserved to patients in which other causes of gradient pressure between the fourth ventricle and subarachnoid space are excluded, for example, tetraventricular hydrocephalus. Surgical procedure is not simple, because it requires meticulous microdissection of cerebello-medullary fissure and upper cervical spine, in already operated patients. To avoid migration of the stent, it should be carefully sutured to the dura mater or thick arachnoid membrane.

Endoscopic ultrasonic aspiration of posterior fossa abscess in Dandy-Walker malformation: case report.

BACKGROUND: Dandy-Walker malformation (DWM) is a posterior fossa malformation characterized by a huge posterior fossa cyst in communication with the fourth ventricle. Hydrocephalus is associated with more than 80% of cases and is usually treated by shunting. Despite infection being a common complication of the shunt, abscess formation within the cyst was reported only once. CASE REPORT: A neonate affected by DWM developed a posterior fossa abscess following a shunt infection. The purulent collection was refractory to standard treatment (antibiotics and burr hole drainage); therefore, an endoscopic approach was performed in order to remove the purulent collection under direct vision. This material was aspirated with the help of an endoscopic ultrasonic aspirator. The outcome was favorable, with a resolution of infection and re-implantation of the ventriculo-peritoneal shunt. Surprisingly, post-operative radiological examination showed substantial modification of the anatomy of the posterior fossa with disappearing of the Dandy-Walker cyst. To the best of our knowledge, this is the first documented report of a true Dandy-Walker malformation that modified its anatomical appearance over time. DISCUSSION AND CONCLUSION: Endoscopic aspiration of intracranial purulent collection should be considered a valid option to manage complicated cases. An endoscopic ultrasonic aspirator may make the procedure more effective and faster.

Acute ischemic stroke secondary to ventriculoperitoneal shunt dysfunction in a child with Moyamoya syndrome.

Background: Patients with brain vascular disease and hydrocephalus may be predisposed to acute ischemic stroke in case of shunt dysfunction and subsequent increased intracranial pression. Patients with brain tumor may develop hydrocephalus as a consequence of obstruction of cerebrospinal fluid pathways and radiation-induced moyamoya syndrome secondary (RIMS) to radiotherapy (RT). Case Description: A 15-year-old male patient, affected by hydrocephalus and RIMS, presented acute cerebral ischemia after an episode of shunt malfunction. The shunt was promptly revised and the areas of ischemia visible at magnetic resonance imaging significantly decreased. Conclusion: Children who receive RT for brain tumor, particularly if the circle of Willis region is involved, require close surveillance for the development of vasculopathy and consequent stroke. This surveillance must be even tighter if the patient has been treated with ventricular shunt for the possible synergistic interaction between the two causes on reducing cerebral perfusion and increasing the risk of acute ischemic events.

Changes in venous drainage after posterior cranial vault distraction and foramen magnum decompression in syndromic craniosynostosis.

OBJECTIVE: The authors' objective was to measure the effect of posterior cranial vault distraction (PCVD) plus foramen magnum decompression (FMD) on dural sinus volume and venous flow in patients with syndromic craniosynostosis. METHODS: The volumes of the sagittal, straight, transverse, and sigmoid sinuses of 5 consecutive patients with syndromic craniosynostosis who underwent PCVD+FMD were calculated in cubic centimeters with T2-weighted volumetric MRI sequences before surgery, immediately after surgery, and after the end of the distraction process. Tridimensional reconstructions of phase-contrast magnetic resonance angiography (PC-MRA) images were obtained with multiplanar reconstruction (MPR). RESULTS: The average total volume of all dural sinuses increased immediately after surgery (from 10.06 cm3 to 12.64 cm3) and continued to increase throughout the 30-day distraction period (from 12.64 cm3 to 14.71 cm3) (p = 0.04), except that the right sigmoid sinus remained stable after the initial increase. The most important increases were observed for the left transverse sinus (+113.2%), right transverse sinus (+104.3%), left sigmoid sinus (+91.3%), and sagittal sinus (+41.8%). Less important modifications were evident for the right sigmoid sinus (+33.7%) and straight sinus (+23.4%). Significant improvements in venous flow were noted on the tridimensional reconstructions of the PC-MRA images. Venous obstruction grading score improved in 4 patients (average [range] 2.4 [ 2-5]) (p = 0.023) and remained stable in 1 patient. All patients had chronic tonsillar herniation (CTH) (mean [range] 16.6 [8-26] mm), and 3 had syringomyelia. CTH showed improvement on the last follow-up MRI evaluation in 4 patients (mean [range] 10.5 [0-25] mm) and worsened from 15 mm to 19 mm in 1 patient. Syringomyelia improved in 2 patients and remained unchanged in 1. CONCLUSIONS: This study has provided the first radiological evidence of the impact of craniofacial surgery on dural sinus anatomy and venous drainage. The venous anomalies described in patients with syndromic craniosynostosis are not static, and PCVD+FMD triggers a dynamic process that can lead to significant modifications of intracranial venous drainage. The traction exerted by the distracted bone flap onto the occipitoparietal dura mater adherent to the inner calvaria may account for the enlargement of the dural sinus throughout the distraction period. The impact of these modifications on venous pressure, intracranial pressure, CTH, and hydrocephalus remains to be determined.