The improved management of craniosynostosis has let emerge concomitant anatomical, functional and clinical features that are frequently faced later in life (especially by syndromic patients) and might variably encompass the cranial nerves due to stenosis of bone foramina, abnormal intracranial pressure, or anomalous gene-driven development. Clinical consequences vary according to the affected nerves, the severity and the pathogenesis of nerve involvement but also might depend on early appropriate treatment. Vision, smell and hearing, but also feeding, swallowing and facial mimic or esthesia might be affected with a possible dramatic impact on the overall development of the child and on its quality of life. A systematic literature review regarding cranial nerves involvement in craniosynostosis was performed, including case series and case reports. According to PRISMA criteria, PubMed and Scopus were searched up to February 2025 by two independent reviewers. Relevant English-language case reports and case series were included, while duplicate or aggregated data were excluded. Reference lists were screened, and disagreements were resolved by consensus. Sixty-three papers were considered. Data extracted from the papers were subjected to statistical analysis only for the optic nerve, owing to the paucity of data concerning the other cranial nerves. Optic nerve involvement was reported in 140 patients (44 papers) and included papilledema (69 patients), optic nerve atrophy (69 patients), and optic nerve hypoplasia (5 patients). Visual function was reduced in 65 patients, normal in 9. In sixty-eight patients (49

BackgroundCranioplasty (CP) is a surgical intervention aiming to re-establish the integrity of skull defects. Autologous bone and different heterologous materials are used for this purpose, with various reported related complications, especially in children.This study aims to evaluate the rate of complication in a multicentric cohort of pediatric patients treated by porous hydroxyapatite (PHA) CP implantation and to assess the reliability of post-marketing clinical data collected by a manufacturing company. MethodsThe authors proactively collected clinical data from 20 institutions in different European countries for patients under the age of 16 treated with a PHA implant. The data were obtained by conducting an on-site interview with physicians in charge of the patients (Post-Marketing Surveillance, PMS group). The endpoints were the incidence of adverse events and related implant removal. The clinical data were compared to the company-based register including all patients under the age of 16 who received the same implant from January 1, 2004 to December 31, 2020, and the collecting complications voluntarily reported by surgeons (Database, DB group). ResultsThe two groups were similar in terms of demographic characteristics and rate of complications. In the PMS group, a total of 11 (16.9%) complications were reported in the group of 65 patients that were proactively collected. Both fractures and infections were the most common complications with 4 cases each (6.2%). In the case of both infections and fractures, revision surgery was required for only one patient (1.5%). Three (4.5%) cases of displacements were reported, and in one (1.5%) case, a surgical revision was required, for a total of 3 (4.5%) cases requiring surgical revision. The average follow-up was 26.7 months. ConclusionsDifferent from a previous study on adult age, pediatric neurosurgeons are more prone to report even to the manufacturing company complications related to skull reconstruction in children. Therefore, these data can be compared with those of other clinical studies. The PHA CP in this series of 65 patients presents a complication rate collected on-site that is similar to other heterologous materials.

OBJECTIVE:To evaluate the efficacy of the screening protocol and the clinical follow-up to detect urological anomalies (UA) in patients with ARM (ARM-P) in our institution. Secondary aim was to define the prevalence of UA and their relationship with severity of ARM.METHODS:ARM-P were selected from a prospectively maintained database from 2000 to 2016. Exclusion criteria were: incomplete or with less than 3 years of follow-up and absence of surgical correction of the anorectal anomalies. Data from urological screening and follow-up were collected. Patients were divided into complex malformations (Group 1) and less complex malformations (Group 2).RESULTS:One hundred seventeen of 149 were included in the study period (62 group 1, 55 group 2). UA were detected in 36/117 at birth (30.7%) with a difference between groups (P = .0005). VUR was detected in 16 (6 with hydronephrosis, 10 with normal ultrasound at birth). A bladder ultrasound after potty training showed 18 lower urinary tract dysfunction (LUTD; 15 with UA detected at birth, 3 with normal ultrasound). 8 LUTD were found during clinical follow-up, confirmed by ultrasound. Spinal MRI detected spinal cord anomalies (SCA) in 52/117 (44.4%). Considering the subgroups with neurogenic bladder and SCA there was a difference between groups (13/33 vs 1/19).CONCLUSION:This study suggests that ARM-P are at increased risk of UA, most of which were detected on neonatal ultrasound. While screening protocol can show 88% of UA, follow-up can detect 12.9 % of total abnormalities without difference between groups. This data has to be considered when planning follow-up for these patients.

Intraparenchymal cavities communicating with the ventricles may appear in patients with chronic obstructive hydrocephalus despite no identifiable surgerical, vascular or traumatic causes. The rate, features, pathogenesis, evolution and clinical impact of intraparenchymal diverticula have not been outlined, yet.

Compressive cervical myelopathy is a well-known life-threatening complication in mucopolysaccharidosis (MPS) patients. Glycosaminoglycan accumulation in the growing cartilage results in dens dysplasia, atlanto-axial instability, and subsequent periodontoid fibrocartilaginous tissue deposition with upper cervical stenosis.Chiari malformation type 1 (CM1) is a congenital downward cerebellar tonsil ectopia determined by clivus and posterior cranial fossa underdevelopment, possibly leading to progressive spinal cord cavitation (syringomyelia) and severe neurological impairment.We present a boy affected with Hunter syndrome (MPS II) and cerebellar tonsil ectopia who developed a holocord syringomyelia at the age of 6 years. The child underwent atlanto-occipital decompressive surgery with rapid clinical and neuroimaging improvement.Sharing a primary mesenchymal involvement of the cervical-occipital region, the coexistence of CM1 in MPS might be not unexpected and complicate further the disease course. In these patients, strict monitoring and prompt treatment might be of foremost importance for preventing major neurological complications.

Background and Aims Shaken Baby Syndrome (SBS) is a severe form of child abuse caused by violent shaking leading to severe head injuries, causing mild-severe long-term disabilities and death. The study aims to explore medium/long-term consequences of SBS comparing children undergoing neurosurgery with hematoma evacuation/cranioplasty with those with hematoma evacuation or no surgery. Methods A cohort of 21 children with SBS, admitted to Pediatric Department/Padua Hospital (2003–2011), was followed-up. Each clinical record was reviewed collecting information on onset, acute course, ophthalmologic examinations, neuroimaging, treatment and procedures, family history and social background. Cases were followed-up at 3, 6 and 12 months after trauma, every year. The assessment included fundus evaluation and visual function, neuroradiological exams (MRI). Results 21 cases were reviewed (M:F=2:1.1), 12 foreigners, 9 italians. Mean age at onset: 5.7 months (range 1.2–18). Mean age at last follow-up evaluation: 30.4 months (range 5–82). Mean follow-up duration: 24.6 months (range 1–73.5). In acute phase 8/19 underwent neurosurgical intervention and 1/21 underwent eye surgery. 20/21 showed retinal hemorrhages, 21/21 cerebral hemorrhages, 11/21 cerebellar hemorrhages, 5/10 spinal subdural hematoma, 4/21 skull fractures, 6/21 other body region fractures. At last follow-up evaluation resulted: 2/21 hemiplegia, 1/21 paraplegia, 1/21 tetraplegia, 2/21 cortical visual impairment, 3/21 visual field deficits, 4/21 strabismus. 10/21 underwent cognitive and behavioural assessment demostrating in 6 cases global delay and in 4 cases delay in specific functions (locomotor, eye and hand coordination, performance scale). Surgical procedure’s video will be shown. Conclusions SBS may influence child development and therapeutical surgical approach seems crucial.

Object. Although a rarely reported occurrence, late failure of endoscopic third ventriculostomy (ETV) may occur in children as a result of a variety of factors. Delay in recognition of symptoms can lead to harmful deterioration in the patient's condition. The authors undertook this study to assess the capacity of cine phase-contrast MR imaging to identify late failure in asymptomatic pediatric patients treated with ETV for hydrocephalus.Methods. This study was a retrospective evaluation of cases involving patients who underwent ETV between January 1, 1999, and December 31, 2008, at the pediatric neurological surgery service of the University of Padua. Before 2004, patients were routinely followed up with cine MR imaging at 3, 6, and 12 months after ETV. In 2004, a protocol of annual cine MR follow-up was instituted as a result of a case of fatal late failure. The authors evaluated all cases of late failure identified through cine MR imaging and performed a statistical analysis to investigate the relationship between ETV failure and several variables, including the cause of hydrocephalus for which ETV was originally indicated.Results. In a series of 84 patients (age range 6 days-16 years), 17 patients had early ETV failure. Of the remaining 67 patients, 5 (7%) were found to have no CSF flow through the fenestration and recurrent ventriculomegaly when assessed with cine MR imaging at 1, 2, 3, 4, and 7 years after ETV. The patient in whom ETV failure was identified 1 year postoperatively had Dandy-Walker malformation. The patients in whom ETV failure was identified 2, 3, and 4 years postoperatively all had undergone ETV for treatment of postinfective hydrocephalus. The patient in whom ETV failure was identified 7 years postoperatively had a cystic arachnopathy in the fourth ventricle after cerebellar astrocytoma removal.Conclusions. Patients who undergo ETV for infective hydrocephalus and Dandy-Walker malformation should receive long-term follow-up, because late closure of the stoma may occur progressively and slowly. Intraoperative observation of thickened arachnoid membranes at the level of the interpeduncular cisterns at the first ETV and a progressive decreasing of CSF flow through the stoma on routine cine MR imaging should be considered unfavorable elements entailing a significant risk of deterioration. (DOI: 10.3171/2011.1.FOCUS10303)

( fig. 2 ), CSF was drained and nonfunctional herniated neural tissue was removed ( fig. 3 ); a watertight dural closure and an aesthetic skin reconstruction were then performed. No attempt was made to repair the calvarial defect because of its small size (1.5 cm in diameter). One month after surgery the aesthetic result was excellent ( fig. 4 ), and at the 2-year follow-up the child was following the normal neurodevelopmental pattern. Vision was preserved, with an unchanged ocular exam. A 5-month-old infant born with a giant occipital encephalocele was treated in Haiti during a humanitarian mission. At the neurologic exam the baby was normal except for a nonpurposeful gaze. Magnetic resonance imaging showed a large multiloculated cerebrospinal fluid (CSF) cyst with minimal herniation of the nonfunctional right occipital lobe; the tentorium cerebelli and right transverse sinus were in the normal position ( fig. 1 ). In the operating room, with the baby in the prone position Received: November 25, 2010 Accepted after revision: April 12, 2011 Published online: June 23, 2011

STUDY DESIGN:A case report and literature review of the treatment of "noncommunicating" syringomyelia. OBJECTIVE.: The aim of this report is to document the timing and the treatment of hydromyelia holocord after surgical treatment for both tethering and retethering of spinal lipoma.SUMMARY OF BACKGROUND DATA:Syringomyelia associated with spinal lipoma presents a different pathogenesis and treatment in comparison to the "communicating" hydromyelia in the myelomeningocele. After the primary retethering operation performed in symptomatic patients, recurrent retethering can occur with an increase of the syringomyelia signs and symptoms.METHODS:Syringomyelia treated with a thin silastic tube passed from the syrinx to the subarachnoidal space for drainage and decompression. Prior operations were: (1) initial untethering at birth, (2) second untethering at 5 years of age, (3) posterior fossa and cervical decompression.RESULTS:Magnetic resonance imaging 6 months post shunt operation demonstrated decompression of the hydromyelia holocord and syringobulbia with improvement of motor function of the legs and improvement in sensory symptoms.CONCLUSION:Usefulness of syrinx-subarachnoidal shunt is demonstrated in this case report after unsuccessful decompression and detethering. When the enlargement of the ependymal channel is greater than 50% of the spinal cord's diameter, neurologic, and urological symptoms are evident and the patient benefitted from cord untethering and syrinx drainage. (1) The terminal "noncommunicating" syringomyelia in lumbar sacral lipoma has been reported to be associated with retethering in spinal lipoma in the 25% of the cases. (2) The rise of distal syringomyelia isn't only linked to the kind of the spinal lipoma, but also to the difficulty to obtain the untethering and a smooth cerebrospinal fluid flow between the subarachnoidal space and the ependymal canal. (3) In patients with hydromyelia holocord greater than the 50% of the spinal cord's diameter a myelotomy and insert an ependymal channel/syrinx to the subarachnoidal space shunt can resolve of the syrinx. In this case, the enlargement of the ependymal channel in "noncommunicating" syringomyelia associated with lumbosacral lipoma is greater than 50% of the spinal cord's diameter; neurologic and urological symptoms occurred and the patient benefited from cord untethering and concurrent syrinx drainage.

BACKGROUND:The growing teratoma syndrome (GTS) consists of a mature teratoma paradoxically enlarging during or after chemotherapy for malignant nongerminomatous germ cell tumors.METHODS AND RESULTS:We report two cases of GTS occurring in association with NSGCT of the pineal gland. Although an unusual event, clinicians and radiologists should be aware of its natural history.CONCLUSIONS:When normalized tumor markers after chemotherapy are associated with imaging features of a growing mass, the hypothesis of GTS must be taken in consideration. When early diagnosed, GTS can be managed surgically with good results.

Objective: Although endoscopic third ventriculostomy (ETV) is a well established procedure for the treatment of congenital and acquired hydrocephalus, there is still a controversy with regard to the higher risk of failure in younger infants compared to older children after such a procedure. The aim of this article is to define the effectiveness of third ventriculostomy for hydrocephalus in infants less than 6 months of age better and to determine possible positive predictive factors.Materials and Methods: In a series of 126 consecutive patients who underwent ETV between January 2000 and December 2002, 21 procedures were performed on infants ranging in age from 23 to 180 days, and without a previous history of shunting. The follow-up period was 49 to 82 months.Results: The overall Success rate of ETV was 67% (14/21). All failures occurred in the,early postoperative period (average 38 days, range 25-88 days). The success rate varied with the etiology of the patient's hydrocephalus. The best results were obtained in patients with acqueductal stenosis, Dandy-Walker malformation and myelomeningocele. The least favorable results (50% failure rate) occurred in infants treated for post-hemorrhagic and post-meningitic hydrocephalus.Conclusion: This study does not demonstrate a correlation between the age of the infants and the ETV success rate. The specific etiology of hydrocephalus is the most relevant prognostic factor in infants under six months of age.

The treatment of the transsphenoidal sphenopalatine encephalocele in infants has not been thoroughly described in the literature. Pterional and subfrontal transbasal approaches have been reported as the advised treatment for transethmoidal encephalocele, but their feasibility for transsphenoidal encephalocele remains controversial, particularly in neonates. The potential harm to vital structures within the herniated tissue and intraoperative bleeding have been considered the major cause of poor postoperative results. The authors present the case of a full-term newborn with a cleft palate and a large transsphenoidal sphenopalatine encephalocele, which filled the nasopharyngeal space and provided MR imaging evidence of an intralesional pituitary gland. The lesion is unusual, and the extracranial intraoral endoscopic approach with an optimal outcome and sparing of the pituitary tissue has never been described in detail in an infant of this age.

Anorectal malformations (ARMs) are associated with a variety of spinal dysraphisms, of which clinical impact is often underestimated. A 6-year-old girl, with a history of rectovaginal fistula, presented with gait disturbance, asthenia, and worsening of fecal incontinence. The spinal magnetic resonance imaging (MRI) at 12 months of age had revealed a tethered cord and a little intradural lipoma. Within the lipoma, a small cystic lesion, interpreted as a cerebrospinal fluid (CSF)-filled loculation, was also described. A consecutive MRI showed a marked increase in size of the CSF-like cyst that was clearly hyperintense on diffusion-weighted imaging (DWI) and presented reduced apparent diffusion coefficient values (855 +/- 109 s/mm), not compatible with CSF values. This lesion, interpreted as an (epi)dermoid cyst, was removed and histologically confirmed; the spinal cord was untethered. The child's lower limb motor deficit resolved rapidly after surgery, and the fecal incontinence slowly returned to the previous bowel habit. There is a growing interest in recognizing and defining spinal dysraphism in ARM patients because some abnormalities may carry severe clinical consequences. For this purpose, a standardized MR protocol is required, in which DWI plays a pivotal role to disclose associated dysembriogenetic lesions, in particular when a CSF loculation is detected. (c) 2008 Elsevier Inc. All rights reserved.