Undiagnosed sagittal synostosis as cause of "idiopathic" intracranial hypertension.

PURPOSE: Idiopathic intracranial hypertension (IIH) is a rare condition in children, but if diagnosed needs to be promptly treated to avoid clinical sequalae. The main purpose of this paper was to test our clinical experience with a cohort of normocephalic children with craniosynostosis who do not present in the routine way to craniofacial services, due to the normal head shape and age, diagnosed with IIH. METHODS: We retrospectively reviewed all children who were referred to neurosurgery from 2012 to 2022 for management of IIH on our prospectively kept database. We determined what treatments were offered and if there was an associated craniosynostosis. RESULTS: In total, 19 children were identified with an average age at referral of 11.5 years (st dev 4.0 years) with 11 male and 8 female. The most common presenting symptoms and signs were papilloedema (18/19), headaches (15/19), visual deterioration (9/19), nausea and vomiting (7/19) and diplopia (4/19). Five out of 19 children (26.3%) had a sagittal suture fused that was not identified at the time of treatment and all children were normocephalic. CONCLUSION: There is a cohort of children with IIH who will have concomitant craniosynostosis and ideally would benefit from cranial vault expansion as primary surgery rather than cerebrospinal fluid (CSF) diversion. We suggest all children with IIH requiring neurosurgical intervention have cross-sectional imaging to look for occult craniosynostosis prior to intervention.

Trampoline-Associated Cranial and Spinal Injuries: A 10-Year Study in a Pediatric Neurosurgery Center.

OBJECTIVE:  There has been an increasing use of trampolines for recreation by children in recent years. Many studies have explored the different types of injuries sustained due to falls from trampolines, but so far none have focused specifically on cranial and spinal injuries. In this study, we describe the pattern of cranial and spinal injuries sustained by pediatric patients that were associated with the use of trampolines and their management in a tertiary pediatric neurosurgery unit over a period of 10 years. METHODS:  This is a retrospective study of all children less than 16 years of age with suspected or confirmed trampoline-associated cranial or spinal injuries, managed by a tertiary pediatric neurosurgery unit from 2010 to 2020. Data collected included the patient's age at the time of injury, gender, neurological deficits, radiological findings, management, and clinical outcome. The data were analyzed to highlight any trends in the pattern of injuries. RESULTS:  A total of 44 patients with a mean age of 8 years (ranging from one year and five months to 15 years and five months) were identified. 52% patients were male. 10 patients (23%) had a reduced Glasgow Coma Scale (GCS) score. In terms of imaging findings, 19 patients (43%) had a radiologically positive head injury, nine (20%) had a craniovertebral junction (CVJ) injury, including the first (C1) and second (C2) cervical vertebrae, and six (14%) had an injury involving other parts of the spine. No patient sustained concurrent head and spinal injuries. Eight (18%) patients had normal radiological findings. Two (5%) had incidental findings on radiology that required subsequent surgery. A total of 31 patients (70%) were managed conservatively. 11 patients (25%) underwent surgery for their trauma, of which seven were cranial. Two further patients underwent surgery for their incidental intracranial diagnoses. One child died from an acute subdural hemorrhage. CONCLUSIONS:  This study is the first to focus on trampoline-associated neurosurgical trauma and report the pattern and severity of cranial and spinal injuries. Younger children (less than five years of age) are more likely to develop a head injury, whereas older children (more than 11 years of age) are more likely to develop a spinal injury following the use of a trampoline. Although uncommon, some injuries are severe and require surgical intervention. Therefore, trampolines should be used prudently with the appropriate safety precautions and measures.

Prevalence and severity of lower urinary tract symptoms in the third trimester of pregnancy.

INTRODUCTION AND HYPOTHESIS: Pregnancy is a risk factor for urinary disorders, mainly in the third trimester. Lower urinary tract symptoms (LUTS) are often underreported by health care professionals and significantly impact the quality of life of pregnant women. Our objective is to analyse lower urinary tract function during the third trimester of pregnancy and assess the impact of traditional risk factors for pelvic floor dysfunctions on bladder health in pregnant women. METHODS: This is a secondary analysis of a multicentre cross-sectional study. Third-trimester pregnant women aged 18 years or older anonymously filled in the "Italian Pelvic Floor Questionnaire for pregnant and postpartum women" questionnaire, validated for pelvic floor disorders in pregnancy and postpartum. RESULTS: A total of 927 pregnant patients completed the questionnaire. Among them, 97.3% complained of at least one urinary disorder. Frequency was the symptom reported most often (77.3%), whereas nocturnal enuresis was the least reported (17%). Despite the high prevalence of LUTS in our sample, only 13.4% reported that they negatively impact their quality of life. Overweight and obesity, advanced maternal age, smoking, family history of pelvic floor disorders and poor pelvic floor contraction capacity were confirmed to be risk factors for the onset of LUTS, even in our population. CONCLUSIONS: Urinary symptoms are extremely common in the third trimester and significantly affect the quality of life of pregnant women. Since overweight, obesity, smoking and reduced pelvic floor contractility emerged as modifiable risk factors for the development of these symptoms, prevention and adequate counselling are cornerstones of pregnancy care.

The role of surgery in relieving calcified shunt site-related pain in patients with functioning VP shunt.

PURPOSE: Shunt calcification is a known late sequela of ventriculoperitoneal (VP) shunt insertion and is associated with shunt malfunction. However, in some patients, while shunt functionality is preserved despite calcification of the catheters, they experience nociceptive symptoms. In this paper, the authors present their surgical experience in managing patients with a functional VP shunt and experiencing pain secondary to shunt calcification. METHODS: We analysed outcomes of patients presenting with pain at the level of a calcified shunt who underwent surgical untethering of the calcified catheter from the soft tissues. This procedure was commenced by the senior author in 2015. Patients were collected prospectively from the databases of two institutions. Evidence of shunt calcification was confirmed on neuroimaging. RESULTS: Seven patients, two male and five female, were included. The mean age at untethering was 13.5 years. The mean time interval between primary shunt surgery and symptom onset was 12 years (range 6-16 years). The commonest site of tethering was the neck (50%) followed by abdomen and chest (both 25%). Six patients underwent untethering of the catheter from soft tissues. One patient had removal of a redundant segment of calcified shunt left in situ during a previous revision. All patients experienced pain relief following shunt untethering. CONCLUSION: Untethering of calcified VP shunt catheters from soft tissue can be considered an effective treatment of shunt site pain and offered to patients presenting with a functional VP shunt.

ERF-related craniosynostosis and surgical management in the paediatric cohort.

INTRODUCTION: ERF mutation is one of the most recently identified genetic aberrations associated with syndromic craniosynostosis. Data on the pattern of craniosynostosis, surgical management of ERF-related craniosynostosis and outcomes is limited. We report on our single-centre experience in paediatric cohort of patients with syndromic craniosynostosis secondary to ERF mutation. METHODS: A retrospective review of all paediatric craniofacial cases was performed over an 8-year period (2014-2022). All patients with genetically confirm ERF-related craniosynostosis were identified, and clinical parameters including, age, sex, pattern of craniosynostosis, associated tonsillar herniation and follow-up period were further analysed from electronic clinical and imaging systems. All patients were selected and discussed in multidisciplinary craniofacial meeting (composed of neurosurgical, maxillofacial, plastics and genetics teams) prior to any surgical intervention. RESULTS: Overall, 10 patients with ERF-related craniosynostosis were identified with a male-to-female ratio of 4:1 with mean age at the time of surgery of 21.6 months with a mean follow-up period of 5.2 years. ERF-confirmed cases led to variable craniosynostosis pattern with multi-sutural synostosis with concurrent sagittal and bilateral lambdoid involvement as the most common pattern (7/10). No patient pre-operatively had evidence of papilloedema on ophthalmological assessment. Eight out of 10 patients had associated low-lying tonsils/hind brain hernia pre-operatively. Eight out of 10 patients required surgery which included 2 fronto-orbital advancement, 3 calvarial remodelling, 2 posterior calvarial remodelling/release and 1 insertion of ventriculoperitoneal shunt. CONCLUSION: Involvement of sagittal and lambdoid sutures is the most common pattern of craniosynostosis. ERF-related craniosynostosis can have variable pattern of suture fusion, and management of each patient requires unique surgical planning and execution based on clinical needs for the optimal outcomes.

A 10-year retrospective observational study on the utility and prescription standards of dexamethasone in pediatric neuro-oncosurgery in a tertiary care center.

OBJECT: This study aimed to retrospectively assess dexamethasone utility in pediatric CNS tumor patients over a 10-year period, to better understand dosing variability, and highlight optimal practice. METHODS: All pediatric CNS tumor cases managed operatively for a 10-year period at a single center were reviewed. Information was gathered on demographics, dexamethasone doses, course durations, weaning regimes, PPI co-prescription, adverse events, and route of administration. Comparison within these groups was analyzed through use of statistical testing. RESULTS: One hundred twenty-seven patients received 193 dexamethasone courses. Median age was 7 years, with a median weight of 27.9 kg. Most common tumor type was astrocytoma (24.8%). Median daily dose was 8 mg, with twice-daily dosing most common. Median course duration was 8 days, ranging from 1 to 1103 days. Median weaning duration was 11.5 days. Daily dose was not correlated with patient weight and the median daily dose per kg was 0.2319 mg/kg. Incidence of adverse effects was 14.5% across all course lengths, with weight gain most common. The short-term course duration (<14 days) had the lowest adverse event incidence, with direct correlation between course length and adverse effect incidence. Dexamethasone dose per kg was not significantly different between patients with and without adverse effects. No relationship was noted between adverse effects incidence and administration route (intravenous compared to oral). 64.2% of patients received concurrent PPI with 35.8% receiving no PPI, with 1 gastrointestinal side effect noted in the PPI-receiving population. CONCLUSIONS: Large variation was seen in practice, with prescriptions appearing based on clinician preference and symptom severity rather than patient age or weight. Future guidelines should consider lower dose regimens than are currently presented with less frequent dosing as these may benefit quality of life. Weaning period can be relatively rapid for most patients, taking place in 2-3 days. PPI co-prescription does not seem to add significant benefit. We recommend using a standardized guideline of 0.2 mg/kg/day (max 8 mg/day) given OD or BD, with PPI cover where necessary. For acute presentations, we recommend limiting dexamethasone treatment to <14 days. These recommendations can be adjusted for individual cases to yield optimal results.

Posterior Calvarial Distraction in older paediatric population: single centre paediatric neurosurgery craniofacial unit outcomes.

PURPOSE: Posterior calvarial distraction (PCD) is a safe and effective technique used to increase cranial vault volume and therefore reduce intracranial pressure in children with complex craniosynostosis. Optimal timing and method used for PCD is controversial. This procedure is usually performed in children younger than 2 years. Literature regarding calvarial distraction in older children is sparse and limited. We report our single-centre experience with PCD in children aged 6 and above to outline the applications, benefits and challenges of employing this technique in an older paediatric population. METHODS: A retrospective analysis of a database on craniofacial cases from 2006 to 2021 was performed. Patients undergoing PCD were identified and children aged 6 and above at the time of operation were included. Data on demographics and clinical outcomes were obtained from electronic records and relevant imaging was reviewed. All cases were reviewed prior to a decision for surgery by the multidisciplinary craniofacial team (composed of neurosurgery, maxillofacial and plastics teams) and underwent surgery in our paediatric craniofacial centre. RESULTS: Overall, 98 PCD cases were identified during the study period, of which 20 cases were identified as having undergone PCD at age 6 or above with mean age of 8.8 years (range 6-18). The most common indication was pansynostosis associated with raised intracranial pressure. Four cases had calvarial remodelling previously and represented with symptoms of raised intracranial pressure sometime after their initial surgery requiring PCD as rescue procedure. Average duration of inpatient stay was 5.85 days. The average duration of follow-up was 3.5 years (0.3 to 11 years). Mean distraction distance achieved was 22.5 mm (18-29 mm). Five patients experienced complications related to wound infection or distractor. Follow-up assessment in all patients demonstrated evidence of vault expansion and symptomatic improvement and resolution of intracranial pressure signs. Comparison with younger cohort did not reveal any difference in any parameters except lower rate of transfusion in the older cohort compared to young cohort (5% vs 38%). CONCLUSION: Posterior calvarial distraction in older children is safe and effective for vault expansion and treatment of raised intracranial pressure in selected cases. A multidisciplinary craniofacial team approach is crucial for appropriate case selection and management in order to optimise outcomes.

Prevalence and severity of pelvic floor disorders in pregnant and postpartum women.

OBJECTIVE: This multicenter study aimed to evaluate risk factors, prevalence and severity of pelvic floor disorders (PFDs) as well as their consequences on women's emotional well-being, using a questionnaire validated specifically for pregnancy and postpartum. METHODS: Prospective study conducted in eight teaching hospitals in Italy and Italian-speaking Switzerland. Pregnant and postpartum women completed the Italian Pelvic Floor Questionnaire for Pregnancy and Postpartum anonymously. Prevalence of, severity of, and risk factors for PFDs were evaluated for all the four domains considered: bladder, bowel, prolapse, and sexual function. RESULTS: A total of 2007 women were included: 983 of the patients were bothered by at least one kind of PFD: bladder, bowel, and sexual dysfunction were more frequently reported. There were no significant differences in PFD prevalence between pregnancy and postpartum, except for bladder disorders, which were more prevalent in pregnancy. Familiarity for PFDs, pelvic floor contraction inability, cigarette smoking, body mass index more than 25 (calculated as weight in kilograms divided by the square of height in meters), and age more than 35 years were confirmed risk factors for the development of PFDs during pregnancy and postpartum. CONCLUSION: Almost half of the women included in the study suffered from PFD-related symptoms with important consequences on quality of life. Validated questionnaires are fundamental in early diagnosis and treatment of PFDs.

Posterior Fossa Decompression and Duraplasty with and without Arachnoid Preservation for the Treatment of Adult Chiari Malformation Type 1: A Systematic Review and Meta-Analysis.

BACKGROUND: The best surgical treatment for adult Chiari malformation type 1 remains widely debated. OBJECTIVE: This study aimed to assess the efficacy of posterior fossa decompression and duraplasty with arachnoid preservation compared with arachnoid dissection for the treatment of adult Chiari malformation type 1. METHODS: Two reviewers (M.O.-G. and M.A.) performed a PubMed, MEDLINE, and Embase literature search using the following terms: ("Chiari" OR "Chiari 1") AND ("duraplasty" OR "arachnoid preservation" OR "arachnoid spar∗" OR "posterior fossa surgery" OR "posterior fossa decompression" OR "foramen magnum decompression"). Studies assessing the efficacy of posterior fossa decompression with duraplasty for the treatment of patients aged >18 years with Chiari malformation type 1 were included. Case reports with <10 patients, editorials, and non-English studies were excluded. RESULTS: Of 195 studies identified, 24 were included for meta-analysis. In the 1006 participants, there was no difference in postoperative clinical or radiologic improvement between the 2 techniques. Patients who underwent posterior fossa decompression with duraplasty and arachnoid dissection had a greater prevalence of total complications (0.20, 95% confidence interval [CI], 0.13-0.29 vs. 0.09, 95% CI, 0.05-0.14; Q = 6.47; P = 0.01) and cerebrospinal fluid-related complications (0.15, 95% CI, 0.10-0.22 vs. 0.05, 95% CI, 0.02-0.12; Q = 4.88; P = 0.03) compared with arachnoid preservation. Furthermore, the prevalence of reoperation in the arachnoid dissection group was 25 times greater than in the arachnoid preservation group (0.08, 95% CI, 0.06-0.10 vs. 0.003, 95% CI, 0.00-0.02; Q = 10.73; P > 0.001). CONCLUSIONS: Posterior fossa decompression and duraplasty with arachnoid preservation is a beneficial technique to treat Chiari malformation type 1 and reduces the risk of complications, particularly cerebrospinal fluid-related complications and the rate of reoperation.

Guideline for the management of long tunnelled external ventricular drains in chronic hydrocephalus.

This article reports on the journey of a child with an inoperable hypothalamic-origin pilocytic astrocytoma causing hydrocephalus, which was refractory to treatment with shunts, and required a new approach. With multidisciplinary support, excellent nursing care and parental education, the child's hydrocephalus was managed long term in the community with bilateral long-tunnelled external ventricular drains (LTEVDs). This article describes the patient's journey and highlights the treatment protocols that were created to achieve this feat. Despite the difficulties in initially setting up these protocols, they proved successful and thus the team managing the patient proposed that LTEVDs are a viable treatment option for children with hydrocephalus in the context of inoperable tumours to help maximise quality of life.

Third ventricular colloid cyst causing acute hydrocephalus during early pregnancy: Clinical lessons from a case.

BACKGROUND: Colloid cysts of third ventricle are rare lesions which may present with symptoms of acute hydrocephalus. We report a case of acute obstructive hydrocephalus secondary to third ventricular colloid cyst and its management during 1st trimester pregnancy. CASE DESCRIPTION: A 31-years-old lady presented to the emergency department with reduced consciousness. Computed tomography head showed obstructive hydrocephalus and an obstructing lesion near foramen of Monro suggestive of third ventricle colloid cyst. She underwent endoscopic resection of colloid cyst. Her pregnancy was only confirmed after surgery and she delivered a healthy neonate at full term. She has remained clinically well and had resumed her work as a general practitioner. CONCLUSION: This case illustrates that urinary pregnancy test may show false negative result but pregnancy should not preclude radiological investigation and neurosurgical intervention in patients with deteriorating neurological function.

Management of patients with a failed kidney transplant: what should we do?

The number of kidney transplant recipients returning to dialysis after graft failure is steadily increasing over time. Patients with a failed kidney transplant have been shown to have a significant increase in mortality compared with patients with a functioning graft or patients initiating dialysis for the first time. Moreover, the risk for infectious complications, cardiovascular disease and malignancy is greater than in the dialysis population due to the frequent maintenance of low-dose immunosuppression, which is required to reduce the risk of allosensitization, particularly in patients with the prospect of retransplantation from a living donor. The management of these patients present several controversial opinions and clinical guidelines are lacking. This article aims to review the leading evidence on the main issues in the management of patients with failed transplant, including the ideal timing and modality of dialysis reinitiation, the indications for an allograft nephrectomy or the correct management of immunosuppression during graft failure. In summary, retransplantation is a feasible option that should be considered in patients with graft failure and may help to minimize the morbidity and mortality risk associated with dialysis reinitiation.

Evaluation and Management of Patients with Hydrocephalus in Craniosynostosis.

BACKGROUND: Hydrocephalus in presence of craniosynostosis, though relatively rare, occurs in 4%-10% cases, with an increased incidence in syndromic craniosynostosis. The optimum management in these patients is unknown. MATERIALS AND METHODS: A search was performed on the departmental craniofacial database to identify all patients with craniosynostosis and hydrocephalus from January 2000 to December 2020. Diagnosis was confirmed by a meticulous review of the notes and previous imaging. These patients were grouped into two groups based on the primary treatment they received: either a cerebrospinal fluid (CSF) diversion procedure or a calvarial remodeling procedure. By analyzing the outcomes for each group, we endeavor to rationalize and outline our management strategy for this complex cohort of patients. RESULTS: Sixty-four of 989 patients were confirmed to have hydrocephalus. Of these, 55 patients underwent calvarial expansion while nine had CSF diversion as a primary procedure. Our study demonstrates that the complication rate is lower in the primary calvarial expansion group. Furthermore, the need for a CSF diversion procedure was avoided in a significant number of these patients as a direct result. CONCLUSION: In the vast majority of patients with craniosynostosis and hydrocephalus, calvarial expansion surgery should be the preferred primary management option.

DelCFHR3-1 influences graft survival in transplant patients with IgA nephropathy via complement-mediated cellular senescence.

IgA nephropathy (IgAN) is a frequent cause of chronic kidney disease (CKD) and progressive renal impairment. A native renal biopsy diagnosis of IgAN is a predictor of graft loss, with a relative risk of 47% but it is difficult to predict graft survival and progressive allograft dysfunction in these patients. Deletion of complement factor H-related genes 1 and 3 (delCFHR3-1) has been associated with a decreased risk of developing IgAN on native kidneys, but the impact on the graft in IgAN-transplanted patients is unknown. We hypothesized that delCFHR3-1 is also associated with the processes that influence graft survival in transplant recipients with IgAN and tested whether cellular senescence is involved in mediating graft damage. We found that patients carrying two copies of CFHR1-3 had a worse outcome (P = .000321) and presented increased FHR1 deposits at glomerular and tubulointerstitial level associated with higher expression of the senescence marker p16INK4a (P = .001) and tubulointerstitial fibrosis (P = .005). Interestingly, FHR1 deposits were associated with increased complement activation as demonstrated by C5b-9 deposits. These data support both the role of FHR1 in mediating complement activation and tubular senescence, and suggest the possibility of genotyping delCFHR3-1 to predict graft survival in IgAN-transplanted patients.

First study on PCBs, organochlorine pesticides, and trace elements in the Eurasian otter (Lutra lutra) from southern Italy.

Persistent organic pollutants (POPs), such as organochlorine pesticides (OCPs) and non dioxin-like polychlorinated biphenyls (ndl-PCBs), or also other toxic and potentially toxic elements tend to accumulate in the trophic web, and are associated with a wide range of deleterious effects in animals and humans. Although these contaminants have been banned since more than 30 years, their occurrence remains widespread in most of natural ecosystems. The Eurasian otter (Lutra lutra), being a top predator may cumulate large amounts of these contaminants, and could represent a keystone species serving as sentinel of ecosystem health in freshwater environments. The aim of this paper was to investigate the concentration of OCPs, ndl-PCBs, and essential and not essential elements in tissue samples of Eurasian otter in southern Italy. Six PCBs, seven OCPs, and thirteen trace elements were detected with high concentrations in few cases, however in general, the amounts of toxic compounds seem unlikely to hazard short-term conservation of this endangered mammal. The present paper is the first to provide baseline information concerning the presence of these substances in the Eurasian otter samples and from its fragile ecosystem in Italy.

Fertility preservation in women affected by gynaecological cancer: the importance of an integrated gynaecological and psychological approach.

Gynaecological cancer treatment significantly affects the fertility of women in reproductive age. Surgery, chemotherapy and radiotherapy are the mainstays of ovarian, cervical and endometrial cancers and anatomically or functionally impact the uterus and ovaries. Moreover, the sexual function and psychological wellbeing of patients are highly weakened after a cancer diagnosis: depression, anxiety and impairment of quality of life represent a relevant concern for patient care. The potential loss of fertility could be more distressing than cancer itself. For this reason, it is of paramount importance to try to preserve fertility in women affected by gynaecological cancers. Recently, tailored fertility preservation therapies have been developed to meet the childbearing demand from more than half of women between 18 and 40 years with a diagnosis of cancer. Currently, fertility preservation techniques play a significant role in improving the quality of life of women with gynaecological cancer. In this scenario, we propose a narrative overview of the recent literature about the importance of a multidisciplinary approach in the management of fertility preservation in the case of gynaecological cancers.

External validation of a new classification of spinal lipomas based on embryonic stage.

OBJECTIVE: Contention exists regarding appropriate classification and management of spinal lipomas (SLs). Given the heterogeneity of SLs, omissions and overlap between surgically incomparable groups exist in conventional classification systems. The new classification of spinal lipoma (NCSL) recently proposed by Morota et al. delineates morphology by embryological pathogenesis and the resultant operative difficulty. Here, the authors aimed to validate the NCSL by applying it to patients who had been operated on at their institution. METHODS: All children who had undergone resection for SL between 2014 and 2018 were included in this analysis. MRI studies were independently reviewed and classified by three adjudicators. Baseline characteristics, inter-adjudicator agreement, coexisting anomalies and/or malformations, and postoperative outcomes and complications were analyzed. RESULTS: Thirty-six patients underwent surgical untethering for SL: NCSL type 1 in 5 patients (14%), type 2 in 14 patients (39%), type 3 in 4 patients (11%), and type 4 in 13 patients (36%). All classification was agreed on first assignment by the adjudicators. Radical or near-radical resection, which was attempted in all patients, was always possible in those with type 1 and 4 SL, but never in those with type 2 and 3 SL. Neurological stabilization and/or improvement were observed in all patients at the last follow-up. CONCLUSIONS: The NCSL was found to be a logical and reproducible system to apply in this SL population. All cases were successfully classified with a high degree of inter-assessor agreement. Widespread establishment of a commonly adopted and clinically useful classification system will enable clinicians to improve patient selection as well as discussion with patient representatives during the decision-making process.

Custom-Made Three-Dimensional Models for Craniosynostosis.

The use of computer-aided design and computer-aided manufacturing in oral and maxillofacial surgery is an ever-growing field.The availability of 3D models, cutting guides, and customised surgical instruments gives surgeons the opportunity to modify and improve their surgical procedures.Here, we discuss the use of computer-aided design-computer-aided manufacturing to improve the management of a case of nonsyndromic metopic synostosis through the construction of: A cutting guide for the cranium, custom-made orbital protectors, a 3D model of the predicted postoperative meninges to allow off the table bone recontouring, and a template frontal bar to allow more specific recontouring of the frontal bar.

A novel management proposal for intrinsic brainstem neurenteric cysts: case report.

Neurenteric cysts (NCs) are rare congenital lesions with epithelial mucin-secreting walls. They can occur anywhere along the neural axis, and an intrinsic midbrain cyst is extraordinary. Surgical management may pose a challenge due to the location of the lesion and adhesion of the cyst wall to the surrounding brainstem. The authors describe the first case of pediatric NC that was treated successfully with intracystic interferon-α (IFN-α).A 16-month-old baby girl presented with a 2-week history of progressive croup, vomiting, and swallowing difficulty. MRI revealed a 1.8-cm cystic intrinsic lesion in the pontomedullary region. She initially underwent posterior fossa craniotomy and drainage of the cyst under intraoperative neurophysiology monitoring. Three weeks following the procedure, her symptoms recurred, and follow-up MRI demonstrated cystic recurrence. She underwent repeat aspiration of the cyst and biopsy of the cyst wall, and INF-α-2b was injected into the cystic cavity. Her symptoms improved and completely resolved after 5 months. A 9-month follow-up brain MRI study showed complete resolution of the NC. Intracystic IFN-α injection after cystic content aspiration may be a safe treatment option for the management of intrinsic brainstem NCs. Long-term clinical and radiological follow-up is recommended.

De novo distal middle cerebral artery aneurysm post-excision of intracerebral arteriovenous malformation in an 8-year old.

Cerebral arteriovenous malformations (AVMs) are frequently associated with concurrent aneurysms. These aneurysms are commonly haemodynamically related to the AVM and can be classified into extranidal or intranidal in reference to the AVM nidus. An aneurysm arising from an artery that does not angiographically contribute to the blood flow to the AVM is uncommon. A distal middle cerebral artery (dMCA) aneurysm itself is also a rare presentation, especially in paediatric population. We present a rare case of dMCA aneurysm that was noted after successful surgical management of a ruptured AVM in an 8-year-old child and its management. BACKGROUND: About 10-30% of patients with cerebral arteriovenous malformation (AVM) have an associated artery aneurysm. The majority of these aneurysms are flow-related to the malformation. These aneurysms can be classified into extranidal or intranidal in reference to the AVM nidus Rammos et al Am J Neuroradiol 37:1966-1971, [1]. An aneurysm arising from a different artery that does not angiographically contribute to the blood flow associated with the AVM is less common and would generally be regarded as unrelated to the AVM. Distal cerebral artery aneurysm itself is also a rare presentation, comprising of 1-7% of all middle cerebral artery aneurysm. In children, mycotic infection and dissection are the two most common causes for aneurysm in this location. Unlike in adults, berry aneurysms are uncommon in children. We describe a young patient who was found to have distal middle cerebral artery (dMCA) aneurysm in follow-up DSA (Digital Subtraction Angiogram) after the initial successful surgical treatment for a cerebral frontal AVM. In this particular case, endovascular repair is thought to be the best strategy to treat the aneurysm. However, there still remains a lack of consensus of the best management strategy (surgery or endovascular) in treating flow-related aneurysms in general. This is usually based on an individual case scenario and the treatment is tailored depending on various factors including the expertise of the treating team.