Practical Algorithm for the Management of Multisutural Craniosynostosis with Associated Chiari Malformation and/or Hydrocephalus.

INTRODUCTION: The association between multisutural craniosynostosis with Chiari malformation (CM), venous hypertension, and hydrocephalus is widely described in the literature, especially in children with paediatric craniofacial syndromes. Some efforts have been done in the last years to understand the complex pathogenetic mechanisms underlying this association, and several theories have been proposed. In particular, it is now accepted that the hypothesis of the overcrowding of the posterior fossa due to precocious suture fusion is the cause of the cerebellar herniation in syndromic and non-syndromic patients, against the theory of intrinsic cerebellar anomalies, ventriculomegaly, and venous hypertension. However, whatever the pathophysiological mechanism, it is still unclear what the best management and treatment of CM and hydrocephalus are in multisutural craniosynostosis patients. The aim of this study was to report our 25 years' experience in treating paediatric patients affected by these rare pathologies in order to propose a simple and effective therapeutic flow chart for their management. MATERIALS AND METHODS: We retrospectively collected data of each patient who underwent a cranial vault remodelling (CVR) for complex multisutural craniosynostosis in our institution in the last 25 years, while monosutural craniosynostosis was excluded. We recorded data concerning type of craniosynostosis and craniofacial syndromes, presence of ventriculomegaly, and CM at presentation and clinical and radiological follow-up. Therefore, we evaluated the final outcomes (improved, stable, deteriorated) of these patients and created a practical flow chart that could help physicians choose the best surgical treatment when different pathological conditions, as Chiari malformation I (CMI) or hydrocephalus, affect complex craniosynostosis children. RESULTS: Thirty-nine patients (39 out of 55; 70.9%), with an isolated multisutural craniosynostosis at presentation, underwent a two-step CVR as first surgery; 36 patients (92.3%) had an improved outcome, 2 patients (5.1%) had a stable outcome, and 1 patient (2.56%) had a deteriorated outcome. Other eight children (8 out of 55; 14.5%) had a radiological evidence of asymptomatic CMI at presentation. In this group, we performed CVR as first surgery. As for the final outcome, 7 patients had an improved outcome (87.5%) with good aesthetic result and stability or resolution of CMI. Finally, 7 patients (7 out of 55; 12.7%) presented a various combination of CMI and ventriculomegaly or hydrocephalus at presentation. Among them, 3 patients had an improved outcome (42.8%), and 4 patients had a deteriorated outcome (57.1%). DISCUSSION: The prevalence of one pathological condition with associated symptoms over the others was the key factor leading our therapeutic strategy. When craniosynostosis is associated with a radiological CM, the assessment of clinical symptoms is of capital importance. When asymptomatic or pauci-symptomatic, we suggest a CVR as first step, for its efficacy in reducing tonsillar herniation and solving CM symptoms. When craniosynostosis is associated with ventricular enlargement, the presence of intracranial hypertension signs and symptoms forces physicians to first treat hydrocephalus with a ventriculo-peritoneal shunt or endoscopic third ventriculostomy. For patients with various degrees and severity of ventriculomegaly and associated CM, the outcomes were very heterogeneous, even when the same therapeutic strategy was applied to patients with similar starting conditions and symptoms. This is maybe the most unexpected and least clear part of our results. Despite the proposed algorithm comes from a clinical experience on 85% successfully treated patients with multiple craniosynostosis, more extensive and deep studies are needed to better understand CM and hydrocephalus development in such conditions.

Ultrasonography as first line imaging for the diagnosis of positional plagiocephaly.

BACKGROUND: In the last years, numerous studies evaluated different tools for the diagnosis of positional plagiocephaly (PP). The purpose of this study was to evaluate ultrasonography (US) as a first line screening test of lambdoid sutural patency in child with PP and to compare our results with the literature. METHODS: All consecutive patients who referred to our Institute from January 2016 to October 2017 with the suspicion of PP, were included in the study and performed US examination of the lambdoid sutures. A 3-6-month clinical follow-up was performed by a pediatric neurosurgeon or a pediatrician to confirm the diagnosis of PP. RESULTS: Thirty-five children performed US examination and in all cases the diagnosis of PP was confirmed. No cases of anticipated suture fusion were examined during this period. The concordance between US findings and clinical exam follow-up was 100%. CONCLUSIONS: Ultrasonography of the lambdoid sutures represents an ideal first-line screening test and reliable alternative to other diagnostic techniques for lambdoid sutural patency in child with PP, being radiation free, fast and cheap.

Peculiar bony involvement of sinus pericranii in children: Extensive diploic erosion in three "karstic" variants.

OBJECT: Sinus pericranii (SP) is a rare, benign, extradural venous anomaly whose extracranial connection consists in an enlarged subgaleal drainage composed of a network of thin-walled veins that form a varix on the external table of the skull. In the present series of three patients we present three variantesof SP which have never been described, characterized by an extensive diploic erosion causing a "karstic" effect. METHODS: A systematic review was conducted in compliance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline. A comprehensive search for relevant articles was performed on PubMed from 1992 to 2022 using the following search words: "sinus pericranii". RESULTS: The search produced 77 articles with 137 patients. In all the cases analysed, there were not reported any patients with extensive diploic erosion, namely "karstic" variants. So, the authors present clinical history, neuroimages, treatment strategies and outcome of 3 patients with a "karstic" SP with the aim to describe this clinicradiological entity. CONCLUSIONS: In this study, our group identified a peculiar variant of SP characterized by bony involvement with extensive diploic "karstic" erosion of diploe, with possible tendency to enlarge during time.

Technical description of a novel device for external ventricular drainage in neonatal and pediatric patients: Results from a single referral center experience.

BACKGROUND: Since external ventricular drainage (EVD) related infections are usually due to skin flora, an extradural intra/extra-cranial accessory device, developed for pediatric patients under three years of age undergoing EVD positioning, is described. The aim of this paper is to provide technical description of this device, underlining the possibility to reduce infective risk and to prevent EVD dislocation. METHODS: Patients undergoing A-D device EVD placement between 1990 and 2017 at authors' institution were retrospectively considered. The device was made of a fully MRI-compatible inert material (Ketron-Peek-1000), composed of two pieces securely fixable to the skull, bridging the catheter directly from the epidural space to the extracranial space without letting it come in contact with the skin. RESULTS: A total number of 350 patients were considered. The mean age was 1.4 years, being the youngest patient a newborn of 25 weeks of gestational age. Mean time of EVD maintenance was 45 days, ranging from 21 to 81 days. 2 cases (0.6%) of EVD related infections were reported, while, pull-out of the ventricular catheter occurred in 3 cases (0.9%). No cases of bone fractures related to the clamp effect provided by A-D device were reported in the series CONCLUSIONS: This device could represent a safe and feasible option to reduce EVD related infections and catheter pull-out in pediatric patients. The encouraging results could strength the aim of the device to allow safer and longer length of CSF drainage. Moreover, the fully MRI-compatible nature and its non-magnetic properties allow to use it with neuronavigation systems.

Limits and pitfalls of indirect revascularization in moyamoya disease and syndrome.

Moyamoya vasculopathy is a rare chronic cerebrovascular disorder characterized by the stenosis of the terminal branches of the internal carotid arteries and the proximal tracts of anterior and middle cerebral arteries. Although surgical revascularization does not significantly change the underlying pathogenic mechanisms, it plays a pivotal role in the management of affected individuals, allowing to decrease the risk of ischemic and hemorrhagic complications. Surgical approaches may be direct (extracranial-intracranial bypass), indirect, or a combination of the two. Several indirect techniques classifiable according to the tissue (muscle, periosteum, galea, dura mater, and extracranial tissues) or vessel (artery) used as a source of blood supply are currently available. In this study, we reviewed the pertinent literature and analyzed the advantages, disadvantages, and pitfalls of the most relevant indirect revascularization techniques. We discussed the technical aspects and the therapeutical implications of each procedure, providing a current state-of-the-art overview on the limits and pitfalls of indirect revascularization in the treatment of moyamoya vasculopathy.

Radiation-Induced Moyamoya Syndrome in Children with Brain Tumors: Case Series and Literature Review.

BACKGROUND: Over the last decades, significant advancements have been achieved in the treatment of pediatric brain tumors as a result of radiation therapy (RT). With the increasing diffusion of this treatment, iatrogenic damage to cerebrovascular tissues contouring the radiation target volume has become the subject of debate, especially radiation-induced moyamoya syndrome (RIMS). METHODS: A systematic literature search was performed on the association between moyamoya vasculopathy and cranial irradiation in children. Large case series of patients with moyamoya were analyzed and clinicoradiologic data were collected reviewing pediatric patients treated with RT for primary brain tumors at our institution. RESULTS: The risk of developing RIMS is higher in younger children, in patients with optic pathway glioma, and in those receiving higher radiation doses. Headache is the most common presenting symptom and cerebral infarction is frequent. The preferred surgical techniques were pial synangiosis and encephaloduroarteriosynangiosis. In our case series, surgical revascularization led to neovascularization, with clinical improvement or stability in all patients. Medical therapy did not significantly affect the clinical course. CONCLUSIONS: Pediatric patients receiving involved field RT for the treatment of brain tumors have an increased risk of developing RIMS. Prompt diagnosis and early surgical revascularization play a pivotal role in decreasing the clinical impact of this complication. The use of new techniques, such as the intensity-modulated RT, and the increasing dose saving for the organs at risk, are essential to prevent iatrogenic vasculopathy. The combination of appropriate medical therapy and surgery will improve patient management and clinical outcome.

Pure Bilateral Lambdoid and Posterior Sagittal Synostosis (Mercedes-Benz Syndrome): Case Report and Literature Review.

BACKGROUND: Bilateral lambdoid and posterior sagittal synostosis is a rarely encountered multisutural craniosynostosis accounting for 0.3%-0.7% of all craniosynostoses. It has been named "Mercedes-Benz Syndrome" (MBS) because of the bone ridges that resemble the Mercedes-Benz logo. The typical head shape is usually described as anterior turricephaly combined with mild brachycephaly. We describe a case of pure MBS without involvement of other sutures. Since we believe that MBS is a different pathology when other sutures are involved, we will discuss our case with a review of the literature, including all reported cases of bilateral lambdoid and posterior sagittal synostosis with no other sutures involved but sagittal and bilateral lambdoid. CASE DESCRIPTION: A 3-month-old female baby came to our attention because of a turrycephalic appearance of the head. Findings of the neurologic examination were unremarkable. Computed tomography scan showed premature fusion of posterior third of sagittal suture and bilateral lambdoid suture. Magnetic resonance imaging did not show relevant intracranial abnormalities. She underwent sagittal decompression extended posteriorly below the lambdoid suture combined with biparietal decompression to obtain expansion of both parieto-frontal bones and posterior fossa. Post-operative course was uneventful. Follow-up at 3 months showed a good aesthetic result, and results of the neurologic examination were unremarkable. CONCLUSIONS: Pure MBS is a rare condition that unlike other multisutural complex craniosynostosis is rarely associated with genetic syndromes, has a low rate of associated intracranial anomalies, and has less need for more skull-remodeling surgery. The choice of the most suitable surgical procedure must be tailored on the patient, considering preoperative head appearance and eventually associated anomalies.

Dermoid and Epidermoid Cysts of Scalp: Case Series of 234 Consecutive Patients.

BACKGROUND: Dermoid and epidermoid cysts are among the most frequent lesions of the scalp in the pediatric population. Optimal management of a suspected dermoid or epidermoid cyst in children is debated: Some authors advocate conservative management on the basis of the benign histology of these entities, while others prefer surgical excision. OBJECTIVE: Our objective is to demonstrate that excision of dermoid and epidermoid cysts is a safe procedure and that early surgery is effective in preventing potential complications related to cyst growth. METHODS: We retrospectively collected data on patients who underwent surgery for excision of proven dermoid or epidermoid cysts between January 2006 and October 2017. RESULTS: In 234 patients, 237 cysts were excised. Mean age at presentation was 26.99 ± 32.7 months; 48.7% of patients were operated on between 1 and 3 years of age, and 32.9% were younger than 1 year of age. Cysts were more frequently located in the frontal bone than in occipital and supraorbital regions. In 36.28% of cases there was no significant cranial impingement, while 21.94% of the cysts eroded through a partial thickness of the cranium, 12.23% were in the full thickness of the skull, and 0.84% had epidural extension. We identified 22 intradiploic cysts. Statistical analysis demonstrated significant association between frontal and pterional localization and bone erosion. Neither major complications nor cyst recurrence were observed. CONCLUSIONS: Excision of dermoid and epidermoid cysts is a safe procedure for neurosurgeons dealing with this disease, even in young patients. Early resection is recommended due to the potential adverse effects that may occur if these cysts are left untreated.

Diagnosis of cerebral vasospasm and risk of delayed cerebral ischemia related to aneurysmal subarachnoid haemorrhage: an overview of available tools.

In the first weeks following aneurysmal subarachnoid haemorrhage, cerebrovascular alterations may impact the outcome significantly. Diagnosis of cerebral vasospasm and detection of alterations at risk of delayed cerebral ischemia are key targets to be monitored in the post-acute phase. Available tools include clinical monitoring, as well as studies that can detect possible arterial narrowing, alterations of perfusion, metabolism and neurophysiology. Each technique is able to investigate possible vascular impairment and has different advantages and limits. All available techniques have been described. Among these, the most practical have been selected and compared for their peculiar characteristics. Based on this analysis, a flowchart to monitor these patients is finally proposed.

Naso-ethmoidal encephalocele with bilateral orbital extension: report of a case in a western country.

INTRODUCTION: Encephalocele is a rare congenital malformation of the central nervous system with protrusion of cranial content (meninges, brain, and ventricles in different combinations) beyond the normal confines of the skull. Anterior encephaloceles occur with a high frequency in Southeast Asia, while in the Western countries occipital encephaloceles prevail. The treatment of an anterior (naso-ethmoidal) encephalocele involves a neurosurgeon or a multidisciplinary team (neurosurgeon, maxillofacial surgeon, plastic surgeon, and ENT surgeon) dealing with craniofacial surgery. Goals of surgery include removal or repositioning of nonfunctional cerebral tissue, closure of the dura, and reconstruction of skeletal and cutaneous structures. The prognosis depends from the anatomical site, volume of neural contents, and the presence of coexisting malformations. CASE REPORT: We report the case of an Italian child suffering from a naso-ethmoidal encephalocele with bilateral orbital extension. The surgical treatment was performed in two steps. CONCLUSION: Sincipital encephalocele is a complex pathology without a unique standardized surgical treatment. Its low incidence in Western countries can make its management particularly tricky.

Pituicytomas: radiological findings, clinical behavior and surgical management.

BACKGROUND: Pituicytomas are rare low-grade glial neoplasms originating in the neurohypophysis or infundibulum that appear as solid, noninfiltrative masses occupying the sella turcica, the suprasellar space or both. Due to their high tendency to bleed and the possible firmness and adhesion to surrounding structures, preoperative diagnosis of pituicytoma is a goal for the surgeon, but it is actually a challenge because of the absence of distinctive clinical or neuroradiological findings. METHODS: We report the new cases of three patients who underwent transsphenoidal surgery for suspected pituitary adenomas, which were determined to be pituicytomas at histological and immunohistochemical analysis. We also review previously reported cases of pituicytoma in the literature. RESULTS: To date, 51 cases of pituicytoma have been reported in addition to our own cases. The natural history and clinical characteristics of this rare tumor are outlined. CONCLUSIONS: Macroscopic aspects are variable, and final diagnosis is made from the typical histological and immunohistochemical pattern. The therapy of choice is gross total surgical resection since subtotal removal can lead to local regrowth, but the time to recurrence is unpredictable and highly variable. The role of adjuvant therapy is currently undefined.