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Background: Median and paramedian craniofacial clefts are associated with hypertelorism, anterior encephalocele, positional abnormalities of the maxilla, and nasal deformity. Cleft lip and palate, eyelid coloboma, and widow's peak are frequently present., Methods: The authors collected data from 30 patients (mean age, 5.8 years; range, 4 months to 18 years) operated between 1986 and 2017 with median or paramedian craniofacial clefts of differing degrees of severity. Malformations of the different anatomic units and their surgical treatment were assessed, as well as complication rates., Results: All patients presented nasal malformations and either telecanthus (n = 16) or hypertelorism (n = 14). Most patients (n = 23) had anterior encephalocele. All patients underwent nasal corrections, and most of them had medial canthopexy (n = 24). Excision of encephalocele was associated with fronto-orbital remodeling. Medialization of the orbits was performed in 11 patients, mainly by box shift (n = 9). Patients from outside Switzerland (n = 23) were operated at an older age than those in the native patient group. Because of staged reconstruction, 13 patients had more than one operation. Surgical complications included three infections and one expander exposition. One patient had bone resorption of a frontal bone flap. Nasal correction needed more than one procedure in 5 patients, and medial canthopexy had to be repeated in 7 patients. Esthetic results were satisfactory, permitting social integration., Conclusion: Median and paramedian craniofacial clefts need adapted and carefully planned corrections respecting the growth of anatomic units. The quality of the medial canthal and nasal reconstruction is to a large extent responsible for the overall result., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Positional plagiocephaly in the newborn corresponds to a posterior flattening and asymetry of the head. Its incidence has significantly increased since the "back to sleep" campain in the nineteen nineties to prevent sudden infant death syndrome. The posterior deformation usually worsens during the first six months of life when the skull is susceptible to posterior constant pressure which induces the deformation. Spontaneous outcome can be favorable. Treatment is always conservative and consists in positionnal exercise, physiotherapy and osteopathy. In some cases treatment with a helmet is recommended for a period of three months usually. Posterior positionnal plagiocephaly has no consequences on the brain's developement an is considered as an esthetic issue. In most cases good results are obtained after management with a custom fitted helmet when needed.

Introduction: Hypertrophy of the calvarium has different aetiologies, among them the rare Proteus syndrome., Case Report: We report here the case of a young girl initially treated for relapsing right then left large chronic subdural haematoma, who progressively developed craniofacial hypertrophy consistent with the diagnosis of Proteus syndrome. Calvarium hypertrophy was shaved and remodelled combining midface advancement, essentially for cosmetic purposes. During the first calvarium remodelling, important bleeding of the bone required large volume of blood replacement. Haemostasis workup revealed platelets aggregation anomalies. Bleeding issues during subsequent surgeries were controlled with tranexamic acid and desmopressin acetate., Discussion: Other manifestations of Proteus syndrome, such as a right hypertrophy of the face with hypoplasia of its middle third, a pigmented epidermal nevus and asymmetric limbs and scoliosis, appeared progressively over time. Blood and fibroblast phosphatase and tensin homolog mutation was not found., Conclusion: Literature review of operated patients with Proteus syndrome did not reveal an association with platelets anomalies. A complete haemostasis workup following this unexpected haemorrhagic complication is recommended for this rare pathology.

Purpose: This study describes the epidemiological patterns of hydrocephalus in toddlers in our setting in order to determine the proportion of those who could benefit from endoscopic third ventriculostomy (ETV)., Methods: This prospective and descriptive study included all toddlers operated on for hydrocephalus from 1 March 2008 to 31 March 2010 at the Yaounde Central Hospital., Results: Forty-six toddlers were included representing 72% of all hydrocephalus cases managed at the Neurosurgery Unit during the study period. The mean age was 6.9 ± 1.6 months. The delay before treatment varied from 5 days to 15.8 months (mean = 3.7 ± 0.5 months). The commonest clinical presentation was macrocrania (78.3%). Of the toddlers, 58.7% presented with a probable blindness (loss of ocular pursuit); dilated and non-reactive pupils were found in nine patients (19.6%). The diagnosis was based on transfontanellar echography (TFE), CT scan or combined TFE and CT scan. Identified aetiologies were aqueduct stenosis (28.7%), haemorrhage (18%), Dandy-Walker's syndrome (14.3%), meningitis (10.8%), myelomeningocele (10.8%), agenesis of Monro's foramen (3.6%), brain abscess (3.2%) and posterior fossa tumour (3.6%). No specific cause was found in 7% of cases. The treatment was ventriculoperitoneal shunting in 42 cases (91.3%) and ETV in two cases (4.3%). Infections (11.1%) and shunts' obstruction (5.4%) were the main complications., Conclusion: Cases of hydrocephalus in toddlers are frequent in our setting. Regardless of the patient's age, the most prevailing aetiologies (infections, haemorrhage, myelomeningocele) and technological conditions (neuroendoscope) are less favourable for ETV. The use of ETV in the treatment of hydrocephalus in sub-Saharan Africa is still marginal and needs to be encouraged in selected cases. The prevention of non-tumoral hydrocephalus is of critical importance.

Primary leptomeningeal melanocytosis is rarely encountered in the pediatric population. Despite being considered a nonmalignant condition, it uniformly carries a poor prognosis given the ineluctable progression of meningeal infiltration. The case of a previously healthy 10-year-old girl who was first thought to suffer from recurrent hemiplegic migraine is reported. She later developed insidious subacute hydrocephalus. Meningeal biopsy performed during shunt insertion demonstrated an unexpectedly large number of melanocytes consistent with meningeal melanocytosis. Subsequently, the child developed recurrent shunt dysfunction and showed evidence of malignant transformation. The steps to reaching the proper diagnosis are discussed, and the current literature on this rare clinical entity as well as on related central nervous system melanocytic lesions that can occur in the pediatric population is reviewed.

Inflammatory myofibroblastic tumours (IMT) are rare, challenging lesions with respect to differential diagnosis, biological behaviour and treatment. We reviewed the literature and report a unique case of a large (9 × 8 × 8 cm) IMT in the nasal region of a 6-year-old girl responsible for important facial deformation. Following surgical resection, without any craniofacial reconstruction, the dysmorphism regressed spontaneously with a good result at 2 years of follow-up. To date, this is the largest IMT reported. Surprisingly, after simple excision of the tumour the craniofacial bones and soft tissues regained spontaneously their normal anatomical position only after a few months. The bone plasticity was likely due to the young age of patient and the preservation of periosteum and muscles during the surgical excision. We conclude that a conservative approach may be considered as alternative to reconstructive surgery in particular cases in young subjects., (Copyright © 2010 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Spinal magnetic resonance angiography (MRA) is difficult to perform because of the size of the spinal cord vessels. High-field MR improves resolution and imaging speed. We examined 17 patients with spinal vascular diseases with dynamic contrast-enhanced three-dimensional MR sequences. In three patients, the artery of Adamkievicz could be seen; we could also detect all arteriovenous malformations and dural fistulas. MRA has the potential to replace diagnostic spinal angiography and the latter should be used only for therapeutic purposes.

Background: Congenital ocular motor apraxia (COMA) occasionally shares with Joubert syndrome (JS) and related disorders (JSRDs) a peculiar malformation, the 'molar tooth sign' (MTS). In JSRDs, the absence of superior cerebellar peduncles (SCP) decussation is reported., Objective: To investigate whether COMA demonstrates similar abnormal axonal pathways., Materials and Methods: Eight healthy age-matched controls, three children with clinical COMA and one child with clinical JSRD underwent examination with a 1.5-T MRI scanner. Diffusion-weighted imaging (DWI), colour-coded fractional anisotropy maps and three-dimensional diffusion tensor imaging (DTI) tractography of the cerebellorubral network were analyzed., Results: On DTI cartography, the 'red dot' originally supposed to represent the SCP decussation in the midbrain was present in controls as well in those with COMA but absent in the single case with JS. In none of the subjects including controls was 3-D FT able to depict the SCP decussation. When seeded, the red dot resulted in the ventral tegmental decussation (VTD). It was normal in controls and in patients with COMA but was absent in our single patient with JSRD. MTS was identified in alla patients with COMA and in the patient with JSRD., Conclusion: MTS can be present in both COMA and JSRD but the underlying anatomy depicted by fibre tracking is distinct. The main difference is the integrity of the VTD in COMA.

In this report, the authors describe 4 recent cases of posterior giant dural venous sinus ectasia in neonates diagnosed during pregnancy and encountered at 3 different institutions. Posterior giant venous sinus ectasia was diagnosed in 4 patients using antenatal ultrasonography and confirmed in 2 patients using prenatal MR imaging and in 3 patients using postnatal MR angiography. In 2 children angiography was performed at the age of 6 months. The pregnancy was terminated in 1 case, and the fetus underwent an autopsy. The 3 children who were born presented with various degree of cardiac insufficiency and were admitted to the intensive care unit after birth. Signs of increased intracranial pressure were present immediately after birth, including a bulging fontanel. No endovascular treatment was used in these cases. Surgery was performed in 2 cases as an attempt to alleviate increased intracranial pressure symptoms, without any real benefit. A slow venous flow in the ectasia was shown by ultrasonography in the case in which the pregnancy was terminated. Angiography or MR angiography did not show an obvious arteriovenous malformation in any of the cases, but an arteriovenous fistula secondary or contributing to the formation of the venous ectasia is one of the physiopathological hypotheses of the cause of this condition. Interestingly, spontaneous progressive thrombosis and regression of the intravascular component of the venous sinus ectasia was observed in all cases. The clinical outcome was acceptable in 1 child (who had a moderate handicap after the surgery) and good for the other 2 children (who had normal neurological development). Stratified thrombi of different ages are found in these giant venous ectasias and develop within the leaves of the dura close to the confluence of the major posterior venous sinuses. Therefore, it appears that the formation of a progressive thrombosis represents the normal evolution of these giant dural venous sinus ectasias, which explains the favorable outcome in some cases without specific surgical treatment, except for resuscitation techniques.

Plexiform neurofibromas of the orbit, sometimes extending to the temporal region and the face, are considered to be a rare but devastating and disfiguring complication of neurofibromatosis type 1. The first symptoms appear in infancy and the involvement of the orbit and the face is present in nearly all children after the age of 5. The disease is unilateral in most cases but can exceptionally involve both sides of the face. Progressive deformation of the orbital frame due to the expanding plexiform neurofibroma and buphthalmos occurs in a large proportion of cases. The associated sphenoidal dysplasia, which is thought to be, according to the most recent hypothesis, genetically determined, will inescapably increase the burden to the orbital content, cause pulsating proptosis and will endanger noble structures, finally resulting in loss of vision. Using the Jackson classification, the authors report their personal series of 22 cases (19 operated). Until now, there has been no effective medical treatment for plexiform neurofibroma and surgery remains the standard care for these patients. Controversies remain about the timing of the first operation and today most multidisciplinary teams involving plastic, maxillofacial, ophthalmologic, and neurosurgeons favor early intervention to try to minimize the secondary deformation of the orbital and facial skeleton. A number of cases of plexiform neurofibromas are illustrated within the three Jackson groups and treatment results of the rare elephantiasis neuromatosa cases are presented. Special techniques such as preoperative embolization of heavily vascularized plexiform neurofibroma are also discussed., (Copyright 2010 Elsevier Masson SAS. All rights reserved.)

The authors report the case of a 58-year-old man presenting with a 3-year history of clinical signs of progressive cervical spondylotic myelopathy (CSM). Magnetic resonance imaging showed a severe stenosis of the cervical spinal canal at C3-4 and C5-6 levels due to multiple discopathies. High signal intensities on T2-weighted MR images of the spinal cord and low signal intensities on T1-weighted images at the C2-6 levels were noted, as was contrast enhancement at the C3-4 level. The patient underwent a bilateral decompressive laminectomy at C3-6. The patient did not show any clinical improvement. Thus, further cervical MR imaging was performed and the differential diagnosis of an intramedullary tumor was considered in view of the persisting intramedullary enhancement. This diagnosis prompted a second operation involving a posterior midline myelotomy and excision of an intramedullary ependymoma at the C3-4 level. Intramedullary tumors should be considered in the differential diagnosis of CSM with an atypical pattern of MR imaging features.

Pediatric particularities and management of pediatric hemorrhagic strokes are reviewed. Etiologies of hemorrhagic strokes in children are quite different than in adults. Arterio-venous malformations are much more frequently encountered than aneurysms, cavernous malformations and other non structural causes. Modem imagery allows to diagnose the cause of the hemorrhage with a good security and the management is based on the association of neurosurgery, interventional neuroradiology and radiosurgery. These cases must be handled in specialized tertiary care hospital where these three modalities of treatment are available 24 hours/day.

Introduction: The association of a medulloblastoma and a syringomyelia has been already described in rare instances albeit without symptoms related to the syrinx., Case Report: The case of a 23-year-old man operated in infancy for a medulloblastoma and then treated solely with adjuvant chemotherapy is reported. He was also operated in infancy for a scaphocephaly. With a very long time delay, he has developed a Chiari I and a symptomatic cervico-dorsal syringomyelia. The symptoms attributed to the syrinx consisted of a unilateral prurigo over the left arm which was so severe to lead to self-mutilation., Discussion: Clinical and magnetic resonance imaging follow-up after cervico-dorsal decompression shows a significant improvement of the symptoms together with a reduction of the size of the syrinx. This case is discussed in the light of the presumed pathophysiology of the syrinx and its exceptional clinical presentation.

Failure to thrive is frequently seen in breastfed infants. The most common diagnosis is insufficiency of breast milk in an otherwise healthy child. However, several differential diagnoses need to be considered. Progressive feeding difficulties and failure to thrive can be the first manifestation of group B streptococcal ventriculitis. This rare disease does not present with acute symptoms of sepsis or meningitis but evolves insidiously with no fever. Diagnosis is therefore often delayed and made only when intracranial hypertension develops. Cerebrospinal fluid (CSF) culture confirming the group B streptococcal infection and cerebral imaging are the necessary investigations for diagnosis. To our knowledge, only 10 cases have been previously reported.

In 1992, the American academy of paediatrics has recommended that infants be placed on their backs to sleep, because prone sleeping has been correlated with sudden infant death syndrome. Following this article, medical paediatric community has documented an exponential increase in the diagnosis of posterior cranial deformities, which were considered as the consequence of unrelieved pressure onto the occiput during infant sleep. These last 15 years, management of posterior positional plagiocephaly has evolved but is still not standardized; it varies according to local specificities, and medical or parental preferences. Treatment of deformational plagiocephaly includes preventive counseling, repositioning adjustments and exercises, physiotherapy, osteopathy, treatment by dynamic cranial orthosis. On extremely rare occasions, corrective surgery is proposed. This article aims at reviewing the epidemiologic, diagnostic, and various therapeutic options of posterior positional plagiocephaly.

Phacomatoses, or neurocutaneous disorders, are a group of congenital and hereditary diseases characterized by developmental lesions of the neuroectoderm, leading to pathologies affecting the skin and the central nervous system. There is a wide range of pathologies affecting individuals at different moments of life. The genetics is variable: while neurofibromatosis 1 and 2, tuberous sclerosis and von Hippel-Lindau disease are all inherited as autosomal dominant traits, Sturge-Weber syndrome is sporadic. Other neurocutaneous disorders can be inherited as autosomal recessive traits (i.e., ataxia-telangiectasia), X-linked (i.e., incontinentia pigmenti) or explained by mosaicism (i.e., hypomelanosis of Ito, McCune-Albright syndrome). In this review, we discuss the major types of neurocutaneous disorders most frequently encountered by the neurosurgeon and followed beyond childhood. They include neurofibromatosis types 1 and 2, tuberous sclerosis, Sturge-Weber syndrome and von Hippel-Lindau disease. In each case, a review of the literature, including diagnosis, genetics and treatment will be presented. The lifespan of the disease with the implications for neurosurgeons will be emphasized. A review of cases, including both pediatric and adult patients, seen in neurosurgical practices in the Lille, France and Lausanne, Switzerland hospitals between 1961 and 2007 is presented to illustrate the pathologies seen in different age-groups. Because the genes mutated in most phacomatoses are involved in development and are activated following a timed schedule, the phenotype of these diseases evolves with age. The implication of the neurosurgeon varies depending on the patient's age and pathology. While neurosurgeons tend to see pediatric patients affected with neurofibromatosis type 1, tuberous sclerosis and Sturge-Weber syndrome, there will be a majority of adult patients with von Hippel-Lindau disease or neurofibromatosis type 2.