Management of median and paramedian craniofacial clefts.

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.

[Positional plagiocephaly in infancy: diagnosis and management].

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.

Proteus syndrome revealing itself after the treatment of a bilateral subdural haematoma.

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.

Hydrocephalus in toddlers: the place of shunts in sub-Sahara African countries.

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.

Primary leptomeningeal melanocytosis in a 10-year-old girl: a challenging diagnosis with a poor prognosis.

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.

A facial inflammatory myofibroblastic tumour in a 6-year-old girl: plastic surgery lessons from a rare case.

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.

Dynamic MR angiography (MRA) of spinal vascular diseases at 3T.

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.

MRI with fibre tracking in Cogan congenital oculomotor apraxia.

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.

Giant dural venous sinus ectasia in neonates.

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.

[Hemorrhagic strokes in children: etiology and management].

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.

[Orbitotemporal facial involvement in type 1 neurofibromatosis (NF1)].

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.

Coincidence of cervical spondylotic myelopathy and intramedullary ependymoma: a potential diagnostic pitfall.

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.

Symptomatic syringomyelia occurring as a late complication of posterior fossa medulloblastoma removal in infancy in a boy also suffering from scaphocephaly.

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.

[A 6-week-old infant with failure to thrive: insidious presentation of group B streptococcal ventriculitis].

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.

[Treatment of posterior positional plagiocephaly].

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.

[Phacomatosis and genetically determined tumors: the transition from childhood to adulthood].

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.

Cefuroxime prophylaxis is effective in noninstrumented spine surgery: a double-blind, placebo-controlled study.

Study Design: Double-blind, placebo-controlled randomized clinical trial.

Objective: To assess the efficacy of 1 preoperative 1.5 g dose of cefuroxime in preventing surgical site infection after surgery for herniated disc.

Acute haemorrhagic presentation of an intracranial meningioma.

Acute haemorrhagic presentation of a meningioma is an extremely rare event. In a review of the literature, 44 such cases were found and only eight of them presented with sudden onset of coma. The outcome in these eight cases was, with few exceptions, poor.

Dysembryoplastic neuroepithelial tumour as a cause of coma.

Dysembryoplastic neuroepithelial tumour (DNT), a non-evolutive intracranial cortical lesion, is generally associated with epileptic seizures principally among youths. A case of a DNT which presented with uncommon clinical features, characterized by severe intracranial hypertension and progressive blindness warranting emergency surgery, is documented. In addition to the classical radiological and neuropathological features characteristic of DNTs there was a large haemorrhagic cystic haematoma as a result of repeated dissections and/or ruptures of the abnormal vessels in areas, explaining some of the atypical clinical symptoms.

A 10-year experience in paediatric spontaneous cerebral hemorrhage: which children with headache need more than a clinical examination?

Introduction: When a child is seen in a clinic with a headache, stroke is certainly not the first on the list of differential diagnoses. In western countries, stroke is typically associated with adults and the elderly. Although rare, haemorrhagic strokes are not exceptional in the paediatric population, as their incidence is around 1/100 000/year.

Spontaneous spinal epidural hematomas in children: can we prevent a negative prognosis?--reflections on 2 cases.

Spontaneous spinal epidural hematomas in children are very rare and, until now, have not been described in infants. Spontaneous spinal epidural hematomas is characterized by a sudden onset of acute back pain followed by acute neurological deterioration within a few hours, but in younger children the initial symptoms are often nonspecific, leading to a delay in diagnosis and treatment. Although some cases have been reported, controversy persists as to its origin, diagnosis, and timing of treatment.

Association of multiple vertebral hemangiomas and severe paraparesis in a patient with a PTEN hamartoma tumor syndrome. Case report.

The PTEN hamartoma tumor syndrome, manifestations of which include Cowden disease and Bannayan-Riley-Ruvalcaba syndrome, is caused by various mutations of the PTEN gene located at 10q23. Its major criteria are macrocephaly and a propensity to develop breast and thyroid cancers as well as endometrial carcinoma. Minor diagnostic criteria include hamartomatous intestinal polyps, lipomas, fibrocystic disease of the breasts, and fibromas.

Clinical applications of diffusion tensor tractography of the spinal cord.

Diffusion tensor imaging (DTI) can visualize the white matter tracts in vivo. The aim of this study was to assess the clinical utility of DTI in patients with diseases of the spinal cord. Fourteen subjects underwent magnetic resonance imaging of the spine at 1.

Frontosphenoidal synostosis: a rare cause of unilateral anterior plagiocephaly.

Introduction: When a child walks in the clinic with a unilateral frontal flattening, it is usually associated in our minds with unilateral coronal synostosis. While the latter might be the most common cause of anterior plagiocephaly, it is not the only one. A patent coronal suture will force us to consider other etiologies, such as deformational plagiocephaly, or synostosis of another suture.