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.

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.

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.

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.

Cervical nerve root synovial sarcoma in a child with chromosomal (X;18) translocation. Case report and review of the literature.

We report on an 11-year-old female with a history of cervicobrachialgia and progressive weakness of the right arm. Cervical spine MRI showed an enhancing heterogeneous intradural mass occupying the right C6-C7 foramen. She underwent a right C6-C7 foraminotomy with a complete macroscopic removal of the lesion.

NFIA haploinsufficiency is associated with a CNS malformation syndrome and urinary tract defects.

Complex central nervous system (CNS) malformations frequently coexist with other developmental abnormalities, but whether the associated defects share a common genetic basis is often unclear. We describe five individuals who share phenotypically related CNS malformations and in some cases urinary tract defects, and also haploinsufficiency for the NFIA transcription factor gene due to chromosomal translocation or deletion. Two individuals have balanced translocations that disrupt NFIA.

Implantation of an empty carbon fiber cage or a tricortical iliac crest autograft after cervical discectomy for single-level disc herniation: a prospective comparative study.

Object: The authors conducted a study to evaluate and compare prospectively the implantation of either an empty carbon fiber composite frame cage (CFCFC) or an iliac crest autograft after anterior cervical discectomy (ACD) for cervical disc herniation with monoradiculopathy.

Methods: Thirty-six consecutive patients with one-level radiculopathy due to single-level cervical disc herniation were treated by ACD, and implantation of either an empty CFCFC (24 patients) or an iliac crest autograft (12 patients). Radiological and clinical assessments were performed preoperatively, immediately postoperatively, and at 3, 6, and 12 months postoperatively.

Meningeal solitary fibrous tumour in a child.

Introduction: Meningeal solitary fibrous tumour is a relatively recent pathological entity that has rarely been described in children. With radiological techniques, it cannot be distinguished from meningiomas, and the diagnosis has to be confirmed histologically.

Case Report: We discuss the possible histogenesis of this tumour and the need for recognizing this lesion as a separate entity.

Atypical infections in tsunami survivors.

After a tsunami hit Asia in December 2004, 2 survivors had severe infections due to multidrug-resistant and atypical bacteria and rare fungi weeks afterwards. Treating these infections is challenging from a clinical and microbiologic point of view.

The Geneva and Lausanne (French-speaking Switzerland) experience: in favor of the transsphenoidal approach when feasible.

Background: The authors present their current attitude towards management of craniopharyngiomas in children. Radical surgery cannot be performed when one is not sure about its potential danger to the visual pathways and the hypothalamus.

Methods: Most of the surgeries that have been performed in our institution via an intracranial approach were incomplete and followed by radiotherapy, in the last 10 years with stereotactic conformational radiotherapy.