The Use of the Three-Dimensional Printed Polyether Ether Ketone Implant in Secondary Craniosynostosis Revision.

Background: Skull deformities may be seen in patients years after craniosynostosis correction. These deformities cause psychosocial distress in affected patients. In this series, the authors describe the use of patient specific polyether ether ketone (PEEK) implants for correction of skull deformities after cranial vault remodeling for craniosynostosis.

Virtual Planning for Exchange Cranioplasty in Cranial Vault Remodeling.

The use of virtual surgical planning and computer-aided design/computer-aided manufacturing has gained popularity in the surgical correction of craniosynostosis. This study expands the use of virtual surgical planning and computer-aided design/computer-aided manufacturing in cranial vault reconstruction by using these methods to reconstruct the anterior vault using a single endocortically-plated unit constructed from the posterior calvarium. This technique was designed to reduce the risk of undesirable contour deformities that can occur when multiple bone grafts are used to reconstruct the anterior vault and fronto-orbital rim.

Outcomes following endoscopic endonasal resection of sellar and supresellar lesions in pediatric patients.

Purpose: The endoscopic endonasal approach (EEA) is a credible surgical alternative for the resection of sellar and suprasellar lesions such as pituitary adenomas, craniopharyngiomas, and Rathke cleft cysts. However, its application to pediatric patients poses several unique challenges that have not yet been well evaluated. The authors evaluate the safety, efficacy, and outcomes associated with the use of the EEA for treatment of these pathologic entities in pediatric patients.

Pedicled nasoseptal flap reconstruction for craniopharyngiomas in pediatric patients.

Purpose: Though the use of the pedicled nasoseptal flap (NSF), a reconstructive technique used after endoscopic endonasal approaches (EEA) for resection of craniopharyngiomas, has been shown to reduce the occurrence of post-operative cerebrospinal fluid (CSF) leaks in adults, less is known about its use in pediatric populations, specifically in children under the age of 7. The goal of this retrospective cohort study is to determine the viability of the pedicled NSF for pediatric patients.

Methods: Retrospective review of 12 pediatric patients (ages 2-16) undergoing 13 NSF reconstructions after resection of craniopharyngiomas.

Hydrocephalus associated with childhood nonaccidental head trauma.

OBJECTIVE The incidence of posttraumatic ventriculomegaly (PTV) and shunt-dependent hydrocephalus after nonaccidental head trauma (NAHT) is unknown. In the present study, the authors assessed the timing of PTV development, the relationship between PTV and decompressive craniectomy (DC), and whether PTV necessitated placement of a permanent shunt. Also, NAHT/PTV cases were categorized into a temporal profile of delay in admission and evaluated for association with outcomes at discharge.

Delay in Arrival to Care in Perpetrator-Identified Nonaccidental Head Trauma: Observations and Outcomes.

Background: Children who sustained nonaccidental head trauma (NAHT) are at severe risk for mortality within the first 24 hours after presentation.

Objective: Extent of delay in seeking medical attention may be related to patient outcome.

Methods: A 10-year, single-institution, retrospective review of 48 cases treated at a large tertiary Children's Hospital reported to the New York State Central Registrar by the child protection team was conducted.

Choroid plexus papilloma and Pierpont syndrome.

The authors describe the case of a child who presented with hydrocephalus and phenotypic features characteristic of a multiple congenital anomalies/mental retardation syndrome. Dysmorphic facies, medial plantar lipomatosis, and developmental delay were observed in this case and are identical to documented findings of Pierpont syndrome diagnosed in 3 boys. This is the fourth case reported to date and is the first documented case of an oncological process- an intraventricular atypical choroid plexus papilloma tumor-found in association with Pierpont syndrome.

Role of Rac1-regulated signaling in medulloblastoma invasion. Laboratory investigation.

Object: Medulloblastomas are the most common malignant brain tumors in children. These tumors are highly invasive, and patients harboring these lesions are frequently diagnosed with distant spread. In this study, the authors investigated the role of Rac1, a member of the Rho family of small guanosine triphosphatases, in medulloblastoma invasion.

Seizure propagation in a patient with musicogenic epilepsy.

Musicogenic epilepsy (ME) is a condition in which seizures are triggered by music. We describe a novel investigation of the mechanisms of ME using noninvasive (ictal 2-flurodeoxyglucose (2-FDG) positron emission tomography (PET)) and invasive (subdural arrays and depth electrodes) methodology in one patient with ME. Ictal PET defined onsets in the right mesial temporal lobe.

Resting tremor secondary to a pineal cyst: case report and review of the literature.

Background: Patients with pineal lesions typically present with the classical signs of increased intracranial pressure (headache, nausea, vomiting) and/or Parinaud's syndrome. Rare symptomatology of secondary parkinsonism attributed to pineal lesions has been previously reported in the literature. We describe an unusual case of a pineal cyst in a patient with the presenting sign of a resting tremor.

Case report: spontaneous intracranial hypotension in association with the presence of a false localizing C1-C2 cerebrospinal fluid leak.

Background: Spontaneous intracranial hypotension is an increasingly recognized cause of new-onset, daily, persistent headaches. Although these headaches are similar to post-lumbar puncture headaches, characteristic differences include intracranial pachymeningeal enhancement, subdural fluid collections, and downward displacement of the brain. The identification of upper cervical epidural fluid collections as a false localizing sign in patients with SIH has provided significant insight into the selection of management options.

Trigonocephaly: surgical considerations and long term evaluation.

Trigonocephaly accounts for approximately 10% of all craniosynostosis. Severe trigonocephaly results in a triangular-shaped forehead, superior-lateral orbital depression, hypotelorism, and compensatory occipital-parietal calvarial changes. Radiographic findings include ovoid orbits with parallel medial borders, thickened keel-shaped frontal bone, small ethmoid sinuses, and a short anterior cranial fossa with pitched sphenoid wings.

Chiari malformation and odontoid panus causing craniovertebral stenosis in a child with Crouzon's syndrome.

Crouzon's disease is a well-known disorder affecting multiple organ systems, specifically a craniofacial disorder with highly variable penetrance and severity of deformity. Crouzon's patients typically have anomalies of the skull base leading to gross distortion of the cranium and in some cases the cervicocranium. We present a 5-year-old girl with Crouzon's disease who suffered from an acquired Chiari I malformation after insertion of a ventriculoperitoneal shunt and a coexistent ventral odontoid panus.

Alloplastic reconstruction of large cranio-orbital defects: a comparative evaluation.

Norian CRS, Bone Cement (Synthes CMF), and Mimix (Lorenz) have been used to reconstruct large cranio-orbital defects in 85 patients. Resorbable mesh (Macropore), used in combination in selected patients, obviates dura pulsations that have been postulated to cause fragmentation of alloplastic material. Norian is composed of monocalcium phosphate, monohydrate, alpha-tricalcium phosphate, and calcium carbonate.

Precocious puberty associated with a pineal cyst: is it disinhibition of the hypothalamic-pituitary axis?

Accelerated development of secondary sexual characteristics or sexual precocity is a well-known entity. Most authors recognize two groups of patients, those described as having central precocious puberty (CPP) and those with precocious pseudopuberty. CPP results from premature activation of the hypothalamic-pituitary-gonadal axis and pseudopuberty is caused by lesions that secrete gonadotropin-like substances or hormones.

Craniovertebral instability with spinal cord compression in a 17-month-old boy with Sly syndrome (mucopolysaccharidosis type VII): a surgical dilemma.

Study Design: A case study with review of surgical technique in craniovertebral stabilization for young children with mucopolysaccharidosis.

Objectives: To describe an interesting patient with a rare metabolic disorder and review surgical technique for craniovertebral instability in this rare patient population.

Summary Of Background Data: Craniovertebral instability has been reported in patients with mucopolysaccharidosis and poses a problem for spinal surgery because of the inherent metabolic disorder and age at presentation.

New method of pediatric cranioplasty for skull defect utilizing polylactic acid absorbable plates and carbonated apatite bone cement.

Cranial defect repair in the pediatric population requires a variety of special considerations. The pediatric skull has a dynamic nature that prohibits the use of rigid fixation, which is commonly applied in the adult population. A technique using a combination of polylactic acid plates and carbonated apatite bone cement has been devised by our group.

Rapid neurological deterioration associated with minor head trauma in chronic hydrocephalus.

Case Report: An 8-year-old developmentally normal boy (status: post third ventriculostomy and resection of posterior fossa low-grade glioma 4 years earlier and with known history of ventriculomegaly/arrested hydrocephalus) presented to the emergency room with vomiting and lethargy after a minor head trauma. Computed tomography scan of the head revealed no acute changes since previous studies. However, the patient's neurological status rapidly declined in the emergency room, where an emergency ventriculostomy demonstrated increased intracranial pressure.

The role of surgical placement and pump orientation in intrathecal pump system failure: a technical report.

Intrathecal pump catheter complications are the most common cause of failure in drug delivery. A previous report has documented that intra-abdominal positioning of the intrathecal pump may predispose the pump-catheter neck to premature catheter breakdown and leakage. Based on this report, we reviewed over 100 intrathecal pump cases to determine the frequency of malpositioning and its role in the pathogenesis of catheter failure.

Pediatric cranial fixation: a survey of pediatric neurosurgeons.

To date, there are no broad-based studies defining the standard of care for pediatric neurosurgeons in the area of cranial fixation. Thus, the techniques for cranial fixation remain largely surgeon dependent. Over the past few years, there have been several new cranial fixation devices approved for use in the United States.

Recurrent intrathecal baclofen pump catheter leakage: A surgical observation with recommendations.

Intrathecal baclofen pump catheter complications are the most common cause for failure in drug delivery. A 4-year-old boy presented with repeated signs of baclofen tolerance despite progressive increases in dosing. External computerized interrogation of the pump and radiograph evaluation of the pump-catheter-intrathecal sac system were normal.