Intracranial infantile hemangioma - rare entity and common pitfalls: A comprehensive multidisciplinary approach from Neurosurgery, Neurooncology and Neuropathology.

Objective: To review the rare entity of pediatric intracranial hemangiomas and discuss surgical pitfalls and pathophysiology in regard to cerebral edema formation.

Materials And Methods: We describe an extremely rare case of intracranial infantile hemangioma in a neonate with massive cerebral edema, surgically resected urgently because of acute herniation. We review the literature of 46 other pediatric cases of intracranial hemangioma, including congenital capillary hemangiomas.

New surgical paradigm for open neural tube defects.

Introduction: An open neural tube defect (ONTD) features an exposed, unclosed neural plate in the form of an expanded, flat, and frequently hefty neural placode. Traditional philosophy of ONTD repair aims at preserving function at any cost, which often means stuffing the entire thick and unwieldy but non-functional placode into a tight dural sac, increasing the likelihood of future tethering of the spinal cord. The same philosophy of attempting to save the whole perimetry of the placode also sometimes leads to inadvertent inclusion of parts of the squamous epithelial membrane surrounding the placode into the reconstructed product, only to form inclusion dermoid cyst causing further injury to the neural tissues.

The Pantheon Variant of the Classic Cathedral Dome Procedure for Parieto-Occipital Skull Deformities.

Objective: To describe a new technique for the reconstruction of parieto-occipital skull deformities.

Methods And Results: The technique is a variant of the previously described "cathedral dome procedure" used for frontal skull deformities. The authors apply the same principle of remodeling by making meridional slat craniotomies surrounding the depressed dome of the posterior skull deformity, followed by "green-stick fracture-reshaping" of the meridional slats to elevate and support the excised depressed dome.

Traumatic Posterior Fossa Subdural Hygroma and Secondary Occlusive Hydrocephalus.

Background: Infratentorial subdural hygromas causing secondary occlusive hydrocephalus are extremely rare in children. Only a handful of cases have been reported in the literature.

Methods: We present a case of a 6-month-old infant with an occipital fracture and slow enlargement of a posterior fossa subdural hygroma that culminated in obstructive hydrocephalus.

Use of intraoperative lateral pelvic X-ray to localize and ensure coccyx removal during sacrococcygeal teratoma resection.

En bloc removal of the coccyx during sacrococcygeal teratoma resection is necessary to decrease the risk of recurrence. However, variable anatomy often makes the border between the coccyx and sacrum difficult to identify. We describe the use of intraoperative lateral pelvic X-ray to localize this border and ensure complete coccygectomy.

Unjoined primary and secondary neural tubes: junctional neural tube defect, a new form of spinal dysraphism caused by disturbance of junctional neurulation.

Introduction: Primary and secondary neurulation are the two known processes that form the central neuraxis of vertebrates. Human phenotypes of neural tube defects (NTDs) mostly fall into two corresponding categories consistent with the two types of developmental sequence: primary NTD features an open skin defect, an exposed, unclosed neural plate (hence an open neural tube defect, or ONTD), and an unformed or poorly formed secondary neural tube, and secondary NTD with no skin abnormality (hence a closed NTD) and a malformed conus caudal to a well-developed primary neural tube.

Methods And Results: We encountered three cases of a previously unrecorded form of spinal dysraphism in which the primary and secondary neural tubes are individually formed but are physically separated far apart and functionally disconnected from each other.

Limited dorsal myeloschisis associated with dermoid elements.

Introduction: Limited dorsal myeloschisis (LDM) originates from undisjointed neural and cutaneous ectoderms during primary neurulation. Its hallmark is a fibroneural stalk causing tethering on the dorsal spinal cord rostral to the conus. Its treatment is a relatively straightforward resection of the LDM stalk from the spinal cord.

Isolated unilateral rupture of the alar ligament.

Only 6 cases of isolated unilateral rupture of the alar ligament have been previously reported. The authors report a new case and review the literature, morbid anatomy, and pathogenesis of this rare injury. The patient in their case, a 9-year-old girl, fell head first from a height of 5 feet off the ground.

Innervation of the brain, intracerebral Schwann cells and intracerebral and intraventricular schwannomas.

The cerebral vasculature and the choroid plexus are innervated by peripheral nerves. The anatomy of the vascular supply to the brain and its related perivascular nerves is reviewed. Intracerebral and intraventricular schwannomas most likely come from neoplastic transformation of Schwann cells investing the perivascular nerves and nerves within the choroid plexus.

Traumatic rupture of the neurocentral synchondrosis of the axis in a child.

The authors report the first case of unilateral traumatic rupture of the C-2 neurocentral synchondrosis. A 26-month-old child was in a vehicular collision that caused his head to be rotated sharply to the left with the neck flexed. He had severe neck pain but was neurologically normal.

Surgical treatment of complex spinal cord lipomas.

Purpose: This paper shows the long-term benefits of total/near-total resection of complex spinal cord lipomas and meticulous reconstruction of the neural placode, and specifically, its advantage over partial resection, and over non-surgical treatment for the subset of children with asymptomatic virgin lipomas.

Methods: The technique of total resection and placode reconstruction, together with technical nuances, are described in detail. We added 77 patients with complex lipomas to our original lipoma series published in 2009 and 2010, to a total of 315 patients who had had total or near-total resection and followed for a span of 20 years.

Limited dorsal myeloschisis: a not-so-rare form of primary neurulation defect.

Purpose: Limited dorsal myeloschisis (LDM) is a distinctive form of spinal dysraphism characterized by two constant features: a focal "closed" midline skin defect and a fibroneural stalk that links the skin lesion to the underlying cord. The embryogenesis is hypothesized to be incomplete disjunction between cutaneous and neural ectoderms, thus preventing complete midline skin closure and allowing persistence of a physical link (fibroneural stalk) between the disjunction site and the dorsal neural tube.

Objective: We utilize the experience gained from the management of 63 patients with LDM to illustrate these features.

Parallel angulated frontal bone slat cuts for treatment of metopic synostosis and other frontal skull deformities: the "cathedral dome procedure".

Purpose: This study aims to describe a new procedure for the treatment of metopic synostosis and other frontal skull deformities.

Method: The procedure comprises a supraorbital bandeau widened with an interpositional graft and rounded laterally to eliminate the acute angle, and parallel angulated slat cuts in the frontal bones. Greenstick fracturing of the medial bases of these slats along a parasagittal hinge line causes fanning of the slats and expansion of the frontal flap both anteriorly and laterally making the forehead contour wider and more rounded.

Terminal myelocystocele: surgical observations and theory of embryogenesis.

Background: The structural complexity of terminal myelocystocele (TMC) precludes a recognizable link to spinal cord development and therefore a plausible embryogenetic theory.

Objective: To demystify TMC using clinical, imaging, surgical, electrophysiological, and histopathological data and to propose a theory of embryogenesis.

Methods: Our series consisted of 4 newborns and 6 older children.

Retained medullary cord in humans: late arrest of secondary neurulation.

Background: Formation of the caudal spinal cord in vertebrates is by secondary neurulation, which begins with mesenchyme-epithelium transformation within a pluripotential blastema called the tail bud or caudal cell mass, from thence initiating an event sequence proceeding from the condensation of mesenchyme into a solid medullary cord, intrachordal lumen formation, to eventual partial degeneration of the cavitatory medullary cord until, in human and tailless mammals, only the conus and filum remain.

Objective: We describe a secondary neurulation malformation probably representing an undegenerated medullary cord that causes tethered cord symptoms.

Method: We present 7 patients with a robust elongated neural structure continuous from the conus and extending to the dural cul-de-sac, complete with issuing nerve roots, which, except in 2 infants, produced neurological deficits by tethering.

Limited dorsal myeloschisis: a distinctive clinicopathological entity.

Background: Limited dorsal myeloschisis (LDM) is a distinctive form of spinal dysraphism characterized by 2 constant features: a focal "closed" midline defect and a fibroneural stalk that links the skin lesion to the underlying cord. The embryogenesis is hypothesized to be incomplete disjunction between cutaneous and neural ectoderms, thus preventing complete midline skin closure and allowing persistence of a physical link (fibroneural stalk) between the disjunction site and the dorsal neural tube.

Objective: To illustrate these features in 51 LDM patients.

Long-term outcome of total and near-total resection of spinal cord lipomas and radical reconstruction of the neural placode, part II: outcome analysis and preoperative profiling.

Objective: To show the long-term benefits of total and near-total resection of complex spinal cord lipomas and reconstruction of the neural placode.

Methods: We analyzed 238 patients with dorsal, transitional, and chaotic lipomas who had total resection as described in part I for overall progression-free survival probability (PFS, Kaplan-Meier analysis) over 16 years. We also analyzed subgroup proportional recurrence hazard (Cox analysis) of 6 outcome predictors of sex, lipoma type, age, preoperative symptoms, previous surgery, and postoperative cord-sac ratio.

Long-term outcome of total and near-total resection of spinal cord lipomas and radical reconstruction of the neural placode: part I-surgical technique.

Objective: Partial resection of complex spinal cord lipomas is associated with a high rate of symptomatic recurrence caused by retethering, presumably promoted by a tight content-container relationship between the spinal cord and the dural sac, and incomplete detachment of the terminal neural placode from residual lipoma. Since 1991, we have performed more than 250 total/near-total resections of complex lipomas with radical reconstruction of the neural placodes. Sixteen years of follow-up have proven the long-term benefits of this technique.

Atlanto-occipital dislocation--part 2: The clinical use of (occipital) condyle-C1 interval, comparison with other diagnostic methods, and the manifestation, management, and outcome of atlanto-occipital dislocation in children.

Objective: The diagnosis of atlanto-occipital dislocation (AOD) remains problematic as a result of a lack of reliable radiodiagnostic criteria. In Part 1 of the AOD series, we showed that the normal occiput-C1 joint in children has an extremely narrow joint gap (condyle-C1 interval [CCI]) with great left-right symmetry. In Part 2, we used a CCI of 4 mm or greater measured on reformatted computed tomographic (CT) scans as the indicator for AOD and tested the diagnostic sensitivity and specificity of CCI against published criteria.

Atlanto-occipital dislocation: part 1--normal occipital condyle-C1 interval in 89 children.

Objective: Although atlanto-occipital dislocation has long been recognized as an extremely unstable and often lethal injury, no single radiodiagnostic criterion published to date has achieved failure-proof status. This is because most existing diagnostic tests exploit bony landmarks remote from the injured condyle-C1 (OC1) joint so that patient positioning could inadvertently line up these landmarks and conceal actual disruption of the joint. Many of the landmarks used are wide apart and/or noncoplanar; their measurements are subject to errors related to x-ray angle, target-film distance, and superimposed bony outlines.

The combined use of hydroxyapatite and bioresorbable plates to repair cranial defects in children.

Object: Hydroxyapatite cement (HAC) is used with increasing frequency by craniofacial surgeons for building facial and skull base structures and by neurosurgeons for cranioplasty. Failures of HAC in cranioplasty have been attributed to breakage due to subjacent cerebrospinal fluid (CSF) pulsations through the dura mater. The authors describe a technique that involves inserting a resorbable MacroPore perforated plate to dampen CSF pulsations and then pouring HAC over the plate to fill a cranial defect and complete skull contouring.

Clear cell meningioma: case report and review of the literature.

Clear cell meningioma (CCM) is a rare variant of meningioma. Only 17 cases have been previously reported in children. Although it has bland cytologic features, it has a higher rate of recurrence than does conventional meningioma.

Progressive cranial nerve palsy following shunt placement in an isolated fourth ventricle: case report.

Cranial nerve palsy is rarely seen after shunt placement in an isolated fourth ventricle. In the few reports of this complication, neuropathies are thought to be caused by catheter injury to the brainstem nuclei either during the initial cannulations or after shrinkage of the fourth ventricle. The authors treated a child who suffered from delayed, progressive palsies of the sixth, seventh, 10th, and 12th cranial nerves several weeks after undergoing ventriculoperitoneal shunt placement in the fourth ventricle.