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.

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.

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.