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Principles of Intraoperative Management of Early-Onset Scoliosis
Published in Alaaeldin (Alaa) Azmi Ahmad, Aakash Agarwal, Early-Onset Scoliosis, 2021
Ashok N. Johari, Rashid Anjum, Vrushali Ponde
The patients should be evaluated for any associated anomalies to avoid any last-minute surprises, especially in cases of EOS that include the congenital types as well. Associated cardiac, renal, and other anomalies in congenital scoliosis can be seen in up to a third of cases. Neuraxial abnormalities, including occult spinal dysraphism, is seen in as many as 50% of the cases of congenital scoliosis, whereas its incidence in infantile idiopathic scoliosis is less than 20% [9–10].
Developmental Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James H. Tonsgard, Nikolas Mata-Machado
This is the most common form of closed spinal dysraphism. It is characterized by failure of fusion of the vertebral body dorsal to the spinal cord. Radiologic findings in addition to the defect in the posterior vertebral arch may include widening of the spinal canal, fusion of vertebral bodies, and fused or malformed lamina. Abnormalities of overlying skin are common including nevi, dermal sinus, dimple, hemangioma, lipoma, or hair. It commonly involves the lamina of L5 and S1. The incidence is about 5% of the population. When there is any associated neurologic deficit, it is called an occult spinal dysraphism.
The cases
Published in Chris Schelvan, Annabel Copeman, Jacky Davis, Annmarie Jeanes, Jane Young, Paediatric Radiology for MRCPCH and FRCR, 2020
Chris Schelvan, Annabel Copeman, Jacky Davis, Annmarie Jeanes, Jane Young
Spinal dysraphism is a spectrum of disorders ranging from mildly deficient lumbar spinous processes (spina bifida occulta) to an open defect with exposed abnormal spinal cord and CSF leakage (spina bifida cystica).
Effect of untethering on occult tethered cord syndrome: a systematic review
Published in British Journal of Neurosurgery, 2022
Hamid Rezaee, Ehsan Keykhosravi
During the searching process, 798 articles were selected for the primary assessment; however, 692 of them were extracted due to irrelevancy. Out of the remaining articles, 32 papers were duplicates that were removed, and the rest of the studies were assessed for eligibility. Out of the remaining 74 articles, a total of 13 studies were performed on patients with occult spinal dysraphism and other similar diseases that were excluded from the review. Moreover, a number of other articles were removed due to lack of access to the full-text version (n = 6), insufficient data (n = 2), experimental design (n = 8), animal studies (n = 0), editorial letters, short communications, or brief reports (n = 14), books (n = 1), narrative and review articles (n = 9), and case reports or case series (n = 11). Eventually, 10 reports were found about the clinical outcomes of SFT for the management of OTCS from 1 October to 6 November 2020, which were included in this review.
Congenital Spinal Lipomatous Malformations. Part 1. Spinal Lipomas, Lipomyeloceles, and Lipomyelomeningoceles
Published in Fetal and Pediatric Pathology, 2020
Rare cervicothoracic intramedullary lipomas, which are congenital malformations of mature adipose tissue rostral to the lumbar spinal cord, can pose both severe neurological deficits and surgical challenges. Symptoms usually appear in the third decade, although these lipomas have been seen in children [137]. An embryologic origin has yet to be well characterized but there is no heterogeneous histopathologic component [138–143]. These subpial spinal cord lipomas appear to be part of midline encephalocraniocutaneous lipomatosis that includes corpus callosum lipomas [144, 145]. Most but not all cases have spinal dysraphism [146, 147]. Subcutaneous cervicothoracic lipomas that may accompany cervicothoracic intramedullary lipomas are more likely to be developmental than neoplastic. One symptomatic cervicothoracic intramedullary lipoma was identified concomitantly with a symptomatic conus medullaris lipoma [148]. Older surgical-radiological classifications do not always distinguish between cervicothoracic intramedullary lipomas and congenital lumbosacrococcygeal lipomatous malformations [4, 21].
Unilateral Anophthalmia and Congenital Frontal Cranioschisis Associated with Extradural Neuroglial Heterotopia: new Insights into a Possible New Malformative Spectrum
Published in Fetal and Pediatric Pathology, 2023
Javier Arredondo Montero, Mónica Bronte Anaut, Carlos Bardají Pascual
This 4-year-old girl of African ethnicity with congenital right anophthalmia presented with a progressively growing deforming frontonasal tumor causing a significant psychological impact. The examination revealed the presence of a soft, rubbery, non-pulsatile, frontonasal mass, with smaller frontal nodules in apparent dependence of the main lesion (Figure 1: Left). No neurological alterations were observed on systematic examination. The patient did not present findings suggestive of spinal dysraphism. The rest of the examination by organs and apparatus showed no alterations apart from the right anophtalmia.