The term "spinal dysraphism" was coined in 1940 by Dr Lichtenstein to designate incomplete fusion or malformations of structures in the dorsal midline of the back, particularly congenital abnormalities of the vertebral column and spinal cord. Raphes develop on the face and head, brancheal arches, sternum, and spinal column. When dysraphism occurs in these sites, failure of closure of fontanelles, cleft lip and palate, brancheal cysts, and abnormalities of the ribs and spine result. A review of 200 cases of occult spinal dysraphism showed the condition to be more common in female patient and to be associated with cutaneous signs in more than 50% of instances. The age at which neurologic symptoms appeared in recorded cases is from birth to 76 years, the average being three years. A case of spinal dysraphism with a tail-like cutaneous structure is presented. The cutaneous manifestations accompanying spinal dysraphism that may lead to early recognition of this syndrome and early institution of treatment are discussed.
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http://dx.doi.org/10.1001/archderm.114.4.573 | DOI Listing |
Birth Defects Res
January 2025
School of Nursing, Ulster University Belfast, Belfast, Northern Ireland, UK.
Introduction: While improved medical and surgical care for children with pina bifida has improved their survival, some may have lower cognitive, behavioral and educational performance. The paper assesses the effect of spina bifida on cognitive, behavioral, and educational outcomes in 5-11 year olds.
Methods: A cross-sectional study design was used where data were collected from parents/guardians and teachers using Behavior Rating Inventory of Executive Function, second edition (BRIEF2), Strengths and Difficulties Questionnaire (SDQ), and Teacher Academic Attainment Scale (TAAS).
Surg Neurol Int
December 2024
Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University, Iizuka, Japan.
Background: Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex is a rare, life-threatening congenital malformation primarily treated with abdominogenital repair. The optimal indication and timing of neurosurgical interventions for the associated spinal cord lesions remains insufficiently studied. We reviewed spinal dysraphism in OEIS to evaluate the best timing for neurosurgical intervention.
View Article and Find Full Text PDFSurg Neurol Int
December 2024
Department of Medicine, Fatima Memorial College of Medicine and Dentistry (FMHCMD), Lahore, Pakistan.
Background: The presence of a human tail is a rare condition resulting from an embryonic remnant that fits the definition of a caudal appendage. It may be a vestigial (true) or a pseudotail. Both may be considered markers of underlying intraspinal abnormalities.
View Article and Find Full Text PDFJ Pediatr Urol
January 2025
Department of Women and Children's Health, School of Life Course Sciences, Kings College London, London, UK; Children's Bladder Service, Evelina London Children's Hospital, Westminster Bridge Road, London, SE1 7EH, UK.
Introduction: The Mirabegron-anticholinergic (MAC) combination has proven effective as a step-up strategy in managing paediatric neurogenic bladder following anticholinergic medication and botulinum toxin (BTX) therapy. This study assesses the long-term efficacy of MAC in children with neurogenic bladder.
Patients And Methods: A retrospective chart review was conducted from 2015 to 2023, including consecutive paediatric patients receiving Mirabegron (25/50 mg) with an anticholinergic agent (solifenacin 16, tolterodine 7, oxybutynin 7, trospium 1).
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