Subdural hemorrhages (SDHs) in the pediatric population are associated with a high mortality and morbidity and may present in the context of abusive head trauma. Diagnostic investigations for such cases often include evaluation for rare genetic and metabolic disorders that can have associated SDH. Sotos syndrome is an overgrowth syndrome associated with macrocephaly and increased subarachnoid spaces and rarely with neurovascular complications. Here, we report two cases of Sotos syndrome, one with SDH during infancy who underwent repeated evaluation for suspected child abuse prior to the Sotos syndrome diagnosis and the other with enlarged extra-axial cerebrospinal fluid spaces, demonstrating a possible mechanism for SDH development in this setting. These cases suggest that some individuals with Sotos syndrome may be at elevated risk of developing SDH in infancy and that Sotos syndrome should be on the differential diagnosis during a medical genetics evaluation in cases of unexplained SDH, especially in the setting of macrocephaly.
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http://dx.doi.org/10.1055/a-2052-8750 | DOI Listing |
World J Clin Cases
January 2025
Shanghai XiRong Information Science and Technology Co., Ltd, National Science and Technology Park, School of Life Sciences and Technology, Tongji University, Shanghai 200092, China.
Sotos syndrome is characterized by overgrowth features and is caused by alterations in the gene. Attention-deficit/hyperactivity disorder (ADHD) is considered a neurodevelopment and psychiatric disorder in childhood. Genetic characteristics and clinical presentation could play an important role in the diagnosis of Sotos syndrome and ADHD.
View Article and Find Full Text PDFOphthalmic Plast Reconstr Surg
December 2024
Department of Ophthalmology, Jones Eye Institute, University of Arkansas for Medical Sciences.
Cureus
October 2024
Spine Surgery, Shiga General Hospital, Shiga, JPN.
There are few reports of syndromic scoliosis accompanied by a congenital vertebral anomaly. We report a case of Sotos syndrome with a concomitant congenital wedged vertebra whose kyphoscoliosis progressed rapidly and presented with myelopathy during the growth-spurt period. A 12-year-old male suffering from Sotos syndrome with T10-wedged vertebra presented with paraparesis and urinary dysfunction.
View Article and Find Full Text PDFMol Genet Genomic Med
November 2024
Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
Background: Sotos syndrome (SS) is a rare disorder characterized by overgrowth, distinctive facial features, and intellectual disability that is primarily caused by NSD1 pathogenic variants or 5q35 microdeletions.
Methods: We retrospectively analyzed the clinical characteristics and 339 anthropometric measurements over an average of 4.3 years of follow-up in 57 Korean children with SS.
Prenat Diagn
December 2024
Department of Obstetrics and Gynecology, Nanfang Hospital, Southern Medical University, Guangzhou, China.
Objective: This study aims to elucidate two distinct fetal ultrasound features associated with aberrant brain sulcus formation as potential prenatal markers for Sotos syndrome caused by mutations in the NSD1 gene.
Method: This retrospective study investigated three fetuses across two pregnancies, including a pair of monochorionic diamniotic twins, all diagnosed with Sotos syndrome via whole exome sequencing (WES). Comprehensive clinical and laboratory data were collected and analyzed.
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