Barbara Jasiewicz1, Magdalena Stachura2, Tomasz Potaczek1, Slawomir Duda1, Piotr Michno3, Stanislaw Kwiatkowski2. 1. Faculty of Medicine, Department of Orthopaedics and Rehabilitation, Jagiellonian University, Zakopane, Poland. 2. Faculty of Medicine, Department of Pediatric Neurosurgery, Jagiellonian University, Krakow, Poland. 3. Department of Orthopaedics, Division of Pediatric Orthopaedics, Umeå University Hospital, Umeå, Sweden.
Abstract
Context: Spine duplication is a rare condition, with various extents and severe additional anomalies. The goal of this study was to describe a unique case of a boy with split notochord syndrome who was followed up from birth until maturity. Findings: Physical examination at birth showed defects of the abdominal wall and cloacal exstrophy with visible urether outlets. A transposed anus was present in the perineal region. Split bony elements of the spine with nonpalpable sacral bone were noted. A soft, skin-covered lump, with the consistency of a lipoma, was present in the sacral area. There was asymmetry of the lower limbs: the left was hypoplastic, with a deformed foot and hip. Computed tomography revealed a normal shape of the Th12 and L1 vertebrae, whereas the L2 was split. Downward from L3, there were two vertebrae at each level, with two spinal canals. The spinal cord divided into two "semicords" at the level of L1. Neurologic status and the shape of the spine remained unchanged during puberty. The last follow-up was performed at the age of 18 years. He managed to walk independently in prosthesis with visible limping. Conclusion: Spine deformities are always suspected in neonates with lipoma in the sacral region, which may sometimes be serious. Walking ability and quality of life depend on neurologic deficits; even with long duplication and double sacrum, walking can be a feasible option.
Context: Spine duplication is a rare condition, with various extents and severe additional anomalies. The goal of this study was to describe a unique case of a boy with split notochord syndrome who was followed up from birth until maturity. Findings: Physical examination at birth showed defects of the abdominal wall and cloacal exstrophy with visible urether outlets. A transposed anus was present in the perineal region. Split bony elements of the spine with nonpalpable sacral bone were noted. A soft, skin-covered lump, with the consistency of a lipoma, was present in the sacral area. There was asymmetry of the lower limbs: the left was hypoplastic, with a deformed foot and hip. Computed tomography revealed a normal shape of the Th12 and L1 vertebrae, whereas the L2 was split. Downward from L3, there were two vertebrae at each level, with two spinal canals. The spinal cord divided into two "semicords" at the level of L1. Neurologic status and the shape of the spine remained unchanged during puberty. The last follow-up was performed at the age of 18 years. He managed to walk independently in prosthesis with visible limping. Conclusion: Spine deformities are always suspected in neonates with lipoma in the sacral region, which may sometimes be serious. Walking ability and quality of life depend on neurologic deficits; even with long duplication and double sacrum, walking can be a feasible option.
Authors: Emily N Morgan; Sean M Caskey; William E Bronson; Glen O Baird; Bryan J Tompkins; Paul M Caskey Journal: J Pediatr Orthop B Date: 2016-05 Impact factor: 1.041