Congenital deformities of the spine lead to an imbalance in the longitudinal growth of the spine. These growth abnormalities may lead to three main patterns of deformity: scoliosis (the most common), kyphosis or lordosis (the least common). Despite the recent improvements in imaging and the routine use of neuromonitoring in the surgical treatment of congenital kyphosis, this surgery may be associated with a high rate of complications such as neurologic deficit, pulmonary thromboembolic events, infection, deep vein thrombosis, implant failure, and dural injury.
View Article and Find Full Text PDFNeuromuscular Diseases (NMD) are associated with decreased bone strength due to altered muscle-bone interaction. However, the evaluation of bone quality remains a certain challenge in these patients. The purpose of this scoping review is to investigate the recent literature regarding the assessment of Bone Mineral Density (BMD) in this population.
View Article and Find Full Text PDFGiven the complexity of neurocutaneous syndromes, a multidisciplinary approach has been advocated in order to provide optimum care. Retrospective analysis of a cohort of 157 patients during a 3-year period, seen at a newly developed neurocutaneous clinic in a pediatric tertiary care hospital in Athens (Greece); and systematic chart review of the patients diagnosed with neurofibromatosis type 1 during this time period. The most frequent neurocutaneous syndromes were neurofibromatosis type 1 (NF1) in 89 patients and tuberous sclerosis complex in 17.
View Article and Find Full Text PDFUnlabelled: New technology and instrumentation techniques are continually entering the spine field, leaving the scoliosis surgeon with a wide variety of options for the treatment of adolescent idiopathic scoliosis. All-screw constructs are currently the most popular. However, they remain controversial because of possible complications, and also because they have been associated with a decrease in thoracic kyphosis, not observed with hybrid instrumentation.
View Article and Find Full Text PDFCongenital insensitivity to pain with anhidrosis is a type IV hereditary sensory and autonomic neuropathy, presenting early in life. This disorder results from defective neural crest differentiation with loss of the first-order afferent system, which is responsible for sensations of pain and temperature; a neuronal loss in the sympathetic ganglia is also present. A case of a 33-year-old patient with congenital insensitivity to pain with anhidrosis is presented.
View Article and Find Full Text PDF