Figure 7.
A 13-years-old Hurler patient: 10 years post-HSCT X-ray of spine and pathology at lumbar spine. Left (X-ray): Severe humpback of L2 is seen. The patient underwent spinal surgery at 13.5 years of age. Mild invagination of the superior and inferior endplates of the lower dorsal vertebrae is observed, and the vertebrae appear flattened. Moderate posterior scalloping of the lumbar vertebrae is observed. There is a mild dextroscoliosis of the lumbar spine. Appearance of the spine is not significantly changed since 6 years of age. Deformity of spine is much milder than that seen in an untreated patient with a severe form. Right (pathology from surgical remnants): no storage vacuoles appear in chondrocytes of lumbar spine at 13.5 years of age and the size and morphology of chondrocytes are normal.