The acro-osteolyses are a heterogeneous group of disorders characterized by bone resorption. The disorder may occur as familial, idiopathic, or secondary to vascular, inflammatory, or neurologic conditions. Acro-osteolysis is rare in association with spinal dysraphism. It is even rarer for it to be the presenting symptom in spinal dysraphism. We report here four patients in whom the diagnosis of spinal dysraphism was established while investigating for the various causes of acro-osteolysis. All four patients presented with trophic changes and acro-osteolysis. Hyperhidrosis in the affected limb was seen in three patients. One patient had leg pain, the others had no sensory or motor deficits. Magnetic resonance imaging showed spinal dysraphism in all four patients.
Diastematomyelia is a rare congenital malformation in which the spinal cord is divided completely or incompletely by osseous or fibrocartilagenous septum. The division is often asymmetrical and is thought to occur due to splitting of the notochord secondary to adhesions between ecto-and entoderm. Most cases are often associated with the abnormalities of the vertebral bodies, including fusion defects, hemi-vertebrae, hypoplasia, kyphoscoliosis, spinabifida and meningomyelocele. In this report, we describe a case of isolated diastematomyelia of mid-lumbar region detected at 17 weeks of gestation by ultrasound scan. The most striking feature being, detection of the wide lumbar spine from L2 to L4 with intact skin. MRI revealed the split cord with intervening bony spur. Ultrasound scan was performed after birth of the infant to evaluate the defect that confirmed diastematomyelia. Surgical repair of the defect was performed at around 6 months of age. A total of 16 cases of prenatally diagnosed diastematomyelia have been reported in the literature, all with a specific sonographic appearance. A 31-year-old white woman, gravida 4 para 3, was referred at 17 weeks of gestation for evaluation of the fetal spine. There had been suspicion about the fetal spine on sonogram at the dating scan. MSAFP screening at 16 weeks of gestation was normal. A detailed evaluation of the fetal anatomy was performed at 17 weeks and 5 days of gestation. There was evidence of widened lumbar spine from L2/3 to L5 with intact skin. Coronal view of lumbar spine showed the presence of a midline echogenic bony spur. This feature could be associated with neurological problems (lower limb weakness and bladder problems) of variable severity. However, at that time, the limb movements were entirely normal. At 24 weeks of gestation, a repeat ultrasound scan was done and the findings were consistent with fetal diastematomyelia. MRI at 28 weeks of gestation confirmed mid-lumbar diastematomyelia. Images of axial views showed the cord split by the bony spur. Brain appeared normal with no evidence of Chiari malformation. At that time the couple declined paediatric neurologist consultation.At 38 weeks of gestation, spontaneous vaginal delivery resulted in the birth of a 2980-g female infant. On physical examination, the newborn was noted to have a circular patch of hair growth in the lower back region with a sacral dimple. There were no signs of neurological deficits, and the baby was moving its limbs. Bladder and bowel function were normal. An ultrasound scan was performed on the infant on day 1 of life, which showed diastematomyelia with split tethered cord and a bony spur. The thoracic spine and spinal cord appeared normal.At 6 months of age surgery was undertaken. This involved the untethering of the spinal cord and the removal of the bony spur with the dural sheath around, so recreating a single tube of dura. The filum terminale was found to be divided. The baby is now 15 months old and has no residual complications, medical or neurological.
Urinary epithelial cell counts were determined in 102 patients with active plaque psoriasis. Cell counts were significantly higher in psoriatic patients than in the controls. A significant fall in the cell counts was observed after regression of psoriasis.
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