Diagnosis is based on the following criteria:

From Desai et al.:

The real cause is still unknown.

Theories to the etiology

• Failure of caudal notocord

• Arrest of development of the caudal cell mass

• Trauma

There is localized agenesis or dysgenesis of the lumbar, thoracolumbar, or rarely the lumbosacral spine. There is kyphosis and kyphoscoliosis and focal abnormali­ties of the spinal cord and its roots. The spinal cord is thinned or even discernible. Distally there may be a bulky, low lying segment of the cord caudal to the defect. Desai et al., reported a case of thoracic amyelia associated with SSD. Dysphasia may involve cord above and below the defect. Other associated anomalies may be present: closed spinal dysraphism including diastatomyelia, dermal sinus, lipoma, thickened fi lum terminale, and spina bifi da. More details can be found in an article by Tortori-Donati et al., other abnormalities may be encountered in the spine, kidney, bladder, heart, ribs, feet, dysraphism, and amyelia.

1. Clinical fi ndings include: gibbus, unstable spine, paraparesis or paraplegia, asso-

ciated anomalies, and deformities of the lower extremities

2. Neuro-radiography: varies according to the extent and level of the dysgenesis.

• Kyphosis or kyphoscoliosis,

• defect of the missing vertebrae,

The common procedures are decompression and arthrodesis. Other procedures per­formed as indicated, e.g., release of tight fi lum terminale, disconnecting a neuren­teric cyst and corrective surgeries on deformities of the lower limbs. Since the series of Tortori-Donati et al., is one of the largest I’ll quote the procedures they had in their series followed up to 23 years. In their series of 100 spinal surgery was done in seven children; three patients had arthrodesis of the spine.

1 had posterior arthrodesis at age 1 year 1 had 2 posterior arthrodesis at age of 1 and 2.5 years 1 had posterior arthrodesis at age of 5 years, anterior arthrodesis at age of 5.2 and

another posterior arthrodesis at age of 5.5

Bristol et al., do not allow instrumentation in infants except when the second

graft is not enough by itself.

Sickle cell anemia or sickle hemoglobin (Hb S) disease is due to the characteristic phenomena of Hb S, which under conditions of low oxygen tension is crystallizes and this leads to sickling of the erythrocytes, which become trapped in the terminal arteri­oles and capillaries resulting in more hypoxia and consequently more sickling which results in thrombosis and ischemic infarction. Tissues that have blood at low oxygen tension e.g., the pulmonary arterioles and the renal parenchyma are more liable to sick­ling although tissues that have relatively oxygenated blood, e.g., the brain or spinal cord are not exempt. The sickle cells are rigid and less fl exible and more fragile so they hemolyze. The possible complications of sickle cell anemia fall into fi ve categories:

• painful crisis especially in the bone

• infection

• vascular disease

• An opposite view that chronic stasis leads to thrombosis.

• The sickle cells occlude the vasa vasara of large vessels producing ischemia of the vessel wall, which leads to thrombosis.

More detailed studies can be seen in the works of Kaul et al., and Mohandes and

The disease has a racial predisposition usually African descent. Other manifesta­tions of sickle cell disease may be manifested: anemia, bone pain, cerebral isch­emia, fat embolism etc. Neurological manifestations are usually vague initially and may be misdiagnosed. From the case described by Wolman and Hardy, there was pain, loss of sensation, muscle weakness, and eventually paraparesis or tetraparesis.