Congenital dermal sinus (CDS) results from the failure of neuroectoderm separation from the cutaneous ectoderm during the process of neurulation. Although cutaneous markers are frequently associated with CDS, these patients seek medical attention only after an infectious or neurological complication. Common infections secondary to dermal sinus include meningitis and abscess in subcutaneous, extradural, or subdural region. Intramedullary spinal cord abscess (ISCA) in children is an extremely rare infection which may clinically resemble spinal cord neoplasm. We report a rare case of lumbar ISCA and thoracic syrinx secondary to CDS.
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CASE REPORTA 2-year-old male child presented with history of intermittent low-grade fever since 2 months. He developed bowel and bladder incontinence with inability to move both the lower limbs since 15 days. His perinatal and developmental history was uneventful, with no history of any significant illness.On examination, a skin dimpling was noted in the lumbosacral region. Neurological examination revealed flaccid paraplegia with complete sensory loss below D12 for touch, pain, temperature, and pressure. Deep tendon reflexes were absent in both the lower limbs; cremasteric reflexes were absent with bilateral extensor plantar response and relaxed anal tone.Laboratory investigations revealed elevated white blood cell count (23,100/mm 3 ), elevated erythrocyte
10.5005/jp-journals-10039-1135sedimentation rate (76 mm/hr), and blood culture report showed methicillin-resistant Staphylococcus aureus. Magnetic resonance imaging of lumbosacral spine showed absent posterior elements at S1 and S2 level with tethered cord (Fig. 1). Enlarged and edematous cord with intramedullary abscess as noted from L1 to S2 level with a sinus tract extending to the skin from thecal sac at S2 level. Syrinx was noted from D3 to D12 level (Fig. 2).
Sickle cell disease (SCD) is a hereditary blood disorder characterised by an abnormality in the oxygen carrying capacity of haemoglobin molecule
in red blood cells. WHO has recognised 14 communities in South Gujarat amongst the tribal groups with high prevalence of SCD. Traumatic brain
injuries (TBI) can lead to increased secondary damage due to presence of hypoxia and hypotension; which are worsened in the presence of SCD
leading to grave outcomes. In one of this rare kind of study; 11 patients of TBI with SCD were analysed. 5(45.45%) patients had history of no
trauma or minor trauma with a mortality of 3(27.27%) patients out of 11. All (100%) patients had hypoxia and hypotension at the time of
presentation. Aresearch on bigger scale is needed to identify specic interventions that may improve outcomes in this population.
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