Diagnostic basis for hirsutism and abnormal pigmentation of the lumbosacral skin

  Patients with tethered cord syndrome, especially children, should be alerted to one clinical manifestation of the disease: hairy, abnormally pigmented skin in the lumbosacral region. Tethered cord syndrome (TCS) is a syndrome in which the spinal cord or conus is stretched due to various congenital and acquired causes, resulting in a series of neurological dysfunctions and deformities. TCS is also known as hypospadias because the spinal cord is most often stretched in the lumbosacral medulla, causing the cones to be abnormally low.  The clinical manifestations of spinal cord embolism syndrome are complex. The clinical manifestations of spinal cord embolism syndrome are complicated by the different timing of symptoms, the different combinations of symptoms, and the different combined congenital malformations, but they can all be attributed to the different neurological dysfunctions that occur when the spinal cord cones are stretched at different times and to different degrees under different etiologies and triggers. Common clinical symptoms and signs are: 1. Pain is the most common symptom. It manifests as indescribable pain or discomfort that can radiate, but is often not characterized by cutaneous segmental distribution. In pediatric patients, the site of pain is often difficult to locate or located in the lumbosacral region and may radiate to the lower extremities. In adults, it is widely distributed and can be located in the deep anorectum, mid buttocks, tail, perineum, lower extremities and low back, either unilaterally or bilaterally. The nature of the pain is mostly diffuse, radiating and electric shock-like pain, and rarely occult pain. The pain is often aggravated by prolonged sitting and forward flexion of the torso, and rarely by coughing, sneezing and twisting. A positive straight leg raise test may be confused with the pain of a herniated disc. A blow to the lumbosacral region may cause severe discharge-like pain with transient lower limb weakness.  2. Motor disorders Mainly progressive weakness and walking difficulties of the lower limbs, which may involve unilaterally or bilaterally, but the latter is more common. Sometimes the patient complains of unilateral involvement, but examination reveals bilateral changes. The lower extremities may show both upper and lower motor neuron damage, i.e., disuse atrophy with increased muscle tone and hyperactive tendon reflexes. In children, there is no or only lower limb movement disorder in the early stage, and the symptoms appear with age, and progressive aggravation, which can be manifested as asymmetry of lower limb length and thickness, ectropion deformity, skin atrophy ulcer, etc.  3.Sensory disorders Mainly numbness or hypoesthesia of skin sensation in the saddle area.  4.Bladder and rectal dysfunction Bladder and rectal dysfunction often appear at the same time. The former includes urine loss, urinary frequency, urinary urgency, urinary incontinence and urinary retention, while the latter includes constipation or fecal incontinence. In children, enuresis or incontinence is most common. Based on bladder function measurements, they can be divided into spastic small bladder and hypotonic large bladder. The former is often combined with spastic gait, urinary frequency, urinary urgency, stress incontinence and constipation, which are manifestations of upper motor neuron damage; the latter is manifested by low-flow incontinence, increased residual urine volume and fecal incontinence, which are manifestations of lower motor neuron damage.  5. Lumbosacral skin abnormalities 90% of children have subcutaneous masses, 50% have skin sinus tracts, spinal membrane bulge, hemangioma and hirsutism. About 1/3 of the children had subcutaneous lipomas growing laterally and spondylolisthesis on the other side. Subcutaneous masses in the lumbosacral region can be very large and attract parental attention because of aesthetic problems. Individual children may have a skin flab in the sacral area, forming a tail. The above skin changes in less than half of the adults.  6.Promoting and aggravating factors ① growth and development period of children; ② adults are seen in activities that suddenly pull the spinal cord, such as kicking upward, bending forward, childbirth, sports or traffic accidents in which the hip joint is forced to bend forward; ③ spinal stenosis; ④ trauma, such as back trauma or landing on the hip when falling.