Hairy, abnormally pigmented skin on the lumbosacral region
Introduction
Introduction Patients with tethered cord syndrome, especially children, should be alert to a clinical manifestation of this disease: the lumbosacral skin is hairy and abnormally pigmented. Tethered cord syndrome (TCS) is a syndrome in which a spinal cord or a cone is pulled due to various congenital and acquired causes, resulting in a series of neurological dysfunctions and malformations. Because the spinal cord is pulled more often in the lumbosacral medulla, causing the cone to be abnormally low, it is also called the lower spinal cord.
Cause
Cause
The cause of hairy and abnormal pigmentation in the lumbosacral region:
1. Various congenital spinal dysplasia: such as meningocele, spinal cord fissure, spinal meningocele, etc. due to insufficiency of the end of the neural tube. Most of the cases after birth were repaired within a few days. The purpose was to repair the abnormal nerve tissue as normal as possible. It is important to prevent cerebrospinal fluid leakage, but after the spinal dural tube is rebuilt. The adhesion produced during the healing process causes tethering at the end of the spinal cord.
2. Spinal cord lipoma and intradural and extrahepatic lipoma: caused by premature separation of neuroectodermal and epidermal ectoderm, the adipocytes of mesodermal leaves enter the neuroectodermal leaves that are not yet locked. Adipose tissue can enter the center of the spinal cord, or it can be connected to the subcutaneous fat tissue through a separate vertebral arch to fix the conus of the spinal cord. Moreover, the cases after the early childhood are related to the inflammation of the fat existing in the subarachnoid space, resulting in fibrosis around the nerve roots and adhesions caused by tethering.
3. Latent sinus: It is the neuroectodermal and epidermal ectoderm not well differentiated, and the locally formed cord-like tissue from the skin through the subcutaneous, spinal, causing tethering to the spinal cone. It can also be proliferated by the tissue of the latent sinus wall to produce dermoid cysts and epidermoid cysts and teratomas, which can surround or pull the spinal nerves and cause tethering.
4. Spinal fissure: The mechanism of the longitudinal fissure of the spinal cord is thought to be caused by abnormalities other than the nerve, that is, the abnormal development of the vertebrae; it is also considered to be an abnormal occurrence of the nerve, which is followed by abnormal development of the spine. The spinal cord is separated from the left and right, with a dural tube with both split and non-dividing types. That is, type I: double dura capsule double spinal cord type, that is, the spinal cord is in the longitudinal fissure, completely separated by fibers, cartilage or osteophytes, divided into two, each with its dura mater and arachnoid, spinal cord partition Pulling, causing symptoms. Type II: the common spinal capsule double spinal cord type, the meninges in the longitudinal fissure, separated by fiber septum, 2 parts, but there is a common dura mater and arachnoid, generally no clinical symptoms.
5. Terminal silk tension: It is due to the process of immature development of the distal end of the spinal cord to degenerate to form the terminal filament, which makes the terminal filament thicker than the normal terminal filament, and the remaining part causes the tethered cord.
6. Neurogenic intestinal cyst: The so-called neurogenic intestinal cyst is a state in which the mesenteric margin of the intestine and the tissue in front of the spine form a traffic due to the patent of the notch of the spinal cord. According to the degree of patent ductus arteriosus and communication, there may be manifestations of bone defects associated with the anterior spine, called the intestinal fistula and the intestinal cyst inside and outside the spinal canal.
7. Contrasts such as adhesions after lumbosacral sulcus and sulcus: Some scholars can account for 10% to 20% of all surgical cases.
Examine
an examination
Related inspection
Adrenocorticotropic hormone (ACTH) stimulation test
Examination and diagnosis of cutaneous and abnormal pigmentation in the lumbosacral region:
The clinical manifestations of tethered cord syndrome are more complicated. Because patients with tethered cord syndrome have different symptoms, different combinations of symptoms, and different congenital malformations, their clinical manifestations are complicated, but these clinical manifestations can be attributed to different causes and incentives. Different neurological dysfunctions occur when the spinal cone is subjected to different times and degrees of traction. Common clinical signs and symptoms are:
1. Pain: It is the most common symptom. It is manifested as pain or discomfort that is difficult to describe, and can be radiated, but often without the distribution of skin segments. The painful part of a child patient is often difficult to locate or is located in the lumbosacral region and can be radiated to the lower extremities. Adults are widely distributed and can be located in the deep rectum, mid-hip, tail, perineum, lower extremities and lower back, either unilaterally or bilaterally. The nature of the pain is mostly diffuse pain, radiation pain and electric shock, and there is little pain. Pain is often exacerbated by sedentary and forward flexion of the body, rarely aggravated by coughing, sneezing, and twisting. The straight leg elevation test is positive and may be confused with the pain of disc herniation. Suffering from lumbosacral tears can cause severe discharge-like pain with short-term lower limb weakness.
2. Movement disorders: mainly due to progressive weakness and difficulty walking, can involve unilateral or bilateral, but the latter are more common. Sometimes the patient complained of unilateral involvement, but the examination revealed changes on both sides. The lower extremities can have both upper and lower motor neuron damage manifestations, namely, disuse muscle atrophy with increased muscle tone and hyperreflexia. In the early stage of children, there are no or only lower extremity dyskinesia, symptoms appear with age, and progressive aggravation, can be expressed as lower limb length and thickness asymmetry, valgus deformity, skin atrophic ulcer.
3. Sensory disorder: mainly the saddle area skin feels numb or feels diminished.
4. Bladder and rectal dysfunction: bladder and rectal dysfunction often occur simultaneously. The former includes enuresis, frequent urination, urgency, urinary incontinence and urinary retention, the latter including constipation or fecal incontinence. Children with enuresis or urinary incontinence are most common. According to the bladder function test, it can be divided into a small bladder and a low-tension bladder. The former often combined with gait, urinary frequency, urgency, stress urinary incontinence and constipation, the performance of motoneuron damage; the latter showed low-flow urinary incontinence, increased residual urine volume and fecal incontinence, etc. The performance of motor neurons damaged.
5. Abdominal skin abnormalities: 90% of children have subcutaneous masses, 50% have skin sinus, meningocele, hemangioma and hirsutism. About 1/3 of the children had subcutaneous lipomas on the lateral side and the other side had meningocele. The subcutaneous mass of the lumbosacral region can be very large, which is caused by the attention of parents due to aesthetic problems. Individual sick children can have skin lice and form a tail. The above skin changes are less than half in adults.
6. Promoting and aggravating factors: 1 Child's growth and development; 2 Adults are seen in activities that suddenly pull the spinal cord, such as upward kicking, bending forward, childbirth, exercise or traffic accident, the hip is forced to flex forward. 3 spinal canal stenosis; 4 trauma, such as back trauma or hips landing when falling.
Diagnosis of tethered cord syndrome is not difficult based on typical medical history, clinical manifestations, and adjuvant examination. Because the disease is often asymptomatic or the development of symptoms is concealed in the early stage, a small number of patients have acute onset, although it can not improve neurological dysfunction after treatment. Therefore, it is important to improve the understanding of this disease and to make early diagnosis and timely treatment. For those with the following clinical manifestations, especially children, should be alert to this disease: 1 lumbosacral skin hairy, abnormal pigmentation, hemangioma, skin fistula, cutaneous sinus or subcutaneous mass; 2 feet and legs asymmetry, weakness; 3 recessive spina bifida; 4 unexplained urinary incontinence or repeated urinary tract infection.
Diagnosis of tethered cord syndrome: 1 pain is extensive and cannot be explained by single root nerve damage; 2 adults have obvious incentives before symptoms appear; 3 bladder and rectal dysfunction, urinary tract infection often occurs; 4 sensorimotor Obstructive progressive aggravation; 5 have different congenital malformations, or have a history of lumbosacral surgery; 6MRI and / or CT spinal canal angiography found abnormal position of the spinal cone and / or end silk thickening.
Diagnosis
Differential diagnosis
Symptoms of lumbosacral skin that are hairy and abnormally pigmented:
Tethered cord syndrome needs to be differentiated from lumbar disc herniation, lumbar muscle strain, myalgia, and spinal cord tumor. Adults also need to be differentiated from spinal stenosis. CT and MRI scans can help to confirm the diagnosis.
The abnormal skin of the lumbosacral region is the clinical manifestation of the tethered cord syndrome. Individual infants may have skin fistulas and form tails.
