Spina bifida and related deformities

Introduction to spina bifida and related malformations Spina bifida and related malformations belong to a neural tube insufficiency malformation. Spina bifida can be a wide range of neural tube infusion, called complete spina bifida or total spine, or partial spina bifida. During the embryonic phase, the neurogenic ganglion formed and developed into a nerve groove to the caudal end. On the 21st day, the neural crest on both sides of the nerve sulcus merged into the dorsal side and merged into a neural tube. The fusion begins at the head and tail from the thoracic segment, and the tail is closed at the second week, and the head end is closed about 2 days earlier than the tail. After the neural tube is closed, it gradually detaches from the epidermis and moves to the deep part of the body wall. The head of the neural tube develops into a cerebral vesicle, and the rest develops into a spinal cord. At the 11th week of the embryo, the bony spinal canal was completely healed. basic knowledge Sickness ratio: 0.0001% Susceptible people: no special people Mode of infection: non-infectious Complications: meningitis hydrocephalus

Spina bifida and related malformations

Causes:

The exact cause is still unclear, and the widely accepted view is that the embryonic neural tube is incompletely closed or the neural tube is closed again for some reason.

Pathogenesis

When the neural tube is healed by internal and external adverse factors, it may cause craniotomy, spina bifida and related malformations. Spina bifida can be a wide range of neural tube infusion, which is complete spina bifida or total spine. Partial spina bifida.

There are several types of partial spina bifida:

1. Recessive spina bifida can be associated with gliosis in the spinal cord or even enlargement of the central canal, resulting in spinal cord injury symptoms (spinal cord syndrome).

2. The dural bulge out of the dura mater to the subcutaneous through the laminar defect, forming a cyst-like mass in the midline, which is filled with cerebrospinal fluid; the nerve root can enter the bulge and adhere to it.

3. The spinal cord bulge has a large spina bifida defect. In addition to the cerebrospinal fluid entering the bulging sac, the spinal cord also enters the bulging sac.

4. Fat meningocele and fatty spinal cord bulging in the bulging sac associated with lipoma.

5. The spinal cord cyst bulge is accompanied by a localized enlargement of the central canal of the spinal cord (spontaneous hydrocephalus).

6. The spinal cord valgus central tube splits directly into the body surface, often with cerebrospinal fluid overflow.

7. The anterior sulcus of the spine bulges forward into the body cavity.

Spina bifida and related malformation prevention

Prevention: Pay attention to the perinatal care of pregnant women and avoid adverse factors such as infection, metabolic diseases and poisoning during embryos.

Spina bifida and related malformations Complications meningitis hydrocephalus

The bulging sac easily breaks the infection and causes meningitis. Epithelial sinus can also cause recurrent episodes of bacterial meningitis. Spina bifida is also often associated with other congenital malformations, such as hydrocephalus, cranial fissures, split lips, and fissures.

Spinal fissure and related malformations Symptoms Urinary incontinence Enuresis Muscle atrophy Spinal bone Metamorphosis Spinal horn deformity Nutritional disorder Cerebellar ataxia Spinal physiologic flexion disappears Spinal glial hyperplasia

Complete spina bifida is mostly stillbirth, clinical significance is not significant, the main manifestations of partial spina bifida are as follows:

1. Recessive spina bifida is the most common, accounting for 1/1000 of the surviving newborns. It is more common in the lumbosacral region. One or more lamina is incompletely closed. The contents of the spinal canal are not bulging, and there is no external manifestation. Occasionally found in the film, occasionally the lumbosacral skin is pigmented, the skin is umbilical lacuna, hair overgrowth or skin fat pad, sometimes the above structure is attached to the dura by a fiber rope through the laminar fissure, The nerve roots, even the spinal cord, fix the spinal cord on the spinal canal, limiting the upward movement of the spinal cord during development, and may be associated with gliosis in the spinal cord or even enlargement of the central canal, resulting in spinal cord syndrome, recessive spina bifida itself. There are very few symptoms, and some low back pain and enuresis may be associated with this. Skin sinus and dermoid cysts may cause recurrent episodes of meningitis.

2. The meningocele is more common in the waist and lumbosacral region; it can also occur in other parts. The dura mater bulges outward through the lamina defect and reaches the subcutaneous area. Together with the skin, it forms a cyst-like mass in the midline, which is filled with cerebrospinal fluid. Or only cover a thin layer of epidermis, the position of the spinal cord and nerve roots can be normal or adhere to the spinal canal, and the nerve root can enter and adhere to the bulging sac.

3. The spinal cord bulging is rarer than the meningocele, and the spina bifida is larger. In addition to the cerebrospinal fluid entering the bulging sac, the spinal cord also enters the bulging sac. The bulging sac has a broad base and the capsular surface covers the skin. The color is dark, the light is not very light, and sometimes the skin is transparent. The blood vessels, nerve roots and spinal cord can be seen through the skin.

4. Fat meningocele and fatty spinal cord bulging in the bulging sac associated with lipoma.

5. The meningeal spinal cord cyst bulges out of the meninges and the spinal cord bulge with a localized central canal enlargement of the spinal cord (spontaneous hydrocephalus), the latter shaped like an intramedullary cyst, which can protrude into the bulging sac.

6. Spinal valgus is the most severe form of partial spina bifida. The central canal of the spinal cord splits directly into the body surface, forming an exposed granulation surface, often with cerebrospinal fluid overflow.

7. Spinal anterior fissure is a rare type of spina bifida that bulges forward into the body cavity.

Most of the recessive spina bifida does not produce symptoms for life. It is accidentally found on X-rays for other reasons. A few patients have enuresis or mild urinary incontinence. It may also be one of the causes of low back pain. Individual recessive spina bifida All of the bulging patients may have varying degrees of neurological damage as follows.

The lumbosacral deformity produces nerve damage of the joint segment, and the calf and foot muscles exhibit symmetric relaxation and atrophy; the muscle function above the knee is relatively rare; the tendon reflex mostly disappears, the foot, the perineum and the hind limb The skin may have sensation loss, pain and temperature sensation disorders are often obvious, patients have varying degrees of urinary incontinence, lower limbs and various nutritional and autonomic dysfunction, such as skin temperature drop, bruising, edema, ulcers, chronic Osteomyelitis and foot deformity, some children have no obvious neurological symptoms at birth, and the nerve damage is gradually worsened during the growth period due to the impact of the bulging sac on the spinal cord and nerve roots or the traction syndrome. .

The deformity of the neck segment produces slack spasm of the upper limbs, muscle atrophy, neurotrophic disorders and paralytic spasms of the lower extremities. These symptoms may have existed at birth or gradually occur during the growth phase, and cerebellar cohorts may occur in high neck deformities. Disorders and convulsions of quadriplegia.

Spinal fissure and related deformity examination

Blood, urine, routine examination, cerebrospinal fluid examination, blood immunology.

1. Radiological examination of plain radiographs, CT and MRI.

2. Prenatal diagnosis of fetal amniotic fluid chromosomes, genetic testing.

Diagnosis and differentiation of spina bifida and related malformations

The diagnosis of meningocele is not difficult. X-ray films can detect bone defects. CT and MRI have replaced myelography, which provides a reliable diagnostic basis for spina bifida and meningocele.

Pay attention to the identification of other masses, the cystic mass along the medial line on the dorsal side, more or less light transmission, fullness when crying, etc., can be used as the main points of identification with other masses.

The material in this site is intended to be of general informational use and is not intended to constitute medical advice, probable diagnosis, or recommended treatments.