Can mild spina bifida worsen?

abstract

Spina bifida is a congenital spinal dorsal canal insufficiency. Complete spina bifida is stillborn. Partial spina bifida can be divided into recessive and dominant, and the latter includes meningocele and spinal meningocele.

A cystic mass containing cerebrospinal fluid was formed, with no spinal cord tissue and a few nerve roots. Spinal meningocele contains nerve roots and underdeveloped spinal cord in the bulging meningeal sac. If the skin on the surface of the capsule is not completely closed, cerebrospinal fluid will overflow. It may be accompanied by other congenital abnormalities such as cervical rib, syringomyelia and hydrocephalus.

clinical picture

(1) The detection rate of spina bifida occulta in routine photos can be as high as 10%. Most of them are asymptomatic, and a few have local pain and discomfort. There may be pigmentation, subcutaneous lipoma, a tuft of hair or pits containing hair near the skin with thin layer missing, and the latter may have pipes leading to deep dermal sinus (tunnel). In a few patients, the terminal filament or cauda equina nerve adhered to the missing area of lamina, or abnormal fibrous tissue extended into the spinal canal. With the increase of age, the spinal cord moves up, the round vertebra or cauda equina nerve of the spinal cord is pulled or compressed, and sphincter dysfunction such as urgency and enuresis gradually occurs, and the muscle strength of the distal end of the lower limb decreases, resulting in nutritional disorders. Asymptomatic patients may have acute cauda equina syndrome after strenuous activity or accidental impact: bladder dysfunction, impotence, numbness of lower limbs and foot drop. In patients with congenital dermal sinus, bacteria can invade the spinal canal through the ostium, causing various acute or chronic intraspinal infections and purulent meningitis, which is easy to recur.

It is most common in the lumbosacral segment of the spine. Subtle spina bifida usually has only 1 to several laminae defects, and most of the spinal membranes are intact. A few patients have abnormal fibrous tissue extending into the spinal canal from under the skin and attaching to the spinal cord membrane or even the nerve tissue in the spinal canal. Some cases are complicated with abnormal development of epidermal ectoderm-congenital dermal sinus. Spinal meningocele is a cystic swelling of spinal meninges caused by local loss of lamina.

(2) Meningocele accounts for 3% ~ 4% of dominant spina bifida. Spinal cord and nerve roots are usually not involved. When crying and coughing, the meninges swell and beat. If there are a few nerve roots in the bulging meningeal sac, patients will have different degrees of limb paralysis and sphincter dysfunction. In some patients, due to the adhesion and fixation of the lower end of the spinal cord, when the length of the spinal column increases, the spinal cord cannot move up relatively, resulting in the dysfunction of the round vertebra-cauda equina nerve or the aggravation of the original symptoms.

(3) Meningocele accounts for more than 90% of dominant spina bifida. The spinal cord function of the diseased segment is seriously impaired, including limb paralysis, sensory loss, fecal incontinence and neurogenic bladder symptoms. Most children are accompanied by hydrocephalus. This is because the lower end of the spinal cord and the lower end of the spinal canal are adhered and fixed during embryonic development, which causes part of medulla oblongata and cerebellum to be pulled out of the cranial cavity downward and herniated into the spinal canal through the foramen magnum (Arnold-Chiari malformation), thus blocking the circulation of cerebrospinal fluid and causing hydrocephalus.

Diagnostic description

For teenagers who complain of low back pain or spinal nerve injury, and patients with bacterial meningitis or purulent infection in spinal canal, the possibility of occult spina bifida should be considered. If hair hyperplasia, vascular nevus, pigmentation or fat mass are found on the surface skin of lumbosacral vertebra, X-ray film of spine should be taken. Spinal meningocele or meningocele, because the swelling source pulsates when the sick child cries or coughs, will immediately attract the attention of family members after birth, further clarify the scope, degree and nature of the lesion, and carry out ct or MRI examination.

Treatment instructions

Patients with spina bifida occulta without neurological dysfunction do not need treatment. For patients with nervous dysfunction such as urgency or enuresis, the fibrous band extending into the spinal canal can be surgically removed and the stretched terminal filament cut off to reduce the compression and stretching of the cauda equina. When congenital dermal sinus is complicated with central infection, antibiotics should be given and the infected focus including the initial part should be surgically removed. If the whole body and local conditions permit, the meningocele of spinal cord should be removed as soon as possible and the abnormal nerve tissue in the capsule should be repaired. For those with hydrocephalus, cerebrospinal fluid shunt can be performed first. Some patients with hydrocephalus after meningocele resection should be treated immediately.

Personally, I suggest conservative treatment first, and then consider surgery if the effect is not satisfactory.

Y answer adoption rate: 37.5%; Reported on August 27th, 2008 +04 10: 27.

Spina bifida is a congenital malformation on the spinal axis, which is mainly caused by mesoderm development when neural tube is closed in embryonic stage. The key is the lack of spinal canal. The most common form is that there is no spinous process and lamina, and the spinal canal is open to the rear, which is more common in sacrococcygeal region, followed by cervical segment, and less in other parts. Lesions can involve one or more vertebrae, some of which have both spinal curvature and foot deformity. Spina bifida is often accompanied by abnormal development of spinal cord and spinal nerves or other deformities, and a few are accompanied by cranial fissure.

[pathological classification]

According to the degree of pathological changes, people with bulging spinal canal contents can be called dominant or cystic spina bifida, and vice versa.

(1) Cystic spina bifida

Most of them occur on the posterior midline of the spine, and a few lesions are on one side. According to the pathological relationship between swelling and nerve and spinal cord tissue, it can be divided into:

1. Spinal meningocele: The inner wall of the cyst cavity of simple meningocele is composed of dura mater and arachnoid membrane, and the cyst cavity is filled with cerebrospinal fluid, which shows the normal shape and position of the spinal cord and its nerve roots. The cyst cavity forms a thin neck through lamina defect, and sometimes the neck is closed by adhesion.

2. Spinal meningocele: There are many types. It is characterized by the deformation of part of the spinal cord itself, the spinal cord and nerve roots bulge backward at the fracture site, and adhere to the cyst wall and surrounding tissues to varying degrees, and also has the characteristics of meningocele.

3. Spinal meningocele: also known as spinal cord exposure, open or complete spina bifida, this type is the most serious and rare. It is characterized in that both spinal canal and spinal membrane are open, and sometimes the spinal cord itself is completely divided into double spinal deformity. Due to extravascular violence, the surface of the lesion is purplish red, similar to granulation tissue. Because some central spinal canal is also split with spinal cord, cerebrospinal fluid often leaks from the split or around the spinal cord in the ward. Because the spinal cord itself is deformed, the nervous system symptoms are extremely serious, mostly complete paralysis and incontinence. When the child was born, the local area was flat, and later it increased with the increase of intracranial pressure, but it did not become a cyst.

(2) spina bifida occulta

It is most common in the lumbosacral region and often involves the fifth lumbar vertebra and the first sacral vertebra. Most of the skin in the lesion area is normal, with a few manifestations of pigmentation, telangiectasia, skin depression and local hirsutism. Infants have no obvious symptoms; In the process of growing up, if it is found that urination is as normal as that of children of the same age, or that enuresis is still frequent at night at school age, it should be considered that it may be caused by the tension of the spinal cord caused by the terminal silk. Adult spina bifida occulta is asymptomatic in most cases, and it is only found by accident during X-ray plain film examination. A few cases have a history of enuresis and lumbago and leg pain. However, there are various pathological changes in spinal canal of spina bifida, such as scar, adhesion or lipoma, which make the spinal cord and nerve roots compressed or involved, accompanied by nervous system symptoms, mostly manifested in different degrees of low back pain and muscle atrophy. Horseshoe deformity and dysfunction of urination and defecation.

[Clinical manifestations]

After the birth of children with cystic spina bifida, there are cystic masses protruding on the longitudinal axis of the spine, which are round or oval, with different sizes, some with thin neck or pedicle, and some with large base without neck. Tumors often increase with age, and the surface skin is normal, or thin and easy to break, and some are thin and translucent, such as cerebrospinal fluid in the capsule, and flashlight irradiation, such as spinal cord and nerve tissue in the capsule, is opaque or can see the shadow of the tissue in the capsule. When the child is crying, the tension of the mass is high. When it is quiet, the back mass is soft and the tension is not high. The root of the mass can touch the edge of the bone defect, indicating that the cyst is connected with the spinal canal. X-ray photos show enlargement of spinal canal, spinous process and lamina defect. For example, when the child is in a quiet state, the mass tension is high and the front chimney is raised, which may be accompanied by hydrocephalus.

Spinal meningocele has different degrees of nervous system symptoms and signs. Careful examination can reveal the weakness of children's lower limbs or foot deformity. Acupuncture on children's lower limbs or feet has no or weak response. Children can find incontinence when they are older. Severe flaccid paralysis of both lower limbs.

After spinal cord exposure, there is no local mass, cerebrospinal fluid leaks out, and serious neurological dysfunction often occurs, making it impossible to survive.

Although there is no lump on the back, flaky hair areas or fine hairs or flaky vascular nevus often appear on the affected skin. In some areas, the skin color is thick, or brown, or black, or red. Sometimes hidden pores can be seen on the spinal axis, and some are actually a sinus opening, which squeezes out mucus or bean dregs-like secretions. There are many dermoid or epithelioid tumors in the spinal canal. Cracking can cause low back pain, enuresis, weakness of lower limbs or neuralgia of lower limbs, but most of them are asymptomatic.

[therapy]

Almost all cystic spina bifida require surgical treatment. If the capsule wall is extremely thin or ruptured, emergency or early surgery is needed. In other cases, it is best to operate within 1 ~ 3 months after birth to prevent the cyst wall from breaking and the lesion from getting worse. If the cyst wall is thick, in order to reduce the operative mortality, the child can also be operated after the old age (1.5 years old). The purpose of the operation is to remove the swollen cyst wall, relieve the adhesion between the spinal cord and the nerve root, reintegrate the swollen nerve tissue into the spinal canal, repair the soft tissue defect, and avoid the continuous involvement of nerve tissue to aggravate the symptoms. When the opening of the spinal cord membrane cannot be directly sutured, the dorsal fascia should be turned over for repair. The dressing should be tight, and prone or lateral position should be adopted 2 ~ 3 days after operation and suture folding to prevent urine and urine from wetting and polluting the incision.

For those with long-term dysuria or nocturnal enuresis or occult spina bifida with persistent neurological symptoms, after careful examination, corresponding surgical treatment should be given. The purpose of the operation is to remove the fibers and adipose tissue that compress the nerve roots. When the nerve root is free, careful operation or microscope operation can avoid nerve injury.

Patients with complete paralysis of both lower limbs at birth, incontinence or meningocele with obvious hydrocephalus are usually difficult to return to normal after operation. Even aggravate symptoms or have other complications. The prognosis of myelocele is very poor, and there is no ideal surgical treatment at present. More children died of infection and other complications shortly after birth.