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All Posts in Category: Diseases

Developmental abnormalities of the central neural tube

 

 

If spinal convergence is not complete, a part of the meninges (meningocele) and/or the spinal cord (meningomyelitis) may be left unprotected and uncovered in the back. This condition is called spina bifida and has different grades, depending on the type of tissue missing. It is a condition that is usually surgically corrected immediately after birth and must have been detected by ultrasound during pregnancy. It is often associated with loss of cerebrospinal fluid, or hydrocephalus. If not treated in a timely manner, there can be serious neurological consequences or infection.
In other cases, even with complete central neural tube closure, there may be other problems, such as congenital dermoid cysts and fistulas, which predispose for types of meningitis and other infections, and usually require surgical repair. There are also disorders of the spinal cord itself (diastematomyelia) or the spinal cord sheaths (neurenteric cysts) or the ligament that holds the spinal cord at the base of the spine (tethered spinal cord). The earlier these conditions are diagnosed, the more effective the treatment (usually surgical intervention).

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Infantile Hydrocephalus

This is probably the most common pediatric neurosurgical condition. The causes may vary. Hydrocephalus may generally be due to obstruction in the the circulation, insufficient reabsorption or overproduction of cerebrospinal fluid. It is often necessary to divert the cerebrospinal fluid circulation by placing a valve. The fluid can then drain into the abdomen, or more rarely into a large vein, and from there to the heart or to the chest, between the lungs and the thoracic wall. Early diagnosis of this condition prevents possible serious neurological problems that can be life threatening for the child. Sometimes the diagnosis is made before birth, with ultrasound during pregnancy. Although it is usually a benign condition, sometimes the valve can become blocked or infected, and the increased intracranial pressure could be life-threatening. Early diagnosis and decompression can be life saving.

Symptoms

Symptoms of increased intracranial pressure are vomiting, headache, dizziness, drowsiness, and the fundoscopy may reveal papilledema, an important sign of increased intracranial pressure. The valve may need repair on numerous occasions during the child’s lifespan. Modern imaging methods such as cine-MRI may give us new information about the pathophysiology of hydrocephalus.

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Craniosynostoses

 

 

The surface of the bones of the skull increases during childhood. We can imagine them as islands that grow and eventually join together, forming sutures. In babies, one can feel the gaps between the bones (called fontanelles). Sometimes, however, sutures may form prematurely or asymmetrically between the left and right sides.

The exact cause of this malformation is still unknown.
The incidence of craniosynostoses is estimated at 2 per 2,000 newborns for which 10-20% are hereditary. Craniosynostosis may occur in the context of a syndrome, i.e. in combination with other malformations, e.g. syndactyly (Apert syndrome), maxillary hypoplasia (Crouzon syndrome), etc.

Skull dysraphia may affect one or more sutures. If there is premature fusion of the sagittal suture, the result is an increase in the anterior-posterior diameter and then scaphocephaly (dolichocephaly or macrocephaly) may occur.
If bilateral fusion of the coronal suture occurs, the result is a smaller but flattened head, i.e. brachycephaly occurring within the context of other malformations, such as Crouzon syndrome.
Premature unilateral fusion of the coronal suture results in elevation of the eye orbit and intense asymmetry, i.e. plagiocephaly, which is more common in girls and second in frequency among all cranial stenoses, following scaphocephaly.
Finally, trigonocephaly is the rarest. This is observed after premature fusion of the metopic suture. The forehead becomes triangular, exhibiting a short distance between the eyes.
Premature fusion of all sutures results in a pointed head, called oxycephaly, or microcephaly. In this case, there is an increase in intracranial pressure, and a risk for neurological damage.

Treatment may be conservative or surgical. In the second case, cranioplasty is performed. In the first case, a special helmet can be used, custom-made for every child.
In scaphocephaly, surgery is recommended mainly for aesthetic reasons. When the malformations are complex or involve multiple sutures, surgical treatment is necessary in order to avoid neurological damage.
Surgery should be carried out before the infant reaches 6 months of age. In simple synostosis, it is usually sufficient to remove the suture with or without covering the ends with silicone, to delay fusion.
More complex malformations may require complex craniectomy, reconstruction of the eye orbit, and multiple surgeries performed by craniofacial surgeons and specialized neurosurgeons.

 

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Chiari malformation and Syringomyelia

 

 

Chiary malformation includes a group of diseases sharing an anatomical disorder at the position of the lower part of the brain and the cerebellum. Thus, part of the cerebellum may be outside the skull, migrating downwards to the craniocervical junction, thus compressing the spinal cord. There are 4 grades for the ectopic position of the anatomical elements. This disorder is usually accompanied by a disturbance of the circulation of the cerebrospinal fluid, both around and inside the spinal cord or the brain. MRI imaging can help us diagnose this disorder. Clinically, in Chiari malformation Type I, which is the most common, the symptoms may include severe headache, symptoms at the upper and lower extremities, disturbance of the sensation of hot and cold, gait instability, urination and bowel movement disorders, etc. Sometimes this syndrome is accompanied by other disorders, such as spine scoliosis.
Syringomyelia involves an excessive amount of cerebrospinal fluid inside the center of the spinal cord. The symptoms are similar to those seen in Chiari malformation, since these two conditions may coexist (cause-effect relationship). Syringomyelia may also develop following spinal cord injury (injuries, ischemia).
Chiari malformation is treated with surgery, with an incision in the back of the neck. An incision is made in the dura mater, to create an additional anatomical space for the circulation and drainage of the cerebrospinal fluid. Of course, the operation is performed under general anesthesia, it lasts about 2 hours and requires a hospital stay for 4-5 days. With the gradual balancing of the cerebrospinal fluid circulation, the symptoms gradually subside; syringomyelia may also subside. In the case of post-traumatic syringomyelia, unfortunately the course of the disease may not be as favourable, and it is more difficult to treat. In many cases, even the drainage of cerebrospinal fluid may not have the desired clinical results.

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Arachnoid cysts

 

 

These are congenital disorders formed by the pathological development of one of the membranes of the brain, namely the arachnoid mater. Normally this meninge is a thin membrane that covers the brain, leaving only minimal space, where the cerebrospinal fluid is located and circulating. If, due anatomical damage, this membrane allows for the accumulation of this fluid, this causes cysts to form, trapping fluid and exercising pressure on the brain. The diagnosis is possible via computed tomography and magnetic resonance imaging. There may be no clinical symptom, or there may be seizures, severe headaches, increased intracranial pressure, and other symptoms. These are benign conditions that may require a simple surgery, in which the fluid is drained towards its normal circulatory pathway, so that the brain can be decompressed.

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Central Nervous System Infections

These infections may affect the brain, the meninges, the cerebrospinal fluid, the surrounding tissues, and the bones of the skull and spine, as well as the spaces between these structures. They may be caused by a germ, a virus, or a fungus. Infections may sometimes be the result of open injury, extension of the infection from neighboring tissues (otitis, inflammation of the teeth, etc.), or even postoperative complications. With early diagnosis and appropriate treatment it is possible to save a patient’s life and prevent the onset of a severe neurological disability.

Viral infections

There are viruses, such as herpes simplex virus type I (HSV I), that are present in most people’s body anyway. Sometimes, either the virus is activated or the body’s defenses are weakened, which results in the onset of infection. The virus can infect peripheral nerves causing severe pain along the nerve, and a skin rash. If the brain is affected, then encephalitis is caused, which must be diagnosed and treated in time. Diagnosis of these infections requires CT/MRI and lumbar puncture. Clinical symptoms are behavioral disturbances, seizures, severe headache, drowsiness, weakness in one side of the body, and others. Treatment is based on antiviral drugs. Other viruses that cause similar infections are HSV 2, HIV (AIDS virus), and others.

Microbial infections

These include meningeal infections (meningitis), which are the most common central nervous system infections affecting infants, children, and adults. The germs that are usually responsible are streptococcus, pneumococcus (meningococcus), Haemophilus influenzae, and more rarely listeria, Escherichia Coli, and others. Prompt diagnosis and immediate start of antibiotic treatment save the patient’s life.

Parasitic infections

In the past, echinococcosis or cysticercosis were common due to poor personal and food hygiene, especially vegetables. Nowadays, they are still here, albeit on a smaller scale. Surgery may be needed to remove any cysts in the brain. Diagnosis is made by CT/MRI, and special serology tests. There is also special medication treatment.

Fungal infections

These can be caused by a pathogenic fungal infection, or occasional fungal infections that do not normally cause infections, unless the patient’s immune system is suppressed, as is the case in many patients with other serious chronic diseases. Fungi, such as candida albicans, aspergillus, and cryptococcus may be responsible for these infections. The diagnosis is made by CT/MRI, and examination of the cerebrospinal fluid; treatment is based on antifungal drugs.

Epidural Cerebral Abscess

This is a collection of pus between the dura mater and the bones of the skull. Bone infection may coexist. The infection could be extending from the sinuses (as in sinusitis). The diagnosis is made by CT. Treatment is based on antibiotics for 6 weeks and may require surgery and cleansing of the sinuses. Prognosis is very good, if there is an early treatment.

Subdural Cerebral Empyema

Inflammation is found between the meninges and the brain, therefore the brain is much more vulnerable to the infectious agent. The cause is usually a neglected inflammation of the sinuses, a skull fracture, postoperative infection, meningitis, and others. The ensuing cerebral edema (swelling of the brain) is a directly life-threatening condition for the patient. Surgery must be performed at a timely manner, to clear the pus and decompress the brain, otherwise it is a condition that leads to the death of the patient.

Intracerebral Abscess

This is a collection of pus in the brain. It can be caused by sinus infections, craniocerebral injury, or the transmission of germs through blood. The diagnosis is made by CT, following administration of contrast fluid. Sometimes, these lesions may look like a brain tumor and the first invasive approach is a biopsy of the lesion. If their size and position exert pressure on the brain, surgical removal of the entire lesion is required, and the patient always receives strong antibiotic treatment for 6 weeks. Repeated CT scans are required to confirm the effectiveness of the treatment.

Epidural empyema of the spinal cord

In immunosuppressed patients, or patients who have had surgery on the spine or epidural anesthesia or lumbar puncture, this infection may develop. Pressure on the spinal cord causes typical neurological symptoms and immediate surgical decompression must be performed to preserve spinal cord function. The diagnosis is possible via magnetic resonance imaging. Long-term administration of antibiotics to which the germ that caused the infection is sensitive (following a culture-antibiogram) is required. Tuberculosis of the spine may also cause a similar infection.

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