Summer 2014 Internship in Pediatric Neurosurgery

Subtitle

Common Pediatric Neurosurgical Disorders

Hyrocephalus is the buildup of too much cerebrospinal fluid in the brain cavities (ventricles). Normally, this fluid is in charge of cushioning the brain tissues. Nevertheless, when you have an excess of this fluid, though, it puts harmful pressure on your brain. As en effect, Hydrocephalus can permanently damage the brain, causing problems with physical and mental development.

Spina bifida is a neural tube defect , which literally means “cleft spine,”. This disorder is characterized by the incomplete development of the brain, spinal cord, and/or meninges ( which  is the protective covering around the brain and spinal cord). Spina Bifida takes place when the spinal column of the fetus doesn't close completely during the first month of pregnancy. This can damage the nerves and spinal cord severely.

Craniosynostosis consists of premature fusion of 1 or more cranial sutures, often resulting in an abnormal head shape. It may result from a primary defect of ossification (primary craniosynostosis) or, more commonly, from a failure of brain growth (secondary craniosynostosis). Craniosynostosis, or simply synostosis, is the early growing together (or fusion) of two or more bones of the skull. A newborn's skull is made up of many separate bones that are not yet fused together. Because the brain grows quickly in the first two years of life, it is important that the skull bones remain open. It is important to know that Synostosis interferes with normal growth of the brain and skull.

Skeletal dysplasias (dwarfism, as it is also known) are heterogeneous disorders characterized by abnormalities of cartilage and bone growth, resulting in abnormal shape and size of the skeleton and disproportion of the long bones, spine, and head.

Arachnoid cysts are fluid-filled sacs that develop between the central nervous system (brain and spinal cord) and the arachnoid membrane, one of three protective membranes that surround the central nervous system. Cells from the arachnoid layer produce cerebrospinal fluid, the fluid that protects and nourishes the brain and spinal cord. Consequently, arachnoid cysts are filled with cerebrospinal fluid. Most arachnoid cysts are harmless and produce no symptoms. If the cyst is large enough, however, it may press on the central nervous system, which can cause headaches, lethargy, and neurological damage.

A cavernous malformation (abbreviated as CM or also called cavernomas) is an abnormal cluster of capillaries and venules that periodically bleed and give rise to a "popcorn-like" lesion in the brain or spinal cord with very thin walls that contains blood of different ages. CMs are also known as cavernoma, cavernous angioma, cavernous hemangioma or capillary hemangioma. They have also been called "angiographically occult" or "occult" arteriovenous malformations.

A brain aneurysm is a balloon or bubble-like growth that typically develops where a major artery branches into smaller arteries, often at the base of the brain. Aneurysms have the potential to leak or rupture, causing bleeding into the brain or the surrounding area called the subarachnoid space. This subarachnoid hemorrhage can cause a stroke, leading often to brain damage or death.

Astrocytomas are a type of cancer that affects the brain. They originate in a particular kind of glial cells in the cerebrum called astrocytes. This type of tumor does not usually spread outside the brain and spinal cord and it does not usually affect other organs. Astrocytomas are the most common glioma and can occur in most parts of the brain and occasionally in the spinal cord. These tumors are “graded” on a scale from I to IV based on how normal or abnormal the cells look. 

Ependymomas are also another type of glial cell tumor. They usually develop in the lining of the ventricles (passageways in the brain) or in the spinal cord. The most common place they are found in children is near the cerebellum. Ependymomas often blocks the flow of the cerebral-spinal fluid, causing increased intracranial pressure. Ependymomas can be slow growing, compared to other brain tumors, but may recur after treatment is completed.

Meningiomas are the most common benign intracraneal tumor. They originate from arachnoid cap cells. The arachnoid is one of three protective layers (collectively known as the meninges) surrounding the brain and the spinal cord. Although the majority of meningiomas are benign, these tumors can grow slowly until they are very large if left undiscovered, and, in some locations, can be severely disabling and life-threatening. Most patients develop a single meningioma; however, some patients may develop several tumors growing simultaneously in other parts of the brain or spinal cord. .

A pituitary tumor is an abnormal growth of cells in the pituitary gland, which is the main hormone-producing gland in the body. The pituitary gland is located in the center of the brain behind the nose and eyes. A tumor in the pituitary gland can disrupt the normal balance of hormones in the body. This may cause a person to become sick. Also, eventually, some pituitary tumors will press against the optic nerves, causing vision problems.

Oligodendrogliomas are described as soft, grayish-pink tumors. They often contain mineral deposits (called calcifications) and can cause diverse areas of hemorrhage, and/or cysts. 

Choroid Plexus Tumor is found in the choroid plexus — the part of the brain within its spaces called ventricles that make cerebrospinal fluid (which surrounds and cushions the brain and the spinal cord). More importantly, as it develops, choroid plexus tumors can cause hydrocephalus.  This results in increased pressure on the brain and enlargement of the skull.

Cervical and Lumbar Discs Hernitation- Intervertebral disc herniation (in the Cervical and Lumbar portions of the Spine) is the displacement of the cushion or disc that is located between the vertebral bones. These intervertebral discs normally act as “shock absorbers” for the spine.

Medullobastomas are tumors located in the cerebellum, the part of the brain that controls balance and other complex motor functions. Generally, they grow most in the central part of the cerebellum, and less frequently in the outer parts of the cerebellum. This tumor, as well, is considered most common malignant (cancerous) central nervous system tumor in children.

Intracerebral Hemorrhage is a sudden leakage of blood into brain tissue. The blood leaks out of a very small artery and pushes into the brain tissue. As an effect, this causes to the brain and the patient exhibits symptoms of a stroke. 

Most Used Drugs in Pediatric Neurosurgery

Lidocaine or thyopental-Dugs directed to reduce high Intracranial Pressure peaks associated with neurosurgical procedures, as well as reducing the brain metabolic rate so that the organ oxygen consumption can be reduced to perform later on, an orotracheal intubation.

Hypoosmolar Soluions (Lacated Ringer)-  Its main function is to prevent interstitial edema, reducing excessive loss of blood’s volume by manipulating blood’s osmolalilty.

Corticosteroid- Their main role is to reduce brain swelling and consequently Intracranial Pressure, apart from possessing an anti-hypertensive properties. 

Versed- is a benzodiazepine a drug used for sedation during neurosurgical procedures with the sole objective of making the patient  to remain calm and relaxed. 

Instruments Used in Pediatric Neurosurgery

CT or CAT (Computerized Tomography or Computerized Axial Tomography scan)- is a medical computerized equipment that employs in order to generate cross-sectional images of the neurovascular system. From the produced images, the medical specialist is able to look at the inside of the body.  

MRI scan(Magnetic Resonance Imaging scan)- is a medical computarized equipment that provides  a diagnostic procedure through generating cross-sectional images of the brain and other organs, structures within the body without using X-ray or other forms of radiation. This produces more detailed images of the brain than a CT scan as it uses  a powerful magnetic field, radio frequency pulses and a computer. 

MR Angiography (Magnetic Resonance Angiography)- is a medical diagnostic procedure and a technique based on Magnetic Resonance Imaging to image the blood vessels. This diagnostic procedure is very important in order to evaluate the arteries as they may suffer from stenosis (abnormal narrowing of arteries), occlusion (sudden blockage of a blood vessels), or aneurysms (vessel wall dilatations, at risk of rupture). Through this procedure, surgeons can predict and avoid blood supply problems prior to surgery.

Operating Microscope- is an optical microscope specifically designed to be used by neurosurgeons when performing microsurgery. Its usage is essential to neurosurgeons  while performing  brain surgery because it allows them to  visualize the procedure in a broad perspective  or take photographs or videos of the lesions in the neurosvascular system.

Coagulation Instrument-Coagulation Instrument or Analyzer (as it is also called) is used to evaluate the platelet function and coagulation of the patient.

Tyndallers- This is one of the most popular medical equipment used for crucial surgery just like brain surgery. Tyndallers are designed to get inside the brain so as to open up the damaged tissues.

Craneotome- is considered a  type of surgical drill that is used for making holes or  large openings in the skull (craniotomy). 

 Retractor- it is used to separate the edges of a wound and/or  to can hold back underlying organs , nerves and tissues. This is done with the main purpose of accessing specific body parts during the surgery. 

 Forceps- are widely utilized in surgery for grasping and holding body structures, tissues and/or organs. 

Surgical suture (commonly known as stitches)- it is commonly used to hold body tissues after a surgical procedure has been done.  

Bone wax-  often described as waxy substance, it is put to use because it contributes to control  bleeding from the bone  during surgical procedures.

Cottonoid- are a type pf  synthetic fibers employed to protect delicate tissue and nervous structures during a neurosurgical procedure. 

Floseal-  Apart from being classified as an hemostatic agent, its main role consists of  increasing  the human blood coagulation when excessive bleeding occurs during surgery. 

Tisseel-is a fibrin sealant widely used to coagulate human blood, stop hemorrhages and seal tissues while and after a surgery. 

Dissecting forceps- these forceps are commonly used to work more easily with tissues, other materials (like for example needles and other instruments)  while simultaneously performing a surgery. 

Biopsy needle-it  is used  to obtain a sample of cells from the body in order to submit these cells laboratory testing afterwards. . Needle biopsy, as well,  may be used to take suspicious or foreign tissue or fluid samples from muscles, bones and organs.

Scalpel- is it used in virtually all neurosurgical procedure for merely anatomical dissection.

Retractor- its main function consists of either separating  the edges of a wound or  holding  back organs and/or tissues in order so that body parts under the incision may be accessed. 

Love-Kerrison Rongeur-  It is generally used to open a " window" in bone (often a small hole in the skull) with the purpose of exposing areas for operation.

Metzenbaum scissors- are a type of surgical scissors specifically designed for cutting delicate tissue. 

Stille Lour Rongeur-  it is employed greatly in neurosurgery due to the fact that is a very useful instrument by cutting away segments of bone or other hard bony tissue by ensuring a great grip on the bone. Also, it can be utilized to enlarge holes to increase the access and visibility to the surgical field. 

 Cautery- is in charge of "burning"  parts of a body; in other words, is used to  to  destroy tissue in a surgery with the intention of  mitigate damage, remove an undesired growth, or minimize other potential medical harmful possibilities such as infections.

Treatments for Common Neurosurgical Disorders

Hydrocephalus- is often treated by surgically inserting a shunt system, named ventriculostomy. This system changes the flow of CSF from the CNS to another area of the body where it can be absorbed as part of the normal circulatory process. A shunt is a flexible but  plastic tube consisting of a catheter, and a valve. One end of the catheter is placed within a ventricle inside the brain or in the CSF outside the spinal cord. The other end of the catheter is commonly placed within the abdominal cavity, but may also be placed at other sites in the body such as a chamber of the heart or areas around the lung where the CSF can drain and be absorbed. A valve located along the catheter maintains one-way flow and regulates the rate of cerebrospinal fluid flow.

Spina Bifida- The key point for treating "myelomeningocele" (another name that refers to Spina Bifida) are to prevent infection from developing in the exposed nerves and tissue through the spinal defect, and to protect the exposed nerves and structures from additional trauma.  Typically, a child born with spina bifida will have surgery to close the defect and minimize the risk of infection or further trauma within the first few days of life.

Craniosynostosis- is treated by surgery that opens the fused sutures creating space for brain growth. This procedure is called "craniotomy". 

Astrocytomas- their treatment options depend on the type, size, location of the tumor, if and how far it has spread, previous treatment received, and the patient’s overall health. Tumor re-growth can be treated with additional surgery, another form of radiation, a different chemotherapy drug (or combination of drugs), or any number of new approaches to treatment currently in the stage of development and research. 

 Meningioma- Surgery is the primary treatment for Meningiomans. Radiation therapy may be used to tumors that cannot be removed with surgery (the ones that are classified as malignant/anaplastic tumors or recurrent tumors). 

Brain Aneurisms- they are treated surgically through the so called  "open surgical clipping" surgery. This is performed by a neurosurgeon that makes an incision in the skin over the head to create  an opening in the bone in order to later on, dissect through the spaces of the brain to place a clip across the aneurysm where it arises from the blood vessel. This prevents the blood flow from entering the aneurysm.

Oligodendroglioma- the standard treatment for oligodendroglioma is surgical removal of as much of the tumor tissue as possible. Biopsy is typically performed on tumors that are not accessible to confirm the diagnosis and determine the grade of tumor. Recurrent low-grade oligodendrogliomas can be treated with surgery, radiation therapy and chemotherapy.

Choroid Plexus Tumor- Surgery, in this case, is the most probable treatment required if this tumor needs to be completely removed.  Tumor removal, as an effect, relieves the brain form suffering from hydrocephalus. 

Cervical and Lumbar Discs Hernitation- Disc herniations in the Cervical and Lumbar areas of the spine can be successfully treated with medication, physical therapy, and steroid injections performed by a pain management specialist. If these treatments are not effective, surgery is generally, the last  option.

Medullobastomas- Treatment for medulloblastomas consists of surgical removal of as much tumor as possible, radiation, and then chemotherapy (in older children and adults).

Intracerebral Hemorrhage- Once the cause and location of the Intracerebral Hemorrhage is determined, medical or surgical treatment is performed to stop the bleeding, remove the clot, and relieve the pressure on the brain. If left alone the brain will eventually absorb the clot within a couple of weeks, but the damage to the brain caused by high intracranial pressure and blood toxins may be irreversible.

Some Concepts used in the Neurosurgery Jargon

Myelopathy- this term describes any neurological deficit related to the spinal cord. Myelopathy is usually due to compression of the spinal cord by osteophyte or extruded disk material in the cervical spine.

Encephalomalacia- refers to the softening or loss of brain tissue after cerebral infarction, cerebral ischemia, infection, craniocerebral trauma, or other injury. The term is usually used during gross pathological inspection to describe blurred cortical margins and decreased consistency of brain tissue after infarction.

Spasticity- Indicates increased involuntary, velocity-dependent muscle tone that causes resistance to movement. The condition may occur secondary to a disorder or trauma, such as a tumor, a stroke, multiple sclerosis, cerebral palsy, or a spinal cord, brain, or peripheral nerve injury.

Midline Shifting- means when a person’s brain actually shifts beyond the imaginary “center line” of the brain. The shift is normally caused by a traumatic event involving the brain or head, and can indicate further problems with the brain, such as intracranial pressure or tumor growths. The brain normally shifts away from a traumatized section, causing neurological abnormalities that affect the behavior of the patient.

Radiculopathy- is a pinched nerve in the spine. It occurs when surrounding bones, cartilage, muscle, or tendons deteriorate or are injured. The trauma causes these tissues to change position so that they exert extra pressure on the nerve roots in the spinal cord.

Scaphocephaly- this concept indicates the condition where the head is disproportionately long and narrow. Scaphocephaly can result from the premature fusion of the sagittal suture or from external deformation. Scaphocephaly is particularly common among infants who are born prematurely.

Cerebral edema- this term makes reference to the excess accumulation of cerebrospinal fluid in the intracellular or extracellular spaces of the brain.

Ventriculomegaly- term employed to refer to the brain condition that occurs when the lateral ventricles become dilated.

Brain Herniation- is when brain tissue, cerebrospinal fluid, and blood vessels are moved or pressed away from their usual position inside the skull as a result of mass effect.

Nervous System Anatomy and Functions

Cerebrum – is the top, front portion of the brain and consists of two hemispheres. The cerebrum can be divided into four lobes: frontal lobe, parietal lobe, occipital lobe, and temporal lobe.  The cerebrum main function is to  manage many everyday activities, including motor function, cognitive abilities (thinking and reasoning) sensory impulse interpretation, speech and language, bowel and bladder control, sexual ability, and emotional control.

Frontal lobe- Is involved in motor function, problem solving, spontaneity, memory, language, initiation, judgment, impulse control, and social and sexual behavior.

Parietal lobe- carries out some very specific functions. This is due to the fact that this lobe has a lot of responsibilities and at the same time, is   able to process sensory information within seconds( such as taste, temperature and touch are integrated, or processed). 

Brain Ventricles- are a communicating network of  brain cavities filled with cerebrospinal fluid (CSF) . The ventricular system is composed of 2 lateral ventricles, the third ventricle, the cerebral aqueduct, and the fourth ventricle. The choroid plexuses located in the ventricles produce CSF, which fills the ventricles and subarachnoid space, following a cycle of constant production and reabsorption.

Hypothalamus- is primarily responsible for hormone production. The hormones produced by this area of the brain controls body temperature, thirst, hunger, sleep, circadian rhythm, moods, sex drive, and the release of other hormones in the body among  many essential processes of the body (including behavioral, autonomic, and endocrine functions).

Occipital lobe- is in charge of in visuospatial processing, discrimination of movement and color.

Temporal lobe- plays an important role in organizing sensory input, auditory perception, language and speech production, as well as memory association and formation.

Pituitary gland- serves as an important link between the nervous and endocrine systems by releasing many hormones which affect growth, sexual development, metabolism and human reproduction.

Pineal gland-  is involve in the  production of several important hormones including melatonin. Melatonin, on the other hand, influences sexual development and sleep-wake cycles. 

Thalamus- It is mainly involved in sensory perception and regulation of motor functions. As a regulator of sensory information, the thalamus also controls sleep and awake states of consciousness.

Corpus Callosum- is a thick band of nerve fibers that apart from dividing  the cerebrum into left and right hemispheres,  it connects the left and right sides of the brain allowing for communication between both hemispheres through the transfer of motor, sensory, and cognitive information between the brain hemispheres.

Cerebellum- is in charge of receiving  information from the sensory systems, the spinal cord, and other parts of the brain. Also, it regulates motor movements, and coordinates voluntary movements such as posture, balance, coordination, and speech, resulting in smooth, balanced muscular activity. More importantly, it is also involved motor behaviors.

Cerebral Amygdala- is an almond-shape set of neurons located deep in the brain's medial temporal lobe. These has been shown to play a key role in the processing of emotions.

Spinal Cord- is a long, thin, tubular bundle of nervous tissue and support cells that extends from the brain. The brain and spinal cord together make up the central nervous system. The spinal cord functions primarily in the transmission of neural signals between the brain and the rest of the body, but also contains neural circuits that can independently control numerous reflexes and central pattern generators. Last but not least, the spinal cord has two functions: serve to conduct motor and sensory information and serve as a center for coordinating certain reflexes.