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Arnold-Chiari malformation is a chronic illness that affects the cerebellum, a brain constituent that is responsible for body balance. The downward dislocation of the cerebellum and brainstem through the foramen magnum results in the malformation of the brain due to the inadequacy of space at the indented lower rear of the skull. As a result, pressure on the cerebellum hinders the flow of the cerebrospinal fluid, which protects and surrounds the brain and spinal cord (Hewitt & Gabata, 2011). A wide range of symptoms such as dizziness, body weakness, vision problem, numbness, headache, coordination and balance problems are allied to the condition. Arnold-Chiari malfunction is classified into three major types, which include type I, type II, and type III. Type I does not result in any symptoms, and is recognized accidentally when assessing other chronic conditions. Type II is characterized by myelomeningocele, which is an instance of spina bifida that is caused by failure of closure of backbone and spinal canal during the process of birth. Type III is the most detrimental form of Arnold-Chiari malfunction and can lead to persistent neurological shortcomings. Type III is also associated to hydrocephalus, syringomyelia and spinal curvature (Hewitt & Gabata, 2011). The causalities of this chronic condition include brain and spinal structural faults during the cause of fetal development, genetic mutations and inadequacy of essential vitamin in the maternal diet. The latter refers to congenital Chiari malformation, which is caused by excessive drainage of spinal fluid from the lumbar or thoracic segments of the spine because of injury or infection.
Statistical Data of People Dealing With/Afflicted By the Condition
During the years 2009 to 2011, approximations of 364 patients were referred for Arnold-Chiari malformation in San Francisco. Each patient had to meet the requirement of having any Arnold-Chiari malformation symptom that conforms to medical referral form or basichondrocranium abnormality linked to Arnold-Chiari malformation or syringomyelia. It was discovered that 29% of the referred patients had already resorted to surgical treatment of the chronic condition. Other patients’ registration requirements included admittance of the entire preoperative records, radiographic and neurological results. However, an exception of patients with neurological discrepancies associated to surgery also took place. The history of pregnancy and birth, maternal exposure to harmful material, probable environmental disclosure, medical and family and symptoms were classified in a database. Patients were demanded to undertake physical assessment, an entire neurological examination and Magnetic Resonance Imaging of the head and spine. The patients’ inabilities were evaluated using Karnofsky performance scale of 0 to 100.
Magnetic Resonance Image was employed in the examination of morphological characteristics of Posterior Cranial Fossa. Cranial and intracranial illness was not evidenced in patients who undertook Magnetic Resonance Imaging for imprecise complaints among 50 randomly selected patients. Posterior Cranial Fossa investigation involved measuring the length of the supraocciput, the slope of tentorium and the degree of cerebellar herniation. Other patients were examined qualitatively. The tentorium slope was defined as normal, increased or decreased depending on measurement considerations for the 50 control patients (Goel & Cacciola, 2011). The odontoid retroflexion was described as a proclivity of the odontoid process’ tip beyond 15 degree posterior.
The volume of Posterior Cranial Fossa is the spherical space surrounded by the tentorium, occipital bone, petrous ridges and clivus. Cavalieri method is used to determine the volumetric extent of the posterior cranial fossa, since it is simple and gives an accurate outcome. Total PCF volume, cerebrospinal fluid volume of the PCF and brain volume of the PCF were measured using the Cavalieri method. Among the 364 afflicted patients, 275 were female, whereas 85 were male. Lifelong complaints that included headaches and clumsiness accounted for 37% and many patients approved an impulsive commencement of indications, 24% recalled that trauma is the notable symptom among others (Rosenberg, 2009).
Psychosocial Problems of the Afflicted Patient Population
Psychosocial problems are notable in Arnold-Chiari Malformation patients and the basic indications such as depression, poor sleep and fatigue justify the association of these challenges to patients. Some patients affirm that they experience “brain fog”, a condition that results to the difficulty in thinking, comprehending and intricacy of word finding. The first psychosocial challenge is Attention Deficit Disorder (ADD), which is popular among children patients. This disorder has been evaluated and known to occur at an incidence rate of 3% to 5% in children. The worsening part is that it does not lessen due to age, but is propagated to adulthood. This disorder is likely to lead to substance abuse among men, whereas it is likely to create mood and anxiety problems among women. The recognition of the problem demands symptoms appearance for more than six months to maladaptive level and incorporates areas of lack of concentration, hyperactivity and impulsivity.
Cases of short-term manifestations such as sedative properties are connected to Arnold-Chiari malformation. This psychosocial challenge is attributed to medications taken by patients to control pain, in particular the opiate class, which are from opium. Continued usage of these drugs for a longer time is likely to affect our thinking processes and mental capability. Other types of drugs recognized to lead to tranquilizing probabilities used by Arnold-Chiari patients include seizure, anti-histamine and anti-anxiety medications among others, often have indications about somnolence.
Another psychosocial challenge is the declination in cognitive skills with an increase in age among adult patients. The late diagnose of the type I Arnold-Chiari malformation, mostly around forty to fifty years, when patients have the expected impacts of aging in brains is connected to this disorder. The aging process and feelings of brain malfunction cannot be reversed despite surgery aiding the patient’s disorder (Parker, 2004). The decrease in this skill is likely to hamper with the brain leading to slow mental process of information and auditory processing, which is the capability to evaluate, blend and segment sounds.
Pain is the most common disorder among chiari malformation patients. If it is not managed for a long time, it can be the causality of patients’ withdrawal from family and friends. Pain hinders people from managing their families, holding steady jobs and facing personal future. This further aggravates to other problems, such as loss of income and intrusion of poverty, and lack of sufficient parental guidance among children. In addition to, pain drastically hinders people’s aptitude to concentrate, communicate, multi-task and mobility among others.
Hormonal abnormalities attributed to Arnold-Chiari Malformation have significant effects on concentration abilities among patients. This is connected to abnormality of the pituitary function, which may have a negative influence on hormone levels. Anomaly in the thyroid, both too and too low have an impact on concentration. Other shifts in hormonal levels such as changes in menopause have been affirmed to hamper cognitive functions.
Sleep-disordered challenges particularly linked to patients with sleep apnea. Sleep apnea is a condition of prolonged termination in breathing. Arnold-Chiari Malformation is likely to reduce cognitive skills such as thinking. Patients may present central apnea, hypoventilation and hypoxia conditions. Sleep apnea patients have a depression of the respiratory center of cranial nerve dysfunction entailing windpipe airway or vocal cords, which poses a psychosocial challenge to patients.
Depression is another psychosocial challenge affecting chiarists. Presently, depression is associated with poor functioning of or lower levels of specific chemicals in the brain referred to as neurotransmitters, which are inter-cell signal conveyors. Other factors that play a key role in initiating depression include isolation, limited social support, low self-esteem, stress and awareness of helplessness among patients. The depression undergone by these patients is called clinical depression typified by long lasting sadness, incapability of doing previously enjoyed duties, reduced appetite that results from changed eating habit and changes in sleeping habits (Parker, 2004). On the contrary, depression is treatable through exercise, counseling and medication, and patients can recover from it.
A research study to link Arnold-Chiari malformation complications to grafts was done in September 30, 2011 by surgeons at Duke. There has been a debate as to whether the dura should be opened during surgery, and the type of graft of the implant to employ when it is opened as a procedure during Chiari decompression. Previously, various materials such as cadaver, collagen, derivative materials, patient’s body tissue and synthetic materials have been substituted for dura. These materials aroused many arguments among surgeons, and none of them has been convincing. The choice of graft lies on the surgeons’ preference and experience. The research reveals that some surgeons have increased their experience and complication rates through using specific arrangement of graft materials and sealants. Certainly, a forceful switch in the graft materials led to the increased complication, which demanded investigations. Researchers used the EnDura graft, a derivative of collagen material, though it was eliminated narrowing their choice to cadaver or durepair. This switch resulted to complications.
The researchers went through records of 114 successive pediatric surgical patients from 2004 to 2008 to verify the connection between complications and grafts. Some of the surgical complications encountered included aseptic meningitis, Cerebrospinal fluid seep outs and psudomeningocele. The analysis revealed that 76% of the patients had obtained an EnDura graft prior to its market elimination while other patients were almost equally divided between Durepair and Cadaver, 11% and 13% respectively. It was also noted that many surgeons opted to apply dural sealant to help close the graft, with tisseal being used popularly accounting for 66%and DuraSeal garnering 10%. It was realized that the highest number of complications among patients who obtained Durepair, accounting for 42% complication rate and 25% reoperation. Cadaver with relative lower complication rate of 27% and 13% reoperation followed this. These complications aroused from the third week after surgery and thereafter. Analysis of the complication from the perspective of time that when it was done, before or after elimination of EnDura revealed that complication vaulted to 35% from 18% after the switch (Hewitt & Gabata, 2011).
The researchers concluded that it was not worthy proceeding further since some grafts had been eliminated from the United States and Canada’s market. In addition, these findings should be authenticated by studies from other institutions. Essentially, patients facing surgery should have knowledge of the graft in relation to surgeon’s experience to avoid complications.
The second research study was on the new scoring system proposed for Arnold-Chiari malformation. There are two primary issues related to Chiari results include lack of a consistent, logical objective, quantitative result measure and less clinical follow-ups. Lack of an effective outcome measure hinders the comparison and assessment of the little research study that is available on chiarists’ results and methodological techniques. It is also impossible to compare varying records on the advantages of opening the Dura during the research when a different measure of success is employed on the subject. Moreover, reading the significant improvement is not helpful, patients, since each patient will infer it variably.
A new scoring system was designed and proposed by University of Chicago for Arnold-Chiari malformation outcomes. It is called Chicago Chiari Outcome Scale, CCOS. The scoring system entails four classes namely pain symptoms, non-pain symptoms, functionality and surgical complications. Additionally, the system rates patients basing on post and pre surgical information on a maximum scale of four for each class. The scores are totaled out of 16. This research comprised of 94 patients scoring between 13 and16, 45 scored between 9 and 12, while 7 were in the 4-8 range. It revealed a wider improvement, constant, worse scale, with the majority of the improved scoring in the highest range. This is indicated in the following table (Reynolds & Fletcher-Janzen, 2009).
Under the pain category, symptoms such as headaches, neck and shoulder pains are captured. A maximum score of four is awarded upon resolution of the pre-surgical and post-surgical symptoms and no emergence of new Chiari type pain indications. A score of three is awarded upon improved pre-surgical pain symptoms implying that the symptoms can be managed through medicinal control (Goel & Cacciola, 2011). Existence of post and pre surgical symptom earns two points, which cannot be managed by medicines. The least point is awarded upon worsening of any pain symptom.
The non-pain category follows the same criteria as the pain category, but centers on primarily on symptoms that are linked to Arnold-Chiari Malformation. Such symptoms include dysphagia, ataxia, sensory disturbances, vertigo, weaknesses and drop attacks. Other neurological indications are also incorporated in this category. The functionality category is designed evaluate the patient’s ability to focus on daily responsibilities such as school, work and family care. A maximum score of four is awarded when a patient can fully attend to these responsibilities, whereas less than 50% impairment gets three. When a person suffering from Arnold-Chiari malformation is restricted from attending more than 50%, he scores two, and one implies a total inability to participate in his or her responsibilities.
According to Hewitt & Gabata (2011), complications category covers complications such as cerebrospinal fluid leaks, demand for more surgery and lumbar punctures. A maximum score of four implies that there is no complication along the period. Persistent but controllable complications get a score of two, and relentless complications obtained the minimum score of one. However, certifying this scale is not appropriate since there is no gold standard for assessment thus it can be quite tricky. This system need to be studied before it gains acceptance. On the contrary, it can offer comprehensive information regarding patients and equip researchers with a new effective tool to employ in research.
Some community-based programs have been initiated to fight against Arnold-Chiari malformation. For example, project shunt in Guatemala and ThinkFirst. Project shunt has been operating since 1997 when Healing the Children,a voluntary organization approached the University of Michigan. Guatemala has registered cases of spina bifida and myelomeningocele. The Healing the Children were searching for pediatric assistance to handle neurosurgery problems. University of Michigan has organized annual trips to Guatemala to perform neurosurgical operations for the past 13 years. The university admits performing 26 operations in 2008 saving children affected with the condition. The program receives finance from donations, well-wishers and other Non Governmental Organizations such as International Financing Corporation. The program is presumed to consume a budget of approximately 235000 dollars, with a larger portion designated to the provision of health care to Chirian children in Guatemala.
Another community-based program is the Chiari and Syringomyelia Program initiated by UCLA Neurosurgery. This program offers complete diagnosis and options for chiari malformation and syringomyelia patients. The program estimates to have treated 1000 patients within the last 20 years. Its diagnostic services include clinical assessment, with extensive experience in the diagnosis. The program uses a multidisciplinary team approach, with expertise neurosurgery, neurology and rehabilitations to patients. Treatment services involve minimal invasive surgical control in posterior decompression operations and transoral odontoidectomy. The UCLA, chiari and Syringomyelia sources its funds from professional services offered and well-wishers. The budget is assumed 350000 dollars.
Minimization of Chiari malformation
Some occurrences of the disease are asymptomatic and have less obstruction to the patients’ activities. Medications might be used to reduce pain and ease symptoms. The only existing solution to minimize the disorder is surgery to correct or stop the development of the damage to the body’s nervous system. Surgery involves removal of the lamina of the first cervical vertebrae and a portion of the occipital bone to relieve pressure (Icon Health Publications, 2003). The surgery also requires the opening of the dura mater followed by the expansion of the space below; as a result, a graft is frequently used to envelop the expanded posterior fossa.
An additional way of minimizing the effect of the condition is to detethering of the spinal cord as an option to relieve firmness of the brain against the skull opening. This prevents decompression surgery and linked trauma. This approach is not authenticated in the medical literature since it has reported only a few cases of survival of patients (Icon Health Publications, 2003).
Arnold Chiari Malformation has several psychosocial challenges, which include attention deficit disorder, declination of cognitive skills, Pain, hormonal abnormalities and depression among others. Some research studies that are associated to the disorder include Chicago Chiari Outcome scale, scoring system that entails four classes namely pain symptoms, non-pain symptoms, functionality and surgical complications. Community based programs include the UCLA Chiari and Syringomyelia program that aim at reducing the disorder among patients. The disease has no cure; therefore, measures are supposed to take measures such setting emotions aside to reduce stress to prolong their lives. Neurosurgeons advocate for surgery as the only medically authenticated way for minimizing impacts on patients.