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By R. Ugolf. Delaware State University. 2018.

Families should be given all the records and support that are needed for them to get a meaningful second opinion purchase 5 mg proscar visa. If this second opinion is similar to that given by the primary physician discount 5 mg proscar with mastercard, the family is often greatly comforted in moving ahead. However, there is still variability in med- ical treatment for children with CP, so depending on the family’s choice of opinions, the recommendations may be slightly to diametrically opposed. In a circumstance where the recommendation of another physician dif- fers significantly, the primary physician must be clear with the family and place the second opinion in the perspective of their recommendation. Some- times the words used may sound very different, but the recommendations are very similar. In other circumstances, the recommendation may be dia- metrically opposed and the primary physician must recognize this and ex- plain to the family the reasons for their recommendation. When recom- mendations are diametrically opposed, clear documentation, including the discussions concerning the other opinion, is especially important. This situ- ation has a high risk for disappointment. Often, families have great difficulty in choosing between divergent opinions, even when one opinion is based on published scientific data and the other opinion is completely lacking in any scientific basis (Cases 1. Therefore, a family may base their decision on other family contacts, a therapist’s recommendations, or the personality of the physician. Physicians must understand that it is the family’s responsibility and power to make these choices; therefore, with rare exception, no matter how medically wrong the physician believes these decisions are, the family must be given the right to choose. Only in rare, directly life-threatening circum- stances will a child protective service agency even consider getting involved, and then this involvement is usually very temporary. With a long and chronic condition such as CP, temporary intervention by a child protective agency generally is of no use in interacting with families. With clear documentation of the recommendations, the physician must let the family proceed as they choose; however, we always tell them that we would be happy to see them back at any time. When they undergo treatment against their primary physi- cian’s advice and return, usually after several years, the physician should not make the previous situation a conflict. The family usually feels guilty and may not want to discuss past events. Occasionally, they will come back and 16 Cerebral Palsy Management Case 1. Her development was noted to be significantly delayed Her parents were assured that good treatment was avail- early on, and her CP was recognized within the first 2 years able to get rid of her pain; they were informed of the treat- of her life. By school age, Judy was not able to walk, but ment options, and it was strongly recommended that these was able to do some speaking, and there was concern options be pursued. Surgery was scheduled emergently and about her educational ability. At age 7 years, she was seen was completely successful in alleviating her pain. This pediatrician thought that she had excellent sons chooses alternative medical treatments instead of cognitive ability, but also noted that she was developing well-recognized appropriate medical treatment. This type significant contractures, and recommended follow-up with of behavior may be very difficult for a physician to accept. However, she was not seen by a This family only saw us once when their daughter was 10 pediatric orthopaedist until age 10 years, when she started years old, and then did not come back for more treat- to develop some pain in the right hip. In these situations a physician can only make the in a regular school and was complaining of pain in the hip recommendations, but cannot force the families to follow during the school day. An evaluation demonstrated a through with treatment. This girl clearly would have been completely dislocated right hip and severe subluxation of much better served by a reconstruction at age 10 years; the left hip; however, this hip was an excellent candidate however, the family had complete control. This family’s for reconstruction because, at age 10 years, she had sub- choice of treatment was not inappropriate enough legally stantial growth remaining. Hip reconstruction was recom- whereby the physician would have gained anything by re- mended to the family and details were given. For reasons porting the family to child protective services or making that were never quite clear, this family pursued many other any other efforts to try to force them to have treatment. In addition to the spinal cord stimulator, other ticity or pain. There is nothing that the primary caring alternative medicine treatments were pursued. The hip physician can do except try to persuade the family and pain would get better intermittently and then would flare then accept their decisions. However, it is very important up, requiring her to be in bed for several days. By 14 years to always leave the family the option of coming back of age Judy had periods of relative comfort between bouts when they are ready and then provide appropriate treat- of severe pain, until age 15 when the pain became more ment, as was done in this situation. By age 15 years, as she entered high Six weeks after this girl’s surgery, at which point all her school with normal cognitive and educational achieve- hip pain was gone, the family noted that she was having ments, the pain got so severe that she could no longer sit difficulty sitting because of her scoliosis. At this point, her parents kept her very keen on moving ahead and having the scoliosis cor- home in bed and gave her a variety of different pain med- rected. This is a circumstance where although the family ications. She was out of school for 1 year, spending most feels extremely guilty and are often very hesitant to return of her time in bed, when her parents finally came back because of fear that the physician will be angry with them, with a request to have her hip reconstructed because they once the appropriate treatment has been performed and now perceived she could no longer deal with the pain. She was perceived to be normal until after having been extremely edematous, and the mild 18 months of age when her development was noted to wound drainage was not a concern. The patient tinued to make progress and by age 3 years had started was still afebrile, was continuing to make good progress walking independently and was speaking.

Motor neuron purchase 5 mg proscar amex, or efferent neuron— system response cheap proscar 5mg; usually requires interneurons a cell that carries impulses away Motor neuron Carries impulses away from the CNS toward the from the CNS. Motor impulses leave effector, a muscle, or a gland Effector A muscle or gland outside the CNS that carries out a the cord through the ventral horn of response the spinal cord gray matter. Injection of anesthetic into the epidural space in the lumbar region of the spine (an “epidural”) is 2 Sensory often used during labor and childbirth. The spinal route neuron also can be used to administer pain medication. This process of demyelination (quadriceps muscle) slows the speed of nerve impulse conduction and disrupts nervous system communication. Although the cause of MS is not completely understood, there is strong evidence that it involves an attack on the myelin sheath by a person’s own immune system, a situation described as autoimmunity. Genetic makeup, in combination with environmental fac- tors, may trigger MS. Some research suggests that a prior viral or bacterial infection, even one that occurred many Figure 9-14 The patellar (knee-jerk) reflex. Numbers indi- years before, may set off the disease. ZOOMING IN How many MS is the most common chronic CNS disease of young total neurons are involved in this spinal reflex? The disease affects women transmitter is released at the synapse shown by number 5? MS progresses at different rates depending on the contracting, is one example of a spinal reflex. If you tap individual, and it may be marked by episodes of relapse the tendon below the kneecap (the patellar tendon), the and remission. At this point, no cure has been found for muscle of the anterior thigh (quadriceps femoris) con- MS, but drugs that stop the autoimmune response and tracts, eliciting the knee-jerk reflex (Fig. Such stretch reflexes may be evoked by appropriate tap- Amyotrophic (ah-mi-o-TROF-ik) lateral sclerosis is a ping of most large muscles (such as the triceps brachii in the disorder of the nervous system in which motor neurons arm and the gastrocnemius in the calf of the leg). The progressive destruction causes muscle flexes are simple and predictable, they are used in physical atrophy and loss of motor control until finally the affected examinations to test the condition of the nervous system. Poliomyelitis (po-le-o-mi-eh-LI-tis) (“polio”) is a Medical Procedures Involving the viral disease of the nervous system that occurs most com- Spinal Cord monly in children. Polio is spread by ingestion of water contaminated with feces containing the virus. It is sometimes necessary to remove the gastrointestinal tract leads to passage of the virus into a small amount of cerebrospinal fluid (CSF) from the the blood, from which it spreads to the CNS. CSF is the fluid that circu- tends to multiply in motor neurons in the spinal cord, lates in and around the brain and spinal cord. This fluid leading to paralysis, including paralysis of the breathing is taken from the space below the spinal cord to avoid muscles. Because the spinal cord is Polio has been virtually eliminated in many countries only about 18 inches long and ends above the level of through the use of vaccines against the disease—first the the hip line, a lumbar puncture or spinal tap is usually injected Salk vaccine developed in 1954, followed by the done between the third and fourth lumbar vertebrae, at Sabin oral vaccine. A goal of the World Health Organiza- about the level of the top of the hipbone. The sample tion (WHO) is the total eradication of polio by worldwide that is removed can then be studied in the laboratory vaccination programs. Anesthetics or medications are Tumors Tumors that affect the spinal cord commonly sometimes injected into the space below the cord. The arise in the support tissue in and around the cord. They anesthetic agent temporarily blocks all sensation from are frequently tumors of the nerve sheaths, the meninges, the lower part of the body. Symptoms are caused by pressure on the thesia has an advantage for certain types of procedures or cord and the roots of the spinal nerves. These include THE NERVOUS SYSTEM: THE SPINAL CORD AND SPINAL NERVES 191 Box 9-1 Hot Topics Spinal Cord Injury: Crossing the DivideSpinal Cord Injury: Crossing the Divide pproximately 11,000 new cases of spinal cord injury occur ◗ Using neurotrophins to induce repair in damaged nerve tissue. Aeach year in the United States, the majority involving males Certain types of neuroglia produce chemicals called neu- ages 16 to 30. Because neurons show little, if any, capacity to re- rotrophins (e. Intravenous injec- The factor called Nogo is an example. Successfully transplanted donor duces swelling at the site of injury and improves recovery. Spinal tains the cell bodies of the sensory neurons. A ganglion cord tumors are diagnosed by magnetic resonance imag- (GANG-le-on) is any collection of nerve cell bodies lo- ing (MRI) or other imaging techniques, and treatment is cated outside the CNS. Fibers from sensory receptors 9 by surgery and radiation. The ventral roots of the spinal nerves are a combina- Injuries Injury to the spinal cord may result from tion of motor (efferent) fibers that supply muscles and wounds, fracture or dislocation of the vertebrae, hernia- glands (effectors). The cell bodies of these neurons are lo- tion of intervertebral disks, or tumors. The most common cated in the ventral gray matter (ventral horns) of the causes of accidental injury to the cord are motor vehicle cord.

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There may be a sympathetic blockade-type effect decreasing the overreacting peripheral basal motor response that creates blue feet when the feet get cold proscar 5 mg otc. In this report purchase proscar 5mg fast delivery, a significant number of men reported a de- creased time and rigidity of erections, and two men reported losing the ability to ejaculate. This complication should be men- tioned to patients for whom it might be a concern. A small group of children require a very high dose of intrathecal baclofen, sometimes 2000 to 3000 mg per day. Also, some children who are on a lower dose suddenly need increased doses if their spasticity is increasing 6 months to 2 years after the implantation. If a child has had an increasing need for baclofen, or is requiring a sudden increase in baclofen after having been stable, catheter malfunction should be considered. After the full workup for catheter malfunction, or after demonstration that the catheter is function- ing, another option for dosing is to use a drug holiday. In this treatment, the intrathecal baclofen is reduced and then slowly decreased to zero to avoid a withdrawal psychosis. The pump may be left in the turned-off position for 1 month and then the drug slowly reintroduced. This drug holiday should allow the nervous system to redevelop a sensitivity to the drug. Another way to use this concept of a drug holiday is to give large intrathecal boluses sev- eral times a day instead of continuous dosing. Therefore, instead of giving a continuous dosing rate of 2000 mg, the child may be given 1000 mg just before bedtime, and then another 1000 mg over a 30-minute period the first thing in the morning. These different dosing regimens may provide a better benefit in some children compared with continuous administration. The current role of intrathecal baclofen in the treatment of children with severe spasticity is primarily in nonambulatory children. From a theoretical standpoint, this treatment should also be ideal for the 3- to 8-year-old spas- tic ambulatory child for whom a rhizotomy could be considered. The size of the pump and the need for long-term maintenance, with filling at least every 3 months, has made it difficult to convince parents and physicians that this is a good treatment option. Also, there are no objective published data that allow one to develop confidence. This question would be an excellent project for a well-controlled study similar to the randomized rhizotomy studies. As better engineered pumps are designed and medication that has more stability is found, so that the pump only needs to be filled every 6 months to 1 year, the intrathecal pump will become an even better option, especially for high-functioning children. Also, there are other medications that may be even better choices than baclofen; however, each of these needs to be trialed and tested in children with spasticity. Neurologic Control of the Musculoskeletal System 115 Rhizotomy Central nervous system surgical approaches to reducing spasticity are most commonly done at the spinal cord level, with posterior dorsal rhizotomy be- ing the most widely used procedure. This procedure involves cutting the dorsal sensory nerve rootlets, which contain the afferent sensory nerves, from the muscle spindles as well as other sensory nerves. By using peripheral motor stimulation and recording the electrical activity in the proximal sen- sory nerves, abnormal rootlets are identified and then sectioned. Many rootlets are not quite normal or not very abnormal, which makes choosing the abnormal ones very subjective. Evidence exists that there is no difference between selective nerve sectioning based on electrical stimulation and just random sectioning. The operative procedure may be done as popularized by Peacock et al. There is no apparent differ- ence between outcomes of the two procedures based on published reports; however, the Peacock technique is more popular in North America. The Fazano technique involves doing only a T12–L1 laminectomy in which the rootlets are separated at the end of the conus. This exposure may lead to thora- columbar kyphosis as a late spinal deformity. The Peacock approach involves a laminec- tomy from L1 to L5 with separation of the rootlets as they exit the spinal canal. The long-term spinal deformity, which occurs as a consequence of the Peacock technique, is progressive lumbar lordosis. Rhizotomy has been described for 100 years, and has had a series of advocates and periods of popularity, but has never developed a stable level of acceptance in medical practice. Outcome of Rhizotomy Since the modern popularization of rhizotomy by Fazano and Peacock in the 1980s, there have been many reports in the literature of its use in children with CP. A search at the time of this writing revealed 111 citations, the ma- jority reporting small, individual surgeon’s experiences. There seems to be a universal agreement that spasticity is reduced acutely after the dorsal rhizo- tomy procedure. There are no studies with good follow-up to maturity; all the long-term studies consider 5 to 10 years as long term. Also, the majority of the studies have no controls with respect to other treatments or for the ef- fects of growth and development. There are two well-designed studies that are very short term, 1 year or less, which randomized the children to a physical therapy-only group or a physical therapy and rhizotomy group. Therefore, the general feeling is if spas- ticity were removed, everything would be better, which is the general tone of many articles reporting the outcomes of rhizotomy. There are no direct com- parisons of rhizotomy to intrathecal baclofen, except for cost comparison. Although there may be less need for orthopaedic surgery after a dorsal rhi- zotomy has been performed, others have shown that there definitely is still significant skeletal deformity occurring throughout development, possibly necessitating more orthopaedic surgery.

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If the child has poor oral motor con- trol purchase proscar 5mg online, the stomach contents may be aspirated into the lungs buy 5mg proscar otc, causing chronic aspiration syndrome with reactive airways disease. Even when no direct as- piration occurs, reactive airways disease may be present secondary to the esophagitis, which is mediated through vagal nerve irritability, caused by the inflammatory process in the esophagus. The treatment of mild to moderate gastroesophageal reflux is with a smooth muscle agonist such as cisapride and an H2 blocker such as cimetidine or ranitidine. If there is no response in 4 to 8 eight weeks, addi- tional workup with endoscopy and muscle biopsy is often indicated. If this biopsy is inconclusive, a 24-hour pH probe may be done to measure the acid in the lower third of the esophagus. The conservative treatment for hip pain is resting the joint by stopping unnecessary movement. If the pain is severe and it is unclear if the gastro- intestinal system is involved, injecting the hip joint with bupivacaine and a deposteroid can be very helpful. The bupivacaine will demonstrate how much pain relief comes with anesthesia of the hip joint, and the steroids are a safe way to temporarily decrease inflammation in the presence of gastritis or esophagitis. The hip should then be treated with reconstruction or palli- ation as described in Chapter 10, which discusses hip problems (Case 3. It is important that the treatment of the hip and gastrointestinal system is done concurrently, rather than one specialist waiting to address the remain- ing problem after the other fixes his problem. The gastrointestinal prob- lems occasionally occur combined with a rapid increase in scoliosis in which 3. The naproxen was increased evaluated because her caretakers felt she was having se- to 500 mg twice a day and the caretakers felt she was vere hip pain limiting her sitting tolerance, which made now very comfortable. Nine months later while still on transfers difficult. The physical examination demonstrated naproxen, she developed a severe gastrointestinal bleed pain with any significant motion of the right hip, and a from a gastric ulcer requiring a prolonged hospital radiograph showed a dislocated hip with severe degen- course. Following this, the hip pain returned, and she was erative changes (Figure C3. She was started on then scheduled for palliative treatment of her hip with the naproxen, 375 mg twice a day. One week later the care- implantation of shoulder prosthesis (Figure C3. When the gastrointestinal problem is under medical management, a spinal fusion should be done. In some cases, the spinal fusion will stop the gas- troesophageal reflux completely; however, in other cases, the reflux will get worse. This worsening reflux can still be managed medically. When reflux does not improve, the next treatment is surgical reconstruction of the gastro- esophageal junction by fundoplication. This procedure is easier and probably longer lasting when the spine is corrected first. There are no published data on the advantage of correcting the spine first, but this has been the experi- ence of our facility. After 2 months, John however, the hips were normal on radiographs and there developed a severe abdominal distension, and it became was no pain relief with injection of the hip joint. The clear that he had a chronic infection in his ventriculo- gastrointestinal workup was also normal. John had a ven- peritoneal shunt when he developed severe ascites. Other Common Problems Incurred and Encountered in the Workup of Pain in the Noncommunicative Child Although the most common cause of discomfort or pain in noncommu- nicative children is by far the gastrointestinal system combined with hip or scoliosis pain, other problems do occur. There may be problems with ven- triculoperitoneal shunts including shunt occlusion or peritonitis. Peritonitis associated with a shunt catheter may be very difficult to diagnose early in the course (Case 3. Many noncommunicative children spend consider- able time reclined and are at more risk for developing sinusitis. The si- nusitis is often very hard to diagnose and initially shows up as an increased uptake on the bone scan. Sinusitis may present with behavior changes, such as refusing to sit or to stand, instead of crying in pain (see Case 3. Ab- scesses of the teeth may have an element of increased drooling or biting as- sociated with the discomfort. An acute surgical abdomen may be very difficult to diagnose and usually leads to the child’s death if not correctly diagnosed before the child comes to see the orthopaedist. Constipation is another com- mon problem in nonambulatory children that can cause severe chronic dis- comfort. The constipation may also lead to urinary incontinence or urinary retention. Urinary calculi may be a cause of intermittent severe pain. Genitourinary Problems Most urinary problems in children with CP do not present as part of an un- known pain problem syndrome, although this can happen. The most com- mon genitourinary problem is undescended testicles in boys with spasticity. Often, these boys are not carefully checked throughout middle childhood when orthopaedists see them most frequently. Recognizing undescended tes- ticles is easy if the boys are examined, especially if the examination is done during a concurrent hip examination while the child is under anesthesia for hip or lower extremity surgery. The boys should be referred to a urologist for an evaluation if the testicle cannot be palpated.

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