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The general purpose of the following treatment methods is to divert the flow of CSF from the blocked aqueduct, which is causing the buildup of CSF, and allow the flow to continue. Another goal of these treatments is to reduce the stress within the ventricles. Studies have not shown that either of the following treatments results in a higher IQ of the patient, and there is not statistical difference in a patient's quality of life based on treatment method. The following treatment methods are not used for aqueductal stenosis caused by tumor compression; if the obstruction is a direct result of tumor compression, CSF flow may be normalized by the surgical removal of the tumor.
An endoscopic third ventriculostomy (ETV) is a procedure where an incision is made in the bottom of the third ventricle to make a drainage point for CSF to flow out of. The procedure is minimally invasive and is performed endoscopically. The goal in the surgery is to create a path for communication between the third ventricle and the subarachnoid space outside the brain for reabsorption of CSF. ETV has a higher failure rate than shunting during the first 3 postoperative months, but after this time the risk of failure progressively drops to become half as high as the failure risk for shunting.
This treatment does not place a foreign body into the patient so there is a much lower risk of infection as compared to a shunt procedure. Along with not implanting a device, this procedure avoids mechanical issues like disconnection, over or underdrainage, and valve dysfunction. The surgery begins by entering the right or left lateral ventricle endoscopically through a burr hole. The third ventricle is identified and entered as well, and an incision is made in the floor of the ventricle and enlarged as necessary with tools such as forceps or Fogarty catheters. If a membrane prevents CSF flow between the ventricle and the subarachnoid space, then an incision is made in the membrane as well. Ideally this procedure can be performed near the midline of the brain with minimal side-to-side motions of the endoscope so as to not tear tissues and cause further complications.
Research has found that this procedure has a 75% success rate, that 72% of ETV surgeries are still correctly functioning after 15 years, and that patients have shorter hospital stays recovering as compared to shunting. If the procedure does not successfully cure the aqueductal stenosis, a second surgery can be performed to enlarge the incision or implant a shunt. Problems that can lead to these failures and require additional surgery include the stoma becoming closed or a new membrane forming across the stoma over time. Currently there is no universal decision about whether this should be performed in children, as infants have a higher tendency to have a membrane form over the incision which means that an additional surgery would have to be performed.
The optimal management of laryngotracheal stenosis is not well defined, depending mainly on the type of the stenosis.
General treatment options include
1. Tracheal dilation using rigid bronchoscope
2. Laser surgery and endoluminal stenting
3. Tracheal resection and laryngotracheal reconstructionr
Tracheal is used to temporarily enlarge the airway. The effect of dilation typically lasts from a few days to 6 months. Several studies have shown that as a result of mechanical dilation (used alone) may occur a high mortality rate and a rate of recurrence of stenosis higher than 90%.
Thus, many authors treat the stenosis by endoscopic excision with laser (commonly either the carbon dioxide or the neodymium: yttrium aluminum garnet laser) and then by using bronchoscopic dilatation and prolonged stenting with a T-tube (generally in silicone).
There are differing opinions on treating with laser surgery.
In very experienced surgery centers, tracheal resection and reconstruction (anastomosis complete end-to-end with or without laryngotracheal temporary stent to prevent airway collapse) is currently the best alternative to completely cure the stenosis and allows to obtain good results. Therefore, it can be considered the gold standard treatment and is suitable for almost all patients.
The narrowed part of the trachea will be cut off and the cut ends of the trachea sewn together with sutures. For stenosis of length greater than 5 cm a stent may be required to join the sections.
Late June or early July 2010, a new potential treatment was trialed at Great Ormond Street Hospital in London, where Ciaran Finn-Lynch (aged 11) received a transplanted trachea which had been injected with stem cells harvested from his own bone marrow. The use of Ciaran's stem cells was hoped to prevent his immune system from rejecting the transplant, but there remain doubts about the operation's success, and several later attempts at similar surgery have been unsuccessful.
Potential surgical treatments include:
- Anterior cervical discectomy and fusion - A surgical treatment of nerve root or spinal cord compression by decompressing the spinal cord and nerve roots of the cervical spine with a discectomy in order to stabilize the corresponding vertebrae.
- Laminoplasty - A surgical procedure relieve pressure on the spinal cord by cutting the lamina on both sides of the affected vertebrae (cutting through on one side and merely cutting a groove on the other) and then "swinging" the freed flap of bone open.
- Laminectomy - A surgical procedure in which the lamina of the vertebra is removed or trimmed to widen the spinal canal and create more space for the spinal nerves and thecal sac.
The definitive treatment of pyloric stenosis is with surgical pyloromyotomy known as Ramstedt's procedure (dividing the muscle of the pylorus to open up the gastric outlet). This surgery can be done through a single incision (usually 3–4 cm long) or laparoscopically (through several tiny incisions), depending on the surgeon's experience and preference.
Today, the laparoscopic technique has largely supplanted the traditional open repairs which involved either a tiny circular incision around the navel or the Ramstedt procedure. Compared to the older open techniques, the complication rate is equivalent, except for a markedly lower risk of wound infection. This is now considered the standard of care at the majority of children's hospitals across the US, although some surgeons still perform the open technique. Following repair, the small 3mm incisions are hard to see.
The vertical incision, pictured and listed above, is no longer usually required, though many incisions have been horizontal in the past years.
Once the stomach can empty into the duodenum, feeding can begin again. Some vomiting may be expected during the first days after surgery as the gastrointestinal tract settles. Rarely, the myotomy procedure performed is incomplete and projectile vomiting continues, requiring repeat surgery. Pyloric stenosis generally has no long term side-effects or impact on the child's future.
Infantile pyloric stenosis is typically managed with surgery; very few cases are mild enough to be treated medically.
The danger of pyloric stenosis comes from the dehydration and electrolyte disturbance rather than the underlying problem itself. Therefore, the baby must be initially stabilized by correcting the dehydration and the abnormally high blood pH seen in combination with low chloride levels with IV fluids. This can usually be accomplished in about 24–48 hours.
Intravenous and oral atropine may be used to treat pyloric stenosis. It has a success rate of 85-89% compared to nearly 100% for pyloromyotomy, however it requires prolonged hospitalization, skilled nursing and careful follow up during treatment. It might be an alternative to surgery in children who have contraindications for anesthesia or surgery, or in children whose parents do not want surgery.
Potential non-surgical treatments include:
- Education about the course of the condition and how to relieve symptoms
- Medicines to relieve pain and inflammation, such as acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs)
- Exercise, to maintain or achieve overall good health, aerobic exercise, such as riding a stationary bicycle, which allows for a forward lean, walking, or swimming can relieve symptoms
- Weight loss, to relieve symptoms and slow progression of the stenosis
- Physical therapy, to provide education, instruction, and support for self-care; physical therapy instructs on stretching and strength exercises that may lead to a decrease in pain and other symptoms
To treat Lutembacher's syndrome, the underlying causes of the disorder must first be treated: mitral stenosis and atrial septal defect. Lutembacher's syndrome is usually treated surgically with treatments such as:
- percutaneous transcatheter therapy for MS
- Device closure of ASD
Percutaneous transcatheter treatment for the MS can include transcatheter therapies of such as balloon valuloplasty.
In terms of treatment for pulmonary valve stenosis, valve replacement or surgical repair (depending upon whether the stenosis is in the valve or vessel) may be indicated. If the valve stenosis is of congenital origin, balloon valvuloplasty is another option, depending on the case.
Valves made from animal or human tissue (are used for valve replacement), in adults metal valves can be used.
MR Imaging is best suited to evaluate patients with Shone's complex. Routine blood tests should be done prior to cardiac catheterization. The surgeons will repair the mitral valve and al the partial surgical removal of supramitral ring is done. This surgical method is preferred to the valve replacement procedure.
Classifying cardiac lesions in infants is quite difficult, and accurate diagnosis is essential. The diagnosis of Shone’s complex requires an ultrasound of the heart (echocardiogram) and a cardiac catheterization procedure, that is, insertion of a device through blood vessels in the groin to the heart that helps identify heart anatomy.
ECG leads must be placed in reversed positions on a person with dextrocardia. In addition, when defibrillating someone with dextrocardia, the pads should be placed in reverse positions. That is, instead of upper right and lower left, pads should be placed upper left and lower right.
When heart transplantation is required in a person with situs inversus, reconstruction of the venous pathways to accommodate a normal donor heart is a major, but not insurmountable, challenge.
Possible side effects from this non-invasive procedure could be:
- fever
- Chest pain
- Shortness of breath
- Unusual swelling or weight gain
- Swelling, bleeding, change in skin color at site of initial catheterization in groin, or pain in the groin.
If any of the above symptoms occur, it is important to contact your doctor to prevent another lapse of mitral stenosis. To ensure good health, routine doctors visits, diet, weight loss, doctor-approved exercise, and use of antibiotics in dental and other procedures are recommended.
It is initially treated with medications, including diuretics, and medications for blood pressure control. When high-grade renal artery stenosis is documented and blood pressure cannot be controlled with medication, or if renal function deteriorates, surgery may be resorted to. The most commonly used procedure is a minimally-invasive angioplasty with or without stenting. It is unclear if this approach yields better results than the use of medications alone. It is a relatively safe procedure. If all else fails and the kidney is thought to be worsening hypertension and revascularization with angioplasty or surgery does not work, then surgical removal of the affected kidney (nephrectomy) may significantly improve high blood pressure.
When treated early, that is, before the onset of pulmonary hypertension, a good outcome is possible in patients with Shone’s syndrome. However, other surgical methods can be employed depending upon the patient’s medical background. The single most important determinant of poor outcome during the surgical management of patients with Shone's syndrome is the degree of involvement of the mitral valve and the presence of secondary pulmonary hypertension.
If restenosis occurs without a stent, it is usually treated with more angioplasty. Once restenosis has occurred and been treated by angioplasty, the chances of restenosis occurring again are increased by a factor of 2. This treatment is also used if restenosis occurs at either the proximal or distal end of the stent.
If restenosis occurs within a stent (also known as in-stent stenosis), it may be treated with repeated angioplasty and insertion of another stent inside the original, sometimes with a drug-eluting stent.
Over the past 5 years, ISR is preferentially treated with a drug eluting balloon, which is a balloon coated with the same anticancer drugs that prevent restenosis. The Balloon avoids the need for a double layer of metal which is used when an in-stent restenosis is treated with another stent within the original stent
Alternative treatments include brachytherapy, or intracoronary radiation. The radiation kills cells and inhibits tissue growth (similar to a patient undergoing cancer therapy).
Tricuspid valve stenosis itself usually doesn't require treatment. If stenosis is mild, monitoring the condition closely suffices. However, severe stenosis, or damage to other valves in the heart, may require surgical repair or replacement.
The treatment is usually by surgery (tricuspid valve replacement) or percutaneous balloon valvuloplasty. The resultant tricuspid regurgitation from percutaneous treatment is better tolerated than the insufficiency occurring during mitral valvuloplasty.
The definitive treatment for Heyde's syndrome is surgical replacement of the aortic valve. Recently, it has been proposed that transcatheter aortic valve implantation (TAVI) can also be used for definitive management. Direct surgical treatment of the bleeding (e.g. surgical resection of the bleeding portion of the bowel) is only rarely effective.
Medical management of symptoms is possible also, although by necessity temporary, as definitive surgical management is required to bring levels of von Willebrand factor back to normal. In severe bleeding, blood transfusions and IV fluid infusions can be used to maintain blood pressure. In addition, desmopressin (DDAVP) is known to be effective in people with von Willebrand's disease, including people with valvular heart disease. Desmopressin stimulates release of von Willebrand factor from blood vessel endothelial cells by acting on the V2 receptor, which leads to decreased breakdown of Factor VIII. Desmopressin is thus sometimes used directly to treat mild to moderate acquired von Willebrand's disease and is an effective prophylactic agent for the reduction of bleeding during heart valve replacement surgery.
Angioplasty with or without stenting is the best option for the treatment of renal artery stenosis due to fibromuscular dysplasia.
Treatment is symptomatic, often addressing indicators associated with peripheral pulmonary artery stenosis. Laryngotracheal calcification resulting in dyspnea and forceful breathing can be treated with bronchodilators including the short and long-acting β2-agonists, and various anticholinergics. Prognosis is good, yet life expectancy depends on the severity and extent of diffuse pulmonary and arterial calcification.
The effectiveness of non surgical treatments is unclear as they have not been well studied.
- Education about the course of the condition and how to relieve symptoms
- Medicines to relieve pain and inflammation, such as acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs)
- Exercise, to maintain or achieve overall good health, aerobic exercise, such as riding a stationary bicycle, which allows for a forward lean, walking, or swimming can relieve symptoms
- Weight loss, to relieve symptoms and slow progression of the stenosis
- Physical therapy to support self-care. Also may give instructs on stretching and strength exercises that may lead to a decrease in pain and other symptoms.
- Lumbar epidural steroid or anesthetic injections have low quality evidence to support their use.
Treatment options are either surgical or non-surgical. Overall evidence is inconclusive whether non-surgical or surgical treatment is the better for lumbar spinal stenosis.
There is no known cure for FMD. However, treatment focuses on relieving symptoms associated with it. Medical management is the most common form of treatment. The best approach to medically managing these patients is constantly being reevaluated as more information is learned about the disease.
The evidence for the use of medical interventions for lumbar spinal stenosis is poor. Injectable but not nasal calcitonin may be useful for short term pain relief. Epidural blocks may also transiently decrease pain, but there is no evidence of long-term effect. Adding steroids to these injections does not improve the result; the use of epidural steroid injections (ESIs) is controversial and evidence of their efficacy is contradictory.
Non-steroidal anti-inflammatory drugs (NSAIDs), muscle relaxants and opioid analgesics are often used to treat low back pain, but evidence of their efficacy is lacking.
For newborns with transposition, prostaglandins can be given to keep the ductus arteriosus open which allows mixing of the otherwise isolated pulmonary and systemic circuits. Thus oxygenated blood that recirculates back to the lungs can mix with blood that circulates throughout the body. The arterial switch operation is the definitive treatment for dextro- transposition. Rarely the arterial switch is not feasible due to particular coronary artery anatomy and an atrial switch operation is preferred.
The Norwood procedure is a procedure to correct fetal aortic stenosis that occurs after birth. This typically consists of three surgeries creating and removing shunts. The atrial septum is removed, the aortic arch is reconstructed to remove any hypoplasia, and then the main pulmonary artery is connected into this reconstructed arch, resulting in the right ventricle ejecting directly into systemic circulation. In the end, the right ventricle is pumping blood to systemic circulation and to the lungs. However, this procedure carries a very high risk of failure and the patient will likely require a heart transplant.
Another treatment option is to correct the stenosis in utero. In this procedure, fetal positioning is crucial. It is important that the left chest is located anteriorly, and that there are no limbs between the uterine wall and the apex of the left ventricle. The LV apex needs to be within 9 cm of the abdominal wall and the left ventricle outflow track has to be parallel to the intended cannula course in order for the wire to be blindly directed at the aortic valve. A 11.5 cm long, 19-gauge cannula and stylet needle passes through the mother’s abdomen, uterine wall, and fetal chest wall into the left ventricle of the fetus. Then a 0.014 inch guide wire is passed across the stenosis aortic valve, where a balloon is inflated to stretch the aortic annulus.
An alternative to the Norwood procedure is known as the hybrid procedure, was developed in 2008. In the hybrid procedure, bilateral pulmonary artery bands are positioned to limit pulmonary flow while, at the same time, placing a stent in the ductus arteriosus to hold it open. This maintains the connection between the aorta and the systemic circulation. A balloon atrial septostomy is also done. This ensures that there is enough of a connection between the two atria of the heart to provide open blood flow and mixing of oxygen rich and poor blood This procedure spares the baby from undergoing open heart surgery until they are older. They typically come back at 4–6 months of age when they are stronger for the open heart surgery.