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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.
The procedure is performed in general anesthesia. It is useful to place pulse oximeter probes on "both hands" and "one foot" so that test occlusion of one arch or its branches will allow confirmation of the anatomy. In addition blood pressure cuffs should also be placed on one leg and both arms to confirm the absence of a pressure gradient when the intended point of division of the lesser arch is temporarily occluded with forceps.
After the surgery, some patients require intubation and mechanical ventilation for several days to allow adequate tracheal toilet, but most patients can have the tubes removed soon after the surgery. The obstructive airway symptoms may be worse in the first postoperative weeks. Only a few patients have immediate relief of stridor, but many obtain immediate relief of problems with swallowing (dysphagia). After extubation, it might be necessary to maintain positive airway pressure by appropriate flows of a humidified oxygen/air mixture.
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.
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.
Surgical treatment involves resection of the stenosed segment and re-anastomsis. Two complications specific to this surgery are Left recurrent nerve palsy and chylothorax, as the recurrent laryngeal nerve and thoracic duct are in the vicinity. Chylothorax is a troublesome complication and is usually managed conservatively by adjusting the diet to eliminate long chain fatty acids and supplementing medium chain triglycerides. When conservative management fails surgical intervention is required. Fluorescein dye can aid in the localisation of chyle leak.
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.
Treatments for the condition vary depending on its severity. The most immediate and effective treatment in the majority of cases is a surgical repair to close the fistula/s and reconnect the two ends of the esophagus to each other. Although this is usually done through an incision between the ribs on right side of the baby, a technique using three small incisions (thoracoscopy) is being used at some centers. In a minority of cases, the gap between upper and lower esophageal segments may be too long to bridge. In some of these so-called long gap cases, though, an advanced surgical treatment developed by John Foker, MD, may be utilized to elongate and then join together the short esophageal segments. Using the Foker technique, surgeons place traction sutures in the tiny esophageal ends and increase the tension on these sutures daily until the ends are close enough to be sewn together. The result is a normally functioning esophagus, virtually indistinguishable from one congenitally well formed. Unfortunately, the results have been somewhat difficult to replicate by other surgeons and the need for multiple operations has tempered enthusiasm for this approach.
The optimal treatment in cases of long gap esophageal atresia remains controversial. Traditional surgical approaches include gastrostomy followed by gastric pull-up, colonic transposition and jejunum transposition. Gastric pull-up has been the preferred approach at many specialized centers, including Great Ormond Street (London) and Mott Children's Hospital (Ann Arbor). Gastrostomy, or G-tube, allows for tube feedings into the stomach through the abdominal wall. Often a cervical esophagostomy will also be done, to allow the saliva which is swallowed to drain out a hole in the neck. Months or years later, the esophagus may be repaired, sometimes by using a segment of bowel brought up into the chest, interposing between the upper and lower segments of esophagus.
Post operative complications sometimes arise, including a leak at the site of closure of the esophagus. Sometimes a stricture, or tight spot, will develop in the esophagus, making it difficult to swallow. This can usually be dilated using medical instruments. In later life, most children with this disorder will have some trouble with either swallowing or heartburn or both. Esophageal dismotility occurs in 75-100% of patients.
Tracheomalacia—a softening of the trachea, usually above the carina (carina of trachea), but sometimes extensive in the lower bronchial tree as well—is another possible serious complication. Even after esophageal repair (anastomosis) the relative flaccidity of former proximal pouch (blind pouch, above) along with esophageal dysmotility can cause fluid buildup during feeding. Owing to proximity, pouch ballooning can cause tracheal occlusion. Severe hypoxia ("dying spells") follows and medical intervention can often be required.
A variety of treatments for tracheomalacia associated with esophageal atresia are available. If not severe, the condition can be managed expectantly since the trachea will usually stiffen as the infant matures into the first year of life. When only the trachea above the carina is compromised, one of the "simplest" interventions is aortopexy wherein the aortic loop is attached to the rear of the sternum, thereby mechanically relieving pressure from the softened trachea. An even simpler intervention is stenting. However, epithelial cell proliferation and potential incorporation of the stent into the trachea can make subsequent removal dangerous.
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.
Treatment of a laryngeal cleft depends on the length and resulting severity of symptoms. A shallow cleft (Type I) may not require surgical intervention. Symptoms may be able to be managed by thickening the infant's feeds. If symptomatic, Type I clefts can be sutured closed or injected with filler as a temporary fix to determine if obliterating the cleft is beneficial and whether or not a more formal closure is required at a later date. Slightly longer clefts (Type II and short Type III) can be repaired endoscopically. Short type IV clefts extending to within 5 mm below the innominate artery can be repaired through the neck by splitting the trachea vertically in the midline and suturing the back layers of the esophagus and trachea closed. A long, tapered piece of rib graft can be placed between the esophageal and tracheal layers to make them rigid so the patient will not require a tracheotomy after the surgery and to decrease chances of fistula postoperatively. Long Type IV clefts extending further than 5 mm below the innominate artery cannot be reached with a vertical incision in the trachea, and therefore are best repaired through cricotracheal resection. This involves separating the trachea from the cricoid cartilage, leaving the patient intubated through the trachea, suturing each of the esophagus and the back wall of the trachea closed independently, and then reattaching the trachea to the cricoid cartilage. This prevents the need for pulmonary bypass or extracorporeal membrane oxygenation.
In adults and children found to have coarctation, treatment is conservative if asymptomatic, but may require surgical resection of the narrow segment if there is arterial hypertension. The first operations to treat coarctation were carried out by Clarence Crafoord in Sweden in 1944. In some cases angioplasty can be performed to dilate the narrowed artery, with or without the placement of a stent graft.
For fetuses at high risk for developing coarctation, a novel experimental treatment approach is being investigated, wherein the mother inhales 45% oxygen three times a day (3 x 3–4 hours) beyond 34 weeks of gestation. The oxygen is transferred via the placenta to the fetus and results in dilatation of the fetal lung vessels. As a consequence, the flow of blood through the fetal circulatory system increases, including that through the underdeveloped arch. In suitable fetuses, marked increases in aortic arch dimensions have been observed over treatment periods of about two to three weeks.
The long term outcome is very good. Some patients may, however, develop narrowing (stenosis) or dilatation at the previous coarctation site. All patients with unrepaired or repaired aortic coarctation require follow up in specialized Congenital Heart Disease centers.
Tet spells may be treated with beta-blockers such as propranolol, but acute episodes require rapid intervention with morphine or intranasal fentanyl to reduce ventilatory drive, a vasopressor such as phenylephrine, or norepinephrine to increase systemic vascular resistance, and IV fluids for volume expansion.
Oxygen (100%) may be effective in treating spells because it is a potent pulmonary vasodilator and systemic vasoconstrictor. This allows more blood flow to the lungs by decreasing shunting of deoxygenated blood from the right to left ventricle through the VSD. There are also simple procedures such as squatting and the knee chest position which increase systemic vascular resistance and decrease right-to-left shunting of deoxygenated blood into the systemic circulation.
The formation of a TIF is a medical emergency and requires immediate intervention. Blood volume control, management of the hemorrhage, and adequate oxygenation should be insured in these patients. In a majority of TIF cases (85%), hyperinflation of the tracheostomy cuff will control the bleeding, while the patient is prepared for surgery. However, if this fails the tracheostomy cuff must be removed, and the patient must be intubated from above. Next, pressure from the index finger can be applied on the bleeding site from within the tracheostomy to control the bleeding. In addition, the "Utley Maneuver", which involves digital compression of the artery against the posterior wall of the manubrium of the sternum following a right infraclavicular incision, may be used to urgently control the bleeding When the bleeding is controlled the patient should be immediately transferred on the operating room.
An extracranial shunt is essentially a sturdy tube with a catheter on one end to drain the third ventricle. The shunt also has a valve which serves to maintain one-way flow of the CSF and regulates the flow rate. The end with the catheter is placed in the third ventricle to drain the excess CSF and the other end is placed in the peritoneal cavity or atrium of the heart (making it a ventriculoperitoneal or ventriculoatrial shunt, respectively). The excess CSF which is diverted to a cavity is then reabsorbed by the surrounding tissue where it is drained to.
The procedure to insert this device is a technically straightforward endoscopic surgery with a low mortality rate (essentially 0% mortality since the 1970s). If the shunt has an adjustable valve the current method of setting the valve pressure is to choose one setting, observe the patient to see if CSF flow improves and the symptoms lessen over time, and adjust the pressure setting as needed if improvement isn't seen. For example, if there is not enough CSF flow, another surgery is performed to lower the valve pressure so that less force needs to be applied to open the valve and thereby drain more CSF.
This treatment method has several possible problems with it (with a 50% failure rate in 2 years), and unfortunately shunt malfunctions and associated complications cause a death rate of 1.2% per year. Problems which can necessitate a secondary surgery to fix them include: mechanical failure, incorrect catheter size, inappropriate valve drainage pressure, and infection.
- Inappropriate valve pressure can lead to "overdraining" or "underdraining", both of which should be treated by adjusting the valve pressure. Overdraining occurs when the valve pressure is too low and CSF flows out of the third ventricle too quickly. The ventricle then collapses and blood vessles can be torn in the process. This in turn can lead to headache, hemorrhage, or slit ventricle syndrome. Underdraining occurs when the valve pressure is too high and CSF flows out too slowly. This results in symptoms of hydrocephalus as the CSF is still collecting rather than being absorbed or diverted.
- Risk of infection is due to the fact that a foreign object is being introduced into the body. Infection can have symptoms of fever and soreness of the neck and shoulders.
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.
The Blalock-Thomas-Taussig procedure, initially the only surgical treatment available for tetralogy of Fallot, was palliative but not curative. The first total repair of tetralogy of Fallot was done by a team led by C. Walton Lillehei at the University of Minnesota in 1954 on an 11-year-old boy. Total repair on infants has had success from 1981, with research indicating that it has a comparatively low mortality rate.
Total repair of tetralogy of Fallot initially carried a high mortality risk, but this risk has gone down steadily over the years. Surgery is now often carried out in infants one year of age or younger with less than 5% perioperative mortality. The open-heart surgery is designed to relieve the right ventricular outflow tract stenosis by careful resection of muscle and to repair the VSD with a Gore-Tex patch or a homograft.> Additional reparative or reconstructive surgery may be done on patients as required by their particular cardiac anatomy.
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.
Treatment is not necessary in asymptomatic patients.
The treatment options for mitral stenosis include medical management, mitral valve replacement by surgery, and percutaneous mitral valvuloplasty by balloon catheter.
The indication for invasive treatment with either a mitral valve replacement or valvuloplasty is NYHA functional class III or IV symptoms.
Another option is balloon dilatation. To determine which patients would benefit from percutaneous balloon mitral valvuloplasty, a scoring system has been developed. Scoring is based on 4 echocardiographic criteria: leaflet mobility, leaflet thickening, subvalvar thickening, and calcification. Individuals with a score of ≥ 8 tended to have suboptimal results. Superb results with valvotomy are seen in individuals with a crisp opening snap, score < 8, and no calcium in the commissures.
Treatment also focuses on concomitant conditions often seen in mitral stenosis:
- Any angina is treated with short-acting nitrovasodilators, beta-blockers and/or calcium blockers
- Any hypertension is treated aggressively, but caution must be taken in administering beta-blockers
- Any heart failure is treated with digoxin, diuretics, nitrovasodilators and, if not contraindicated, cautious inpatient administration of ACE inhibitors
Acute decompensated heart failure due to AS may be temporarily managed by an intra-aortic balloon pump while pending surgery. In those with high blood pressure nitroprusside may be carefully used. Phenylephrine may be used in those with very low blood pressure.
Percutaneous transcatheter therapy is used to repair the mitral valve and sometimes the septum. In percutaneous balloon mitral valvuloplasty, using a catheter, a ballon such as the Inoue ballon is placed into blood vessels in the groin area and the balloon guided to the heart. If a hole is not already present, a small hole may need to be inserted the atria and inserted into the mitral valve through the left atrium; the balloon is then inflated. The balloon inside the mitral valve will be inflated and deflated several times to wide the valve opening until the opening is satisfactory; the balloon will then be deflated and removed.
The advantage to using percutaneous procedures instead of open-heart surgery is not needing general anesthesia, blood transfusions, and the recovery time is quicker. The drawback to this procedure is the lack of repeating and transseptal procedures if they are needed later. Also if the patient later develops a relapse of MS, surgery will need to be performed where using more evasive techniques. Additionally, if a hole is needed to be inserted into the atria to obtain access to the mitral valve, there is a risk of developing ASD secondarily.
For infants and children, balloon valvuloplasty, where a balloon is inflated to stretch the valve and allow greater flow, may also be effective. In adults, however, it is generally ineffective, as the valve tends to return to a stenosed state. The surgeon will make a small incision at the top of the person's leg and proceed to insert the balloon into the artery. The balloon is then advanced up to the valve and is inflated to stretch the valve open.
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
Sometimes CHD improves without treatment. Other defects are so small that they do not require any treatment. Most of the time CHD is serious and requires surgery and/or medications. Medications include diuretics, which aid the body in eliminating water, salts, and digoxin for strengthening the contraction of the heart. This slows the heartbeat and removes some fluid from tissues. Some defects require surgical procedures to restore circulation back to normal and in some cases, multiple surgeries are needed.
Interventional cardiology now offers patients minimally invasive alternatives to surgery for some patients. The Melody Transcatheter Pulmonary Valve (TPV), approved in Europe in 2006 and in the U.S. in 2010 under a Humanitarian Device Exemption (HDE), is designed to treat congenital heart disease patients with a dysfunctional conduit in their right ventricular outflow tract (RVOT). The RVOT is the connection between the heart and lungs; once blood reaches the lungs, it is enriched with oxygen before being pumped to the rest of the body. Transcatheter pulmonary valve technology provides a less-invasive means to extend the life of a failed RVOT conduit and is designed to allow physicians to deliver a replacement pulmonary valve via a catheter through the patient’s blood vessels.
Most patients require lifelong specialized cardiac care, first with a pediatric cardiologist and later with an adult congenital cardiologist. There are more than 1.8 million adults living with congenital heart defects.
To prevent an TIF, intubation time should be limited to less than 2 weeks and proper techniques should be used when performing tracheotomies. The occurrence of an TIF can be reduced by using more flexible and blunt tracheostomy tubes and insuring that the tubes are properly aligned in the patients. Placing the tracheostomy between the second and third tracheal rings can minimize the risk of an TIF. Repetitive head movements, especially, hyperextension of the neck should be avoided as since this movement results in contact between the innominate artery and the underside of the tube.
Mitral valvuloplasty is a minimally invasive therapeutic procedure to correct an uncomplicated mitral stenosis by dilating the valve using a balloon.
Under local anaesthetic, a catheter with a special balloon is passed from the right femoral vein, up the inferior vena cava and into the right atrium. The interatrial septum is punctured and the catheter passed into the left atrium using a "trans-septal technique." The balloon is sub-divided into 3 segments and is dilated in 3 stages. First, the distal portion (lying in the left ventricle) is inflated and pulled against the valve cusps. Second, the proximal portion is dilated, in order to fix the centre segment at the valve orifice. Finally, the central section is inflated, this should take no longer than 30 seconds, since full inflation obstructs the valve and causes congestion, leading to circulatory arrest and flash pulmonary edema.
With careful patient pre-selection, percutaneous balloon mitral valvuloplasty (PBMV) is associated with good success rates and a low rate of complications. By far the most serious adverse event is the occurrence of acute severe mitral regurgitation. Severe mitral regurgitation usually results from a tear in one of the valve leaflets or the subvalvular apparatus. It can lead to pulmonary edema and hemodynamic compromise, necessitating urgent surgical mitral valve replacement.
Other serious complications with PBMV usually relate to the technique of trans-septal puncture (TSP). The ideal site for TSP is the region of the fossa ovalis in the inter-atrial septum. Occasionally, however, the sharp needle used for TSP may inadvertently traumatize other cardiac structures, leading to cardiac tamponade or serious blood loss.
Although the immediate results of PBMV are often quite gratifying, the procedure does not provide permanent relief from mitral stenosis. Regular follow-up is mandatory, to detect restenosis. Long-term follow-up data from patients undergoing PBMV indicates that up to 70-75% individuals can be free of restenosis 10 years following the procedure. The number falls to about 40% 15 years post-PBMV.