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Treatment depends on the anatomy of the malformation as determined by angiography or Magnetic Resonance Imaging (MRI).
This is based on MRI scan, magnetic resonance angiography and CT scan. A cerebral digital subtraction angiography (DSA) enhances visualization of the fistula.
- CT scans classically show an enlarged superior ophthalmic vein, cavernous sinus enlargement ipsilateral (same side) as the abnormality and possibly diffuse enlargement of all the extraocular muscles resulting from venous engorgement.
- Selective arteriography is used to evaluate arteriovenous fistulas.
- High resolution digital subtraction angiography may help in classifying CCF into dural and direct type and thus formulate a strategy to treat it either by a balloon or coil or both with or without preservation of parent ipsilateral carotid artery.
Cerebral angiography is the diagnostic standard. MRIs are usually normal.
Testing for a malformed vein of Galen is indicated when a patient has heart failure which has no obvious cause. Diagnosis is generally achieved by signs such as cranial bruits and symptoms such as expanded facial veins. The vein of Galen can be visualized using ultrasound or Doppler. A malformed Great Cerebral Vein will be noticeably enlarged. Ultrasound is a particularly useful tool for vein of Galen malformations because so many cases occur in infancy and ultrasound can make diagnoses prenatally. Many cases are diagnosed only during autopsy as congestive heart failure occurs very early.
The Cognard et al. Classification correlates venous drainage patterns with increasingly aggressive neurological clinical course.
The mainstay of treatment for CCF is endovascular therapy. This may be transarterial (mostly in the case of direct CCF) or transvenous (most commonly in indirect CCF). Occasionally, more direct approaches, such as direct transorbital puncture of the cavernous sinus or cannulation of the draining superior orbital vein are used when conventional approaches are not possible. Spontaneous resolution of indirect fistulae has been reported but is uncommon. Staged manual compression of the ipsilateral carotid has been reported to assist with spontaneous closure in selected cases.
Direct CCF may be treated by occlusion of the affected cavernous sinus (coils, balloon, liquid agents), or by reconstruction of the damaged internal carotid artery (stent, coils or liquid agents).
Indirect CCF may be treated by occlusion of the affected cavernous sinus with coils, liquid agents or a combination of both.
The radiocephalic arteriovenous fistula (RC-AVF) is a shortcut between cephalic vein and radial artery at the wrist. It is the recommended first choice for hemodialysis access. Possible underlying causes for failure are stenosis and thrombosis especially in diabetics and those with low blood flow such as due to narrow vessels, arteriosclerosis and advanced age. Reported patency of fistulae after 1 year is about 62.5%.
The procedure was invented by doctors James Cimino and M. J. Brescia in 1966. Before the Cimino fistula was invented, access was through a Scribner shunt, which consisted of a Teflon tube with a needle at each end. Between treatments, the needles were left in place and the tube allowed blood flow to reduce clotting. But Scribner shunts lasted only a few days to weeks. Frustrated by this limitation, James E. Cimino recalled his days as a phlebotomist (blood drawer) at New York City's Bellevue Hospital in the 1950s when Korean War veterans showed up with fistulas caused by trauma. Cimino recognized that these fistulas did not cause the patients harm and were easy places to get repeated blood samples. He convinced surgeon Kenneth Appell to create some in patients with chronic kidney failure and the result was a complete success. Scribner shunts were quickly replaced with Cimino fistulas, and they remain the most effective, longest-lasting method for long-term access to patients' blood for hemodialysis today.
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.
The treatment of arterial tortuosity syndrome entails possible surgery for aortic aneurysms, as well as, follow ups which should consist of EGC. The prognosis of this condition has it at about 12% mortality
Treatment for fistula varies depending on the cause and extent of the fistula, but often involves surgical intervention combined with antibiotic therapy.
Typically the first step in treating a fistula is an examination by a doctor to determine the extent and "path" that the fistula takes through the tissue.
In some cases the fistula is temporarily covered, for example a fistula caused by cleft palate is often treated with a palatal obturator to delay the need for surgery to a more appropriate age.
Surgery is often required to assure adequate drainage of the fistula (so that pus may escape without forming an abscess). Various surgical procedures are commonly used, most commonly fistulotomy, placement of a seton (a cord that is passed through the path of the fistula to keep it open for draining), or an endorectal flap procedure (where healthy tissue is pulled over the internal side of the fistula to keep feces or other material from reinfecting the channel). Treatment involves filling the fistula with fibrin glue; also plugging it with plugs made of porcine small intestine submucosa have also been explored in recent years, with variable success. Surgery for anorectal fistulae is not without side effects, including recurrence, reinfection, and incontinence. High rate of recurrence and more chances of complications like incontinence are always there in fistula surgeries (Anal Fistula).
It is important to note that surgical treatment of a fistula without diagnosis or management of the underlying condition, if any, is not recommended. For example, surgical treatment of fistulae in Crohn's disease can be effective, but if the Crohn's disease itself is not treated, the rate of recurrence of fistula is very high (well above 50%).
In terms of the diagnosis of arterial tortuosity syndrome can be done via genetic testing, as well as the following listed below:
- CT
- MRI
- Echocardiogram
- Physical exam(for specific characteristics)
"Diagnosis" is by examination, either in an outpatient setting or under anaesthesia (referred to as — Examination Under Anaesthesia). The fistula may be explored by using a fistula probe (a narrow instrument). In this way, it may be possible to find both openings. The examination can be an anoscopy. Diagnosis may be aided by performing a fistulogram, proctoscopy and/or sigmoidoscopy.
Possible findings:
- The opening of the fistula onto the skin may be observed
- The area may be painful on examination
- There may be redness
- An area of induration may be felt — thickening due to chronic infection
- A discharge may be seen
CLASSIFICATIONS of ANAL FISTULA
- Park's Classification: This was done in 1976 by Parks et al from UK. This was done in the era when MRI or Endoanal Ultrasound was not there. It classified the fistula in four grades
- St James University Hospital Classification: This was done by Morris et al in the year 2000. This classification was improvement over Parks classification as it was based on MRI studies. It classified the fistula in five grades.
- Garg Classification: This was done by Pankaj Garg in 2017. This classification is improvement over both Parks and St James University Hospital Classification. This was based on MRI studies and operative findings in 440 patients. It classified the fistula in five grades. The grades of this classification correlate quite well with the severity of the disease. Grade I & II are simpler fistulas and can be managed by Fistulotomy whereas grade III-V are complex fistulas in which fistulotomy should be not be done. They should be managed by Fistula experts. Unlike Park's and St James University Hospital Classification, this correlation is quite accurate with Garg's classification. Therefore this new classification is useful to both surgeons and radiologists
TIF is a rare condition with a .7% frequency, and an mortality rate approaching 100% without surgical intervention. Immediate diagnosis and intervention of an TIF is critical for the surgical intervention success. 25-30% of TIF patients who reach the operating room survive. Recently, the incidence of TIF may have declined due to advances in tracheostomy tube technology and the introduction of the bedside percutaneous dilatational tracheostomy (PDT).
Various types of fistulas include:
Although most fistulas are in forms of a tube, some can also have multiple branches.
Smith (2015) conducted a study that looked into specific biological markers that correlate to Moyamoya disease. Some of the categories of these biomarkers include phenotypes - conditions commonly related to Moyamoya, radiographical markers for the diagnosis of Moyamoya, and proteins as well as cellular changes that occur in cases of Moyamoya.
Similar to Moyamoya Disease, there are conditions that are closely associated with Moyamoya Syndrome. Some of the more common medical conditions that are closely associated with Moyamoya Syndrome include trisomy 21 (Down's Syndrome), sickle cell disease, and neurofibromatosis type 1. There is also evidence that identifies hyperthyroidism and congenital dwarfing syndromes as two of the more loosely associated syndromes that correlate with the possibility of being diagnosed with Moyamoya Disease later in life.
There is also research that has shown that certain radiographic biomarkers that lead to the diagnosis of Moyamoya Disease have been identified. The specific radiographic markers are now considered an acceptable key component to Moyamoya Disease and have been added to the INternational Classification of Diseases (ICD). These biomarkers of Moyamoya are "stenosis of the distal ICA's up to and including the bifurcation, along with segments of the proximal ACA and MCA...dilated basal collateral vessels must be present" Some other common findings that have not been added to the classification index of those with Moyamoya Disease which are found using radiography involve very distinct changes in the vessels of the brain. These changes include newly formed vessels made to compensate for another change noted, ischemia and cerebrovascular reserve, both found on MRI. Functional changes include evidence of ischemia in vessels of the brain (ICA, ACA, MCA, specifically). It is important to also note that the radiographic biomarkers, in order to be classified as Moyamoya Disease, all findings must be bilateral. If this is not the case and the findings are unilateral, it is diagnosed as Moyamoya Syndrome.
There are also several protein biomarkers that have been linked to the Moyamoya Disease diagnosis. Although the sample size of the studies performed are small due to the rarity of the disease, the findings are indicative of a correlation between the disease and several specific protein biomarkers. Other studies have confirmed the correlation of Moyamoya and adhesion molecule 1 (ICAM-1) being increased as compared to normal vascular function counterparts Furthermore, it has been concluded that the localization of inflammatory cells suggests that the inflammation stimulus iteself may be responsible for the proliferation and occlusion in the ICA, ACA, and MCA found in those with Moyamoya Disease.
Other conditions in which infected perianal "holes" or openings may appear include Pilonidal cysts/sinuses.
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.
Just like berry aneurysm, an intracerebral arteriovenous fistula can rupture causing subarachnoid hemorrhage.
Clinical features can be found in the subhyoid portion of the tract and 75% present as midline swellings. The remainder can be found as far lateral as the lateral tip of the hyoid bone.
Typically, the cyst will move upwards on protrusion of the tongue, given its attachment to the embryonic duct, as well as on swallowing, due to attachment of the tract to the foramen caecum.
The natural history of this disorder is not well known. The long term outlook for patients with treated moyamoya seems to be good. While symptoms may seem to improve almost immediately after the in-direct EDAS, EMS, and multiple burr holes surgeries, it will take probably 6–12 months before new vessels can develop to give a sufficient blood supply. With the direct STA-MCA surgery, increased blood supply is immediate.
Once major stroke or bleeding take place, even with treatment, the patient may be left with permanent loss of function so it is very important to treat this condition promptly.
Dr. Michael Scott, MD discusses the success rate for Moyamoya surgery in
It is surgically corrected, with resection of any fistula and anastomosis of any discontinuous segments.
A cirsoid aneurysm is the dilation of a group of blood vessels due to congenital malformations with AV (arterio venous) shunting. Cirsoid means resembling a varix.
Sometimes, a minor traumatic episode, such as a fall or bump on the head, can lead to the formation of a cirsoid aneurysm. Often these are trivial traumatic episodes
Cirsoid aneurysm, in general, is a hemangioma of an artery. It most commonly occurs over the head, usually the superficial temporal artery and also its branches. It can also occur in places where medium vessels lie over bones without much intervening tissues between them and the skin.
The superficial temporal artery is the most commonly involved artery.
This condition takes several different forms, often involving one or more fistulas connecting the trachea to the esophagus (tracheoesophageal fistula).
When an infant is born with an anorectal malformation, it is usually detected quickly as it is a very obvious defect. Doctors will then determine the type of birth defect the child was born with and whether or not there are any associated malformations. It is important to determine the presence of any associated defects during the newborn period in order to treat them early and avoid further sequelae. There are two main categories of anorectal malformations: those that require a protective colostomy and those that do not. The decision to open a colostomy is usually taken within the first 24 hours of birth.
Sonography can be used to determine the type of imperforate anus.