Branchial Cleft Anomalies

230px-Bilateral_Branchial_Cleft_Sinus_fistulography_rightA branchial cleft cyst is a congenital epithelial cyst that arises on the lateral part of the neck due to failure of obliteration of the second branchial cleft (or failure of fusion of the second and third branchial arches) in embryonic development.
The cyst wall is composed of either squamous or columnar cells with lymphoid infiltrate, often with prominent germinal centers. The cyst may contain granular and keratinaceous cellular debris. Cholesterol crystals may be found in the fluid extracted from a branchial cyst.  If the cyst connects to the either the skin on the outside or mucous membrane on the inside it is called a sinus.  If it connects to both it is called a fistula.

Pathophysiology
Branchial cleft cysts are remnants of embryonic development and result from a failure of obliteration of one of the branchial clefts, which in fish develop into gills.

Types
First branchial cleft fistulae typically originate in the angle of the mandible and extend to the external auditory canal. They are often associated with the facial nerve. Rare ~5%.

Second branchial cleft fistulae are most common (~95%). They are found along the anterior border of the Sternocleidomastoid muscle, pass through the carotid bifurcation and into the tonsillar pillar.

Third and fourth branchial cleft fistulae are rare. The external opening occurs about 2/3 of the way down the SCM anteriorly, similar to second branchial cleft cysts. The tract ascends along the carotid sheath posteriorly to the internal carotid artery, under the glossopharyngeal nerve, and over the vagus nerve and hypoglossal nerve to open into the piriform sinus.

Symptoms
Most branchial cleft fistulae are asymptomatic, but they may become infected. The cyst, however, usually presents as a smooth, slowly enlarging lateral neck mass that may increase in size after an upper respiratory tract infection.

Treatment
branchial_cleft_sinus_surgery_200x200Bilateral branchial cleft sinuses during surgery. Conservative (i.e. no treatment), or surgical excision. As complete surgical excision may be difficult (due to the close proximity of the internal jugular vein and carotid vessels that lie deep to the swelling), they can recur.

Frontal Sinus Surgery

©2004, The New York Eye and Ear Infirmarydefault

Frontal sinusotomy is a broad term which encompasses both external and intranasal approaches to open and/or create a permanent communication from the sinus to the nose.

Endoscopic frontal sinusotomy is defined as creating a permanent opening from the frontal sinus into the nose. This simple definition includes a range from relatively simple to highly complex procedures (Weber, 2001). The complexity of endoscopic frontal sinusotomy is determined by the site of obstruction to the outflow tract or disease within the sinus, and variations in frontal and ethmoid sinus anatomy.

Nose Bleed (Epistaxis)

images-1I think most of us ENTs (in independent practice) would open our offices after hours for a colleague with a nosebleed. I’d much rather treat a nosebleed in my office than the ED (no offense meant toward my ED colleagues). I have most of the instruments and lighting that I would need there, ready to go. Definitely would favor an ED rather than a UCC.

Some “anterior” nosebleeds can bleed like stink. This is because bleeding is from a higher pressure arteriole. If you have Afrin or Neosynephrine at home (and it’s actively bleeding), I would advise using (after blowing to evacuate clots, as clot begets bleeding by depleting the clotting factors). Then firmly pinch the lower collapsible soft part of the nose (thumb and finger, tongue blades, clothes pin, I’ve even seen patients use a “chip clip”) for 10-15 minutes BY THE CLOCK. (Remember normal Ivy bleeding time is 3-10 minutes — but bleeding times are sadly no longer being done most places.)

If you have a bit of knowledge of internal nasal anatomy, you could try wetting an appropriate sized piece of cotton with decongestant and carefully slipping it into the nostril before pinching the nose.  Keep the head elevated. Pressure on the upper lip below the nose can decrease the blood pressure in anterior septal arterioles. Ice on the forehead and/or back of the neck might help.  If you are in the ER, use their LET Solution on a cotton ball—has the topical 1:1000 epi and is incredibly effective at constricting the offending arterioles.

Finally, and I’ve brought this up on other threads before, if you have some bacon (and no religious objections) you could cut off a generous portion (as fatty as possible) and gently stuff it snuggly into the nostril before going to the ED. Pork fat has a water soluble compound that promotes platelet adhesion (making them “super platelets”) and I have used salt pork with a number of patients on anti-coagulants as well as with thrombocytopenia. It has not failed me yet — this is after other, more “traditional” methods have failed.

Try to avoid sticking dry or abrasive materials into the nose (e.g. Kleenex, toilet paper, or even dry cotton). To prevent future bleeds I recommend: humidification, avoidance of NSAIDs (especially Toradol.

Take 250-500mg Vitamin C daily (promotes hydroxylation of proline and collagen formation).

Ameloblastoma

images-4Ameloblastoma is a rare, noncancerous (benign) tumor that develops most often in the jaw near the molars. Ameloblastoma begins in the cells that form the protective enamel lining on your teeth.

images-2Ameloblastoma occurs in men more often than it occurs in women. Though it can be diagnosed at any age, ameloblastoma is most often diagnosed in adults in their 40s or 50s.

images-3Despite being benign, ameloblastoma can be very aggressive, growing into the jawbone and causing swelling and pain. Very rarely, ameloblastoma cells can spread to other areas of the body, such as the lymph nodes in the neck and the lungs.

Ameloblastoma is the most common type of odontogenic lesion.

RRP/HPV

gardasil_logo_tcm1908-197175There are over 200 known serotypes of HPV. About 79 million Americans are currently infected with HPV. About 7% of adults have HPV in their oral cavity. Only about 3,000 to 13,000 patients in the United States have Recurrent Respiratory Papilllomatosis. Virtually all of them have active HPV in thier oral cavity. These patients are afflicted with HPV 6 and 11, which affects primarily mucous membranes. It is looking more and more like oral to oral transmission trumps oral genital transmission. It seems to be a different animal all together than cervical HPV. Serotypes 16 and 18 are the types associated with carcinogenesis. There currently is no evidence that spouses should be vaccinated and it does not really seem to be easily transmitted between adults.PRIMARILY HPV 6 & 11 (RARELY TYPE 16 & 18).

This disease has become epidemic, however the treatments and the preventative measures are also gaining momentum.  HPV is the whole reason we really did PAP smears.  The viral changes led to cervical uterine dysplasian and even carcinogenesis.  It was even suggested to consider C-section in women with active infections.

images-1Recurrent Laryngeal RRP may require multiple surgeries.  It seems the best initial response is with the CO2 Laser, however use of the microdebrider (PIPE) may actually give better voice outcomes

Adjuvant therapies such as Acyclovir, MTX, Ribavarin, Mumps vaccine, PPI, Alpha Interferon, Hsp E7, Retinoids, and Intralesional Cidofovir have been touted, but lack strong prospective efficacy studies.  Typically I use the CO2 laser.  Have patients eat green leafy vegetables (Indole-3-carbinols) modulates estrogen metabolism and 1/3 of patients respond.  Inject about 75 mg of Cidofovir in 1 cc after the resection.  I may consider placing on Celebrex (Cox 2 inhibitor) which modulates the over expression of epidermal growth factor by inhibiting cyclooxygenase-2 and prostaglandin E2.   I also may consider injecting Avastin (Bevacizumab) recombinant monoclonal antibody against vascular endothelial growth factor.

Alpha-gal (Meat) Allergy

Alpha-gal allergies are a reaction to Galactose-alpha-1,3-galactose, whereby the body is overloaded with immunoglobulin E antibodies on contact with the carbohydrate. Alpha-gal is found in all mammals apart from primates (including humans). Bites from the lone star tick, which transfer this carbohydrate to the victim, have been implicated in the development of this delayed allergic response which is triggered by the consumption of mammalian meat products.[1] Despite myths to the contrary, an alpha-gal allergy does not require the afflicted to become a vegetarian, as poultry and fish do not trigger a reaction.

The allergy most often occurs in the central and southern United States, which corresponds to the distribution of the lone star tick.[3] In the Southern United States, where the tick is most prevalent, allergy rates are 32% higher than elsewhere.[4] However, as doctors are not required to report the number of patients suffering the alpha-gal allergies, the true number of affected individuals is unknown.

Allergy

The allergy was first formally identified as originating from tick bites in a 2007 paper by Sheryl van Nunen. Prior to the paper’s publication, Thomas Platts-Mills and Scott Commins, were attempting to discover why some patients were reacting negatively to the carbohydrate in the cancer drug Cetuximab.[6][7] They had previously hypothesized that a fungal infection or parasite could lead to the allergy.[6][8] It wasn’t until Platts-Mills was bitten by a tick and developed alpha-gal allergies, that his team also came to the conclusion that there was a link between tick bites and the allergy.[8]

Alpha-gal allergies are very similar to Pork-Cat Syndrome and hence misidentification can occur.[9]

Cause

Alpha-gal allergies develop after a person has been bitten by the Lone Star Tick in the United States, the European Castor Bean Tick, and the Paralysis Tick in Australia.[6][7] Alpha-gal is not naturally present in apes (including humans), but is in all other mammals. If a tick feeds on another mammal, the alpha-gal will remain in its alimentary tract.[2] The tick will then inject the alpha-gal into a person’s skin, which in turn will cause the immune system to release a flood of immunoglobulin E antibodies (a.k.a. IgE) to fight off the foreign carbohydrate.[2][6] Researchers still do not know which specific component of tick saliva causes the reaction.[10]

A 2012 preliminary study found unexpectedly high rates of alpha-gal allergies in the Western and North Central parts of the United States, which suggests that the allergy may be spread by unknown tick species.[4] Examples of alpha-gal allergies were even present in Hawaii, where none of the ticks identified with the allergies live.[10] Human factors were suggested but no specific examples were provided.[4]

Symptoms

A typical allergic reaction to alpha gal has a delayed onset, occurring 4–8 hours after the consumption of mammalian meat products, instead of the typical rapid onset with most food allergies. After the delayed onset, the allergic response is typical of most food allergies, and especially an IgE mediated allergy, including severe whole-body itching, hives, angioedema, gastrointestinal upset, and possible anaphylaxis.[11] These symptoms are caused by too many IgE antibodies attacking the allergen, in this case the alpha-gal.[6] In 70% of cases the reaction is accompanied by respiratory distress and as such is particularly harmful to those with asthma.[12]

Alpha-gal allergies are the first food allergies to come with the possibility of delayed anaphylaxis.[12][13] It is also the first food-related allergy to be associated with a carbohydrate, rather than a protein.[13][14]

Treatment and medical issues

Blood tests for IgE response indicating alpha gal allergy have not been approved by the U.S. Food and Drug Administration (FDA), and must usually be purchased by private individuals, but are available and are in use.

Alpha-gal is present in cancer drugs, as well as the IV fluid replacements Gelofusine and Haemaccel.

There has been at least one instance of a man with an alpha-gal allergy going into anaphylaxis after receiving a heart-valve.[6] Some researchers have suggested that the alpha-gal which is prevalent in pig’s tissue, and used for xenografts, may contribute to organ rejection.

Unlike most food allergies, the alpha-gal allergy will recede with time, as long as the person is not bitten by another tick. The recovery period can take anywhere from eight months to five years.

A new type of cancer treatment called HyperAcute immunotherapies, which utilizes humans’ usual immunity to alpha-gal is being tested by NewLink Genetics Corporation.[16] The treatment uses modified alpha-gal cells to provoke a strong reaction in the immune system, but targeted towards cancer, rather than attacking the alpha-gal cells themselves.[17] As of November 2013, the treatment was in a Phase 3 Trial with the FDA.[16]

 

 

 

Celiac Syndrome

The Controversy of Celiac Disease (Gluten Sensitivity)

The problem here is that we define a complex autoimmune disease spectrum by a traditional and end-stage histologic finding. Better tools are on the way… There is a spectrum of gluten intolerant disorders. I vote that she has gluten intolerance which is also called celiac syndrome. Treatment is a gluten free diet. Only the top 30% of gluten intolerant people meet the criteria of celiac disease with positive blood work and biopsy. The other 70% are not able to be diagnosed by current criteria but definitely have gluten intolerance or celiac syndrome.

A number of people without celiac disease may feel better on a restrictive diet, but I suspect for a lot of them it’s because their diet is now healthier. I have seen a lot of this and found out that it’s the lack of processed and junk foods rather than the lack of gluten that works for them. Just something to think about.

 

Fungal Issues and Gluten Sensitivity

Fungal Allergies and Sinus Issues

Overview

yeast_top_01_large-300x74Yeast, Candida, Thrush, Monilia, Mold, and Fungus are all terms that we routinely use interchangeably and incorrectly. This is often confusing to both physicians and patients. Mycology is the “study of” fungi and we really have very little of it in medical school. There are really separate pathologic conditions we must differentiate in order to understand the condition. Unfortunately, there is no rule that you cannot have a combination of things affecting you.

Fungal Sinusitis

Fungal sinusitis is really nebulous term that implies there are fungi creating problems within the paranasal sinuses. By definition there really must be some objective findings on a CAT scan of the sinuses. If indeed there are fungal organisms in the sinuses, these can cause inflammation via a number of mechanisms. First, by the fact that they are sitting there in close contact with our sinonasal mucosa, it is more likely that we develop sensitivities or allergies to them. No matter if there are allergies to the fungi or not, removing them from the sinonasal passages is paramount.

Allergic Fungal Sinusitis

When there are fungal organisms inappropriately occupying the sinuses they will often elicit an allergic response. The CT is often impressive for multiple fungal organisms and the allergy tests seem to correlate. Dr. Bradley Marple and colleagues at Dallas Southwestern really described this condition called Allergic Fungal Sinusitis. The treat ment is meticulous surgical debridement and subsequent allergic desensitization.

Eosinophilic (non allergic) Fungal Sinusitis

Mayo clinic physicians have discovered a non allergic (but immune mediated) inflammatory response to molds which causes sinusitis as well. They have termed this Eosinophilic Fungal Sinusitis. It is primarily Cell Mediated (not IgE Mediated) and seems to be related to a specific mold Alternaria. Again, removing any and all molds and then controlling the immune response is the treatment. They actually propose rinsing the sinonasal passages with antifungal agents.

Invasive Fungal Sinusitis

Invasive fungal disease really is largely limited to immunocompromised patients. We often see these as urgent life threatening conditions on the oncology ward. We immediately try to remove all of the offending fungi, however the survival of the patient is really more dependent on the patient mustering some type of an immunologic response. Whether or not the patient has allergies, it rarely makes sense to desensitize an immunocompromised patient.
Candidiasis and Mold Allergies

Fungi can also cause patients problems even if there sinuses are not loaded with organisms. In these patients, the CAT scan is less impressive and surgery is not in the treatment regimen.

Mold Allergies

Allergy to molds is a common ailment and can really keep patients miserable on a year round basis. Molds are often airborne20and unfortunately can cause symptoms on a year congestion, and fatigue. They can readily be diagnosed by either skin or blood testing for IgE against each type of mold. Again, removing the fungi and then desensitizing the patient is the treatment.

Candidiasis/Yeast

Candidiasis is really a diagnosis of exclusion, and a questionable one at that. Dr. William Crooks defined the condition as an overgrowth of yeast primarily within our digestive tract that leads to a chronic inflammatory state. It too is treated with elimination of the molds as much as possible, and then setting up conditions to avoid recurrent yeast overgrowth. These treatment options really seem to help a lot of patients, I am just not sure we understand the pathophysiology of what is really going on. In treating these patients we often try dietary manipulation that would preclude fungal overgrowth. This is often a low carbohydrate or gluten free type of diet. I suspect this may be the major reason these people feel better.

Gluten Sensitivity

Gluten intolerance (Celiac Disease) is probably an under recognized entity.  There is certainly a large portion of the population that has some sensitivity to gluten and feels much better avoiding it.  Whether or not they meet the criteria for Celiac seems academic.

Summary

For whatever reason, many patients seem to get clinical benefit from antifungals, probiotics, and or dietary changes. This makes sense, in that lessening exposure to substances patients are sensitive to, would make them feel better. Likewise, desensitizing them with immunotherapy, also seems to be helpful.

 

 

 

 

 

What is new in post op tonsillectomy?

Recovering From Throat Surgery

Unknown-4Recovering from throat surgery such as tonsillectomy, uvuloplasty, and or base of tongue surgery can be excruciating. There is no pain therapy that is out of bounds. I give 12 mg Decadron intra operatively and generously inject Exparel into the operated on area.

Topically Gelclair can be very helpful. It forms a barrier (similar to but also superior to Carafate). Magic mouth rinse with topical anethestics or Numbing throat sprays (Chloraseptic) can be temporarily helpful.

confezioniI personally use 10 mg of ocycontin po q 12, Lortab Elixir, Motrin Elixir and Amoxacillin or Zithromax Elixir. A Medrol Dose pack can be utilziled. Toradol can also be helpful, although bleeding risk must be considered.

Higher dose steroids (Prednisone) and or more powerful sustained release narcotics such as Oxycontin or Dilaudid can be considered.

Post on recent Angioedema Case

201312howarth-fig-1This is a case of angioedema, a significant swelling of tissues deep to the skin and mucous membranes. Angioedema differs from conditions such as urticaria where there is swelling of the skin. Angioedema most commonly affects the tongue and face. It can also affect abdominal organs.

Angioedema without urticaria can be induced by NSAIDs, or it can be part of a histamine-driven allergic reaction, or be driven by accumulation of bradykinin.1 The patient had not taken any NSAIDs and there was no response to treatments directed towards a histamine-driven allergic process. Reasonable explanations of this presentation of angioedema without urticaria include:13

  • ACE inhibitor angioedema, which occurs in ACE inhibitor users at a prevalence greater than 1:1000. Most cases are much milder than this one.
  • Hereditary angioedema is a condition with a prevalence of about 1:10 000 or less.2 There is a deficiency in C1 esterase inhibitor, or C1 esterase inhibitor is present but not functional, allowing accumulation of active C1 esterase. This in turn leads to, among other things, a periodic accumulation of bradykinin in tissues that results in angioedema.
  • Acquired C1 esterase inhibitor deficiency is an autoimmune condition in which there is autoimmune inactivation of C1 esterase inhibitor.4 This renders the C1 esterase inhibitor ineffective and excess C1 esterase activity can develop. This can result in accumulation of bradykinin in tissues. This condition is very rare with less than 150 described cases.
  • Hereditary angioedema without C1 esterase inhibitor deficiency appears to be due to abnormalities in clotting factor XII and occurs with a prevalence of less than 1:100 000 people.5 The majority of cases are female.

The absence of a family history made either type of hereditary angioedema very unlikely. Further this would be an extraordinarily delayed onset for either type of hereditary angioedema.

The patient was investigated for C1 esterase inhibitor deficiency. Functional C1 esterase inhibitor level was 105% (reference range 70–130%). This ruled out hereditary angioedema and acquired C1 inhibitor deficiency. With respect to hereditary angioedema without C1 esterase inhibitor, over 90% of patients with this very rare condition have a positive family history and most cases are female.

From the above we can conclude the diagnosis is ACE inhibitor angioedema. Neither of the treating doctors recognised the condition as ACE inhibitor angioedema at time of presentation and they treated it as an allergic reaction.

Discussion

ACE inhibitor angioedema has long been a known adverse drug reaction occurring with the whole group of ACE inhibitors. It has been estimated by various means to occur in 0.1–0.42% of patients on an ACE inhibitor.68 It occurs most commonly in the first year of treatment with an ACE inhibitor but can occur after years of use.9,10 Patients may have multiple episodes of ACE inhibitor angioedema before the condition is recognised, and patients with initially mild episodes may progress to having severe life-threatening episodes.9,10

The condition is usually not correctly diagnosed on initial presentation and even in fatal cases the condition often appears to have been unrecognised.11,12 The incidence is likely to be underestimated, which is concerning because of the potential for the condition to be lethal.

The typical case involves swelling of the tongue, but the lips, pharynx, larynx and submandibular tissues can also be involved.9,12 In severe cases failure to secure an airway has led to death by asphyxia.11,12 There have been a number of reported cases where the condition has manifested only as angioedema of the gut. Such cases are usually initially diagnosed with primary abdominal complaints, such as irritable bowel or ischaemic colitis.13,14 Such patients are at risk of undergoing laparotomy.15

There are a number of risk factors for development of ACE inhibitor angioedema. African American patients have repeatedly been shown to be at approximately four times the risk of other patients.6,8,16 Smoking also increases the risk of developing this condition.16 A history of an ACE inhibitor-induced cough is associated with a 9-fold increase in the risk of angioedema.16 Thus it is important to take patients with an ACE inhibitor-induced cough off this group of drugs.

The lack of response to adrenaline, antihistamines and steroids in this case is typical and is due to the condition being related to accumulation of bradykinin and not histamine. With ACE inhibition the breakdown of bradykinin is partly dependent on dipeptidyl peptidase 4 (DPP4). Levels of DPP4 may be reduced in patients who have ACE inhibitor angioedema.1719 The incidence of angioedema with the combination of an ACE inhibitor and a DPP4 inhibitor seems to be 4–5 times higher than the risk with an ACE inhibitor alone.19

A concern for the future is that DPP4 inhibitors are rapidly entering the market for the treatment of type 2 diabetes. These drugs help control type 2 diabetes by inhibiting the breakdown of incretins. As ACE inhibition is usually seen as first-line therapy for hypertension in patients with type 2 diabetes, the incidence of angioedema, a potentially lethal condition, may become more common now that DPP4 inhibitors are regularly being used. If, as in this case, patients can develop angioedema after many years of stable ACE inhibitor use then adding a DPP4 inhibitor may become the destabilising trigger for an episode of angioedema.

Treatment options

In the long term, after an episode of ACE inhibitor angioedema, the patient must not take this class of drug again. Frequently, the patient will be switched to an angiotensin II receptor blocker (ARB). These drugs can also induce angioedema though at a much lower rate than ACE inhibitors. Further, ARBs do not seem to induce severe episodes.20

The acute management of this condition is not clear-cut. The use of antihistamines, steroids and adrenaline is often quoted although there does not seem to be any evidence for efficacy with this line of treatment and certainly in this case there was no apparent benefit. There is often an automatic response among clinicians to give an antihistamine for any condition that could be an immediate hypersensitivity reaction. In a case such as this there is a risk with the use of a sedating antihistamine as the airway may be about to become compromised and a sedating drug may lead to respiratory decompensation.

There are case reports of ACE inhibitor angioedema being successfully treated with fresh frozen plasma.2123 These reports have appeared for over a decade now. There is a recent study of seven patients given fresh frozen plasma for this condition, all of whom had failed to respond to antihistamines, steroid and adrenaline.24 All seven rapidly improved after fresh frozen plasma. Thus, faced with a patient exhibiting severe ACE inhibitor angioedema, a trial of a couple of units of fresh frozen plasma would seem very reasonable to hopefully shorten the attack or to reduce the need for what could be a very difficult intubation.

There are also reports of the successful use of the bradykinin receptor antagonist icatibant,25 a short peptide that has a structure related to bradykinin. It can be administered by subcutaneous injection and is used by patients with hereditary angioedema, often by self-administration. It is not available in rural hospitals and it costs about $3000 per dose.26 By comparison, fresh frozen plasma is available in most rural hospitals and is relatively cheap at $300 a bag.27 Fresh frozen plasma, however, has the disadvantage of having to be thawed, and the minor risk of being a blood product.

Clearly, cases of the severity of this one require urgent airway management and may require emergency cricothyroidotomy.