Tracheal Stenosis

Tracheal stenosis is a narrowing of the trachea, or “windpipe”. Tracheal stenosis causes shortness of breath, which is made worse during Noisy breathing can be heard when tracheal stenosis is severe. tracheal-stenosis-canonicalOr (narrowing due to a birth defect) is a rare condition in which the cartilage support structure of the trachea can cause a narrowing of the airway. A normal tracheal cartilage is C-shaped with a softer, posterior membrane which consists of muscle. Abnormalities of this cartilage can include tracheal cartilaginous sleeves, prone to obstruction and crusting, and complete tracheal rings, in which the trachea consists of several or more complete rings of cartilage with a narrowed diameter. Symptoms of tracheal stenosis can be present shortly after birth affected children may present with rapidly worsening symptoms in the face of an upper respiratory infection. Evaluation for tracheal stenosis may include X-rays and more advanced imaging such as CT or MR imaging. Imaging can provide information not only about the extent of airway narrowing.

Endoscopic view of tracheal stenosis

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For more help contact Tracheal Stenosis Foundation Click photo to enlarge it

https://www.facebook.com/stopts.org

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pyloric stenosis and Esophageal Atresia may coexist.

Pyloric Stenosis

Pyloric stenosis, also called infantile hypertrophic pyloric stenosis, is a condition caused by an enlarged pylorus. The pylorus is a muscle that opens and closes to allow food to pass through the stomach into the intestine. When this muscle becomes enlarged, feedings are blocked from emptying out of the stomach. The retained feedings cause the infant to vomit.

What causes pyloric stenosis?

There is no known reason for enlargement of the pylorus. The main symptom of pyloric stenosis is vomiting undigested breast milk or formula soon after a feeding. Vomiting usually begins at four weeks of age but can happen as early as two weeks after birth. Once vomiting begins it becomes more frequent, and severe, and is often described as “forceful” or “projectile”. Infants with pyloric stenosis may become fussy and, since they cannot keep down all their feedings, are hungry between feedings and are not able to gain weight normally. If the vomiting continues infants may become ill from dehydration.

Pyloric stenosis is one of the most common conditions requiring surgery in infants. It is more common in boys than girls and usually affects children who are born at full term. It rarely occurs in premature infants. Although not thought to be hereditary, pyloric stenosis occurs more commonly in children of parents who had pyloric stenosis themselves as infants.

Click on Photo’s to enlarge

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How is pyloric stenosis diagnosed?

An examination of the abdomen may allow the doctor to feel the enlarged pyloric muscle (called an “olive”). If the pylorus cannot be felt, pyloric stenosis can be diagnosed by ultrasound study or by x-rays taken after the infant drinks a liquid called “contrast.” This is a study which examines the stomach and small intestine and is called an upper GI.

How is pyloric stenosis treated?

Pyloric stenosis does not get better by itself and must be corrected with an operation. The operation is called a “pyloromyotomy” where the surgeon cuts through the muscle fibres of enlarged pyloric muscle in order to widen the opening into the intestine. Prior to the operation, your child will be admitted to the hospital for intravenous fluids. Feedings will be held temporarily and restarted after the operation. A blood sample will be taken to check for dehydration and electrolyte abnormalities. Electrolytes measure the amount of sodium, potassium, carbon dioxide and chloride in the blood. The surgeon will schedule the operation as soon as the infant’s electrolyte levels are normal and the dehydration is corrected. This may take a day of treatment with intravenous fluids. A pyloromyotomy can be done using a small telescope and two miniature instruments through several small band-aid sized incisions, or it can be done through a very small incision on the abdomen.

http://pedsurg.ucsf.edu/conditions–procedures/pyloric-stenosis.aspx

Hypertrophic pyloric stenosis (HPS) is commonly encountered in paediatric practice. The typical infant presents with nonbilious projectile vomiting and dehydration (with hypochloremic hypokalemic metabolic alkalosis) if the diagnosis is delayed. Premature infants tend to present at 3-6 weeks from birth—not at 3-6 weeks from the due date—and these infants may have borderline normal muscle thickness because they are comparatively smaller. Hypertrophic pyloric stenosis is rarely seen in children older than 6 months.

This condition accounts for one-third of nonbilious vomiting occurrences in infants and is the most common reason for laparotomy before age 1 year. A striking male preponderance is seen, with a male-to-female ratio of 4-6:1. In addition, pyloric stenosis and,

(CLICK ON THIS READ WRITING)  oesophagal atresia may coexist.

http://emedicine.medscape.com/article/414368-overview

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Annular Pancreas

What Is Annular Pancreas?

The pancreas is an organ in the posterior (back) of your abdomen, just behind the stomach. A duct connects the pancreas to a part of the small intestine called the duodenum. It is responsible for excreting insulin into the bloodstream to transform glucose into energy. It also secretes enzymes into the intestines to directly aid in the digestion of food.

Normally, your pancreas sits next to your duodenum in your abdomen. In some people, tissue from the pancreas may surround the duodenum. When this happens, your duodenum can become restricted or blocked. This may impact your ability to digest food. This condition is known as annular pancreas.

What Are the Causes of Annular Pancreas? 

Down’s syndrome. 

tracheoesophageal fistula, an abnormal connection between the oesophagus and the trachea. 

intestinal atresia, the failure of a portion of the intestinal tract to completely form. 

pancreas divisum, a birth defect in which ducts of the pancreas don’t join together.

The annular pancreas also occurs with polyhydramnios, or excess amniotic fluid during pregnancy. Annular pancreas is a rare condition that occurs in only 1 in every 20,000 newborns. It occurs more frequently in males than females.

For more on this click below

http://www.healthline.com/health/annular-pancreas#Causes2

http://radiopaedia.org/articles/annular-pancreas

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Roux-En-Y Diversion

Note I couldn’t find a lot on this

Introduction: GERD is a highly prevalent disease process and, with the advent of PPI, it has been successfully treated with medical management. In those patients that fail maximal medical management, Nissen fundoplication is the procedure of choice. Following a failed fundoplication, an acid-diverting procedure like RYGB is highly successful.

Materials and Methods: A 60-year-old male with a past medical history of fibromyalgia, arthritis, anxiety, depression, aspiration pneumonia, presented with a history of severe, longstanding GERD with aspiration and dysphagia; he had failed maximal medical therapy and a Nissen fundoplication.
The patient underwent a laparoscopic Roux –en –Y gastric bypass as an acid-diverting procedure. Intraoperative findings were large amounts of adhesions between the colon, small bowel, omentum, stomach, liver, and abdominal wall. An intraoperative EGD was performed in order to identify the GE junction. The hiatus was closed. An ante colic side-to-side gastrojejunostomy between the pouch and alimentary limbs was performed on the posterior wall with a linear stapler, on the anterior wall with a double layer of interrupted sutures, and checked for leakage with air. A side-to-side jejunojejunostomy between the biliopancreatic and alimentary limbs was performed 100 cm from the gastrojejunostomy. The patient tolerated the procedure well.

Result: The recovery of the patient was uneventful, with a normal UGI on POD2 without leak or obstruction. The patient noted that his symptoms of reflux and aspiration improved dramatically.

Conclusion: Roux-en-Y gastric bypass has been shown to be the most effective bariatric procedure that consistently leads to weight reduction and improvement of GERD symptoms. RYGB is the procedure of choice for patients with intractable GERD, following failed standard fundoplication procedures.

roux-en-y

http://www.sages.org/meetings/annual-meeting/abstracts-archive/laparoscopic-roux-en-y-gastric-bypass-for-intractable-gerd-following-a-failed-nissen/

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Billroth I or II anastomosis is mostly applied for distal gastrectomy. However reflux of duodenal contents into remnant stomach even oesophagus and extra-esophagus can be a challenging problem after the procedure.

The erosive duodenal mixed with gastric fluid is much harmful to the end organ mucosa causing severe digestive even respiratory symptoms or complications. The main medication for gastroesophageal reflux disease (GERD), often a proton pump inhibitor (PPI), is often found to have limited effect for duodenal gastroesophageal reflux (DGER) .

Laparoscopic fundoplication has been proved highly effective and becoming a standard antireflux procedure for GERD, however, a fundoplication may not sufficient to stop DGER, so we added Roux-en-Y diversion to the procedure. The purpose of this report is to describe the outcome of laparoscopic Dor fundoplication plus Roux-en-Y diversion in six patients with intractable duodenogastroesophageal reflux after BillrothI and II reconstruction.

roux_en_y_gastric_bypass

For the full PDF click here and enlarge on their site

http://www.clinicsinsurgery.com/pdfs_folder/cis-v1-id1015.pdf

http://www.americanjournalofsurgery.com/article/S0002-9610(03)00213-7/abstract

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 March 16, 2016

Is Acid Reflux Genetic?

Click on the RED underlined words to those links

 Is Acid Reflux Genetic?

There is abundant evidence showing a link between our genes and acid reflux. Studies in people with acid reflux symptoms and GERD have identified common markers in our DNA associated with acid reflux.

Studies in Twins

One of the best ways to study the link between a particular condition and genetics is to research it in twins. Identical twins share the same DNA. If both twins have a particular disease, there is likely a genetic cause.

One study published in Alimentary Pharmacology & Therapeutics journal found that twins were more likely to both have GERD. The study included 481 identical and 505 fraternal twins. The correlation was stronger in identical twins compared to fraternal twins. This suggests that genetics play a role in causing acid reflux.

An earlier study published in the Gut journal found that one twin was 1.5 times more likely to suffer from GERD if their identical twin had the condition. The study compared heartburn incidence in more than 2,000 sets of identical twins.

Family Studies

If acid reflux is genetic, this means that multiple family members are more likely to have the condition. Research at the University of Amsterdam found a pattern of inheritance of GERD among multi-generational family members. Of the 28 family members who participated in the study, 17 members from four generations were affected with GERD. However, the researchers couldn’t pinpoint the specific gene.

Studies in People with Barrett’s Esophagus

Barrett’s oesophagus is a serious complication of GERD. It is linked to an increased risk of developing oesophagal cancer. Genetics may play an especially important role in Barrett’s oesophagus.

A study reported in the journal Nature Genetics found specific gene variants on chromosomes 6 and 16 were linked to a higher risk of Barrett’s oesophagus. The study found that the closest protein-encoding gene to these variants is FOXF1, which is connected to the development and structure of the oesophagus. A 2013 article in the International Journal of Cancer also reported a link among FOXF1, Barrett’s oesophagus, and oesophagal cancer.

A 2016 study in Nature Genetics found a significant genetic overlap among the following diseases: GERD – Barrett’s oesophagus –  oesophagal cancer.

The researchers concluded that GERD has a genetic basis, and they hypothesised that all three diseases are linked to the same gene locus.

Other Studies

Many other studies have shown a link between genetics and GERD. For example, a study published in the American Journal of Gastroenterology found that a specific polymorphism (a variation in DNA) called GNB3 C825T was present in all 363 GERD patients included in the study. The polymorphism wasn’t present in the study’s healthy control population.

Much more to read here.

http://www.healthline.com/health/gerd/genetics#Overview1

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Here to help you the Parent

Button batteries pose ‘deadly’ risk to toddlers

Surgeons are warning of the potentially deadly risk posed to young children by button batteries. If accidentally swallowed, the small, round batteries can get lodged in the oesophagus and burn a hole through its lining. London’s Great Ormond Street Hospital has seen a big increase in cases in the past year. Surgeon Kate Cross said: “Button batteries should be treated like poison and kept out of reach of children.”

Great Ormond Street Hospital says a decade ago they rarely treated button battery injuries, but there has been a dramatic rise recently, and now are seeing about one child a month.

Kate Cross, consultant neonatal and the paediatric surgeon said: “If the battery gets enveloped in the mucosa of the oesophagus it creates an electrical circuit and the battery starts to function, releasing an alkali which is like caustic soda, which can erode through the wall to the windpipe.”If the battery is facing a different way it can burn into the aorta, a major blood vessel, and there have been cases in Britain where the child has bled to death. “That is why it is important to get the message out to parents but also other health professionals because this is a time critical problem.”

button battary

Watch what damage one of these flat Button batteries can do watch video

http://www.bbc.co.uk/news/health-37410343

Parents – let us fuel your knowledge on battery risks to children

There is warning parents about the dangers of children swallowing these batteries as with more and more compact electronic devices appearing in the home, the risk of children swallowing these small batteries is increasing. We all know that very young children find out about the world by putting things in their mouths, but what many parents don’t realise is that lithium batteries react with saliva so that they leak acid within as little as an hour.

Therefore, if a child swallows a battery it can cause severe trauma, such as burning a hole in their throat or stomach or further damage to other internal organs within a few hours. The Queensland Injury Surveillance Unit in Australia estimates that four children a week are admitted to hospital after swallowing batteries. Meanwhile, the National Capital Poison Center in the USA reports that there are around 3,500 incidents a year where swallowed batteries require urgent treatment.

https://safetygonesane.wordpress.com/2013/10/07/parents-let-us-fuel-your-knowledge-on-battery-risks-to-children/

Swallowed a Button Battery? A battery in the Nose or Ear? 

For Parents in the USA Only (Help Phone No 24-7)

Every year in the United States, more than 3,500 people of all ages swallow button batteries. These are used to power hearing aids, watches, toys, games, flashing jewellery, singing greeting cards, remote control devices, and many other items. The National Capital Poison Center in Washington, DC, operates a 24/7 hotline for battery ingestion cases. Call 202-625-3333 for guidance.

Most button batteries pass through the body and are eliminated in the stool. However, sometimes batteries get “hung up”, and these are the ones that cause problems. A battery that is stuck in the esophagus is especially likely to cause tissue damage. An electrical current can form around the outside of the battery, generating hydroxide (an alkaline chemical) and causing a tissue burn. When a battery is swallowed, it is impossible to know whether it will pass through or get “hung up”.

If anyone ingests a battery, this is what you should do:

Immediately call the 24-hour National Battery Ingestion Hotline at 202-625-3333.

If readily available, provide the battery identification number, found on the package or from a matching battery.

In most cases, an x-ray must be obtained right away to be sure that the battery has gone through the esophagus into the stomach. (If the battery remains in the esophagus, it must be removed immediately. Most batteries move on to the stomach and can be allowed to pass by themselves.)  Based on the age of the patient and size of the battery, the National Battery Ingestion Hotline specialists can help you determine if an immediate x-ray is required.

Don’t induce vomiting. Don’t eat or drink until the x-ray shows the battery is beyond the esophagus.

Watch for fever, abdominal pain, vomiting, or blood in the stools. Report these symptoms immediately.

Check the stools until the battery has passed.

Your physician or the emergency room may call the National Button Battery Ingestion Hotline at the National Capital Poison Center (202-625-3333) for consultation about button batteries. Expert advice is available 24 hours a day, 7 days a week.

Button batteries may also cause permanent injury when they are placed in the nose or the ears. Young children and elderly people have been particularly involved in this kind of incident. Symptoms to watch for are pain and/or a discharge from the nose or ears. DO NOT use nose or ear drops until the person has been examined by a physician, as these fluids can cause additional injury if a battery is involved.

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Myotonic Muscular Dystrophy (MMD)

Myotonic muscular dystrophy (MMD) is a form of muscular dystrophy that affects muscles and many other organs in the body. Myotonic dystrophy is more than just a muscle disease. Both MMD1 and MMD2 affect several aspects of physical and mental functioning. Like many of you, we were surprised to learn that such a wide range of symptoms — muscle weakness, involuntary clenching of hands and jaw, swallowing problems, eye problems, heart disorders, extreme fatigue and other difficulties, could be caused by a form of muscular dystrophy. When MMD1 begins in early childhood (but not at birth), the first symptoms tend to be more behavioral and cognitive than physical. Some people with the juvenile-onset form struggle with planning ahead, decision-making and visual-spatial processing. Babies with congenital MMD1 have very weak muscles and a lack of muscle tone (hypotonia). They appear floppy, have trouble breathing, and suck and swallow poorly. As the child matures into adulthood, typical features of adult-onset MMD1 usually emerge.

https://www.mda.org/quest/article/juvenile-onset-mmd1-can-cause-cognitive-behavior-challenges

Adult-Onset MMD1 and MMD2

https://www.mda.org/disease/myotonic-muscular-dystrophy/signs-and-symptoms/adult-onset-MMD

Very interesting read

https://www.mda.org/sites/default/files/publications/Facts_MMD_P-212_0.pdf

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