How we treat tracheomalacia 2017

Esophageal and Airway Treatment Center | Boston Children’s Hospital

Talked though by the World Famous Dr Jennings

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Tracheomalacia and complex congenital airway problems released 11 Jan 2017

Tracheomalacia and complex congenital airway problems and treatment 

 Here on the 12th Jan 2017

By Dr Jennings

Published on 11 Jan 2017

Surgical Grand Rounds Jan 11 2017 on Complex Congenital Airway Treatment by Dr Jennings and colleagues. In detail discussion of current treatments for tracheomalacia, tracheal compression, tracheal anomalies and vascular rings including aberrant subclavian artery, circumflex aorta and double aorta. He also discusses treatment for tracheal strictures with resection and slide tracheoplasty and slide bronchoplasty

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Tracheomalacia 

Published on Nov 1, 2015

Severe Tracheomalacia Assessment and Treatment

VERY INTRESTING

Dr Russell Jennings explains the current diagnostic methods for children with noisy breathing, with a focus on tracheomalacia. He also goes into some of the current effective treatment techniques, including the posterior tracheopexy.

Watch the talk from Dr Russell Jennings: https://www.youtube.com/watch?v=YotyiMFLMM0&sns=em

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EA/TEF Tracheomalacia Baby struggling to feed

Published on Jul 1, 2012 (Thank you to the Parents that posted this so we can show that from the struggle and first you as Parents will get though. Read the positive comments below) 

Baby ****** is about 7 weeks old here and had just come home from his first stay in the NICU after he was born 32 weeks preemie with an EA/TEF repair needed and NEC. He has tracheomalacia and laryngomalacia with extreme GERD. He ultimately had many ‘blue spells’ and one “blue death” resulting in Mommy resuscitating him at home and 2 subsequent Nissen Fudoplication surgeries and frequent hospitalizations for a host of bronchial and GI issues. A later video was also posted of the child older and much happier. This was a very difficult time for us, but if you are going through this … HANG IN THERE. They need TLC but will improve with time and are exceedingly happy to be alive.

https://www.youtube.com/watch?v=NA7qdN1P-sc

THANK YOU to the Parents

Anterior Tracheal Suspension 

Tracheomalacia (TM) is a disorder of the airway consisting of a weakness of the anterior tracheal cartilaginous rings and laxity of the posterior membranous trachea, resulting in dynamic airway obstruction. The clinical symptoms range from a chronic cough and wheezing to life-threatening apnea with cyanosis and profound airway obstruction (acute life-threatening events). TM in infancy occurs either in isolation or in association with a variety of anatomic conditions, including a wide range of congenital heart lesions, as well as primary tracheal and esophageal disorders. TM is also associated with chromosomal abnormalities, such as 22q11.2 deletion syndrome, and trisomy 21.

Severe TM is a life-threatening condition with reported mortality rates as high as 80%. Prolonged intubation and chronic positive pressure ventilation are particularly common in affected patients following reparative congenital heart surgery; in the most severe cases, tracheostomy is required. Although often life-saving in these patients, tracheostomy results in a high risk of medical, social, and developmental complications. Patients requiring tracheostomy are technology-bound until their anterior tracheal wall matures and they effectively outgrow their malacia. Most concerning, patients with tracheostomy and persistent severe TM remain at risk of sudden death, recurrent infections, and equipment failure.

Aortopexy has been accepted as the standard surgical treatment to alleviate TM associated with congenital heart disease and carries a success rate of 85 to 90%. In our experience, innominate artery-pexy coupled with aortopexy has been particularly effective in relieving TM secondary to isolated innominate artery compression. However, it has also been our experience that aortopexy alone has failed to relieve symptoms in a number of cases of more complex TM and tracheobronchial malacia.

For the rest of this REPORT Clink on the link as there are detailed photos to explain this

www.optechtcs.com/article/S1522-2942%2811%2900075-4/fulltext

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Laryngomalacia

Newborn making abnormal sounds while breathing? Watch out, for it may be a case of Laryngomalacia that is the actual cause. Read and know all about the causes, symptoms, diagnosis and treatment options for this disorder.

Laryngomalacia Definition

It is a congenital malformation of the laryngeal cartilage, meaning it is present at birth. However, it is not a serious condition in the majority of cases.

This anomaly of the larynx is also referred to as “Soft Voice Box” or “Soft Larynx.”

Laryngomalacia ICD9 Code

The ICD9 Code for this disease is 748.3.

Who Suffers from Laryngomalacia?

The condition is quite common in infants and young children although it may also arise in some adults. Older adults, particularly those suffering from weakened throat muscles due to neuromuscular disorders, can suffer from this disease. However, the disorder is much more common in infants.

Laryngomalacia Symptoms

A person affected by this disorder tends to make abnormal noises, especially a whistling sound (stridor) while breathing. Stridor refers to a high-pitched noise that can be heard when a child breathes in (inspires). It can sound like snoring through a congested nose or high-pitched squeaking. In some cases, the sound is audible to anyone present near the sufferer. In the rest of cases, the noise can be heard only with the help of a stethoscope.

Picture of Laryngomalacia

Picture 1 – Laryngomalacia

In some infants, the area at the throat base can be found to sink in with inspiration. In others, the region under the diaphragm is observed to drop.

While stridor is mainly audible during the inspiration of infants, it can also be heard at the time of expiration (breathing out).

Some other features of the condition include: Click Link

http://www.primehealthchannel.com/laryngomalacia.html

Laryngopharyngeal Reflux

https://www.verywell.com/laryngopharyngeal-reflux-lpr-1742320

MORE INFO

Laryngomalacia is the most common cause of stridor in newborns, affecting 45–75% of all infants with congenital stridor. The spectrum of disease presentation, progression, and outcomes is varied. Identifying symptoms and patient factors that influence disease severity helps predict outcomes. Findings. Infants with stridor who do not have significant feeding-related symptoms can be managed expectantly without intervention. Infants with stridor and feeding-related symptoms benefit from acid suppression treatment. Those with additional symptoms of aspiration, failure to thrive, and consequences of airway obstruction and hypoxia require surgical intervention. The presence of an additional level of airway obstruction worsens symptoms and has a 4.5x risk of requiring surgical intervention, usually supraglottoplasty. The presence of medical comorbidities predicts worse symptoms. Summary. Most with laryngomalacia will have mild-to-moderate symptoms and not require surgical intervention. Those with gastroesophageal reflux and/or laryngopharyngeal reflux have symptom improvement from acid suppression therapy. Those with severe enough disease to require supraglottoplasty will have minimal complications and good outcomes if multiple medical comorbidities are not present. Identifying patient factors that influence disease severity is an important aspect of care provided to infants with laryngomalacia.

http://www.hindawi.com/journals/ijpedi/2012/753526/

There is a FaceBook Group for this

https://www.facebook.com/LoveForMalaciaBabies

Q-A Help

http://www.babycenter.com/400_babies-with-laryngomalacia_13357800_926.bc

Bronchitis

For more on Breathing go to this page to

http://www.birth-defect.org/breathing-with-tof-and-tef/

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Bronchitis – acute

Acute bronchitis is swelling and inflammation in the main passages that carry air to the lungs. The swelling narrows the airways, which makes it harder to breathe. Another symptom of bronchitis is a cough. Acute means the symptoms have been present only for a short time.

acute

Bronchitis is an inflammation of the airways in the lungs. The main tubes that air flows through in the lungs are called bronchi, and branching off them are smaller tubes called bronchioles. When these tubes become inflamed it causes narrowing, constriction, and blockage of the airways, which leads to symptoms of bronchitis. Bronchitis can be acute, lasting less than six weeks, or chronic.

Sometimes, bacteria also infect your airways. This is called a secondary infection.

Chronic Bronchitis

Chronic bronchitis is a recurrent disorder where there is chronic inflammation, swelling, and narrowing of the airways. It is defined as a cough with production of mucus (sputum) for at least a 3-month period, for two years in a row. Chronic bronchitis is usually the result of lung damage from chronic medical disorders.

broncites

bronchitis_s2_diagram

Can Bronchitis Be Treated At Home?

If symptoms of bronchitis are not severe, home remedies include:

  • Drink plenty of fluids
  • Take over-the-counter fever-reducing medications such as aspirin, acetaminophen (Tylenol), ibuprofen (Advil, Motrin), naproxen (Aleve)  if advised by a Doctor PLUS Do not give aspirin to children.
  • Get plenty of rest
  • Use a humidifier or steam in the bathroom.

How Is Bronchitis Diagnosed?

 

Even after acute bronchitis has cleared, you may have a dry, nagging cough that lasts for 1 to 4 weeks.

Sometimes it can be hard to know you have pneumonia or bronchitis. If you have pneumonia, you are more likely to have a high fever and chills, feel sicker, or be more short of breath.

Pneumonia

READ HERE ON THIS WEBSITE THE STORY OF A YOUNG CHILD’S FIGHT WITH Pneumonia …….. Flown across Australia BY THE AIR DOCTOR’S …

www.birth-defect.org/from-the-heart/

Pneumonia is inflammation or infection in one or both of the lungs (sometimes called a chest infection). Pneumonia in children can be caused by viruses or bacteria. Viruses are the most common cause of pneumonia.

Due to the infection, the small airways in the lungs become swollen and make more sticky fluid (mucus). This blocks the airways and reduces the amount of oxygen that is able to get into the body. Pneumonia can affect one part (lobe) of the lung or all of the lung.

The doctor will determine if your child has pneumonia by asking you questions and examining your child. Blood tests are sometimes helpful to find the likely cause of pneumonia (i.e. bacteria or virus). An X-ray of your child’s chest can confirm the diagnosis. If your child has pneumonia the X-ray will show fluid (consolidation) in the lungs.

Pneumonia often comes after a respiratory infection, such as a cold or flu. Most children with pneumonia get better quite quickly and completely.

Signs and symptoms

Signs and symptoms of pneumonia vary depending on your child’s age and the cause of pneumonia.

Children often have one or more of the following:

  • high fever;
  • fast and/or difficult breathing – your child’s breathing will become hard work and you can often see the ribs ‘sucking in’ when they are breathing;
  • cough;
  • vomiting;
  • be irritable or more tired than usual;
  • pain in the chest, especially when coughing;
  • tummy (abdominal) aches or pain.

Most children refuse to eat. This is often concerning for parents but remember that your child will begin to eat again once they feel better. It is important to give your child fluids to prevent dehydration.  Offer small sips of water and offer babies the breast or bottle more often.

Treatment at home

Most children with pneumonia are able to be treated at home.

  • They will need a lot of rest.
  • They need to drink small amounts of fluids often so they do not get dehydrated.
  • They may be more comfortable sleeping propped up on a couple of pillows, rather than laying completely flat.
  • If your child has chest pains they may need some pain relief, such as paracetamol (e.g. Panadol).
  • Do not give cough medicines. They do not help children with pneumonia.
  • Smoking around your child should be avoided at all times.

Bacterial Pneumonia – If your child’s pneumonia is caused by bacteria they will be given an antibiotic medicine to take. Children with bacterial pneumonia usually improve a lot within 24 to 48 hours of starting antibiotics. Their fever will come down, they will have more energy and their breathing will become easier. They may, however, continue to cough for days to weeks. It is very important to complete the whole course of antibiotics, even if your child seems remarkably better. Treatment will continue for seven to 10 days.

Viral pneumonia – Antibiotics do not cure viruses and are not given for viral pneumonia. Recovery is usually slower, over two to four weeks.

IMG_8513_resized[1]

Treatment in hospital

Some children with pneumonia need to be admitted to hospital. This is usually if they:

  • are less than one year old;
  • are not able to take medicine by mouth;
  • have become dehydrated;
  • have severe breathing problems.

While in hospital your child will be watched closely and the following treatments may be necessary:

  • Antibiotics might be given directly into a vein through a drip (intravenous or IV). These will only be given if the pneumonia is thought to be bacterial.
  • Some children may need oxygen to help them to breathe more easily.
  • Children who are dehydrated will need to be given fluids by a drip (intravenous or IV therapy).

When to come back

You should contact a doctor if your child is being treated for pneumonia and:

  • their breathing becomes more difficult;
  • they become more drowsy or sleepy, or are hard to wake;
  • they are continuing to vomit and are unable to drink much;
  • you are worried about your child at any stage during the illness or you have other questions.

Some children need to be checked after a few days. Your doctor will tell you when to come back.

About six weeks after the illness your child will need to see a doctor to make sure that they have had a complete recovery.

Prevention

  • Keep your child up to date with their immunisations. Yearly flu shots may be beneficial in some children.  Your family doctor can give you more advice about flu shots. 
  • Teach primary school aged children not to share eating or drinking utensils, toys and food or drinks with other children. This is difficult to do in young children, especially in créche, childcare or kindergarten, as they often put shared toys in their mouth.  Immunised children will have a much smaller risk of becoming infected with pneumonia in these instances.
  • Teach children to wash their hands after coughing or sneezing to prevent the spread of germs.

Key points to remember

  • Most children with pneumonia recover quite quickly and completely.
  • If your child has pneumonia they will need to rest and drink small amounts of fluid often.
  • Cough medicines do not help children with pneumonia.
  • It is very important to complete the course of antibiotics if they have been prescribed.

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July 7, 2015

Antibiotic efficacy is linked to bacterial cellular respiration

Bacteriostatic and bactericidal antibiotic treatments result in two fundamentally different phenotypic outcomes the inhibition of bacterial growth or, alternatively, cell death. Most antibiotics inhibit processes that are major consumers of cellular energy output, suggesting that antibiotic treatment may have important downstream consequences on bacterial metabolism. We hypothesised that the specific metabolic effects of bacteriostatic and bactericidal antibiotics contribute to their overall efficacy. We leveraged the opposing phenotypes of bacteriostatic and bactericidal drugs in combination to investigate their activity. Growth inhibition from bacteriostatic antibiotics was associated with suppressed cellular respiration whereas cell death from most bactericidal antibiotics was associated with accelerated respiration. In combination, suppression of cellular respiration by the bacteriostatic antibiotic was the dominant effect, blocking bactericidal killing. Global metabolic profiling of bacteriostatic antibiotic treatment revealed that accumulation of metabolites involved in specific drug target activity was linked to the buildup of energy metabolites that feed the electron transport chain. Inhibition of cellular respiration by knockout of the cytochrome oxidases was sufficient to attenuate bactericidal lethality whereas acceleration of basal respiration by genetically uncoupling ATP synthesis from electron transport resulted in potentiation of the killing effect of bactericidal antibiotics. This work identifies a link between antibiotic-induced cellular respiration and bactericidal lethality and demonstrates that bactericidal activity can be arrested by attenuated respiration and potentiated by accelerated respiration. Our data collectively show that antibiotics perturb the metabolic state of bacteria and that the metabolic state of bacteria impacts antibiotic efficacy.

Bacteriostatic antibiotics inhibit cell growth whereas bactericidal antibiotics induce cell death. Classifying an antibiotic as bacteriostatic or bactericidal is based on an operational in vitro test, which offers a limited perspective on the physiologic activity of the antibiotic. Although the clinical value of bactericidal activity in the treatment of infection is a point of debate, evidence supports a preference for bactericidal antibiotics for certain high-risk infections. The use of antibiotic combinations to treat bacterial infections is increasingly common, but the predictability of this approach is limited. It is well-known that bacteriostatic –

bactericidal combination treatments result in attenuation of bactericidal activity in vitro across a range of drugs and organisms. Clinically, this effect can have negative consequences in high morbidity infections like meningitis, or positive effects by inhibiting lysis and exotoxin released in toxin-mediated syndromes (21, 22). How bacteriostatic antibiotics can block bactericidal lethality, however, is not well-understood.

Recent lines of evidence have suggested that antibiotics induce cellular metabolic shifts as a secondary response to their target interaction. The generation of antagonistic metabolic responses may be one possible means by which bacteriostatic and bactericidal antibiotics interact. The predominant cellular process targeted by bacteriostatic antibiotics is a translation, which is thought to account for a major portion of the energy consumption in the cell at steady state. Consequently, disruption of this process may cause significant changes in cellular energy dynamics. In support of this notion, the proteomic response to the bacteriostatic antibiotic chlortetracycline involves downregulation of major metabolic pathways, potentially suggesting a reduction in metabolic rates. In comparison with the bacteriostatic response, evidence suggests that bactericidal

agents may increase cellular metabolic rates and that bactericidal antibiotic efficacy may relate directly to the metabolic state. The regulation of genes involved in central metabolism and respiration.

Significance

The global burden of antibiotic resistance has created a demand to better understand the basic mechanisms of existing antibiotics. Of significant interest is how antibiotics may perturb bacterial metabolism, and how bacterial metabolism may influence antibiotic activity. Here, we study the interaction of bacteriostatic and bactericidal antibiotics, the two major phenotypic drug classes. Interestingly, the two classes differentially perturb bacterial cellular respiration, with major consequences for their intrinsic activity both alone and in combination. Of note, bacteriostatic antibiotics decelerate cellular respiration, generating a metabolic state that is prohibitive to killing. Further, we show that the efficacy of bactericidal drugs can be improved by increasing basal respiration, and we identify a respiration-related drug target that potentiates the activity of bactericidal antibiotics.

www.pnas.org/content/112/27/8173.full.pdf

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5 Types of EA -OA

Types of EA or OA

The Federation of Esophageal Atresia and Tracheo-Esophageal Fistula

There are 5 types of EA or OA The types are found below, without Sue lewis-Jones from a UK Charity sending us this link back in 2013 when this website was put together, since then has grown to what you see now, two year’s on we are raising awareness for these conditions. We thank Sue for her help.

www.we-are-eat.org/tell-me-about-esophageal-atresia/types-of-ea/

From Sue Lewis-Jones Trustee of a UK Charity

 

Isolated EA
(Gross type A, Vogt type 2)
Pure Esophageal Atresia, with no TEF.About 8% of EA patients have this type (which is also known as Gross type A, or Vogt type 2).
EA with Proximal TEF
(Gross type B, Vogt type 3A)
EA, with a proximal TEF, or a TEF connecting between the upper pouch of the oesophagus and the trachea.This is rare, with only about 0.8% of EA patients have this type. With this type of EA, food and saliva can travel directly into the lungs, before surgical correction is performed
EA with Distal TEF
(Gross type C, Vogt type 3B) .EA, with a distal TEF, or a TEF connecting between the lower pouch of the oesophagus and the trachea. This is by far the commonest type of EA, with about 89% of EA patients having this type.With this type of EA, gastric contents and acid can travel directly into the lungs, before surgical correction is performed.
EA with Dual TEF’s (Gross type D, Vogt type 3C) Dual TEF, where there is both a TEF connecting between the upper pouch of the oesophagus and the trachea and a TEF connecting the lower pouch of the oesophagus and the trachea. This is rare, with only about 1.4% of EA patients have this type.
H-type TEF (no EA) (Gross type E) H-Type TEF, where there is a TEF connecting between the oesophagus and the trachea, but there is no EA.Children with an H-Type TEF, unlike the other types, are often diagnosed later in infancy or childhood (and rarely as adults), rather than becoming evident shortly after birth. People with an H-type TEF can swallow, but often cough and choke during swallowing, especially with liquids, and may present with recurrent cases of pneumonia. The exact prevalence of this form is not known (because they tend to be diagnosed late), but is thought to be about 4% (this is known as Gross type E).

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Acid Reflux and Shortness of Breath June 15, 2015

Part 1 of 4: Overview

Difficulty breathing is one of the more frightening symptoms of acid reflux and the chronic form of the condition, which is called gastroesophageal reflux disease (GERD). GERD can be associated with breathing difficulties such as bronchospasm and aspiration. These difficulties can sometimes lead to life-threatening respiratory complications.

Shortness of breath, also called dyspnea, occurs with GERD because stomach acid that creeps into the esophagus can cause it to narrow. When gastric acid reaches the vocal folds, airways, and lungs, it can cause a swelling of the passages. This can lead to atypical asthma reactions. Such airway damage can affect breathing by causing coughing or wheezing and making swallowing solid foods more difficult.

http://www.healthline.com/health/gerd/shortness-of-breath#Overview1

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Post Nasal Drip

A relatively common disorder among people with allergies and other conditions like acid reflux

A relatively common disorder among people with allergies and other conditions like acid reflux is a post-nasal drip. A post-nasal drip has several causes and symptoms that can disrupt your day and cause discomfort. Like any medical condition, it is important to get this treated right away if you have it. Knowing post-nasal drip symptoms helps toward identifying what is wrong and being able to tell a doctor what you are experiencing. Post-nasal drip symptoms are varied and can last a long time if they are not identified and treated. Some of the most severe signs of post-nasal drip (click on red writing) are similar to the signs of normal allergy irritation and may be ignored by some people. If you know the symptoms for this condition and experience any of them, it is important to let your doctor know.

nose

One of the most common and severe post-nasal drip symptoms is coughing. People with a post-nasal drip tend to start out with a mild cough that is commonly mistaken for a cough from allergies or irritation. This cough can become severe and in some cases painful. Coughing associated with post nasal drip tends to be persistent and is often accompanied by wheezing. Wheezing is more commonly from the presence of post nasal drip than asthma. The excess mucus from post-nasal drip can cause you to wheeze and cough frequently and the symptoms will not subside until the drip is corrected.

http://www.therabreath.com/how-to-stop-post-nasal-drip.html

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Airway reflux as a cause of respiratory disease  Alyn H. Morice 1 June 2013

Summary Reflux is the cause of much respiratory disease. More commonly still, it is the unrecognised agent provoking the symptoms of respiratory disease. That the reflux entering the airways from the gastrointestinal tract is central to the diagnosis, therapy and understanding of respiratory pathology has been missed because the paradigm of peptic disease has been applied. Airway reflux is however unlike gastro-oesophageal reflux disease (GORD). GORD is liquid acid reflux causing heartburn and indigestion. Airway reflux consists of a mainly gaseous non-acid mist which, when deposited in the upper and lower airways leads to inflammation, fibrosis, bronchoconstriction and cough. Here, the hypothesis that airway reflux is responsible for chronic “idiopathic” cough, late onset asthma, exacerbations of COPD, ‘idiopathic’ pulmonary fibrosis and even the lung disease of cystic fibrosis is outlined. The exclusive focus of clinicians on the extrinsic origins of these conditions and the rejection of an obvious intrinsic aetiology causes millions of patients to be denied an explanation for their symptoms and simple, effective, treatments. In many cases idiopathic should be no longer considered idiopathic.

Introduction

Reflux is the cause of all respiratory disease. An exaggeration of course, but I hope to convince you in this article that there is more than a germ of truth in this outrageous statement. There have been two barriers to the revelation that reflux is a major contributor to respiratory pathology. First, we have been labouring under the diagnostic criteria of reflux as gastroesophageal reflux disease (GORD). There is no doubt that GORD exists and the gastroenterologists have defined and characterised the disease which causes heartburn, dyspepsia and oesophagitis; however, they have been very reluctant to accept that this is merely the tip of the reflux iceberg, with extra-oesophageal reflux being out of their home territory. Respiratory professionals, being largely ignorant of reflux, its aetiology and manifestations, have accepted the wisdom of the gastroenterologists, since this is “their” area. So blinkered has this attitude become that, with one or two exceptions, one should not talk to a gastroenterologist about what has been termed airway reflux. You might as well talk to a tree!

http://breathe.ersjournals.com/content/9/4/256

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Long-term respiratory complications of congenital esophageal atresia with or without tracheoesophageal fistula: an update.

Despite early surgical repair, congenital esophageal atresia with or without tracheoesophageal fistula (EA ± TEF) has long-term effects on respiratory and gastrointestinal function. This review updates summarizes research published since 2003 on long-term respiratory complications in patients with a history of EA ± TEF. Pulmonary hypoplasia appears to not be rare in patients with EA ± TEF. Tracheomalacia is common and is associated with respiratory symptoms in childhood. Aspiration, associated with esophageal dysmotility and/or gastroesophageal reflux, may lead to reduced pulmonary function and bronchiectasis. Pulmonary function is generally normal, although lower than in control patients, and restrictive defects tend to be commoner than obstructive defects. Abnormal airway reactivity is common and, along with respiratory symptoms, is associated with atopy. However, the inflammatory profile in EA ± TEF patients based on bronchial biopsies and exhaled nitric oxide differs from typical allergic asthma. Recent studies suggest that in older patients, respiratory symptoms tend to be associated with atopy, but abnormal lung function tends to be associated with gastroesophageal reflux and with chest wall abnormalities. Early detection and management of aspiration may be important to help prevent decrements in pulmonary function and serious long-term complications in EA ± TEF patients.

www.ncbi.nlm.nih.gov/pubmed/23679034

Aortopexy for Innominate Artery Compression of the Trachea

Abstract

Tracheomalacia is a rare congenital condition that results from the improper formation of the cartilaginous tracheal rings but is often seen in children who have oesophagal atresia with/without trachea-esophageal fistula. This causes the trachea, which is the main airway, to be floppy, and as the child grows, can result in breathing difficulties. These difficulties are usually manifested as breathing noises that may change with position and improve during sleep, or that get worse with coughing, crying, or feeding. Upper respiratory infections can also be more common. While most cases of tracheomalacia resolve by 18 to 24 months of age, a small percentage either continue or cause such severe breathing or feeding issues that surgical intervention is warranted. In cases where the innominate artery is the cause of compression of the weakened trachea, an aortopexy to elevate the vessel up to the sternum and away from the trachea is performed.

Case Overview

Focused History

Patients with tracheomalacia present primarily with breathing difficulties, the most common of which is stridor. Many times this occurs in the setting of prior oesophagal atresia-tracheoesophageal fistula repair, with the symptoms beginning only after repair of the atresia. Care should be taken to elicit symptoms consistent with cyanosis or feeding difficulties, as these can indicate a more severe problem than that indicated by the physical examination. Since most cases of tracheomalacia resolve without intervention, it is crucial to take into account the age of the patient and the overall clinical course up to the present when making a decision for surgery.

Physical Findings

Findings consistent with airway compromise guide management. Specifically, breathing noises that may change with position and improve during sleep, or that get worse with coughing, crying, or feeding. Upper respiratory infections can also be more common, along with high-pitched breathing and rattling or noisy breaths. Expiratory stridor at rest, biphasic stridor and cyanosis are findings that are indicative of severe airway compromise. In addition, care should be taken to rule out gastroesophageal reflux disease and, in patients with prior repair, recurrent tracheoesophageal fistula, as these latter two conditions can cause the same symptoms, or even co-exist with tracheomalacia.

Imaging studies

The diagnosis of tracheomalacia is confirmed by trachea bronchoscopy, or direct visualisation of the trachea, at which time a recurrent fistula should also be ruled out in those patients with the prior EA-TEF repair. Ancillary studies include CT angiogram of the chest to help document compression due to the innominate artery, as well as a barium swallow to rule out gastroesophageal reflux disease in those children where the presence of reflux is suspected.

Natural history

Most cases of tracheomalacia resolve with time, with the more mature airways proving more resilience to compression due to increasing diameter of the trachea and maturation of the cartilaginous rings. However, in patients with severe respiratory compromise due to tracheomalacia, progressive pulmonary illness with severe infection and ultimately loss of life can be expected.

Options for treatment

Other than supportive care, including treatment of upper respiratory tract infections, medical management for tracheomalacia includes use of ipratropium bromide and, in severe cases, continuous positive airway pressure, or CPAP

Rationale for treatment in this case

The rationale for aortopexy resides in the severity of the condition being treated. In children with severe respiratory compromise due to compression of the trachea by the innominate artery, aortopexy is the only viable treatment option. Only after the child has failed more conservative measures is aortopexy recommended. While prior attempts have been made at endobronchial stenting, the high complication rate associated with that procedure has caused it to fall into disuse.

Special consideration

Obstruction of the main bronchus by the innominate artery must be documented to ensure that the appropriate patients are being selected for surgery. Prior cases of tracheoesophageal repair require documentation that the fistula has not recurred.

Discussion

Tracheomalacia may be subdivided into primary and secondary classifications. Primary tracheomalacia is a congenital absence of the trachea-supporting cartilage normally present, while secondary tracheomalacia is a result of some external insult to the trachea, such as the previously mentioned tracheoesophageal fistula. In addition, vascular rings and an aberrant innominate artery are also causes of secondary tracheomalacia. While classification is helpful in understanding the disease process, a successful aortopexy relies on the diagnosis of an obstructing innominate artery, regardless of the classification. The first description of the diagnosis and treatment of compression of the trachea due to an anomalous innominate artery was published over sixty years ago1. Subsequently, tracheomalacia associated with tracheoesophageal fistula2,3 has been added as an aetiology of tracheomalacia treatable with aortopexy. The traditional approach to aortopexy has been via a thoracotomy incision, where access to the mediastinum is gained by incising the pleura, done either through the left or right chest. Multiple other approaches have been described in addition to the thoracotomy5, including thoracoscopic and partial sternotomy, with no approach having a clearly better outcome with respect to the others, although the true comparison is difficult due to the rarity of the disease and the procedure. Due to its high natural resolution rate, breathing difficulties due to tracheomalacia should be treated surgically only in very select patients, after all, non-operative options have been explored and/or exhausted. The recovery from the procedure itself is relatively rapid, and other than the failure of the surgery to correct the respiratory compromise, long-term complications are few but tend to be musculoskeletal in nature and more commonly associated with the thoracotomy approach6.

Equipment

A paediatric chest wall retractor is needed, as well as an infant bronchoscopy set.

There is a Video on this link (But I don’t want to show it here)

jomi.com/article/10/aortopexy-innominate-artery-compression-trachea/

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What is Stridor? (Noisy Breathing)

Stridor is noisy breathing that occurs due to obstructed airflow through a narrowed airway. Stridor is not in and of itself a diagnosis, but rather is a symptom or sign that points to a specific airway disorder. The timing and the sound of your child’s noisy breathing provides clues to the type of airway disorder: CLICK ON BOTH LINKS

www.chop.edu/conditions-diseases/stridor-noisy-breathing#.VhlXUSv582Y

patient.info/doctor/stridor

Recognising croup and stridor in children

www.americannursetoday.com/recognizing-croup-and-stridor-in-children/

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Hiatus hernia

hytias hurn

A hiatus hernia is when part of the upper stomach slides upwards into the chest. It does this by pushing through the natural opening in the diaphragm muscle through which the oesophagus (the pipe that goes from the mouth to the stomach) passes. Hiatus hernias are common, especially in people over 50.

Your stomach usually sits completely below your diaphragm. The diaphragm is the sheet of muscle that separates your chest (where your lungs and heart are) from your abdomen (tummy).

hytias hurn. 2

Your diaphragm has an opening in it called the hiatus. Your oesophagus passes through the hiatus. A hiatus hernia develops when part of your stomach slides back up through the hiatus and into your chest.

A hiatus hernia is very common – around three in 10 people over 50 will get it. However, this number may be higher, because many people with a hiatus hernia have very mild symptoms or none at all, and may not realise that they have the condition.

How a sliding hiatus hernia forms

When the junction between the oesophagus and the stomach, as well as a portion of the stomach itself, slides up above the diaphragm

youtu.be/vYP1mHLEGEw

There are two types of a hiatus hernia, a sliding hiatus hernia and a rolling hiatus hernia. Around nine out of 10 people with a hiatus hernia have the sliding type. This is when both the stomach and the junction where your oesophagus and stomach meet slide upwards into your chest. A rolling hiatus hernia is where just part of your stomach pushes up into the chest.

How a rolling hiatus hernia forms

When a portion of your stomach goes through the diaphragm and lies beside the oesophagus

youtu.be/imgDFUjQ4Vc

Symptoms of hiatus hernia

Most hiatus hernias don’t cause any symptoms. If you do get symptoms, they are unlikely to be serious.

A sliding hiatus hernia can cause a problem called gastro-oesophageal reflux disease (GORD). This is when acid or bile in the stomach rises back up into your oesophagus.

The most common symptom of GORD is heartburn, which causes a warm or burning sensation in your chest. The burning feeling from heartburn can go all the way up to your throat. You may be more likely to notice heartburn after smoking, drinking alcohol or coffee, or eating chocolate. It can also get worse when you bend over or when you’re lying down.

Other symptoms of a hiatus hernia and GORD include:

  • feeling sick or being sick
  • a cough or wheezing when breathing, especially at night – this is caused by breathing in the acid that has come up from your stomach
  • your mouth filling with saliva
  • finding it difficult or painful to swallow

You can have a hiatus hernia without GORD, and you can have GORD without having a hiatus hernia, but they often go together.

Treatment of hiatus hernia

Treatment for a hiatus hernia generally aims to ease the symptoms of acid reflux. The treatment you need will depend on how severe your symptoms are and how much they are affecting you.

hytias hurn. 3

Self-help

You can reduce the symptoms of a hiatus hernia and prevent further problems by making the following lifestyle changes.

  • If you’re overweight or obese, lose excess weight.
  • Eat small meals often and try not to eat just before you go to bed.
  • Giving up smoking and cutting down on alcohol will help.
  • Wear loose comfortable clothes.
  • Raise the head of your bed and try not to stoop or bend down.
  • Cut down or stop eating food or drinks that make your symptoms worse. See our frequently asked questions for more information.

Medicines

You can take medicines to help reduce the symptoms of reflux from a sliding hiatus hernia. The most common are antacids, which work by neutralising the acid in your stomach. How well antacids work varies from person to person. If you find they don’t relieve your symptoms, your GP or pharmacist may suggest a different medicine. The two main types that you may be prescribed are called H2 receptor antagonists and proton pump inhibitors. Both of these reduce the amount of acid produced by your stomach.

Surgery

If you have tried other treatments and they haven’t worked, or your symptoms are severe, your doctor may suggest an operation. You will also need to have surgery if a rolling hernia becomes strangulated.

There are a number of different types of operation to repair a hiatus hernia. They involve putting the gastro-oesophageal junction back into your abdomen and tightening up the hiatus (the opening).

Your surgeon will also strengthen the oesophagal sphincter by wrapping the stomach around it. The oesophageal sphincter is a bundle of muscles which stops what is in your stomach from coming back out. This operation is called a fundoplication and is usually done as a laparoscopic (keyhole) procedure.

What is Dysmotility

Dysmotility is a condition in which muscles of the digestive system become impaired and changes in the speed, strength or coordination in the digestive organs occurs. In the normal.

small intestine, liquefied food and secretions including digestive enzymes are pushed onwards by waves of muscular contraction. When these contractions are impaired, the contents are trapped and cause distention with symptoms such as bloating, nausea, vomiting and even malnutrition. There are many causes of abnormal intestinal motility that can be separated into two different groups.

www.ddc.musc.edu/public/organs/smallBowel.html

abnormalities of the muscle of the intestine (myopathy)

abnormalities in the neural (nerve) control of the muscle (neuropathy)

WATCH:-www.youtube.com/watch?t=11&v=hHASSPQtVPUNerve and muscle abnormalities may be genetically inherited. In this situation, it is common to find many patients’ family members with contraction problems, not only of the intestinal muscle but also of the bladder. There are secondary causes of intestinal dysmotility. Examples of this include systemic Lupus erythematosus, amyloidosis, neurofibromatosis, Parkinson’s disease, diabetes, scleroderma, thyroid disorders, and muscular dystrophies. Certain medications can also cause intestinal dysmotility. Additionally, any disorders that cause inflammation to the intestine can cause significant dysmotility (i.e. radiation therapy, celiac disease)

www.ddc.musc.edu/public/symptomsDiseases/diseases/smallBowel/celiacSprue.html

www.ddc.musc.edu/public/symptomsDiseases/diseases/smallBowel/amyloidosis.html

Symptoms

Symptoms may be absent or trivial. Some patients may have severe ongoing attacks due to a blockage is known as an intestinal pseudo-obstruction. Patients who develop bacterial overgrowth because of poor motility may develop diarrhoea, while others may be constipated. Associated disorders of motility in other organs such as the oesophagus, stomach and colon will produce other symptoms. Patients with severe dysmotility will develop malnutrition because they are unable to eat adequately.

Diagnosis

Barium X-ray showing the small bowel.

Blood Tests Blood tests help to assess the degree of malnutrition, anaemia and any salt imbalance. They may also make a specific diagnosis such as diabetes, thyroid problems and systemic disorders such as Lupus.

X-ray Studies X-ray studies (barium) help to delineate the extent of bowel involvement by demonstrating areas of distended intestines, and by excluding mechanical obstruction. This must be done because treatment of pseudo-obstruction and true obstruction are very different.

www.ddc.musc.edu/public/tests/diagnosticRadiology/bariumMeal.html

Motility/Transit Studies Motility/transit investigations help to define the degree of contractile abnormality as well as a propulsive abnormality of the intestines. They are sometimes useful (by assessing the pattern of contraction) in deciding whether a neuropathy or myopathy is present.

Transit Time Measurement

A transit time X-ray measures the time it takes for food to go through the colon. The person takes special capsules by mouth twice a day for five days. Each capsule has twelve X-ray markers. Each day, the person takes a total of twenty-four markers. This averages out to one every hour.

On the sixth day, an X-ray of the abdomen is taken and we count the number of markers left in the person’s colon. This tells about how long it takes for food to go through the colon. If there are eighty-six markers, it takes eighty-six hours. If there are only twenty-four markers, it takes twenty-four hours.

The average normal transit time is thirty-five hours. The upper limit of normal is seventy-two hours. If it takes more than seventy-two hours to go through the body, and all other tests are normal, then we make the diagnosis of colonic inertia (slow transit).

www.ddc.musc.edu/public/symptomsDiseases/diseases/colon/colonicInertia.html

It is important to avoid the use of all laxatives and enemas during the five days before a transit time X-ray. However, it is fine to continue Natural Vegetable Powder, or other fibre supplements, in a normal dose.

www.ddc.musc.edu/public/procedures/upperEndoscopy.html

WATCH :- www.youtube.com/watch?v=ETiWBHORNCg

Biopsies Biopsy samples of the intestine are obtained at endoscopy or surgery, and may detect the cause of the dysmotility.

Treatment

Mechanical obstruction (i.e. blockage) must be excluded before a patient is diagnosed as having pseudo-obstruction or intestinal dysmotility as a cause of their symptoms. Specific treatments are available for some causes of dysmotility, including abnormalities in salt balance and endocrine problems such as thyroid disease.

Unfortunately, many causes of dysmotility cannot be cured, and symptomatic treatment is offered. Medicines can stimulate intestinal motility and help with propulsion of intestinal contents. Dietary modifications are advised. It is important that adequate calories are taken, usually in the form of meal supplements. Patients should avoid gas forming foods, carbonated beverages, and foods that are difficult to digest. Patients may need to be admitted to hospital for intravenous fluids, and decompression of the intestine with a tube placed in the stomach. Occasionally, nutrition will have to be supplied through a vein.

When only a short segment of the small intestine is involved, surgical resection may be appropriate. However, patients must be carefully selected as surgery can lead to scarring (adhesions) within the abdominal cavity, with further disturbance of intestinal motility.

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Recurrent Tracheoesophageal Fistula

2014

Diagnosing recurrent tracheoesophageal fistula (TEF) in a child with congenital oesophagal atresia repair during the neonatal period who has recurrent or persistent respiratory symptoms is a real challenge, since medium leakage in the gastrointestinal tract may be missed on standard contrast studies. We present a case that illustrates the importance of high-pressure administration of contrast medium in the oesophagus via a catheter, for visualisation of recurrent TEF, and the additional utility of flexible bronchoscopy for diagnosis.

A 3 ½-year-old boy was evaluated for recurrent lower respiratory tract infections. He had a history of congenital oesophagal atresia with distal TEF repair in the neonatal period. At the age of 2 ½ years, the child was hospitalised for complicated pneumonia with pneumothorax and pneumomediastinum, requiring admission to the intensive care unit and chest tube placement. Chest CT showed diffuse ground glass opacities and areas of atelectasis in both lungs. In view of the seriousness of the clinical signs, recurrent TEP was considered in the differential diagnosis. The patient had a history of anastomotic leakage, a known risk factor for fistula recurrence,3 yet there were no signs of contrast medium leak or oesophagal stenosis in the gastroduodenal tract.

FULL REPORT

http://www.archbronconeumol.org/en/recurrent-tracheoesophageal-fistula-in-children/articulo/S1579212914003486/

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Schatzki Ring or Schatzki-Gary ring

Jun 17, 2016

Schatzki rings are fixed anatomic mucosal ring structures in the distal oesophagus. They are distinct from lower oesophagal muscular rings (A rings), which are transient, smooth, circumferential indentations in the oesophagus. Lower oesophagal rings were described first by Templeton in 1944. The vast majority of Schatzki ringspatients with lower oesophagal rings are asymptomatic. In fact, lower oesophagal rings are found during 6-14% of all routine upper gastrointestinal barium examinations. Lower oesophagal rings that cause symptoms (true Schatzki rings) occur in only 0.5% of patients undergoing these examinations. Schatzki rings are a common cause of intermittent dysphagia. Barium esophagography is the preferred method of detection of Schatzki rings. Oesophagal rings may be missed during barium esophagography if distention of the distal oesophagus is inadequate; therefore, adequate distention of the oesophagus during images Schatzki ringsbarium esophagography is imperative. A modified Valsalva manoeuvre also may help in demonstrating the ring by distending the associated hiatal hernia. (As seen above on this page) Some people may have a Schatzki’s ring since birth. Sometimes, it is due to Gastro Esophageal Reflux Disease (GERD). Structuring, or narrowing, can be caused by acid in the stomach entering up into the oesophagus causing the oesophagus tissue to scar. Scar tissue may develop into a narrowing or tightness that could form a structure which sometimes causes food, pills and/or liquids to get stuck.

For more on this

http://www.gandhofcny.com/education/diseases/esophageal-ring-stricture/

http://healthool.com/schatzki-ring/

Read from Patient’s themselves

http://www.medicinenet.com/schatzki_ring/patient-comments-1645.htm

The most famous and common ring in the oesophagal  is the B ring or Schatzki ring. The pathogenesis and treatment of oesophagal rings and webs are evolving. Most of these structural lesions are asymptomatic but can cause dysphagia. Some authors believe the Schatzki ring is a protective barrier against gastroesophageal reflux (GER) Esophageal rings usually exist as a single lesion but can be multiple, as shown in the photo on left. tef ringSeveral names have been coined for when multiple rings are found in the oesophagal, including multiple oesophagal rings or webs, congenital oesophagal  stenosis, ringed oesophagal, corrugated oesophagal, and feline oesophagal. Oesophagal rings and webs are folds that block your oesophagal either partially or completely. Rings are bands of normal oesophagal tissue that form constrictions around the inside of the oesophagal. They occur in the lower oesophagus. Webs, which arise in the upper esophagus, are thin layers of cells that grow across the inside of the oesophagus. Either condition may make it difficult to swallow solid food. Experts aren’t sure what causes oesophagal rings and webs. The condition may be congenital (inherited) or may develop after birth. Most esophageal rings and webs do not cause any symptoms, and are discovered when people have barium X-rays or endoscopy for unrelated reasons. When rings or webs do cause symptoms, the most common complaint is difficulty swallowing solids. Foods, especially meats and breads, may feel like they get stuck in the same place. People with oesophagal rings and webs commonly have reflux symptoms. When esophageal webs occur together with iron deficiency anaemia the condition is known as Plummer-Vinson syndrome.

The association of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia is known as Plummer-Vinson syndrome (PVS) in the United States and Paterson-Brown-Kelly syndrome in the United Kingdom. The term sideropenic dysphagia has also been used because the syndrome can occur with iron deficiency (sideropenia), but it is not associated with anaemia.

Cricoid ring 2017

Background:

Knowledge regarding the shape, size, and variability of the cricoid ring is important to properly choose the correct endotracheal tube (ETT) in the pediatric patient. Studies have measured the size of the cricoid ring using methodologies such as moulages, magnetic resonance imaging, and video-bronchoscopy. In the present study, computed tomography (CT) -based images were used to determine the shape, size, and configuration of the cricoid ring in the pediatric population taking into considerations growth and development. This is a retrospective review using 130 CT images of children ranging in age from 1 month to 10 years undergoing radiological evaluation unrelated to airway symptomatology. The CT scans were obtained in spontaneously breathing patients during either natural sleep or procedural sedation. Anteroposterior (AP) and transverse (T) diameters were measured at the cricoid ring using these images.

READ FULL Report here very long

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5389240/

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