Tracheoesophageal fistula
From Wikipedia, the free encyclopedia
| Tracheoesophageal fistula Classification and external resources |
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| ICD-10 | J95.0, Q39.1-Q39.2 |
| ICD-9 | 530.84, 750.3 |
| DiseasesDB | 30034 |
| eMedicine | med/3416 |
| MeSH | D014138 |
A tracheoesophageal fistula (TEF) is an abnormal connection (fistula) between the esophagus and the trachea. TEF is a common congenital abnormality, but when occurring late in life is usually the sequela of surgical procedures such as a laryngectomy.
Contents |
[edit] Causes
Congenital TEF can arise due to failed fusion of the tracheoesophageal ridges during the third week of embryological development. [1]
A fistula, from the Latin meaning ‘a pipe,’ is an abnormal connection running either between two tubes or between a tube and a surface. In tracheo-oesophageal fistula it runs between the trachea and the oesophagus. This connection may or may not have a central cavity; if it does, then food within the oesophagus may pass into the trachea (and on to the lungs) or alternatively, air in the trachea may cross into the oesophagus.
[edit] Associations
Babies with TEF or EA are unable to feed properly. Once diagnosed, prompt surgery is required to allow the baby to take in food. Many TEF children have few problems after surgery, however a number develop feeding difficulties and chest problems. Some TEF babies are also born with other abnormalities, most commonly those described in VACTERL association - a group of anomalies which often occur together, including heart, kidney and limb deformities.
[edit] Classification
Fistulae between the trachea and esophagus in the newborn can be of diverse morphology and anatomical location,[2][3] however, various pediatric surgical publications have attempted a classification system comprised of the below specified types.
| Type | Description |
| Type C | Proximal esophageal atresia (esophagus continuous with the mouth ending in a blind loop superior to the sternal angle) with a distal esophagus arising from the lower trachea or carina. (Most common, up to 90% of cases.) |
| Type A | Proximal and distal esophageal bud--a normal esophagus with a missing mid-segment. |
| Type D | Proximal esophageal termination on the lower trachea or carina with distal esophagus arising from the carina. |
| Type H | A variant of type D: if the two segments of esophagus communicate, this is termed an H-type fistula due to its resemblance to the letter H. |
| Type B | Proximal esophageal termination on the lower trachea with distal esophageal bud. |
The letter codes are usually associated with the system used by Gross,[4] while number codes are usually associated with Vogt.[5]
(For the purposes of this discussion, proximal esophagus indicates normal esophageal tissue arising normally from the pharynx, and distal esophagus indicates normal esophageal tissue emptying into the proximal stomach.)
[edit] Clinical presentation
Tracheoesophageal fistula is suggested in a newborn by copious salivation associated with choking, coughing, and cyanosis coincident with the onset of feeding.
[edit] Treatment
It is surgically corrected, with resection of any fistula and anastomosis of any discontinuous segments. Surgical repair is associated with complications, including
- Stricture, due to gastric acid erosion of the shortened esophagus.
- Leak of contents at the point of anastomosis.
- Recurrence of fistula.
[edit] common problems
Every child is unique and will be affected in different ways by TOF/OA. However, there are some common problems. These are:
The “TOF cough” Children with TOF/OA often have a loud, barking cough, known as the “TOF cough”. It’s caused by a floppiness (tracheomalacia) of part of the trachea (windpipe) and can get worse when a child has a cold or other respiratory problems. Whilst it can sound alarming, it doesn’t necessarily mean the child is ill.
Respiratory problems, asthma and chest infections Babies with TOF/OA can experience short periods when they find it hard to breathe, due to a floppy trachea. This is most likely to happen when a child is breathing heavily, for example when coughing or crying. Children usually grow out of this by the age of two and then only a minority of babies suffer severely.
Babies may also experience respiratory problems such as asthma and chest infections. These are treated with inhalers or antibiotic treatments.
Swallowing and getting food stuck Children with TOF/OA often have abnormal swallowing mechanisms and will have to learn to cope with their particular feeding problems. Some children will need to receive tube feeding to make sure they stay healthy. At first, many will need a special diet, but most will (with their support of their school) cope with school dinners or a packed lunch.
Reflux Children with TOF/OA often experience gastro-oesophageal reflux (GOR). This is where the acidic stomach contents pass back into the lower oesophagus, causing pain and often reluctance to eat. Reflux can usually be treated using a combination of practical measures and prescribed medications. Occasionally it requires further surgery
[edit] Surgery
Content provided by Professor L Spitz PhD FRCS, Nuffield Professor of Paediatric Surgery, Institute of Child Health (University College London) and Great Ormond Street Hospital for Sick Children, London.
In occasional circumstances the operation can be safely postponed until the daylight hours rather than having to be carried out at a time when the surgeon may not be at his or her best and when staffing is at emergency levels. However, the lack of availability of a regular operating theatre may dictate that the surgery has to be undertaken at night rather than waiting until the following morning.
The only exception to this rule is the infant who has respiratory difficulties, particularly the premature infant with respiratory distress syndrome. Such babies often require mechanical ventilation and in the presence of a distal tracheo-oesophageal fistula, the pressures necessary to keep the stiff lungs inflated may cause some of the air to escape down the fistula into the stomach. The stomach becomes distended, worsening the respiratory status, and the stomach may even rupture, resulting in a life- threatening situation.
In this case, an emergency operation will be needed to close off the fistula. If the infant’s condition improves dramatically, the surgeon may continue with the procedure, but usually the operation is left at this stage and the repair postponed for a few days until the infant is in a stable condition.
Another circumstance which may delay surgery or dictate a different approach is where the infant has a major congenital heart abnormality. Detailed investigations of the heart will be required and medical treatment for cardiac failure instituted. Occasionally cardiac surgery may be advisable before correction of the oesophageal atresia.
Primary anastomosis The aim of surgery for oesophageal atresia is to divide the tracheo- oesophageal fistula and to perform a ‘primary anastomosis,’ joining the two ends of the oesophagus during the initial operation.
Occasionally, primary anastomosis will not be possible at the first operation because the gap between the two ends of the oesophagus is too wide.
Nowadays it is very rare for an otherwise healthy baby to die from TOF or OA. If the infant’s birthweight is greater than 1500 grams and there is no evidence of a major congenital heart anomaly, the chances of survival are close to 100%.
If the baby weighs less than 1500 grams at birth or has a major congenital heart anomaly, the chances of survival are reduced to 60%.
If the baby’s weight is below 1500 grams and there is a major heart anomaly, the chances of survival are much further reduced, to around 20-30%.
The operation Following assessment and stabilisation, the infant can be prepared for surgery. Consent for the procedure is obtained and the risks of the operation and the chances of the baby’s survival are carefully explained to the parents.
The baby is then taken to the operating theatre where anaesthesia is induced by an experienced paediatric anaesthetist.
Incision The usual incision is along a curved line on the right side of the chest close to the back to just under the tip of the shoulder blade. It should not extend close to the nipple.
Procedure The chest is entered between the fourth and fifth ribs and, without opening the membrane which encloses the lung, the area of the oesophageal atresia is approached. The fistula is carefully identified and traced towards its entry into the trachea, where it is divided and the defect in the trachea closed with fine interrupted sutures.
The proximal (upper) oesophagus is now identified with the help of the anaesthetist applying pressure on the tube in the upper oesophageal pouch. The upper pouch is mobilised sufficiently to allow anastomosis (joining) to take place with the least possible tension. If there is significant tension at the anastomosis, the infant may be electively paralysed and mechanically ventilated (i.e. put on a ‘life- support machine’) to reduce the likelihood of a leak in the anastomosis.
The anastomosis is performed using fine interrupted sutures which should provide a watertight seal.
Transanastomotic tube Most surgeons will pass a trans- anastomotic tube (trans = across; anastomosis = join) from the nose through the oesophagus into the stomach to remove any pressure in the stomach during the first few days after the operation, and to allow feeding to occur on the third or fourth postoperative day.
Chest drain Some surgeons leave a chest drain in position for up to one week, others have omitted this as it does not appear to be of any great value in detecting an anastomotic leak (a defect in the join that allows oesophageal contents to leak into the surrounding tissues).
Closure The wound is closed in layers, finishing with a stitch which is buried in the skin, underneath the surface. This type of stitch does not require removal.
Although nowadays surgeons prefer to use keyhole surgery to decrease the risk of infection and leave less scaring. Unfortunately this was not around in 1994, leaving many children with hideous scars!!!!!
[edit] Scarring
TOF/OA children can be left with many scars. If the child has air pumped into them and their stomach bursts then they will have a large scar on the stomach area, the TOF procedure -before keyhole- leaves a large scar going from the side of the chest right around to the back, tracing the shoulder blade. Feeding tubes also leave scars and if a child has more than one fitted for any reason they are left with multiple scars that resemble extra navels.
==Contacts==
TOFS is a charity involved in the following activities. To provide information and support to families of TOF, OA and VACTERL children as well as to medical professionals. A range of leaflets is offered on these conditions and a quarterly newsletter, CHEW. A book, The TOF Child, is also available. New parents who wish it are put in contact with each other. The TOFS Conference is organised every other year for both parents and medical professionals. It includes a programme of talks and seminars. TOFS is committed to funding research to determine the cause of these conditions, as well as studies of the various treatments for TOF and its complications. The TOFS website has extensive information about the group and its activities; alternatively, please contact the TOFS office at the address above.
[edit] References
- ^ Esophageal Atresia and Tracheoesophageal Fistula - February 15, 1999 - American Academy of Family Physicians. Retrieved on 2007-11-11.
- ^ Spitz L (2007). "Oesophageal atresia". Orphanet journal of rare diseases 2: 24. doi:. PMID 17498283.
- ^ Kovesi T, Rubin S (2004). "Long-term complications of congenital esophageal atresia and/or tracheoesophageal fistula". Chest 126 (3): 915–25. doi:. PMID 15364774.
- ^ Gross, RE. The surgery of infancy and chilhood. Philadelphia , WB Saunders; 1953.
- ^ Vogt EC. Congenital esophageal atresia. Am J of Roentgenol. 1929;22:463–465.
[edit] External links
- TOFS: The Tracheo Esophageal Support Group
- Swiss embryology (from UL, UB, and UF) rrespiratory/patholrespi01
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