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{{Infobox medical condition
{{Infobox medical condition (new)
| Nme = Tracheoesophageal fistula
| name = Tracheoesophageal fistula
| Image = Tracheoesophageal_Fistula_Types.JPG
| synonyms =
| image = Tracheoesophageal_Fistula_Types.svg
| Caption =
| caption =
| DiseasesDB = 30034
| pronounce =
| field =
⚫
| ICD10 = {{ICD10|J|95|0|j|95}}, {{ICD10|Q|39|1|q|38}}-{{ICD10|Q|39|2|q|38}}
| ICD9 = {{ICD9|530.84}}, {{ICD9|750.3}}
| symptoms =
| ICDO =
| complications =
| OMIM =
| onset =
| MedlinePlus =
| duration =
| types =
⚫
| eMedicineSubj = med
| causes =
⚫
| eMedicineTopic = 3416
| MeshID = D014138
| risks =
| diagnosis =
| differential =
| prevention =
| treatment =
| medication =
| prognosis =
| frequency =
| deaths =
}}
}}
A '''tracheoesophageal fistula''' ('''TEF''', or '''TOF'''; see [[American and British English spelling differences#Simplification of ae and oe|spelling differences]]) is an abnormal connection ([[fistula]]) between the [[esophagus]] and the [[Vertebrate trachea|trachea]]. TEF is a common [[congenital]] abnormality, but when occurring late in life is usually the sequela of surgical procedures such as a [[laryngectomy]].
A '''tracheoesophageal fistula''' ('''TEF''', or '''TOF'''; see [[American and British English spelling differences#ae and oe|spelling differences]]) is an abnormal connection ([[fistula]]) between the [[esophagus]] and the [[Vertebrate trachea|trachea]]. TEF is a common [[congenital]] abnormality, but when occurring late in life is usually the sequela of surgical procedures such as a [[laryngectomy]].


==Causes==
==Presentation==
[[File:H-Fistel neugeb 05032014.jpg|thumb|Radiograph with oral contrast showing h-type tracheoesophageal fistula in a newborn]]
⚫
Congenital TEF can arise due to failed fusion of the [[tracheoesophageal ridges]] after the fourth week of embryological development.<ref name="pmid10068713">{{cite journal |author=Clark DC |title=Esophageal atresia and tracheoesophageal fistula |journal=American Family Physician |volume=59 |issue=4 |pages=910–6, 919–20 |date=February 1999 |pmid=10068713 |doi= |url=http://www.aafp.org/afp/990215ap/910.html}}</ref>
⚫
Tracheoesophageal fistula is suggested in a newborn by copious [[salivation]] associated with [[choking]], [[coughing]], vomiting, and [[cyanosis]] coincident with the onset of feeding. Esophageal atresia and the subsequent inability to swallow typically cause [[polyhydramnios]] in utero. Rarely it may present in an [[adult]].<ref>{{cite journal | author = Newberry D., Sharma V., Reiff D., De Lorenzo F. | year = 1999| title = A "little cough" for 40 years | journal = Lancet | volume = 354 | issue = 9185| page = 1174 | pmid = 10513712 | doi = 10.1016/s0140-6736(99)10014-x | s2cid = 39432557}}</ref>


===Complications===
⚫
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-esophageal fistula it runs between the trachea and the esophagus. This connection may or may not have a central cavity; if it does, then food within the esophagus may pass into the trachea (and on to the lungs) or alternatively, air in the trachea may cross into the esophagus.
Surgical repair can sometimes result in complications, including:{{cn|date=May 2022}}
⚫
* [[stenosis|Stricture]], due to [[gastric acid]] erosion of the shortened esophagus
⚫
* Leak of contents at the point of anastomosis
⚫
* Recurrence of fistula
* [[Gastro-esophageal reflux disease]]
* [[Dysphagia]]
* [[Asthma]]-like symptoms, such as persistent coughing/wheezing
* Recurrent [[lower respiratory tract infection|chest infections]]
* [[Tracheomalacia]]


⚫
===Associations===
⚫
TEF can also occur due to pressure [[necrosis]] by a [[tracheostomy tube]] in apposition to a [[nasogastric tube]] (NGT).<ref>Dr. Lorne H. Blackbourne, ''Advanced Surgical Recall, 3rd Ed.'', pg. 206.</ref>

⚫
==Associations==


Neonates with TEF or esophageal atresia are unable to feed properly. Once diagnosed, prompt surgery is required to allow the food intake. Some children do experience problems following TEF surgery; they can develop dysphagia and thoracic problems. Children with TEF can also be 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. 6% of babies with TEF also have a [[laryngeal cleft]].<ref>{{Cite book
Neonates with TEF or esophageal atresia are unable to feed properly. Once diagnosed, [[Surgery|prompt surgery]] is required to allow the food intake.<ref name=":0">{{Cite web|date=2017-10-18|title=Oesophageal atresia and tracheo-oesophageal fistula|url=https://www.nhs.uk/conditions/oesophageal-atresia/|access-date=2020-11-13|website=nhs.uk|language=en}}</ref> Some children experience problems following TEF surgery; they can develop dysphagia and thoracic problems. Children with TEF can also be 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. 6% of babies with TEF also have a [[laryngeal cleft]].<ref>{{Cite book | last = Bluestone | first = Charles D. | title = Pediatric otolaryngology, Volume 2 | publisher = [[Elsevier Health Sciences]] | year = 2003 | pages = 1468 | url = https://books.google.com/books?id=y5rpEdeKtJEC&pg=PA1469 | isbn =0-7216-9197-8 }}
| last = Bluestone
| first = Charles D.
| title = Pediatric otolaryngology, Volume 2
| publisher = [[Elsevier Health Sciences]]
| year = 2003
| pages = 1468
| url = https://books.google.com/books?id=y5rpEdeKtJEC&pg=PA1469
| isbn =0-7216-9197-8 }}
</ref>
</ref>


==Classification==
==Cause==
⚫
Congenital TEF can arise due to failed fusion of the [[tracheoesophageal ridges]] after the fourth week of [[Prenatal development|embryological development]].<ref name="pmid10068713">{{cite journal |author=Clark DC |title=Esophageal atresia and tracheoesophageal fistula |journal=American Family Physician |volume=59 |issue=4 |pages=910–6, 919–20 |date=February 1999 |pmid=10068713 |url=http://www.aafp.org/afp/990215ap/910.html}}</ref>
⚫
Fistulae between the trachea and esophagus in the newborn can be of diverse morphology and anatomical location;<ref name="pmid17498283">{{cite journal |author=Spitz L |title=Oesophageal atresia |journal=Orphanet journal of rare diseases |volume=2 |issue= 1|pages=24 |year=2007 |pmid=17498283 |doi=10.1186/1750-1172-2-24 |pmc=1884133}}</ref><ref name="pmid15364774">{{cite journal |vauthors=Kovesi T, Rubin S |title=Long-term complications of congenital esophageal atresia and/or tracheoesophageal fistula |journal=Chest |volume=126 |issue=3 |pages=915–25 |year=2004 |pmid=15364774 |doi=10.1378/chest.126.3.915}}</ref> however, various pediatric surgical publications have attempted a classification system based on the below specified types.


⚫
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-esophageal fistula it runs between the trachea and the esophagus. This connection may or may not have a central cavity; if it does, then food within the esophagus may pass into the trachea (and on to the lungs) or alternatively, air in the trachea may cross into the esophagus.{{citation needed|date=August 2020}}
⚫
Not all types include both esophageal agenesis and tracheoesophageal fistula, but the most common types do.

⚫
TEF can also occur due to pressure [[necrosis]] by a [[tracheostomy tube]] in apposition to a [[nasogastric tube]] (NGT).<ref>Dr. Lorne H. Blackbourne, ''Advanced Surgical Recall, 3rd Ed.'', pg. 206.</ref>

==Diagnosis==
TEF should be suspected once the baby fails to swallow after their first feeding during the first day of life. Esophageal atresia can be diagnosed by Ryle nasogastric tube; if the Ryle fails to pass into the stomach, then this indicates esophageal atresia and loss of communication between stomach and esophagus. TEF may be diagnosed by MRI which clarifies the atretic esophagus (if presents) and TEF, as well as its location and anatomy. Gastrograffin contrast swallow should not be used if TEF is suspected, due to its high risk of allergy and severe intractable chest infection.{{citation needed|date=August 2020}}

===Classification===
⚫
Fistulae between the trachea and esophagus in the newborn can be of diverse morphology and anatomical location.<ref name="pmid17498283">{{cite journal |author=Spitz L |title=Oesophageal atresia |journal=Orphanet Journal of Rare Diseases |volume=2 |issue= 1|pages=24 |year=2007 |pmid=17498283 |doi=10.1186/1750-1172-2-24 |pmc=1884133 |doi-access=free }}</ref><ref name="pmid15364774">{{cite journal |vauthors=Kovesi T, Rubin S |title=Long-term complications of congenital esophageal atresia and/or tracheoesophageal fistula |journal=Chest |volume=126 |issue=3 |pages=915–25 |year=2004 |pmid=15364774 |doi=10.1378/chest.126.3.915}}</ref> However, various pediatric surgical publications have attempted a classification system based on the below specified types.{{cn|date=May 2022}}

⚫
Not all types include both esophageal agenesis and tracheoesophageal fistula, but the most common types do.{{cn|date=May 2022}}


{| class="wikitable"
{| class="wikitable"
| '''Gross''' || '''Vogt'''<ref name="urlLong-term Complications of Congenital Esophageal Atresia and/or Tracheoesophageal Fistula -- Kovesi and Rubin 126 (3): 915 Figure 1 -- Chest">{{cite web |url=http://www.chestjournal.org/cgi/content/full/126/3/915/F1 |title=Long-term Complications of Congenital Esophageal Atresia and/or Tracheoesophageal Fistula -- Kovesi and Rubin 126 (3): 915 Figure 1 -- Chest |work= |accessdate=2008-10-11}}</ref> || '''Description''' || '''EA?''' || '''TEF?'''
! Gross || Vogt<ref name="urlLong-term Complications of Congenital Esophageal Atresia and/or Tracheoesophageal Fistula -- Kovesi and Rubin 126 (3): 915 Figure 1 -- Chest">{{cite web |url=http://www.chestjournal.org/cgi/content/full/126/3/915/F1 |title=Long-term Complications of Congenital Esophageal Atresia and/or Tracheoesophageal Fistula -- Kovesi and Rubin 126 (3): 915 Figure 1 -- Chest |access-date=2008-10-11}}</ref> || Description || EA? || TEF?
|-
|-
| - || Type 1 || Esophageal agenesis. Very rare, and not included in the classification by Gross.<ref name="isbn3-540-40738-3">{{cite book |author= P. Puri |editor= M. E. Höllwarth|title=Pediatric Surgery (Springer Surgery Atlas Series) |publisher=Springer |location=Berlin |year=2005 |isbn=3-540-40738-3 |oclc= |doi= |accessdate= |page=30}}</ref> || Yes || No
| - || Type 1 || Esophageal agenesis. Very rare, and not included in the classification by Gross.<ref name="isbn3-540-40738-3">{{cite book |author= P. Puri |editor= M. E. Höllwarth|title=Pediatric Surgery (Springer Surgery Atlas Series) |publisher=Springer |location=Berlin |year=2005 |isbn=3-540-40738-3 |page=30}}</ref> || Yes || No
|-
|-
| Type A || Type 2 || Proximal and distal esophageal bud—a normal esophagus with a missing mid-segment. || Yes || No
| Type A || Type 2 || Proximal and distal esophageal bud—a normal esophagus with a missing mid-segment. || Yes || No
|-
|-
| Type B || Type 3A || Proximal esophageal termination on the lower trachea with distal esophageal bud. || Yes || Yes
| Type B || Type 3A || Proximal esophageal termination on the lower trachea with distal esophageal bud. || Yes || Yes
|-
|-
| '''Type C''' || Type 3B || 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 of trachea|carina]]. ('''Most common''', up to 90% of cases.) || Yes || Yes
| '''Type C''' || Type 3B || 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 of trachea|carina]]. ('''Most common''', up to 90% of cases.) || Yes || Yes
|-
|-
| Type D || Type 3C || Proximal esophageal termination on the lower trachea or carina with distal esophagus arising from the carina. || Yes || Yes
| Type D || Type 3C || Proximal esophageal termination on the lower trachea or carina with distal esophagus arising from the carina. || Yes || Yes
|-
|-
| Type E (or H-Type) || - || A variant of type D: if the two segments of esophagus communicate, this is sometimes termed an ''H-type'' fistula due to its resemblance to the letter H. TEF without EA. || No || Yes
| Type E (or H-Type) || - || A variant of type D: if the two segments of esophagus communicate, this is sometimes termed an ''H-type'' fistula due to its resemblance to the letter H. TEF without EA. || No || Yes
|}
|}
Line 58: Line 77:
The letter codes are usually associated with the system used by Gross,<ref>Gross, RE. The surgery of infancy and childhood. Philadelphia, WB Saunders; 1953.</ref> while number codes are usually associated with Vogt.<ref>Vogt EC. Congenital esophageal atresia. Am J of Roentgenol. 1929;22:463–465.</ref>
The letter codes are usually associated with the system used by Gross,<ref>Gross, RE. The surgery of infancy and childhood. Philadelphia, WB Saunders; 1953.</ref> while number codes are usually associated with Vogt.<ref>Vogt EC. Congenital esophageal atresia. Am J of Roentgenol. 1929;22:463–465.</ref>


An additional type, "blind upper segment only" has been described,<ref name="isbn0-7216-0187-1">{{cite book |author1=Cotran, Ramzi S. |author2=Kumar, Vinay |author3=Fausto, Nelson |author4=Nelso Fausto |author5=Robbins, Stanley L. |author6=Abbas, Abul K. |title=Robbins and Cotran pathologic basis of disease |publisher=Elsevier Saunders |location=St. Louis, Mo |year=2005 |isbn=0-7216-0187-1 |oclc= |doi= |accessdate= |page=800}}</ref> but this type is not usually included in most classifications.
An additional type, "blind upper segment only" has been described,<ref name="isbn0-7216-0187-1">{{cite book |author1=Cotran, Ramzi S. |author2=Kumar, Vinay |author3=Fausto, Nelson |author4=Nelso Fausto |author5=Robbins, Stanley L. |author6=Abbas, Abul K. |title=Robbins and Cotran pathologic basis of disease |publisher=Elsevier Saunders |location=St. Louis, Mo |year=2005 |isbn=0-7216-0187-1 |page=800}}</ref> but this type is not usually included in most classifications. (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.)

(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.)

==Clinical presentation==
⚫
Tracheoesophageal fistula is suggested in a newborn by copious [[salivation]] associated with [[choking]], [[coughing]], vomiting, and [[cyanosis]] coincident with the onset of feeding. Esophageal atresia and the subsequent inability to swallow typically cause [[polyhydramnios]] in utero. Rarely it may present in an adult.<ref>D. Newberry, V. Sharma, D. Reiff, and F. De Lorenzo. A "little cough" for 40 years. Lancet, 354(9185):1174, Oct 1999. PMID 10513712</ref>


==Treatment==
==Treatment==
It is surgically corrected, with [[Segmental resection|resection]] of any fistula and [[anastomosis]] of any discontinuous segments. Surgical repair is associated with complications, including
It is surgically corrected, with [[Segmental resection|resection]] of any fistula and [[anastomosis]] of any discontinuous segments.<ref name=":0" /> Babies often need to spend time in a [[neonatal intensive care unit]] for feeding with a [[stomach]] [[feeding tube]].<ref name=":0" /> [[Antibiotic]]s, [[Pain management|pain relief]], a [[Chest tube|chest drain]], [[Oxygen therapy|oxygen]], and [[Ventilator|ventilation]] may all be needed.<ref name=":0" />
⚫
* [[stenosis|Stricture]], due to gastric acid erosion of the shortened esophagus.
⚫
* Leak of contents at the point of anastomosis.
⚫
* Recurrence of fistula.
* Increased gastro oesophageal reflux.


==References==
==References==
{{reflist|2}}
{{Reflist}}


== External links ==
== External links ==
*{{commonscatinline}}
*[http://www.tofs.org.uk/ TOFS: Tracheoesophageal Fistula Support Group]
{{Medical resources
* {{EmbryologySwiss|rrespiratory/patholrespi01}}
| DiseasesDB = 30034
⚫
| ICD10 = {{ICD10|J|95|0|j|95}}, {{ICD10|Q|39|1|q|38}}-{{ICD10|Q|39|2|q|38}}
| ICD9 = {{ICD9|530.84}}, {{ICD9|750.3}}
| ICDO =
| OMIM =
| MedlinePlus =
⚫
| eMedicineSubj = med
⚫
| eMedicineTopic = 3416
| MeshID = D014138
}}


{{Congenital malformations and deformations of digestive system}}
{{Congenital malformations and deformations of digestive system}}

Latest revision as of 12:33, 12 August 2024

Tracheoesophageal fistula
SpecialtyMedical genetics Edit this on Wikidata

A tracheoesophageal fistula (TEF, or TOF; see spelling differences) 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.

Presentation

[edit]
Radiograph with oral contrast showing h-type tracheoesophageal fistula in a newborn

Tracheoesophageal fistula is suggested in a newborn by copious salivation associated with choking, coughing, vomiting, and cyanosis coincident with the onset of feeding. Esophageal atresia and the subsequent inability to swallow typically cause polyhydramnios in utero. Rarely it may present in an adult.[1]

Complications

[edit]

Surgical repair can sometimes result in complications, including:[citation needed]

Associations

[edit]

Neonates with TEF or esophageal atresia are unable to feed properly. Once diagnosed, prompt surgery is required to allow the food intake.[2] Some children experience problems following TEF surgery; they can develop dysphagia and thoracic problems. Children with TEF can also be 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. 6% of babies with TEF also have a laryngeal cleft.[3]

Cause

[edit]

Congenital TEF can arise due to failed fusion of the tracheoesophageal ridges after the fourth week of embryological development.[4]

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-esophageal fistula it runs between the trachea and the esophagus. This connection may or may not have a central cavity; if it does, then food within the esophagus may pass into the trachea (and on to the lungs) or alternatively, air in the trachea may cross into the esophagus.[citation needed]

TEF can also occur due to pressure necrosis by a tracheostomy tube in apposition to a nasogastric tube (NGT).[5]

Diagnosis

[edit]

TEF should be suspected once the baby fails to swallow after their first feeding during the first day of life. Esophageal atresia can be diagnosed by Ryle nasogastric tube; if the Ryle fails to pass into the stomach, then this indicates esophageal atresia and loss of communication between stomach and esophagus. TEF may be diagnosed by MRI which clarifies the atretic esophagus (if presents) and TEF, as well as its location and anatomy. Gastrograffin contrast swallow should not be used if TEF is suspected, due to its high risk of allergy and severe intractable chest infection.[citation needed]

Classification

[edit]

Fistulae between the trachea and esophagus in the newborn can be of diverse morphology and anatomical location.[6][7] However, various pediatric surgical publications have attempted a classification system based on the below specified types.[citation needed]

Not all types include both esophageal agenesis and tracheoesophageal fistula, but the most common types do.[citation needed]

Gross Vogt[8] Description EA? TEF?
- Type 1 Esophageal agenesis. Very rare, and not included in the classification by Gross.[9] Yes No
Type A Type 2 Proximal and distal esophageal bud—a normal esophagus with a missing mid-segment. Yes No
Type B Type 3A Proximal esophageal termination on the lower trachea with distal esophageal bud. Yes Yes
Type C Type 3B 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.) Yes Yes
Type D Type 3C Proximal esophageal termination on the lower trachea or carina with distal esophagus arising from the carina. Yes Yes
Type E (or H-Type) - A variant of type D: if the two segments of esophagus communicate, this is sometimes termed an H-type fistula due to its resemblance to the letter H. TEF without EA. No Yes

The letter codes are usually associated with the system used by Gross,[10] while number codes are usually associated with Vogt.[11]

An additional type, "blind upper segment only" has been described,[12] but this type is not usually included in most classifications. (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.)

Treatment

[edit]

It is surgically corrected, with resection of any fistula and anastomosis of any discontinuous segments.[2] Babies often need to spend time in a neonatal intensive care unit for feeding with a stomach feeding tube.[2] Antibiotics, pain relief, a chest drain, oxygen, and ventilation may all be needed.[2]

References

[edit]
  1. ^ Newberry D., Sharma V., Reiff D., De Lorenzo F. (1999). "A "little cough" for 40 years". Lancet. 354 (9185): 1174. doi:10.1016/s0140-6736(99)10014-x. PMID 10513712. S2CID 39432557.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  2. ^ a b c d "Oesophageal atresia and tracheo-oesophageal fistula". nhs.uk. 2017-10-18. Retrieved 2020-11-13.
  3. ^ Bluestone, Charles D. (2003). Pediatric otolaryngology, Volume 2. Elsevier Health Sciences. p. 1468. ISBN 0-7216-9197-8.
  4. ^ Clark DC (February 1999). "Esophageal atresia and tracheoesophageal fistula". American Family Physician. 59 (4): 910–6, 919–20. PMID 10068713.
  5. ^ Dr. Lorne H. Blackbourne, Advanced Surgical Recall, 3rd Ed., pg. 206.
  6. ^ Spitz L (2007). "Oesophageal atresia". Orphanet Journal of Rare Diseases. 2 (1): 24. doi:10.1186/1750-1172-2-24. PMC 1884133. PMID 17498283.
  7. ^ Kovesi T, Rubin S (2004). "Long-term complications of congenital esophageal atresia and/or tracheoesophageal fistula". Chest. 126 (3): 915–25. doi:10.1378/chest.126.3.915. PMID 15364774.
  8. ^ "Long-term Complications of Congenital Esophageal Atresia and/or Tracheoesophageal Fistula -- Kovesi and Rubin 126 (3): 915 Figure 1 -- Chest". Retrieved 2008-10-11.
  9. ^ P. Puri (2005). M. E. Höllwarth (ed.). Pediatric Surgery (Springer Surgery Atlas Series). Berlin: Springer. p. 30. ISBN 3-540-40738-3.
  10. ^ Gross, RE. The surgery of infancy and childhood. Philadelphia, WB Saunders; 1953.
  11. ^ Vogt EC. Congenital esophageal atresia. Am J of Roentgenol. 1929;22:463–465.
  12. ^ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo: Elsevier Saunders. p. 800. ISBN 0-7216-0187-1.
[edit]
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