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The History of Esophageal
Atresia and
Tracheoesophageal Fistula
The earliest description of oesophageal atresia starts over
300 years ago in the year 1670 by Dr. William Durston of
England in a paper titled "Description of A' Monstrous
Birth in Plymouth. Durston was called after the delivery
of con-joined twin girls by the attending mid-wife and one
of the twin girls had oesophageal atresia.( put link here
to article and picture)
In another paper 27 years later in 1697 a Dr. Gibson details
the First description of oesophageal atresia with distal
fistula today what is the most commonly seen type of oesophageal
atresia. The top or proximal part of the esophagus and the
lower or distal part of the oesophagus from the stomach
and through the diaphram attaches to the trachea called
oesophageal atresia with a tracheoesophageal fistula. Often
called a type C today. (put link to article)
Almost a 100 years pass and in 1793 a Dr. Hunter describes
the use of an eel skin as a gavage tube or feeding tube.
In 1821 Dr. Martin reports the second case of oesophageal
atresia and distal fistula 124 years after the first description
by Gibson.
In 1840 Dr. Thomas Hill reports on the first know case of
esophageal atresia and rectal agenesis. No anal opening
between the cheeks of your butt so you can't pass gas (commonly
called farting) nor can you poop. His description of the
birth defects of the trachea, esophagus and fistula (TEF)
including the anal defect could be considered for first
describing the multiple birth defects we see today coined
VATER Association. You could use the acronyms FATE, FEAT,
for his description. (link to article)
By the year 1841 Dr. Hirschprung had seen four cases of
oesophageal atresia with fistula adding to the 10 already
in literature. This is the physician whom Hirschprungs Disease
is named after.
In 1869 the first suggestion of surgical treatment by Dr.
Timothy Holmes on babies born with oesophageal atresia and
fistula.
A different type of fistula is described in literature with
Lambs description of the 'H' fistula in 1873. Usually there
is no missing part of the oesophagus but there is a small
connection between the oesophagus and trachea in the shape
of the letter H. An example would be when you write the
capital letter H you make two vertical lines and connect
the lines with a horizontal line. The two horizontal lines
are the oesophagus and trachea connected by horizontal line
the piece of tissue called a fistula. Today also described
as both an 'H' fistula and 'N' fistula.
Morell Mackenzie in 1880 writes an article on 'Malformations
of the Oesophagus' adding the one he has now seen to the
56 others already written in literature.
The above history describes the observation of babies born
with oesophageal atresia with and without fistula and the
beginning of babies seen with multiple congenital anomalies
( more that one birth defect). There was no surgical intervention
during this period of over 200 years to try and correct
these birth defects for many reasons, one being that there
was so little information on these birth defects no one
knew what the problem was until the baby died and then it
was after autopsy on the baby to see what went wrong inside.
"The First Operation and the Surgical Era Begins"
In 1888 in London Dr. Charles Steele performed the first
operation on a baby with oesophageal atresia. Steele goes
on to describe how he was asked to consult on a baby 24
hours old. After a sound was passed down the infants throat
about 5 inches it would go no further. It was thought either
a membrane was blocking the oesophagus or the oesophagus
ended in a blind pouch. If there was a membrane they could
open it. If two ends of the oesophagus nothing could be
done. The parents agreed to operate to give their baby a
chance at survival. The next day the baby was opened and
found to have oesophageal atresia. The operation ended ,
wound closed, the baby rested peacefully and the baby passed
24 hours later. Dr. Steele has been credited with being
the first to do a gastrotomy and others believe a gastrostomy.
(link to article)
In 1898 Hoffman operated on a baby with oesophageal atresia
and is believed to be the first to do a gastrostomy on a
baby with oesophageal atresia.
The 20th century begins and in 1913 Dr. Richter tries to
separate a baby with oesophaageal atresia with a tracheoesophageal
fistula
In 1923 Dr. Smith tries ligation of the oesophagus at the
cardia.
In 1928 Dr. Scott does a gastrostomy and divides the oesophagus
at the cardia.
In 1936 two doctors Gage and Oschner do a cervical oesophagostomy.
Open the neck on either side and bring out part of the oesophagus.
They ligate the oesophagus and do a gastrostomy.
In 1936 T. H.Lanman tried the first direct approach to correct
esophageal atresia as did Robert Shaw.
In 1938 Dr. Shaw attempts a primary anastamosis. (bringing
the two ends of the oesophagus together. I have many letters
of communication between Dr. John Alexander and Dr. Robert
Shaw who were friends. Dr. Shaw was trying to get published
in the Journal of the American Medical Association his case
of esophageal atresia. Alexander writes' I am enclosing
the disgusting reply from the J.A.M.A. about your article.
I have sent it to the Journal of Pediatrics.
Also in 1938 Dr. Gamble does a cervical oesophagostomy and
divides the stomach.
All the surgical attempts to correct the birth defect of
oesophageal atresia with or without a tracheoesophageal
fistula were not successful during this period of surgical
intervention.
The Survival Era Begins
The First survivor of Esophageal with Distal Tracheoesophageal
fistula is a baby girl and patient of Dr. William Ladd (
Father of Pediatric Surgery) in Boston in 1939. Dr. Ladd's
patient had a distal fistula and he use a staged approach
to the operation. This staged approach means that one part
of the operation is disconnecting the fistula from the lower
part of the trachea and then feeding the baby by the gastrostomy
tube and now letting the baby grow and gain strength for
the next part of the operation. (link to Millie) picture
of Ladd
In the same year and at almost the same time in Minnesota
Dr. Leven does a similar operation on a baby boy and this
boy survives. This boy was born one day before the baby
girl. The wonderful part of this story is that this kind
and gentle man Dr. Leven adopts his patient and this boy
becomes his only child. I have tried for years to locate
this patient with no luck at this time. This would make
a wonderful movie. (link to picture)
In 1941 Dr. Cameran Haight and Dr. Harry Towsley from Michigan
successfully operate on a baby girl born with esophageal
atresia with a tracheoesophageal fistula. This is the first
time the lower part of the esophagus is disconnected and
rejoined to the top part of the esophagus called a primary
anastamosis (sewing the two ends together) and the patient
survives. Medical history is made. Haight had tried this
on ten other patients and none survived. This baby girl
survived 12 days before her operation was to take place.
(link to pictures and story)
In 1939 Dr. Imperatori described the success of his operation
on a patient with an H'fistula.
I came across an article in 1997 by a geneticist in Minnessota
who described a mother who was born with esophageal atresia
and tracheoesophageal fistula, was a nurse, and had a daughter
born with the same thing. It was this mother who asked this
geneticist if she would consider doing research on her and
her daughter. I contacted this geneticist and she told me
she knew of a man who was 73 years old and had survived
an operation for an H' Fistula. She would not share this
information with me but I will keep trying to verify this.
The history of parents and children, aunts and uncles, brother
and sisters, twins and triplettes having esophageal atresia
and or tracheoesophageal fistula is something eventually
we will write about. I know a doctor who wrote about 5 in
one family from the mid-west but I have not been able to
locate them. I have located twins both born with esophageal
atresia and will share this at a later date. There is also
a paper about a father who was born with imperforate anus
and he had 5 children with this
In the year 1935 a baby boy from New York is born with Esophageal
atresia. He is taught from the age of 2 to chew his food
and then spit it in a funnel into the opening in his abdomen.
He would come under the care of Dr. George Humphreys in
1941 and Dr. Humphreys would take a part of the small intestine
(the jejunum) and use this for his esophagus. (link to picture
and george
In 1928 a Dr. Abel reported a patient he had seen with esophageal
atresia without a fistula and he stated he was able to manipulate
the esophagus of his patient and this patient survived.
I have not found the article. I have only read about it
in print. If anyone comes across it I would ask if you would
send me the reference and I will try and research the references.
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TEF/Vater®
International
is
a nonprofit organization founded by Greg and Terri Burke after their daughter,
Jaclyn, was born with esophageal atresia in 1990. To those children,
born and unborn, with esophageal atresia, tracheo-esophageal fistula,
and/or the VATER/VACTERL Association, and to the very special parents
and medical staff who love and care for them, this organization is dedicated
phone
301-952-6837
| fax
301-952-9152
| email
info@tefvater.org
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