First Documented Case of Esophageal Atresia with a Tracheoesophageal Fistula and Anal/Rectal Malformation
Congenital Malformation Some time in August last I was called, in the night, to visit Dr. Webster's family of Hill.
Mrs. W. Had been confined, the previous afternoon, of a male child, and was then comfortable. Nothing unusual appeared in the child at birth; its exterior was perfect, so far as had been discovered, and its features were
uncommonly beautiful. Very soon, however, it was seized with spasms, attended with a livid appearance over the face and neck, and in some degree over the whole body, more especially when anything was put into its mouth.
The nurse told me, on my arrival, that she believed the child could not swallow. Finding nothing in the mouth to obstruct the passage to the throat, and in order to satisfy myself as to what the nurse had stated, I directed
her to give a teaspoonful of warm drink. The child made no effort to swallow, but was immediately convulsed, accompanied with the lividity above mentioned, and the drink which had been given was returned by the mouth and
nose, mixed with bloody mucus. Supposing there might possibly be some spasmodic stricture of the oesophagus, and that gently stimulating the rectum would removed the difficulty, I advised an injection of warm water per
anum. But in attempting to comply with the direction, no outward passage could be found; not even the vestige of an anus was seen; all was smooth as the hand, except the raphe, which extended from the scrotum to near the
point of the coccygis. All the circumstances of the case considered, it was not thought to be advisable to attempt any operation, and I took my leave, expecting soon to hear that the child was dead. It remained,
however, much the same till the next day; it would sometimes lay quiet, as though nothing ailed it; and the anxious parents, desiring that something might be done, if possible, to relieve the little sufferer from its impending
fate, sent for me again, and two others of the faculty. The result of this consultation was, that an attempt be made to open a passage into the rectum. An incision was made in the integuments, about an inch long, half
way between the scrotum and coccygis, and an abscess lancet introduced about an inch and a half in the direction to pierce the lower portion of the gut, if it were in its natural situation. No portion of the intestine was
reached, and, of course, the operation failed—and the poor little thing was again left to its fate. It lingered a day or two, without any change of symptoms, and expired. Post-mortem Examination.— The abdomen was
considerably swollen, from gaeous distension of the intestines. No other abnormal appearance was noticed in the alimentary canal, except at its extremities. On dissecting out the lower portion of the track, it was found
that the rectum, instead of pursuing its natural course down the concave surface of the os sacrum and coccygis, took an anterior direction towards the bladder, and terminated in a cul de sac upon the posterior portion of the neck
of that fiscus. The bladder could be inflated from the rectum by means of a blow-pipe, though the communication could not be traced by a common probe. The condition of the upper end of the tube was still more
remarkable. The pharynx terminated in another cul de sac, about two inches below the fauces. The oesophagus, tracing it upward from the cardiac orifice of the stomach, diminished in size as it ascended, till it finally
ended in a few scattered fibres, passing through an opening in the posterior part of the trachea, to be inserted upon its inner surface. I have procured a drawing of the dissection, which I enclose in this communication, and
which, together with the communication, is at your disposal. Thomas P. Hill Sandbornton, NH, Dec. 4, 1839
(Communicated for the Boston Medical and Surgical Journal)
Congenital Obliteration of Oesophagus with other Malformations
On December 30th, 1896, Mrs. T. was delivered of a
female child at full term after a natural labour, this being her second confinement. When the child was born, I noticed that its hands presented a very unusual appearance, the fingers pointing upwards toward the elbow; there
was entire absence of both radii and both thumbs. When at rest, the outer side of each hand (that is the index finger and it metacarpal bone) rested against the forearm, but by manipulation the hands could be brought down at
right angles with the forearm but no further, and there was great resistance to supination. The ulna in each arm was a little curved and seemed shorter than normal, but in all other respects the child seemed perfectly
developed. On the third day, as soon as the mother's breasts became full of milk, she noticed that as the baby sucked the nipple, after drawing two or three times, the milk returned through its nose, and she had to take it from
the breast. I examined the child's mouth, and found nothing to account for it, and as this regurgitation continued and the child began to waste, I suspected some obstruction in the oesophagus. I did not pass a bougie,
as the nurse told me that the baby had frequent attacks of cyanosis with much choking, and it seemed to fragile and weak that I did not think it would be wise to attempt it. In this way the child continued to live, but gradually
wasting; it eventually died exhausted when it was 13 days old. I obtained permission to make a post-mortem examination, and I found that the upper part of the oesophagus was simply a cul de sac
extending about ¾ inch below the larynx, and that the lower part, as it came up from the stomach, opened into the trachea near the point of its bifurcation. The stomach and intestines were quite empty and very think, just as one would expect to see in a case of starvation. On opening the heart, I found the interventricular septum was not complete, being deficient in its upper part.
The case is an extremely interesting one, not only on account of the rare malformations, but also on account of the length of time the child lived without any nourishment whatever. C. Kessick Bowes, M.B. March 6, 1897
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