Talk:Paper - Congenital absence of the appendix of the caecum (1915): Difference between revisions

From Embryology
(Created page with "KA CASE OF CONGENITAL ABSENCE OF THE LEFT KIDNEY AND URETER. By Recinatp J. Guapstone, M.D. Aberd., F.R.C.S. Eng., F.R.S. Edin., Lecturer and Demonstrator in Anatomy at King...")
 
mNo edit summary
 
Line 1: Line 1:
KA CASE OF CONGENITAL ABSENCE OF THE LEFT KIDNEY
CONGENITAL ABSENCE OF THE APPENDIX OF THE CAICUM.
AND URETER. By Recinatp J. Guapstone, M.D. Aberd.,
By ReainaLp J. GLADSTONE, M.D. Aberd., F.R.C.S. Eng., F.R.S. Edin.,
F.R.C.S. Eng., F.R.S. Edin., Lecturer and Demonstrator in Anatomy
Lecturer and Demonstrator of Anatomy, University of London,
at King’s College, University of London.
King’s College.


CONGENITAL anomalies of the urino-genital system are of interest not
CoNGENITAL absence of the cecal appendix is an extremely rare abnormality.
only as teratological curiosities, but they are also of interest to the
R. J. Berry, writing in the Anatomischer Anzeiger in 1895 (1), after having
embryologist on account of the light that they sometimes throw upon
reviewed the literature on the anatomy of the appendix up to that date,
normal developmental processes. Further, as in the case described below,
and having reported 100 cases of his own, states that “some authors have
they frequently have a very special and important bearing on surgical
described total absence of the appendix: I do not, however, believe that
practice.
the appendix is ever absent except as the result of a previous excision.
Later, in 1907 (2), the same authority refers to a specimen belonging to
Professor Fawcett, and writes: “The fact remains that it is the only case on
record of congenital absence of the appendix vermiformis.


DESCRIPTION OF CASE.
In the systematic examination of 1852 subjects, in which special
attention was given to the anatomy of the cecum and appendix, there
was only one case in which the appendix was absent, namely, Faweett’s.


Complete absence of the left kidney and ureter was discovered during
Number of Absence
the dissection of a male subject in the Anatomy Department, King’s
Cases examined. of Appendix.
College, London.| The man, who was excessively fat, was certified to
Monks and Blake (3) . . 641 0
have died from chronic bronchitis, aged 56. The right kidney (fig. 1)
Fawcett and Blatchford (5). . 350 1
was normal in position, and, as is usual in cases of single kidney, was
Ribbert . . . . . . 161 0
greatly enlarged. It was rather more than double the average weight
Berry (2) . . . . . 100 0
of the kidney in the male.
Treves (14). . . . . 100 0
1352 1


The measurements and weight of the “single kidney,” compared with
Other undoubted cases of congenital absence of the appendix have,
the normal organ, are as follows :—
however, been recorded by Zuckerkandl, Bryant, Huntington, Meckel,
Robinson, and others. I have myself seen three specimens. Two of these
are in the Museum of the Royal College of Surgeons, England: Specimen
. 549 of the Teratological Series, described in the catalogue as “ Part of the
ileum and cecum of a human fcetus in which the appendix is absent and
the cecum is very short,” presented by Sir J. Bland Sutton; and Specimen
549-11, presented last year by Dr E. A. Chill. In this specimen also the
cecum was rounded and very short. It occurred in a new-born child,
in which there was also an epi-cephalocele. The third specimen (fig. 1),
Congenital Absence of the Appendix of the Caecum 415


which forms the subject of this communication, was discovered in the
 
Anatomy Department at King’s College, University of London. It
occurred in a woman who was certified to have died, aged sixty-three,
 
from arterial sclerosis and heart failure. There was no scar in the
Single Kidney. Normal Kidney.
abdominal wall, nor were there any signs of inflammation in the region
|
of the cecum. The cacum was in the usual situation, and was normal in
Length . . .| 52ins, (150 mm.) 4} ins. (105 mm.) |
Width sos : 23 ,, (72 4, ) 24 ,, (60 ,, ) |
Thickness . . . 24 ” (55 2” ) 13 ”? (30 ” ) |
Weight . . . 11 oz. (315 grm.) 44 oz, (130 grm.)
 
 
 
 
The right kidney was elongated in the vertical diameter, and distinctly
lobulated, but was otherwise normal in appearance. Both suprarenal
bodies were present, and they were normal in size and position. The
left, however, differed in form; for, owing to absence of the left kidney,
 
1 T have to thank the students who were engaged in the dissection of the subject, Mr
H. O. Gunewardine and Mr A. M. G. Mishad, who first drew my attention to the defect.
Case of Congenital Absence of the Left Kidney and Ureter 419
 
the upper pole of which normally exerts pressure on the under surface of
the suprarenal body, this surface, instead of being concave, was convex.
 
 
 
 
 
 
Right suprarenal body.
 
Left suprarenal
body.
 
Renal ; ae:
arteries. 3-8
 
1
 
Renal veins. ( Left spermatic
2. vessels.
 
Hypertrophied
 
right kidney.
 
Deferent duct.
 
Orifice of ureter.
Fic. 1.—Absence of left kidney and ureter,
 
The ureter on the left side was completely absent, and there was no
indication of a left ureteral orifice in the interior of the bladder. The
pelvis of the right ureter was larger than usual, but in other respects it
appeared to be quite normal.
 
The testis, epididymis, deferent duct, and seminal vesicle of the left
420 Dr Reginald J. Gladstone
 
side were present but imperfectly developed. The veins of the spermatic
cord were large and somewhat varicose. The cavity of the tunica vaginalis
was obliterated. On section, the testis appeared to the naked eye white
and fibrous. It was dense in consistence, and the tubules appeared to be
imperfectly developed, and imbedded in dense fibrous tissue. The epididymis appeared to be formed almost entirely of a venous plexus
supported by a loose connective tissue. The deferent duct was represented
by a thin fibro-muscular band, which was with difficulty distinguished
from the other constituents of the cord. It pursued the normal course,
and terminated in the usual position at the base of the prostate gland
by joining with a fibrous cord representing the duct of the seminal vesicle.
The left seminal vesicle was, however, quite rudimentary, and no lumen
could be recognised in it; nor could the opening of the common ejaculatory
duct be found in the floor of the prostatic urethra.
 
The bladder was contracted and its walls greatly hypertrophied As
previously stated, only one ureteral orifice, the right, was present. The
hypertrophy was obviously due to urethral obstruction. There was,
however, no definite stricture, but rather a general narrowing of the
canal, which was sufficiently marked to prevent the passage of a mediumsized sound. Whether the small size of the canal was a congenital stenosis,
or the result of inflammation, it is difficult to decide, more especially as I
have been unable to obtain any history of the case. As, however, there
were no obvious signs of inflammation, I am inclined to regard the small
size of the urethra as a congenital defect associated with imperfect
development of the left half of the urogenital sinus.
 
MicroscopicaAL APPEARANCE OF A LONGITUDINAL SECTION
OF THE TESTICLE.
 
The organ was cut entire, stained with hematoxylin and counterstained
with eosin. The outer fibrous tunic (t. albuginea) was greatly thickened.
The substance of the organ (fig. 2) did not show the normal subdivision
into compartments or loculi, by septa converging from the periphery to
the mediastinum, but consisted of an irregular network of coarse bands, in
the meshes of which the seminiferous tubules were embedded. The
tubules differed markedly in appearance in different parts of the organ.
In some parts the epithelium of the tubules had entirely disappeared, and
the walls were greatly thickened, so that the lumen was reduced to a mere
chink. Some tubules, on the other hand, were more or less completely
filled with epithelium, the cells being mostly large, and rounded or
polygonal in shape. They contained large spherical nuclei, the chromatic
Case of Congenital Absence of the Left Kidney and Ureter 421
 
elements of which stained deeply with hematoxylin. Scattered about in
the region of the mediastinum and between the tubules were other cells
of an epithelial type; these, the] “interstitial cells,’ formed irregular


   
   


Fic, 2.—Section through left testicle from case in which left kidney was absent,
Fic. 1.—A symmetrical type of cecum, with absence of the appendix.
 
showing imperfect development of seminiferous tubules and group of
interstitial cells,
 
groups, which, owing to the degeneration of the epithelial cells in the
tubules, appeared more than usually conspicuous.
 
In the epididymis the tubules were large and replaced by a vascular
plexus. The walls of the tubules were greatly thickened, and the
‘epithelium degenerated.
 
Dr Shattock, who very kindly examined one of the sections for me,
422 Dr Reginald J. Gladstone
 
stated that the condition was very similar to that of an undescended
testicle, and that he believes the thickening of the walls of the tubules
to be due to irritation produced by toxic products from the degenerating
epithelium.
 
ARTERIES.
 
The left renal artery was entirely absent. The right kidney received
three arteries, which arose close together from the abdominal aorta.
They occupied a normal position behind the inferior vena cava. The
first or highest of these was given off at the level of the superior mesenteric
artery. It supplied the upper part of the organ and a branch to the right
suprarenal. One of the main terminal branches entered the anterior
surface of the kidney above the hilum; another passed into the upper part
of the hilum. The second and third branches arose from the right side of
the abdominal: aorta, one in front of the other, and both of them about
half an inch below the level of the first. The second passed in front of
the renal vein and pelvis of the ureter, the third behind both. They
supplied the middle and lower part of the kidney.
 
The left suprarenal body received its blood-supply from the left inferior
phrenic, the aorta, and the left spermatic arteries.
 
The right suprarenal body was supplied by the right inferior phrenic,
aorta, and highest right renal artery.
 
There were two left spermatic arteries; one arose at the level of the
superior mesenteric artery from the left side of the aorta, the other about
half an inch below this from the same side of the aorta. They joined
below to form a single vessel.
 
VEINS.
 
The “left renal” vein was represented by a small trunk formed by the
union of the left suprarenal vein with the left spermatic vein. The
suprarenal was joined by the left inferior phrenic vein. The main vein
received a small tributary from the second left lumbar vein. This vessel
probably represented the communication of the left posterior cardinal
vein with the transverse anastomosis joining the subcardinal and posterior
cardinal veins; this anastomosis normally forms the terminal part of the
left renal vein.
 
Two right renal veins were present, a larger one above, which joined
the inferior vena cava just below the level of the vessel representing the left
renal vein, and a smaller vein below, which joined the inferior vena cava
half an inch below the level of the former.
Case ‘of Congenital Absence of the Left Kidney and Ureter 423
 
STRUCTURE AND NATURE OF ENLARGEMENT.
 
On making a longitudinal section through the enlarged right kidney,
it was obvious that the pyramids were also enlarged, and that the general
structure of the organ was coarser than in the normal kidney. Further,
on counting the number of pyramids visible in the section, I found
that, notwithstanding the large size of the organ, there were only
nine, which is approximately the number visible in a similar section of
a normal kidney.
 
In making comparison with the normal kidney, I counted the number
of pyramids visible in a mesial longitudinal section of twenty kidneys
obtained from the dissecting room, and found that the average in twelve
right kidneys was 99, and in eight left kidneys 9-5. Thus in the
normal kidneys, though only half the size, there were in the average
rather more pyramids visible in the mesial section, than in the enlarged
single kidney.
 
Moreover, on examining sections of the enlarged “single kidney” with
the microscope, and comparing these with the normal, the glomeruli
and tubules were seen to be much larger than in the normal kidney.
The average diameter of twenty-five glomeruli in the “single kidney ”
was 0°2 mm.; in a normal human kidney used for comparison the
average was 0:13 mm. There was a similar enlargement of the tubules
of the “single kidney.”
 
Further, on counting the number of glomeruli visible in a given area of
the “single kidney,” which I accomplished by enumerating those falling
within an area of 16 squares ruled on a’ glass inserted inside the ocular of
the microscope, I found that the average number falling within these
16 squares in 50 counts was 2°8 in the “single kidney,” 5°5 in the normal.
The number of glomeruli falling within the given area, in the “single
kidney,” thus averages about half that in the normal.
 
Average number of glomeruli seen
in 50 counts of 16 squares.
 
——— 0S
Single kidney. Normal kidney.
 
2°8 55 3 = approx. 2.
 
The enlargement of the kidney is thus a pure hypertrophy, or enlargement by increase in the size of the constituent elements, and not a hyperplasia, or enlargement by increase in their number. In other words, the
total glomeruli in the enlarged “single kidney” must have been approximately equal in number to those contained in a normal kidney, in spite of
the great difference in size between the two.
424, Dr Reginald J. Gladstone
 
The credit of first pointing out that the enlargement in a case of “single
kidney” is a pure hypertrophy is due to Professor A. E. Boycott, who cut serial
sections of blocks having a definite weight from kidneys in the rabbit. One of these
was taken from a case of single kidney, the others from two “controls.” He
counted the glomeruli with the aid of camera lucida drawings. My observations
on the human subject must therefore be regarded as confirmatory of his work
carried out in the rabbit.
 
PRACTICAL CONSIDERATIONS.
 
The possibility of one kidney being absent has an important bearing on
modern surgical practice, and more especially with reference to the operation
of nephrectomy.
 
In considering these cases it is necessary to distinguish between :—
 
1. Congenital absence of one kidney, including those cases in which one
kidney is rudimentary and functionless.
 
2. Fusion of two kidneys into a single mass (horse-shoe and disc-shaped
kidneys).
 
3. Atrophy or destruction of a kidney as the result of disease.
 
Secondly, it is of importance to have some idea as to the frequency
of these conditions; and thirdly, we must consider the signs and symptoms
which would lead one to expect the absence of one kidney, or the fusion of
two into a single mass.
 
The frequency of congenital absence of one kidney, fusion of two
kidneys, and atrophic kidneys has been very thoroughly worked out by
Sir Henry Morris, who gives the following averages derived from the
reports of post-mortem examinations conducted at ‘four London hospitals,
combined with published statistics of other writers :—
 
1. Congenital absence of one kidney . lin 3,992 or 25 per 1000
2. Fusion of two kidneys (horse-shoe, etc.) 19 in 18,244 or 1 per 1000
3. Atrophied, small, shrunken, or wasted
 
kidneys. . . . 59 in 8,178 or 7 2 per 1000
 
Congenital absence of one e kidney is thus seen to be an extremely rare
abnormality. From the surgical standpoint, however, in neither of the
first two groups should nephrectomy be performed; and probably; in the
greater number of cases belonging to the third group, the operation would
be contraindicated, as the atrophic kidney, if the other was removed, would
be unequal to the strain which would suddenly be thrown upon it. The
total number of cases, therefore, falling into one or other of these three
groups in which nephrectomy is contraindicated is about 8 per 1000.
This number is, however, considerably increased by the fact that when one
kidney is absent, or is atrophied, the other kidney is frequently diseased.
Thus it is probable that the proportion of cases in which one kidney is
Case of Congenital Absence of the Left Kidney and Ureter 425
 
absent would be greater in those subjects suffering from symptoms of
renal disease than in the total number of cases examined “post mortem ”
in a general hospital. Moreover, the importance of recognising the condition is so great, that in all cases in which nephrectomy is contemplated a
careful examination should be made to ascertain the existence and condition
of the opposite kidney.
 
The most important signs of absence of one kidney are :—
 
1. Absence of an ureteral orifice on one side of the bladder.


2. Absence of the “kidney shadow” when the patient is examined with
size and form. It was completely covered by peritoneum. The “teniz
X-rays.
coli” converged to a point, on the medial and posterior aspect of the
cecum, 1} inches below the ileo-cxcal junction. No trace of an appendix
was, however, visible from the outside. On examining the mucous
membrane lining the interior of the cacum, a small patch of what appeared
to be adenoid tissue was found opposite the point of convergence of the
three teeniz coli, and in the position in which one would expect to find
the orifice of the appendix.


3. Some congenital defect of the external organs of generation on the
The distal end or fundus of the cecum was formed by a sacculation
same side.
416 Dr Reginald J. Gladstone


In one instructive case described by Guthrie and Wilson, and
which lay to the right of and below the point previously mentioned to which
operated on by W. J. Mayo, absence of the left kidney and ureter was
the longitudinal muscular bands converged. This case therefore differs
diagnosed by cystoscopic examination previous to an operation for the
from those described by Huntington; in one of which the cecum was round
removal of a tumour, which proved to be the uterus and upper part of
and globular and the muscular bands converged to the lowest point of the
the vagina distended with retained menstrual fluid. The lower portion of
pouch, and a second in which the cecum turned upward and to the left, and
the vagina, the left ovary and Fallopian tube were completely absent.
ended in a sharp point, to which several lobes of epiploic fat were attached.
The labia majora were rudimentary, and the labia minora absent. The
case emphasises the importance of cystoscopic examination of the bladder,
and also the importance of examining the external genital organs, which,
in congenital absence of one kidney, are frequently imperfectly developed
on the same side as that on which the kidney is absent.


In the case which I have described, the atrophic condition of the left
The very rare occurrence of total absence of the appendix is a matter
testicle and deferent duct would, taken in conjunction with absence of the
of some surprise. On considering the subject from the standpoint of
ureteral orifice, have formed a very important clue to the absence of the
comparative anatomy, one would expect that an organ which is absent in
left kidney.
such a large number of orders and species of vertebrate animals, and only
present in higher types (anthropoid apes, certain. rodents, and the wombat),
would frequently be absent in man.


Abnormalities of the genital organs are, however, not the only. con' genital defects which may accompany absence of one kidney. In a
Vestigial structures are usually, though not always, variable in the
specimen (558°4) presented by Frank Deas to the Royal College of Surgeons,
extent of their development, and are frequently found to be absent
England, last year, absence of the right kidney was associated with imperforate anus and hydrocephaly. In specimen 7022, also added to the
altogether. This is especially the case when the structure or organ has no
Museum of the Royal College of Surgeons last year, imperfect differentiation of the sexual organs in a newly born child is combined with an
apparent functional importance. The fact that although the cecal appendix varies very considerably in its length, form, and disposition, it is so
imperforate condition of the rectum. There was also an imperfect penis
rarely absent, supports the views put forward by Berry, Keith, and others
and scrotum, and no sexual glands.
regarding its functional importance.


In my own experience of teratological specimens, I find that one
From the standpoint of ontogeny, one would also expect congenital
congenital defect is nearly always associated with other defects, and often
absence of the cecum and appendix to be a much more frequent occurrence
of some quite distant part. The frequent occurrence in one subject of
than it is. In a human embryo of 7:5-mm. length (Harvard College) the
cecal diverticulum is recognisable as a slight swelling on the distal limb
of the intestinal loop. In an embryo of 17 mm. (Mall) this bulging is more
pronounced and forms a rounded swelling, such as is seen in Specimen 629,
R.C.S. Eng. In a later stage (in an embryo also 17 mm. in length) it
becomes conical. Between 40 and 50 mm. a narrower distal part which
will become the appendix can be distinguished from a wider proximal part
which will become the cecum. It is not, however, until after birth that a
sudden diminution in the calibre of the gut marks off the root of the
appendix from the cecum, which is at this stage symmetrical in form, the
appendix coming off from its lowest point. The asymmetrical form is not
developed until a few years after birth. ,


congenital defects in different and often distant parts of the body
Arrest of development may occur at any of these stages and give
VOL. XLIX. (THIRD SER. VOL. X.)—JULY 1915. 30
rise to:—
426 Dr Reginald J. Gladstone


indicates, therefore, that special care should be taken to ascertain whether
“Absence of the cecum and appendix” (Robinson); “ Rudimentary
- one kidney is absent, in all those cases in which nephrectomy is proposed
cecum without appendix” (Sutton, Chill); “A cecum having a blunt
and some congenital defect, eg. an “accessory auricle” or “club foot,” is
conical form without appendix” (Huntington); “A caecum having a
present.
rounded symmetrical form, with the longitudinal muscular bands converging towards its apex, but without appendix” (Huntington); “ Asymmetrical form of cecum without appendix” (Author).
 
Congenital Absence of the Appendix of the Cacum 417
It is curious that, since writing the above sentence, I have received a letter
from Mr Frank Deas, informing me that the child from whom specimen 558-4 was
 
prepared also ‘had a curious deformity of the hands, which resembled feet.” This
was not stated in the description in the catalogue.
 
Absence of the ureteral orifice on one side does not necessarily involve
complete absence of the kidney on the same side; it may be present but in
a cystic condition, as in a case of Sir J. Bland Sutton’s, reported by Sir
H. Morris, or it may be otherwise diseased, as in a specimen (648°6) in the
Museum of the Royal College of Surgeons, England, presented last year by
Dr F. Mott. In this case the termination of the left ureter was congenitally
deficient, and the left kidney was saccular and filled with a caseous substance. In these and similar cases, however, the kidney is functionally
absent and from the surgical standpoint useless.
 
Two ureteral orifices may be present and yet one kidney may be
absent, or quite rudimentary. The pelvis of the ureter in these cases is
also small or absent altogether. The condition, if suspected, might be
diagnosed by passing with a catheterising cystoscope an ureteral catheter
(which is opaque to X-rays) as far as the renal pelvis. The renal pelvis
may then be injected with collargol 10 per cent. in water, and the patient
then examined with the X-rays. This should be done on both sides, as the
condition is sometimes bilateral. Such a case occurred last year in a
female subject in the Anatomy Department of King’s College, London.
Both kidneys and ureters were atrophied, as were also the ureters, ovaries,
and Fallopian tubes. The main blood-vessels of the abdomen and pelvis
were also about half their normal size. The method of injecting with
collargol would be useful also in the diagnosis of “horse-shoe” and other
forms of fused kidney. In these cases the kidney and pelves of the ureters
are usually situated near the middle line, and are lower in position than
the normal kidney.
 
Should an operation for nephrectomy have already been commenced,
without a previous cystoscopic and X-ray examination having been made,
and it is found that the kidney (apart from the disease) is nearly double
the normal size, and that there is more than one renal artery and vein,
these conditions should lead one to suspect the absence of the opposite
kidney, and the operation should not be completed without first having
ascertained that the opposite kidney is present.
Case of Congenital Absence of the Left Kidney and Ureter 427
 
ETIOLOGY.
 
The causation of congenital absence of one kidney and its ureter is, like
that of other congenital abnormalities, obscure, but it is probable that the
defect originates in an arrest of development of the distal end of the
Wolffian duct, and of its union with that part of the cloaca from which the
urogenital sinus is derived. The normal outgrowth of the renal diverticulum is therefore checked. The mesonephros, the genital gland, and the
upper or proximal end of the Wolffian duct, if formed, subsequently atrophy.
This atrophy in the male subject is probably due in part, at least, to there
being no outlet for the escape of the testicular secretion.
 
The frequent cystic condition of the atrophic forms of kidney may be
explained by a faulty union, or a complete failure in the union of the
tubules of the metanephric blastema with the excretory tubules of the
renal diverticulum.
 
The kidney is congenitally absent on the left side about twice as
frequently as on the right. In twenty cases, including my own, the left
kidney was absent in fourteen, the right in six. This may possibly be due
to the fact that the umbilical cord is most frequently to the right side of
the tail in young embryos of from 4-10 mm. length. It is during this
period that the Wolffian duct first penetrates the wall of the cloaca and the
renal diverticulum grows out from its lower end. Now, as the cloaca will
be pulled over to the right with the umbilical cord, it will be farther away
from the growing lower end of the Wolffian duct on the left than on the
right side. This may account for greater frequency in the failure to unite
with the cloaca on the left than on the right side.
 
SuMMARY.
 
In conclusion, I will give a brief summary of the more important points
to be learnt from a consideration of these cases.
 
1. The enlargement of the remaining kidney, when one is congenitally
absent, is a pure hypertrophy.
 
2. The importance of cystoscopic and X-ray examinations, and of
noting the presence of other congenital defects, in all cases in which
nephrectomy is contemplated.
 
3. Congenital absence of the kidney and ureter is in all probability
due to an arrest in the development of the distal end of the Wolffian duct,
and to a failure in its union with the cloaca.
428 Case of Congenital Absence of the Left Kidney and Ureter


REFERENCES TO LITERATURE.
REFERENCES TO LITERATURE.


Boycort, A. E., “A Case of Unilateral Aplasia of the Kidney in a Rabbit,”
1) Berry, R. J. A., Anat. Anz., Jahrg. x., 1895.
Jour. Anat. and Physiol., vol. xlv. p. 20.
 
Branca, Aupert, et Feuizet, G., “Sur les cellules intersticielles du testicule
ectopique,” Paris, C’. R. Soc. biol., vol. liii, 1901, p. 311.
 
GREENFIELD, Path Soc. Trans., xxviii. p. 164.
 
GuturRiz, DonaLp, and Witson, L. B., Ann. Sury. Lond., vol. 1., 1909, p. 907.


Herrincuam, Kidney Diseases, 1912.
)
) Berry, R. J. A., Lntercolonial Med. J. of Australasia, vol. xii., 1907, p. 305.
) Brake, see Monks.


Moorz, Craven, Jour. Anat. and Physiol., 1899, p. 400.
) BiatcHForD, see FAWCETT.


Morris, H., Surgical Diseases of the Kidney and Ureter, vol. i. p. 18.
) Fawcerr and Biarcurorp, J. Anat. Physiol., vol. xxxiv.


Petersen, Med. Times and Gazette, 1882, Oct. 7.
(6) Keiru, A., Proc. Anat. Soc., Nov. 1903, p. 7.


Poik, W., New York Med. Jour., Feb. 17, 1883.
(7) Huntinetox, The Anatomy of the Human Peritoneum and Abdominal
Cavity, 1903.


Wer, R. F., New York Med. Jour., Dec. 27, 1884.
(8) Hurpon, see KELLY.
(9) KeipeL and Mati, Manual of Human Embryology.
(10) Keniy, H. A., and Hurpon, E., 7he Vermitorm Appendix and its Diseases.
(11) Monks and Bakes, Boston Medical and Surgical J., Nov. 1902.
(12) Quaty, R., vol iii. part iv.
(13) Ransonorr, J. Amer. Med. Ass., 1888.
(14) Treves, F., Zntestinal Canal in Man, London, 1885.
(15) ZucKERKANDL, E., Anat. Hefte, Bd. 4, 1894.


Wizson, L. B., see GUTHRIE.
(1
(2
(3
(4
(5

Latest revision as of 14:01, 4 March 2020

CONGENITAL ABSENCE OF THE APPENDIX OF THE CAICUM. By ReainaLp J. GLADSTONE, M.D. Aberd., F.R.C.S. Eng., F.R.S. Edin., Lecturer and Demonstrator of Anatomy, University of London, King’s College.

CoNGENITAL absence of the cecal appendix is an extremely rare abnormality. R. J. Berry, writing in the Anatomischer Anzeiger in 1895 (1), after having reviewed the literature on the anatomy of the appendix up to that date, and having reported 100 cases of his own, states that “some authors have described total absence of the appendix: I do not, however, believe that the appendix is ever absent except as the result of a previous excision.” Later, in 1907 (2), the same authority refers to a specimen belonging to Professor Fawcett, and writes: “The fact remains that it is the only case on record of congenital absence of the appendix vermiformis.”

In the systematic examination of 1852 subjects, in which special attention was given to the anatomy of the cecum and appendix, there was only one case in which the appendix was absent, namely, Faweett’s.

Number of Absence Cases examined. of Appendix. Monks and Blake (3) . . 641 0 Fawcett and Blatchford (5). . 350 1 Ribbert . . . . . . 161 0 Berry (2) . . . . . 100 0 Treves (14). . . . . 100 0 1352 1

Other undoubted cases of congenital absence of the appendix have, however, been recorded by Zuckerkandl, Bryant, Huntington, Meckel, Robinson, and others. I have myself seen three specimens. Two of these are in the Museum of the Royal College of Surgeons, England: Specimen . 549 of the Teratological Series, described in the catalogue as “ Part of the ileum and cecum of a human fcetus in which the appendix is absent and the cecum is very short,” presented by Sir J. Bland Sutton; and Specimen 549-11, presented last year by Dr E. A. Chill. In this specimen also the cecum was rounded and very short. It occurred in a new-born child, in which there was also an epi-cephalocele. The third specimen (fig. 1), Congenital Absence of the Appendix of the Caecum 415

which forms the subject of this communication, was discovered in the Anatomy Department at King’s College, University of London. It occurred in a woman who was certified to have died, aged sixty-three, from arterial sclerosis and heart failure. There was no scar in the abdominal wall, nor were there any signs of inflammation in the region of the cecum. The cacum was in the usual situation, and was normal in


Fic. 1.—A symmetrical type of cecum, with absence of the appendix.

size and form. It was completely covered by peritoneum. The “teniz coli” converged to a point, on the medial and posterior aspect of the cecum, 1} inches below the ileo-cxcal junction. No trace of an appendix was, however, visible from the outside. On examining the mucous membrane lining the interior of the cacum, a small patch of what appeared to be adenoid tissue was found opposite the point of convergence of the three teeniz coli, and in the position in which one would expect to find the orifice of the appendix.

The distal end or fundus of the cecum was formed by a sacculation 416 Dr Reginald J. Gladstone

which lay to the right of and below the point previously mentioned to which the longitudinal muscular bands converged. This case therefore differs from those described by Huntington; in one of which the cecum was round and globular and the muscular bands converged to the lowest point of the pouch, and a second in which the cecum turned upward and to the left, and ended in a sharp point, to which several lobes of epiploic fat were attached.

The very rare occurrence of total absence of the appendix is a matter of some surprise. On considering the subject from the standpoint of comparative anatomy, one would expect that an organ which is absent in such a large number of orders and species of vertebrate animals, and only present in higher types (anthropoid apes, certain. rodents, and the wombat), would frequently be absent in man.

Vestigial structures are usually, though not always, variable in the extent of their development, and are frequently found to be absent altogether. This is especially the case when the structure or organ has no apparent functional importance. The fact that although the cecal appendix varies very considerably in its length, form, and disposition, it is so rarely absent, supports the views put forward by Berry, Keith, and others regarding its functional importance.

From the standpoint of ontogeny, one would also expect congenital absence of the cecum and appendix to be a much more frequent occurrence than it is. In a human embryo of 7:5-mm. length (Harvard College) the cecal diverticulum is recognisable as a slight swelling on the distal limb of the intestinal loop. In an embryo of 17 mm. (Mall) this bulging is more pronounced and forms a rounded swelling, such as is seen in Specimen 629, R.C.S. Eng. In a later stage (in an embryo also 17 mm. in length) it becomes conical. Between 40 and 50 mm. a narrower distal part which will become the appendix can be distinguished from a wider proximal part which will become the cecum. It is not, however, until after birth that a sudden diminution in the calibre of the gut marks off the root of the appendix from the cecum, which is at this stage symmetrical in form, the appendix coming off from its lowest point. The asymmetrical form is not developed until a few years after birth. ,

Arrest of development may occur at any of these stages and give rise to:—

“Absence of the cecum and appendix” (Robinson); “ Rudimentary cecum without appendix” (Sutton, Chill); “A cecum having a blunt conical form without appendix” (Huntington); “A caecum having a rounded symmetrical form, with the longitudinal muscular bands converging towards its apex, but without appendix” (Huntington); “ Asymmetrical form of cecum without appendix” (Author). Congenital Absence of the Appendix of the Cacum 417

REFERENCES TO LITERATURE.

1) Berry, R. J. A., Anat. Anz., Jahrg. x., 1895.

) ) Berry, R. J. A., Lntercolonial Med. J. of Australasia, vol. xii., 1907, p. 305. ) Brake, see Monks.

) BiatcHForD, see FAWCETT.

) Fawcerr and Biarcurorp, J. Anat. Physiol., vol. xxxiv.

(6) Keiru, A., Proc. Anat. Soc., Nov. 1903, p. 7.

(7) Huntinetox, The Anatomy of the Human Peritoneum and Abdominal Cavity, 1903.

(8) Hurpon, see KELLY. (9) KeipeL and Mati, Manual of Human Embryology. (10) Keniy, H. A., and Hurpon, E., 7he Vermitorm Appendix and its Diseases. (11) Monks and Bakes, Boston Medical and Surgical J., Nov. 1902. (12) Quaty, R., vol iii. part iv. (13) Ransonorr, J. Amer. Med. Ass., 1888. (14) Treves, F., Zntestinal Canal in Man, London, 1885. (15) ZucKERKANDL, E., Anat. Hefte, Bd. 4, 1894.

(1 (2 (3 (4 (5