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CAUSE OF " ESSENTIAL H^EMATURIA NOT UNDERSTOOD. 591

There are a certain number of cases of renal haematuria with-
out ascertainable renal lesion, the so-called essential hcematuria,
which at present are unexplained ; cases in which the haemorrhage
is so profuse, so unprovoked, so recurrent or persistent, that no
amount of rest, and no remedies which have been tried have
influenced it ; and in which no explanation is forthcoming even
after a post-mortem examination. The haemorrhage in some
of these cases probably depends upon some functional derange-
ment or actual lesion of the vaso-motor nervous system, in others
possibly upon a morbid condition of the blood or blood vessels due
to some toxic cause.

I have known at least three fatal cases of the sort, two after
nephrotomy, and one in which no operation was performed. In the
first case* upon which I operated the patient was a young man
of twenty, who for six months had suffered from severe attacks
of haematuria uninfluenced by exercise or rest, but attended
with pain in the loins, chiefly in the right loin. The repeated
and considerable losses of blood had rendered him very anaemic,
weak and emaciated, and his condition was becoming progressively
worse. The right kidney was cut into and thoroughly examined,
with negative result ; nothing was found to explain the haematuria,
and no improvement in the symptoms resulted. Ten days after
the nephrotomy I performed nephrectomy, because of the con-
tinuing haematuria and the supervention of haemorrhage from the
kidney operated upon. Haematuria, however, continued after
the nephrectomy, and the patient died the next day. There
was nothing in the naked-eye appearances of the kidney removed,
or of the left kidney, or of the bladder, or any part of the
urinary system to explain the cause of the haemorrhage. Neither
a microscopic nor a bacteriological examination of the kidneys
was, I regret to say, made, their naked-eye appearances being
so normal that further examination was unfortunately omitted.
In the second case operated f upon for similar symptoms there
were no naked-eye or microscopical characters existing to account
for the symptoms.

The essential haematuria of hot or tropical countries is caused

by parasites, such as the bilharzia or filaria or the plasmodium

malariae ; in the colder countries neither parasites in the blood

or urinary organs nor any lesion of the kidney is discoverable.

* " Hunterian Lectures," Table IV., No. 30, and Table VIII., No. 3.



592 SURGERY OF THE KIDNEY AND URETER.

Some of the German authors, following Senator and Klemperer,
describe cases of " essential " renal haematuria under the name
of " angeio-neurotic," and to this class I believe the two cases
I have referred to belong. Schede, Klemperer, Xitze, Debaisieux,
and others have reported cases of the sort for which nephrotomy
or nephrectomy has been performed. Nitze speaks of having
operated in seven cases of " essential " haematuria four by nephrec-
tomy and three by renal incision. In Schede's case the haematuria
had lasted many weeks, and the kidney after nephrectomy was
microscopically examined and found to be quite healthy.*

Though the clinical diagnosis of these cases is impossible, yet
when there is any indication as to which kidney is the source of
the haemorrhage we have exploratory nephrotomy to fall back upon.
It is satisfactory to learn that in a good many cases of undiagnos-
able haematuria which have been operated upon the haematuria
has ceased and has not recurred. The explanation of this is
possibly the removal of intra-capsule tension by the relief of renal
congestion, \vhether due to blood or nervous cause in the first place.
It is, however, difficult to understand why, though the bleeding
ceases for a time, it does not in all these cases recur after the
kidney is healed. In neither of the cases upon which I performed
nephrotomy, was the bleeding controlled by the operation. In
one case in which (in March, 1896) I performed simultaneously
nephrotomy and nephropexy for haematuria associated with hae-
mato-nephrosis in a movable kidney, the haematuria did not cease
till some weeks after the nephrotomy, and then after an interval
of more than a year, the kidney being well fixed, recurred to a
less degree, and has done so two or three times subsequently.
In another case in which I performed nephrotomy for haemato-
nephrosis and haematuria there were subsequent recurrences of
haematuria for a time until the kidney atrophied ; and at the
patient's death, fourteen months after the operation, retro-perito-
neal carcinoma, unconnected with tjie atrophied kidney, together
with secondary deposits in the liver, were found. There was no
tumour to be discovered, though often sought for during life.

In some of the cases of the so-called essential or angeio-neurotic
kind there is reason to think the haemorrhage comes from both
kidneys, even when there is some other local symptom such as

* See also Harris, Med. Journ. Philadelphia, March 19, 1898, on
cases of Essential Haematuria.



NEPHRALGIC H.EMATURIA DUE TO RENAL IRRITATION. 593

pain or nephrectasis pointing to one kidney rather than to the
other. This clearly was so in the fatal case upon which I
operated, and to which I have referred above.

Nephralgic haematuria. Since the time of Sydenham much
has been written and many cases have been published to show
that renal neuralgia gives rise to haematuria in the absence of any
appreciable lesion of the kidney. Many cases of nephralgia are
attributed to malarial poisoning. Tift'any of Maryland speaks
of having seen a number of cases of malarial haematuria in which
there was apparently no history of intermittent or remittent
fever. He seems to have gone some way towards proving the
malarial origin of the haematuria and pigmented urine in some
of these cases by finding the plasmodium malarias in the blood.
Maurice Raynaud, Rolf, Legueu, Broca, Sabatier, and others
have attributed haematuria to nerve causes. Nephralgic haema-
turia has been ascribed to a profound disturbance of the vaso-
rhotor system of the kidney, just as symmetrical gangrene is
ascribed to exaggerated vaso-motor disturbance in the fingers
and toes and other peripheral parts in Raynaud's disease.

In many of the cases, however, some other condition, such
as oxaluria, lithaemia, movable kidney, and chronic nephritis, has
been present by which possibly both the pain and the haema-
turia may have been caused. In other cases there have been
found adhesions about the kidney and ureter, or calculous particles
in the renal calyces, to which rather than to an idiopathic dis-
turbance of the vaso-motor system of the kidney the renal
congestion and tension and the consequent haematuria and pain
were much more probably due.*

As a correct clinical diagnosis of these cases cannot be made,
it is consoling to know that in many of them complete relief
follows from lumbar nephrotomy.

Senator and Klemperer have given evidence as to the exist-
ence of very abundant haematuria in cases of proved haemophilia.
Senator relates the case of a young girl affected in this way,
upon whom Sonnenburg performed nephrectomy. When it occurs
it is usually in young men or boys. Sometimes pain is associated
with the haemorrhage, but often not.

Haematuria, with or without pain, but without other ascer-
tainable or recognisable cause, in. a member of a family of

* "Hunterian Lectures," lecture ii., p. 52.
VOL. I. 2 M



594 SURGERY OF THE KIDNEY AND URETER.

" bleeders," should be treated as due to this constitutional ten-
dency, and not submitted to nephrotomy but to nephrectomy,
if all other remedies have failed and life is threatened, and not
ttefore. No operation which can possibly be avoided is per-
missible in the case of these persons.

Hsematuria, in some cases to a rather abundant degree, has
been met with in association with movable kidney. I have
explored the movable kidney in several instances, on account of
pain and hsematuria, and I have found it congested ; and after
the operation the symptoms have entirely ceased. In some of
these cases I have been uncertain as to how much of the
relief ought to be attributed to the fixation and how much to
the nephrotomy ; and whether the symptoms were not caused
by chronic nephritis. In some instances, where the kidney on
exposure and section has had the appearances of chronic
nephritis, I have not fixed it, though I found it very movable,
and yet the symptoms have ceased after the operation, which
looks as if the relief was the result of the nephrotomy. This
cannot be positively inferred, however, because after such an
operation, the kidney is apt to become firmly fixed, even when
no sutures are inserted into its substance or capsule.

When hsematuria occurs with a movable kidney, lumbar
exploration ought to be performed and nephrorraphy practised,
after full examination of the kidney and downward catheterisation
of the ureter have shown that no other tangible cause for the
haemorrhage exists.

Guyon has pointed out that hsematuria occurs in rare cases
during pregnancy. If not caused by direct pressure it is
probably due to congestion of the renal vessels, which are apt
to be influenced by uterine and ovarian conditions.

When there is tumour without hcematuria. Having diagnosed
a tumour of the abdomen to be renal, how can we decide whether
the tumour is due to a malignant new growth or to some other
form of renal enlargement ? It is likely to be malignant, if
the tumour occurs in a child under ten, and still more so if
under six or seven years of age ; or in an adult between forty
and seventy ; if it has grown rapidly since it was first discovered,
whether preserving the renal outline or not ; if it has not varied and
does not vary in size and painfulness, with marked variations in
the amount of urine passed ; if there are no crystals, calculous



H.EMATURIA AND TUMOUR IN OTHER AFFECTIONS. 595

fragments, or pus or casts in the urine ; if by repeated and
careful microscopic examination of the urine little masses of new
growth are detected ; if there is a progressive diminution in the
amount of urea ; if there is no antecedent history of calculus ;
if with the growth of the tumour the patient emaciates
markedly and becomes sallow ; if enlarged glands, intra-abdominal,
pelvic, or inguinal, are discoverable : if there is a recent vari-
cocele on the same side as the tumour distinctly increasing with
the growth of the tumour ; if there are signs of venous obstruc-
tion in the inferior cava ; or if there is any nodule of malignant
new growth in any other part of the bod}'.

Too much reliance must not be attached to any one
individually of the indications just mentioned, but if several
of them co-exist in any case in which the tumour is diag-
nosed as renal the probability of malignant new growth is
considerable.

When hwmaturia and tumour co-exist. In renal calculous
affections, in tuberculous affections of the kidney, in cystic
disease, in hsemato-nephrosis, and in hydro-nephrosis from various
causes, haematuria and renal tumour co-exist, and will have
to be diagnosed from malignant renal tumour.

From calculous affections it is diagnosed by the characters
of the haematuria above described ; by the absence of tenderness
on pressure, which is so frequent in calculous affections when
the kidney is manipulated between the two hands; by the non-
occurrence of renal colic after exercise, jolting, or fatigue ; by
the want of an antecedent history of lithiasis or of former
attacks of hsematuria and pain followed by long periods of
quiescence years before the tumour first appeared.*

From tuberculous affections by the characters of the haema-
turia ; by the absence of pus and tubercle bacilli in the urine ;
by the absence of tuberculous lesions in other organs ; by the absence
of an elevated temperature at night ; and by the absence of
the family history of tubercle.

From poly cystic disease by the abundance of the hsematuria,
hsematuria being rare in cystic disease, but when it occurs it
resembles the haematuria of malignant tumour by its unprovoked
onset and its tendency to recur without assignable cause ; by
the absence of polyuria, or anuria, or uraemia, either or all of
' " Himterian Lectures." pp. 70-74.



596 SURGERY OF THE KIDNEY AND URETER.

which are met with as early symptoms in polvcystic disease, but
very rarely indeed occur in cases of new growths of the kidney ;
by the absence of that irregular botryoidal outline which is so
characteristic of conglomerate cysts ; and by the absence of a
tumour on both sides bilateral enlargement being not at all un-
frequent in polvcystic disease. In malignant disease the amount
of urea may be diminishing but without polyuria or anuria ; in
polvcystic disease the lessening amount of urea is associated with
a great increase in the amount of water, or on the contrary
with a suppression of the secretion in great part or entirely.

In cases of serous cysts the hsematuria is either absent
or slight, the tumour may fluctuate at some stage of its growth,
whilst at others it is very dense and hard, and more resembles
a rounded circumscribed solid tumour in the abdominal parietes.
I have known a case in which there were sudden fainting fits
caused by haemorrhages into the cyst cavity.* Cancer may be
engrafted upon such a renal cyst.j

The difficulties which surround the diagnosis of these very
rare cases of greatly enlarged simple cysts are considerable, and
it is sometimes impossible to distinguish them not only from
cases of nephrectasis, but from solid tumours of the kidney or
of some other intra-abdominal organ or tissue.

The diagnosis of hydatid cysts of the kidney, which are
very rare, from malignant tumour must in most cases remain
uncertain, unless daughter cysts or booklets are discovered in the
urine, or the tumour gives the characteristic hydatid fremitus,
or fluctuates.

Another source of error in the diagnosis of malignant tumour
is due to the fact that hydro-nephrosis is in some cases ac-
companied by haeniaturia, and may therefore be mistaken for a
new growth of the kidney. This is especially likely to occur if
the cause of the hydro-nephrosis happens to be a tumour villous
papilloma or carcinoma of the bladder. I have 011 several
occasions been consulted on account of hsematuria with or without
renal enlargement, which has been regarded as due to some
affection of the kidney, when the disease has really been a new
growth in the bladder around the orifice of the ureter of the sus-
pected side. Such growths may cause continuous or intermittent

* Path. Soc. Trans., vol. xxii., p. 171.

t Ibid., vol. xxi., p. 253 and vol. xxii., p. 171.



RENAL ENLARGEMENT CAUSED BY VESICAL GROWTHS. 597

hydro-nephrosis, and spontaneous profuse irregularly intermittent
hgematuria having all the characters of renal tumour.

The diagnosis can be made by bimanual examination of
the bladder under chloroform after emptying it of its
contents ; then with one finger in the rectum and the fingers
'of the other hand depressing forcibly the abdominal wall in
the hypogastrium, the collapsed vesical walls can be thoroughly
felt and any difference in thickness or resistance appreciated.
The induration and unyielding feeling of an infiltrating malignant
growth is readily detected by the experienced fingers ; and a
soft villous papilloma large enough to give rise to the symptoms
of hydro-nephrosis can be felt within the vesical cavity like a
piece of saturated sponge between the fingers (see a paper with
recorded cases by the author in the Lancet, October 31, 1896).
The cystoscope may be employed whenever a new growth of
the kidney is suspected ; but with the bimanual method of
examination just described all the information requisite for a
correct diagnosis can be obtained in these cases without re-
sorting to the intra-vesical inspection.

Unless due to the cause just stated it is not common for the
hsematuria in hydro-nephrosis to be either abundant or bright red
as it is in renal tumour.

In some of the cases of renal distension from, vesical new
growth which have been under my charge high temperature
and shivering have been prominent symptoms.

Diagnosis of the variety of new growth. In the adult it
is generally almost impossible to diagnose with any degree of
certainty the nature of the tumour. Sarcoma is more frequent
than carcinoma in the earlier decades of manhood (twenty to
forty), epithelioma in the later (forty to seventy) ; sarcoma causes
hsematuria less frequently, secondary growths more slowly, and
has a duration of from five to six years, instead of from three
to four years as has carcinoma.

But these distinctions cannot be relied upon. It is impossible
clinically to diagnose adenoma from epithelioma, and even with
the microscope the distinction is far from easy and not always
possible.

It is often quite impossible to make the diagnosis of malig-
nant from benign renal tumours and from pararenal growths
except by an exploratory incision, and often a microscopic



598



SURGERY OF THE KIDNEY AND URETER.



examination of the growth does not permit of a decisive
judgment being formed. Very variable opinions are ex-
pressed by equally competent microscopists about the nature of
the same tumour. The facts of the tumour being encapsuled
and that on section the microscopic characters are those of an
adenoma do not prove the non-malignancy of the growth or
give a certain guarantee against recurrence. In the case of a
lady whom I saw in consultation, a renal tumour removed and



Necrosis centre of the
growth.




Fig. 91. Carcinoma of the upper half of the kidney confined within the renal
capsule. There were secondary deposits in the brain, lungs, and hronchial
glands. Pyelitis existed. From a man aged 38. (See also Figs. 92 and 93.)

described by Mr. Stanley Boyd as an adenoma, recurred within a
few months, and formed a tumour much larger than the original mass.*
The diagnosis of adhesions and secondary deposits is of import-
ance from the point of view of treatment, but it is not always
possible to exclude these without an exploratory incision. The long
duration of the symptoms, the absence of mobility and ballotte-
ment of the tumour, the oedema of the tissues in the loin, vari-
cocele, oedema of the leg 011 the affected side, and the detection
of enlarged glands within the abdomen along the brim of the
* Path. Hoc. Trans., vol. xlix., ]>. 175.



CANCER GRAFTED ON ANOTHER RENAL DISEASE.



599



pelvis or in the groin -will indicate extension of the disease and

make the prospects of an operation unfavourable. A careful

examination of all the other organs

of the body for signs of secondary

growths should be made.

Prognosis. When once the disease

has set in, sarcoma and carcinoma and

other malignant forms of new growths

advance steadily. The usual causes of

death are exhaustion, haemorrhage, or

uraemia ; the more rare are ulceration,

or bursting of the growth into the

peritoneum or through the diaphragm

into the lung causing pulmonary

abscess, or secondary new growths

in the brain (Figs. 91, 92, 93), vertebral

column and spinal cord, stomach or

intestine. The cases in which the

course and progress of the disease

seem to have been arrested for many

years, and then to have advanced

again, must be accepted with some

reserve. It is not improbable that

the earlier
symptoms in
these and
s u c h - 1 i k e
cases were

due to some other disease, and that the
malignant growth was engrafted upon it.
This was almost certainly the case in the
cancer which was associated with a large
renal cyst, and which I reported years
ago to the Pathological Society of London
(see the Transactions, vol. xxii., p. 172).
It is not necessary for the renal

tumour to have attained a large size before it causes secondary

deposits in other organs. Thus, in the case from which the speci-
mens illustrated in Figs. 91, 92, and 93 were taken, the renal new

growth was the size of a Tangerine orange, occupying the upper





Fig. 93. Secondary Tumour in
the Right Temporo-Sphen-
oidal Lobe. From the same
patient as Fig. 91.



Fig. 92. Secondary Tumour
of Brain. Exact size of
the tumour. From same
patient as Fig. 91.



600 SURGERY OF THE KIDNEY AND URETER.

pole of the kidney and contained within the renal capsule; the
lower part of the kidney was natural and no tumour was detect-
able during life ; and cases are on record in which new growths in
the neck or in the limbs have been secondary to a growth in the
kidney which was only detected during an operation or after death.

The duration of the tumour between the discovery of the
first symptom and the date of death, when no operation has
been performed, is from three to four years for carcinoma and
five to six years for sarcoma.

Guillet has collected five cases which survived from four to
ten years, and six cases from ten to sixteen year's.

The liability to secondary deposits, although not so decided in
the case of the kidney as in many other organs, is still great,
and is of course of extreme importance when considering the
advisability of an operation. Likewise, it is also of the greatest
importance in considering the prognosis and treatment of a renal
tumour to make sure that a primary growth in some other
organ has not been overlooked. Death may supervene very
rapidly if the mediastinal glands become secondarily affected.
In a case sent to me by Dr. Casley of Ipswich, in June, 1899,
there was a soft friable carcinoma affecting a portion of the left
kidney, the rest of the organ being quite healthy. It was for-
tunately decided that no operation was advisable, as the patient
died somewhat suddenly eighteen days later from dyspnoea due
to the pressure of carcinomatous deposits in the mediastinal
glands and the glands at the roots of the bronchi.

The following statistics will show the frequency with which
secondary deposits occur in malignant disease of the kidney.

Roberts in fifty-one cases found secondary growths in thirty-
one, while in the remaining twenty the kidney alone was attacked.
Dickinson found secondary growths in fourteen out of fifty-nine
cases. Rohrer in 115 cases found secondary growths in fifty,
and Guillet in forty-seven out of seventy cases. The lungs appear
to be the most frequent seat of secondary deposits, but the liver
and lumbar glands are also frequently affected while the suprarenals
are often implicated by direct continuity. Guillet has attempted
to compare the relative infect ivity between renal carcinoma and
sarcoma : he found secondary growths in thirty-two out of thirty-
eight cases of carcinoma and in four out of eight cases of sarcoma
in adults,, while in sarcoma of children he found secondary deposits



IMPROVEMENT IX THE MORTALITY OF NEPHRECTOMY. 601

in eleven out of twenty-four cases. From the consideration of
numerous statistics, Guillet concludes that secondary deposits occur in
about two-thirds of all cases of malignant disease of the kidney,
that they are very frequent in carcinoma, and occur in about
half the cases of sarcoma.

Treatment. Death is the inevitable result of every malig
riant tumour of the kidney if left to itself ; and so rapid is its
advance in children that the fatal termination usually .supervenes
very quickly upon the first discovery of the existence of the
tumour. If therefore nephrectomy can cure even a large minority
the operation would not only be justifiable but would be hailed
with satisfaction by surgeons and patients, even though death
in some instances is somewhat hastened by it. The chance
of cure by operation would be well worth the risk when the
alternative is certain death, especially if the chance can be shown
to be three or four to one in favour of the operation. This
point then ought to l)e first investigated.

Thanks to the better recognition of the signs of renal tumour
a diagnosis is nowadays made at a much earlier period than
formerly : at a period, that is, when the growth is yet small or
of but medium size. Moreover, the technique of nephrectomy
is better understood and better practised, so that the operation has
been performed under more favourable conditions than formerly,
with the result that not only has the whole of the disease been
more frequently removed but the immediate mortality of the
operation has been reduced between half and two-thirds.

Dickinson, writing in 1882, stated that so far as he knew
the kidney had been removed in eleven instances for malignant



Online LibraryHenry MorrisSurgical diseases of the kidney and ureter including injuries, malformations and misplacements (Volume 1) → online text (page 55 of 63)