anaesthetic management of phaeochromogytoma: a review of 26 cases
TRANSCRIPT
Actu anaesth. Scandinav. 1961, 5, 153-177.
ANAESTHETIC MANAGEMENT OF PHAEOCHROMOCYTOMA
A Review of 26 Cases
BY JEAN PRICE and OLE SECHER
During the past decade reports have appeared with increasing frequency of cases of phaeochromocytoma diagnosed clinically and operated upon success- fdy . Owing to the special difficulties arising with tumours of this type these operations were at first attended by a considerable risk and had a mortality of approximately 25 yo (J. B. GRAHAM (195 1)26, V. &CAR and E. M. PAPPER (1951)4). This risk has been significantly reduced during the past few years as a better understanding of the physiological changes occurring in connection with the removal of the tumour has been attained. The majority of cases are reported singly, but several large series of cases are also available in the litera- ture: V. APGAR and E. M. PAPPER (1951)4, 14 cases; J. E. THOMPSON and J. G. ARROWWOOD (1954)48, 14 cases; W. F. KVALE, J. T. PRIESTLEY and G. M. ROTH (1954)82, 25 cases; G. F. CAHILL and E. M. PAPPER (1956)'0, 47 cases; E. M. LANCE, W. R. CATE, G. W. LIDDLE and H. W. SCOTT (1958)35, 9 cases; E. DAHL IVERSEN (1959)11, 18 cases; and D. M. HUME (1960)31, 21 cases.
In the Scandinavian literature 69 cases of phaeochromocytoma have been reported (two cases are described repeatedly in two articles) : N. ALWALL and H. B. WULFP (1948)2, 1 case; L. AWN, Y. ISCREN and C. G. AMAN (1960)6, 1 case; P. BASTRUP MADSEN (1951)8, 1 case; A. BINDSLEV (1941)', 1 case; E. BORCH-JOHNSEN (1937)*, 1 case; V. 0. BJ~RK, H. LINDERHOLM, H. LUBLIN, B. PERNOW and B. TORNBERG ( 1959)9, 1 case; E. DAHL-IVERSEN ( 1959)11, 18 cases; P. EFFERSBE, T. GERTZ and A. LUND (1952)12, 1 case; A. ENCEL and N. TOLOCEN (1951)13, 2 cases; J. ESPENSEN and E. DAHL-IVERSEN (194f5)16, 2 cases; U. S. VAN EULER, A. LUND, A. OLSON and P. SANDBLOM (1953)16, 1 case; M. FABER (1951)17, 2 cases; M. FOG-POUUEN (1949)18, 1 case; R. FRIEDBERG (1956)20, 1 case; M. FROM-F'EDERSEN (1951)21, 1 case; P. CARSTEN and J. E. HEDIN ( 1958)22, 1 case; N. GJBL, R. DYBKJBR and J. FUNDER ( 1957)2S, 1 case; H. GORMSEN (1938)24, 2 cases; G. GQRTZ (1946)20, 1 case; J. HEDBY
From the Department of Anaesthesia, Rigshospitalet (University Hospital), Copenhagen, Denmark. Received July 11, 1961.
154 JEAN PRICE AND OLE SECHER
and H. LUBLIN (1951)28, 1 case; E. J. HOIST (1938)29, 3 cases; J. HOPFMEYER (1938y, 1 case; M. KASZNER ( 1955)33, 1 case; W. MATHISEN (1960)%, 10 cases; A. HANCHE OISEN (1960)38, 1 case; J. RIISHEDE (1951)M, 2 cases; J. RIISHEDE (1955)41, 1 case; T. M. SCHEINEN (1960)42, 1 case; P. SCHLEISNER (1954)43, 1 case; K. SIGROTH (1951)46, 1 case; J. P. STROMBECK and T. P. HEDBERG ( 1939)48, 1 case; M. SULAMAA and G. R. WALLGREN ( 1954)47, 1 case; E. WAALER (1946)49, 1 case; U. WAALBERG (1949)w, 1 case; K. WINKLER (1958)61, 1 case; K. WIRD-LQKK and K. G. TILLINGER (1953)62, 1 case.
TABLE 1 . 69 cases of phaeochromocytoma from the Scandinavian literature.
Diagnosed ......... 55 52 40 12 3 2 Undiagnosed. ..... 12 7 6 1 5 5 + 2 diagnosed cases in which treatment was not recorded.
A detailed study of these papers has been made, and the results are sum- marised in table 1. This table reveals a high mortality in the surgical patients- a fact which corresponds to the findings of the authors quoted in the opening paragraph of this paper. However, it should be noticed that these results are spread over the period from 1937 to 1960, and the highest mortality is found among the cases occurring early in this period.
E. DAHL-IVERSEN’S series, which is by far the largest in Scandinavia, con- sists of 18 cases with only two postoperative deaths; at the present time, the prognosis can therefore be considered good. In the group headed “undiagnosed” three of the six cases operated upon were cured. This may be explained by the fact that a diagnosis of phaeochromocytoma was first made by histological examination of the tissue removed at operation. The patients who received medical treatment, were either too poor for operation, or died before operation could be performed.
Phaeochromocytomata give rise to many problems which will directly con- cern the anaesthetist, and it should therefore be of interest to report the results from a large series based mainly on the cases reported by E. DAHL-IVERSEN, and supplemented by some previously unreported cases-giving a total of 26 cases. The literature contains reports not only of pre-operatively diagnosed cases, but also of a considerable number of cases in which the tumour was not diagnosed until the patient was undergoing an operation for some quite un- related condition. Extreme fluctuations in blood pressure often occur during these operations and may result in a state of shock, either during the surgical procedures or in the immediate postoperative period. Death is reported to
ANAESTHETIC MANAGEMENT OF PHAEOCHROMOCYTOMA 155
occur in 50 yo of these cases (see, for example, E. WAALER ( 1946)49, J. RIISHEDE (1951)40, V. APCAR and E. M. PAPPER (1951)4, T. R. STEEN (1957)44, J. C. S. AINLEY-WALKER and J. N. WOODWARD (1959)l).
The clinical picture, diagnosis and treatment of phaeochromocytoma have been dealt with most adequately in several excellent papers, and a large number of literature references are quoted (R. A. MILLAR ( 1957)37, J. RIISHEDE (1951)Sg and D. M. HUME (1960)31), to which the reader is referred. Here only a short account will be given.
Clinical Picture Phaeochromocytoma is a type of tumour which arises from the chromaffin
tissue in the organism, in the majority of cases from the adrenal medulla and more frequently from the right adrenal than from the left. The tumour may also be extra-adrenal ( 10-15yo) arising in the region of the sympathetic chain in the lumbar or thoracic paravertebral areas along the main vessels, in the organ of Zuckerkandl at the bifurcation of the aorta, around the coeliac plexus or the carotid and aortic bodies.
In 10% of the cases-most frequently children or young adults-the tumour may be multiple or bilateral; approximately 10% are malignant and show metastases. Malignant cases are usually without endocrine symptoms.
The disease occurs equally often in both sexes, and cases have been recorded in all age groups. The size of the tumour may vary from that of a pea to a child's head. Although this tumour is rare, and in the majority of cases histologically benign, it is, nevertheless, physiologically malignant, and death will usually occur within about 10 years after the development of symptoms unless the patient is subjected to operation.
The principal symptom of phaeochromocytoma is attacks of paroxysmal hypertension occurring in patients who show an otherwise normal blood pres- sure, but in some cases the disease may simulate essential hypertension, either made worse by paroxysmal attacks, or without any paroxysms at all.
A typical attack begins with abdominal pain, headache, anginal pain' and anxiety. Then follow palpitation, dyspnoea, nausea and perhaps vomiting, cold extremities and profuse sweating, dilated pupils, extreme exhaustion and, in some cases, fainting. Such a crisis may be over in a few minutes, but can last several hours or more than a day. During the attack the systolic blood pressure is elevated to very high values, and there will be very marked fluc- tuations. After the attack hyperglycaemia, glucosuria and albuminuria may be present. Very severe attacks may progress to lung oedema, cerebral hae- morrhage, shock and death.
Attacks may begin spontaneously or may be provoked by some specific cause, such as exertion, psychic trauma, specific movements, pressure in the renal region, etc. As the attacks increase in frequency, they will be more
156 JEAN PRICE AND OLE SECHER
prolonged, and hypertension between the attacks will gradually become a permanent feature of the disease.
The disease may be combined with thyroid enlargement and may be mistaken for essential hypertension, chronic nephritis, diabetes or thyreotoxicosis.
Cases are on record in which the tumour did not give rise to any symptoms (silent cases) until the patient was operated upon for an independent disease. Even a careful retrospective examination of the history proved unsuccessful in revealing signs of hyperadrenalaemia.
Diagnosis Diagnosis is relatively simple in typical cases, whereas atypical cases may
present diagnostic difficulties. Symptoms of the disease are a result of the high production and excretion
of noradrenalin by the tumour. The normal adrenal medulla produces a mixture of adrenalin-noradrenalin in a ratio of 85/15, whereas the phaeochro- mocytoma produces a mixture of an almost reversed ratio.
Inability to make a definite diagnosis in the doubtful cases is due to the considerable difficulty experienced in provoking an attack which is completely typical.
Pressure in the lumbar region will in some cases provoke an attack; this test is more successful when the patient is in anaesthesia and curarised, but it is not completely reliable. An attack can be provoked medically by injecting histamine 0.05 mg intravenously, or tetraethylammonium 300-500 mg; in the presence of phaeochromocytoma a considerable rise in blood pressure will result from the release of noradrenaline. This elevation of blood pressure may, in severe cases, proceed to a genuine attack with a crisis. Better results are obtained by using drugs which have an adrenolytic action4rugs which block the action of noradrenaline: dipenamine, which has a very prolonged action, or phentolamine (Regitin@), which given in a dose of 5 mg intravenously results in a short period of blood-pressure depression of about 35 mm Hg or more if the test is positive.
A definite diagnosis may be made by measuring the noradrenalin con- centration in a 24-hour specimen of urine: 0-50 pg is the normal range; 0-100 pg may be found in cases of essential hypertension; values of above 200 pg (between 190 and 1500 pg) are found in patients with a phaeochromocytoma (A. ENCEL and U. S. v. EULER (1950)13). During an attack the blood con- centration of adrenaline or noradrenaline may also be found to be elevated (A. LUND (1950)56).
Radiography may be helpful in making the diagnosis by means of retro- peritoneal pneumography and/or aortography. The results are generally con- sidered to be of the greatest value when the tumour is large, but in the experi- ence of Professor E. DAHL-IVERSEN even small tumours may be detected by this method.
ANAESTHETIC MANAGEMENT OF PHAEOCHROMOCYTOMA 157
Treatment Treatment of patients with a phaeochromocytoma consists in surgical
removal of the tumour, whereas medical treatment is unable to affect the course of the disease to any significant degree.
Localisation of the tumour is the primary problem, especially when the growth is very small and situated extra-adrenally. Furthermore, exploration of the operating field with even the slightest pressure on the tumour will cause an enormous rise in blood pressure. Immediately after removal of the tumour the blood pressure will show a rapid fall to subnormal levels. These fluctuations in blood pressure can be treated to some degree with various remedies, viz. phentolamine to reduce the pressure during the period of palpation and removal of thk tumour, and a continuous noradrenaline drip when the pressure falls seriously after removal of the tumour. The length of time during which nor- adrenaline will be necessary varies greatly with each case-perhaps from a few hours until a day or more, but the drip must be maintained until a stable blood pressure is obtained. One of the cases reported below is of particular interest in this respect; the patient received remarkably high doses of nor- adrenaline during the first few postoperative days.
Clinical Material Our series consists of 26 patients subjected to operation in the University
Hospital. Of these cases, 18 have previously been reported-one by the Surgical Department R (12), and 17 in E. DAHL-IVERSEN’S series which consisted of 17 surgically treated patients and one who died before operation could be per- formed. The previously unreported cases originate from the three surgical de- partments as follows: C, 6 cases; D, 1 case; and R, 2 cases.).
In these 26 cases (table 2), 42 operations were performed with the removal of 35 tumours and two postoperative deaths. These two deaths occurred among the first eight patients in the series and before noradrenaline was available in this hospital.
In 13 cases a single tumour was found in the right suprarenal gland and in six cases in the left suprarenal gland. In one of the six cases the tumour was found at autopsy, the patient having died in shock shortly after being subjected to an exploratory procedure on the right side. A single extra-adrenal tumour on the right side was found in one patient, and a single extra-adrenal tumour on the left side at autopsy in a second patient, who died after discharge, fol- lowing an operation for removal of the left suprarenal gland.
No phaeochromocytoma was found in two cases-one patient’s symptoms having been found to be due to chronic carbon monoxide poisoning (case 20)
*) We wish to thank the heads of the three departments, Professors E. DAHL-IVERSEN, E. HUSFELDT and F. THERKELSEN, for permission to use the material.
158 JEAN PRICE AND OLE SECHER
-and the last three cases had multiple tumours. Of these three, one patient had two extra-adrenal tumours, one above the right ovary and the second in the bladder wall. The remaining two patients had six tumours each. In the first case these were all on the left side-one suprarenal and the other five extra-adrenal, and were removed at two operations, five years apart. In the- second case there were two extra-adrenal tumours on the right side and four extra-adrenal tumours on the left side.
The 26 patients were 14 men and 12 woman whose ages ranged from 15 to 69 years. The diagnosis was suspected prior to operation in all cases. Sustained hypertension was present in 14 patients and paroxysmal in 12. In one patient the attacks had been present for 30 years, but the hypertension was still not sustained. No case of malignancy was foind in this series.
Premedication Atropine in combination with morphine and pethidine was given to 16
patients, and only in one case-a boy of 16-which is reported below were difficulties encountered which may have been due to the use of atropine. Hypnophen (barbiturate mixture) was given to this patient at the second attempt of operation. Scopolamine was given instead of atropine in 17 cases, and seven patients received pethidine alone.
Anaesthesia In 21 of the 42 operations performed, the anaesthesia consisted of a short-
acting barbiturate, nitrous oxide-oxygen, and Relaxan@ or Laudolissin@ for re- laxation, supplemented by pethidine when required. In four operations, succi- nylcholine chloride was used instead of Flaxedil, and additional relaxation was obtained with ether. Five of the earlier cases were anaesthetised with nitrous oxide and ether, and one of the patients was operated upon ten times in this anaesthesia. In one operation cyclopropane was used to supplement the anaes- thesia, but this patient maintained a constantly normal blood pressure through- out the procedure. Six cases received Regitin during the operation to control the rising blood pressure, and ten of the cases required a noradrenaline drip for the maintainance of blood pressure after the removal of the tumour.
The choice of anaesthetic agent appears to be unimportant providing that the airway is always kept free and anoxia religiously avoided-the slightest trace of anoxia stimulates production of the catechol amines. A curarising drug was used in all our more recent cases and no ill-effects were seen, although
Synonyms for the drugs used : Narcodorm@ = Enibomalum N. F. N. (short-acting barbiturate). Relaxan" = FlaxediP = Gallamonum N. F. N. Laudolissin' = Laudexonum N. F. N. Regitin" = Phentolaminum N. F. N.
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inci
sion
. T
umou
r re
mov
ed.
Exp
lora
- tio
n on
the
le
ft si
de.
Lum
bar
inci
sion
. T
umou
r re
mov
ed.
Lum
bar
inci
sion
. T
umou
r re
mov
ed.
Lum
bar
inci
sion
. T
umou
r re
mov
ed.
1. E
xpl.
lapa
- ro
tom
y.
2. L
. Sym
pa-
thec
tom
y.
3. R
. Sym
pa-
thec
tom
y.
Mor
phin
e A
trop
ine
Mor
phin
e .4
trop
ine
Peth
idin
e
Peth
idin
e
Mor
phin
e Sc
op.
Mor
phin
e A
trop
ine
Bar
bitu
rate
50
0 m
l bl
ood
B.P
. 15
0/10
0 <
N
,O
2701
200
Rel
axan
Bar
bitu
rate
65
0 m
l bl
ood
B.P
. 17
0/10
0 <
N
*O
2701
190
Rel
axan
Bar
bitu
rate
30
0 m
l bl
ood
B.P
. 1
4/7
0. N
o R
elax
an N
,O
fluc
tuat
ion
Cyc
lo
duri
ng
oper
atio
n.
Bar
bitu
rate
N
orad
rena
line
Rel
axan
N,O
dr
ip
Peth
idin
e M
etao
xedr
ine
400
ml
bloo
d
Bar
bitu
rate
R
egiti
n R
elax
an N
,O
Nor
adre
nalin
e dr
ip
300
ml
bloo
d
Bar
bitu
rate
N
orad
rena
line
Rel
axan
N,O
dr
ip
550
ml
bloo
d
Nor
adre
nalin
e dr
ip 2
day
s
e
m
W
c.
Q1
TABLE
2 (c
ontin
ued)
. .p
Post
oper
ativ
e %
I ::. I Dcpt.
1 Sex 1 Age
1 Side 1 Ope
ratio
n 1 Pru
ned.
I Anaes
thes
ia
duri
ng o
p.
1951
4. R
emov
al
R. s
upra
- re
nal.
5. L
.-sid
ed
expl
. 6.
R.-s
ided
ex
pl.
thor
aco-
to
my
(L) .
8. E
xpl. L.
rena
l re
gion
. 9.
Exp
l. la
pa-
roto
my.
7. E
xpl.
R
10. T
hora
cola
- M
orph
ine
at.
pa
roto
my
Scop
. ad
r.
(R).
25
1959
R
M
64
1. T
hora
co-
Peth
idin
e la
paro
tom
y
Rem
oval
of
supr
aren
al
+ ph
aeo-
ch
rom
o-
cyto
ma.
roto
my.
(I-).
2. E
xpl.
lapa
- Pe
thid
ine
N,O
-eth
er
1500
ml b
lood
Pe
thid
ine
Nar
codo
rm
Rel
axan
N
,O-p
ethi
- di
ne
Nar
codo
rm
500
ml b
lood
R
elax
an
dine
N
SO-p
thi-
+ E 3 ij
B t U 0 fi
Pre-
oper
ativ
e co
nditi
on u
n-
chan
ged.
26
1957
D
F
56
L
1.
Lum
bar
Mor
phin
e X
arco
dorm
90
0 m
l bl
ood
expl
. (K
).
Sco
p.
Rel
axan
N
,O-p
ethi
- di
ne
2. T
hora
cola
- N
arco
dorm
60
0 xn
l bl
ood
paro
totn
y R
elax
an
P4.
X20
-pet
hi-
Rem
oval
di
ne
supr
aren
al
3
glan
d.
3 c. 5 2 z
-
- m
n
k
166 JEAN PRICE AND OLE SECHER
some authors suggest that the possibility of the release of histamine by these drugs is a definite contra-indication to their use. We preferred Laudolissin in the cases in which tachycardia was present pre-operatively, as release of histamine is less with this drug than with Relaxan.
Six cases of particular interest will be described in more detail with reference to some of the difficulties encountered in the pre-operative, operative and post- operative periods.
CASE R E P O R T S
Case ]&-One of the most interesting of the series. The patient was a 69-year-old man, who for 30 years had complained of attacks, at intervals of days or weeks, of severe headache, palpitations, tachycardia, tremor of the hands and paraesthesiae in the fingers. The attacks averaged 3 minutes in duration, and while they lasted he was pale and felt very ill, although he had never fainted. He had never experienced disturbances of vision. Blood pressure recordings during attacks were around 210/110 mm Hg, but between attacks the pressure readings were normal, 14O/80-120/60 mm Hg. Excretion of noradrenaline and adrenaline
mm Hg. Case No.16 69 years male
200. Left suparenal tumor. Anhsthesia: Narcodorm 100 mg.
Laudolissin 40 18 '
NP-02
Fig. ].-Case 16, man aged 69. Shows the steep fall in blood pressure following removal of the tumour and the difficulty experienced in maintaining the blood pressure. Noradre- naline was given in a drip and by direct injection. During the last hour of anaesthesia it was possible to keep the pressure constant with a double-strength noradrenaline drip, but after the end of anaesthesia repeated noradrenaline injections every 5-10 minutes were
required.
ANAESTHETIC MANAGEMENT OF PHAEOCHROMOCYTOMA 167
in the urine was measured during an attack, and a total value of 6.1 pg was found in 19 ml urine. Abdominal aortography revealed the presence of a tumour, measuring 5 x 5 cm, above the left kidney. The histamine test was positive.
During the night prior to operation a crisis developed. This may have resulted from anxiety as to the outcome of the operation. He sweated a great deal and the blood pressure fluctuated considerably, varying between 140 mm Hg and 220 mg Hg systolic. At 8 a.m., premedication was given consisting of pethidine 75 mg and scopolamine 0.4 mg. Half an hour later the patient was extremely drowsy, but could be roused sufficiently to answer questions. There was a little vomiting; the blood pressure was still elevated to around 220/130, and the pulse was 144. Postponement of the operation was ordered. By 10 a.m. the blood pressure had fallen to normal levels, and the patient was still heavily under the influence of the premedication; it was therefore decided to take him down for operation.
Anaesthesia consisted of an intravenous barbiturate (Narcodorm) and nitrous oxide, with Laudolissin for maintainance of relaxation. Operation proceeded quite uneventfully until after the removal of a tumour weighing 32 g from the left adrenal gland. The blood pressure fell immediately to 50 mm Hg systolic and a noradrenaline drip containing 7 mg noradrenaline in 1000 ml glucose was set up. This proved quite ineffective, and it was necessary to give 0.5 mg noradrenaline intravenously about every 5 minutes during the rest of the operation. At the end of operation the patient could not be moved from the operating table owing to the difficulty in maintaining the blood pressure-this was only possible by continuing to give a noradrenaline drip with 8 mg per 400 ml glucose supple- mented by large doses of noradrenaline by direct injection (see fig. 1). At 8.45 p.m., a new noradrenaline drip was started containing 20 mg noradrenaline + 100 mg hydro- cortisone in 500 ml glucose. This concentration was later made even stronger, and repeated intravenous doses of noradrenaline, each of 0.25 mg, were still necessary. At 4 a.m. the following morning a blood pressure of around 100 mg Hg systolic could be maintained by the drip alone-now with a concentration of 28 mg noradrenaline in 500 ml glucose. By 8 a.m. the patient had received a total of 98.75 mg noradrenaline since the operation. The noradrenaline drip was continued during the next five days and from the time of operation the patient received a total of 383.3 mg noradrenaline. He made a complete recovery with no renal or cerebral damage and was later discharged.
Case I 1 was a 17-year-old girl transferred from the Medical Department with the diagnosis of a phaeochromocytoma. She had been admitted to hospital complaining of severe sweating, dyspnoea on exertion and palpitations. Examination revealed a constantly raised blood pressure with no marked paroxysms. There was tachycardia-pulse rate 140; serum noradrenaline was 1.7 pg ”/, and urinary noradrenaline 295 pg”,. Basal metabolism was very high, +83%; later the rate fell to +390,;. Retroperitoneal pneumography showed no adrenal enlargement.
Anaesthesia consisted of Narcodorm-nitrous oxide-ether, and a right-sided exploration was performed. A phaeochromocytoma, the size of a plum, was removed from the right adrenal and 5 minutes later a second tumour, the size of a hazelnut, was also extirpated. Following the removal of the second tumour the blood pressure fell from 210/120 to 170/100, but not further. The rest of the operation was uneventful.
During the postoperative period the blood pressure remained steadily elevated, and the patient continued to sweat profusely. A fortnight later an exploratory procedure was performed on the left side. On this occasion, the anaesthesia was Narcodorm, Relaxan and nitrous oxide. A large phaeochromocytoma was found in the centre of the adrenal gland. This tumour was enucleated, but as may be seen from the anaesthetic chart (fig. 2) , the blood pressure still remained elevated. Exploration was continued and three additional small phaeochromocytomata were found. Not until the fourth tumour had been removed did the blood pressure finally f a l l to normal. These four tumours weighed 10 g, 4 g, 2 g and 2 g, re- spectively. Postoperatively, urinary noradrenaline excretion showed normal values.
.paaourai WM mourni ise[ aqi w arnssaid u! doip daais e Lq pa~o1103 s~noruni in03 aqi
6u
89 I I3H33S 370 CINV Z3IIid Wd
ANAESTHETIC MANAGEMENT OF PHAEOCHROMOCYTOMA 169
by a further 0.1 mg 15 minutes later. Operation was abandoned, and the patient trans- ferred back to the Medical Department.
Nine days later the patient was again taken to the operating theatre, having been treated for some days previously with phenemal5 mg + 5 mg + 10 mg. On the day prior to operation a test dose of scopolamine 0.2 mg was given and resulted in a marked rise in blood pressure. Therefore it was decided not to use scopolamine at all, and instead the boy received hypnofen 250 mg intramuscularly and morphine 8 mg. In the corridor outside the operating theatre he was immediately given 100 Narcodorm intravenously and was almost asleep when taken into the theatre. Here he was fully anaesthetised with a further 150 mg Narcodorm and intubated on succinylcholine chloride. The blood pressure was 260/150 mm Hg at this time. Anaesthesia was continued with nitrous oxide, and Laudolissin was given for relaxation. A phaeochromocytoma, the size of a mandarin and weighing 47 g, was removed from the adrenal through a right-sided incision. Within10
mm Hg.
300.
280.
260-
240.
220 - 200-
180 - 160-
140-
120.
100-
80-
60-
40-
20: { II
o a
2 .: i f u s
Case No. 17 Ilt operi
I\ 1 Anssthcsia:
P P
t t t 4
Fig. 3.-Case 17, woman aged 56. Demonstrating the marked fluctuations in blood pressure during the period of exploration and immediate postoperative difficulties in stabilising the blood pressure. Continuous drip with noradrenaline was not necessary. Tumour finally
localised in the bladder wall.
170 JEAN PRICE AND OLE SECHER
minutes of the removal of the tumour the blood pressure fell to 90/70, and noradrenaline drip was commenced. The rest of the operation was uneventful, and so was the postoperative course, apart from the development of a small necrotic area in the left antebrachial region as a result of the noradrenaline drip.
Case I7.-This patient was a 56-year-old woman with a five-year history of hypertension in the region of 200/100. During the past four years there had been hypertensive attacks of a duration of 15-30 minutes, and on the last occasion 2 hours. The attacks were typical and were accompanied by coldness and clamminess of the skin, headache, palpitations, nausea, vomiting and anxiety. At the end of the attacks the patient had a sensation of warmth, and the skin became red. Urinalysis revealed a noradrenaline concentration of 1014 pg in a 24-hour specimen, with adrenaline 97 pg. There was dyspnoea on exertion, eye symptoms and the B.M.R. was +35%. As the tumour could not be localised, it was decided to carry out an exploratory surgical procedure.
On March 10, 1959, exploration of the left adrenal region was performed, but no tumour was found. During the procedure the blood pressure remained roughly normal, apart from a rise to 260 systolic on one occasion, and the procedure was well tolerated. One month later exploration of the right adrenal region was carried out-again with negative results, apart from the discovery of a leiomyoma in the intestine. This was removed after pressure on the tumour had produced a blood-pressure rise; it was thought to be a phaeochromocytoma until the pathological report was received.
On June 29, 1959, a laparotomy was performed. After a prolonged exploratory pro- cedure accompanied by palpation a phaeochromocytoma, the size of a thumb nail, was discovered in the tissue directly above the right ovary. Palpation in this region was ac- companied by a rise in blood pressure to unrecordable heights. The pulse rate increased to about 200. Removal of the tumour was followed by a sharp fall in blood pressure, which was counteracted by noradrenaline. Postoperative examination revealed a continued high urinary excretion of noradrenaline, but the patient was now free from her attacks, although the blood pressure level was still high.
Two months later the patient was re-admitted for follow-up examination. The adre- naline-noradrenaline excretion was so high that the presence of another active phaeochro- mocytoma was suspected. On October 2, 1959, prolonged surgical exploration was again carried out.
As is seen from the anaesthetic chart (fig. 3), palpation in the abdomen produced marked fluctuations in blood pressure, and, finally, a tumour was detected in the bladder wall. After removal of the tumour the blood pressure fell sharply, and the postoperative course was uneventful. After this operation-the fourth-the adrenaline-noradrenaline levels were within normal limits, and the patient was discharged in well-being.
Case 24 has previously been reported from Surgical Department R, the University Hospital (Ref. No. 12).
This patient was a young man in his thirties suffering from attacks of violent headache, which began with transient impairment of vision, palpitations and a cold sensation in the fingers and toes. The headache was synchronous with the pulse, and he was nauseated and vomited and sweated profusely. He was treated over a period of 3 years in various hospitals before it was discovered that during an attack his blood pressure was in the region of 220- 300 systolic and 130-170 diastolic. Physical examination of the patient between attacks revealed no abnormality. By means of fluorometric measurement3 a content of 2.4 ,ug% adrenaline + noradrenaline was found in the blood during one attack. One month after the diagnosis had been made he was operated upon for the first time, but no tumour could be found. During the next 18 months he was subjected to another eight negative exploratory procedures. Finally, careful palpation under anaesthesia + curare revealed the site of the tumour, and at the tenth operation a phaeochromocytoma, weighing 21 g, situated behind the inferior vena cava and the renal vein was removed through a thoraco-abdominal
ANAESTHETIC MANAGEMENT OF PHAEOCHROMOCYTOMA 171
incision. The attacks now ceased, but the patient had became psychologically disturbed by his long treatment and many operations and dependent on the large number of pethidine injections he had received during his illness, so that it was not very long before he again began having attacks which simulated the previous ones. No blood-pressure elevation was present, however, and the adrenaline excretion was not raised.
Case I0 was a patient operated upon for the first time in 1955, when she was 19 years old. She had eye symptoms consisting of flickering and spots before the eyes for which she had consulted an ophthalmologist. She also complained of headache, palpitations and sweating. The blood pressure was elevated in the region of 160/125. Urinalysis revealed 5.3 pgadrenaline and85 pg noradrenaline. At operation aphaeochromocytoma was removed from the left suprarenal region. The patient remained well with a blood pressure around 135/100 for 5 years. Then the attacks recommenced with a blood pressure fluctuating up to 225/100. Examination of the urine showed an excretion of 2039 pg adrenaline + nor- adrenaline in 24 hours.
The second operation took place on April 20, 1961, and five tumours were removed from the tissue surrounding the upper pole of the left kidney. Not until the fifth tumour was removed did the characteristic blood-pressure fall occur. A fortnight after the operation this patient had a total urinary adrenaline excretion of 688 pg in 24 hours.
DISCUSSION
Only two of the 26 patients with phaeochromocytoma operated upon in the University Hospital presented any real problems with regard to anaesthetic management. In the first case described-that of the 69-year-old man with a 30-year history of hypertensive attackssome of the difficulties may have arisen from the fact that operation was performed while the patient was in, or just emerging from, a crisis. That was the view of the surgeon who performed the operation, but is a difficult point to clarify. The patient had no history of sustained hypertension, and his hypertensive attacks had occurred over a period of 30 years without any marked ill-effects. When operation was commenced, the blood pressure was around the patient’s normal level, and he was adequately premedicated and very sleepy. The procedure was completely uneventful until after the tumour had been removed. If the crisis had played a large part in the picture, wide fluctuations in blood pressure should have been expected during the first half of the operation. This was not the case, and the great difficulty encountered in maintaining the blood pressure after removal of the tumour is more likely to have been a result of the very prolonged period during which the patient had experienced a high level of catachol amines in the body- over 30 years. Therefore the process of adjustment to a new physiological level could be expected to present grave difficulties.
Regitin was not given to this patient and was actually used only in six of the 26 cases subjected to operation. Its prolonged action-30 minutes or more -was felt to be undesirable in cases in which the tumour could easily be isolated, as the most important problem of management in these cases is the maintenance of blood pressure after removal of the tumour. Regitin does not prevent large fluctuations in the blood pressure during its period of action,
172 JEAN PRICE AND OLE SECHER
and the prolonged hypotension produced may make the situation worse; the tachycardia which is a side effect of this drug is also undesirable. Regitin is possibly of most help when a prolonged exploratory procedure is expected. Piperoxan may be a more satisfactory hypotensive agent owing to its shorter action. The fall in blood pressure is fully developed within a few minutes. The pressure remains low for a few more minutes and then gradually rises, reaching its former level after 25 minutes. However, this drug was not tried out in our series.
It was fortunate that there were no further complications in this case as the administration of such high doses of noradrenaline are potentially dangerous in themselves. Prolonged noradrenaline therapy produces a ganglion-blocking effect in the patient, and if administratiori of this agent is continued for any length of time after the operation, difficulties may arise on withdrawal of the drug. Cerebral and renal complications were also avoided despite the pro- longed period of hypotension experienced by the patient.
Local complications may also arise during the administration of nor- adrenaline. Necrosis of tissue may follow a leakage around the venipuncture or diffusion through the vein wall. As the risk of necrosis increases with the concentration of the solution, a noradrenaline drip with a high concentration should be avoided whenever possible. Generalised vasoconstriction in a limb from noradrenaline may end in gangrene; for this reason a noradrenaline drip should never be put up in the leg, but preferably in the upper part of the arm or in the femoral vein. Should local vasoconstriction occur, immediate in- filtration with acetylcholine is reported to reverse the condition (A. HAENNI and E. C. BONARD (1955)a7). Use of noradrenaline drip in these cases should be limited to as weak a solution as possible and for as short a period as possible. Only four cases in our series received a continuous noradrenaline drip for one day or more.
Case 14, the 16-year-old boy, went into a state of circulatory collapse on the first occasion he was anaesthetised. This occurred after intubation and after he had been placed in the left lateral position prior to operation. The pulse became very rapid ; the blood pressure rose, and ventricular tachycardia and lung oedema developed. As premedication he had received both morphine and atropine and the possibility of this having played a part must be considered. His arrival in the operating theatre with a blood pressure of 180/120 and pulse rate of 1011 would, however, suggest that this was not the case. Relaxan could have caused the marked tachycardia, and the fall in blood pressure when the boy began to breathe spontaneously would suggest that controlled respiration had had some influence. As controlled ventilation with its increased intrathoracic pressure normally lowers the blood pressure by impeding the venous return to the heart to some extent, it does not seem that the explanation lies here. Positioning of the patient on his left side would seem to be a much more plausible cause. The tumour removed at the operation 9 days later was as
ANAESTHETIC MANAGEMENT OF PHAEOCHROMOCYTOMA 173
large as a mandarin; therefore placing the patient on his side and at the same time attempting to avoid all pressure in the region of the tumour would present difficulties. I t would seem that the trouble arose due to a large leakage of secretion from the tumour during the turning of the patient.
On the second occasion on which the boy was anaesthetised .all precautions were taken with regard to premedication and a completely quiet induction, but in spite of this the blood pressure rose just as high as previously. But no signs of cardiac failure developed, and the operation was completed uneventfully.
Apart from these two cases no particular anaesthetic problems arose in connection with the patients in our series. Fluctuations in blood pressure during exploration were common and were usually also seen at the intubation, but quite a wide margin in blood-pressure levels are usually tolerated and are harmless, provided that constant observation of the patient’s condition is main- tained (A. FAULCONER (1957)’s). It is interesting, but probably natural, that the pulse rate does not fluctuate as much as the blood pressure. The steep drop in blood pressure occurring after the removal of a tumour is an indication that only a single tumour was present. A partial drop in blood pressure, as in cases 10 and 11, indicates that multiple tumours are present, and only when the last tumour is removed will the blood pressure drop to a low level. Case 10 also seems to indicate that “silent” tumours can be present and develop after an active tumour has been removed, or that further active tumours can develop later in life.
The normal requirements of good anaesthesiaquiet induction, good re- laxation, scrupulous care in the avoidance of anoxia, adequate analgesia-are obviously very important in these cases, but our experience does not suggest that any specific pre-operative preparation is of paramount importance. One author suggests that preparation for several days prior to operation with Regitin is desirable, and that this should be supplemented at the start of anaesthesia (D. M. HUME (1960)31). This author also recommends a relatively strong solution of noradrenaline for the drip after removal of the tumour. We feel that the possibility of having to treat the patient with heavy doses of nor- adrenaline is increased if Regitin is given routinely to all cases, owing to the prolonged hypotension resulting from the drug when the main source of the excess secretion of noradrenaline, i.e. the tumour, is removed. The dangers of this treatment appear to outweigh the possible benefits of the hypotension obtained with Regitin in the early part of the procedure, because, as stated above, we do not think that the possible fluctuations in blood pressure during exploration is so important, and anyway they cannot be avoided. Our experience suggests that, on the whole, patients with phaeochromocytoma tolerate the anaesthesia and operation fairly well. In the case of the patient subjected to 10 operations-nine exploratory-there were no complications as a result of the exploratory procedures, and he was not prepared for the operations in any special way.
174 JEAN PRICE AND OLE SECHER
V. APGAR and E. M. PAPPER (1951)’, in their paper on anaesthetic manage- ment, lay particular stress on the dangers of anaesthesia and surgery in the patient with a phaeochromocytoma-suspected or unsuspected. They suggest that surgery in the unsuspected case of phaeochromocytoma may carry a mortality of 50%, and in the suspected case a mortality of 24%. These figures appear to be questionable, even at the time when they were published and cannot stand at the present, as may be seen from our series and from the cases reported by others. In the 50% mortality in the unsuspected cases the calculation was made from collected cases taken from a period of over halfa century. Obviously the poor standard of anaesthesia in the larger part of this period has contributed to the high mortality rate. The fact that 70% of all phaeochromocytomata are found at autopsy suggest that many patients with this disease must be operated upon for unrelated conditions without being harmed; the explanation may be that it is only in those cases in which trouble ensues that the diagnosis can be made-post mortem. In APGAR and PAPPER’S own series, four patients with unsuspected phaeochromocytomata were oper- ated but all died-one not until 4 years later-whereas 14 patients with suspected phaeochromocytomata were subjected to exploratory procedures with no mortality. These figures cannot be regarded as real evidence in support of the view that a pre-operative diagnosis lowers the mortality, as, firstly, the numbers are too small to be significant; secondly, there is no control over the number of patients with an undiagnosed phaeochromocytoma who were also operated upon for other conditions and survived; and, thirdly, the only point of practical importance which arises with a prior knowledge of the patient’s disease is the possibility of elaborate preparation for surgery. As stated, our cases were not prepared in any specific way except in case 14, and this was at the second operation. Five of our cases had previously been operated upon for other conditions, and five were operated upon more than once for the removal of their phaeochromocytomata without complications.
SUMMARY
A review is presented of all the cases of phaeochromocytoma reported in the Scandinavian literature-69 cases in all. The results of operation or medical treatment in these cases are summarised in table 1. A short survey of the clinical picture, diagnosis and treatment of the disease follows. Our own series of 26 operated cases from the University Hospital is then presented with details of the cases and the operative results. A more detailed description is given of six cases which presented particular problems and points of interest. The first of these cases was an elderly man in whom great difficulty was experienced in maintaining the blood pressure after removal of the tumour. A total of 383.3 pg noradrenaline was given in the first five days after operation. The second case was a young girl with multiple turnours. Four tumoun were removed at
ANAESTHETIC MANAGEMENT OF PHAEOCHROMOCYTOMA 175
the second operation and the elevated blood-pressure level which persisted until the removal of the last tumour is clearly demonstrated on the anaesthetic chart. The third case described is that of a young boy who developed circulatory failure and lung oedema after being placed in the lateral position following induction of anaesthesia. Operation at a later date was uneventhl. The fourth case illustrates the difficulty which may be encountered in localising extra- adrenal tumours. This patient was a woman of 56 operated upon four times before the last tumour was found in the bladder wall. The marked fluctuations in blood pressure occurring during exploration are shown on the anaesthetic chart.
The next case has previously been published. The patient was a young man who was operated upon nine times over a period of 18 months before a phaebchromocytoma was found behind the inferior vena cava. The last patient was a young woman re-operated five years after the removal of a phaeochro- mocytoma4uring which time she had been symptom-free except for the last year. At the second operation five tumours were removed from the left renal regibn. A discussion of the problems encountered in the cases in our series follows.
ZUSAMMENFAS S UNG
Es wird ein uberblick uber alle Falle von Phaeochromocytom gegeben, die in der skandinavischen Literatur beschrieben wurden (insgesamt 69 Falle). Die Ergebnisse der Operation oder der konservativen Behandlung dieser Falle sind in Tabelle 1 zusammengefasst. Es folgt eine kurze Besprechung des kli- nischen Bildes, der Diagnose und Behandlung dieser Krankheit. Dann wird unsere eigene Serie von 26, an der Kopenhagener Universitatsklinik operier- ten, Falle in ihren Einzelheiten und den operativen Ergebnissen dargelegt. Eine ausfiihrlichere Beschreibung wird von 6 Fallen gegeben, die besondere Probleme und interessante Fragen aufgeworfen haben. Der erste dieser Falle war ein alterer Mann, bei dem der Blutdruck nach Entferning des Tumors nur sehr schwer aufrecht erhalten werden konnte ; insgesamt wurden 383,3 mg Noradrenalin in den ersten 5 Tagen nach der Operation gegeben. Der zweite Fall war ein junges Madchen mit multiplen Tumoren. Vier Tumoren wurden bei der zweiten Operation entfernt und der erhohte Blutdruckspiegel, der bis zur Entferning des letzten Tumors bestand, wird deutlich im Anaesthe- siebericht demonstriert. Der dritte beschriebene Fall betrifft einen Knaben, der nach Einleitung der Narkose bei der Umlagerung zur Seitenlage Kreis- laufversagen und Lungenodem entwickelte. Die zu einem spateren Zeitpunkt vorgenommene Operation war ohne Besonderheiten. Der vierte Fall zeigt die Schwierigkeiten auf, denen man bei der Lokalisation von extraadrenalen Tumoren begegnen kann. Diese Patientin war eine 56-jahrige Frau, die vier- ma1 operiert werden musste, bevor der letzte Tumor in der Blasenwand ge- funden wurde. Die ausgepragten Blutdruckschwankungen wahrend der Ex- ploration werden im Anaesthesiebericht aufgezeigt.
176 JEAN PRICE AND OLE SECHER
Der nachste Fall wurde bereits friiher veroffentlicht. Dieser Patient war ein junger Mann, der neunmal innerhalb eines Zeitabschnittes von 18 Monaten operiert wurde, bis schliesslich ein Phaeochromocytom hinter der Vena cava inferior gefunden wurde. Die letzte Patientin, eine junge Frau, wurde 5 Jahre nach der Entfernung eines Phaeochromocytoms re-operiert. Wahrend dieser Zeit war sie bis auf das letzte Jahr symptomfrei. Bei der zweiten Operation wurden 5 Tumoren aus der linken Nierengegend entfernt. Es folgt eine Dis- kussion der Probleme, die mit den Fallen unsere Serie aufgetreten sind.
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