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Angioma of the renal artery causing hypertension
A N G I O M A OF T H E R E N A L A R T E R Y C A U S I N G H Y P E R T E N S I O N S. KARANI, M.B., M.R.C.P., D.P.H, L. MORRIS, M.B., F.F.R., and I. RUSSELL, M.B., B.S. From the Departments of Medicine and Radiodiagnosis, St Nicholas' Hospital, Plumstead, London, S.E. 18 THE relationship between renal artery disease and hypertension is well established, and the commoner lesions resulting in hypertension have been well documented. The purpose of this communication is to record a rare renal artery lesion causing hypertension. CASE REPORT A twenty-seven year old male clerk was referred to hospital complaining of numbness of the left side of his face and body, dizziness, loss of balance, and blurring of vision in both eyes. The symptoms were of six weeks' duration. He had been discharged fit from the Royal Air Force seven years previously. Examination revealed a well developed, nervous man. The blood pressure was 255/130 mm. Hg. Both ocular fundi showed papillitis. There was slight right facial weakness. The heart was not enlarged, and there were no other physical signs. Chest x-ray was normal, as were the blood count and blood urea estimation. Administration of sodium amytal produced a reduction in the systolic blood pressure of 45 ram. Hg. Aortography revealed a slightly enlarged left kidney supplied by two renal arteries, but which was otherwise normal. On the right side there were two renal arteries, both of which, but especially the lower, supplied an extensive
cirsoid vascular malformation of the kidney hilus. In addition there was a small similar malformation on the surface of the kidney, apparently supplied by the upper renal artery (Figs. 1 and 2). One of the films (Fig. 1) showed a faint contrast shadow to the right of the lumbar spine, and this was thought to represent early filling of the inferior vena cava. A second injection with the catheter placed lower showed a tortuous vessel along the line of the ureter representing a cirsoid malformation of the ureteric artery (Fig. 3). The pyelogram phase (Fig. 4) showed deformity of the right renal pelvis and upper ureter, and this film taken together with the arteriogram phase demonstrated the relationship of the abnormal vessels to the deformity they were causing in the pelvis and ureter. Left nephrectomy was carried out by Mr J. Gabe using the posterior approach. The arteriographic findings were confirmed. Immediately following operation the blood pressure fell to 180/80 mm. Hg., but during the following twenty-four hours it rose to 290/170 mm. Hg. At this stage Serpasil was administered by intramuscular injection, and over the following ten days the blood pressure fluctuated around 210/ 140 mm. Hg. The blood urea and blood electrolytes were normal. Serpasil was given orally, and the patient was discharged on the tenth post-operative day, Three weeks later he was symptom-free, and the blood pressure was 210/140 mm. Hg. The blood urea was normal. There was still bilateral papillitis. Serpasil was continued. Twelve weeks after operation his blood pressure was 180/
Fro. I Aortogram showing two normal renal arteries on the left side. Two renal arteries on the right side supply a cirsoid malformation in the hilum of the kidney. There is probably early filling of the inferior vena cava (------~). 287
288
CLINICAL
RADIOLOGY
FIG. 2 Demonstrating the malformation in the kidney hilum, and a small malformation on the surface of the lower pole of the kidney (---+).
FIG. 3 FIG. 4 FIG. 3 - - A o r t o g r a m filling only the lower renal artery together with a pyelogram. The deformities of the pelvis and ureter are shown, also their relationship to the vascular malformation causing them. A tortuous ureteric artery is seen (---+). F m 4.--Pyelogram phase. The irregular deformity of the pelvis and upper ureter are shown. 85 m m . Hg. T h e fundi were clear, h e felt extremely well a n d was b a c k at work. Serpasil h a d been discontinued. Pathological examination (Dr I. W i l l i a m s ) . - - T h e kidney m e a s u r e d 10 by 6 by 4 cm. T h e k i d n e y itself s h o w e d n o m a c r o s c o p i c abnormality. I n the h i l u m o f the kidney there was a t o r t u o u s vessel.
M i c r o s c o p y o f t h e kidney s u b s t a n c e showed n o gross a b n o r m a l i t y o f t h e g l o m e r u l i or the t u b u l a r structures. There was n o obvious v a s c u l a r change. Sections o f t h e kidney h i l u m showed s o m e degree o f irregular thickening o f the i n t i m a of the arteries, with s o m e medial h y p e r t r o p h y . T h e adventitia was n o r m a l
ANGIOMA OF THE R E N A L ARTERY C A U S I N G H Y P E R T E N S I O N
DISCUSSION A review of the available literature has revealed only three similar cases investigated by aortography (Isaac, Brem, Temkin and Movius, 1957). The cause of the hypertension is presumed to be suppression of blood flow to the kidney as a result of the vascular malformation itself, as well as some degree of pre-renal shunting due to an arteriovenous fistula, the latter being suspected in the present case because of early filling of the inferior vena cava seen in one of the films. These changes probably produce a Goldblatt phenomenon. Irrespective of the exact mechanism of production of the hypertension, it may be noted from the present case, and those recorded by Isaac et al (1957), that removal of the abnormal kidney may be beneficial. This empirical observation is probably sufficient to warrant nephrectomy in the absence of any obvious abnormality in the other kidney. The radiological features are most striking. The deformity of the renal pelvis and the upper ureter on the pyelogram are fairly characteristic, and arteriography will confirm these findings. Glyn Thomas and Levin (1961) showed similar pyelographic deformity resulting from the development of tortuous collateral channels from the ureteric artery in a case of renal artery stenosis. In our case we were able to rule out renal artery stenosis as a
289
primary cause on the basis of the operative findings, and also the fact that the renal arteries themselves were involved in the malformation. In addition, there was a similar malformation on the kidney surface unrelated to the kidney substance. On the basis of these features we concluded that the vascular malformation was probably congenital in origin. Other conditions which may cause deformity of the pelvis and upper ureter similar to those seen in our case in the pyelogram phase include pyeloureteritis cystica, retroperitoneal metastases, primary retroperitoneal tumours and periureteric fibrosis. SUMMARY A case of cirsoid malformation of the renal artery, causing hypertension, is presented. The lesion was considered to be congenital in origin. There was a satisfactory response to nephrectomy. The striking and almost characteristic radiological features of this lesion are illustrated. REFERENCES ISAAC, F., BREM, T. H., TEMKIN, E., & Mowus, H. J. (1957). Radiology, 68, 679. GLYN THOMAS, R., & LEVIN, N. W. (1961). Brit. J. Radiol. 34, 438.
BOOK REVIEW Die Supervolt-Therapie. Edited by J. BECKER and G. SCHUBERT. Pp. 584, 421 illus. 1961. Stuttgart: George Thieme Verlag. Price, D.M. 145. " DIE SUPERVOLT-THERAPIE" is a large detailed textbook written in sections by radiotherapists and physicists at Heidelberg and Hamburg and edited by Professor Becker, Director of the Heidelberg University Radiotherapy Centre and by Professor Schubert, Director of the Hamburg University Gynaecological Department. It is written entirely in German. The word "supervolt-thetapie" is used to mean x-rays, gamma-rays and electrons of energy over IMV, though there is also discussion of other high energyparticles-protons, deuterons, c~-particles and neutrons. The book begins with a section on physics dealing with the absorption and scatter of the different rays, followed by a description of the different supervoltage sources---cascade and Van-de-Graaf generators, linea r and cyclical accelerators (betatron, cyclotron, synchrotron and synchrocyclotron), telecurie units and nuclear reactors. Full descriptions are given of dosimetry, practical methods and the theoretical background. There is next a one-hundred page section on radiobiology as applied to supervoltage, and then a long description of technical methods used in clinical radiotherapy. Finally, there is the major part of the book describing the use of supervoltage radiotherapy for different types of tumours and the results to be expected. This is a textbook of megavoltage radiotherapy, complete COO
in itself, and of course does not discuss superficial or kilovoltage x-ray therapy n o r the use of implants, moulds or artificial radio-active isotopes. All the authors refer to the recent English and American literature, often very extensively. Some of the methods described might seem a little unusual, for example the use of high energy electrons to treat a breast cancer by a tangent pair, the patient compressing her breast on to the applicator (Fig. 4, page 384). There is also an interesting section on the use of positive and negative grids or " sieves," which seems a deliberate use of inhomogeneous irradiation, perpetuated at a depth when using supervoltage radiation. It seems curious and perhaps unnecessary to do this when there is no difficulty about either skin reaction or adequacy of depth dose. A few English names are misspelt but the whole book seems in the best German academic tradition and is extremely well printed and produced, with many very clear diagrams and tables. There are thirty-five pages of references, titles being given in full. " D i e Supervolt-Therapie" appears designed as a textbook for the German-speaking radiologist who is already experienced in older methods of radiotherapy and who wishes to use one of the newer high energy units, whether this be a cobalt unit, betatron or some other generator; and can be strongly recommended for this purpose. It will also be very useful in other countries as a guide to German ideas on the correct use of megavoltage radiation. K. E. HALNAN.
cirsoid vascular malformation of the kidney hilus. In addition there was a small similar malformation on the surface of the kidney, apparently supplied by the upper renal artery (Figs. 1 and 2). One of the films (Fig. 1) showed a faint contrast shadow to the right of the lumbar spine, and this was thought to represent early filling of the inferior vena cava. A second injection with the catheter placed lower showed a tortuous vessel along the line of the ureter representing a cirsoid malformation of the ureteric artery (Fig. 3). The pyelogram phase (Fig. 4) showed deformity of the right renal pelvis and upper ureter, and this film taken together with the arteriogram phase demonstrated the relationship of the abnormal vessels to the deformity they were causing in the pelvis and ureter. Left nephrectomy was carried out by Mr J. Gabe using the posterior approach. The arteriographic findings were confirmed. Immediately following operation the blood pressure fell to 180/80 mm. Hg., but during the following twenty-four hours it rose to 290/170 mm. Hg. At this stage Serpasil was administered by intramuscular injection, and over the following ten days the blood pressure fluctuated around 210/ 140 mm. Hg. The blood urea and blood electrolytes were normal. Serpasil was given orally, and the patient was discharged on the tenth post-operative day, Three weeks later he was symptom-free, and the blood pressure was 210/140 mm. Hg. The blood urea was normal. There was still bilateral papillitis. Serpasil was continued. Twelve weeks after operation his blood pressure was 180/
Fro. I Aortogram showing two normal renal arteries on the left side. Two renal arteries on the right side supply a cirsoid malformation in the hilum of the kidney. There is probably early filling of the inferior vena cava (------~). 287
288
CLINICAL
RADIOLOGY
FIG. 2 Demonstrating the malformation in the kidney hilum, and a small malformation on the surface of the lower pole of the kidney (---+).
FIG. 3 FIG. 4 FIG. 3 - - A o r t o g r a m filling only the lower renal artery together with a pyelogram. The deformities of the pelvis and ureter are shown, also their relationship to the vascular malformation causing them. A tortuous ureteric artery is seen (---+). F m 4.--Pyelogram phase. The irregular deformity of the pelvis and upper ureter are shown. 85 m m . Hg. T h e fundi were clear, h e felt extremely well a n d was b a c k at work. Serpasil h a d been discontinued. Pathological examination (Dr I. W i l l i a m s ) . - - T h e kidney m e a s u r e d 10 by 6 by 4 cm. T h e k i d n e y itself s h o w e d n o m a c r o s c o p i c abnormality. I n the h i l u m o f the kidney there was a t o r t u o u s vessel.
M i c r o s c o p y o f t h e kidney s u b s t a n c e showed n o gross a b n o r m a l i t y o f t h e g l o m e r u l i or the t u b u l a r structures. There was n o obvious v a s c u l a r change. Sections o f t h e kidney h i l u m showed s o m e degree o f irregular thickening o f the i n t i m a of the arteries, with s o m e medial h y p e r t r o p h y . T h e adventitia was n o r m a l
ANGIOMA OF THE R E N A L ARTERY C A U S I N G H Y P E R T E N S I O N
DISCUSSION A review of the available literature has revealed only three similar cases investigated by aortography (Isaac, Brem, Temkin and Movius, 1957). The cause of the hypertension is presumed to be suppression of blood flow to the kidney as a result of the vascular malformation itself, as well as some degree of pre-renal shunting due to an arteriovenous fistula, the latter being suspected in the present case because of early filling of the inferior vena cava seen in one of the films. These changes probably produce a Goldblatt phenomenon. Irrespective of the exact mechanism of production of the hypertension, it may be noted from the present case, and those recorded by Isaac et al (1957), that removal of the abnormal kidney may be beneficial. This empirical observation is probably sufficient to warrant nephrectomy in the absence of any obvious abnormality in the other kidney. The radiological features are most striking. The deformity of the renal pelvis and the upper ureter on the pyelogram are fairly characteristic, and arteriography will confirm these findings. Glyn Thomas and Levin (1961) showed similar pyelographic deformity resulting from the development of tortuous collateral channels from the ureteric artery in a case of renal artery stenosis. In our case we were able to rule out renal artery stenosis as a
289
primary cause on the basis of the operative findings, and also the fact that the renal arteries themselves were involved in the malformation. In addition, there was a similar malformation on the kidney surface unrelated to the kidney substance. On the basis of these features we concluded that the vascular malformation was probably congenital in origin. Other conditions which may cause deformity of the pelvis and upper ureter similar to those seen in our case in the pyelogram phase include pyeloureteritis cystica, retroperitoneal metastases, primary retroperitoneal tumours and periureteric fibrosis. SUMMARY A case of cirsoid malformation of the renal artery, causing hypertension, is presented. The lesion was considered to be congenital in origin. There was a satisfactory response to nephrectomy. The striking and almost characteristic radiological features of this lesion are illustrated. REFERENCES ISAAC, F., BREM, T. H., TEMKIN, E., & Mowus, H. J. (1957). Radiology, 68, 679. GLYN THOMAS, R., & LEVIN, N. W. (1961). Brit. J. Radiol. 34, 438.
BOOK REVIEW Die Supervolt-Therapie. Edited by J. BECKER and G. SCHUBERT. Pp. 584, 421 illus. 1961. Stuttgart: George Thieme Verlag. Price, D.M. 145. " DIE SUPERVOLT-THERAPIE" is a large detailed textbook written in sections by radiotherapists and physicists at Heidelberg and Hamburg and edited by Professor Becker, Director of the Heidelberg University Radiotherapy Centre and by Professor Schubert, Director of the Hamburg University Gynaecological Department. It is written entirely in German. The word "supervolt-thetapie" is used to mean x-rays, gamma-rays and electrons of energy over IMV, though there is also discussion of other high energyparticles-protons, deuterons, c~-particles and neutrons. The book begins with a section on physics dealing with the absorption and scatter of the different rays, followed by a description of the different supervoltage sources---cascade and Van-de-Graaf generators, linea r and cyclical accelerators (betatron, cyclotron, synchrotron and synchrocyclotron), telecurie units and nuclear reactors. Full descriptions are given of dosimetry, practical methods and the theoretical background. There is next a one-hundred page section on radiobiology as applied to supervoltage, and then a long description of technical methods used in clinical radiotherapy. Finally, there is the major part of the book describing the use of supervoltage radiotherapy for different types of tumours and the results to be expected. This is a textbook of megavoltage radiotherapy, complete COO
in itself, and of course does not discuss superficial or kilovoltage x-ray therapy n o r the use of implants, moulds or artificial radio-active isotopes. All the authors refer to the recent English and American literature, often very extensively. Some of the methods described might seem a little unusual, for example the use of high energy electrons to treat a breast cancer by a tangent pair, the patient compressing her breast on to the applicator (Fig. 4, page 384). There is also an interesting section on the use of positive and negative grids or " sieves," which seems a deliberate use of inhomogeneous irradiation, perpetuated at a depth when using supervoltage radiation. It seems curious and perhaps unnecessary to do this when there is no difficulty about either skin reaction or adequacy of depth dose. A few English names are misspelt but the whole book seems in the best German academic tradition and is extremely well printed and produced, with many very clear diagrams and tables. There are thirty-five pages of references, titles being given in full. " D i e Supervolt-Therapie" appears designed as a textbook for the German-speaking radiologist who is already experienced in older methods of radiotherapy and who wishes to use one of the newer high energy units, whether this be a cobalt unit, betatron or some other generator; and can be strongly recommended for this purpose. It will also be very useful in other countries as a guide to German ideas on the correct use of megavoltage radiation. K. E. HALNAN.