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Cerebral arteriovenous fistula
Clinical Radiology (1994) 49, 144-145
Correspondence Letters are published at the discretion of the Editor. Opinions expressed by correspondents are not necessarily those of the Editor. Unduly long letters may be returned to the authors for shortening. Letters in response to a paper may be sent to the author of the paper so that the reply can be publishedin the same issue. Letters shouM be typed double spaced and shouM be signed by all authors personally. References should be given in the style specified in the Instructions to Authors at the front of the Journal.
LARGE BOWEL EXAMINATION IN THE FRAIL ELDERLY PATIENT SIR - We read with interest the article by J. J. Day on the use of CT for examination of large bowel in the frail elderly patient [1]. There is obvious benefit in performing CT of the large bowel without the use of rectal contrast; however, I am unsure whether the technique is sensitive enough to be of use except for patients with a severely limited life expectancy, Previous attempts to identify large bowel lesions revealed the absolute necessity o f using rectal air as a contrast medium. Failure to use air resulted in a 32% incidence of missed colonic carcinoma (11/34) as opposed to 5% incidence in the group with rectal insuftlation (3/56). These workers also noted the significant help of performing the examination on the prepared colon [2]. Further studies need to be performed to evaluate the sensitivity of CT. In particular optimal bowel preparation, and the requirement for rectal contrast need to be established, as does the correct use of oral contrast and the CT technique itself. Only when the above have been established should the technique be extended to a larger group of elderly patients. The authors claim that a negative CT report can be assumed to be correct unless there are very serious signs and symptoms, but how reliable is this? A recent study showed that the sensitivity o f colonic symptoms for the demonstration of colonic disease was only 44% [3]. Even in a frail elderly patient it is important to detect a carcinoma at the earliest stage possible. Any avoidable delay in making the diagnosis in these patients will inevitably increase the surgical risk of operation. A patient's life expectancy may be limited but how can one judge that, and further where does one draw the line? One final point about the study is that reliance was made on verbal consent from this group of elderly patients to participate in the trial. Results state that 11 out of the 36 patients examined by both techniques were unable to make a valid comparison due to dementia or illness. One must be wary of performing such a study on these patients who might not be capable of giving informed consent. G. T. ROTTENBERG U. PATEL
Department of Diagnostic Imaging The Middlesex Hospital Mortimer Street London W1
References
multiple. Demonstration of this abnormal vein, classically using venographic techniques, is often important in diagnosis of the syndrome itself. Deep venous aplasia/hypoplasia has been quoted in between 18 % [4]40% [5] of affected limbs. These figures may well be overestimates due to selective flow of contrast up the lateral channel at venography and failure to opacify the deep veins. The tissue overgrowth that occurs in the Klippel-Trenaunay syndrome has been shown to be due to venous obstruction without a change in blood flow [6]. It is important to differentiate this syndrome from the morphologically similar, but unrelated, Parkes-Weber Syndrome [7]. This syndrome involves limb hypertrophy and varicosities which result from the presence of multiple, usually microscopic, arteriovenous fistulae. D. C. HOWLETT A. B. AYERS
Department of Radiology St Thomas' Hospital Lambeth Palace Road London SE1 7EH
References
1 Dunn WK, Jaspan T. Case report: cerebral arteriovenous fistula in the Klippel-Trenaunay Weber syndrome, Clinical Radiology 1993; 48:134-136. 2 Klippel M, Trenaunay P. Du naevus variquex osteo-hypertrophique. Archives Gbnbrale de Medicine (Paris) 1900;185:641 672. 3 Browse NL, Burnand KG. Lea Thomas M. Disease of the veins; pathology, diagnosis and treatment. London: Edward Arnold, I988. 4 Baskerville PA, Ackroyd JS, Thomas ML, Browse NL. The KlippelTrenaunay syndrome: clinical, radiological and haemodynamic features and management. British Journal of Surgery 1985;72:232236. 5 Lea Thomas M. Phlebography of the lower limb. Edinburgh: Churchill Livingston, 1982. 6 Lindenauer SM. The Klippel-Trenaunay syndrome; varicosity, hypertrophy and haemangioma with no arteriovenous fistula. Annals of Surgery 1965;162:256. 7 Parkes-Weber F. Angioma formation in connection with hypertrophy of the limbs and hemi-hypertrophy. British Journal of Dermatology 1907; 19:231.
1 Day J J, Freeman AH, Coni NK, Dixon AK. Barium enema or computed tomography for the frail elderly patient? Clinical Radiology 1993;48:48-51.
2 Balthazar EJ, Megibow AJ, Hulnick D, Naidich DP. Carcinoma of the colon: detection and preoperative staging by CT. American Journal o f Roentgenology 1988;150:301 306. 3 Mclntyre AS, Long RG. Prospective survey of investigations in outpatients referred with iron deficiency anaemia. Gut 1993;34:11021107.
CEREBRAL ARTERIOVENOUS FISTULA SlR- We would like to comment on the recent paper by Dunn and Jaspan [1] presenting a case of cerebral arteriovenous fistula in a patient with what they describe as the Klippel Trenaunay-Weber syndrome. The Klippel Trenaunay syndrome is a rare mesodermal abnormality first described in 1900 [2]. It comprises a triad of cutaneous naevus, bone and soft tissue hypertrophy and venous anomalies, usually confined to a single lower limb. The venous anomalies include varicose veins, deep venous aplasia/ hypoplasia and an abnormal venous channel [3]. This channel tends to run up the posterolateral aspect of the affected limb and has communication with the deep venous system. The communication is usually single via the profunda femoris or internal lilac vein, but may be
SIR--I am grateful to Dr Howlett et al. for their comments on the differentiation of the syndrome described in 1900 by Klippel and Trenaunay [1] and the patients identified shortly afterwards by Weber [2,3] with a similar morphological appearance who had associated arteriovenous fistulas within the affected limb. I should like to point out that while some authors feel that these patients should be differentiated as the Parkes-Weber syndrome, the majority agree that the KlippelTrenaunay and Parkes-Weber syndromes are variants of the same condition which is usually referred to by the combined term of the Klippel-Trenaunay-Weber syndrome [4-8]. This condition is considered to be a member of a group of conditions termed the neurocutaneous syndromes or phakomatoses [9]. W. K. D U N N
Department of Neuroradiology University Hospital Clifton Boulevard Nottingham NG7 2UH
References
i Klippel M, Trenaunay P. Du naevus variquex osteo-hypertrophique. Archives Gbnkrale de Medicine (Paris) 1900;3:641 672. 2 Weber FP. Angioma formation in connection with hypertrophy of limbs and hemihypertrophy. British Journal of Dermatology 1907; 19:231 235. 3 Weber FP. Haemangiectatic hypertrophy of limbs - congenital phle-
Correspondence Letters are published at the discretion of the Editor. Opinions expressed by correspondents are not necessarily those of the Editor. Unduly long letters may be returned to the authors for shortening. Letters in response to a paper may be sent to the author of the paper so that the reply can be publishedin the same issue. Letters shouM be typed double spaced and shouM be signed by all authors personally. References should be given in the style specified in the Instructions to Authors at the front of the Journal.
LARGE BOWEL EXAMINATION IN THE FRAIL ELDERLY PATIENT SIR - We read with interest the article by J. J. Day on the use of CT for examination of large bowel in the frail elderly patient [1]. There is obvious benefit in performing CT of the large bowel without the use of rectal contrast; however, I am unsure whether the technique is sensitive enough to be of use except for patients with a severely limited life expectancy, Previous attempts to identify large bowel lesions revealed the absolute necessity o f using rectal air as a contrast medium. Failure to use air resulted in a 32% incidence of missed colonic carcinoma (11/34) as opposed to 5% incidence in the group with rectal insuftlation (3/56). These workers also noted the significant help of performing the examination on the prepared colon [2]. Further studies need to be performed to evaluate the sensitivity of CT. In particular optimal bowel preparation, and the requirement for rectal contrast need to be established, as does the correct use of oral contrast and the CT technique itself. Only when the above have been established should the technique be extended to a larger group of elderly patients. The authors claim that a negative CT report can be assumed to be correct unless there are very serious signs and symptoms, but how reliable is this? A recent study showed that the sensitivity o f colonic symptoms for the demonstration of colonic disease was only 44% [3]. Even in a frail elderly patient it is important to detect a carcinoma at the earliest stage possible. Any avoidable delay in making the diagnosis in these patients will inevitably increase the surgical risk of operation. A patient's life expectancy may be limited but how can one judge that, and further where does one draw the line? One final point about the study is that reliance was made on verbal consent from this group of elderly patients to participate in the trial. Results state that 11 out of the 36 patients examined by both techniques were unable to make a valid comparison due to dementia or illness. One must be wary of performing such a study on these patients who might not be capable of giving informed consent. G. T. ROTTENBERG U. PATEL
Department of Diagnostic Imaging The Middlesex Hospital Mortimer Street London W1
References
multiple. Demonstration of this abnormal vein, classically using venographic techniques, is often important in diagnosis of the syndrome itself. Deep venous aplasia/hypoplasia has been quoted in between 18 % [4]40% [5] of affected limbs. These figures may well be overestimates due to selective flow of contrast up the lateral channel at venography and failure to opacify the deep veins. The tissue overgrowth that occurs in the Klippel-Trenaunay syndrome has been shown to be due to venous obstruction without a change in blood flow [6]. It is important to differentiate this syndrome from the morphologically similar, but unrelated, Parkes-Weber Syndrome [7]. This syndrome involves limb hypertrophy and varicosities which result from the presence of multiple, usually microscopic, arteriovenous fistulae. D. C. HOWLETT A. B. AYERS
Department of Radiology St Thomas' Hospital Lambeth Palace Road London SE1 7EH
References
1 Dunn WK, Jaspan T. Case report: cerebral arteriovenous fistula in the Klippel-Trenaunay Weber syndrome, Clinical Radiology 1993; 48:134-136. 2 Klippel M, Trenaunay P. Du naevus variquex osteo-hypertrophique. Archives Gbnbrale de Medicine (Paris) 1900;185:641 672. 3 Browse NL, Burnand KG. Lea Thomas M. Disease of the veins; pathology, diagnosis and treatment. London: Edward Arnold, I988. 4 Baskerville PA, Ackroyd JS, Thomas ML, Browse NL. The KlippelTrenaunay syndrome: clinical, radiological and haemodynamic features and management. British Journal of Surgery 1985;72:232236. 5 Lea Thomas M. Phlebography of the lower limb. Edinburgh: Churchill Livingston, 1982. 6 Lindenauer SM. The Klippel-Trenaunay syndrome; varicosity, hypertrophy and haemangioma with no arteriovenous fistula. Annals of Surgery 1965;162:256. 7 Parkes-Weber F. Angioma formation in connection with hypertrophy of the limbs and hemi-hypertrophy. British Journal of Dermatology 1907; 19:231.
1 Day J J, Freeman AH, Coni NK, Dixon AK. Barium enema or computed tomography for the frail elderly patient? Clinical Radiology 1993;48:48-51.
2 Balthazar EJ, Megibow AJ, Hulnick D, Naidich DP. Carcinoma of the colon: detection and preoperative staging by CT. American Journal o f Roentgenology 1988;150:301 306. 3 Mclntyre AS, Long RG. Prospective survey of investigations in outpatients referred with iron deficiency anaemia. Gut 1993;34:11021107.
CEREBRAL ARTERIOVENOUS FISTULA SlR- We would like to comment on the recent paper by Dunn and Jaspan [1] presenting a case of cerebral arteriovenous fistula in a patient with what they describe as the Klippel Trenaunay-Weber syndrome. The Klippel Trenaunay syndrome is a rare mesodermal abnormality first described in 1900 [2]. It comprises a triad of cutaneous naevus, bone and soft tissue hypertrophy and venous anomalies, usually confined to a single lower limb. The venous anomalies include varicose veins, deep venous aplasia/ hypoplasia and an abnormal venous channel [3]. This channel tends to run up the posterolateral aspect of the affected limb and has communication with the deep venous system. The communication is usually single via the profunda femoris or internal lilac vein, but may be
SIR--I am grateful to Dr Howlett et al. for their comments on the differentiation of the syndrome described in 1900 by Klippel and Trenaunay [1] and the patients identified shortly afterwards by Weber [2,3] with a similar morphological appearance who had associated arteriovenous fistulas within the affected limb. I should like to point out that while some authors feel that these patients should be differentiated as the Parkes-Weber syndrome, the majority agree that the KlippelTrenaunay and Parkes-Weber syndromes are variants of the same condition which is usually referred to by the combined term of the Klippel-Trenaunay-Weber syndrome [4-8]. This condition is considered to be a member of a group of conditions termed the neurocutaneous syndromes or phakomatoses [9]. W. K. D U N N
Department of Neuroradiology University Hospital Clifton Boulevard Nottingham NG7 2UH
References
i Klippel M, Trenaunay P. Du naevus variquex osteo-hypertrophique. Archives Gbnkrale de Medicine (Paris) 1900;3:641 672. 2 Weber FP. Angioma formation in connection with hypertrophy of limbs and hemihypertrophy. British Journal of Dermatology 1907; 19:231 235. 3 Weber FP. Haemangiectatic hypertrophy of limbs - congenital phle-