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SIMULTANEOUS CENTRAL VENOUS AND ARTERIAL BLOOD SAMPLING FOR CATECHOLAMINE ASSAY IN PHAEOCHROMOCYTOMA
526 THORACIC VAGECTOMY FOR VAGAL BRADYCARDIA IN CHILDREN
SIR,-We have now performed four selective right thoracic vagectomies for progressive bradycardia in childhood, with excellent results. In progressive vagal bradycardia the resting heartrate gradually falls resulting in lethargy, poor feeding, erratic breathing, and, if untreated, progresses to cardiac and then respiratory standstill. In the early phase the children often have epileptiform seizures but never experience postictal symptoms. A heartrate which drops to 80/s or below appears to be significant and epileptiform activity may be an attempt to increase the heartrate. These seizures, during which the heartrate may be over 100/s, are often followed by a drop in rate to 20 - 30/s when the child or even to zero in advanced cases. Since our first case report we have successfully operated on 3 more children with generalised hypervagism and progressive bradycardia. That is, before surgery, when not receiving atropine, at times their heartrates would drop to zero; after surgery they had heartrates between 80 and 130/s depending upon their degree of
relaxes,
activity. types of vagal bradycardia; local-resulting from such as pressure from a tumour or adhesions; and central-due to immaturity or abnormality of the. central vagal nuclei, as in our cases, which results in generalised hypervagism with gastrointestinal and, to a lesser degree, respiratory symptoms, as well as cardiac symptoms the most lethal component of which is progressive bradycardia. The disease may also provide a clue about the cause of and possible treatment for at least one form of sudden infant death syndrome (sits).22 When the bradycardia is vagal in origin there is an immediate response to atropine given intravenously or intramuscularly, 0’ I mg/kg. In the progressive form, tachyphylaxis soon raises the effective dose of atropine to high levels, and the viscous mucus in the respiratory and gastrointestinal tracts can cause problems. The response to atropine, which is the key to diagnosis, means that the bradycardia is due to vagal inhibition of impulses which arise in the sino-atrial node which is innervated by the right vagus nerve, and has minimal innervation from the left. The nerve fibres (about 3 branches) to the node come off the right recurrent laryngeal nerve, and 2 or 3 more branches come directly from the trunk of the right vagus, just distal to the origin of the recurrent laryngeal. By cutting only these fibres the recurrent laryngeal nerve is spared and there is no right vocal cord paralysis, no gastrointestinal tract effects (as would result from a truncal vagotomy), and few or no pulmonary effects, such as bronchospasm. Vagectomy is performed through a third intercostal space right lateral thoracotomy. The posterior pleura is incised from the azygos vein to the origin of the right subclavian artery; the vagal trunk is identified and followed up to the origin of the right recurrent laryngeal nerve. The first 3 fibres arising from the recurrent laryngeal are then divided, as are all branches of the vagal trunk distal to the recurrent laryngeal up to a point just above the azygos vein. If the heartrate is stable on atropine, surgery can be performed without a temporary venous pacemaker in place. If unstable, its use as an added safety factor is strongly advised. The procedure is well tolerated. Feeding can usually be started on the first or second day postoperatively. All our patients had been discharged by the fifth postoperative day. Vagectomy has proved simpler, more dependable, and better tolerated for a longer period than insertion of a pacemaker-especially in the small child. Our oldest patient, who had the operation in 1971 aged 3 years, is now a teenager. Our most recent patient is aged 4 years and has Down syndrome. The central type ofvagal bradycardia has been found most often in children with There
are two
phenomena
1
Coryllos EV, Kenigsberg K, Delaney T, Mofenson H. Thoracic vagectomy for vagal bradycardia. NY State J Med 1976; 76: 706. 2. SIDS-one possible etiology and its management. Presentation spring scientific sessions of New York Society for Thoracic Surgery, memorial Hospital, New York City, 1981.
system abnormalities, e.g. Down syndrome, cerebral microcephaly, palsy. Vagectomy is a simple, low-risk procedure for the treatment of progressive vagal bradycardia in children. Results have been good and lons-lastins. with low morbiditv. ELIZABETH V. CORYLLOS M. MOHTASHEMI T. DELANEY Nassau Hospital, Mineola, New York, U.S.A. J. GREENSHER central
nervous
or
SIMULTANEOUS CENTRAL VENOUS AND ARTERIAL BLOOD SAMPLING FOR CATECHOLAMINE ASSAY IN PHAEOCHROMOCYTOMA
SIR,-Central venous blood sampling for catecholamine assay is a diagnostic procedure for preoperative localisation of phaeochromocytomas. It has been recommended when radiographic studies fail to localise the tumour.1 Central venous blood sampling can be particularly useful in localising small, multiple, or metastatic tumours, as well as excluding an intrathoracic or neck localisation. Furthermore, it may avoid an unnecessary laparotomy.When a phaeochromocytoma is multiple, metastatic, or has abnormal venous drainage, multiple plasma catecholamine peaks may be detected. This also occurs if the tumour’s catecholamine secretion rate varies significantly during sequential central venous blood sampling, and might be a pitfall in accurate localisation of phaeochromocytomas. To avoid drawing false conclusions from the catheterisation date, we suggest that catecholamine levels be measured in central venous and arterial (brachial) blood samples, drawing the arterial sample 15 seconds after the corresponding central venous sample has been taken. Case 1 (see In this
figure).
patient the diagnosis of phaeochromocytoma was suggested by continued hypertension and abnormally high urinary excretion of 4-hydroxy 3-methoxy mandelic acid (HMMA)2 (15-25 3 mg/g creatinine) and free catecholamines3 (noradrenaline: 1256—2427 g/gcreatinine, adrenaline: 757-1478 pglg creatinine). Central venous blood sampling (no drug treatment) showed two 1.
Manger WM, Gifford RW, Jr. Pheochromocytoma. Central venous blood sampling for catecholamine assay. New York: Springer-Verlag, 1978: 266-75 2. Pisano JJ, Crout JR, Abraham D. Determination of 3-methoxy-4-hydroxymandelic acid in urine. Clin Chim Acta 1962; 7: 285-91. V, Brunori CA, Valori C A sensitive and specific fluorimetric method for the determination of noradrenaline and adrenaline in human plasma. Clin Chim Acta 1970; 30: 587-94.
3. Renzini
Plasma catecholamine concentration (NA= = noradrenaline; A= adrenaline) during central venous blood sampling (CVBS) from right atrium (RA) to lumbar 5 (L,), and simultaneous arterial blood
sampling (ABS).
527 Since corresponding arterial catecholamine levels were excluded variation in the tumoral secretion rate and stable, multiple phaeochromocytomas. In fact the lumbar 3 peak suspected (figure) was due to an additional vein discharging into the vena cava, as later confirmed both by retrograde phlebography and surgical resection.
peaks.
we
measured IgA levels in 20 patients with rotator cuff lesions. In our group of frozen-shoulder patients, we did not detect B27 in a single patient. Additionally the IgA levels were similar in frozen shoulder, rotator cuff, and control groups. Department of Rheumatology, Westminster Hospital,
M. A. STODELL
London SW1
Case2 The second
patient was investigated because of recurrence of abnormal excretion of HMMA (32-55 mg/g and hypertension creatinine) and noradrenaline (4265-7564 jL
tumour
Institutes of Medical Pathology and Clinical Medicine, University of Perugia, 06100 Perugia, Italy
GEREMIABOLLI PIERPAOLO DE FEO PIETRO COMPAGNUCCI MARIA G. CARTECHINI FAUSTO SAMTEUSANIO PAOLO BRUNETTI
FROZEN SHOULDER
SiR,—The aetiology of frozen shoulder or capsulitis remains unknown. Bulgen and co-workers, of Cambridge, have implicated a component and postulate a localised autoimmune reaction. These workers have reported a 36% incidence of HLA B27 in frozen shoulder associated with a low serum IgA level which persists after recovery. To our knowledge, these findings remain unconfirmed. In a review of the frozen shoulder, Kessel et al.could not find a genetic linkage or any disturbance of IgA levels in a series of 50 patients. We have studied 21 patients with frozen shoulder using the same criteria for diagnosis as Bulgen et al. We have also
hereditary
Centre for Rheumatic Diseases, Baird Street,
PREVENTION OF INTRAVENTRICULAR HAEMORRHAGE BY PHENOBARBITONE Ji,——
1 Bulgen DY, Hazleman BL,
Voak D HLA-B27 and frozen shoulder. Lancet 1976; i: 1042-44 Bulgen D, Hazleman B, Ward M, McCallum M. Immunological studies in frozen shoulder Ann Rheum Dis 1978; 37: 135-38. 3 Kessel L, Bayley I, Young A. The frozen shoulder. BrJ Hosp Med 1981, 25: 334-38.
1 11C
C2LL-iLL11; ICpUi
L Ui 1-l 1-,IUIIII amu U-WUljCIo
V1.Ub. 1, p.
215) states that ultrasound scans of the brain were done on the 3rd, 4th or 5th postnatal day. If the results of the study are to be accepted fully, we think that clarification of the time of scanning is called for. Since the end of 1978 we have prospectively scanned the brains of all small preterm infants admitted to our neonatal unit. The scanners used were the ADR 2130 linear-array system with 5 MHz and 7 MHz probes, and more recently the Siemens Diasonics RA 1 sector scanner with a 7 5 MHz probe. The infants’ brains were repeatedly examined through the skull and through the anterior fontanelle. In our experience about a third of all haemorrhages first appeared during or after the third day. These late haemorrhages were usually small (grade I). Donn and co-workers’ study shows a notable lack of grade I haemorrhages in the treatment group. If the age at scanning was, in general, later in the control group than in the treatment group, the conclusion that phenobarbitone was effective might be open to question. P. L. HOPE Department of Paediatrics, School of Medicine,
University College London, London WC1E
6JJ
R. J. THORBURN A. L. STEWART E. O. R. REYNOLDS
INTENSIVE CARE AND THE VERY LOW BIRTH WEIGHT INFANT
SIR,-Stewart et al. recently published a paperl showing the decrease in mortality for very low birth weight (VLBW) infants during the years 1946 to 1977, with a stable and reasonably low handicap-rate of 6 to 8% since 1960. Chalmers and Mutchcommenting on this paper, suggested that with a stable handicap rate and decreasing mortality the absolute number of handicapped children would rise. However, discussion is hard to interpret since it is difficult to obtain exact figures for welldefined populations of VLBW infants. Although Stewart et al. claim that they reviewed only results from studies where all live births in the population were accounted for, nevertheless, they reported results obtained in patients referred after birth to neonatal intensive care units.3-6 We have found no increase in the handicap-rate nor in the absolute number of handicapped children during the 21-years from 1959 to 1979 in the group of VLBW babies born in Wilhelmina Gasthuis, the Amsterdam University Hospital. AL, Reynolds EOR, Lipscomb AP Outcome for infants of very low birthweight: survey of world literature Lancet 1981; i: 1038-40 Chalmers I, Mutch L. Are current trends in perinatal practice associated with an increase or a decrease in handicapping conditions? Lancet 1981; i: 1415. Steiner ES, Sanders EM, Philips ECK, Maddock CR. Very low birth weight children at school age; comparison of neonatal management methods Br Med J 1980; 281:
1. Stewart 2. 3.
1237-40. ER, Mandelkorn T, Woodrum DE, Wennberg RP, Parks CR, Hodson WA. Morbidity and mortality of infants weighing less than 1000 grams in an intensive care nursery Pediatrics 1972; 50: 40-49 5. Stewart AL, Turcan DM, Rawlings G, Reynolds EOR. Prognosis for infants weighing 1000 g or less at birth Arch Dis Child 1977; 52: 97-104. 6. Pape KE, Buncic RJ, Ashby S, Fitzhardinge PM. The status at two years of low-birthweight infants bom in 1974 with birth weights of less than 1001 gm. J Pediatr 1978; 92: 253-60.
4. Alden
4 Tell GP, Haour F, Saez J. Hormonal regulation of membrane receptors and cell responsiveness. Metabolism 1978; 27: 1566-92.
R. D. STURROCK
Glasgow G4
SIR,-We have now performed four selective right thoracic vagectomies for progressive bradycardia in childhood, with excellent results. In progressive vagal bradycardia the resting heartrate gradually falls resulting in lethargy, poor feeding, erratic breathing, and, if untreated, progresses to cardiac and then respiratory standstill. In the early phase the children often have epileptiform seizures but never experience postictal symptoms. A heartrate which drops to 80/s or below appears to be significant and epileptiform activity may be an attempt to increase the heartrate. These seizures, during which the heartrate may be over 100/s, are often followed by a drop in rate to 20 - 30/s when the child or even to zero in advanced cases. Since our first case report we have successfully operated on 3 more children with generalised hypervagism and progressive bradycardia. That is, before surgery, when not receiving atropine, at times their heartrates would drop to zero; after surgery they had heartrates between 80 and 130/s depending upon their degree of
relaxes,
activity. types of vagal bradycardia; local-resulting from such as pressure from a tumour or adhesions; and central-due to immaturity or abnormality of the. central vagal nuclei, as in our cases, which results in generalised hypervagism with gastrointestinal and, to a lesser degree, respiratory symptoms, as well as cardiac symptoms the most lethal component of which is progressive bradycardia. The disease may also provide a clue about the cause of and possible treatment for at least one form of sudden infant death syndrome (sits).22 When the bradycardia is vagal in origin there is an immediate response to atropine given intravenously or intramuscularly, 0’ I mg/kg. In the progressive form, tachyphylaxis soon raises the effective dose of atropine to high levels, and the viscous mucus in the respiratory and gastrointestinal tracts can cause problems. The response to atropine, which is the key to diagnosis, means that the bradycardia is due to vagal inhibition of impulses which arise in the sino-atrial node which is innervated by the right vagus nerve, and has minimal innervation from the left. The nerve fibres (about 3 branches) to the node come off the right recurrent laryngeal nerve, and 2 or 3 more branches come directly from the trunk of the right vagus, just distal to the origin of the recurrent laryngeal. By cutting only these fibres the recurrent laryngeal nerve is spared and there is no right vocal cord paralysis, no gastrointestinal tract effects (as would result from a truncal vagotomy), and few or no pulmonary effects, such as bronchospasm. Vagectomy is performed through a third intercostal space right lateral thoracotomy. The posterior pleura is incised from the azygos vein to the origin of the right subclavian artery; the vagal trunk is identified and followed up to the origin of the right recurrent laryngeal nerve. The first 3 fibres arising from the recurrent laryngeal are then divided, as are all branches of the vagal trunk distal to the recurrent laryngeal up to a point just above the azygos vein. If the heartrate is stable on atropine, surgery can be performed without a temporary venous pacemaker in place. If unstable, its use as an added safety factor is strongly advised. The procedure is well tolerated. Feeding can usually be started on the first or second day postoperatively. All our patients had been discharged by the fifth postoperative day. Vagectomy has proved simpler, more dependable, and better tolerated for a longer period than insertion of a pacemaker-especially in the small child. Our oldest patient, who had the operation in 1971 aged 3 years, is now a teenager. Our most recent patient is aged 4 years and has Down syndrome. The central type ofvagal bradycardia has been found most often in children with There
are two
phenomena
1
Coryllos EV, Kenigsberg K, Delaney T, Mofenson H. Thoracic vagectomy for vagal bradycardia. NY State J Med 1976; 76: 706. 2. SIDS-one possible etiology and its management. Presentation spring scientific sessions of New York Society for Thoracic Surgery, memorial Hospital, New York City, 1981.
system abnormalities, e.g. Down syndrome, cerebral microcephaly, palsy. Vagectomy is a simple, low-risk procedure for the treatment of progressive vagal bradycardia in children. Results have been good and lons-lastins. with low morbiditv. ELIZABETH V. CORYLLOS M. MOHTASHEMI T. DELANEY Nassau Hospital, Mineola, New York, U.S.A. J. GREENSHER central
nervous
or
SIMULTANEOUS CENTRAL VENOUS AND ARTERIAL BLOOD SAMPLING FOR CATECHOLAMINE ASSAY IN PHAEOCHROMOCYTOMA
SIR,-Central venous blood sampling for catecholamine assay is a diagnostic procedure for preoperative localisation of phaeochromocytomas. It has been recommended when radiographic studies fail to localise the tumour.1 Central venous blood sampling can be particularly useful in localising small, multiple, or metastatic tumours, as well as excluding an intrathoracic or neck localisation. Furthermore, it may avoid an unnecessary laparotomy.When a phaeochromocytoma is multiple, metastatic, or has abnormal venous drainage, multiple plasma catecholamine peaks may be detected. This also occurs if the tumour’s catecholamine secretion rate varies significantly during sequential central venous blood sampling, and might be a pitfall in accurate localisation of phaeochromocytomas. To avoid drawing false conclusions from the catheterisation date, we suggest that catecholamine levels be measured in central venous and arterial (brachial) blood samples, drawing the arterial sample 15 seconds after the corresponding central venous sample has been taken. Case 1 (see In this
figure).
patient the diagnosis of phaeochromocytoma was suggested by continued hypertension and abnormally high urinary excretion of 4-hydroxy 3-methoxy mandelic acid (HMMA)2 (15-25 3 mg/g creatinine) and free catecholamines3 (noradrenaline: 1256—2427 g/gcreatinine, adrenaline: 757-1478 pglg creatinine). Central venous blood sampling (no drug treatment) showed two 1.
Manger WM, Gifford RW, Jr. Pheochromocytoma. Central venous blood sampling for catecholamine assay. New York: Springer-Verlag, 1978: 266-75 2. Pisano JJ, Crout JR, Abraham D. Determination of 3-methoxy-4-hydroxymandelic acid in urine. Clin Chim Acta 1962; 7: 285-91. V, Brunori CA, Valori C A sensitive and specific fluorimetric method for the determination of noradrenaline and adrenaline in human plasma. Clin Chim Acta 1970; 30: 587-94.
3. Renzini
Plasma catecholamine concentration (NA= = noradrenaline; A= adrenaline) during central venous blood sampling (CVBS) from right atrium (RA) to lumbar 5 (L,), and simultaneous arterial blood
sampling (ABS).
527 Since corresponding arterial catecholamine levels were excluded variation in the tumoral secretion rate and stable, multiple phaeochromocytomas. In fact the lumbar 3 peak suspected (figure) was due to an additional vein discharging into the vena cava, as later confirmed both by retrograde phlebography and surgical resection.
peaks.
we
measured IgA levels in 20 patients with rotator cuff lesions. In our group of frozen-shoulder patients, we did not detect B27 in a single patient. Additionally the IgA levels were similar in frozen shoulder, rotator cuff, and control groups. Department of Rheumatology, Westminster Hospital,
M. A. STODELL
London SW1
Case2 The second
patient was investigated because of recurrence of abnormal excretion of HMMA (32-55 mg/g and hypertension creatinine) and noradrenaline (4265-7564 jL
tumour
Institutes of Medical Pathology and Clinical Medicine, University of Perugia, 06100 Perugia, Italy
GEREMIABOLLI PIERPAOLO DE FEO PIETRO COMPAGNUCCI MARIA G. CARTECHINI FAUSTO SAMTEUSANIO PAOLO BRUNETTI
FROZEN SHOULDER
SiR,—The aetiology of frozen shoulder or capsulitis remains unknown. Bulgen and co-workers, of Cambridge, have implicated a component and postulate a localised autoimmune reaction. These workers have reported a 36% incidence of HLA B27 in frozen shoulder associated with a low serum IgA level which persists after recovery. To our knowledge, these findings remain unconfirmed. In a review of the frozen shoulder, Kessel et al.could not find a genetic linkage or any disturbance of IgA levels in a series of 50 patients. We have studied 21 patients with frozen shoulder using the same criteria for diagnosis as Bulgen et al. We have also
hereditary
Centre for Rheumatic Diseases, Baird Street,
PREVENTION OF INTRAVENTRICULAR HAEMORRHAGE BY PHENOBARBITONE Ji,——
1 Bulgen DY, Hazleman BL,
Voak D HLA-B27 and frozen shoulder. Lancet 1976; i: 1042-44 Bulgen D, Hazleman B, Ward M, McCallum M. Immunological studies in frozen shoulder Ann Rheum Dis 1978; 37: 135-38. 3 Kessel L, Bayley I, Young A. The frozen shoulder. BrJ Hosp Med 1981, 25: 334-38.
1 11C
C2LL-iLL11; ICpUi
L Ui 1-l 1-,IUIIII amu U-WUljCIo
V1.Ub. 1, p.
215) states that ultrasound scans of the brain were done on the 3rd, 4th or 5th postnatal day. If the results of the study are to be accepted fully, we think that clarification of the time of scanning is called for. Since the end of 1978 we have prospectively scanned the brains of all small preterm infants admitted to our neonatal unit. The scanners used were the ADR 2130 linear-array system with 5 MHz and 7 MHz probes, and more recently the Siemens Diasonics RA 1 sector scanner with a 7 5 MHz probe. The infants’ brains were repeatedly examined through the skull and through the anterior fontanelle. In our experience about a third of all haemorrhages first appeared during or after the third day. These late haemorrhages were usually small (grade I). Donn and co-workers’ study shows a notable lack of grade I haemorrhages in the treatment group. If the age at scanning was, in general, later in the control group than in the treatment group, the conclusion that phenobarbitone was effective might be open to question. P. L. HOPE Department of Paediatrics, School of Medicine,
University College London, London WC1E
6JJ
R. J. THORBURN A. L. STEWART E. O. R. REYNOLDS
INTENSIVE CARE AND THE VERY LOW BIRTH WEIGHT INFANT
SIR,-Stewart et al. recently published a paperl showing the decrease in mortality for very low birth weight (VLBW) infants during the years 1946 to 1977, with a stable and reasonably low handicap-rate of 6 to 8% since 1960. Chalmers and Mutchcommenting on this paper, suggested that with a stable handicap rate and decreasing mortality the absolute number of handicapped children would rise. However, discussion is hard to interpret since it is difficult to obtain exact figures for welldefined populations of VLBW infants. Although Stewart et al. claim that they reviewed only results from studies where all live births in the population were accounted for, nevertheless, they reported results obtained in patients referred after birth to neonatal intensive care units.3-6 We have found no increase in the handicap-rate nor in the absolute number of handicapped children during the 21-years from 1959 to 1979 in the group of VLBW babies born in Wilhelmina Gasthuis, the Amsterdam University Hospital. AL, Reynolds EOR, Lipscomb AP Outcome for infants of very low birthweight: survey of world literature Lancet 1981; i: 1038-40 Chalmers I, Mutch L. Are current trends in perinatal practice associated with an increase or a decrease in handicapping conditions? Lancet 1981; i: 1415. Steiner ES, Sanders EM, Philips ECK, Maddock CR. Very low birth weight children at school age; comparison of neonatal management methods Br Med J 1980; 281:
1. Stewart 2. 3.
1237-40. ER, Mandelkorn T, Woodrum DE, Wennberg RP, Parks CR, Hodson WA. Morbidity and mortality of infants weighing less than 1000 grams in an intensive care nursery Pediatrics 1972; 50: 40-49 5. Stewart AL, Turcan DM, Rawlings G, Reynolds EOR. Prognosis for infants weighing 1000 g or less at birth Arch Dis Child 1977; 52: 97-104. 6. Pape KE, Buncic RJ, Ashby S, Fitzhardinge PM. The status at two years of low-birthweight infants bom in 1974 with birth weights of less than 1001 gm. J Pediatr 1978; 92: 253-60.
4. Alden
4 Tell GP, Haour F, Saez J. Hormonal regulation of membrane receptors and cell responsiveness. Metabolism 1978; 27: 1566-92.
R. D. STURROCK
Glasgow G4