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98
Thorax 1997;52:98–99
index was 19.1 kg/m
2
. Her clotting screen was
Thromboembolism
normal. A routine two dimensional echo-
cardiogram revealed a 3 cm clot at the catheter
related to a Port-a-Cath
tip of the Port-a-Cath in the right atrium. The
device was functioning well and the patient was
device in a patient with
asymptomatic. A bolus dose of 50 mg re-
combinant tissue plasminogen activator (rt-PA)
cystic fibrosis
was given intravenously over an hour on two
occasions during the next 16 hours in an at-
tempt to lyse the clot and she was then com-
menced on intravenous heparin infusion. She
B Yung, J S Elborn, I A Campbell,
remained well until three hours after the last
Y Summers, M Beckles,AAWoodcock
dose of rt-PA when she became acutely unwell
and complained of pleuritic chest pain with
severe dyspnoea of sudden onset.
On examination she was unwell with tachy-
Abstract
pnoea and tachycardia. Her blood pressure
The case is described of a potentially life
was 120/90 mm Hg and oxygen saturation was
threatening complication relating to the
92% on oxygen at 8 l/min via nasal cannulae
use of a totally implantable venous access
(her normal oxygen saturation was 92% on
device (Port-a-Cath) in a 28 year old
air). Her electrocardiogram and chest radio-
patient with cystic fibrosis. The device was
graph were unchanged. A diagnosis of acute
inserted in 1990 and used repeatedly for
pulmonary embolism was made. Her clotting
antibiotic therapy without any com-
status was checked and a further heparin bolus
plications. In 1995, during assessment
dose was given because of an inadequate
for double lung transplantation, a 3 cm
KCCT ratio of 1.05. A computed tomographic
thrombus was found at the tip of the cath-
(CT) contrast enhanced pulmonary angiogram
eter in the right atrium. Embolisation of
confirmed the presence of clots in the right
the thrombus to the pulmonary arteries
main pulmonary artery (fig 1) and the artery to
occurred after the infusion of recombinant
the right lower lobe. A repeat echocardiogram
tissue plasminogen activator (rt-PA).
showed no evidence of the clot at the tip of the
Thrombus formation may be associated
catheter. The patient’s condition was stable
with totally implantable venous access de-
and she continued to receive heparin and sub-
vices and thromboembolism may occur
sequently warfarin.
following the use of thrombolytic agents
Two days later she became more dyspnoeic
in the treatment of such thrombosis.
with production of purulent sputum. Intra-
(Thorax 1997;52:98–99)
venous antibiotics were started and she was
also given a 50 mg dose of rt-PA via her Port-
Keywords: cystic fibrosis, totally implantable venous
a-Cath. A repeat CT pulmonary angiogram
access devices, thromboembolism.
performed 24 hours later showed persistence
of the clots in the pulmonary arteries. She was
commenced on streptokinase infusion (250 000
Totally implantable venous access devices are
units over 30 minutes) but developed an ana-
used in oncological patients and in patients
phylactic reaction three minutes into the in-
with cystic fibrosis.
12
These devices provide
fusion and the infusion was stopped. Following
safe effective means of intermittent venous
repeat clotting studies two further doses of rt-
access and are well accepted by patients.
34
We
PA were given over the next six hours but a
report a potentially life threatening com-
repeat CT pulmonary angiogram 24 hours later
plication related to the use of a Port-a-Cath
was unchanged. She was then treated with four
device in a patient with cystic fibrosis.
Section of Respiratory
doses of intravenous urokinase (4400 units/kg
Medicine, University
on each occasion, given over 30 minutes, with
of Wales College of
hydrocortisone cover) over the next 18 hours.
Medicine, Llandough
Hospital NHS Trust,
Case report Her clinical condition improved and a repeat
Penarth, South
A 28 year old patient with cystic fibrosis (delta CT pulmonary angiogram performed 24 hours
Glamorgan CF64 2XX,
F508 heterozygous), who had deteriorated after later revealed resolution of clot in the right
UK
B Yung
the birth of her son in 1990, was being assessed main pulmonary artery and in the artery to the
J S Elborn
for double lung transplantation. She frequently right lower lobe. She was discharged three days
I A Campbell
required intravenous antibiotics for chronic later on warfarin. A repeat echocardiogram six
weeks later revealed no evidence of a recurrencePseudomonas aeruginosa infection of her lungs
North West Lung
Centre, Wythenshawe
and venous access became increasingly diffi- of the clot on the Port-a-Cath which still re-
Hospital, Manchester
cult. In June 1990 a Port-a-Cath was implanted mains fully functional. Following this case, we
M23 9LT, UK
via the right cephalic vein and she subsequently electively performed two dimensional echo-
Y Summers
M Beckles
had many antibiotic courses through this device cardiograms on all 11 of our patients with cystic
A A Woodcock
without complication. There was no previous fibrosis with Port-a-Cath devices and no further
Correspondence to:
history or family history of thromboembolism case of thrombosis was identified.
Dr B Yung, Department of
and she was not taking the contraceptive pill.
Cystic Fibrosis, Royal
Brompton Hospital, London
In January 1995 she was referred for as-
SW3 6NP, UK.
sessment for lung transplantation. Her forced Discussion
Received 20 June 1995
expiratory volume in one second (FEV
1
) was Port-a-Cath devices have been used in on-
Accepted for publication
31 July 1995
0.76 l (26% predicted) and her body mass cological patients since 1982
1
and are as-
group.bmj.com on July 13, 2011 - Published by thorax.bmj.comDownloaded from
Transverse myelitis in cystic fibrosis 99
Thrombolytic agents are effective in the
Figure 1 Computed
tomographic pulmonary
treatment of pulmonary embolism, accelerating
angiogram showing a
clot lysis, hastening pulmonary tissue per-
marked filling defect in the
fusion, reversing right heart failure, and im-
right main pulmonary
artery (arrowed).
proving pulmonary capillary blood volume.
8
They are particularly useful in the treatment of
pulmonary embolism when the haemodynamic
status is compromised. A serial contrast en-
hanced helical CT pulmonary angiogram can
be used for diagnosis and monitoring the sub-
sequent response to thrombolytic therapy.
9
We
suggest that, in patients with totally implantable
venous access devices, thromboembolism
should be considered as a differential diagnosis
should a patient develop sudden chest pain and
dyspnoea, especially if this occurs soon after
the infusion of thrombolytic agents or heparin.
BY was supported by the Cystic Fibrosis Trust. We would like
to thank staff at the ECG department, Llandough Hospital for
sociated with a lower incidence of infection
and thrombosis than external venous access
devices, as well as being more cosmetically
performing the echocardiograms.
acceptable.
5
Pattison reported on the use of the
Port-a-Cath in patients with cystic fibrosis in
1 Niederhuber JE, Ensminger W, Gyves JW, Liepman M, Doan
K, Cozzi E. Totally implanted venous and arterial access
1986.
2
Since then, studies have shown that
system to replace external catheters in cancer treatment.
Surgery 1982;92:706–11.
Port-a-Cath devices are well accepted by this
2 Pattison J, Heaf DP. Totally implantable vascular access in
group of patients and are safe and reliable
treatment of cystic fibrosis. Lancet 1986;i:799.
3 Cassey J, Ford WDA, O’Brien L, Martin AJ. Totally im-
for effective intermittent intravenous antibiotic
plantable system for venous access in children with cystic
therapy.
34
Potential complications include
fibrosis. Clin Pediatr 1988;27:91–5.
4 Morris JB, Occhionero ME, Gauderer MWL, Stern RC,
catheter occlusion, subcutaneous infiltration,
Doershuk CF. Totally implantable vascular access devices
infection, migration of the catheter, pain from
in cystic fibrosis: A four year experience with fifty eight
patients. J Pediatr 1990;117(1 Pt 1):82–5.
the portal and, rarely, venous thrombosis with
5 Brothers TE, Von Moll LK, Niederhuber JE, Roberts JA,
or without superior vena cava obstruction.
346
Walker-Andrews S, Ensminger WD. Experience with sub-
cutaneous infusion ports in three hundred patients. Surgery
We were able to identify only one previous case
1988;166:295–301.
of deep venous thrombosis of the iliac veins
6 Peckham DG, Hill J, Manhire AR, Knox AJ. Resolution
of superior vena cava obstruction following thrombolytic
followed by pulmonary embolism related to the
therapy in a patient with cystic fibrosis and a long-term
use of a Davol Femoral Infusaport.
7
Our patient
indwelling catheter. Respir Med 1994;88:627–9.
7 Sola JE, Stone MM, Wise B, Colombani PM. Atypical throm-
is the first to be reported with thrombo-
botic and septic complications of totally implantable venous
embolism related to the use of Port-a-Cath
access devices in patients with cystic fibrosis. Pediatr Pul-
monol 1992;14:239–42.
devices implanted in the upper limbs, a serious
8 Goldhaber SZ. Contemporary pulmonary embolism throm-
and potentially life threatening complication.
bolysis. Chest 1995;107(Suppl 1):45–51S.
9 Curtin JJ, Mewissen MW, Crain MR, Lipchik RJ. Post con-
We presume that embolism of the thrombus
trast CT in the diagnosis and assessment of response to
from the Port-a-Cath occurred following the
thrombolysis in massive pulmonary embolism. J Comput
Assist Tomogr 1994;18:133–5.
use of rt-PA.
Thorax 1997;52:99–101
verse myelitis developed following bron-
chial artery embolisation but recovery was
Transverse myelitis: a
rapid and nearly complete. Haemoptysis
did not recur during four years of follow
reversible complication
up.
(Thorax 1997;52:99–101)
of bronchial artery
Division of
Keywords: cystic fibrosis, bronchial artery, em-
embolisation in cystic
Respiratory Medicine
bolisation, transverse myelitis.
K L Fraser
R H Hyland
fibrosis
D E Tullis
A 28 year old woman who was diagnosed with
Department of
cystic fibrosis in infancy had moderate lung
Radiology
disease (forced vital capacity 75% predicted,
H Grosman
K L Fraser, H Grosman, R H Hyland,
forced expiratory volume in one second 61%
D E Tullis
The Wellesley Hospital,
predicted) and was colonised with Pseudomonas
University of Toronto,
aeruginosa and Burkholderia cepacia. She was
Toronto, Ontario M4Y
1JS, Canada
generally well with few hospital admissions for
pulmonary exacerbations. In July 1991 she de-
Correspondence to:
Dr D E Tullis.
Abstract veloped haemoptysis associated with a pul-
monary exacerbation, resulting in a significantThe case history is presented of a young
Received 14 December 1995
Returned to authors
woman with cystic fibrosis and life threat- fall in her haemoglobin level. She improved
29 March 1996
ening haemoptysis. Angiography revealed with conservative treatment.
Revised version received
12 August 1996
enlarged bronchial vessels, one of which Haemoptysis recurred in October 1991 and
Accepted for publication
supplied the contralateral lung. Trans- her haemoglobin fell from 13.4 g/dl to 1 1.4 g/dl.
3 October 1996
group.bmj.com on July 13, 2011 - Published by thorax.bmj.comDownloaded from
100 Fraser, Grosman, Hyland, Tullis
The right fifth intercostal artery, when in-
Figure 1 Injection of right
fifth intercostal (solid
jected with contrast, filled more proximally
black arrow) with remote
positioned intercostal vessels through com-
filling of abnormally
municating branches parallel to the spine until
hypertrophied non-
bronchial systemic artery
eventually filling an abnormally enlarged non-
(open arrow) supplying the
bronchial systemic artery supplying the right
right upper lobe.
upper lobe. This large abnormal vessel could
only be embolised remotely via the right fifth
intercostal artery. The catheter became wedged
into this vessel during the lengthy embolisations
and there was angiographic “hold up” or stag-
nation of contrast medium in the proximal
intercostal vessels on “control” injections into
the right fifth intercostal artery (figs 1–3).
The left thyrocervical trunk gave rise to an
enlarged bronchial vessel which crossed the
midline and supplied the right lower lobe (a
rare congenital anomaly). Since this was a very
large vessel, it was embolised with a com-
bination of Ivalon and absorbable gelatin
Figure 2 Enlargement of
abnormally hypertrophied
vessel shown in fig 1.
sponge particles (Gelfoam; Upjohn, Ontario,
Canada). A fourth enlarged bronchial artery
arising from the right thyrocervical trunk and
supplying the right mid lung was cannulated
and embolised with the same combination.
A 5 French end hole embolisation Cobra
catheter (Cook Inc, Bloomington, Indiana,
USA) was used for the first two vessels em-
bolised and a 5 French HIH (head hunter)
catheter (Cook Inc) for the enlarged bronchial
arteries arising from each of the thyrocervical
trunks. The procedure lasted approximately
four hours and a total of 300 ml of non-ionic
contrast medium was injected. Digital sub-
traction angiography was used entirely during
the procedure in an attempt to keep the volume
Figure 3 Abnormal non-
bronchial systemic artery
of contrast medium used to a minimum and
successfully embolised
to assess better the vascular supply to the cord.
(black arrow). Stasis of
No obvious vascular supply to the cord, how-
contrast medium in residual
right fifth intercostal artery
ever, was visualised during the entire study.
(black arrowheads) with
One limitation might have been the lack of
communicating branches
a high resolution digital system although the
with other intercostal
vessels and no visualisation
patient was anaesthetised, thus eliminating res-
of vascular supply to the
piratory motion artefact during the digital sub-
spinal cord.
traction imaging. The contrast medium used
was non-ionic (Omnipaque 300; Sanofi-
Winthrop, Ontario, Canada) which is con-
sidered safer than ionic based media. Injections
into each vessel of 2–4 ml contrast medium per
injection were carried out manually by the
angiographer.
All four abnormal vessels were successfully
embolised. The haemoptysis stopped, the
patient was extubated and reported no neuro-
logical symptoms for the remainder of the day.
The following morning the patient complained
of paralysis of the left leg and was found to
have right sided sensory disturbance to the level
She required a total of four units of blood.
Conservative treatment was not successful and
three days after admission she was scheduled
for bronchial arteriography and embolisation.
On the morning of the scheduled procedure
she had massive haemoptysis with respiratory
of T4. Posterior column function was intact,
compromise necessitating intubation. Bron-
compatible with a diagnosis of transverse my-
chial artery angiography was performed. The
elitis.
first artery recognised as a bronchial artery
Treatment was initiated immediately with
arose from the left side of the aorta at around
intravenous methylprednisolone 125 mg daily
T7. As embolisation was performed using
for three days and there was rapid neurological
polyvinyl alcohol microspheres 300–600 lm
improvement. At the time of discharge 25 days
(Ivalon; Locam Medical Distributors, Laborat-
later some sensory deficit and minimal foot
ories Nycamed, Paris, France) more and more
drop persisted. With rehabilitation therapy no
enlarged collateral vessels supplying the left
discernible motor weakness remained, al-
lung became apparent and were further em-
bolised. though neurological examination revealed re-
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Transverse myelitis in cystic fibrosis 101
duced sensation to pin prick and light touch Our patient is similar in that contrast material
was injected into the fifth right intercostal artery
on the right to the level of the umbilicus.
and in the subsequent neurological course.
The patient remained well with no recurrent
Given the delayed onset of the neurological
massive haemoptysis until August 1995 when
event and the reversible nature of the lesion,
she succumbed to an infective exacerbation of
and given the lack of visualisation of the anterior
her pulmonary disease.
spinal artery complex and the amount of em-
bolic material used, we postulate that this,
too, is a case of a contrast-induced spinal cord
lesion. This may have been promoted by the
Discussion
wedging of the catheter into the right fifth
The bronchial artery circulation is quite vari-
intercostal origin and the persistence of contrast
able and complex. Arterial origins arise mainly
medium in this vessel and its branches during
from the thoracic aorta or its branches. Up to
this lengthy, although life saving, procedure.
20% arise from various other vessels and the
The anterior spinal cord is supplied by the
remaining 10% originate from the anterior sur-
anterior spinal artery which originates from
face of the aortic arch. In conditions of chronic
branches of the vertebral arteries and from
inflammation such as cystic fibrosis and tuber-
anterior medullary branches of intercostal and
culous bronchiectasis, the bronchial circulation
lumbar arteries.
11
All spinal cord arteries are
expands greatly.
1
The same vessels that supply
functional end arteries. In the thoracic area the
the bronchial arteries may also supply the oeso-
supply to the anterior spinal artery is usually
phagus, mediastinal lymph nodes, and the
from a single anterior medullary branch. It has
spinal arteries through a complex anastomotic
been suggested that, in patients who suffer
network. This expanded systemic circulation is
neurological sequelae following injection of
contrast medium, the fifth right intercostal ar-the primary source of bleeding in haemoptysis.
2
tery sometimes gives rise to the anterior vas-
Many patients with cystic fibrosis have under-
cular supply of the thoracic cord at T4 to T6.
10
gone bronchial artery embolisation for treat-
One cannot predict ahead of time in which
ment of haemoptysis.
3–6
We are aware of one
patient this occurs.
previous case in which an hypertrophied bron-
This case illustrates that transverse myelitis
chial vessel has supplied the contralateral lung.
7
is a risk of bronchial artery embolisation, even
In that report, the patient had undergone mul-
when current guidelines and proper angio-
tiple previous embolisations and the authors
graphic procedures are followed. However, the
hypothesised that the previous interventions
prognosis for a nearly total recovery is good.
may have contributed to the enlargement of
the vessel. Our patient had no previous history
1Deffebach ME. Clinical relevance of the bronchial cir-
of embolisation, suggesting that this un-
culation. Pulm Perspect 1991;8:8–10.
2 Porter DK, VanEvery MJ, Anthracile RF, Mack JW. Massive
common circulation may arise simply as a result
hemoptysis in cystic fibrosis. Arch Intern Med 1983;143:
287–90.
of chronic inflammation.
3 Fellows KE, Khaw KT, Schuster S, Shwachman H. Bron-
Transverse myelitis is a well recognised com-
chial artery embolization in cystic fibrosis: technique and
long–term results. J Pediatr 1979;95:959–63.
plication of arteriography
89
and is a theoretical
4 Sweezey NB, Fellows KE. Bronchial artery embolization
risk of bronchial artery embolisation because
for severe hemoptysis in cystic fibrosis. Chest 1990;97:
1322–6.
of the anatomical variation of a shared origin
5 Cohen AM, Doershuk CF, Stern RC. Bronchial artery
of bronchial arteries with intercostal vessels
embolization to control hemoptysis in cystic fibrosis.
Radiology 1990;175:401–5.
which supply radiculomedullary branches to
6 Tonkin ILD, Hanissian AS, Boulden TF, et al. Bronchial
the anterior spinal circulation. However, there
arteriography and embolotherapy for hemoptysis in
patients with cystic fibrosis. Cardiovasc Intervent Radiol
are no reports in the literature of transverse
1991;14:241–6.
7 Cohen AM, Antoun BW, Stern RC. Left thyrocervical trunk
myelitis following bronchial artery em-
bronchial artery supplying right lung: source of recurrent
bolisation. Kardjiev et al reported five cases in
hemoptysis in cystic fibrosis. AJR 1992;158:1131–3.
8 Feigelson HH, Ravin HA. Transverse myelitis following
which similar neurological events followed the
selective bronchial arteriography. Radiology 1965;85:663–
injection of contrast medium into the right
5.
9 DiChiro G. Unintentional spinal cord arteriography: a warn-
fifth intercostal artery. The procedures were
ing. Radiology 1974;112:231–3.
carried out for diagnostic purposes only and a
10 Kardjiev V, Symeonov A, Chankov I. Etiology, pathogenesis
and prevention of spinal cord lesion in selective angio-
different contrast medium (ionic) from ours
graphy of the bronchial and intercostal arteries. Radio-
was used. All patients made a full recovery
logy 1974;112:81–3.
11 Stoll JF, Bettmann MA. Bronchial artery embolization to
although it took almost three months for one
control hemoptysis: a review. Cardiovasc Intervent Radiol
1988;11:263–9.
patient’s symptoms to resolve.
10
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doi: 10.1136/thx.52.1.98
1997 52: 98-99Thorax
B Yung, J S Elborn, I A Campbell, et al.
device in a patient with cystic fibrosis.
Thromboembolism related to a Port-a-Cath
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