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Thromboembolism related to Port-a-Cath device in a patient with cystic fibrosis


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The case is described of a potentially life threatening complication relating to the use of a totally implantable venous access device (Port-a-Cath) in a 28 year old patient with cystic fibrosis. The device was inserted in 1990 and used repeatedly for antibiotic therapy without any complications. In 1995, during assessment for double lung transplantation, a 3 cm thrombus was found at the tip of the catheter in the right atrium. Embolisation of the thrombus to the pulmonary arteries occurred after the infusion of recombinant tissue plasminogen activator (rt-PA). Thrombus formation may be associated with totally implantable venous access devices and thromboembolism may occur following the use of thrombolytic agents in the treatment of such thrombosis.
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Thorax 1997;52:98–99
index was 19.1 kg/m
. Her clotting screen was
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-
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.
These devices provide
fusion and the infusion was stopped. Following
safe eective means of intermittent venous
repeat clotting studies two further doses of rt-
access and are well accepted by patients.
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
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 di- 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
) 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
and are as- on July 13, 2011 - Published by thorax.bmj.comDownloaded from
Transverse myelitis in cystic fibrosis 99
Thrombolytic agents are eective 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.
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.
suggest that, in patients with totally implantable
venous access devices, thromboembolism
should be considered as a dierential 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 sta 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.
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
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 eective intermittent intravenous antibiotic
plantable system for venous access in children with cystic
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.
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
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.
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
(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
D E Tullis
A 28 year old woman who was diagnosed with
Department of
cystic fibrosis in infancy had moderate lung
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 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
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
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- on July 13, 2011 - Published by thorax.bmj.comDownloaded from
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
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.
All spinal cord arteries are
expands greatly.
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 suer
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.
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.
gone bronchial artery embolisation for treat-
One cannot predict ahead of time in which
ment of haemoptysis.
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.
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
1Deebach 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:
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
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:
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
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
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-
dierent 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
patient’s symptoms to resolve.
10 on July 13, 2011 - Published by thorax.bmj.comDownloaded from
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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... Progressive hypoxaemia occurs as a result of pulmonary sepsis and associated obstruction of the airways [1]. Despite being a chronic inflammatory condition with a purported increased incidence of thrombophilia [2,3], thromboembolic disease is rarely reported except in association with totally implantable venous access devices (TIVADs) [4][5][6][7][8] which are often inserted when frequent courses of intravenous antibiotics are required. ...
... Initially, the hypoxaemia was attributed to an infective exacerbation, but other diagnoses were later considered as oxygen saturation did not improve with her FEV 1 . These included interstitial lung disease (in view of the RA) and pulmonary embolism (a rare diagnosis in CF with few reported cases -all in the presence of a TIVAD [4,9]). A PFO with right-to-left shunting was later confirmed, demonstrating that this is possible without significant pulmonary hypertension. ...
Full-text available
Cystic fibrosis is usually associated with chronic pulmonary sepsis and frequent infective exacerbations. We report a very unusual cause of severe hypoxaemia in a woman with cystic fibrosis caused by thrombus formation in the right atrium. A 21-year-old Caucasian woman with cystic fibrosis and a totally implantable venous access device presented with severe hypoxaemia. This was initially treated with antibiotics but her oxygen levels did not improve significantly. Subsequently, a transient ischaemic attack occurred. Further investigations, including a contrast echocardiogram and a cardiac magnetic resonance scan, revealed the presence of a large right atrial thrombus and right-to-left intracardiac shunt through a patent foramen ovale. This case highlights the need to consider a right-to-left shunt in chronic respiratory diseases when hypoxaemia is out of proportion to the degree of lung function impairment. Totally implantable venous access devices should always be considered as a source of thrombus formation.
... Taniguchi reported that the aim of chemotherapy used TIVAPs were less than nutritious it. However, there is no evidence demonstrating any decrease in the infection rates [19]. Actually, there were a few catheter related infections in our institute, whose reasons are the use of catheter for cancer chemotherapy and we followed general maintenance and access procedures follow as; 1) wash hands, 2) wear clean gloves, 3) perform sterilization by povidone iodine, 4) puncture by needle and make sure metal sound, 5) 3-5 mL of blood and discard blood draws, 6) fix the needle under sterilization, 7) flush with 10-20 mL of normal saline by pulsing flush. ...
Full-text available
Gluteal intramuscular injection requires accurate needle insertion into the specific muscle area for accurate, safe, and efficacious delivery of medication for long-acting anti-psychiatric drugs. With ultrasonography, accurate evaluation of the "Distance from the Epidermis to the Under-Fascia (DEUF)" can be determined. However, a nurse in the clinical settings, ultrasonographic measurements during intramuscular injection is impractical and difficult. The purpose of this study was to estimate the thickness of subcutaneous fat by using a near-infrared ray measuring device.This study was approved by the Ethics Committee of Tokushima University Hospital. There were 39 subjects (21 men and 18 women) aged 20 to 60 years (Mean ± SD: 44.97 ± 15.20 years old). Bilateral DEUF at Ventrogluteal and Dorsogluteal sites (AVIUS: Hitachi-Aloka Medical Co.) were evaluated on B-mode scan as the gold standard. At the same sites, the thickness of the subcutaneous fatty tissue was measured using the PoccoStick (HORIC: smallsize instrument measuring the thickness of the subcutaneous fat with a near-infrared ray device). The right Ventrogluteal site thickness of the fatty tissue was significantly overestimated by PoccoStick (15.18 ± 8.77 mm) measurements, as compared to the ultrasonography (12.42 ± 5.26 mm) (p<0.01) results. The left-side Ventrogluteal site and both the right and left Dorsogluteal sites measurements did not show significant differences. This illustrated and supported the credible use of inexpensive devices such as the PoccoStick in estimating the DEUF. The development of a more highly accurate small-sized instrument is recommended.
... Documented pulmonary embolism is very rare, but has been described with upper-extremity indwelling catheters. 16 This study was not designed to examine whether prophylactic warfarin (1 mg/d) is effective in prophylaxis against thrombosis, as is seen in other patient population groups. Bern et al 17 randomized 82 oncology patients with indwelling catheters to warfarin, 1 mg/d, or placebo. ...
Study objective Patients with cystic fibrosis (CF)frequently require recurrent courses of IV antibiotics to treat acuteexacerbations of their pulmonary disease. Over time, CF patients oftenlose peripheral access, and indwelling central venous catheters areplaced. We attempted to determine the type and incidence of cathetercomplications so that CF patients could be fully informed of the risksprior to placement of these catheters. Design Thecharts of all CF patients who attended the Adult Cystic Fibrosis Clinicof the University of Washington Medical Center from January 1989through December 1998 were reviewed. Demographic information wasobtained along with the type and duration of catheter, type and numberof complications, and the use of anticoagulant medication. Measurements and results Of the 218 CF patients whoattended the clinic, 65 patients (30%) had indwelling catheters inplace at some time during the study period. A total of 87 catheterswere placed into these 65 patients. The total number ofcatheter-days for first indwelling catheters was 68,220. The totalnumber of catheter-days for all catheters was 75,660 (210catheter-years). Thirty-five catheter-related complications wereidentified, occurring in 26 patients. Complications included thrombosis(n = 14), infections (n = 9), mechanical problems (n = 6),pneumothorax (n = 3), superior vena cava syndrome/stenosis (n = 2),and air embolism (n = 1), for an overall complication rate of0.463/1,000 catheter-days. Conclusion We concludethat indwelling catheters are relatively safe in patients with CF. Goodinfection control policies appear to prevent most infectiouscomplications. The most common complication is that of thrombosis, which may be recurrent in some patients. Consideration should be givento prophylactic warfarin therapy despite the potential risk ofsignificant hemoptysis in this patient population.
... Thrombus formation is the most unwanted complication developed after Port-A catheter implantation. Moreover, severe thromboembolism may occur following the use of thrombolytic agents for treating such thrombosis, which can be fatal[3].We report a case with suspected upper venous occlusionafter Port-A catheter implantation. Nuclear medicine flow studies for the evaluation of upper extremity deep venous thrombosis provide a good demonstration of obstructed venous return in this patient. ...
... In addition, many CF patients (23% according to UK CF database 2002) have a totally implantable venous access device (TIVAD) such as a Port-a-Cath 1 , and a recognized complication (reported in 3-40%) is line occlusion and thrombosis, with the clot often attached to the tip of the catheter. [1][2][3][4][5][6][7][8][9] These complications are more likely if the child has an underlying thrombotic tendency, and this tendency may be enhanced in the presence of lung infection and inflammation. 10,11 The aim of this study was to screen our pediatric CF clinic to determine the underlying incidence of thrombophilia and see if there was any correlation with clinical status. ...
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In some children with cystic fibrosis (CF), percutaneous long lines occlude sooner than expected (due to thrombophlebitis or thrombosis), and many have a totally implantable venous access device (TIVAD), a recognized complication of which is thrombosis. This complication is more likely if the child has an underlying thrombotic tendency, which may be enhanced in the presence of inflammatory lung disease. There are no reports of an identified association of heritable thrombophilia with CF, although individual cases have been recognized. Our aim was to determine the incidence of thrombophilia in children with CF. In a tertiary pediatric CF center, blood was screened for thrombophilia at annual review, and retested if abnormal. A thrombotic abnormality was found in 41/204 (20%) patients. These included activated protein C resistance (10/204, 5%) with a prevalence similar to that expected, but the following abnormalities had an increased prevalence: antithrombin deficiency (2/204, 1%), protein S deficiency (11/204, 5%), protein C deficiency (8/204, 4%), and lupus anticoagulant (18/204, 9%). There were no differences found in those with thrombophilia for the following parameters: age, gender, genotype, lung function, presence of Pseudomonas aeruginosa, prothrombin time, serum IgE, aspergillus-specific IgE, liver function, and blood inflammatory markers. Fifteen children had TIVADs, 4 of whom had evidence of thrombophilia. In conclusion, a significant proportion of patients had a thrombophilic abnormality. We recommend that thrombophilia screening be performed prior to insertion of a TIVAD, and also in those with a history of venous thrombosis, blocked TIVADs, or recurring problems with long lines. Pediatr Pulmonol. 2005; 39:306–310. © 2005 Wiley-Liss, Inc.
... Placing the port on the upper arm after subclavian puncture may reduce the potential risk to that of a port placed on the chest. In fact thrombotic complications appear to be particularly associated with a larger TIVAS [17][18][19]. None was noted with PAS Port use in this series or in the series by YUNG et al. [7], but a 4-14% rate was reported for the Infuse-A-Port (Infusaid Corporation, Sharon, MA, USA) and Port-A-Cath in cystic fibrosis [6,7,9], and a 4-16% rate in cancer patients [12,14]. ...
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An implantable venous access system provides a reliable and painless entry site for intravenous treatment. This study reports the authors' experience with such a system in adult patients with cystic fibrosis. Sixty five (87%) of 75 PAS Ports were placed successfully in 57 patients with cystic fibrosis. Because of early difficulties in advancing the catheter in patients whose veins had been traumatized by repeated courses of intravenous antibiotics, a technique was developed whereby venous entry was gained by direct subclavian puncture. This catheter insertion method was used in 53 (82%) attempts and the catheter was then passed by subcutaneous tunnelling to the port site on the ventral aspect of the upper arm. Fifty seven (88%) insertions were successful under local anaesthetic. The major early and late complications were iatrogenic pneumothorax (six cases) and infection (five cases), respectively. Late complications were more common when there was coexisting disease, e.g. diabetes mellitus, or an acute severe respiratory exacerbation, or when the Port was used for parenteral feeding. In conclusion, the PAS Port can be inserted safely by direct subclavian puncture. It was well tolerated and universally liked by the patients.
Background People with cystic fibrosis (CF) have an increased risk of thrombosis due to acquired thrombophilia secondary to chronic systemic inflammation and central venous catheter use for treatment of pulmonary infections. The objective of this study is to determine the safety and effectiveness of a risk-stratified, venous thromboembolism (VTE) prophylaxis intervention. Methods This single-center, IRB-approved, retrospective study assessed patients with CF admitted to our institution for treatment of a pulmonary exacerbation from 2017 to 2019. Data and outcomes were manually extracted from the electronic medical record and internal CF clinical database. Subject characteristics, calculated VTE risk, prophylaxis interventions prescribed, VTE incidence, and adverse events were captured. Results A total of 135 CF patients had 354 admissions for pulmonary exacerbations in the time frame of the study. The majority of admissions (88.7%) were classified as moderate or high risk for VTE using the algorithm. Overall, VTE prophylaxis intervention determined by the algorithm was initiated in 36.2% of admissions. During the study period, no VTE events occurred. Four minor bleeding adverse effects were reported in patients receiving VTE chemical prophylaxis with enoxaparin (4.2%). Conclusions This study provides the first reported outcomes following implementation of a risk-stratified VTE prophylaxis algorithm in hospitalized young people with CF. In this population at increased risk, use of risk-stratified prophylaxis was safe and effective in preventing VTE. Additional work to improve and maintain adherence to the algorithm and VTE prophylaxis interventions at our institution is planned and similar care should be considered at other pediatric CF care centers.
Port-a-cath insertion, for long-term intravenous antibiotic therapy, is an ideal solution for patient with cystic fibrosis. However, indwelling lines are liable to many complications including catheter thrombosis especially in patients having cystic fibrosis with hypercoagulable state. An endovascular technique for insertion of a port-a-cath in a patient having cystic fibrosis with occluded superior vena cava is reported. The technique is described in detail. In addition, a review of literature for the various methods of saving a failed central venous access in these patients was performed. The line was successfully inserted and remained patent without need of any further intervention for 20 months. In this report, several endovascular skills were used for central venous access salvage that can be used in similar situations with chronic superior vena cava occlusion, which may not be suitable for thrombolysis or stenting.
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Totally implantable vascular access devices (ports) were introduced into clinical practice to replace external catheters in patients undergoing prolonged intravenous therapy. Today, they are also designed to provide repeated access to the vascular system for the delivery of blood products and are also used for the withdrawal of blood samples. As regards cancer patients, the availability of these continuous reliable intravenous catheterisation devices has provided an opportunity to deliver more complex, multi-drug regimens, in safety and with a good quality of life. One hundred and eight patients underwent the subcutaneous positioning of a totally implanted reservoir (via the right subclavian vein) for aggressive combination chemotherapy in an operating room suite, using local anaesthesia. No patients suffered from air embolism, severe bleeding, brachial plexus injury, cardiac tamponade, haemothorax or hydrothorax. No patients experienced catheter occlusion or catheter-related venous thrombosis. One patient suffered from a pocket infection. Two patients experienced pneumothorax, but neither of them required catheter removal or required chest drainage. Indications, contraindications and complications are discussed. With careful routine care, totally subcutaneously implanted ports are very safe and provide reliable access to the venous system.
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An 11 year old boy with cystic fibrosis suffered a stroke, producing right sided weakness. Four years previously a totally implantable venous access device (Port-a-Cath) had been inserted. Magnetic resonance angiography revealed a filling defect in the left middle cerebral artery. Transoesophageal echocardiography demonstrated a thrombus attached to the tip of the Port-a-Cath and also the presence of a patent foramen ovale. After an initial period of anticoagulation the defect was closed using a septal occlusion device introduced via a cardiac catheter. The boy's neurological signs completely resolved and he remains free from further thromboembolic episodes. Whilst pulmonary embolism has been described before in relation to a totally implantable venous access device, this is believed to be the first description of a paradoxical embolism in relation to such a device.
Severe bronchial hemorrhage in 13 patients with cystic fibrosis was treated by catheter embolization of bronchial arteries. Indications were either excessive bleeding persisting for several days, or bleeding serious enough to interfere with pulmonary drainage and recurring over weeks or months. In follow-up ranging from one to 30 months, cessation of major bleeding was achieved in 12 of 13 patients (93%), although 5 of 13 patients (40%) did have recurrence of minor hemoptysis. No neurologic or other major complications were encountered. However, there are potential risks and this approach at present should be limited to patients with life-threatening bleeding and carried out only by experienced angiographers.
The use of vascular access systems in patients with cystic fibrosis (CF) is well accepted, with lower overall complications and maintenance costs than percutaneous silastic catheters. We report our 6 year experience with 22 infusaports in 15 CF patients. Our patients had indwelling catheters for an average of 539 days per catheter (range, 14-2,224 days). These infusaports were used for home antibiotic therapy, blood sampling, and total parenteral nutrition. The overall complication rate was relatively low, 1 in every 1,483 catheter days. Infectious complications were extremely infrequent at a rate of 1 in 5,929 catheter days. The rate of mechanical complications was 1 in 1,976 catheter days. However, superior vena caval syndrome or deep venous thrombosis was associated with 3 of 22 catheters (13.6%). Due to this high incidence of major thrombotic events with the attendant risk of pulmonary embolism, all patients with CF using infusaports and without evidence of liver disease or bleeding problems receive aspirin prophylaxis.
Bronchial arteriography and embolotherapy were performed to control hemoptysis in 11 patients with advanced stages of cystic fibrosis. Two patients suffered massive, 1 moderate, and 8 mild but recurrent hemoptysis. The embolization procedures were performed with Gelfoam, Ivalon, and coils in one to four separate procedures. Altogether, 19 of 20 procedures were successful, with follow-up periods ranging from 9 months to 8 years. No serious complications were encountered except for one femoral artery pseudoaneurysm which required surgical repair. Rapid digital subtraction angiography and "roadmapping" were considered helpful in avoiding the complication of reflux embolization and expediting the procedures. Bronchial embolization is a life-saving procedure for severe hemoptysis in patients with cystic fibrosis and is considered safe enough to include the indications of moderate and mild/recurrent hemoptysis to improve the quality of life in these patients.
Severe hemoptysis in cystic fibrosis can be life-threatening because of acute blood loss or because it interferes with sustaining physical therapy of the chest. Hemoptysis was controlled in 19 of 20 cystic fibrosis patients by means of embolization with a combination of 250-590-microns particles of polyvinyl alcohol foam and absorbable gelatin pledgets. Repeat embolization was needed in eight patients to achieve or maintain effective hemostasis. One or more aberrant bronchial arteries were found in seven patients (35%), and a spinal artery branching from a vessel that also supplied bronchial circulation was present in 11 patients (55%). The data suggest that although embolization has a wider applicability than previously reported, care should be taken during angiography to evaluate the bronchial circulation because aberrant bronchial vessels and spinal arteries arising from bronchial circulation are common.
We studied the long-term outcome after BAE for life-threatening hemoptysis in patients with CF. Data from pulmonary function tests were available for 18 of the 25 patients followed. A case-control comparison revealed that these 18 patients died sooner than hemoptysis-free patients with CF matched for age, sex, and pulmonary function (p less than 0.02), with the excess mortality occurring within the first three months after BAE. Of all 25 patients followed, six died of cardiorespiratory failure within three months of BAE; in two of them, hemoptysis was a contributing cause of death. The 19 patients who lived more than three months after BAE had a mean survival after embolization of 3.5 years (five were still alive at the end of the study). Most patients experienced long intervals (greater than 1 year) free of major hemoptysis. Extended follow-up (mean, 35 months) revealed a higher incidence of recurrent severe bleeding than previously reported for 13 of these patients followed a mean of 11 months. Repeat BAE for severe recurrence was performed successfully in eight of nine patients, without complication.
Thirteen children with cystic fibrosis had a totally implantable reservoir surgically inserted for repeated courses of intravenous antibiotics. The youngest patient was 17 months at the time of implantation. The catheters have been in place for an average of 463 ± 200 days. Forty seven courses of intravenous antibiotics have been given over a total of 832 treatment days. Three patients had complications. One experienced catheter separation from the portal, one catheter became occluded, and a third catheter fractured and migrated to the right atrium but was successfully retrieved percutaneously. The catheters were accepted well by patients and parents. We conclude that implantable reservoir systems, although moderately expensive and not totally free of complications, offer another modality in the management of cystic fibrosis patients.
Subcutaneous infusion ports (SIP) were inserted for chronic venous access during 329 procedures in 300 patients over the past five years at the University of Michigan Medical Center, with a total follow-up experience of 318 patient years. Seventy-four per cent of the SIP were surgically implanted while patients were hospitalized. The SIP were used for chemotherapeutic agents (83.0 per cent), blood products (29.0 per cent) or hyperosmolar total parenteral nutrition (8.5 per cent) and accessed a median of three occasions. Eighty-four per cent were used in an outpatient setting at least part of the time. Thirty-nine per cent of SIP were associated with complications, including local infection or sepsis (16.4 per cent), thrombosis of the catheter or central vein (9.7 per cent) and extravasation from the port secondary to needle dislodgement (6.4 per cent). The risk of complication was slightly higher in those SIP first used ten to 14 days after placement as compared with those used earlier or later (p less than 0.05). In 23 of 32 episodes, clinically diagnosed local infection unassociated with systemic sepsis or skin necrosis was successfully treated without removal of the port using aggressive intravenous and oral antibiotics. Treatment of thrombosis of the catheter with either urokinase or streptokinase infusion was successful in ten of 15 attempts. Seventy-two (22 per cent) of SIP were eventually removed, either after completion of the chemotherapy (20) or because of a complication (52) with 29 SIP being replaced. There was no correlation between the risk of infection or thrombosis and the perioperative use of antibiotics, frequency of SIP use or preoperative white blood cell count, platelet count, coagulation profile, blood urea nitrogen or albumen concentration. SIP provide an excellent method of chronic venous access, having a lower rate of infection and thrombosis in historical comparison with external vascular access devices.