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Embolotherapy in Pediatrics 307
Fig. 23.1a–d. Liver trauma in a 15-year-old boy. a CT-scan revealed the presence of an arterial pseudoaneurysm. b Selective
catheterization of the hepatic artery confi rmed the pseudoaneurysm. c With a coaxial system (Tracker: Renegade) we overpass
the pseudoaneurysm and occlude the exit branch with a coil. Then, we occlude the pseudoaneurysm with glue followed by the
occlusion of the afferent artery by a coil. d Control angiogram revealed a complete occlusion of the pseudoaneurysm
a
b
c d
Fig. 23.2a,b. A 10-year-old girl
with severe hematuria post renal
transplant biopsy. a Angiogram
shows the presence of a pseu-
doaneurysm. b With a coaxial
system, a selective catheteriza-
tion of the pseudoaneurysm
was performed. The occlusion
was performed with a coil
ba
308
J. Dubois and L. Garel
topenia, microangiopathic hemolytic anemia, and
localized consumption coagulopathy in association
with rapid evolutive hemangioendothelioma. This
syndrome requires an aggressive treatment, and
carries a mortality rate of 20 to 30%.
Aspirin, dipyridamole, antifibrinolytic agents,
aminocaproic acid, corticosteroid, interferon,
embolization, cyclophosphamide, pentoxifylline,
radiotherapy, and antiplatelet aggregating agents
have been tried with variable success [12–14]. Hepa-
rin has been shown to boost the growth of KHE [12]
and worsens the clinical situation [15, 16].
Embolization aims at reducing the high flow.
Embolization is performed by arterial approach
using polyvinyl alcohol particles or alcohol
(Fig. 23.4a–d).
23.3.3.3
Liver Hemangiomas
The differential diagnosis of hemangioma of the
liver include hepatic angiosarcoma, hepatic epithe-
lioid hemangioendothelioma, or metastatic disease
like neuroblastoma. No treatment is required in case
of asymptomatic hepatic hemangiomas. The main
indications for treatment of hepatic hemangiomas
are congestive heart failure, patients who requires
mechanical ventilatory support, feeding problem,
or Kasabach-Merritt phenomenon. Steroid is the
initial drug. Interferon or vinblastine is reserved
for refractory cases. We believe that embolization
Fig. 23.3a,b. A 10-year-old boy with a pseudoaneurysm of the femoral artery related to cardiac catheterization. a Color ultra-
sonography shows the presence of a pseudoaneurysm. b Under ultrasonography, direct puncture of the pseudoaneurysm was
performed with a 22-G Cathlon. 800 units of Thrombostat permit to occlude the pseudoaneurysm
a b
The indications of embolotherapy in vascular
anomalies are: (1) hemangiomas refractory to medi-
cal treatment, (2) hemangioendotheliomas with
Kasabach-Merritt phenomenon, (3) liver heman-
gioma with cardiac failure, and (4) arteriovenous
malformations.
23.3.3.1
Hemangiomas Refractory to Medical Treatment
Ten to 20% of hemangiomas need to be treated
[11]. Medical treatment is the first choice using ste-
roids, interferon, or vincristine. Embolotherapy is
only indicated in cases of ineffective medical treat-
ment. Embolization is mostly performed in cases of
hepatic hemangioma with cardiac failure, heman-
gioendothelioma complicated by Kasabach-Merritt
phenomenon, and uncontrolled proliferative hem-
angioma with functional disorder (e.g. tongue with
feeding problem).
The embolization provides the control of the
growth of hemangioma in its proliferative phase.
23.3.3.2
Kaposiform Hemangioendothelioma
Mueller [12] and Enjolras [13] reported that
Kasabach-Merritt phenomenon (KMP) is caused
by kaposiform hemangioendothelioma (KHE) or
tufted angioma. The KMP consists of thrombocy-
Embolotherapy in Pediatrics 309
in combination with medical treatment is the best
alternative in symptomatic liver hemangiomas.
Pre-embolization mapping is mandatory, assessing
the potential involvement of intercostal or phrenic
arteries in addition to the hepatic artery and the
portal system [17–19].
Five patterns of angiographic findings had been
described by Kassarjian et al. [20]. Embolic mate-
rial is selected depending on the vascular type. The
first type, the most classical appearance, is early
filling of abnormal vascular channels, stagnation of
contrast material, and no evidence of a direct shunt-
ing (Fig. 23.5a–h). Type 2 shows high-f low nodules
without direct shunts. Large particles can be used in
type 1 and 2. Type 3 is made of arteriovenous shunts,
type 4 of portovenous shunt, and type 5 of both arte-
riovenous and portovenous shunts.
The embolization is performed by arterial
approach for types 1, 2, 3, and 5, and by transhepatic
transvenous approach for portovenous shunts in
type 4. Platinum fiber microcoils are generally safe
in types 3, 4 and 5, and permit the occlusion of the
shunts. Glue (n-butyl-2-cyanoacrylate) is the most
effective material in patients with direct arteriove-
nous and arterioportal shunting arising from mul-
tiple sources [21]. Medical antiangiogenesis drugs
Fig. 23.4a–d. A 10-year-old boy with refractory scrotal and perineal hemangioendothelioma with recurrent scrotal bleeding.
a Selective catheterization of the perineal branch from the internal pudendal artery through an anastomosis from the common
femoral artery was performed. The opacifi cation demonstrates an important stagnation of contrast in the tumor. b The embo-
lization was performed with Gelfoam. c Catheterization of the hypogastric artery with selective catheterization of the perineal
branch from the internal pudendal artery shows a tumor blush. d (Control) angiogram after devascularization of the tumor
with particles
a b
c d
310
J. Dubois and L. Garel
Fig. 23.5a–h. A 2-month-old girl on steroid treatment with progressive liver hemangioma and feeding problem. a MR imaging
shows a large liver mass in the right lobe of the liver. b–d The aortogram demonstrates a vascular tumor supplied by the right
branch of the hepatic artery with stagnation of the contrast and normal draining vein, illustrative of type 1 liver hemangioma.
e Selective catheterization of the right hepatic artery was performed. f,g With a coaxial system, multiple feeders were catheter-
ized followed by an embolization with particles. h Postembolization opacifi cation of the right hepatic artery shows a signifi cant
devascularization of the tumor
b
d f
h
a
c
e
g
should be maintained after embolization until
nearly complete regression of the lesions.
Vascular malformations of the liver are rare,
except for the venous malformations misnamed
hemangioma in adults. Most arteriovenous malfor-
mations of the liver are seen in hereditary hemor-
rhagic telangiectasia (Rendu-Weber-Osler) with
hepatic ischemia, congestive heart failure, and
portal hypertension. Because of the risk of increas-
ing the hepatic failure, embolization is not recom-
mended in diffuse lesions. Liver transplantation is
indicated in such instances.
Arterioportal fistula in cases of hereditary hem-
orrhagic telangiectasia, Ehlers-Danlos syndrome,
or patients with biliary atresia and cirrhosis can be
treated by embolization.
Embolotherapy in Pediatrics 311
Pure venous malformations are rare and asymp-
tomatic in children. Most of them are seen specifi-
cally in patients with blue rubber bleb nevus syn-
drome which is a familial condition with multiple
venous malformations of the skin, musculoskeletal
system, and viscera.
23.3.3.4
Arteriovenous Malformations
Arteriovenous malformations (AVM) are an impor-
tant challenge for interventional angiographers.
These high flow vascular malformations are abnor-
mal communications between arteries and veins.
We do not understand exactly why some AVMs
respond well to embolization while other AVMs
progress ineluctably despite embolotherapy. That
is the reason why we favor a conservative manage-
ment for quiescent, stable, and non-bleeding AVM
with an annual MRI and cardiac ultrasound. If the
AVM progresses, bleeds, or disfigures, angiography
is essential to provide the road-map necessary for
embolization. The angiographic characteristics of
AVMs are dilatation and lengthening of afferent
arteries, with early opacification of enlarged effer-
ent veins [22]. To destroy the AVM and reduce the
risk of recurrence, a superselective catheterization
with microcatheters is necessary combined with
percutaneous puncture when feasible [23]. The best
agent to destroy the nidus is dehydrated alcohol.
The amount of ethanol needed and the pressure
of injection are evaluated with contrast media test
injections. The maximum dose is 1 ml/kg. Over
l ml/kg, the elevated serum ethanol levels put the
patients at risk for respiratory depression, cardiac
arrhythmias, seizures, rhabdomyolysis, and hypo-
glycemia [24]. The ethanol penetrates to the cap-
illary level and totally devitalizes normal tissues.
Balloon occlusion, tourniquets, blood pressure
cuffs inf lated above systolic pressure, or a combi-
nation of these can be useful if vascular occlusion
is necessary to induce stasis. Temporary compres-
sion of the venous drainage during the injection
slows the blood f low, but one should relieve the
occlusion slowly to avoid a significant modification
of the pressure within the AVM. Coagulation dis-
turbances have been reported in response to dehy-
drated alcohol that could increase the risk of bleed-
ing, thrombosis, or hematoma. In these patients, in
which the embolization is followed by surgery, the
use of glue or coils as a substitute for dehydrated
alcohol is recommended [25]; further studies are
needed to evaluate the specific changes that occur
with the dehydrated alcohol. N-butyl-2-cyanoacry-
late or coils are used for large AVM or to avoid
neuropathy when the AVM is close to nerves.
Many complications are reported particularly
with alcohol embolization, such as pulmonary
embolus, cardiovascular collapse, neuropathy, skin
blisters, radiculopathy, finger numbness, and focal
skin necrosis [23, 24]. Arterial line monitoring and
Swan-Ganz catheters are recommended for large
AVM embolization [24] (Figs. 23.6a–d, 23.7a–d).
AVM can be associated with hereditary hemor-
rhagic telangiectasia (HHT), also known as Osler-
Weber-Rendu syndrome. It is an autosomal domi-
nant inherited disease of the vascular connective
tissue characterized by epistaxis, telangiectasia,
and visceral arteriovenous malformation. The
organs mostly affected are the lungs (Fig. 23.8a–c),
liver, brain, and the gastrointestinal tract. HHT is
difficult to treat and requires a multidisciplinary
approach for its management.
23.3.4
Renal Embolization
Renal scarring secondary to vesicoureteral reflux
may be the cause of renovascular hypertension.
Renal ablation is an alternative to nephrectomy to
remove to involved kidney. The selective emboliza-
tion should be performed with alcohol to prevent
collateral revascularization. The efficacy is debated
considering that embolization may delay the defini-
tive treatment [26]. Gelfoam and coils are less valu-
able than alcohol because of collateral revascular-
ization (Fig. 23.9a,b).
Selective renal embolization can be useful and
effective in cases of refractory urinoma following
partial nephrectomy or blunt trauma. The goal of
the embolization is to ablate the fragment of renal
parenchyma that is producing and leaking urine
[27]. The embolization is done through selective
catheterization of the vessel adjacent to the leakage.
We recommend the use of particles (polyvinyl alco-
hol) as embolic agent.
23.3.5
Epistaxis
The differential diagnoses of epistaxis in children
includes trauma, foreign-body impaction, bleeding
diathesis, vascular disorder, vascular anomalies
312
J. Dubois and L. Garel
Fig. 23.6a–d. A 17-year-old boy with arteriovenous malformation (AVM) of the third digit. a Selective catheterization of the bra-
chial artery was performed and showed an AVM of the third digit. b,c Percutaneous approach was performed. Under fl uoroscopy,
with prior mapping of the AVM from the arterial side, we puncture directly the AVM with a 25-G butterfl y needle at two sites.
The acquisition shows the opacifi cation of the AVM. We injected 2 cc of alcohol. d Control angiogram by arterial approach shows
a signifi cant devascularization of the AVM but a severe arterial spasm. We monitored the coloration and skin temperature. No
arterial ischemia was seen; because of the signifi cant venous congestion, a nail resection was performed without any sequelae
a b
c d
(reported but rare in Sturge-Weber syndrome), and
nasal hemangioma. The most common indication of
embolotherapy in children presenting with epistaxis
is nasopharyngeal angiofibroma. A benign tumor,
most often seen in the adolescent boy, juvenile
angiofibromas originate from the sphenopalatine
foramen and extend into the paranasal sinuses, the
infratemporal fossa, the middle fossa, and the orbit.
The optimal treatment consists of preoperative
embolization followed by radical surgical excision.
CT-scan and MR are essential both for the diagnosis
and the assessment of the tumoral extent.
The arteries supplying juvenile angiofibromas
arise from branches of the external carotid artery
(ECA). The tumor blush is intense and persistent.
There is no arteriovenous shunting within the
lesion. The contralateral ECA should be explored in
all cases that reach the midline. The distal internal
maxillary artery is the first vessel to investigate.
Large tumors are also supplied by other branches
Embolotherapy in Pediatrics 313
Fig. 23.7a–d. An 11-year-old boy with an arteriovenous malformation (AVM) of the left shoulder. a Selective catheterization of
the posterior circumfl ex artery from the humeral artery was performed with a Tracker 38. Angiogram demonstrated multiple
dilated arteries with early venous drainage. b A Tracker 18 was introduced through the Tracker 38 for selective catheterization
of the arterial feeders. Alcohol was used for embolization. The control angiogram revealed a complete devascularization of the
AVM. c 8 weeks later a residual AVM was seen at the control angiogram. d We used the same coaxial system and alcohol for the
devascularization of the residual AVM with a good result
a b
c d
Fig. 23.8a–c. A 6-year-old girl with Rendu-Weber-Osler syndrome. a At pulmonary angiogram, small aneurysms (arrows) are
seen in the left lung. Multiple areas of telangiectasia with diffuse arteriovenous shunting are seen predominantly in both lower
lobes (arrowhead). b On selective injection of the right renal artery, several small aneurysms (arrowhead) associated with
telangiectasias (arrows) are seen. c After selective injection of the superior mesenteric artery, a small aneurysm (arrow) and
diffuse area of telangiectasia are demonstrated in the proximal jejunum
ba c
314
J. Dubois and L. Garel
revascularization) is usually performed with par-
ticles or tissue adhesive (Fig. 23.10a,b). When the
tumor has invaded the cavernous sinus, the pitu-
itary fossa, the suprasellar area, or the intracranial
intradural area, permanent preoperative balloon
occlusion of the internal carotid artery could be
helpful [28, 29].
Fig. 23.9a,b. Alcohol embolization of atrophic kidney for refractory hypertension. a Selective catheterism of left renal artery.
b Renal artery opacifi cation after alcohol embolization shows a complete devascularization of the kidney. Normalization of
blood pressure was noted
a b
such as the accessory meningeal, the ascending
pharyngeal, and the ascending palatine arteries. In
case of intracranial tumor extension, the hemody-
namics (anastomoses) between the internal max-
illary system and the ipsilateral internal carotid
artery must be analyzed carefully. Distal emboli-
zation (proximal occlusion would result in tumor
Fig. 23.10a,b. Juvenile nasopharyngeal angiofi broma in an 11-year-old boy. Preoperative embolization with particles of the arte-
rial branches of the internal maxillary artery. a Selective external carotid angiogram shows a vascular tumor fed by multiple
branches of the internal maxillary artery. b After multiple selective catheterization with a coaxial system (Tracker 38 and Tracker
18), embolization was performed with particles. Postembolization angiogram shows signifi cant devascularization
a b
Embolotherapy in Pediatrics 315
23.3.5
Hemoptysis
Most patients who may benefit from bronchial artery
embolization are teenagers with cystic fibrosis (CF)
and major hemoptysis. The hazards of general anes-
thesia with positive pressure ventilation in CF patients
have been addressed recently in the literature. A
fatal pulmonary hemorrhage during induction was
reported by Mcdougal and Sherrington [30]. The
technique is well established: femoral artery access,
5-F catheter, precise vascular mapping, coaxial tech-
nique with Tracker 18 catheters, secure catheter
placement prior to performing embolization, and use
of polyvinyl alcohol particles (size: 355–500 microns
or 500–710 microns if microfistulas are observed)
(Fig. 23.11a–d). Coil embolization or surgical ligation
of bronchial arteries should be avoided because they
hinder subsequent catheterization of the proximally
occluded vessel. Careful attention should be paid
for the identification of spinal arteries arising from
the bronchial or intercostal arteries. The localizing
value of emergency bronchoscopy or multidetector
CT scanning prior to embolization remains to be
evaluated.
Bronchial artery embolization in CF patients is
very effective immediately and on short-term basis.
Many patients will require repeated embolizations
during t he follow-up [31]. De spite the reported severe
complications of bronchial artery embolization, the
procedure has proved to be safe if performed by
experienced angiographers.
Fig. 23.11a–d. Hemoptysis in a 17-year-old boy with cystic fi brosis. a Selective catheterization of the right bronchial artery with
a 5-F hook catheter shows dilated and tortuous arteries in the superior and middle pulmonary lobes. b With a coaxial system
(Renegade 18), an embolization was performed with particles (Contour 500-700 microns). Postembolization angiogram shows
devascularization of the bleeding site. c Selective catheterization of the bronchointercostal trunk. Multiple dilated and tortu-
ous arteries are seen. d Selective catheterization with the same coaxial system. Embolization was performed with particles.
Postembolization angiogram shows complete devascularization
a
b
c d
316
J. Dubois and L. Garel
23.3.6
Gastrointestinal Bleeding
In children, localized gastrointestinal bleeding is
usually secondary to duodenal ulcer and less fre-
quently to gastric ulcer, Meckel’s diverticulum, and
vascular malformations. Diffuse bleeding can occur
in vasculitis and coagulopathy.
Gastrointestinal vascular anomalies are often
associated with known syndromes such as Klippel-
Trenaunay, Rendu-Osler-Weber, blue rubber bleb
nevus, and Proteus syndromes.
In our experience, venous malformations are the
more common vascular anomaly encountered in
cases of bleeding (Fig. 23.12a,b).
In children with gastrointestinal arteriovenous
malformations, the angiographic examination con-
firms the diagnosis and the extent of the AVMs.
Embolization is usually not recommended in small
bowel and colonic lesions because of the risk of
necrosis [32, 33]. Embolotherapy is sometimes con-
sidered preoperatively to lower the risk of operative
bleeding.
23.3.7
Embolization or Sclerotherapy of Varicoceles
Varicoceles are present in 15%–20% of preadoles-
cents and adolescents. The treatment of varicoceles
in this age group is controversial. The treatment
can be surgical, endoscopic or radiological (sclero-
therapy or embolization of the internal spermatic
vein). Most of the varicoceles are seen on the left
side. Bähren et al. [34] described five types of left
varicoceles according to the anatomy of the internal
spermatic vein (ISV).
Irrespective of the type of varicocele, the sclero-
therapy procedure is the same. Our technical pro-
tocol for percutaneous endovascular occlusion
of the ISV is as follows: IV sedation by Ketamine/
Midazolam, femoral vein approach, 7-F Cobra
catheter with coaxial 3-F or Tracker 18 for distal
sclerotherapy by sodium tetradecyl sulfate (STS)
followed by more proximal coil occlusion, bed-rest
for 4 hours, and discharge 6 hours post-procedure
(Fig. 23.13a,b). Results are assessed by the referring
surgeon 2 months later.
In our series, we have found a high incidence (44%)
of anatomical variants in the pediatric population.
Technical difficulties of retrograde sclerotherapy
were seen in type IVb, collaterals from segmental
renal veins to the internal spermatic vein with a com-
petent ostial valve (12% of our cases, failure rate 50%)
and in type V, double renal veins (14% of our cases,
failure rate 33%). Our overall results (failure rate 10%)
are comparable to the recently reported pediatric
series [35, 36], in the radiological and surgical litera-
ture. The issue of radiation related to interventional
procedure has been addressed in the literature. If the
Fig. 23.12a,b. A 13-year-old boy presented with recurrent lower gastrointestinal bleeding. Lower gastrointestinal endoscopy was
normal. a,b Selective injection of the right colic artery shows dysplasic veins (arrowhead) in the ascending colon
a b