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Congenital symptomatic intrahepatic arteriovenousfistulas in newborns: management of 2 caseswith prenatal diagnosis

Mario Limaa,*, Marianna Lallaa, Antonio Aquinoa, Marcello Dominia, Stefano Tursinia,Giovanni Ruggeria, Gabriella Pelusia, Antonia Pignab, Gianni Tanic,Gian Luigi Pilud, Daniela Prandstrallere, Nunzio Salfif

aDepartment of Pediatric Surgery, University of Bologna, 40138 Bologna, ItalybPediatric Intensive Care Unit, University of Bologna, 40138 Bologna, ItalycPediatric Radiology, University of Bologna, 40138 Bologna, ItalydDepartment of Obstetrics and Gynecology, University of Bologna, 40138 Bologna, ItalyeDepartment of Pediatric Cardiology, University of Bologna, 40138 Bologna, ItalyfInstitute of Pathology, University of Bologna, Bologna, 40138 Bologna, Italy

0022-3468/$ – see front matter D 2005

doi:10.1016/j.jpedsurg.2005.06.033

T Corresponding author. Tel.: +3

63663657.

E-mail address: m.lima@med.unibo

Index words:Hepatic arteriovenous

malformation;

Prenatal diagnosis;

Vascular embolization;

Hepatic resection

AbstractBackground: Fetal and neonatal hepatic arteriovenous fistulas are rare and associated with a high

mortality rate; they can be prenatally detected by ultrasonography. Management of these malformations

can be a challenge for pediatric surgeons.

Methods: Two patients with a prenatal diagnosis of intrahepatic arteriovenous shunts were treated at ourinstitution in the last 2 years. A hepatic complex arteriovenous malformation fed respectively by

prominent branches of the hepatic artery and of the celiac trunk rising from dilated suprarenal aortae and

draining into suprahepatic veins was detected. In the first case, an embolization was performed; in the

second, the surgical resection of the vascular malformation was the treatment of choice.

Results: The first patient died after embolization and before surgery for hemodynamic complications.

The second patient, at a follow-up of 16 months, is alive and doing well.

Conclusion: Hepatic resection is the treatment of choice for localized intrahepatic arteriovenous

malformation. Theoretically, embolization could be curative or reduce the size of a malformation,

making consequent hepatic resection feasible. Results do not support this theory because of the high rate

of complications recorded that brought in every case, ours included, to the death of the child.

D 2005 Elsevier Inc. All rights reserved.

Neonatal hepatic vascular anomalies are rare and are

associated with high mortality rates. Prenatal diagnosis is

Elsevier Inc. All rights reserved.

9 051 6364985; fax: +39 051

.it (M. Lima).

very important and useful in alerting perinatal care

assistance; diagnosis must be confirmed early at birth to

permit an appropriate definitive treatment. The management

of these malformations should be adjusted to individual

requirements; it cannot be rigid and must look after the size,

extent, and localization of the hepatic vascular anomalies

Journal of Pediatric Surgery (2005) 40, E1–E5

M. Lima et al.E2

and their associated complications. In general, hemangio-

mas (hemangioendothelioma, HE) are benign tumors not

present at birth, appear few days after, grow up rapidly in

the first year of life, and then tend to regress spontaneously

slowly in 5 or 6 years; the treatment is indicated only for

life-threatening hemodynamic disturbances or disfiguring

lesions and consists of pharmacological (corticosteroids or

interferon) or surgical therapy. Infantile hepatic heman-

gioendothelioma (IHHE) is a peculiar type of capillary

hemangioma: it has a proliferating and an involuting phase

and is the most common symptomatic vascular tumor, with

85% of cases presenting in the first 6 months of life. On the

contrary, the arteriovenous malformations (AVMs) are

present at birth even if they become evident in infancy

and childhood. Because they are malformations of the

vascular channels (capillary, venous, or arterial), they never

regress spontaneously. A variety of approaches to treatment

have been tried, without univocal results. Many different

therapies are described: radiotherapy [1], arterial emboliza-

tion [1-3], hepatic artery ligation [4,5], hepatic lobectomy

[6], and laser therapy. We present our experience with

2 cases of arteriovenous fistula (AVF), where different

approaches have been used, with a critical review of the

management based on the literature.

1. Patients

Two female patients with a prenatal diagnosis of intra-

hepatic AVF were treated at our department in September

2002. For the cases, the diagnosis was obtained between the

25th and the 27th week of gestational age. In the first

patient, a fluid area within the fetal abdomen, close to the

inferior vena cava (IVC), was noted; color Doppler imaging

demonstrated the presence of ba vessel in the fluid area,

which enters the inferior vena cava.Q In the second patient, a

fluid-filled area affecting the upper part of the left hemi-

abdomen, characterized by pulsatile venous blood flow

suspected to be connected to the IVC, was described.

Complications associated to intrahepatic AVM were a

moderate cardiomegaly with minimal pericardial effusion

in the first case and severe cardiomegaly with initial signs of

heart failure in the second. The 2 fetuses were monitored

during pregnancy with seriated ultrasound (US) controls.

Fig. 1 First case. Embolization of the

To avoid hepatic rupture, a cesarean delivery was

performed on the mothers at 35 and 37 weeks of gestation.

None of the patients had cutaneous angiomata or diffuse

angiomatic lesions. Also, none of the patients had a Down’s

syndrome. A postnatal US confirmed the antenatal diagnosis

in both cases.

1.1. Child 1

A dilatation of the suprarenal aorta and a considerable

dilatation of the left hepatic artery were seen at prenatal

US. The patient manifested since birth clinical signs of

congestive heart failure and severe thrombocytopenia with

disseminated intravascular coagulopathy (Kasabach-Merritt

syndrome), which required platelet transfusions and treat-

ment with cardiokinetics and diuretics. In consideration of

the size, extent, and localization of the lesion, affecting

almost the entire liver, and the clinical condition of the

patient, surgery seemed to be not indicated and the

management was carefully discussed in a multidisciplinary

session with radiologists, neonatologists, and anesthetists.

For this case, embolization resulted to be the most

appropriate treatment whereas surgery was planned to be

performed after embolization, when a reduced extent of the

lesion and better clinical conditions of the child would be

achieved. Then, on the first day of life, an arteriography was

performed, which showed a hepatohepatic AVF fed by

branches of the right hepatic artery and by a dilated left

hepatic artery and drained by left suprahepatic veins.

Embolization was feasible only for the branches of the right

hepatic artery (Fig. 1); on the left side, embolization was

impossible owing to the inappropriate size of the catheter.

1.2. Child 2

At birth, the child was not symptomatic, although a mild

dilatation of her heart was detected at echocardiography and

treated by diuretic supply. Postnatal US showed a dilatation

of the abdominal aorta (b, 7 mm) and of the celiac trunk (b,5 mm), confirming the diagnosis of intrahepatic AVM. On

day 2 of life, an angiographic magnetic resonance imaging

was performed: it showed a hepatohepatic AVF affecting the

left hepatic lobe fed by arterial branches of the celiac trunk

and drained by left suprahepatic veins (Fig. 2). Further

echocardiography revealed a moderate dilated right ventricle

right branches of the hepatic artery.

Fig. 2 Second case. Angiographic magnetic resonance imaging.

Top panel, a hepatohepatic AVF affects the left hepatic lobe.

Bottom panel, the AVM is fed by arterial branches of the celiac

trunk and drains into left suprahepatic veins.

Congenital symptomatic intrahepatic arteriovenous fistulas in newborns E3

with normal biventricular function and patent ductus arterio-

sus; thus, diuretics and cardiokinetics were administered. On

the second day of life, the child underwent a left hepatec-

tomy. At laparotomy, the malformed hepatic area could be

distinguished on the surface of the liver because it was tender

and surrounded by pulsating blood vessels. This area

resulted to be within the left hepatic lobe, so a left

hepatectomy was successfully carried out without significant

intraoperative blood loss (70 mL). The histology of the

resected hepatic lobe showed an intrahepatic AVM.

2. Results

2.1. Child 1

Immediately after embolization, the child’s clinical con-

ditions improved. Nevertheless, on days 2 and 3 of life, the

hemodynamic status and the coagulopathy of the patient

worsened, until the child died of congestive heart failure.

Postmortem autopsy revealed anAVMof the left hepatic lobe,

cardiomegaly, and congenital heart and lung malformations.

2.2. Child 2

A postoperative echocardiogram revealed normal ven-

tricular size and function; thus, pharmacological therapy

was suspended. Six months after the operation, an abdom-

inal US revealed a normal hepatic parenchyma with normal

vascular flow. After a 20-month follow-up, the patient is

healthy and requires no therapy.

3. Discussion

With the term neonatal vascular hepatic anomalies,

several vascular lesions are indicated. The etiology,

natural history, and pathological findings of hepatic

AVM are different from vascular tumors such as HE

and IHHE [7,8]. Vascular malformations are developmen-

tal anomalies and therefore mostly present at birth; these

malformations consist of dysplastic vessels lined by a

quiescent endothelium: they are classified by their

predominant channel type (capillary, venous, arterial,

lymphatic, or a combination) and by flow characteristics

(slow flow and fast flow). Within the liver, they act as

low-resistance AV shunting and can yield consequences

on systemic circulation (congestive heart failure), hepatic

hemodynamic status (portal hypertension and, rarely,

disorders of hepatic function), and thrombocytopenia

secondary to local platelet sequestration (consumptive

coagulopathy or Kasabach-Merritt syndrome). These

different clinical signs depend on the type of vascular

shunt. Two types of shunt have been described: (1)

hepatohepatic AV shunt (from the hepatic artery directly

into the IVC and to the right side of the heart bypassing

the liver) that leads to congestive cardiac failure [9,10]

and (2) hepatoportal shunt between hepatic arteries and

portal veins (with consequent congestive increase of portal

pressure and gastrointestinal symptoms such as abdominal

distension, persisting vomiting, gastrointestinal bleeding,

and watery diarrhea).

The higher the blood flow owing to AVM toward the

right side of the heart is, the higher the cardiac failure is but

the less important portal hypertension becomes. The earlier

the symptoms are noted, the worse the prognosis is [8].

Cardiac failure is reported in up to 58% of affected

newborns and is associated with a 50% to 90% mortality

rate [11].

Our 2 cases both belong to the form of hepatohepatic

AV shunt.

Congenital hepatic AVFs are very rare and can be

successfully diagnosed during pregnancy with US and color

flow Doppler imaging [12,13]. Peculiar features at antenatal

diagnosis are hepatomegaly and prominent intrahepatic

blood vessels and those at color Doppler imaging include

M. Lima et al.E4

an abnormal intrahepatic blood flow. At birth, the prenatal

diagnosis of hepatic vascular malformation is confirmed by

US (pulse wave Doppler and color Doppler imaging),

magnetic resonance imaging that shows intrahepatic AVFs

as large bflow voidsQ and localized mass with increased

signal intensity on T1-weighted images [2], and eventually

by an arteriography that shows the anomalous blood supply

and the AV shunting within the vascular anomaly, giving

criteria of its resectability [11].

Many different therapies are described to treat the broad

spectrum of hepatic vascular anomalies (AVM, HE, IHHE):

radiotherapy, steroid therapy, arterial embolization, hepatic

artery ligation, hepatic lobectomy, and laser therapy.

Because of the rarity and the different peculiarities of the

types of lesions (AVM vs HE), it is difficult to assess the

efficacy of the different forms of treatment.

1. Radiotherapy is effective only for vascular tumors (HE,

IHHE) and not for AVM. Furthermore, because of the

side effects (eg, hepatitis, cirrhosis, leukemia, and

hepatosarcoma), radiotherapy should not be considered

unless other therapies are unsuccessful [2,14].

2. Steroids have been reported to be effective in the man-

agement of hepatic HE in infants. Intrauterine steroid

therapy (methylprednisolone 2-4 mg/d for 2-4 weeks)

for a fetus with congestive heart failure is described in

the literature to be effective in improving fetal hemo-

dynamic, decreasing liver vascularity and delaying early

emergency delivery [8]. Arteriovenous malformation

does not respond to steroids.

3. Transfemoral percutaneous catheter embolization is

widely recommended because it can temporarily control

life-threatening congestive heart failure if done in

addition with pharmacological therapy. A wide range

of embolic agents have been described. For an effective

treatment, the fistula must be accurately localized and a

superselective catheterization should be achieved and

the nonabsorbable embolic agent should occlude totally

all feeding arteries as close as possible to the lesion.

Complications such as incomplete occlusion, rapid

development of collateral vessels, necrosis, and hematic

transportation of embolic agents from the site of

application are described [8,15].

4. Hepatic artery ligation is not always successful because

it may fail to control symptoms [14]; described

complications are the rapid development of collateral

vessels, liver necrosis, and abscess [2].

5. Partial hepatic resection is recommended only for well-

defined localized lesions not associated with hemody-

namic complications or for emergencies such as

spontaneous bleeding and rupture of the mass [10];

morbidity is low and mortality is negligible. Potential

complications are bleeding, hepatic necrosis, and

abscess [2]. Care must be taken to clamp the vessels

tributary to the AVM and to resect the whole AVM

within a convenient resection margin.

6. Laser therapy has been described in the course of

laparotomy or laparoscopy to treat both hepatic AVM

and HE [8].

After a review of the literature, we found 22 cases of

antenatal diagnosis of hepatic vascular anomalies treated

in various forms: 16 cases involved patients with HE

[13,16-19]; 2, IHHE [20,21]; 2, diffuse hemangiomatosis

[13,22]; and 2, hepatic AVM [9,12].

Therefore, assembling the 3 cases reported in the

literature with our 2 patients, only 4 cases of antenatal

diagnosis of AVM are described. The treatments undertaken

were as follows: (1) arterial embolization of the feeding

arteries of the hepatic vascular mass (one described in

literature and one in our series)—in these 2 cases, the

occlusion of the arteries was incomplete and the mortality

rate was 100% ([9]; our first patient); (2) in one patient, a

hepatic left artery ligation was performed—the child was

alive and well [12]; (3) in one case, a left hepatectomy (our

second patient) was done—with good results.

In conclusion, embolization can cure an AVM only in

selected cases: when technically possible, the catheteriza-

tion of all anomalous vascular communications or as an

adjunct to surgery (arterial embolization, done 24-72

hours before surgical resection, temporarily occludes the

nidus and reduces the size of the malformation, thus

reducing the impact of intraoperatoric complications).

Finally, we believe that surgical treatment should be

considered as the best choice of management for localized

intrahepatic AVMs.

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