Endovascular techniques used in children for diagnostic and therapeutic purposes are relatively similar compared to those used for adults. Most of these procedures performed in the pediatric population have been extrapolated from the adult experience. However, vascular interventional radiology in pediatrics differs from adult vascular interventions in several aspects, and we frequently face the need for miniaturization of equipment for appropriate use in the pediatric population. Despite anatomical and technical issues, significant clinical and technical success rates have been achieved with endovascular techniques in the pediatric population.1
Furthermore, when the patient is evaluated for moderate sedation or general anesthesia, it is important to consider the maintenance of body temperature control, fluid balance, iodine contrast dose, radiation safety, and equipment selection. Also, technique standardization of any angiographic/vascular procedure is limited due to the fact that different devices are necessary to perform procedures safely in patients with a large range of body size. Children have both advantages and disadvantages and may require alternative techniques. Complications may be similar to those reported in adults, but specific problems may be seen with different prevalence (i.e., vessel thrombosis).1,2
There has been tremendous growth in pediatric vascular interventions, and most recently, pediatric embolotherapy has become technically feasible mainly due to the availability of smaller diagnostic catheters and microcatheters. This chapter will focus on some specific indications for embolotherapy in pediatrics, emphasizing particular issues to be considered in this age group.1–3
For all procedures, informed consent is obtained from the parent, guardian, or the patient if they are of legal age to consent. In these forms, the procedure is described, therapeutic alternatives are discussed, and procedure-related complications are mentioned. Routine blood work and coagulation studies are requested. For therapeutic procedures, the coagulation references are platelet count over 50,000/µL, prothrombin time less than 18 seconds, partial thromboplastin time less than 32 seconds, and international normalized ratio less than 1.2. If the blood tests are abnormal, coagulation has to be optimized to avoid procedural bleeding, and a consultation with a hematologist is recommended. Patients are not routinely blood matched, except for those procedures when a possibility of significant blood loss exists, such as transarterial or transjugular intrahepatic portosystemic shunt procedures in coagulopathic patients.4
Patient’s diet is held for an appropriate time as determined by age, alimentary requirements, type of sedation, and need for general anesthesia. Regarding diet, solids may be consumed until 8 hours before sedation or general anesthesia in 6 months of age or older patients. In younger patients, breast milk and formula are permitted until 4 hours before the procedure. Also, patients are able to take clear fluids by mouth up to 2 hours before the procedure. Antibiotics are not routinely prescribed before the embolization procedure, with particular exception for splenic embolization and patients with congenital heart defects. For the former, we usually use a recommended broad-spectrum antibiotic regimen (ceftriaxone/clindamycin) just before and for 5 days after the embolization procedure. For the latter, we use a prophylactic single dose of cefazolin or cefotaxime, 50 mg/kg.2,4
In all cases, the angiography room must be warm, particularly when procedures are performed in newborns and infants. Patients must be at a constant body temperature, and covers, hat, Bair Hugger (Arizant Healthcare, Inc., St Paul, Minnesota), heat lamp, and warming blanket are frequently necessary. Also, any solutions used including the contrast medium should be slightly heated. In pediatrics, the amount of contrast media is a limiting factor. The accepted dose of nonionic contrast should not exceed 5 mL/kg.1–4
A sedation protocol is essential to establish a successful pediatric interventional practice. The choice of whether to use conscious sedation or general anesthesia is influenced by many factors, but for embolization procedures, general anesthesia is preferred.3,5
When planning for sedation, special consideration should be given to children who are very ill, especially those with hepatic and/or renal failure. These patients are at greater risk for acute drug toxicity, hypotension, respiratory depression, and prolonged sedation because of their inability to metabolize and excrete most sedation drugs. Therefore, general anesthesia is often preferred for this group.2–4 General anesthesia is also preferred when the procedure is expected to be lengthy or painful, when there is a history of failed or difficult sedation, or when there are contraindications or unacceptable risks to sedation.3Hence, general anesthesia is usually indicated for embolization procedures.1–5
Children are more radiation-sensitive than adults and have a longer lifespan during which to manifest radiation-induced cancers. Therefore, it is prudent and important to use appropriate radiation safety techniques when performing pediatric interventional procedures.6 Radiologists, radiologic technologists, and all supervising physicians have a responsibility to minimize the radiation dose to individual patients and to staff while maintaining the necessary diagnostic image quality, following the “as low as reasonably achievable (ALARA)” principle. Vascular procedures might be associated with a moderate radiation dose. Also, lengthy or repeated procedures may result in significant radiation exposure, making it extremely necessary to respect and follow the radiation safety protocols. Examination protocols should vary, taking into account patient body habitus, such as height and/or weight, body mass index, or lateral width. The dose reduction devices that are available on imaging equipment should be active; if not, manual techniques should be used to moderate the exposure while maintaining the necessary diagnostic image quality.7
Some techniques for decreasing patient radiation dose should be considered, using reduced-dose protocols, the last-image-hold feature, pulsed fluoroscopy, tightly collimating to the area of interest, and minimizing the use of magnification. Periodically, radiation exposures should be measured and patient radiation doses estimated by a medical physicist in accordance with the appropriate technical standards.5–7
Vascular access in children based on palpation and anatomic landmarks can be challenging. Ultrasound (US) guidance for the arterial puncture may increase the chances of successfully gaining access to these small-caliber arteries.5,8In children weighing more than 30 kg, femoral access is obtained with use of an 18-gauge needle, and a 4-Fr or 5-Fr catheter is inserted. In children weighing between 10 and 30 kg, a 20-gauge needle might be used for arterial puncture and a 4-Fr catheter is used.
Arterial puncture in children weighing less than 10 kg can be technically challenging and should not be done with anything larger than a 20-gauge needle. For smaller children, the use of 3-Fr devices is recommended.4,5Vascular sheaths are placed in all patients because these procedures are often lengthy and can require catheter exchanges. To prevent vessel thrombosis, an initial bolus of heparin is given (75 to 100 units/kg). In case of lengthy procedures, a subsequent heparin injection should follow. Arterial spasm is frequent in this population and may be treated with intra-arterial injection of papaverine (1 mg/kg) or nitroglycerin (2 to 3 units/kg).1,2,4,5
Embolic agents used in pediatric embolotherapy are the same as those used in adult patients. Cautions should be considered in case of alcohol use. A maximum dose of 1 mL/kg (or 60 mL) per session should never be exceeded.1,2Microcatheters allow practitioners to perform many of these pediatric embolization procedures, and coaxial systems (guide catheter/microcatheters) are ideal for this purpose.
Diagnostic arteriography is gradually being replaced with noninvasive imaging. Indications for diagnostic vascular studies still occur, but frequently, these diagnostic studies are done in conjunction with a therapeutic intervention (i.e., embolization).
Transcatheter embolization is currently a common procedure in pediatric tertiary centers performed by physicians trained in endovascular procedures and aware of technical modifications and alternatives needed for this age group of patients.1,4,5
It is important to ensure the relevance of the procedure and decision must be taken based on a multidisciplinary approach. This chapter will focus on some particular entities almost exclusively found in pediatric patients, such as vascular tumors and congenital shunts and fistulas. Vascular malformations, a pathology very frequently found in pediatric interventional radiology practice, will not be discussed in this chapter (see Chapter 13).
In 1982, Drs. Mulliken and Glowacki proposed a classification of vascular congenital anomalies based on the clinical, biologic, and cytologic findings. According to this classification, vascular anomalies were divided into either vascular tumors (hemangioma, hemangioendothelioma, and other vascular tumors) or vascular malformations (capillary, venous, lymphatic, arteriovenous, and combined lesions).9,10
Vascular malformations are composed of irregularly dilated, malformed, or dysplastic vessels, with variably thickened vascular channels lined with mature endothelial cells. These lesions are usually present at birth and do not regress. Vascular malformations exhibit a predictable group of clinical patterns that vary in severity and rate of progression. These vascular malformations were named and classified based on the affected vascular channel (arterial, capillary, venous, or lymphatic or in combination).9–11 Later, a complementary hemodynamic classification was described in 1993, dividing vascular malformations into high-flow and low-flow lesions.12
Sclerotherapy is the technique used to treat low-flow vascular malformations. High-flow lesions are treated with superselective transarterial, direct, and transvenous access, with flow reduction techniques, to deliver an adequate dose of sclerosant and embolic agents to the malformation nidus.9–11,13
Vascular tumors demonstrate rapid neonatal growth, endothelial hypercellularity, increased cellular turnover, and in the case of infantile hemangiomas, a later involutive phase. Hemangiomas are the most frequent tumors in infancy.10,11,13,14
Main indications of embolotherapy in vascular tumors are hemangiomas refractory to medical treatment, hemangioendotheliomas with Kasabach-Merritt phenomenon, and liver hemangioma with cardiac failure.1,2,10,11,14
The most frequent vascular tumors in infancy are infantile hemangiomas. Congenital hemangiomas (noninvoluting congenital hemangiomas [NICH] or rapidly involuting congenital hemangiomas [RICH]), hemangioendotheliomas, tufted angiomas, and sarcomas are other vascular tumors seen in children.10,11,14
Hemangiomas are the most common tumors in infancy, with predominance in the females (3:1). Generally, they appear in the first week of life, and 60% of the cases are located at the head and neck. As mentioned, the classic infantile hemangioma presents a first stage of rapid proliferation of approximately 3 to 12 months of duration, with a period of stability and a stage of slow regression lasting from 2 to 10 years. Frequently, nearly 80% of these tumors do not need treatment.
The diagnosis of the hemangiomas is mainly clinical. Doppler US and magnetic resonance imaging are useful diagnostic imaging methods, demonstrating the hypervascularity and extension of these tumors. Angiography and the endovascular techniques are only reserved for therapeutic purposes.2,10,11,14
Hemangiomas Refractory to Treatment
Hemangiomas will only need treatment in 10% to 20% of cases, such as periocular hemangiomas with orbital compromise and/or vision impairment; visceral hemangiomas associated with cardiac insufficiency; hemangiomas with persistent ulceration; hemangiomas compromising the airway; facial hemangiomas with rapid growth and distortion, with presumption of important sequels; symptomatic muscular hemangiomas; and in the presence of Kasabach-Merritt phenomenon.
In case a hemangioma needs treatment, medical treatment is usually the first option. The most common first-line therapy is propranolol (2 to 3 mg/kg/day) that typically has excellent results. Other treatments include steroids, interferon, and vincristine.10,14–16
Embolization and/or surgery are required when medical alternatives are ineffective, mostly in cases of liver hemangiomas with cardiac failure, hemangioendothelioma complicated by Kasabach-Merritt phenomenon, and uncontrolled proliferative hemangioma with functional disorder that do not respond to pharmacologic treatment.10,17,18 Embolization of hemangiomas imposes a precise, distal, intratumoral occlusion. Particulated agents (gelfoam and 300 µm microparticles of polyvinyl alcohol or acrylic microspheres) are usually employed for this purpose.17,19
Patients’ weight is a bounding factor in the use of contrast media in these procedures. Therefore, in some cases, a first procedure will be focused on the embolization of main tumor-feeding vessels. If symptoms persist, this procedure can be repeated.1,2,5,17
The Kasabach-Merritt phenomenon (KMP) consists of severe thrombocytopenia, microangiopathic hemolytic anemia, and localized consumption coagulopathy, in association with a rapid tumor growth, with reported death rates of 20% to 30%. KMP is actually related to another type of vascular tumor (kaposiform hemangioendothelioma, tufted angioma) and not to the “classic” hemangioma. These tumors present a more invasive and aggressive behavior. Hence, the KMP phenomenon, due to the associated high death rate, needs to be treated.2,11,17,18 Medical treatment is recommended. Steroids are the first-choice treatment, keeping interferon and vincristine for steroid-refractory treatment. Embolization is needed if medical treatment fails and is always associated with medical treatment.2,10,14,18 Embolization, performed by arterial approach, aims at reducing the high flow. In cases of KMP, a distal intratumoral occlusion (Fig. 60.1A–D) is also necessary; therefore, particles are also frequently used in these cases.2,10,15,17,18
No treatment is required in case of asymptomatic hepatic hemangiomas, but these cases should be closely followed. The main indications for treatment of hepatic hemangiomas are congestive heart failure or KMP.15–17,19 Again, if treatment is needed, medical treatment with propranolol is a first-line therapy. Other treatments include steroids, interferon, and vincristine.10,14–16
In nonresponding patients or in cases where an emergent endovascular treatment is indicated due to a rapid and deleterious progression, embolization is indicated.17 When a hepatic hemangioma needs treatment, a complete vascular mapping is necessary, identifying the arterial vascularization of the tumor (hepatic and extrahepatic feeders) and the patency of the portal vein.19 Different patterns of angiographic findings have been described for hepatic hemangiomas.19 Embolic material is selected depending on the vascular pattern. The most classical appearance is an early filling of abnormal vascular channels without evidence of direct shunting. Others may show high-flow nodules without direct shunts. Embolization is performed by arterial approach for these types, and large particles (≥500 µm) can be used for this purpose. Fatal complications have been reported with the use of small-caliber particles in such cases due to migration to the pulmonary circulation. Hepatic necrosis has also been described with small particles (<300 µm).17,19,20 The choice of the embolic agent is based on the size of the shunts. Also, in small and ill patients, the amount of fluid needed for the delivery of the embolic agent should be minimized. There are no specific recommendations about “contrast:saline solution” for particles infusion; this solution should maintain enough radiopacity to be clearly seen under fluoroscopy. We usually use a 70% contrast/30% saline solution for this purpose.
Hemodynamic patterns including arteriovenous shunts, portovenous shunts, or both arteriovenous and portovenous shunts have been described. Embolization with platinum microcoils are generally safe in such cases and permit the occlusion of the shunts. Glue (N-butyl cyanoacrylate) is also an effective material in patients with direct arteriovenous and arterioportal shunting arising from multiple sources.17,19,20Glue dilution is based on shunt flow. For shunts involving arterial feeders, a 50:50 (glue:Ethiodol) or greater solution is preferred. Whenever possible, flow reduction techniques might be considered.
Staged procedures are prudent to minimize the appearance of complications such hepatic ischemia, hepatic necrosis, or death.17
Knowing the portal involvement in tumor supply is extremely important for embolization planning. Arterial embolization in cases of portovenous fistulas might be ineffective in reducing the cardiac symptoms and may induce hepatic necrosis. In such cases, embolization of portovenous fistulas from transhepatic or transvenous approach is advocated. If necessary, after the venous occlusion, the arterial embolization may be considered. Clinical treatment (propanolol, steroids, and or vincristine) should be maintained after embolization until nearly complete regression of the lesions.2,17,19,20 The differential diagnosis of liver hemangiomas includes mesenchymal hamartoma, hepatic angiosarcoma, hepatic epithelioid hemangioendothelioma, or metastatic disease, such as neuroblastoma.10,17,20
CONGENITAL PORTOSYSTEMIC SHUNTS
Congenital portosystemic shunts are rarely seen. These abnormal communications can be congenital or acquired, asymptomatic (frequently diagnosed incidentally), or diagnosed due to the presence of shunt complications. Most of them occur in infants.21,22 These shunts are related to the lack of complete involution of one or several fetal vessels, establishing abnormal vascular communications between any vein of the portal system and any vein of the inferior vena cava system. They may exist inside or outside the liver, may be single or multiple, and vary in size. These anomalous communications can cause a partial or complete diversion of the portal flow to a systemic vessel.23 Some small intrahepatic portosystemic shunts located between the portal branches and hepatic veins may resolve spontaneously by age 1 to 2 years; others, mostly the large shunts, such as extrahepatic, persistent ductus venosus, or still patent intrahepatic shunts, persist throughout life and carry risks of complications. They differ from the acquired intrahepatic and extrahepatic portosystemic shunts occurring as a consequence of portal hypertension.24
Associated risks of severe complications exist including neonatal cholestasis, benign and malignant liver tumors, hepatopulmonary syndrome, portopulmonary hypertension, and encephalopathy. The presence of an anomalous portosystemic shunt should be considered in patients with central, not cardiogenic, cyanosis and normal thoracic imaging results (radiography and/or computed tomography [CT]). Hence, the severity of some of these complications and the potential reversibility after shunt occlusion make necessary the treatment of these abnormal communications.23–29
The diagnosis of these entities may be obtained with US and Doppler US imaging, allowing identification of the anomalous communication.
Once a congenital portosystemic shunt is found in a child, either during the investigation of a complication during the neonatal period or later as a fortuitous finding, the first step is to be sure that the shunt is not the consequence of portal hypertension or, during early infancy, of a liver hemangioma that would require a specific treatment.23,24
Congenital portosystemic venous shunts are best classified into intrahepatic and extrahepatic varieties. In the former, the connections are created between branches of the portal vein, after its division, and the hepatic veins or inferior vena cava. In extrahepatic shunts, the anastomoses are established between the portomesenteric vasculature, before division of the portal vein, and a systemic vein.21–24,30,31 Therefore, these shunts are divided into two broad types: (1) intrahepatic shunts located between the portal vein or one or several of its branches on one side and the inferior vena cava or a hepatic vein on the other, including the ductus venosus (Fig. 60.2A–D), and (2) extrahepatic shunts, which join directly the portal trunk or one of its branches of origin to the inferior vena cava or one of its branches.24
As far as the time of closure is concerned, there is no doubt that closure should be carried out when one of the complications is present, with the exception of neonatal cholestasis, which resolves spontaneously. Symptomatic or complicated portosystemic shunts must be closed and this can be performed in one or two steps—by endovascular techniques or surgically—depending on the level of portal pressure during an occlusion test.23,24,26
Imaging studies sometimes fail to show a patent intrahepatic portal system, and there are reports of extreme hypoplasia of the portal trunk, even suggesting that the portal vein and its intrahepatic branches are lacking, a condition known in the literature as congenital absence of the portal vein or Abernethy malformation. In such cases, acute closure of the shunt may have devastating consequences, including acute portal hypertension, gastrointestinal bleeding, and severe damage to the gut. However, it is also suggested that the hypoplastic portal vein and/or branches are able to expand after closure of the shunt and that revascularization of these “disconnected” intrahepatic portal branches may occur.23,24,32,33
Indications for Shunt Closure
With the exception of neonatal cholestasis, which resolves spontaneously, closure of the shunt is mandatory whenever a complication is present. When no complication is detected, closure of the shunt can be delayed in cases of a small intrahepatic shunt found in early infancy because there is a reasonable hope of spontaneous shunt regression during the first year. In all other cases, closure of the shunt should be considered early, to prevent complications, for different reasons:
a. Hepatopulmonary syndromse and pulmonary hypertension may be present during the very first years of life.
b. The regression of pulmonary hypertension cannot be ascertained once irreversible lesions of the pulmonary arteries are present.
c. Chronic hyperammonemia and high blood levels of manganese have adverse effects on the developing brain.
d. The plasticity of the intrahepatic portal system may be better in younger children.
The plasticity of the intrahepatic portal system allows revascularization of the liver after shunt closure, even when no intrahepatic portal structures can be detected on imaging studies. This leaves little or no place for liver transplantation in the management of these children.23,24,26,29,32,34
Closure of congenital shunts in children should start by measuring portal pressure while transiently occluding the shunt during catheterization with an occlusion balloon (Fig. 60.3A–E). Depending on the shunt anatomy, whether attempting to treat the shunt with endovascular techniques or surgically, if the portal pressure is too high to allow shunt closure, then a two-step surgical treatment is indicated. In such cases, surgical banding of the shunt is recommended to maintain portal pressure around 20 mm Hg, followed by closure of the shunt a few months later, after checking by imaging studies that the intrahepatic portal branches have developed satisfactorily.23,24,26,35
Embolization of this kind of communications with different conventional embolic agents (i.e., stainless-steel and platinum coils, acrylate) has been described; however, this might be complicated or even impossible as a result of the diameter of the fistula.25,36,37 The anatomic characteristics of the vascular malformations (large communications) are amenable to be treated with the Amplatzer occlusion devices. Several reports exist in the literature of closure of these portosystemic shunts with Amplatzer plugs. When possible, the endovascular treatment with this device to occlude large communications is highly recommended as it can obviate complex surgeries. A patent ductus venosus can be successfully managed in this way, except when it is too wide or short to safely block the device in its lumen (Fig. 60.3A–E), in which case surgery must be undertaken.23,25,38–41
End-to-side shunts such as those joining a splenic or mesenteric vein to an affluent of the inferior vena cava such as a renal vein or an iliac vein can be easily closed percutaneously. On the other hand, when the shunt is side-to-side, surgery is certainly preferable, at least in young children, to the placement of a covered stent that would require lifelong anticoagulation treatment and would probably become inadequate in size with growth.23–26,33 Side-to-side shunts between the main portal vein and the inferior vena cava are frequently indications for surgery, usually performed in one step. However, in some cases, depending on the anatomy and location of the communication, the endovascular techniques may be useful for resolution of this type of shunt.25 On the contrary, end-to-side shunts between the main portal vein and the inferior vena cava usually require a two-step surgical procedure to avoid acute severe portal hypertension.23,24,33,34
A major complication related to vascular embolization in such cases is portal vein thrombosis, generally related to improper location or migration of the embolic agent. This serious complication is different from the portal vein thrombosis that may occur after occlusion of arterioportal fistulas, which will be discussed later. Neither anticoagulation nor antiaggregation is indicated after endovascular treatment of such portosystemic shunts.23–25 Follow-up control imaging is usually performed with Doppler US and demonstrates redirection of portal flow with no filling of the anomalous communication and subsequent regression of symptoms.
Interventional radiology techniques have an important role in the diagnosis and management of these congenital shunts involving the portal vein. The appropriate decision about the optimal treatment timing and approach, surgical or endovascular, should be taken based on a multidisciplinary management of such patients.
CONGENITAL ARTERIOPORTAL FISTULAS
Arterioportal fistulas (APFs) are a rare but treatable cause of portal hypertension associated with gastrointestinal hemorrhage in early childhood. APF may be primary (congenital) or secondary (acquired). Congenital APFs are defined as an intrahepatic communication between the hepatic artery and the portal venous system, without any connection with the systemic venous circulation or a clear secondary cause, and with onset before 18 years of age.17,22,42 Less than 10% of APFs involving the hepatic artery are congenital. The presence of multiple or diffuse feeding vessels to the fistula is a frequent and characteristic feature of congenital lesions.43 Among the most commonly reported causes of acquired APF are blunt abdominal trauma, surgical procedures such as the Kasai procedure and segmental liver transplantation, hepatic artery aneurysm, cirrhosis, biliary atresia, and tumors.42,44
Endovascular treatment of this entity aims at permanent occlusion of the fistula and restoration of normal liver hemodynamics. Transarterial embolization is a minimally invasive, effective, and safe therapy that allows resolution of the pathology with a shorter hospital stay and decreased morbidity. It is the treatment of choice among other options, such as surgical ligation of the involved hepatic artery, partial hepatectomy, portocaval shunt, and liver transplantation.43–45
In most patients with APF, clinical symptoms are related to the development of portal hypertension that appears in the first year of life. The severity of the symptoms is proportional to the size of the fistula and the flow within it. The fistula progressively decreases and then reverses the normal antegrade flow in the portal vein while the hepatic artery flow, and diameter, increase due to the fact that arterial flow directly communicates with a territory of low pressure.42,43,46
The hepatic artery is usually enlarged in all patients. This hemodynamic alteration also produces a decrease in blood flow distal to the fistula in the abdominal aorta beyond the celiac trunk (aortic tapering). Some authors have suggested that this steal effect compromises perfusion of the superior and inferior mesenteric circulations, producing bowel hypoxia, thus worsening intestinal edema.43
In a review of the largest series, the most commonly found clinical features were upper gastrointestinal bleeding, failure to thrive, chronic diarrhea with steatorrhea, and protein-losing enteropathy. In these same series, the most important findings on physical examination at diagnosis were splenomegaly, hepatomegaly, ascites, and edema. The variable clinical presentation might be related to variations in the angioarchitecture, size of the fistula, flow within the malformation, and, consequently, the degree of portal hypertension.43,44
In contrast to patients with arteriovenous fistulas in other locations, congestive heart failure is rare in patients with APF. This seems to be related to the protective effect of the hepatic sinusoids interposed between the fistula and the right heart cavities. The typical symptoms of chronic malabsorption, diarrhea, and abdominal pain are likely associated with mesenteric vascular congestion. Protein-losing enteropathy, steatorrhea, and malabsorption contribute to patient malnutrition and may be due to edema and dilation of the lymphatic system and pancreatic hypoperfusion.46
No cases of spontaneous closure of an APF have been reported; therefore, intervention is warranted in all symptomatic patients. Transcatheter embolization of the fistula by means of endovascular techniques has been proposed as the first therapeutic option with a high success rate in patients with unilateral lesions or in those with few feeding arteries. Congenital APF may have a single feeding artery or multiple or diffuse feeding vessels (Fig. 60.4A–F).43–45,47The presence of the former characteristic thus increases the chance of successful occlusion of an APF via endovascular procedures. However, in many series, more than one intervention has been necessary to achieve occlusion. Occasionally, after an apparently technically successful procedure, new collateral feeders not seen during the first intervention might appear and act as persistent anomalous arterial to portal connections keeping the fistula patent. In these patients, nonsurgical treatment is preferable because the success rate is not higher with surgery.43,44 A differential diagnosis might be established with hepatic arteriovenous malformations, a fast-flow vascular malformation. This entity may present a single or several arterial feeders and show a characteristic nidus not seen in APF. Hepatic arteriovenous malformations may be focal or diffuse, and embolization is usually, but not always, an option due to the risk of hepatic ischemia and necrosis.17 Metallic coils are the recommended agents for permanent arterial embolization of APF. The use of Amplatzer plugs has also been reported for this indication. Acrylic or polyvinyl alcohol microspheres have also been described in case small feeders are present. Microspheres larger than 500 µm should be used based on that this caliber is large enough to occlude the arterioportal connection, avoiding the risks of migration to the portal system and hepatic ischemia. There are no reports about the use of liquid embolic agents, such as Onyx (Covidien, Irvine, California) and/or acrylate for APF embolization.17,43–45,47
Portal vein thrombosis is a major complication during the embolization procedure and in the early posttreatment period. This complication may be related to nontarget embolization, directly locating the embolic agent at the wrong site, or the material moving from the original site to the portal system because of high flow in the fistula. Another reason for this severe complication is related to sudden flow changes after embolization of the fistula. Therefore, immediately after the procedure, heparinization is recommended. In our experience, after the intervention, for thrombosis prophylaxis, subcutaneous low-molecular-weight heparin was administered for 3 days once the end point (anti-Xa = 0.5 to 1 IU/mL) had been achieved. The Doppler US follow-up in the early posttreatment period is crucial to detect this vascular complication.17,43,45
TIPS AND TRICKS
• Attention should always be drawn to the maintenance of patient body temperature control, fluid balance, and total dose of iodine contrast used.
• Appropriate radiation safety techniques when performing pediatric interventional procedures must be considered, following the ALARA principle.
• For transarterial embolization procedures, general anesthesia is preferred.
• US guidance for the arterial puncture increases the chances of a successful access in small patients.
• Vascular sheaths should always be placed at the access site.
• Microcatheters and coaxial systems are ideal for embolization in pediatrics.
• Percutaneous sclerotherapy is the technique used to treat low-flow vascular malformations. On the other hand, high-flow lesions are treated with superselective transarterial, direct, and transvenous access.
• The diagnosis of the hemangiomas is mainly clinical. Angiography and further endovascular techniques are only reserved for therapeutic purposes when medical treatment fails.
• The presence of an anomalous portosystemic shunt should be considered in patients with central, not cardiogenic, cyanosis and normal thoracic imaging.
• In pediatric interventional radiology, optimal clinical results are obtained as a result of a multidisciplinary discussion, management, and patients’ follow-up.
• The accepted dose of nonionic contrast should not exceed 5 mL/kg. Usually, we use undiluted contrast material; however, in patients weighing less than 10 kg, we often dilute 1:1 with saline.
• Radiation safety tricks may include reduced-dose protocols, the last-image-hold feature, pulsed fluoroscopy, tight collimation, and minimizing the use of magnification.
• Arterial puncture in children weighing less than 10 kg should not be done with anything larger than a 20-gauge needle.
• Arterial spasm, frequent in this population, may be treated with intra-arterial injection of papaverine (1 mg/kg) or nitroglycerin (2–3 units/kg).
• Considering patients’ weight and the use of contrast media for embolization procedures, in some cases, a first procedure will be focused on the embolization of main tumor-feeding vessels. If symptoms persist, this procedure can be repeated.
• Embolization of hemangiomas imposes a precise, distal, intratumoral occlusion. Particulated agents (gelfoam and ≥300 µm microparticles) are usually employed for this purpose.
• Regarding treatment of hepatic vascular tumors, staged procedures are prudent to minimize the appearance of complications such as hepatic ischemia or necrosis.
• Treatment of portosystemic shunts should be considered when a complication is present (with the exception of neonatal cholestasis, which may resolve spontaneously).
• If treatment is needed, an occlusion test for portal pressure assessment should be performed. Then, endovascular or surgical treatment can be carried out, in one or two steps, depending on the test result.
• Metallic coils are the recommended agents for permanent arterial embolization of congenital APFs. Vascular plugs may also be useful for this purpose.
• Immediately after the embolization of congenital APF, heparinization is recommended to avoid portal vein thrombosis.
Embolotherapy has demonstrated its effectiveness with current available materials and equipment in a wide range of indications in pediatric patients. These techniques should be performed by specifically trained physicians, with knowledge of the pediatric pathology and experience in the management of the specific considerations related to this age group. A multidisciplinary discussion, management, and follow-up are essential for the continuous evolution of endovascular techniques in pediatrics.
1. Dubois J, Garel L, Culham G. Pediatric Interventional angiography. In: Baum S, Pentecost MJ, eds. Abram’s Angiography Interventional Radiology. Philadelphia, PA: Lippincott Williams & Wilkins; 2006:1046–1067.
2. Dubois J, Garel L. Embolotherapy in pediatrics. In: Golzarian J, Sun S, Sharafuddin MJ, eds. Vascular Embolotherapy. Berlin, Germany: Springer-Verlag; 2006:297–320.
3. Kaye RD, Sane SS, Towbin RB. Pediatric intervention: an update—part I. J Vasc Interv Radiol. 2000;11:683–697.
4. Kaye RD, Sane SS, Towbin RB. Pediatric intervention: an update—part II. J Vasc Interv Radiol. 2000;11:807–822.
5. Donaldson JS. Pediatric vascular procedures: arterial and venous. In: Slovis T, ed. Caffey’s Pediatric Diagnostic Imaging. Philadelphia, PA: Mosby Elsevier; 2008:3100–3116.
6. Sidhu M, Strauss KJ, Connolly MB, et al. Radiation safety in pediatric interventional radiology. TechVasc Interv Radiol. 2010;13:158–166.
7. Connolly B, Racadio J, Towbin R. Practice of ALARA in the pediatric interventional suite. Pediatr Radiol. 2006;36:163–167.
8. Jaques PF, Mauro MA, Keefe B. US guidance for vascular access. Technical note. J Vasc Interv Radiol. 1992;3:427–430.
9. Legiehn GM, Heran MK. A step-by-step practical approach to imaging diagnosis and interventional radiologic therapy in vascular malformations. Semin Intervent Radiol. 2010;27:209–231.
10. Dubois J, Alison M. Vascular anomalies: what a radiologist needs to know. Pediatr Radiol. 2010;40:895–905.
11. Ernemann U, Kramer U, Miller S, et al. Current concepts in the classification, diagnosis and treatment of vascular anomalies. Eur J Radiol. 2010;75:2–11.
12. Jackson IT, Carreno R, Potparic Z, et al. Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment. Plast Reconstr Surg. 1993;91:1216–1230.
13. Legiehn GM, Heran MK. Classification, diagnosis, and interventional radiologic management of vascular malformations. Orthop Clin North Am. 2006;37:435–474.
14. Chen TS, MD, Eichenfield LF, Friedlander SF. Infantile hemangiomas: an update on pathogenesis and therapy. Pediatrics. 2013;131:99–108.
15. Sans V, de la Roque ED, Berge J, et al. Propranolol for severe infantile hemangiomas: follow-up report. Pediatrics. 2009;124:423–431.
16. Leaute-Labreze C, Dumas de la Roque E, Hubiche T, et al. Propranolol for severe hemangiomas of infancy. N Engl J Med. 2008;358:2649–2651.
17. Burrows PE, Dubois J, Kassarjian A. Pediatric hepatic vascular anomalies. Pediatr Radiol. 2001;31:533–545.
18. Garcia-Monaco R, Giachetti A, Peralta O, et al. Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon: successful treatment with embolization and vincristine in two newborns. J Vasc Interv Radiol. 2012;23:417–422.
19. Kassarjian A, Dubois J, Burrows PE. Angiographic classification of hepatic hemangiomas in infants. Radiology. 2002;222:693–698.
20. Christison-Lagay ER, Burrows PE, Alomari A, et al. Hepatic hemangiomas: subtype classification and development of a clinical practice algorithm and registry. J Pediatr Surg. 2007;42:62–67.
21. Alonso-Gamarra E, Parrón M, Pérez A, et al. Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review. Radiographics. 2011;31:707–722.
22. Gallego C, Miralles M, Marín C, et al. Congenital hepatic shunts. Radiographics. 2004;24:755–772.
23. Bernard O, Franchi-Abella S, Branchereau S, et al. Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis. 2012;32:273–287.
24. Franchi-Abella S, Branchereau S, Lambert V, et al. Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr. 2010;51:322–330.
25. Alonso J, Sierre S, Lipsich J, et al. Endovascular treatment of congenital portal vein fistulas with the Amplatzer occlusion device. J Vasc Interv Radiol. 2004;15:989–993.
26. Stringer MD. The clinical anatomy of congenital portosystemic venous shunts. Clin Anat. 2008;21:147–157.
27. Eroglu Y, Donaldson J, Sorensen LG, et al. Improved neurocognitive function after radiologic closure of congenital portosystemic shunts. J Pediatr Gastroenterol Nutr. 2004;39:410–417.
28. Kim T, Murakami T, Sugihara E, et al. Hepatic nodular lesions associated with abnormal development of the portal vein. AJR Am J Roentgenol. 2004;183:1333–1338.
29. Morikawa N, Honna T, Kuroda T, et al. Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation. J Pediatr Surg. 2008;43:e35–e38.
30. Howard ER, Davenport M. Congenital extrahepatic portocaval shunts—the Abernethy malformation. J Pediatr Surg. 1997;32:494–497.
31. Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts. Pediatr Radiol. 2003;33:614–620.
32. Barsky MF, Rankin RN, Wall WJ, et al. Patent ductus venosus: problems in assessment and management. Can J Surg. 1989;32:271–275.
33. Takehara Y, Mori K, Edagawa T, et al. Presumed hypoplastic intrahepatic portal system due to patent ductus venosus: importance of direct occlusion test of ductus venosus under open laparotomy. Pediatr Int. 2004;46:484–486.
34. Yoshimoto Y, Shimizu R, Saeki T, et al. Patent ductus venosus in children: a case report and review of the literature. J Pediatr Surg. 2004;39:E1–E5.
35. Yagi H, Takada Y, Fujimoto Y, et al. Successful surgical ligation under intraoperative portal vein pressure monitoring of a large portosystemic shunt presenting as an intrapulmonary shunt: report of a case. Surg Today. 2004;34:1049–1052.
36. Gupta V, Kalra N, Vyas S, et al. Embolization of congenital intrahepatic porto-systemic shunt by n-butyl cyanoacrylate. Indian J Pediatr. 2009;76:1059–1060.
37. Yamagami T, Yoshimatsu R, Matsumoto T, et al. Successful embolization using interlocking detachable coils for a congenital extrahepatic portosystemic venous shunt in a child. J Pediatr Surg. 2007;42:1949–1952.
38. Gillespie MJ, Golden A, Sivarajan VB, et al. Transcatheter closure of patent ductus venosus with the Amplatzer vascular plug in twin brothers. Pediatr Cardiol. 2006;27:142–145.
39. Chiu SN, Chien YH, Wu MH, et al. Transcatheter closure of portal-systemic shunt combining congenital double extrahepatic inferior vena cava with vascular plug. J Pediatr. 2008;153:723.
40. Cho YK, Chang NK, Ma JS. Successful transcatheter closure of a large patent ductus venosus with the Amplatzer vascular plug II. Pediatr Cardiol. 2009;30:540–542.
41. Evans WN, Galindo A, Acherman RJ, et al. Congenital portosystemic shunts and AMPLATZER vascular plug occlusion in newborns. Pediatr Cardiol. 2009;30:1083–1088.
42. Norton SP, Jacobson K, Moroz SP, et al. The congenital intrahepatic arterioportal fistula syndrome: elucidation and proposed classification. J Pediatr Gastroenterol Nutr. 2006;43:248–255.
43. Teplisky D, Tincani EU, Lipsich J, et al. Congenital arterioportal fistulas: radiological treatment and color Doppler US follow-up. Pediatr Radiol. 2012;42:1326–1332.
44. Vauthey JN, Tomczak RJ, Helmberger T, et al. The arterioportal fistula syndrome: clinicopathologic features, diagnosis, and therapy. Gastroenterology. 1997;113:1390–1401.
45. Kumar N, de Goyet JV, Sharif K, et al. Congenital, solitary, large, intrahepatic arterioportal fistula in a child: management and review of the literature. Pediatr Radiol. 2003;33:20–23.
46. Iñon AE, D’Agostino D. Portal hypertension secondary to congenital arterioportal fistula. J Pediatr Gastroenterol Nutr. 1987;6:471–473.
47. Raghuram L, Korah IP, Jaya V, et al. Coil embolization of a solitary congenital intrahepatic hepatoportal fistula. Abdom Imaging. 2001;26:194–196.