eMedicine Specialties > Pediatrics: Surgery > Vascular Surgery
Congenital Arterial and Venous Anomalies, Surgical Treatment
Updated: Apr 10, 2009
Introduction
Arterial and venous malformations due to congenital abnormalities are encountered infrequently in everyday practice. These malformations represent a heterogeneous group of isolated or multiple congenital abnormalities sometimes associated with complex congenital syndromes. Relatively few basic experiments have addressed the problem of congenital arteriovenous malformations (AVMs). Correct recognition and classification of these rare abnormalities may sometimes be difficult. No systematic classification of arterial and/or venous vascular malformations due to congenital abnormalities is currently available. For the student and the diagnostician, a classification based on the etiology of communication is helpful.
The congenital AVMs are primarily divided into 4 categories: (1) hemangiomas, (2) predominantly venous malformations, (3) multifistulous AVMs, and (4) single fistula. Hemangiomas are either neoplastic or nonneoplastic. Predominantly venous malformations are further divided as those with demonstrable shunting (Parkes-Weber Syndrome) and those with no shunting, which are usually cavernous lesions (Klippel-Trenaunay syndrome). Multifistulous AVMs can be macrofistulous communications, which are demonstrable on angiography, or microfistulous communications, which are not demonstrable on angiography. A single fistula is a patent ductus, septal defect, peripheral arteriovenous fistulas (AVFs), or another anomaly of the great vessels.
Vascular malformations, as opposed to hemangiomas, are not neoplastic. These lesions have no endothelial proliferation and show no growth on tissue cultures.
In general, congenital AVMs, like other congenital anomalies, remain incompletely understood in terms of pathogenesis. From a clinical standpoint, these are malformations and not neoplasms. As generally isolated anomalies, they are rarely genetically transmitted, they are often stable, and they require no treatment.
Congenital vascular malformations may involve arterial, venous, and lymphatic structures. They can appear in a variety of forms and present many diagnostic and therapeutic challenges. Two-thirds of all congenital vascular malformations are predominantly venous. Most of the venous malformations are asymptomatic and should be managed conservatively. However, the clinical presentation of patients with venous malformations associated with lymphatic anomalies is variable, and treatment may be challenging.
The early work of Woodlard on the embryology of the vascular system shed light on our understanding of these congenital anomalies. In the early stages of development, the vascular system consists of interlacing blood spaces in the primitive mesenchyma. Because of a genetic factor in the capillary endothelium, these interlacing networks differentiate into arteries and veins. Focal persistence of the primitive vascular elements constitutes the congenital vascular malformations.
According to Woodlard, the 3 stages of development are (1) the stage of undifferentiated capillary network (Hemangiomas result from arrest of development during this stage.), (2) the retiform stage (AVM results from arrest of development during this stage.), and (3) the vascular maturation stage (A port-wine stain is an example.).
Frequency
The overall incidence of congenital vascular malformations in the general population is 1.5%. Approximately two thirds are malformations of venous predominance. Abnormalities of the deep venous trunks have been observed in association with large superficial compensatory varices in these types of malformations. Knowledge of the integrity of the deep venous system is important for management of these malformations because excision of the enlarged superficial veins may be deleterious if aplasia or hypoplasia of the deep venous trunks exists.
Patients with congenital vascular malformations were evaluated at Children's Hospital of Mexico City (1963-1983, 223 children) and the Walter Reed Army and National Naval Medical Centers (1984-1998, 169 children). Of the 392 patients, 257 (65.6%) had malformations of venous predominance. Prevalences of phlebectasia, aplasia or hypoplasia of venous trunks, aneurysms, and avalvulia were recorded.
Pathophysiology
Pathologists differentiate vascular malformations into 4 categories, as follows:
- Venous angiomas, which are characterized by an extensive network of veins separated by normal parenchyma
- Telangiectasis lesions (ie, capillary angiomas), which generally are benign lesions of the brain stem
- Cavernous angiomas, which are typically developmental malformations in the brain substance that can be demonstrated by means of brain CT or MRI
- AVMs, which are usually larger than the other types of lesions but can vary in size and consist of a feeding artery or arteries, a nidus, and draining veins
In terms of pathophysiology, congenital AVMs are always due to 3 separate and distinct phenomena: (1) the local effect of the fistula (warmth, aneurysmal dilatation of the veins), (2) central and distal effects (distal ischemia depending on the amount of shunting), and (3) high-output cardiac failure. The frequency and severity of the symptoms and signs depends on the magnitude and location of the fistulas.
Congenital AVFs can occur almost anywhere in the body. They most commonly involve the extremities, lower more than upper. Among fistulas of the extremities, those involving the femoral vessels are most common. Congenital AVFs have also been described in the head and neck, where they contribute to major clinical deformities. Patients with an AVM of the brain have a dramatic presentation. Pelvic AVMs are usually extensive and manifest with vaginal bleeding or compressive symptoms on other pelvic organs. Congenital visceral fistulas are seen in lung, kidney, and alimentary tract. Alimentary-tract AVMs are usually a part of congenital telangiectatic syndromes. Congenital AVMs can sometimes result in portal hypertension.
Presentation
Patients are usually asymptomatic. Bruits may be audible.
Other congenital anomalies can coexist. Some coexisting anomalies occur in response to abnormal local blood flow (eg, overgrowth, undergrowth, and focal gigantism). The observed association of hyperhidrosis and café au lait spots suggests connections between abnormal vascular and neurologic anomalies.
No sex predominance is observed except in infantile hemangiomas.
Gradual growth occurs in proportion to the growth of the individual.
Clinical detection is variable. The lesion sometimes manifests as a local skin discoloration, a local bruit or thrill, or a local pulsatile mass. Detection can be based on the effects on adjacent structures or on secondary complications, such as hemorrhage, venous ulceration, or ischemia of the regional tissues due to steal phenomenon. Local trauma may direct attention to the congenital malformation. Some believe that trauma activates congenital AVMs to open new channels as they enlarge or become symptomatic. The enlargement is due to enlargement of vascular channels and not cellular proliferation. High-output cardiac failure can be an initial manifestation; this usually occurs in lesions in infancy and extremely large pelvic or intra-abdominal lesions in adults. Vague local pain is sometimes the presenting symptom. Local varicosities can also be seen in some patients. Local gigantism is another clinical manifestation.
Vascular malformations can occur anywhere in the body, though certain anatomic sites, such as the pelvis, extremities, and intracranial circulation, seem to be most commonly affected. Pelvic lesions can manifest in multiple ways:
- Asymptomatic
- Pelvic pain
- Pain referred to leg
- Sexual dysfunction
- High output heart failure
- Pressure effects on pelvic organs
- Hemorrhage
Bleeding from cranial lesions could cause a subarachnoid, intracerebral, or intraventricular hemorrhage.
AVMs usually appear with an intracranial bleed, but patients may have a variety of symptoms and signs, including headache, cranial bruit, convulsive seizure, mental deterioration, or hemispheric neurologic deficit.
If large enough, these malformations can be diagnosed by using CT and MRI of the brain. The final diagnosis is made by means of 4-vessel cerebral angiography, with which all the feeding arteries, nidus, and venous drainage can be demonstrated.
AVMs tend to bleed earlier in life than aneurysms; the peak incidence is in individuals aged 30-40 years. Available data suggest that, subsequent to diagnosis, the annual cumulative risk of rebleeding in intraparenchymal AVMs is 3-4%. The risk might be slightly increased in the first year after an initial hemorrhage, but it appears to remain relatively constant from that point for the remainder of the patient's life.
Indications
Techniques for management of AVMs include microsurgical excision, embolization with glue or thrombogenic particles, and focused radiation. Depending on the clinical condition of the patient and the characteristics of the AVM (size, location, arterial supply, venous drainage), a decision is made regarding which of the 3 modalities should be used.
Relevant Anatomy
Venous angiomas are characterized by an extensive network of veins separated by normal parenchyma. These lesions seldom bleed or give rise to clinical symptoms.
Telangiectasis lesions (ie, capillary angiomas) are generally benign lesions of the brainstem.
Cavernous angiomas are typically developmental malformations in the brain substance that can be demonstrated by means of brain CT or MRI. They may clinically manifest as growing masses, intracerebral hemorrhage, or intractable seizures. Symptomatic lesions that are accessible are usually removed surgically.
AVMs are usually larger than the other types of lesions, but they can vary in size. AVMs consist of a feeding artery or arteries, a nidus, and draining veins. The superficial portion of a malformation may cover part of the cerebral surface, but the lesion frequently extends like a cone down to the ventricular surface.
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Further Reading
Keywords
congenital arterial anomaly, venous anomaly, congenital arterial malformation, congenital venous malformation, congenital vascular malformation, hemangioma, arteriovenous malformation, AVM, symptomatic vascular malformation, SVM
