Vascular Malformations In Children Research Articles

Volvulus of a jejunal lymphatic vascular malformation presenting with bilious vomiting and the radiological appearances of malrotation

Bile-stained vomiting in infancy is abnormal. Malrotation with small bowel volvulus is probably the most worrying of possible causes because if untreated for too long, it can result in massive bowel necrosis. As a consequence, if examination or plain radiography does not suggest another cause, an upper gastrointestinal (GI) series is often performed to exclude malrotation. If the examination is normal however and symptoms settle, should investigation stop, or how far should it be pursued? Small bowel lymphatic vascular malformations in children are extremely rare but carry a risk of obstruction, volvulus, and loss of small bowel. We present a case of a 5-year-old boy referred to our unit with bilious vomiting and the radiological findings of malrotation on upper GI series. Interestingly, a previous upper GI series undertaken during the first year of life for intermittent bilious vomiting was normal. Operative findings were of a 16-cm cystic lymphatic vascular malformation of the jejunal wall causing a 360 degrees volvulus. The mesentery was derotated, and the cyst removed with the attached section of jejunum. This case highlights the need to reinvestigate patients with bilious vomiting despite a previous normal upper GI series. The case raises the issue of whether bilious vomiting, with a normal upper GI series, should be investigated further with an abdominal ultrasound scan to identify more unusual causes such as jejunal lymphatic vascular malformations.

Cavernomes du système nerveux central chez l'enfant

Cavernomas represent 1.7 to 18% of all vascular malformations in children and 25% are observed in children under 18 years of age. Cases observed in neonates and infants have been published, but the mean age varies from 9.1 to 10.2 years. There is no predominance between boys and girls. Clinical presentation In children, hemorrhage is a common manifestation with an incidence varying from 27.3 to 78% versus 8 to 37% in adult patients. Isolated headaches occur in 2.8% of patients and elevated ICP is observed in 20.1%. Epilepsy is reported in 16 to 60% of children, depending on the series. Neurological deficits are observed in 22.7% of patients and are more severe for deep-seated and brainstem cavernoma. About 14.2% of the cases are discovered fortuitously in asymptomatic patients. Spinal cord deficits are observed in 5% of the cases. Location Using data in the literature plus our personal series of 57 cases, 79.4% of lesions are in the supratentorial compartment and 20.6% in the posterior fossa, the majority located in the brainstem, most of them in the pons. Spinal cord cavernomas represent 5% and multiples cavernomas (12.6%) of the reviewed cases. Management Appropriate management of cavernomas has long been a subject of much debate. Today, a consensus has been reached to favor medical management of asymptomatic and non hemorrhagic lesions and surgical management of symptomatic and/or hemorrhagic cavernomas whatever the localization. Progress in neuroimaging, surgical mapping, intraoperative monitoring and microsurgical techniques has greatly contributed to improved approach to those lesions. Results Results obtained in 217 cases were reviewed. Near 70% of the children are neurologically intact, 19.3% are improved or stable, 2.7% worsened and 1.13% died. Results for epilepsy are very encouraging, surgery is efficient in almost all the children except for temporal lobe cavernomas where invasive presurgical evaluation is recommended. Deep-seated and brainstem cavernomas can safely be removed in most of the cases. Only two children died from recurrent hemorrhage due to residual lesions.

Incidence of vascular malformations in spontaneous intra-cerebral haemorrhage in children

To assess the incidence of vascular malformations in children presenting with non-traumatic intra-cerebral haemorrhage and outline the need for radiological investigations. Between 1993 and 2002, 26 children presented with spontaneous intra-cerebral haemorrhage at a mean age of 26 months (range 1-192 months). Twenty-two children had, as first treatment, surgical removal of the haematoma and any malformation found. One patient with no evidence of vascular malformation had conservative treatment, two had embolisation and one had stereotactic radiosurgery of arteriovenous malformations (AVMs). The diagnosis of vascular malformation was confirmed histologically and/or radiologically in 16 (61%) patients. Of these 16 patients, 7 were AVMs, 1 thrombosed middle cerebral artery (MCA) aneurysm, 1 cavernous angioma, 6 aggregates of abnormal vessels, 1 vein of Galen aneurysm. Pre-operatively, 12 patients had magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) and 7 were positive for vascular malformation (1 false positive) with no false negatives. Digital subtraction angiography (DSA) was performed pre-operatively in 7 patients, 4 were positive with no false negatives. There were no re-bleeds at the follow-up period. Five (19%) patients died from the haemorrhage, 7 (27%) had severe neurological deficit and 14 (54%) had no neurological deficit. Residual malformation after surgery requiring additional treatment was found in 3 (16%) patients. All patients with confirmed malformations were followed-up with DSA. MRI/MRA has high sensitivity and specificity in identifying vascular malformations in children presenting acutely with spontaneous intra-cerebral haemorrhage. This may prove useful when pre-operative DSA is not promptly available. After acute clot evacuation, there is high incidence of residual malformation and such patients should be followed-up with DSA.

Classification and imaging of vascular malformations in children.

Accurate diagnosis and classification of haemangiomas and vascular malformations is essential for prediction of outcome and rational therapeutic intervention. The purpose of this review is to demonstrate how radiological techniques, including projectional radiography, ultrasound and colour flow imaging, magnetic resonance imaging, and conventional venography and arteriography can aid in the diagnosis and classification of vascular malformations in children, and to guide the radiologist in the use of modern nomenclature.

Interstitial laser therapy for vascular malformations in children

Interstitial laser therapy for vascular malformations in children

Intraoperative Angiography in the Management of Pediatric Vascular Disorders

We reviewed our experience with intraoperative angiography in the management of 7 cerebral aneurysms and 15 arteriovenous malformations (AVMs) in children over the past 5 years. The patients ranged from 4 months to 18 years of age with a mean age of 9 years. In 4 of the 22 cases, angiography provided information that led to changes during surgery. In 3 cases, the intraoperative angiogram revealed residual AVMs that were then completely resected. In 1 patient with an anterior circulation aneurysm, intraoperative studies revealed findings which resulted in clip repositioning. Intaoperative angiography added a mean of 43 (+/-19) min to the operative time, with a mode of 40 minutes. There was 1 complication, a groin hematoma that readily resolved. Intraoperative angiography prolongs to operative course; however, it is a useful adjunct to the management of vascular disorders in children. We now routinely use and recommend intraoperative angiography to assist with the surgical management of vascular malformations in children.

Imaging of hemangiomas and vascular malformations in children

Imaging of hemangiomas and vascular malformations in children

MR characteristics of low-flow facial vascular malformations in children and young adults

The purpose of this report is to describe the magnetic resonance (MR) imaging characteristics of low-flow vascular malformations of the face. We studied six patients ranging in age from 1 month to 20 years old, with surgically proven or clinically certain low-flow vascular malformations of the face. T1-weighted MR images of five lesions showed homogeneous intermediate to low signal intensity, similar to the signal intensity of muscle. T1-weighted images of one lesion showed mild high signal intensity and image of another demonstrated fatty infiltration. After contrast administration, images of four lesions showed moderate to marked enhancement, three diffused and one patchy. On T2-weighted sequences, five lesions were heterogeneously of high signal and one of low signal intensity. Three lesions exhibited low-signal septa, and two revealed low-signal vascular flow voids on both types of sequences. Satellite nodules were present in three instances. Low-flow vascular malformations involving the face in children and young adults have a heterogeneous and nonspecific appearance on MR images. They should be considered in the differential diagnosis of all facial masses presenting in children and young adults. Enhancement with contrast material is essential to localize the mass.

Intravascular Treatment of Vascular Malformations in Children

Intravascular Treatment of Vascular Malformations in Children

Facial vascular malformations in children. Conventional and digital, diagnostic and therapeutic angiography.

The authors present their experience with conventional and digital angiography of vascular malformations of the head and neck in children. 22 hemangioendotheliomas, 8 venous angiomas, and 3 arteriovenous fistula were studied. 22 patients were embolised. DSA offers many advantages during the diagnostic as well during the therapeutic phase of angiography. Embolization appears to have a major role in treatment of such vascular malformations.

The association of extracranial and intracranial vascular malformations in children.

This review is an attempt to relate the types of angiomas and other cutaneous vascular lesions appearing in the face with abnormalities in the formation of cerebral vessels including arteries at their origin, veins and venous sinuses, and with other less constant alterations that affect other organs, particularly the heart and cerebellum. After angiographic study of a series of patients exhibiting diverse types of vascular nevi (capillary and cavernous hemangiomas, nevus flammeus, and lymphangiomas), a correlation was established for each type of dermal nevus with a corresponding group of intracranial vascular anomalies. An explanation of these malformations based on the embryological development of the principal cerebral vessels during the 7th and 8th weeks of gestation is offered. Involvement of the facial and scalp dermatome innervated by the first sensory trigeminal branch correlates most consistently with the presence of abnormal cerebral vessels. The capillary hemangioma occurs preferentially in girls and frequently is associated with developmental anomalies of major cerebral arteries characterised by the absence of some adult vessels and the persistence of others that should have disappeared during embryogenesis such as the fetal trigeminal artery. The complete angiographic exploration of the cerebral vessels from their origin in the aortic arch to the phase of intracranial venous evacuation may reveal unexpected important findings, and is recommended for children with major facial vascular nevi, even if they are neurologically asymptomatic.

Intracranial vascular malformations in children: computed tomographic and angiographic evaluation.

Thirty-nine cases of intracranial vascular malformations in children were reviewed. All patients underwent preoperative computed tomography (CT) and 35 had complete preoperative angiographic work-ups. The CT features of intracranial vascular malformations in children are described. Among the 39 patients, there were 30 parenchymal arteriovenous malformations, four dural arteriovenous malformations, three cavernous angiomas, and two venous angiomas. Combined CT and angiography allowed a highly specific diagnosis in 77% of the cases.

Congenital intracranial vascular malformations in children.

The clinical, laboratory, and other features of 16 children with congenital intracranial vascular malformations seen over a period of 15 years are presented. 13 patients had an arteriovenous (AV) malformation, 2 patients an aneurysm of the vein of Galen, and 1 a congenital internal carotid/internal jugular fistula. Based on onset of symptoms the patients ranged in age from 3 months to 16 years with an average age of 7½ years; however, 5 patients had their first clinical manifestation before 6 years of age. Unfortunately, definitive diagnosis could not be made until an average of 15½ years. The most important clinical manifestations were focal or generalized seizures and spontaneous intracranial bleeding, each occurring in 6 patients. Of 2 patients who presented with hydrocephalus, 1 had had an unexplained episode of cardiac failure during infancy. Inequality in the size of the legs, and periodic headaches were the initial manifestations in 2 other patients. Analysis of this series of patients with intracranial AV malformations suggests the following diagnostic recommendations: careful auscultation of the skull for bruits should be performed in all infants and children with cardiac failure of unknown aetiology; patients with focal seizures refractory to anticonvulsant therapy should be re-examined at frequent intervals to detect focal neurological deficit which otherwise may go unnoticed; and, finally, patients with a seizure disorder who develop focal signs of neurological deficit deserve a complete diagnostic investigation, including contrast studies.