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The purpose of our study was to examine the coagulation status in patients with vascular anomalies who had undergone sclerotherapy or embolization.Ours was a prospective pilot study of 29 patients who had undergone sclerotherapy or embolization of large vascular anomalies. Fibrinogen, platelet, and d-dimer levels and prothrombin time were obtained before, immediately after, and on the day after the procedure.Five patients with venous malformations had positive d-dimer levels before the procedure. A subgroup analysis revealed a relationship between the type of agent used and the change in coagulation status. Specifically, a positive relationship was found between the use of dehydrated alcohol or sodium tetradecyl sulfate and a disruption in coagulation profiles as evidenced by a decrease in platelets and fibrinogen, an increase in prothrombin time, and a conversion from negative to positive d-dimers. In contrast, sclerotherapy or embolization with cyanoacrylic, polyvinyl alcohol foam particles, or platinum microcoils was not associated with coagulation disturbances.The coagulation disturbances that occur in response to dehydrated alcohol or sodium tetradecyl sulfate sclerotherapy or embolization could compromise the patient's clotting ability. Patients who receive dehydrated alcohol or sodium tetradecyl sulfate during a preoperative sclerotherapy or embolization may experience coagulation disturbances that could increase the risk of bleeding, thrombosis, or hematoma. This patient population may benefit from the use of glue, foam, or coils as a substitute for dehydrated alcohol or sodium tetradecyl sulfate.

This study was designed to evaluate safety, efficacy, and success of adding IV midazolam to an established IV pentobarbital protocol for pediatric sedation for radiologic imaging. Outcomes included sedation and discharge times as well as adverse eventsThis prospective study compared two different sedation protocols developed by the radiology sedation committee and approved by the hospital sedation committee at our institution. Patients in the pentobarbital group received IV pentobarbital alone, and patients in the pentobarbital--midazolam group received a combination of IV pentobarbital and midazolam. A total of 1070 infants and children were enrolled, and sedation data were entered into a computer database and reviewed at bimonthly radiology sedation committee meetings for safety, efficacy, efficiency, failed sedations, and adverse outcomes.Mean age distribution, sex, American Society of Anesthesiologists physical status classification, fasting status, weight, and types of examinations were similarly distributed between the two study groups. Analysis of variance indicated longer times were required to sedate and to discharge patients who had received pentobarbital--midazolam (p0.001 for both times), even after adjusting for differences in the patients' ages and weights. The pentobarbital--midazolam group required more time to be successfully sedated and more time to discharge from the recovery room. The rates of adverse events and failed sedations were similar for both groups.Midazolam does not have a beneficial effect on pentobarbital sedation and has no effect on the rate of adverse events. The prolonged time needed both to sedate and to discharge (timed from the initial dose of sedation) pediatric patients who have received midazolam should discourage physicians from combining it with pentobarbital for pediatric sedation.

A 13-year-old girl who recently developed hypertension was diagnosed to have an occluded right renal branch artery and was treated successfully with percutaneous transluminal angioplasty (PTA). To our knowledge, PTA has not been reported as a treatment for totally occluded renal branch arteries, and there is no data available regarding the success rate and possible complications.

Hepatic haemangiomas in infants are rare. An infant with both a hepatic haemangioma and a severe hypothyroid condition, unresponsive to conventional thyroxine therapy, will be described. This case presented here is the perioperative management of a critically ill infant who had myocardial depression secondary to hypothyroidism and a hepatic haemangioma that required embolization. To our knowledge, this is the first published report describing intravenous triiodothyronine as a therapeutic modality to stabilize a hypothyroid infant prior to undergoing a general anaesthetic.

To evaluate the serum ethanol level in children and adults after ethanol embolization or sclerotherapy for vascular anomalies.Serum ethanol level was evaluated in 71 patients with vascular anomalies who underwent ethanol embolization, sclerotherapy, or both. Blood used to determine serum ethanol level was drawn at the end of each procedure.The relationship between serum ethanol level and amount of ethanol administered was statistically significant. Results of stepwise linear regression analysis showed that the amount of ethanol administered (P.001) and type of malformation (P =.02) were multivariate predictors of serum ethanol level. Results of logistic regression analysis revealed that the only uni- or multivariate predictor of legal intoxication was the amount of ethanol administered (P =.018). Five patients fulfilled the criteria for legal intoxication, and the mean amount of ethanol administered to these patients was 0.87 mL per kilogram of body weight +/- 0.18 (SD) (range, 0.55-0.99 mL/kg).The volume of ethanol administered is the most reliable predictor of serum ethanol level and legal intoxication. Patients who receive up to 1.0 mL/kg ethanol during embolization or sclerotherapeutic procedures may have elevated serum ethanol levels that could put them at risk of respiratory depression, cardiac arrhythmias, seizures, rhabdomyolysis, and hypoglycemia.

We tested the accuracy of a low flow (50 cc/min) sidestream capnographysystem equipped with an experimental 30-foot nasal cannula to monitor ventilatory status in children. End-tidal CO2 and respiratory rate, both at room air and in the presence of supplemental oxygen, were recorded simultaneously from the experimental 30-foot nasal cannula and the standard, FDA approved, 10-foot nasal cannula. The 30-foot nasal cannula was as accurate as the 10-foot nasal cannula in measuring respiratory rate and end-tidal CO2 in children. When supplemental oxygen was delivered by facemask, there was no dilutional effect on the respiratory rate or end-tidal CO2 recorded with either the 10-foot or 30-foot nasal cannulas inplace.

Background. Sedation for diagnostic imaging and interventional radiologic procedures in pediatrics has greatly increased over the past decade. With appropriate patient selection and monitoring, serious adverse effects are infrequent, but failure to sedate and paradoxical reactions do occur. Objective. The purpose of this study was to determine, among patients undergoing sedation for radiologic procedures, the incidence of sedation failure and paradoxical reaction to pentobarbital and to identify potentially correctable causes. Materials and methods. Records of 1665 patients who were sedated in the radiology department from 1 November 1997 to 1 July 1998 were reviewed. Patients failing sedation or experiencing paradoxical reaction were compared with respect to sex, age group, diagnosis, scan type, time of day, NPO status, use of IV contrast and type of sedation agent using the Fisher exact test, Pearson chi-square, analysis of variance (ANOVA), the Student t-test, and logistic regression. Results. Data analysis revealed a sedation failure rate of 1 % and paradoxical reaction rate of 1.2 %. Stepwise multiple logistic regression revealed that the only significant independent multivariate predictor of failure was the need for the administration of a combination of pentobarbital, fentanyl, and midazolam IV. Conclusion. The low rate of sedation failure and paradoxical reactions to pentobarbital was near optimal and probably cannot be improved with the currently available sedatives.

MR imaging is an invaluable tool for the evaluation of congenital abnormalities and vascular birthmarks of the extremities in children. These abnormalities of the immature musculoskeletal system are often underestimated by radiography. MR imaging is useful for diagnosis, assisting in therapy, showing response to treatment, and determining prognosis. Localized and generalized abnormalities of the lower extremities and issues pertinent to their MR imaging are illustrated in this article.

This article reviews the role of modern diagnostic imaging in the evaluation of patients with vascular birthmarks. There are two main categories of vascular anomalies: hemangiomas and vascular malformations. The findings on plain radiography, sonography, computed tomography (CT), magnetic resonance imaging (MRI) and angiography, and the appropriate sequence of investigation for the different vascular anomalies are discussed.

Central nervous system vascular disorders in the neonate comprise structural anomalies or malformations of arteries and veins and physiologic alterations of cerebral blood flow, which can mimic structural vascular disease. Clinical, imaging, and transcatheter therapeutic aspects of neonatal cerebral vascular malformations are described. Symptomatic high-flow vascular malformations characteristically present with cardiac failure and associated systemic problems in the neonate, whereas infants typically present with macrocephaly and hydrocephalus and older children with hemorrhage, developmental delay, or focal deficits. Neonatal cerebral hemorrhage is typically primary or associated with immaturity, parturitional trauma, or coagulopathy. Likewise, cerebral ischemic lesions are more likely secondary to hypoxic, ischemic events than to thromboembolic or structural cerebrovascular occlusive disease. The role of the current noninvasive imaging modalities in each of these clinical problems is reviewed and illustrated.

Mutations in gene RASA1 have been historically associated with capillary malformation–arteriovenous malformation, but sporadic reports of lymphatic involvement have yet to be investigated in detail. To investigate the impact of RASA1 mutations in the lymphatic system, we performed investigational near-infrared fluorescence lymphatic imaging and confirmatory radiographic lymphangiography in a Parkes–Weber syndrome (PKWS) patient with suspected RASA1 mutations and correlated the lymphatic abnormalities against that imaged in an inducible Rasa1 knockout mouse. Whole-exome sequencing (WES) analysis and validation by Sanger sequencing of DNA from the patient and unaffected biological parents enabled us to identify an early-frameshift deletion in RASA1 that was shared with the father, who possessed a capillary stain but otherwise no overt disease phenotype. Abnormal lymphatic vasculature was imaged in both affected and unaffected legs of the PKWS subject that transported injected indocyanine green dye to the inguinal lymph node and drained atypically into the abdomen and into dermal lymphocele-like vesicles on the groin. Dermal lymphatic hyperplasia and dilated vessels were observed in Rasa1-deficient mice, with subsequent development of chylous ascites. WES analyses did not identify potential gene modifiers that could explain the variability of penetrance between father and son. Nonetheless, we conclude that the RASA1 mutation is responsible for the aberrant lymphatic architecture and functional abnormalities, as visualized in the PKWS subject and in the animal model. Our unique method to combine investigatory near-infrared fluorescence lymphatic imaging and WES for accurate phenoptyping and unbiased genotyping allows the study of molecular mechanisms of lymphatic involvement of hemovascular disorders.

OBJECTIVE: Infantile hemangioma and arteriovenous malformation (AVM) of the liver have a similar presentation but a different natural history, and therefore require different treatment. This study was undertaken to clarify differential diagnosis and management of these two biologically distinct vascular disorders. STUDY DESIGN: We retrospectively analyzed the records of 43 children with hepatic vascular anomalies treated during the past 27 years. RESULTS: Ninety percent were hemangiomas (n = 39); 10% were AVM (n = 4). Infants with AVM or large solitary hemangioma had hepatomegaly, congestive heart failure, and anemia as presenting symptoms at birth. Multiple hepatic hemangiomas manifested at 1 to 16 weeks of age with the same clinical triad, plus multiple cutaneous lesions (19/23). The mortality rate after treatment of hepatic AVM was 50% (2/4). The mortality rates after treatment of liver hemangiomas were as follows: resection of solitary lesions, 20% (2/10); embolization, 43% (3/7); corticosteroids, 30% (3/10); and interferon alfa-2a, 15% (2/13). CONCLUSION: Solitary hepatic hemangioma cannot always be distinguished from hepatic AVM without radiologic studies. Multiple hepatic hemangiomas are differentiated from hepatic AVM by coexistence of multiple cutaneous hemangioma and by radiologic imaging. We recommend combined embolization and surgical resection for hepatic AVM and for solitary symptomatic hemangioma, if drug therapy fails. Pharmacologic treatment is used for symptomatic multiple liver hemangiomas. Embolization allows interim control of heart failure. A decreased mortality rate after interferon alfa-2a therapy is encouraging. (J P EDIATR 1996;129:346-54)

Contrast angiography can demonstrate the vascular components of a vascular malformation, but can be technically challenging in small patients with complex venous anomalies. We reviewed the role of magnetic resonance venography (MRV) in the evaluation of children with predominantly low-flow, vascular malformations of the extremities. MRV (2D time-of-flight technique) and magnetic resonance (MR) imaging examinations were performed in ten young patients with congenital predominantly low-flow vascular malformations of the extremities. MR imaging was used to characterize and determine the extent of the malformations, and MRV to evaluate the deep and superficial venous channels. In all patients, MRV studies were reviewed in conjunction with contrast angiograms, considered the gold standard, to confirm the findings. All significant channel anomalies seen with contrast angiography were identified with MRV. In addition, MRV demonstrated some veins that were not intentionally opacified during contrast studies. MRV demonstrates both the superficial and deep conducting veins, whereas contrast angiography is a more directed study, evaluating only those channels intentionally opacified. Together, MR imaging and MRV data can non-invasively form the basis for determining the prognosis and choosing the individual treatment of congenital vascular malformations of the extremities.

✓ The authors report a unique case involving a 2-year-old child with idiopathic moyamoya disease who presented with cerebral infarctions and seizures. On initial evaluation, computerized tomography (CT) showed a left parietal infarct and angiograms demonstrated early moyamoya disease with no evidence of arteriovenous malformation (AVM). Approximately 9 years later, angiography and magnetic resonance (MR) imaging revealed an AVM centered on the same region of the left parietal lobe. Angiographic, CT, and MR images are presented that demonstrate the progression of moyamoya disease and de novo development of the AVM in the infarct site. The possible role of angiogenesis in the etiology of acquired AVMs and moyamoya disease is discussed.

THE patient with an anterior mediastinal mass presents the anesthesiologist with a formidable challenge. Severe respiratory and cardiovascular complications, including the inability to resuscitate, can ensue on induction of general anesthesia. We describe the anesthetic management of a child with a large dynamic vascular malformation located in the neck and mediastinum who underwent percutaneous sclerotherapy.

To evaluate selective embolization for management of posttraumatic priapism in preadolescent boys and color Doppler sonography for diagnosis of the causative lesion and for planning embolization.Five preadolescent boys with posttraumatic priapism underwent selective angiography and embolization. Color Doppler sonography was performed in the last three patients.Selective angiography showed an intracavernosal arteriovenous fistula in two patients, pseudoaneurysm of the cavernosal artery in two patients, and asymmetric cavernosal arterial flow in the other patient. After successful embolization, detumescence was achieved in all patients. Color Doppler sonography enabled localization and characterization of the lesion causing the priapism in the three patients.Angiography with selective embolization is a safe and effective method to correct posttraumatic priapism in children. Color Doppler sonography is a valuable preangiographic study, as it allows for characterization and localization of the causative lesion and obviates diagnostic cavernosal aspiration.

We performed a cross-sectional evaluation of deep vein thrombosis (DVT) related to the use of central venous lines (CVLs) in all pediatric patients receiving home total parenteral nutrition at our institution (N = 12). All children (5 months to 17 years of age) were examined with bilateral upper limb venography. All CVLs were flushed daily with heparin (200 units). At the time of evaluation, 49 CVLs had been placed in the 12 children. Of the 39 CVLs removed, 27 (66%) were blocked; venograms had not been previously obtained except of one child. Eight children had clinical evidence of superficial collateral circulation in the upper portion of the chest and the upper extremities; five had intermittent symptoms of superior vena cava obstruction. On venography, 8 of the 12 children had extensive evidence of DVT; two were unilateral and six bilateral. Five children were treated with warfarin (0.12 to 0.28 mg/kg per day) to achieve an international normalized ratio of 1.4 to 1.8. Neither bleeding nor further CVL-related DVT has occurred. We conclude that the risk of CVL-related DVT in children requiring home total parenteral nutrition is high, and that venography should be performed early in the event of CVL blockage. A multicenter, controlled trial assessing optimal warfarin therapy in this patient population is indicated.

The purpose of this article is to study the incidence, risk factors, and treatment of gross hemoglobinuria and oliguria following sclerotherapy for venous malformations.The clinical records and imaging studies of 131 patients with venous malformations (57 male and 74 female patients; age range, 2-58 years) who underwent sclerotherapy at our institution between July 1993 and August 2007 were reviewed. Demographic data, the location and estimated size of the malformation, the type and dose of the sclerosing agents, development of postprocedural hemoglobinuria and oliguria, and the treatment given were documented and analyzed.Four hundred seventy-five sclerotherapy procedures were performed on 131 patients, with the number of procedures per patient ranging from 1 to 21 (mean, 3.6 procedures). Sodium tetradecyl sulfate was used in 47% of the procedures, ethanol in 27%, and both agents in 26%. Transient hemoglobinuria occurred after 34% of the sclerotherapy procedures, and 57% of these were associated with transient oliguria, with increased risk with higher adjusted doses (sclerosant volume/weight of patient) for both agents. Resolution of the hemoglobinuria and oliguria with hydration, alkalinization, and diuretics occurred in all patients. The risk of hemoglobinuria increased with higher adjusted dose (sclerosant volume/weight of patient) for both agents and with sclerotherapy of venous malformations affecting the lower extremities and multiple locations.Transient hemoglobinuria and oliguria are common complications of sclerotherapy for venous malformation. Nevertheless, with proper fluid management, all the patients promptly recovered. The risk correlates with the volume of sclerosant (adjusted to patient's weight) and is higher for lower extremity and multiple locations.

Background and Purpose— PHACE is an acronym for posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Several case reports of arterial ischemic stroke (AIS) in individuals with PHACE have been published, but risk factors for AIS in PHACE have not been clearly defined. The objective of this article is to review all cases of stroke in PHACE in children and describe clinical characteristics that may be associated with an increased risk of AIS. Methods— A literature and registry search was conducted to identify patients with PHACE who had experienced AIS. Data were analyzed to determine age of onset, presenting signs and symptoms, and clinical features among this cohort compared with PHACE without AIS. Results— Twenty-two individuals with PHACE and AIS were identified. Imaging of the arteries of the head and neck was reported in 20 of 22. Narrowing or nonvisualization of at least 1 great cerebral vessel was present in 19 of 20 and of those, 15 had ≥2 vessels involved. Aortic arch anomalies were reported in 13 of 22 individuals. Conclusions— Aplasia, hypoplasia, or occlusion of a major cerebral artery appears to be a significant risk factor for AIS in children with PHACE, especially when >1 vessel is involved or if there is coarctation of the aorta.

PTEN hamartoma tumor syndrome (PHTS) presents in a spectrum that encompasses the eponymous disorders Cowden and Bannayan-Riley-Ruvalcaba. Herein, we delineate the distinctive histopathology of a predominantly intramuscular lesion in PHTS, often called "arteriovenous malformation," because of certain imaging and histopathologic features. Cases were identified by review of lesions resected from patients with PHTS registered at our Vascular Anomalies Center and of unusual intramuscular vascular anomalies in our pathology database from 1985 to 2008. Thirty-four patients with this lesion were identified: 20 had a clinical diagnosis of, or were suspected to have, PHTS (genetically confirmed in 16). In 4 patients without clinical manifestations of PHTS, 2 had PTEN mutations, 1 did not, and in 1 the mutation was intronic. In the remaining 10, there was insufficient clinical information to fully assess whether they had manifestations of PHTS. Lesions manifested by 15 years of age, normally with pain and swelling, and were most often located in the lower extremity. The major mass was usually intramuscular, but often there were fascial and subcutaneous components and not infrequently a cutaneous vascular stain. Magnetic resonance imaging generally showed an infiltrative soft tissue lesion involving the muscle, fascia, and subcutis with frequently enlarged, serpiginous vessels, small arteriovenous fistulae with disproportionately dilated draining veins, and a prominent adipocytic component. Some lesions involved contiguous muscles, and 20% were multifocal. Resected specimens ranged in size from 1.2 to 25 cm; in 1 patient, amputation was necessary. Histopathologically, these unencapsulated masses, often with a nodular appearance at scanning magnification, consisted of: (1) a variable admixture of mature adipocytic and dense and/or myxoid fibrous tissues (50% to 90% of surface area); (2) a vascular component (10% to 50% of surface area) with: (a) clusters of venous channels, some with excessively and irregularly muscularized complex walls and lumens, and others with thin walls resembling pulmonary alveoli, (b) tortuous, thick-walled arteries with concentric muscular hyperplasia and relatively small lumens, (c) numerous small vessels (arteries, veins, and indeterminate channels), and (d) occasional arteriovenous communications; (3) lymphoid follicles (50%); (4) foci of bone (20%); and (5) hypertrophic nerves with "onion bulb" proliferation of periaxonal spindled cells (9%). We designate this disorganized overgrowth of essentially mesenchymal elements as PTEN hamartoma of soft tissue. It differs from other vascular and connective tissue lesions that occur in patients with PHTS. PTEN hamartoma of soft tissue is histopathologically distinctive, and its identification should prompt a thorough investigation for PHTS.

BACKGROUND We wished to determine the nature and incidence of changes in the iliofemoral arteries after balloon dilation angioplasty (BDA) for aortic arch obstruction in children and to determine the reliability of gradient magnetic resonance imaging (MRI) in their detection. METHODS AND RESULTS Sixty-three children, including 62 with and 1 without arch obstruction, underwent MRI of the iliofemoral vessels. Of these, 36 patients had undergone transfemoral BDA (7 after previous transfemoral diagnostic catheterization), 12 had undergone diagnostic transfemoral catheterization but not BDA, and 15 had no history of femoral arterial catheterization. The iliofemoral arteries were normal on MRI in all 15 children without catheterization. Among the 36 children who had undergone BDA, the ipsilateral iliofemoral artery was normal in 15, mildly narrowing in 7, and severely stenotic or occluded in 14 (39%), including 6 of 9 patients treated for acute femoral artery thrombosis and 8 with no history of femoral artery thrombosis. Two patients had documentation of progressive obstruction. Six patients had concordant conventional angiography. There was a significant correlation between the number of balloon catheters used for the angioplasty and severe occlusive changes. Nine of 19 patients who had undergone diagnostic transfemoral catheterization had severe obstructive changes on MRI; 8 of 9 weighed < 10 kg at catheterization. CONCLUSIONS Obstructive lesions of the iliofemoral arteries are common after transfemoral BDA of arch obstructions (58%) and can be reliably evaluated with gradient MRI. Catheter size and manipulation are the main contributing factors.

CLOVES syndrome is a complex disorder of congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal/scoliosis/spinal anomalies. We report the occurrence of spinal-paraspinal fast-flow lesions within or adjacent to the truncal overgrowth or a cutaneous birthmark in 6 patients with CLOVES syndrome.

Vascular malformations (VM) are classified by flow characteristics and channel content. They can involve any organ and tissue plane, and occur in focal and diffuse forms. Slow-flow vascular malformations (venous and lymphatic malformations) are typically treated by sclerotherapy, whereas fast-flow lesions (arteriovenous malformations) are managed with embolizations. Some VMs, such as VMs of the rectum or uterus, are best dealt with surgically. This review will present a summary of the conditions, their imaging features, and some useful endovascular therapeutic techniques.

The trend to perform early primary repair of tetralogy of Fallot prompted us to review our experience in the current era with the traditional protocol consisting of palliation during infancy, if required, and repair after infancy. During a 10-year period, 270 infants with tetralogy of Fallot presented aged less than 18 months. Thirteen infants (4.8%) had major noncardiac lesions that precluded definitive care for their congenital heart disease. Twenty infants (7.4%) had major associated cardiac lesions (atrioventricular septal defect or absent pulmonary valve syndrome, or both). Survival in this group was poor, with only 58% +/- 12% reaching the age of 10 years. Four of the seven deaths occurred before intracardiac repair was performed. The remaining 237 infants presented with isolated tetralogy of Fallot. Eight-nine percent +/- 2.3% survived to age 10 years. Sixty percent of these infants required palliation, and survival in these infants did not differ from that in those who never required palliation. However, 19 infants (8%) required palliation in the first month of life. In these children, survival to age 10 years was significantly lower (77%), secondary palliation was frequently required (n = 11), and a transannular patch or conduit at the time of repair (10 of 14 patients) was more likely needed than it was in children who had not undergone a palliative procedure during the neonatal period. The survival in infants with tetralogy of Fallot is unlikely to be different, regardless of whether primary repair or a staged repair is carried out. The quality of survival, including the exercise capability and absence of arrhythmias, must be assessed to determine which protocol is superior.

A patient with pulmonary atresia and a ventricular septal defect is described in whom an arterial branch from the distal ascending aorta supplied segments of both lungs. The branch is considered to represent a persistent fifth aortic arch. The possible morphogenesis and differential diagnosis of a communication between the ascending aorta and the pulmonary artery in pulmonary atresia with ventricular septal defect are discussed.

Objectives The objectives of this study were to determine the anatomic and physiological factors most responsible for the severe symptoms and poor prognosis of infants with scimitar syndrome. Background Whereas the diagnosis of scimitar syndrome is often made incidentally in older children and adults who undergo chest radiography for diverse reasons, infants in whom the diagnosis is made typically present with severe symptoms and have a poor prognosis. Methods The clinical, catheterization and imaging data of 13 consecutive infants with scimitar syndrome who underwent cardiac catheterization in the 1st 6 months of life were reviewed, with emphasis on the pulmonary artery pressure, pulmonary and cardiovascular anatomy, therapeutic interventions and outcome. Results Twelve of the 13 infants had pulmonary hypertension at the time of diagnosis. Six patients died despite specific treatment. Eleven of 13 infants had associated cardiac malformations and 9 had large systemic arterial collateral channels to the right lung. Seven patients had anomalies involving the left side of the heart, especially varying degrees of hypoplasia of the left heart or aorta, and six of these patients died. Ten patients underwent surgical or transcatheter therapy in the 1st year of life. Systemic arteries to the right lung were ligated in three patients and occluded by transcatheter embolization in four. Balloon angioplasty was carried out in two patients, one with stenosis of the left-sided pulmonary veins and one with stenosis of the anomalous right pulmonary vein. The latter had placement of a balloon-expandable stent. In both patients, pulmonary vein stenosis progressed. Six patients had surgical repair of associated cardiovascular anomalies, and two required repair of extracardiac congenital anomalies. Occlusion of the anomalous systemic arteries was generally associated with clinical improvement, but congestive heart failure and pulmonary hypertension recurred in those patients with associated cardiovascular anomalies, whose condition subsequently responded after correction of the shunt lesions. Conclusions The severe symptoms and pulmonary hypertension found in infants with scimitar syndrome have many causes. Anomalous systemic arterial supply, pulmonary vein stenosis and associated cardiovascular anomalies play a significant role, and the ultimate outcome of individual infants depends on the feasibility of treating these anomalies in early infancy.

Recently, a case of right isomerism with totally anomalous pulmonary venous connection (TAPVC) to the innominate vein through a vertical vein that ascended in the mediastinum posterior to the left-sided main bronchus was encountered. This retrobronchial vertical vein that has not been described previously in patients with TAPVC to the innominate vein prompted the performance of a retrospective analysis of 44 cases with TAPVC to the innominate vein in which angiography had been performed. The 44 cases consisted of 36 patients with situs solitus, 7 with right isomerism, and 1 with left isomerism. The spatial relation of the vertical vein to the adjacent pulmonary artery and main bronchus could be defined in 41 cases. The vertical vein was retrobronchial in 4 of 5 cases with right isomerism in which the location of the vertical vein could be defined with certainty. No case with situs solitus or left isomerism was associated with a retrobronchial vertical vein. In all 4 patients with a retrobronchial vertical vein, angiographic evidence of obstruction of pulmonary venous drainage was present, and was due to an extrinsic bronchial compression in 2 and to an intrinsic narrowing of the vertical vein in 2.

Bannayan-Riley-Ruvacalba syndrome (BRRS) belongs to the PTEN hamartoma tumor syndromes and is characterized by a high risk of malignancy in early adulthood added to local destructive effects of hamartomas in childhood. There is no standard treatment for this condition and patients are usually offered symptomatic surgical relief. Rapamycin has been reported to be effective in the management of other conditions associated with PTEN mutation. We report here a case of BRRS in a 6-year-old male with progressive loss of function of left hand and forearm associated with pain. He was treated with oral rapamycin and regained pain-free full mobility.

BACKGROUND AND PURPOSE: KTS is a rare limb overgrowth disorder with slow-flow vascular anomalies. This study examines the presumed association between KTS and spinal AVMs. MATERIALS AND METHODS: We performed a MEDLINE search of articles and reviewed textbooks of spinal diseases to study the association between KTS and spinal AVM. Our goal was to ascertain the basis on which the diagnosis of KTS was established and to evaluate the evidence of its association with spinal AVMs. In addition, the data base of the Vascular Anomalies Center at Children9s Hospital Boston was queried for patients with KTS, and the association with spinal AVM was investigated. RESULTS: Twenty-four published reports on spinal AVMs in 31 patients with KTS were reviewed. None of these references provided solid evidence of the diagnosis of KTS in any patient. Clinical data were either incompatible with the diagnosis of KTS or were inadequate to establish the diagnosis. Alternative possible diagnoses (CLOVES syndrome and CM-AVM) were suggested by the first author for 9 of the patients reported in these articles. The medical records of 208 patients with the diagnosis of KTS were analyzed; not a single patient had clinical or radiologic evidence of a spinal AVM. CONCLUSIONS: An association between KTS and spinal AVM, as posited in numerous references, is most likely erroneous. The association has neither been reliably proved in the limited published literature nor encountered in a large cohort.

The authors present a series of 13 multiple arteriovenous malformations (MAVMs) in the pediatric population (16.9% of their overall series of brain AVMs in this group). Two types of MAVMs can be distinguished: congenital and acquired. Congenital MAVMs may be of the nidus or fistula type. They may be uni- or bilateral, placed in one or several cerebral lobes, separated or close one to another, or even systematized (Wiburn-Mason syndrome). The symptoms created by these MAVMs are the same as those encountered in the presence of other AVMs, with hemorrhage as revealing symptom in 31% of patients. The responsibility of one particular nidus in the onset of clinical signs is often difficult to determine. From an angioarchitectural point of view, it seems that venous drainage changes are mainly responsible for the symptomatology. The natural history of these MAVMs is difficult to assess; spontaneous regression has been noted in 15% of cases. Acquired cerebral MAVMs can be due to angiogenesis ("sprouting" or "non-sprouting") around a true AVM because of previous hemorrhage or ischemia, or to pial shunts associated with dural arteriovenous malformations. The treatment of MAVMs is difficult. Embolization seems to the authors the best therapeutic modality available, as surgery or radiosurgery are often unable to treat these multifocal lesions. Anatomical cure is rarely obtained; the therapeutic strategy has to be targeted on the symptomatic lesions.

Twelve children were identified with subaortic stenosis after Fontan's operation. All had absent resting and isoproterenol-provoked pressure gradient before the Fontan procedure. Six had a univentricular heart of left ventricular morphology, three had a single ventricle of right ventricular morphology, one had tricuspid atresia with transposition of the great arteries, one had pulmonary atresia, intact ventricular septum, and hypoplastic right ventricle, and one had corrected transposition with hypoplastic systemic ventricle. The median interval between the Fontan operation and the recognition of subaortic stenosis was 2.5 years. Ten patients underwent surgical treatment after a prior Fontan operation: Five had myectomy and enlargement of ventricular septal defect with two operative deaths; two had placement of a valved conduit from the ventricular apex to the descending aorta, and both died postoperatively; two with single ventricle had subaortic myectomy, and one had enlargement of ventricular septal defect and pulmonary aortic connection. Complete heart block developed in only one patient. Postoperative testing with Doppler echocardiography with color flow imaging demonstrated good relief of subaortic stenosis. All six children who survived the operation are well 4 months to 4 years later. Subaortic stenosis is a progressive lesion that may develop after a Fontan operation. Its surgical treatment continues to carry a significant mortality. Myectomy and enlargement of ventricular septal defect achieve direct relief of the obstruction with minimal risk of heart block.

Objectives. This study was designed to assess the impact of implantation of balloon-expandable stents on right ventricular outflow obstruction in children with congenital heart disease. Background. Intravascular stenting has been established as a useful treatment in adults with coronary and peripheral vascular disease. Its application in the treatment of infants and children with pulmonary, systemic and right ventricular conduit obstruction resistant to balloon angioplasty is limited. Methods. A total of 24 stainless steel stents were implanted in 17 patients. Five stents were placed within right ventricular to pulmonary artery conduits, 17 in branch pulmonary arteries and 1 in an aortopulmonary collateral vessel. Follow-up time has ranged from 1 to 14 months, with 6 patients having hemodynamic and angiographic studies >1 year after stent placement. The mean age at implantation was 7.4 ± 5.6 years and the mean weight 33 ± 16 kg. Results. Optimal stent position was obtained in 22 of 24 implantations. In one patient the stent slipped from the delivery balloon and was left positioned in the inferior vena cava. No embolization or thrombotic event has been documented. Among patients with right ventricular to pulmonary artery conduit obstruction, the gradient was immediately reduced from 85 ± 30 mm Hg to 35 ± 20 mm Hg after stent implantation; however, three patients required conduit replacement because of persistent obstruction with elevated right ventricular pressures (82 ± 16 mm Hg). In 10 of 11 patients with pulmonary artery stenosis, clinical improvement was noted in association with enlargement of vessel diameter by 92% ± 90% (range 17% to 355%) and the gradient reduction of 22 ± 24 mm Hg to 3 ± 4 mm Hg. Conclusions. These data support the view that intravascular stenting will become an important adjunct in the management of children with congenital heart disease.

The clinical impact of percutaneous balloon angioplasty on the management of patients with native or postoperative pulmonary arterial stenosis was reviewed. Seventy-four patients underwent 110 angioplasty procedures. Mean age at dilation was 6.7 +/- 5.3 years (range 0.2 to 18.1), 17 patients were aged less than 1 year, mean follow-up was 37.7 +/- 22.8 months (range 16 to 96), and 34 patients (44%) had follow-up angiography. Pulmonary artery dilation was acutely successful in 53% of patients, 17% had recurrent stenosis, and 5% had complications. The impact on subsequent care was favorably influenced in 26 of 74 patients (35%) with either complete resolution of stenosis (n = 7), optimizing future surgical conditions (n = 14), reduction in right ventricular pressure by greater than 20% (n = 3), or improvement of ipsilateral lung perfusion (n = 2). No patient previously considered inoperable was subsequently considered suitable for surgical repair owing to the intervention. No correlation was found between success and cardiac diagnosis (p = 0.48), site of stenosis (p = 0.78), balloon-vessel ratio (p = 0.42), or whether the stenotic area consisted of native or synthetic material (p = 0.22). No predictive factors for success could be defined, and often there was only a transient clinical impact. Due to the low complication risk and potential for a beneficial result, it still appears prudent to offer angioplasty as an initial therapeutic modality in this setting.

Objective To define the clinical spectrum of regional congenital anomalies associated with large cutaneous hemangiomas of the lower half of the body, clarify risk for underlying anomalies on the basis of hemangioma location, and provide imaging guidelines for evaluation. Study design We conducted a multi-institutional, retrospective case analysis of 24 new patients and review of 29 published cases. Results Hemangiomas in our series tended to be "segmental" and often "minimal growth" in morphology. Such lesions were often extensive, covering the entire leg. Extensive limb hemangiomas also showed potential for extracutaneous anomalies, including underlying arterial anomalies, limb underdevelopment, and ulceration. The cutaneous hemangioma and underlying anomalies demonstrated regional correlation. Myelopathies were the most common category of associated anomalies. Conclusions We propose the acronym "LUMBAR" to describe the association of Lower body hemangioma and other cutaneous defects, Urogenital anomalies, Ulceration, Myelopathy, Bony deformities, Anorectal malformations, Arterial anomalies, and Renal anomalies. There are many similarities between LUMBAR and PHACE syndrome, which might be considered regional variations of the same. Although guidelines for imaging are suggested, prospective studies will lead to precise imaging recommendations and help determine true incidence, risk and long-term outcomes.

A young girl with an arteriovenous malformation involving the right upper extremity developed rapidly progressive bony destruction that did not respond to embolization. Treatment with marimastat, starting at 3 years of age, resulted in rapid resolution of pain and gradual healing of bony destruction, associated with regression of the intraosseous arteriovenous shunts. New arteriovenous shunts with bony destruction developed over the years and responded to an increase in the dose of marimastat. Interruption of therapy resulted in recurrence of pain and formation of new lesions. The patient has been treated in this way for 12 years with no adverse effects from the drug.

The authors present four consecutive cases of multiple dural arteriovenous (AV) shunts in children. This entity represents a rare but severe clinical situation. The etiology of the shunts is not known. There is clinical and radiological evidence that they are evolutionary lesions. The clinical presentation in this series was usually by cardiac manifestations (one case), cerebrospinal fluid disorders (two cases), neurological symptoms (three cases), including intracranial hemorrhage (two cases), and cranial bruits (four cases). No radical treatment leading to anatomical cure of all dural AV shunts was thought to be possible using current methods, including modern endovascular and surgical techniques, in any of the four cases. Therefore, treatment was only symptomatic and directed at some of the AV shunts. Targeted arterial embolization with permanent embolic agents represents the most rational technique for symptomatic relief in these patients. However, clinical recurrence often happens without evidence of recanalization. Secondary multifocal pial AV shunts opening into the abnormal sinus occurred in two of the cases; they may have been induced by venous sump from the sinus draining the dural AV shunts. Mechanical occlusion (or excision) in multiple dural AV shunts in children does not represent a satisfactory goal, as the shunts can be the expression of a more complex and yet unknown disease.