[Role of duplex Doppler for superficial "angiomas"]. / Place de l'échodoppler dans les "angiomes" superficiels.

Diagnosis of superficial vascular anomalies, previously called "angiomas", is basically clinical. Ultrasound and duplex Doppler imaging is a simple and helpful tool to confirm the clinical diagnosis and/or to suggest further required imaging modalities. The purpose of this work is to demonstrate the usefulness of duplex Doppler and ultrasound for exploring vessel and soft tissue components of vascular anomalies.

[Vascular lesions of the orbit in children]. / Pathologies vasculaires de l'orbite de l'enfant.

Vascular lesions of the orbit in children are most often hemangiomas and lymphangiomas. Hemangioma is a tumor that tends toward spontaneous regression. Lymphangioma is a malformation that tends to increase in size with growth spurts that can occur until adulthood. The treatment is decided according to the functional impact.

[Abnormal origin of the left coronary artery from the pulmonary artery: a retrospective study of 36 cases]. / Anomalie de naissance de la coronaire gauche à partir de l'artère pulmonaire: étude rétrospective de 36 patients.

The aim of this study was to show the evolution of diagnostic techniques revealing an abnormal origin of the left coronary artery and present the follow-up results of patients operated for this malformation at different ages. This retrospective study includes 36 children or adults, with a mean age at the moment of of 28 months old, ranging from 7 days to 39 years. In 9% of cases, patients were asymptomatic. Before 1990, 81% of children had a cardiac catheterization, versus 25% after. Indeed, echocardiography with color Doppler enabled the diagnosis of abnormal origin of the left coronary artery in 70% of cases. The pre-operative mortality is at 21% (12.5% after 1990). Twenty-three patients had surgery: left coronary artery reimplantation (n=16), bypass (n=5), at mean of 44 months old for the entire series, but 21 months old after 1990. The follow-up was clinical, ECG and radiographic and echocardiographic after 1975; when possible an exercise test, cardiac nuclear imaging and more recently coronary CT scan and MRI have been performed. In conclusion, children are operated earlier and the follow-up should focus on the detection of occlusion of the re-implanted left coronary artery by echocardiography, with or without stress, exercise test, cardiac nuclear imaging, MRI and sometimes, coronary angiography.

[Early surgical treatment of cutaneous hemangiomas]. / Le traitement chirurgical précoce des hémangiomes cutanés.

Infantile hemangioma appears after the birth as a vascular tumor, which is known for its characteristic evolution in 3 phases: rapid augmentation then stabilization and involution on several years with in the best cases, classical "restitutio ad integrum". Usual surgical attitude is abstention and surgery is proposed only in order to treat sequel. But some particular situations require early surgery for life-threatening lesions or in case of functional impairment. Surgery consists in these cases in a simple volumetric diminution of the tumor with no aim for esthetic improvement. Other kinds of hemangiomas require an early surgical treatment before their complete involution. In some particular locations, deformation or growth delay can occur due to the lesion's development. In some cases, hemangiomas present a delayed involution with minor regression capacity; these are mainly located on the median part of the face and have principally a subcutaneous development. Early surgery can be proposed in order to avoid definitive deformation or growth impairment of adjacent structures. It should be performed before school age and before occurrence of psychological difficulties. Surgery is indicated in a perspective of esthetic improvement. Surgical procedure consists first in modeling excision followed by simple repair technique as linear suture or purse string closure; complex surgical procedures inducing their own sequels are usually inappropriate.

[Percutaneous angioplasty of native coarctation of the aorta. Results in children and young adults]. / Angioplastie percutanée de la coarctation native de l'aorte. Expérience d'un centre chez l'enfant et l'adulte jeune.

The authors report the experience of one centre in the treatment of native coarctation of the aorta by percutaneous angioplasty. Between 1999 and August 2005, 22 patients (7 girls, 15 boys) underwent dilatation of their coarctation by a balloon catheter at an average age of 11.8 +/- 5 years and body weight of 44 +/- 21 kg. In 20 patients, balloon angioplasty alone was performed and, in the other two, the angioplasty was associated with the insertion of a covered CP stent. The dilatation was performed with a balloon/coarctation ratio of 2.18 +/- 0.6 (1.7 to 3.5) and a balloon/isthmus ratio of 1.0 +/- 0.23 (0.7 to 1.87). After the procedure, ascending aortic pressure decreased from 134.7 +/- 23.4 mmHg to 125 +/- 22.7 mmHg (p= 0.0003); descending aortic pressure increased from 93.4 +/- 14.9 mmHg to 104.8 +/- 21.7 mmHg (p= 0.003); transisthmic pressure gradient decreased from 41.7 +/- 14.1 mmHg to 19.8 +/- 9.5 mmHg (p< 0.0001) and the diameter of the coarctation increased from 5.9 +/- 2.6 mm to 9.3 +/- 2.6mm (p= 0.0015). The two patients treated by covered CP stents had excellent immediate results without significant residual gradients. After dilatation (sometimes repeated, N= 13), a gradient > 20 mmHg persisted in ten patients (36% of cases) but no predictive factor was found on statistical analysis. There were no cases of acute aneurysm. In one patient, the femoral pulse decreased but returned to normal with heparin therapy. No patient required emergency surgical treatment after the angioplasty. During the clinical follow-up, echocardiography and MRI showed no signs of aneurysm and one patient had mild irregularity of the isthmic region. Three patients underwent surgery by resection suture for persistent coarctation (one combined with a Ross procedure); another patient was treated by a CP stent. In conclusion, percutaneous angioplasty of native coarctation of the aorta gives satisfactory results with few complications in bigger children and young adults. The results can be improved by using a slightly higher balloon/coarctation ratio.

[The effect of an endothelin receptor antagonist in Eisenmenger syndrome: a single-center experience of 11 patients]. / Effet d'un antagoniste des récepteurs de l'endothéline dans le syndrome d'Eisenmenger: expérience d'un centre à propos de 11 patients.

The Eisenmenger syndrome is a complication of congenital heart disease with significant left-to-right shunts, such as large ventricular septal defects, and corresponds to fixed pulmonary hypertension with shunt reversal. Bosentan, an inhibitor of endothelin A and B receptors, is a new molecule previously validated in the treatment of primary pulmonary hypertension. The authors report their monocentric experience of bosentan in 11 consecutive patients with the Eisenmenger syndrome treated for at least one year. This retrospective study comprised 7 females and 4 males with an average age of 34 years (range 17 to 51 years). The underlying lesion was ventricular septal defect (n = 4), atrial septal defect (n = 3), pulmonary atresia with septal defect (n = 4 of which 2 were treated palliatively). Before treatment, the patients were classified according to the NHYA functional class (I, II, IIIa and IIIb, IV or, respectively from 1 to 5) with a distribution in this series between Classes IIIa and IV (average 3.81 +/- 0.75) and from 3 to 10 on Borg's dyspnoea scale (average 6.54 +/- 2.29). The ambient oxygen saturation (SaO2) at rest was, on average 77 +/- 9%, the haemoglobin concentration 16.6 +/- 2.4 g/dl; hepatic transaminase levels were normal. The 6 minute walk test before treatment was 216 +/- 111 m with marked desaturation on exercise (49 +/- 18%). With Bosentan, patients were globally much better clinically with a decrease in dyspnoea, improvement in NHYA class and increased 6 minute walking perimeter. Improvement in NYHA class was observed from 3 months' treatment (3.0 +/- 0.8, p = 0.0002) and was sustained to one year (2.54 +/- 0.7, p< 0.001). An improvement of dyspnoea on Borg's scale was observed from the second month's treatment (5.56 +/- 1.65, p = 0.0201) and persisted throughout follow up to one year (3.81 +/- 1.32, p < 0.0001). Similarly, the 6 minute walking perimeter increased from the first control at 6 months (323 +/- 82 m, p < 0.0001) and at one year (322 +/- 62 m, p <0.0004). Finally, although a significant increase in SaO2 was observed at 6 months (p = 0.0032), this was hardly significant at one year (82 +/- 10 %, p = 0.0512). Transaminase levels did not rise significantly at the follow up visits (p = ns) and the haemoglobin concentration was unchanged. No patient died during the study period. This study showed a clear functional improvement in patients with Eisenmenger's syndrome treated with bosentan. The drug was well tolerated clinically with few adverse effects and a good safety margin of usage.

[Pulmonary atresia with an intact interventricular septum]. / Atrésie pulmonaire à septum interventriculaire intact. Expérience d'un centre à propos de 35 patients.

We report this centre's experience of the treatment and follow up of pulmonary atresia with an intact septum. 35 infants were seen during the neonatal period. Opening via catheterisation was attempted in 21 patients with initial success in 10, and 11 failures leading to urgent surgery (surgical opening, n=9) and isolated Blalock-Taussig anastomosis (n=2). Fourteen other patients underwent immediate surgery: surgical opening, n=3, and Blalock alone, n=11. Four patients died in the neonatal period: 1 after successful opening via catheterisation, 3 others after Blalock anastomosis. Five others with a Blalock anastomosis died suddenly later. By the end of follow up, 16 patients had undergone biventricular type repair, of which 7 required additional procedures. 10 others had undergone cavo-pulmonary type repair, including 4 infants in whom the initial strategy of biventricular repair had failed. The only predictive factors at birth for subsequent progression to biventricular type repair were: larger tricuspid diameter at echography (10.9 +/- 2.25 mm versus 6.34 +/- 1.74 mm, p = 0.0007) or at angiography (10.07 +/- 2.09 mm versus 8.04 +/- 2.42 mm, p = 0.039), and the right ventricular morphology (p = 0.0011) with more tripartite ventricles, and less bipartite or even unipartite ones in the biventricular group.

[Vincristine treatment for function- and life-threatening infantile hemangioma]. / Traitement par vincristine des hémangiomes graves du nourrisson.

AIM: To evaluate the efficacy of vincristine treatment for function- and life-threatening hemangiomas. PATIENTS AND METHOD: Nine infants, eight girls and one boy, received vincristine treatment (VCR) for endangering hemangiomas. In six cases, the hemangiomas involved head and neck in a segmental unilateral or bilateral distribution (3/6 also had laryngeal and 2/6 tracheal location causing respiratory distress, 5/6 had eyelid and orbital involvement); one infant had disseminated neonatal hemangiomatosis (skin, liver, kidney); two infants had liver hemangiomas with cardiac failure. VCR was prescribed after failure of high-dosage corticosteroid treatment in six, and of both corticosteroids and interferon alpha 2b (5 months) in one; two infants received VCR as first line treatment. RESULTS: A dosage of 1 mg/m(2) IV injection was delivered, with weekly injections first, and then tapering, increasing the interval between injections, depending on the clinical response. The nine infants received from 5 to 25 injections (average: 16), for a length of treatment of 1.5-8 months (average: 5.5 months). In seven patients a clear clinical response was observed at the end of the first month of treatment, while a slow protracted response was noted in two. Transient mild side effects were present in four patients. DISCUSSION: Corticosteroid treatment, although a worldwide recognized treatment of problematic hemangiomas, cannot always control the growth of alarming hemangiomas. Interferon alpha 2a and 2b have proven a 90% effectiveness: treatment for cortico-resistant, function- and life-threatening, hemangiomas.

[Pulmonary valvulotomy with the Nykanen radiofrequency guide in pulmonary atresia with intact interventricular septum]. / Valvulotomie pulmonaire avec le guide à radiofréquence Nykanen dans l'atrésie pulmonaire à septum interventriculaire intact.

We report 2 cases of pulmonary atresia with intact interventricular septum where perforation of the pulmonary valve was performed using a new Nykanen radiofrequency guide from Bayliss. The valvulotomy was successful in the two neonates, but one required secondary surgery to enlarge the pulmonary infundibulum. Moreover, in one of the two cases, this anomaly was associated with a right aortic arch without systemico-pulmonary vessels, which is a rare combination in this pathology. This technique is effective and safe, and represents a good alternative to open surgery in the forms with tripartite right ventricle and without coronaro-cardiac fistulae.

[Important of magnetic resonance angiography with gadolinium injection in pulmonary vein diseases]. / Intérêt de l'angiographie par résonance magnétique avec injection de gadolinium dans le bilan des pathologies des veines pulmonaires.

The study of the pulmonary veins by echocardiography is sometimes difficult especially when the ultrasonic window is restricted. Conventional angiography is the classic reference examination but it exposes the patient to ionising radiation and requires the injection of an iodine contrast product. Another technique that can provide the essential information is magnetic resonance angiography (MRA) with injection of gadolinium. It was performed in 9 patients for suspected congenital or acquired anomalies of the pulmonary veins between June 1999 and December 2001. The patient's ages varied from 1 month to 10 years. The examinations were carried out on a 1.5 T Vision machine (Siemens, Erlangen, Germany) without "cardiac gating" or apnoea after parental consent. MRA with gadolinium injection showed 5 drainage anomalies (3 partial pulmonary venous refluxes in the superior vena cava, 2 scimitar syndromes) and 3 stenoses (one due to compression by an aneurysm of the left pulmonary artery, a second secondary to pericardial agenesis, and a third secondary to hypoplasia of an isolated vein). MRA allowed three dimensional visualisation of these anomalies. This is a rapid, non-invasive and certain imaging technique which does not expose the patient to ionising radiation. It is therefore of significance in the investigation of anomalies of the pulmonary veins complementing echocardiography, and could in future replace cardiac catheterisation.

[Percutaneous closure of patent ductus arteriosus with the Amplatzer duct occluder. Results of 29 patients]. / Fermeture percutanée du canal artériel avec le dispositif Amplatzer duct occluder. Série de 29 patients.

The authors report their experience of percutaneous closure of large patent ductus arteriosus with the Amplatzer duct occluder. The age of the patients ranged from 3 months to 72 years (average 24 months) with body weights of 3.9 to 74 Kg (average 16 Kg). The smallest angiographic diameter of the ductus was 4.0 +/- 1.3 mm at angiography (range 1.8 to 6 mm). The occluder was inserted under local anaesthetic with control aortography in all but one case who required general anaesthesia. In two small babies, the implantation was performed after venous puncture alone with transthoracic echocardiographic monitoring. The implantation was successful in all but one case in which there was a distensible duct. Early angiographic control showed suppression of the shunt in 61% of patients. Secondary haemolysis was observed in one case and was corrected by a second catheterisation 4 days later, with occlusion of the duct with a balloon catheter. The patients were followed up clinically, radiologically and echocardiographically. Doppler echocardiography showed absence of a residual shunt in 24 patients (86%) one month after the procedure, in 25 patients (89%) 3 months after the procedure and in 26 patients (93%) one year after the procedure. Two patients were left with mild or moderate shunts 12 and 16 months after the attempted occlusion. No cases of aortic or pulmonary obstruction were observed. The authors conclude that the Amplatzer duct occluder is a reliable device for closing large patent ductus arteriosus. It may be proposed in young symptomatic children over 4 Kg in body weight. A high rate of occlusion is obtained with a minimal risk of complications.

[Left monoplegia revealing pulmonary arteriovenous fistual in an adolescent: occclusion with an Amplatzer occluder]. / Monoplégie gauche révélatrice d'une fistule artérioveineuse pulmonaire chez un adolescent: occlusion avec un bouchon d'Amplatz.

A 16 year old adolescent was admitted to hospital for investigation of a transient left monoplegia. The clinical, electric and radiological examinations were all normal. Echocardiography showed no abnormality of cardiac structures or function. Contrast echocardiography was performed and showed a right-to-left shunt suggesting a pulmonary arteriovenous fistula which was confirmed at angiography. The fistula was closed at catheterisation by a detachable balloon: in a second procedure, the balloon was deflated and contrast studies showed a recurrence of the right-to-left shunt. An Amplatzer 8/6 duct occluder was implanted with excellent results and no recurrence at 3 months.

Experience in one centre using the buttoned device for occlusion of atrial septal defect: comparison with the Amplatzer septal occluder.

We report our experience using the buttoned device to close defects within the oval fossa and probe-patent oval foramens, comparing the findings with those obtained with the Amplatzer septal occluder. From 1992 to 1997, we used the buttoned device to close defects in 73 consecutive patients, 64 with defects in the oval fossa and nine with patent foramens. We compared this experience with a further series of 62 patients seen from 1997 to 1999 in whom the Amplatzer septal occluder was used. Successful implantation was achieved in three-quarters of those with septal defects in whom the buttoned device was used, in all of those in whom the buttoned device was used for patent foramens, and in nine-tenths of those in whom closure was attempted using the Amplatzer occluder. Immediate surgery was needed in 3 patients in whom a buttoned device was used, one because of embolization and two with residual shunts and a straddling device. Similar immediate surgery was needed to retrieve one embolized Amplatzer occluder. During follow-up, surgery was needed in a further 7 patients, all having had insertion of a buttoned device, because of atrial perforation in one and a significant residual shunt in the remainder. At late follow-up, the rate of complete occlusion was 69% in the patients in whom the buttoned device was used to close a septal defect, 100% when the buttoned device was used for patent foramens, and 95% in those treated with the Amplatzer occluder. Our experience shows that the Amplatzer occluder produced a significantly higher rate of occlusion for larger defects, and with a shorter fluoroscopy time than the buttoned device. The Amplatzer septal occluder, therefore, is our preferred device for closure of defects within the oval fossa.

[Long-term results of anatomic repair of transposition of great vessels]. / Résultats à long-terme de la réparation anatomique d'une transposition des gros vaisseaux.

The long-term complications after anatomical repair of transposition of the great arteries (TGA) were analysed in a prospective study of 30 successive patients, from August 1996 to October 1999, who were presumed asymptomatic and investigated 10 years after surgery. All underwent clinical examination, ECG, stress Thallium 201 myocardial scintigraphy, Doppler echocardiography, Holter ECG, pulmonary perfusion scintigraphy, right and left cardiac catheterization with selective coronary angiography. Five patients had coronary lesions (4 thromboses and 1 coronary-pulmonary artery fistula). The other abnormalities observed were mild bilateral stenosis of the two pulmonary arteries (1 case), grade 1 aortic regurgitation (6 cases), including 1 case of aortic root dilatation. Type B to E coronary circulations (Yacoub classification) were not significantly correlated with coronary artery disease in this series (p = 0.06). For the diagnosis of these lesions, myocardial scintigraphy and Doppler echocardiographic detection of wall motion abnormalities had a sensitivity of 50% and respective specificities of 88% and 35%. Long-term results after anatomical repair of TGA are satisfactory. However, the high incidence of coronary lesions makes regular follow-up and systematic coronary angiography necessary in all children.

[Severe arterial hypertension and pheochromocytoma in childhood. Case report and review of the literature]. / Hypertension artérielle menaçante et phéochromocytome chez l'enfant. A propos d'un cas et revue de la littérature.

Pheochromocytoma was diagnosed in a 14 year old boy twenty-four months after the onset of symptoms. The diagnosis was made during a severe hypertensive crisis. Previously, the child had been followed up for delayed growth and functional psychiatric symptoms. After investigation, the tumour was removed by a trained surgical team. Pheochromocytoma usually arises from the adrenal gland and secretes catecholamines. It is less common in children than in adults. The presentation is very variable with hypertension as the main sign. The diagnosis is based on the finding of raised urinary catecholamines and CT scanning with scintigraphy for localising the tumour. These cases should be managed by teams familiar with this pathology because of the risk of catecholamine discharge, the need for complementary investigation of associated disease, the medical preoperative preparation and the specificity of the surgical intervention.

[Arm ischemia revealing Kawasaki disease]. / Ischémie de la main révélant une maladie de Kawasaki.

UNLABELLED: An aneurysm of the medium arteries is one of the major complications of Kawasaki syndrome. Eleven cases of giant coronary aneurysm have been reported. CASE REPORT: We report a new case in a nine-month-old child. Ischemia of the left hand was the first symptom. Diagnosis was made 30 days later. Anticoagulant and fibrinolytic treatments did not prevent necrosis of four fingers. CONCLUSION: The prognosis of Kawasaki disease depends on early diagnosis and management. Infants younger than seven months of age are more susceptible to developing coronary and midartery aneurysms, which therefore justifies a close observation during the acute phase and later on. In the case of a coronary artery aneurysm, Doppler echography of the upper and lower limbs is mandatorily recommended. Anticoagulant therapy should be started once the diagnosis of mid-artery aneurysms is made. Ischemia of the extremities can lead to necrosis and amputation. Until now, anticoagulants, vasodilators and fibrinolytic agents were not proven to be effective.

[Teleradiographic analysis of velopharyngeal insufficiency in CATCH 22 association]. / Analyse téléradiographique de l'insuffisance vélo-pharyngée dans l'association CATCH 22.

The CATCH 22 association (Cardiac defect, Abnormal facies, Thymic hypoplasia, Cleft palate, Hypocalcemia) combines certain signs common to various syndromes (Di-George syndrome, velocardiofacial syndrome, etc.) in combination with a 22q11 chromosomal microdeletion. Velopharyngeal insufficiency can be a complementary sign, or even a presenting sign of this chromosomal anomaly. After clinical examination of these patients, velopharyngeal insufficiency appeared to be due to a deep nasopharynx rather than to a short soft palate. We therefore reviewed 11 patients with teleradiography. We studied the length of the soft palate, the depth of the nasopharynx, the ratio of the depth of the nasopharynx over the length of the soft palate, and we compared our results with those observed in reference populations. The results demonstrate nasopharyngeal disproportion with a normal length of the soft palate and increased depth of the nasopharynx. This anatomical finding therefore encourages us to propose sphincteroplasty rather than velopharyngoplasty.

[Giant multifocal venous malformation with monomelic predominance]. / Malformation veineuse multifocale majeure à prédominance monomélique.

INTRODUCTION: Venous malformations are usually easy to recognise. We describe one case in which the clinical aspect was suggestive of Maffucci's syndrome. OBSERVATION: A 44-year-old male, had numerous angiomatous nodules which could be emptied by pressure since infancy. The progressive increase in volume of these lesions on the arms and forearms led to enormous deformation and major disability. The thoracic area and the right foot were also affected to a lesser degree. Two endobuccal lesions were also found on the clinical examination The cutaneous lesions were tender and occasionally hyperhidrosis was present. The presence of phleboliths on Xray and the presence of venous and capillary cavities with numerous thrombi confirmed the diagnosis of venous malformation. Following the failure of the Ethibloc(R) embolization, eight excisions were made in a two-year-period with a good functional result. Elastic strapping was then applied. DISCUSSION: The diagnosis of venous malformation was based on clinical, radiological and histological findings. The absence of chondroma excluded the diagnosis of Maffucci's syndrome. Spindle cell hemangioendothelioma, "blue rubber bleb nevus" and glomangiomatosis were confirmed by the histological findings. The absence of port-wine stain or bone hypertrophy and the presence of multiple nodules excluded Klippel-Trenaunay syndrome. The presence of multifocal involvement with oral lesions, the severity of the deformation and the relative success of the surgical procedure make this case unusual.