[Solupred in pediatrics].
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Administration, Oral↗
Biomedical subjects
O Enjolras
Publications and source records attributed to O Enjolras.
Percutaneous sclerotherapy for venous malformation of the lips: a retrospective study of 23 patients.
Labial venous malformations are relatively common. Depending on their size, they are responsible for functional and cosmetic handicap. When treatment is indicated, it will be based on percutaneous sclerotherapy, using Ethibloc or Aetoxysclerol, with surgery in some patients. Our purpose was to review 23 patients with soft-tissue venous malformations of the lips. Follow-up ranged from 6 months to 4 years. Sclerotherapy alone or with surgical resection achieved good results in 18 patients. In six patients mild improvement was obtained. No worsening of the initial clinical situation occurred, and no persistent complication was observed. We discuss the indications for treatment and the different types of sclerosing agent. Percutaneous sclerotherapy is safe and is effective for small and medium-size labial malformations. For larger lesions the treatment is more complex and combined long term sclerotherapy and surgery procedures may be required over several years.
Adolescent↗
Residual lesions after Kasabach-Merritt phenomenon in 41 patients.
BACKGROUND: Kasabach-Merritt phenomenon (KMP) is the association of a vascular tumor and thrombocytopenic coagulopathy. Vascular tumors are either kaposiform hemangioendothelioma or tufted angioma but not "true" common hemangioma of infancy. There is a conspicuous absence in the literature regarding the late outcome and possible residual lesions after apparent clinical cure of KMP. OBJECTIVE: The purpose of the study was to analyze these residua in a large number of patients. METHODS: Clinical data on 41 patients who had KMP were accrued in an international cooperative study. The emphasis was on the residual lesions after resolution of the thrombocytopenia and other coagulation abnormalities. Imaging studies (follow-up magnetic resonance imaging studies available for 10 patients) and histologic specimens (30 specimens available for 26 patients, 18 biopsies done during the KMP and 12 concerning the sequelae) were reviewed. RESULTS: Residual lesions after "cure" of KMP were common. They exhibited 3 clinical patterns: type I lesions (n = 28) showed a cutaneous red stain, with or without associated red papules. The stain might overlap a minor fibrotic infiltration or a significant poorly delineated diffuse fibrotic infiltration. These cutaneous vascular lesions varied in size and appearance over time and were occasionally painful. Type II lesions were telangiectatic streaks and swelling (n = 5), and type III lesions showed a minor, firm, irregular, subcutaneous mass assessed by palpation or deep infiltration evidenced by computed tomography or magnetic resonance imaging (n = 8). A fourth feature was sequelae in muscles and/or joints. Histologically, tufted angioma was more common in the specimens from residual lesions, whereas kaposiform hemangioendothelioma was more common during the active phase of KMP. Imaging findings were remarkably reproducible and revealed a persistent vascular tumor. CONCLUSION: Residua of tumors associated with KMP are common after the resolution of thrombocytopenia and coagulopathy. They are (more or less) prominent dormant vascular tumors, not "scars" and, clinically as well as histologically, they differ markedly from involuted hemangioma.
Adolescent↗
Vascular tumors and vascular malformations: evidence for an association.
BACKGROUND: The International Society for the Study of Vascular Anomalies classification is an updated biologic classification that distinguishes vascular tumors from vascular malformations on the basis of clinical and histologic characteristics and biologic behavior. OBJECTIVE: We propose that in a minority of cases there is an association between vascular tumors and vascular malformations. METHODS: We retrospectively identified clinical cases in our medical centers and others reported in the medical literature that demonstrate an association between vascular tumors and vascular malformation clinically or histologically. RESULTS: We identified several clinical or histologic settings in which vascular tumors and vascular malformations were associated: (1) coexistence of hemangioma and vascular malformation at the same anatomic site or in close proximity; (2) pyogenic granuloma arising within a vascular malformation; (3) hemangioma associated with developmental vascular anomalies; (4) spindle-cell hemangioendothelioma and venous malformation; (5) kaposiform hemangioendothelioma and lymphatic malformation. CONCLUSION: The biologic classification of vascular birthmarks distinguishing vascular tumors from vascular malformations is clinically useful and forms the framework for our understanding of vascular anomalies. These examples suggest that in a small minority of patients there is an association between vascular tumors and vascular malformations.
Cardiovascular Abnormalities↗
[Effectiveness and dangers of interferon-alpha in the treatment of severe hemangiomas in infants].
UNLABELLED: Alarming hemangiomas, due to their site or repercussions, require pharmacological treatment. Corticosteroid therapy is indicated by first intention. In the event of failure, interferon alpha is proposed. CASE REPORTS: Case 1. A five-week-old infant was admitted to hospital for an extensive hemangioma of the left side of the face and neck with necrosis of the upper lip and ear. Prednisolone (2 mg/kg/day) by intravenous route brought about no improvement. Interferon alpha 2a (3 MU/m2/day of Referon by subcutaneous injection) enabled regression of lesions from the sixth month of treatment. After 11 months of treatment, the hemangioma had all but disappeared and interferon therapy was stopped. Repair surgery was planned at 24 months of age. Case 2. A one-month-old infant suffered from a hemangioma of the right side of the face with orbital invasion and risk of amblyopia. Prednisone (2 mg/kg/day) by oral route was ineffective. Interferon alpha 2a enabled regression of the hemangioma and the eye opened from the third month of treatment. Interferon therapy was stopped after 14 months. Initial repair surgery intervention was possible at two years of age. Spastic paraplegia was diagnosed at 18 months of age. The brain and medullar magnetic resonance imaging was normal. No etiology could explain the neurological attack. The possible toxic effect of interferon alpha is discussed. CONCLUSION: Interferon alpha is an effective treatment for hemangiomas. It significantly reduces spontaneous regression time. The uncertainty of long-term effects in infants with hemangiomas incites its indication to be limited to alarming corticosteroid-resistant forms and necessitates prolonged neurological surveillance.
Amblyopia↗
[Arteriovenous malformations: a study of 200 cases].
OBJECTIVE: The aim of this study was to analyze the natural history of arteriovenous malformations by reviewing 200 consecutive cases observed in all localizations between 1992 and 1996 in a multidisciplinary angioma clinic. PATIENTS AND METHODS: Files concerning 200 arteriovenous malformations were reviewed with a standardized observation sheet applying the severity criteria defined by the International Society for the Study of Vascular Anomalies. We used the Schobinger staging system which includes 4 grades of severity: grade I=dormancy, grade II=expansion, grade III=destruction, grade IV=cardiac decompensation. RESULTS: There was no predominance by gender. Cephalic localizations were the most common. The malformation was present at birth as indicated by history taking in 40 p. 100 of the cases. Progression during childhood (grade II) was observed in 84 p. 100. At the first consultation, the patients generally were more often in grade II than in grade I or III. Bone destruction was observed in 3 cases, signs of cardiac decompensation in 5. Arteriovenous malformations were part of a more complex syndrome in 9 cases. We noted a flare-up in the prepuberty or puberty period in 75 p. 100 of the cases and the possible role of puberty (64 cases), trauma (39 cases) and pregnancy (25 p. 100 of the adult women). Explorations were generally completed (Doppler, arteriography) by grade II, expressing the need for a map of expanding lesions. Finally it was difficult to assess posteriorly the beneficial or deleterious effect of the often multiple treatments prescribed for these patients. DISCUSSION: The review of these 200 cases pointed out the "pediatric" nature of the problem of arteriovenous malformations in the large majority of the cases and the often misleading presentation of these vascular anomalies, particularly grade I malformations. A false aspect of capillary malformation could raise the risk of inappropriate treatment. This review also confirmed known factors of exacerbation (puberty, pregnancy, trauma) and demonstrated the severity of these vascular anomalies which can progress with loco-regional expansion and invasion without a cellular proliferation component. The analysis of treatments used showed that embolization alone cannot definitively and totally control a superficial arteriovenous malformation and that no one treatment, even combined embolization and large excision, can be a guarantee to provide total cure.
Adolescent↗
[Sturge-Weber syndrome. The current neuroradiologic data].
Sturge-Weber syndrome (SWS) is a rare congenital sporadic disease with neuro-ocular and cutaneous vascular findings. Clinically, the full-blown condition consists of a facial port-wine stain (PWS) involving the V1 facial trigeminal skin area, alone or in combination with V2 and V3PWS, seizures and ocular abnormalities (glaucoma and choroidal angioma). Radiologically, a leptomeningeal (pial) capillary and venous malformation, mostly located in the parieto-occipital area, cerebral atrophy and calcifications are demonstrated. An ipsilateral enlarged choroid plexus may be an early anatomic symptom. Developmental venous anomalies (DVA) of the brain are sometimes associated. MR with gadolinium enhancement is the optimal neuro-diagnostic imaging technique for the screening of infants with an at-risk V1PWS, as well as for the follow-up of patients with evidence SWS. Accelerated myelination in the involved hemisphere may be an early diagnostic feature before 6 months of age. Later, hyperintensity of white matter on T2 is considered a symptom of gliosis. Clinically, progression of the diseases is associated with anatomic changes and correlates with the extent of the pial vascular anomaly, extent and severity of cerebral atrophy, and white matter abnormalities. A neonatal neuro-imaging work-up, using CT or MRI, may not demonstrate the pial anomaly and should be repeated after 6 to 12 months in an at-risk infant with V1PWS.
Brain↗
Cultured endothelial cells from human arteriovenous malformations have defective growth regulation.
Vascular malformations are frequent in newborns, and they persist throughout life, which differentiates them from vascular tumors (eg, hemangiomas). Arteriovenous malformations are high-flow vascular malformations. They are considered nonmalignant but can expand and become a significant clinical risk when extensive. To characterize endothelial cells from arteriovenous malformations (AMEC), we cultured cells obtained from surgical specimens and studied their properties. After selection, the cells that grew out from explants had phenotypic and antigenic features (platelet endothelial cell adhesion molecule, von Willebrand factor) of human endothelial cells. Their spontaneous proliferation rate was higher (1.8 to 6.4 times) than that of human umbilical vein, arterial, or microvascular endothelial cells. The proliferation rate of AMEC was not sensitive to the inhibitory activity of various cytokines (interleukin-1beta, tumor necrosis factor-alpha, transforming growth factor-beta, Interferon-gamma). In basal conditions, intercellular adhesion molecule (ICAM-1) was detected at a higher level of expression (6- to 10-fold) on AMEC, but these cells failed to express E-selectin or the vascular cell adhesion molecule (VCAM-1) after cytokine stimulation. Expression of c-ets-1 proto-oncogene was shown by in situ hybridization. The low response to cytokines, the higher propensity to proliferate, and the ets-1 expression suggest that AMEC have a defective regulation of proliferation that may be due to a reduced apoptotic process.
Adolescent↗
An association between autosomal dominant cerebral cavernomas and a distinctive hyperkeratotic cutaneous vascular malformation in 4 families.
Cerebral cavernomas (CCMs) are vascular malformations that may be inherited as an autosomal dominant condition for which a gene, CCM1, was mapped to chromosome 7. Poorly defined cutaneous malformations were sometimes described in association with CCMs. During a national survey, 57 French CCM families were studied. Co-occurrence of CCMs and a distinctive cutaneous vascular malformation was observed in 4 families. Ten individuals belonging to these families showed similar hyperkeratotic cutaneous capillary venous malformations (HCCVMs). In 3 families, the histology showed orthokeratosis and hyperkeratosis as well as dilated capillaries in the dermis extending to the hypodermis and confirmed the diagnosis of HCCVM. Genetic analysis strongly supports linkage of these families to the CCM1 locus on chromosome 7. The HCCVM seems to be a peculiar cutaneous vascular malformation associated with CCMs. These data strongly suggest that HCCVMs and CCMs in these families are due to the same genetic abnormality.
Adult↗
[Angiomas in children at a turning point of understanding and management].
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Age Factors↗
[Superficial vascular malformations: classification and histopathology].
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Arteriovenous Malformations↗
[52 patients with cystic lymphatic vascular malformations. Percutaneous sclerotherapy--simple, fast and repeatable].
Cystic lymphatic malformations are haemodynamically inactive vascular malformations that never regress. During a nine-year period, 52 patients with cervico-facial superficial cystic lymphatic malformations underwent percutaneous sclerotherapy with Ethibloc (a corn-based alcohol derivative). In 57 percent (30/52) of cases, the outcome of sclerotherapy alone was excellent or good: in 19 percent (10/52), the procedure had to be combined with surgery for aesthetic reasons; and in the remaining 24 percent (12/52), the treatment failed. The results suggest percutaneous sclerotherapy with Ethibloc to be safe and effective, and that it is to be recommended as the treatment of choice in cases of superficial cystic lymphatic malformations.
Adult↗
Therapeutic approach to arteriovenous malformations of the tongue.
Arteriovenous malformations (AVM) are developmental errors with a high complication rate because they are hemodynamically active. The aim of our study is to evaluate management of the rare subgroup of tongue AVM. From 1982 to 1994, 25 patients with AVM of the tongue presented to our department. All patients were discussed in our multidisciplinary staff consultation and were treated by embolization, surgery, or were followed-up clinically with no intervention. Thirteen patients were asymptomatic in a hemodynamically quiescent phase and did not require any kind of intervention. The 12 others underwent embolization procedures following which 6 became stable, 2 had complementary surgery, 1 was lost to follow-up, 1 had two minor bleeding episodes without the need for hospitalization, and 2 were improved. Arteriovenous malformations of the tongue must be followed clinically and treated only if they become active. Permanent embolization with a polymerizing liquid administered through supra-selective catheterization or by direct puncture of the malformation is recommended.
Adolescent↗
Neurotoxicity of interferon alfa in children treated for hemangiomas.
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Antineoplastic Agents↗
[Absence of HHV-8 virus detected in immature hemangiomas in infants].
INTRODUCTION: The aim of our study was to search for the presence of HHV-8 DNA sequences in Biopsy specimens from hemangioma of the infancy. MATERIAL AND METHODS: The study included 9 biopsies from hemangioma. DNA of human beta-globin gene and HHV-8 were searched for by PCR using specific primers. Amplified products were revealed after an hybridization with an internal probe digoxigenin-labelled. RESULTS: Human beta-globin gene could be detected in all samples illustrating the absence of PCR inhibitor. HHV-8 could never be detected in samples analyzed. DISCUSSION: Our study does not imply any causative role of HHV-8 in the pathogenesis of hemangioma. This result must be confirmed by serologic studies.
Biopsy↗
[Kasabach-Merritt syndrome on a congenital tufted angioma].
INTRODUCTION: Kasabach-Merritt syndrome is a very rare disease of infancy, with profound thrombocytopenia and a mild to severe consumption coagulopathy; this biological phenomenon is difficult to control. CASE REPORT: A 1-month old boy had a congenital plaque-like lesion in the calf. It was a biopsy-proven tufted angioma. Five weeks later, Kasabach-Merritt syndrome developed. After failure of ticlopidine + aspirin, and oral betamethasone treatment, thrombocytopenia was cured with vincristine treatment, then the leg lesion slowly continued to shrink after cessation of the treatment. It had disappeared before the age of 1 year. DISCUSSION: We highlighted two points: 1) Kasabach Merritt does not appear as a complication of a classic hemangioma (infantile, "cellular", "capillary", involuting-type), as it has long been thought. In our experience, it develops on a different endothelial cell proliferation, in this case a congenital tufted angioma, but it can also engraft on a kaposiform hemangioendothelioma. 2) These patients are difficult to treat because, up to now, no single treatment has given constant by good results. Vincristine was recently introduced in the treatment of Kasabach-Merritt syndrome, with excellent, rapid outcome. CONCLUSION: What seems a therapeutic progress in a difficult field needs further control.
Antineoplastic Agents, Phytogenic↗
[Importance of bFGF ("basic fibroblast growth factor") for diagnosis and treatment of hemangiomas].
OBJECTIVES: Hemangiomas of infancy follow a characteristic three-phases course: proliferation, involution, regressed Proliferative endothelial cells predominate during the proliferative phase. Moreover it has been shown that patients with active angiogenesis have elevated levels of urinary bFGF (basic Fibroblast Growth Factor). PATIENTS AND METHODS: Here we report our preliminary results of urinary bFGF assay (ELISA) for the diagnosis and follow up of severe hemangioma. We also assayed bFGF in normal infants, in patients with large vascular malformations and in infants with Kasabach-Merritt syndrome. RESULTS: In the control group, urinary bFGF was elevated in new borns but nul or very low in infants. Urinary bFGF levels were normal, i.e. very low in 4 patients with a vascular malformation. In infants with a clinically proliferative hemangioma, urinary bFGF was elevated in 8 among the 10 studied. bFGF levels guided treatment in 9 patients. Urinary bFGF was elevated in 4 patients with Kasabach-Merritt syndrome. DISCUSSION: Angiogenesis is regulated by angiogenic and inhibitory factors. The angiogenic factor bFGF is an autocrine growth factor for endothelial cells and hemangioma endothelial cells expressing bFGF in their cytosol during the proliferative phase. As suggested by J. Folkman and his group, assay of urinary bFGF appears useful in differentiating between hemangioma and vascular malformation and for follow up of treated patients.
Adrenal Cortex Hormones↗
[Maffucci syndrome: a false venous malformation? A case with hemangioendothelioma with fusiform cells].
BACKGROUND: Maffucci syndrome occurs as a sporadic disease. Progressive onset of both cutaneous vascular lesions (considered to be of venous type) and bony enchondromatous tumors (similar to those seen in Ollier disease) occurs throughout childhood. We report a case of a woman with Maffucci syndrome whose cutaneous vascular lesions revealed spindle cell hemangioendothelioma. CASE REPORT: An italian woman developed severe bone distortion and dwarfism due to multiple enchondromas, first diagnosed as Ollier disease during childhood. At puberty, multiple vascular nodules appeared mainly on the limbs, leading to the diagnosis of Maffucci syndrome. Clinical data suggested the diagnosis of cutaneous venous anomalies: blue color of some nodules, phleboliths, arteriographic pattern. Histopathological examination of the skin specimen showed features of spindle cell hemangioendothelioma, e.g. nodules of dense spindle cell infiltration in combination with dysplastic vessels. DISCUSSION: The few reports available do not clearly evidence the underlying histopathology progression of the lesions over years in a given patient. Skin lesions are classified among venous malformations. Bony enchondromatous involvement of the limbs is common and reported in 9 out of 10 patients. Both vascular and bony lesions classically develop from childhood to adulthood. Spindle cell hemangioendothelioma is a vascular tumor recently described. Cellular spindling in association with vascular spaces must not be misdiagnosed as Kaposi sarcoma. Some of the reported cases of spindle cell hemangioendothelioma had Maffucci syndrome. It is unknown whether Maffucci syndrome occurs in association with venous malformation or whether it is always present in the cutaneous vascular lesions of the disease.
Adult↗