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Biomedical subjects

O Enjolras

Publications and source records attributed to O Enjolras.

At least 55 records · Page 3Linked to original sources
PubMed1998

[Cutaneous malacoplakia: a pediatric case].

INTRODUCTION: Cutaneous malakoplakia is an inflammatory disease characterized by granulomatous accumulation of distinctive phagocytic macrophages. It occurs mainly in visceral or orificial areas; the condition is rarely purely cutaneous, and appears to be extremely rare in childhood. CASE REPORT: A facial cutaneous crusted lesion was diagnosed as cutaneous malakoplakia in an immunocompetent child. The lesion had been excised twice and it had recurred, and the diagnosis was made possible only with a third biopsy, after a 2-year chronic expansion. This third biopsy revealed a dense granulomatous inflammation with numerous phagocytic histiocytes containing abundant fine granules and round Michaelis-Gutmann bodies, both staining with PAS, Perls and von Kossa. Biopsy cultures revealed only growth of two different streptococcus (group B) strains. The lesion resolved after a 4-month period of antibiotic therapy, including roxithromycin, ampicillin and trimethoprim-sulfamethoxasole. DISCUSSION: Diagnosis of malakoplakia is mainly made by histopathologic examination of tissue excision or biopsies. There are no specific clinical features. Most reported cases of this uncommon phagocytic reaction to common bacteria have developed in the genitourinary areas (71 p. 100); purely cutaneous localisation, as in our patient, are rare (4 p. 100). Intracytoplasmic granules may result from phagolysosomes and incomplete bacterial killing, with subsequent deposit of iron and calcium in the phagocytic macrophages. A number of reported cases have affected immunocompromised patients with either congenital immunodeficiency or secondary immunodeficiency. The most effective treatment option is based on a protracted antibiotherapy, using drugs that easily permeate the macrophages, e.g. quinolones and trimethoprim-sulfamethoxasole. Lesion may recur after surgical excision.

Biopsy↗
PubMed1998

[Sinusoidal hemangioma].

INTRODUCTION: Sinusoidal hemangioma (SH) was described by two pathologists, Calonje and Fletcher, in 1991. This uncommon cutaneous benign vascular lesions with distinctive histologic features was considered a subset of lesions known as "cavernous hemangiomas". It is made of dilated interconnecting ("sinusoidal"), thin-walled channels with a single-layered lining endothelium and a lobular architecture. PATIENTS AND METHODS: Clinical, radiological (CT, MRI and arteriography), evolutive, and histologic data of 4 patients, whose lesions appeared very similar clinically and histologically, were reviewed and compared to the patterns of the so-called SH. RESULTS: There were 2 males and 2 females. Lesions had an infantile onset in 3 (congenital in 2). They exhibited a protracted course over years in the 4, and they all invaded the right forehead, eyelids and orbit areas. Clinically, they appeared as multilobulated, building, firm tumors under a normal shiny skin. Radiologically, lesions were strikingly nodular, with criteria of slow-flow vascular lesions on MRI and arteriograms. Histologically, all lesions were comprised of well-circumscribed nodules with a lobulated architecture, made of greatly dilated, blood-filled, thin-walled vascular channels, with scanty fibrous stroma between them. Interconnecting channels had the distinctive sinusoidal pattern. There were some elongated pseudopapillary structures. Islands of spindle cells were noted in 2 lesions. DISCUSSION: On the one hand histologic features of the lesions in our 4 patients are identical to those described by Calonje and Fletcher as SH, a lesion not to be confused with classic, involuting-type, hemangioma of infancy and childhood, and with venous or lymphatic malformations of the same location. SH fits into the group of lesions defined by pathologists as "cavenous hemangiomas". On the other hand the lesions in our 4 patients differed from the clinical description of SH as a solitary acquired small (less than 3 cm) nodule in adults. Otherwise the four lesions described herein constitute a distinctive clinicopathologic entity with a striking aggressive local growth although there is a histologic malformative pattern. The presence in 2 cases of islands of spindle cells led us to discuss the differences with spindle cell hemangioendothelioma.

Adolescent↗
PubMed1998

[Sclerosing tufted angioma. Apropos of 4 cases involving lower limbs].

BACKGROUND: Tufted angioma belongs to the family of vascular skin proliferations. Clustered capillaries is the characteristic histological feature. Classically, tufted angioma is an acquired disease observed in children or young adults with lesions predominating on the neck, the shoulders and the upper trunk. CASE REPORTS: Four children were seen for flat skin lesions which were either initially or secondarily erythematous. The lesions were observed at birth or within the first weeks of life. In all 4 cases, the lesions progressed by infiltration, leading to sclerous plaques within a few weeks. The hip area was involved in case n(o) 2 and the thigh in the other cases. Pathology reported tufted angioma. A repeated biopsy 4 years after the first biopsy demonstrated the progressive nature of the fibrosis in case n(o) 2. Magnetic resonance imaging, performed in 3 of the 4 cases, demonstrated deep penetration of the vascular proliferation. The lesions were in contact with bony structures in case n(o) 2, reached the hypodermis in case n(o) 3 and infiltrated between the adducter muscles in case n(o) 4. DISCUSSION: Among tufted angiomas, there is a clinical variant characterized by very early discovery (congenital or within the first months of life), preferential localization on the lower limbs and progression to sclerosis. Although non-specific, hypersudation and lanuginous hypertrichosis are also highly suggestive signs. The main clinical and histological differential diagnosis is angio-eccrine hamartoma and, to a lesser degree, Kaposi hemangioendothelioma which does not predominate on the lower limbs. This particular clinical presentation of tufted angioma should lead to wide and sufficiently deep biopsy to allow precise diagnosis prior to initiating therapy. The possibility of spontaneous involution would allow abstention with regular surveillance to detect reactivation and progression or functional impairment. Such unfavorable progression has lead certain authors to propose high-dose systemic corticosteroid therapy or alpha-interferon.

Biopsy↗
PubMed1998

[Vascular "tumors" and the rules of their surgical management].

After defining vascular tumors and malformations, formerly called angiomas, the authors adopt the classification of the International Society for the Study of vascular Anomalies. The various groups of malformations, after definition of the subject and the problems raised, are illustrated by a number of clinical cases and the rules of surgical management are discussed. Capillary malformations, formerly called portwine stains, can be treated by dye laser, but sometimes tissue and orthognathic reconstructive surgery in the presence of a skeletal malformation. Lymphatic malformations (lymphangiomas), optimally assessed by CT or MRI, can be treated by a variety of approaches: sclerotherapy, surgery. Venous malformations raise very different problems in the cervicocephalic region and on the limbs and trunk, but always require a multidisciplinary approach with, according to the site, size and repercussions, Ethibloc percutaneous sclerotherapy, embolization, surgery. Arteriovenous malformations require complete surgical treatment, usually preceded by embolization; reconstruction consists of local flaps or skin expansion in simple cases, and revascularized free flaps in difficult cases. If the malformation is cosmetically and functionally acceptable, the authors propose conservative management. The first-line treatment of haemangiomas is pharmacological (corticosteroids, interferon), but surgery may be indicated in two situations: early and late. There is a renewed interest in early surgery, subtended by several factors including cosmetic concerns and the development of new technologies, including the Cavitron. Late surgery retains its classical cosmetic and functional indications. Two key-words dominate the rules of therapeutic management of all types of vascular malformations: a multidisciplinary approach and modesty.

Adolescent↗
PubMed1997

Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas.

OBJECTIVE: In 1940 Kasabach and Merritt described an infant with a vascular anomaly, extensive purpura, and thrombocytopenia; they called his lesion "capillary hemangioma." Hemangioma is a benign tumor that grows in infancy and is characterized by proliferation of endothelial cells and regression during childhood. Although Kasabach-Merritt syndrome (KMS) is frequently mentioned as a possible complication of hemangioma, our experience suggests that the anatomic vascular lesion underlying the thrombocytopenia is not a "true," classic, involuting type of hemangioma of infancy and childhood. STUDY DESIGN: We reviewed the clinical and hemostasis data and the response to treatment in 22 cases of KMS, and we analyzed the biopsy specimens of 15 of them. RESULTS: Clinically none of the 22 patients had classic hemangioma. There was no female preponderance. All patients had severe thrombocytopenia (lowest platelet count = 3000/mm3) and consumption of fibrinogen. Histologically, none had the typical "capillary," involuting type of hemangioma of infancy: they exhibited either a tufted angioma or a kaposiform hemangioendothelioma pattern; all specimens also contained numerous abnormal lymphatic-like vessels; lymphatic malformation was the major component in two patients. The infants exhibited a heterogeneous response to a number of therapeutic regimens, as noted in other reports. Severe morbidity was present; three of our patients died, and one had leg amputation. "Residua" were, in fact, residual vascular neoplasia, variable in duration, and not a stable fibrofatty residuum, as in classic involuted hemangioma; only the hematologic phenomenon was "cured" after a period of years. CONCLUSIONS: KMS is a distinctive disease of infancy, but the underlying vascular lesion is not a "true," classic, involuting type of hemangioma of infancy. This is a different vascular tumor with a resemblance pathologically to either tufted angioma or kaposiform hemangioendothelioma in association with lymphatic-like vessels. Whether the underlying lesion in KMS is a single anatomic entity or heterogeneous cannot be definitely concluded from this study. We need a better understanding of the pathogenesis of KMS to improve our therapeutic management.

Biopsy↗
PubMed1997

Extensive pure venous malformations in the upper or lower limb: a review of 27 cases.

BACKGROUND: Extensive pure venous malformations (VMs) involving the entire lower or upper limb and adjacent trunk form a particular group of rare vascular malformations. OBJECTIVE: Our purpose was to review 27 cases of extensive limb VMs and describe their characteristics and management. METHODS: Cases of extensive limb VMs were investigated, treated, and observed for a mean of 7 years. RESULTS: Eleven cases involved the upper limbs and 16 involved the lower limbs. All involved skin and muscle. In 81% of cases in the lower limb there was also knee joint involvement that created severe functional impairment. Ultrasonography with Doppler (duplex scans), computed tomographic scans, and magnetic resonance imaging were helpful noninvasive diagnostic procedures in these patients, whereas arteriography and phlebography were less informative. Muscle involvement was present in 100% of patients and bone abnormalities in 63%. Leg length was either normal or there was slight limb undergrowth, except in three patients who had minor overgrowth of the affected limb. Coagulation profiles demonstrated localized intravascular coagulation in 88% of patients. The majority of patients had conservative management (elastic stockings). In a few, percutaneous sclerotherapy or partial excision of skin and muscle VMs was beneficial. Knee joint involvement required synovectomy and VM excision during childhood in 7 of 16 patients. CONCLUSION: Extensive limb VMs are characterized by diffuse involvement of the skin, muscle, and joints, and by a specific localized intravascular coagulopathy with general consequences. This group of vascular malformations should be separated from the Klippel-Trenaunay and Parkes Weber syndromes.

Abnormalities, Multiple↗
PubMed1997

Early single photon emission computed tomography in Sturge-Weber syndrome.

OBJECTIVES: Functional cerebral imaging PET and SPECT have shown hypometabolism and hypoperfusion in the area of vascular malformation in children with epilepsy due to Sturge-Weber syndrome. However, data are scarce in infants and do not exist in patients with Sturge-Weber disease without epilepsy. The pattern of perfusion during the first two years of life was studied including patients before the onset of seizures. METHODS: Twenty two infants with later confirmed Sturge-Weber disease underwent SPECT examination using TOMOMATIC 564 (Medimatic) and xenon-133 at ages ranging from 8 days to 25 months. Twelve had never had seizures before SPECT and seven underwent a second SPECT a mean seven months later. Cerebral blood flow (CBF) was measured in the whole hemisphere and in the part of the cortex involved in the vascular malformation on both sides as well as a "pathological to normal" index for the hemisphere and vascular malformation. These values were compared with normal age paired values. RESULTS: Compared with controls, CBF and the indices in the hemisphere and vascular malformation were significantly decreased in patients who already had had seizures before SPECT, whereas they were significantly increased in 75% of the patients who had never had any seizures. On second SPECT, the indices were decreased in all patients, including the four who still remained non-epileptic. CONCLUSIONS: SPECT therefore detects CBF asymmetry in infants with Sturge-Weber disease, which tends to shift with age. The cortex involved in the vascular malformation is hyperperfused during the first year of life before first seizures. The classic hypoperfusion appears after one year of age, even in non-epileptic patients.

Brain↗
PubMed1997

Vascular endothelial growth factor confers a growth advantage in vitro and in vivo to stromal cells cultured from neonatal hemangiomas.

Neonatal hemangioma is a common benign proliferation of unorganized structures containing stromal and capillary endothelial cells. We tested the hypothesis that such cell proliferation might result from the release by stromal cells of endothelial cell mitogens. Stromal cells cultured from biopsies of surgically removed life-threatening hemangiomas released an endothelial cell mitogen in vitro that was indistinguishable from vascular endothelial growth factor (VEGF) based on independent criteria such as affinity chromatography for heparin or anti-VEGF IgG and radioreceptor assay. A functional product of the KDR gene encoding a cognate VEGF receptor was also expressed by these stromal cells. Transient transfection with antisense oligonucleotides targeted on the translation initiation codon of KDR abolished its tyrosine phosphorylation and mitogenic response of neonatal hemangioma cells to VEGF, confirming the existence of an autocrine loop of proliferation. When grafted in nude mice, these stromal cells elicited an angiogenic response that was blocked by neutralizing anti-VEGF IgG. These results might provide a clue to the importance of stromal cells in the pathogeny of neonatal hemangiomas.

Animals↗
PubMed1997

Management of hemangiomas.

A majority of hemangiomas are harmless lesions and they regress spontaneously without significant sequelae. However, there are also distressing, endangering, and even life-threatening hemangiomas. For them, the outcome has improved over the last 10 years, and mortality figures have dropped due to promptness in treatment and to more effective therapeutic regimens. Parents need frequent consultations and support even when we decide not to treat the child, and pictures should be taken to follow the progress of involution.

Adrenal Cortex Hormones↗
PubMed1997

[Kasabach-Merritt syndrome of the leg associated with osteolysis or Gorham sign].

INTRODUCTION: Kasabach-Merritt syndrome and Gorham's sign are two uncommon and severe, sometimes life-threatening, complications in infants with vascular lesions. Their association has been described in rare cases. CASE REPORT: An infant with a vast congenital angiomatous lesion including an extensive lymphatic component, developed active regional osteolysis then suddenly suffered disseminated intravascular coagulation of the leg. Medical treatment was unsatisfactory. After unsuccessful use of low molecular weight heparin, pentoxifyllin and alpha interferon, amputation of the leg was required to avoid a fatal outcome. DISCUSSION: Kasabach-Merritt syndrome does not develop on classic immature hemangiomas, despite some contradictory statements in the literature. In our case, a complex tumor developed in association with a lymphatic malformation. The association of Kasabach-Merritt syndrome with osteolysis (Gorham's sign) does not appear to be fortuitous. Therapeutic management of these severe complications is difficult and requires case by case analysis.

Disarticulation↗
PubMed1996

Congenital hemangioma: evidence of accelerated involution.

OBJECTIVE: To study the course of hemangiomas that proliferate in utero, are fully grown at birth, and begin to regress during early infancy. DESIGN: We analyzed retrospectively 31 infants with congenital hemangioma seen at Tarnier-Cochin Hospital (Paris) and Children's Hospital (Boston). Diagnosis was made by clinical and radiologic examination and, if necessary, by biopsy. Age, gender, location, appearance, and evolution were noted for each infant. RESULTS: Only 3 of 23 congenital hemangiomas were diagnosed in utero by ultrasonography. The three most common morphologic forms were raised violaceous tumor with ectatic veins (n = 8), raised grayish tumor with multiple tiny telangiectasias, surrounded by a pale halo (n = 8), and flat infiltrative tumor with violaceous overlying skin (n = 5). Two congenital hemangiomas had associated thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon). All the untreated congenital hemangiomas (n = 24) regressed by the time the infants were 14 months of age, leaving either atrophic skin or extra skin. Seven congenital hemangiomas required therapy for complications: three tumors responded to systemic corticosteroid administration and four were resected. CONCLUSION: Hemangiomas can proliferate in utero and manifest as fully developed tumors at birth. These congenital hemangiomas can regress rapidly. This phenomenon raises new questions about the pathogenesis of this tumor.

Antineoplastic Agents, Hormonal↗
PubMed1996

[Surgical treatment of superficial vascular malformations. Indications for tissue expansion].

The aim of this work was to demonstrate the feasibility of surgical exeresis of superficial vascular malformations using tissue expansion. A retrospective analysis of data from 15 patients who underwent surgery over a 9 year period for arteriovenous (n = 6) and venous malformations of the trunk and limbs was made. Indications for treatment were pain in six patients or complications of an arteriovenous malformation. Twenty-eight expanders were used in 15 patients. Most of the complications observed (25% of the cases) were minor. The program had to be interrupted due to complications in only one case (7%). Mean duration of tissue expansion was 105 days (30-165). Mean delay to cicatrization was 40 days and mean duration of the treatment program was 156 days. Indication for surgical exeresis of superficial vascular malformations can be widened due the contribution of tissue expansion. With acquired experience, the risk of complications has been reduced. The duration of the treatment protocol is the main drawback.

Adolescent↗
PubMed1996

Cerebral developmental venous anomalies associated with head and neck venous malformations.

PURPOSE: To study cerebral developmental venous anomalies in patients with extensive venous malformations of the head and neck. METHODS: All patients had undergone carotid angiography 10 to 15 years previously. Four-vessel cerebral angiography was carried out in 40 patients with venous malformations. All patients had a physical examination, 16 had CT, and 22 were examined with MR imaging. One patient had MR angiography. RESULTS: Eighteen developmental venous anomalies were noted in 8 (20%) of 40 patients. Four patients had multiple anomalies, and these were bilateral in 1 patient. Developmental venous anomalies seen in association with cervicofacial, cutaneous, and mucosal venous malformations were remarkable in their absence of neurologic events and associated cavernoma; significance of ectatic venous convergence, extension, and preponderance of deep drainage routes; and frequency with which they multiple in occurrence. CONCLUSION: Developmental venous anomalies have a remarkable prevalence of 20% in patients with extensive superficial venous malformations. Therefore, it is important to search for a cerebral developmental venous anomaly when confronted with a cervicofacial venous malformation.

Adolescent↗
PubMed1996

[Cervicofacial superficial venous malformations and developmental abnormalities of the cerebral venous system].

INTRODUCTION: Cerebral developmental venous anomalies (DVA) are uncommon trajectories of the brain venous drainage. They must be differentiated from cavernomas. Patients with head-and-neck venous malformations can have DVA in their brain, a finding much more frequent than in general population. OBSERVATIONS: Three patients had DVA in their brain; this was an incidental discovery when investigating their facial or cervical superficial venous anomalies using arteriography, CT, MRI and MRA. COMMENT: DVA are uncommon variations of the brain venous system. In a vast majority of patients, this anatomical variant appears to be tolerated well, in contrast to cerebral cavernoma that can cause intracranial hemorrhage and neurologic symptoms. We want to stress the fact that the frequency of DVA occurring in patients with cervicofacial VM is much higher than in general population. This pattern is often a fortuitous finding in the work-up of these patients and it must be known in order to stop confusing it with true cerebral venous "angioma": the carvernoma.

Adult↗
PubMed1995

Incontinentia pigmenti: late sequelae and genotypic diagnosis: a three-generation study of four patients.

Late cutaneous signs of incontinentia pigmenti (IP) are often subtle and misdiagnosed. We focus on these somewhat confusing clinical markers in a family, and on the genotypic diagnosis based on DNA analysis. An infant was born with a typical IP rash. Dermatologic examination of the women in her family revealed that her mother, her maternal aunt, and her grandmother had subtle skin signs reminiscent of IP. The four family members proved to be informative for DNA markers in the Xq28 region. Familial cases of IP are sometimes missed due to the lack of recognition of some late skin signs that are not always hyperpigmented streaks. Subtle, faint, hypochromic or atrophic lesions in a linear pattern may occur. Thus an accurate diagnosis of the women in a particular family also requires anamnestic data and recognition of extracutaneous anomalies. When a clinical diagnosis has been made, DNA marker analysis allows us to offer a prenatal diagnosis with minimal risk of error in case of further pregnancy. However, early testing of chorionic villus samples does not allow one to predict the severity of the disease in an affected fetus.

Adult↗