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Biomedical subjects

O Enjolras

Publications and source records attributed to O Enjolras.

At least 73 records · Page 4Linked to original sources
PubMed1995

Cervical occult spinal dysraphism: MRI findings and the value of a vascular birthmark.

Spinal dysraphism is easily recognized in the overt form as a meningocele or myelomeningocele. The closed form or occult spinal dysraphism (OSD) can be overlooked. It occurs predominantly at the lumbosacral level, but OSD at the cervical level, although very rare, also occurs. The value of magnetic resonance imaging investigations in preparation for surgical treatment is emphasized. We discuss the value of various midline posterior skin anomalies as indicators of an underlying developmental defect in the neural axis. Hallmarks for OSD in the inferior third of the back are well known. They can also occur at the cervical level. Among these warning cutaneous midline changes, a vascular stain alone is rarely a clue for OSD whatever the spinal level involved, and specifically in the nuchal area.

Female↗
PubMed1995

'Membranous aplasia cutis' with hair collars. Congenital absence of skin or neuroectodermal defect?

BACKGROUND: The skin and the nervous system are both derived from ectoderm. Separation of neural ectoderm from epithelial ectoderm occurs concurrently with the closure of the neural tube. This chronologic association may explain the cutaneous abnormalities often found overlying neural tube defects. A ring of dark long hair encircling a congenital scalp lesion (the hair collar sign) is one such marker and is often associated with encephaloceles, meningoceles, and heterotopic brain tissue. OBSERVATIONS: We describe six children with aplasia cutis who displayed the hair collar sign. Aplasia cutis is a relatively heterogeneous disorder; however, these lesions had a unique and strikingly similar appearance. This subtype of aplasia cutis, which we have termed membranous aplasia cutis, shares several clinical and histologic features with cranial neural tube defects. CONCLUSIONS: We propose that membranous aplasia cutis is a form fruste of a neural tube defect and may be derived from a similar embryological defect. Recent advances in the understanding of cranial neural tube closure may provide support for this hypothesis.

Atrophy↗
PubMed1994

[Multifocal epithelioid hemangioendothelioma with partial remission after interferon alfa-2a treatment].

INTRODUCTION: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumour of the soft tissue having an intermediate malignancy. Cutaneous presentations have exceptionally been reported. OBSERVATION: A young woman, initially affected by an EHE with cutaneous and bone involvement, then with pulmonary and hepatic localizations, was in partial remission after one year of treatment with alpha 2a interferon. DISCUSSION: In a critical review of the literature, we describe the main features of this tumour and the difficulties to classify this entity in the vascular tumors' spectrum. Histologically, epithelioid hemangioendothelioma has a very characteristic appearance and is clearly different from other vascular tumors as angiolymphoid hyperplasia with eosinophilia and Kimura's disease. The assumption of a multicentric or a metastatic origin when multiple tumor deposits are found, is still not elucidated. Different therapeutic approaches are proposed: surgery, interferon, interleukin-2, retinoids. Randomized studies would be necessary to confirm our results with alpha 2a interferon but are probably difficult to realize because of the lack of cases.

Adult↗
PubMed1994

[Proteus syndrome. 8 cases].

INTRODUCTION: Proteus syndrome most often includes: corporal hemi-hypertrophia, gigantism of the extremities with hyperplasia of the palms and soles, vascular abnormalities and varied types of other hamartomatous tumours. The dysmorphic growth generates functional orthopaedic and orthognatic disabilities that increase with age. OBSERVATIONS: We report, on eight cases, our experience in management of Proteus syndrome abnormalities and the benefits of new techniques in imaging and interventional vascular radiology. The correction of limb orthopaedic abnormalities is complicated with elephantiasic enlargement of soft tissues. In some patients the absence of a deep venous network contra-indicates transcutaneous occlusion of dysplatic vessels. Facial asymmetry and orthognatic anomalies are better managed after permanent teeth have appeared. COMMENTARIES: The management of these disabilities is carried out by staff of different specialities but should be organized by a practitioner who regularly follows the child and detects early the functional disturbances. Aesthetic corrections are performed later.

Child↗
PubMed1993

Serious childhood angiomas: unsuccessful alpha-2b interferon treatment. A report of four cases.

Over a 4-year period, we managed four children with alarming haemangiomas (two cases of Kasabach-Merritt syndrome and two life-threatening haemangiomas). Systemic steroid therapy was ineffective. Other treatments (radiotherapy, anti-platelet drugs) were also ineffective in the Kasabach-Merritt patients. On the basis of recent reports on the effects of interferon on endothelial cells, we used alpha-2 interferon therapy, but obtained no response.

Arm↗
PubMed1993

The current management of vascular birthmarks.

Two vascular birthmarks are hemangiomas and vascular malformations. Hemangiomas grow by cellular proliferation. Their hallmark is rapid neonatal growth. Spontaneous regression begins when the infant is 6 to 10 months old, but it may continue until 8 to 10 years of age. Hemangiomas are infrequently life-threatening. Pharmacologic treatment is indispensible; unsightly sequelae require surgical treatment. Vascular malformations consist of dysplastic vessels and are present on a lifelong basis. They are either slow-flow (capillary, venous, lymphatic) or fast-flow anomalies with arteriovenous shunting. Complex combined vascular malformations are observed as well. Ten years ago angiographic studies clearly demonstrated the differences among the various lesions. Today a noninvasive diagnostic approach is recommended, particularly in children. Ultrasonography, Doppler flow imaging, and magnetic resonance imaging are the most informative techniques, revealing the extent of tissue involvement and differentiating fast-flow from slow-flow anomalies. Risks and management differ depending on the type of vascular malformation.

Adolescent↗
PubMed1993

[Surgical indications in angioma of the face].

Hemangiomas are common vascular tumors of children. They always regress. Surgery takes place for late sequelae, such as cutaneous scarring and distortion of the facial structures. Early surgery is beneficial for the nasal cryano hemangioma, or for some eyelid hemangiomas. Venous malformations need to be manage from childhood to adulthood as they slowly worsen. Percutaneous embolization with Ethibloc and surgery are performed in order to minimize, or to improve the skin, muscle and bone distortion. Arteriovenous malformations are the most dangerous vascular anomalies of the face. Superselective arterial embolization prepares the surgical excision of the nidus. Difficulties in covering the excised area are frequently solved by flaps or cutaneous expansion.

Adolescent↗
PubMed1993

[Surgical indications in angiomas of the face].

Hemangiomas are common vascular tumors of children. They always regress. Surgery takes place for late sequelae, such as cutaneous scarring and distortion of the facial structures. Early surgery is beneficial for the nasal cyrano hemangioma, of for some eyelid hemangiomas. Venous malformations need to be manage from childhood to adulthood as they slowly worsen. Percutaneous embolization with Ethibloc and surgery are performed in order to minimize, or to improve the skin, muscle and bone distortion. Arteriovenous malformations are the most dangerous vascular anomalies of the face. Superselective arterial embolization prepares the surgical excision of the nidus. Difficulties in covering the excised area are frequently solved by flaps or cutaneous expansion.

Adolescent↗
PubMed1993

Percutaneous embolization with Ethibloc of lymphatic cystic malformations with a review of the experience in 70 patients.

Cystic lymphatic malformations (CLM) are superficial vascular hemodynamically inactive malformations of the lymphatic compartment. We propose a new approach which uses a sclerosing agent as an alternative to surgical resection. In the past nine years we treated 70 patients with CLM. Fifty-five percent were younger than five years of age with a male preponderance and most (80%) of the CLM were located in the maxillofacial region. They usually presented with functional impairment from the mass effect; others had infections, bleeding, or inflammation. The CLM were injected under fluoroscopic control with a sclerosing agent, Ethibloc, which dries up the pockets and reduces the mass. On follow-up the results were good in 62%, unchanged in 5%, and continued progression in 20%. Fifteen percent underwent surgery failures (24%) occurred in mixed forms of cystic and cellular lymphangiomas. Complications were minors. Percutaneous embolization is useful for CLM, with minimal risk, absence of scar, and it avoids surgery. It should be the first line of treatment for these lesions.

Adult↗
PubMed1992

[Diagnostic orientation in the presence of superficial vascular malformation].

Diagnosis relies first on clinical evaluation of the patients. Subsequently, modern diagnostic imaging techniques, such as CT scan, MRI and Echo-Doppler, confirm the clinical approach and define the type of vascular anomaly. Angiographic analysis is not performed for all cases: indications are carefully thought out.

Arteriovenous Malformations↗
PubMed1992

[Immature hemangiomas in children].

Immature haemangiomas affect only infants and children and follow a peculiar course: first an expansion phase, then an involution phase which ends in complete cure in the majority of cases. Because of this favorable outcome, all inopportune treatments must be avoided, and abstention is the best attitude. However, early corticosteroid therapy must be prescribed when the lesion is located in a dangerous area, such as the eyelids or the lips. Specific treatments must be instituted only in very special cases, such as visceral or giant cutaneous haemangiomas or Kasabach-Merritt syndrome.

Age Factors↗
PubMed1992

[Superficial venous malformations].

Mature vascular malformations of the venous compartment are haemodynamically inactive and persist throughout the patient's life. They have no systemic repercussions, except in patients with disorders of coagulation (e.g. disseminated intravascular coagulation), but they have local, cosmetic and functional repercussions. Percutaneous embolization, notably with Ethibloc, has modified their prognosis favourably. This simple and effective procedure is mainly used for superficial venous malformations of the face.

Angiomatosis↗
PubMed1992

[Arteriovenous malformations].

Arteriovenous malformations (AVM) are the most dangerous of vascular malformations, being haemodynamically active. These malformations proceed in two phases: a phase of quiescence which may last throughout life, and an unpredictable phase of activity sometimes triggered by a trauma or by hormonal variations (puberty, pregnancy, oral contraceptives). Treatment must be careful usually limited, and considered in the phase of activity. A multidisciplinary consensus is necessary.

Arteriovenous Malformations↗
PubMed1992

[Disseminated complex vascular malformations].

Diffuse complex vascular malformations are sporadic or hereditary. They usually concern one type of vessel: capillary, venous or arterial (with AV fistula). They involve skin, mucosal membranes and internal organs. They lead to a rich and heterogeneous symptomatology, depending on the lesional localizations. Five out of them are described: cutis marmorata, Rendu Osler disease, Bean syndrome, Maffucci syndrome, and Proteus syndrome.

Abnormalities, Multiple↗
PubMed1992

[Systematized complex vascular malformations].

Various types of systematized vascular syndromes are described, either in the head or in the limbs. The Sturge-Weber syndrome includes a facial portwine stain, ocular anomalies, and a pial vascular malformation. Complex combined vascular malformations with limb overgrowth need careful investigations to clearly define the vascular component and the management.

Arteriovenous Malformations↗
PubMed1992

[Cystic lymphatic malformations of the head and neck. Role of interventional radiology].

Cystic lymphatic malformations are hemodynamically inactive vascular malformations of the lymphatic compartment. Over a 9 years period, 52 patients with superficial cystic lymphatic malformations of the head and neck underwent embolization by direct injections of Ethibloc (Ethicon, Ethnon Laboratories, Neuilly/France) under fluoroscopic control. Results were graded as excellent or good in 57% of patients after embolization alone. Treatment was completed by surgery in 19% of patients. Embolization was considered a failure in 24% (12 patients), although 10 of these patients showed regression of the cystic component after embolization. This safe, efficacious percutaneous technique has emerged as the first treatment to be considered for cystic lymphatic malformations.

Child, Preschool↗