[Cicatricial pemphigoid: treatment with mycophenolate mofetil]. / Pemphigoïde cicatricielle: traitement par mycophénolate mofétil.

BACKGROUND: The severity of cicatricial pemphigoid (CP) varies. First-intent treatment of mild or moderate cases is dapsone. In life or sight-threatening cases, intravenous cyclophosphamide pulses are efficient but may have digestive side effects and imply repeated hospitalizations. Mycophenolate mofetil (MMF) is an oral and well tolerated immunosuppressant agent which has proved its efficacy in pemphigus and some bullous pemphigoid. In CP, encouraging case reports have been previously published. We report herein a retrospective study about 14 patients who have received MMF since 2000. PATIENTS AND METHODS: There were 5 men and 9 women, with a mean age of 69 years. MMF was introduced in 3 different clinical situations: immediately in relay to cyclophosphamide in 7 patients with severe CP (group I); in case of a mild-severe relapse at distance from with dranal of cyclophosphamide in 3 patients (group II); as first-intent immunosuppressant agent in 4 patients whose disease was not under control with high-dose dapsone, but not life - or sight-threatening (group III). In all these patients, the disease was invalidating and not controlled by dapsone +/- sulfasalazine, but did not threaten life or sight. The aim was to achieve satisfying control of the disease with an oral and well tolerated immunosuppressant agent, and to maintain good quality of life. The dose of MMF was 1.5 or 2 g per day. The criteria of MMF efficacy was the healing of previous lesions and the absence of new progressive lesions. RESULTS: MMF was efficient in obtaining or maintaining a good control of the disease in 10/14 patients, as long as the underlying treatment with dapsone (2 mg/kg/d) was maintained. In 7/10 cases, it was possible to decrease the dapsone dose in order to improve hematological tolerance. In the 3 other cases, a relapse occurred when the dose of dapsone was decreased. MMF was inefficient in controling the disease in 4/14 patients (29 p. 100). Clinical and biological tolerance of MMF was good in 13/14 patients. DISCUSSION: In this series, MMF was proposed to heterogenous patients, who presented at that time a mild-moderate disease and for whom we wanted in improve the quality of life. MMF seems to be an interesting drug, capable of obtaining or maintaining satisfactory control of the disease and permitting the decrease of dapsone doses in some mild-severe CP. However MMF must not replace cyclophosphamide in severe sight or life-threatening forms of CP.

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.

[Hemangiomas and superficial vascular malformations: classification]. / Hémangiomes et malformations vasculaires superficielles: classification.

Superficial vascular malformations of the face, trunk and limbs are better known today, and they can be divided up into simple and complex vascular malformations. Simple vascular malformations may form five major categories: immature hemangiomas of infants, port-wine stains, capillarovenous angiodysplasias, and arteriovenous fistulae and malformations. Complex angiodysplasias are systematized (Sturge-Weber and Bonnet-Dechaume-Blanc syndromes, Cobb's metameric angiomatosis, Klippel-Trenaunay and Parkes Weber's syndromes) or disseminated (Weber-Osler-Rendu disease and blue rubber-bleb nevus syndrome). Various modalities of treatment may be contemplated, according to the type of malformations, and an interdisciplinary consultation is essential to decide whether a watch-and-wait policy, a physical method (laser), embolization, fibrosing injections, vascular, maxillofacial or plastic surgery, or a successive combination of various techniques should be resorted to.

[Exploration strategy for superficial vascular malformations]. / Stratégie d'exploration des malformations vasculaires superficielles.

Transillumination of cystic lymphangiomas, MRI of venous vascular malformations, pulsed Doppler and arteriography of arteriovenous malformations, echo-Doppler and radiomeasurement of malformations in the lower limbs are key examinations in the exploration strategy for superficial vascular malformations.

[Venous vascular malformations and their treatment with Ethibloc]. / Les malformations vasculaires veineuses et leur traitement par Ethibloc.

The use of Ethibloc must be contemplated as a first-intention treatment for venous malformations of the face. This sclerosing agent has transformed the functional, esthetic and psychological prognosis for these vascular malformations.

[Cystic lymphatic malformations and their treatment]. / Les malformations lymphatiques kystiques et leur traitement.

Lymphangiomas are remarkably well amenable to treatment with direct aspiration then embolization with a sclerosing agent derived from maize: Ethibloc.

Alveolar soft-part sarcoma of the cheek.

Alveolar soft-part sarcoma is a rare malignant tumour of uncertain histogenesis, the two main sites of which are the lower extremities in adults and the head and neck in children. We know of only three case reports of intraoral locations without tongue involvement. Here, we describe the first case of an alveolar soft-part sarcoma of the cheek which presented in a patient over 30 years old and had an extremely rapid and eventually fatal evolution. We have also reviewed hypotheses about its aetiology.

[2 cases of unilateral nasal obstruction related to rhinolithiasis]. / Deux cas d'obstruction nasale unilatérale liée à une rhinolithiase.

Authors report two cases of 31 and 42 year old women who for many years had a unilateral nasal obstruction: first case due to a large polyp, second one due to a mucosal inflammation but both caused by a rhinolithiasis. The latter is formed over several years around a vegetable seed or a piece of material neglected since childhood. The rhinolithiasis is a calcareous concretion formed in layers visible by X-rays. This type of growth is usually enhanced by weak economic development of certain countries.

[Extracorporeal lithotripsy in the treatment of salivary lithiasis. A prospective study apropos of 27 cases]. / La lithotripsie extra-corporelle dans le traitement de la lithiase salivaire. Une étude prospective à propos de 27 cas.

In a prospective study, we evaluated the efficacy of extracorporeal lithotripsy (ECL) in the treatment of salivary stones. We define the indications for the treatment of submaxillary and parotid stones, depending on the diameter and the location of the stone. Twenty-seven patients were treated by ECL. All had a unique stone, in the salivary duct, with a diameter greater than 2 mm; the site was the parotid gland for ten cases, the submaxillary gland for seventeen cases. After six months, the results from a clinical and ultrasonography point of view, were the following: total efficacy or complete desintegration of the stone: 9 cases; partial efficacy or fragmentation of the calculi, with residual fragments with a diameter of less than 2 mm, which could be flushed out with possible spontaneous clearance: 10 cases; failure or the lack of the reduction in the size of the stone or partial fragmentation into concrements with a diameter of more than 2 mm: 8 cases. Based on our experience and the results reported in the medical literature, we propose an therapeutic approach to symptomatic salivary stones. ECL is the most preferable treatment for stones of the parotid duct or parotid gland. The treatment of stones of the submaxillary gland depends on the site and the size of the calculi; ECL should be proposed as first-line treatment when the stone diameter ranges from 2 to 10 mm and when it is situated in the pelvis of the duct or in the proximal duct; surgical resection of the submandibular gland is indicated when the stone is situated in the gland itself or when the diameter is more than 10 mm; when it is situated in the distal duct, a marsupialization should be performed.

[Anatomical and functional evaluation 3 and 5 years later of 94 cases of chronic cholesteatomatous otitis media. Clinical and therapeutic implications]. / Bilan anatomique et fonctionnel à 3 et 5 ans de 94 cas d'otite moyenne chronique cholestéatomateuse. Implications cliniques et thérapeutiques.

Anatomical and functional results were evaluated at 3 and 5 years after surgery for chronic cholesteatomatous otitis media in 94 patients treated by the same surgeon between 1975 and 1983. Open techniques predominated and were justified by seven clinical or epidemiologic factors present alone or in association in 80% of cases. Many patients were non-attenders at follow up (40% at 3 years and 70% at 5 years), recovery from their cholesteatoma being noted in 84% and 78% of those attending at 3 and 5 years respectively. Useful audition was recovered in 52%, including 2 of 3 due to an open technique. Petro-mastoid hollow-out with arrangement of cavity and minimal ossicular reconstruction is considered the most effective treatment for this type of affection.

[Anatomical and functional evaluation, after 3 and 5 years, of 94 cases of chronic cholesteatomatous otitis media. Clinical and therapeutic implications]. / Bilan anatomique et fonctionnel à 3 et 5 ans de 94 cas d'otite moyenne chronique cholestéatomateuse. Implications cliniques et thérapeutiques.

Anatomic and functional results are reported after 3 and 5 year follow up of 94 cases of chronic cholesteatomatous otitis operated upon and kept under surveillance by the same surgeon between 1975 and 1983. Open technics dominated and were justified by seven clinical and epidemiologic factors found alone or in combination in 80% of patients. Results must allow for the high rate of non-attenders (40% at 3 years and 70% at 5 years). Recovery from their cholesteatoma was obtained in 84 and 78% of patients after 3 and 5 years respectively, 52% recovering useful hearing, 2 out of 3 of these due to an open technic. Modified radical mastoidectomy is considered to be undoubtedly the most effective treatment for this type of affection.

[Bucco-sinusal site of primary leiomyosarcoma]. / Localisation bucco-sinusienne d'un leiomyosarcome primitif.

The leiomyosarcoma, a malignant smooth muscle tumor, is common in uterus and gastro intestinal track. Only 27 cases of oral cavity localisation have been report in literature since 1908. We report a case of primary leiomyosarcoma of palate in 26 years old woman. The diagnosis is made by histopathological examination after biopsy. The extension is revealed by clinical symptoms and scan examination. The treatment is based on wide surgery Chemotherapy and radiotherapy could be proposed. The prognosis of the lesion depends of metastatic development, few cases have been present with metastases developed after one year, two years and five years. Generally their localisation are liver, lungs or lymph nodes. In our case after two years post operative a second lesion is revealed and the patient died quickly.

[Fibrous dysplasia and ossifying fibroma of the base of the skull. Apropos of 6 cases]. / Dysplasie fibreuse et fibrome ossifiant de la base du crane. A propos de 6 cas.

The authors report 6 cases of fibrous dysplasia (F.D.) of the base of the skull and review the literature. They confirm the impossibility of histological differentiation between ossifying fibroma and monostic dysplasia with cranio-facial sites. They stress the value of CAT scan in the assessment of spread and that of dynamic isotope scan in the differential diagnosis with plaque-shaped hyperostotic meningioma, in the diagnosis of polyostotic forms and in postoperative surveillance. They stress the risk of visual sensorial impairment: visual by stenosis of the optic canals and auditory by stenosis of the E.A.M. Operative indications are influenced by this risk, thereby explaining the need for prolonged surveillance.

[Value of Gore Tex in the reconstruction of the nasofrontal canal]. / Intérêt du Gore Tex dans la reconstitution du canal naso-frontal.

Surgical technic used in treatment of chronic frontal sinusitis is described, and the interest emphasized of the employ of fragments of Gore Tex prosthetic fragments for calibration and maintenance of permeability of the newly formed nasofrontal canal. Their perfect tolerance allows them to be left in place and their permeability can be surveilled by CT scan imaging or fibroscopy.

[Diagnostic problems of tuberculoid cervical adenitis. Apropos of 70 cases]. / Problèmes diagnostiques des adénites cervicales tuberculoides. A propos de 70 observations.

Pathologic criteria in tuberculoid adenitis enable differentiation between follicular adenitis with or without caseous necrosis and that involving suppurative necrosis. The absence of specific etiology is emphasized. Seventy cases of tuberculoid adenitis are analyzed. Of the 62 cases of adenitis labelled tuberculous, bacterial confirmation was obtained in only 22 (35%), suggesting probable diagnosis by excess for the remaining 40 cases. Related bacteriologic problems concern isolation of B.K. of the 3 cases classified as sarcoidosis, it was important to obtain data from broncho-alveolar lavage and serum conversion enzyme assay. Four patients with adenitis were diagnosed as having cat-scratch disease from positive specific intradermal reactions, the probable discovery of the responsible agent thus providing a bacteriologic diagnosis.

[Labial incompetence and maxillodental dysmorphosis]. / Incompétence labiale et dysmorphose maxillo-dentaire.

A definition of labial incompetence is given and emptrasis placed on the fact that this is a symptom of maxillodental dysmorphism (MDD) and not of nasal dyspermeability. Pathogenic features are discussed, the relevant clinical and paraclinical diagnostic features of MDD outlined, and the different alveolodental and/or bony (maxillary or mandibular) dysmorphias responsible for labial incompetence described. Principles of orthodontic and surgical treatment are centered on re-establishment of a correct articulation and therefore labial competence.

[Mucoceles of the maxillary sinus. Diagnostic and therapeutic problems. Apropos of 5 cases]. / Mucocèles du sinus maxillaire. Problèmes diagnostiques et thérapeutiques. A propos de 5 cas.

Five cases of the rarely observed maxillary sinus mucocele are reported, one patient with bilateral lesions presenting primary dilatation of the bronchi. A previous history of surgery or injury to sinuses was obtained in all cases, the diagnosis being confirmed by computed tomography imaging. Recovery is usually complete after the Caldwell-Luc operation. One case with exclusion of the sinus is described and the literature reviewed.

[Tympanic and jugular paragangliomas. I. Clinical symptomatology and evaluation of extension. Apropos of 25 cases]. / Paragangliomes tympaniques et jugulaires. I. Sémiologie clinique et bilan d'extension. A propos de 25 cas.

Based on a review of 25 cases of tympanic and jugular paragangliomas treated and followed up in the Lariboisière hospital, Paris between 1978 and 1986, clinical aspects and extension of these tumors are studied. The often long delay in making the diagnosis is related to lack of knowledge of presenting symptomatology, frequent discordance between functional and physical symptomatology exists, but remarkable progress has been made in assessment of extension of tumors using new imaging techniques, particularly angiography, which can also guide the preoperative embolization and surgical procedures.

[Tympanic and jugular paragangliomas. II. Angiographic, surgical and irradiation treatment. Results. Indications. Apropos of 25 cases]. / Paragangliomes tympaniques et jugulaires. II. Traitement angiographique, chirurgical, et par irradiation. Résultats. Indications. A propos de 25 cas.

Results of treatment are reviewed in 25 cases of tympanic and jugular paragangliomas treated and followed up in the Lariboisière hospital, Paris between 1978 and 1986. Therapy has been transformed by the use of embolization but its non-negligible neurologic accident risks, whatever the carotid artery territory involved, justify prior weighing of indications for its application. New surgical technics allow more extensive exeresis, but intrapetrous extension to the nerve compartment of the jugular foramen and the anterior pericarotid region still raises difficult surgical problems and appears to be the principal reason for failure of treatment. Radiotherapy is very effective and applicable for inoperable forms, as a complement to incomplete surgical removal and for recurrences.

[Olfactory esthesioneuroma. Clinical, histological and therapeutic aspects; apropos of 6 cases]. / Les esthésioneuromes olfactifs. Aspects cliniques, histologiques, et thérapeutiques; à propos de six observations.

On the basis of 6 cases, the authors review the clinical, histological and therapeutic aspects of olfactory esthesioneuromas. These rare tumours, showing varying rates and degrees of progression from one patient to another, generally have a severe prognosis. Diagnosis is based upon precise histological criteria which may be clarified by electromicroscopic data. In difficult cases it may be useful to seek the aid of immuno-histochemical techniques in order to demonstrate the presence in tumour cells of specific neuronal enolase and the labelling of such cells by anti-protein S-100 antibodies. The treatment of choice would appear to be radio-surgical completed by chemotherapy similar to that used in neuroblastomas.