[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.

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.

[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.

Perichiasmatic granuloma occuring after radical mastoidectomy: MR findings.

A case of chronic chemical meningitis occurring after a radical mastoidectomy is reported. Imaging and surgical findings were suggestive of a dissemination of cholesteatoma debris within the subarachnoid spaces. Chemical meningitis has been described in epidermoid and dermoid cyst rupture. This report illustrates that clinicians should be aware of this possible complication. Skull base imaging is mandatory before considering the diagnosis of idiopathic meningitis. Only treatment of the abnormal communication between cerebrospinal fluid and middle ear may eradicate the origin of this rare meningitis.

["Beret" flap and other flaps in the reconstruction of cancer related loss of substance of the ear]. / Le lambeau "en béret" et les autres lambeaux dans la reconstruction des pertes de substance carcinologiques de l'oreille.

Major tissue loss involving the ears and periauricular area due to carcinologic exeresis is an important problem in reconstructive surgery. We present the "béret" flap, a frontal flap with an occipital pedicle, emphasizing the simplicity of the procedure and its reliability. We present our personal experience and data in the literature and recall the main clinical and histological features of these tumors as well as the clinical course and treatment.

[Concomitant radiochemotherapy in locally advanced ORL cancers: a prospective study of 68 cases]. / Radiochimiothérapie concomitante dans les cancers ORL localement avancés: étude prospective de 68 patients.

From January 1988 to July 1992, 68 patients presenting with a locally advanced and unresectable carcinoma of the head and neck region were given 3 to 5 courses of chemotherapy (5-FU 1 g/m2, day 1 to 3, cisplatin 4 mg/m2, day 1 and 2, 15 mg/m2 day 8) combined with simultaneous radiotherapy. Patients were enrolled in two protocols. In the initial one a 70 Gy total dose was delivered in 105 days, and in the latter the same dose was given in only 63 days. Toxicity was moderate and acceptable. In the 62 evaluable patients, a complete response was observed in 62%, and partial response in 24%. The complete response rate was higher (80%) in the population enrolled in the second protocol. Overall the median survival is 18 months. At 2 and 3 years, the overall survival is 48% and 30% respectively. These results suggest an influence of overall treatment duration on complete response rate and survival.

[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.

[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.

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.

[Port wine hemangioma]. / Angiome plan.

Port wine stains are mainly due to an increase in the number or size of dermal capillary vessels. They are a natural disgrace which, in their most common form (i.e. isolated and non-systematized), requires a cosmetic treatment. It is for this purpose that various therapies were formerly prescribed with inconstant results. Since the development of lasers, notably the argon laser and, most recently, the pulsed dye laser, these treatments have become obsolete. Before they fall into oblivion, it must be remembered that until the seventies radiotherapy was part of the old methods and that all cases of port wine stain now seen in adults must be followed up with particular attention if they have received radiotherapy in their childhood.

[Temporo-masseter vascular malformations]. / Malformations vasculaires temporo-massétérines.

Temporo-masseterine vascular malformations are mature capillarovenous malformations. Being congenital lesions, they are present at birth and progress throughout life, particularly before adolescence. The clinical diagnosis rests on the finding that the malformation swells when the patient is in supine position. Its extent is determined by MRI. Treatment is radiosurgical and always begins with one or several embolizations using Ethibloc. Surgical resection, which is exceptionally complete, may be performed to make the face symmetrical.

[Surgical landmarks of the temporo-frontal branch of the facial nerve]. / Repères chirurgicaux du rameau temporo-frontal du nerf facial.

The temporal branches of the facial nerve, and in particular the rami for the frontalis muscle, have been the subject of many studies with the main objective of avoiding injury. The usual reference points, sometimes difficult to localise, anatomical variations in their branching and anastomoses are the main points of our investigation. This anatomical dissection study of 30 cadavres, with easy reference points available both superficially and in the depths allow a simple, non complicated localisation of the frontal branch and its anastomosis in a precise way on the mandibular condyle, the zygomatic arch and the temporal region. The distances described a, b, c, are useful in common surgical practice: temporo-mandibular joint surgery, facial lifting, temporal and temporo-parietal fascia flaps, and contralateral neurotomy for frontal symmetrization among others.

[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.

[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.