Erdheim-Chester disease presenting as malignant exophthalmos.
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Biomedical subjects
Publications and source records attributed to Y Raulo.
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Treatment of temporomandibular ankylosis complicating masseter muscle cellulitis in three patients, as a result of a dental lesion, required wide excision of bone structure and, in two cases, of adjacent infected soft tissues.
Current limitations for surgical excision and solutions to problems of bone and dura mater repair of nasosinusal tumors invading the anterior region of the base of skull are discussed in relation to 14 cases. None of these patients had been operated upon by first intention using the upper approach Exploration of the median part of the anterior region is limited posteriorly by the posterior border of the jugum and the base of the anterior clinoid processes. Pre-operative discovery of invasion of the dura mater is a contraindication for surgery. Posterior and dural extension of the tumor is evaluated by combined CT Scan and tomography, but these examinations do not always confirm the absence of dura mater involvement before surgery. A double approach should be used when doubt as to invasion of the base exists. Dura mater repair involves suturing, a free periosteal flap, and a pediculated cranial periosteum flap anteriorly on the fronto-orbital region. Reconstruction of the base requires the use of the periosteal flap, alone or associated with bone grafts depending on the size and location of the bone breach. Short and long term postoperative complications were nor observed.
A 30 year old woman presented with a tumor of calvaria which had grown slowly over a period of 10 years. There was no history of symptoms other than an isolated episode of seizure. Infiltration of the scalp noted at physical examination and radiologic findings suggested the diagnosis slowly progressive sarcoma but the diagnosis of meningioma was established by histologic examination which also showed the important vascularization of the tumor (estimated blood loss was 300cm3). Taking into consideration the histologic benignity of the tumor and the complete thrombosis of the longitudinal sinus, removal of the lesion was planned after complete embolization of the vessels branching from the external carotid artery to supply the tumor. A two stage procedure was performed. During the first operative stage portion of the meningioma infiltrating the bone (800 g; 15 X 12 X 2 cm) and scalp was removed. In as second stage the intracranial "en plaque" meningioma was removed with no significant bleeding. The resected dura was replaced by combining a pericranial graft with a dermal graft. The cranial valt was restored with a bone homograft. The patient tolerated the procedure well and is in good health 18 months following surgery.
A thirty-nine-year-old caucasian man presented with an ulceration of the scalp, developing on an alopecia. The scalp indeed had been burnt by boiling water when the boy was two. During childhood and youth microtraumatisms induced superficial wounds which always healed easily. Nine months ago, a new ulceration followed a new trauma; the wound did not heal, and lay on a dark brown area. A biopsy diagnosed a malignant melanoma. The whole alopecic area was resected, under the ulceration and the pigmented area, as well as the external surface and the diploe. The defect was covered with a free transplant of omentum, revascularized by microsurgical anastomoses to the external carotid artery and the external jugular vein in the parotid bed. The omentum was immediately covered with split-thickness skin grafts. Histologic investigations demonstrated the association of a melanoma developing on a Hutchinson melanotic freckle and a squamous cell carcinoma.
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This paper reviews the evolution of the surgical approach to the facial deformities in Crouzon's and Apert's syndromes. Since the first high maxillary osteotomy performed via an extracranial approach, various procedures have been used. These procedures were, successively, the intracranial route permitting simultaneous advancement of the mid-face and frontal bone in adult; then the use of trephine holes to protect the dura and brain during osteotomies obviating the need for a craniotomy. Likewise, more precise craniofacial fixation by interosseous wiring and bone grafting frequently obviate the need for intermaxillary fixation. More recently the fronto-facial advancement in one piece has been used in children between 4 and 10 years of age. The advantages and disadvantages of this procedure are considered.
Before regarding the treatment of facial deformities in mandibulo-facial dysostosis, the clinical, radiological and anatomical findings are reviewed. The plastic surgical correction of the main deformities are then considered. The aims of the treatment are: close the palpebral coloboma, build the zygomatic bones and zygomatic arches, correct the malformation of the auricles and the macrostomia, re-establish normal dental occlusion if necessary and harmonize the profile by reducing the nose and excessive vertical dimension of the mental symphysis.
Three cases of rarely observed bilateral parotid gland tumors are reported. In two cases the tumors were of different histological types (mixed tumor on one side and an adenocarcinoma on the other side) while in the third case they were both of the mixed variety. No clear explanation for the possible bilateral nature of these tumors was found, diagnosis being made either during the initial examination or after an interval of several years.
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The most frequently observed maxillary complications of labiopalatine clefts are endognathia and mesognathia. Their treatment, which consists essentially of a maxillary osteotomy, enables correction not only of dental articulation disorders but also ensures improved upper lip projection and harmonious support of the base of the alae nasi.
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