Acromegaly in Carney complex.

PURPOSE: Carney complex (CNC) is a rare autosomal dominant syndrome, characterized by mucocutaneous pigmentation, cardiac, cutaneous myxomas and endocrine overactivity. It is generally caused by inactivating mutations in the PRKAR1A (protein kinase cAMP-dependent type I regulatory subunit alpha) gene. Acromegaly is an infrequent manifestation of CNC, reportedly diagnosed in 10% of patients. METHODS: We here report the case of a patient who was concomitantly diagnosed with Carney complex, due to a new mutation in PRKAR1A ((NM_002734.3:c.80_83del, p.(Ile27Lysfs*101 in exon 2), and acromegaly. In parallel, we conducted an extensive review of published case reports of acromegaly in the setting of CNC. RESULTS: The 43-year-old patient was diagnosed with an acromegaly due to a GH-secreting pituitary microadenoma resistant to somatostatin analogs. He underwent transsphenoidal surgery in our tertiary referral center, which found a pure GH-secreting adenoma. In the literature, we identified 57 cases (24 men, 33 women) of acromegaly in CNC patients. The median age at diagnosis was 28.8 ± 12 year and there were 6 cases of gigantism. Acromegaly revealed CNC in only 4 patients. 24 patients had a microadenoma and two carried pituitary hyperplasia and/or multiple adenomas, suggesting that CNC may result in a higher proportion of microadenoma as compared to non-CNC acromegaly. CONCLUSIONS: Although it rarely reveals CNC, acromegaly is diagnosed at a younger age in this setting, with a higher proportion of microadenomas.

[Quality of life for patients treated for head and neck carcinoma]. / Qualité de vie chez les patients traités pour un cancer de la sphère ORL.

A large consensus admits that quality of life is a multifactorial concept including at least physical, psychical and social dimensions of the disease as well as symptoms related to the disease and to the requested treatments. Quality of life is actually considered as one of the major assessment criteria for taking care of patients with cancer and to evaluate results of clinical trials. Self-evaluation by the patient is considered as the gold standard to evaluate the clinical symptoms. This evaluation is not unambiguous. Medical doctors underestimate patients' symptoms. In the field of surgery, development of organ preservation strategies should be considered as one of the major improvement observed in the modern era of head and neck oncology. The role of xerostomia, the most frequent complication reported after head and neck radiation therapy, is major in this field. However, odynophagia is considered as the most detrimental component of quality of life. Radiation oncologists should realize the role of these parameters in order to include these concepts as relevant in the global evaluation of treatments.

[Acute pseudo-membranous laryngitis in epidermolysis bullosa acquisita]. / Laryngite pseudo-membraneuse sévère dans le cadre d'une épidermolyse bulleuse acquise.

Epidermolysis bullosa acquisita is a rare entity belonging to the auto-immune cutaneous blistering disorders of the dermo-epidermal junction. Clinical manifestations are generally cutaneous including the development of sub-epidermal blisters. Mucosal manifestations should be systematically looking for, but laryngeal involvement remains uncommon. We report an 81-year-old woman who presented with dysphagia, dyspnea and dysphonia as the presenting features of laryngeal involvement of an epidermolysis bullosa acquisita. This is the tenth reported case in the literature. We describe our diagnostic approach and the therapeutic management, comparing them with the literature.

[Partial allotransplantation of the face including the mandible: a feasibility study]. / Allotransplantation partielle de face incluant l'os mandibulaire: étude de faisabilité.

OBJECTIVE: Facial grafts are useful in that they allow the repair of severe facial defects in one step in contrast to the actual available techniques which require staged procedures with limited cosmetic and functional results. The aim of our study was to determine whether it would be possible to include part of the mandible in a partial allotransplant of the face. MATERIAL AND METHODS: An anatomical study on the arterial and venous vascularisation of the face and the mandible was performed on 7 heads. Then nine heads were used to describe an anatomical model of harvesting two-thirds of the lower face. RESULTS: The study determined that a graft could be viable with a facial artery, inferior dental artery and two veins facial. Thus, a reliable method for harvesting hemi-mandible and total mandible is developed. The average sampling time was 4 hours and thirty minutes. Harvesting a total mandibular graft was more tedious because of the loss of joint laxity caused by the absence of mandibular osteotomy. CONCLUSION: Partial allotransplant of the face including the mandible is feasible. In such transplantations, functional difficulties related to the temporo-mandibular joint and orthognathic problems need to be overcome.

[Management of cervical cellulitis with and without mediastinal extension: report of 17 cases]. / Prise en charge des cellulites cervicales avec on sans extension médiastinale: à propos de 17 cas.

OBJECTIVES: Cervical cellulitis is infrequent but serious. The aim of our study was to describe the way we care and to identify certain factors that promote the development of such a condition. PATIENTS AND METHODS: We conducted a retrospective study covering the period 2004 to 2009 and included patients with cervical cellulitis with or without mediastinal extension surgically supported by ENT department of the University Hospital of Dijon. Data were collected clinical, radiological, treatment, type of surgery and complications. RESULTS: Seventeen patients met our inclusion criteria, four of which had a form associated with mediastinitis. Eight patients had taken NSAIDs and/or corticosteroids and fifteen patients antibiotics before their hospitalization. All have benefited from surgery with an average of 1.35 interventions (range 1 to 3) and support postoperative resuscitation. In both cases the outcome was unfavourable. CONCLUSION: The use of NSAIDs and/or corticosteroids was a factor in promoting this type of infection. In the context of surgical treatment, it does not seem necessary to surgically reoperate systematically.

[Acute group A beta-hemolytic streptococcus membranous laryngotracheobronchitis in an adult]. / Laryngotrachéobronchite aiguë membraneuse à streptocoque bêta-hémolytique du groupe A de l'adulte.

OBJECTIVE: To describe the first case of a group A beta-hemolytic streptococcus laryngotracheobronchitis as well as the other possible etiologies in a case of acute dyspnea. METHODS: We report the case of a 46-year-old woman with a doubtful vaccination status who presented clinical features of croup (laryngotracheobronchitis). She developed respiratory distress and required endotracheal intubation. RESULTS: The endoscopy demonstrated a great deal of crust and pseudomembrane detachment. Bacterial culture grew group A beta-hemolytic streptococcus. Progression was good with antibiotics and corticosteroid treatment. CONCLUSION: Bacterial acute pseudomembranous croup (laryngotracheobronchitis) is rare. We must search for the most frequent diagnoses such as diphtheria and epiglottitis. This is the first case to be reported in the literature.

[Zenker's diverticulum: technique and results of endoscopic treatment with Endo-GIA 30 stapler]. / Diverticule de Zenker : technique et résultats du traitement endoscopique a la pince GIA 30.

OBJECTIVES: The purpose of this project was to analyze the endoscopic management of Zenker diverticulum using an Endo-GIA stapler inserted transorally to perform an oesophageal diverticulostomia. PATIENTS AND METHODS: Between January 97 and December 2006, 30 consecutively treated symptomatic patients (13 men; median age 67 years; range 45-91) with Zenker's diverticulum were enrolled into this retrospective study. Ninety six percent complained about dysphagia with slimming in 33%. The diagnosis of Zenker's diverticulum is based on anamnesis and radiological examinations with applied contrast medium of the upper digestive tract. RESULTS: Twenty-six patients had the endoscopic approach. A patient profited in same time from a resection by external cervical approach following a tearing of the mucous membrane after installation from staled diverticulotomy. Finally 3 patients failed endoscopic exposure. On the 26 patients operated by strict endoscopic treatment, the average duration of intervention was 29 minutes. Overall, an onset of liquid intake on 2.3 postoperative days and the average length of stay was 6 days. Mean follow-up was 40 months. A recurrency was noted in the 2 cases, one was reoperated with the same technique. CONCLUSION: This endoscopic technique using an Endo-Gia stapler is an efficient, safe and minimally invasive method for the treatment of Zenker's diverticulum. With a low rate of morbidity, it is a good technique especially for patients with impaired of health or associated diseases.

[Frontal sinus pathology and epilepsy]. / Epilepsie et pathologie du sinus frontal.

OBJECTIVES: Because of its location and the fragility of its physiology, the frontal sinus is the first of the facial sinuses to cause complications. In this context, orbital sepsis, cranial vault osteitis, meningitis, cerebral abscess, longitudinal sinus thrombophlebitis can occur. A more uncommon consequence of frontal sinusitis is isolated epilepsy. METHODS: We report two cases of patients admitted in our department after a generalised epilepsy seizure with, on the CT-scan, an opacity of the frontal sinus with a posterior wall lysis. RESULTS: We operated on quickly both patients after the seizure via an eyebrow approach. The first one had a purulent collection of the frontal sinus, the second an infected cholesteatoma. Both had a stenosis of the nasofrontal canal and a lysis of the sinus posterior wall with a bare dura mater. The surgical treatment consisted in the cleaning of the sinus associated with an antibiotic treatment in one case and the cholesteatoma matrix removal in the other. The nasofrontal canal was calibrated for respectively four and two months. An antiepileptic treatment was administered for one year. Four years later the nasofrontal canal is pervious and the frontal sinus sound in both patients. CONCLUSION: An epilepsy seizure can follow a frontal sinusitis. It does not convey the existence of an endocranial complication but requires researching it. The posterior wall lysis of the sinus with a bare dura mater is sufficient to lead to a seizure in case of sinus infection.

[Intratumoral chemotherapy: experimental data and applications to head and neck tumors]. / La chimiothérapie intratumorale: aspects expérimentaux et applications aux tumeurs de la tête et du cou.

OBJECTIVE: Intratumoral chemotherapy consists in the direct intratumoral injection of the anticancer drugs. Despite its simple and logical principle it remains relatively little used. MATERIAL AND METHODS: This work reviews and analyses the national and international literature about experimental and clinical studies of intratumoral chemotherapy. RESULTS: Numerous experimental studies validated its theoretical advantages compared with the intravenous one: drug intratumoral concentration increase, antitumoral activity improvement and systemic toxicity decrease. But they also underlined its limits: the high clearance and the non-homogeneous drug diffusion. Research works led to the improvement of the results and performed clinical trials with slow release devices (microspheres, collagen matrix with or without vasoconstrictive agent), anticancer drug in an aqueous solution with a vasoconstrictive agent, intratumoral injection in association with electrochemotherapy or radiotherapy. These trials showed the feasibility of this technique with, in recurrent tumors, response rate between 27 and 50% and an increase in quality of life. The more frequent adverse effects were pain in 24 to 80% of cases, ulceration, necrosis and oedema of the treated locations in 53 to 87,4% of cases and during the use of vasoconstrictive agents systemic effects like arterial hypertension and extrasystoles. CONCLUSIONS: Intratumoral chemotherapy is an effective therapeutic even when used after the classical treatments. Improvements are necessary to define the best drugs, injection technique, treatment periodicity and indications. Intratumoral chemotherapy deserves better interest at the moment where drugs and antibodies limit their action to the cancer cells preserving the healthy ones.

Telithromycin (HMR 3647) achieves high and sustained concentrations in tonsils of patients undergoing tonsillectomy.

Telithromycin, the first ketolide antimicrobial to be developed for clinical use, has potent activity against group A beta-haemolytic streptococci (GABHS), including macrolide-resistant strains. The penetration of telithromycin into tonsils was assessed in 22 adults undergoing tonsillectomy at 3, 12 or 24 h after the fourth dose of oral telithromycin 800 mg once daily. Telithromycin rapidly penetrated tonsillar tissues, achieving a mean concentration of 3.95 mg/kg at 3 h post dose, 3.4 times greater than the corresponding plasma concentration (1.22 mg/l. The mean tonsil:plasma concentration ratio increased to 13.1 at 24 h post dose, indicating slower elimination from tonsils than plasma. Tonsillar and plasma concentrations exceeded the MIC(50) for GABHS throughout the 24-h dosing period. These findings suggest that telithromycin may be an effective new alternative treatment for GABHS tonsillopharyngitis.

[Pharyngeal stenosis: treatment by resection-end to end anastomosis]. / Sténose pharyngée: traitement par résection-anastomose.

OBJECTIVES: Pharyngeal stenosis occurs frequently after laryngectomy or total laryngectomy extended to the pharynx. Oral feeding might become impossible or is limited to liquids. In such cases, dilations can be performed, but surgery becomes necessary when they are unsuccessful. The objective of this report is to show that pharyngeal resection anastomosis is one of the existing techniques which can be helpful in such cases. METHODS: A 67-year-old woman had been operated on a laryngeal cancer 18 years earlier when she presented with a 2 cm height pharyngeal stenosis responsible for a chronical dysphagia to solids. The multiple dilations performed were unsuccessful and she underwent a pharyngeal resection and end to end anastomosis by lateral cervicotomy. RESULTS: The outcome was uneventful. Normal pharyngeal permeability and swallowing were restored and are still maintained with a 18 months follow-up. CONCLUSION: Rehabilitation technics using flaps--pectoralis myocutaneous, lingual, platysma or jejunum flaps--is not always mandatory in patients presenting with pharyngeal stenosis. A pharyngeal resection end to end stenosis, pharynx anastomosis can also be successfully performed in stenosis of limited height.

[MRI screening of vestibular schwannomas without gadolinium: usefulness of the turbo gradient spin echo T2-weighted pulse sequence]. / Dépistage du schwannome vestibulaire en IRM sans gadolinium: intérêt de la séquence turbo gradient spin écho pondérée T2.

The purpose of this retrospective MRI work was to evaluate the use of turbo gradient spin echo (TGSE) high resolution imaging for the detection of eighth nerve schwannomas, without injection of gadolinium. The TGSE sequence (slice thickness: 3 mm with 1.5 mm interleaving; matrix: 512) was compared with a reference sequence: T1-weighted spin echo (SE) after gadolinium injection (slice thickness: 3 mm, matrix: 256). Among 380 internal auditory meatus (IAM) explored, 34 abnormalities were detected on T2-weighted TGSE images compared with 19 on contrast-enhanced T1-weighted SE images. This new sequence has a 100% sensitivity, a 96% specificity and a 100% negative predictive value. Using a rigorous protocol for IAM analysis with the TGSE sequence, gadolinium injection may no longer be needed systematically for vestibular schwannoma screening, but might only be necessary when an abnormality or a doubt persists after TGSE. This approach allows about 20% cost reduction for each patient, a savings of 11,433 euros considering only the true negatives observed in this study.

[Labyrinthine fistulae and cholesteatoma]. / Les fistules labyrinthiques d'origine cholestéatomateuse.

Labyrinthine fistula is a potentially serious complication of cholesteatoma. During or after surgery, cholesteatoma can induce partial or total destruction of cochleovestibular functions. We operated 38 patients from 1983 to 1996. The site of the fistula was the external semi-circular canal in 90% of the cases. The facial nerve canal was eroded in 66% of the cases. We performed 11 CT scans; only 7 evidenced the fistula. We removed the matrix of the cholesteatoma during the initial surgery in 35 cases and in 3 left the fistula in situ for subsequent excision at a second operation. Postoperative hearing loss compared with the preoperative situation was observed in 66% of the patients. Deafness was observed in 4 ears (11%). Hearing improved after surgery in 23% of the patients. We consider that a closed technique with immediate removal of the cholesteatoma matrix is indicated for most fistulae but that second-intention resection (combined approach tympanoplasty) is the better choice when the fistula is wide and the ear is infected. In some cases (old patient, one functional ear, better ear) an open technique may be preferred without risk for the cochleovestibular functions.

[Mastoid cortical bone grafts in ossiculoplasty]. / L'os cortical mastoïdien dans les ossiculoplasties.

Ossicular homografts have been left for safety reasons with regard to viral transmission diseases. Several means are usable to reconstruct ossicular chain: synthetic prosthesis and autologous bone. On grounds of disponibility, biocompatibility, cost and use easiness we have been using mastoid cortical bone since 1995. We have studied hearing results and tolerance of 45 ossiculoplasties performed with cortical bone. Two years after, we have been obtaining as good or even better functional results with cortical bone graft than with auto or homologous ossicular bones (air bone gap inferior or equal to 20 DB in 89 % of the cases) and no extrusion. Thus, cortical bone seems to be, the better material when autologous ossicular bones are not available.

[General physicians' viewpoint on the evaluation of health care quality in the hospital]. / Le point de vue des médecins généralistes sur l'évaluation de la qualité des soins à l'hôpital.

The principal objective of this work was to determine whether the evaluation of general practitioners' satisfaction vis-à-vis a health care establishment impacted that establishment to improve the quality of the services it provides. Ten doctors were randomly selected and 100% agreed to respond to a semi-directive interview. Two independent doctors analysed each corpus and identified 15 themes, of which 5 systematically came up: the organisation of the care of patients, the availability of hospital doctors, the transmission of information, the speed of emergency admissions, and the patient-hospital doctor relationship. The opinion of private practice doctors usefully complements the evaluation of patient satisfaction used to identify the weaknesses of an establishment. It is proposed to include this evaluation in the procedure of continued improvement of the quality of care within establishments.

[A difficult diagnosis of a congenital minor ear anomalie]. / Un cas trompeur d'aplasie mineure de l'oreille.

We report an unusual congenital middle ear anomalie with an inflammatory tympanic membrane, a total opacity of the middle ear on the CT scan and a tumor in the mesotympanum.

[Pathogenic hypothesis of a rare malformation of the stapes]. / Hypothèse pathogénique d'une malformation rare de l'étrier.

We report an exceptional stapedial malformation. The stapes presents here three legs. We propose a pathogenic hypothesis with a supernumerary branch of the stapedial artery.