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.

[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.

[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.

[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.

[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.

[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.

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.

[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.

[Spontaneous hematoma of the intra-orbital optic nerve]. / Hématome spontané du nerf optique intra-orbitaire.

A young male was observed with papilledema and optic nerve enlargement on CT scanning. Visual acuity and field were not affected. A microsurgical procedure, through a para-latero-nasal approach, revealed a blue domed cyst around the neural structure. Incision of the cyst released old hemorrhagic fluid and biopsies of the cyst wall were collected. Microscopy showed granuloma and hemosiderin pigments. The post-operative course was uneventful, and the papilledema resolved in six weeks. The clinical presentation and surgical findings, the histopathology and the post-operative course lead us to conclude that this is a case of spontaneous hematoma of the optic nerve. Although intracranial and chiasmal optic nerve hematomas have been described since 1966, this is the first report to our knowledge of a hematoma involving the intraorbital portion of the optic nerve. The para-latero-nasal approach seems to be a easy way of acquiring good visualization without any osteotomy, when an operating microscope is available. This report may alert ophthalmologists to consider a cyst as a rare cause of papilledema.

[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.

[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.

[Malignant change affecting stenotic scarring (caustic) of the oesophagus (author's transl)]. / La dégénérescence cancéreuse des sténoses cicatricielles (par caustique) de l'oesophage.

The authors report four cases of carcinoma of the oesophagus developing at the site of old burns. They review the classical features of this type of lesion, found in their own cases: the role of trauma from dilatations and the very long time elapsing before malignant change occurs (29 to 57 years, according to the individual cases). They discuss the prophylactic value of oesophagectomy with oesophagoplasty following severe burns with stenotic scarring. Finally, they note that whilst the treatment of these forms of carcinoma of the oesophagus is in general palliative, curative surgery may nevertheless be envisaged.

[Three case histories of cortical and sub-cortical auditory lesions. Audiological and tomodensimetrical confrontations (author's transl)]. / Trois observations d'atteinte cortico-sous-corticale de l'audition. Confrontations audiologiques et tomodensimétriques.

The authors describe three case histories of auditory lesions involving the cortex or subcortex of the temporal lobes. In two of the cases there are minor lesions of the hemisphere producing an amusia without verbal deafness or agnosia of non-verbal sounds. In one of the two patients, the total transitory deafness makes the use of the term cortical deafness debatable. In the third case, a bilateral vascular lesion of the temporal lobes had produced a pure verbal deafness without loss of tone discrimination or loss of the capacity to recognise changes in rhythm or sound frequency. In these three patients in whom there was no anatomical verification, the data given by the scanner and the study of evoked cortical potentials recorded over the two cerebral hemispheres allowed a fairly good study of the correlations between the topography of the lesions and their semiotic consequences.

[The surgical treatment of scar tissue stenosis of the crico-tracheal junction (author's transl)]. / Le traitement chirurgical des sténoses cicatricielles de la joncation crico-trachéale.

On the basis of 6 personal cases, the authors report the special conditions associated with surgery for stenosis of the crico-tracheal junation : proximity of the recurrent laryngeal nerves, difficulty of accurately matching the upper and lower ends, difficulty in passage of the suture materials. They nevertheless conclude that in the treatment of stenosis at this level of the respiratory tract, resection-anastomosis would appear to give better results than those obtained by Rethi's operation. Alongside major surgery, minor therapeutic techniques and, in particular, the laser used endoscopically also have a role to play : either in the treatment of moderate stenosis, or in the prevention and treatment of recurrent stenoses after resection-anastomosis.

[Metastases to the thyroid from adenocarcinomas. Review of the world literature around five personal cases (author's transl)]. / Les métastases tkhyroïdiennes des cancers épithéliaux. Revue de la littérature mondiale a propos de 5 cas personnels.

The authors propound five personal clinical cases of intra-thyroid metastases from epithelial carcinomas. Looking through the world literature, they found 134 other such cases. Analyzing these cases, there is a marked preponderance of renal cancers. The other most frequency primary cancers are of the breast, tract, lungs and skin epitheliomas. Undeclared metastases discovered at autopsy seem to be much more frequency (approximately 7.7% of those who die of their cancer have such metastases). This discrepancy between the rarity of clinical metastases and the frequency of microscopic metastases is explained by their very great latency and their very slow progress. This justifies the attempt at a curative treatment every time this is possible: surgery plus cobalt therapy. This can produce a fairly long survival rate: one year and four months on average when the metastasis is discovered before the primary cancer; two years and then months when the metastasis is discovered after.

[Non-traumatic rhinorrhea from a latero-sellar fistula of the temporal fossa adjacent to an empty sella turcica. Computed tomography aspects; surgical treatment]. / Rhinorrhée non traumatique par fistule latérosellaire de la fosse temporale au voisinage d'une selle turcique vide. Aspects tomodensitométriques; guérison chirurgicale.

The authors report a case of non traumatic rhinorrhea due to an osteo-dural fistula communicating between the latero-sellar part of the left middle cranial fossa and the sphenoidal sinus. This fistula, probably congenital, was adjacent to an intrasellar arachnoidocele ("empty" sella turcica), but was not responsible for it. This lesion was demonstrated by CT scan associated with the injection of Metrizamide. The surgical treatment (trans-sphenoidal approach) allowed complete cure of the fistula and its associated symptoms.

[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.

[Trimetazidine and degenerative deafness. Effect on hearing and integration]. / Trimétazidine et surdités dégénératives. Effets sur l'audition et l'intégration.

This study was conducted in order to assess the activity of trimetazidine dosed at 60 mg/day in patients presenting degenerative loss of hearing. Ten centers participated in this multicenter double-blind placebo-controlled trial which spanned 6 months. Included were 251 patients (118 women, 133 men) presenting with pure perception bilateral symmetrical deafness concerning predominantly the acute frequency range. 228 patients terminated the study, including 115 in the trimetazidine group and 113 in the placebo group. The evolution over a 6-month treatment period was significantly better with trimetazidine as regards the following parameters: audibility as assessed by pure-tone audiometry, namely at the acute frequency range (p = 0.002), intelligibility as assessed by speech audiometry (p = 0.008), subjective evaluation of hearing loss and its impact on the patient's social life. Results confirm the value of trimetazidine hearing loss management, based on clinical, subjective criteria as well as audiometric ones. Parameters reflecting best trimetazidine's efficacy were intelligibility and the psychological/behavioral impact of the impairment, allowing for better social integration of the subject.

[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.