Laryngeal graft after total laryngectomy in humans: A SWiM analysis.

Evaluation of the results of laryngeal transplantation (LT) in humans. Analysis of 3 bibliographic databases with the keywords "larynx, transplantation, autograft". In total, 626 abstracts were read and 25 articles selected. The main objective was to analyze the characteristics of laryngeal transplant patients. The accessory objectives comprised analysis of operative technique, immunosuppressive treatment and results. Four articles were selected for analysis. Two patients were transplanted after total laryngectomy for laryngeal carcinoma and 2 after laryngeal trauma. Three of the 4 patients had true transplantation with arterial, venous and neural microanastomosis. Two patients were decannulated and the tracheostomy tube was maintained in the other 2. Three of the 4 patients had good-quality phonation and could feed without a gastric tube. One patient died of carcinoma progression and 1 patient had to be explanted 14 years after transplantation. The number of LTs reported is too small for scientific determination of the place of this intervention in laryngology. The published results could, at first sight, suggest that the future of LT is uncertain. However, several elements, also suggest that otolaryngologists should continue to take an interest in this technique.

Audiological phenotyping evaluation in KBG syndrome: Description of a multicenter review.

OBJECTIVES: Our objective was to reinforce clinical knowledge of hearing impairment in KBG syndrome. KBG syndrome is a rare genetic disorder due to monoallelic pathogenic variations of ANKRD11.The typical phenotype includes facial dysmorphism, costal and spinal malformation and developmental delay. Hearing loss in KBG patients has been reported for many years, but no study has evaluated audiological phenotyping from a clinical and an anatomical point of view. METHODS: This French multicenter study included 32 KBG patients with retrospective collection of data on audiological features, ear imaging and genetic investigations. RESULTS: We identified a typical audiological profil in KBG syndrome: conductive (71%), bilateral (81%), mild to moderate (84%) and stable (69%) hearing loss, with some audiological heterogeneity. Among patients with an abnormality on CT imaging (55%), ossicular chain impairment (67%), fixation of the stapes footplate (33%) and inner-ear malformations (33%) were the most common abnormalities. CONCLUSION: We recommend a complete audiological and radiological evaluation and an ENT-follow up in all patients presenting with KBG Syndrome. Imaging evaluation is necessary to determine the nature of lesions in the middle and inner ear.

Ruptured dural arteriovenous fistula and sinus venous thrombosis following surgical resection of a vestibular schwannoma: Case report and review of the literature.

Dural arteriovenous fistula (DAVF) is a rare vascular malformation. Strong evidence suggests that the development of DAVF in adults is acquired and multifactorial. The link between cerebral venous thrombosis and DAVF is probably explained by dynamic changes in the venous drainage pattern. We report the case of a 34-year-old man admitted to the emergency department for seizure and headaches. The patient had a medical history of right vestibular schwannoma resection 9 months earlier, complicated by untreated asymptomatic sigmoid sinus thrombosis. At admission, CT scan revealed a spontaneous temporal intracerebral hemorrhage associated with ventricular hemorrhage due to the rupture of a DAVF diagnosed by complementary CT angiography. External ventricular drainage was performed in emergency, followed by endovascular exclusion of the DAVF. Good neurological outcome was achieved, with complete exclusion of the vascular malformation. This clinical case underlines the absence of guidelines on the use of anticoagulation drugs to treat postoperative venous sinus thrombosis and to potentially prevent DAVF as a late complication following cerebellopontine angle surgery.

Short-term hearing outcome of malleus removal for surgery: An observational cohort study.

OBJECTIVES: The main goal of the present study was to assess hearing outcome for malleus removal in cholesteatoma surgery compared to a group with malleus conservation. The secondary aim was to compare the auditory involvement of the stapes between the two groups. MATERIAL AND METHODS: A single-center observational study included adult patients operated on by ossiculoplasty for acquired cholesteatoma between 2015 and 2019. Endpoints comprised improvement in pure-tone average air-bone gap (PTA-ABG) and air-bone gap (ABG) at conversational frequencies and, independently, per frequency. 136 patients were included: 95 with conserved malleus (M+) and 41 with malleus removal (M(). Mean time to audiometric follow-up was 9 months in both groups. RESULTS: The PTA-ABG improvement was 4.4±12.6dB for the M+ group and 3.8±13.4dB for the M- group, with no significant significance (P=0.8). Better results (not exceeding 7.5dB) were found for the M+ group at 2 and 8kHz (P=0.3 and P=0.052 respectively). Presence or absence of the stapes did not affect the results in either group. CONCLUSION: Those results suggest a negligible role of the malleus in early hearing outcome of tympanoplasty for cholesteatoma. A slight improvement was observed in the M+ group at 2 and 8kHz, but its interpretation remains uncertain.

Stapes surgery in osteogenesis imperfecta: retrospective analysis of 18 operated ears.

PURPOSE: To evaluate short and long term results of stapes surgery in patients with osteogenesis imperfecta (OI), METHODS: Retrospective case series of 18 primary stapes surgeries performed on 11 hearing-impaired OI patients with evidence of stapes fixation, in a Tertiary referral center. We analysed pre-operative and post-operative hearing results at 1 month and at least 1 year RESULTS: The main operative findings were stapes fixation, thickened footplate and fragile or fractured stapes crura. No revision surgery was necessary. Hearing improvement was achieved in 94% of the cases. We obtained an air-bone gap closure to within 10 dB in 46% of the cases and to within 15 dB in 92% of the cases at 1-year follow-up. The mean hearing gain in air conduction (at 0.5, 1, 2 and 4 kHz) was 18.4 dB at 1 month and 22.4 dB at 1 year. CONCLUSION: Stapes surgery in OI gives good results with few complications in our series. A hearing gain is often obtained in spite of the sensorineural hearing loss caused by the natural progression of the disease.

Retropharyngeal lipostructure in the treatment of velopharyngeal insufficiency: A prospective study and update.

INTRODUCTION: Retropharyngeal lipostructure is a recent procedure in velopharyngeal insufficiency (VPI), offering an effective alternative to heavier surgery. OBJECTIVES: To update and assess retropharyngeal lipostructure as a treatment for VPI in the University Hospital Center of Rouen (France). TYPE OF STUDY: Single-center prospective study, from May 2012 to May 2014. PATIENTS AND METHODS: Six patients (4 girls, 2 boys) presenting with VPI were treated by retropharyngeal lipostructure. Age at surgery ranged between 6 and 12 years. Four of the patients bore a 22q11 microdeletion. Treatment was indicated in case of Borel-Maisonny type 2b (n=2) or 2m (n=4) despite well-conducted speech therapy and of≥50% velopharyngeal sphincter closure on nasal endoscopy. Patients were assessed preoperatively and at 3 months, by a multidisciplinary team. Borel-Maisonny type was assessed by a speech therapist. Nasality was measured on assisted vocal evaluation (EVA®). Sphincter closure was assessed on dynamic MRI. RESULTS: Between 6 and 8cm3autologous fat was injected. At 3months, 4 children showed 1-grade improvement in Borel-Maisonny type. Nasality decreased systematically, from a mean 14.5% preoperatively to 10.5% postoperatively. MRI showed improvement in all cases, with complete closure in occlusive vowels in 3 children. CONCLUSION: EVA® and MRI provide precise objective assessment of VPI. Retropharyngeal lipostructure is a simple, relatively non-invasive, reproducible technique, providing good results in VPI.

Sensorineural hearing loss in patients with vestibular schwannoma correlates with the presence of utricular hydrops as diagnosed on heavily T2-weighted MRI.

PURPOSE: The purpose of this study was to assess whether the volume of the vestibular endolymphatic space correlates with the degree of hearing loss using heavily T2-weighted fast imaging employing steady-state acquisition with cycle phase (FIESTA-C) MRI. MATERIALS AND METHODS: A total of 23 patients with vestibular schwannoma, as diagnosed on typical image findings, who underwent FIESTA-C MRI were included. There were 13 women and 10 men with a mean age of 63.5±9.3 (SD) years (range: 49-88years). Two radiologists independently evaluated the volume of the utricle and saccule. Correlation between tumor volume, vestibular endolymphatic space volume and degree of hearing loss - as evaluated with the levels of pure-tone average and speech recognition threshold - were searched for. RESULTS: The mean saccular, utricular and tumor volumes were 3.17±1.1 (SD) mm3 (range: 1.45-5.7mm3), 14.55±5 (SD) mm3; (range: 6.6-23.9mm3) and 17.4±5.5 (SD) mm3; (range: 8.3-25.4mm3), respectively. There was a moderate correlation between the volume of the utricle and the degree of hearing loss as evaluated with the levels of pure-tone average (rho=0.5; P=0.015) and speech recognition threshold (rho=0.58; P=0.004). There were no significant correlations between saccular and tumor volumes and the degree of hearing loss. CONCLUSION: The volume of the utricle in patients with obstructive vestibular schwannoma moderately correlates with the degree of hearing loss.

Treatment of unilateral vocal fold paralysis with ansa cervicalis to recurrent nerve anastomosis in a young adolescent: European case report.

BACKGROUND: Laryngeal re-innervation in paediatric unilateral vocal fold paralysis is a relatively new treatment option, of which there has been little reported experience in Europe. METHODS: In this European case report of a 13-year-old boy with dysphonia secondary to left-sided unilateral vocal fold paralysis after cardiac surgery, the patient underwent re-innervation using an ansa cervicalis to recurrent laryngeal nerve transfer, in combination with fat augmentation, after 12 years of nerve denervation. Perceptual analysis data, and acoustic and laryngoscopy recordings were acquired pre-operatively, and at one and two years post-operatively. RESULTS: The patient's perceptual voice quality was improved. He experienced subjective improvement and is very satisfied with the result. As expected, laryngoscopy at one and two years after surgery showed no physiological mobility of the vocal fold concerned, but improved closure during phonation was achieved. Electromyography showed evidence of re-innervation. CONCLUSION: Laryngeal re-innervation could be considered as a treatment option for unilateral vocal fold paralysis in children and adolescents, even after a long-term delay.

Modified first or second cervical nerve transplantation technique for the treatment of recurrent laryngeal neuropathy in horses.

BACKGROUND: In horses, the only established method for reinnervation of the larynx is the nerve-muscle pedicle implantation, whereas in human medicine, direct nerve implantation is a standard surgical technique for selective laryngeal reinnervation in human patients suffering from bilateral vocal fold paralysis. OBJECTIVES: (1) To describe a modified first or second cervical nerve transplantation technique for the treatment of recurrent laryngeal neuropathy (RLN) in horses and (2) evaluate the outcomes of reinnervation using direct nerve needle-stimulation of the first cervical nerve and exercising endoscopy before and after surgery. STUDY DESIGN: Case series. METHODS: Nerve transplantation surgery, in which the first or second cervical nerve is tunnelled through the atrophied left cricoarytenoideus dorsalis muscle, was performed in combination with ipsilateral laser ventriculocordectomy. Ultrasound-guided stimulation of the first cervical nerve at the level of the alar foramen was used to confirm successful reinnervation post-operatively. Exercising endoscopy was performed before and after surgery. The exercising RLN grade of the left arytenoid was blindly determined at the highest stride frequency for each examination. RESULTS: Surgery was performed in 17 client-owned animals with RLN. Reinnervation was confirmed by nerve stimulation and subsequent arytenoid abduction observed in 11 out of 12 cases between 4 and 12 months post-operatively. Fourteen horses had exercising endoscopy before and after surgery. Nine horses had an improved exercising RLN grade, four horses had the same exercising grade and one horse had a worse exercising grade after surgery. MAIN LIMITATIONS: A sham-operated control group was not included and follow-up beyond 12 months and objective performance data were not obtained. CONCLUSIONS: The modified first or second cervical nerve transplantation technique, using tunnelling and direct implantation of the donor nerve into the cricoarytenoideus dorsalis muscle, resulted in reinnervation in 11 out of 12 cases and improved exercising grade in 9 out of 14 horses within 12 months after surgery.

Lateral skull base chondroblastoma resected with facial nerve posterior transposition.

INTRODUCTION: Chondroblastoma is a rare tumor that can involve the temporal bone. Because it is a benign tumor, functional surgery must be proposed. We report a case of a patient with a massive chondroblastoma operated on with preservation of the facial nerve, and description of the surgical technique. CASE PRESENTATION: A 37-year-old man presented with a 9-month history of a growing left pre-auricular mass and hearing loss. Neuroimaging showed an osteolytic mass invading the temporal bone and temporomandibular joint. Excision was performed via a transpetrosal and transcochlear approach with posterior transposition of the facial nerve. EMG monitoring was effective in preventing facial palsy. Four years later, no sign of recurrence was observed. DISCUSSION: Chondroblastoma is a locally aggressive tumor, especially when located in the petrous bone and temporomandibular joint. The suggested treatment is a complete excision.

Transalar transsphenoidal meningoencephalocele presenting in the form of recurrent meningitis: Report of two cases and discussion of the diagnosis and treatment.

INTRODUCTION: Skull base meningoencephaloceles are rare congenital malformations. The two cases of transalar transsphenoidal malformation reported here differ from the classical transsphenoidal meningoencephalocele. CASE REPORTS: Case 1 was a three-and-a-half-year-old boy and case 2 was a 36-year-old man. Both cases presented with clinical features of recurrent meningitis. Surgical management of case 1 was performed via an intradural infratemporal fossa craniotomy with reoperation 2years later. In the second case, surgery was initially performed via an endonasal approach and then via pterional craniotomy. Reoperation via Sekhar's transpetrosal approach was also a failure. Only closure of the trigeminal-pontine angle via a suboccipital retrosigmoid approach allowed repair of the defect. DISCUSSION: Surgical access to the pterygopalatine fossa is complex due to its anatomical position and its anatomical relations with nerves and vessels. An endoscopic approach appears to be a valuable alternative to classical craniotomy. CONCLUSION: In the two cases reported here, neurosurgery allowed lasting closure of the defect.

[A tamponade complicating an acute eosinophilic pericarditis due to a myeloproliferative/myelodysplastic syndrome]. / Une tamponnade compliquant une péricardite à éosinophiles au cours d'un syndrome myéloprolifératif/myélodysplasique.

Cardiac involvement in eosinophilia is potentially fatal and requires early diagnosis and prompt treatment. We report here the case of a 71-year-old female patient with eosinophilia>10,000/mm(3) for 2 months due to a myeloproliferative/myelodysplastic syndrome, with a rapidly progressive exertional dyspnea explained by an important circumferential eosinophilic pericarditis. Due to a rapid evolution to a tamponade, an emergent surgical drainage was performed. Subsequent medical treatment combined high-dose corticosteroids (1mg/kg/day) with hydroxyurea and imatinib. The outcome was favourable with regression of the effusion, of the volume overload symptoms and decrease in eosinophilia.

Olfaction preservation after removal of large tuberculum sellae meningiomas via a superior interhemispheric approach. A quantitative and qualitative study.

INTRODUCTION: We present a prospective series of tuberculum sellae meningioma (TSM) resected via a superior interhemispheric (IH) approach in 10 patients who preoperatively and postoperatively underwent extensive olfaction testing using a standardised test battery. PATIENTS AND METHODS: This prospective longitudinal study evaluated the olfactory function after TSM resection. The resection was performed via a superior interhemispheric (IH) approach. The quantitative and qualitative analyses of the olfactory function were assessed with the Biolfa(®) olfactory test (at 6 months). RESULTS: Between November 2009 and April 2012, 10 consecutive patients with symptomatic TSM and preserved olfactory function were operated via a superior IH approach. For the self-evaluation criteria of the olfactory function, the mean preoperative visual analog scale score was 8.8. The mean preoperative total quantitative (/27) scored 18.2 ± 6.3 for this cohort of 10 patients. In the postoperative period, the mean total score decreased non-significantly to 15.8 ± 8.8 (Wilcoxon test, P = 0.085). The mean preoperative qualitative score (/8) was 5.5 ± 1.7 and in the postoperative period decreased, non-significantly, to 4.7 ± 2.6 (Wilcoxon test, P = 0.12). The olfactory function was quantitatively and qualitatively preserved in 6 patients (60%), but a postoperative deterioration occurred in 2 (20%) and an anosmia in 2 (20%). Size and invasive characteristics of the meningioma determined the post-surgical deterioration. CONCLUSION: Olfaction is an important factor of emotional and social life, which needs to be integrated into the challenge regarding the resection of TSM. The risks of nerve damage are reviewed.

High numbers of mobilized CD34+ cells collected in AML in first remission are associated with high relapse risk irrespective of treatment with autologous peripheral blood SCT or autologous BMT.

The faster hematopoietic recovery after autologous peripheral blood SCT (APBSCT) in patients with AML may be offset by an increased relapse risk as compared with autologous BMT (ABMT). The EORTC and GIMEMA Leukemia Groups conducted a trial (AML-10) in which they compared, as second randomization, APBSCT and ABMT in first CR patients without an HLA compatible donor. A total of 292 patients were randomized. The 5-year DFS rate was 41% in the APBSCT arm and 46% in the ABMT arm with a hazard ratio (HR) of 1.17; 95% confidence interval=0.85-1.59; P=0.34. The 5-year cumulative relapse incidence was 56% vs 49% (P=0.26), and the 5-year OS 50% and 55% (P=0.6) in the APBSCT and ABMT groups, respectively. APBSCT was associated with significantly faster recovery of neutrophils and platelets, shorter duration of hospitalization, reduced need of transfusion packed RBC and less days of intravenous antibiotics. In both treatment groups, higher numbers of mobilized CD34+ cells were associated with a significantly higher relapse risk irrespective of the treatment given after the mobilization. Randomization between APBSCT and ABMT did not result in significantly different outcomes in terms of DFS, OS and relapse incidence.

[Which prostheses to use in mesh sacrocolpopexy? Experimental and clinical study]. / Promontofixation : quelles prothèses choisir ? Étude expérimentale et clinique.

BACKGROUND: Sacrocolpopexy is the standard surgical treatment of genital prolapse of the upper vaginal wall. Nowadays, the laparotomy approach is progressively supplanted by the laparoscopic procedure for the same anatomical results. About sacrocolpopexy, to date it still remains details of the technique, which differ with surgical teams maintaining controversy. Among them, the choice of the meshes certainly creates debate. OBJECTIVES: To state the basic physicochemical principles which are necessary for surgeons to select the most suitable prosthetic material to obtain the most beneficial anatomic and functional outcomes for patients. MATERIAL AND METHODS: The concepts of prosthetic biocompatibility, strength, shrinkage, deformation and elasticity are discussed. They are illustrated by experimental animal references and also human clinical references. RESULTS: Macroporous polypropylene and polyester prostheses (pore size>1 mm) are properly integrated. Collagen prosthetic coating improves tissue integration. Absorbable and nonabsorbable ultralight prostheses expose patients to a high risk of recurrence. Multifilament polyester wide pore-side prostheses have less retraction and are more flexible than monofilament polypropylene prostheses. DISCUSSION AND CONCLUSION: The prosthetic cut-off weight below which the mesh does not offer any guarantee of strength is not precisely known. Moreover, the benefit of weight reduction is not proved. Currently, heavy weight multifilament polyester prostheses with wide pore size, more than 1mm, appear to be the most appropriate meshes for sacrocolpopexy without vaginal incision.

Clofarabine in combination with a standard remission induction regimen (cytosine arabinoside and idarubicin) in patients with previously untreated intermediate and bad-risk acute myelogenous leukemia (AML) or high-risk myelodysplastic syndrome (HR-MDS): phase I results of an ongoing phase I/II study of the leukemia groups of EORTC and GIMEMA (EORTC GIMEMA 06061/AML-14A trial).

This study aims to determine the maximum tolerated dose (MTD) of clofarabine combined with the EORTC-GIMEMA 3 + 10 induction regimen (idarubicin + cytosine arabinoside) in adults with untreated acute myelogenous leukemia or high-risk myelodysplastic syndrome. In this phase I trial, 25 patients (median age 56 years) received 5 days of clofarabine as 1-h infusion (arm A) or push injection (arm B) at the dose level of 5 × 10 or 5 × 15 mg/m(2)/day in an algorithmic dose escalation 3 + 3 design. A consolidation course (intermediate dose cytosine arabinoside, idarubicin) was planned for patients in complete remission (CR). Primary endpoint was safety and tolerance as measured by dose limiting toxicity (DLT); secondary endpoints were response rate, other grade III/IV toxicities, and hematological recovery after induction and consolidation. Five DLTs were observed (in arm A: one DLT at 10 mg/m(2)/day, three at 15 mg/m(2)/day; in arm B: one DLT at 15 mg/m(2)/day). Three patients receiving 15 mg/m(2)/day were withdrawn due to adverse events not classified as DLT. Prolonged hypoplasia was observed in five patients. CR + complete remission with incomplete recovery were achieved in 21 patients (11/12 (92 %) receiving clofarabine 10 mg/m(2)/day; 10/13 (77 %) receiving clofarabine 15 mg/m(2)/day). Clofarabine, 5 × 10 mg/m(2)/day, resulted in one DLT and no early treatment withdrawals. MTD of clofarabine combined with cytosine arabinoside and idarubicin is 5 × 10 mg/m(2)/day.

BRAF mutation in papillary thyroid carcinoma: predictive value for long-term prognosis and radioiodine sensitivity.

OBJECTIVES: BRAF pV600E mutation is the most common oncogenic event and the most specific mutation for papillary thyroid carcinoma (PTC). Many studies over the last decade have shown a direct relationship between BRAF mutation and aggressive tumour characteristics, resulting in poor prognosis. However, several recent studies have suggested that BRAF mutation is not associated with poor prognosis of PTC. The present study was designed to evaluate the association between BRAF mutation with clinicopathological factors and tumour recurrence. MATERIAL AND METHODS: In this retrospective study, BRAF mutation status was examined by direct sequencing on paraffin-embedded tumour specimens from 46 patients undergoing surgery for PTC in our institution from 1985 to 2000. The relationship between BRAF mutation and gender, advanced age, extrathyroid extension, multifocal tumour, cervical lymph node metastasis, tumour size and advanced pT stage of PTC and its predictive role for the risk of tumour recurrence were investigated with a median follow-up of 10.1 (±6.5)years. RESULTS: BRAF mutation was detected in 20 of the 46 patients (43.5%) included in the study. No statistically significant correlation was demonstrated between the presence of BRAF mutation and the various clinicopathological factors studied. No significant difference in tumour recurrence rate or radioiodine sensitivity was observed between the two subgroups: mutant BRAF and wild-type BRAF. CONCLUSION: Although BRAF mutation appears to play a role in local tumour progression, it is not a risk factor for poor prognosis or tumour recurrence in PTC.

Tacrolimus enhances the recovery of normal laryngeal muscle fibre distribution after reinnervation.

OBJECTIVES: To assess the recovery of various muscle fibre types in the posterior cricoarytenoid muscle after laryngeal reinnervation in the rat, and to determine the influence of tacrolimus on this process. METHODS: Four groups of rats underwent resection and anastomosis of the left vagus nerve, and were administered either tacrolimus at a low dose or an immunosuppressive dose, or cyclosporin A at a low dose or an immunosuppressive dose. A fifth group received surgery alone, and a sixth group received neither surgery nor drug treatment (healthy group). Muscles were removed for immunohistochemical analysis 45 days after surgery. RESULTS: There was no difference in the proportion of types 1, 2a and 2b muscle fibres, comparing the immunosuppressive tacrolimus group and the healthy group, whereas there were fewer type 1 fibres in the group receiving surgery alone, compared with the healthy group (7 vs 12.1 per cent, respectively; p = 0.0303). CONCLUSION: Tacrolimus enhanced the recovery of normal laryngeal muscle fibres after reinnervation in the rat, indicating a possible role in laryngeal transplantation.