How reliable is assessment of true vocal cord-arytenoid unit mobility in patients affected by laryngeal cancer? a multi-institutional study on 366 patients from the ARYFIX collaborative group.

PURPOSE: In clinical practice the assessment of the "vocal cord-arytenoid unit" (VCAU) mobility is crucial in the staging, prognosis, and choice of treatment of laryngeal squamous cell carcinoma (LSCC). The aim of the present study was to measure repeatability and reliability of clinical assessment of VCAU mobility and radiologic analysis of posterior laryngeal extension. METHODS: In this multi-institutional retrospective study, patients with LSCC-induced impairment of VCAU mobility who received curative treatment were included; pre-treatment endoscopy and contrast-enhanced imaging were collected and evaluated by raters. According to their evaluations, concordance, number of assigned categories, and inter- and intra-rater agreement were calculated. RESULTS: Twenty-two otorhinolaryngologists evaluated 366 videolaryngoscopies (total evaluations: 2170) and 6 radiologists evaluated 237 imaging studies (total evaluations: 477). The concordance of clinical rating was excellent in only 22.7% of cases. Overall, inter- and intra-rater agreement was weak. Supraglottic cancers and transoral endoscopy were associated with the lowest inter-observer reliability values. Radiologic inter-rater agreement was low and did not vary with imaging technique. Intra-rater reliability of radiologic evaluation was optimal. CONCLUSIONS: The current methods to assess VCAU mobility and posterior extension of LSCC are flawed by weak inter-observer agreement and reliability. Radiologic evaluation was characterized by very high intra-rater agreement, but weak inter-observer reliability. The relevance of VCAU mobility assessment in laryngeal oncology should be re-weighted. Patients affected by LSCC requiring imaging should be referred to dedicated radiologists with experience in head and neck oncology.

Epidural hemostasis by autologous fat graft in minimally invasive surgery for lumbar spinal stenosis: In vivo experimental study.

BACKGROUND: Epidural hemostasis needs to use small, adapted material in minimally invasive surgery, including bilateral decompression via a unilateral approach for lumbar spinal stenosis. Most surgeons avoid external material for hemostasis because of possible neural tissue damage or complications. We compared epidural hemostasis in minimally invasive surgery by fat graft versus gelatin sponge. METHODS: The design was a prospective randomized controlled in-vivo human experimental study. The 24 levels operated on for lumbar spinal stenosis were evaluated in two groups: Group A (control group: gelatin sponge) and Group B (experimental group: fat graft). International Normalized Ratio and Prothrombin Time were assessed preoperatively. Number of cotton hemostats and systolic and diastolic blood pressure were assessed intraoperatively. Epidural hemorrhage area, spinal cord size and ratio of epidural hemorrhage area to spinal cord size were evaluated on early postoperative lumbar MRI. RESULTS: Mean epidural hemorrhage area in groups A and B was respectively 1.3±0.5 and 1.2±0.6cm2, and mean spinal cord size 1.2±0.6 and 1.8±0.6cm2 on early postoperative axial lumbar MRI. The two groups did not significantly differ in ratio of epidural hemorrhage/spinal cord size or number of intraoperative hemostats (P=0.36, and P=0.71). CONCLUSIONS: The autologous fat graft ensured sufficient and safe epidural hemostasis without serious adverse events in minimally invasive spinal surgery, and is preferable as autologous tissue is easily and quickly harvested. The surgeon feels safe with this technique and does not need external hemostatic agents.

Analysis of clinical characteristics and surgical results of upper lumbar disc herniations.

BACKGROUND: Upper lumbar disc herniation (ULDH) is described mostly in levels L1-2, L2-3 and L3-4 of the lumbar spine in the literature and accounts for less than 10% of all disc herniations. The aim of our study was to evaluate the clinical characteristics and surgical results of ULDH. METHODS: In all, 367 patients treated for upper lumbar disc herniation and 2137 treated for lower lumbar disc herniation (LLDH) between January 2008 and January 2017 were included. They were followed up postoperatively at 12 months by radiological investigations, back and leg pain Visual Analogue Scale (VAS), and Oswestry Disability Index (ODI). Preoperative data comprised age group (20-40, 40-60, 60-80 years), gender (male, female), lumbar level (L1-2, L2-3, L3-4), side of disc herniation (left, right) and symptom duration (0-3, 3-6, 6-9 months). RESULTS: ODI and VAS scores improved significantly postoperatively for all patients. Of the 367 ULDH patients, 169 were female (54%) and 198 male (46%), with a mean age of 55.8±10.1 years (range, 35-71). In 174 (47.4%) patients symptom duration was 3 months, in 99 (27.0%) 3-6 months, and in 94 (25.6%) 6-9 months. At 12 months, ODI, back and leg pain VAS scores showed a significant difference in improvement according to ULDH symptom duration<3 months and to LLDH symptom duration<6 months. CONCLUSION: Microdiscectomy in ULDH provided sufficient and safe decompression of neural structures, with significant reduction in symptoms and disability. We suggest that early surgical treatment is an important factor for good outcome in ULDH.

Comparison of the superb microvascular imaging technique and the color Doppler techniques for evaluating children's testicular blood flow.

OBJECTIVE: We have compared conventional Color Doppler (CD) and Power Doppler (PD) techniques, which are used for evaluating the testicular blood flow in small children, and the Superb Microvascular Imaging (SMI), which is a new technique. We have also investigated their contributions to testicular evaluations. PATIENTS AND METHODS: We evaluated blood flow in testicles using a grading system with CD, PD and SMI techniques. We determined the average duration of the three techniques. RESULTS: There was a statistically significant difference between the SMI and CD techniques for all patients (p < 0.001, p = 0.001). When we compared the PD and SMI, either as much or more vascular information was obtained (p = 0.106). There was a statistically significant difference between the application durations of the tests (p < 0.05). CONCLUSIONS: Superb Microvascular Imaging yields more detailed vascular information in blood flow in testicles in small children, than either CD or PD. Furthermore, this technique decreases the duration of the examination at a significant level. Superb Microvascular Imaging may represent an alternative method that can be used safely for evaluating blood flow in the testicles of small children. Additional studies may increase the reliability of SMI.

RECURRENT RAB3GAP1 MUTATIONS IN THE TURKISH POPULATION.

Warburg Micro Syndrome (WARBM, MIM 600118) is a rare, severe autosomal recessive neurodevelopmental disorder characterized by microcephaly, microphthalmia, microcornea, congenital cataract, cortical dysplasia, corpus callosum hypoplasia, intellectual disability, hypotonia and hypogonadism. RABS, small G proteins belonging to the RAS superfamily, are master regulators of vesicle trafficking in the cell. The identification of mutations in the RAB3GAP1 and RAB3GAP2 genes, which together encode the RAB3GTPase-activating protein, a key regulator in calcium-mediated exocytosis of neurotransmitters and hormones, has underpinned abnormal development of the brain, eye and genitalia as cardinal features of this syndrome. More than 100 patients have been reported with WARBM, with mutations in the RABGAP1, RABGAP2, RAB18 and TBC1D20 genes. The objective of the study was to describe the recurrent RAB3GAP1 mutations and compare the clinical features of the patients with WARBM in the Turkish population. Here we report two brothers with Warburg Micro Syndrome 1 from a non-consanguineous Turkish family with clinical features similar to those previously reported in Turkish patients with RAB3GAP1 mutations. We found that the c.748+1G>A splice-site mutation in RAB3GAP1 intron 8 is common and has so far only been detected in patients of Turkish ethnic origin. Although one of our patients has a distal extra crease on the 4th finger and another has nephrolithiasis, there does not appear to be any specific phenotypic findings associated with this mutation.

Anophthalmia-plus syndrome with unusual findings. A clinical report and review of the literature.

We present a male child at 3 years old with Anophthalmia-Plus Syndrome (APS). He has asymmetry of the face and head, left choanal atresia, a sunken facial appearance, microphthalmia in the right eye, severe microphthalmia in the left eye, bilateral low-set ears, scarring from cleft palate surgery. Magnetic resonance imaging (MRI) sections revealed decreased right globe volume, an undeveloped left globe, decreased left optical nerve thickness, Chiari type 2 malformation, left choanal atresia and cleft palate. Echocardiography and abdominal ultrasonography were normal. The patient has a 45 dB conductive hearing loss in the left ear. Repeated thyroid function tests were evaluated as compatible with central hypothyroidism. We report a Fryns Anophthalmia-Plus Syndrome in a child with unusual findings including central hypothyroidism, chiari type 2 malformation, conductive hearing loss and developmental regression. Summary of the features reported in the present case and all 14 previous cases that might be defined as APS.

MDCT angiography assessment of tetralogy of Fallot with atresic left pulmonary artery.

A 24-year-old female patient was admitted to clinic with a 10-year history of dyspnoea. A chest radiograph showed mild cardiomegaly and echocardiography revealed classic findings of Fallot's tetralogy (TOF). Multidetector-computed tomography (MDCT) angiography was performed to evaluate the additional vascular anomalies. MDCT undoubtedly revealed left pulmonary arterial atresia as well as complex intracardiac and vascular anatomic features of TOF. We described both image findings of MDCT angiography for TOF and additional vascular anomalies in this patient (Fig. 3, Ref. 8).

Evaluating adult cor triatriatum with total anomalous pulmonary venous connections by multidetector computed tomography angiography.

A 19-year-old female patient was admitted to our hospital with dyspnea, chest pain, and shortness of breath. A chest radiograph showed mild cardiomegaly. Echocardiography revealed an extra chamber in the heart. To evaluate this abnormality, ECG-gated 16-detector-row computed tomography angiography was performed. Multidetector computed tomography (MDCT), showing cor triatriatum with total anomalous pulmonary venous connections (TAPVC), clearly revealed cardiac and vascular anatomy. ECG-gated cardiac MDCT is a useful tool for detection and characterisation of cor triatriatum and related anomalies.

The value of routine clinical and radiologic studies in predicting neoplastic invasion of cricoarytenoid units.

BACKGROUND AND PURPOSE: Evaluation of the laryngeal cartilages is essential for the treatment strategy of patients with laryngeal carcinoma. Our aim was to assess the accuracy of preoperative clinical examinations and CT for preoperative evaluation. MATERIALS AND METHODS: A prospective comparative study was performed at a university department on 30 patients with advanced laryngeal carcinomas. All patients were men and underwent total laryngectomy. The preoperative mobility of vocal folds was evaluated and classified as mobile, impaired, or fixed. CT was performed by using multidetector row CT. Cricoarytenoid units (CAUs) from the laryngectomy specimens were cut into sections. Histologic findings were compared with the clinical and radiologic findings. RESULTS: The accuracy of combined clinical and CT staging in CAUs with normal mobility, impaired mobility, and absent mobility and negative CT findings was 96.5%, 50%, and 80%, respectively. These scores were 40%, 54.5%, and 83.3% for CAUs with normal mobility, impaired mobility, and absent mobility and positive CT findings, respectively. CONCLUSIONS: Arytenoid mobility and >1 cartilage abnormality on CT were found to be reliable indicators of neoplastic cartilage invasion in patients with laryngeal carcinoma.

An unusual cause of dyspnea: myelomatous involvement of cricoid cartilage. A case report.

Extraosseous multiple myeloma involving the cricoid cartilage is exceedingly rare. CT is invaluable for detecting this lesion. Herein, we describe a patient with worsening dyspnea due to involvement of the cricoid cartilage with multiple myeloma. CT not only detected the lesion but also revealed additional classical lytic lesions of multiple myeloma. In patients with worsening dyspnea laryngeal involvement should be included in the differential diagnosis.

Is routine bilateral neck dissection absolutely necessary in the management of N0 neck in patients with supraglottic carcinoma?

The supraglottic larynx has a rich lymphatic network that places patients with supraglottic laryngeal carcinomas at high risk for early dissemination of the disease into the cervical lymphatics. Therefore, elective neck treatment of clinically N0 neck in patients with supraglottic carcinomas is widely accepted as a standard approach. However, the issue whether elective neck treatment should routinely be directed on both sides of the neck is still controversial. The present study is aimed at determining whether T2-T4 stage supraglottic carcinomas require bilateral neck dissection in the management of N0 necks. We designed a prospective study on 72 patients with N0 supraglottic laryngeal carcinoma. Patients were divided into three groups according to the site and extension of the primary tumors. Group I consisted of 21 patients with lateralized (clear lateral) lesion reaching but not crossing the midline. Group II comprised 25 patients with cancer largely involving one side and crossing to the midline. Group III included 26 patients with carcinoma equally involving both sides of the larynx or growth into the midline larynx. All patients underwent bilateral lateral neck dissection in conjunction with various types of laryngectomies selected to the status of the primary. Of the 72 patients, 16 were found to have occult regional metastases in pathologic examination (9 pN1, 4 pN2b, 3 pN2c). The prevalence of occult metastases proportionally increased with T stage from 8.3 to 22.7 and 31.2%, respectively, for T2, T3 and T4. Bilateral neck metastases were found in 2 of 26 patients (7.7%) with central lesions. There was only one patient (4%) with both ipsilateral and contralateral lymph node metastasis in group II. None of the 21 patients with lateral lesion (group I) had contralateral neck metastasis. Routine bilateral elective neck dissection may not be a part of the surgical procedure in all supraglottic laryngeal carcinoma patients. Bilateral neck dissection should be preferred for cases with central tumors and lateral tumors with positive nodes in the ipsilateral side of the neck.

Functional lingual thyroid as unusual cause of progressive Dysphagia.

Lingual thyroid is an uncommon developmental anomaly and is the result of failure of the thyroid to descend from the foramen caecum to its prelaryngeal site. The lingual thyroid with a functioning thyroid gland in the neck is even more rare. In this report, we describe a 40-year-old female patient with a foreign body sensation and progressive dysphagia caused by ectopic lingual thyroid that is 5 x 4 x 3 cm in size. Here we present a patient with functional lingual thyroid gland who had had thyroidectomy 20 years ago. Lingual thyroid, which is like a malignant mass, was excised by external approach without complications.