Oculosympathetic paresis after selective neck dissection: A ‘distant’ complication

Publication date: Available online 17 February 2018
Source:Oral Oncology
Author(s): S. Mumtaz, J. Parrish, M. Singh




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The Infection Returns: A Case of Pulmonary Sporotrichosis Relapse after Chemotherapy

Background. Pulmonary sporotrichosis is a rare disease caused by a dimorphic fungus, Sporothrix schenckii. It is rarely found in association with malignancy. We present a case of pulmonary sporotrichosis recurrence after chemotherapy. Case Presentation. A 44-year-old man, treated for pulmonary sporotrichosis in the past, presented with dysphagia and was found to have squamous cell carcinoma of the esophagus. After undergoing chemotherapy, extensive cavitary lesions were observed on thoracic computed tomography scan. A bronchoalveolar lavage revealed the presence of Sporothrix schenckii sensu lato. Despite treatment with itraconazole, he eventually required a left pneumonectomy for progressive destructive cavitary lesions involving the left lung. Conclusion. This case highlights the importance of considering past fungal infections, albeit cured, in patients initiating immunosuppressive therapy.

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Serlopitant for the treatment of chronic pruritus: results of a randomized, multicenter, placebo-controlled phase 2 clinical trial

The substance P/neurokinin 1 receptor (NK1R) pathway is critical in chronic pruritus; anecdotal evidence suggests antagonism of this pathway can reduce chronic itch.

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Editorial Board

Publication date: March 2018
Source:Operative Techniques in Otolaryngology-Head and Neck Surgery, Volume 29, Issue 1





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An approach to successful slide tracheoplasty in the low birth weight neonate with single lung

Publication date: Available online 17 February 2018
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): Matthew S. Yong, Joanne Harrison, Robert G. Berkowitz, Sarath Ranganathan, Igor E. Konstantinov
The advent of the slide tracheoplasty technique and a multi-disciplinary approach has improved outcomes of congenital tracheal stenosis. However, tracheal surgery in younger patients with pulmonary malformations, especially low birth-weight neonates, has been associated with increased mortality. Patients with very low birth-weight, pulmonary malformations and prematurity may be palliated prior to definitive tracheal surgery due to the poor prognosis. We report a successful and unique approach of delaying tracheal reconstruction to allow growth and development in the premature, very low birth-weight neonate (1046g) with left lung agenesis.



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A novel missense mutation in GIPC3 causes sensorineural hearing loss in an Iranian family revealed by targeted next-generation sequencing

Publication date: May 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 108
Author(s): Samira Asgharzade, Mohammad Amin Tabatabaiefar, Javad Mohammadi-asl, Morteza Hashemzadeh Chaleshtori
BackgroundRecent studies have confirmed the utility of targeted next-generation sequencing (NGS), providing a remarkable opportunity to find variants in known disease genes, especially in genetically heterogeneous disorders such as hearing loss (HL).MethodsAfter excluding mutations in the most common autosomal recessive non-syndromic HL (ARNSHL) genes via Sanger sequencing and genetic linkage analysis, we performed NGS in the proband an Iranian family with ARNSHL. The NimbleGen sequence capture array captures codingsequences (CDSs) and 100 bp of the flanking sequence of 129 common deafness genes (cat# Oto-DA3). NGSwas performed on the IlluminaHiSeq2000. BWA, SAMtools, Picard, GATK, Variant Tools, ANNOVAR, and IGV were applied for Bioinformatics analyses. Data filtering with allele frequencies (<5% in the 1000 Genomes Project and 5400 NHLBI exomes) and PolyPhen2/SIFTscores (>0.95) prioritized 1indel (insertions/deletions) and 3 missense variants in this family. Eventually, Sanger sequencing, segregation pattern, the frequency in 50 healthy matched normal controls, and evolutionary conservation of amino acid residues revealed the pathogenic variant.ResultsWe identified a novel missenseGIPC3 mutation, c.472G > A (p.Glu158 Lys). The pathogenicity of GIPC3c.472G > A was supported by its absence in the population databases and the healthy-matched controls.Sanger sequencing confirmed co-segregation of the mutation with HL.ConclusionsThis study is the first report of the contribution of theGIPC3 gene to HL in the Iranian population.Targeted NGS allows easier detection of mutations in relatively uncommon deafness genes in families with ARNSHL.



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Development and standardization of Arabic words in noise test in Egyptian children

Publication date: May 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 108
Author(s): Tayseer Taha Abdel Rahman
ObjectivesTo develop and establish norms of Arabic Words in Noise test in Egyptian children.MethodTotal number of participants was 152 with normal hearing and ranging in age from 5 to 12 years. They are subdivided into two main groups (standardization group) which comprised 120 children with normal scholastic achievement and (application group) which comprised 32 children with different types of central auditory processing disorders.Arabic version of both Speech perception in noise (SPIN) and Words in Noise (WIN) tests were presented in each ear at zero signal to-noise ratio (SNR) using ipsilateral Cafeteria noise fixed at 50 dB sensation level (dBSL).ResultsThe least performance in WIN test occurred between 5 and 7 years and highest scores from 9 to 12 years. However, no statistically significant difference was found among the three standardization age groups. Moreover, no statistically significant difference was found between the right and left ears scores or among the three lists. When the WIN test was compared to SPIN test in children with and without abnormal SPIN scores it showed highly consistent results except in children suffering from memory deficit reflecting that WIN test is more accurate than SPIN in this group of children.ConclusionsThe Arabic WIN test can be used in children as young as 5 years. Also, it can be a good cross check test with SPIN test or used to follow up children after rehabilitation program in hearing impaired children or follow up after central auditory remediation of children with selective auditory attention deficit.



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Clinical and sociodemographic characteristics associated with disease severity in juvenile recurrent respiratory papillomatosis: A study of 104 patients in a tertiary care pediatric hospital

Publication date: Available online 17 February 2018
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): Lee Marvin Reyes, Juan León Aguilar, Perla Villamor, Carlos De La Torre, Alicia Álvarez, Edgar Mantilla, Hiram Álvarez-Neri
BackgroundJuvenile recurrent respiratory papillomatosis (JRRP) is generally aggressive and with a high recurrence rate. Currently, there is no definite curative treatment for JRRP. Therefore, a greater understanding of the aspects that influence the severity and prognosis of the disease is required.ObjectiveThe aim of this study was to establish the clinical and socioeconomic characteristics of pediatric patients with JRRP and its relationship with the severity of the disease in a tertiary care pediatric hospital.ResultsA strong relationship was observed between the severity of the disease and the age at the time of diagnosis, and having a tracheostomy. A moderate association was found between the severity of the disease and the age at the time of the study, the area of origin and the recurrence rate. None of the socioeconomic statuses had a correlation with the severity of the JRRP.ConclusionsJRRP is associated with multiple surgeries due to the recurrence and aggressiveness of the disease. The socioeconomic status does not seem to influence the severity of the disease, whereas younger patients and users of tracheostomy should receive a more strict follow-up given the increased risk of severe disease.



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Frequency-specific auditory brainstem response testing with age-appropriate sedation

Publication date: Available online 17 February 2018
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): Yael Levit, Dror Mandel, Idit Matot
ObjectiveAuditory brainstem response (ABR) testing is the gold-standard procedure for hearing evaluation in pediatric patients who cannot complete a behavioral hearing test. The amount of audiological information obtained depends on the quality of the patient's sleep during the test. In this retrospective database review, we aimed to assess the amount and the characteristics of the audiological information obtained in ABR testing in pediatric patients with age-appropriate sedation.MethodsA retrospective chart review was conducted on 501 consecutive ABR sedation sessions performed between January 2014 and June 2016 at the Tel Aviv Medical Center. Oral triclofos was used for the sedation of younger patients (3–24 months) and intravenous propofol for older patients (>24 months). The dataset included 370 triclofos sessions (in 337 patients) and 131 propofol sessions (in 126 patients).ResultsNone of the children developed complications, and all were discharged on the same day of the evaluation. Among the hearing-impaired children, a mean of 10 (1.8 SD) ABR threshold measurements was obtained from propofol-sedated patients and 9.4 (2.8 SD) measurements from those sedated with triclofos (P = 0.039). The major characteristics of the hearing loss, including its degree, type, and configuration, were obtained from all propofol-sedated patients and from 95% of those sedated with triclofos.ConclusionsA comprehensive evaluation of hearing status can be obtained in ABR testing with age-appropriate sedation. An average number of ∼10 threshold measurements were obtained during ABR testing with age-appropriate sedation, thus allowing for the evaluation of the degree, type and configuration of the hearing loss.



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Caregivers' perception of speech and language status and related needs in children with cleft lip and palate

Publication date: Available online 17 February 2018
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): B. Subramaniyan, Roopa Nagarajan, R. Vaidyanathan, B. Rajashekhar, B.W.C. Sathiyasekaran
Background and PurposeComprehensive management for communication disorders in individuals with CLP was provided in a community based program in two rural districts in Tamilnadu, India. This program provides services at the community to empower the families about CLP and treatment options.ObjectiveTo document the caregiver perceptions' of communication status and needs in children with repaired CLP.MethodSix Focus Group Discussions were conducted in Thiruvannamalai and Cuddalore districts in Tamil Nadu, India. Participants were 55 Caregivers of children (5–12 years of age) with repaired CLP.ResultsMost participants were concerned about their child's communication and few expressed concerns about specific errors in speech observed in their children. The caregivers recognized the need for and were willing to avail speech correction services. The focus group discussions highlighted some factors that need to be considered while planning a service delivery program for speech correction. The caregivers' expressed inability to independently carryout home training programs attributing it the lack of cooperation from their children. However they were open to receiving services if it was logistically convenient.ConclusionThis study provided the caregiver perceptions' of needs relating to communication in children with CLP. These need to be incorporated in the existing program to develop a model for speech intervention that would be feasible, sustainable and have good compliance.



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Foreword

Publication date: February 2018
Source:European Annals of Otorhinolaryngology, Head and Neck Diseases, Volume 135, Issue 1, Supplement
Author(s): B. Fraysse, V. Couloigner, D. Chevalier




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International consensus (ICON) on the ENT role in diagnosis of obstructive sleep apnea syndrome

Publication date: February 2018
Source:European Annals of Otorhinolaryngology, Head and Neck Diseases, Volume 135, Issue 1, Supplement
Author(s): M.C. Ouayoun, F. Chabolle, A. De Vito, C. Heiser, V.K. Paramasivan, F.A.W. Rabelo, B. Rotenberg, M.V. Suurna
During the 2017 IFOS ENT World Congress, an international expert panel was asked to clarify the role of ENT in the diagnosis process of the obstructive sleep apnea syndrome (OSA) in adults around the world. OSA is a major public health issue throughout the world. OSA is a highly prevalent disease with heavy clinical, social and economical outcomes. This high prevalence raises serious difficulties of diagnosis accessibility if only somnologists are able to confirm OSA diagnosis. First of all, the panellists reviewed the impact of OSA. Secondly, they defined the ENT role stressing ENT legitimacy, professional expertise and academic and institutional tasks. They also defined when somnologists were necessary. For the international panel, the ENT is a major player in the OSA diagnosis process.



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Auditory effects of autologous fat graft for TORP stabilization in the middle ear: a cadaveric study

Total ossicular replacement prostheses (TORP) are often used to re-establish ossicular coupling of sound in an ear lacking a stapes supra-structure. The use of TORPs, however, is associated with a 2/3 five yea...

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Takotsubo syndrome in a premenopausal patient

Takotsubo syndrome is a rare clinical condition, with a pathophysiology that is not fully understood. Characterised by an acute and usually reversible heart failure, the condition is often preceded by a stressful event. For the diagnosis of Takotsubo syndrome to be possible, the absence of coronary artery disease as a cause is required. We report a case of Takotsubo syndrome in a 47-year-old woman of fertile age. Electrical and echocardiographic presentations were classical in the patient. However, abnormally elevated cardiac biomarkers were registered. The patient showed signs of clinical improvement, with a follow-up angiography excluding coronary artery disease and therefore leading to a diagnosis of Takotsubo syndrome.

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Bladder necrosis: 'A man without a bladder

Since the use of antibiotics, bladder necrosis has become a rare condition. We report a case of bladder necrosis in a 90-year-old man following urinary retention. After insertion of a transurethral catheter (TUC), 2 L of urine was evacuated. In the following days, the TUC became intermittently blocked. Adequate bladder drainage could not be obtained despite intensive rinsing and placement of a suprapubic catheter. On surgical exploration necrosis of almost the entire bladder wall, except for the trigone, was encountered. Surgical debridement of the non-viable bladder wall without opening the abdominal cavity was conducted, and a TUC was placed in the Retzius cavity to ensure evacuation of urine. Since the patient was haemodynamically unstable, construction of a urinary diversion was waived and urinary drainage of the Retzius cavity by the TUC was accepted, resulting in adequate urinary drainage without compromising renal function.

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Disabling pansclerotic morphoea of childhood

Disabling pansclerotic morphoea (DPM) of childhood is a severe and often fatal variant of deep morphoea. It usually starts in childhood and rarely seen in adults. The course of the disease is progressive with lifelong morbidity in the form of joint contractures and immobility. The causes of mortality include complications such as sepsis, gangrene and cardiopulmonary involvement. Herein, we discuss the case of a 15-year-old girl with limb deformity and finger contractures, that is, bone involvement. The diagnosis of DPM of childhood was fortuitously made after the correction of limb deformity, when the patient was seen in the dermatology department for evaluation of skin discolouration on the thighs.

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When a polyp is not a polyp: incidental finding of a sigmoid schwannoma at first colonoscopic screening

Schwannomas, peripheral nerve sheath tumours arising from Schwann cells, are often associated with inherited disorders such as neurofibromatosis. Gastrointestinal schwannomas, while rare, have been reported in those without personal or family history of neurofibromatosis. Diagnoses of these lesions, however, typically follow evaluations prompted by symptomatic presentations associated with abdominal pain, rectal bleeding, change in bowel habits or positive results on faecal occult blood tests performed for colorectal cancer screening. Further, management of these predominantly benign lesions commonly incorporates surgical resection. We present the case of a sigmoid schwannoma found in an asymptomatic individual on first screening colonoscopy and treated with complete endoscopic polypectomy with anticipated surveillance colonoscopy.

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Pulmonary inflammatory myofibroblastic tumour misdiagnosed as a round pneumonia

Description

A 9-year-old male patient, previously healthy, presented with acute-onset chest pain. The pain was on the right lateral chest, 10/10 in severity and non-reproducible. The patient had 1-week history of cough that had been improving; no fever, weight loss, dyspnoea, palpitations or night sweats were reported. He had positive sick contacts. Examination was remarkable for slight decrease in breath sounds at the right lower lung field posteriorly. Initial chest radiograph showed a well-circumscribed opacity in the right lower lobe posteriorly with adjacent consolidation seen on the lateral view. The lesion appeared to be distinct from the cardiomediastinal silhouette (figure 1). Sonography was performed to characterise the nature of the lesion; however, it was difficult to visualise and only revealed mixed echotexture (figure 2). Contrast-enhanced CT scan of the thorax was then obtained. CT showed a right posterior basal segment consolidation with hypo-enhancing components...

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Late diagnosis: a case of rapidly progressive extranodal NK/T cell lymphoma, nasal type

Extranodal natural killer (NK)/T cell lymphoma, nasal type is a condition that has poor prognosis. Accurate diagnosis of lymphoma is made by pathological findings. We report a case of extranodal NK/T cell lymphoma, nasal type affecting the lung and liver and which was difficult to diagnose because of negative biopsy results from multiple sites. A 39-year-old man who had dry cough and fever for 1 month was referred to our hospital. He had pancytopenia and elevated serum levels of lactate dehydrogenase and soluble interleukin-2 receptor. Hepatosplenomegaly and multiple lung nodules were found on imaging study. Specimens of bronchoscopic lung, percutaneous liver, bone marrow and random skin biopsies were all negative. Open lung biopsy was not definitive. Unfortunately, disease progression was rapid and fatal before results of pleural fluid cytology and a second liver biopsy showed extranodal NK/T cell lymphoma, nasal type. This report focused on diagnostic planning for rapidly progressive extranodal NK/T-cell lymphoma, nasal type.

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A quintessential syndrome with a rare marvelling aetiology: Rosai-Dorfman disease presenting as Conus-Cauda syndrome

A 19-year-old woman presented with a history of severe lower backache and asymmetric proximal lower limb weakness during the past 3 months. In addition, she also suffered from lower motor neuron-type bladder and bowel symptoms. On examination, paraparesis was noted. Further, sensory examination suggested patchy asymmetric sensory loss in both lower limbs with saddle anaesthesia and areflexia. A clinical diagnosis of Conus-Cauda syndrome was made and contrast-enhanced MRI of the lumbar and sacral spine was done, which confirmed the presence of a mass lesion within the spinal canal involving the cauda equina extending up to the sacral level. She underwent partial resection of the lesion following which the neurological deficits and lower backache resolved. Histopathological evaluation and immunohistochemical analyses uncovered Rosai-Dorfman disease. There was no evidence of disease elsewhere in the body. Since the patient improved significantly following surgery and exhibited no further neurological worsening, she remains under close follow-up.

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NMDAR (N-methyl-D-aspartate receptor) encephalitis in a patient with MS (multiple sclerosis): a rare and challenging case

We present a rare case of N-methyl-D-aspartate receptor (NMDAR) encephalitis in a 41-year-old Caucasian woman, who initially presented with prominent neuropsychiatric symptoms on the background of pre-existing multiple sclerosis. Here, the authors navigate the muddy water between neurology and psychiatry, describing the caveats of antibody testing with a misdiagnosed case of acute and transient psychotic episode. NMDAR encephalitis in MS is a rare condition, which can be easily confused with a new onset psychotic episode. This case report can be helpful in recognition and diagnosis of this rare condition. Making the right diagnosis is important since it can prevent an unnecessary radical treatment and long-term neuropsychiatric complications.

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2017 Thank you to our reviewers

The Editor would like to publicly acknowledge the people listed below who served as reviewers on the journal during 2017. Without their efforts, the quality of the journal could not be sustained.

A-Cienfuegos, Javier

A.C, Jyothi

Aaronson, Nicole

Abba-Aji, Adam

Abbas, Ossama

Abbas, Syed Hussain

Abbey-Mensah, Geraldine

Abbott, Iain

Abd El-Latif, Amani N

Abdalla, Ahmed

Abdallah, Hassane

Abdel Hay, Sameh

Abdel Razek, Ahmed

Abdeldayem, Hussein

Abdelgadir, Ibtihal

Abdul Haium, Abdul Alim

Abduljabbar, Fahad

Abdullah, Ibrahim

Abdullah, Nurul

Abdussalam, Abdullah

Abe, Kazuo

Abe, Tomomi

Abegunde, Ayokunle

Abeygunasekara, Anuruddha

Abid, Noina

Abou Dargham, Hanadi

Abourazzak, Sana

Abreo, Kenneth

Abreo, Mohini

Abu Amna, Fatima

Abu Bakar, Mohd Zulkiflee

Abu Freha, Naim

Abu Saadeh, Feras

Abu-Arafeh, I

Abu-Zaid, Ahmed

Abuomara, Hossamaldin

Acar, Fahrettin

Aceto, P

Acheampong, Derrick

Acien, Pedro

Adachi, Masanori

Adam, D.N

Adam, Ervin

Adam, Ishag

Adams, Daniel

Adams, Derick

Adams, G. G. W

Adams, Nicholas

Adams, Phil

Adamski, Jill

Adebajo, Ade

...

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A child with tubulointerstitial nephritis and uveitis (TINU) syndrome

Tubulointerstitial nephritis and uveitis syndrome is an uncommon disease, probably underdiagnosed in clinical practice. Its aetiology and pathogenesis remain unknown. This syndrome is defined by an association of uveitis and tubulointerstitial nephritis, with no evidence of systemic disease or infection that might cause both ocular and renal inflammation. Renal and ocular manifestations may not occur simultaneously, making the diagnosis even more challenging. Treatment includes topical and oral corticosteroids. Renal involvement usually resolves spontaneously with full recovery of kidney function, however uveitis can persist or recur years after its initial presentation. We report a case of a 13-year-old girl with tubulointerstitial nephritis and uveitis syndrome.

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Editorial Board

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Comorbidities of pyoderma gangrenosum: a retrospective multicentric analysis of 126 patients

Abstract

Pyoderma Grangrenosum (PG) is a rare neutrophilic dermatosis with challenging diagnosis and unclear underlying mechanisms. Multifactorial causes are purposed, including neutrophilic dysfunction, aberrant cellular immunity and cytokinic activities, and genetic predispositions¹. An association with a systemic disease is classically described in 25 to 75% of cases, including inflammatory bowel diseases (IBD), inflammatory rheumatological diseases (IRD) and hematological disorders. Association of PG with cardiovascular disorders have also been proposed ^2,3. Due to its rarity, the literature on PG-associated comorbidities remains limited to date.

This article is protected by copyright. All rights reserved.

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Extraordinary disease-free survival in a rare malignant extrarenal rhabdoid tumor: a case report and review of the literature

Malignant extrarenal rhabdoid tumor of the gastrointestinal tract is rarely reported in the literature. It is characterized by poor prognosis and aggressive metastatic features.

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Auditory effects of autologous fat graft for TORP stabilization in the middle ear: a cadaveric study

Abstract

Background

Total ossicular replacement prostheses (TORP) are often used to re-establish ossicular coupling of sound in an ear lacking a stapes supra-structure. The use of TORPs, however, is associated with a 2/3 five year failure rate due to their anatomic instability over time in the middle ear. The use of autologous fat to try and stabilize TORPs may improve long-term results with this challenging ossicular reconstruction technique.

Methods

A cadaveric temporal bone model was developed and laser Doppler vibrometry was used to measure and record round window membrane vibration in response to sound stimulation under the following conditions: normal middle ear, middle ear filled with fat, normal middle ear with TORP prosthesis, TORP prosthesis with fat around its distal end and TORP prosthesis with fat filling the middle ear. Fourteen temporal bones were used.

Results

There was a significant decrease in round window membrane velocity after filling the middle ear with fat in both the normal middle ear (− 8.6 dB; p < 0.0001) and prosthesis conditions (− 13.7 dB; p < 0.0001). However, there was no significant drop in round window membrane velocity associated with using fat around the distal end of the TORP prosthesis as compared to the prosthesis without fat condition (p > 0.05).

Conclusions

Autologous fat around the distal end of a TORP prosthesis may not be associated with any additional hearing loss, as demonstrated in this cadaveric model. The additional hearing loss potentially caused by using fat to completely surround the prosthesis and fill the middle ear is probably not clinically acceptable at this time, especially given the unknown way in which the fat will atrophy over time in this context.

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