Impact of anatomical abnormalities on velopharyngeal insufficiency in patients with submucous cleft palate.

BACKGROUND: Submucous cleft palate (SMCP) is a congenital anomaly characterized by the presence of Calnan's triad. However, in clinical practice, it is common for individuals to exhibit one or two anatomical abnormalities within the triad. Furthermore, the definition of SMCP has been diverse and ambiguous in literature. Therefore, this study aimed to analyze the correlation between anatomical abnormalities and development of velopharyngeal insufficiency (VPI). METHODS: We conducted a retrospective analysis of 99 patients referred to our clinic for speech issues or anatomical abnormalities identified during routine oral examinations from January 2012 to June 2023. A single surgeon performed all physical examinations. We evaluated the presence of bony notch, zona pellucida, and bifid uvula, assigned a score to each abnormality, and analyzed their correlation with VPI. The correlation of each of the abnormalities with VPI development was examined, along with the relationship between the number of abnormalities and VPI. RESULTS: Among the 99 patients, 27 were diagnosed with VPI. Only the bony notch had a significant correlation with VPI development. The incidence of VPI tended to increase with the presence of more anatomical abnormalities. VPI occurred in approximately 40% of patients exhibiting all three anatomical abnormalities. CONCLUSION: The study findings highlight the importance of meticulous intraoral examinations in patients with SMCP and careful monitoring of patients with a bony notch or two or more anatomical abnormalities.

Hair Tourniquet Syndrome Involving the Uvula Secondary to an Airway Foreign Body.

Hair tourniquet syndrome is a rare condition that can cause ischemia and necrosis secondary to hair fibers constricting a patient's appendages. Typically, the syndrome affects patients aged two to six months. Hair tourniquet syndrome often involves the toes, fingers, or genitalia, and it has been rarely reported to have oropharyngeal manifestations. Accurate and timely treatment of this syndrome is imperative to save the involved appendage. We discuss a case of a six-month-old female who presented to the emergency room (ER) with increased agitation and was found to have hair tourniquet syndrome of the uvula, requiring the removal of the foreign body in the operating room (OR).

The burdens, associated factors, and reasons for traditional uvulectomy in Ethiopia: A systematic review and meta-analysis.

INTRODUCTION: Traditional uvulectomy is a harmful procedure in which the entire or a portion of the uvula is removed by traditional practitioners. It causes complications like septicemia, transmission of infectious diseases, anemia, excessive bleeding, infection, tetanus, meningitis, and death. A summary of national data was lacking, thus, the study aimed to determine the pooled national burden and determinants of traditional uvulectomy in Ethiopia. METHODS: Studies were retrieved from PubMed, EMBASE, CINHAL (EBSCO), Google Scholar, Web of Sciences, MEDLINE, Cochrane Library, SCOPUS, and Google databases. Finally, 19 studies were included. The methodological quality of the included studies was assessed using the JBI checklist. Data synthesis and statistical analysis were conducted using STATA Version 17 software. Heterogeneity and publication bias were assessed. Forest plots were used to present the pooled and summarized with random-effects meta-analysis models. RESULTS: Totally 19 articles with 23,559 study participants were included in this systematic review and meta-analysis. The overall pooled prevalence of uvulectomy in Ethiopia was 44 % (95 % CI: 31%-57 %). The highest prevalence was observed among studies conducted in the Tigray region (63 % (95 % CI: 34%-94 %), between the year 2011-2014 (58 % (95 % CI: 29%-87 %)) and, community setting (57 % (95 % CI: 36%-78 %). Mothers' educational status (AOR: 1.66, 95 % CI: 1.31-2.01) and residence (AOR: 1.70, 95 % CI: 1.16-2.23) were found to be significantly associated with traditional uvulectomy in Ethiopia. Frequently cited reasons for traditional uvulectomy were to prevent swelling, pus, and rupture of the uvula, for better care, prevention of sore throats and coughs, religion, and culture. CONCLUSION: The pooled result revealed that almost half of children are still subjected to traditional uvulectomy in Ethiopia. There is a need to intensify awareness creation campaigns against the practice by giving special attention to rural residents and uneducated ones.

[Functional and aesthetic significance of the uvula in cleft palate repair]. / Rol' yazychka v dostizhenii funktsional'nogo i esteticheskogo rezul'tata uranoplastiki.

OBJECTIVE: To investigate functional and aesthetic role of uvula in cleft palate repair. MATERIALS AND METHODS: Forty-one patients aged from 1 year 2 months to 7 years were included in this study with congenital cleft lip and/or palate. The morphological investigation of the resected hemi- uvula was done. Palatoplasty was performed in all cases. RESULTS: According to morphological results, most of the resected hemi-uvula consisted of vascularized fibrous tissue, covered with epithelium. In three groups of patients (with unilateral, bilateral and isolated cleft palate), the duration of the surgery and intraoperative blood loss did not exceed similar values for conventional methods. The volume of infusion therapy revealed a deficit of fluid intake of no more than 30%, which indicates early restoration of swallowing function. CONCLUSION: The technique of preserving one of the «hemi-uvulas¼ lead to excellent aesthetic results and increasing functionality. Resection of one of the «hemi-uvulas¼ is safe and physiological.

Bifid Uvula-An Enigma.

Facial development involves an intricate regulatory mechanism that accounts for numerous craniofacial abnormalities, common being orofacial clefts. Although cleft in the secondary palate accounts for one-third of orofacial clefts stills remains an under-researched domain. Hence, in this work, the authors put forth two non-syndromic, asymptomatic cleft uvulae reported among bimodal male patients of the Indian-Asiatic population who came up for dental screening. Most of the time, isolated/asymptomatic cleft uvula patients will be reluctant to further investigations and treatment. Although bifid uvula looks benign in most patients, it may sometimes be associated with catastrophic complications. To conclude, whenever bifid uvula is an incidental finding, it is the responsibility of the healthcare worker to plan a thorough patient workup as a primary preventive measure to rule out any complications whenever feasible. It can help us overcome many future unforeseen sequelae and emergency management due to bifid uvula.

Obstructive Sleep Apnea: A Case Report.

Obstructive sleep apnea (OSA) is characterized by repeated episodes of upper airway blockage and collapse during sleep accompanied by awakenings with or without oxygen desaturations. During obstructive sleep apnea events, the oropharynx in the back of the throat compresses, causing arousal, oxygen desaturation, or both, leading to fragmented sleep. The hyperplastic uvula is a common clinical finding in patients with obstructive sleep apnea. The various diagnostic and treatment modalities of obstructive sleep apnea are discussed in this article.

Benign Symptomatic Squamous Papilloma of the Uvula: A Case Report and Review of the Literature.

Squamous papillomas are benign lesions that are associated with human papillomavirus infection. Oral squamous papilloma of the uvula is uncommon and rarely causes symptoms. In this case report, we present a rare case of symptomatic squamous papilloma of the uvula where the patient complained of mass-related symptoms such as dysphagia and choking sensation. The lesion was surgically excised using electrocautery with excellent outcomes. In addition, we reviewed literature related to the lesion.

In Vivo Localization of the Human Velocity Storage Mechanism and Its Core Cerebellar Networks by Means of Galvanic-Vestibular Afternystagmus and fMRI.

Humans are able to estimate head movements accurately despite the short half-life of information coming from our inner ear motion sensors. The observation that the central angular velocity estimate outlives the decaying signal of the semicircular canal afferents led to the concept of a velocity storage mechanism (VSM). The VSM can be activated via visual and vestibular modalities and becomes manifest in ocular motor responses after sustained stimulation like whole-body rotations, optokinetic or galvanic vestibular stimulation (GVS). The VSM has been the focus of many computational modelling approaches; little attention though has been paid to discover its actual structural correlates. Animal studies localized the VSM in the medial and superior vestibular nuclei. A significant modulation by cerebellar circuitries including the uvula and nodulus has been proposed. Nevertheless, the corresponding neuroanatomical structures in humans have not been identified so far. The aim of the present study was to delineate the neural substrates of the VSM using high-resolution infratentorial fMRI with a fast T2* sequence optimized for infratentorial neuroimaging and via video-oculography (VOG). The neuroimaging experiment (n=20) gave first in vivo evidence for an involvement of the vestibular nuclei in the VSM and substantiate a crucial role for cerebellar circuitries. Our results emphasize the importance of cerebellar feedback loops in VSM most likely represented by signal increases in vestibulo-cerebellar hubs like the uvula and nodulus and lobule VIIIA. The delineated activation maps give new insights regarding the function and embedment of Crus I, Crus II, and lobule VII and VIII in the human vestibular system.

A Case of Recurrent Isolated Uvula Oedema Secondary to Obesity and Obstructive Sleep Apnoea.

A 34-year-old male presented as an emergency with sudden onset globus, stertor and choking whilst asleep. He had similar previous episodes that self-resolved. The patient's observations were all within normal range. On examination, he had a grossly enlarged, non-erythematous uvula and there were no signs of respiratory distress or stridor. He was managed with intravenous dexamethasone and an attempt at needle aspiration of the uvula was made but there was no clinical improvement in the patient's condition. Despite no improvement with therapy, he was monitored overnight for any signs of airway compromise and discharged the following morning. His symptoms completely resolved on follow-up in the otolaryngology clinic a week later. He was diagnosed with Quincke's oedema caused by his obesity and background of obstructive sleep apnoea. We discuss the various aetiologies, assessment, and management of Quincke's oedema.

The human egomotion network.

All volitional movement in a three-dimensional space requires multisensory integration, in particular of visual and vestibular signals. Where and how the human brain processes and integrates self-motion signals remains enigmatic. Here, we applied visual and vestibular self-motion stimulation using fast and precise whole-brain neuroimaging to delineate and characterize the entire cortical and subcortical egomotion network in a substantial cohort (n=131). Our results identify a core egomotion network consisting of areas in the cingulate sulcus (CSv, PcM/pCi), the cerebellum (uvula), and the temporo-parietal cortex including area VPS and an unnamed region in the supramarginal gyrus. Based on its cerebral connectivity pattern and anatomical localization, we propose that this region represents the human homologue of macaque area 7a. Whole-brain connectivity and gradient analyses imply an essential role of the connections between the cingulate sulcus and the cerebellar uvula in egomotion perception. This could be via feedback loops involved updating visuo-spatial and vestibular information. The unique functional connectivity patterns of PcM/pCi hint at central role in multisensory integration essential for the perception of self-referential spatial awareness. All cortical egomotion hubs showed modular functional connectivity with other visual, vestibular, somatosensory and higher order motor areas, underlining their mutual function in general sensorimotor integration.

Quincke's Disease Presenting After Cocaine Exposure.

Quincke's disease is a very rare form of upper airway angioedema, and it is characterized by a well-localized edematous reaction. Its epidemiology is not documented due to the rarity of this condition. Causes include allergic reactions, infectious diseases, and trauma, among others.  This article describes a rare case of a 27-year-old man who presented to the Emergency Department with isolated uvular edema associated with cocaine exposure.

Modeling the effect of gravity on periodic alternating nystagmus.

Periodic alternating nystagmus (PAN) is a rare oscillatory ocular motor disorder. The effects of gravity on the dynamic behavior of PAN can be studied by monitoring the nystagmus while changing head orientation. Previous studies of patients with PAN reached different conclusions about the effect of changing the orientation of the head relative to gravity on the ongoing PAN, either no effect or a damping of the nystagmus within several minutes. What neuronal circuits could account for the difference in the effects of gravity among PAN patients? We modeled how the brain resolves the tilt-translation ambiguity in normal individuals and added an unstable, oscillatory vestibular system generating PAN. PAN was suppressed in our patient in ear-down positions, in a similar pattern to that of a previously reported patient. This effect was simulated by reducing the gain of the projection of the "rotation feedback" loop to the velocity-storage integrator to approximately 5% of its normal value. With normal "rotation feedback" PAN is expected to dissipate quickly as soon as the head is rotated away from upright position. Moreover, by disconnecting the rotation feedback completely (gain = zero) the model simulated PAN that was reported to be unaffected by gravity. Thus, understanding the effect of this single parameter, the gain of the rotation feedback, can explain the observed variability among our own and previous studies.

Hair Tourniquet of the Uvula.

Hair tourniquet syndrome (HTS) is a condition, where a strand of hair encircles the base of an appendage. We report a rare case of HTS of the uvula and review relevant literature.

Cleft Palate in Apert Syndrome.

Apert syndrome is a rare genetic disorder characterized by craniosynostosis, midface retrusion, and limb anomalies. Cleft palate occurs in a subset of Apert syndrome patients. Although the genetic causes underlying Apert syndrome have been identified, the downstream signaling pathways and cellular mechanisms responsible for cleft palate are still elusive. To find clues for the pathogenic mechanisms of palatal defects in Apert syndrome, we review the clinical characteristics of the palate in cases of Apert syndrome, the palatal phenotypes in mouse models, and the potential signaling mechanisms involved in palatal defects. In Apert syndrome patients, cleft of the soft palate is more frequent than of the hard palate. The length of the hard palate is decreased. Cleft palate is associated most commonly with the S252W variant of FGFR2. In addition to cleft palate, high-arched palate, lateral palatal swelling, or bifid uvula are common in Apert syndrome patients. Mouse models of Apert syndrome display palatal defects, providing valuable tools to understand the underlying mechanisms. The mutations in FGFR2 causing Apert syndrome may change a signaling network in epithelial-mesenchymal interactions during palatogenesis. Understanding the pathogenic mechanisms of palatal defects in Apert syndrome may shed light on potential novel therapeutic solutions.

Tapia's syndrome in post-operative patient following orotracheal intubation.

Introduction: Tapia's syndrome is a rare condition that manifest due to unilateral extracranial nerve extension of cranial nerve 10 and 12 which occurred as a rare complication of Orotracheal Intubation in patient undergoing Laparoscopic appendectomy. Case presentation: A 30 year old male, a known case of normal variant Right bundle branch block and Gilbert syndrome underwent orotracheal intubation prior to general anesthesia for emergency laparoscopic appendectomy. Postoperatively while assessing the patient there was deviation of tongue on left side. Clinical findings and investigations: On examination of throat, atrophic and deviated uvula toward the right side was found. While protruding his tongue, tongue was deviated towards left side.Neurological examination revealed sluggish Gag Reflex. Brain Ct was done, which showed normal scan. Interventions and outcome: Tablet Prednisolone and logopedic therapy in combination helped in early recovery; which is almost 8 weeks. Patient was completely recovered in 2 month with gradual improvement of phonation, tongue tone and mobility. Relevance and impact: The intent of this report is to show how important it is for anesthesia providers and surgeons to understand Tapia's syndrome, its causes, and the fact that it can occur despite seemingly normal airway care and neck posture. We believe that by raising awareness of this uncommon issue, practitioners will be able to early identify this complication.

Molecular signalling involved in upper airway remodelling is enhanced in patients with obstructive sleep apnoea.

OBJECTIVE: This study aimed to elucidate whether molecular signalling involved in upper airway remodelling is enhanced in patients with obstructive sleep apnoea. METHOD: Twenty patients with mild obstructive sleep apnoea (control group) and 40 patients with moderate to severe obstructive sleep apnoea (obstructive sleep apnoea group) who desired uvulopalatopharyngoplasty were recruited for the study. After uvulopalatopharyngoplasty, surgical specimens of the uvula were subjected to haematoxylin and eosin, Masson's trichrome and immunohistochemical staining. Western blot and reverse transcriptase-polymerase chain reaction were used to evaluate the protein and messenger RNA expressions. RESULTS: The obstructive sleep apnoea group showed more severe inflammation, increased collagen deposition and higher immunohistochemical staining intensity for TGF-ß and MMP-9 as well as higher protein and messenger RNA expression of MMP-9, VEGF, TGF-ß, p38 MAPK, SMAD 2/3, AKT and JNK in the uvula than control group. CONCLUSION: Patients with obstructive sleep apnoea demonstrated more severe inflammation, increased airway remodelling, and increased protein and messenger RNA expression of pro-inflammatory and pro-fibrotic cytokines in the uvula than control participants.

Modified Uvuloplasty for Achieving Aesthetically Desired Uvula in Cleft Palate Repair.

Background: Uvula is a delicate structure in comparison with the rest of the palate. During cleft palate repair, surgeons may find it challenging to suture both the hemi-uvulae meticulously with conventional repair technique and achieve satisfactory aesthetic results. We have adopted few surgical steps in conventional uvular repair to make this procedure simpler and more effective in terms of aesthetic outcome. Methods: From 2016 to 2019, 386 patients underwent primary palatoplasty in our centre. Their uvula was repaired with conventional technique with having those modifications. Out of 386, 211 patients were followed for the 6 months postoperatively. Results: The uvular appearance was satisfactory in 82% of the cases. Undesired appearances of uvula were noted as deviated, bifid and small in 1%, 7% and 10% of cases, respectively. In none of the cases, complete dehiscence of the uvula was seen. Conclusion: The authors advocate simple modifications in conventional uvuloplasty to improve uvular aesthetics. This modified uvuloplasty is easy to practice and very promising in achieving an aesthetically desired uvula in cleft palate surgery.