[Characterization of otorhinolaryngologic manifestations in children with mucopolysaccharide storage disease typeâ and type â ¡].

Objective:To analyze the characteristics of otorhinolaryngological clinical manifestations in children with Mucopolysaccharide（MPS） type Ⅰ and type II in order to improve the knowledge of otorhinolaryngologists about this disease. Methods:Clinical data related to 55 children with MPS type Ⅰ and type II were retrospectively analyzed to investigate the clinical manifestations of MPS in ENT. Results:All 40 patients（72.72%） with MPS had at least one ENT symptom during the course of the disease, with 95% of them having an ENT symptom prior to the diagnosis of MPS; upper airway obstruction was the most common ENT symptom（34, 85.00%）, followed by recurrent upper respiratory tract infections（23, 57.50%）, and lastly, hearing loss（11, 27.50%）; all 26 patients had undergone at least one surgical procedure, of which 15（57.69%） had undergone ENT surgery, and all of these patients underwent ENT surgery before diagnosis. The most common ENT surgery was adenoidectomy. Conclusion:Early clinical manifestations of MPS patients are atypical, but the early and prevalent appearance of otolaryngologic symptoms and increased awareness of the disease among otolaryngologists has a positive impact on the prognosis of MPS.

Nasal Disorders.

Nasal obstruction, rhinorrhea, and epistaxis are common presenting concerns in primary care clinics. Nasal disorders affect the quality of life for many children and families. Rarely, these complaints may represent a life-threatening condition among infant obligate nasal breathers or cases of unusual pathology. The most common causes of rhinorrhea and nasal obstruction vary by age and include physiologic, infectious, allergic, foreign body, irritant, and traumatic causes. Less commonly, children may have congenital malformations, sinonasal masses, or autoimmune disease. The most common causes of epistaxis are inflammatory, environmental, and traumatic causes and medication misuse, but rarely, children may have predisposing anatomic, hematologic, or vascular abnormalities or even sinonasal tumors. In this article, we provide a thorough review of the common nasal disorders treated every day in primary care clinics and mention briefly some of the rare but serious cases that may be overlooked without considering a full differential diagnosis.

Intraoperative prevention of nasal mucosal injury and surgical field contamination during single-nostril transnasal endoscopic pituitary adenomas resection.

BACKGROUND: The common complications of transnasal endoscopic pituitary adenomas resection include nasal hemorrhage, olfactory disorder, nasal adhesion, and intracranial infection. Consequently, the protection of nasal mucosa and the prevention of surgical field contamination are critical. METHOD: We presented a step-by-step description of the methods of the disinfection and protection of nasal mucosa and the prevention of surgical field contamination during transnasal endoscopic pituitary adenomas resection, and these comprehensive measures to prevent these complications have not been documented. CONCLUSION: These measures effectively reduce the risk of nasal mucosal injury and surgical field contamination, and are easy to perform.

Two-flap technique with interpositional dermofat graft for anterior oronasal fistula closure in patients with cleft: A case series.

BACKGROUND: Oronasal fistula at the anterior hard palate is one of the common sequelae after cleft surgery, and the leakage negatively affects the patient's quality of life. Although several surgical techniques have been proposed for reconstruction, it remains challenging because of the scarred regional tissue with a high rate of fistula recurrence. In this study, we present the anterior oronasal fistula repair using a two-flap technique with an interpositional dermofat graft (DFG). METHODS: A retrospective review of anterior oronasal fistula repair performed by the senior author between April 2018 and August 2022 at the Craniofacial Center was conducted. Patients who underwent a fistula repair using the technique were further identified and investigated. RESULTS: Thirty-four operations were performed using the technique, and 31 fistulas were completely closed, with a success rate of 91.2%. The fistula symptom improved but persisted postoperatively in 3 patients, of whom 2 patients underwent a second fistula repair using the same procedure, resulting in successful closure. Fistula recurrence was significantly correlated with fistula size (p = 0.04). The DFG was simultaneously utilized for nasal dorsum and/or vermillion reconstruction in 28 cases. CONCLUSION: The two-flap technique enabled tension-free approximation, and the interpositional DFG facilitated watertight closure of the fistula, resulting in a high success rate of anterior oronasal fistula repair. The fistula closure could be combined with other revisional procedures for cleft-related deformities, where the DFG was simultaneously utilized.

Primary rhinoplasty in patients with bilateral cleft lip: Longitudinal results and comparison between closed and semi-open approaches.

BACKGROUND: Although several studies have shown that primary rhinoplasty in patients with cleft lip provides good outcomes with limited effect on nasal growth, the surgical procedure remains to be standardized. The purpose of this study was to evaluate the long-term outcome of primary semi-open rhinoplasty with Tajima reverse-U incision, compared with that of closed rhinoplasty. METHODS: Consecutive nonsyndromic patients with complete bilateral cleft lip and palate (n = 52) who underwent primary semi-open rhinoplasty between 2001 and 2016 were reviewed. Patients who underwent primary closed rhinoplasty (n = 61) and control group individuals were recruited for comparison. Computer-based standardized measurements of 2D photographs and panel assessments by laypersons were collected and statistically analyzed. RESULTS: In the comparative analysis at preschool age, semi-open rhinoplasty significantly improved the typical nasal deformities, including transversely oriented wide nostrils, short columella, and de-projected nasal tip, more effectively than closed rhinoplasty. Without major drawbacks, these parameters in the semi-open group were well maintained closer to those in the control group till skeletal maturity. After primary rhinoplasty, 54% of patients in the closed group and 4% in the semi-open group underwent intermediate rhinoplasty at preschool age. CONCLUSION: This study showed that the patients who underwent primary semi-open rhinoplasty achieved long-term and persistent outcomes that were closer to the normal nasal morphology compared with the patients treated with closed rhinoplasty, while avoiding intermediate rhinoplasty during the preschool to adolescent periods.

Novel classification system for adult Asian secondary bilateral cleft lip with nasal deformity that guides surgical strategy.

BACKGROUND: Bilateral cleft lip is a congenital defect often accompanied by secondary lip and nose deformity. The current classification system for secondary cleft lip deformity has limitations in guiding surgical planning. In this article, we report a method for secondary bilateral cleft lip classification that can guide surgery on the basis of the pathological anatomy of the columellar and upper lip. METHODS: Photographs of patients were retrospectively classified into four types on the basis of the ratio of columellar height to alar base width (CH/AW) and upper lip protrusion (UP) to lower lip, as follows: type I - with CH/AW ≥ 0.2 and UP ≥ 0; type II - with CH/AW ≥ 0.2 and UP <0; type III - with CH/AW < 0.2 and UP ≥0; type IV - with CH/AW < 0.2 and UP < 0. Surgical treatments and the change of the nasal profile were documented. RESULTS: A total of 105 patients from January 2008 to December 2018 were included in this study. The nasal profile was significantly improved in type III and IV patients with postoperative CH/AW values close to normal. The upper lip was distinctively retruded in type II and IV patients before treatment, and the postoperative view revealed improved upper lip protrusion with UP values close to normal. Ninety-eight patients reported satisfactory outcomes after treatment. CONCLUSIONS: The new classification method described provides key information regarding the deformity of different types of secondary bilateral cleft lip patients and provides clear guidance for surgical planning on the basis of the anatomical defect of each type.

Intralesional methotrexate as non-surgical therapy for giant keratoacanthoma on the nose.

Keratoacanthoma is an epithelial tumour derived from hair follicles. Clinical and histopathological features of keratoacanthoma can resemble that of squamous cell carcinoma. Different treatment alternatives have been described over the years including intralesional methotrexate injection. We present an interesting case of treatment of solitary keratoacanthoma lesion on the nose with intralesional methotrexate as non-surgical therapy.

Do Buccal Flaps Improve Velopharyngeal Insufficiency in Conversion Furlow Palatoplasty for Patients with Cleft Palate?

SUMMARY: Velopharyngeal insufficiency (VPI) is a complication following primary palatoplasty that can lead to hypernasality of the voice and other speech problems. The conversion Furlow palatoplasty for VPI can be performed with the addition of buccal flaps to provide additional tissue for palatal repair. In this study, the authors aimed to determine the effectiveness of buccal flaps with conversion Furlow palatoplasty in secondary management of VPI. A retrospective review of patients undergoing surgical repair of VPI between 2016 and 2020 was performed. Patients underwent either conversion Furlow palatoplasty alone (FA) or conversion Furlow palatoplasty with buccal flaps (FB) for VPI after primary straight-line repair of the palate. The authors reviewed medical records to collect demographics, operative information, and preoperative and postoperative speech scores. Of the 77 patients in the study, 16 (21%) had a revision that incorporated buccal flaps. The median age at cleft palate revision surgery was 8.97 years in the FA group and 7.96 years in the FB group ( P = 0.337). In the FA group, four patients (7%) developed a postoperative fistula, compared with zero patients in the FB group. The average time to follow-up after revision surgery was 3.4 years (range, 7 months to 5.9 years). Both cohorts demonstrated a decrease in hypernasality and total parameter scores postoperatively. The use of buccal flaps in revision Furlow palatoplasty could decrease the risk for postoperative complications. The use of data from a larger patient population from multiple institutions is warranted to determine true significance. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Pediatric Inferior Turbinate Hypertrophy: Diagnosis and Management. A YO-IFOS Consensus Statement.

OBJECTIVE: Pediatric inferior turbinate hypertrophy (PedTH) is a frequent and often overlooked cause or associated cause of nasal breathing difficulties. This clinical consensus statement (CCS) aims to provide a diagnosis and management framework covering the lack of specific guidelines for this condition and addressing the existing controversies. METHODS: A clinical consensus statement (CCS) was developed by a panel of 20 contributors from 7 different European and North American countries using the modified Delphi method. The aim of the CCS was to offer a multidisciplinary reference framework for the management of PedTH on the basis of shared clinical experience and analysis of the strongest evidence currently available. RESULTS: A systematic literature review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) criteria was performed. From the initial 96 items identified, 7 articles were selected based on higher-evidence items such as randomized-controlled trials, guidelines, and systematic reviews. A 34-statement survey was developed, and after three rounds of voting, 2 items reached strong consensus, 17 reached consensus or near consensus, and 15 had no consensus. CONCLUSIONS: Until further prospective data are available, our CCS should provide a useful reference for PedTH management. PedTH should be considered a nasal obstructive disease not necessarily related to an adult condition but frequently associated with other nasal or craniofacial disorders. Diagnosis requires clinical examination and endoscopy, whereas rhinomanometry, nasal cytology, and questionnaires have little clinical role. Treatment choice should consider the specific indications and features of the available options, with a preference for less invasive procedures. LEVEL OF EVIDENCE: 5 Laryngoscope, 134:1437-1444, 2024.

Surgical Management of Submucous Cleft Palate by Radical Muscle Dissection Veloplasty: Speech Outcomes in Patients with 22q11.2 Deletion Syndrome.

OBJECTIVE: To evaluate the benefit of anatomical muscle dissection repair for velopharyngeal insufficiency (VPI) in patients with submucous cleft palate (SMCP) with 22q11.2 deletion syndrome. DESIGN: Retrospective blinded randomised analysis of a surgeon's management over 10 years. SETTING: The study was performed at a specialised Paediatric hospital in the United Kingdom. PATIENTS: Children with SMCP and 22q11.2 deletion syndrome. INTERVENTIONS: All participants underwent radical muscle dissection veloplasty. OUTCOMES MEASURED: Pre- and post- operative measurements included severity of anatomical defect, speech samples and lateral images which were digitised, randomised then externally and blindly analysed using validated techniques. Stata software was used to perform statistical analysis. RESULTS: 57 children with 22q11.2 deletion syndrome were included in this analysis. Intra-operatively, the majority of cases were identified as SMCP Grade I anomalies. Post-operatively, a statistically significant improvement in hypernasality, resting palate length, palate length at maximum closure, palate excursion and gap size at maximum closure was observed. Secondary surgery was performed for 59% of patients by ten years. CONCLUSION: Muscle dissection repair improves hypernasality, palate closure function and the closure gap in patients with 22q11.2 deletion syndrome. Although over 50% of patients may require further surgery, muscle dissection repair should be a first step due to its utility at a younger age, when invasive investigations are impossible, its lower morbidity, speech and language benefits or altering the plans for less obstructive secondary surgery when it lead to reduced velo-pharyngeal gap and improved palate mobility even when adequate velo-pharyngeal closure was not achieved.

The Serpentine Sign: A Reliable Endoscopic and Radiographic Finding in Empty Nose Syndrome.

OBJECTIVE: Empty nose syndrome (ENS) is a relatively uncommon disease that greatly impacts the quality of life and presents diagnostic challenges. We sought to identify objective clinical findings unique to patients with ENS, and in doing so identified compensatory mucosal hypertrophy in an alternating, undulating swelling on endoscopy and coronal computerized tomography (CT) that we have termed the "Serpentine Sign." Here, we investigated whether this radiographic finding is a reliable manifestation in ENS patients. METHODS: Retrospective review was undertaken to identify ENS patients with past turbinoplasty, an ENS6Q score of at least 11/30, and symptomatic improvement with the cotton placement test. Control patients without complaints of ENS symptoms (ENS6Q < 11) were identified for comparison. ENS and control patients had coronal CT imaging available to evaluate for the Serpentine Sign, as well as ENS6Q scores, and histologic analysis of nasal tissue. RESULTS: 34 ENS and 74 control patients were evaluated for the presence of the Serpentine Sign. Of the 34 patients with ENS, 18 exhibited this radiographic finding on CT imaging (52.9%) compared to 0 of the 74 control patients (p < 0.0001). Further analysis demonstrated that ENS patients with the Serpentine Sign had lower median scores on ENS6Q than ENS patients without (17.5 vs. 22, p = 0.033). Histology revealed disorganized subepithelium rich in seromucinous glands similar to the nasal septum swell body. CONCLUSION: The "Serpentine Sign" is a unique presentation of hypertrophic change to the nasal septum soft tissue that is specific to ENS patients and may serve as a reliable radiographic and endoscopic finding in diagnosis. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:1089-1095, 2024.

Sublabial excision versus transnasal endoscopic marsupialization for nasolabial cysts: A systematic review and meta-analysis.

OBJECTIVES: To compare the intraoperative and postoperative outcomes of sublabial excision and transnasal endoscopic marsupialization, the two primary surgical approaches for nasolabial cysts. DESIGN AND SETTING: A comprehensive meta-analysis of studies identified from PubMed, Embase, and the Cochrane Library. PARTICIPANTS: Patients diagnosed with nasolabial cysts who underwent surgical treatment. MAIN OUTCOME MEASURES: Operative time, postoperative pain, overall postoperative complications, admission rate, length of hospital stay, use of general anaesthesia, medical costs, and recurrence rate. RESULTS: The pooled analysis revealed that the transnasal endoscopic marsupialization group had shorter operative time (mean differences [MD], -32.51; 95% confidence interval [CI], -38.52 to -26.51), reduced postoperative pain (MD, -4.25; 95% CI, -7.62 to -0.89), fewer overall postoperative complications (risk difference [RD], -0.68; 95% CI, -0.90 to -0.46), lower admission rates (RD, -0.86; 95% CI, -1.11 to -0.61), shorter hospital stays (MD, -1.74; 95% CI, -2.58 to -0.89), decreased use of general anaesthesia (RD, -0.40; 95% CI, -0.76 to -0.03), and reduced medical costs (MD, -229.69; 95% CI, -338.64 to -120.75). The recurrence rate between the two groups showed no significant difference (RD, -0.01; 95% CI, -0.05 to 0.04). CONCLUSION: Transnasal endoscopic marsupialization presents as a promising alternative to sublabial excision in the treatment of nasolabial cysts. It offers advantages like reduced operative time, decreased postoperative pain, fewer complications, lower admission rates, shorter hospital stays, diminished need for general anaesthesia, and cost savings. Clinicians can leverage these findings to select the most suitable surgical approach for their patients.

Impact of COVID-19 pandemic: increase in complicated upper respiratory tract infections requiring ENT surgery?

PURPOSE: This study investigates the impact of the COVID-19 pandemic on complicated upper respiratory tract infections requiring surgical intervention in a tertiary referral center. The aim is to understand the consequences of pandemic-related measures and their subsequent relaxation on the incidence and characteristics of upper respiratory tract infection-related complications. METHODS: Patients who underwent surgery as a complication of upper respiratory tract infections between December 2014 to February 2023 were included. Demographic information, surgical procedures, microbiological findings, and clinical outcomes were assessed and analyzed comparing pre-pandemic, pandemic and post-pandemic groups. RESULTS: 321 patients were enrolled, including 105 patients (32.7%) in the pediatric population. Comparison of pre-pandemic (n = 210), pandemic (n = 46) and post-pandemic periods (n = 65) revealed a statistically significant increase in complicated otologic infections requiring surgical intervention in the post-pandemic period compared to the pandemic period (p value = 0.03). No statistically significant differences in other surgical procedures or demographic parameters were observed. A statistically significant increase in urgent ear surgery in the pediatric population between the pandemic and the post-pandemic period (p value = 0.02) was observed. Beta-hemolytic group A streptococcal infections showed a statistically significant increase in the post-pandemic period compared with the pandemic period (p value = 0.02). CONCLUSIONS: Relaxation of COVID-19-related restrictions was associated with an increase of upper respiratory tract infection-related otologic infections requiring surgical intervention with an increasing rate of beta-hemolytic group A streptococcal infections. These findings highlight the importance of considering the impact of the pandemic on upper respiratory tract infection complications and adapting management strategies accordingly.

Use of a barrier membrane to repair congenital hard palate defects and to close oronasal fistulae remaining after cleft palate repair: seven dogs (2019-2022).

OBJECTIVE: To describe the use of a barrier membrane in dogs for repair of congenital hard palate defects and closure of oronasal fistulae (ONF) remaining after previous cleft palate (CFP) repair. ANIMALS: 7 client-owned dogs. METHODS: The hard palate defect was closed with medially positioned flaps (Von Langenbeck technique) or pedicle flaps (2-flap palatoplasty) and a membrane composed of autologous auricular cartilage from the pinna or allogenous fascia lata underlying the mucoperiosteal flaps. RESULTS: All palate defects were considered to have a high risk of dehiscence based on their type and size and the characteristics of the surrounding tissue. The barrier membrane was used in 5 dogs for repair of congenital hard palate defects and in 2 dogs for closure of ONF remaining after previous CFP repair. Resolution of clinical signs occurred in all cases. Complete success (ie, complete closure of the palate defect and absence of clinical signs) was achieved in 5 dogs (4 with congenital hard palate defects and 1 with an ONF remaining after previous CFP repair). The persistent ONF in 1 dog with functional success (incomplete closure, but no clinical signs) was smaller than prior to surgery. CLINICAL RELEVANCE: Barrier membranes underlying mucoperiosteal flaps may constitute an alternative technique in dogs for repair of congenital hard palate defects and closure of ONF remaining after previous CFP repair.

Deformity, etiology, solution, sequence (DESS): Facial analysis in rhinoplasty.

PURPOSE: Rhinoplasty is amongst the most challenging surgeries to perfect and can take decades. This process begins during residency; however, residents often have limited exposure to rhinoplasty during their training and lack a standardized method for systematically analyzing and formulating a surgical plan. The DESS (Deformity, Etiology, Solution, Sequence) is a novel educational format for residents that serves to increase their pre-operative comfort with the surgical evaluation and intraoperative planning for a rhinoplasty. MATERIALS AND METHODS: A qualitative study performed at a tertiary academic institution with an otolaryngology residency program evaluating three consecutive residency classes comprised of four residents per class. A 9-item questionnaire was distributed to measure change in resident comfort after utilizing the DESS during their facial plastics rotation. Questionnaire responses highlighted resident comfort with facial nasal analysis, identifying deformities, suggesting surgical maneuvers, and synthesizing a comprehensive surgical plan. RESULTS: Ten of the twelve residents surveyed responded. Of those that responded, comfort in facial nasal analysis, identification of common nasal deformities, surgical planning, and development of an overall surgical plan were significantly improved after completion of the facial plastic rotation. These residents largely attributed their success to the systematic educational format, with an average score of 4.8/5.0 (SD 0.42). CONCLUSION: While rhinoplasty is a challenging artform to master, systematic approaches to analysis and operative planning are vital for teaching and guiding residents. Through this novel methodology, residents display significant improvement in their comfort with facial nasal analysis and overall surgical preparation.

Simplified Septal Stabilization in Closed-Approach Low-Septal-Resection Dorsal Preservation Rhinoplasty.

BACKGROUND: Septal fixation is a basic step in low-septal-resection dorsal preservation rhinoplasty. Inadequate septal stabilization can lead to supratip depression or more severe saddle nose deformity. This paper presents a simple surgical maneuver to stabilize quadrangular septal cartilage instead of suture fixation to the anterior nasal spine. METHODS: Thirty patients who underwent closed-approach low-septal-resection dorsal preservation rhinoplasty between July and December 2021 were included in this retrospective study. Depending on the surgical maneuver performed to stabilize quadrangular septal cartilage, the patients were divided into 2 groups as follows: (1) a group in which the septum was stabilized with transfixion sutures (the transfixion suture group) and (2) a group in which the septum was fixed to the anterior nasal spine with suture (the anterior nasal spine suture fixation group). Standardized postoperative 12-month lateral-view photographs were scanned for the presence of supratip depression or saddle nose deformity that may indicate insufficient septum stabilization. RESULTS: Supratip depression was observed in 1 patient in the transfixion suture group (n = 16) and in 1 patient in the anterior nasal spine suture fixation group (n = 14; P > 0.05). No saddle nose deformity was observed in either group. CONCLUSIONS: Transfixion sutures seem effective in providing septal stabilization to prevent supratip depression or saddle nose deformity in closed-approach low-septal-resection dorsal preservation rhinoplasty. Transfixion sutures can be used for septal stabilization in closed-approach rhinoplasty, allowing the surgeon to take advantage of the ease of placement.

Two novel mutations within FREM1 gene in patients with bifid nose.

BACKGROUND: Bifid nose is a rare congenital deformity and the etiology is unknown. The purpose of this study was to report genetic variation in family of patients with bifid nose. METHODS: Twenty-three consecutive patients who were diagnosed with mild bifid nose were operated with z-plasty from 2009 to 2021. Three underage patients (a pair of twins and a girl) from two family lines, who came to our hospital for surgical treatment, were enrolled. Whole exome sequencing and Sanger sequencing were conducted. Z-shaped flaps were created and the cartilago alaris major were re-stitched. Photographs and CT scan before and after surgery were obtained. Clinical outcomes, complications and patients' satisfaction were evaluated and analyzed. The follow-up time ranges from 2 to 3 years (2.4 ± 1.2 years). RESULTS: Most patients were satisfied with the outcome (96.2%). The nasal deformities were corrected successfully with z-plasty technique in one-stage. FREM1 c.870_876del and c.2 T > C were detected with Whole exome sequencing, which have not been reported before. The results of Sanger sequencing were consistent with those of Whole exome sequencing. CONCLUSIONS: The newly detected mutations of FREM1 have a certain heritability, and are helpful to make an accurate diagnosis and provide a better understanding of bifid nose mechanism. Z-plasty technique can be an effective technical approach for correcting mild bifid nose deformity.