[Clinical characteristics, diagnosis and treatment of nasal cartilage mesenchymal hamartoma in infants].

Objective: To explore the clinical characteristics, pathological features, and diagnosis and treatment strategies of nasal chondromesenchymal hamartoma (NCMH) in infants and young children. Methods: A retrospective analysis was conducted on seven cases of NCMH infants and young children admitted to Beijing Children's Hospital, Capital Medical University from April 2015 to January 2022. The cohort included 5 males and 2 females, aged from 6 days to 2 years and 3 months. General information, clinical symptoms, imaging findings, treatment plans, postoperative complications, recurrence and follow-up time were collected, summarized and analyzed. Additionally, immunohistochemical characteristics of the lesion were examined. Results: The clinical symptoms of 7 children included nasal congestion, runny nose, open mouth breathing, snoring during sleep, difficulty feeding, and strabismus. All patients underwent electronic nasopharyngoscopy examination, with 5 cases of tumors located in the right nasal cavity and 2 cases in the left nasal cavity. No case of bilateral nasal cavity disease was found. All 7 patients underwent complete imaging examinations, with 5 patients underwent MRI and CT examinations, 1 patient underwent CT examination only, and 1 patient underwent MRI examination only. The CT results showed that all tumors were broad-based, with uneven density, multiple calcifications and bone remodeling, and some exhibited multiple cystic components. The MRI results showed that the tumor showed low signal on T1 weighted imaging and high or slightly high signal on T2 weighted imaging. All patients were diagnosed through histopathological examination and immunohistochemistry, including 7 cases of Ki-67 and SMA (+), 5 cases of S-100 and Vimentin (+), and all EMA and GFAP were negative. All patients underwent endoscopic resection surgery through the nasal approach, with 3 cases using navigation technology. Five cases of tumors were completely removed, and two cases of tumors were mostly removed. No nasal packing was performed after surgery, and no postoperative nasal, ocular, or intracranial complication occurred in all patients. Follow up assessments conducted 6 to 84 months post-surgery revealed no instances of tumor recurrence in any of the patients. Conclusions: The clinical symptoms of children with NCHM mainly depend on the size and location of the tumor. Nasal endoscopic surgery is the main treatment method. In cases where critical structures like the skull base or orbit are implicated, staged surgical interventions may be warranted. Long-term follow-up is strongly advised to monitor for any potential recurrence or complications.

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.

[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.

ChatGPT as an information tool in rhinology. Can we trust each other today?

PURPOSE: ChatGPT (Chat-Generative Pre-trained Transformer) has proven to be a powerful information tool on various topics, including healthcare. This system is based on information obtained on the Internet, but this information is not always reliable. Currently, few studies analyze the validity of these responses in rhinology. Our work aims to assess the quality and reliability of the information provided by AI regarding the main rhinological pathologies. METHODS: We asked to the default ChatGPT version (GPT-3.5) 65 questions about the most prevalent pathologies in rhinology. The focus was learning about the causes, risk factors, treatments, prognosis, and outcomes. We use the Discern questionnaire and a hexagonal radar schema to evaluate the quality of the information. We use Fleiss's kappa statistical analysis to determine the consistency of agreement between different observers. RESULTS: The overall evaluation of the Discern questionnaire resulted in a score of 4.05 (± 0.6). The results in the Reliability section are worse, with an average score of 3.18. (± 1.77). This score is affected by the responses to questions about the source of the information provided. The average score for the Quality section was 3.59 (± 1.18). Fleiss's Kappa shows substantial agreement, with a K of 0.69 (p < 0.001). CONCLUSION: The ChatGPT answers are accurate and reliable. It generates a simple and understandable description of the pathology for the patient's benefit. Our team considers that ChatGPT could be a useful tool to provide information under prior supervision by a health professional.

Pathogenic Somatic Mutation of DICER1 and Clinicopathological Features in Nasal Chondromesenchymal Hamartomas: A Series of Nine Cases.

Nasal chondromesenchymal hamartoma (NCMH) is a rare benign polypoid mesenchymal tumor arising in the nasal cavity and/or paranasal sinuses. Recognizing these sporadic, rare lesions is crucial, as surgical complete removal of the mass is the common treatment approach. This retrospective study analyzed the demographics, symptoms, and imaging data of 9 patients diagnosed with NCMH between January 2017 and June 2023, possibly representing the largest single-center adult case cohort to date. Diagnostic techniques included nasal endoscopy, CT/MRI scan, immunohistological studies, and morphologic comparisons. Pathologic specimens were subjected to Sanger sequencing of exons 24 and 25 of DICER1. The average age of 9 cases was 24.4 years, and the oldest was 55 years. Four of the patients were children, ranging from 1 year old to 11 years old, with an average of 4.5 years. Nasal congestion is the most common registered symptom. Endoscopic findings showed that most patients had smooth pink neoplasms or polypoid masses in the nasal meatus. Radiologic scanning revealed soft-tissue density masses that occupied the nasal cavity. Histologically, the characteristic structure of NCMHs is immature cellular cartilage nodules and mature cartilage nodules distributed in a loose mucoid matrix. Five of the 9 patients had somatic DICER1 missense mutations. Four of the patients with DICER1-mutated NCMH exhibited a p.E1813 missense hotspot mutation. We also report a case of a rare p.P1836H missense mutation. The detected DICER1 somatic mutations provide compelling evidence of an association with the DICER1 tumor family. We emphasize the importance of pathologic consultation and the need for pathologists to accumulate experience in NCMH diagnosis to avoid misdiagnosis.

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.

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.

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.

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.

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.

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.

A 3-year follow-up study after nasal reconstruction surgery in patients with empty nose syndrome.

KEY POINTS: Empty nose syndrome (ENS) is characterized by a patent nasal airway and a sense of nasal obstruction. ENS and psychological symptoms improved after surgery and remained stable for up to three years. Identifying residual disease is necessary for enhancing therapeutic outcomes in ENS patients.

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.