Contemporary Surgical Management of Craniofacial Fibrous Dysplasia Using Computer-Assisted Surgery and Intraoperative Navigation.

Craniofacial fibrous dysplasia (CFD) is a rare developmental disease of bone, which typically presents as a painless, expansile mass causing deformity of the craniofacial skeleton. In rare circumstances, compression of neurovascular structures may arise, causing symptoms such as pain, visual impairment, and hearing loss. Traditionally, CFD debulking has been performed with "freehand" techniques using preoperative imaging and anthropometric norms to determine the ideal amount of tissue removal. The advent of computer-assisted surgery, computer-aided design, and computer-aided manufacturing (CAD/CAM) has revolutionized the management of CFD. Surgeons can now fabricate patient-specific osteotomy/ostectomy guides, allowing for increased accuracy in bone removal and improved cosmetic outcomes. This series of 3 cases describe our institution's technique using patient-specific ostectomy "depth guides", which allow for maximum removal of fibro-osseous tissue while sparing deep and adjacent critical structures. These techniques can be widely applied to the craniofacial skeleton to assist in the surgical management of CFD.

Does resident involvement in tonsillectomy affect outcomes?

OBJECTIVE: Tonsillectomy is essentially a solo surgery with a well-described complication profile. It may serve as a good benchmark to evaluate the resident-as-surgeon. This study examined complications such as post-tonsillectomy bleeding in children undergoing tonsillectomy by attending surgeons (AS) or pediatric otolaryngologist-supervised residents. METHODS: Charts were reviewed of all children aged 12 and under who had tonsillectomy +/- adenoidectomy at a children's hospital between Jan 2019 and Dec 2020. Patient age, gender, BMI, indication for surgery, surgical technique, presence of a resident surgeon, primary bleeding, secondary bleeding, treatment of bleeding, other Emergency Room (ER) visits, and clinic phone calls were recorded. Binary logistic regression was performed. RESULTS: 2051 total children (1092 (53.2 %) males and 956 (46.6 %) females) with a mean age of 6.1 years (95 % CI 6.0-6.2) were included. 1910 (93.0 %) underwent surgery for tonsillar obstruction. 1557 (75.9 %) underwent monopolar cautery tonsillectomy. 661 (32.2 %) had a resident surgeon. 274 (13.4 %) had a related ER visit within 15 days. 18 (0.9 %) had a primary bleed and 155 (7.6 %) had a secondary bleed. Binary logistic regression showed that significant predictors of postoperative ER visits were patient age (OR = 1.101, 95 % CI = 1.050-1.154, p < .001) and resident involvement (OR = 0.585, 95 % CI = 0.429-,797, p < .001). Only age was associated with overall postoperative bleeding incidence (OR = 1.131, 95 % CI = 1.068-1.197, p < .001), as well as secondary bleeding (OR = 1.128, 95 % CI = 1.063-1.197, p < .001). There were no significant predictors of primary bleeding. CONCLUSION: Resident involvement in pediatric tonsillectomy is associated with decreased postoperative ER utilization and does not appear to increase common postoperative complications including bleeding and dehydration.

Are children with unilateral sensorineural hearing loss receiving cochlear implants?

INTRODUCTION: The US Food and Drug Administration (FDA) granted its first approval for cochlear implants (CI) in children with bilateral sensorineural hearing loss (SNHL) in 1990. In 2019, the FDA expanded CI indications to include children with unilateral SNHL. OBJECTIVE: The aim of this study was to assess the prevalence of children with unilateral SNHL in the population of new pediatric CI recipients between 2012 and 2021. METHODS: A retrospective analysis using the American College of Surgeons National Surgical Quality Improvement Pediatric database examined patients under 18 years of age with bilateral or unilateral SNHL who underwent CI between 2012 and 2021. Current Procedural Terminology code 69930 identified patients with 'cochlear device implantation, with or without mastoidectomy.' The percentage of children undergoing CI for unilateral versus bilateral SNHL during the study period was calculated and subjected to statistical analysis. RESULTS: 9863 pediatric CI patients were included with a mean age of 5.1 (95 % CI 5.1-5.2) years at the time of implantation. 7.5 % (N = 739) of patients had unilateral SNHL and 92.5 % (N = 9124) had bilateral SNHL. Children with bilateral SNHL undergoing CI were significantly younger (5.0 years versus 6.9 years for those with unilateral SNHL, p < .001). There was a statistically significant difference in the percentage of children receiving CI for unilateral versus bilateral SNHL (3.3 % in 2012 to 14.3 % in 2021, p < .001) before and after the FDA changes. CONCLUSIONS: The proportion of CIs placed for unilateral SNHL has increased annually even before 2019 when the FDA expanded its CI indications to include children with unilateral SNHL for the first time.

Prophylactic Antibiotics After Cleft Lip and Palate Reconstruction: A Review From a Global Health Perspective.

Orofacial clefts are common congenital deformities. Global initiatives have increased access to cleft care and reconstruction surgeries for cleft lip with or without cleft palate (CL/P), but there is no consensus on the use of postoperative prophylactic antibiotics. We conducted a narrative review using PubMed on the use of postoperative prophylactic antibiotics in CL/P surgery. A search of PubMed identified 30 potentially relevant articles, of which 15 were reviewed. There was no consensus among surgeons on prescribing patterns, but there was limited evidence that postoperative antibiotics reduce palatal fistulas. Notably, microbiological screening is not used to guide the choice of antimicrobial or to predict postoperative complications. Based on limited available data, we cannot make any strong evidence-based recommendations on prescribing postoperative antibiotics; however, we recommend that each cleft surgeon performing these procedures in lower-income countries without access to tertiary care centers consider the cost-benefit analysis of prescribing antimicrobials postoperatively, without antimicrobial screening, which showed no benefit.

Acute Herpetic Nasopharyngitis in an Adult Patient: A Case Report and Literature Review.

OBJECTIVE: To report a case of herpes virus-associated nasopharyngitis in an adult patient. METHODS: The patient's medical record was reviewed for demographic and clinical data. For literature review, all case reports or other publications published in English literature were identified using Pubmed with the MeSH terms "herpes," "nasopharyngitis," and "upper respiratory infection." RESULTS: A 40-year-old male presented for nasal congestion and a suspected nasal mass. Computed tomography of the sinuses revealed edematous changes in the nasopharynx which exerted a downward mass effect at the right aspect of the soft palate. Flexible fiberoptic laryngoscopy (FFL) revealed a lesion arising from the posterior aspect of the soft palate with extension into the posterior nasal cavity as well as copious mucopurulent secretions consistent with a superimposed acute sinusitis. Rigid nasal endoscopy demonstrated a friable and ulcerated lesion arising from the aforementioned anatomical location. Biopsy of this lesion and subsequent immunohistochemical analysis revealed a diagnosis of herpetic nasopharyngitis. CONCLUSIONS: Herpetic infection should be in the differential diagnosis of patients presenting with atypical symptoms of nasopharyngitis. Early accurate diagnosis and appropriate specific management can limit the duration of disease course and prevent further complications.

Cricopharyngeal Myotomy in National Surgical Quality Improvement Program (NSQIP): Complications for Otolaryngologists Versus Non-otolaryngologists.

OBJECTIVE: Comparing outcomes after cricopharyngeal myotomy (CM) performed by otolaryngologists (OTO) and non-otolaryngologists (NO). METHODS: A retrospective analysis of the 2014-19 ACS-NSQIP database (American College of Surgeons National Surgical Quality Improvement Program) of patients who underwent open CM (CPT code 43030) as their primary procedure. Analyzed variables include medical comorbidities, operative time, the total length of stay, readmission, reoperation, concurrent procedures, postoperative complications, and postoperative diagnoses. 183 patients were included, 97 (53%) females and 86 (47%) males. 120 had surgery by OTO and 63 by NO.  Results: There were no differences in preoperative morbidity. OTO had more outpatient surgeries compared to NO (p<.001). OTO had a longer mean operating time (p=.008). OTO had a higher proportion of concurrent laryngeal procedures and other unspecified procedures compared to NO, while NO had a higher proportion of concurrent esophageal procedures (p=.028). The total length of stay was not significantly different between the two groups. 5.8% OTO and 7.9% NO patients were readmitted for a related reason (p=.586). Complications were similar between the two groups (p>.05). NO had more postop diagnoses of acquired diverticula and achalasia of the stomach cardia, while OTO had more diagnoses of dysphagia and muscular dystrophy (p<.001).  Conclusion: There were differences in the surgical setting, length of procedure, concurrent procedures, and postop diagnoses between NO and OTO surgeons but similar complication rates.

Ciliary genes arl13b, ahi1 and cc2d2a differentially modify expression of visual acuity phenotypes but do not enhance retinal degeneration due to mutation of cep290 in zebrafish.

Mutations in the gene Centrosomal Protein 290 kDa (CEP290) result in multiple ciliopathies ranging from the neonatal lethal disorder Meckel-Gruber Syndrome to multi-systemic disorders such as Joubert Syndrome and Bardet-Biedl Syndrome to nonsyndromic diseases like Leber Congenital Amaurosis (LCA) and retinitis pigmentosa. Results from model organisms and human genetics studies, have suggest that mutations in genes encoding protein components of the transition zone (TZ) and other cilia-associated proteins can function as genetic modifiers and be a source for CEP290 pleiotropy. We investigated the zebrafish cep290fh297/fh297 mutant, which encodes a nonsense mutation (p.Q1217*). This mutant is viable as adults, exhibits scoliosis, and undergoes a slow, progressive cone degeneration. The cep290fh297/fh297 mutants showed partial mislocalization of the transmembrane protein rhodopsin but not of the prenylated proteins rhodopsin kinase (GRK1) or the rod transducin subunit GNB1. Surprisingly, photoreceptor degeneration did not trigger proliferation of Müller glia, but proliferation of rod progenitors in the outer nuclear layer was significantly increased. To determine if heterozygous mutations in other cilia genes could exacerbate retinal degeneration, we bred cep290fh297/fh297 mutants to arl13b, ahi1, and cc2d2a mutant zebrafish lines. While cep290fh297/fh297 mutants lacking a single allele of these genes did not exhibit accelerated photoreceptor degeneration, loss of one alleles of arl13b or ahi1 reduced visual performance in optokinetic response assays at 5 days post fertilization. Our results indicate that the cep290fh297/fh297 mutant is a useful model to study the role of genetic modifiers on photoreceptor degeneration in zebrafish and to explore how progressive photoreceptor degeneration influences regeneration in adult zebrafish.