An Unusual Case of Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type Masquerading as Dacryocystitis and Sinusitis.

Extranodal natural killer (NK)/T-cell lymphoma, nasal type (NNKTL) is a rare and highly aggressive non-Hodgkin lymphoma originating from NK or γδ T cells infected by Epstein-Barr virus (EBV). In the United States, NNKTL is usually noted in people of Asian or Hispanic descent. Natural killer/T-cell lymphoma, nasal type commonly involves the upper aerodigestive tract, including the nasopharynx, nasal cavity, Waldeyer's ring, and oropharynx. Extensive local destruction and invasion has been noted, especially of the paranasal sinuses, hard palate, and central nervous system; involvement of the nasolacrimal duct with dacryocystitis is yet to be reported. We report a rare case of a Hispanic man with extranodal NNKTL masquerading as persistent dacryocystitis and necrotizing sinusitis unresponsive to antibiotics and surgical intervention. An extensive background of necrosis and inflammation was noted on pathology, and additional analysis with immunohistochemistry and in situ hybridization after repeat biopsy were necessary for accurate diagnosis.

Epidemiology and resource utilization of ludwig's angina ED visits in the United States 2006-2014.

OBJECTIVES: Ludwig's angina is a potentially lethal submandibular space infection. We aim to describe the epidemiological characteristics of Ludwig's angina patients presenting to the emergency department (ED) and to examine outcomes and resource utilization to determine their burden on ED and hospitals. METHODS: Using the Nationwide Emergency Department Sample database, a nationally representative all-payer database, we retrospectively reviewed all ED visits between 2006 and 2014 for patients admitted with a primary diagnosis of Ludwig's angina (International Classification of Diseases, Ninth Revision, 528.3). We collected information including demographics, ED and inpatient charges, airway interventions, length of stay, and mortality. RESULTS: A total of 5,855 patients met our inclusion criteria. In our study population, the mean age was 44.5 years, with 54% males and 46% females. There were 75% insured and 25% uninsured. Overall median ED charges were $1,352 and median inpatient charges were $18,017.54, with a median length of stay of 3 days. As part of their management, 47.2% of the patients received a surgical drainage procedure, 3.3% required a surgical airway, and 4.6% required a nonsurgical airway. The overall mortality rate was 0.3%. CONCLUSION: Ludwig's angina remains a rare and potentially life-threatening condition. The mortality rate appears to be decreased from previous historical accounts, with airway intervention remaining a significant part of management. LEVEL OF EVIDENCE: 3 Laryngoscope, 129:2041-2044, 2019.

Low surgical complication rates in cochlear implantation for young children less than 1 year of age.

OBJECTIVE: To identify risk factors for perioperative morbidity among a large national cohort of pediatric patients undergoing cochlear implantation. STUDY DESIGN: Retrospective study utilizing the American College of Surgeons National Surgical Quality Improvement Program Pediatric database (2012-2013). METHODS: Pediatric cochlear implantation cases were identified using current procedural terminology 69930. Patients were categorized by age, and operative characteristics along with 30-day perioperative outcomes were analyzed. RESULTS: We identified 1,351 cases of pediatric cochlear implantation. The median age was 3.6 years, and 73 patients were less than 1 year of age. Of 21 complication occurrences (1.55%), superficial incisional surgical site infection (SSI) was the most common (n = 13, 61.9%). Thirty-nine patients (2.9%) required readmission. The median operative time was 142 minutes, and the mean postoperative length of stay was 0.58 days. When comparing patients younger than 1 year old to those 1 year or older, no significant differences were noted in complication rate, postoperative length of stay, or reoperation rate. Patients less than 1 year of age were more likely to be readmitted (6.9% vs. 2.7%, P = 0.04) and had longer mean operative times (191 minutes vs. 160 minutes, P = 0.0015). Steroid use was a risk factor for unplanned reoperation, SSI, and readmission. CONCLUSION: Despite a slight increase in readmission rates and operative times among patients less than 1 year of age, cochlear implantation appears to be safe in this population, with complication rates, reoperation rates, and postoperative lengths of stay similar to children undergoing the procedure at the current U.S. Food and Drug Administration-approved age of 1 year and older. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:720-724, 2017.

Otologic manifestations of Fanconi anemia and other inherited bone marrow failure syndromes.

BACKGROUND: The inherited bone marrow failure syndromes (IBMFSs) are diverse disorders with syndrome-specific features; their otologic and audiologic manifestations have not been well described. Our objective was to characterize these in patients with Fanconi anemia (FA), dyskeratosis congenita (DC), Diamond-Blackfan anemia (DBA), and Shwachman-Diamond syndrome (SDS), and to determine the association between physical findings and hearing loss. METHODS: Patients with an IBMFS underwent comprehensive clinical and laboratory evaluations and testing for syndrome-specific gene mutations. Hearing loss was measured by pure tone audiometry and otologic abnormalities by otomicroscopy. RESULTS: Patients included 33 with FA, 37 with DC, 32 with DBA, and nine with SDS. Hearing loss was most frequent in patients with FA (45%) and DBA (14%). The most common type of hearing loss in FA was conductive (65%). Absent or hypoplastic radius, noted in 21% of the patients with FA, was associated with hearing loss in all cases. Otomicroscopy was abnormal in 66% of patients with FA. Characteristic ear abnormalities included small tympanic membrane (66%), malformed malleus (57%), aberrant tympanic bony island (48%), narrow external auditory canal (EAC) (32%), and abnormal course of chorda tympani (34%). Ear malformations were almost always associated with hearing loss. Hearing loss was rare in patients with DC and SDS. CONCLUSIONS: FA is the major IBMFS with associated hearing loss, which is most commonly conductive. Radial hypoplasia or aplasia and characteristic congenital ear malformations are associated with hearing loss in patients with FA. Recognition of these syndrome-specific abnormalities should lead to earlier management of hearing loss.