Intraosseous Hemangioma of the Ethmoid Sinus.

Intraosseous hemangiomas, also referred to as bony hemangiomas, arising within the nasal cavity are exceedingly rare with only 2 cases arising in the ethmoid region. Despite their rarity, they are important to consider in a patient presenting with long-standing nasal congestion. While characteristic findings on computed tomography imaging may be observed, biopsy is diagnostic. En bloc resection using a transnasal endoscopic approach with or without preoperative embolization is the treatment of choice for intranasal intraosseous hemangiomas.

Intrathyroidal Parathyroid Cyst in a Middle-aged Woman.

Parathyroid cyst is a rare entity that can closely mimic thyroid and brachial cleft cysts, particularly when located within the thyroid gland. Most commonly, the cysts are non-functional but can produce compressive symptoms. The cyst contains watery content with elevated PTH levels, specifying parathyroid origin1. Parathyroid cysts should be considered in the differential in patients with a neck mass. This study describes a rare case of a middle-aged woman with a history of a symptomatic intrathyroidal parathyroid cyst discovered intraoperatively.

Combination of Melolabial Interpolation Flap and Nasal Sidewall and Cheek Advancement Flaps Allows for Repair of Complex Compound Defects.

BACKGROUND: The paramedian forehead flap (PMFF) is the repair of choice for large nasal defects involving multiple cosmetic subunits. However, the PMFF may not be optimal for all patients. The combination of a melolabial interpolation flap (MLIF) and a nasal sidewall flap may serve as an alternative. OBJECTIVE: To present the surgical technique and aesthetic outcomes of the alternative reconstruction for surgical defects of the lower nose. MATERIALS AND METHODS: Fourteen patients with multisubunit nasal Mohs defects reconstructed alternatively were identified from 2 academic centers in this retrospective case series. Illustrations and photographs were used to demonstrate surgical technique and outcomes. Final aesthetic results were analyzed using the Patient and Observer Scar Assessment Scale. RESULTS: The physician observer rated the scar outcome a mean score of 10.9 ± 3.3. Patients rated their results as a mean of 9.1 ± 4.7. The mean "Patient" Overall Opinion score was 2.3 ± 2.6 and the mean "Observer" Overall Opinion score was 1.9 ± 0.9. No patients reported problems with nasal airflow or obstruction, and cosmetic complications such as pincushioning or alar buckling were not observed. CONCLUSION: The combined MLIF and nasal sidewall flap is an alternative repair for complex distal nasal defects.

Audiometric assessment of pediatric patients with cystic fibrosis.

BACKGROUND: The purpose of this study was to evaluate hearing impairment in pediatric patients with cystic fibrosis (CF). METHODS: This is a retrospective analysis of the AudGen database generated by Children's Hospital of Philadelphia. Audiograms were analyzed for type of hearing loss (HL), pure-tone-average (PTA), laterality, and change in hearing over time. Medical charts were reviewed to identify factors that influence development and progression of hearing loss. RESULTS: 217 patients with CF were included in this study. 69 (31.8%) had hearing loss on initial audiogram. Chronic otitis media (OR: 2.4, 95% CI: 1.3-4.5, p<0.01), Eustachian tube dysfunction (OR: 2.4, 95% CI: 1.4-5.4, p<0.01), and otorrhea (OR: 6.3, 95% CI: 1.6-24.7, p<0.01) were positive predictors of HL. Children with a diagnosis of diabetes had more decline in hearing over time than those without diabetes (12.4±17.2dB worsening vs. -5.7±9.8dB improvement in PTA, p=0.014). CONCLUSION: This is the largest comprehensive analysis of all types of hearing loss in pediatric patients with CF. Our data suggest that children with more severe sinus disease may be at lower risk for inflammatory middle ear disease and subsequent hearing loss. Patients who develop complications of CF such as diabetes should be monitored frequently, and the use of ototoxic drugs should be limited if possible.

Hearing loss in children with primary ciliary dyskinesia.

OBJECTIVES: To evaluate the type and severity of hearing impairment in pediatric patients with primary ciliary dyskinesia (PCD) and relate these measures to patient demographics, treatment options, and other otologic factors. METHODS: A retrospective analysis of children with a diagnosis of PCD, Kartagener's syndrome, or situs inversus in the AudGen Database was conducted. Audiograms were analyzed for type of hearing loss (HL), severity, laterality, and progression. Medical charts were reviewed to identify factors that influence severity and progression of hearing loss. RESULTS: 56 patients met inclusion criteria and 42 patients had HL. 66.6% had bilateral and 33.3% had unilateral loss (70 total ears with HL). Conductive hearing loss (CHL) was the most common type of HL, though 30% of children had some sensorineural component to their hearing loss. 92.9% of children with HL received at least one diagnosis of otitis media, but HL did not improve in the majority (77.8%) of ears in our study regardless of ear tube placement. CONCLUSIONS: Slight to mild CHL and all types of otitis media are prevalent among patients with PCD, and some of these children have sensorineural hearing loss (SNHL). All patients diagnosed with situs inversus at birth should be evaluated by an otolaryngologist.

Characteristics and Progression of Hearing Loss in Children with Down Syndrome.

OBJECTIVES: To evaluate hearing impairment in children with Down syndrome, and to describe the factors that influence the severity of hearing loss or changes in hearing over time. STUDY DESIGN: Using the Audiological and Genetic Database (AudGenDB), audiograms of children with Down syndrome were analyzed retrospectively for type, severity, and laterality of hearing loss, as well as changes over time. Medical charts and imaging results were reviewed to identify factors influencing hearing loss. RESULTS: Among the 1088 patients with Down syndrome included in the study, 921 had hearing loss in at least 1 ear, 91.1% had bilateral hearing loss, and 8.9% had unilateral hearing loss (1760 total ears with hearing loss). Of the ears with hearing loss, 18.8% (n = 180) had moderate or worse hearing loss. "Undefined" hearing loss and pure conductive hearing loss (CHL) were the most common types, followed by mixed hearing loss and sensorineural hearing loss (SNHL). Three-quarters (75.4%) of the children had experienced chronic otitis media or more than 2 episodes of acute otitis media. Patients with bilateral, mixed hearing loss or a history of seizures were at risk for more severe hearing loss. CHL, absence of cholesteatoma, and placement of first ear tubes before age 2 years were associated with greater improvement in hearing over time, whereas SNHL and mixed hearing loss were associated with progressive decline. CONCLUSION: Children with Down syndrome who have bilateral, mixed hearing loss or a history of seizures are at risk for more severe hearing loss. SNHL and mixed hearing loss should not be overlooked in patients with CHL. All patients with Down syndrome will benefit from serial audiograms, especially those children with SNHL or mixed hearing loss, which is likely to worsen over time.

Hearing loss in children with growth hormone deficiency.

OBJECTIVE: Although insulin-like growth factor 1 (IGF-1) has been shown to be important for inner-ear development in animal models, little is known about the otologic and audiologic findings of children with growth hormone deficiency (GHD). The goal of this study is to evaluate the prevalence, type, and severity of hearing impairment in children with GHD. METHODS: Audiologic, otologic, and demographic data were recorded for children with a diagnosis of GHD in the AudGen database. Data for each patient were selected based on the first encounter with available complete audiometric data or the first encounter with a type of hearing loss documented. The patients were then stratified by type and severity of hearing loss, and otologic issues were documented. A separate cohort comprised of children with GHD without hearing loss was compared as a control. RESULTS: 209 children with GHD met inclusion criteria. 173 (83%) of these patients had hearing loss. 79% of losses were bilateral and 21% were unilateral (309 total ears with hearing loss). 293 of the 309 ears with hearing loss had audiograms with ear-specific thresholds; 47 had conductive, 24 had sensorineural, 65 had mixed and 157 had undefined hearing loss with incomplete audiograms. Pure-tone averages (PTA) were higher among patients with mixed hearing loss compared to patients with all other loss types. CONCLUSION: Hearing loss is prevalent in children with GHD with a predisposition to be bilateral. These findings suggest the need for increased awareness and routine hearing screening for patients with GHD. Further studies may elucidate the etiology of the hearing impairment in children with GHD to better aid pediatricians, endocrinologists, otolaryngologists and audiologists when assessing and managing these children.

Addressing Practice Gaps in Cutaneous Surgery: Advances in Diagnosis and Treatment.

IMPORTANCE: Cutaneous surgery is performed by otolaryngologists, plastic surgeons, oculoplastic surgeons, dermatologic surgeons, and some primary care physicians. Practice gaps exist among cutaneous surgeons, as do differences in how different physicians approach preoperative, intraoperative, and postoperative decision-making. OBJECTIVE: To present the newest and best evidence to close common practice gaps in cutaneous surgery. EVIDENCE REVIEW: We performed a detailed search of peer-reviewed publications that were identified through a search of PubMed/MEDLINE (January 1, 2000, through June 30, 2016) using the literature search terms "cutaneous surgery," "Mohs micrographic surgery," "plastic surgery," in combination with "safety," "cost," "anesthesia," "anti-coagulation," "bleeding," "pain," "analgesia," "anxiety," or "infection," among others. Bibliographies from these references, as well as meta-analyses, were also reviewed. FINDINGS: A total of 73 peer-reviewed studies, including randomized clinical trials, were selected to support the conclusions of the article. Levels of evidence were analyzed for selected studies using recommendations from the American Association of Plastic Surgeons based on guidelines from the Oxford Centre for Evidence-Based Medicine. Large cutaneous surgical resections can be done effectively and safely, taking steps to assure patient comfort under local anesthesia. Medically necessary anticoagulant and antiplatelet medication should be continued during cutaneous surgery. In preparation for surgery, patient anxiety and pain must be addressed. Music and anxiolytics limit anxiety, prevent cardiovascular compromise, and improve patient satisfaction. Cutaneous surgeons and support staff should carefully consider the dose and injection angle of local anesthetic. Postoperative opioids and topical antibiotics might cause harm to patients and should be avoided. Acetaminophen and ibuprofen provide adequate pain control with fewer adverse effects than opioid medications. CONCLUSIONS AND RELEVANCE: Clinicians performing cutaneous surgery should understand the importance of patient safety and comfort, as guided by recent evidence.

Propionibacterium skull base osteomyelitis complicated by internal carotid artery pseudoaneurysm.

The microbiology of skull base osteomyelitis (SBO) is evolving. We present here the first case of SBO caused by Propionibacterium acnes leading to the development of a pseudoaneurysm of the internal carotid artery. Otolaryngologists should recognize this pathogen as a potential cause of invasive temporal bone infection to optimize prompt diagnosis and treatment. Laryngoscope, 127:2337-2339, 2017.

Audiologic and otologic phenotype in children with Duane's Retraction Syndrome: A rare ophthalmologic disorder.

OBJECTIVE: The focus of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with Duane's Retraction Syndrome and to relate these measures to patient demographics and other otologic and audiologic factors. STUDY DESIGN: Retrospective AudGen Database analysis SETTING: Tertiary academic referral center PATIENTS: Pediatric patients in AudGenDB with a diagnosis of Duane's Retraction Syndrome (DRS). INTERVENTIONS: Appropriate audiologic, otologic, and demographic data were recorded. MAIN OUTCOME MEASURE: Seventy-nine patients (n = 79) met inclusion criteria. The first encounter with available audiometric data or the first encounter with hearing loss were documented. Audiograms were stratified by type and severity of hearing loss, and common associated medical issues were documented. RESULTS: 57 children had normal hearing; 22 had hearing loss. 9 ears had pure conductive hearing loss, 1 had pure sensorineural, 14 ears had components of both, and 79 had hearing loss that could not be specified. Multivariate regression revealed episodes of chronic otitis and craniofacial anomalies are associated with worse hearing loss. CONCLUSION: This study presents a detailed characterization of hearing loss in patients with Duane's retraction syndrome. Conductive and sensorineural hearing loss are both prevalent among these children. Careful and early audiologic evaluation of all patients with DRS is important.

Hormone receptor expression in patients with dermatofibrosarcoma protuberans.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma for which the exact etiology is unknown. Case reports exist of DFSP appearing and growing rapidly during pregnancy, suggesting a hormonal role. OBJECTIVE: Our goal was to determine the expression of estrogen receptors (ERs) and progesterone receptors (PRs) in patients with DFSP. METHODS: Archived formalin-fixed, paraffin-embedded tissue from patients with DFSP in the past 20 years at a single institution were analyzed for ER and PR using immunohistochemistry. A semiquantitative scoring method was used to evaluate the expression as positive or negative. Analysis was used to determine whether there was an association between receptor positivity and tumor site, age at diagnosis, sex, race, or disease recurrence. RESULTS: Forty-four patients with DFSP were included in the study. Tumors were 22.7% ER+/PR+, 34.1% ER+/PR-, 9.1% ER-/PR+, and 34.1% ER-/PR-. There was no significant association between expression of ER and PR and sex, age at diagnosis, race, or tumor location. Loss of receptor expression was observed in all recurrent tumors. LIMITATIONS: This study is limited by a lack of follow-up and a new scoring system. CONCLUSIONS: The data presented warrant additional study to determine hormone receptor function and the potential efficacy of antihormone therapies for the treatment of patients with DFSP.

Pediatric epidermoid cysts masquerading as ranulas: A case series.

Pediatric neck masses represent a variety of differential diagnoses. A common pathology in pediatric cystic neck tumors include ranulas, mucus retention cysts due to salivary gland obstruction. Epidermoid cysts are lesions infrequently encountered in the pediatric population and may appear similarly to ranulas on computed tomography imaging. MRI more easily differentiates these masses, and should therefore be the preferred imaging modality. Due to their distinct intraoperative management, ranulas and epidermoid cysts should be distinguished preoperatively through proper workup.

Incidence and Survival of Primary Dermatofibrosarcoma Protuberans in the United States.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma for which data on risk factors, incidence, and survival are limited. OBJECTIVE: The authors sought to establish a comprehensive report on the incidence of and survival from primary DFSP. METHODS: The authors used data from the 18 registries of the Surveillance, Epidemiology, and End Results Program from 2000 to 2010. RESULTS: Overall incidence was 4.1 per million person-years and steady over the decade. Trunk was the most common anatomic site except in older men. Incidence among women was 1.14 times higher than men (95% confidence interval [CI] of rate ratio: 1.07-1.22). Incidence among blacks was almost 2 times the rate among whites (95% CI of rate ratio: 1.8-2.1). Ten-year relative survival of DFSP was 99.1% (95% CI: 97.6-99.7). Increased age, male sex, black race, and anatomic location of the limbs and head as compared with the trunk were associated with higher all-cause mortality. CONCLUSION: This is the largest population-based study of DFSP derived from a cohort of almost 7,000 patients. The epidemiologic profile of DFSP differs from most skin cancers. Incidence is stable and highest among women and blacks. Worse survival is associated with increased age, male sex, black race, and anatomic location of the limbs and head.

Melanoma Incidence in Children and Adolescents: Decreasing Trends in the United States.

OBJECTIVE: To assess trends in the incidence of melanoma in children and adolescents in the US from 2000-2010. STUDY DESIGN: Using the Surveillance, Epidemiology, and End Results cancer registry data, we calculated age-adjusted incidence rates of melanoma in children and adolescents (age <20 years) from 2000-2010, as well as annual percent changes. We analyzed incidence trends using joinpoint regression models. We further stratified incidence rates and trends by age group, sex, race, and melanoma-specific characteristic (histology, anatomic site, Breslow depth, ulceration status, lymph node involvement, and presence of metastasis). RESULTS: We included 1185 pediatric patients (age <20 years) diagnosed with melanoma from 2000-2010. In patients age <20 years overall, we found a significant decreasing incidence (11.58% per year) from 2004-2010. Overall, significant decreasing incidence trends were also noted in males, melanoma located on the trunk, melanoma located on the upper extremities, superficial spreading melanoma, and melanoma with good prognostic indicators. When further subdividing the pediatric population by age group, these significant decreasing incidence trends were most notable in adolescents (age 15-19 years), decreasing 11.08% per year from 2003-2010. Furthermore, in 15- to 19-year-olds, decreasing trends were found to be significant in melanoma located on the trunk, superficial spreading melanoma, and melanoma with good prognostic indicators. CONCLUSIONS: Decreasing trends in melanoma incidence in the pediatric population from 2000-2010 stand in contrast to previous reports of increasing long-term incidence trends. Possible contributors to these decreasing trends include effective public health initiatives, decreased time spent outdoors, and increased sunscreen use.