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Ann Otol Rhinol Laryngol ; : 3489420933645, 2020 Jun 09.
Artigo em Inglês | MEDLINE | ID: mdl-32517491


OBJECTIVES: LMNA-associated familial partial lipodystrophy (FPLD) is a rare autosomal dominant A-type laminopathy characterized by variable loss and redistribution of subcutaneous adipose tissue, dyslipidemia, and insulin resistance. Though A-type lamins play a key role in nuclear membrane structure and regulation of cell proliferation, an association between cancer and LMNA-associated FPLD has not been reported. METHODS AND RESULTS: This report outlines the case of two biological sisters with LMNA-associated FPLD who developed hypopharyngeal squamous cell carcinoma in the absence of any other risk factors for head and neck cancer. CONCLUSION: These observations prompt further investigation into the potential role of A-type lamins in the development and progression of head and neck cancers.

Laryngoscope ; 2020 Feb 14.
Artigo em Inglês | MEDLINE | ID: mdl-32057094


A case is presented showcasing the feasibility of three-dimensional (3D) printing patient-specific fistula plugs as a bridge therapy to definitive management of pharyngocutaneous fistulae. For this patient, a set of 3D printed fistula plugs was successfully used with low morbidity and positive results in the management of this postoperative issue. Utilizing 3D printed fistula plugs could represent a novel yet safe therapy for patients requiring long-term management of similar wounds. Further studies are warranted to evaluate implementation of this practice on a widespread scale. Laryngoscope, 2020.

Otol Neurotol ; 40(9): 1224-1229, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31469794


OBJECTIVE: Previous research has shown that tumor growth during observation of small-to-medium sized sporadic vestibular schwannomas (VSs) occurs almost exclusively within 3 to 5 years following diagnosis. This has led some to consider ending surveillance after this interval. This study seeks to characterize a cohort of patients with tumors that exhibited late growth. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: Adults with sporadic VSs who initially elected observation with serial magnetic resonance imaging (MRI) surveillance. INTERVENTION(S): None. MAIN OUTCOME MEASURE(S): Linear tumor growth was measured in accordance with AAO-HNS reporting guidelines. Delayed growth was defined as growth ≥2 mm in linear diameter that was first detected 5 years or more from the initial MRI. RESULTS: From a total of 361 patients, 172 experienced tumor growth during the interval of observation. Fourteen of these 172 patients (8.1%) experienced late growth occurring at 5 years or beyond. Among patients with delayed growth, the fastest growth rate after extended quiescence was 1.33 mm/yr, and the longest delay before tumor growth detection was 11.1 years. Additional treatment was recommended for six (42.9%) of the patients with delayed growth. Of 68 tumors that remained in the IAC, 11 (16.2%) demonstrated delayed growth. Of 66 tumors that presented in the CPA, 2 (3.0%) demonstrated delayed growth. Initial size was larger for tumors demonstrating early growth compared with those with delayed growth. For tumors within the IAC, those with early growth had a significantly higher median growth rate than those with delayed growth (1.40 vs. 0.45 mm/yr, p < 0.001). CONCLUSIONS: Delayed growth encompassed 8.1% of growing VSs and 3.9% of all observed tumors. Patients with delayed growth exhibited slower growth rates compared with those who were diagnosed with growth early in their observation course. These findings support the need for lifelong surveillance of untreated VSs given the possibility of clinically significant delayed growth. Increasing the time interval between MRI studies after 5 years is a reasonable concession to balance practicalities of cost and convenience with risk of delayed of tumor growth.

Int J Pediatr Otorhinolaryngol ; 120: 25-29, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30753978


OBJECTIVE: To evaluate the prevalence of bilateral hearing loss in children with unilateral enlarged vestibular aqueduct (EVA) at a single institution. METHODS: A retrospective case review was performed at a tertiary care pediatric referral center involving children with radiologic findings of unilateral EVA and normal labyrinthine anatomy of the contralateral ear diagnosed via CT and/or MRI. The main outcome measure of interest is the number of patients with unilateral EVA who were diagnosed with bilateral hearing loss. RESULTS: Sixty-one pediatric patients were identified. The mean audiometric follow-up was 48.2 months (0-150). Three (4.9%) patients with unilateral EVA were noted to have bilateral hearing loss, and this rate was not significantly different (p = 1.0) from the rate reported in a comparison group of patients with contralateral hearing loss (6.0%) without an EVA. The pure-tone average (defined as the average dB HL at 500, 1000, 2000, and 4000 Hz) in the group with bilateral hearing loss was 31.3 dB HL in the better hearing ear and 79.6 dB HL in the worse hearing ear, with the difference being statistically significant (p = 0.02). In the unilateral EVA patients without contralateral hearing loss (n = 56, 91.8%), the PTA was 9.4 dB HL in the better hearing ear and 51.9 dB HL in the worse hearing ear, with the difference being statistically significant (p < 0.001). Two patients (3.3%) with unilateral EVA were found to have hearing within normal limits bilaterally. The EVA was ipsilateral to the worse hearing ear in all cases. CONCLUSION: The prevalence of bilateral hearing loss in children with unilateral EVA appears to be low. Specifically, it may be no different than the rate of contralateral hearing loss in children with unilateral hearing loss without an EVA. The present report is somewhat different than the previously described prevalence in the literature. This difference could be related to the imaging type and diagnostic criteria used, the patients included, the source of the identified patents, and the overall population of patients studied.

Audiometria de Tons Puros/métodos , Perda Auditiva Bilateral/epidemiologia , Perda Auditiva Neurossensorial/complicações , Aqueduto Vestibular/anormalidades , Adolescente , Criança , Pré-Escolar , Feminino , Audição , Perda Auditiva Bilateral/etiologia , Humanos , Lactente , Imagem por Ressonância Magnética , Masculino , Prevalência , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
Otol Neurotol ; 37(8): 1155-61, 2016 09.
Artigo em Inglês | MEDLINE | ID: mdl-27362739


OBJECTIVES: To determine the influence of posterior fossa dimensions on surgical outcomes after vestibular schwannoma management. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary care academic medical center. PATIENTS: Patients with sporadic vestibular schwannomas who underwent surgical resection via retrosigmoid or translabyrinthine techniques. INTERVENTIONS: Diagnostic. MAIN OUTCOME MEASURES: One and two-way analysis of variance (ANOVAs) of the cohort based on volumetric and clinical outcome groupings. RESULTS: A total of 95 patients were identified. A one-way ANOVA looking at the entire cohort of patients showed that outcomes like surgical time (p < 0.001) and whether a Good Outcome (House-Brackmann score of 1 or 2, no complications, and a complete resection) was achieved (p = 0.009) correlated very well with preoperative tumor volume, but not with posterior fossa volume (p = 0.412 and p = 0.345, respectively). However, in medium sized tumors, House-Brackmann group was correlated with posterior fossa volume (p = 0.032). CONCLUSIONS: The present data suggest a small effect of posterior fossa size on some clinical outcomes in medium sized vestibular schwannomas.

Neuroma Acústico/cirurgia , Crânio/anatomia & histologia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos