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Myeloid sarcomas of the head and neck in pediatric patients with myeloid leukemia

September 19, 2016     David W. Cuthbertson, MD; Jyotinder Nain Punia, MD; Vicki L. Owczarzak, MD


Myeloid sarcoma is a rare extramedullary tumor composed of malignant myeloid cells that occur in the presence of myeloid leukemia. We report a case series of pediatric head and neck myeloid sarcomas representative of the epidemiology, symptomatology, laboratorial correlations, prognoses, and treatment of extramedullary leukemia. Presented are 3 cases involving patients ranging from 17 months to 11 years of age. Two patients were successfully treated with chemotherapy, and in the third patient, a large lytic lesion was treated palliatively with proton beam therapy. Knowledge and recognition of myeloid sarcomas is important as they can be locally invasive, and they may also be used as a diagnostic tool or a prognostic indicator for leukemia.

Endoscopic view of a polyp in a concha bullosa

September 19, 2016     Nurdoğan Ata, MD; Fatih Güzelkara, MD

Polyps originating within a concha bullosa are uncommon, with only 5 cases reported in the English literature.

An unusual cause of tinnitus

September 19, 2016     Po-Jun Chen, MD; Hsin-Chien Chen, MD, PhD

Live insects found in the outer ear canal should be immobilized or killed before removal.

Reverse omega technique for single-stage ear lobule reconstruction

September 19, 2016     Mofiyinfolu Sokoya, MD; Andrew A. Winkler, MD

The design of the reverse omega flaps leads to a lack of visible scars, and no major complications have been encountered.

Hailey-Hailey disease

September 19, 2016     Lester D. Thompson, MD

Careful correlation of clinical findings with histologic and immunofluorescence findings usually assists in reaching the diagnosis.

Impact of nasal septal perforations of varying sizes and locations on the warming function of the nasal cavity: A computational fluid-dynamics analysis of 5 cases

September 19, 2016     Lifeng Li, MD; Demin Han, MD, PhD; Luo Zhang, MD, PhD; Yunchuan Li, MD, PhD; Hongrui Zang, MD, PhD; Tong Wang, MD, PhD; Yingxi Liu, PhD


Patients with a nasal septal perforation often exhibit symptoms associated with disturbed airflow, which can have an adverse effect on the warming function of the nasal cavity. The impact of this effect is not fully understood. The warming function is an important factor in the maintenance of nasal physiology. We conducted a study to investigate the impact of septal perforations of various sizes and locations on the warming function during inspiration in 5 patients-3 men and 2 women, aged 25 to 47 years. Three-dimensional computed tomography and computational fluid dynamics were used to model the flux of communication and temperature, and differences among patients were compared. All 5 patients exhibited an impairment of their nasal warming function. As the size of the perforation increased, the flux of communication increased and the warming function decreased. Perforations located in an anterior position were associated with greater damage to the warming function than those in a posterior position. In patients with a large or anteriorly located perforation, airflow temperature in the nasopharynx was decreased. Our findings suggest that septal perforations not only induce airflow disturbance, but they also impair the nasal warming function. Further analysis of warming function is necessary to better explore flow mechanisms in patients with structural abnormalities.

Sensorineural hearing loss after otitis media with effusion and subacute mastoiditis after viral infections of the upper respiratory tract: A comparative study of conservative and surgical treatment

September 19, 2016     Thomas Wilhelm, MD, PhD; Tim Stelzer, MD; Rudolf Hagen, MD, PhD


Involvement of the middle ear after viral infections of the upper airways may lead to serous otitis media with effusion in the absence of bacterial infection. This can be accompanied by a concomitant shading of the mastoid air cells, which could manifest as a reduced opacity on computed tomography (CT) in the absence of a history of chronic mastoiditis or acute inflammatory signs. This can lead to a subsequent impairment of inner ear function. CT scans reveal an extended pneumatization of the temporal bones in affected patients. Inner ear hearing impairment can probably be attributed to a concomitant labyrinthine reaction-the so-called toxic inner ear lesion. If no remission occurs within 5 days after initial conservative treatment (paracentesis or hemorrheologic infusions), surgical treatment with a mastoidectomy can accelerate hearing restoration. We conducted a retrospective, nonrandomized study of short- and long-term hearing outcomes in patients with a toxic inner ear lesion who had been treated with conservative measures alone (CONS group) or with surgery (SURG group) in a tertiary care referral center. Our study group was made up of 52 consecutively presenting patients (57 ears) who had been seen over a 10-year period; there were 20 patients (21 ears) in the CONS group and 32 patients (36 ears) in the SURG group. Initially, 15 CONS patients (75%) and 18 SURG patients (56%) complained of dizziness or a balance disorder. The initial averaged sensorineural hearing loss (over 0.5, 1.0, 2.0, and 3.0 kHz) was 32.4 ± 15.6 dB in the CONS group and 35.4 ± 12.0 dB in the SURG group. At follow-up (mean: 31.7 mo), the SURG group experienced a significantly greater improvement in hearing (p = 0.025). We conclude that patients with viral otitis media and concomitant noninflammatory mastoiditis with impairment of inner ear function (sensorineural hearing loss) experience a better hearing outcome when a mastoidectomy is performed during primary treatment.

Hemorrhagic polyp with large occult sulcus vocalis in a singer

September 19, 2016     Meghan O'Brien, DO; Libby J. Smith, DO

A sulcus can decrease the functional vibratory property of the vocal fold free edge and result in dysphonia.

Instructions for Authors

September 19, 2016    

Parotid gland metastasis in Merkel cell carcinoma of the head and neck: A series of 14 cases

September 19, 2016     Kristine E. Day, MD; William R. Carroll, MD; Eben L. Rosenthal, MD


Merkel cell carcinoma (MCC) is a rare cutaneous cancer of neuroendocrine cell origin that occurs frequently on the head and neck. With a high incidence of local recurrence and regional and distant metastasis, it carries a poor prognosis. We performed a retrospective study to determine the prognostic implications of parotid gland metastasis in MCC of the head and neck. Our study population was made up of 14 patients-13 men and 1 woman, aged 62 to 87 years (mean: 75.9)-who underwent a parotidectomy for the diagnosis of MCC over a period of 10 years and 9 months. Ten patients had a primary skin lesion of the head and neck and 4 presented with a parotid mass and an unknown primary. In all, 13 of the 14 patients were found to have parotid involvement-either a direct extension of MCC into the gland or a positive intraparotid lymph node; some patients had both. All patients underwent tumor excision, and 10 underwent neck dissection. Eleven patients received adjuvant radiotherapy; none received adjuvant chemotherapy. Of the 10 patients who underwent a neck dissection, 6 were found to have a cervical lymph node metastasis on pathologic examination. Follow-up ranged from 1.3 to 39.2 months (mean: 12.4). Three patients were lost to follow-up shortly after surgery, although some information was available on 2 of them. At the final follow-up, mortality data were available on 12 patients; of these, 11 had died. The lone survivor was the patient without a parotid metastasis. Among those known to have died, survival ranged from 1.6 to 49.2 months (mean: 16.0). We conclude that parotid metastasis in patients with MCC of the head and neck is associated with a dismal survival rate that is even worse than the poor survival associated with cervical node involvement.

Primary salivary duct carcinoma arising from the Stensen duct

September 19, 2016     Kenji Noda, MD; Takashi Hirano, MD, PhD; Tomoyo Okamoto, MD; Masashi Suzuki, MD, PhD


This report describes a salivary duct carcinoma (SDC) arising from the extraglandular portion of the Stensen duct. The patient was a 56-year-old man who presented with a palpable, elastic, hard mass without tenderness in the right cheek. Computed tomography revealed a tumor of the extraglandular portion of the Stensen duct. Supraomohyoid right neck dissection and total right parotidectomy were performed, and the histologic diagnosis was SDC of the Stensen duct. Postoperatively, the patient received no additional treatment. Neither recurrence nor metastasis was observed during 4 years of follow-up examination. SDC of the Stensen duct is extremely rare. To our knowledge, there is no report that describes primary SDC arising from that location. We also believe this is the first report that describes the clinical course of primary SDC arising from a Stensen duct.

Onodi cell mucocele: Case report and review of the literature

September 19, 2016     John M. Lee, MSc, MD; Michael Au, MD


Onodi cell mucoceles are rare entities that can cause devastating ocular complications if not treated promptly. Delays in the diagnosis are possible because of the wide range of differential diagnoses of unilateral retrobulbar optic neuropathy. We describe a new case of Onodi cell mucocele in a 39-year-old woman, and we present a comprehensive review of the literature on this entity. To the best of our knowledge, no review of Onodi cell mucoceles has been previously published. Our review found that 69% of patients with an Onodi cell mucocele experienced an improvement in vision after surgical decompression. The vast majority of these patients underwent endoscopic decompression; the timing of surgical decompression did not appear to affect outcomes in terms of vision. Onodi cell mucocele requires a high degree of clinical suspicion for diagnosis and a multidisciplinary approach to management that involves primary care physicians, ophthalmologists, and otolaryngologists. Early surgical treatment via an endoscopic approach is recommended for most patients, regardless of the duration of their ophthalmologic signs and symptoms.

Do ENT outpatient computed tomography facilities perceive accreditation as valuable?

September 19, 2016     Mary Beth Farrell, MS, CNMT, NCT, FSNMMI-TS; Alice Zhao, MD; Gary Heller, MD, PhD; Gavin Setzen

Isolated epiglottic lymphatic malformation in a 2-year-old: Diagnosis and treatment

September 19, 2016     Tate Naylor, BS; Anthony Sheyn, MD; Felicity Lenes-Voit, MD; Eric Berg, MD


Airway obstruction in children has a wide differential diagnosis that includes laryngomalacia, infectious processes, paralysis, extrinsic compression, and other rare anatomic anomalies. Isolated laryngeal lymphatic malformations are rare developments that can manifest with clinically significant airway obstruction. To the best of our knowledge, there have been fewer than 20 reported cases. These laryngeal mucosal lesions are best managed with radiofrequency ablation or laser ablation. We present a case of a 2-year-old child who presented with airway obstruction, initially diagnosed with laryngomalacia, who was subsequently diagnosed and treated for an isolated epiglottic lymphatic malformation.

Chronic invasive fungal sinusitis causing a pathologic Le Fort I fracture in an immunocompetent patient

September 19, 2016     Amy L. Richter, MD; K. Kelly Gallagher, MD


We describe the case of a 77-year-old immunocompetent woman with a history of chronic rhinosinusitis who presented with a pathologic Le Fort I fracture after a forceful sneeze. Imaging revealed diffuse sinus opacification and a Le Fort type I complex fracture involving the maxilla, pterygoid plates, clivus, and right nasal bridge. The patient underwent endoscopic debridement of her sinuses, which revealed mucosal dehiscence and otherwise normal healthy bleeding tissue. Anatomic pathology identified necrotic bone with invasive fungal hyphae. Cultures demonstrated Burkholderia cepacia, diphtheroid organisms, and Enterococcus and Serratia spp. The patient was administered an intravenous antibiotic and antifungal for several months, but interval imaging found no significant improvement in bone healing although the stability of her palate had improved on clinical examination. Chronic rhinosinusitis has been found to be a complication of soft-tissue, orbital, and intracranial infections but, to the best of our knowledge, a pathologic facial fracture secondary to chronic invasive fungal and bacterial rhinosinusitis has not been previously reported in the literature.

Decision making in patients with natural myringostapediopexy: A study of the contralateral ear

September 19, 2016     Viviane Bom Schmidt, MD; Sady Selaimen da Costa, MD, PhD; Leticia Petersen Schmidt Rosito, MD; Neil Sperling, MD; Rodrigo Goncalves Dias, MD


Naturally occurring myringostapediopexy frequently results in minimal hearing loss and is asymptomatic. Management decisions in such ears, however, often hinge on an appraisal of evolution toward cholesteatoma. The study of the contralateral ear has been used by our research team to infer the progression of chronic otitis media. This cross-sectional, comparative study describes the clinical findings of the contralateral ear in a series of patients with myringostapediopexy. This study included a historical and current sample of 46 patients divided into a pediatric (≤18 years) and an adult group. Patient distribution according to sex was similar (52.2% male), and 56.5% were adults. Mean conductive hearing loss ranged from 14.1 to 21.2 dB in ears with myringostapediopexy and from 16.0 to 26.6 dB in the contralateral ears according to the frequency assessed. The contralateral ear was normal in only 19.6% of the cases of myringostapediopexy. Central tympanic membrane perforation was found in 6.5% of the cases; perforation-retraction, in 17.4%; moderate or severe retraction, in 28.3%; and cholesteatoma, in 28.3%. The prevalence of cholesteatoma in the contralateral ear in the pediatric and adult groups was not significantly different (p = 0.5; χ2 test). The presence of significant abnormalities, particularly cholesteatoma, in the contralateral ears suggests a probable unfavorable progression in cases of myringostapediopexy and may influence management decisions.

Neoplastic causes of nonacute facial paralysis: A review of 221 cases

September 19, 2016     John P. Leonetti, MD; Sam J. Marzo, MD; Douglas A. Anderson, MD; Joshua M. Sappington, MD


We conducted a retrospective review to assess the clinical presentation of patients with tumor-related nonacute complete peripheral facial weakness or an incomplete partial facial paresis and to provide an algorithm for the evaluation and management of these patients. Our study population was made up of 221 patients-131 females and 90 males, aged 14 to 79 years (mean: 49.7)-who had been referred to the Facial Nerve Disorders Clinic at our tertiary care academic medical center over a 23-year period with a documented neoplastic cause of facial paralysis. In addition to demographic data, we compiled information on clinical signs and symptoms, radiologic and pathologic findings, and surgical approaches. All patients exhibited gradual-onset facial weakness or facial twitching. Imaging identified an extratemporal tumor in 128 patients (58%), an intratemporal lesion in 55 patients (25%), and an intradural mass in 38 (17%). Almost all of the extratemporal tumors (99%) were malignant, while 91% of the intratemporal and intradural tumors were benign. A transtemporal surgical approach was used in the 93 intratemporal and intradural tumor resections, while the 128 extratemporal lesions required a parotidectomy with partial temporal bone dissection. The vast majority of patients (97%) underwent facial reanimation. We conclude that gradual-onset facial paralysis or twitching may occur as a result of a neoplastic invasion of the facial nerve along its course from the cerebellopontine angle to the parotid gland. We caution readers to beware of a diagnosis of “atypical Bell's palsy.”

A case of radiation-induced mucosal melanoma in an immunohistochemically S-100-negative patient

August 21, 2016     Michael Rodriguez, DO; Yash Patil, MD; Arun Gupta, DO, MPH


We report a case of radiation-induced mucosal melanoma in a 41-year-old woman with a history of childhood rhabdomyosarcoma of the nasal cavity that had been treated with radiotherapy. During the workup for the melanoma, the patient was found to be negative for S-100 protein on immunostaining. While many melanotic markers for the histologic confirmation of melanoma exist, they can be negative in some cases, such as ours. To the best of our knowledge, only 1 case of radiation-induced melanoma has been previously reported in the English-language literature, and in that case the patient was S-100-positive. Although our case is rare, it suggests another possible long-term adverse effect of radiotherapy. We also describe the morphologies and histology associated with diagnosing melanoma in an S-100-negative patient.

Blastomycosis of the nose: A case report

August 21, 2016     Thomas M. Jetmore, MD; Jennifer Phan, MD; Abideen O. Yekinni, MD, FACS


Blastomyces dermatitidis is a thermally dimorphic fungus endemic to the North American soil near the Mississippi, Ohio, and St. Lawrence river valleys, as well as the Great Lakes Basin. It is responsible for blastomycosis, a systemic pyogranulomatous disease. Blastomycosis of the head and neck is decidedly uncommon and often mistaken for cancer. There are only 3 previous case reports of blastomycosis presenting as an intranasal lesion. We report the unusual case of a 24-year-old man with blastomycosis presenting with a 4-week history of a progressively enlarging intranasal mass who had complete resolution of symptoms after treatment with itraconazole and surgical excision of the lesion.

Surgical management of a high jugular bulb

August 21, 2016     Daniel S. Roberts, MD, PhD; Brian Chen, MD; William Slattery, MD

Patient selection for surgical repair of a high dehiscent jugular bulb should include the presence of a codominant sigmoid sinus to avoid elevated postoperative intracranial pressure.