Fungal necrotizing fasciitis of the head and neck in 3 patients with uncontrolled diabetes

March 18, 2014     Saravanam Prasanna Kumar, DNB; Arunachalam Ravikumar, MS; Lakshmanan Somu, MS


Necrotizing fasciitis is an uncommon, rapidly progressive soft-tissue infection that is associated with a high incidence of morbidity and mortality. It is usually caused by bacteria and rarely caused by or complicated by a fungus. We report 3 cases of necrotizing fasciitis of the head and neck in patients with uncontrolled diabetes. Fungi were isolated in all 3 cases. In 1 fatal case, the invasive zygomycete Apophysomyces elegans was isolated. Keys to the management of this condition are (1) early isolation of the causative organism by fungal smear and culture, (2) adequate control of diabetes, (3) maintenance of electrolyte balance, and (4) controlled aggressive surgical debridement at an early stage. We emphasize the importance of fungal smears and cultures in the management of this rapidly spreading infection.

Introduction Necrotizing fasciitis of the head and neck region is a rare, rapidly progressive infection of the soft tissue. It is associated with a high incidence of morbidity and mortality. These infections are characterized by rapidly progressive necrosis of the skin, subcutaneous fat, and fascia, with or without myonecrosis.

Merkel cell carcinoma

March 18, 2014     Jeffrey D. Shiffer, MD; Lester D.R. Thompson, MD

The expected 5-year survival rate for patients with Merkel cell carcinoma is more than 80% if the tumor is less than 2 cm and has not metastasized. Once a tumor has metastasized regionally, the 5-year survival rate drops to about 50%.

Early detection of nasopharyngeal carcinoma using IgA anti-EBNA1 + VCA-p18 serology assay

March 18, 2014     Achmad C. Romdhoni, MD, PhD; Nurul Wiqoyah, MS; Widodo Ario Kentjono, MD, PhD

Nasopharyngeal carcinoma (NPC) is the most common head and neck malignancy in Indonesia. Overall, it ranks fourth in males and sixth in females as the most prevalent type of cancer in that country. The data show that in the year 2011, NPC incidence was considered to be intermediate (6.2/100,000 population per year). Through histopathologic examination, about 70 to 80% of these cases were found to be type III according to the WHO classificaton. NPC carries an excellent prognosis if treated early, but most patients presented with stage III to IV disease, which negatively affected the cure rate and increased the mortality rate. Epstein-Barr virus (EBV) IgA serology has been established as an effective marker for NPC. Therefore, biologic markers, DNA, and/or antibody-based diagnosis is needed to decrease NPC cases. A screening program needs to be developed that will identify people at high risk of NPC and those who are in the early stage of the disease. In this study, 20 samples were collected from posttherapy patients. An otolaryngologic examination, histopathology of nasopharyngeal tissue, and blood testing for serologic markers were performed. IgA anti-EBNA1 + VCA-p18 enzyme-linked immunosorbent assay showed positive impact as a tool for confirming the diagnosis of NPC, but it still has to be combined with other specific diagnostic tools for post-therapy monitoring and for determining prognosis.

Introduction Nasopharyngeal carcinoma (NPC) is rare in most parts of the world, but it is relatively common in Southeast Asia, including Indonesia. Chinese populations, living either in mainland China or elsewhere, have the highest incidence of this tumor worldwide: 10 cases/100,000 population.1

Necrotizing fasciitis of the ear: An unusual case

March 18, 2014     Jessica L. Gullung, MD; Jacob E. Smith, MD; April D. Hendryx, DO; Krishna G. Patel, MD, PhD


We describe a rare case of necrotizing fasciitis of the ear in an otherwise generally healthy 39-year-old man. The ear is rarely involved in this often-fatal disease. The diagnosis was suspected on the basis of clinical examination and confirmed by computed tomography and pathology of a biopsy specimen, which revealed subcutaneous gas formation and dermal infiltration of inflammatory cells, respectively. The patient was promptly treated with surgical debridement, intravenous antibiotics, and hyperbaric oxygen. He recovered well and subsequently underwent otoplastic reconstruction with very satisfactory results. By documenting this case, we aim to heighten awareness of necrotizing fasciitis of the head and neck in an effort to improve survival rates.

Introduction Necrotizing fasciitis is a rare, potentially life-threatening infection. A high index of suspicion with immediate intervention is essential for maximizing patient survival.

Pediatric cervical sympathetic chain schwannoma with Horner syndrome: A rare case presentation

March 18, 2014     Sanjeev Bhagat, MS; Saurabh Varshney, MS; Sampan S. Bist, MS; and Nitin Gupta, MS


Schwannomas are rare, benign, slowly growing neurogenic tumors that originate in peripheral, spinal, or cranial nerves other than the optic and olfactory nerves. In the head and neck, these tumors usually arise from the vagus nerve and the cervical sympathetic chain in the parapharyngeal space. Cervical sympathetic chain schwannomas represent a rare subgroup of schwannomas; fewer than 60 cases have been reported in the literature. These tumors are rarely seen in children and adolescents. Because patients typically present with an asymptomatic neck mass and vague complaints, clinical suspicion is important in making the diagnosis. Neural deficits at presentation are uncommon. The presence of features of Horner syndrome before excision is very rare, having been previously reported in only 10 cases of cervical sympathetic chain schwannoma. We report an extremely rare case of a cervical sympathetic chain schwannoma in a child who presented with Horner syndrome. The lesion was successfully excised.

Introduction Schwannomas are uncommon, benign, slowly growing nerve sheath tumors that originate in neural crest cells.1 They can arise from any peripheral, spinal, or cranial nerve except the optic and olfactory nerves.1-3 Schwannomas that arise from the cervical sympathetic chain are quite exceptional, as fewer than 60 cases have been reported...

Implants in otology

March 18, 2014     Barry E. Hirsch, MD

Progress in technology facilitates progress in patient care.

[Editor's note: This Guest Editorial has been adapted with permission from its publication in the Fall 2012 issue of Soundings, the Pennsylvania Academy of Otolaryngology-Head and Neck Surgery's newsletter.]

Two cases of granular cell tumors of the head and neck at different sites

March 18, 2014     Mustafa Paksoy, MD; Mehmet Eken, MD; Emin Ayduran, MD; Gokhan Altin, MD


Granular cell tumor (GCT) is an uncommon, primarily benign lesion. We describe 2 cases of GCT. Patient 1 was a 38-year-old man who presented with a reddish, vegetative lesion that involved the posterior third of the left true vocal fold. Patient 2 was an 18-year-old girl who presented with a firm, slightly extruded submucosal mass in the posterior lateral third of the tongue. Both lesions were identified as GCTs on biopsy. Both tumors were removed surgically, and both patients exhibited no signs of recurrence during follow-up. It is important that otolaryngologists be familiar with GCT and its management. Although the length of follow-up has varied substantially in the literature, we recommend long-term surveillance, including regular clinical examinations and periodic imaging with contrast-enhanced magnetic resonance imaging, at least until additional studies have clarified the natural history and recurrence patterns of GCT with greater certainty.

Introduction Granular cell tumor (GCT) is an uncommon, primarily benign lesion that was first described in 1926 by Abrikossoff, who called it myoblastoma.1 This tumor is more common among blacks than members of any other race (63% of cases), and it is twice as common in women as in men.2 GCT occurs in all age groups, but it is most prevalent...

Facial nerve palsy associated with a cystic lesion of the temporal bone

March 18, 2014     Na Hyun Kim, MD; Seung-Ho Shin, MD


Facial nerve palsy results in the loss of facial expression and is most commonly caused by a benign, self-limiting inflammatory condition known as Bell palsy. However, there are other conditions that may cause facial paralysis, such as neoplastic conditions of the facial nerve, traumatic nerve injury, and temporal bone leions. We present a case of facial nerve palsy concurrent with a benign cystic lesion of the temporal bone, adjacent to the tympanic segment of the facial nerve. The patient's symptoms subsided after facial nerve decompression via a transmastoid approach.

Introduction Facial nerve palsy results in the loss of facial expression and is most commonly caused by a benign, self-limiting inflammatory condition known as Bell palsy.1 However, other conditions may result in injury of the seventh cranial nerve, such as neoplastic conditions of the facial nerve, traumatic nerve injury, and temporal bone...

Multiple plasma cell granulomas of the larynx in a young man

March 18, 2014     Courtney Shires, MD; Sandeep Samant, MD, FACS


Plasma cell granuloma of the larynx is a rare benign lesion of unknown etiology, with only 21 cases reported previously. We report an additional case of plasma cell granuloma in which a 26-year-old man experienced a 1.5 x 3.4-cm, completely obstructing subglottic lesion. Because of the patient's young age, history of hemoptysis, bleeding from his tracheostomy, and the rarity of plasma cell granulomas, the patient was assumed to have hemangioma until proven otherwise. He presented with a partially obstructing glottic lesion 4 months later. Both the subglottic and glottic lesions were excised endoscopically. Multiple modalities have been used to treat plasma cell granulomas, including radiation, endoscopic CO2 laser ablation, high-dose prednisone, and open excision. In our case, steroids were given in the interim between the 2 excisions. This is the first report of a patient with two laryngeal plasma cell granulomas and the 22nd reported case of laryngeal plasma cell granuloma.

Introduction Inflammatory pseudotumors are a group of benign pseudoneoplastic lesions that possess a variety of histopathologic features, ranging from plasma cell proliferation to abundance of myofibroblasts.1 Pseudotumors containing predominantly mature plasma cells are known as plasma cell granulomas.2

Aggressive inflammatory pseudotumor of the maxillary sinus and orbit

March 18, 2014     Sheldon Chong, MBBS, MS(ORL-HNS); Carren S.L. Teh, MBBS, MS(ORL-HNS); Shashinder Singh, MBBS, FRACS; Mun Kein Seong, MBBS, MPath; Subrayan Viswaraja, MBBS, FRCS(Edin), FRCOphth(UK)


Inflammatory pseudotumor (IPT) is a rare, locally aggressive, benign neoplasm of unknown etiology. It is uncommon in the head and neck region, particularly in the paranasal sinuses. We present an unusual case of IPT of the maxillary sinus and orbit in a 27-year-old woman who presented with cheek swelling, right orbital swelling, double vision, and associated fever and trismus. Computed tomography identified a mass with radiologic features suggestive of a malignancy of the maxillary sinus and orbit; the mass extended into the infratemporal fossa, parapharyngeal space, anterior antral wall, and surrounding soft tissue. A diagnosis of IPT was established on the basis of histologic and immunohistochemical analysis, which identified a proliferation of bland spindle cells and a mixed inflammatory cell infiltrate. Despite its aggressive appearance, IPT is associated with a good prognosis. Our patient was treated successfully with a combination of surgery, steroid therapy, and methotrexate. Chemotherapeutic agents are generally reserved for recalcitrant cases.

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