Pediatric Otolaryngology

Juvenile xanthogranuloma of the tympanic membrane: A case report

September 7, 2012     Michael W. Chu, MD; Alice Werner, MD; Stephanie A. Moody-Antonio, MD
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Abstract

Juvenile xanthogranuloma (JXG) is a benign, non-Langerhans cell histiocytic lesion that generally affects infants and children. These lesions characteristically appear as a solitary, yellow, cutaneous nodule of the head, neck, or trunk. Subcutaneous and extracutaneous forms can involve the gastrointestinal tract, kidney, lung, gonads, pericardium, central nervous system, temporal bone, larynx, and eye. We describe the clinical presentation, imaging, histochemical findings, and management of a solitary JXG of the tympanic membrane in a 17-month-old girl. The patient underwent surgical resection and was without disease several months following surgery and reconstruction of the defect. To the best of our knowledge, this is the first reported case of a JXG of the tympanic membrane.

Allergic fungal rhinosinusitis in children

July 5, 2012     Ross Shockley, MD; Soham Roy, MD
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Allergic fungal rhinosinusitis most often occurs in adolescents or young adults who exhibit nasal obstruction, congestion, purulent or clear rhinorrhea, anosmia, and headache--classic manifestations of chronic sinusitis.

Mesenchymal hamartomas of the pediatric head and neck

July 5, 2012     Matthew L. Carlson, MD; Amy M. Saleh, MD; Keith J. Kaplan, MD; Shelagh A. Cofer, MD
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Abstract

We describe the fifth published report of a mesenchymal hamartoma presenting as a cheek mass. A 5-month-old infant was brought to our institution for evaluation of an enlarging left-sided congenital cheek mass. Over time, the lesion had begun to cause significant facial asymmetry and oral incompetence. Radiologic imaging revealed an approximate 2.5 x  3.5-cm, noncystic lesion located in the left buccal space, separate from the mandible and surrounding the salivary glands. Magnetic resonance imaging (MRI) sequences demonstrated an isointense, T1-weighted lesion with avid gadolinium uptake, and increased intensity of T2-weighted sequences. The patient subsequently underwent biopsy and subtotal resection through a left gingivobuccal incisionwith the goal of improving lip contour and facial symmetry.Histologic examination revealed an admixed arrangement of mature smooth muscle, vascular, adipose, and neural tissue elements within a slightly myxoid stroma, consistent with a mixed mesenchymal hamartoma. An 8-month postoperative MRI demonstrated near-total removal without evidence of regrowth. While rare, hamartomas should be included in the differential diagnosis of a slow-growing pediatric head and neck mass. Gross total resection may provide cure; however, given this lesion’s benign nature, less-than-complete resection should be considered when lesions infiltrate opposing critical structures. Thorough clinical and histologic evaluation is critical to avoid overly aggressive treatment and unnecessary morbidity.

Is routine analysis of pediatric tonsillectomy specimens worth the money?

April 30, 2012     Marc C. Thorne, MD, MPH, Assistant Professor, Associate Program Director
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Although gross pathologic analysis may seem like a good alternative to examining every specimen microscopically, from the point of view of cost-effectiveness, it is the worst option and is quite unlikely to identify significant pathology.

Bilateral congenital lacrimal fistulae: A case report

April 30, 2012     Lei Zhuang, MD; Christin L. Sylvester, DO; Jeffrey P. Simons, MD
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Abstract

A congenital lacrimal fistula is a rare developmental anomaly, usually unilateral. While it is often asymptomatic, some patients present with epiphora or discharge. We report the case of a 4-year-old boy with bilateral lacrimal fistulae. No other systemic, nasal, or ocular anomalies were found. In the absence of significant symptoms, we decided on a course of observation. In this article, we discuss the embryologic basis of congenital lacrimal fistulae, as well as the typical presentation and possible treatment modalities. The presence of a lacrimal fistula is an indication to search for a variety of underlying systemic and ocular anomalies.

Myxoid malignant fibrous histiocytoma presenting as a midline nasal mass

March 31, 2012     Jaimie DeRosa, MD and Jeffrey R. Smit, MD
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Abstract

Myxoid malignant fibrous histiocytoma is a rare type of pediatric non-rhabdomyosarcoma soft-tissue sarcoma. The case of a 5-year-old girl is presented, highlighting the potential for multiple pitfalls and aberrant differential diagnoses that need to be identified for successful treatment of pediatric myxofibrosarcomas. An awareness of these tumors and a call for standardized postsurgical treatment protocols is necessary in order to successfully treat children with this disease.

Juvenile aggressive cemento-ossifying fibroma of the sinonasal tract: Case report

November 22, 2011     Sampan Singh Bist, MBBS, MS, Saurabh Varshney, MS, Rakesh Kumar, MS, Sanjeev Bhagat, MS, and Nitin Gupta, MS
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Abstract

Cemento-ossifying fibroma (COF) is a rare nonodontogenic tumor of the periodontal membrane that arises from the mesodermal germ layer. Clinically, these lesions are usually asymptomatic, slowly growing, and well circumscribed. However, they have demonstrated an aggressive course of development in a very small number of cases, particularly in younger patients. Surgical management via wide local excision is necessary because COF is notorious for recurrence, particularly lesions in the paranasal sinuses. We describe a case of juvenile aggressive COF of the sinonasal tract in a 12-year-old boy. This tumor presented with various overlapping clinical, radiologic, and pathologic features, which led to initial confusion and a diagnostic dilemma. We also discuss the radiologic interpretation of COF, which can be helpful in making the diagnosis and in selecting the appropriate surgical approach in a growing child.

Osteosarcoma of the skull base in a 15-year-old boy

October 26, 2011     Gretchen M. Oakley, BA, Dary J. Costa, MD, Ron B. Mitchell, MD, and Cirilo Sotelo, MD
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Myofibroma of the zygomatic bone in an older child: A case report

July 13, 2011     Engin Acıoğlu, MD, Gül Özbilen Acar, MD, Yalçın Alimoğlu, MD, Harun Cansız, MD, and Sergülen Dervişoğlu, MD
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Abstract

Myofibroma/myofibromatosis is a rare mesenchymal disorder that is part of a heterogeneous group of approximately 20 disorders that are classified primarily according to the proliferation of benign fibrous elements. These lesions can arise during a wide range of ages, with many occurring in the first decade of life, and they are slightly more common in males than females. The etiology of this disease is not well understood. Clinically, patients with myofibroma/myofibromatosis present with various signs, ranging from superficial, cutaneous, purplish macules to freely movable subcutaneous masses to deep-seated fixed lesions. The definitive diagnosis is made on histopathologic grounds. The destructive clinical behavior of myofibroma/myofibromatosis in the setting of insufficient pre- or perioperative diagnostic evaluations (e.g., a failure to perform fine-needle aspiration or frozen-section biopsy) may guide the clinician toward a radical surgical procedure rather than a simple excision.

Ballpoint pen ingestion in a 2-year-old child

July 13, 2011     Anaïs Rameau, MD, CM, MSc, Sumeet M. Anand, MD, and Lily H.P. Nguyen, MD, CM, MSc, FRCS(C)
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Abstract

A 2-year-old girl ingested a ballpoint pen, which was found on chest x-ray to have lodged in the lower esophagus and stomach. The pen, which measured nearly 15 cm in length, was removed via rigid esophagoscopy without complication. To the best of our knowledge, this is the longest nonflexible foreign body ingested by a young child ever reported in the English-language literature. We describe the presentation of this case and the current guidelines for safety as enumerated in the Small Parts Regulations established by the U.S. Consumer Product Safety Commission

Lateral sinus thrombosis in children: A review

June 13, 2011     Borlingegowda Viswanatha, MS, DLO
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Abstract

A retrospective study was undertaken to review the clinical presentation, evaluation, management, and outcome of otogenic lateral sinus thrombosis (LST) in children. All pediatric patients with LST seen in our department between 1999 and 2007 were included; there were 9 cases involving 6 boys and 3 girls whose ages ranged from 8 to 12 years. They had all been treated with antibiotics elsewhere prior to admission, and the duration of symptoms before admission ranged from 5 to 18 days. The most common presenting symptoms were ear discharge, headache, otalgia, and fever. Radiologic evaluation included computed tomography and magnetic resonance imaging. All patients underwent radical mastoidectomy with incision of the lateral sinus and removal of its content. There were no deaths. Pseudomonas and Proteus spp were the most commonly identified organisms. Otogenic LST still poses a serious threat that warrants immediate attention and care. It is often associated with other intracranial complications, such as cerebellar abscess. Computed tomography and magnetic resonance imaging play an important role in the management of this disease. Early and aggressive surgical intervention of this otogenic complication can potentially minimize mortality.

Nasal septal deviation in the pediatric and adult populations

March 1, 2011     Shari D. Reitzen, MD, Wayne Chung, MD, and Anil R. Shah, MD
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Abstract

A significant proportion of the population has nasal septal deviation of varying degrees. Recent reports of such deviation occurring at younger ages suggest a congenital etiology. To the best of our knowledge, no previous clinical studies have compared the septal deviation of adult and pediatric populations with a uniform measure that focuses on the degree of deviation. We retrospectively analyzed computed tomography (CT) and magnetic resonance imaging (MRI) scans obtained from 81 patients who had undergone head and neck imaging for a variety of reasons. These subjects were divided into four age groups: younger than 4 months; 4 months to less than 5 years; 5 to 15 years; and more than 15 years. We used a measure of tortuosity to examine and compare nasal septal deviation among the different age groups. The tortuosity of the septum was measured at four precise points along the length of the septum on thin-section sinus CT and MRI. Tortuosity was defined as the ratio of the “actual” length of the septum to the “ideal” length of the septum, which was defined as the length of a straight line drawn from the superior to the inferior aspect of the septum. We found that subjects younger than 5 years of age exhibited significantly less tortuosity (p ≤ 0.017459) than did the older children and the adults. Therefore, we conclude that nasal septal deviation occurs at a higher frequency in older children and in adults when calculations of tortuosity are used as a measure. Our data may suggest that a noncongenital etiology is responsible for nasal septal deviation. However, given that the growth of the septum continues throughout childhood, our results do not preclude the possibility of a genetic predisposition to the later development of a deviated nasal septum.

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