Pediatric Otolaryngology

Airway management in an infant with alobar holoprosencephaly and cebocephaly associated with maternal diabetes mellitus

April 17, 2013     Rajanya S. Petersson, MD; William A. Carey, MD; Dana M. Thompson, MD
article

Abstract

We report a case of alobar holoprosencephaly (HPE) and cebocephaly associated with uncontrolled maternal type 1 (insulin-dependent) diabetes mellitus. Alobar HPE is the most severe form of HPE. Patients with cebocephaly have ocular hypotelorism and a proboscis with a single, blind-ended nostril. Shortly after our patient was born, we were consulted for airway management, as the parents' goal was to bring their child home. A tracheostomy tube was placed, and choanal atresia repair was eventually performed. The infant was never decannulated, however, and she died at the age of 9 months of acute respiratory distress syndrome secondary to an upper respiratory infection. To the best of our knowledge, this case represents the longest reported survival of an infant with alobar HPE and cebocephaly. Decisions regarding the care of these infants should be made in a collaborative, multidisciplinary fashion, with special attention paid to the primary caregivers' goals of care.

Bilateral mastoid subperiosteal abscesses in an infant

January 24, 2013     Hernan Goldsztien, MD; Kevin D. Pereira, MD
article

The finding of bilateral acute mastoiditis in an infant should prompt a search for underlying predisposing causes.

Sphenoethmoid sinusitis in a child resulting in a disastrous intracranial sequela

October 31, 2012     Aye Jane Sow, MS(ORL-HNS); Jeevanan Jahendran, MS(ORL-HNS); Charng Jeng Toh, MS(Neurosurg); Thean Yean Kew, FRCR
article

Abstract

Localized sphenoethmoid sinusitis in children is a rare occurrence. It is usually overlooked because of the misconception that the sinuses are not developed. We describe a case of localized acute sphenoid and right posterior ethmoid sinusitis that presented as right frontobasal subdural empyema and multiple deep cerebral abscesses. Morbidity from subdural empyema in children is high. Early diagnosis and treatment based on recognition that the etiology might involve the paranasal sinuses affects the overall prognosis.

Tongue base cyst in a 6-week-old boy

October 8, 2012     Joel Y. Sun, BA; Ron B. Mitchell, MD; Seckin O. Ulualp, MD
article

After excision, a histologic finding of an epithelial lining without ectopic thyroid tissue confirms the diagnosis of a lingual thyroglossal duct cyst.

Spontaneous cervicofacial subcutaneous emphysema secondary to pneumomediastinum in an otherwise asymptomatic child

October 4, 2012     Tripti Maithani, MS; Debraj Dey, MS; Chandermohan Sharma, MS
article

Abstract

A 12-year-old boy presented with subcutaneous emphysema of the face, neck, and upper chest. He had no significant history that suggested any specific cause, and initial investigations revealed no underlying pathology. Computed tomography of the chest detected pneumomediastinum. The patient was managed conservatively, and he responded. He was discharged 7 days after admission with a complete resolution of the emphysema. To the best of our knowledge, this is the first reported case of spontaneous pneumomediastinum presenting as cervicofacial swelling in a child without any other symptoms.

Juvenile xanthogranuloma of the tympanic membrane: A case report

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

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
article

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
article

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.

Bilateral congenital lacrimal fistulae: A case report

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

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.

Is routine analysis of pediatric tonsillectomy specimens worth the money?

April 30, 2012     Marc C. Thorne, MD, MPH, Assistant Professor, Associate Program Director
article

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.

Myxoid malignant fibrous histiocytoma presenting as a midline nasal mass

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

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
article

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.

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