Pathology quiz case. Gingival granular cell tumor of the newborn (GGCTN) (congenital epulis).
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to Anthony J Mortelliti.
Explore the source record for details and available documents.
Hemangiomas are the most common tumor of infancy, and the vast majority occur in the head and neck region. In children, laryngeal hemangiomas typically occur below the level of the true vocal folds, in the region of the subglottis, and other sites are exceedingly rare. We present four cases of hemangiomas located in the postcricoid region of the hypopharynx. Because of the location of these lesions, children may present with obstructive symptoms such as dysphagia, intermittent aspiration, hypersalivation, or recurrent respiratory infections. Clinical diagnosis is relatively easily made with flexible laryngoscopy, as the lesions have a propensity to enlarge with crying or straining. When these patients are examined under general anesthesia in a relaxed state, however, the lesions are typically much smaller, and can even go unnoticed. Unlike other reported cases, the postcricoid hemangiomas in our patients were not causing any symptoms and were simply incidental findings. Thus, we believe that the true incidence of postcricoid hemangiomas is likely higher than reports suggest. To our knowledge, we report the longest follow-up (6 years) of a patient with a postcricoid hemangioma and are the first to describe the natural course of such a lesion.
OBJECTIVES: Management of hemoptysis in the pediatric patient requires an otolaryngologist knowledgeable and skilled in the causes and management of this potentially life-threatening condition. This paper will discuss the management of pediatric hemoptysis by the otolaryngologist through an index case of massive hemoptysis seen in a child with isolated unilateral pulmonary artery agenesis (IUPAA). STUDY DESIGN: Literature review and index case. METHODS: Through a Medline search and the experience of the author (A.J.M.), we reviewed the causes and treatment options for hemoptysis in the pediatric patient. RESULTS: Management of hemoptysis by the otolaryngologist requires prompt diagnosis and patient stabilization. This allows the patient access to multiple treatment options which may include diagnostic or therapeutic bronchoscopy, angiography with embolization, and surgical intervention such as resection or revascularization. IUPAA is an unusual, and potentially life-threatening cause of hemoptysis in the pediatric patient. CONCLUSION: Hemoptysis in the pediatric patient requires prompt and thorough evaluation and treatment. An approach for the management of pediatric hemoptysis, including massive hemoptysis, is described.
PURPOSE OF REVIEW: The understanding of velo-cardio-facial syndrome has grown markedly since the initial descriptions of this common genetic disorder nearly 30 years ago. Our knowledge of the syndrome has advanced in part from opportunities to monitor many patients into adulthood because of advances in the fields of cardiothoracic surgery and immunology. Longitudinal study has brought to light psychiatric and behavioral features of the syndrome that are often not apparent until late adolescence or the early adult years. Certain endocrine and immunologic features of the syndrome thought to be resolved in childhood are now witnessed in older patients. Variable expression and lack of disease awareness are two major factors that contribute to the delays in diagnosis in many cases. To address this, there has been a call to delineate screening parameters for patients at risk of carrying the deletion. RECENT FINDINGS: Several areas are highlighted in this review, reflecting the focus of scholarly work on velo-cardio-facial syndrome in the past year. Molecular genetics has shown smaller deletions in many families with the syndrome. The gene TBX1 has been found to be important to the phenotype. Surgical outcomes data reveal the greater challenges involved in correcting velopharyngeal insufficiency. SUMMARY: Defining the genetic basis of velo-cardio-facial syndrome will allow clinicians and basic scientists to make further inroads into understanding the variable expressivity of this syndrome. It is also important to be aware of the continued diagnostic challenges encountered by clinicians in attempts to improve the detection of patients with this syndrome.
Explore the source record for details and available documents.