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Vocal and other otolaryngological manifestations of ectodermal dysplasia
Otolaryngology Head and Neck Surgery
Scientific Posters P205
Volume 115 Number 2
parathyroidectomy will be illustrated. Postoperative management including hungry bone syndrome, calcium replacement, and management of the dialysis patient during the postoperative period will also be discussed. 151
Vocal and Other Otolaryngologlcal Manifestations of Ectodermal Dysplasla GEORGE CHARPIED, PhD, and GREGORY A. GRILLONE, MD, Boston, Mass.
Objective: Anhidrotic ectodermal dysplasia (AED) is a rare disorder characterized by deficient sweating, sparse hair growth, and deficient teeth. There is also a marked decrease in mucus production in the aerodigestive tract, which can result in significant mucosal inflammation and infection. This can have devastating consequences for the patient. However, little is known about the otolaryngologic management of such patients, particularly with respect to the phonatory tract. The objective of this report is to describe vocal and other otolaryngologic management issues in a patient with AED. Methods: We report a case of a 23-year-old Pakistani man with AED and a chronic disabling dysphonia. Medical history was significant for chronic bronchitis, asthma, chronic rhinitis, and gastroesophageal reflux disease. Flexible videolaryngoscopy revealed severe diffuse erythema and desiccation of the larynx, pharynx, and nasal cavity mucosa with marked crusting of secretions. The patient was treated with aggressive humidification, systemic and topical steroids, and omeprazole for gastroesophageal reflux disease. lsotretinoin, which the patient was taking for severe acne and which was thought to be contributing to the dryness, was ultimately discontinued. Results: Within 3 months the patient's voice was subjectively improved, and he was able to resume normal vocal activities at work and school. Improvement was also confirmed by significant changes in both perceptual assessment and acoustic analytic parameters. Conclusions: This case demonstrates the profound effect AED can have on the upper aerodigestive tract, particularly for the phonatory tract, and the need for aggressive medical therapy. The otolaryngologic manifestations of AED are reviewed, and recommendations for management of these patients are outlined.
Postirradiation osteogenic carcinomas are likely to involve the axial skeleton, occur in an older patient population, and present with metastases. Local recurrence is common because of the inability to achieve complete tumor resection in greater that 50% of cases. Claviculectomy has recently been described for improving access to lesions of the lower neck, axilla, and thoracic inlet. It allows for complete visualization of the confluence of the jugular, subclavian, and innominate veins, as well as the subclavian artery. Removal of the clavicle results in minimal functional disability and little cosmetic deformity. We present a case of a radiation-induced osteogenic sarcoma arising from the seventh cervical vertebra. The tumor extended into the neck but also transgressed the thoracic inlet. Surgical resection included a modified radical neck dissection, claviculectomy, and vertebrectomy, which was immediately reconstructed with a titanium prosthesis. The seventh cervical root, along with the vertebral and thyrocervical arteries, was resected. We discuss the surgical management and the adjuvant chemotherapy and radiotherapy this patient received for tumor control. We further present the radiologic and pathologic evaluation. This surgical approach offers excellent visualization and control over the neurovascular structures of the thoracic inlet and anterior mediastinum, with minimal morbidity. 153
An Unusual Airway Foreign Body STEVEN M. GOLD, MD, and SALLY R. SHOTT, MD, Cincinnati, Ohio
Objective: Sharp-pointed foreign bodies in the airway are uncommon. Special care and technique are required to safely remove such objects from the tracheobronchial tree. This case report exhibits a safe and effective method of endoscopic retrieval of sharp foreign bodies from the airway. Methods: Presented is a case report of a 12-year-old child who aspirated a blow dart. Photodocumentation of the tracheal foreign body and radiographs are included. Results: Endoscopic retrieval of sharp, pointed bodies of the tracheobronchial tree requires appropriate instrumentation and technique. The use of optical forceps to shield the pointed tip in the rigid bronchoscope resulted in a safe, uncomplicated extraction. A discussion of this technique and a review of the literature is presented.
152 154
Clavlculectomy and Resection of a Radiation-induced Osteogenlc Sarcoma of the Cervical Vertebra ROGER J. LEVIN, MD, DANIEL E, GELB, MD, NICHOLAS J. HAMILL, MD, and FRED G. FEDOK, MD, Hershey, Pa.
Osteogenic sarcoma of the head and neck is rare, with localization to the cervical vertebrae even more uncommon. Several epidemiologic risk factors have been correlated with the development of osteosarcoma, including history of fibrous dysplasia and prior exposure to ionizing radiation.
Internal Carotid Artery Injury Wlth Associated Lower Cranial Neuropathies TIMOTHY LIAN, MD, and MICHAEL SEIDMAN, MD, Detroit, Mich.
Objective: Aneurysms of the extracranial portion of the internal carotid artery are rare, comprising less than 4% of all aneurysms. Signs and symptoms of cerebral ischemia are common, but isolated cranial neuropathies are unusual.
Scientific Posters P205
Volume 115 Number 2
parathyroidectomy will be illustrated. Postoperative management including hungry bone syndrome, calcium replacement, and management of the dialysis patient during the postoperative period will also be discussed. 151
Vocal and Other Otolaryngologlcal Manifestations of Ectodermal Dysplasla GEORGE CHARPIED, PhD, and GREGORY A. GRILLONE, MD, Boston, Mass.
Objective: Anhidrotic ectodermal dysplasia (AED) is a rare disorder characterized by deficient sweating, sparse hair growth, and deficient teeth. There is also a marked decrease in mucus production in the aerodigestive tract, which can result in significant mucosal inflammation and infection. This can have devastating consequences for the patient. However, little is known about the otolaryngologic management of such patients, particularly with respect to the phonatory tract. The objective of this report is to describe vocal and other otolaryngologic management issues in a patient with AED. Methods: We report a case of a 23-year-old Pakistani man with AED and a chronic disabling dysphonia. Medical history was significant for chronic bronchitis, asthma, chronic rhinitis, and gastroesophageal reflux disease. Flexible videolaryngoscopy revealed severe diffuse erythema and desiccation of the larynx, pharynx, and nasal cavity mucosa with marked crusting of secretions. The patient was treated with aggressive humidification, systemic and topical steroids, and omeprazole for gastroesophageal reflux disease. lsotretinoin, which the patient was taking for severe acne and which was thought to be contributing to the dryness, was ultimately discontinued. Results: Within 3 months the patient's voice was subjectively improved, and he was able to resume normal vocal activities at work and school. Improvement was also confirmed by significant changes in both perceptual assessment and acoustic analytic parameters. Conclusions: This case demonstrates the profound effect AED can have on the upper aerodigestive tract, particularly for the phonatory tract, and the need for aggressive medical therapy. The otolaryngologic manifestations of AED are reviewed, and recommendations for management of these patients are outlined.
Postirradiation osteogenic carcinomas are likely to involve the axial skeleton, occur in an older patient population, and present with metastases. Local recurrence is common because of the inability to achieve complete tumor resection in greater that 50% of cases. Claviculectomy has recently been described for improving access to lesions of the lower neck, axilla, and thoracic inlet. It allows for complete visualization of the confluence of the jugular, subclavian, and innominate veins, as well as the subclavian artery. Removal of the clavicle results in minimal functional disability and little cosmetic deformity. We present a case of a radiation-induced osteogenic sarcoma arising from the seventh cervical vertebra. The tumor extended into the neck but also transgressed the thoracic inlet. Surgical resection included a modified radical neck dissection, claviculectomy, and vertebrectomy, which was immediately reconstructed with a titanium prosthesis. The seventh cervical root, along with the vertebral and thyrocervical arteries, was resected. We discuss the surgical management and the adjuvant chemotherapy and radiotherapy this patient received for tumor control. We further present the radiologic and pathologic evaluation. This surgical approach offers excellent visualization and control over the neurovascular structures of the thoracic inlet and anterior mediastinum, with minimal morbidity. 153
An Unusual Airway Foreign Body STEVEN M. GOLD, MD, and SALLY R. SHOTT, MD, Cincinnati, Ohio
Objective: Sharp-pointed foreign bodies in the airway are uncommon. Special care and technique are required to safely remove such objects from the tracheobronchial tree. This case report exhibits a safe and effective method of endoscopic retrieval of sharp foreign bodies from the airway. Methods: Presented is a case report of a 12-year-old child who aspirated a blow dart. Photodocumentation of the tracheal foreign body and radiographs are included. Results: Endoscopic retrieval of sharp, pointed bodies of the tracheobronchial tree requires appropriate instrumentation and technique. The use of optical forceps to shield the pointed tip in the rigid bronchoscope resulted in a safe, uncomplicated extraction. A discussion of this technique and a review of the literature is presented.
152 154
Clavlculectomy and Resection of a Radiation-induced Osteogenlc Sarcoma of the Cervical Vertebra ROGER J. LEVIN, MD, DANIEL E, GELB, MD, NICHOLAS J. HAMILL, MD, and FRED G. FEDOK, MD, Hershey, Pa.
Osteogenic sarcoma of the head and neck is rare, with localization to the cervical vertebrae even more uncommon. Several epidemiologic risk factors have been correlated with the development of osteosarcoma, including history of fibrous dysplasia and prior exposure to ionizing radiation.
Internal Carotid Artery Injury Wlth Associated Lower Cranial Neuropathies TIMOTHY LIAN, MD, and MICHAEL SEIDMAN, MD, Detroit, Mich.
Objective: Aneurysms of the extracranial portion of the internal carotid artery are rare, comprising less than 4% of all aneurysms. Signs and symptoms of cerebral ischemia are common, but isolated cranial neuropathies are unusual.