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Respiratory distress precipitated by a “Contralateral” mediastinal goiter
P208
Otolaryngology Head and Neck Surgery August 1996
Scientific Posters
linkage analysis, the inherited gene for NF-2 has been localized to the long arm of chromosome 22. It is the loss of tumor-suppressive genetic material that is thought to predispose to the formation of multiple tumors in the central and peripheral nervous systems. Individuals with NF-2 are at risk not o n l y for a c o u s t i c n e u r o m a s but also for meningiomas, spinal ependymomas, astrocytomas, and facial nerve neuromas. NF-2 has an incidence of 3 cases per 100,000 population and affects nearly 1 in 40,000 people in the United States. Identical (monozygotic) twins occur approximately once in every 300 births among white North Americans. Interestingly, since the initial description of NF-2 by Wishart in 1822, no twins with bilateral acoustic tumors have been reported. With discordant phenotypic presentation and the necessary disparate m a n a g e m e n t undertaken, we present the first reported case of identical twins with NF-2.
162
Respiratory Distress Precipitated by a "Contralateral" Medlastlnal Goiter ANDREW J. LERRICK, MD, and GEORGE A. SISSON, MD, Chicago, III.
Patients with substernal goiters may present with respiratory compromise due to tracheal compression at the level of the thoracic inlet. Although most patients with mediastinal goiters are asymptomatic, a few permit the thyroid mass to progress to the point of airway obstruction despite having the cosmetic deformity of an enlarging neck mass. We present two cases of a "contralateral" substernal goiter in which the substernal component was on the opposite side of the trachea from the cervical mass. Both patients reported having a right cervical mass for more than 20 years. Each was referred with mild stridor secondary to tracheal compression. Palpation of the trachea was obscured by the goiter, although tracheal deviation to the left was anticipated. The patients were clinically stable to permit a preoperative CT scan. Despite having a predominantly right-sided suprasternal mass, in both instances the trachea was unexpectedly deviated to the right by a massive left-sided mediastinal goiter. One patient underwent total thyroidectomy after successful digital mobilization of the mediastinal component. The other required resection of the manubrium and clavicular head for mediastinal exposure to the level of the aortic window. Only then was safe, blunt mobilization of the substernal goiter achieved. The entity of a "contralateral" goiter should be considered in patients presenting with respiratory distress, especially with respect to tracheal deviation in the cervical region in the event that an emergency tracheotomy need be performed. In addition to difficulty locating the trachea under emergency conditions, inadvertent injury to the carotid artery could occur.
163
First Branchial Cleft Anomaly Associated With Congenital Cholesteatoma in the Middle Ear KEVIN EARL McLAUGHLIN and IAN N, JACOBS, Decatur and Atlanta, Ga.
First branchial cleft anomalies are rare. They account for less than 1% of all branchial cleft anomalies. Work has classified these branchial cleft anomalies as type I and type II. Type I anomalies are usually cystic lesions found posterior to the pinna and do not communicate with the external auditory canal. Type II lesions are more common, usually occurring as a sinus or fistula coursing from the anterior neck to the bony-cartilaginous junction of the external auditory canal. The middle ear and tympanic membrane are usually normal with both anomalies; however, there have been six reported cases of tympanic membrane and middle ear involvement. We report a seventh case of a 26-month-old man who presented with chronic recurrent left infraparotid infections, chronic left otitis media, conductive hearing loss, and a white mass at the anterior inferior portion of the tympanic membrane. CT scan revealed a soft tissue mass lateral to the parotid and coursing into the h y p o t y m p a n u m and mesotympanum. The patient underwent excision of the first branchial cleft sinus with superficial parotidectomy and facial nerve exploration. The tract coursed lateral to the inferior branch and medial to the superior branch of the facial nerve. The anomalous course has been noted in only two other reported cases. The tract traveled parallel to the external auditory canal and medial to the glenoid fossa and was continuous with the hypotympanic space. Left middle ear exploration revealed frank cholesteatoma that involved the hypotympanum and was medial to the glenoid fossa toward the lateral carotid artery. This is the first reported case of a first branchial cleft anomaly associated with congenital cholesteatoma. The patient underwent a tympanomastoidectomy and meatoplasty. He remains free of disease with good facial movement and left-sided hearing 2 months after surgery. The embryology, classification, assessment, workup, surgical approaches, and pitfalls associated with this unusual first branchial cleft anomaly are discussed in addition to a review of the world literature. 164
Rheumatoid Pannus of the Cervical Spine: An Unusual Cause of Dysphagla WILLIAM C. KINNEY, MD, RAYMOND J. SCHEETZ, MD, and MARSHALL STROME, MD, Cleveland, Ohio
Rheumatoid arthritis (RA) commonly involves the cervical spine; however, otolaryngologic manifestations of RA r e m a i n u n c o m m o n . S y m p t o m s of R A arising from otolaryngologic involvement are predominantly related to synovial inflammation involving the temporal mandibular
Otolaryngology Head and Neck Surgery August 1996
Scientific Posters
linkage analysis, the inherited gene for NF-2 has been localized to the long arm of chromosome 22. It is the loss of tumor-suppressive genetic material that is thought to predispose to the formation of multiple tumors in the central and peripheral nervous systems. Individuals with NF-2 are at risk not o n l y for a c o u s t i c n e u r o m a s but also for meningiomas, spinal ependymomas, astrocytomas, and facial nerve neuromas. NF-2 has an incidence of 3 cases per 100,000 population and affects nearly 1 in 40,000 people in the United States. Identical (monozygotic) twins occur approximately once in every 300 births among white North Americans. Interestingly, since the initial description of NF-2 by Wishart in 1822, no twins with bilateral acoustic tumors have been reported. With discordant phenotypic presentation and the necessary disparate m a n a g e m e n t undertaken, we present the first reported case of identical twins with NF-2.
162
Respiratory Distress Precipitated by a "Contralateral" Medlastlnal Goiter ANDREW J. LERRICK, MD, and GEORGE A. SISSON, MD, Chicago, III.
Patients with substernal goiters may present with respiratory compromise due to tracheal compression at the level of the thoracic inlet. Although most patients with mediastinal goiters are asymptomatic, a few permit the thyroid mass to progress to the point of airway obstruction despite having the cosmetic deformity of an enlarging neck mass. We present two cases of a "contralateral" substernal goiter in which the substernal component was on the opposite side of the trachea from the cervical mass. Both patients reported having a right cervical mass for more than 20 years. Each was referred with mild stridor secondary to tracheal compression. Palpation of the trachea was obscured by the goiter, although tracheal deviation to the left was anticipated. The patients were clinically stable to permit a preoperative CT scan. Despite having a predominantly right-sided suprasternal mass, in both instances the trachea was unexpectedly deviated to the right by a massive left-sided mediastinal goiter. One patient underwent total thyroidectomy after successful digital mobilization of the mediastinal component. The other required resection of the manubrium and clavicular head for mediastinal exposure to the level of the aortic window. Only then was safe, blunt mobilization of the substernal goiter achieved. The entity of a "contralateral" goiter should be considered in patients presenting with respiratory distress, especially with respect to tracheal deviation in the cervical region in the event that an emergency tracheotomy need be performed. In addition to difficulty locating the trachea under emergency conditions, inadvertent injury to the carotid artery could occur.
163
First Branchial Cleft Anomaly Associated With Congenital Cholesteatoma in the Middle Ear KEVIN EARL McLAUGHLIN and IAN N, JACOBS, Decatur and Atlanta, Ga.
First branchial cleft anomalies are rare. They account for less than 1% of all branchial cleft anomalies. Work has classified these branchial cleft anomalies as type I and type II. Type I anomalies are usually cystic lesions found posterior to the pinna and do not communicate with the external auditory canal. Type II lesions are more common, usually occurring as a sinus or fistula coursing from the anterior neck to the bony-cartilaginous junction of the external auditory canal. The middle ear and tympanic membrane are usually normal with both anomalies; however, there have been six reported cases of tympanic membrane and middle ear involvement. We report a seventh case of a 26-month-old man who presented with chronic recurrent left infraparotid infections, chronic left otitis media, conductive hearing loss, and a white mass at the anterior inferior portion of the tympanic membrane. CT scan revealed a soft tissue mass lateral to the parotid and coursing into the h y p o t y m p a n u m and mesotympanum. The patient underwent excision of the first branchial cleft sinus with superficial parotidectomy and facial nerve exploration. The tract coursed lateral to the inferior branch and medial to the superior branch of the facial nerve. The anomalous course has been noted in only two other reported cases. The tract traveled parallel to the external auditory canal and medial to the glenoid fossa and was continuous with the hypotympanic space. Left middle ear exploration revealed frank cholesteatoma that involved the hypotympanum and was medial to the glenoid fossa toward the lateral carotid artery. This is the first reported case of a first branchial cleft anomaly associated with congenital cholesteatoma. The patient underwent a tympanomastoidectomy and meatoplasty. He remains free of disease with good facial movement and left-sided hearing 2 months after surgery. The embryology, classification, assessment, workup, surgical approaches, and pitfalls associated with this unusual first branchial cleft anomaly are discussed in addition to a review of the world literature. 164
Rheumatoid Pannus of the Cervical Spine: An Unusual Cause of Dysphagla WILLIAM C. KINNEY, MD, RAYMOND J. SCHEETZ, MD, and MARSHALL STROME, MD, Cleveland, Ohio
Rheumatoid arthritis (RA) commonly involves the cervical spine; however, otolaryngologic manifestations of RA r e m a i n u n c o m m o n . S y m p t o m s of R A arising from otolaryngologic involvement are predominantly related to synovial inflammation involving the temporal mandibular