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Manifestations and treatment of anhydrotic ectodermal dysplasia in the head and neck
Otolaryngology Head and Neck Surgery Volume 113 Number 2
Scientific Pesters P211
multicentric cervical lesion in the fetus. In anticipation of postnatal upper airway obstruction expected in this neonate, an experienced pediatric airway management team assembled in the delivery suite a full complement of equipment necessary to secure any infant's airway. At the time of planned cesarian section, the newly delivered infant immediately experienced respiratory distress. The airway was emergently secured by open (ventilating) bronchoscopy, followed by tracheotomy without untoward sequelae. The stable airway allowed work-up and surgical removal of a cystic teratoma in a controlled fashion several days later. As prenatal ultrasound diagnosis becomes both more sophisticated and prevalent, the otolaryngologist should be called upon to play an increasing role in perinatal decision-making regarding cases such as this and in planning for airway emergencies at the time of delivery. Assembly of an experience team and essential equipment/resources is paramount when the need for specialized intervention can be reliably predicted.
tomography and magnetic resonance imaging. Imaging studies revealed a complex mass, apparently extending from the skull base, that filled the entire nasopharnx and oropharynx; however, no skull base defect was noted. The patient was taken to the operating room for resection on day 5 of life, several days having been allowed for mild bronchopulmonary dysplasia to resolve. In the operating room a 6 x 10 cm bilobed mass was found, with its base on the posterior nasopharyngea] wall. The mass was carefully dissected out of the nasopharynx. Grossly, it appeared like a duplicate tongue with cystic structures, limb buds, and hair. Choanae, skull base, and posterior nasopharyngeal wall were found to be intact postoperatively. The patient was successfully extubated on postoperative day 4. The unique features of the head and neck epignathi and of polyhydramnios as the presenting sign will be discussed.
177
Manifestations and Treatment of Anhydrotic Ectodermal Dysplasia in the Head and Neck
Cervical Chondroma With Tracheal Compression
PAULINO E. GOCO, MD, ERIC T. ADLER, MD, and KWEON I. STAMBAUGH, MD, Washington, D.C.
DAVID KANAGY, MD, and lIANA SELIGMAN, MD, Maywood and Chicago, Ill.
We describe the case of a 5-year-old girl with a slowly enlarging neck mass causing tracheal compression and airway compromise. The patient was treated for 9 months for the presumed diagnoses of reactive lymphadenopathy and asthma with nebutizers, antibiotics, and corticosteroids. She was referred to Children's Memorial Hospital when her neck mass continued to increase in size despite medical management. Chest x-rays, computed toruography scans, and gross and histologic pathology photographs are presented. The management of cervical chondroma is discussed and the literature reviewed. 178
Obstructing Nasopharyngeal Epignathi Causing Polyhydramnios DANIEL A. DEEMS, MD, PHD, CHRISTOPHER P. POJE, MD, JANE CHA1TEN, MD, and MITCHELL B. AUSTIN, MD, Philadelphia, Pa.
Teratomas are comprised of an unorganized arrangement of tissues foreign to the site of origin. An epignathus is one of the four teratoma classifications. Its hallmark is that of fetal organs and limb components. We present here the case of an infant with an obstructing nasopharyngeal epignathus. The 34-week-old female was delivered by cesarean section because of polyhydramnios to a G2P1 otherwise healthy female. At delivery the infant did not vocalize. Direct laryngoscopy revealed an obstructing nasopharyngeal and oropharyngeal mass. She was successfully intubated around the mass. Physical examination also revealed marked anterior protrusion of the palate by the mass. Upon stabilization of the n e w b o r n ' s airway, she underwent computed
179
Ectodermal dysplasia is a congenital disorder characterized by an alteration or absence of ectodermally derived tissues. The structures most commonly involved are the eccrine glands (causing hypohidrosis or anhydrosis), the hair (resulting in a scanty, fine distribution), the teeth (resulting in hypodontia with peg or conical dentition), and the nails (dystrophy). It is a very rare disease that occurs about once per every 100,000 births. Ectodermal dysplasia has classically been considered an X-linked or autosomal-recessive disorder. Only males can therefore express the complete pattern of the syndrome, and females partially. The most common manifestations are of the anhydrotic type. If the child does not succumb to hyperpyrexia because of the lack of sweat glands, then he/she will likely present to an otolaryngologist for treatment. Pediatricians typically refer patients for assistance with chronically impacted cerumen, nasal obstruction, and ozena (foul smell originating from the nasal cavity). In the literature, two cases of ozena have been the presenting symptom of ectodermal dysplasia. A case of complete expression of ectodermal dysplasia will be presented, along with clinical and endoscopic photographs, radiologic studies, and a succinct description of treatment modalities. A review of the literature, diagnosis, pathophysiology, and possible experimental therapeutic modalities will be included. 180
Pediatric Subacute Acetaminophen Overdose TIMOTHY M. DUNLEVY, MD, and MARYANN P. WALL MD, Chesapeake, Va.
A case of pediatric subacute acetaminophen toxicity after the ingestion of 2.6 gm of acetaminophen by a 6-year-old,
Scientific Pesters P211
multicentric cervical lesion in the fetus. In anticipation of postnatal upper airway obstruction expected in this neonate, an experienced pediatric airway management team assembled in the delivery suite a full complement of equipment necessary to secure any infant's airway. At the time of planned cesarian section, the newly delivered infant immediately experienced respiratory distress. The airway was emergently secured by open (ventilating) bronchoscopy, followed by tracheotomy without untoward sequelae. The stable airway allowed work-up and surgical removal of a cystic teratoma in a controlled fashion several days later. As prenatal ultrasound diagnosis becomes both more sophisticated and prevalent, the otolaryngologist should be called upon to play an increasing role in perinatal decision-making regarding cases such as this and in planning for airway emergencies at the time of delivery. Assembly of an experience team and essential equipment/resources is paramount when the need for specialized intervention can be reliably predicted.
tomography and magnetic resonance imaging. Imaging studies revealed a complex mass, apparently extending from the skull base, that filled the entire nasopharnx and oropharynx; however, no skull base defect was noted. The patient was taken to the operating room for resection on day 5 of life, several days having been allowed for mild bronchopulmonary dysplasia to resolve. In the operating room a 6 x 10 cm bilobed mass was found, with its base on the posterior nasopharyngea] wall. The mass was carefully dissected out of the nasopharynx. Grossly, it appeared like a duplicate tongue with cystic structures, limb buds, and hair. Choanae, skull base, and posterior nasopharyngeal wall were found to be intact postoperatively. The patient was successfully extubated on postoperative day 4. The unique features of the head and neck epignathi and of polyhydramnios as the presenting sign will be discussed.
177
Manifestations and Treatment of Anhydrotic Ectodermal Dysplasia in the Head and Neck
Cervical Chondroma With Tracheal Compression
PAULINO E. GOCO, MD, ERIC T. ADLER, MD, and KWEON I. STAMBAUGH, MD, Washington, D.C.
DAVID KANAGY, MD, and lIANA SELIGMAN, MD, Maywood and Chicago, Ill.
We describe the case of a 5-year-old girl with a slowly enlarging neck mass causing tracheal compression and airway compromise. The patient was treated for 9 months for the presumed diagnoses of reactive lymphadenopathy and asthma with nebutizers, antibiotics, and corticosteroids. She was referred to Children's Memorial Hospital when her neck mass continued to increase in size despite medical management. Chest x-rays, computed toruography scans, and gross and histologic pathology photographs are presented. The management of cervical chondroma is discussed and the literature reviewed. 178
Obstructing Nasopharyngeal Epignathi Causing Polyhydramnios DANIEL A. DEEMS, MD, PHD, CHRISTOPHER P. POJE, MD, JANE CHA1TEN, MD, and MITCHELL B. AUSTIN, MD, Philadelphia, Pa.
Teratomas are comprised of an unorganized arrangement of tissues foreign to the site of origin. An epignathus is one of the four teratoma classifications. Its hallmark is that of fetal organs and limb components. We present here the case of an infant with an obstructing nasopharyngeal epignathus. The 34-week-old female was delivered by cesarean section because of polyhydramnios to a G2P1 otherwise healthy female. At delivery the infant did not vocalize. Direct laryngoscopy revealed an obstructing nasopharyngeal and oropharyngeal mass. She was successfully intubated around the mass. Physical examination also revealed marked anterior protrusion of the palate by the mass. Upon stabilization of the n e w b o r n ' s airway, she underwent computed
179
Ectodermal dysplasia is a congenital disorder characterized by an alteration or absence of ectodermally derived tissues. The structures most commonly involved are the eccrine glands (causing hypohidrosis or anhydrosis), the hair (resulting in a scanty, fine distribution), the teeth (resulting in hypodontia with peg or conical dentition), and the nails (dystrophy). It is a very rare disease that occurs about once per every 100,000 births. Ectodermal dysplasia has classically been considered an X-linked or autosomal-recessive disorder. Only males can therefore express the complete pattern of the syndrome, and females partially. The most common manifestations are of the anhydrotic type. If the child does not succumb to hyperpyrexia because of the lack of sweat glands, then he/she will likely present to an otolaryngologist for treatment. Pediatricians typically refer patients for assistance with chronically impacted cerumen, nasal obstruction, and ozena (foul smell originating from the nasal cavity). In the literature, two cases of ozena have been the presenting symptom of ectodermal dysplasia. A case of complete expression of ectodermal dysplasia will be presented, along with clinical and endoscopic photographs, radiologic studies, and a succinct description of treatment modalities. A review of the literature, diagnosis, pathophysiology, and possible experimental therapeutic modalities will be included. 180
Pediatric Subacute Acetaminophen Overdose TIMOTHY M. DUNLEVY, MD, and MARYANN P. WALL MD, Chesapeake, Va.
A case of pediatric subacute acetaminophen toxicity after the ingestion of 2.6 gm of acetaminophen by a 6-year-old,