Antrochoanal polyps in children.

Although relatively rare, antrochoanal polyps represent one of the most common types of polyp diagnosed in children without cystic fibrosis. In an attempt to better define this entity and discuss treatment options, the histories and operative reports of all 25 children (aged 17 years and younger) diagnosed with an antrochoanal polyp between 1970 and 1997 at our institution were reviewed. All 25 children complained of nasal obstruction on presentation; other presenting symptoms included rhinorrhea (48%), snoring (36%), and mouth breathing (32%). All 25 patients were noted to have a mass in the nose on examination, and 16 (64%) also had a mass noted in the nasopharynx. All but 1 patient underwent surgical removal of the polyp: intranasal avulsion only, 2 patients; Caldwell-Luc procedures, 10 patients; intranasal procedures, 8 patients; and endoscopic procedures, 4 patients. Mean time to first recurrence was 44.5 months. Seven patients (29%) who underwent excision at our institution experienced recurrence, 3 after endoscopic procedures and 4 after intranasal procedures (with or without Caldwell-Luc; 1 of these patients had a second recurrence). Complications were unusual and included bleeding after pack removal (8.3%) and facial paresthesias (10%). Follow-up ranged from 2 days to almost 27 years and was aided by telephone interviews. We conclude that surgical treatment of these lesions is safe and effective. Endoscopic removal may result in a higher recurrence rate.

Pediatric autoimmune neuropsychiatric disorders and streptococcal infections: role of otolaryngologist.

OBJECTIVE: To increase awareness and understanding of the putative role of streptococcal infection in the development of neuropsychiatric disorders in children and to discuss therapeutic options in this group of patients. METHODS: Case illustration and literature review. RESULTS: Two siblings, one with obsessive-compulsive disorder (OCD) and one with a tic disorder, had tonsillectomy for recurrent streptococcal pharyngitis. At the latest follow-up visit (11 mo postoperatively), both patients exhibited significant improvement in their psychiatric illnesses. We discuss these cases as well as the diagnosis, pathophysiology, and treatment of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS). CONCLUSION: PANDAS is an active area of research investigating the relationship between streptococcal infections and the development of obsessive-compulsive disorder or tic disorders (or both) in children. The etiopathogenesis of PANDAS is thought to reflect autoimmune mechanisms and involvement of the basal ganglia of susceptible hosts. Because otolaryngologists evaluate a large portion of pediatric patients with recurrent streptococcal pharyngitis, it is important to be aware of this association and to manage these patients appropriately.

Pseudotumor of infancy and congenital muscular torticollis: 170 cases.

OBJECTIVES: To review pseudotumor of infancy (POI) and congenital muscular torticollis (CMT) and to suggest an algorithm for treatment. STUDY DESIGN: Retrospective review of cases from 1962 to 1998 at a tertiary care center. METHODS: Patients included in this study were 81 boys and 89 girls who had a diagnosis of POI (n = 38) or CMT (n = 132) before 24 months of age. RESULTS: For all patients, the mean age at diagnosis was 4 months; 54.1% had the left side of the neck affected, over 90% had a head tilt, and 2.4% had feeding difficulty as a result of the disorder. Plagiocephaly was present in 39.5% of patients with POI and 63.6% of patients with CMT; a neck mass, in 63.2% and 18.2%, respectively; and facial asymmetry, in 7.9% and 15.9%, respectively. All patients had a complete physical examination; 54.1% had plain cervical radiography, 4.1%, computed tomography, and 2.9%, ultrasonography. Passive range of motion was the initial treatment recommended for 65.3% of patients. Conservative treatment failed for 16 patients; subsequently, they had surgical treatment. Follow-up data were available for 159 patients; 85.5% experienced total resolution and 14.5% experienced subtotal resolution or long-term abnormality. CONCLUSIONS: Children diagnosed with POI or CMT should be treated and observed for at least 12 months or until symptoms resolve. If symptoms persist 1 year after diagnosis despite conservative therapy, surgical treatment should be considered. The majority of children with POI or CMT experience total resolution of symptoms.

Pediatric transseptal transsphenoidal pituitary surgery.

The use of the transseptal transsphenoidal approach to the pituitary gland has gained acceptance as a safe, relatively atraumatic means of removing pituitary tumors. The outcome in adult patients has been successful; however, the outcome in pediatric patients whose noses may still be developing is not well described. We reviewed the outcomes in 41 patients younger than age 18 years who underwent transseptal transsphenoidal pituitary surgery at our institution between 1986 and 1997 (20 boys and 21 girls; age, 3 to 17 years; mean age, 13.4 years). The most common diagnosis was prolactin-secreting adenoma (14 patients), followed by craniopharyngioma (7 patients). Presenting symptoms included headache (20 patients), galactorrhea (13 patients), and menstrual irregularities (11 patients). The most common early complication was transient diabetes insipidus (20 patients). No patient experienced serious bleeding at pack removal and no patient developed a cerebrospinal fluid leak postoperatively. Follow-up ranged from 3 months to 12.7 years, with 7 patients experiencing recurrent or residual disease between 6 days and 2 years after their original transseptal procedure. The most common long-term surgical complication was nasal obstruction in 5 patients, 3 of whom also complained of seasonal allergies. Four patients complained of recurrent sinus infections, and 4 patients noted an external nasal deformity as a result of the surgery.

Pediatric lymphangiomas of the head and neck.

To better define the cause, presentation, diagnosis, and staging of lymphangiomas, we reviewed all cases of lymphangiomas of the head and neck region in children seen at our institution between 1986 and 1996. Sixty-seven children were identified (31 male and 36 female). Age at presentation ranged from birth to 18 years. All but 8 patients presented with an obvious mass, and 5 required tracheotomy because of airway obstruction. The most common location was the submandibular region (37%), followed by the parotid gland (31%). Treatment ranged from observation to extensive and multiple resections. Thirty-one patients underwent only 1 resection, and 2 patients received interferon as part of their treatment. Lesions involving the lip, hypopharynx and/or larynx, the tongue, and the floor of the mouth had high rates of recurrent or persistent disease. We review our experience with these difficult lesions and propose a staging system based on functional deficit, cosmetic changes, sites of involvement, and age at diagnosis.

Pediatric parotid masses.

OBJECTIVE: To evaluate the incidence, types, and treatment outcomes of pediatric parotid lesions. DESIGN: Retrospective case review, histological tissue review, and literature review. SETTING: Tertiary care center. PATIENTS: All patients aged 18 years and younger with parotid masses evaluated and treated at the Mayo Clinic, Rochester, Minn, from January 1, 1970, to December 31, 1997. RESULTS: Parotid masses were identified in 118 children (60 boys and 58 girls). At diagnosis, the ages of patients were from birth through 18 years, and 72 (61.0%) were aged 10 years and older. An asymptomatic mass was the most common presentation. Forty-three patients (36.4%) had infectious or inflammatory lesions, 56 (47.5%) had benign lesions, and 19 (16.1%) had malignant lesions. The most common benign lesions were pleomorphic adenoma (22.9%) and hemangioma (10.2%). The most common malignant lesions were mucoepidermoid carcinoma (6.8%) and acinic cell carcinoma (3.4%). The most common treatment was total parotidectomy (40.7%). Surgical complications included temporary facial nerve weakness in 22 (18.6%) patients, permanent facial weakness in 11 (9.3%), and permanent paralysis in 2 (1.7%). Pleomorphic adenoma recurred in 4 (14.8%) of 28 patients and mucoepidermoid carcinoma in 3 (37.5%) of 8 patients. One patient with adenoid cystic carcinoma died of the tumor. CONCLUSIONS: Although pediatric parotid masses are unusual, they can represent a variety of pathological diagnoses, including malignancy. We advocate prompt evaluation and treatment of these masses, and suggest guidelines for their management, based on diagnosis.

Hearing and otopathology in Crouzon syndrome.

OBJECTIVES: To better establish the incidence and types of otologic and auditory abnormalities in patients with Crouzon syndrome. STUDY DESIGN: Retrospective chart review of the otologic and auditory findings of patients diagnosed with Crouzon syndrome who were seen at our institution between 1978 and 1994. METHODS: Charts were reviewed and data recorded on patient sex, family history, appearance, auricular abnormalities, auditory findings, history of otologic disease, and follow-up. RESULTS: Nineteen patients were identified with the diagnosis of Crouzon syndrome: 12 males and 7 females. Twelve cases represented spontaneous mutations. Eight patients had abnormalities involving the external ear: from malalignment of the pinna (6 patients) to external auditory canal atresia (1 patient). Ten patients had documented hearing loss: 4 with conductive hearing loss, 2 with a mixed hearing loss, and 4 with a sensorineural hearing loss, the etiologies of which ranged from ossicular fixation and serous otitis media to unknown sensorineural deficits. CONCLUSIONS: Patients with Crouzon syndrome can exhibit various pathological features of the ear. Although external malformations are unusual, middle ear disease and hearing loss are common. We advocate close otologic and audiologic follow-up in these patients and note a higher frequency of sensorineural hearing loss than previously reported. Recent genetic advances may allow more accurate and earlier diagnosis of this syndrome.

Intranasal verrucous carcinoma: relationship to inverting papilloma and human papillomavirus.

OBJECTIVES: To establish the incidence, appearance, behavior, and appropriate treatment of intranasal verrucous carcinoma and determine its relationship to inverting papilloma and human papillomavirus (HPV). STUDY DESIGN: Retrospective review of all cases of intranasal verrucous carcinoma seen at the Mayo Clinic from 1960 through May 1996. METHODS: Retrospective chart review and data collection for age, sex, smoking history, location, association with inverting papilloma, treatment, recurrence, and follow-up. Polymerase chain reaction (PCR) testing for the presence of HPV DNA was performed on all specimens. RESULTS: Of the 13 patients identified, most presented with nasal obstruction (10) or a noticeable intranasal lesion (8). The maxillary sinus was the extranasal site most often involved. Five patients had verrucous cancer develop in an inverting papilloma, and one had squamous cell carcinoma with the verrucous component (a hybrid tumor). All but one patient underwent surgery as initial treatment; only one patient had preoperative radiation therapy. Surgical procedures ranged from local excision to a craniofacial resection. Follow-up ranged from 2 months to 32 years (mean, 6.5 y). Four patients had a single recurrence and two tumors recurred a second time. No metastases developed and no one died from the tumor. In seven patients (10 specimens), DNA was successfully amplified for PCR testing, and no HPV DNA was detected. CONCLUSIONS: When verrucous tumors are discovered early, they can be treated effectively with wide local excision. In some cases, a more extensive procedure may be required. A possible role for HPV in the etiology of these tumors was not found.

Long-term clinical experience with transtracheal oxygen catheters.

OBJECTIVE: To evaluate and discuss the use of transtracheal oxygen catheters for the treatment of chronic hypoxemia and to discuss the complications associated with the placement and care of these devices. DESIGN: We conducted a retrospective study at a tertiary medical center and reviewed the pertinent literature. MATERIAL AND METHODS: The medical records of 56 patients who received a transtracheal oxygen catheter between January 1987 and June 1992 at our institution were reviewed for demographic data, diagnosis leading to catheter placement, complications related to catheter use, reason for catheter removal, and duration of use. Follow-up results were established by documentation in the medical records or telephone interview. RESULTS: During the study period, 39 men and 17 women received a transtracheal catheter. More than half the patients (52%) had chronic obstructive pulmonary disease. The duration of use of the catheter ranged from 2 days to more than 6 years, and the most frequent cause for removal of the catheter was death. Of the 56 patients, 42 died with the catheter in place, 24 within the first year after placement. Complications ranged from mucous plugging (38 % of patients) to pneumothorax (4%), and no patient died of a catheter-related complication. Overall, 55% of patients had their catheter for less than 1 year after placement. CONCLUSION: In patients with transtracheal oxygen catheters, problems related to mucous plugging are common, but severe complications such as pneumothorax and pneumomediastinum are uncommon. Although selection factors that would identify ideal candidates for transtracheal oxygen therapy have not been established, such a catheter is best placed in highly motivated patients who can physically manage the daily care of this device.

Verrucous carcinoma of the larynx: a review of 53 patients.

BACKGROUND: Verrucous carcinoma of the larynx is a rare laryngeal neoplasm that can be confused with a benign process. Although usually curable at an early stage, it can be locally aggressive if left untreated. We describe our experience with this tumor and make recommendations for diagnosis and treatment. METHODS: From 1960 through 1990, 53 patients received initial treatment for verrucous carcinoma of the larynx at the Mayo Clinic. We retrospectively reviewed the presentation, diagnosis, treatment, and outcomes in these patients. All cases were confirmed by histologic examination. RESULTS: All but two patients were men, and most were smokers. Most tumors were found in the glottis, and 72% were T1 lesions at presentation. Six patients had hybrid tumors consisting of conventional squamous cell carcinoma along with the verrucous component. Sixteen patients had lesions that had been diagnosed as benign before they were seen at our institution. Treatment ranged from transoral excision to laryngopharyngectomy. Ten patients experienced recurrence, but only two patients died of the tumor. No pure verrucous lesion metastasized, and overall survival was similar to that of an age- and sex-matched cohort from the Western North Central U.S. white population. CONCLUSIONS: When discovered early, verrucous carcinoma of the larynx can be treated by local excision with good results; extensive lesions, however, may require more-aggressive procedures. Close communication between the pathologist and the clinician as well as adequate biopsy is essential for diagnosis. Patients with a conventional squamous cell carcinoma coexistent with a verrucous lesion must be treated as if they had invasive squamous cell carcinoma.

Lymphoma involving the esophagus.

Fewer than 1% of all lymphomas involve the esophagus; however, lymphoma of the esophagus represents an important cause of dysphagia. This study reviewed all cases of biopsy-proven lymphoma involving the esophagus presenting at our institution between 1945 and 1992. Twenty-seven cases were identified. Three were primary esophageal lymphomas. Eleven percent of the cases presented Hodgkin's disease. Eighty-nine percent were non-Hodgkin's lymphoma. Eighty-nine percent of the patients experienced dysphagia. Eleven lymphomas (41%) were located at the gastroesophageal junction, while the other 17 were in the esophagus proper. Seven of these cases occurred at relapse. Three had mediastinal adenopathy with secondary esophageal involvement. Morbidity included tracheoesophageal fistula in 22%, and surgical repair was performed in half of these cases. Vocal cord paralysis occurred in 22%, with minimal sequelae. Esophageal stricture was present in 30%, usually necessitating dilation. The presentation, diagnosis, and management of this problem are multidisciplinary.