Fine needle aspiration biopsy of pediatric head and neck masses.

OBJECTIVE: To determine if fine needle aspiration (FNA) can preclude the requirement for diagnostic open biopsy in suspicious pediatric head and neck masses. METHODS: The records of 40 children presenting to an inner city tertiary care hospital who underwent a total of 50 FNA biopsies during the years 1988-1999 were reviewed. From these 40 patients, 17 children, aged 3 months to 18 years, underwent both clinically indicated FNA biopsy and subsequent open surgical biopsy or excision. Outcome measurements included clinical resolution or surgical pathologic diagnosis. RESULTS: The 17 patients who underwent open surgical biopsy subsequent to the FNA had a total of 21 FNAs performed. Three of these patients had more than one needle biopsy prior to surgery. The histologic diagnosis of the surgical excision confirmed the FNA biopsy cytologic diagnosis in all but two cases. FNA cytologic diagnostic categories included reactive lymph node/non-specific inflammation (25 biopsies), benign cystic process (four), granulomatous disease (eight), malignant neoplasm (three), and benign neoplasm (one). Eight of nine FNAs initially non-diagnostic had either complete resolution of the mass or a diagnosis obtained by subsequent FNA or open biopsy. CONCLUSIONS: FNA is a valuable diagnostic tool in the management of children with the clinical presentation of a suspicious neck mass. The technique reduces the need for more invasive and costly procedures. Early surgical biopsy, however, should be considered in rapidly enlarging masses, in the presence of persistent systemic symptoms, and when repeated FNA cytology is non-diagnostic.

Analysis of a cohort of children with sensory hearing loss using the SCALE systematic nomenclature.

OBJECTIVES: What characteristics identify clinical types of childhood deafness? Which aspects of the otological evaluation best delineate them? To approach these related questions, a classification for deafness consistent with current medical concepts was constructed using a systematic nomenclature and then applied to a pediatric cohort of 168 children with sensorineural hearing loss (SNHL) who were referred for private consultation. A major aim of the analysis was to test the utility of SCALE, the new systematic nomenclature. METHODS: Patients with SNHL were identified through the office records of a single faculty member of the Department of Otolaryngology in a medical school situated in a major US city. Inclusion criteria required bone conduction thresholds above 30 dB or equivalent in at least one of the frequencies from 250 Hz to 4 kHz on either behavioral audiogram or on electrophysiological testing. All identified patients had initial visits during an 8-year period from late 1990 to early 1999. Patients were excluded if age at first consultation was 19 years or more, if records were insufficient to confirm SNHL, or if further evaluation revealed that SNHL had been misdiagnosed. A formal nomenclature was designed to systematically encode clinical features with simple descriptive terms according to an acronym (SCALE [sidedness, component function, age of onset, lesion, and etiology]) for all included patients. RESULTS: One hundred sixty-eight study patients were analyzed; sensory hearing loss was bilateral in 82% (137/168) and unilateral in 18% (31/168). The etiology of this impairment was determined to be intrinsic in 40% of children (67/168), either secondary to genotype (57/ 67), or to named congenital syndromes without known extrinsic cause (10/67). Recessive single gene mutations diagnosed by family history, recognition of syndrome, or determination of homozygous 35delG mutations in the gap junction protein gene, Connexin 26, accounted for bilateral sensory hearing loss in 33 children (24% of all bilateral cases). One girl had an X-linked dominant syndrome (Coffin-Lowry syndrome) with auditory brainstem response-documented childhood onset of SNHL. Nine patients (5%) had chromosomal aneuploidy, and 12 patients (7%) had either a family history of dominant deafness (7/ 12) or a recognizable autosomal dominant syndrome (5/12), most commonly, Waardenburg syndrome type 1 (4/5). Extrinsic causes of deafness were identified in only 13% of children (21/168) and included a relatively large number of referrals from neurosurgery (9/21). Three of these children had chronic middle ear disease and sensory hearing loss associated with inflammatory and bony changes on temporal bone imaging suggestive of chronic osteitis; all had a history of active otitis media during cranial irradiation. Congenital cytomegalovirus infections were documented in only 4 cases, but 41 patients could have had this as a cause or did not have this cause ruled out. An idiopathic cause or origin was assigned to 36% of patients (61/168) including patients with unnamed syndromic patterns of multiple anomalies. CONCLUSIONS: The SCALE nomenclature facilitated complete descriptions of hearing-impaired children and provided a classification scheme applicable to broad categories of human disease. The single most useful diagnostic test was screening for Cx26 mutations. Computed tomography scan of the temporal bones was helpful in establishing etiology for selected patients and invaluable in patients with chronic ear disease. Magnetic resonance imaging scan was a superior diagnostic modality in one child with a posterior fossa arachnoid cyst.

Airway management in children with major craniofacial anomalies.

OBJECTIVES: Delineation of clinical characteristics affecting the airway in a cohort of craniofacially deformed children. What factors differ between patients requiring and those not requiring surgical airway intervention? What factors predispose to the need for tracheotomy? When can decannulation be expected if tracheotomy is required? What interventions aid decannulation? STUDY DESIGN: Five-year retrospective chart review at tertiary center. METHODS: Two hundred fifty-one patients met the following entry criteria: enrollment in the New York University Institute of Reconstructive and Plastic Surgery's Craniofacial Clinic and admission to Tisch Hospital in Manhattan for surgery from 1990 to 1994. Hospital, clinic, and departmental office records were reviewed. All patients had major craniofacial bony anomalies and underwent administration of general anesthesia at least once. RESULTS: Nearly 20% of all children required tracheotomy (47/251). Craniofacial synostosis patients (Crouzon, Pfeiffer, or Apert syndrome) had the highest rate of tracheotomy (48% [28/59]). Mandibulofacial dysostoses patients (Treacher Collins or Nager syndrome) had the next highest rate (41% [28/59]). Patients with oculo-auriculo-vertebral sequence were less likely to undergo tracheotomy (22% [9/41]). Children with craniosynostosis rarely required a surgical airway, unless there was marked associated facial dysmorphism (1% [1/72]). The duration of cannulation was related to the age at tracheotomy in a bimodal distribution. Generally, tracheotomies required before age 4 years remained for several years, whereas those placed after age 4 were removed after several weeks. The presence of a cleft palate correlated with reduced risk for tracheotomy, but the presence of a ventriculoperitoneal shunt correlated with an increased risk for tracheotomy. Procedures selectively used to improve the airway included midface advancement, mandibular expansion, tonsillectomy and adenoidectomy, uvulopalatopharyngoplasty, anterior tongue reduction, and endoscopic tracheal granuloma excision. CONCLUSIONS: The likelihood for surgical airway management is related to specific craniofacial diagnosis. The length of tracheal cannulation is greatest for infants and young children who manifest severe airway compromise, often because of nasal obstruction in combination with other anatomic factors. Early tracheotomy is advocated for these patients to promote optimal growth and development. Choanal atresia is often misdiagnosed in these infants; nasal obstruction is actually secondary to midface retrusion. Staged surgical interventions can allow eventual successful decannulation in nearly all cases of craniofacial syndromes.

Hearing loss in Turner syndrome.

A retrospective study was undertaken to answer the following questions: Is the sensorineural hearing loss (SNHL) in Turner syndrome progressive? Can the occurrence of hearing loss be explained by the parental origin of the intact X chromosome? Twenty-four individuals recruited through the Turner Syndrome Society completed a questionnaire and submitted sufficient medical records to determine their otologic status. The majority (21/24) have had problematic otitis media (OM), and two thirds (16/24) have SNHL. In seven of the Turner subjects (age range: 12 to 51 years), gradual progressive SNHL began in late childhood or early adulthood. Molecular techniques showed no correlation between parental origin of the retained X chromosome and hearing status in 17 Turner subjects and at least one of their parents. SNHL and frequent OM appear to be independent variables that are both present in Turner syndrome. It is postulated that the presence of unpaired genes on the X chromosome may account for hearing loss and other phenotypic abnormalities seen in this syndrome.

Database for sensorineural hearing loss.

We are creating a bank of EBV immortalized lymphoblast cells and extracted DNA taken from the blood of deaf children and their relatives, in order to study the molecular basis of hereditary deafness. We have established a corresponding database for sensorineural hearing loss that records clinical data for each entered specimen. The purpose of this paper is to present the content and design of the computerized relational database. The data model is designed first to identify known etiologies of deafness, either acquired or syndromic, and then to characterize the clinical features of the deaf individual, and both their affected and non-affected family members. The application operates in a graphical environment of visual prompts and message panels. The database is organized by sections which record demographic data, presenting complaints, otologic history, birth and perinatal history, developmental history, symptoms of chronic airway obstruction, family history, neurologic history, congenital infections, hospitalizations and surgical history, medication history, vestibular findings, audiometry, radiology, medical conditions and syndromes and physical examination. The database was developed on a commercially available software product. Our database is presented as a model for use by other clinicians and investigators.

Complications of tympanostomy tubes in an inner city clinic population.

While both prophylactic antibiotics and tympanostomy tube insertion have a role in the treatment of recurrent acute otitis media (AOM) and otitis media with effusion (OME) in children previous work has shown that patients in our urban clinic are not compliant with prophylactic antibiotics. Concerned about the potential for decreased compliance in a non-compliant population, we performed a retrospective review to assess the incidence of complications from the insertion of tympanostomy tubes in the same pediatric clinic population. A total of 391 tubes were placed in 165 patients. Follow-up ranged from 0-49.4 months with a mean of 21.3 months. Three ears (1.14%) had persistent perforations. Tympanosclerosis was found in 30 ears (11.1%). No ear showed a chronic retraction or cholesteatoma. Six ears (1.70%) developed postoperative otorrhea. Thirty-five patients had at least one episode of otorrhea outside of the perioperative period, and a total of 60 episodes (19.6% of ears) occurred during the study period. The mean pure tone average prior to tube placement was 25.0 dB, with tubes in place was 2.44 dB and after the last set of tubes had extruded was 6.97 dB. Our study shows that the incidence of complications of tympanostomy tubes was minimal in our inner city clinic population.

Clinical diagnosis of pediatric obstructive sleep apnea validated by polysomnography.

The decision to perform tonsillectomy and adenoidectomy for treatment of pediatric obstructive sleep apnea syndrome is often made on a clinical basis without formal polysomnography. To examine the accuracy of the clinical diagnosis of pediatric obstructive sleep apnea syndrome, we prospectively evaluated 30 children with obstructive symptoms by a standardized history, physical examination, and review of a tape recording of breathing during sleep. On the basis of this clinical evaluation, patients were divided into three predictive groups: (1) definite obstructive sleep apnea syndrome, (2) possible obstructive sleep apnea syndrome, and (3) unlikely to have obstructive sleep apnea syndrome. Nocturnal polysomnography was used to determine the presence or absence of true sleep apnea. Ten of 18 (55.6%) patients predicted clinically to have definite obstructive sleep apnea syndrome had positive nocturnal polysomnographies. Two of six (33.3%) patients predicted to have possible obstructive sleep apnea syndrome had positive nocturnal polysomnographies. One of six (16.7%) patients predicted to be unlikely to have obstructive sleep apnea syndrome had a positive nocturnal polysomnography. Six nocturnal polysomnographies negative by conventional criteria were suspicious for apnea, but considering these positive for obstructive sleep apnea syndrome did not improve the specificity of the clinical prediction. Our results show that clinical assessment of obstructive sleep apnea syndrome in children is sensitive (92.3%) but not specific (29.4%) for making the diagnosis of obstructive sleep apnea syndrome as compared with nocturnal polysomnography and may contribute to the decision to obtain nocturnal polysomnography in specific circumstances.

The role of flexible bronchoscopy in children with AIDS: an update of the New York University experience.

The clinical courses of children with acquired immunodeficiency syndrome (AIDS) who underwent diagnostic flexible bronchoscopy at Bellevue Hospital from 1987-1992 were reviewed to determine the value of the procedure in patient management. Twenty-eight children (age 13 days to 12 years) underwent 31 bronchoscopies for indications including respiratory distress, fever and abnormal chest radiograph. Procedures were well tolerated. Complications were limited to transient hypoxia and epistaxis. Although 58% of bronchoscopies yielded a diagnosis (Pneumocystis carinii, Streptococcus viridans, Pseudomonas aeruginosa, Cytomegalovirus, atypical mycobacterium, giant cell pneumonia, and mechanical obstruction), empiric medical therapy was altered in only 16% of cases. Bronchoscopic diagnoses are correlated with Centers for Disease Control (CDC) classification, immune status, treatment and outcome.

Compliance with prophylactic antibiotics for otitis media in a New York City clinic.

Although previous efficacy studies have reported high compliance rates among children treated by private physicians with prophylactic antibiotics for prevention of otitis media, compliance rates in a lower socioeconomic, urban clinic population have not been well described. Eighty children who were placed on daily low dose antibiotics in the Pediatric Otolaryngology Clinic at Bellevue Hospital were prospectively followed in order to estimate compliance in this population. Compliance was estimated by the parent's stated word alone. Of the 77 patients with records adequate for analysis, only 36 (46.8%) families claimed compliance, 18 (23.4%) admitted non-compliance, and 23 (29.9%) did not reliably return for follow-up clinic visits. Statistical analysis revealed that no single characteristic of the patient population or the treatment regimen strongly influenced compliance. The factors examined included: the child's age, sex, race and otologic diagnosis, the type of prophylaxis prescribed, the parent's ability to speak English, national origin, consistency of follow-up, and method of payment. Based on these results, less than half of the children attending an inner city clinic compiled with maintenance medication. The clinician should consider alternate treatment modalities, such as the insertion of middle ear ventilation tubes, if poor compliance with prolonged antibiotic treatment is suspected.

Noncongenital hereditary hearing loss in children. Prospective documentation.

Younger siblings of children with sensorineural hearing loss of possible hereditary cause underwent interval audiologic examination. Seven siblings (in unrelated families) were found to have progressive sensorineural hearing loss despite early audiograms documenting normal hearing levels for age. Continued testing of these children allowed for early identification and intervention. We advocate regular otolaryngologic and audiologic follow-up even after normal audiologic assessments are made for younger siblings of children with documented sensorineural hearing loss, unless a definite nongenetic origin of the hearing loss in the older child is known. Recessive sensorineural hearing loss with onset in infancy or childhood may present with no antecedent family history and with normal behavioral audiograms early in life.

Fibrous histiocytoma of the trachea: management of a rare cause of upper airway obstruction.

We describe a case of fibrous histiocytoma of the trachea diagnosed in a 17-year-old female who presented with symptoms of 'asthma'. Management included rigid bronchoscopy with biopsy and debulking of this obstructing tumor, later excised with partial tracheal resection. Although tracheal tumors are quite rare in children, the majority (6/9) of reported cases of fibrous histiocytoma of the trachea have been described in the pediatric age group. The possibility of a tracheal neoplasm as a cause of wheezing, stridor or hemoptysis in children should be recognized. Control of the airway without tracheotomy may facilitate surgical cure via tracheal resection in such cases.

Pediatric human immunodeficiency virus infection: an otolaryngologist's perspective.

Children with human immunodeficiency virus (HIV) frequently have recurrent otitis media, chronic rhinorrhea, parotitis, cough and other common pediatric otolaryngologic problems. As these complaints often occur before more unusual opportunistic infections or pulmonary conditions prompt a diagnosis of acquired immunodeficiency syndrome (AIDS), members of our specialty are liable to see HIV-positive children before infection with the virus has been recognized. Children with HIV infection are also likely to be referred to us after diagnosis, as is any immunosuppressed child with otolaryngologic infections. These children may require procedures such as bronchoscopy, sinus irrigations or tympanocentesis. The subject of this review is the natural history of pediatric HIV infection with special emphasis on otolaryngologic manifestations and recommendations for safe techniques of examination and treatment.

Otitis media and hearing loss in Turner syndrome.

Twenty-two phenotypic females with Turner syndrome underwent prospective otologic evaluation including a standard history, physical examination, audiogram, and tympanogram. Eight of these patients had computed tomography of the temporal bones. Eighty-two percent of the patients had a history of chronic or recurrent ear infections. Eleven patients (50%) had previous myringotomy and tube placement and 4 (16%) had undergone tympanoplasty or tympanomastoidectomy for sequelae of otitis media. Ten patients (45%) had middle ear effusions evident on examination. Sixteen patients (73%) had hearing loss in at least one ear at the time of examination. Sensorineural losses were evident in 37% of patients. No malformations of the otic capsule were noted on computed tomography. The high prevalence of both hearing loss and otitis media in Turner syndrome warrants otologic and audiologic assessment of patients with this chromosomal anomaly.

Otolaryngologic management of posttransplant lymphoproliferative disease in children.

Twelve of 14 children (86%) given a pathologic diagnosis of posttransplantation lymphoproliferative disease (PTLD) at the Children's Hospital of Pittsburgh presented with head and neck symptoms, and are included in this retrospective analysis. Upper airway obstruction was the most common symptom, evident in nine children (75%). Ten children (83%) had febrile illnesses with dysphagia, odynophagia, and evidence of hypertrophy of components of Waldeyer's ring. Associated findings included cervical adenopathy, sinusitis, and otitis media. The two remaining children had an intratracheal and paratracheal mass, respectively. Excision of obstructing lymphoid tissue with proper handling of the specimen is advocated for diagnosis and relief of airway obstruction. Systemic therapy is necessary for treatment of PTLD and includes reduction of immunosuppression. Erythromycin causes elevation in cyclosporine levels and should be avoided in patients taking this drug.

Diagnostic flexible bronchoscopy in human immunodeficiency virus (HIV)-positive children.

Twelve children with laboratory evidence of human immunodeficiency virus (HIV) infection underwent diagnostic flexible bronchoscopy with washings or bronchoalveolar lavage at Bellevue Hospital Center from October 1987 to April 1989. The patients included 7 boys and 5 girls ranging from age 3.5 months to 10 years 5 months. Indications for bronchoscopy included respiratory distress with or without focal changes on chest radiograph in 11 patients, and persistent but asymptomatic right middle lobe collapse in one child. The etiology of pneumonia was diagnosed in 7 children and included Pneumocystis carinii, (PCP) (17%), Streptococcus viridans (17%), mechanical obstruction (17%) and cytomegalovirus (CMV) (8%). Bronchoscopy was non-diagnostic in 5 cases. Techniques for maximal yield of information using flexible bronchoscopy in HIV-positive children are discussed.

Pathogenesis of cholesteatoma.

Aural cholesteatomas consist of accumulations of desquamating keratinized epithelium within the middle ear or other pneumatized portion of the temporal bone. Cholesteatomas may be classified according to presumed etiology as congenital or acquired. The theoretical mechanisms of the pathogenesis of the two types are discussed.