Postmeningitic pediatric hearing loss from non-type b Haemophilus influenzae.

BACKGROUND: Postmeningitic hearing loss from Haemophilus influenzae (H. influenzae) is increasingly due to encapsulated serotypes other than type b (Hib) and nontypeable strains (collectively, nHiB H. influenzae). Pediatric hearing loss after nHib H. influenzae meningitis remains poorly described. METHODS: Retrospecive case series of nHiB H. influenzae meningitis cases identified from a microbiologic database at Children's Hospital Colorado from 2000 to 2020. Literature regarding nHiB H. influenzae and H. influenzae postmeningitic hearing loss was also reviewed. RESULTS: Eleven cases of nHib H. influenzae meningitis (median age 15.9 months) were identified due to serotype f (36 %), serotype a (27 %), and nontypable strains (36 %). Seven (64 %) patients were male, 55 % were white and 18 % were Hispanic or Latino. Hearing loss was initially identified in 4 children (40 %), with two patients with moderate conductive hearing loss (CHL) and one child with unilateral moderate sensorineural (SNHL) hearing loss patients recovering normal hearing. One patient with bilateral profound sensorineural hearing loss and associated labyrinthitis ossificans required cochlear implantation. All children (4) with identified hearing loss were noted to have additional intracranial sequelae, which included empyema (2), sinus thrombosis (2), and seizures (2). Of patients receiving steroids, 25 % had hearing loss on initial testing, compared to 66 % of those who did not receive steroids. CONCLUSIONS: nHib H. influenzae can cause both transient and permanent postmeningitic hearing loss. Steroids may offer otoprotection in nHib H. influenzae meningitis similar to Hib meningitis. Given the limited literature, further study is needed to better characterize hearing outcomes after nHib H. influenzae meningitis.

Pediatric primary salivary gland tumors.

OBJECTIVES: To characterize the presentation and treatment of children presenting with primary salivary gland neoplasms. METHODS: A retrospective review of primary salivary tumor patients presenting to Children's Hospital Colorado between January 2000 and August 2020. RESULTS: Fifty children were identified with primary salivary gland tumors, comprising of 39 (78 %) benign and 11 (22 %) malignant lesions. Pleomorphic adenoma was the most common benign tumor (36/39, 92 %), while acinic cell carcinoma was the most common malignancy (7/11, 64 %). The parotid gland was the most common site, followed by the submandibular gland (66 % vs. 34 %). No tumors were found in the sublingual glands. Benign neoplasms accounted for 70 % of parotid lesions and 94 % of submandibular tumors. No significant differences in age (13.6 years, SD 4 vs. 13.0 years, SD 4.3) were noted between patients with benign and malignant disease, but tumors in females were more frequently malignant (M:F 1:1.3 vs. 1:2.7 for benign and malignant tumors, respectively). Neck dissection and/or facial nerve sacrifice were required in 27 % (3/11) and 9.1 % (1/11) of malignancies, respectively. Local recurrence was observed in 7.7 % (3/39) of benign cases and 9.1 % (1/11) of malignant cases. No salivary malignancies required chemotherapy, though one patient with neurofibromatosis received imatinib prior to resection. Two patients with locoregional malignancy received adjunctive radiation. The average duration of follow up for benign and malignant disease were 12.6 ± 25 and 45.1 ± 32 months, respectively. CONCLUSIONS: This study presents one of the larger single institutional experiences of pediatric primary salivary neoplasms in the past 20 years, identifying pleomorphic adenoma and acinic cell carcinoma as the most common benign and malignant etiologies, respectively. While this review found most neoplasms presented as a localized mass effectively managed with conservative surgical resection, aggressive tumors required multidisciplinary care.

Hearing outcomes in children with pneumococcal meningitis in the PCV13 era.

INTRODUCTION: Streptococcus pneumoniae, is associated with the highest incidence of post-meningitic SNHL. The exact impact of 13-valent pneumococcal conjugate vaccine (PCV) on pediatric SNHL from pneumococcal meningitis is unknown. We aimed to identify clinical factors associated with post-meningitic SNHL (pmSNHL) from pneumococcal meningitis and describe its rates based on three time periods: pre-PCV, PCV-7 and PCV13 eras. METHODS: A retrospective case-control study was performed for patients 18 years and younger diagnosed with pneumococcal meningitis from January 1, 2010 to December 31, 2020 at Children's Hospital Colorado. Demographic and clinical risk factors between those with or without SNHL were compared. Detailed hearing outcomes of those with resulting SNHL are described. RESULTS: 23 patients with CSF cultures or Meningitis/Encephalitis Panel positive for pneumococcal meningitis were identified. Twenty patients both survived the infection and had audiologic evaluation. Six patients had pmSNHL, with 50 % affected bilaterally. The rate of pmSNHL from S. pneumoniae in the PCV-13 era at our institution was similar to historical rates from the pre-PCV and PCV-7 eras. Similar proportions of patients with pmSNHL completed PCV vaccination (66.7 %) compared to those without (71.4 %). Non-PCV-13 serotypes were responsible 83 % of patients with pmSNHL versus 57 % of patients without pmSNHL. CONCLUSIONS: Despite high rates of PCV-13 uptake in our cohort, pmSNHL was still common, severe, and commonly associated with non-PCV-13 serotypes. Non-PCV-13 serotypes may be contributing to the persistently high rate of post-meningitic SNHL and the severity of SNHL. Newer pneumococcal conjugate vaccines with expanded serotypes may help mitigate the SNHL associated with pneumococcal meningitis.

A Novel Articulating Chip-on-Tip Endoscope for Dynamic Middle Ear Surgical Visualization.

OBJECTIVE: To enhance visualization in pediatric Otolaryngology middle ear surgeries and reduce mastoidectomy instances, we introduce a novel Articulating Chip-on-Tip Endoscope (ACoT Endo). METHODS: The ACoT Endo incorporates a cable-driven distal end camera and off-the-shelf Chip-on-Tip camera to improve visualization. We compared its capabilities with standard endoscopes, evaluating its bending capacity (70 ° ± 2 °) and center axis rotation (360 °). To test the overall functionality of this device, a Mock Ear was created to simulate the anatomy of the human ear, and the ACoT Endo's ability to be used in this cavity is compared to a standard 0 ° Karl Storz endoscope through tests with the Mock Ear and respective endoscopes. RESULTS: The ACoT Endo accurately captured surgical details similar to standard endoscopes in the ENT field. Compared to the 0 ° Karl Storz endoscope, the ACoT Endo demonstrated an increased field of view by approximately 69% and captured area by approximately 249%. ACot Endo allowed the surgeon to effortlessly articulate the camera with the rotation of a finger, while an excision tool was inserted in the middle ear, a procedure that is currently extremely difficult with standard endoscopes. CONCLUSION: The ACoT Endo's dynamic viewing angle and Chip-on-Tip camera enable unparalleled surgical visualization within the middle ear using a single endoscope, offering potential benefits in Otolaryngology procedures. SIGNIFICANCE: By reducing the need for invasive mastoidectomies and providing better visualization tools, the ACoT Endo has significant potential to improve outcomes and safety in pediatric middle ear surgeries.

Olfactory dysfunction and training in children with COVID-19 infection: A prospective study Post-COVID pediatric olfactory training.

OBJECTIVE: Postviral olfactory dysfunction (OD) including corona 2019 viral disease (COVID-19) OD occurs in both adults and children. Despite limited reports of efficacy in treating adult postviral including COVID-19 OD with olfactory training (OT), its effects on children in general, and post-COVID-19 in specific, is unknown. The study aimed at evaluating the effects of OT in a COVID-19 OD pediatric cohort. METHODS: A single-arm prospective study of pediatric COVID-19 OD subjects confirmed by the University of Pennsylvania Smell Identification Test (UPSIT), was conducted. All subjects underwent OT by sniffing 4 odorants (lavender, orange, peppermint, and eucalyptus) for 1 min twice a day for 3 months. Subjects underwent an odorant identification test (OIT) of the 4 odorants each visit. A repeat UPSIT was administered at the 4th visit. RESULTS: The study enrolled a total of 37 subjects [11 males/26 females with mean age/standard deviation (std) of 15.6(2.1) years]. The time interval between COVID-19 and entry was 5.3(2.4) months. The mean pre/post study UPSIT score improvement was 2.3(4.7), p = .09. OIT scores between entry and 3 subsequent visits showed a mean improvement of 1.8(1.5), 1.8(1.9) and 2.3(1.9) odorants, respectively, with P < .001 for all 3 comparisons. CONCLUSIONS: OT subjects were predominantly female teens with substantial OD lasting greater than 5 months. OT did not affect OD as measured by UPSIT but OIT scores improved during OT. We postulate that OT likely has a role in pediatric post-COVID OD recovery, but UPSIT likely is too rigid to detect disparate odorant improvement.

Do Surgical Smoke Evacuators Increase the Risk of Hearing Loss for Operative Personnel During Routine Adenotonsillectomy Surgery?

INTRODUCTION: Aerosol mitigation equipment implemented due to COVID-19 has increased noise levels in the operating room (OR) during otolaryngological procedures. Intraoperative sound levels may potentially place personnel at risk for occupational hearing loss. This study hypothesized that cumulative intraoperative noise exposures with aerosol mitigation equipment exceed recommended occupational noise exposure levels. METHODS: Sound levels generated by the surgical smoke evacuator (SSE) during adenotonsillectomy were measured using a sound level meter and compared to surgery without SSE. RESULTS: Thirteen adenotonsillectomy surgeries were recorded. Mean sound levels with the SSE were greater than the control (72 ± 3 A-weighted decibels (dBA) vs. 68 ± 2 dBA; p=0.015). Maximum noise levels during surgery with SSE reached 82 ± 3 dBA. CONCLUSION: Surgeons performing adenotonsillectomy with aerosol mitigation equipment are exposed to significant noise levels. Intraoperative sound levels exceeded international standards for work requiring concentration. Innovation is needed to reduce cumulative OR noise exposures.

Rosai-Dorfman-Destombes Disease in the Pediatric Head and Neck.

BACKGROUND: Rosai-Dorfman-Destombes disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a rare form of non-Langerhans cells histiocytosis. It has a wide-ranging variability in presentation since first described in 1969 but much of its characteristics in children remain unknown. METHODS: A retrospective chart review of children diagnosed with RDD at a tertiary care children's hospital was conducted from 2000 to 2021. RESULTS: Twelve RDD patients were identified, with an average age of 7 years (SD 4.3). Males comprised 58% of the cohort, and African American ethnicity was most common (42%). Nodal RDD was found in 7 patients (58%). Nine patients (75%) presented RDD within the head and neck, 6 of whom had nodal RDD. The most common presentation was cervical lymphadenopathy, which most often involved levels V (67%), II (56%), III (44%), and I (11%), in order of frequency. Recurrence and persistence of disease after initial treatment was common, with 5 (42%) being disease free at the time of the last follow up. Fifty-eight percent (7/12) developed recurrence or had persistent disease and 4 required adjuvant systemic treatment with corticosteroids and/or chemotherapy. One patient succumbed after developing treatment related acute myelodysplastic leukemia (t-AML) from chemotherapy used to treat recurrent RDD. CONCLUSION: Pediatric RDD presents at a young age and most commonly involving cervical lymphadenopathy. Ongoing surveillance in the setting of persistence or recurrence without clearly defined prognostic risk factors is important.

Concurrent Pediatric Lingual and Submental Dermoid Cysts: Case Report and Literature Review.

This pediatric case report describes the novel finding of concurrent submental and lingual dermoid cysts, which to our knowledge, has not been previously reported in the literature. The etiology of cysts involving the tongue, floor of the mouth, and submental neck is varied, representing congenital, inflammatory, and neoplastic sources. Dermoid cysts involving these regions are uncommon and are most frequently reported in the submental, sublingual, and lingual spaces. Presenting symptoms vary with cyst size and position relative to the mylohyoid muscle. MRI is the preferred modality to differentiate dermoid cysts from other etiologies. While interventional techniques have been utilized to treat dermoid cysts in other head and neck locations, surgical excision remains the preferred treatment for those involving oral and floor-of-mouth structures.

Otitis Media in Children with Down Syndrome Is Associated with Shifts in the Nasopharyngeal and Middle Ear Microbiotas.

Background: Otitis media (OM) is defined as middle ear (ME) inflammation that is usually due to infection. Globally, OM is a leading cause of hearing loss and is the most frequently diagnosed disease in young children. For OM, pediatric patients with Down syndrome (DS) demonstrate higher incidence rates, greater severity, and poorer outcomes. However, to date, no studies have investigated the bacterial profiles of children with DS and OM. Method: We aimed to determine if there are differences in composition of bacterial profiles or the relative abundance of individual taxa within the ME and nasopharyngeal (NP) microbiotas of pediatric OM patients with DS (n = 11) compared with those without DS (n = 84). We sequenced the 16S rRNA genes and analyzed the sequence data for diversity indices and relative abundance of individual taxa. Results: Individuals with DS demonstrated increased biodiversity in their ME and NP microbiotas. In children with OM, DS was associated with increased biodiversity and higher relative abundance of specific taxa in the ME. Conclusion: Our findings suggest that dysbioses in the NP of DS children contributes to their increased susceptibility to OM compared with controls. These findings suggest that DS influences regulation of the mucosal microbiota and contributes to OM pathology.

Growth After Adenotonsillectomy for Obstructive Sleep Apnea: Revisited.

OBJECTIVES/HYPOTHESIS: To reanalyze the growth trajectory and assess longitudinal changes of children undergoing adenotonsillectomy (AT) versus watchful waiting (WW) enrolled in the Childhood Adenotonsillectomy Trial (CHAT) study and to determine if an AT increases the risk of obesity in children. STUDY DESIGN: Reanalysis of prospective cohort investigation. METHODS: The study analyzed publicly available data from CHAT, including 3 months visit data not previously included in a prior publication. Statistical comparisons and mixed-effects modeling were done using age- and sex-specific BMI expressed as a percentage of the 95th percentile (%BMIp95). P < .05 was considered significant. RESULTS: Children in the AT group, especially if underweight at baseline, had an increased rate of weight gain, with 100% of underweight children in the AT group becoming normal weight compared to 20% for WW. However, the rate of weight gain, as measured by the %BMIp95 trajectory for both AT and WW groups, was not significantly different when baseline weight status and obstructive sleep apnea (OSA) resolution were accounted for. Comparisons of %BMIp95 between treatment groups at baseline, 3- and 7-month follow-up visits also failed to identify statistically significant differences (P > .05). Overall for the entire cohort, resolution of OSA was associated with a decreased weight trajectory (P < .001). CONCLUSIONS: AT compared to WW is not associated with an increased risk of excessive weight gain. Otolaryngologists should be aware of this updated analysis when discussing AT surgical outcomes with families. LEVEL OF EVIDENCE: 2 Laryngoscope, 132:1289-1294, 2022.

Olfactory testing as COVID-19 screening in school children; A prospective cross-sectional study.

BACKGROUND: Little is known about olfactory changes in pediatric COVID-19. It is possible that children under-report chemosensory changes on questionnaires, similar to reports in adults. Here, we aim to describe COVID-19-related olfactory dysfunction in outpatient children. We hypothesized that children with COVID-19 will demonstrate abnormal olfaction on smell-identification testing at a higher rate than children with negative COVID-19 testing. METHODS: A prospective cross-sectional study was undertaken from June 2020-June 2021 at a tertiary care pediatric hospital. A consecutive sample of 205 outpatients aged 5-21 years undergoing severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) PCR testing were approached for this study. Patients with prior olfactory dysfunction were excluded. Participants were given a standard COVID-19 symptom questionnaire, a Smell Identification Test (SIT) and home-odorant-based testing within 2 weeks of COVID-19 testing. Prior to study enrollment, power calculation estimated 42 patients to determine difference in rates of SIT results between groups. Data were summarized with descriptive statistics. RESULTS: Fifty-one patients underwent smell identification testing (23 positive (45%) and 28 negative (55%) for COVID-19; mean age 12.7 years; 60% female). 92% of all patients denied subjective change in their sense of smell or taste but only 58.8% were normosmic on testing. There was no difference in screening questionnaires or SIT scores between COVID-19 positive and negative groups. CONCLUSIONS: Unlike adults, there was no statistical difference in olfactory function between outpatient COVID-19 positive and negative children. Our findings suggest a discrepancy between objective and patient-reported olfactory function in pediatric patients, and poor performance of current screening protocols at detecting pediatric COVID-19.

Pediatric Primary Tympanoplasty Outcomes With Autologous and Non-autologous Grafts.

OBJECTIVE: To compare rates of successful tympanic membrane (TM) closure in primary pediatric tympanoplasty between various autologous and non-autologous tissues. METHODS: A retrospective chart review was performed examining all primary pediatric tympanoplasties over a 20-year period at a single institution. RESULTS: In 564 pediatric tympanoplasties, no statistically significant difference existed between success rates of autologous and non-autologous grafts (p = 0.083). Compared with fascia, the hazard ratios (and 95% confidence intervals [CI]) for failure for each graft were as follows: human pericardial collagen (HR 0.90, CI 0.54-1.50, p = 0.680), porcine submucosal collagen (HR 1.07, CI 0.56-2.05, p = 0.830), human acellular dermal collagen (HR 1.66, CI 0.95-2.87, p = 0.073), and "multiple grafts" (HR 0.72, CI 0.26-1.98, p = 0.520). Survival curves demonstrated that 75% of graft failures occurred by 6 months after surgery, the rest occurring between 6 and 12 months postoperatively. Larger perforations encompassing more than or equal to 50% of the TM had lower success rates (HR 1.50, CI 1.02-2.21, p = 0.041) than smaller perforations encompassing less than 50% of the TM. Age was not correlated with success (HR 0.98, CI 0.93-1.03, p = 0.390). CONCLUSION: This study found that non-autologous collagen grafts provide equivalent rates of healing when compared with autologous tissue in primary pediatric tympanoplasty. In addition to the potential for reduced operative time and donor site morbidity, these materials provide a viable graft alternative in fascia-depleted ears.Level of Evidence: Level 4.

Pediatric Head and Neck Tumors Associated with Li-Fraumeni Syndrome.

INTRODUCTION: Cancer predisposition syndromes are germline pathogenic variants in genes that greatly raise the risk of developing neoplastic diseases. One of the most well-known is Li-Fraumeni syndrome (LFS), which is due to pathogenic variants in the TP53 gene. Children with LFS have higher risks for multiple malignancies before adulthood, often with rare and aggressive subtypes. OBJECTIVE: To examine head and neck manifestations of LFS in children treated at a tertiary children's hospital over a 20-year period. METHODS: A retrospective review of LFS children with neoplastic disease presenting in traditional Otolaryngologic head and neck subsites from 2000 to 2019, with patient charts reviewed for relevant clinical, imaging, and operative data. RESULTS: Of the 40 LFS patients initially identified, 27 neoplastic tumors were identified in 20 children within this cohort (20 primary, 7 second primary). Head and neck subsites aside from the brain or orbit were involved in 22% (6/27) of these tumors, representing 20% (4/20) of primary tumors and 29% (2/7) of second primary tumors. Both second primaries within the head and neck were within the radiation fields of the first primary tumor. The mean ages at primary and second primary diagnosis were 4.6 years (SD 3.5) and 12 years (SD 1.4), respectively. The male/female ratio was 1:6 among all patients with head and neck tumors. All 6 head and neck tumors were sarcomas. Rhabdomyosarcoma (N = 3, 50%) was the most common pathology, and the other 3 demonstrated rare tumor pathological subtypes (synovial cell sarcoma, pleomorphic myxoid liposarcoma, mandibular osteosarcoma). The neck was the most common subsite (75%) within this group for primary tumor presentation. CONCLUSION: This study identifies a high potential for head and neck involvement in children with LFS, which has not been previously described in the literature. Otolaryngological care should be included in a multidisciplinary care team surveilling these patients.

Parental Perception of Weight Status for Adenotonsillectomy Patients.

OBJECTIVE: Weight status can affect outcomes in pediatric adenotonsillectomy performed for obstructive sleep disordered breathing. Parents frequently underestimate their child's weight and are unaware weight status may affect adenotonsillectomy success. Accurate understanding of a child's weight status is important for shared decision making with the family and perioperative care. The purpose of this study is to analyze the accuracy of the parent's perception of their child's weight status. METHODS: A retrospective analysis was performed of prospective data collected from families of children undergoing adenotonsillectomy from June 2018 through June 2019. RESULTS: A total of 522 children met the inclusion criteria. Two hundred and thirty-two children were either overweight (n = 46, 9%) or obese (n = 186, 36%). Among parents of this cohort whose children were overweight or obese, 74 (32%) erroneously reported that their child was normal weight. For the 290 nonoverweight children, 99% of parents accurately reported weight status. After adjusting for ethnicity, race, BMI%, and sex, for every 1-year increase in age of the child, the odds of the parent correctly identifying their child as overweight increased by a factor of 1.18 (95% CI: 1.09, 1.27). CONCLUSION: One-third of families with children who were overweight or obese undergoing adenotonsillectomy for obstructive sleep disordered breathing underestimated their child's weight. This study highlights the need to facilitate family understanding of weight status' potential impact on both obstructive sleep disordered breathing severity and adenotonsillectomy success, especially for younger children. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:2121-2125, 2021.

Pediatric sublingual dermoid and epidermoid cysts: A 20-year institutional review.

INTRODUCTION: Pediatric cystic sublingual masses often present a diagnostic dilemma for practitioners. Though uncommon, dermoid or epidermoid cysts can present in the sublingual space at any age and are often misdiagnosed as an inflammatory pseudocyst (ranula) or lymphatic malformation. Imaging may not always identify the underlying etiology, requiring physicians to maintain a high index of suspicion for these relatively rare oral cysts. OBJECTIVES: To describe the presentation and treatment of sublingual dermoid and epidermoid cysts presenting to a tertiary children's hospital over 20 years. METHODS: A retrospective review of all pathology specimens identified as dermoid or epidermoid cysts within the sublingual space from 1999 to 2019. Patient charts were then reviewed for relevant clinical, imaging, and operative data. RESULTS: Twelve pediatric patients were identified (8 female, 4 male) with a mean age of 7.2 years (SD 5.6). Eighty six percent (6/7) of dermoid cysts were found in female patients, while 60% (3/5) of epidermoid cysts were in male patients. Multiple dermoid and epidermoid cysts were each found in one patient (8%). Two epidermoid cysts presented in the neonatal period. Preoperative diagnosis included nondiagnostic "cystic mass" (33%), ranula (25%), lymphatic malformation (LM) (17%), and dermoid/epidermoid cyst (17%). Two thirds of patients (8/12) underwent imaging, with all receiving either MRI or CT. Although MRI was the most likely to suggest the possibility of a dermoid/epidermoid cyst (2/4), ranula was the most common primary radiographic diagnosis (5/8). One patient underwent sclerotherapy for presumed LM one year prior to surgical excision of the cyst. Eleven patients (92%) underwent intraoral excision, one (8.3%) underwent a combined intraoral/extraoral approach. CONCLUSIONS: To our knowledge, this review represents the largest case series of pediatric sublingual dermoid and epidermoid cysts to date. This series contained higher levels of epidermoid cysts and female patients than previously reported in the literature. Identifying more dermoid cysts in females and epidermoid cysts in males is also a new finding. MRI was superior to CT and US regarding the presence of a dermoid/epidermoid cyst. Frequently misdiagnosed, it is important to consider these relatively rare pathologies when treating children presenting with sublingual masses in order to avoid delayed and/or inappropriate treatment.

Head and neck pleomorphic myxoid liposarcoma in a child with Li-Fraumeni syndrome.

INTRODUCTION: Pleomorphic myxoid liposarcoma is a rare and aggressive cancer seen in the pediatric population that has been previously associated with hereditable cancer disorders like Li Fraumeni syndrome. We present a case report and review of the relevant literature. CASE PRESENTATION: Pleomorphic myxoid liposarcoma presenting as a second primary tumor in a child with a strong family history for cancer led to diagnosis of Li-Fraumeni syndrome, which is associated with TP53 tumor suppressor gene inactivation. MANAGEMENT AND OUTCOME: The tumor was fully excised, but postoperative radiation was deferred to limit future radiation-induced tumorgenesis. DISCUSSION: Pleomorphic myxoid liposarcoma is rare but aggressive, and should prompt caregivers to test for potential hereditable cancer disorders. Li-Fraumeni syndrome is associated with early onset neoplasia and development of recurrent primary tumors. Its presence affects treatment decisions and methods of surveillance. Chemoradiation should be used judiciously in this population.

Otolaryngological manifestations of posttransplant lymphoproliferative disorder in pediatric thoracic transplant patients.

OBJECTIVE: To identify the otolaryngological manifestations of posttransplant lymphoproliferative disorder (PTLD) in pediatric heart and lung transplant recipients. METHODS: A 14-year retrospective case series review (1990-2003) of children less than 18 years of age presenting with PTLD after receiving orthotopic heart and lung transplants at St. Louis Children's hospital (St. Louis, MO, USA). RESULTS: One hundred ninety seven cardiac and 246 pulmonary transplant patients were included in this review. Thirteen heart transplant patients developed PTLD with 39% (5/13) presenting in the head and neck. Thirty-two lung transplant patients developed PTLD with 25% (8/32) presenting in the head and neck. PTLD in cardiac transplants most often presented as adenotonsillar hypertrophy or cervical lymphadenopathy. The sinonasal cavity was the most common site of head and neck PTLD in pulmonary transplants patients. CONCLUSIONS: This study suggests that head and neck involvement is more common than currently reported in the literature. As thoracic organ transplants become more frequent in children, otolaryngologists caring for this population should maintain a high index of suspicion for any suggestive findings. Evaluation must include tissue biopsy, as histopathologic examination is required for diagnosis.

Sinonasal posttransplant lymphoproliferative disorder in pediatric lung transplant patients.

OBJECTIVE: To evaluate sinonasal manifestations of posttransplant lymphoproliferative disorder (PTLD) in the pediatric lung transplant population. STUDY DESIGN AND METHODS: Case series of children less than 18 years presenting with PTLD after pulmonary transplantation at St Louis Children's Hospital between Jan 1, 1990 and Dec 31, 2003. RESULTS: Two hundred eighty-two lung transplants were performed in 246 children. Thirty-two cases of histopathologically confirmed PTLD were identified with 8 (25%) presenting in the head and neck. Sinonasal PTLD was the most common site of head and neck involvement (63%), with 40% of patients presenting with occult disease. All patients with sinonasal PTLD had longstanding nasal polyposis related to cystic fibrosis (P = 0.07). CONCLUSIONS: This is, to our knowledge, the first report identifying an increased frequency of sinonasal PTLD after pediatric pulmonary transplantation, particularly in children with cystic fibrosis and associated nasal polyposis. Because sinonasal PTLD may be asymptomatic, this region should receive close scrutiny on surveillance evaluations.

Otologic and audiologic features of Nager acrofacial dysostosis.

OBJECTIVE: To describe the otologic and audiologic characteristics of pediatric patients with Nager acrofacial dysostosis. DESIGN: Retrospective case series. SETTING: Multidisciplinary clinic in a tertiary care children's hospital. SUBJECTS: Patients less than 18 years of age with Nager acrofacial dysostosis. METHODS: Nager syndrome is a mandibulofacial dysostosis associated with preaxial limb abnormalities and multiple craniofacial anomalies. Ten patients with Nager syndrome were reviewed. Relevant literature, 1966 to the present, was reviewed with the assistance of Medline. RESULTS: External and middle ear abnormalities are common in Nager syndrome. All non-atretic ears had significant difficulty with otitis media, requiring an average of two sets of tympanostomy tubes. Cholesteatoma was diagnosed in one patient. Pure conductive hearing loss was identified in eight patients with mixed hearing loss noted in two patients. Conductive hearing loss greater than 30 dB HL was noted in 90% (9/10) of patients, with 40% (4/10) having 55-70 dB HL loss. Although amplification was effective, results of surgical interventions to correct conductive hearing loss were inconsistent. Two patients with mixed hearing loss developed the sensorineural component in later childhood, indicating that progressive or fluctuating sensorineural hearing loss is also possible in this population. CONCLUSIONS: Pediatric patients with Nager acrofacial dysostosis exhibit conductive hearing loss due to middle and external ear pathology. Prolonged ventilation of the middle ear via tympanostomy tubes and amplification with hearing aids are often required. Some patients also demonstrate mixed hearing loss that may be progressive and should be monitored carefully. Early and aggressive management in a multidisciplinary team approach is recommended.