Management of first branchial anomalies in children: 20 years of experience.

PURPOSE: Branchial cleft anomalies (BCAs) are common pediatric head and neck lesions; however, only 1-4% involve the first branchial cleft. The rare occurrence of first BCAs, their presentation at a young age, and the possible facial nerve involvement make diagnosis and treatment challenging. METHODS: A retrospective chart review was conducted for children diagnosed with their first BCA between 2000 and 2020. Data on demographics, presenting symptoms, physical findings, imaging features, previous surgery, and treatment outcomes were collected and analyzed. RESULTS: The cohort included 17 patients with a median age of 5 years at presentation. Seven (41%) had undergone previous surgical intervention before definitive surgery. Eight were classified as Work Type II anomalies, and nine as Work Type I. Sixteen patients (94%) underwent definitive surgical excision at a median age of 6.9. A parotid approach was used in 10 (62%), with dissection of the mass from the facial nerve, and a retro-auricular or end-aural approach was used in 6 (38%). Complete excision was achieved in 14/16 patients (88%). Three patients had transient facial nerve paresis postoperatively. Recurrence was noted in 3/16 patients (18%). Enhancement in imaging was positively correlated with post-operative complications (R = 0.463, P = 0.018). CONCLUSIONS: First, BCA poses a diagnostic and surgical challenge; thus, definitive surgical treatment is often delayed. The surgical approach should be tailored to the type of anomaly (Work type I or II) and possible facial nerve involvement. Risk factors for post-operative complications are a history of recurrent infections and previous surgical interventions. The presence of contrast enhancement in preoperative imaging should alert surgeons to perioperative challenges and the risk of post-operative complications.

Predictors of residual disease following pediatric thyroglossal duct cyst excision.

OBJECTIVES: Residual thyroglossal duct cyst (TGDC) following surgical excision is not uncommon. This study aimed to search for risk factors for residual disease that either required revision surgery or were resolved with only conservative treatment and follow up. METHODS: A retrospective study of consecutive children who underwent surgical excision for thyroglossal duct cysts between 2008 and 2021 at Schneider Children's Medical Center of Israel, a tertiary referral center in Israel. RESULTS: Out of 102 children, 54 (53 %) had an uneventful recovery, 32 (31 %) had post-operative complications which were managed without revision surgery, and 16 (16 %) underwent revision surgery. A comparison of the three groups showed that children who had early post-operative complications (up to one month) were more likely to respond to conservative treatment (57 %). In contrast children with late complications had a higher probability (59 %) of undergoing revision surgery. The presence of a pre-operative cutaneous fistula was significantly associated with revision surgery (p = 0.012). In addition, children with no prior history of neck infection were more likely to have an uneventful recovery (p = 0.005). CONCLUSIONS: TGDC disease has a wide range of clinical presentations both before and after surgery. A significant percentage of children with persistent post-operative symptoms may resolve without revision surgery. The presence of a pre-operative cutaneous fistula and late post-operative complications are the main risk factors for revision surgery.

Intranasal foreign bodies: A 10-year analysis of a large cohort, in a tertiary medical center.

BACKGROUND: Nasal foreign bodies (NFB) are commonly seen in pediatric patients seeking medical attention in the emergency department (ED). We aim to describe the occurrence, clinical presentation and management, of these cases, and to assess various risk factors for complications. METHODS: A retrospective analysis of a computerized patient directory of 562 children admitted to the emergency department during a 10-year period, with NFB, in a tertiary pediatric hospital. RESULTS: Upon admittance, most of the children (82%) were asymptomatic. Among the symptomatic children (18%), the primary symptoms were nasal discharge (10%), epistaxis (8%) and pain (4%). Younger children (under 4 years) were more likely to insert organic materials, compared to older children. Younger children were also admitted sooner to the emergency department and were more likely to present with nasal discharge. The overall complication rate was 5%. None of the children had aspirated the foreign body. Complications included infection (2%), necrosis (0.7%), septal perforation (0.5%), deep mucosal laceration (1.5%) and loss of foreign body (1.9%). Significantly higher rates of symptoms and complications were associated with button batteries. Increased risk for complications were observed according to type of foreign body, multiple attempts to remove it, posterior insertion and left-side insertion. CONCLUSIONS: Nasal foreign bodies in children are common. Mostly, patients are asymptomatic, therefore a high index of suspicion is required, for quick diagnosis and safe removal, without complications.

Acute mastoiditis in infants younger than 6 months: is an alternative treatment protocol needed?

OBJECTIVES: Several studies have reported that younger children suffer from increased incidence and more severe episodes of acute mastoiditis (AM) than older children, whereas other researchers have found the opposite. The aim of our study was to describe the occurrence, clinical, and microbiological aspects of AM in children 6 months or younger, and compare them with the results in an older reference age band. METHODS: The medical files of children hospitalized with a diagnosis of AM during 2001-2016 were retrospectively reviewed. Diagnosis of AM was based on the presence of clinical signs of acute otitis media (AOM) accompanied by two or more of the following: auricle protrusion, retro-auricular erythema, swelling, and local tenderness. Children were divided into two age bands, 6 months or younger, and older than 6 months. RESULTS: Fifty patients in the young age band and 335 in the older reference age band were included. Bilateral AOM was identified in 14 (28%) children under 6 months, and 50 (14.9%) in the reference age band (p < 0.001). Fever, mean WBC, and CRP values were similar in both age bands; 4 (8%) children under 6 months had pre-auricular/zygomatic area swelling, as compared to 1(0.02%) in the reference age band (p < 0.001). Complication rates (subperiosteal abscess, sinus vein thrombosis, and epidural abscess) were similar in both age bands. All children were treated with myringotomy and IV antibiotics. Cortical mastoidectomy with the insertion of ventilation tube was performed in 10 (20%) children in the younger age band and 58(17.3%) in the reference age band. Streptococcus pneumoniae was the most common (38%) isolated bacteria in the younger age band, and Group A streptococcous (GAS) (20.6%) in the reference age band. CONCLUSIONS: AM in children 6 months or younger has similar presentation and characteristics as in older children. Pre-auricular swelling and bilateral AOM are more typical in the younger age band; Streptococcus pneumoniae is the most common pathogen in the younger children. We suggest that the treatment approach should be the same for both groups.

Postoperative Intracochlear Electrocochleography in Pediatric Cochlear Implant Recipients: Association to Audiometric Thresholds and Auditory Performance.

OBJECTIVES: The aim of this study was to compare intracochlear-recorded cochlear microphonics (CM) responses to behavioral audiometry thresholds in young children, with congenital hearing loss, 2 to 5 years after cochlear implantation early in life. In addition, differences in speech and auditory outcomes were assessed among children with and without residual hearing. DESIGN: The study was conducted at a tertiary, university-affiliated, pediatric medical center. CM responses by intracochlear electrocochleography technique were recorded from 102 implanted ears of 60 children and those responses correlated to behavioral audiometry thresholds at 0.125 to 2 kHz frequencies. All children had received Advanced Bionics cochlear implant with High Focus J1 or MidScala electrodes, along with extensive auditory rehabilitation before and after implantation, including the use of conventional hearing aids. Speech Spatial and Hearing Qualities, Category of Auditory Performance scale, and educational settings information were obtained for each participant. Those cochlear implantation (CI) outcomes were compared between children with or without residual CM responses. RESULTS: Two distinctive CM responses patterns were found among the implanted children. Of all ears diagnosed with cochlear hearing loss (n = 88), only in 29 ears, clear CM responses were obtained. In all other ears, no CM responses were obtained at the maximum output levels. The CM responses were highly correlated with coefficients ranging from 0.7 to 0.83 for the audiometric behavioral thresholds at 0.125 to 2 kHz frequency range. Of all ears diagnosed with auditory neuropathy syndrome disorder (n = 14), eight ears had residual hearing and recordable CM postimplantation. The other six ears showed no recordable CM responses at maximum output levels for all tested frequencies. Those showed supposedly better CM responses than the behavioral audiometry threshold, however with poor correlations with tested frequency thresholds. Children with residual hearing showed significantly better auditory outcomes with CI, compared with those without residual hearing. CONCLUSIONS: In children with congenital cochlear hearing loss, the objective CM intracochlear responses can reliably predict the residual audiometric threshold. However, in children with auditory neuropathy syndrome disorder, the CM thresholds did not match the behavioral audiometric responses. Postoperatively, children with recordable CM responses, showing preserved residual hearing, demonstrated better CI outcomes.

[COCHLEAR IMPLANTS IN SINGLE-SIDED DEAFNESS].

INTRODUCTION: In normal hearing, the brain receives bilateral auditory input from both ears. In individuals with only one functioning ear listening in noisy environments and sound localization may become difficult. Historically, the impact of unilateral hearing loss in children had typically been minimized by clinicians, as it was assumed that one normal hearing ear provided sufficient auditory input for speech development and normal hearing experience. Data supporting the negative effects of unilateral deafness has been accumulating during the last decades. The effects of unilateral deafness extend beyond spatial hearing to language development, slower rates of educational progress, problems in social interaction and in cognitively demanding tasks. Until recently, treatments for single sided deafness were limited to routing signals from the deaf ear to the contralateral hearing ear either through conventional CROS aids or through bone anchored technologies. These technologies simply transfer sounds to the single functioning ear which allow sound awareness from the deaf side and minor improvement in hearing in noisy environments and localization. The cochlear implant is a surgically implanted electronic device that contains an array of electrodes which is placed into the cochlea, and stimulates the cochlear nerve. The cochlear implant bypasses the injured parts of the inner ear. Currently it is the only treatment to restore binaural hearing. This review aims to discuss the different aspects, the benefits and disadvantages of cochlear implantation in children with single sided deafness.

Surgical intervention for acute mastoiditis: 10 years experience in a tertiary children hospital.

PURPOSE: To evaluate the clinical course of children with acute mastoiditis (AM) who required surgical intervention. MATERIAL AND METHODS: Clinical and biochemical characteristics at the moment of hospital admission were reviewed for patients who required surgery for AM. Children who were successfully managed conservatively during the last 3 years of study were chosen as a comparison group. RESULTS: During 2008-2017, 570 children were admitted with AM: 82(14%) underwent cortical mastoidectomy, including 31(38%) with decompression of epidural space and sigmoid sinus. The comparison group consisted of 167 children with AM who did not require surgery. The surgical group had a higher rate of acute otitis media before admission. At the time of hospital admission, the surgical group had a higher rate of prolonged fever, otorrhea, and sub-periosteal abscess. Their average temperature, WBC, neutrophil count, and CRP were significantly higher (39.2 vs. 37.9°. C, 20 K vs. 16.5 K, 67 vs. 55.8 percent, 17 vs. 8.8, respectively, p = 0.0001). Fusobacterium necrophorum was the most common pathogen in the surgical group (50%), and group A streptococcus in the comparison group (22%). Sub-periosteal abscess, sinus venous thrombosis, and epidural involvement were diagnosed in 95, 35, and 38 percent of patients, respectively. Average length of IV antibiotic treatment was 20 days in operated children, compared to 5.6 days in the comparison group (p = 0.0001). Since 2013, a significantly higher percentage of children were diagnosed with Fusobacterium mastoiditis (p = 0.0001) who required surgery (p = 0.008). CONCLUSION: In children with AM presenting with, high fever, leukocytosis, elevated CRP, and sub-periosteal abscess, early CT and surgical intervention were frequently required. The increase in Fusobacterium infection might be an explanation for the increase in complicated AM requiring surgery.

Cochlear Implantation and Social-Emotional Functioning of Children with Hearing Loss.

This study examined the contribution of cochlear implants (CIs) to the social-emotional functioning of children who are deaf or hard of hearing (dhh). Sixty-three parents of children who are dhh participated in the study. Thirty children were CI users and 32 used hearing aids (HAs). They completed the Strengths and Difficulties Questionnaire and a background questionnaire. Parents of children with CIs reported lower levels of hyperactivity/inattention and higher levels of pro-social behavior compared to parents of children with HAs. Additionally, older age when hearing loss was detected was related to more pro-social behavior, and age at implantation among CI users was negatively correlated with children's hyperactivity/inattention and conduct problems. These findings add to the existing knowledge about the many benefits of CIs for individuals with hearing loss and emphasize the possible impact of early implantation to children's social-emotional functioning.

Pediatric Cochlear implant soft failure.

PURPOSE: Hard cochlear implant failures are diagnosed by objective tests whereas soft failures are suspected on the basis of clinical signs and symptoms. This study reviews our experience with children in tertiary pediatric medical center who underwent revision cochlear implantation, with emphasis on soft failures. MATERIALS AND METHODS: Children (age<18years) who underwent revision cochlear implantation from 2000 to 2012 were identified by database search. Pre- and post-explantation data were collected. RESULTS: Twenty-six revision surgeries were performed, accounting for 7.4% of all cochlear implant surgeries at our center during the study period. The pre-explantation diagnosis was hard failure in 7 cases (27%), soft failure in 12 (46%), and medical failure in 7 (27%). On post-explantation analysis, 7/12 devices from the soft-failure group with a normal integrity test had abnormal findings, yielding a 63% false-negative rate (12/19) for the integrity test. All children regained their initial performance. Compared to hard failures, soft failures were associated with a shorter median time from first implantation to symptom onset (8 vs 25months) but a significantly longer time from symptom onset to revision surgery (17.5 vs 3months; P=0.004). CONCLUSIONS: Soft cochlear implant failure in young patients poses a diagnostic challenge. A high index of suspicion is important because a delayed diagnosis may have severe consequences for language development. A normal integrity test does not unequivocally exclude device failure and is unrelated to functional outcome after revision surgery. Better education of parents and rehabilitation teams is needed in addition to more accurate diagnostic tests.

Auditory Performance and Electrical Stimulation Measures in Cochlear Implant Recipients With Auditory Neuropathy Compared With Severe to Profound Sensorineural Hearing Loss.

OBJECTIVES: The aim of the study was to compare auditory and speech outcomes and electrical parameters on average 8 years after cochlear implantation between children with isolated auditory neuropathy (AN) and children with sensorineural hearing loss (SNHL). DESIGN: The study was conducted at a tertiary, university-affiliated pediatric medical center. The cohort included 16 patients with isolated AN with current age of 5 to 12.2 years who had been using a cochlear implant for at least 3.4 years and 16 control patients with SNHL matched for duration of deafness, age at implantation, type of implant, and unilateral/bilateral implant placement. All participants had had extensive auditory rehabilitation before and after implantation, including the use of conventional hearing aids. Most patients received Cochlear Nucleus devices, and the remainder either Med-El or Advanced Bionics devices. Unaided pure-tone audiograms were evaluated before and after implantation. Implantation outcomes were assessed by auditory and speech recognition tests in quiet and in noise. Data were also collected on the educational setting at 1 year after implantation and at school age. The electrical stimulation measures were evaluated only in the Cochlear Nucleus implant recipients in the two groups. Similar mapping and electrical measurement techniques were used in the two groups. Electrical thresholds, comfortable level, dynamic range, and objective neural response telemetry threshold were measured across the 22-electrode array in each patient. Main outcome measures were between-group differences in the following parameters: (1) Auditory and speech tests. (2) Residual hearing. (3) Electrical stimulation parameters. (4) Correlations of residual hearing at low frequencies with electrical thresholds at the basal, middle, and apical electrodes. RESULTS: The children with isolated AN performed equally well to the children with SNHL on auditory and speech recognition tests in both quiet and noise. More children in the AN group than the SNHL group were attending mainstream educational settings at school age, but the difference was not statistically significant. Significant between-group differences were noted in electrical measurements: the AN group was characterized by a lower current charge to reach subjective electrical thresholds, lower comfortable level and dynamic range, and lower telemetric neural response threshold. Based on pure-tone audiograms, the children with AN also had more residual hearing before and after implantation. Highly positive coefficients were found on correlation analysis between T levels across the basal and midcochlear electrodes and low-frequency acoustic thresholds. CONCLUSIONS: Prelingual children with isolated AN who fail to show expected oral and auditory progress after extensive rehabilitation with conventional hearing aids should be considered for cochlear implantation. Children with isolated AN had similar pattern as children with SNHL on auditory performance tests after cochlear implantation. The lower current charge required to evoke subjective and objective electrical thresholds in children with AN compared with children with SNHL may be attributed to the contribution to electrophonic hearing from the remaining neurons and hair cells. In addition, it is also possible that mechanical stimulation of the basilar membrane, as in acoustic stimulation, is added to the electrical stimulation of the cochlear implant.

Hearing loss patterns after cochlear implantation via the round window in an animal model.

PURPOSE: The mechanism and the type of hearing loss induced by cochlear implants are mostly unknown. Therefore, this study evaluated the impact and type of hearing loss induced by each stage of cochlear implantation surgery in an animal model. STUDY DESIGN: Original basic research animal study. SETTING: The study was conducted in a tertiary, university-affiliated medical center in accordance with the guidelines of the Institutional Animal Care and Use Committee. SUBJECTS AND METHODS: Cochlear implant electrode array was inserted via the round window membrane in 17 ears of 9 adult-size fat sand rats. In 7 ears of 5 additional animals round window incision only was performed, followed by patching with a small piece of periosteum (control). Hearing thresholds to air (AC) and bone conduction (BC), clicks, 1 kHz and 6 kHz tone bursts were measured by auditory brainstem evoked potential, before, during each stage of surgery and one week post-operatively. In addition, inner ear histology was performed. RESULTS: The degree of hearing loss increased significantly from baseline throughout the stages of cochlear implantation surgery and up to one week after (p<0.0001). In both operated groups, the greatest deterioration was noted after round window incision. Overall, threshold shift to air-conduction clicks, reached 61 dB SPL and the bone conduction threshold deteriorated by 19 dB SPL only. Similar losses were found for 1-kHz and 6-kHz frequencies. The hearing loss was not associated with significant changes in inner ear histology. CONCLUSIONS: Hearing loss following cochlear implantation in normal hearing animals is progressive and of mixed type, but mainly conductive. Changes in the inner-ear mechanism are most likely responsible for the conductive hearing loss.

Radiation-Induced Well-Differentiated Thyroid Cancer: Disease Characteristics and Survival.

BACKGROUND: Radiation exposure is a well-known risk factor for well-differentiated thyroid cancer (WDTC). However, disease characteristics, optimal treatment, time from exposure to disease appearance, and the effect of age at initial exposure on the outcome have yet to be determined. OBJECTIVES: To identify the characteristics of radiation-induced thyroid carcinoma. METHODS: We retrieved the charts of all patients previously exposed to radiation who were diagnosed with WDTC between the years 1985 and 2013 in a tertiary referral center. RESULTS: Forty-four patients were reviewed. Median time from radiation exposure to diagnosis was 23 years. These patients had higher rates of aerodigestive symptoms and distant metastases on presentation than seen in non-radiated patients. Patients who were exposed to radiation before age 15 years tended to develop the disease at a younger age but had a longer latency period (34.7 ± 15.3 vs. 16.3 ± 10 years, P < 0.001) and none had significantly higher rates of vocal cord palsy, hoarseness on presentation, or aggressive variants on histology compared to patients exposed to radiation at an older age. Disease-specific survival (DSS) was the same for both groups and were similar to that seen in the general population (95% 20 year DSS). CONCLUSIONS: Radiation-induced thyroid cancer has a more aggressive presentation and the age at exposure affects the presentation of disease. Nonetheless, appropriate treatment leads to a favorable prognosis.

Pattern of hearing loss following cochlear implantation.

Cochlear implantation is associated with deterioration in hearing. Despite the fact that the damage is presumed to be of sensory origin, residual hearing is usually assessed by air-conduction thresholds alone. This study sought to determine if surgery may cause changes in air- and bone-conduction thresholds producing a mixed-type hearing loss. The sample included 18 patients (mean age 37 years) with an air-bone gap of 10 dB over three consecutive frequencies and measurable masked and reliable bone-conduction thresholds of operated and non-operated ears who underwent cochlear implant surgery. All underwent comprehensive audiologic and otologic assessment and imaging before and after surgery. The air-bone gap in the treated ears was 17-41 dB preoperatively and 13-59 dB postoperatively over 250-4,000 Hz. Air-conduction thresholds in the treated ears significantly deteriorated after surgery, by a mean of 10-21 dB. Bone-conduction levels deteriorated nonsignificantly by 0.8-7.5 dB. The findings indicate that the increase in air-conduction threshold after cochlear implantation accounts for most of the postoperative increase in the air-bone gap. Changes in the mechanics of the inner ear may play an important role. Further studies in larger samples including objective measures of inner ear mechanics may add information on the source of the air-bone gap.

Long-term outcomes and electrophysiological measures of children with inner ear malformations and cochlear implants.

OBJECTIVES: To compared auditory and speech performance outcomes of children with cochlear implants (CI), between those with inner ear malformations (IEMs) and with normal ear anatomy; and to describe differences in electrophysiological measurements. STUDY DESIGN: A retrospective study. SETTING: A tertiary care pediatric medical center. PATIENTS: Forty-one children with IEMs who underwent CI during 2003-2017, and 41 age-matched CI recipients with normal ear anatomy (control group). MAIN OUTCOME MEASURES: Post-CI auditory performance outcomes including educational setting, Categories of Auditory Performance (CAP), and Speech Intelligibility Rating (SIR); and electrophysiological measurements, Including maximal comfortable electrical levels (CLs) and impedances along CI electrodes. RESULTS: The ANOVA on ranks revealed lower CAP scores in the study than control group: H3 = 18.8, P < 0.001. Among children with IEMs, CAP scores were better in children with enlarged vestibular aqueduct (EVA) (P < 0.04). SIR scores of the control group did not differ from those with isolated EVA; however, SIR scores of the IEMs without EVA subgroup were lower than all the other study subgroups (P < 0.01). The proportion of the control group that was integrated with full inclusion educational settings into the regular mainstream schools was higher than for those with IEMs without EVA (47 % vs. 15 %, P < 0.05), but similar to those with isolated EVA. For the study group versus control group, maximal comfortable electrical levels (CLs) were higher)P > 0.03) while impedance measurements were similar. CONCLUSIONS: Outcomes of pediatric recipients with normal anatomy were better than those with IEMs. Among pediatric recipients of CI with IEMs, auditory performance was better and CLs were lower among children with isolated EVA than all other IEM subgroups.

Discovery of a Novel Missense Variant in NLRP3 Causing Atypical Cryopyrin-Associated Periodic Syndromes With Hearing Loss as the Primary Presentation, Responsive to Anti-Interleukin-1 Therapy.

OBJECTIVE: Cryopyrin-associated periodic syndromes (CAPS), also known as NLRP3-associated autoinflammatory diseases, are a spectrum of rare autoinflammatory diseases caused by gain-of-function variants in the NLRP3 gene, resulting in inflammasome hyperactivation and dysregulated release of interleukin-1ß (IL-1ß). Many patients with CAPS develop progressive sensorineural hearing loss (SNHL) because of cochlear autoinflammation, which may be the sole manifestation in rare cases. This study was undertaken to establish the suspected diagnosis of CAPS in a family presenting with autosomal-dominant progressive/acute SNHL and a novel missense variant in the NLRP3 gene of unknown significance (NM_001079821.3:c.1784G>A p.Ser595Asn). METHODS: We conducted an ex vivo functional assessment of the NLRP3 inflammasome in heterozygous individuals (n = 10) and healthy family members (n = 5). RESULTS: The assay revealed hyperactivation of the inflammasome among heterozygous individuals, supporting the hypothesis that this missense variant is a pathogenic gain-of-function variant. Administration of IL-1 receptor antagonist resulted in a substantial clinical improvement among pediatric patients, who exhibited near resolution of hearing impairment within 1 to 3 months of treatment. CONCLUSION: Our findings highlight the crucial role of early diagnosis and treatment with an anti-IL-1 agent in reversing cochlear damage. Furthermore, our results suggest that high- and ultrahigh-frequency ranges need to be included in the auditory assessment to enable early detection of subclinical SNHL. Finally, incorporating functional inflammasome assessment as part of the clinical evaluation could establish the diagnosis in inconclusive cases.

Unilateral Choanal Atresia Presenting With Congenital Respiratory Distress and Recurrent Cyanotic Episodes.

Congenital unilateral choanal atresia (CA) is not considered an emergent condition and should not cause respiratory distress in the newborn. Therefore, surgical repair of unilateral CA is usually delayed. This description of a newborn with congenital unilateral CA that caused significant respiratory distress, recurrent cyanotic episodes, and severe feeding difficulties highlights an exception to that rule.

Hemangiomas of the nasal tip treated with propranolol.

BACKGROUND: Infantile hemangioma is the most common tumor of infancy. There are recent reports of the efficacy of propranolol in the treatment of these hemangiomas. Nasal tip hemangiomas pose a particularly sensitive concern aesthetically and functionally. The treatment of nasal tip hemangiomas is controversial. We assessed the effect of propranolol therapy in hemangiomas of the nasal tip. OBJECTIVES: To evaluate the response of nasal tip hemangiomas to systemic propranolol. METHODS: During 2008-2010 ten infant with nasal tip hemangiomas presented to our tertiary care center. All underwent comprehensive evaluation by a multidisciplinary team and were then treated with oral propranolol at 2 mg/kg/day, with continuous clinical follow-up until age 14-16 months, or in older infants until the proliferative phase resolved. RESULTS: Eight patients demonstrated good clinical improvement. Two patients had partial improvement. One patient discontinued treatment due to wheezing. Three patients had mild sleep disturbance which did not warrant discontinuation of treatment. No rebound was noticed after cessation of treatment. LIMITATIONS: Children presented by referral at variable ages. It is possible that routine initiation of propranolol in neonates at the first sign of nasal hemangioma may reduce the required treatment duration or dose. CONCLUSIONS: Early treatment of hemangiomas of the nasal tip with propranolol prevents lesion proliferation, reduces lesion volume, and prevents nasal and facial deformation. Propranolol appears to be a safe and effective treatment. Its efficacy and safety profiles, relative to other accepted therapies, suggest that it should be considered as the first-line treatment when intervention is required.

Post-operative MRI detection of residual cholesteatoma in pediatric patients - The yield of serial scans over a long follow-up.

OBJECTIVES: Non-echo-planar diffusion weighted magnetic resonance imaging (Non-EPI DWI MRI) is commonly used for follow-up after cholesteatoma surgery. MRI has a critical role in the evaluation of residual disease, where physical examination will commonly demonstrate an intact tympanic membrane. The aim of our study was to assess the timing of residual cholesteatoma identification on serial MRI scans and the yield of MRI follow up after canal wall up tympano-mastoidectomy. METHODS: A retrospective chart review of children that underwent canal wall up tympano-mastoidectomy due to cholesteatoma in Schneider Children's Medical Center during 2004-2016, and were followed up both clinically and with MRI. RESULTS: Seventy-seven children (89 ears) were included, who altogether underwent 166 surgeries (77 revisions). Average follow-up was 66 ± 34.4 months. During follow up, 244 scans were performed; 19 cases of residual disease were diagnosed by MRI and confirmed in surgery. The mean time from surgery and an MRI positive for residual disease was 29.7 ± 16 months (range: 10-66). In 9/19 cases (47%), at least one negative MRI preceded the scan positive for residual disease, and in 4 cases at least two initial scans were negative. CONCLUSIONS: MRI plays an important role in the diagnosis of residual disease after cholesteatoma surgery. In our cohort. Almost half of the cases diagnosed with residual disease had at least one negative scan prior to the positive one, emphasizing the importance of close radiological follow-up with serial scans after surgery.