Treatment practices and response in kaposiform hemangioendothelioma: A multicenter cohort study.

BACKGROUND AND OBJECTIVES: Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare vascular tumors in children historically associated with significant morbidity and mortality. This study was conducted to determine first-line therapy in the absence of available prospective clinical trials. METHODS: Patients from 17 institutions diagnosed with KHE/TA between 2005 and 2020 with more than 6 months of follow-up were included. Response rates to sirolimus and vincristine were compared at 3 and 6 months. Durability of response and response to other treatment modalities were also evaluated. RESULTS: Of 159 unique KHE/TA subjects, Kasabach-Merritt phenomenon (KMP) was present in 64 (40.3%), and only two patients were deceased (1.3%). Over 60% (n = 96) demonstrated treatment response at 3 months, and more than 70% (n = 114) by 6 months (no significant difference across groups). The vincristine group had higher radiologic response at 3 months compared to sirolimus (72.7% vs. 20%, p = .03), but there were no differences between these groups at 6 months. There were no differences in rates of recurrent or progressive disease between vincristine and sirolimus. CONCLUSIONS: In this large, multicenter cohort of 159 patients with KHE/TA, rates of KMP were consistent with historical literature, but the mortality rate (1.3%) was much lower. Overall treatment response rates were high (>70%), and there was no significant difference in treatment response or durability of disease comparing sirolimus to vincristine. Our results support individualized treatment decision plans depending on clinical scenario and patient/physician preferences. Response criteria and response rates reported here will be useful for guiding future treatment protocols for vascular tumors.

Foreign Body in the Bronchus Intermedius: Inadvertent Deployment of a Bravo Wireless pH Probe in the Airway.

The Bravo pH probe is a wireless capsule allowing remote quantification of gastroesophageal reflux. A 14-year-old male presented for Bravo probe placement. Following esophagogastroduodenoscopy, attachment of the Bravo probe was attempted. Immediately, the patient began coughing without oxygen desaturation. Repeat endoscopy did not reveal the probe within the esophagus or stomach. He was then intubated, and fluoroscopy demonstrated a foreign body within the bronchus intermedius. Rigid bronchoscopy was performed to retrieve the probe using optical forceps. This is the first case of pediatric inadvertent airway deployment requiring retrieval. We recommend endoscopic visualization of the delivery catheter entering the cricopharyngeus before Bravo probe deployment, then followed by repeat endoscopy to confirm position of the probe after attachment.

Asthma control in normal weight and overweight/obese asthmatic children following adenotonsillectomy.

OBJECTIVES: Childhood adenotonsillar hypertrophy (ATH) with sleep-disordered breathing (SDB) frequently occurs concomitant with asthma. Adenotonsillectomy and reduction in asthma severity association has been reported. We describe changes in asthma control in nonobese or normal weight and obese/overweight children undergoing adenotonsillectomy for SDB. METHODS: This prospective, nonrandomized cohort trial with 6-month follow-up at a tertiary children's hospital enrolled 41 children with persistent asthma undergoing adenotonsillectomy for SDB. Children with significant chronic medical conditions, premature birth (< 28 weeks), or recent respiratory infection were excluded. Patients were stratified by baseline BMI into nonobese or normal weight (BMI < 85 percentile) and obese/overweight (BMI > 85%). The primary outcome was change in Childhood Asthma Control Test (cACT) scores 3 and 6 months following adenotonsillectomy. Secondary outcome examined improvement in Pediatric Asthma Caregiver's Quality of Life Questionnaire (PACQLQ) 3 and 6 months following adenotonsillectomy. RESULTS: Baseline characteristics were similar except for anthropometric measures and mean PACQLQ (P = .03). Children with nonobese or normal weight (n = 26) had statistically significant improvement in change in cACT at 3 (22.80 ± 2.33 vs. 17.86 ± 3.53, P < .001) and 6 (20.71±3.29 vs. 18.24 ± 4.16, P = .044) months compared with baseline. PACQLQ scores also improved at 3 (6.20 ± 0.87 vs. 4.56 ± 1.12, P < .001) and 6 (6.36 ± 0.72 vs. 4.93 ± 0.96, P < .001) months. Obese/overweight children (n = 10) had significant improvement in cACT scores at 6 months (20.00 ± 3.90 vs. 15.00 ± 6.90, P = .048). Change of cACT scores at 3 months (17.86 ± 3.53 vs. 14.86 ± 6.31, P = .272) was not significantly different. PACQLQ scores improved at 3 (5.47 ± 1.09 vs. 3.70 ± 0.85, P < .001) and 6 (5.75 ± 2.19 vs. 3.67 ± 1.04, P = .016) months. CONCLUSION: Nonobese or normal-weight children undergoing adenotonsillectomy demonstrated significant improvement in asthma control scores at 3 and 6 and obese/overweight children at 6 months. Using the PACQLQ, caregiver quality of life improved for all children at 3 and 6 months. Surgical management of ATH in children with comorbid SBD and asthma is a good treatment option.

Systematic Review and Meta-analysis of the Change in Pharyngeal Bacterial Cultures After Pediatric Tonsillectomy.

OBJECTIVE: To review all available biomedical literature to assess published data regarding the effect of pediatric tonsillectomy on the culture results of potentially pathogenic respiratory pharyngeal bacteria before and after surgery. DATA SOURCES: Biomedical literature databases (PubMed, Embase, Web of Science) from January 1970 to December 2019. REVIEW METHODS: A systematic review of the literature was performed with the assistance of a medical librarian. Inclusion criteria consisted of pediatric patients and extractable data regarding respiratory bacteria culture data before and after tonsillectomy. Meta-analysis with random effects modeling was used on a limited basis. RESULTS: Only 5 studies met the inclusion criteria. The grand mean age was 5.9 years; the sample size range was 31 to 134; and the range of follow-up was 1 to 12 months. Group A beta hemolytic Streptococcus was generally the least commonly cultured pathogenic bacteria on preoperative cultures. Qualitative culture data generally showed an overall decrease in potentially pathogenic bacteria and some increase in nonpathologic respiratory flora after tonsillectomy. Meta-analysis showed significant reductions in postoperative culture rates for group A beta hemolytic Streptococcus (positive post- vs preoperative culture: risk ratio [RR], 0.144; 95% CI, 0-0.342), Haemophilus influenzae (RR, 0.437; 95% CI, 0.266-0.608), and Streptococcus pneumoniae (RR, 0.268; 95% CI, 0-0.567) and mixed results for Moraxella catarrhalis (0.736; 95% CI, 0.446-1.03) but no significant reduction for Staphylococcus aureus (RR, 0.774; 95% CI, 0.157-1.39). CONCLUSION: The majority of published evidence shows that pediatric tonsillectomy appears to reduce the quantity of most cultured potentially pathogenic respiratory bacteria in the pharynx after surgery. The implications and possible benefits of this favorable change in the microbiologic environment after surgery require further study.

Neonatal airway obstruction due to a massive lingual foregut duplication cyst.

Foregut duplication cysts (FDCs) are rare malformations arising along primitively derived alimentary tract. Head and neck cases comprise 0.3% of all FDCs with 60% occurring in the oral cavity. We present a case of neonatal airway obstruction secondary to a prenatally diagnosed massive lingual FDC. Definitive treatment requires surgical excision. Histologically, the cysts are lined gastric and respiratory epithelium. FDC should be a consideration in prenatally diagnosed masses affecting the oral cavity.

Massive infantile myofibromatosis of the upper lip causing airway distress in a newborn.

Infantile myofibromatosis is a rare condition characterized by benign spindle cell tumors most commonly involving the head, neck, and chest. An infant female with a prenatal diagnosis of a large facial mass was delivered via Cesarean at 34 weeks. Sparse prenatal care was received. Following delivery, the neonate was found to have an 8 cm ulcerative mass involving the upper lip and philtrum. Respiratory distress developed, and mask ventilation was difficult secondary to the size of the mass. The patient was successfully intubated after numerous attempts and then transferred to the children's hospital. Additional imaging demonstrated similar masses within bilateral iliopsoas and gluteal muscles, and her right gastrocnemius. A biopsy confirmed infantile myofibromatosis. At two weeks of life, she underwent resection with bilateral myocutaneous advancement flaps and successful extubation. She received adjuvant vinblastine and methotrexate for her pelvic and extremity disease with excellent response. We present the first case of airway distress secondary to myocutaneous myofibromatosis.

Neuromuscular electrical stimulation improves feeding and aspiration status in medically complex children undergoing feeding therapy.

OBJECTIVES: Neuromuscular electrical stimulation (NMES) therapy in the head and neck has been effectively used to rehabilitate swallowing in adult patients with acquired dysphagia. Limited data is available for the effectiveness of NMES in medically complex children with dysphagia and aspiration. This study intends to determine the efficacy of NMES as a therapeutic adjunct to improve aspiration and feeding status in medically complex children with severe dysphagia undergoing comprehensive speech and swallow therapy. STUDY DESIGN: Case series. SETTING: Tertiary children's hospital. SUBJECTS: and Methods: Medically complex children undergoing treatment for dysphagia using NMES during a three year period were included. Duration of treatment routinely ranged from 20 to 26 weeks. Demographic information, pre and post-NMES treatment aspiration status via modified barium swallow (MBS), and pre and post-NMES treatment feeding status were examined. RESULTS: 15 medically complex children underwent NMES as a component of their feeding therapy. The mean age was 2.51 ±â€¯3.20 years. Mean pre-treatment Functional Oral Intake Scale(FOIS) was 3.07 ±â€¯1.94. Following NMES, FOIS improved to 4.47 ±â€¯2.26 (p < 0.0001). Fourteen patients were evaluated with MBS prior to NMES. Pre-treatment aspiration and penetration were noted in 10 and 2 patients respectively. Two patients did not aspirate on pre-treatment MBS. Improvement was noted in 9 of 11 children with post-treatment MBS. CONCLUSION: NMES was safely completed in all children without complication. Improvement in aspiration status was seen in 83.3% of patients with pre- and post-treatment MBS. Feeding status as measured by textures was advanced in all patients with a significant improvement in FOIS. Future prospective studies are required to investigate the specific role in children with acquired and congenital dysphagia.

Endoscopic cricopharyngeal myotomy in infants.

Cricopharyngeal achalasia is a rare cause of dysphagia in young children. Conservative treatments involve medical therapy and serial dilations or injections. Definitive cure can be achieved with cricopharyngeal myotomy. We report two cases of young children, each less than one year of age, who underwent endoscopic laser-assisted cricopharyngeal myotomy. Following surgery, both infants were able to advance to age appropriate diets prior to discharge. Post-operative modified barium swallow demonstrated complete resolution of achalasia in both patients. To the best of our knowledge, this is the first case series of infants undergoing successful endoscopic cricopharyngeal myotomy.

Is inpatient admission necessary following removal of airway foreign bodies?

OBJECTIVE: To determine the need for postoperative admission following airway foreign body retrieval by examining the preoperative presentation, operative details, and postoperative recovery. INTRODUCTION: Inpatient admission following foreign body removal is common, however little evidence supports this practice. In the era of cost containment and prudent utilization of hospital resources, careful examination of the postoperative course following airway foreign body removal is required. METHODS: A retrospective review of cases over a four year period from a pediatric tertiary care pediatric hospital was performed. All children presenting with concerns for airway foreign bodies were included. Children without identification of an airway foreign body during bronchoscopy were excluded. Microlaryngoscopy and bronchoscopy with airway foreign body extraction was performed. Details concerning demographics, operative findings, and pre and postoperative course including pulse oximetry were collected. All respiratory events, intubations, and persistent oxygen requirements were investigated. RESULTS: Thirty five children underwent successful airway foreign body removal. The mean age was 3.2±2.6 years with a preponderance of males (68.6%). The retrieved items included: nuts (31.4%), popcorn (14.3%), seeds (8.6%), and inorganic materials (34.3%). Mean operative time was 29.7±25.6min. The mean length of stay following surgery was 1.3±1.9 days. Most patients (31/35) (88.6%) were extubated prior to transfer to recovery. 30/35 (85.7%) patients required no supplemental oxygen without desaturations following post anesthesia care unit (PACU) recovery. One patient developed laryngospasm requiring reintubation within 15min of surgery. Two patients were intubated prior to transfer for respiratory distress and remained intubated following surgery. Two patients breathing spontaneously prior to surgery were left intubated following surgery secondary to prolonged pneumonia treatment or multifocal foreign bodies with airway edema. All patients subsequently extubated without complication. In total, 30/31 (96.7%) of patients extubated in the operating room returned to room air oxygenation within 2h of surgery. CONCLUSIONS: PACU observation and discharge is feasible in select children following airway foreign body extraction. Patients carefully monitored in the recovery unit without oxygen requirement are candidates for discharge. Inpatient monitoring is advised in 'children with preoperative respiratory distress or a complicated operative course.

CT measured normative cartilage growth in children requiring costochondral grafting.

OBJECTIVE: Careful operative timing is required for children undergoing microtia repair using autologous costochondral grafting. This operation is performed as early as age 6 in efforts to treat children before school matriculation while allowing for sufficient rib growth. There remains a paucity of data regarding cartilaginous growth of the ribs and synchondrosis routinely harvested during microtia repair. This study employs CT imaging to generate normative costochondral growth characteristics in children. STUDY DESIGN: A population-based study was performed. SETTING: Tertiary care children's hospital. SUBJECTS AND METHODS: Chest CTs were reviewed in 360 children ages 3 to 20 years. Measurements included: length of ribs 6, 7, and 8 and the height and width of the synchondrosis between ribs 6 and 7. Growth charts are presented for gender and laterality. RESULTS: At age 6: ribs 6, 7, and 8 measure 5.96 ± 0.69, 7.79 ± 0.84, and 6.33 ± 1.01 cm, respectively. In adulthood the mean length of ribs 6, 7, and 8 are 8.29 ± 1.00, 11.10 ± 1.19, and 8.95 ± 1.99 cm, respectively. The vertical height of the synchondrosis at years 6 and 20 are 2.42 ± 0.39 and 3.59 ± 0.53 cm, respectively. Ribs 6, 7, and 8 as well as the synchondrosis grow in a nearly linear fashion. CONCLUSIONS: Cartilaginous growth of ribs 6 to 8 during early childhood is nearly linear. Synchondrosis height approaches adult auricle width at 8 years. Rib size is consistently larger in males and on the left side. These data are useful for the pediatric otolaryngologist and facial plastics and reconstructive surgeon performing microtia surgery.

Adenoid ciliostimulation in children with chronic otitis media.

OBJECTIVE: Adenoid hypertrophy and chronic adenoiditis are associated with an increased incidence of chronic otitis media. This study intends to determine the relationship between chronic otitis media and dynamic ciliary beat frequency in children undergoing adenoidectomy. STUDY DESIGN: Prospective, controlled study. SETTING: Pediatric tertiary care hospital. SUBJECTS AND METHODS: Children undergoing adenoidectomy were enrolled. Patients were stratified according to their indication for surgery, including adenotonsillar hypertrophy with obstructive sleep apnea, chronic otitis media with effusion, or recurrent episodes of acute otitis media. Adenoids were harvested using the curette. Tissue was sectioned and allowed to equilibrate in basal media for 24 hours. Cilia-bearing tissue was then stimulated using isoproterenol or methacholine. Ciliary beat frequency was serially reordered and analyzed using the Sisson-Ammons Video Analysis software program. RESULTS: Baseline ciliary beat frequency was similar in all groups (N = 47, total). Using isoproterenol, children with chronic otitis media with effusion demonstrated a blunted dynamic ciliary response at 2 and 3 hours relative to control (P = .0176 and P = .0282). Methacholine-stimulated ciliary beat frequency was not different between each group. CONCLUSION: At 2 and 3 hours following isoproterenol stimulation, there was a significant blunting of dynamic ciliary beat frequency in children with chronic otitis media with effusion. This ciliary dysfunction may provide a physiological explanation related to chronic adenoiditis in children with chronic otitis media.

Radiotherapy in parotid acinic cell carcinoma: does it have an impact on survival?

OBJECTIVE: Acinic (or acinar) cell carcinoma (ACC) represents approximately 10% of salivary gland malignant tumors and most commonly occurs in the parotid gland. It carries a propensity for locoregional and distant metastasis. Although it is selectively used as an adjuvant in this tumor, radiotherapy (RT) has not been sufficiently examined in large population studies for survival impact. DESIGN: Retrospective database review. SETTING: Tertiary care center. PATIENTS: A total of 1241 cases of parotid ACC in the Surveillance, Epidemiology, and End Results (SEER) Program database from 1988 to 2007 were identified and analyzed. INTERVENTIONS: Comparison groups were surgery and surgery plus RT. Kaplan-Meier survival curves were generated for oncologic stage and histologic grade. MAIN OUTCOME MEASURES: Overall survival. RESULTS: A total of 969 patients had sufficient staging data for inclusion. When comparing surgery with surgery with adjuvant RT, there was no statistical difference in overall survival when stratifying for stage I (P = .57), stage II (P = .37), stage III (P = .25), and stage IV (P = .24) tumors. Similarly, adjuvant RT did not demonstrate a survival advantage when stratified by histologic grade of tumor. The highest-grade and highest-stage tumors were fewer in number, however. CONCLUSIONS: To our knowledge, this study represents the largest cohort of patients treated for ACC of the parotid. Adjuvant RT does not seem to provide a significant survival advantage for early-stage or lower-grade parotid ACC. Radiotherapy for highest-stage and highest-grade tumors requires further study.

Cigarette smoke inhibits dynamic ciliary beat frequency in pediatric adenoid explants.

OBJECTIVE: Environmental tobacco smoke exposure in children increases the incidence of upper respiratory infections, chronic sinusitis, and chronic otitis media. This study investigated the effects of ex vivo and in vitro smoke exposure on dynamic ciliary beat frequency (CBF) in pediatric adenoid explants. STUDY DESIGN: Blinded and controlled prospective study. SETTING: Tertiary care pediatric hospital. SUBJECTS AND METHODS: Fifty-five children undergoing adenoidectomy for obstructive sleep apnea and adenotonsillar hypertrophy were enrolled in this study. Adenoids were surgically removed using currettage. Hair was collected for nicotine analysis. Tissue was sectioned into 1-mm strips and allowed to equilibrate in DMEM/F12 with 2% fetal bovine serum for 24 hours. Cilia-bearing explant tissues were treated with either DMEM/F12 media, 5% cigarette smoke extract (CSE), or 10% CSE for 24 hours. Cilia were then stimulated using either isoproterenol (10(-9) M) or methacholine (10(-6)M), and CBF was serially recorded using the Sisson-Ammons Video Analysis (SAVA) software. RESULTS: Children with hair nicotine levels ≥ 1 ng/mg consistent with secondhand smoke exposure display blunted dynamic CBF response ex vivo. Explants incubated with CSE in vitro demonstrate significant impairment of isoproterenol and methacholine-induced CBF. CONCLUSION: CBF of adenoid explants increases when stimulated with isoproterenol and methacholine. Ex vivo and in vitro smoke exposure blunted ciliostimulation of CBF in adenoid explants. Smoke exposure impairs ciliary function in the pediatric airway and could potentially contribute to disorders such as chronic rhinosinusitis and chronic otitis media.

Feeding status after pediatric laryngotracheal reconstruction.

OBJECTIVE: To determine the influence of pediatric laryngotracheal reconstruction (LTR) on postoperative feeding status and longitudinal weight gain after surgery. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care pediatric hospital. SUBJECTS AND METHODS: We identified 30 consecutive pediatric patients undergoing LTR from November 2005 to October 2008. Demographics, stenosis grade, surgical procedure, decannulation status, preoperative feeding status and weight, discharge feeding status, and weights at follow-up were collected. Weights were plotted on standardized growth charts at surgery, during the early postoperative period (1-3 months), and during the late postoperative period (10-14 months). Growth percentiles were compared by the use of Wilcoxon signed rank test. RESULTS: Twenty-eight patients (97%) maintained or advanced their feeding status after LTR. Twenty-one patients (72%) were oral feeders at surgery. All of these patients continued the same oral diet postoperatively. Five patients (17%) were dependent on gastrostomy before and after surgery. Three patients (10%) were fed via naso- or orogastric tubes at surgery. Two of these patients were discharged on an oral diet, and one required a gastrostomy tube. The median growth percentiles at the time of surgery, early postoperative, and late postoperative periods were nine, 18, and 32, respectively. Differences between percentiles at the time of surgery compared with early (P = 0.081) and late follow-up (P = 0.074) were not significant. In patients who were not dependent on gastrostomy, there was a significant increase in growth percentile at early follow-up (P = 0.026). CONCLUSION: The performance of LTR does not influence feeding status. An early increase in median growth percentile is observed in oral feeders, but overall long-term median growth percentiles remain stable after LTR.