Infantile Fibrosarcoma With Concurrent Infantile Hemangioma: A Case Report.

This report focuses on the clinical course and treatment of an infant male who had a progressively enlarging tongue mass initially thought to be an infantile hemangioma but was later found to be an infantile fibrosarcoma. Treatment included surgical excision with anticipated difficult mask ventilation with active rhino/enterovirus infection bronchiolitis and recent croup. Complete surgical excision is the mainstay of treatment, and the patient did have negative margins after complete surgical re-excision. The patient has surveillance MRI scans and remains without fibrosarcoma recurrence. This case report highlights complex pediatric airway management and the need for vigilance in healthcare when common presentations, such as infantile hemangioma, also present with a concurrent rare disease at a different anatomic location, such as infantile fibrosarcoma in this case.

Full-Thickness Oral Mucoperiosteal Defects: Challenges and Opportunities.

Regenerative engineering strategies for the oral mucoperiosteum, as may be needed following surgeries, such as cleft palate repair and tumor resection, are underdeveloped compared with those for maxillofacial bone. However, critical-size tissue defects left to heal by secondary intention can lead to complications, such as infection, fistula formation, scarring, and midface hypoplasia. This review describes current clinical practice for replacing mucoperiosteal tissue, including autografts and allografts. Potentially paradigm-shifting experimental regenerative engineering strategies for mucoperiosteal wound healing, such as hybrid grafts and engineered matrices, are also discussed. Throughout the review, the advantages and disadvantages of each replacement or regeneration strategy are outlined in the context of clinical outcomes, quality of life for the patient, availability of materials, and cost of care. Finally, future directions for research and development in the area of mucoperiosteum repair are proposed, with an emphasis on identifying globally available and affordable solutions for promoting mucoperiosteal regeneration. Impact statement Unassisted oral mucoperiosteal wound healing can lead to severe complications such as infection, fistulae, scarring, and developmental abnormalities. Thus, strategies for promoting wound healing must be considered when mucoperiosteal defects are incident to oral surgery, as in palatoplasty or tumor resection. Emerging mucoperiosteal tissue engineering strategies, described in this study, have the potential to overcome the limitations of current standard-of-care donor tissue grafts. For example, the use of engineered mucoperiosteal biomaterials could circumvent concerns about tissue availability and immunogenicity. Moreover, employment of tissue engineering strategies may improve the equity of oral wound care by increasing global affordability and accessibility of materials.

Adenoidectomy and persistent velopharyngeal insufficiency: Considerations, risk factors, and treatment.

IMPORTANCE: Persistent velopharyngeal insufficiency (VPI) is a rare but well-recognized complication of adenoidectomy. VPI can have a significant impact on the communication of a child. OBJECTIVE: To describe the pathophysiology of post-adenoidectomy VPI, identify its associated risk factors, and illustrate the techniques used to treat the entity. EVIDENCE REVIEW: A search of English or translated English articles concerning adenoidectomy, partial adenoidectomy, superior adenoidectomy; and velopharyngeal insufficiency, speech and voice from 1980 to 2021 was performed using Pubmed and Embase. Data from prospective and retrospective studies and their relevant references were pooled. RESULTS: By objective measures, hypernasality is noted in many pediatric patients post-adenoidectomy, but this typically resolves in 3-6 months. Risk factors for the development of post-adenoidectomy VPI include low birth weight, family history of hypernasality, and history of speech problems or nasal regurgitation. The cleft palate, submucous cleft palate, poor palate mobility, and a deep pharynx may indicate susceptibility to VPI. Speech therapy is successful in up to 50% of patients, while surgical intervention may be tailored based on the diagnostic evaluation of the velopharynx with videofluoroscopy or nasoendoscopy. CONCLUSION: We present a comprehensive review of the literature on the pathophysiology, risk factors, and treatment of post-adenoidectomy VPI. We hope to bring awareness to the factors that can lead to a rare but potentially devastating complication in one of the most common procedures performed by Otolaryngologists.

Rigid Video Laryngoscopy for Intubation in Severe Pierre Robin Sequence: A Retrospective Review.

OBJECTIVES/HYPOTHESIS: The anatomy of children with severe Pierre Robin sequence can present a challenge for direct laryngoscopy and intubation. Advanced techniques including flexible fiberoptic laryngoscopic intubation have been described but require highly specialized skill and equipment. Rigid video laryngoscopy is more accessible but has not been described in this population. STUDY DESIGN: Retrospective cohort study. METHODS: A retrospective review was completed at a tertiary care center of all children between January 2016 and March 2020 with Pierre Robin sequence who underwent a mandibular distraction osteogenesis procedure. Intubation events were collected, and a descriptive analysis was performed. A univariate logistic regression model was applied to direct laryngoscopy and flexible fiberoptic laryngoscopy with rigid video laryngoscopy as a reference. RESULTS: Twenty-five patients were identified with a total of 56 endotracheal events. All patients were successfully intubated. Direct laryngoscopy was successful at first intubation attempt in 47.3% (9/19) of events. Six direct laryngoscopy events required switching to another device. Rigid video laryngoscopy was successful at first intubation attempt in 80.5% (29/36) of events. Two cases required switching to another device. Flexible fiberoptic laryngoscopy was found successful at first intubation attempt in 88.9% (8/9) of events. Direct laryngoscopy was 4 times more likely to fail first intubation attempt when compared to rigid video laryngoscopy (P < .05). There was no significant difference between rigid video laryngoscopy and flexible fiberoptic laryngoscopy for intubation. CONCLUSIONS: For children with Pierre Robin sequence rigid video laryngoscopy should be considered as a first attempt intubation device both in the operating room and for emergent situations. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:1647-1651, 2021.

Interferon regulatory factor 6 is required for proper wound healing in vivo.

BACKGROUND: Van der Woude syndrome (VWS) is the most common form of syndromic orofacial cleft caused predominantly by mutations in Interferon Regulatory Factor 6 (IRF6). We previously reported that individuals with VWS have increased risk of wound healing complications following cleft repair compared with individuals with nonsyndromic orofacial clefts (nonsyndromic cleft lip and palate-NSCLP). In vitro, absence of IRF6 leads to impaired keratinocyte migration and embryonic wound healing. However, there is currently no data on tissue repair in adult animals and cells with reduced levels of IRF6 like in VWS. RESULTS: Excisional wounds of Irf6+/- and wild-type animals were analyzed 4 and 7 days post-wounding. Although all wounds were reepithelialized after 7 days, the epidermal and wound volume of repaired wounds was larger in Irf6+/- . These data were supported by increased keratinocyte proliferation in the neoformed epidermis and a less mature granulation tissue with increased cytokine levels. This effect was not cell autonomous, as Irf6+/- neonatal keratinocytes in vitro did not exhibit defects in scratch wound closure or proliferation. Keratinocytes from individuals with VWS also migrated similarly to keratinocytes from NSCLP individuals. CONCLUSIONS: These data support a role for IRF6 in wound healing by regulating keratinocyte proliferation, granulation tissue maturation, and cytokine levels.

Quantitative Endoscopic Phototransducer Investigation of Normal Velopharyngeal Physiology.

PURPOSE: The purpose of this research was to learn the extent to which healthy individuals vary in their ability to achieve velopharyngeal closure for speech. METHOD: Twenty healthy adult volunteers (10 women, 10 men) were tested using an endoscopic phototransducer system that tracks variations in velopharyngeal closure during speech production. Each speaker produced multiple repetitions of three utterances that differed in phonetic content. The data were amplitude normalized and averaged for each speaker. RESULTS: Average phototransducer measurements were similar across subjects for utterances containing only oral phonemes. Average percentage of velopharyngeal closure varied considerably among subjects when producing utterances containing both oral and nasal phonemes (54%-95%). Average percentage of velopharyngeal closure levels were significantly lower (p < .05) for utterances that included nasal consonants. CONCLUSIONS: Phototransducer measurements of velopharyngeal closure for speech are sensitive to nasal phoneme content. The findings suggest that motor programming that accomplishes rapid oral-nasal velopharyngeal valving for speech may differ among healthy subjects. However, such variations in motor programming may not perceptually affect typical speakers. If present in individuals with abnormal velopharyngeal mechanisms, these variations may help explain variations among speakers in speech outcomes after physical and behavioral management.

Development of a preliminary pediatric tracheal growth model from magnetic resonance images.

OBJECTIVES/HYPOTHESIS: To develop a growth model of the minimum cross-sectional area of the normal pediatric trachea with measurements from magnetic resonance images (MRIs) to supplement the clinical criteria used to determine if a child with tracheal stenosis needs surgery. STUDY DESIGN: Retrospective imaging review. METHODS: A total of 81 patients were imaged for a variety of clinical reasons and declared to have normal tracheas fully visible in their T1 magnetic resonance image. Regression analysis was used to identify any contribution that age, gender, and z scores for height and weight have in predicting the minimum cross-sectional area of the trachea. RESULTS: The best-fit model for minimum cross-sectional area is: Area = -0.00451*age(4) + 0.177*age(3) - 2.05*age(2) + 12.6*age + 8.02 (area in mm(2) and age in years). Gender and z scores for height and weight did not provide any additional explanation of variance in tracheal size. CONCLUSIONS: Our study demonstrates the potential to create a growth model of the normal trachea based on cross-sectional area of the trachea using MRIs. Even with the relatively small number of patients used to build it, the model has demonstrated some ability to be used as an objective prediction tool when deciding a treatment path for a patient. With continued development of precise, objective measures to diagnose the severity of the tracheal stenosis, more patients can be given early and accurate prognosis and be treated appropriately.

Changing microbiology of pediatric neck abscesses in Iowa 2000-2010.

OBJECTIVES/HYPOTHESIS: To examine the changing microbiology of pediatric neck abscesses over a 10-year period with particular interest in methicillin-resistant Staphylococcus aureus (MRSA) infections and their associated antibacterial-resistance patterns, including resistance to clindamycin, a frequently used antibiotic for Staphylococcus aureus. STUDY DESIGN: Retrospective chart review at a tertiary academic medical center. METHODS: One hundred and twenty-two consecutive pediatric patients managed between January 2000 and June 2010 with incision and drainage of a proven neck abscess. RESULTS: Seventy-four patients with 76 abscesses were identified. A microorganism was found in 65 (85%) of the 76 abscesses. Forty-three percent grew Staphylococcus aureus (SA), 12% were methicillin-resistant Staphylococcus Aureus (MRSA), and 31% were methicillin-susceptible Staphylococcus aureus (MSSA). There was a significant increase in the incidence of MRSA infections during the study period, with only one case of MRSA diagnosed in the first half of the study (from 2000-2004) compared with seven in the second half (from 2005-2010) (P = 0.023). The second most common bacterial etiology or isolate was Streptococcus pyogenes in 27%, while the remaining 30% grew mixed oral flora and other microorganisms. CONCLUSIONS: These findings demonstrate a statistically significant rise in the proportion of MRSA neck infections in pediatric patients in Iowa. Resistance to clindamycin was highest among MSSA isolates. Clindamycin-resistant S. aureus and Streptococcus pyogenes are established pathogens in neck infections. In communities with similar microbiology patterns, empiric treatment with combination therapy of a beta lactam and vancomycin or trimethoprim/sulfamethoxazole should be initiated until culture results are available.

Pediatric endoscopic airway management with posterior cricoid rib grafting.

OBJECTIVES/HYPOTHESIS: To confirm and extend reported successful treatment of posterior glottic stenosis in pediatric patients using endoscopic laser division of the posterior cricoid plate with augmentation using costal cartilage. STUDY DESIGN: A retrospective chart review and case series. METHODS: Medical records were examined to determine the surgical indications, outcomes, and postoperative complications of this procedure. RESULTS: Twelve patients underwent the procedure, six females and six males, with an average age of 7 years (range, 2-26 years). There were 8/12 (67%) patients successfully decannulated after being tracheostomy dependent. There were no consistent anatomic abnormalities or surgical findings predictive of failure to decannulate. Average hospital stay was 3.6 days (range, 2-9 days). There were no deaths or other major complications; one patient had extrusion. CONCLUSIONS: Endoscopic posterior cricoid grafting is a valuable surgical option for patients with posterior glottic stenosis. The procedure is associated with low morbidity and permits decannulation in the majority of patients.

Complications of otitis media before placement of tympanostomy tubes in children.

OBJECTIVES: To report the incidence of short-term complications from otitis media in children before placement of tympanostomy tubes (TTs) and to compare children treated according to the Agency for Health Care Policy and Research guidelines with those who were treated earlier or later than recommended. DESIGN: Retrospective outcomes review. PATIENTS: Subjects were children aged 10 or younger who had TTs inserted at a tertiary care county hospital from January 1, 1999, to December 31, 2000. Exclusion criteria included prior TT placement, any concurrent head and neck procedure, and craniofacial defects. INTERVENTION: Tympanostomy tube placement. MAIN OUTCOME MEASURES: Any occurrences of otorrhea, tympanic membrane perforation, tinnitus, antibiotic reactions, speech or language delay, febrile seizures, or meningitis before placement of TTs documented in the county hospital records were recorded as complications. Hearing loss was considered separately. RESULTS: Of 147 children who met our criteria, 81 (55.1%) had 1 or more complications from otitis media before placement of TTs. Fifty-five (37.4%) had 2 to 6 complications documented. Adverse reactions to antibiotics were the most common complication, reported in 34 (23.1%). CONCLUSIONS: Most children in this county hospital experienced short-term complications of otitis media before receiving TTs. Even the children treated "on time" according to the guidelines from the Agency for Health Care Policy and Research experienced complications; however, adherence to the guidelines had no significant effect on complications.

Spontaneous tracheal and subglottic tears in neonates.

OBJECTIVES/HYPOTHESIS: Spontaneous rupture of the trachea or subglottis as a complication of difficult delivery has not been reported in the United States literature. There have been a few cases reported in the European literature. The present report describes a series of newborns with this complication and discusses the signs and treatment options of this difficult, life-threatening problem. STUDY DESIGN: Retrospective review. METHODS: Newborns born between 1996 and 2001 who were treated for spontaneous subglottic or tracheal rupture at a tertiary care children's hospital neonatal intensive care unit were reviewed. RESULTS: Four cases of spontaneous subglottic rupture were seen at the hospital. In three of the four cases the tracheas were intubated on an emergency basis after subcutaneous air was noted in the anterior aspect of the neck. In the fourth patient the trachea was not intubated until the subglottic tear was visualized intraoperatively. Two of the four patients died. One died without securing of an airway; the other died of complications of prolonged hypoxia. Eight cases from European literature of spontaneous neonatal subglottic and tracheal tears are reviewed and are compared with the cases presented in the current report. CONCLUSIONS: Early detection of airway rupture by flexible endoscopy is essential for timely diagnosis and appropriate treatment. Standard endotracheal intubation can exacerbate the problem and should be deferred if possible until direct airway visualization can be accomplished. Signs associated with tracheal tears include subcutaneous emphysema, respiratory distress, pneumothorax, and pneumomediastinum. These should lead to emergent consultation with otolaryngologists for examination and securing of the airway.