Primary Repair of Ear Avulsion with Adjuvant Hyperbaric Therapy and Nitroglycerin Ointment.

Traumatic auricular avulsion is a rare and deforming injury. Classically, repair has required microvascular anastomosis. In this publication, we report two separate cases of pediatric auricular avulsion from dog bites. In both cases, the ear was cleaned and surgically reattached. Adjunctive therapies included hyperbaric oxygen and nitroglycerin ointment. There was complete graft take for one patient and 90% graft take for the second, both achieving satisfactory aesthetic outcome. These unique cases highlight the benefits of surgical reattachment of the avulsed portion of the ear followed by hyperbaric oxygen therapy and nitroglycerin ointment.

Head and neck venous thrombosis secondary to pediatric otolaryngologic infection.

OBJECTIVES: Head and neck venous thrombosis is a rare but potentially devastating complication of childhood otolaryngologic infections. This study examines the presentation and management of this condition. METHODS: A retrospective chart review was performed on all pediatric patients with otolaryngologic infections complicated by cranial and cervical venous thrombosis at a tertiary children's hospital from 2007 to 2018. Patient demographics, presentation, site of infection, thrombosis location, implicated pathogen, length of hospital stay, need for surgery, and anticoagulant regimen were assessed. RESULTS: This study included 33 patients (mean age, 7.5 years; age range, 0.8-17 years; 19 [58%] male). The most common infection source was otologic (n = 20), followed by ophthalmic and sinonasal pathology (n = 9), and neck infections (n = 4). The most common site of thrombosis secondary to ear pathology was the sigmoid sinus. The ophthalmic veins were the most common site of thrombosis for ophthalmic/sinonasal infections. Nine CN VI palsies, one CN VII palsy, and one CN III palsy were observed. Twenty-six subjects (79%) required surgical intervention. All those who experienced a nerve palsy required surgery. Length of hospitalization significantly differed with the stay for a neck infection complicated by thrombosis longer compared to otologic and sinonasal infections (F[2,30] = 7.08, p = 0.003). Length of hospital stay was significantly correlated with admission temperature (r = 0.506, p = 0.003) and CRP (r = 0.400, p = 0.03) but not WBC (r = 0.181, p = 0.31). Culture growth predominantly isolated a single causative organism rather than polymicrobial involvement. Forty-eight species were identified, most (n = 41/48, 85%) being Gram-positive bacteria. Alpha-hemolytic Streptococcus was the most common isolate from children with vessel thrombosis secondary to ear infections, with Streptococcus pyogenes predominant in sinonasal infections and Staphylococcus aureus the most common in neck abscesses. There was significant variability in anticoagulation management within the patient population, but no bleeding complications were documented. Most patients had no evidence of underlying thrombophilia (n = 15); for those with positive hypercoagulability screens, the most common positive marker was the presence of lupus inhibitor (n = 6). CONCLUSION: Venous thrombosis resulting from adjacent otolaryngologic infection is a serious complication requiring proper recognition and management. The involved vasculature and cranial nerve findings are dependent on the anatomic location of the underlying infection. Cranial neuropathies in the presence of these infections should prompt evaluation for possible thrombosis.

Update on congenital ear molding.

PURPOSE OF REVIEW: Congenital ear deformities are characterized by distorted, but still present, landmarks of the auricle. Interventions include early treatment with ear molding or delayed surgical treatment. The purpose of this review is to provide an update on the latest information related to ear molding for congenital ear deformities. RECENT FINDINGS: Various ear molding techniques date back to the 1980s, but with the availability of commercially available molding systems, interest and use has blossomed in recent years. As more longitudinal research results are obtained, ear molding has been proven to be a highly effective treatment, although the best technique and length of treatment remains unclear. SUMMARY: While it is clear that earlier intervention is ideal, due to lack of public awareness, patients often present later, and it remains controversial what age to offer ear molding interventions to and still expect success. Ear molding interventions are becoming increasingly covered by insurance due to evidence of preventing long-term psychological morbidities and the need for future surgical interventions.

Prenatal Consultation Outcomes for Infants With Cleft Lip With and Without Cleft Palate.

To assess the clinical impacts of prenatal consultation with a multidisciplinary cleft team on infants with cleft lip with or without cleft palate (CL ± P).Retrospective cases series.Tertiary pediatric hospital.Infants with CL ± P whose mothers received prenatal consultation with a pediatric otolaryngology team from June 2005 to December 2019 were identified. A random sample of infants with CL ± P without prenatal consultation from June 2005 to December 2019 was also identified.The primary outcomes were the length of hospitalization during the first 12 weeks of life, timing of surgical repair, length of postsurgical hospitalization, and number of unplanned clinic visits and phone calls for feeding evaluation.Time to cleft lip repair differed significantly between the 2 groups with repair performed at 13.4 (±0.9) weeks for the prenatal consultation group (n = 73) and 15.3 (±2.1) weeks for the control group (n = 80), (P < .05). If hospitalization was required for feeding difficulties during the first 12 weeks of life, length of stay was 4.9 (± 1.7) days for infants with prenatal consultation and 11.5 (± 7.2) days for control infants (P < .05). Unplanned clinic visits with a speech-language pathologist (SLP) for feeding difficulties were needed for 2.7% of prenatal consultation infants and 11.3% of control infants (P < .05).Prenatal consultation regarding CL ± P resulted in infants with decreased duration of early hospitalizations, earlier cleft lip repair, and decreased engagement with the SLP feeding clinic for feeding difficulties when compared with infants without prenatal consultation.

The Incidence of Velopharyngeal Insufficiency in Stickler Syndrome.

OBJECTIVE: Stickler Syndrome (SS) is an inherited collagenopathy characterized by heterogenous orofacial, ocular, auditory, and skeletal abnormalities. The orofacial manifestations are variable and some patients present with cleft palate and velopharyngeal insufficiency (VPI). The incidence of VPI in SS is poorly studied and no studies have compared the incidence of VPI between Type I (COL2A1) and Type II (COL11A1) SS. The objective of this study is to compare the incidence of VPI between SS subtypes and discuss the surgical techniques used to treat them. DESIGN: Single-institution, retrospective chart review. SETTING: Tertiary pediatric hospital. PATIENTS/PARTICIPANTS: Forty-three children were diagnosed with SS between January 2003 and December 2018. Genetic testing results, genetics notes, craniofacial clinic notes, and operative reports were reviewed. Patients without genetic testing or craniofacial/otolaryngologic evaluation were excluded. Thirty-one patients met criteria and were included. MAIN OUTCOME MEASURE: Primary outcome was VPI incidence. RESULTS: There were 18 patients with Type I SS and 13 with Type II SS. Five (16%) patients had VPI, 2 (11%) with Type I SS compared to 3 (23%) with Type II SS (P > .05). All patients with VPI underwent surgery with either sphincter pharyngoplasty (3) or pharyngeal flap (2). Two patients with Type II SS underwent revision sphincter pharyngoplasty, with one conversion to pharyngeal flap. CONCLUSION: VPI is common for patients with SS. In this study, there was no significant difference in the incidence of VPI between SS subtypes. Future studies are needed to confirm these findings, which could be important for patient counseling and treatment planning.

Demographic Risk Factors for Malnutrition in Patients With Cleft Lip and Palate.

OBJECTIVES/HYPOTHESIS: Patients with cleft lip and/or palate (CLP) are at increased risk of malnutrition. Acute and chronic malnutrition have been associated with elevated risk of postsurgical wound complications, adding morbidity and cost to patients and their families. To study the association between demographic factors, including insurance type, race, and median neighborhood income (MNI), and malnutrition in patients with CLP. STUDY DESIGN: Retrospective cohort study. METHODS: Retrospective review was performed in patients undergoing their first cleft-related surgery at a large tertiary pediatric hospital from 2006 to 2018. Demographic data, weight and height at surgery, type of insurance, race, and primary residential address were collected. Geocoded information on MNI was generated using patient address. World Health Organization Z-scores for weight-for-age (WFA) and height-for-age (HFA) were used as proxies for acute and chronic malnutrition, respectively. Linear regression models were generated to analyze the relationship of insurance type, race, and MNI on WFA and HFA Z-scores. RESULTS: About 313 patients met inclusion criteria. Increasing MNI predicted increasing WFA Z-score (0.05 increase in WFA per $1,000 increase, P = .047) as well as HFA Z-score (0.09 increase in HFA per $1,000 increase, P = .011). The effect of MNI was not independently modified by race for either WFA (P = .841) nor HFA (P = .404). Race and insurance type did not predict WFA or HFA. CONCLUSIONS: Lower MNI is a significant independent risk factor for acute and chronic malnutrition in children with CLP. Combined with previous investigation linking malnutrition to surgical outcomes in this population, this offers a target area for intervention to improve patient outcomes. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:1482-1486, 2022.

Percutaneous Enteral Feeding in Patients With 22q11.2 Deletion Syndrome.

OBJECTIVE: To describe the incidence of percutaneous enteral feeding in patients with 22q11.2 deletion syndrome (22q11.2 DS) and determine factors associated with the need for percutaneous enteral feeding tube placement. DESIGN: Retrospective chart review. METHODS: The records of a 22q11.2 DS clinic and pediatric otolaryngology clinic at a tertiary pediatric hospital were reviewed from January 1, 2009, to December 31, 2019. All patients with confirmed 22q11.2 deletion were identified. Cardiac, otolaryngological, and feeding characteristics were recorded along with surgical history. A patient was defined to have a G-tube if the history was significant for any percutaneous gastric feeding tube placement, including a gastrostomy tube, gastrostomyjejunostomy tube, or a Mickey button. RESULTS: One hundred ninety patients with confirmed 22q11.2 DS by genetic testing were included. Thirty-three percent (n = 63) required G-tube placement. G-tube placement was associated with cardiac diagnosis (P < .01), history of cardiac surgery (P < .01), aspiration (P < .01), nasopharyngeal reflux (P < .01), subglottic stenosis (P < .01), laryngeal web (P = .003), and tracheostomy (P < .01). This suggests these conditions are associated with higher rates of G-tube placement in the 22q11.2 DS population. CONCLUSIONS: Patients with 22q11.2 DS often require supplemental nutritional support in the form of G-tube feeding, most often in the first year of life. Congenital heart abnormalities and surgery along with tracheostomy, subglottic stenosis, laryngeal web, aspiration, and nasopharyngeal reflux are significantly associated with the need for G-tube placement. Understanding associations between comorbid conditions and G-tube placement, especially those involving the head and neck, may assist with counseling of patients with 22q11.2 DS.

Integrated microtia and aural atresia management.

Objectives: To present recommendations for the coordinated evaluation and management of the hearing and reconstructive needs of patients with microtia and aural atresia. Methods: A national working group of 9 experts on microtia and atresia evaluated a working document on the evaluation and treatment of patients. Treatment options for auricular reconstruction and hearing habilitation were reviewed and integrated into a coordinated care timeline. Results: Recommendations were created for children with microtia and atresia, including diagnostic considerations, surgical and non-surgical options for hearing management and auricular reconstruction, and the treatment timeline for each option. These recommendations are based on the collective opinion of the group and are intended for otolaryngologists, audiologists, plastic surgeons, anaplastologists, and any provider caring for a patient with microtia and ear canal atresia. Close communication between atresia/hearing reconstruction surgeon and microtia repair surgeon is strongly recommended.

Nasal septal hematoma in children: Time to diagnosis and resulting complications.

OBJECTIVES: To determine the time from initial injury to diagnosis of nasal septal hematoma (NSH). Additional objectives included determining number of medical evaluations prior to diagnosis and long-term complications. METHODS: A retrospective chart review was performed on all patients diagnosed with NSH at a tertiary pediatric hospital between January 1, 2003 and April 1, 2019 were identified. Time to diagnosis was defined as time between initial trauma to date of diagnosis. Number of evaluations was defined as all medical evaluations prior to diagnosis. RESULTS: Of 2762 charts that were reviewed, 13 patients with NSH were identified. Of those, 92% were male and trauma was the cause in 85% of patients. Median time to diagnosis was 7 days (0-21 days), with an average of 2.2 evaluations (1-4 evaluations). Settings where diagnosis were missed included EDs (N = 9, 82%), primary care (N = 6, 55%), urgent care (N = 1, 9%) and otolaryngology clinic (N = 2, 18%). Four patients (31%) were evaluated by an otolaryngologist in the ED. The median time to otolaryngology outpatient visit was 7.5 days. In five patients (46%), septal hematomas were missed in multiple clinical settings. Seven patients (54%) experienced complications, including saddle nose deformity (N = 3, 23%). CONCLUSIONS: NSH is a rare but serious emergency. To avoid multiple visits and delay in diagnosis, additional education and awareness is needed for providers who evaluate these patients. Early diagnosis will reduce the risk of abscess formation and saddle nose deformity.

A protocol of situation-dependent transfusion, erythropoietin and tranexamic acid reduces transfusion in fronto-orbital advancement for metopic and coronal craniosynostosis.

PURPOSE: Assess the effect of a protocol of preoperative erythropoietin (EPO) and ferrous sulfate in addition to perioperative tranexamic acid (TXA) on blood transfusions in patients with coronal or metopic craniosynostosis undergoing cranial vault remodeling (CVR) with fronto-orbital advancement (FOA). METHODS: Retrospective review of all coronal and metopic craniosynostosis patients undergoing CVR and FOA from March 2010 to June 2019 was performed. Before 2014 ("Control group"), all patients received blood transfusion at the start of surgery. In 2014, a protocol of preoperative EPO and ferrous sulfate with perioperative TXA and non-automatic transfusion was instituted ("Study group"). Patient demographics and anthropometrics, perioperative hemoglobin (Hb) levels, and transfusion details were collected and compared. RESULTS: Thirty-six patients met inclusion criteria. Twenty-one patients were in the control group, and 15 in the Study group. Nineteen patients had metopic synostosis, 11 had unicoronal synostosis, and 6 had bicoronal synostosis. There were no significant differences between groups in demographics, operative time, intraoperative crystalloid volume, craniofacial syndromes, or sutures affected. The Study group had higher preoperative Hb (13.9 ± 1.0 vs. 12.6 ± 0.8 g/dL, p < 0.001), lower intraoperative Hb nadir (7.4 ± 1.8 vs. 9.2 ± 1.2 g/dL) lower intraoperative transfusion rate (66.7% vs. 100%, p = 0.008), lower postoperative transfusion rate (0% vs 28.6%, p = 0.03), and exposure to fewer unique units of packed red blood cells (0.7 ± 0.6 vs. 1.5 ± 0.9 units). CONCLUSION: Our protocol resulted in decreased transfusion needs. These results add valuable information to the growing body of work on transfusion reduction in craniosynostosis surgery.

Malnutrition as a Risk Factor in Cleft Lip and Palate Surgery.

OBJECTIVES/HYPOTHESIS: To assess the prevalence of acute and chronic malnutrition at the time of surgery in patients with cleft lip and/or palate (CLP) at our institution, and to quantify nutrition as a risk factor for postsurgical complications following CLP surgery. STUDY DESIGN: Retrospective cohort study. METHODS: Retrospective review of 855 children undergoing initial cleft lip or palate surgery, or revision surgery after fistula/dehiscence of initial cleft repair. We measured acute and chronic malnutrition using World Health Organization Z-scores of weight-for-age and height-for-age, respectively, and noted any postsurgical fistula or dehiscence. RESULTS: Among patients with cleft lip, 22.3% were at least moderately chronically malnourished at the time of initial repair, and 17.5% were at least moderately acutely malnourished. Among patients undergoing initial repair of cleft palate, 20.9% were at least moderately chronically malnourished, and 8.1% were at least moderately acutely malnourished. Increasing nutritional status, as measured by height-for-age, predicts decreased odds of fistula (OR 0.78, P = .01) after cleft palate surgery. CONCLUSIONS: Chronic malnutrition significantly increases the risk of fistula formation in patient with cleft palate. Preoperative strategies to manage this risk and influence surgical timing can avoid morbid and costly postoperative complications. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2060-E2065, 2021.

Multimodal Imaging Characteristics and Diagnostic Approach to Ancient Schwannoma in a Pediatric Patient.

Ancient schwannoma is an extremely rare benign, peripheral nerve sheath tumor. Despite its benign nature, its characteristic heterogeneous appearance and degenerative changes commonly lead to misdiagnosis of malignancy. Although schwannomas are extremely uncommon in the pediatric population, these neoplasms have been associated with underlying conditions such as neurofibromatosis type two, and appropriate recognition is important to ensure close monitoring of potential future symptoms secondary to other tumors. We report the imaging and laboratory findings of an ancient schwannoma of the vagus nerve in a 10-year-old female, the first documented case of such a tumor in a pediatric patient, and discuss its characteristic findings and diagnostic considerations. Awareness of this rare tumor can help promote correct diagnosis and avoidance of costly, high-risk diagnostic methods.

Comparing autologous versus allogenic rib grafting in pediatric cleft rhinoplasty.

BACKGROUND: Patients with cleft lip with or without cleft palate suffer from varying degrees of nasal deformity, often requiring nasal reconstruction to provide improved form and function. Rib cartilage is an excellent source of grafting material for nasal reconstruction and is available either as an autologous or allogenic graft. There is a paucity of literature comparing outcomes of autologous and allogenic rib grafts in pediatric cleft rhinoplasty. METHODS: A retrospective chart review was performed on patients who underwent cleft rhinoplasty with autologous or allogenic rib grafting at a tertiary pediatric hospital between January 1, 2003 and December 31, 2017. Outcome data were gathered over a 6-month postoperative period. RESULTS: There were 23 cleft rhinoplasties performed with rib graft, 12 with autologous rib and 11 with allogenic rib. Those in the autologous group tended to be older than those in the allogeneic group (15.6 ± 4.4 v 12.4 ± 5.2 years, p = 0.13). The most common types of grafts used were columellar strut (20/23), shield graft (9/23), and unilateral or bilateral batten grafts (7/23). Length of stay was significantly longer for patients who underwent autologous rib grafting compared with those with allogenic rib grafting (25.8 ± 4.7 v 11.9 ± 7.2 h, p < 0.05). Each group reported one complication. CONCLUSION: Autologous and allogenic rib grafts are safe and effective in pediatric rhinoplasty. The most common grafts used in this sample were columellar strut, batten, and shield grafts. Autologous rib grafts were more likely to be used in older patients and require longer hospital stay compared to allogenic grafts.

Correlation of air-bone gap and size of Enlarged Vestibular Aqueduct in children.

OBJECTIVE: Enlarged vestibular aqueduct (EVA) is an inner ear malformation that represents an important cause of pediatric hearing loss. While certain elements in the history or audiogram may suggest EVA, it is most often diagnosed using computed tomography (CT). The present investigation was conducted to determine if the size of the audiometric air-bone gap (ABG) is correlated with the size of the vestibular aqueduct in the pediatric population using three vestibular aqueduct measurements. These included the fundus, midpoint, and porous widths of the vestibular aqueduct. STUDY DESIGN: This is a retrospective cohort study. SETTING: This study took place at a tertiary care referral center. PATIENTS: Fifty-five children (33 female; 22 male) with a confirmed diagnosis of unilateral or bilateral EVA as determined by prior imaging of the inner ear were included in the study. MAIN OUTCOME MEASURES: Associations of EVA measurements with ABGs at 0.5 and 1 kHz were evaluated using Pearson correlation coefficients. RESULTS: All of the correlation coefficients were positive, indicating that as EVA measurements increased so did the ABG. Only the correlation between fundus width and ABG at 1 kHz was not statistically significant. CONCLUSIONS: ABGs measured during audiometric testing correlate with the size of the EVA and ABGs can be clinical predictors of the severity of the bony abnormality. These data support the third window theory of conductive hearing loss in pediatric EVA.

Minimizing transfusion in sagittal craniosynostosis surgery: the Children's Hospital of Minnesota Protocol.

PURPOSE: To assess the success of a protocol using preoperative erythropoietin (EPO) and iron with perioperative tranexamic acid (TXA) in reducing blood transfusion in sagittal craniosynostosis surgery. METHODS: A retrospective chart review of all sagittal craniosynostosis patients undergoing open repair at our institution since 2010 was conducted. A novel protocol of preoperative EPO with iron and perioperative TXA, along with a shift away from automatic transfusion, was initiated in 2014. Perioperative hemoglobin levels, length of stay, and transfusion rates were compared between the historical control and the study group receiving the protocol. RESULTS: A total of 36 patients met inclusion criteria. Twenty-eight patients were male and 8 were female. Twenty-two patients were in the control group receiving neither TXA nor EPO and automatically received a transfusion, while 14 were in the study group and received the full protocol. There were no significant demographic differences between groups. Within the control group, 100% of patients were transfused compared with 14.3% of the study group (p < 0.0001). The study group also had a shorter postoperative length of stay in the hospital (mean, 3.4 days; range, 3-6) than the control (mean, 4 days; range, 2-5.5, p = 0.038). The study group had a higher preoperative hemoglobin than the control (13.6 vs. 11.8 g/dL, p = 0.0001). CONCLUSION: Our protocol of preoperative EPO and iron with perioperative TXA increased the preoperative hemoglobin and was associated with a low transfusion rate without negatively impacting postoperative course.

Vincristine-induced vocal cord paresis and paralysis in children.

OBJECTIVES: To describe three new cases of vincristine-induced vocal cord paresis or paralysis (VIVCPP) in children and to review the diagnosis and management of this neuropathy. METHODS: Retrospective case series. Diagnosis of VIVCPP was confirmed by laryngoscopy in all children. RESULTS: Less than 20 cases of VIVCPP in children have been previously documented in the literature. Of the three children in our case series, one had unilateral vincristine-induced vocal cord paresis and two had bilateral VIVCPP. The first two patients each had two separate episodes of paresis, lasting 4 months and 1 month respectively. In the last patient, whose medical course was complicated by many additional factors, vocal cord paralysis persisted for over three years. CONCLUSIONS: Clinicians must evaluate children with suspected VIVCPP for concomitant symptoms and signs of vincristine neuropathies and examine the vocal cords via laryngoscopy. The effects of vincristine neurotoxicity can be waxing and waning, demonstrate delayed onset and persist well beyond drug cessation. Further studies are needed to identify effective neuroprotectants and delineate appropriate vincristine dosing in patients with vincristine neurotoxicity and cancer.

Microtia and Related Facial Anomalies.

Infants may be born with external ear deformities or malformations that can present a diagnostic clue to an affiliated syndrome while also presenting the possibility of surgical intervention. Microtia is a malformation of the ear that is associated with other craniofacial or systemic anomalies in 50% of cases. Surgical correction of microtia and associated facial anomalies is complex and must be integrated thoughtfully into the overall care plan for children with complex medical needs. Familiarity with types of ear and facial anomalies and their association with more global concerns can allow perinatal practitioners to better serve their patients.