Pediatric Functional Rhinoplasty.

Surgery on the growing pediatric nose poses many questions regarding the indications and proper timing. Historically, surgeries on the pediatric nose led to facial growth alterations so many urged caution and waiting until skeletal maturity at the age of 18. However, animal studies showed some promise that facial growth may not be affected. Investigations into effects of pediatric sinus surgery and septal surgery on facial growth showed no issues. The external nose is somewhat different in that minor variations potentially can grow to a cosmetic deformity. However, numerous studies on facial growth show, to date, that it is not affected, and that surgery is safe. The caveat that one uses is the techniques of cartilage sparing and preservation, which have been studied and shown to be safe. There are ways to measure the effectiveness of surgery in relieving nasal airway obstruction both objectively and, more recently, subjectively. It is essential to treat pediatric nasal airway obstruction to avoid other facial growth problems, such as mouth breathing, snoring, malocclusion, and obstructive sleep apnea.

Predictive Factors for Perinatal Outcomes of Infants Diagnosed With Micrognathia Antenatally.

INTRODUCTION: Advances in fetal imaging have allowed us to identify abnormalities previously not appreciated. With this study, we hope to identify factors predicting a difficult airway at birth and review the perinatal outcomes of these patients. METHODS: Sixteen patients with antenatally diagnosed micrognathia were reviewed from a tertiary care hospital database from 2011 to 2016. Jaw index (JI), amniotic fluid index (AFI), glossoptosis, gastric size, and oropharynx obliteration were assessed. The airway support required at birth, specialist team involvement, and outcomes were evaluated. RESULTS: Nine (56.3%) of 16 patients had JI <5th percentile, 3 (33.3%) of 9 had difficult intubation, 2 (22.2%) of 9 needed an emergency tracheostomy, and 1 (11.1%) of 9 died. Seven patients had polyhydramnios, 2 (28.6%) of 7 had difficult intubation, 2 (28.6%) of 7 required tracheostomy, and 1 (14.3%) of 7 died. Twelve patients had either JI <5th percentile or abnormal AFI, 5 (41.7%) of 12 had difficult intubation, 2 (16.7%) of 12 required tracheostomy, and 1 (8.33%) of 12 died. For the group without otolaryngology consultation, 8 (50%) of 16, 1 (12.5%) of 8 had difficult intubation and 1 (12.5%) of 8 died because airway was not secured after 45 minutes of resuscitation. CONCLUSION: Jaw index <5th percentile or abnormal AFI predicts a difficult airway. A multidisciplinary approach with otolaryngology involvement for airway intervention may be required at birth.

A case of rare isolated agnathia and literature review.

Agnathia is a rare congenital malformation with unknown etiology characterized by absence of the mandible, microstomia, and tongue aplasia, often found to have other anomalies including holoprosencephaly. The purpose of this paper was to describe the symptoms and imaging of a case of isolated agnathia and to conduct a comprehensive literature review of reported patients with isolated agnathia. Case reports of isolated agnathia are very rare, with most infants as stillborn. We report a child's management of isolated agnathia with microstomia and tongue aplasia. A literature review was performed with focus on diagnosis, airway, and feeding management of isolated agnathia. Polyhydramnios was a common pregnancy complication reported in 25 out of the 39 patients in the case study. Five infants were stillborn, while 23 died within the neonatal period. Of the deceased infants within the neonatal period, 19 died within minutes to hours while four died within days to weeks. There are nine patients with agnathia that survived past infancy. The results of this study suggest that isolated agnathia is a rare malformation which requires a multi-disciplinary approach for airway and feeding management.

Novel deletion of 6p21.31p21.1 associated with laryngeal cleft, developmental delay, dysmorphic features and vascular anomaly.

Interstitial deletions involving chromosome region 6p21.31p21.2 have not been previously reported in the literature. Here, we present a 2 year old girl with global developmental delay, severe speech delay, dysmorphic features, laryngeal cleft, anterior descending aorta that occluded the left main bronchus and a novel de novo deletion of chromosome 6: arr[hg19] 6p21.31p21.2 (35462950-36725083)x1. The deletion, which was diagnosed by array comparative genomic hybridization and further confirmed with fluorescence in situ hybridization, was approximately 1.26 Mb and contained 28 RefSeq genes. The deleted region includes 24 protein coding genes and 4 non-coding genes. This represents a novel microdeletion that has not been previously reported in the literature.

Nasal erosion as an uncommon sign of child abuse.

While various forms of facial trauma, bruising, burns, and fractures are frequently seen in cases of child abuse, purposeful nasal erosion has rarely been identified as a form of abusive injury. Progressive destruction of nasal tissue in children provokes a wide differential diagnosis crossing multiple subspecialties: infectious, primary immunodeficiencies, inflammatory conditions, malignancy, and genetic disorders. Progressive nasal erosion also can be a manifestation of child abuse. The proposed mechanism is repetitive mechanical denudation of the soft tissue and cartilage resulting in chronic inflammation, bleeding, and ultimately destruction of the insulted tissue. We report 6 cases of child abuse manifesting as overt nasal destruction.

Reducing Surgical Revisions in Intracranial Complications of Pediatric Acute Sinusitis.

Objective (1) To describe the demographics and clinical course of children with intracranial complications of sinusitis. (2) To elucidate factors that predict revision surgery in this population, such as type of initial surgery. Study Design Case series with chart review. Setting Tertiary care academic children's hospital. Subjects and Methods A 15-year retrospective review identified 71 patients with intracranial complications of acute sinusitis. Primary outcome was need for revision surgery. Secondary outcomes were readmission, length of hospitalization, and long-term complications. Results This study is the largest to date examining this disease process. Overall, 69 (97%) patients had surgery; 33 (46%) required revision surgery. Half of the patients with frontal sinus opacification underwent frontal sinus surgery at presentation (endoscopic, trephination, or cranialization). There was no difference in revision surgery between patients who had frontal sinus surgery and those who did not. Patients with frontal sinus surgery did not have a higher rate of complications or chronic sinusitis ( P > .05). Subdural abscess was associated with multiple surgical procedures (odds ratio, 20.0; P < .01). Thirty-four patients (49%) required neurosurgery. These patients had a longer length of stay (odds ratio, 11.0; P < .01) and a higher readmission rate ( P = .02). During the mean 92-month follow-up, 22 patients (33%) had long-term complications, and there was 1 death (1.4%). Conclusion Almost half of this cohort required multiple surgical procedures. In particular, patients with subdural abscess had significantly higher rates of revision surgery. Type of frontal sinus surgery was not correlated with need for revision surgery and was not associated with an increased rate of complications.

Difficult airway intubation simulation using Bonfils fiberscope and rigid fiberscope for surgical training.

INTRODUCTION: Pediatric otolaryngologists are frequently called to assist in difficult airway management in newborns with Pierre Robin Sequence (PRS) who have microretrognathia, glossoptosis, and an anterior larynx. The Bonfils fiberscope (BF) is a curved rigid scope designed to provide superior visualization in the anterior larynx. OBJECTIVE: (1) to assess whether BF provides an improvement in intubation success rate, time to intubation, or airway visualization as compared to rigid fiberscope (RF) in a difficult airway simulation setting and (2) to determine whether a training program for BF can improve time to intubation through practice trials. METHODS: Six right-handed trainees completed five trials on each of the three following airway models using the BF and RF: normal anatomy, anterior larynx and PRS. The normal larynx model was intubated only with RF. Main outcome measures were the time needed for tracheal intubation and Cormack-Lehane classification (1-4). RESULTS: The majority of the intubation trials showed a statistically significant difference between first and last completion times (p < .05) suggesting construct validity. Only the anterior larynx trials with BF did not demonstrate an improvement in time to intubation between first and last attempts (p < .3125). For the PRS retrognathia model, there was no statistically significant difference in time to intubation between using the BF and the RF (p < .44); in the anterior larynx model, the RF yielded a faster time to intubation than the BF on the final trial attempts (p < .0313). By Cormack-Lehane classification measures, laryngeal visualization by the BF was better than RF in the PRS manikin (p < .0022) while there was no significant difference in grade scores for the anterior larynx manikin (p < .45). All six trainees reported an improved visualization of the larynx with the BF compared to the RF for both the anterior larynx and PRS manikins; at the end of the trial runs, all participants noted an improvement in comfort level using the BF. CONCLUSION: The difficult airway simulation model is feasible for surgical training. BF adds superior visualization of the anterior larynx in PRS. Otolaryngology training programs may include BF as a supplemental tool in addition to RF as a part of the airway equipment training since there is significant improvement in time to intubation with consecutive practice trials and superior laryngeal visualization.

Mature and immature pediatric head and neck teratomas: A 15-year review at a large tertiary center.

INTRODUCTION: Pediatric head and neck teratomas account for less than 4% of congenital teratomas. The distinct presentations and outcomes of mature and immature head and neck teratomas have not been well established. OBJECTIVES: To review the management and outcomes of pediatric head and neck teratomas. To distinguish differences between mature and immature tumors with respect to the age at presentation and surgery, tumor size and location, alpha fetoprotein (AFP) levels, airway management, and recurrence. METHODS: A 15-year retrospective chart review of patients treated for head and neck teratomas at Texas Children's Hospital was performed. A total of 20 patients were included. Wilcoxon rank and Fisher's exact tests were used for statistical analysis. RESULTS: Immature teratomas were associated with both higher AFP levels (80800 ng/ml, p = 0.02) and maximum tumor dimensions (14.4 cm, p = 0.0034) than mature teratomas (24400 ng/ml and 6.44 cm). Patients with immature tumors were younger at the time of surgical resection (19.8 days, p = 0.025) compared to those with mature tumors (348 days). 89% of immature teratomas involved anterior neck localization compared to 27% for mature teratomas (p = 0.0098); 88% of the immature teratomas required an EXIT (Ex Utero Intrapartum Treatment) procedure compared with 40% of the mature teratomas (p = 0.0656). Recurrence was noted in only two cases: an immature teratoma 51 months after incomplete resection and a mature teratoma 33 months after complete resection. Long-term consequences of surgical resection included cleft palate (38.9%), dysphagia (33.3%), facial nerve paresis/paralysis (16.7%) and tracheotomy (16.7%). CONCLUSION: Immature teratomas had higher AFP levels, tumor dimensions, frequency of anterior neck localization, and requirement of EXIT than mature teratomas. Given that there was no significant difference between the recurrence rates of immature and mature teratomas, follow-up vigilance should be maintained equally regardless of tumor maturity.

Tracheal Cartilaginous Sleeve in Syndromic Craniosynostosis: An Underrecognized Source of Significant Morbidity and Mortality.

Tracheal cartilaginous sleeve (TCS) is a rare and previously unrecognized source of morbidity and mortality in patients with certain craniosynostosis syndromes. There is a paucity of reporting on this airway anomaly, and the true incidence of TCS is largely unknown. The purpose of this study was to investigate the incidence of TCS among patients with syndromic craniosynostosis at our institution. Patients with syndromic craniosynostosis who underwent direct bronchoscopy and laryngoscopy were evaluated retrospectively by pediatric otolaryngologists for the presence of TCS and associated anomalies. Among patients with a diagnosis of syndromic craniosynostosis in our craniofacial database, 10 (37%) were found to have previous direct bronchoscopy and laryngoscopy reports. Of these 10 patients, 2 had Crouzon syndrome, 3 had Pfeiffer syndrome, 3 had Apert syndrome, 1 had Muenke syndrome, and 1 had Antley-Bixler syndrome. Eighty percent (8/10) of these patients were found to have some evidence of TCS. The most commonly observed associated findings included the following: tracheostomy dependency (7/10; 70%), hearing loss (6/10; 60%), obstructive sleep apnea (5/10; 50%), cervical spine anomalies (5/10; 50%), developmental delay (5/10; 50%), and enlarged cerebral ventricles (4/10; 40%). Larger multicenter studies are required to further characterize this airway anomaly and its impact on this patient population. Our results confirm the importance of thorough airway evaluation at initial presentation and the need for validated screening protocols.

Incidental radiographic findings of thyroglossal duct cysts: Prevalence and management.

OBJECTIVES/HYPOTHESIS: To determine the epidemiology of incidental thyroglossal duct cysts (TGDC) discovered on imaging studies obtained in the head and neck area in children and to discuss subsequent management. METHODS: A retrospective chart review was performed at Texas Children's Hospital of all computed tomography (CT) and magnetic resonance (MR) imaging studies obtained in the head and neck region between July 2011 and July 2014. Images obtained for the purpose of evaluating a neck or lingual mass were excluded from the study, as were patients with previously known TGDCs. Data including age, sex, location of TGDC, size, presence of symptoms, referral to Otolaryngology, and intervention were recorded. RESULTS: A total of 60,663 CT and MR studies of the head, brain, sinus, neck, and C-spine during this time period were reviewed; of these 69 (0.1%) cases contained incidental discovery of probable TGDCs with more males (40) than females (29). Ages ranged from 3 days to 17 years old, with the mean age at 5 years. Locations varied, with majority at base of tongue (83%) followed by hyoid (13%) then infrahyoid straps (4%). Sizes ranged from 2 to 28 mm with average size at 8 mm. 11 of these patients were referred to an Otolaryngologist; 9 were asymptomatic and decision was made to observe, the other two subsequently underwent surgical excision secondary to mass effect and dysphagia with histologic confirmation of diagnosis. CONCLUSION: TGDCs commonly present as an anterior neck mass, however the majority of incidentally discovered TGDCs on imaging are located at the base of tongue. Management of these findings should include referral to an Otolaryngologist for further evaluation with the decision to intervene surgically based on development of clinical symptoms.

Congenital hairy polyp of the oropharynx presenting as an esophageal mass in a neonate, a case report and literature review.

PURPOSE: To review the literature of congenital hairy polyps and describe the clinical presentation, operative management, and histologic findings of a congenital hairy polyp arising from the palatopharyngeus muscle in a neonate with recurrent choking episodes. METHODS: Chart review of a 2-month-old male referred to a tertiary care pediatric hospital. RESULTS: We present a case of a 2-month-old male who presented to the emergency room with recurrent episodes of choking and vomiting. The patient was previously healthy with no prior medical or neonatal history. The parents noted a small fleshy mass in the patient's oropharynx that he would chew on and swallow after several minutes. However, on physical exam, there was no evidence of oropharyngeal mass. The patient did not have respiratory distress. Imaging revealed a 22×7×11mm oblong, fatty mass in the lower cervical and upper thoracic esophagus with a thin stalk extending proximally to the upper collapsed esophagus. Intraoperative recorded laryngoscopy revealed a pedunculated soft palate mass attached to the right superior palatopharyngeus muscle. Histopathology revealed ectodermal and mesodermal elements in a polypoid structure lined by keratinizing squamous epithelium with adnexal structures and central mature adipose tissue, consistent with congenital hairy polyp resembling an accessory tragus of the ear and branchial anomaly. At 6-week follow up, the patient was doing well and gaining weight appropriately with no further choking episodes. There was no evidence of velopharyngeal dysfunction on follow up exam. The surgical site was completely healed and there was no evidence of recurrence. DISCUSSION: Congenital hairy polyps of the naso- and oropharynx are rare but may present as airway or esophageal masses, causing respiratory distress or choking episodes in a pediatric patient. The pathologic findings of keratinizing squamous epithelium, adnexal structures, adipose and cartilage tissues resemble congenital accessory tragus and may be considered a branchial arch anomaly.

Propranolol for the treatment of subglottic hemangiomas.

INTRODUCTION: Infantile subglottic hemangiomas are rare causes of airway obstruction. They begin to proliferate at 1-2 months of age and can cause biphasic stridor with or without respiratory distress. Diagnosis requires direct visualization by direct laryngoscopy and bronchoscopy. Various therapeutic options have been utilized for treatment, including tracheotomy, open surgical excision, laser ablation, intralesional steroid injection, systemic steroids, and now oral propranolol. METHODS: We present a retrospective chart review of infantile subglottic hemangiomas over a 5-year span (January 2005-2010) at a tertiary care pediatric hospital. IRB approval was obtained, and charts were reviewed to find patients with subglottic hemangiomas, including patient characteristics, presentation, workup, medical and surgical management, and outcomes. A case presentation demonstrates diagnostic, management, and treatment strategies and dilemmas encountered. RESULTS: Nine patients were found to have infantile subglottic hemangiomas. Six of nine patients were treated with laser excision, with five of the six having localized subglottic hemangiomas. In 2009, three of four patients were initiated on propranolol as first-line treatment; the fourth had comorbidities which precluded this. Of the three, two showed improvement, while a third, who also had bearded hemangioma, required tracheotomy. DISCUSSION: Infantile subglottic hemangiomas are rare but essential in the differential diagnosis of biphasic stridor. Although propranolol has been effective in treating cutaneous and airway hemangiomas, our experience suggests that this is not consistent for subglottic hemangiomas. In an area where airway compromise can be lethal, we must extend caution and monitor these patients closely as they may require adjuvant therapy.

Expression of the proto-oncogene eIF4E in inflammation of the oral cavity.

OBJECTIVE: eIF4E (4E) is elevated in 100% of head and neck squamous cell carcinoma (HNSCC) and in premalignant lesions of the larynx. However, it is not elevated in normal mucosa. In this study, we hypothesize that 4E is not significantly elevated in inflammation unlike its expression in premalignant lesions of the oral cavity. STUDY DESIGN: Biopsies from the oral cavity were divided into 5 groups: (1) normal mucosa, (2) chronic inflammation, (3) mild dysplasia from leukoplakic lesions, (4) mild dysplasia in surgical margins of patients with HNSCC, and (5) HNSCC. Immunohistochemical qualitative analysis was then performed. RESULTS: None of the 15 specimens in group 1 and 100% of the 15 specimens in group 5 expressed 4E. Of the 29 specimens in group 2 only 4/29 (13%) overexpressed 4E compared with 10/31 (32%) in group 3 and 9/21 (42%) in group 4. There was a significant difference between groups 2 and 3 and groups 2 and 4 (P < 0.0001 and P < 0.003 respectively) but no significant difference between groups 1 and 2 (P = 0.13) and between groups 3 and 4 (P = 0.30). CONCLUSION: 4E is not significantly elevated in inflammation of the oral cavity thus fulfilling one of the criteria that biomarkers require to be useful in a clinical setting.