Otologic safety of intratympanic N-acetylcysteine in an animal model.

OBJECTIVE: N-acetylcysteine (NAC) is an anti-oxidant and mucolytic effective against bacterial biofilms, making it useful in the treatment of chronically discharging ears that are unresponsive to traditional treatment methods. The objective of this study was to evaluate the otologic safety of intratympanic NAC combined with Ciprodex® in an animal model. METHODS: Baseline distortion product otoacoustic emissions (DPOAE) and auditory brainstem response (ABR) measurements were performed for both ears on thirteen guinea pigs from the animal care research facilities of the McGill University Health Center. This was followed by intratympanic administration of control solution (Ciprofloxacin 0.3%/Dexamethasone 0.1%) to the left ear and experimental solution (1.25% NAC/Ciprofloxacin 0.3%/Dexamethasone 0.1%) to the right ear. Three additional intratympanic injections were performed over the next fourteen days. DPOAE and ABR measurements were repeated 3-4 weeks after the initial procedure. Outcome measures included differences in DPOAE and ABR thresholds after intervention, clinical evidence of vestibular dysfunction and histological evidence of ototoxicity. RESULTS: There were no significant differences in the ABR thresholds and DPOAE results of the control and experimental ears at baseline and after intervention. There was neither clinical manifestation of vestibular dysfunction nor histological evidence of ototoxicity. CONCLUSION: Our study suggests that intratympanic 1.25% NAC with ciprofloxacin and dexamethasone is safe in guinea pigs and support its potential use in the treatment of chronically discharging ears. Further studies in humans are required to analyze its efficacy relative to conventional treatments. LEVEL OF EVIDENCE: Animal Research.

Screening for von Willebrand disease does not impact posttonsillectomy bleeding in a low-risk population.

BACKGROUND: Bleeding is an important complication in children following tonsillectomy. Screening with coagulation tests prior to procedure is common to assess bleeding risk in the perioperative period, although ASH/ASPHO Choosing Wisely guidelines recommend against routine PT/PTT testing. Our aim was to compare von Willebrand factor antigen (VWF:Ag) and activity levels among patients with postoperative bleeding following tonsillectomy to evaluate for potential risk for bleeding. PROCEDURE: Eligible subjects were aged 0-18 without significant personal or family history of major bleeding. Postoperative bleeding diaries were collected and symptoms measured using a postoperative bleeding score. Plasma VWF levels were drawn at time of anesthesia administration. RESULTS: Postoperative bleeding occurred in 248 cases out of 1399 total subjects. Median VWF:Ag was 86 in patients with postoperative bleeding scores of 1-2, 86 for scores 3-4, 84 for scores 5-6, and 83 for scores >6, with no significant difference among groups (p = .98). Additionally, no difference was observed for subjects with multiple days of postoperative bleeding as compared to those with only 1 day of postoperative bleeding. Finally, no difference in VWF:Ag was observed for subjects whose first reported bleed occurred early in the postoperative course compared to those whose first reported bleed occurred later. VWF:Ag does not correlate with severity of bleeding, time of onset of first bleeding event, or recurrence of bleeding in healthy children with no personal or family history of bleeding who have postoperative bleeding following tonsillectomy. CONCLUSIONS: This data does not support routine von Willebrand disease screening prior to tonsillectomy.

Low VWF levels in children and lack of association with bleeding in children undergoing tonsillectomy.

von Willebrand disease is a common bleeding disorder, but diagnosis can be difficult in young children who have not had bleeding challenges. We sought to evaluate the correlation between bleeding and von Willebrand factor (VWF) levels in children undergoing surgical challenge with tonsillectomy. Children ages 0 to 18 undergoing tonsillectomy without a personal or family history of bleeding were enrolled prospectively following informed consent and institutional review board approval. VWF levels were obtained at the time of surgery. VWF antigen (VWF:Ag) and VWF activity (VWF:GPIbM) were tested via enzyme-linked immunosorbent assay. Bleeding score was calculated using the International Society of Hematology bleeding assessment tool (BAT). Surgical and postoperative bleeding were determined using questionnaires filled out by the surgeon and patient/family. A total of 1399 subjects were enrolled with evaluable data, with a median age of 5 years. The median VWF:Ag was 85 IU/dL and the median VWF:GPIbM was 100 U/dL. Median BAT for the entire population was 0, including those with postoperative bleeding. There was no difference in VWF level between those who experienced postoperative bleeding and those who did not, with median VWF:Ag 85 vs 85 (P = .89) and mean VWF:GPIbM 98 vs 100 (P = .5). Interestingly, there was a difference in VWF levels with age, with median VWF:Ag 81 for those younger than 3 years, 82 for those 3 to 6 years, 90 for those 7 to 10 years, and 100 for those 11 to 18 years. A similar trend was noted for VWF:GPIbM. Of the 2 to 6 year olds, 5% had VWF:Ag <50, which would meet criteria for low VWF, but only 1.8% had an abnormal BAT at study entry and only 2.5% bled after surgery. Only 1 subject with low VWF had an elevated postoperative BAT >2. These data suggest that low VWF levels do not correlate with bleeding in children undergoing tonsillectomy. In addition, VWF levels outside the adult normal range in young children may be more common than previously thought and do not necessarily predict surgical bleeding.

Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis.

BACKGROUND: Recurrent Respiratory Papillomatosis (RRP) is a rare disease characterized by the growth of papillomas in the airway and especially the larynx. The clinical course is highly variable among individuals and there is poor understanding of the factors that drive an aggressive vs an indolent course. METHODS: A convenience cohort of 339 affected subjects with papillomas positive for only HPV6 or HPV11 and clinical course data available for 1 year or more, from a large multicenter international study were included. Exploratory data analysis was conducted followed by inferential analyses with frequentist and Bayesian statistics. RESULTS: We examined 339 subjects: 82% were diagnosed prior to the age of 18 years, 65% were infected with HPV6, and 69% had an aggressive clinical course. When comparing age at diagnosis with clinical course, the probability of aggressiveness is high for children under five years of age then drops rapidly. For patients diagnosed after the age of 10 years, an indolent course is more common. After accounting for confounding between HPV11 and young age, HPV type was minimally associated with aggressiveness. Fast and Frugal Trees (FFTs) were utilized to determine which algorithms yield the highest accuracy to classify patients as having an indolent or aggressive clinical course and consistently created a branch for diagnostic age at ~5 years old. There was no reliable strong association between clinical course and socioeconomic or parental factors. CONCLUSION: In the largest cohort of its type, we have identified a critical age at diagnosis which demarcates a more aggressive from less aggressive clinical course.

Safety of Ultrasound-Guided Botulinum Toxin Injections for Sialorrhea as Performed by Pediatric Otolaryngologists.

OBJECTIVE: To evaluate incidence of complications and hospital readmission as a result of ultrasound-guided botulinum toxin injections to manage sialorrhea. STUDY DESIGN: Case series with chart review. SETTING: Children's Hospital of Wisconsin. SUBJECTS AND METHODS: A case series with chart review was performed of all cases of ultrasound-guided injection of botulinum toxin by pediatric otolaryngologists from March 5, 2010, to September 26, 2014,. Primary outcomes included complications such as dysphagia, aspiration pneumonia, and motor paralysis. Secondary outcomes included hospitalization, intubation, and nasogastric tube placement. RESULTS: There were 48 patients, 111 interventions, and 306 intraglandular injections identified. Botulinum toxin type A and type B were utilized in 4 and 107 operative interventions, respectively. Type A was injected into 4 parotid and 4 submandibular glands, utilizing doses of 20 U per parotid and 30 U per submandibular gland. Type B was injected into 98 parotid and 200 submandibular glands, with average dosing of 923 U per parotid and 1170 U per submandibular gland, respectively. There were 2 instances of subjectively worsening of baseline dysphagia that self-resolved. No cases were complicated by aspiration pneumonia or motor paralysis. No patients required hospital readmission, intubation, or nasogastric tube placement. CONCLUSION: Prior published data indicated 16% complication incidence with ultrasound-guided injection of botulinum toxin. Our study found a low complication rate (0.6%) with ultrasound-guided injections of botulinum toxin to manage sialorrhea, without cases of aspiration pneumonia or motor paralysis. Of 306 intraglandular injections, there were 2 cases of worsening baseline subjective dysphagia that self-resolved.

CPCJ 50th Anniversary Editorial Board Commentary: Otolaryngology--then and now.

This review continues the series of articles by the Editorial Board reflecting upon the first volume of the journal published in 1964 and celebrating the 50th anniversary of The Cleft Palate-Craniofacial Journal. This editorial examines the contributions of Otolaryngology-Head and Neck Surgery to interdisciplinary cleft care. The aerodigestive tract has many functions that are impacted by the cleft anomaly. Progress in the development of selected therapies will be reviewed.

Ulcerative lesions as a rare cause of laryngotracheitis in the pediatric population.

OBJECTIVE: The goal of this study is to describe a unique finding of ulcerative lesions of the larynx in two pediatric patients presenting with prolonged acute laryngotracheitis and compare to previously described reports to determine the typical clinical picture, need for intervention, and management model. METHODS: We present two cases of ulcerative lesions of the larynx in immunocompetent children, one with PCR positive HSV, which presented as severe croup requiring intensive care unit admission. Literature review was completed to assess for current knowledge of this entity. Our cases are discussed in the context of previously reported cases of HSV laryngotracheitis. Descriptive analysis was completed focusing on presentation, physical exam findings, treatment, length of therapy, and outcomes. RESULTS: Literature review uncovered six case reports including 10 individual cases of prolonged croup with findings of HSV laryngitis and one retrospective review describing 15 cases of prolonged croup found to be caused by ulcerative laryngitis. All patients underwent direct laryngoscopy and bronchoscopy for evaluation. Analysis was completed comparing the studies to our patients with significant findings including high intubation rate of 77%, ulcerative stomatitis in 63%, and treatment with antiviral medication directed at HSV in 85% with improvement in symptoms. CONCLUSION: It is important to consider HSV as a possible pathogen in cases of prolonged or atypical croup. Laryngoscopy should be used for diagnostic intervention and identification of ulcerative lesions. Stomatitis may be an indication for earlier direct inspection. Treatment with anti-viral therapy and with discontinuation or taper of steroid is suggested.

Outcomes of tonsillectomy in neurologically impaired children.

OBJECTIVES/HYPOTHESIS: Children with neurologically impaired (NI) swallow can undergo tonsillectomy safely and effectively. STUDY DESIGN: Retrospective review. METHODS: Forty-five children with documented dysphagia were compared to age- and procedure-matched normal children for operating room and clinical experience. Three further studies were limited to the NI children only: preoperative and postoperative video swallow studies (VSS), and polysomnography (PSG) for sleep-disordered breathing history. Long-term telephone follow-up of clinical outcomes was performed. RESULTS: There were no differences between the two groups measured by tonsil size or intraoperative or late post-tonsillectomy hemorrhage, but lowest measured oxygen levels were statistically different. No intraoperative complications, early post-tonsillectomy hemorrhage, hospital readmission, or mortality occurred in either group. Three NI children each had an episode of aspiration pneumonia (early or late) without sequelae. Of the 32 VSS available for review, postoperative aspiration incidence was significantly improved, but with new-onset aspiration occurring in five children. Of available matched pre- and postoperative PSG, 91% confirmed resolution of identified preoperative obstructive sleep apnea. Long-term telephone follow-up of 20 NI children revealed improved breathing (95%), communication (90%), and feeding efficiency (55%). CONCLUSIONS: This study suggests tonsillectomy in NI children can be performed safely with appropriate monitoring and precautions with a 48-hour hospital postoperative stay recommended. Swallowing safety appears to improve both objectively and subjectively in most NI children following tonsillectomy. Both preoperative and postoperative VSS are recommended for any NI child undergoing tonsillectomy. Long-term follow-up identified improved quality of life measures for the majority of the NI children queried.

Age of child, more than HPV type, is associated with clinical course in recurrent respiratory papillomatosis.

BACKGROUND: RRP is a devastating disease in which papillomas in the airway cause hoarseness and breathing difficulty. The disease is caused by human papillomavirus (HPV) 6 or 11 and is very variable. Patients undergo multiple surgeries to maintain a patent airway and in order to communicate vocally. Several small studies have been published in which most have noted that HPV 11 is associated with a more aggressive course. METHODOLOGY/PRINCIPAL FINDINGS: Papilloma biopsies were taken from patients undergoing surgical treatment of RRP and were subjected to HPV typing. 118 patients with juvenile-onset RRP with at least 1 year of clinical data and infected with a single HPV type were analyzed. HPV 11 was encountered in 40% of the patients. By our definition, most of the patients in the sample (81%) had run an aggressive course. The odds of a patient with HPV 11 running an aggressive course were 3.9 times higher than that of patients with HPV 6 (Fisher's exact p = 0.017). However, clinical course was more closely associated with age of the patient (at diagnosis and at the time of the current surgery) than with HPV type. Patients with HPV 11 were diagnosed at a younger age (2.4y) than were those with HPV 6 (3.4y) (p = 0.014). Both by multiple linear regression and by multiple logistic regression HPV type was only weakly associated with metrics of disease course when simultaneously accounting for age. CONCLUSIONS/SIGNIFICANCE ABSTRACT: The course of RRP is variable and a quarter of the variability can be accounted for by the age of the patient. HPV 11 is more closely associated with a younger age at diagnosis than it is associated with an aggressive clinical course. These data suggest that there are factors other than HPV type and age of the patient that determine disease course.

Ibuprofen therapy and nasal polyposis in cystic fibrosis patients.

OBJECTIVE: To assess the effects on nasal polyposis from high-dose ibuprofen therapy used in children with cystic fibrosis (CF) pulmonary disease. DESIGN: Retrospective case series. MAIN OUTCOME MEASURE: Presence or absence of nasal polyps. RESULTS: Twenty-two patients treated with high-dose ibuprofen therapy to benefit pulmonary function were identified from 235 patients with CF. Sinonasal disease was present in 19 patients, of whom 12 had nasal polyposis. All 12 patients had observed absence of nasal polyps at some point during their ibuprofen course. Nasal polyps were present in five patients during ibuprofen therapy, and all resolved with increased ibuprofen doses. Polyps occurred in six of eight patients after ibuprofen therapy ceased. Five of the 12 patients required endoscopic sinus surgery for polyposis. CONCLUSION: High-dose ibuprofen therapy chronically administered at appropriate weight-based dosing is a possible treatment option for children and young adults with CF polyposis. More testing is indicated.

Long-term efficacy of intra-oral surgery for sialorrhea.

OBJECTIVES: To investigate the efficacy and quality of life impact of intra-oral surgery for sialorrhea and to provide long-term outcome measures. METHODS: A retrospective review and telephone survey of patients seen in a multi-disciplinary saliva control clinic who underwent surgery between 1999 and 2003. RESULTS: Sixteen of 31 patients underwent 4-duct ligation, 12 patients underwent submandibular gland duct ligation, and three patients underwent a 3-duct ligation procedure. The mean presurgical drooling score (maximum 10) was 9.5, 8.8, and 9, respectively. Recurrence was seen in 68% of patients at a mean of four months. Additional surgery was required in five patients. Phone survey was accomplished in 10 patients. Two of 10 caregivers reported sustained improvement of sialorrhea at two and three years. CONCLUSIONS: Intra-oral surgery provides minimal long-term control of sialorrhea. Additional medical and surgical therapy was needed in a significant portion of patients in this long-term review.

Botulinum toxin as treatment for a unique case of subcutaneous emphysema.

A developmentally delayed 11-year-old male developed extensive spontaneous subcutaneous emphysema 6 weeks after a laryngotracheal separation. Computed tomography demonstrated a small amount of air at the distal end of the laryngeal stump and significant esophageal air. Aerophagia was diagnosed with air presumed diverted through the laryngeal stump due to cricopharyngeal hypertension. Cricopharyngeal botulinum toxin injection was coordinated with a minimal neck dissection to drain the subcutaneous emphysema. The subcutaneous emphysema gradually improved and the patient was discharged home. His subcutaneous emphysema has not returned after four total botulinum toxin injections every 3 months.

Ofloxacin otic drops vs neomycin-polymyxin B otic drops as prophylaxis against early postoperative tympanostomy tube otorrhea.

OBJECTIVES: To evaluate the incidence of tympanostomy tube (TT) sequelae, tube otorrhea, and tube obstruction immediately postoperatively in patients receiving TT for otitis media and to compare patients receiving postoperative otic drops with controls. DESIGN: Blinded randomized control trial. SETTING: A tertiary pediatric otolaryngology practice. SUBJECTS: The study population comprised 306 patients undergoing TT placement. INTERVENTIONS: The 306 patients were enrolled into the following 3 groups: (1) those receiving no postoperative otic drop prophylaxis (control group), (2) those receiving ofloxacin otic drops (FLOX group), and (3) those receiving neomycin sulfate-polymyxin B sulfate-hydrocortisone otic drops (COS group). RESULTS: Overall otorrhea rates postoperatively were 14.9% for the control group, 8.1% for the FLOX group, and 5.5% for the COS group. When controlling for disease severity, the rate of otorrhea was significantly higher for the control group than for both the FLOX (P = .04) and COS (P = .01) groups. Nonpatent, plugged, tube rates were added to otorrhea rates for a TT failure analysis postoperatively. The control group demonstrated a significantly greater failure rate (29.9%) than both the FLOX (12.1%) and COS (7.7%) groups. The only differences between the patients in the 2 groups receiving drops were that ofloxacin was more well liked by patients (P = .04) and caused less pain (P = .004). CONCLUSIONS: Nonpatency and otorrhea are the most frequent sequelae immediately following TT placement. Few studies have compared different treatment regimens in a randomized controlled trial. These results demonstrate that otic drops clearly provide benefit postoperatively in preventing TT plugging and otorrhea but primarily in patients who have middle ear fluid at the time of TT placement. In addition, consideration of drop choice should be based on patient tolerance and medication safety profiles.

Recurrent respiratory papillomatosis in children: masquerader of common respiratory diseases.

BACKGROUND: Recurrent respiratory papillomatosis in children is an uncommon but potentially life-threatening benign tumor of the respiratory tract with laryngeal predilection. The diagnosis of recurrent respiratory papillomatosis may be challenging unless there is a high index of suspicion and awareness of the variable presentations. METHODS: We reviewed the medical charts of children with recurrent respiratory papillomatosis treated at a tertiary children's hospital. The presentation of recurrent respiratory papillomatosis is illustrated by a series of case reports. We provide a paradigm to assist in the early diagnosis of children with recurrent respiratory papillomatosis. RESULTS: Five patients, aged 2 to 6 years, were erroneously diagnosed with recurrent croup, asthma, laryngeal hemangioma, and tracheomalacia after presenting with variable degrees of chronic dyspnea, cough, stridor, dysphonia, weak cry, and syncope. Once the diagnosis of recurrent respiratory papillomatosis was made, recurring surgical ablation of papillomata was initiated. CONCLUSIONS: Any child presenting with a voice disturbance with or without stridor is recommended to have diagnostic flexible fiber-optic laryngoscopy. Recurrent respiratory papillomatosis should be considered in children when other common pediatric airway diseases either do not follow the natural history or do not respond to treatment of the common disorder.

Unique challenges of obtaining regulatory approval for a multicenter protocol to study the genetics of RRP and suggested remedies.

OBJECTIVE: Investigations that seek to generalize findings or to understand uncommon diseases must be conducted at multiple centers. This study describes the process of obtaining regulatory approval for a minimal risk genetic study in a multi-center setting as undertaken by the Recurrent Respiratory Papillomatosis (RRP) Task Force. STUDY DESIGN AND SETTING: Sequential cohort of American children's hospitals. A single protocol was submitted to each Institutional Review Board (IRB). RESULTS: Documentation was prepared for 14 IRBs over 2.5 years. The median time between enlistment and approval at the first 8 sites was 15 months. Institutions varied considerably in their requirements and in the issues that were raised. Protocols were submitted sequentially and accumulated experience was used in the preparation of applications to subsequent IRBs. Nevertheless, there was no correlation between the accumulated experience and the number of issues that were raised. CONCLUSION: Despite uniform federal standards, all local IRBs required unique and individualized submissions. For multicenter studies, investigators should seriously consider the establishment of cooperative authorization agreements. On a simpler level, a standardized format for applications needs to be adopted nationwide. EBM RATING: B-3b.

Bilateral epistaxis in children: efficacy of bilateral septal cauterization with silver nitrate.

OBJECTIVE: To evaluate the rate of control of epistaxis in patients with bilateral complaints using silver nitrate and to assess short-term complications from this therapy. STUDY DESIGN AND SETTING: Retrospective chart review with prospective incident case identification in a tertiary referral pediatric otolaryngology practice. SUBJECTS AND INTERVENTIONS: Forty-six consecutive patients presenting to a single pediatric otolaryngologist with complaints of bilateral epistaxis received bilateral silver nitrate cauterization to the anterior septum and were seen in follow-up 2 months later to assess for control of epistaxis and complications from this therapy. RESULTS: Ninety-three percent (43/46) of patients followed-up at 2 months after therapy. About 93% (40/43) had total or near-total resolution of their epistaxis. Five percent (2/43) of patients returned to the clinic after initial successful control for a second cauterization during the period of study. No significant complications of infection, perforation, long-term crusting, tattooing or mucocutaneous or allergic reactions were identified. CONCLUSIONS: Bilateral epistaxis can be treated at a single visit with bilateral silver nitrate therapy in children. This treatment is highly effective, well-tolerated, and appears to have a low rate of complications.

Four mutations in Epidermodysplasia verruciformis 1 (EVER1) gene are not contributors to susceptibility in RRP.

OBJECTIVE: Epidermodysplasia verruciformis is a skin disease characterized by abnormal susceptibility to human papilloma viruses. Recently four mutations in the Epidermodysplasia verruciformis 1 gene (EVER1, also known as TMC6) have been associated with the disease. Because of the phenotypic similarity between Epidermodysplasia verruciformis and recurrent respiratory papillomatosis, we decided to investigate whether any of these mutations accounts for the susceptibility to human papilloma viruses in subjects with recurrent respiratory papillomatosis (RRP). METHODS: Allele-specific PCR and restriction fragment length polymorphisms (RFLPs) were employed for genotyping a cohort of 101 patients with recurrent respiratory papillomatosis. RESULTS: None of these four mutations were found in the studied subjects. CONCLUSION: The absence of these mutations in RRP patients might indicate that EVER 1 alleles are not associated with susceptibility to RRP, or that other, as yet unidentified, mutations in the Epidermodysplasia verruciformis 1 gene, might account for the susceptibility to RRP.

Pediatric myasthenia gravis and velopharyngeal incompetence.

OBJECTIVE: To determine the clinical course of velopharyngeal incompetence in children with myasthenia gravis (MG). METHODS: A 30-year retrospective study was performed using the medical records of 538 children who presented with velopharyngeal inadequacy (VPI) to a tertiary care academic pediatric center. Children with velopharyngeal incompetence due to myasthenia gravis were identified and their clinical courses were reviewed. RESULTS: Four children were identified with velopharyngeal incompetence associated with myasthenia gravis. All four children required intervention for improvement of speech intelligibility. A speech prosthesis was the uniform intervention. CONCLUSION: Neonatal myasthenia gravis patients should be followed long-term as symptoms may recur as speech impairment. In addition, a high index of suspicion for this entity is required for early diagnosis due to the highly variable presentation and clinical course.

Foreign body granuloma of the external auditory canal.

External auditory canal polyps are most commonly inflammatory in nature but may also manifest more severe disease. Prolonged conservative therapy may delay the correct diagnosis and appropriate intervention. A case is presented of a child with chronic otorrhea treated for 4 months with topical drops and antibiotics. On referral, a large external auditory canal polyp was confirmed to represent a foreign body granuloma covering a large electrical cap, with erosion approaching the facial nerve. External auditory canal polyps that fail to respond promptly to conservative medical therapy warrant a computed tomography scan and surgical exploration with biopsy.

Anterior lingual thyroglossal cyst: antenatal diagnosis, management, and long-term outcome.

OBJECTIVE: To describe the long-term outcome of a large anterior lingual thyroglossal duct cyst, diagnosed antenatally by ultrasound that completely obstructed the oral cavity and had almost completely replaced the anterior two-thirds lingual musculature. METHODS: Longitudinal observation. RESULTS: Anticipated airway obstruction at birth did not occur. Complete cyst removal occurred at day 2 of life with a rim of lingual muscle observed present only on the left side. Complete and safe oral feeding was achieved by day 11 of life. Progressive muscle mass development and function with mild asymmetry was observed during sequential assessments. Vigorous speech therapy was started at age 7 months achieved 80-100% intelligible speech with minimal misarticulations at age 33 months. CONCLUSION: This case demonstrates the remarkable compensatory ability of the tongue to achieve almost normal function with minimal anterior musculature that is critical to deglutition and articulation. Early speech therapy appears key to improving functional outcomes of speech. A strong central nervous system basis for suckle development is suggested by the rapid development of a safe and effective suckle and swallow soon after cyst resection and in spite of in utero tongue fixation.