Jordan Virbalas

The Role of Tonsillectomy in the Treatment of Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS)

IMPORTANCE Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) in children describes neuropsychiatric symptom exacerbations that relate temporally to streptococcal infections. Recent case reports suggest tonsillectomy may effectively reduce these symptoms; however, no consensus treatment guidelines exist. This study examines whether tonsillectomy improves neuropsychiatric symptoms in children with PANDAS who have incomplete response to antibiotic therapy. OBSERVATIONS Ten patients met strict diagnostic criteria for PANDAS. Comparisons were made between parental reports of symptom severity at diagnosis, after antibiotic treatment (in 10 patients), and after tonsillectomy (in 9). From a baseline severity score of 10, antibiotics alone improved symptoms to a median (interquartile range [IQR]) score of 8 (6.5-10.0) (P = .03). Nine children who subsequently underwent tonsillectomy reported symptom improvement in comparison with treatment with antibiotics alone, including those with no response to antibiotics. Symptom severity improved at all periods after tonsillectomy compared with antibiotics alone. The median score [IQR] 3 months postoperatively was 3 (0.0-6.5) (P = .01); 6 months postoperatively, 3 (0.0-5.0) (P = .02); 1 year postoperatively, 3 (0.0-5.0) (P = .02); and 3 years postoperatively, 0.5 (0.0-2.3) (P = .03). Four of the 9 had complete resolution after tonsillectomy. CONCLUSIONS AND RELEVANCE This PANDAS cohort whose neuropsychiatric symptoms did not respond sufficiently to antibiotics may have gained benefit from tonsillectomy.

Pectus excavatum in children with laryngomalacia

BACKGROUND Laryngomalacia is the most common congenital laryngeal anomaly and is associated with several disorders including gastric reflux, sleep apnea, hypotonia and failure to thrive. Pectus excavatum (PE) is the most common chest wall deformity affecting 1-300/1000 individuals. Though many authors presume a relationship between PE and laryngomalacia, there is no published data to establish this association. GOAL To test the hypothesis that patients referred to our pediatric otolaryngology clinic for evaluation of laryngomalacia exhibit higher rates of PE than the general population. METHODS Retrospective review of prospectively enrolled children who presented with laryngomalacia (January 2008-June 2012) to a tertiary care, hospital based, pediatric otolaryngology practice. Each chart was examined for a concurrent diagnosis of pectus deformity. RESULTS Of the 137 laryngomalacia patients, 9 (6.6%) had documented PE. This represents a significantly increased rate of PE when compared to children without laryngomalacia (p = 0.001). Four of the 9 children with PE underwent supraglottoplasty for laryngomalacia, a significantly greater proportion than the 9/128 of the children with isolated laryngomalacia who underwent supraglottoplasty (p = 0.004). CONCLUSIONS This study suggests an association between laryngomalacia and PE. Pediatric otolaryngologists should be cognizant of this relationship, though further studies are needed to elucidate the nature of this association.

Upper Airway Obstruction

1. Jordan Virbalas, MD* 2. Lee Smith, MD† 1. *Department of Otorhinolaryngology–Head and Neck Surgery, Albert Einstein College of Medicine, Bronx, NY. 2. †Department of Otolaryngology, Steven and Alexandra Cohen Children’s Medical Center, Hofstra North Shore LIJ School of Medicine, New Hyde Park, NY. * Abbreviations: BT: : bacterial tracheitis CT: : computed tomography HIB: : Haemophilus influenzae type B The differential diagnosis of upper airway obstruction in children includes infectious and noninfectious causes (Table). When evaluating a child with stridor, the clinician must know how to differentiate between various anatomical anomalies (laryngomalacia, tracheomalacia, and subglottic stenosis) and infectious conditions (croup, epiglottitis, and bacterial tracheitis) to promptly implement appropriate management. View this table: Table. Common Causes of Upper Airway Obstruction After completing this article, readers should be able to 1. Know that upper respiratory tract infections and airway obstruction in young infants can lead to respiratory distress. 2. Know the clinical presentation of laryngomalacia. 3. Know the risks of examination of patients with suspected epiglottitis. 4. Know how to treat a child with epiglottitis. 5. Know the clinical manifestations of laryngotracheitis (croup). 6. Know the appropriate management of croup. 7. Differentiate the clinical and radiographic findings of viral croup from those of epiglottitis and bacterial tracheitis. 8. Distinguish between viral and noninfectious croup. 9. Recognize the signs and symptoms of bacterial tracheitis. 10. Know the typical clinical course of bacterial tracheitis, including biphasic illness, precipitous worsening, requirement for intubation, and relatively prolonged intubation. 11. Know the treatment of bacterial tracheitis. 12. Know the microbiology of bacterial tracheitis. 13. Know that tracheomalacia can occur as a complication of long-term mechanical ventilation in children. 14. Know that tracheoesophageal fistula may result in tracheomalacia. 15. Know the clinical manifestations of tracheomalacia. ### Definitions Laryngomalacia refers to the prolapse of supraglottic structures into the laryngeal airway on inspiration, which usually manifests as a primarily inspiratory stridor in young children. ### Introduction Laryngomalacia is the most common congenital laryngeal anomaly and is the most frequent congenital cause of stridor in infants. Often symptoms are not present at birth, and affected children typically develop stridor in the first 2 …

Laryngotracheal Reconstruction Outcomes in Hypotonic Children

IMPORTANCE Children with poor muscle tone may demonstrate upper airway obstruction due to several mechanisms including obstructive sleep apnea, laryngopharyngeal reflux, and laryngomalacia. Though hypotonia has been shown to compromise the pediatric airway, and some authors suggest that neurologic deficits can compromise the success of laryngotracheal reconstruction (LTR), to our knowledge no studies have evaluated the effect of neurologic diagnoses or hypotonia on outcomes in LTR. OBJECTIVE To determine whether hypotonic children with subglottic stenosis have lower rates of successful decannulation after LTR compared with children without neurologic deficit. DESIGN, SETTING, AND PARTICIPANTS A retrospective medical chart review was conducted for 27 children aged 0 to 6 years, who underwent LTR for subglottic stenosis between December 2007 and December 2012 at a tertiary care childrens hospital. Children were classified based on documented neurologic findings. Group 1 comprised those children without neurologic impairment (n = 16). Group 2 included those children with a documented neurocognitive or neuromuscular diagnosis but without evidence of hypotonia (n = 7). Group 3 comprised hypotonic children (n = 4). INTERVENTIONS Laryngotracheal reconstruction. MAIN OUTCOMES AND MEASURES The number of procedures performed after LTR to optimize the airway and whether the child was successfully decannulated. RESULTS All 16 of the neurologically intact patients (100%) were decannulated. Among children with a neurologic deficit, 5 of 7 (71%) were ultimately decannulated. No hypotonic children 0 of 4 were decannulated. The difference in rates of decannulation between unaffected and normotonic children with a neurologic deficit was not statistically significant (P = .08). However, the difference in outcomes between hypotonic children and neurologically intact patients was statistically significant (P < .001). CONCLUSIONS AND RELEVANCE Findings from this study suggest that hypotonic children may experience poorer rates of post-LTR decannulation compared with children without neurologic deficit. Dynamic upper airway obstruction may be unappreciated in hypotonic children. Future research may be directed at the appropriate evaluation and treatment of children with poor muscle tone and subglottic stenosis.

Vascular Anomalies in Otolaryngology–Head and Neck Surgery Resident Education:

The evaluation and treatment of vascular anomalies is rapidly evolving. In recent years, improved imaging, medical therapies, interventional radiology procedures, and technical advances have led to improved functional and aesthetic outcomes with reduced morbidity. With management of vascular anomalies becoming increasingly complex, we wanted to assess the opinions of otolaryngology–head and neck surgery resident trainees regarding education in this evolving subspecialty. The results of our survey show that a significant majority of trainees feel that vascular anomalies are best managed by a multidisciplinary team, consistent with practice in large vascular anomalies centers. While training in this area does not seem to be deficient, it may be helpful to identify those otolaryngology residents who are interested in gaining exposure to patients with vascular anomalies, so that they may seek additional subspecialty experiences to complement their otolaryngology training.

Injection laryngoplasty in children with cystic fibrosis and abnormal swallow.

OBJECTIVE Children with cystic fibrosis (CF) are uniquely vulnerable to the pulmonary complications of chronic aspiration. We present a case series of children with CF and evidence of chronic aspiration who underwent injection laryngoplasty to improve the safety and efficacy of their swallow. STUDY DESIGN Retrospective chart review. SETTING Tertiary care childrens hospital. METHODS/SUBJECTS A chart review was performed on three consecutive cases of children with CF and evidence of aspiration on modified barium swallow (MBS) evaluated at a tertiary care, academic childrens medical center. RESULTS Three patients with CF underwent injection laryngoplasty for evidence of aspiration or laryngeal penetration on MBS evaluation. Normal laryngeal anatomy was identified intraoperatively in each case. At the time of the procedure, patients were 22 months, 70 months, and 24 months old, and follow-up information was available for 7 months, 11 months, and 12 months post-procedure, respectively. Presenting symptoms included chronic cough, cough with oral liquids, and recurrent pneumonia. Each patient underwent successful injection into the interarytenoid space. Post-operatively, modified barium swallow demonstrated resolution of aspiration or penetration in all patients. No procedure-related complications were encountered. CONCLUSION Patients with CF are highly susceptible to pulmonary infections, and aggressive treatment of chronic aspiration is often necessary. Injection laryngoplasty may be effective in normalizing swallowing in these children. Future study will elucidate the duration of effect and if this technique improves long-term pulmonary outcomes in CF patients.

Obstacles to communication in children with cri du chat syndrome

BACKGROUND Cri du chat syndrome (CCS) is a genetic disorder resulting from the deletion of the short arm of chromosome 5. Perhaps the most distinctive characteristic of this syndrome is the congenital high-pitched cry, which frequently brings these patients to the attention of an otolaryngologist. Speech and language development in children with CCS is notable for a reduced receptive vocabulary and a profound deficit in expressive language. Currently, no clear guidelines have been established for the treatment of the speech and language difficulties exhibited by these patients. In this article, we present a case report and discuss the current literature regarding the challenges to effective communication in CCS. METHODS Case report. CASE We present a 7-year-old girl with CCS who sought help to improve her ability to communicate. The patient presented with a persistent high-pitched voice unchanged since birth and a breathy dysphonia. Findings on examination were significant for an abnormally oriented larynx with atrophic vocal folds. She continues to undertake intensive speech therapy to assist in her language development. CONCLUSION CCS is a genetic disorder that universally results in profound deficits in expressive speech. Although patients with CCS commonly present with a high-pitched voice and marked laryngeal abnormalities, they are unlikely to benefit from surgical intervention. Speech and language therapy, including augmentative communication devices, may enhance effective communication and improve the quality of life of these patients.

Radiology quiz case 1. Diagnosis: Parathyroid cyst (PC).

A 50-YEAR-OLD WOMAN PRESENTED TO THE otolaryngology clinic after the incidental finding of a mass in the lower part of the left side of her neck. Because she had been unaware of the mass until a recent visit to her primary care physician, she did not know how long the mass had been present or if it had changed in size. She reported no symptoms associated with the mass. She denied shortness of breath, dysphagia, hoarseness, and pain. She also had no hypothyroid or hyperthyroid symptoms. A review of systems revealed no abnormalities. She was in good general health, with no other notable medical, surgical, or family history. Physical examinationshowedthat thecenterof themass was located approximately 4 cm to the left of the midline and 3 cm below the level of the cricoid cartilage. The mass was firm,minimallymobileonswallowing,andnontender. Therewasnolocalwarmthorerythemaoftheoverlyingskin. Nobruitswereappreciated.Theresultsof therestof thehead and neck and general physical examinations were normal. Magnetic resonance imaging of the neck (Figure 1 and Figure 2) revealed a cystic mass in the lower part of the left side of the neck that measured approximately 4.0 3.0 2.3 cm. Ultrasonography of the neck confirmed the presence of a uniloculated cyst in the region of the left lower lobe of the thyroid gland (Figure 3). What is your diagnosis?