Masses of the Sternoclavicular Area: Case Series and Review of the Literature.

OBJECTIVE: Masses of the sternoclavicular area are rare, and are not well described in the literature. We aim to present a series of patients with masses in this location and to review all reported English language cases of sternoclavicular masses in pediatric patients. METHODS: This is a case series of pediatric patients with masses of the sternoclavicular area presenting to a tertiary care pediatric hospital from 2010 through 2017. Data was collected by using ICD-9 and ICD-10 codes to query the electronic medical record. Chart review included age at presentation, mass characteristics, medical and surgical interventions, and pathology results. A review of the literature was then performed. RESULTS: Ten patients with masses overlying the sternoclavicular area were identified. Four patients presented with abscess and were treated with incision and drainage. Three of these patients were then treated with staged excision once infection cleared. Two additional patients were treated with primary excision. Four patients were treated with observation. The most common histopathologic finding was epidermoid. One patient was found to have a dermoid cyst, and 1 had a congenital cartilaginous rest. CONCLUSION: Epidermoids and dermoids are the most common masses overlying the sternoclavicular area. Controversy remains regarding the embryologic origin of sternoclavicular masses. The differential for masses in this area also includes branchial remnants, bronchogenic cysts, ganglion cysts, or septic arthritis.

Undifferentiated recurrent fevers in pediatrics are clinically distinct from PFAPA syndrome but retain an IL-1 signature.

Autoinflammatory disorders of the innate immune system present with recurrent episodes of inflammation often beginning in early childhood. While there are now more than 30 genetically-defined hereditary fever disorders, many patients lack a clear diagnosis. Many pediatric patients are often grouped with patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome despite failing to meet diagnostic criteria. Here, we categorize these patients as syndrome of undifferentiated recurrent fever (SURF), and identify the unique features which distinguish them from the PFAPA syndrome. SURF patients were more likely to report gastrointestinal symptoms of nausea, vomiting and abdominal pain, and experienced inconsistent responses to on-demand steroid therapy compared to PFAPA patients. For this previously undefined cohort, an optimal course of therapy remains uncertain, with medical and surgical therapies largely driven by parental preference. A subset of patients with SURF underwent tonsillectomy with complete resolution. Flow cytometric evaluation demonstrates leukocytic populations distinct from PFAPA patients, with reduced CD3+ T cell numbers. SURF patient tonsils were predominantly characterized by an IL-1 signature compared to PFAPA, even during the afebrile period. Peripheral blood signatures were similar between groups suggesting that PFAPA and SURF patient tonsils have localized, persistent inflammation, without clinical symptoms. These data suggest that SURF is a heterogenous syndrome on the autoinflammatory disease spectrum.

Immune Dysregulation in the Tonsillar Microenvironment of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome.

Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis (PFAPA) syndrome is an inflammatory disorder of childhood classically characterized by recurrent fevers, pharyngitis, stomatitis, cervical adenitis, and leukocytosis. While the mechanism is unclear, previous studies have shown that tonsillectomy can be a therapeutic option with improvement in quality of life in many patients with PFAPA, but the mechanisms behind surgical success remain unknown. In addition, long-term clinical follow-up is lacking. In our tertiary care center cohort, 62 patients with PFAPA syndrome had complete resolution of symptoms after surgery (95.3%). Flow cytometric evaluation demonstrates an inflammatory cell population, distinct from patients with infectious pharyngitis, with increased numbers of CD8+ T cells (5.9% vs. 3.8%, p < 0.01), CD19+ B cells (51% vs. 35%, p < 0.05), and CD19+CD20+CD27+CD38-memory B cells (14% vs. 7.7%, p < 0.01). Cells are primed at baseline with increased percentage of IL-1ß positive cells compared to control tonsil-derived cells, which require exogenous LPS stimulation. Gene expression analysis demonstrates a fivefold upregulation in IL1RN and TNF expression in whole tonsil compared to control tonsils, with persistent activation of the NF-κB signaling pathway, and differential microbial signatures, even in the afebrile period. Our data indicates that PFAPA patient tonsils have localized, persistent inflammation, in the absence of clinical symptoms, which may explain the success of tonsillectomy as an effective surgical treatment option. The differential expression of several genes and microbial signatures suggests the potential for a diagnostic biomarker for PFAPA syndrome.

Utility of concurrent direct laryngoscopy and bronchoscopy with drug induced sleep endoscopy in pediatric patients with obstructive sleep apnea.

OBJECTIVES: The goal of this report was to find the frequency of synchronous airway lesions (SAL) identified during microdirect laryngoscopy and bronchoscopy (MDLB) that influenced treatment decisions beyond the information provided by drug induced sleep endoscopy (DISE) alone in children with obstructive sleep apnea (OSA) at a tertiary care pediatric hospital. METHODS: This was a retrospective chart review of all pediatric patients who underwent drug induced sleep endoscopy in conjunction with direct laryngoscopy and bronchoscopy as part of a comprehensive airway evaluation for obstructive sleep apnea at a tertiary care pediatric hospital. RESULTS: Three hundred thirty-five patients with obstructive sleep apnea were evaluated with both sleep endoscopy and direct laryngoscopy with bronchoscopy. Five percent of patients had SAL identified on MDLB contributing to airway obstruction. Three patients (0.9%) who underwent MDLB for OSA required surgical correction of SAL that was identified. CONCLUSION: In a limited subset of patients, direct laryngoscopy with bronchoscopy provides additional positive findings to aid with treatment planning for obstructive sleep apnea.

Diagnosis and treatment of pediatric frontotemporal pits: report of 2 cases.

In contrast to more common nasal and cervical lesions, the frontotemporal pit is a rarely encountered lesion that is often associated with a dermoid and may track intracranially. Due to delays in diagnosis, the propensity to spread intracranially, and the risk of infection, awareness of these lesions and appropriate diagnosis and management are important. The authors present 2 cases of frontotemporal pits from a single institution. Epidemiology, presentation, and management recommendations are discussed.

Functional Electrical Stimulation of the Feline Larynx With a Flexible Ribbon Electrode Array.

OBJECTIVES: Success of laryngeal reanimation through neurorrhaphy has been limited by synkinesis and preoperative muscle atrophy. The objective of this study was to investigate the use of epimysial electrode arrays as a means of delivering electrical stimulation to the posterior cricoarytenoid muscles in order to control laryngeal abduction. METHODS: Ribbon electrode arrays with 4 or 8 electrode contacts were used. Four cats underwent implantation of electrode arrays along the surface of the posterior cricoarytenoid muscles. The glottis was visualized with a 0° telescope while electrodes were stimulated at different amplitudes and pulse-width durations. Recordings of stimulated vocal folds were analyzed, and the degree of vocal fold abduction was measured in order to create recruitment curves for the left and right posterior cricoarytenoid. Recruitment curves from electrode channels within the array were compared. RESULTS: Electrodes oriented along the medial aspect of the posterior cricoarytenoid stimulated graded physiologic degrees of abduction depending on the amplitude of stimulation. Electrodes oriented laterally along the posterior cricoarytenoid stimulated greater degrees of simultaneous adduction with abduction. CONCLUSION: Acute studies of ribbon surface electrode arrays placed onto the posterior cricoarytenoid reproduce graded degrees of abduction necessary for the precise function of respiration and speech.

Nasal endoscopy: new tools and technology for accurate assessment.

In this chapter the technique of nasal endoscopy is discussed. Standardized reporting of nasal endoscopy findings is essential in order to allow communication between different surgeons, speech therapists and endoscopists. Representative videos are provided for the normal examination, for coronal and sagittal velopharyngeal closure patterns, as well as for other anatomical variants of interest. Recommendations for tailoring surgical therapy based on the nasopharyngeal closure pattern are given, although the experience of the surgeon is an important factor for success of any surgical procedure for velopharyngeal insufficiency. Limitations and new frontiers of the technique of nasal endoscopy are also outlined.

Cricoid reduction laryngoplasty for treatment of dysphonia after pediatric laryngotracheal reconstruction.

Dysphonia is common after pediatric laryngotracheal reconstruction (LTR). Surgical techniques designed to rehabilitate the voice after LTR have not yet been proposed. Herein we describe a case of a patient with severe glottal insufficiency after LTR who was treated with a cricoid reduction laryngoplasty in order to surgically rehabilitate the voice. Removal of the posterior costal cartilage graft was effective at improving the quality of the voice and left the patient with an adequate airway even during moderate exercise.

Recurrent contralateral hearing loss after 2 craniotomies for vestibular schwannoma: etiologic implications.

OBJECTIVE: To describe recurrent sudden sensorineural hearing loss after contralateral vestibular schwannoma resection and re-resection. STUDY DESIGN: Clinical capsule report. SETTING: Tertiary academic referral hospital. PATIENT: A patient who underwent 2 craniotomies for vestibular schwannoma. RESULTS: In 2003, a patient experienced contralateral low-frequency sensorineural hearing loss after undergoing translabyrinthine resection of a vestibular schwannoma. This resolved after a course of oral steroids. Seven years later, in 2010, the patient developed tumor recurrence. After retrosigmoid resection, the patient experienced a similar episode of transient, contralateral, low-frequency predominant sensorineural hearing loss. CONCLUSION: The recurrence of contralateral hearing loss after craniotomies years apart suggests that patient specific anatomic risk factors predispose an individual to hearing loss after contralateral cerebellopontine angle surgery. Patients with previous history of contralateral hearing loss should be counseled that they may be at increased risk for recurrent loss in the setting of re-resection.

Supraglottitis in the era following widespread immunization against Haemophilus influenzae type B: evolving principles in diagnosis and management.

OBJECTIVES/HYPOTHESIS: To review the demographics, presentation, interventions, and outcomes of acute supraglottitis in the post-Haemophilus influenzae type B (Hib) vaccination era and make updated recommendations for treatment. STUDY DESIGN: Retrospective review. METHODS: Patients with the discharge diagnosis of acute epiglottitis or supraglottitis from two tertiary hospitals from 1995 to 2005 were identified. Patient characteristics, signs and symptoms at presentation, interventions, hospital course, and outcomes were reviewed and analyzed. RESULTS: Sixty adults and one child were identified. The most common presenting symptom was odynophagia (100%), followed by dysphagia (85%) and voice change (75%). Thirteen patients (21%) required airway intervention; 11 patients were intubated, and two required tracheotomy. Stridor, respiratory distress, tachycardia, tachypnea, rapid onset of symptoms, and shortness of breath were all associated with the need for airway intervention. Patients without any of these symptoms recovered without airway intervention. A total of 62% of patients were admitted to the intensive care unit (ICU), and the average length of ICU stay was 2.3 days. All patients were treated with intravenous antibiotics, most commonly ceftriaxone and ampicillin/sulbactam, and 87% of patients received at least one dose of steroids. The average overall length of stay was 3.8 days. There were no deaths. The use of corticosteroids was associated with shorter ICU and overall lengths of stay. CONCLUSIONS: The patient demographics, presentation, and course of supraglottitis have changed since the widespread use of the Hib vaccine. Recognizing the signs and symptoms associated with airway obstruction is important in the safe and effective management of this condition.