Tracheostomy speaking valve modification in children: A standardized approach leads to widespread use.

BACKGROUND: Tracheostomy is associated with negative effects on voice, speech, and feeding/swallowing. Speaking valves have beneficial effects in these areas but are often contra-indicated in children with airway problems due to high transtracheal pressure (TTP). Valves are modified by drilling to reduce excessive TTP. We hypothesized that a standardized approach to assessment and valve modification by drilling improves valve tolerance and allows widespread successful use. METHODS: Following development of a standardized multidisciplinary protocol for patient selection, valve modification and valve prescription at our center, we retrospectively collected information from clinical notes relating to clinical indication and medical history of all children undergoing speaking valve assessment from February 2014 to June 2017. We designed a questionnaire which was delivered to the parents of children receiving both modified and standard valves enquiring about voice, feeding, communication, and suctioning. RESULTS: Data on 45 children were collected. Thirteen had normal TTP and were given standard valves and 32 had high TTP, all of whom had their valves modified resulting in good tolerance. 17 Children were on positive pressure ventilation at the time of placement. The survey response rate was 83%. Parents report a high degree of satisfaction with modified valves and report positive effects in terms of voice, speech, and feeding/swallowing similar to those reported for standard valves. CONCLUSIONS: Speaking valves can be successfully and safely modified in children, providing valves to many patients previously deemed unsuitable. We report positive parental experiences of these modified valves in line with those reported with standard valves.

International Pediatric Otolaryngology Group: Consensus guidelines on the diagnosis and management of type I laryngeal clefts.

INTRODUCTION: The diagnosis and management of type I laryngeal clefts can be controversial and varies across centers and surgeons. Using existing peer-reviewed literature to develop an expert-based consensus will help guide physicians in the treatment of these patients as well as develop research hypotheses to further study this condition. OBJECTIVE: To provide recommendations for the diagnosis and management of type I laryngeal clefts. METHODS: Determination of current expert- and literature-based recommendations, via a survey of the International Pediatric Otolaryngology Group, using a modified Delphi method. SETTING: Multinational, multi-institutional, tertiary pediatric hospitals. RESULTS: Consensus recommendations include diagnostic workup, medical management, pre-operative, intra-operative and post-operative considerations for type I laryngeal clefts. CONCLUSIONS: This guide on the diagnosis and management of patients with type I laryngeal clefts is aimed at improving patient care and promoting future hypothesis generation and research to validate the recommendations made here.

Prophylactic total thyroidectomy using the minimally invasive video-assisted approach in children with multiple endocrine neoplasia type 2.

BACKGROUND: There have been few reports of prophylactic thyroidectomy using the minimally invasive video-assisted thyroidectomy (MIVAT) approach in children with multiple endocrine neoplasia 2 (MEN2). METHODS: We conducted a retrospective review of a prospectively maintained database of patients who underwent MIVAT for total thyroidectomy. RESULTS: Six children underwent MIVAT; RET codon mutations identified were 634, 620, 611, and 918. Mean operative time was 93 minutes (range, 68-105 minutes). Five patients were discharged on the first postoperative day; however, 1 patient had a postoperative hematoma and was discharged 2 days postoperatively. There were no cases of laryngeal nerve palsy or postoperative hypoparathyroidism. High levels of satisfaction with postoperative cosmesis were reported. Calcitonin levels have been undetectable at follow-up thus far (mean follow-up, 42.8 months). CONCLUSION: Although our outcomes are similar to those reported using the traditional approach, it is important to note that MIVAT is essentially the same operation, just performed through a smaller incision, with resulting benefits in terms of pain, cosmesis and, perhaps, morbidity.

Extrinsic bronchial compression due to patent ductus arteriosus closure device.

Interventional cardiology provides a valuable nonoperative approach for the modern management of patent ductus arteriosus (PDA) in patients with non-complex congenital heart disease. We describe a patient with a right-sided aortic arch who developed severe bronchomalacia after PDA device closure that necessitated extensive surgical repair. Consequently, we advise that in infants with a right-sided aortic arch and PDA inserting into the right pulmonary artery, device closure is challenging due to the potential risk of bronchial compression and subsequent development of bronchomalacia. Consideration should be given to surgical closure or use of a softer duct occlusion device.

Subglottic injury: a clinically relevant animal model.

OBJECTIVES/HYPOTHESIS: To develop a clinically aligned, reproducible model for subglottic injury. STUDY DESIGN: Prospective randomized control pilot study. METHODS: Juvenile (3-month-old) New Zealand White rabbits underwent intubation with a 3-cm length of an endotracheal tube that was chosen so that there would be no air leak below 20 cm of water. This tube was one or two sizes above the appropriate tube for the animal. It was held in situ with a suture placed at the trachea and secured over a button in the neck for a period of 1 week. Animals were sacrificed 1 week postextubation, and larynges were harvested. A range of histological techniques and gross morphology were utilized to examine the injury caused at the level of the subglottis. Unintubated animals constituted controlled specimens. RESULTS: Intubated animals demonstrated considerable histopathology including evidence of ulceration, inflammation, granulation tissue, perichondritis, and chondritis when compared with control animals. Morphometric analysis demonstrated a significant increase in lamina propria thickness (P = .0013), mucosal thickness (P ≤ .0001), and in goblet cell areal density (P = .014). Analysis of mucin types found a significant decrease in acidic (P = .0001) mucin coinciding with a significant increase in mixed mucin types (P = .0013). CONCLUSIONS: Our model provides a reliable and reproducible technique for acute/subacute injury to the subglottis secondary to intubation, which is consistent with previous histological findings of early changes associated with acquired subglottic stenosis (SGS). Future uses of this model could include the examination of current adjunctive therapies and their effects on limiting progression to SGS.

Reversal of laryngotracheal separation in paediatric patients.

OBJECTIVE: Laryngotracheal separation (LTS) is an effective and reliable definitive treatment for intractable aspiration. A major advantage of this treatment for intractable aspiration is its' potential reversibility. Should the underlying disorder improve, a reversal of the procedure may be attempted. This has been successfully achieved in the adult population. To our knowledge, no previous cases have been reported of successful reversal of LTS in children. METHODS: A retrospective review from 2003 to 2010 identified four cases of intractable aspiration treated with LTS in our department. Two of these patients displayed objective evidence of sufficient recovery of their underlying aspiration to consider reversal. Patient selection for reversal was dependent upon successful oral intake for 9 months along with videofluoroscopic evidence of normal or minimally impaired swallow. RESULTS: Two children who were successfully treated for intractable aspiration with LTS demonstrated objective evidence of recovery sufficient to attempt reversal. Both children underwent successful surgical reversal of LTS using a cricotracheal resection with end-to-end anastamosis, similar to that used in treatment of subglottic stenosis. Both children can now tolerate oral diet and their speech and language development is in line with their overall developmental level. CONCLUSIONS: Laryngotracheal separation is an effective and reliable definitive treatment for intractable aspiration facilitating protection of the airway and allowing safe swallowing with unimpeded respiration, but with the major drawback of loss of phonation. To our knowledge, we document the first two cases of successful LTS reversal in children.

Use of the laryngeal microdebrider blade in treating Teflon-induced vocal fold granuloma: a case report.

Vocal fold granuloma induced by polytef (Teflon) injection is rare. This condition requires surgical intervention in order to restore the voice. In this report, we discuss the use of an endoscopic laryngeal microdebrider blade in successfully treating this condition. To the best of our knowledge, this procedure has not been previously described in the literature.

Invasive rhino-maxillary mucormycosis diagnosed before HSCT.

We report a case of successful allogeneic hematopoietic stem cell transplantation (HSCT) with full myeloablative conditioning in a patient with pre-existing invasive mucormycosis. The mucormycosis involved the maxilla, the nasal septum, and the hard palate. Sustained antifungal therapy and aggressive surgery both before and after HSCT were required.

Intractable pulmonary aspiration in children: which operation?

Intractable aspiration is a life-threatening medical problem. There are many surgical procedures for the treatment of intractable aspiration. Our experience with three children was reported here. After failing initial conservative measures, the first two patients had supraglottic laryngeal closure performed through an endoscopic approach with a small hole left superiorly for phonation. However, both of them developed dehiscence of the closure postoperatively, necessitating a more definitive procedure to stop the aspiration. Their symptoms of aspiration were only relieved after they underwent laryngotracheal separation. In the third child, laryngotracheal separation was performed as the initial procedure and the symptoms of recurrent aspiration were relieved. In conclusion, the treatment of intractable aspiration requires a definitive procedure from the outset and we would suggest, based on literature review and on our experience, that laryngotracheal separation is the most effective and reliable option, albeit lack of phonation is inevitable. A comprehensive review of all procedures used for treatment of this condition was also undertaken.

Detection of bone marrow micrometastases in the rib marrow of head and neck cancer patients: a prospective pilot study.

Flow cytometry has been validated as an objective method of demonstrating and quantifying micrometastases. Micrometastases within bone marrow indicate a poor prognosis in patients with upper gastrointestinal, colorectal and breast epithelial tumours. We prospectively sought to assess the feasibility of testing rib marrow for bone marrow micrometastases in head and neck cancer and to report their frequency in a cohort of patients. Nine patients were enrolled in the study. Bone marrow was obtained before manipulation of the primary tumour. Micrometastatic cells were detected by staining contaminant cytokeratin-18 positive cells and using the twin techniques of immuncytochemistry and flow cytometry. Cellular marrow was retrieved in 100% of cases. Micrometastases were detected in one out of nine epithelial tumours on both flow cytometry and immunocytochemistry. The detection rate appeared to be independent of TN staging. We were unable to culture the cells. Preoperative detection of bone marrow micrometastases may reflect transient shedding of cells, metastatic potential or residual disease. This prospective study confirms the feasibility of using rib marrow in future studies investigating micrometastases in head and neck cancer.

Multiple airway abnormalities in a patient with Rothmund-Thomson syndrome.

Rothmund-Thomson syndrome (RTS) is a rare autosomal recessive disorder that is characterized by skin manifestations, juvenile cataracts, and extracutaneous manifestations. The presence of congenital airway abnormalities has not previously been documented in Rothmund-Thomson syndrome. We report the case of a child with Rothmund-Thomson syndrome suffering from multiple airway abnormalities. Our case highlights the importance of suspecting multiple airway abnormalities in any child with respiratory distress with other systemic anomalies. This necessitates meticulous anatomical and dynamic examination of the laryngotracheo-broncheal tree.

Tympanosclerosis in the rat tympanic membrane: an experimental study.

OBJECTIVES/HYPOTHESIS: Tympanosclerosis is a pathologic condition affecting the middle ear and tympanic membrane. It is a common condition in humans, most notably after grommet insertion. However, the pathogenesis of this disease is unclear. The aim of this study was to investigate the development and progression of tympanosclerosis in the pars tensa of rat tympanic membranes at various time intervals after inducing sterile middle ear effusions. METHODS: Fifty-six male-specific, pathogen-free CD Wistar rats (100-170 g) underwent unilateral eustachian tube obstruction. Contralateral ears served as controls. Only specimens from animals with sterile effusions were included in this study. Light and electron microscopic analysis was performed on the pars tensa of rats with induced effusions ranging from 1 to 12 months. RESULTS: Histologic changes consistent with tympanosclerosis were seen in the majority of animals with effusions from 3 months and greater. The process started in the submucosal connective tissue layer and progressed to involve all connective tissue sublayers. The extent of calcium deposition and fibrosis across the membrane was related to the duration of otitis media with effusion. Atrophy was not found in any specimens. CONCLUSIONS: Tympanosclerosis is a progressive disorder, which appears to be the main response of the rat pars tensa to prolonged sterile otitis media with effusion. The extent of involvement of the different sublayers of the pars tensa was closely related to the duration of otitis media with effusion. We propose that this new rat model for prolonged otitis media with sterile effusion is therefore a suitable animal model for the study of tympanosclerosis in the pars tensa.

Complete vascular ring detected by barium esophagography.

The diagnosis of a vascular ring can be made on the basis of characteristic findings on barium esophagography. We report a case of a double aortic arch in a 9-month-old girl that was diagnosed in this manner, and we briefly review the anatomic characteristics of vascular rings.