DOZ047.70: Prevalence of abnormal triple endoscopy findings for tracheoesophageal fistula patients in a multidisciplinary aerodigestive clinic

2019 ◽  
Vol 32 (Supplement_1) ◽  
Author(s):  
S Chowdhuri ◽  
B Osterbauer ◽  
M Bansal ◽  
C Hochstim ◽  
V Bhardwaj

Abstract Introduction Tracheoesophageal fistula (TEF) is a rare congenital defect involving malformations of the esophagus and trachea, which can be life-threatening if left untreated. Children who have undergone TEF repair often present with symptoms such as cough, vomiting, and growth failure. Although the variety of these symptoms implies a necessity for a multidisciplinary approach to care of TEF repair patients, many are still followed by a single discipline such as gastroenterology or pulmonology. The purpose of this study is to describe the experience of Children's Hospital Los Angeles Aerodigestive team in evaluating TEF patients using an interdisciplinary approach. In particular, we aim to explore the findings obtained during triple endoscopy in order to confirm the necessity of care by multiple specialties, ideally in the setting of an Aerodigestive Clinic. Methods Consent was obtained for all children (birth to 18 years) seen in the Children's Hospital Los Angeles (CHLA) Aerodigestive (AERO) Clinic between June 2016 and August 2018. All patients with a diagnosis of TEF were included in the study and data collected included: age, sex, presenting diagnoses and symptoms, and triple endoscopy findings. Results Of the first 109 patients in the AERO Clinic, 18 had TEF. Of these, 7 (39%) were female with a mean age of 3.4 years (SD 3), and 44% were Hispanic. Ten TEF patients underwent triple endoscopy and all had at least one abnormal finding. Six were diagnosed with laryngeal cleft (LC); four type I, one type II, and one was unspecified. Other abnormal findings included: bronchomalacia (7), tracheomalacia (8), esophagitis (4), gastritis (2), and duodenitis (1). Additionally, 7 had an abnormal bronchoalveolar lavage (BAL) finding, with 6 culture positive results. Conclusion TEF patients can present with feeding and respiratory difficulties months to years after their surgical repair. The triple endoscopy findings of TEF patients presented in this study illustrate that symptoms may arise from the gastrointestinal or respiratory tract. Our experience with TEF patients at CHLA indicates that a multidisciplinary approach to evaluation and management is necessary to serve the complex needs of this population.

2008 ◽  
Vol 18 (2) ◽  
pp. 76-86 ◽  
Author(s):  
Lauren Hofmann ◽  
Joseph Bolton ◽  
Susan Ferry

Abstract At The Children's Hospital of Philadelphia (CHOP) we treat many children requiring tracheostomy tube placement. With potential for a tracheostomy tube to be in place for an extended period of time, these children may be at risk for long-term disruption to normal speech development. As such, speaking valves that restore more normal phonation are often key tools in the effort to restore speech and promote more typical language development in this population. However, successful use of speaking valves is frequently more challenging with infant and pediatric patients than with adult patients. The purpose of this article is to review background information related to speaking valves, the indications for one-way valve use, criteria for candidacy, and the benefits of using speaking valves in the pediatric population. This review will emphasize the importance of interdisciplinary collaboration from the perspectives of speech-language pathology and respiratory therapy. Along with the background information, we will present current practices and a case study to illustrate a safe and systematic approach to speaking valve implementation based upon our experiences.


2020 ◽  
pp. 1-12
Author(s):  
Mackenzie Postel M S ◽  
◽  
Julia T Chu ◽  
Henry H Tran ◽  
Shamlal Mangray ◽  
...  

Background: Pediatric salivary gland-type neoplasms (SGTNs) pose a significant diagnostic problem due to histo-morphological heterogeneity. Previous reports have shown that Mucin 4 (MUC4) expression is associated with adult mucoepidermoid carcinoma (MEC). We hypothesize that MUC4 is also a sensitive marker for distinguishing MEC from other SGTNs in the pediatric population. Objective: To evaluate MUC4 expression in pediatric SGTNs. Methods: A retrospective review of 74 SGTNs diagnosed between 1993–2015 at Children’s Hospital Los Angeles, Boston Children’s Hospital, and Rhode Island Hospital was performed. H&E sections of 31 MECs were compared to 3 adenoid cystic carcinomas (AdCCs), 6 acinic cell carcinomas (AcCCs), 30 pleomorphic adenomas (PAs), 3 mammary analogue secretory carcinomas (MASCs), and one sialoblastoma (SB). Samples underwent immunohistochemical staining for MUC4, with expression score criteria: 0% positivity = 0, 1-10% = +, 11-50% = ++, 51-90% = +++, >90% = ++++. Results: All MECs were MUC4-positive, with 25 (80.65%) having an expression score ≥ +++. AdCCs and PAs demonstrated no to minimal MUC4-positivity. Subsets of AcCCs and MASCs were unexpectedly MUC4-positive. As a novel marker for pediatric MEC, MUC4’s sensitivity = 100%, specificity = 79.41%, positive predictive value = 75.86%, and negative predictive value = 100%. Conclusion: MUC4 is a sensitive marker for pediatric MEC


Author(s):  
Yu.O. Volkov ◽  
Ad.A. Mamedov ◽  
L.M. Makarova ◽  
E.A. Ryzhov ◽  
B.A. Tkachenko

The article presents a rare clinical case of hamartoma of the tongue in combination with a cleft palate, diagnosed in a newborn child, as a manifestation of a severe congenital malformation of the maxillofacial region. The preparation and successful implementation of the first stage of treatment of this combined pathology and subsequent rehabilitation in a multidisciplinary children's hospital are described in detail on the basis of continuity in the work of specialists and an interdisciplinary approach.


2018 ◽  
Vol 55 (5) ◽  
pp. 711-720 ◽  
Author(s):  
Zakia Hammal ◽  
Jeffrey F. Cohn ◽  
Erin R. Wallace ◽  
Carrie L. Heike ◽  
Craig B. Birgfeld ◽  
...  

Objective: To compare facial expressiveness (FE) of infants with and without craniofacial microsomia (cases and controls, respectively) and to compare phenotypic variation among cases in relation to FE. Design: Positive and negative affect was elicited in response to standardized emotion inductions, video recorded, and manually coded from video using the Facial Action Coding System for Infants and Young Children. Setting: Five craniofacial centers: Children’s Hospital of Los Angeles, Children’s Hospital of Philadelphia, Seattle Children’s Hospital, University of Illinois–Chicago, and University of North Carolina–Chapel Hill. Participants: Eighty ethnically diverse 12- to 14-month-old infants. Main Outcome Measures: FE was measured on a frame-by-frame basis as the sum of 9 observed facial action units (AUs) representative of positive and negative affect. Results: FE differed between conditions intended to elicit positive and negative affect (95% confidence interval = 0.09-0.66, P = .01). FE failed to differ between cases and controls (ES = –0.16 to –0.02, P = .47 to .92). Among cases, those with and without mandibular hypoplasia showed similar levels of FE (ES = –0.38 to 0.54, P = .10 to .66). Conclusions: FE varied between positive and negative affect, and cases and controls responded similarly. Null findings for case/control differences may be attributable to a lower than anticipated prevalence of nerve palsy among cases, the selection of AUs, or the use of manual coding. In future research, we will reexamine group differences using an automated, computer vision approach that can cover a broader range of facial movements and their dynamics.


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