Publications by authors named "Ellen M Friedman"

35 Publications

Volumetric brain magnetic resonance imaging analysis in children with obstructive sleep apnea.

Int J Pediatr Otorhinolaryngol 2020 Nov 7;138:110369. Epub 2020 Sep 7.

Department of Otolaryngology and Head and Neck Surgery, Baylor College of Medicine, Houston, TX, USA; Department of Pediatric Otolaryngology and Head and Neck Surgery, Texas Children's Hospital, Houston, TX, USA.

Objectives: Pediatric Obstructive Sleep Apnea (OSA) is associated with neurocognitive deficits. Cerebral structural alterations in the frontal cortex, cerebellum, and hippocampus have been reported in adult OSA patients. These brain areas are important for executive functioning, motor regulation of breathing, and memory function, respectively. Corresponding evidence comparing cerebral structures in pediatric OSA patients is limited. The objective of this study is to investigate MRI differences in cortical thickness and cortical volume in children with and without OSA.

Study Design: Prospective, single institutional case-control study.

Methods: Forty-five children were recruited at a pediatric tertiary care center (27 with OSA; mean age 9.9 ± 1.9 years, and 18 controls; mean age 10.5 ± 1.0 years). The OSA group underwent magnetic resonance imaging (MRI), polysomnography (PSG) and completed the Pediatric Daytime Sleepiness Scale (PDSS) and the Child's Sleep Habits Questionnaire (CSHQ). High-resolution T1-weighted MRI was utilized to examine cortical thickness and gray and white matter volume in children with OSA compared to a healthy group of demographically-comparable children without OSA selected from a pre-existing MRI dataset.

Results: Children with OSA showed multiple regions of cortical thinning primarily in the left hemisphere. Reduced gray matter (GM) volume was noted in the OSA group in multiple frontal regions of the left hemisphere (superior frontal, rostral medial frontal, and caudal medial frontal regions). Reduced white matter (WM) volume in both the left and right hemisphere was observed in regions of the frontal, parietal, and occipital lobes in children with OSA.

Conclusion: This study noted differences in cortical thickness and GM and WM regional brain volumes in children with OSA. These findings are consistent with other pediatric studies, which also report differences between healthy children and those with OSA. We found that the severity of OSA does not correlate with the extent of MRI alterations.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ijporl.2020.110369DOI Listing
November 2020

Professionalism, Quality, and Safety for Pediatric Otolaryngologists.

Otolaryngol Clin North Am 2019 Oct 5;52(5):969-980. Epub 2019 Aug 5.

Department of Otolaryngology, Head and Neck Surgery, UT Southwestern Medical Center, Dallas, TX, USA. Electronic address:

Professionalism, quality, and safety have become essential components of pediatric otolaryngology. Professionalism, as defined by Osler, refers to the long tradition of physicians carrying out the noble cause of providing health care to patients and families. The importance of professionalism cannot be overstated and now is widely understood to be a core competency of every practicing physician. The attention to quality and safety is also a central tenet of current surgical practice. Quality is doing the right thing at the right time for the right persons. Safety is providing care to patients that is free from undue harm.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.otc.2019.06.010DOI Listing
October 2019

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

PLoS One 2019 13;14(6):e0216697. Epub 2019 Jun 13.

Departments of Microbiology and Immunology, and Otolaryngology-Head and Neck Surgery and Center for Genomic Sciences, Institute of Molecular Medicine and Infectious Disease, Drexel University College of Medicine, Philadelphia, Pennsylvania, United States of America.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0216697PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6563955PMC
February 2020

Optimizing Performance by Preventing Disruptive Behavior in Radiology.

Radiographics 2018 10;38(6):1639-1650

From the Department of Radiology (M.H.W.) and Center for Professionalism in Medicine (E.M.F.), Baylor College of Medicine, 1 Baylor Plaza, Houston, TX 77030; and Department of Radiology, Lucile Packard Children's Hospital at Stanford, Palo Alto, Calif (L.F.D.).

Disruptive behaviors impede delivery of high-value health care by negatively impacting patient outcomes and increasing costs. Health care is brimming with potential triggers of disruptive behavior. Given omnipresent environmental and cultural factors such as constrained resources, stressful environments, commercialization, fatigue, unrealistic expectation of perfectionism, and burdensome documentation, a burnout epidemic is raging, and medical providers are understandably at tremendous risk to succumb and manifest these unprofessional behaviors. Each medical specialty has its own unique challenges. Radiology is not exempt; these issues do not respect specialty or professional boundaries. Unfortunately, preventive measures are too frequently overlooked, provider support programs rarely exist, and often organizations either tolerate or ineffectively manage the downstream disruptive behaviors. This review summarizes the background, key definitions, contributing factors, impact, prevention, and management of disruptive behavior. Every member of the health care team can gain from an improved understanding and awareness of the contributing factors and preventive measures. Application of these principles can foster a just culture of understanding, trust, support, respect, and teamwork balanced with accountability. The authors discuss these general topics along with specific issues for radiologists in the current medical environment. Patients, providers, health care organizations, and society all stand to benefit from better prevention of these behaviors. There is a strong moral, ethical, and business case to address this issue head-on. RSNA, 2018.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1148/rg.2018180019DOI Listing
October 2018

Twelve tips for the introduction of emotional intelligence in medical education.

Med Teach 2019 07 21;41(7):746-749. Epub 2018 Jul 21.

b Center for Professionalism , Baylor College of Medicine , Houston , Texas , USA.

Emotional intelligence (EI) is the ability to recognize, understand, and manage emotions in yourself and in others. EI has long been recognized as a critical component for individual and organizational success within the business realm, and there is emerging evidence that enhancing EI is equally important in the medical setting. EI can improve interpersonal communications, enable constructive conflict resolution, and promote a culture of professionalism. As healthcare becomes increasingly team-based, proficiency in EI will be required to build consensus among multidisciplinary stakeholders, and effect change in attitudes and behaviors that result in improved patient safety and clinical outcomes. Based on the existing literature and the authors' experiences, these 12 tips provide practical suggestions on how to introduce EI into a medical curriculum. These tips have broad applicability, and can be implemented in courses on topics such as professionalism, leadership development, empathy, patient safety, or wellness.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1080/0142159X.2018.1481499DOI Listing
July 2019

Emailing Test Results to Patients-Results.

Authors:
Ellen M Friedman

JAMA 2016 Sep;316(12):1319

Center for Professionalism in Medicine, Baylor College of Medicine, Houston, Texas.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1001/jama.2016.12251DOI Listing
September 2016

Removal of Foreign Bodies from the Ear and Nose.

Authors:
Ellen M Friedman

N Engl J Med 2016 07;375(2):194

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1056/NEJMc1603663DOI Listing
July 2016

A PIECE OF MY MIND. You've Got Mail.

Authors:
Ellen M Friedman

JAMA 2016 Jun;315(21):2275-6

Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, and Center for Professionalism in Medicine, Baylor College of Medicine, Houston, Texas.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1001/jama.2016.1757DOI Listing
June 2016

A Five-Year Analysis of Airway Foreign Body Management: Toward a Better Understanding of Negative Bronchoscopies.

Ann Otol Rhinol Laryngol 2016 Jul 17;125(7):591-5. Epub 2016 Mar 17.

Laryngology Fellow, The Emory University School of Medicine, Atlanta, Georgia, USA.

Objective: To identify characteristics in patients who undergo positive and negative bronchoscopy for a suspected airway foreign body (AFB).

Methods: Review medical records between 2008 and 2012.

Results: There were 145 patients who went to the operating room with the pre-bronchoscopy diagnosis of suspected AFB during the study period. There was an overall negative bronchoscopy rate of an average of 37%, with an annual range between 21% and 50%. The findings of history or suspicion of choking, asymmetric breath sounds, and wheezing were statistically more common in patients with an AFB. Chest roentograms (CXR) had a sensitivity and specificity of 62% and 57%. Twenty patients had a chest computed tomography (CT) scan, and 100% were clinically significant. Four CT scans were diagnostic of an AFB, and 16 patients avoided bronchoscopy after negative CT.

Conclusions: In current clinical practice, it is difficult to identify patients with an AFB without performing bronchoscopy. This results in a significant number of negative bronchoscopes. Certain elements in the history and physical exam were more common in patients who were found to have an AFB. Our preliminary data suggest that chest CT scans may be useful to decrease the number of negative bronchoscopies.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/0003489416637387DOI Listing
July 2016

VIDEOS IN CLINICAL MEDICINE. Removal of Foreign Bodies from the Ear and Nose.

Authors:
Ellen M Friedman

N Engl J Med 2016 Feb;374(7):e7

From the Texas Children's Hospital and Baylor College of Medicine, Houston.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1056/NEJMvcm1207469DOI Listing
February 2016

Successful endoscopic ablation of a pyriform sinus fistula in a child: case report and literature review.

Pediatr Surg Int 2016 Jun 28;32(6):623-7. Epub 2016 Jan 28.

Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, 6701 Fannin Street, Suite 1210, Houston, Texas, 77030, USA.

Recurrent thyroid infections are rare in children. When present, patients should be evaluated for anatomic anomalies such as pyriform sinus fistulae. We describe a 12-year-old girl with history of recurrent thyroid abscesses secondary to a pyriform sinus fistula and managed with concurrent endoscopic ablation and incision and drainage.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00383-016-3868-3DOI Listing
June 2016

Outcomes variability in non-emergent esophageal foreign body removal: Is daytime removal better?

Int J Pediatr Otorhinolaryngol 2015 Oct 29;79(10):1630-3. Epub 2015 Jun 29.

Baylor College of Medicine, Texas Children's Hospital Director of the Center for Professionalism in Medicine, United States. Electronic address:

Objective: The objective of this study is to investigate differences between esophageal foreign body removal performed during standard operating room hours and those performed after-hours in asymptomatic patients.

Methods: A retrospective chart review at a tertiary children's hospital identified 264 cases of patients with non-emergent esophageal foreign bodies between 2006 and 2011. Variables pertaining to procedure and recovery times, hospital charges, complications, length of stay, American Society of Anesthesiology (ASA) classification, and presence of mucosal injury were summarized and compared between cases performed during standard operating hours and those performed after-hours.

Results: Cases performed during standard hours had significantly longer average wait times compared with after-hours cases (13.1h versus 9.0h, p<0.001). No other clinical characteristics or outcomes were significantly different between groups. Longer wait times are not associated with mucosal injury or postoperative complications.

Conclusion: There were no significant differences in procedure time, charges, or safety in after-hours removal of non-emergent esophageal foreign bodies compared to removal during standard operating hours. OR wait time was about 4h longer during standard hours compared with after-hours. This study could not assess the factors to determine the impact in differences in hospital resource utilization or work force, which may be significant between these two groups.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ijporl.2015.06.027DOI Listing
October 2015

A cooperative approach to diagnosis of rare diseases: primitive myxoid mesenchymal tumor of infancy.

Ann Clin Lab Sci 2014 ;44(3):310-6

Bobby R. Alford Department of Otolaryngology - Head and Neck Surgery, Baylor College of Medicine, Houston, TX Department of Otolaryngology, Texas Children's Hospital, Houston, TX, USA.

Primitive Myxoid Mesenchymal Tumor of Infancy (PMMTI) is a recently recognized locally aggressive myofibroblastic tumor. It is a low- to intermediate-grade fibroblastic malignancy with a high local recurrence rate but low metastatic potential and is composed of primitive spindled cells in a myxoid background. We present the eleventh reported case of PMMTI, occurring in the sinonasal tract of a 3-year old child. This case is novel in both the relatively older age of the child, the location of the tumor, and the role that immunohistochemical stains, and cytogenetic analysis played in differentiating it from similar diagnoses that differ considerably in their chemosensitivity and recurrence rates. Close collaboration between the pathologist and surgeon was crucial as different diagnoses would have led to vastly different treatment strategies for the patient.
View Article and Find Full Text PDF

Download full-text PDF

Source
May 2015

The effect of a speaking valve on laryngeal aspiration and penetration in children with tracheotomies.

Laryngoscope 2014 Jun 13;124(6):1469-74. Epub 2013 Nov 13.

Department of Pediatric Otolaryngology, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas, U.S.A.

Objectives/hypothesis: Alterations in swallowing can occur after tracheotomy and can result in varying degrees of aspiration. In tracheotomized adult patients, use of a Passy Muir Speaking Valve (PMV) has been shown to decrease laryngeal penetration and aspiration of foods and liquids. The objective of this study was to determine if the PMV has a similar effect on laryngeal penetration and aspiration in tracheotomized children.

Study Design: This is a prospective case-control study.

Methods: Pediatric patients with tracheotomies who were able to tolerate the PMV were identified. Modified barium swallow (MBS) was performed with and without the PMV. Two consistencies, thin liquids and purées, were used. Two speech language pathologists (SLPs), who were blinded to the PMV status, reviewed the recorded MBSs. Three swallows of each consistency were graded on an 8 point Penetration-Aspiration Scale. Residue in the vallecula, piriform sinuses, and posterior pharyngeal wall was graded.

Results: Twelve patients were included for analysis. Laryngeal penetration and aspiration was decreased with purées over liquids (P = 0.5 and P = 0.005, respectively) with either the sham valve or the PMV. The presence of the PMV decreased piriform sinus residue (P = 0.01); however, it did not demonstrate a decrease in laryngeal aspiration or penetration.

Conclusion: Unlike in adults, the presence of PMV did not decrease laryngeal aspiration or penetration in children with tracheotomies. It did, however, improve piriform sinus residue.

Level Of Evidence: 3b.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/lary.24457DOI Listing
June 2014

Is voice therapy effective in the management of vocal fold nodules in children?

Laryngoscope 2013 Dec 20;123(12):2930-1. Epub 2013 Sep 20.

Department of Otolaryngology Head and Neck Surgery, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, U.S.A.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/lary.23830DOI Listing
December 2013

Juvenile recurrent respiratory papilloma: variable intersurgical intervals.

Laryngoscope 2012 Dec 30;122(12):2844-9. Epub 2012 Jul 30.

Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA.

Objectives/hypothesis: To analyze the patterns of surgical frequency in pediatric patients undergoing surgery with CO(2) laser ablation for juvenile onset recurrent respiratory papillomatosis (JORRP). The hypothesis is that over time there is a high variability in surgical frequency independent of the use of an adjuvant therapy.

Study Design: Retrospective case review.

Methods: All pediatric patients treated surgically with the CO(2) laser for JORRP by two senior surgeons at a tertiary pediatric hospital over an 11-year period were evaluated. Regression analysis was performed. An online survey was conducted of the American Academy of Pediatric Otolaryngology membership on their practice patterns regarding JORRP.

Results: Twenty-nine patients were identified, and of those, 20 were included in regression analysis. Several distinct patterns were noted. Only five of the 20 patients (25%) had a constant rate of procedures throughout the observation period. Three (15%) of the patients had continual decrease and one (5%) of the patients had a continual increase in the surgical rate throughout the study period. Eleven (55%) had a fluctuation in the pattern of their recurrences. When queried on how they approach when to repeat intervention for JORRP patients, more than 16 responses were given, and the most common determinants included standard set interval, previous operative findings, and previous interval.

Conclusions: The natural fluctuation in intersurgical intervals without the use of any adjuvant therapy confounds the use of intersurgical interval as an outcome measure for the success of adjuvant therapy. Accelerations and decelerations were noted but cannot be explained.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/lary.23534DOI Listing
December 2012

Postoperative analgesic and behavioral effects of intranasal fentanyl, intravenous morphine, and intramuscular morphine in pediatric patients undergoing bilateral myringotomy and placement of ventilating tubes.

Anesth Analg 2012 Aug 5;115(2):356-63. Epub 2012 Jun 5.

Department of Anesthesiology & Pediatrics, Texas Children's Hospital, Baylor College of Medicine, 6621 Fannin, Suite 3300, Houston, TX, USA.

Background: Bilateral myringotomy and placement of ventilating tubes (BMT) is one of the most common pediatric surgical procedures in the United States. Many children who undergo BMT develop behavioral changes in the postanesthesia care unit (PACU) and require rescue pain medication. The incidence of these changes is lower in children receiving intraoperative opioids by the nasal, IM, or IV route compared with placebo. However, there are no data to indicate which route of administration is better. Our study was designed to compare the immediate postoperative analgesic and behavioral effects of 3 frequently used intraoperative techniques of postoperative pain control for patients undergoing BMT under general anesthesia.

Methods: One hundred seventy-one ASA physical status I and II children scheduled for BMT were randomized into 1 of 3 groups: group 1-nasal fentanyl 2 μg/kg with IV and IM saline placebo; group 2-IV morphine 0.1 mg/kg with nasal and IM placebo; or group 3-IM morphine 0.1 mg/kg with nasal and IV placebo. All subjects received a standardized general anesthetic with sevoflurane, N(2)O, and O(2) and similar postoperative care. The primary end point of the study was the pain scores measured by the Faces, Legs, Activity, Cry, and Consolability (FLACC) scale in the PACU.

Results: There were no significant differences in peak FLACC pain among the 3 groups (mean [95% CI] 2.0 [1.2-2.8] for intranasal fentanyl, 2.7 [1.7-3.6] for IV morphine, and 2.9 [2.1-3.7] for IM morphine, respectively). There were no differences in the scores on the Pediatric Anesthesia Emergence Delirium (PAED) scale, incidence of postoperative emergence delirium (PAED score ≥ 12), emesis, perioperative hypoxemia, or need for airway intervention, and postoperative rescue analgesia. There were also no differences in the duration of PACU stay or parental satisfaction among the groups.

Conclusion: In this double-blind, double-dummy study, there was no difference in the efficacy of intranasal fentanyl, IM and IV morphine in controlling postoperative pain and emergence delirium in children undergoing BMT placement. The IM route is the simplest and avoids the potential for delays to establish vascular access for IV therapy and the risks of laryngospasm if intranasal drugs pass through the posterior nasopharynx and irritate the vocal cords.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1213/ANE.0b013e31825afef3DOI Listing
August 2012

Potocki-Lupski syndrome: a microduplication syndrome associated with oropharyngeal dysphagia and failure to thrive.

J Pediatr 2011 Apr 17;158(4):655-659.e2. Epub 2010 Dec 17.

Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA.

Objective: Failure to thrive (FTT) is a feature of children with Potocki-Lupski syndrome (PTLS) [duplication 17p11.2]. This study was designed to describe the growth characteristics of 24 subjects with PTLS from birth through age 5 years in conjunction with relevant physical features and swallow function studies.

Study Design: We evaluated 24 individuals with PTLS who were ascertained by chromosome analysis and/or array comparative genome hybridization. Clinical assessments included review of medical records, physical examination, otolaryngological examination, and swallow function studies. Measures of height and weight were converted to Z-scores.

Results: The mean weight-for-age and weight-for-length Z-scores at birth were lower (P < .01) than the reference standard and did not change with age. A history of poor feeding, hypotonia, and FTT were reported in 92%, 88%, and 71%, respectively. Individuals with hypotonia had lower weight-for-age and body mass index-for-age Z-scores (P = .01). Swallow function studies demonstrated at least one abnormality in all subjects.

Conclusions: FTT is common in children with PTLS. We hypothesize that oropharyngeal dysphagia and hypotonia likely contribute to FTT in patients with PTLS and recommend that once a diagnosis is established, the individual be assessed for feeding and growth issues and be availed of oromotor therapy and nutritional services.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jpeds.2010.09.062DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3059370PMC
April 2011

Differential diagnosis of pediatric tumors of the nasal cavity and paranasal sinuses: a 45-year multi-institutional review.

Ear Nose Throat J 2010 Nov;89(11):534-40

Department of Head and Neck Surgery, University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.

We conducted a retrospective case-series review to identify the various diagnoses of neoplasms of the nasal cavity and paranasal sinuses in a pediatric population. Our study group was made up of 54 children-23 boys and 31 girls, aged 8 months to 16 years (mean: 9 yr). All patients had been diagnosed with a tumor of the nasal cavity or paranasal sinuses between Jan. 1, 1955, and Dec. 31, 1999, at one of four university-based, tertiary care referral centers. We compiled data on tumoral characteristics (location, size, and histopathology), morbidity and mortality, and rates of recurrence. Lesions included adnexal neoplasm, ameloblastic fibro-odontoma, basal cell carcinoma, benign fibrous histiocytoma, blue nevus, chondrosarcoma, compound nevus, epithelioma adenoides cysticum, esthesioneuroblastoma, Ewing sarcoma, fibrosarcoma, giant cell granuloma, granulocytic sarcoma, hemangioma, hemangiopericytoma, Langerhans cell histiocytosis, lymphangioma, lymphoma, melanoma, neuroblastoma, neurofibroma, ossifying osteofibroma, osteochondroma, osteosarcoma, port wine stain, rhabdomyosarcoma, Spitz nevus, and xanthogranuloma. To the best of our knowledge, this is the largest such study of its kind to date. We believe that the large size of this study and the data on disease incidence will allow clinicians to be better informed of the differential diagnosis of neoplasms of the nasal cavity and paranasal sinuses in the pediatric population.
View Article and Find Full Text PDF

Download full-text PDF

Source
November 2010

Lingual tonsil hypertrophy complicating upper gastrointestinal endoscopy.

J Pediatr Gastroenterol Nutr 2010 Sep;51(3):247

Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/MPG.0b013e3181eb6858DOI Listing
September 2010

The effect of intraoperative dexmedetomidine on postoperative analgesia and sedation in pediatric patients undergoing tonsillectomy and adenoidectomy.

Anesth Analg 2010 Aug 7;111(2):490-5. Epub 2010 Jul 7.

Texas Children's Hospital, 6621 Fannin St., Suite A-300, MC 2-1495, Houston, TX 77030, USA.

Background: The immediate postoperative period after tonsillectomy and adenoidectomy, one of the most common pediatric surgical procedures, is often difficult. These children frequently have severe pain but postoperative airway edema along with increased sensitivity to the respiratory-depressant effects of opioids may result in obstructive symptoms and hypoxemia. Opioid consumption may be reduced by nonsteroidal antiinflammatory drugs, but these drugs may be associated with increased bleeding after this operation. Dexmedetomidine has mild analgesic properties, causes sedation without respiratory depression, and does not have an effect on coagulation. We designed a prospective, double-blind, randomized controlled study to determine the effects of intraoperative dexmedetomidine on postoperative recovery including pain, sedation, and hemodynamics in pediatric patients undergoing tonsillectomy and adenoidectomy.

Methods: One hundred nine patients were randomized to receive a single intraoperative dose of dexmedetomidine 0.75 microg/kg, dexmedetomidine 1 microg/kg, morphine 50 microg/kg, or morphine 100 microg/kg over 10 minutes after endotracheal intubation.

Results: There were no significant differences among the 4 groups in patient demographics, ASA physical status, postoperative opioid requirements, sedation scores, duration of oxygen supplementation in the postanesthetic care unit, and time to discharge readiness. The median time to first postoperative rescue analgesic was similar in patients receiving dexmedetomidine 1 microg/kg and morphine 100 microg/kg, but significantly longer compared with patients receiving dexmedetomidine 0.75 microg/kg or morphine 50 microg/kg (P < 0.01). In addition, the number of patients requiring >1 rescue analgesic dose was significantly higher in the dexmedetomidine 0.75 microg/kg group compared with the dexmedetomidine 1 microg/kg and morphine 100 microg/kg groups, but not the morphine 50 microg/kg group. Patients receiving dexmedetomidine had significantly slower heart rates in the first 30 minutes after surgery compared with those receiving morphine (P < 0.05). There was no significant difference in sedation scores among the groups.

Conclusions: The total postoperative rescue opioid requirements were similar in tonsillectomy patients receiving intraoperative dexmedetomidine or morphine. However, the use of dexmedetomidine 1 microg/kg and morphine 100 microg/kg had the advantages of an increased time to first analgesic and a reduced need for additional rescue analgesia doses, without increasing discharge times.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1213/ANE.0b013e3181e33429DOI Listing
August 2010

Potocki-Shaffer syndrome: comprehensive clinical assessment, review of the literature, and proposals for medical management.

Am J Med Genet A 2010 Mar;152A(3):565-72

Department of Pediatrics, Baylor College of Medicine, Houston, Texas 77030, USA.

Potocki-Shaffer syndrome is a rare contiguous gene deletion syndrome due to haploinsufficiency of the 11p11.2p12 region and is characterized by craniofacial abnormalities, developmental delay, intellectual disability, multiple exostoses, and biparietal foramina. In this study, six patients with the Potocki-Shaffer syndrome were identified and evaluated using a multidisciplinary protocol that included assessments by a geneticist, ophthalmologist, otolaryngologist, orthopedist, nephrologist, audiologist, and neuropsychologist. Diagnostic studies included skeletal survey, magnetic resonance imaging of the brain, renal ultrasound, complete blood count, comprehensive metabolic panel, thyroid studies, and urinalysis. Using array comparative genomic hybridization, we further characterized the deletion in five of these patients. The results of these evaluations were combined with a comprehensive review of reported cases. Our data highlight the characteristic facial features, biparietal foramina, moderate-to-severe developmental delay and intellectual disability, myopia and strabismus, and multiple exostoses seen with this disorder. We also identify for the first time an association of Potocki-Shaffer syndrome with sensorineural hearing loss and autistic behaviors. Finally, we provide recommendations for the health maintenance of patients with Potocki-Shaffer syndrome.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/ajmg.a.33245DOI Listing
March 2010

Tonsillectomy in post-transplant lymphoproliferative disease in children.

Laryngoscope 2010 Mar;120(3):608-11

Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, Texas 77030, USA.

Objectives/hypothesis: To review the role of tonsillectomy in diagnosis and management of post-transplant lymphoproliferative disorder (PTLD).

Study Design: Case series using a retrospective chart review.

Methods: A retrospective review of post-transplant lymphoproliferative disease at a tertiary academic medical center from January 2004 to April 2008. Data extracted includes patients' gender, age at transplantation, year of transplantation and tonsillectomy, type of transplant, presenting symptoms, type of immunosuppression, and outcome.

Results: Six out of 25 (24%) patients who underwent tonsillectomy were found to have PTLD. The patient's characteristics, gender, age at transplantation, year of transplantation and tonsillectomy, type of transplant, presenting symptoms, type of immunosuppression, and outcomes are discussed.

Conclusions: In our series, tonsillar hypertrophy, male gender, young age at transplantation, and liver transplantation were associated with higher rates of PTLD. Given the devastating nature of PTLD, early detection with tonsillectomy and institution of treatment is critical.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/lary.20799DOI Listing
March 2010

Optimal utilization of histopathologic analysis of tonsil and adenoid specimens in the pediatric population.

Int J Pediatr Otorhinolaryngol 2010 Feb 26;74(2):161-3. Epub 2009 Nov 26.

Bobby R Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, TX 77030, USA.

Objective: To evaluate the optimal utilization of histopathologic analysis of tonsil and adenoid specimens in the pediatric population.

Methods: A retrospective review was performed on 7837 tonsil and adenoid specimens submitted from January 2004 to April 2008. The records were reviewed for the patients' age, sex, and pathologic analysis. The time and cost per analysis of each specimen were determined.

Results: Histopathology was performed on 347 specimens based on clinical suspicion by the surgeon, a difference of 0.5 cm or more among tonsils, gross abnormalities, and history of malignancy, transplant, or immunocompromise. Malignancy was diagnosed in 0.026% of patients. Post-transplant lymphoproliferative disease was diagnosed in 6 of 24 immunocompromised patients. The use of these criteria resulted in a savings of $518,088.47 and 461 h of dedicated technician time per year.

Conclusions: Histologic examinations in selected specimens should be based on specific criteria that should be determined by each hospital based on hospital size, finances and input from their pathologists and otolaryngogists. Storage of a representative specimen for possible retrospective review may be useful.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ijporl.2009.10.028DOI Listing
February 2010

Cervical thymic anomalies--the Texas Children's Hospital experience.

Laryngoscope 2009 Oct;119(10):1988-93

Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA.

Objectives/hypothesis: To review the presentation and management of cervical thymic cysts and ectopic thymic tissue at Texas Children's Hospital over the last 25 years.

Study Design: Case report and case series using retrospective chart review.

Methods: A case report is presented of a recently diagnosed thymic cyst highlighting diagnostic, management, and treatment strategies available for optimizing management of patients with significant mediastinal extension. We then present a retrospective review of cervical thymic anomalies at a tertiary academic medical center over a 25-year span (1983-present). Data extracted include patients' characteristics, clinical presentation, diagnostic workup, surgical management, and postoperative complications.

Results: Fifteen patients were found to have a pathological diagnosis of cervical thymic cyst, and 10 patients had a diagnosis of ectopic thymic tissue in the neck. This is the largest case series of cervical thymic anomalies presented in the literature to date. Patients' characteristics, diagnostic techniques, and treatment strategies are discussed.

Conclusions: Cervical thymic anomalies are a rare but necessary part of the differential diagnosis of a cervical mass. Computed tomography scan can both narrow the preoperative differential diagnosis and aid in surgical planning for thymic cyst excision. A full discussion of the embryology, clinical presentation, and management of cervical thymic cysts and a review of the current literature is presented.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/lary.20625DOI Listing
October 2009

Advanced pediatric mastoiditis with and without intracranial complications.

Laryngoscope 2009 Aug;119(8):1610-5

Bobby R. Alford Department of Otolaryngology, Texas Children's Hospital, Houston, USA.

Objectives/hypothesis: Recently, several groups have noticed an increase in cases of advanced pediatric mastoiditis and intracranial complications. The objective of this study was to review the bacteriology of advanced mastoiditis in pediatric patients, with the hypothesis that a difference in bacteriology might explain the development of an intracranial complication.

Study Design: Retrospective chart review.

Methods: All pediatric patients with coalescent mastoiditis requiring surgery treated at a tertiary care children's hospital between 2002 and 2007 were reviewed. Every patient included was treated either with mastoidectomy alone (for coalescent mastoiditis without an intracranial complication) or with transtemporal craniotomy (for coalescent mastoiditis with an intracranial complication). All patients had surgical specimens sent for pathology, Gram stain, and aerobic and anaerobic cultures.

Results: One hundred eight pediatric patients with coalescent mastoiditis were identified: 58 (53%) presented with coalescent mastoiditis alone, 17 (16%) presented with coalescent mastoiditis and an intracranial complication, and 33 (31%) were excluded because they were treated with myringotomy and tubes alone, had incomplete data, or had an unclear diagnosis. Streptococcus pneumoniae was the most commonly cultured organism in patients with and without intracranial complications. Anaerobic isolates were present in 29.4% of patients with intracranial complications and 5.7% of patients without intracranial complications (P = .015).

Conclusions: Nearly a quarter of pediatric patients with coalescent mastoiditis presented with a simultaneous intracranial complication. There was an increased incidence of anaerobic organisms in patients with intracranial complications compared to those without, indicating the importance of culture and antibiotic coverage appropriate for anaerobes. This series demonstrates the role of aggressive surgical management and close collaboration with the infectious disease service for long-term intravenous antibiotic therapy in treating pediatric patients with advanced mastoiditis.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/lary.20259DOI Listing
August 2009

Nasal and sinus disease in cystic fibrosis.

Paediatr Respir Rev 2008 Sep 31;9(3):213-9. Epub 2008 Jul 31.

Department of Pediatrics, Texas Children's Hospital, Houston, Texas 77030, USA.

Paranasal and sinus disease is present in almost every patient with cystic fibrosis. However, symptoms are rarely reported. Some aspects including polyposis and microbial flora change with patient age. Endoscopy and computerised tomography have broadened our understanding of how this disease affects the sinuses, including an increased recognition of polyposis than previously thought and identification of several disease specific entities such as abnormalities of the lateral nasal wall and uncinate process. Few randomised, controlled trials evaluating medical or surgical treatments of CF sinus disease exist. Sinus surgery may provide some benefit, though there are no established selection criteria for appropriate candidates. A link between sinus disease and lower respiratory tract function may contribute to general health and survival following lung transplantation. Complications of sinonasal disease in CF are rare and include mucoceles and periorbital abscesses.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.prrv.2008.04.003DOI Listing
September 2008

The usefulness of computed tomography scans in cystic fibrosis patients with chronic sinusitis.

Am J Rhinol 2007 Nov-Dec;21(6):706-10

96th Surgical Operations Squadron/SGCXL, Eglin Air Force Base, Florida, USA.

Background: This study was performed to evaluate the usefulness of computed tomography (CT) scans of the sinuses in children with cystic fibrosis (CF) as an outcomes measurement for medical and surgically treated chronic rhinosinusitis.

Methods: Patients with CF who had sinus scans performed at the study institution from January 1999 to September 2003 were identified. The scans and any available prior scans were scored according to the Lund-MacKay system. A retrospective chart review determined if hospitalization with i.v. antibiotic therapy or operative intervention had occurred between scans and if the scans showed improvement secondary to the intervention.

Results: One hundred thirty-four patients were identified who met inclusion criteria. A total of 290 scans were reviewed, with the average number of CT scans per patient of 2.24 (range, 1-10). The average Lund-MacKay score for scans closest in time to the first sinus surgery was 14.5. (range, 7-24), and for the first postoperative scans, the score was 14.7 (8-24). There was no significant difference between the preoperative and postoperative Lund-MacKay score after initial surgery (p = 0.99) or in subsequent scans despite medical or surgical interventions (p = 0.90).

Conclusion: CT scanning of the sinuses does not appear to be useful in determining outcomes of intervention in CF patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.2500/ajr.2007.21.3104DOI Listing
March 2008

Doxycycline sclerotherapy as the primary treatment for head and neck lymphatic malformations.

Otolaryngol Head Neck Surg 2007 Dec;137(6):962-4

Bobby R Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, TX, USA.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.otohns.2007.08.013DOI Listing
December 2007

Characterization of Potocki-Lupski syndrome (dup(17)(p11.2p11.2)) and delineation of a dosage-sensitive critical interval that can convey an autism phenotype.

Am J Hum Genet 2007 Apr 26;80(4):633-49. Epub 2007 Feb 26.

Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA.

The duplication 17p11.2 syndrome, associated with dup(17)(p11.2p11.2), is a recently recognized syndrome of multiple congenital anomalies and mental retardation and is the first predicted reciprocal microduplication syndrome described--the homologous recombination reciprocal of the Smith-Magenis syndrome (SMS) microdeletion (del(17)(p11.2p11.2)). We previously described seven subjects with dup(17)(p11.2p11.2) and noted their relatively mild phenotype compared with that of individuals with SMS. Here, we molecularly analyzed 28 additional patients, using multiple independent assays, and also report the phenotypic characteristics obtained from extensive multidisciplinary clinical study of a subset of these patients. Whereas the majority of subjects (22 of 35) harbor the homologous recombination reciprocal product of the common SMS microdeletion (~3.7 Mb), 13 subjects (~37%) have nonrecurrent duplications ranging in size from 1.3 to 15.2 Mb. Molecular studies suggest potential mechanistic differences between nonrecurrent duplications and nonrecurrent genomic deletions. Clinical features observed in patients with the common dup(17)(p11.2p11.2) are distinct from those seen with SMS and include infantile hypotonia, failure to thrive, mental retardation, autistic features, sleep apnea, and structural cardiovascular anomalies. We narrow the critical region to a 1.3-Mb genomic interval that contains the dosage-sensitive RAI1 gene. Our results refine the critical region for Potocki-Lupski syndrome, provide information to assist in clinical diagnosis and management, and lend further support for the concept that genomic architecture incites genomic instability.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1086/512864DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1852712PMC
April 2007