The Medially-Invasive Cholesteatoma: An Aggressive Subtype of a Common Pathology

2020 ◽  
Vol 130 (1) ◽  
pp. 38-46
Author(s):  
Geoffrey Casazza ◽  
Matthew L. Carlson ◽  
Clough Shelton ◽  
Richard K. Gurgel

Objective: Describe the outcomes of treatment for patients with cholesteatomas that are medially invasive to the otic capsule, petrous apex, and/or skull base. Study Design: Retrospective case series Setting: Two tertiary care academic centers. Patients: Patients surgically managed for medially-invasive cholesteatoma at two tertiary care institutions from 2001 to 2017. Interventions: Surgical management of medially-invasive cholesteatomas. Main Outcome Measures: The presenting symptoms, imaging, pre- and post-operative clinical course, and complications were reviewed. Results: Seven patients were identified. All patients had pre-operative radiographic evidence of invasive cholesteatoma with erosion into the otic capsule beyond just a lateral semicircular canal fistula. Five patients had a complex otologic history with multiple surgeries for recurrent cholesteatoma including three with prior canal wall down mastoidectomy surgeries. Average age at the time of surgery was 41.3 years (range 20-83). Two patients underwent a hearing preservation approach to the skull base while all others underwent a surgical approach based on the extent of the lesion. Facial nerve function was maintained at the pre-operative level in all but one patient. No patient developed cholesteatoma recurrence. Conclusions: The medially-invasive cholesteatoma demonstrates an aggressive, endophytic growth pattern, invading into the otic capsule or through the perilabyrinthine air cells to the petrous apex. Surgical resection remains the best treatment option for medially-invasive cholesteatoma. When CSF leak is a concern, a subtotal petrosectomy with closure of the ear is often necessary.

Author(s):  
Shashidhar S. Suligavi ◽  
Mallikarjun N. Patil ◽  
S. S. Doddamani ◽  
Chandrashekarayya S. Hiremath ◽  
Afshan Fathima

<p class="abstract"><strong><span lang="EN-US">Background:</span></strong>Tracheo- bronchial foreign bodies have always posed a challenge to the ENT surgeon as they present with varied symptomatology ranging from a simple cough and fever to more grave respiratory distress. It requires a strong suspicion, early diagnosis and timely intervention to reduce the overall morbidity and mortality .This study was undertaken to highlight our experiences in handling cases of tracheo- bronchial foreign bodies (FB) at our setup.</p><p class="abstract"><strong><span lang="EN-US">Methods:</span></strong>It is a retrospective case series study conducted in S. Nijalingappa Medical College between January 2011 and January 2015.  </p><p class="abstract"><strong><span lang="EN-US">Results:</span></strong>Most commonly affected were children between 1year to 3years of age. Chronic cough and wheeze were the commonest presenting symptoms. Vegetative foreign body was found to be the commonest variety of foreign body. The mortality rate in our study was 4.7% (n=3).</p><p class="abstract"><strong><span lang="EN-US">Conclusions:</span></strong>A good clinical acumen, team work, early diagnosis and timely intervention are all needed to reduce the overall mortality and morbidity associated with tracheo- bronchial foreign bodies.</p>


Author(s):  
Erin Mamuyac Lopez ◽  
Zainab Farzal ◽  
Kelly Marie Dean ◽  
Craig Miller ◽  
Justin Cates Morse ◽  
...  

Objectives: The frequency of endoscopic skull base surgery in pediatric patients is increasing. This study’s aim is to systematically review the literature for endoscopic skull base surgery outcomes in children/adolescents ages 0-18 years. Design: A systematic review of the literature was performed in PubMed and SCOPUS databases querying studies from 2000-2020 using PRISMA guidelines. Final inclusion criteria included: case series with 10+ patients with pediatric patients age ≤18 years, endoscopic or endoscopic-assisted skull base surgery, and outcomes reported. Setting: Tertiary care medical center Participants: Children/adolescents ages 0-18 years who underwent endoscopic skull base surgery Main Outcome Measures: Patient demographics, pathology, reconstructive technique, intra-operative findings, intra-operative and post-operative surgical complications. Results: Systematic literature search yielded 287 publications. Of these, 12 studies discussing a total of 399 patients age 0-18 years met inclusion criteria for final analysis. 7 of 12 studies discussed a single pathology. The most common pathology was a skull base defect causing CSF leak. The majority of skull base repairs were made with free tissue grafts. The most common post-operative complication was CSF leak (n=40). Twelve cases of meningitis occurred post-operatively with two of these episodes resulting in death. Conclusions: Endoscopic skull base surgery has been performed recently in the pediatric population in a variety of disease states. Inconsistent individual-level data and reporting standards are present in existing studies posing challenges for comparative analysis. Standardized reporting will aid future reviews and meta-analysis for rare skull base pathology.


2019 ◽  
Vol 162 (2) ◽  
pp. 234-240 ◽  
Author(s):  
Claire M. Lawlor ◽  
Natasha D. Dombrowski ◽  
Roger C. Nuss ◽  
Reza Rahbar ◽  
Sukgi S. Choi

Objective To discuss the presentation, evaluation, and management of pediatric laryngeal web. Study Design Retrospective case series. Setting Single tertiary care center. Subjects All patients with laryngeal web at Boston Children’s Hospital in the past 22 years. Methods No exclusion criteria. Charts mined for age at presentation, presenting symptoms, degree/location of web, associated syndromes, number/type of surgical procedures, and postoperative outcomes. Results Thirty-seven patients were included (13 male, 24 female). Average age at diagnosis was 3.7 years (0-19.5 years). Mean follow-up was 4.4 years (range, 0-16.4 years). There were 26 congenital webs (70.2%) and 11 acquired webs (29.8%). Presenting symptoms were vocal (29 patients, 78.4%) and respiratory (22 patients, 60%). Underlying syndromes or synchronous airway lesions included the following: premature (n = 5), congenital heart disease (n = 18), subglottic stenosis (n = 5), 22q11.2 deletion syndrome (n = 10), and recurrent respiratory papillomatosis (n = 4). There were 20 type 1 webs, 6 type 2 webs, 8 type 3 webs, and 3 type 4 webs; 10 had subglottic extension of the laryngeal web. Twelve patients were managed conservatively with observation. Eighty-four interventions were performed: 18 open and 66 endoscopic (sharp division, 32; dilation, 33; mitomycin C, 14; laser, 5; keel, 6; triamcinolone injection, 8; stent, 15; removal of granulation tissue, 5). Tracheotomy was required in 11 patients, and 5 patients were decannulated. Voice improved in 12 patients, with respiratory symptoms in 12 patients. Web recurred in 17 patients. One patient died due to airway complications. Conclusions Pediatric laryngeal web is an uncommon but challenging lesion. Patients need to be evaluated for comorbid syndromes and synchronous airway lesions. Management includes open and endoscopic procedures. Procedures should be tailored to the child’s presentation.


2013 ◽  
Vol 31 (15_suppl) ◽  
pp. 10584-10584
Author(s):  
Maryann Shango ◽  
Lili Zhao ◽  
Monika Leja ◽  
Jonathan B. McHugh ◽  
Scott Schuetze ◽  
...  

10584 Background: Primary cardiac sarcoma (PCS) is the most common primary cardiac malignancy, but is a rare primary site of sarcoma. We present 21 cases from a tertiary care center to better understand this uncommon malignancy. Methods: A cancer center-based registry and pathology database were searched to identify pts diagnosed with PCS from 1992-2013 at University of Michigan. Kaplan-meier method was used to estimate survival. Cox proportional hazard model was used to associate variables to occurrence of metastases (mets) or death. Results: Atotal of 21 pts (F12, 9M) with PCS were identified, median age 36 (range 11-74). The most common presenting symptoms included dyspnea (16) and chest pain (6; 5 with associated pericardial effusion). Histologies included: angiosarcoma (9), leiomyosarcoma (4), undifferentiated pleomorphic (3), spindle cell (2), fibrosarcoma (1), rhabdomyosarcoma (1) and synovial (1). Sites of origin were R atrium (7), R ventricle (2), L atrium (10) and pericardium (2). Ten pts presented with mets; most common sites were lung (8), liver (2), brain (2), pancreas (2) and bone (2). Surgery was attempted in 12 pts, achieving 1 R0 resection. Pts received a median of 1 (0-7) systemic therapies. Median overall (OS) was 12.6 mos (range 3-79) from diagnosis. Pts without prior surgery were more likely to have mets or death (p=0.038). Brain mets were common, occurring in 7 of 21 pts after a median of 7 mos (range 1-75) from diagnosis. Median OS after diagnosis of brain mets was 8 mos. Of the 7 pts who developed brain metastasis, 5 had PCS originating in the left heart. Of the 2 pts with PCS in the right heart, one was evaluated for and had a right to left shunt. The likelihood of developing brain mets did not correlate with age, chemotherapy, or surgery. Conclusions: PCS portends an extremely poor prognosis, marked by inability to achieve complete resection and a high incidence of disseminated disease at diagnosis. Metastatic disease to the brain was much more common in PCS (33%) as compared to STS of any origin (approximately 1-8%), particularly in pts with PCS originating in the left heart. Clinicians should have a low threshold for brain imaging evaluation of PCS pts.


2021 ◽  
pp. 000348942110072
Author(s):  
Jenna H. Barengo ◽  
Andrew J. Redmann ◽  
Patrick Kennedy ◽  
Michael J. Rutter ◽  
Matthew M. Smith

Objectives: Examine the presentation and clinical course of patients with bacterial tracheitis (BT). Identify if socioeconomic differences exist among children who present with BT. Methods: This was a retrospective case series from a tertiary care pediatric medical center. The study group included patients less than 18 years old who were diagnosed with BT from January 2011 to March 2019. Patients with a tracheostomy and those who developed BT after prolonged hospitalization were excluded. Patient demographics were compared with the demographics of the counties surrounding the hospital. Results: 33 patients with BT met inclusion criteria. The most common presenting symptoms were difficulty breathing, stridor, and sore throat (81.8% each), followed by cough (78.8%). Median length of stay was 3 days [interquartile range (IQR):2-4]. 19 patients (57.5%) were admitted to the intensive care unit. Intubation was required for 13 patients (39.4%), for a median length of 2 days [IQR:2-2]. Methicillin sensitive staphylococcus aureus was the most common bacterial etiology (33%). Mean presenting age was 8.58 years [95% confidence interval:7.3-9.9] and 14 patients were female (42.4%). 31 patients were white (93.9%), 1 was black (3%), and 1 was Hispanic (3%). BT patients were more likely to have private insurance compared to comparison (81.8% vs 63.4%, P < .001). Conclusion: Children who presented with BT were more likely to be privately insured than a comparison population.


Author(s):  
Deepak A. V. ◽  
K. J. Jacob ◽  
Sumi P. Maria

Background: Peripartum hysterectomy is a life-saving procedure resorted to when conservative measures fail to control obstetric haemorrhage. Several predisposing factors, suboptimal care and lack of infrastructure may lead to this emergency procedure. We wanted to find out factors associated with peripartum hysterectomy and the adverse maternal outcomes at our centre.Methods: A retrospective case series analysis of 40 cases of peripartum hysterectomy performed over a period of 5 years from January 2010 to December 2014 at Government Medical College Hospital, Thrissur, Kerala was done.Results: The incidence of peripartum hysterectomy was 0.29%. The most common indication for peripartum hysterectomy was hysterectomy was uterine atony (50%). Thirty-five women (88%) were between 20 and 35 years. Most of the subjects were unbooked. There were two maternal deaths (case fatality rate of 5%) following peripartum hysterectomy during this period. All the subjects required blood transfusion.Conclusions: Prompt performance hysterectomy before the patient’s clinical condition deteriorates is the key to success. The incidence of adherent placenta is increasing, so every effort should be taken to reduce the caesarean section rates globally. 


Author(s):  
Josee Paradis ◽  
Agnieszka Dzioba ◽  
Hamdy El-Hakim ◽  
Paul Hong ◽  
Frederick K. Kozak ◽  
...  

Abstract Background To evaluate the clinical presentation of choanal atresia (CA) in tertiary centers across Canada. Methods Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with CA at a participating center. Results The health charts of 215 patients (59.6% female) with CA were reviewed and included in this study. The mean age of patients at time of CA presentation was 0.4 months (range 0.1 to 7.2 months) for bilateral CA and 37.8 months (range 0.1 to 164.1 months) for unilateral cases. The most common presenting symptoms for bilateral CA in decreasing order were respiratory distress (96.4%), feeding difficulties (68.2%), and rhinorrhea (65.5%), and for unilateral cases in decreasing order were rhinorrhea (92.0%), feeding difficulties (24.7%), and respiratory distress (18.0%). For the majority of patients (73.2%), the obstruction comprised mixed bony and membranous tissue, with only 10.5% presenting with a purely membranous obstruction. Familial history of CA was confirmed in only 3.3% of cases. One half of patients with CA presented with one or more associated anomalies and 30.6% had a syndrome. Conclusions The present investigation is the first national multi-institutional study evaluating the clinical presentation of CA over three decades. The present cohort of CA patients presented with a breadth of co-morbidities with highly variable presentations, with bilateral cases being more severely affected than unilateral cases. Further investigation into hereditary linkages to CA development is warranted. Graphical abstract


2019 ◽  
Vol 24 (3) ◽  
pp. 246-257 ◽  
Author(s):  
Jennifer L. Quon ◽  
Lily H. Kim ◽  
Peter H. Hwang ◽  
Zara M. Patel ◽  
Gerald A. Grant ◽  
...  

OBJECTIVETransnasal endoscopic transsphenoidal approaches constitute an essential technique for the resection of skull base tumors in adults. However, in the pediatric population, sellar and suprasellar lesions have historically been treated by craniotomy. Transnasal endoscopic approaches are less invasive and thus may be preferable to craniotomy, especially in children. In this case series, the authors present their institutional experience with transnasal endoscopic transsphenoidal approaches for pediatric skull base tumors.METHODSThe authors retrospectively reviewed pediatric patients (age ≤ 18 years) who had undergone transnasal endoscopic transsphenoidal approaches for either biopsy or resection of sellar or suprasellar lesions between 2007 and 2016. All operations were performed jointly by a team of pediatric neurosurgeons and skull base otolaryngologists, except for 8 cases performed by one neurosurgeon.RESULTSThe series included 42 patients between 4 and 18 years old (average 12.5 years) who underwent 51 operations. Headache (45%), visual symptoms (69%), and symptoms related to hormonal abnormalities (71%) were the predominant presenting symptoms. Improvement in preoperative symptoms was seen in 92% of cases. Most patients had craniopharyngiomas (n = 16), followed by pituitary adenomas (n = 12), Rathke cleft cysts (n = 4), germinomas (n = 4), chordomas (n = 2), and other lesion subtypes (n = 4). Lesions ranged from 0.3 to 6.2 cm (median 2.5 cm) in their greatest dimension. Gross-total resection was primarily performed (63% of cases), with 5 subsequent recurrences. Nasoseptal flaps were used in 47% of cases, fat grafts in 37%, and lumbar drains in 47%. CSF space was entered intraoperatively in 15 cases, and postoperative CSF was observed only in lesions with suprasellar extension. There were 8 cases of new hormonal deficits and 3 cases of new cranial nerve deficits. Length of hospital stay ranged from 1 to 61 days (median 5 days). Patients were clinically followed up for a median of 46 months (range 1–120 months), accompanied by a median radiological follow-up period of 45 months (range 3.8–120 months). Most patients (76%) were offered adjuvant therapy.CONCLUSIONSIn this single-institution report of the transnasal endoscopic transsphenoidal approach, the authors demonstrated that this technique is generally safe and effective for different types of pediatric skull base lesions. Favorable effects of surgery were sustained during a follow-up period of 4 years. Further refinement in technology will allow for more widespread use in the pediatric population.


2014 ◽  
Vol 2 (1) ◽  
pp. 9-14
Author(s):  
Sheikh Shawkat Kamal

Aim: To evaluate the role of newly created transcanal endoscopic antrostomy hole as a passage in restoring the aeration of epitympanum and mastoid antrum.Materials & Methods: Study design: Retrospective case series study. Study duration: From January 2013 to January 2014. Study place: Private tertiary care hospital. Patients: 27  ears of 23 adult patients (age ranging from 15years to 54years) underwent transcanal endoscopic tympanoplasty for their chronic middle ear diseases in presence of inflamed middle ear mucosa along with radiological shadows of stagnant fluid in their epitympanum and mastoid antrum. Structured three months follow-up was ensured in every case. Intervention: A hole was created at a selected site of posterior meatal wall purely through transcanal endoscopic approach that established direct communication between mastoid antrum and external auditory canal. Thereafter this newly created passage was used for three purposes- assessment of the condition of mastoid antrum, performing the water test for checking epitympanic patency and in few cases placement of temporary tube for postoperative ventilation and drainage of middle ear. Main out come measures: The feasibility, performance and management of transcanal endoscopic antrostomy hole as passage for reestablishing the aeration of epitympanum and mastoid antrum.Results: The chosen site for antrostomy hole was found effective and safe in providing convenient entrance into the mastoid antrum in every case in this study with out facing technical complexity and failure. Postoperative healing of skin over antrostomy hole was found complete in all ears without any inward growth of skin in to mastoid antrum. Available post operative CT scan imaging of temporal bones showed improved aeration in their epitympanum and mastoid antrum.Conclusions: The role of transcanal antrostomy hole has been proved worthy in restoring ventilation pathway to epitympanum and mastoid antrum during tympanoplasty. This antrostomy hole has the potentiality to be considered in future for placement of long term mastoid ventilation tube in order to treat persistent atelectatic middle ear.


2017 ◽  
Vol 28 (10) ◽  
pp. 913-919 ◽  
Author(s):  
Margaret A. Meredith ◽  
Jay T. Rubinstein ◽  
Kathleen C. Y. Sie ◽  
Susan J. Norton

Background: Children with steeply sloping sensorineural hearing loss (SNHL) lack access to critical high-frequency cues despite the use of advanced hearing aid technology. In addition, their auditory-only aided speech perception abilities often meet Food and Drug Administration criteria for cochlear implantation. Purpose: The objective of this study was to describe hearing preservation and speech perception outcomes in a group of young children with steeply sloping SNHL who received a cochlear implant (CI). Research Design: Retrospective case series. Study Sample: Eight children with steeply sloping postlingual progressive SNHL who received a unilateral traditional CI at Seattle Children’s Hospital between 2009 and 2013 and had follow-up data available up to 24 mo postimplant were included. Data Collection and Analysis: A retrospective chart review was completed. Medical records were reviewed for demographic information, preoperative and postoperative behavioral hearing thresholds, and speech perception scores. Paired t tests were used to analyze speech perception data. Hearing preservation results are reported. Results: Rapid improvement of speech perception scores was observed within the first month postimplant for all participants. Mean monosyllabic word scores were 76% and mean phoneme scores were 86.7% at 1-mo postactivation compared to mean preimplant scores of 19.5% and 31.0%, respectively. Hearing preservation was observed in five participants out to 24-mo postactivation. Two participants lost hearing in both the implanted and unimplanted ear, and received a sequential bilateral CI in the other ear after progression of the hearing loss. One participant had a total loss of hearing in only the implanted ear. Results reported in this article are from the ear implanted first. Bilateral outcomes are not reported. Conclusions: CIs provided benefit for children with steeply sloping bilateral hearing loss for whom hearing aids did not provide adequate auditory access. In our cohort, significant improvements in speech understanding occurred rapidly postactivation. Preservation of residual hearing in children with a traditional CI electrode is possible.


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