Transcanal Endoscopic Ear Surgery for Congenital Cholesteatoma: A Multi-institutional Series

2021 ◽  
pp. 019459982110675
Author(s):  
Carolyn M. Jenks ◽  
Patricia L. Purcell ◽  
Gaia Federici ◽  
Domenico Villari ◽  
Livio Presutti ◽  
...  

Objective To assess outcomes of transcanal endoscopic ear surgery (TEES) for congenital cholesteatoma. Study Design Case series with chart review of children who underwent TEES for congenital cholesteatoma over a 10-year period. Setting Three tertiary referral centers. Methods Cholesteatoma extent was classified according to Potsic stage; cases with mastoid extension (Potsic IV) were excluded. Disease characteristics, surgical approach, and outcomes were compared among stages. Outcomes measures included residual or recurrent cholesteatoma and audiometric data. Results Sixty-five cases of congenital cholesteatoma were included. The mean age was 6.5 years (range, 1.2-16), and the mean follow-up was 3.9 years (range, 0.75-9.1). There were 19 cases (29%) of Potsic stage I disease, 10 (15%) stage II, and 36 (55%) stage III. Overall, 24 (37%) patients underwent a second-stage procedure, including 1 with Potsic stage II disease (10%) and 21 (58%) with Potsic stage III disease. Eight cases (12%) of residual cholesteatoma occurred. One patient (2%) developed retraction-type (“recurrent”) cholesteatoma. Recidivism occurred only among Potsic stage III cases. Postoperative air conduction hearing thresholds were normal (<25 dB HL) in 93% of Potsic stage I, 88% of stage II, and 36% of stage III cases. Conclusion TEES is feasible and effective for removal of congenital cholesteatoma not extending into the mastoid. Recidivism rates were lower with the TEES approach in this large series than in previously reported studies. Advanced-stage disease was the primary risk factor for recidivism and worse hearing result. As minimally invasive TEES is possible in the youngest cases, children benefit from early identification and intervention.

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5330-5330 ◽  
Author(s):  
Ritsuro Suzuki ◽  
Dai Chihara ◽  
Naoko Asano ◽  
Ken Ohmachi ◽  
Tomohiro Kinoshita ◽  
...  

Abstract [Background] Mantle cell lymphoma (MCL) is an aggressive B-cell lymphoma, characterized by the overexpression of cyclin D1 derived from t(11;14)(q13;q32) and poor prognosis. Most MCLs show nodal presentation, but also accompany extranodal involvement, such as bone marrow, peripheral blood or gastrointestinal tract. As a result, many MCLs present with advanced stage disease. Since only a small portion of patients show limited-stage disease, minimal data exist on treatment of patients diagnosed with limited stage disease. Nevertheless, the treatment strategy of MCL is recommended according to the clinical stage of limited- (stage I or non-bulky II) vs. advanced-stage, as well as other types of lymphoma. [Patients and methods] We recently collected 633 patient data of MCL (Chihara, et al. Ann Oncol 2015). Information of clinical stage was available in 626 patients. The patient data were retrospectively analyzed the by the clinical stage at initial presentation. [Results] The clinical stage was I in 24 patients (4%), II in 33 (5%), III in 70 (11%), and IV in 499 (80%). Only one patient presented with bulky stage II. Detailed demographic information by the clinical stage are listed in Table. Age and sex were not significantly different by clinical stage. Limited stage patients were associated with better performance status (PS), less B symptoms, no extranodal involvement, and lower lactate dehydrogenase (LDH) level and white blood cell (WBC) count. Most patients in any stage were treated with cytotoxic chemotherapy, but more patients in limited stage received radiotherapy. The proportion of high-dose cytarabine (HDCA)-containing regimen over CHOP/CHOP-like was higher in advanced stage patients. Complete and overall response rates were 92% and 96% in stage I, 58% and 94% in stage II, 66% and 86% in stage III, and 52% and 82% in stage IV, respectively (P = 0.02). However, the higher response rate in limited stage patients did not translate into better prognosis. The median survival was 11.0 years in stage I, 13.4 years in stage II, 11.5 years in stage III, and 5.6 years in stage IV (Figure). The prognosis was not significantly different among patients with stage I, II, and III (P = 0.33). [Conclusion] Prognosis of limited-stage MCL was almost similar to that of stage III MCL. Although the present study includes several limitations including a retrospective nature and limited number of patients, prognosis of patients with limited-stage MCL was not satisfactory. The significance of radiotherapy, as well as the optimal choice of chemotherapy, for limited-stage MCL needs re-evaluation. Table Table. Figure Figure. Disclosures Suzuki: Chugai: Honoraria; Kyowa Hakko kirin: Honoraria; Bristol-Myers Squibb: Honoraria. Asano:Jannsen: Honoraria; Chugai: Honoraria. Kinoshita:Ono: Research Funding; Gilead: Research Funding; Zenyaku: Honoraria, Research Funding; Takeda: Research Funding; Chugai: Honoraria, Research Funding; Eisai: Honoraria, Research Funding; Solasia: Research Funding; Janssen: Honoraria; Kyowa Kirin: Honoraria. Suzumiya:Chugai: Honoraria, Research Funding; Astellas: Research Funding; Eisai: Honoraria, Research Funding; Takeda: Honoraria; Toyama Chemical: Research Funding; Kyowa Hakko kirin: Research Funding. Ogura:SymBio Pharmaceuticals: Consultancy, Honoraria; Celltrion, Inc.: Consultancy, Honoraria.


2018 ◽  
Vol 48 (4) ◽  
Author(s):  
Simone Carvalho dos Santos Cunha ◽  
Katia Barão Corgozinho ◽  
Franciele Basso Fernandes Silva ◽  
Kassia Valéria Gomes Coelho da Silva ◽  
Ana Maria Reis Ferreira

ABSTRACT: Our retrospective study evaluated the survival of 24 dogs with unresectable malignant melanoma treated with radiation therapy. Fifteen dogs were treated with radiation therapy (RT) and chemotherapy (CT), five with surgery followed by RT and CT, three with palliative RT, and one with electrochemotherapy associated with RT. All dogs were treated with an orthovoltage Stabilipan I. The protocol used was three or four weekly fractions of 8 Gy. Carboplatin was administered every 21 days, a total of four times. Five percent of dogs were classified as having stage I melanoma, 17% as stage II, 50% as stage III, and 17% as stage IV. Sixty-four percent had a partial response to treatment, 29% achieved complete remission, and 7% remained in a stable disease state. The mean survival time was 390 days for stage I, 286 days for stage II, 159 days for stage III, and 90 days for stage IV. We concluded that radiation therapy can be considered a viable alternative for the palliative treatment of canine oral melanoma.


1986 ◽  
Vol 32 (1) ◽  
pp. 159-161 ◽  
Author(s):  
B A Faraj ◽  
V M Camp ◽  
D R Murray ◽  
M Kutner ◽  
J Hearn ◽  
...  

Abstract We determined the concentration of L-dopa in the plasma of 98 patients with biopsy-proven melanoma, a dermatological neoplasm that is characterized biochemically by abnormal tyrosine metabolism. For 21 patients previously diagnosed as having melanoma but who were clinically free of disease (stage I), the mean concentration of L-dopa in plasma, 1.01 (SD 0.12) micrograms/L, was not significantly different from that of 32 normal controls, 1.23 (SD 0.16) micrograms/L. However, L-dopa was increased significantly (p less than 0.001) in the plasma of all of 65 patients with active disease (stage II), 2.08 (SD 0.46) micrograms/L, and was highest in 12 patients with stage III malignant melanoma, 8.40 (SD 3.50) micrograms/L. The development of metastases in four patients with stage II melanoma was accompanied by an increase in the concentration of plasma L-dopa. These studies suggest that measurement of plasma L-dopa may be useful in the diagnosis of melanoma.


2012 ◽  
Vol 38 (5) ◽  
pp. 468-473 ◽  
Author(s):  
T. Lequint ◽  
K. Naito ◽  
T. Awada ◽  
S. Facca ◽  
P. Liverneaux

The treatment of ulnar nerve compression at the elbow remains controversial. No single technique has yet proven its superiority. We describe a technique combining the advantages of the mini-invasive approach with those of transposition. We present the results of 30 patients, of mean age 52 years, who underwent anterior subcutaneous transposition of the ulnar nerve using a mini-invasive approach with a follow-up of more than six months. The incision measures 3 cm. The results were evaluated by measuring pain intensity, quick disabilities of the arm shoulder and hand (DASH), grip strength and pinch, and McGowan score, pre- and post-operatively. All parameters were improved post-operative. The mean pain score went from 5.5 to 4, the quick DASH from 48 to 38, mean grip strength from 28 to 31 kg, and mean pinch strength from 4.7 to 6.4 kg. The McGowan score was also improved; pre-operatively, there were 16 patients at stage III, seven patients stage II, seven patients stage I, and post-operatively there was one patient stage III, three patients stage II, 16 patients stage I, and 10 patients stage 0. Analysis of our series shows that a 3 cm incision without endoscopy allows subcutanous transposition, with results at least as good as those with other techniques. The advantages of our technique are that it is easy, has a limited approach, preserves blood supply, allows placement of the nerve in a favourable environment, and decreases nerve stretching during elbow flexion.


2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Leily Mohajerzadeh ◽  
Ahmad Khaleghnejad ◽  
Mohsen Rouzrokh ◽  
Shahin Shamsian ◽  
Javad Ghoroubi ◽  
...  

Background: Wilms’ tumor (nephroblastoma) is the major renal cancer in children. Objectives: The aim of this study was to assess the individuality of Wilms’ tumor and the consequences of management attained in our referral subspecialty center. Methods: In this study, we composed the data of children with Wilms’ tumor in 2 decades; 55 cases between 1992 and 2002 and 49 patients between 2006 and 2016 were diagnosed with Wilms’ tumor. Demographic characters, a form of presentation, tumor stage, related underlying disease, histopathology consequences, type of management, and the survival rates were assessed. Results: In the first decade, 24 patients were females and 31 were males (M/F = 1.2); in the other groups, 30 were females and 19 were males (M/F = 0.61). The mean age was 45.2 months at the time of diagnosis for the first group and the mean age was 36 months for the other group. In the first decade, the surgical stage after the operation was as follows: stage I (32.7%), stage II (16.36%), stage III (38.1%), stage IV (9%), and stage V (1.8%) who did not operate. In second decade, 49 patients were as follows: stage I (14.3%), stage II (40.8%), stage III (24.5%), stage IV (10.2%), and stage V (10.2%). In 54.5% of the first group, histology was favorable, and in 43.6% of the first group, histology was unfavorable; in the second group, 95.4% were the favorable type. The patients were managed based on protocols of the National Wilms’ Tumor Study. In the first decade, relapse-free was 71% and 4-year survival rates were estimated at 86%, and in the second decade, pulmonary metastasis was observed at 28.6%, liver metastasis in 2.3%, recurrence in 5%, and 4-year survival rates were estimated at 90%. Conclusions: This study demonstrated development in the management of children with Wilms’ tumor in recent 20 years, with comparable relapse-free and survival rates to the National Wilms’ Tumor study. But with more adjustment in treatment protocols, the superior outcome will be attainable.


2020 ◽  
pp. 019459982097118
Author(s):  
Liliya Benchetrit ◽  
Megan Kwock ◽  
Evette Ronner ◽  
Sheli Goldstein ◽  
Edina Shu ◽  
...  

Objective To compare postoperative pain and analgesic use in children following transcanal endoscopic ear surgery (TEES) vs non–transcanal endoscopic ear surgery (non-TEES). Study Design Prospective case series. Setting Tertiary care center. Methods Surveys using the Wong-Baker FACES Pain Rating Scale and recording the frequency and dosage of consumed analgesics were administered prospectively to caregivers of children undergoing otologic surgery between May 2018 to February 2020. Pain intensity and medication use were recorded twice daily for 6 days, starting on postoperative day 0. Mean pain scores and mean number of consumed analgesic doses were compared between groups. Results Survey response rate was 57.9%. Among 53 patients who completed the survey, 35 (66.0%) underwent TEES and 18 (34.0%) underwent non-TEES. Mean pain ratings on postoperative days 0 and 1 were significantly lower among children undergoing TEES (2.2 and 2.1) vs non-TEES (4.0 and 4.1), P = .045 and P = .008, respectively (Mann-Whitney U test). The mean pain ratings across the 6 days were similar in TEES (1.7) and non-TEES (2.6) ( P = .140, Mann-Whitney U test). The mean number of analgesic doses consumed per half-day over the 6 days was significantly lower among children undergoing TEES (0.3) vs non-TEES (0.6; P = .049, Mann-Whitney U test). Conclusion Postoperative pain following TEES and non-TEES in children was overall low. Children undergoing TEES had a small but statistically significant decrease in pain on postoperative days 0 and 1 and decreased use of pain medications compared to non-TEES.


2020 ◽  
Vol 15 ◽  
Author(s):  
Athira K ◽  
Vrinda C ◽  
Sunil Kumar P V ◽  
Gopakumar G

Background: Breast cancer is the most common cancer in women across the world, with high incidence and mortality rates. Being a heterogeneous disease, gene expression profiling based analysis plays a significant role in understanding breast cancer. Since expression patterns of patients belonging to the same stage of breast cancer vary considerably, an integrated stage-wise analysis involving multiple samples is expected to give more comprehensive results and understanding of breast cancer. Objective: The objective of this study is to detect functionally significant modules from gene co-expression network of cancerous tissues and to extract prognostic genes related to multiple stages of breast cancer. Methods: To achieve this, a multiplex framework is modelled to map the multiple stages of breast cancer, which is followed by a modularity optimization method to identify functional modules from it. These functional modules are found to enrich many Gene Ontology terms significantly that are associated with cancer. Result and Discussion: predictive biomarkers are identified based on differential expression analysis of multiple stages of breast cancer. Conclusion: Our analysis identified 13 stage-I specific genes, 12 stage-II specific genes, and 42 stage-III specific genes that are significantly regulated and could be promising targets of breast cancer therapy. That apart, we could identify 29, 18 and 26 lncRNAs specific to stage I, stage II and stage III respectively.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
P Milani ◽  
L Obici ◽  
R Mussinelli ◽  
M Basset ◽  
G Manfrinato ◽  
...  

Abstract Background Cardiac wild type transthyretin (ATTRwt) amyloidosis, formerly known as senile systemic amyloidosis, is an increasingly recognized, progressive, and fatal cardiomyopathy. Two biomarkers staging systems were proposed based on NT-proBNP (in both cases) and troponin or estimated glomerular filtration rate, that are able to predict survival in this population. The availability of novel effective treatments requires large studies to describe the natural history of the disease in different populations. Objective To describe the natural history of the disease in a large, prospective, national series. Methods Starting in 2007, we protocolized data collection in all the patients diagnosed at our center (n=400 up to 7/2019). Results The referrals to our center increased over time: 5 cases (1%) between 2007–2009, 33 (9%) in 2010–2012, 90 (22%) in 2013–2015 and 272 (68%) in 2016–2019. Median age was 76 years [interquartile range (IQR): 71–80 years] and 372 patients (93%) were males. One hundred and seventy-three (43%) had atrial fibrillation, 63 (15%) had a history of ischemic cardiomyopathy and 64 (15%) underwent pacemaker or ICD implantation. NYHA class was I in 58 subjects (16%), II in 225 (63%) and III in 74 (21%). Median NT-proBNP was 3064 ng/L (IQR: 1817–5579 ng/L), troponin I 0.096 ng/mL (IQR: 0.063–0.158 ng/mL), eGFR 62 mL/min (IQR: 50–78 mL/min). Median IVS was 17 mm (IQR: 15–19 mm), PW 16 mm (IQR: 14–18 mm) and EF 53% (IQR: 45–57%). One-hundred and forty-eight subjects (37%) had a concomitant monoclonal component in serum and/or urine and/or an abnormal free light chain ratio. In these patients, the diagnosis was confirmed by immunoelectron microscopy or mass spectrometry. In 252 (63%) the diagnosis was based on bone scintigraphy. DNA analysis for amyloidogenic mutations in transthyretin and apolipoprotein A-I genes was negative in all subjects. The median survival of the whole cohort was 59 months. The Mayo Clinic staging based on NT-proBNP (cutoff: 3000 ng/L) and troponin I (cutoff: 0.1 ng/mL) discriminated 3 different groups [stage I: 131 (35%), stage II: 123 (32%) and stage III: 127 (33%)] with different survival between stage I and II (median 86 vs. 81 months, P=0.04) and between stage II and III (median 81 vs. 62 months, P&lt;0.001). The UK staging system (NT-proBNP 3000 ng/L and eGFR 45 mL/min), discriminated three groups [stage I: 170 (45%), stage II: 165 (43%) and stage III: 45 (12%)] with a significant difference in survival: between stage I and stage II (86 vs. 52 months, P&lt;0.001) and between stage II and stage III (median survival 52 vs. 33 months, P=0.045). Conclusions This is one of the largest series of patients with cardiac ATTRwt reported so far. Referrals and diagnoses increased exponentially in recent years, One-third of patients has a concomitant monoclonal gammopathy and needed tissue typing. Both the current staging systems offered good discrimination of staging and were validated in our independent cohort. Funding Acknowledgement Type of funding source: None


2019 ◽  
Vol 10 (2) ◽  
pp. 164-170 ◽  
Author(s):  
Valdano Manuel ◽  
Humberto Morais ◽  
Aida L. R. Turquetto ◽  
Gade Miguel ◽  
Leonardo A. Miana ◽  
...  

Introduction: Single ventricle physiology management is challenging, especially in low-income countries. Objective: To report the palliation outcomes of single ventricle patients in a developing African country. Methods: We retrospectively studied 83 consecutive patients subjected to single ventricle palliation in a single center between March 2011 and December 2017. Preoperative data, surgical factors, postoperative results, and survival outcomes were analyzed. The patients were divided by palliation stage: I (pulmonary artery banding [PAB] or Blalock–Taussig shunt [BTS]), II (Glenn procedure), or III (Fontan procedure). Results: Of the 83 patients who underwent palliation (stages I-III), 38 deaths were observed (31 after stage I, six after stage II, and one after stage III) for an overall mortality of 45.7%. The main causes of operative mortality were multiple organ dysfunction due to sepsis, shunt occlusion, and cardiogenic shock. Twenty-eight survivors were lost to follow-up (22 after stage I, six after stage II). Thirteen stage II survivors are still waiting for stage III. The mean follow-up was 366 ± 369 days. Five-year survival was 28.4 % for PAB and 30.1% for BTS, while that for stage II and III was 49.8% and 57.1%, respectively. Age (hazard ratio, 0.61; 95% confidence interval: 0.47-0.7; P = .000) and weight at surgery (hazard ratio, 0.45; 95% confidence interval: 0.31-0.64; P = .002) impacted survival. Conclusion: A high-mortality rate was observed in this initial experience, mainly in stage I patients. A large number of patients were lost to follow-up. A task force to improve outcomes is urgently required.


2011 ◽  
Vol 2011 ◽  
pp. 1-7 ◽  
Author(s):  
Chang-Ming Huang ◽  
Jian-Xian Lin ◽  
Chao-Hui Zheng ◽  
Ping Li ◽  
Jian-Wei Xie ◽  
...  

Objectives. To investigate the prognostic impact of the number of dissected lymph nodes (LNs) in gastric cancer after curative distal gastrectomy.Methods. The survival of 634 patients who underwent curative distal gastrectomy from 1995 to 2004 was retrieved. Long-term surgical outcomes and associations between the number of dissected LNs and the 5-year survival rate were investigated.Results. The number of dissected LNs was one of the most important prognostic indicators. Among patients with comparable T category, the larger the number of dissected LNs was, the better the survival would be (). The linear regression showed that a significant survival improvement based on increasing retrieved LNs for stage II, III and IV (). A cut-point analysis yields the greatest variance of survival rate difference at the levels of 15 LNs (stage I), 25 LNs (stage II) and 30 LNs (stage III).Conclusion. The number of dissected LNs is an independent prognostic factor for gastric cancer. To improve the long-term survival of patients with gastric cancer, removing at least 15 LNs for stage I, 25 LNs for stage II, and 30 LNs for stage III patients during curative distal gastrectomy is recommended.


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