Airway Hemangiomas in PHACE Syndrome: A Multicenter Experience.

OBJECTIVE: To describe the prevalence and clinical characteristics of airway findings in a multi-institutional cohort of PHACE patients. STUDY DESIGN: Multicenter retrospective case series. SETTING: Multidisciplinary vascular anomalies clinics at 2 institutions. METHODS: Data were collected from the electronic medical record, including clinical presentation, airway findings, treatment, and outcomes. RESULTS: Of 55 PHACE patients, 22 (40%) had airway hemangiomas. Patients with airway involvement were more commonly female (P = .034, odds ratio [OR] 23, 95% confidence interval [CI] 1.3-410) and of Caucasian ethnicity (P = .020, OR 5.3, 95% CI 1.3-21). Anatomically, patients with bilateral S3 involvement had higher rates of airway disease (P = .0012, OR 15, 95% CI 2.9-77). Most patients with airway hemangiomas had stridor (68%). Of the patients managed in the propranolol era (2008 or later, n = 35), 14 had airway involvement. All 14 were treated with propranolol, whereas 13 (62%) of 21 nonairway patients were treated with propranolol. The average treatment duration was longer in the airway patients (22.1 vs 16.7 months). All patients who underwent tracheostomy (n = 4) did so before 2008. CONCLUSION: Risk factors for airway involvement in PHACE include female gender, Caucasian ethnicity, and stridor. Since the widespread use of propranolol, fewer patients have required surgical management of their airway disease. Given the high prevalence of airway involvement even in patients without stridor, assessment of the airway is a crucial component of a comprehensive PHACE workup.

Indications and clinical outcomes of fully covered self-expandable metallic tracheobronchial stents in patients with malignant airway diseases.

BACKGROUND: Airway stenting is an efficacious approach in management of malignant airway disease (MAD) with improvement in survival outcome. OBJECTIVE: To determine the indications and long-term clinical outcomes of tracheobronchial stenting in patients with MAD. METHODS: A cross-sectional review of 51 patients who underwent airway stenting from June 2011 to June 2019 was done. Paired t-test was used to compare mean difference of clinical characteristics between pre- and post-airway stenting. Kaplan-Meier curves were used to assess overall survival. RESULTS: A total of 51 patients had stent insertion with mean age 46.63±17.10years including 27(52.9%) females. Mainly 37(72.5%) patients had esophageal and 06(11.8%) had lung cancer. The main indications were bronchial stenosis 18(35.3%), tracheal stenosis 11(21.6%) and Tracheo-esophageal/bronchial fistula 13(25.5%). Obstruction was intrinsic, extrinsic and mixed in 20(39.2%), 13(25.5%) and 5(9.8%) patients, respectively. There was statistically significant mean difference in pre- and post-procedure oxygen saturation (mean (M)=89.8, standard deviation (SD)=6.70 vs M =95.5,SD=2.54.p =0.001) and performance status (M =3.65,SD =0.6 vs M =2.59, SD=0.83.p =0.001). Overall median survival was 16±3.44 weeks, highest amongst patients with intrinsic obstruction (27±6.51 weeks). CONCLUSION: Airway stenting is an effective endoscopic procedure to re-establish airway patency in MAD with minimal complications..

An Open-label, Single-arm Study of CRYO2 for Debulking at the Site of Central Airway Obstruction or Stenosis.

BACKGROUND: An open-label, single-arm study was conducted to assess the safety of a cryosurgery unit named CRYO2 for debulking at the site of obstruction or stenosis. PATIENTS AND METHODS: In order to treat central airway tumor-related stenosis, debulking at the stenotic site of the airway was performed using CRYO2 under general or local anesthesia. The primary endpoint was the incidence of moderate to massive hemorrhage. RESULTS: Incidence of moderate to massive hemorrhage during surgery was 3.8% (1/26) (95% confidence interval(CI)=0.1-19.6%). Technical success was 96.2% (25/26), with a 95% confidence interval of 80.4-99.9%. CONCLUSION: CRYO2 for debulking at the site of obstruction or stenosis can be performed safely.

Risk and outcomes of second malignant neoplasms in chronic myeloid leukemia survivors.

The risk of second malignant neoplasms (SMN) in chronic myeloid leukemia (CML) survivors remains unclear. We utilized the Surveillance, Epidemiology and End Results 18 (SEER 18) registries to evaluate the risk and subsequent outcomes of SMN in CML survivors. There were 3407 patients included. Of these, 170 (4.99%) developed a SMN with SIR of 1.40 (95% C.I. 1.19-1.62). An increased risk was noted for cancers of the respiratory tract, genitourinary (GU) tract and skin excluding basal cell and squamous cell carcinoma. Using 3:1 matching (3 de novo malignancies to 1 post-CML SMN case), we compared survival data for cancers of the respiratory, GU and gastrointestinal (GI) tract. Patients with GU malignancies developing after CML had worse overall survival than patients without prior CML diagnosis (P = 0.018). There was no difference in survival between post-CML and non-post-CML patients with respiratory or GI malignancies.

[Endoscopic early diagnosis of carcinomas in upper respiratory and digestive tract]. / Endoskopische Frühdiagnostik von Karzinomen im oberen Aerodigestivtrakt.

Modern endoscopic imaging techniques make it possible to detect tumor diseases of the upper respiratory and digestive tract and treat them minimally invasive - with a good oncologic outcome and maintaining the functionality of the tissue.Horizontal techniques permit the inspection of big mucous membrane surfaces, searching vor areas suspicious of dysplasia or tumor. They can be used as screening techniques. Vertical techniques serve for precise examination of in-depth expansion, infiltration chraracteristics and dignity of known lesions. Cellular techniques deal with the detection of cellular changes in vivo. As the techniques have different advantages and disandvantages, it is recommended to combine several techniques for best diagnostic gains.

Management of Infantile Hemangiomas of the Airway.

Infantile hemangiomas (IHs) of the airway are far less common than their cutaneous counterparts, and their symptoms mimic those of viral croup. As a result, by the time these lesions are diagnosed, they are often advanced and causing airway compromise. Fortunately, the evolution of propranolol as an effective and safe pharmacotherapy has simplified management of IH and reduced the likelihood of complications previously seen with steroid therapy and surgery. Nevertheless, the otolaryngologist must be prepared with an alternate plan to manage lesions refractory to pharmacotherapy. This article reviews the clinical presentation and current management of IHs of the airway.

The Management of Vascular Malformations of the Airway: Natural History, Investigations, Medical, Surgical and Radiological Management.

Vascular malformations may affect nearly all aspects of the upper airway. Each type of malformation has a characteristic pattern of disease. These lesions may be focal or diffuse, and require directed management strategies. Physicians treating these entities should have a high level of suspicion to consider airway evaluation even in the absence of overt symptoms. However, cutaneous head and neck venous malformations or other lesions affecting the lips, oral cavity, or tongue can herald the presence of coexisting airway lesions. A multidisciplinary approach is critical in achieving comprehensive treatment.

A Case Series of the Probability Density and Cumulative Distribution of Laryngeal Disease in a Tertiary Care Voice Center.

OBJECTIVE: To examine the distribution of clinic and operative pathology in a tertiary care laryngology practice. METHODS: Probability density and cumulative distribution analyses (Pareto analysis) was used to rank order laryngeal conditions seen in an outpatient tertiary care laryngology practice and those requiring surgical intervention during a 3-year period. RESULTS: Among 3783 new clinic consultations and 1380 operative procedures, voice disorders were the most common primary diagnostic category seen in clinic (n = 3223), followed by airway (n = 374) and swallowing (n = 186) disorders. Within the voice strata, the most common primary ICD-9 code used was dysphonia (41%), followed by unilateral vocal fold paralysis (UVFP) (9%) and cough (7%). Among new voice patients, 45% were found to have a structural abnormality. The most common surgical indications were laryngotracheal stenosis (37%), followed by recurrent respiratory papillomatosis (18%) and UVFP (17%). CONCLUSIONS: Nearly 55% of patients presenting to a tertiary referral laryngology practice did not have an identifiable structural abnormality in the larynx on direct or indirect examination. The distribution of ICD-9 codes requiring surgical intervention was disparate from that seen in clinic. Application of the Pareto principle may improve resource allocation in laryngology, but these initial results require confirmation across multiple institutions.

Lymphocyte profile and cytokine mRNA expression in peripheral blood mononuclear cells of patients with recurrent respiratory papillomatosis suggest dysregulated cytokine mRNA response and impaired cytotoxic capacity.

OBJECTIVES: Recurrent respiratory papillomatosis (RRP) is a relatively rare, chronic disease caused by Human Papilloma Virus (HPV) 6 and 11, and characterized by wart-like lesions in the airway affecting voice and respiratory function. The majority of HPV infections are asymptomatic and resolve spontaneously, however, some individuals are afflicted with persistent HPV infections. Failure to eliminate HPV 6 and 11 due to a defect immune responsiveness to these specific genotypes is proposed to play a major role in the development of RRP. METHODS: We performed a phenotypic characterization of peripheral blood mononuclear cells (PBMC) collected from 16 RRP patients and 12 age-matched healthy controls, using immunoflow cytometry, and monoclonal antibodies against differentiation and activation markers. The cytokine mRNA profile of monocytes, T helper-, T cytotoxic-, and NK cells was assessed using RT-qPCR cytokine analysis, differentiating between Th1-, Th2-, Th3/regulatory-, and inflammatory immune responses. RESULTS: We found a dominance of cytotoxic T cells, activated NK cells, and high numbers of stressed MIC A/B expressing lymphocytes. There was an overall suppression of cytokine mRNA production and an aberrant cytokine mRNA profile in the activated NK cells. CONCLUSION: These findings demonstrate an immune dysregulation with inverted CD4+ /CD8+ ratio and aberrant cytokine mRNA production in RRP patients, compared to healthy controls.

The Different Clinical Aspects of Pediatric Primary Airway Tumors in the Larynx, Trachea, and Bronchi.

Primary airway tumors are rare in children and no literature reviewed their characteristics each location. We evaluate the clinical characteristics and outcomes of Korean children with primary airway tumors, from the larynx to bronchi. A retrospective chart review of children with primary tumors of the larynx, trachea, and bronchi at Asan Medical Center from January 2000 to July 2016 was conducted. Nineteen children were diagnosed with primary airway tumors of the larynx (47.4%), trachea (10.5%), and bronchi (42.1%). Median follow-up duration was 2.8 years and there were recurrences in 21.1%. Laryngeal tumors were associated with a younger median age at onset (2 months) and diagnosis (4 months), and most were relatively small (median size = 5.3 mm) and symptomatic. Tracheal and bronchial tumors were found in older children (age at onset and diagnosis > 11 years) and large (> 15.0 mm). Most (75%) patients with bronchial tumors were asymptomatic and all the patients with tracheal tumors were symptomatic. This study suggests that we should consider different the locations in primary airway tumor based on the age at onset and diagnosis, initial symptoms or signs, and size of tumor.

COMPARISON BETWEEN THORACIC RADIOGRAPHIC FINDINGS AND POSTMORTEM DIAGNOSIS OF THORACIC DISEASES IN DYSPNEIC COMPANION RATS (RATTUS NORVEGICUS).

Companion rats are often presented to veterinarians for respiratory difficulties. Dyspnea in rats is most commonly due to infectious pneumonia, and thoracic neoplasia can go undiagnosed ante mortem due to a mistaken interpretation of pneumonia. In domestic carnivores, pulmonary nodular patterns have been shown to correlate with lung neoplastic diseases and infectious diseases. The main objective of this retrospective case series study was to determine whether certain radiographic criteria could be correlated with the presence of thoracic infectious disease and neoplastic disease in companion rats. A secondary objective was to determine whether the patient's sex and age were different between rats diagnosed with infectious versus neoplastic disease. Medical records and thoracic radiographs of dyspneic companion rats presented to the University of California at Davis, William R. Pritchard Veterinary Medical Teaching Hospital during the time period from January 2000 to December 2014 were reviewed. Rats with postmortem confirmation of thoracic lesions were included in the study. Thoracic radiographs were evaluated for positioning, lesion distribution, lung lobe involved, pulmonary pattern, mediastinal and pleural lesions by three observers blinded to diagnosis. Thirty rats were included in the study, including 23 rats with an infectious disease and seven with neoplasia. Mediastinal lesions were significantly more prevalent in the group diagnosed with thoracic neoplasia (P = 0.031), in particular cranially (P = 0.048). Although there was an overlap between the two groups, findings indicated that the presence of cranial mediastinal lesions may be helpful for differentiating neoplastic from infectious disease in rats.

Hernia of the swim bladder (aerocystocele) with concurrent mycotic granulomatous inflammation and swim bladder carcinoma in a wild mullet (Mugil cephalus).

We describe a hernia of the swim bladder, with a concurrent mycotic granulomatous inflammation, and carcinoma of the swim bladder in a wild mullet (Mugil cephalus) referred for an exophytic dorsal mass. Grossly, the mass was white, soft, and composed of multiple cystic gas-containing chambers connected by a funnel-shaped tissue segment to the coelomic swim bladder. Histologically, the mass was characterized by cysts of variable size, multifocally contiguous with the subepithelial rete mirabile, supported by abundant fibrous tissue. The skin covering the herniated swim bladder was focally ulcerated and replaced by abundant granulation tissue in which multiple scattered granulomas centered on pigmented fungal hyphae were observed. These granulomas were also seen in the remaining coelomic portion of the swim bladder as well as in the spleen, perivisceral pancreas, and peritoneal adipose tissue; the fungus was molecularly identified as Cladosporium spp. Focally, arising from the herniated swim bladder epithelium, an unencapsulated poorly demarcated, moderately cellular neoplasm, composed of islands, lobules, and acini of neoplastic epithelium, was found.

Ultrasound-guided Lung Biopsy in the Hands of Respiratory Physicians: Diagnostic Yield and Complications in 215 Consecutive Patients in 3 Centers.

BACKGROUND: The aim of the study was to determine the diagnostic yield and prevalence of complications of ultrasound-guided transthoracic needle aspiration biopsies (US-TTNAB) performed by respiratory physicians after implementation of the procedure in an everyday clinical setting at 3 different centers. METHODS: Patients were included if they during the period from January 2012 to August 2014 had a registered US-TTNAB procedure code or if a US biopsy registration form had been filled out at either of the participating centers. Histology or cytology results were used as a reference test for diagnoses that could be made based on these results. Reference test for the remaining diagnoses was clinical follow-up. The diagnostic yield of US-TTNAB was defined as the proportion of patients in which the result of the US-TTNAB was consistent with the reference test. RESULTS: A total of 215 patients in which a primary US-TTNAB had been performed were identified. The most common biopsy sites were lungs and pleurae with a total of 164 (76.3%) patients and 31 patients (14.4%), respectively. US-TTNAB diagnostic yield was 76.9% (95% CI, 70.3%-83.4%) for malignant diagnoses and 47.6% (95% CI, 31.9%-63.4%) for nonmalignant diagnoses. The most common complications of US-TTNAB were pneumothorax (2.5%; 95% CI, 0.03%-4.6%) and pain at the biopsy site (2%; 95% CI, 0.04%-3.9%). No fatalities related to US-TTNAB were observed. CONCLUSION: US-TTNAB performed by respiratory physicians is a safe procedure with a low risk of complications and the diagnostic yield to establish a malignant diagnosis is acceptable.

Acoustical and Perceptual Voice Profiling of Children With Recurrent Respiratory Papillomatosis.

OBJECTIVES: Respiratory papillomatosis is a condition characterized by benign papillomatous (wart-like) growths in the respiratory tract. The condition tends to recur after treatment, requiring multiple procedures to control growth of the lesions. In such cases, the condition is known as recurrent respiratory papillomatosis (RRP). This study aims at providing comparative measures of acoustic as well as perceptual voice analysis of children with RRP. METHODS: Ten children with a history of RRP for a minimum of 3 years were selected under purposive sampling. A detailed case history, Clinical Assessment Scale of RRP, and laryngostroboscopy findings were done. This was followed by perceptual voice assessment using Buffalo III Voice Screening Profile, and acoustical voice assessment using Dr. Speech software (by Tiger DRS, Inc.). Group statistics and t test were used at a significance level of 0.05 (SPSS package Version 12.0 is used which is manufactured by IBM Corporation). RESULTS: Both groups with RRP showed substantial difference in habitual F0, S/Z ratio, Signal to Noise Ratio (SNR), Harmonic to Noise Ratio (HNR), and Maximum Phonation Time (MPT) voice parameters. On perceptual analysis, all children with RRP had hoarse voice quality, and stridors are present. CONCLUSIONS: All children with RRP have affected voice parameters in some extent which depend on the period and area of lesion.

CFD modeling and image analysis of exhaled aerosols due to a growing bronchial tumor: towards non-invasive diagnosis and treatment of respiratory obstructive diseases.

Diagnosis and prognosis of tumorigenesis are generally performed with CT, PET, or biopsy. Such methods are accurate, but have the limitations of high cost and posing additional health risks to patients. In this study, we introduce an alternative computer aided diagnostic tool that can locate malignant sites caused by tumorigenesis in a non-invasive and low-cost way. Our hypothesis is that exhaled aerosol distribution is unique to lung structure and is sensitive to airway structure variations. With appropriate approaches, it is possible to locate the disease site, determine the disease severity, and subsequently formulate a targeted drug delivery plan to treat the disease. This study numerically evaluated the feasibility of the proposed breath test in an image-based lung model with varying pathological stages of a bronchial squamous tumor. Large eddy simulations and a Lagrangian tracking approach were used to model respiratory airflows and aerosol dynamics. Respirations of tracer aerosols of 1 µm at a flow rate of 20 L/min were simulated, with the distributions of exhaled aerosols recorded on a filter at the mouth exit. Aerosol patterns were quantified with multiple analytical techniques such as concentration disparity, spatial scanning and fractal analysis. We demonstrated that a growing bronchial tumor induced notable variations in both the airflow and exhaled aerosol distribution. These variations became more apparent with increasing tumor severity. The exhaled aerosols exhibited distinctive pattern parameters such as spatial probability, fractal dimension, and multifractal spectrum. Results of this study show that morphometric measures of the exhaled aerosol pattern can be used to detect and monitor the pathological states of respiratory diseases in the upper airway. The proposed breath test also has the potential to locate the site of the disease, which is critical in developing a personalized, site-specific drug delivery protocol.

Influence of pharyngolaryngeal anomalies diagnosed through indirect laryngoscopy in the prediction of difficult intubation.

OBJECTIVE: To determine the pharyngolaryngeal anomalies not usually included in the evaluation of difficult airway, in order to investigate the influence of these anomalies in the prediction of difficult intubation. To do this, indirect laryngoscopy with a 70° rigid laryngoscope was performed on all patients during the preoperative period. METHODS: This is an observational, prospective study on 300 consecutive patients who were scheduled for endotracheal intubation under general anesthesia. In addition to assessing the airway in the preoperative period by demographic and clinical predictors of difficult airway, rigid indirect laryngoscopy was performed to diagnose pharyngolaryngeal anomalies. Later, under general anesthesia and direct laryngoscopy it was checked to see if there was difficulty in intubating the larynx, and its association with all previous variables was investigated. A logistic regression model for prediction purposes was developed, and its power of discrimination was achieved by assessing the area under the curve. RESULTS: During the examination by indirect laryngoscopy 46 anomalies were found, which were as follows: 31 abnormalities of the epiglottis (22 omega epiglottis, 9 flaccid or hypertrophic epiglottis); 6 findings of hypertrophic lingual tonsils, 3 cases of upper airway tumors, and 6 patients with tongue disorders. Intubation difficulty was found in 14 cases (4.66%). The regression model found, and its coefficients to develop it were: f(x)=1.322+(2.173 thyromental distance <6.5 cm)+(1.813 omega epiglottis)-(1.310*cm opening mouth). Global power of discrimination was 0.83, with a 95% confidence interval from 0.709 to 0.952). CONCLUSION: Indirect laryngoscopy allowed pharyngolaryngeal anomalies to be diagnosed, including omega epiglottis, which was one of the variables included in the logistic regression model.

[The indication of panendoscopy in the search for synchronous and metachronous head and neck cancer]. / Rôte de la panendoscopie dans la recherche de cancers synchrones et métachrones de ta sphère ORL.

Head and neck cancer result from excessive tobacco and alcohol consumption. The survival has not improved in the last decades despite better loco-regional control, mainly because of secondary cancers. Head and neck cancer is associated to a high rate of synchronous esophageal and lung tumors and an annual 4% rate of new metachronous malignancies. The role of panendoscopy is evolving: a flexible esophagoscopy with Lugol's iodine staining should be performed and a chest CT-scan should replace bronchoscopy. During follow-up, metachronous malignancy should be searched for in the head and neck region; investigation beyond the head and neck is questionable since prognosis is poor.