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1.
Fetal Diagn Ther ; 50(6): 472-479, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37544292

RESUMO

INTRODUCTION: Large congenital neck tumors can cause neonatal death due to airway obstruction. The aim of this study was to report outcomes of the first cohort of fetuses with neck masses and suspected airway obstruction managed with fetal laryngoscopy (FL) and fetal endoscopic tracheal intubation (FETI) to secure fetal airways and avoid ex utero intrapartum treatment (EXIT) procedure. METHODS: A prospective observational cohort of consecutive fetuses with neck masses that were candidates for an EXIT procedure due to suspicion of laryngeal and/or tracheal occlusion on ultrasonographic (US) or magnetic resonance imaging (MRI) examination were recruited for FL in a tertiary referral center in Queretaro, Mexico. FETI was performed if the obstruction was confirmed by FL. Maternal and perinatal outcomes were evaluated. RESULTS: Between January 2012 and March 2023, 35 cases with neck masses were evaluated. Airway obstruction was suspected in 12/35 (34.3%), either by US in 10/35 (28.6%) or by fetal MRI in 2/35 (5.7%). In all cases, FL was successfully performed at the first attempt at a median gestational age (GA) of 36+5 (range, 33+5-39+6) weeks+days, with a median surgical time of 22.5 (12-35) min. In 4 cases, airway patency was confirmed during FL and an EXIT procedure was avoided. In 8/12 cases (66.7%), airway obstruction was confirmed during fetoscopy and FETI was successfully performed at a median GA of 36+3 (33+2-38+5) weeks+days, with a median surgical time of 25.0 (range, 12-45) min. No case required an EXIT procedure. All patients underwent conventional cesarean delivery with no maternal complications and all neonates were admitted to the neonatal intensive care unit with a correctly positioned endotracheal tube (ETT) immediately after delivery. Three neonatal deaths (37.5%) were reported due to postnatal unplanned extubation, failed ETT replacement, and tumoral bleeding. CONCLUSION: In fetuses with neck masses and suspected airway obstruction, FL and FETI are feasible and could replace EXIT procedures with good maternal and perinatal outcomes.


Assuntos
Obstrução das Vias Respiratórias , Laringoscopia , Gravidez , Feminino , Recém-Nascido , Humanos , Laringoscopia/efeitos adversos , Intubação Intratraqueal/efeitos adversos , Intubação Intratraqueal/métodos , Feto , Cuidado Pré-Natal , Obstrução das Vias Respiratórias/diagnóstico por imagem , Obstrução das Vias Respiratórias/cirurgia , Obstrução das Vias Respiratórias/etiologia
2.
J Avian Med Surg ; 34(2): 181-185, 2020 Jul 21.
Artigo em Inglês | MEDLINE | ID: mdl-32702958

RESUMO

A 17-year-old, female cockatiel (Nymphicus hollandicus) was presented for a 1-week history of respiratory distress. The clinical signs were consistent with tracheal obstruction; therefore, an air sac cannula was placed to reduce respiratory compromise. Radiographic images of the patient revealed a round, intraluminal, soft tissue opacity in the trachea. The obstruction could be visualized via transtracheal illumination, but tracheoscopy was not feasible because of the size of the patient. Attempts to manually extirpate the obstruction were unsuccessful, and the trachea subsequently avulsed in the midcervical region. Tracheal resection and anastomosis was performed, and the obstruction was removed with the associated tracheal ring. The patient recovered uneventfully and was doing well at follow-up evaluations at 9 days, 24 days, and 10 months after the surgical procedure. The histologic diagnosis was ossifying myelolipoma arising from bone marrow within the tracheal ring.


Assuntos
Doenças das Aves/diagnóstico , Cacatuas , Mielolipoma/veterinária , Neoplasias da Traqueia/veterinária , Anastomose Cirúrgica/veterinária , Animais , Doenças das Aves/diagnóstico por imagem , Doenças das Aves/cirurgia , Diagnóstico Diferencial , Feminino , Mielolipoma/diagnóstico , Neoplasias da Traqueia/diagnóstico
3.
Mol Genet Metab ; 117(2): 150-6, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26432669

RESUMO

Patients with severe tracheal obstruction in Morquio A syndrome are at risk of dying of sleep apnea and related complications. Tracheal obstruction also leads to life-threatening complications during anesthesia as a result of the difficulty in managing the upper airway due to factors inherent to the Morquio A syndrome, compounded by the difficulty in intubating the trachea. A detailed description of the obstructive pathology of the trachea is not available in the literature probably due to lack of a homogenous group of Morquio A patients to study at any one particular center. We present a series of cases with significant tracheal obstruction who were unrecognized due to the difficulty in interpreting tracheal narrowing airway symptoms. Our goal is to provide the guidelines in the management of these patients that allow earlier recognition and intervention of tracheal obstruction. Sagittal MRI images of the cervical spine of 28 Morquio A patients (12±8.14years) showed that19/28 (67.9%) patients had at least 25% tracheal narrowing and that narrowing worsened with age (all 8 patients over 15years had greater than 50% narrowing). Eight out of 28 patients were categorized as severe (>75%) tracheal narrowing when images were evaluated in neutral head and neck position. Of the 19 patients with tracheal narrowing, compression by the tortuous brachiocephalic artery was the most common cause (n=15). Evidence of such tracheal narrowing was evident as early as at 2years of age. The etiology of tracheal impingement by the brachiocephalic artery in Morquio A appears to be due to a combination of the narrow thoracic inlet crowding structures and the disproportionate growth of trachea and brachiocephalic artery in relationship to the chest cavity leading to tracheal tortuosity. In conclusion, tracheal narrowing, often due to impression from the crossing tortuous brachiocephalic artery, increases with age in Morquio A patients. Greater attention to the trachea is needed when evaluating cervical spine MRIs as well as other imaging and clinical investigations, with the goal of establishing a timely treatment protocol to reduce the mortality rate in this patient population.


Assuntos
Obstrução das Vias Respiratórias/diagnóstico , Mucopolissacaridose IV/patologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Estudos Retrospectivos , Traqueia/patologia , Adulto Jovem
4.
Ear Nose Throat J ; : 1455613231201015, 2023 Sep 25.
Artigo em Inglês | MEDLINE | ID: mdl-37743805

RESUMO

BACKGROUND: Foreign body aspiration into the trachea is a common occurrence in children. While most foreign bodies in the airways can be removed using rigid tracheoscopy, there are instances where tracheostomy or tracheotomy is needed. This article presents a series of cases in which tracheostomy was necessary to remove foreign bodies, and summarizes the relevant experiences and lessons learned. METHODS: We reviewed a total of 7 cases in which tracheostomy and rigid tracheoscopy were combined for foreign body removal out of a larger cohort of 1559 cases at Shenzhen Children's Hospital in Guangdong, China over a 20-year period. RESULTS: The combined use of tracheostomy and rigid tracheoscopy resulted in successful removal of foreign bodies in all 7 patients reviewed in this study. Six patients developed complications of tracheotomy, mainly including mediastinal emphysema (5/7), subcutaneous emphysema (3/7), and pneumothorax (1/7). Tracheotomy was temporary in all patients. CONCLUSION: In cases where a foreign body in the trachea is too large or has irregular, sharp edges, is embedded in submucosal tissues with edema, cannot be passed smoothly through the vocal cords, or local endoscopic facilities are inadequate, a tracheotomy may be necessary. However, this approach should be used with caution, and close collaboration with anesthesiologists and careful management of intraoperative emergencies are crucial for improving the success rate of the procedure. Additionally, the complications should be noted, paying particular attention to early complications such as pneumothorax, pneumomediastinum, and bleeding.

5.
Indian J Otolaryngol Head Neck Surg ; 75(3): 2456-2459, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37636645

RESUMO

Tracheitis sicca is a rare condition where there is drying and crusting of tracheal mucosa which may lead to tracheal obstruction and respiratory distress. Reported here is a case of young boy, who presented with cough, cold and respiratory distress worsening over a period of 5 days. On admission the patient was administered with dexamethasone and was intubated. However, saturation was not maintained despite assisted ventilation, and multiple episodes of self extubation always presented with stridor. Hence, otorhinolaryngology reference was taken and decision was taken to perform laryngotracheobronchoscopy. Endoscopy done showed extensive crusting in the trachea causing complete obstruction of airway above the level of carina. The crusting was meticulously removed, sent for culture, and the obstruction was relieved. The patient maintained saturation after the procedure, and was ultimately extubated and discharged.

6.
JTCVS Tech ; 22: 317-330, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38152216

RESUMO

Objectives: The study objectives were to test an innovative T-tube procedure involving ablative bronchoscopy for the treatment of total airway occlusion and to orchestrate a safe and nontraumatic maneuver to treat intricate subglottic stenosis amenable for substituting the conventional surgical intervention. Methods: This was an uncontrolled single-center cohort study on 1254 patients from January 2001 to June 2021. Patients underwent the modified T-tube procedure treatment for tracheal stenosis. Only 42 patients were included in the study because they had full records for subglottic total occlusion sitting tracheostomy. The ablative bronchoscopy, aided by a fixed suspending laryngoscope, was applied to retunnel their total airway occlusion. T-tube revision and removal were conducted under general anesthesia with laryngeal mask airway aid during follow-up. Results: The primary outcome was 90-day mortality. The secondary outcome was 90-day morbidity. The 42 patients included in the study had a mean age of 52.29 years (range, 9-84 years) with 22 men (52.38%). Their mean length of hospital stay was 13.67 days (range, 2-45 days). Their mean operation time was 73 minutes (range, 43-256 minutes). Their mean length of the tracheal stenosis was 2.8 cm (range, 0.8-6.3 cm). Outcomes were good in 29 patients (69.05%), satisfactory in 10 patients (23.81%), and considered failures in 3 patients (7.14%). A total of 16 patients (38.10%) underwent decannulation, and 3 patients (7.14%) were shifted to a Shiley tracheostomy. All 42 patients had a median follow-up of 6.2 years (range, 1.5-16.3 years). Conclusions: The modified T-tube procedure, which offered both resilience and versatility, improved the conventional technique in treating those patients experiencing total tracheal stenosis and who were unqualified for conventional open surgery.

7.
Cureus ; 15(1): e33322, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36741619

RESUMO

A man in his early twenties with obesity was found dead in his apartment. The deceased was found naked and surrounded by empty bottles of electrolytes. An autopsy performed approximately 6 days postmortem and gross inspection revealed the absence of injury and no apparent extrinsic cause of death. It was decided to dissect to investigate the cause of death. The deceased had become morbidly obese (weight, 98 kg; height, 160 cm; body mass index, 38.3). Shortly before his death, he presented at a clinic complaining of gastric discomfort and heartburn, but other than hypertension (155/91 mmHg) no specific abnormality was found. He was normothermic (36.6℃), and his blood oxygen saturation was normal (97%). Postmortem computed tomography of the thorax revealed a mediastinal mass obstructing the trachea, an upper-airway obstruction, and a narrowed thoracic cavity due to upward compression by an enlarged fatty liver. Autopsy confirmed that the tracheal mass was fatty tissue within the thymus and that upward pressure from an enlarged fatty liver had compressed the thoracic cavity. The deceased likely developed nocturnal chronic hypoxia because of compression by the mediastinal fat mass as well as intermittent hypoxia because of obstructive sleep apnea when lying supine. Chronic and intermittent hypoxia, diabetes, and obesity activate the sympathetic nervous system, increasing the risk of hypertension, heart failure, and arrhythmias. Histological findings showed pulmonary congestion and edema, reflecting heart failure as well as myocardial fragmentation and waving, showing hyper-contraction and hyper-relaxation, respectively. Hypertension, feeling overheated, and myocardial hyper-contraction can be explained as sympathetic nerve over-activation. Intra-cardiac coagulation and a renal cortical pallor suggested subacute death from cardiogenic shock due to heart failure. Postmortem computed tomography before autopsy detected airway obstruction and revealed the cause and pathophysiology of unexpected death in a young man with morbid obesity. Therefore, this could be a potentially useful clinical practice for determining the cause of death postmortem.

8.
Cureus ; 15(1): e33868, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36819365

RESUMO

The number of endotracheal intubations increased in the United States during the COVID-19 pandemic with an associated rise in laryngotracheal injury. Our patient had a complete laryngeal occlusion just proximal to the first tracheal ring. The Neodymium-doped Yttrium Aluminum Garnet (Nd-YAG) laser is often used to resolve sub-laryngeal occlusions, and without access to the Nd-YAG laser, we had to find an alternative solution. Few centers have the access to an Nd-YAG laser, the optimal choice for sub-laryngeal occlusion and our novel approach allowed us to reestablish tracheal continuity and the patient's ability to speak.

9.
World J Clin Cases ; 11(16): 3847-3851, 2023 Jun 06.
Artigo em Inglês | MEDLINE | ID: mdl-37383126

RESUMO

BACKGROUND: Primary adenoid cystic carcinoma in the trachea (TACC) is a rare tumour. Tracheal bronchoscopy is always chosen as a routine approach to obtain a pathological diagnosis, but it can be associated with an increased risk of asphyxia. CASE SUMMARY: We describe a case of TACC in a patient evaluated by chest computed tomography (CT) with three-dimensional reconstruction imaging and diagnosed by transoesophageal endoscopic ultrasonography. The pathological diagnosis confirmed tracheal adenoid cystic carcinoma. CONCLUSION: We highlight the importance of CT and provide a successful exploration of transoesophageal biopsy as a safe alternative approach.

11.
Anaesth Rep ; 8(2): 120-122, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33089217

RESUMO

Tracheal tumour is rare but can lead to upper airway obstruction and acute respiratory distress. Its management includes surgical resection, radiotherapy or interventional bronchoscopy. Ventilation or difficulties with tracheal intubation can occur during the peri-operative course resulting in serious adverse consequences. We report the case of an 83-year-old man with an obstructive tracheal chondrosarcoma resected by rigid bronchoscopy undergoing veno-venous extracorporeal membrane oxygenation. Such support should be considered when the patient's airway patency cannot be ensured by conventional methods.

12.
Respir Med Case Rep ; 30: 101130, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32596130

RESUMO

This report presents a case of endotracheal metastasis in which elective veno-venous extracorporeal membrane oxygenation (VV ECMO) was used to undergo tracheal laser-surgery prior to establishment of a definitive airway. Specifically, we describe the respiratory and airway management in an adult patient from the preclinical phase throughout elective preoperative ECMO implantation to postoperative ECMO weaning and decannulation in the Intensive Care Unit. This case report lends further supports to the idea that the extracorporeal membrane oxygenation could be electively used to provide safe environment for surgery in situations where the standard maneuvers of sustaining adequate gas exchange are anticipated to fail.

13.
Front Vet Sci ; 7: 409, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32793644

RESUMO

A 4-year-old Siberian Husky mix was referred to the emergency service of the University of California Davis Veterinary Medical Teaching Hospital after being found unconscious in a housefire. Upon arrival, the dog was conscious and panting with normal breathing effort. The dog was initially treated with oxygen therapy to minimize the risk of carbon monoxide toxicosis. Progressive agitation with paroxysmal episodes of increased respiratory effort and increased upper airway sounds were noted ~48 h after presentation. Hypoxemia was then documented. Clinical signs continued to progress despite supportive measures, and five days after initial presentation mechanical ventilation was deemed indicated. Following anesthetic induction, endotracheal intubation was performed. Capnography and peak inspiratory pressures recorded on the mechanical ventilator were consistent with airway obstruction. Diffuse intraluminal tracheal obstruction with grossly necrotic tracheal tissue was confirmed using fiber optic tracheoscopy. The patient was humanely euthanized due to grave prognosis. At necropsy, the tracheal lumen was obstructed by sloughed, necrotic tracheal mucosa. This is the first report describing a severe delayed intrathoracic large airway complication secondary to smoke inhalation in a dog.

14.
J Biomed Mater Res B Appl Biomater ; 107(6): 1844-1853, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30521126

RESUMO

Tracheal stenting currently using non-degradable stents is commonplace for treatment of trauma, prolonged intubation related adult airway obstructions, and pediatric patients-associated tracheal stenosis conditions. Degradable tracheal stent placement will avoid complications of stent removal and restenosis. Widespread reports exist on degradable magnesium alloys success for orthopedic and cardiovascular applications but none to date for intra tracheal use. This research explores the use of pure Mg, AZ31, and Mg-3Y alloys for degradable tracheal stent assessment. In vitro evaluation of magnesium, prototype stents in a bioreactor simulate the airway environment and corrosion. Micro-CT imaging and biocompatibility evaluation helped assess the 24-week degradation of intraluminal alloy stents following implantation in a rat tracheal in vivo bypass model. Histological analysis indicate tissue response of the harvested stented trachea segments after each time point. Corrosion studies for each alloy indicate significant differences between the simulated and control in vitro conditions. AZ31 exhibited the lowest volume loss of 6.8% in saline, while pure Mg displayed the lowest volume loss of 4.6% in simulated airway fluid (SAF), both at 1-week time points. Significant differences in percentage of total volume lost after 6 months were determined between the alloys over time. MgY alloy displayed the slowest corrosion losing only 15.1% volume after 24 weeks of immersion. Additionally, in vitro magnesium alloy corrosion was not significantly different from the percentage of total volume lost in vivo at 1-week time point. The study demonstrates promise of magnesium alloys for intraluminal tracheal stent application albeit viability of a clinically translatable model warrants further studies. © 2018 Wiley Periodicals, Inc. J Biomed Mater Res Part B: Appl Biomater 107B: 1844-1853, 2019.


Assuntos
Implantes Absorvíveis , Ligas , Materiais Revestidos Biocompatíveis , Magnésio , Teste de Materiais , Traqueia , Animais , Criança , Feminino , Humanos , Ratos , Ratos Endogâmicos Lew , Traqueia/metabolismo , Traqueia/patologia , Traqueia/cirurgia
15.
Int J Surg Case Rep ; 65: 217-220, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31733618

RESUMO

INTRODUCTION: Semi-solid nutrients have several advantages, including reduced cases of diarrhea and aspiration pneumonia, and are usually administered via percutaneous endoscopic gastrostomy owing to its high viscosity. Administering semi-solid nutrients via a nasogastric tube was recently introduced in clinical practice; however, its safety has not been well confirmed. PRESENTATION OF CASE: An 82-year-old man with a right occipital hemorrhage and severe diarrhea consulted the nutritional support team. Administrations of semi-solid nutrients (HINE E-GEL®) via the nasogastric tube was initiated, which gradually alleviated his symptoms. Fourteen days after initiation, he suddenly had pulmonary failure owing to a tracheal obstruction caused by the reflux and aspiration of semi-solid nutrients. Intubation and subsequent reflex cough expectorated sputum with gel-form particles, which quickly stabilized his pulmonary condition. After this, his hospital course was stable, and he was referred to another hospital for further rehabilitation. DISCUSSION: Semi-solid nutrients administered via the nasogastric tube have different ingredients compared with those administered via percutaneous endoscopic gastrostomy. HINE E-GEL®, for example, contains pectin and calcium phosphate that changes from liquid to semi-solid inside the stomach via chemical reactions under acidic conditions. Data on the viscosity of HINE E-GEL® in vivo are insufficient. Uncertainty regarding the form and viscosity of HINE E-GEL® inside the stomach complicates clinical practice. CONCLUSIONS: Although semi-solid nutrients have several advantages, including reduced diarrhea and gastroesophageal reflux, evidence on semi-solid nutrients via the nasogastric tube is insufficient. It should be noted that semi-solid nutrient reflux can be more fatal than liquid nutrients.

16.
Int J Pediatr Otorhinolaryngol ; 111: 1-6, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29958589

RESUMO

We describe the case of a child with isolated absence of cartilaginous tracheal rings and a trifurcate carina. At 6 months of age, the patient presented to our multidisciplinary airway clinic with stridor and recurrent severe respiratory infections requiring hospitalization. Radiographs showed airway narrowing. Exam demonstrated biphasic stridor. Flexible fiberoptic laryngoscopy demonstrated only mild laryngomalacia. Operative bronchoscopy demonstrated severe tracheomalacia with absence of any visible tracheal rings and a trifurcate carina. Subsequent CT imaging corroborated these findings and did not demonstrate any other major abnormality. The patient did not require operative intervention and his subsequent course was uncomplicated.


Assuntos
Anormalidades Múltiplas/diagnóstico , Broncoscopia , Laringomalácia/diagnóstico , Laringoscopia , Traqueomalácia/diagnóstico , Humanos , Lactente , Laringomalácia/complicações , Masculino , Sons Respiratórios/etiologia , Traqueomalácia/complicações
17.
Mol Genet Metab Rep ; 14: 59-67, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29326877

RESUMO

Morquio A syndrome (mucopolysaccharidosis IVA, MPS IVA) is a lysosomal storage disease caused by a deficiency of N-acetylgalactosamine-6-sulfate sulfatase, resulting in systemic accumulation of the partially degraded glycosaminoglycans (GAGs), keratan sulfate and chondroitin-6-sulfate. The accumulation of these GAGs leads to distinguishing features as skeletal dysplasia with disproportionate dwarfism, short neck, kyphoscoliosis, pectus carinatum, tracheal obstruction, coxa valga, genu valgum, and joint laxity. In the absence of autopsied cases and systemic analysis of multiple tissues, the pathological mechanism of the characteristic skeletal dysplasia associated with the disease largely remains a question. Here we report an autopsied case of a 23-year-old male with MPS IVA, who developed characteristic skeletal abnormalities by 4 months of age and died of severe tracheal obstruction and hypoventilation originating from respiratory muscle weakness from neurological cord deficit due to cord myelopathy at the age of 23. We analyzed postmortem tissues pathohistologically, including the thyroid, lung, lung bronchus, trachea, heart, aorta, liver, spleen, kidney, testes, humerus, knee cartilage, and knee ligament. Examination of the tissues demonstrated systemic storage materials in multiple tissues, as well as severely ballooned and vacuolated chondrocytes in the trachea, humerus, knee cartilage, and lung bronchus. This autopsied case with MPS IVA addresses the importance of tracheal obstruction for morbidity and mortality of the disease, and the pathological findings contribute to a further understanding of the pathogenesis of MPS IVA and the development of novel therapies.

18.
Thorac Surg Clin ; 27(2): 133-137, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28363367

RESUMO

Straight back syndrome is a rare condition usually associated with mitral valve prolapse and "pseudo-heart disease" caused by a very narrow anterior-posterior chest due to the loss of the normal dorsal curvature of the thoracic spine. More rarely, the narrowed upper chest may compress the trachea causing extrinsic tracheal obstruction. If severe enough, this requires operative repair by effectively opening up the upper chest by a variety of techniques to allow the trachea enough room to avoid compression.


Assuntos
Obstrução das Vias Respiratórias/etiologia , Manúbrio/cirurgia , Anormalidades Musculoesqueléticas/complicações , Procedimentos Ortopédicos/métodos , Vértebras Torácicas/anormalidades , Estenose Traqueal/etiologia , Obstrução das Vias Respiratórias/cirurgia , Humanos , Anormalidades Musculoesqueléticas/diagnóstico , Síndrome , Estenose Traqueal/cirurgia
19.
Expert Opin Orphan Drugs ; 4(9): 941-951, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-28217429

RESUMO

INTRODUCTION: Morquio A syndrome is characterized by a unique skeletal dysplasia, leading to short neck and trunk, pectus carinatum, laxity of joints, kyphoscoliosis, and tracheal obstruction. Cervical spinal cord compression/inability, a restrictive and obstructive airway, and/or bone deformity and imbalance of growth, are life-threatening to Morquio A patients, leading to a high morbidity and mortality. It is critical to review the current therapeutic approaches with respect to their efficacy and limitations. AREAS COVERED: Patients with progressive skeletal dysplasia often need to undergo orthopedic surgical interventions in the first two decades of life. Recently, we have treated four patients with a new surgery to correct progressive tracheal obstruction. Enzyme replacement therapy (ERT) has been approved clinically. Cell-based therapies such as hematopoietic stem cell therapy (HSCT) and gene therapy are typically one-time, permanent treatments for enzyme deficiencies. We report here on four Morquio A patients treated with HSCT approved in Japan and followed for at least ten years after treatment. Gene therapy is under investigation on mouse models but not yet available as a therapeutic option. EXPERT OPINION: ERT and HSCT in combination with surgical intervention(s) are a therapeutic option for Morquio A; however, the approach for bone and cartilage lesion remains an unmet challenge.

20.
Laryngoscope ; 124(7): 1663-7, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24254967

RESUMO

OBJECTIVES/HYPOTHESIS: To improve understanding of aspects of end-of-life care that may not be intuitive to the otolaryngology community. DATA SOURCES AND REVIEW METHODS: A comprehensive review of the literature was performed by searching Medline, Embase, and Google Scholar databases. Primary manuscripts' bibliographies were reviewed to identify any nonindexed references. Prospective consultation by means of one-on-one interviews was sought from nonotolaryngology key stakeholders in the areas of hospice nursing care and patient advocacy in order to identify pertinent issues. RESULTS: We identified over 1,000 articles published from 1965 to 2013 on the topic of tracheal stents, as well as over 40,000 on hospice/end-of-life care. Three articles focusing specifically on palliative care and airway stenting were identified, of which three were case reports and none were definitive reviews. There are a number of significant issues and concepts unique to hospice care. These are likely unfamiliar to all except for head and neck oncology-specialized otolaryngologists. An example is that hospice care focuses on quality of life rather than prolongation of life (such as curative surgery). Patients with nonoperable tracheal obstruction from malignancy face an unpleasant demise from suffocation. For those patients, stenting can relieve suffering by restoring airway patency. CONCLUSIONS: Airway stenting can be a valid palliative care option, even for terminal patients receiving hospice care, when performed to relieve airway obstruction and improve quality of life. End-of-life ethics is an underdeveloped area of otolaryngology that should be explored.


Assuntos
Ética Médica , Otolaringologia/ética , Cuidados Paliativos/ética , Stents , Traqueostomia/instrumentação , Humanos , Cuidados Paliativos/métodos , Traqueostomia/ética
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