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Orphanet Journal of Rare Diseases Dec 2011A laryngo-tracheo-esophageal cleft (LC) is a congenital malformation characterized by an abnormal, posterior, sagittal communication between the larynx and the pharynx,... (Review)
Review
A laryngo-tracheo-esophageal cleft (LC) is a congenital malformation characterized by an abnormal, posterior, sagittal communication between the larynx and the pharynx, possibly extending downward between the trachea and the esophagus. The estimated annual incidence of LC is 1/10,000 to 1/20,000 live births, accounting for 0.2% to 1.5% of congenital malformations of the larynx. These incidence rates may however be underestimated due to difficulty in diagnosing minor forms and a high mortality rate in severe forms. A slightly higher incidence has been reported in boys than in girls. No specific geographic distribution has been found. Depending on the severity of the malformation, patients may present with stridor, hoarse cry, swallowing difficulties, aspirations, cough, dyspnea and cyanosis through to early respiratory distress. Five types of laryngo-tracheo-esophageal cleft have been described based on the downward extension of the cleft, which typically correlates with the severity of symptoms: Type 0 laryngo-tracheo-esophageal cleft to Type 4 laryngo-tracheo-esophageal cleft. LC is often associated with other congenital abnormalities/anomalies (16% to 68%), mainly involving the gastro-intestinal tract, which include laryngomalacia, tracheo-bronchial dyskinesia, tracheo-bronchomalacia (mostly in types 3 and 4), and gastro-esophageal reflux disease (GERD). The syndromes most frequently associated with an LC are Opitz/BBB syndrome, Pallister Hall syndrome, VACTERL/VATER association, and CHARGE syndrome. Laryngeal clefts result from failure of fusion of the posterior cricoid lamina and abnormal development of the tracheo-esophageal septum. The causes of the embryological developmental anomalies leading to LC are not known but are thought to be multifactorial. LC appears to be mostly sporadic although some familial cases with suspected autosomal dominant transmission have been reported. The age of diagnosis depends mainly on the severity of the clinical symptoms and therefore on the extent of the LC. Diagnosis is made either based on clinical manifestations or on investigations, such as endoscopy, X-ray, CT scan, performed for other conditions. Differential diagnoses include tracheo-bronchial fistula, gastro-esophageal reflux disease and neurological swallowing disorders, as well as laryngomalacia and laryngeal palsy. Prenatal diagnosis of LC has never been reported, although associated anomalies may be detected on fetal ultrasonography. Once the cleft is diagnosed, it is essential to determine its length to orient the management and treatment approach. Management involves maintenance of satisfactory ventilation, prevention of secondary pulmonary complications as a result of repeated aspirations, and adequate feeding. Endotracheal intubation may be required for respiratory distress in severe cases. Treatment requires endoscopic or external surgery to close the cleft. Surgery should be performed as early as possible to avoid complications related to aspiration and gastric reflux, except in type 0 and type 1 cases in which conservative measures must first be attempted. The prognosis is variable depending on the severity of the LC and associated malformations. Early diagnosis and appropriate treatment and management help to reduce mortality and morbidity.
Topics: Abnormalities, Multiple; Animals; Child; Child, Preschool; Congenital Abnormalities; Endoscopy; Esophagus; Female; Humans; Infant; Infant, Newborn; Larynx; Male; Prognosis; Rats; Trachea
PubMed: 22151899
DOI: 10.1186/1750-1172-6-81 -
Journal of Education & Teaching in... Jul 2021This simulation provides training for emergency medicine residents in the stepwise management of a patient who presents with bleeding from a tracheoinnominate artery...
AUDIENCE
This simulation provides training for emergency medicine residents in the stepwise management of a patient who presents with bleeding from a tracheoinnominate artery fistula. Additional learners who might benefit from this simulation are otolaryngology and general surgery residents as well as critical care fellows.
INTRODUCTION
Hemorrhage from a tracheoinnominate artery fistula (TIAF) is a rare but life-threatening complication in a patient with a recent tracheostomy. This complication occurs in 0.7% of tracheostomy patients with a mortality of 50-70%.1 Seventy-five percent of patients with a TIAF will present within the first three weeks of surgery and 50% of patients will present with a sentinel bleed that briefly resolves.1 Key elements of a history and exam that should raise a provider's concern for this diagnosis include a recent tracheostomy (within the last 4 weeks), a percutaneous tracheostomy, prior radiation, chronic steroid use, a neck or chest deformity or a sentinel bleed.2 Survival from a TIAF hinges upon emergent, operative repair by an otolaryngologist and cardiothoracic surgeon. Cuff hyperinflation and the Utley Maneuver are critical bedside interventions to temporize this massive bleed and stabilize the patient for definitive, operative repair.
EDUCATIONAL OBJECTIVES
By the end of this simulation, learners will be able to: 1) perform a focused history and physical exam on any patient who presents with bleeding from the tracheostomy site, 2) describe the differential diagnosis of bleeding from a tracheostomy site, including a TIAF, 3) demonstrate the stepwise management of bleeding from a suspected TIAF, including cuff hyperinflation and the Utley Maneuver, 4) verify that definitive airway control via endotracheal intubation is only feasible in the tracheostomy patient when it is clear, upon history and exam, that the patient can be intubated from above, 5) demonstrate additional critical actions in the management of a patient with a TIAF, including early consultation with otolaryngology and cardiothoracic surgery as well as emergent blood transfusion and activation of a massive transfusion protocol.
EDUCATIONAL METHODS
This case was written with a modified, low-fidelity manikin, traditionally used for training in nasogastric tube placement and tracheostomy care. We modified this manikin to simulate a hemorrhage from the tracheostomy site.3 The patient in our case had a history of laryngeal cancer, and thus we occluded his larynx for this simulation. As a result of this obstruction, he was unable to be intubated from above. We provided confederates, a bedside nurse and family member, to assist the learners throughout the case. We also utilized a simulation technician to operate dynamic vital signs on a simulated cardiac monitor. It would be technically challenging to adapt this case to a high-fidelity simulator due to potential for damage of the internal electrical elements by the large amount of artificial blood from the tracheostomy tube. However, a mechanical pump provided a useful means of active bleeding in this low-fidelity manikin.
RESEARCH METHODS
We provided a pre- and post-simulation questionnaire for the 33 emergency medicine residents who participated in this simulation. There were 11 residents from each of the PGY-1, PGY-2 and PGY-3 year-groups. Thirty-two residents (97%) completed the pre-survey and 33 residents (100%) completed the post-survey. For our questions, we used a 5-point Likert Scale to assess a resident's knowledge of the learning objectives within this simulation.
RESULTS
Responses from our pre- and post- survey indicated a significant improvement in knowledge about a tracheoinnominate artery fistula as well as the general management of tracheostomy complications in the emergency department.
DISCUSSION
This simulation is a useful educational tool for instructing emergency medicine residents on optimal management of tracheostomy emergencies such as a TIAF. The interprofessional teaching by an emergency medicine attending and mid-level (PGY-3) otolaryngology resident allowed for a richer and more detailed discussion during the debriefing. Throughout the case, the emergency medicine attending played the role of a bedside nurse and offered supportive, clinical cues when bleeding recurred. The otolaryngology resident played the role of a family member and offered helpful cues during the history and exam portion of the case. Following the case, both content experts provided useful clinical insight during the debriefing. If staffing availability permits, it might be advantageous to use additional simulation-trained personnel to play the roles of the nurse and family member, thus allowing the emergency medicine attending and otolaryngology content experts to simply view the case from the control room and perform the debriefing.
TOPICS
Tracheostomy, surgical airway, tracheoinnominate artery fistula, bleeding from tracheostomy site, complications with tracheostomies, hemorrhagic shock.
PubMed: 37465068
DOI: 10.21980/J8K05R -
Developmental Biology Sep 2021Trachea-esophageal defects (TEDs), including esophageal atresia (EA), tracheoesophageal fistula (TEF), and laryngeal-tracheoesophageal clefts (LTEC), are a spectrum of... (Review)
Review
Trachea-esophageal defects (TEDs), including esophageal atresia (EA), tracheoesophageal fistula (TEF), and laryngeal-tracheoesophageal clefts (LTEC), are a spectrum of life-threatening congenital anomalies in which the trachea and esophagus do not form properly. Up until recently, the developmental basis of these conditions and how the trachea and esophagus arise from a common fetal foregut was poorly understood. However, with significant advances in human genetics, organoids, and animal models, and integrating single cell genomics with high resolution imaging, we are revealing the molecular and cellular mechanisms that orchestrate tracheoesophageal morphogenesis and how disruption in these processes leads to birth defects. Here we review the current understanding of the genetic and developmental basis of TEDs. We suggest future opportunities for integrating developmental mechanisms elucidated from animals and organoids with human genetics and clinical data to gain insight into the genotype-phenotype basis of these heterogeneous birth defects. Finally, we envision how this will enhance diagnosis, improve treatment, and perhaps one day, lead to new tissue replacement therapy.
Topics: Animals; Digestive System Abnormalities; Disease Models, Animal; Esophagus; Humans; Organoids; Trachea
PubMed: 34023332
DOI: 10.1016/j.ydbio.2021.05.015 -
Indian Journal of Otolaryngology and... Oct 2022Laryngeal cancer treatment is often wrought with challenges, pharyngocutaneous fistula formation (PCF) in patients undergoing either primary or salvage laryngectomy for...
Laryngeal cancer treatment is often wrought with challenges, pharyngocutaneous fistula formation (PCF) in patients undergoing either primary or salvage laryngectomy for laryngeal and hypopharyngeal for squamous cell carcinoma is an important one. We aimed to study the factors before and at the surgery that are associated with PCF formation in a South Indian tertiary care hospital. A retrospective chart review of 127 patients who underwent total laryngectomy (TL) between May 2014 and April 2019 at our centre were done. Data was collected, including patient age and gender, comorbidities (Diabetes mellitus, COPD and hypothyroidism), smoking, tumor stage and site, prior tracheostomy, prior radiation, concurrent neck dissection and type of pharyngoplasty, Preoperative hemoglobin and albumin levels, surgical margin status and development of a PCF was also done. Further details specific to the development of a PCF were recorded for that subset of patients including the length of time to fistula, mode of closure, time of closure and modality of management. The overall incidence of PCF was 16.5% (21 of 127 patients), and the median time from TL to the diagnosis of PCF was 6 days (range, 3-20 days). The analysis was done separately for laryngectomies without any pharyngeal reconstruction (112/127 patients). In patients treated with a primary TL, the incidence of PCF was 12.20% (10 of 82) and 26.66% (8 of 30) after salvage TL. Subset analysis for type of pharyngoplasty repair showed 12% (15/127) underwent different types of vascularised/muscular flap for smaller residual pharyngeal mucosa, of which 20% (3/15) developed PCF and one patient developed haematoma needing exploration and re-suturing. The predictive factors for PCF were hypopharynx cancer (< 0.05), surgical margin positivity ( < 0.0001), female gender ( < 0.05), absence of prior tracheostomy (< 0.05) and tumor extension into pyriform sinus mucosa ( < 0.05). Preoperative patient factors of gender and site of primary along with histological margin positivity and extension of tumor to the pyriform sinus mucosa were significant risk factors for PCF formation. Pre-Op radiotherapy remains a strong clinical suspicion but not statistically significant.
PubMed: 36452720
DOI: 10.1007/s12070-020-02046-x -
Acta Otorhinolaryngologica Italica :... Jun 2015Our objective was to evaluate recurrence patterns of hypopharyngeal and laryngeal carcinoma after chemoradiation and options for salvage surgery, with special emphasis... (Review)
Review
Our objective was to evaluate recurrence patterns of hypopharyngeal and laryngeal carcinoma after chemoradiation and options for salvage surgery, with special emphasis on elderly patients. In a retrospective study all patients who underwent chemoradiation for hypopharyngeal and laryngeal carcinoma in a tertiary care academic center from 1990 through 2010 were evaluated. Primary outcome measures were the survival and complication rates of patients undergoing salvage surgery, especially in elderly patients. Secondary outcome measures were the predictors for salvage surgery for patients with locoregional recurrence after failed chemoradiotherapy. A review of the literature was performed. Of the 136 included patients, 60 patients had recurrent locoregional disease, of whom 22 underwent salvage surgery. Fifteen patients underwent a total laryngectomy with neck dissection(s) and 7 neck dissection without primary tumour surgery. Independent predictors for salvage surgery within the group of 60 patients with recurrent disease, were age under the median of 59 years (p = 0.036) and larynx vs. hypopharynx (p = 0.002) in multivariate analyses. The complication rate was 68% (14% major and 54% minor), with fistulas in 23% of the patients. Significantly more wound related complications occurred in patients with current excessive alcohol use (p = 0.04). Five-year disease free control rate of 35%, overall survival rate of 27% and disease specific survival rate of 35% were found. For the 38 patients who were not suitable for salvage surgery, median survival was 12 months. Patients in whom the tumour was controlled had a 5-year overall survival of 70%. In patients selected for salvage surgery age was not predictive for complications and survival. In conclusion, at two years follow-up after chemoradiation 40% of the patients were diagnosed with recurrent locoregional disease. One third underwent salvage surgery with 35% 5-year disease specific survival and 14% major complications. Older patients selected for salvage surgery had a similar complication rate and survival as younger patients.
Topics: Adult; Aged; Carcinoma, Squamous Cell; Chemoradiotherapy; Combined Modality Therapy; Female; Humans; Hypopharyngeal Neoplasms; Laryngeal Neoplasms; Male; Middle Aged; Retrospective Studies; Survival Rate; Treatment Outcome
PubMed: 26246660
DOI: No ID Found -
Brazilian Journal of Otorhinolaryngology 2020Pharyngocutaneous fistula is the most significant complication after salvage total laryngectomy in patients who have received previous treatment with radiotherapy with... (Review)
Review
INTRODUCTION
Pharyngocutaneous fistula is the most significant complication after salvage total laryngectomy in patients who have received previous treatment with radiotherapy with or without chemotherapy.
OBJECTIVE
Our purpose is to review the fistula rate in radiated patients undergoing salvage total laryngectomy, to determine if the use of pectoralis major flap interposition reduces the incidence and duration of fistula and to examine other risk factors.
METHODS
We made a retrospective review of patients undergoing salvage total laryngectomy for exclusively larynx cancer after failure of primary curative radiotherapy between 2000 and 2017. General data from patients, risk factors and other complications were analyzed.
RESULTS
We identified 27 patients whose mean age was 66.4 years, mainly male (92.5%). The primary closure group without pectoralis major flap included 14 patients, and the group with pectoralis major flap closure included 13 patients. Pharyngocutaneous fistula was present in 15 patients (55.5%). Global pharyngocutaneous fistula rate was higher in the group of patients without pectoralis major flap comparing with those were the flap was interposed (78.6% versus 30.8%, p=0.047). Also the pharyngocutaneous fistulas which need to be repaired with surgery (64.3% versus 7.7%, p=0.03) and large pharyngostomes (64.3% versus 0%, p=0.0004) were present in a higher rate in the group closed primary without pectoralis major flap. We did not find other risk factors with statistical significance. Oral diet initiation (84 days versus 21.5 days, p=0.039) and the duration of hospitalization (98.3 days versus 27.2 days, p=0.0041) were much lower in patients with a preventive pectoralis major flap. Two patients died as a consequence of complications of large pharyngostomes.
CONCLUSIONS
Prophylactic pectoralis major flap reduced the incidence, severity and duration of fistula and should be considered during salvage total laryngectomy.
Topics: Adult; Aged; Aged, 80 and over; Cutaneous Fistula; Female; Humans; Laryngeal Neoplasms; Laryngectomy; Male; Middle Aged; Neoplasm Staging; Postoperative Complications; Retrospective Studies; Risk Factors; Severity of Illness Index; Surgical Flaps
PubMed: 30683565
DOI: 10.1016/j.bjorl.2018.11.007 -
The Laryngoscope May 2021A paucity of data exists regarding surgical outcomes for patients undergoing total laryngectomy for a dysfunctional larynx. Herein, we present the largest study...
OBJECTIVE
A paucity of data exists regarding surgical outcomes for patients undergoing total laryngectomy for a dysfunctional larynx. Herein, we present the largest study evaluating the method of closure on postoperative fistula rate and swallowing ability.
METHOD
We performed a retrospective review of patients undergoing total laryngectomy for a dysfunctional larynx after primary radiation or chemoradiation therapy for laryngeal carcinoma from 1998 to 2020. Demographic information, operative details, length of hospitalization, fistula formation, method of fistula treatment, and need for enteral feeding 6 months after surgery were analyzed.
RESULTS
A total of 268 patients were included. Flaps were performed in 140 (52.2%) patients, including radial forearm free flaps (RFFF), pectoralis flaps, and supraclavicular flaps. Sixty-four (23.9%) patients developed postoperative fistulas. There was no significant difference in the fistula rate between flap and primary closure methods (P = .06). However, among patients who had a flap, RFFF had a significantly lower fistula rate (P = .02). Significantly more patients who had initial closure with a pectoralis flap required an additional flap for fistula repair than those who underwent RFFF or primary closure (P < .05). Last, whereas 87 patients (32.5%) required an enteral feeding tube 6 months after surgery, significantly fewer patients who underwent RFFF were feeding tube-dependent (P = < .0001).
CONCLUSION
Herein, we present the largest study of outcomes after total laryngectomy for dysfunctional larynx. Postoperative fistula rates are high, 23%; however, the majority of patients, 67%, will not require long-term enteral support. The RFFF is an excellent option demonstrating the lowest rates of postoperative fistula and enteral feeding tube dependence.
LEVEL OF EVIDENCE
4 Laryngoscope, 131:E1510-E1513, 2021.
Topics: Aged; Aged, 80 and over; Carcinoma; Chemoradiotherapy; Cutaneous Fistula; Enteral Nutrition; Female; Humans; Laryngeal Neoplasms; Laryngectomy; Larynx; Male; Middle Aged; Neoplasm Staging; Postoperative Complications; Radiation Injuries; Plastic Surgery Procedures; Retrospective Studies; Surgical Flaps; Treatment Outcome
PubMed: 33037821
DOI: 10.1002/lary.29154 -
Cureus Sep 2021Since the first total laryngectomy was performed in the late 18th century, several improvements and variations in surgical techniques have been proposed for this... (Review)
Review
Since the first total laryngectomy was performed in the late 18th century, several improvements and variations in surgical techniques have been proposed for this procedure. The surgical techniques employed in total laryngectomy have not been comprehensively discussed to date. Thus, the main objective of this article was to address controversial aspects related to this procedure and compare different surgical techniques used for a total laryngectomy procedure from the beginning to the end. Although the management paradigms in laryngeal and hypopharyngeal squamous cell carcinomas have shifted to organ-preserving chemoradiotherapy protocols, total laryngectomy still plays a prominent role in the treatment of advanced and recurrent tumors. The increased incidence of complications associated with salvage total laryngectomy has driven efforts to improve the surgical techniques in various aspects of the operation. Loss of voice and impaired swallowing are the most difficult challenges to be overcome in laryngectomies, and the introduction of tracheoesophageal voice prostheses has made an enormous difference in postoperative rehabilitation and quality of life. Advancements in reconstruction techniques, tumor control, and metastatic management, such as prophylactic neck treatments and paratracheal nodal dissection (PTND), as well as the use of thyroid gland-preserving total laryngectomy in selected patients have all led to the increasing success of modern total laryngectomy. Several conclusions regarding the benchmarking of surgical techniques cannot be drawn. Issues regarding total laryngectomy are still open for discussion, and the technique will continue to require improvement in the near future.
PubMed: 34707956
DOI: 10.7759/cureus.18181