Tracheal agenesis with anomalies found in both VACTERL and TACRD associations

Tracheal agenesis (TA) was diagnosed by endoscopy after esophageal intubation in a 34-week newborn. Diagnosis, work-up, and management approach are discussed. Similar to previous reports, this case of tracheal agenesis presented with multiple associated defects found at autopsy. Tracheal agenesis had previously been reported as a part of the VATER (vertebral defects, anal atresia, tracheoesophageal fistula and/or esophageal atresia, radial dysplasia, renal defects) and VACTERL (VATER plus cardiovascular and limb defects) associations/syndromes. More recently, cases of TA have been reported with associated anomalies described as TACRD (tracheal agenesis/atresia, complex congenital cardiac abnormalities, radial ray defects, and duodenal atresia) association/syndrome. We present a case of TA with anomalies found in both VACTERL and TACRD associations, which support the speculation that both are different manifestations of a spectrum of mesodermal dysplasia.     

Agenesis of the trachea: phenotypic expression of a rare cause of fatal neonatal respiratory insufficiency in six patients

Tracheal agenesis is a rare congenital malformation, which is usually fatal in the newborn period. Its incidence is approximately 1 in 50,000 births. Presentation is with respiratory insufficiency and no audible cry. Other anomalies are found in most cases. Six cases of tracheal agenesis were seen in our hospital since 1988. Their medical records were reviewed. Three of our cases classify as Floyd's type III, two as Floyd's type II and one as Floyd's type I. Associated anomalies were found in five cases. The classification of tracheal agenesis, associated anomalies and potential therapeutic options are discussed.     

Thyroid paraganglioma.

Paraganglioma of the thyroid gland is a rare neoplasm. Only eight cases have previously been reported. The neoplasm was confined to the thyroid in all but two patients; laryngeal involvement was seen in one and tracheal involvement in another. A review of these previously reported cases is presented along with the details of an additional case with laryngeal involvement. Laryngeal and thyroid paragangliomas are compared and contrasted. The anatomic and embryologic basis of these tumors and the diagnosis and treatment are discussed. We suggest changing the nomenclature and reporting of these lesions on the basis of their apparent common origin. Surgery remains the treatment of choice in most paragangliomas, including those involving the larynx and thyroid. Although the number of reported cases is small, there have been no cases of distant metastases or local recurrence after adequate surgical removal.     

不同咽喉入路方式在声门上型喉癌手术中的应用

目的分析声门上型喉癌保留喉功能手术中进入咽喉腔的合理入路。方法对21例行声门上喉部分切除术的患者分别采用会厌谷入路、声带前连合上方入路及咽侧入路进入咽喉腔,完整切除肿瘤并行相应修复方式,保留喉功能。结果所有患者手术顺利,术中进入咽喉腔及肿瘤切除的过程中符合肿瘤切除的"无瘤"原则,术后病理未出现手术切缘阳性,无咽瘘。全部患者均于术后10～30 d拔除鼻饲管经口进食,20例患者于术后14～60 d拔除气管套管,所有患者均发音成功。结论声门上型喉癌行保留喉功能手术时,根据肿瘤原发部位及局部浸润情况,采取不同入路进入咽喉腔既获得了满意的手术视野及疗效,同时遵循了肿瘤切除的"无瘤"原则。     

Surgical treatment of laryngeal tumors with subglottic extension and tracheal tumors with composite nasal septal cartilage graft: technique and outcome

OBJECTIVE: Composite nasal septal cartilage grafts (CNSCG) are effective grafting materials in laryngeal and tracheal reconstruction following tumor resection. METHODS: Between 1985 and 2005, we used CNSCG for the reconstruction of defects following resection of laryngeal tumors with subglottic extension (20 cases), subglottic mesenchymal tumors (2 cases), invasive thyroid carcinoma (4 cases), tracheal tumors (3 cases) and esophagus carcinoma with tracheal invasion (1 case) in total of 30 patients. RESULTS: The patients with subglottic tumors were decanulated within 5-7 days except one case. We achieved satisfactory voice and swallowing without any sign of recurrence. Overall complications consisted of subglottic stenosis in one case, and unilateral paralysis of recurrent laryngeal nerve in two cases. One patient with subglottic laryngeal carcinoma died due to neck and distant metastases 4 years after the operation. All patients are well with a mean follow-up period 9 years. Three patients with tracheal tumors underwent lateral resection and reconstructed with CNSCG. Satisfactory healing of the grafts was seen in all cases without local recurrence or complication with a mean follow-up period of 62 months. One of the patients had distant metastases 3 years after the operation. The patient with esophagus carcinoma and tracheal invasion was treated by total esophagectomy, gastric pull-up, tracheal resection and CNSCG reconstruction. He died at postoperative 5th day due to mediastinitis as a complication of gastric pull-up. CONCLUSION: Free composite cartilage graft is a reliable material in the reconstruction of defects after surgery of laryngeal tumors with subglottic extension, invasive thyroid and esophagus tumors and well-selected tracheal tumors.     

Squamous metaplasia of the mucosa of the human laryngeal ventricle: a clinicopathological study with reference to the possibility of de novo carcinogenesis]

Primary carcinomas are rarely encountered in the laryngeal ventricle of Morgagni. They are classified into the subgroup of supraglottic laryngeal carcinoma in the TNM classification system of malignant tumors. However, detailed investigations have not hitherto been made on the possible occurrence of carcinomas in this region of the larynx. To find out whether the laryngeal ventricle could be the site of carcinogenesis, the author examined squamous metaplasia in the mucosal epithelium of 60 human laryngeal ventricles which were taken from 38 larynges removed operatively from patients with laryngeal carcinoma (14 cases), hypopharyngeal carcinoma (20 cases), carcinoma of the upper esophagus (two cases), tracheal carcinoma (one case) or thyroid carcinoma (one case). The patients consisted of 31 males and 7 females, with an age distribution of 44 to 80, average 62.7. The laryngeal ventricles were selected for investigation only when they did not show any carcinomatous invasion macroscopically. Macroscopic observations were performed after gross staining with pyronin Y, which permits differentiation of squamous metaplasia from respiratory epithelia. Tissues were embedded in paraffin, sectioned serially and stained with hematoxylin and eosin. The clinical records of the patients were also made. The results are summarized as follows: 1. Squamous metaplasia of the stratified ciliated epithelium was found in 38 out of 60 specimens (63%) of the laryngeal ventricles examined. 2. The metaplasia seemed to be precipitated by smoking or exposure to radiation, along with aging. 3. The incidence and extent of metaplasia of the ventricular mucosa depended on the disease entity which had necessitated total laryngectomy, and the metaplasia was most prominent in cases with the supraglottic type of laryngeal carcinoma. 4. The metaplastic epithelium was occasionally associated with hyperplasia or dysplasia of the epithelium. 5. Microinvasive carcinoma was found in one laryngeal ventricle with severe metaplasia. These findings suggested a possible de novo carcinogenic process in the laryngeal ventricle through squamous metaplasia of the respiratory epithelium. From this observation, the author proposes to call carcinoma of this region of the larynx the ventricular type of laryngeal carcinoma.     

一期整复手术治疗喉气管外伤45例

目的通过分析一期整复手术对喉气管外伤患者喉气管功能恢复的影响,探讨喉气管外伤合理的救治方法。方法对45例喉气管外伤患者均施行一期喉气管探查术,其中闭合性喉气管外伤15例,开放性喉气管外伤30例。结果术后顺利拔管40例,其中仍有声嘶12例;未能拔管4例,术后均发生喉狭窄,其中2例经二次手术喉内放置硅胶管扩张后拔管,2例喉腔肉芽生长反复多次钳取、激光切除,致瘢痕狭窄再行手术,延迟拔管;1例吻颈患者在行喉气管整复术后放弃治疗,2?d后死亡。结论在处理喉气管外伤中应尽可能一期重建喉气管的结构,保留、恢复患者的喉气管功能,早期正确处理是防止喉狭窄的关键。     

Tracheal agenesis

Tracheal agenesis, though seemingly rare and fatal to date, has been reported with increasing frequency. Establishment of the diagnosis necessitates a high index of suspicion in an infant in respiratory distress an birth, without a cry and in whom intubation is difficult. We report a case of tracheal agenesis.     

Reliability of frozen section in determining tumor thickness intraoperatively in laryngeal cancer

OBJECTIVES: The relationship between locoregional lymph metastasis and tumor thickness in head and neck cancer has been well documented in recent years. Determining tumor thickness by frozen section may help the surgeon decide intraoperatively whether to perform elective neck dissection, whereas paraffin section results could be obtained at a later time for this decision. The aim of this study was to evaluate the accuracy of tumor thickness measurements obtained by macroscopic measurement and by frozen section intraoperatively in laryngeal cancer. STUDY DESIGN: Prospectively we compared the tumor thickness results obtained by gross visual examination, by frozen section, and by paraffin section in 20 total, near-total, and horizontal supraglottic laryngectomy specimens. METHODS: The sections were stained with hematoxylin and eosin and tumor thickness was measured under a light microscope with an ocular micrometer. RESULTS: A strong correlation was found between frozen section and paraffin section tumor thickness measurements (Pearson correlation coefficient = 0.993, P <.001). Paired t test showed a 4.59 mm mean difference between macroscopic and paraffin section measurements, and a 0.76 mm mean difference between frozen and paraffin section measurements. CONCLUSION: Assessment of tumor thickness in laryngeal cancer intraoperatively by frozen section is a reliable method.     

喉气管狭窄的手术治疗

目的 探讨喉气管狭窄的手术方法及疗效。 方法 回顾分析山东省千佛山医院耳鼻咽喉头颈外科2009至2015年16例喉气管狭窄手术的临床资料,其中3例喉气管钝性挫伤、1例喉乳头状瘤术后反复狭窄、1例喉癌术后肉芽生成分别实施支撑喉镜下CO₂激光切除术。1例喉癌术后气管内鳞癌、多形性腺瘤及气管腺样囊性癌在鼻内镜下行切除术。1例长时间气管内插管致气管狭窄及1例气管囊腺癌者行气管袖状切除断端吻合术。1例气管腺样囊性癌、2例甲状腺癌及1例气管外伤后狭窄行喉气管重建术。1例食管癌晚期致喉狭窄及1例喉气管外伤老年患者行镍钛记忆合金支架植入术。 结果 随访12~24个月,除2例外,其余患者均手术后拔管。其中1例喉气管重建术放置T形管后肉芽再次生长;另1例镍钛记忆合金支架植入术后出现排异反应,取出支架,此2例均无法拔管,自动出院后失访。 结论 喉气管狭窄的手术治疗应根据狭窄的位置及程度选择不同的手术方法,可获得良好的效果。     

Inner surface imaging of laryngeal and tracheal stenosis by spiral-CT: role of a new diagnostic procedure

BACKGROUND: Inner-surface-reconstruction calculated from helical-CT data sets offers a new diagnostic option for upper airway assessment. Using a special software, it is possible to create a continuous overview on the inner surface of hollow viscera on a monitor, similar to an endoscopic view. However, reports on the clinical value of this examination procedure with regard to laryngeal or tracheal stenosis are rare. METHOD: We compared the results of this "virtual endoscopy" with real endoscopic or intraoperative findings in 11 patients suffering from laryngeal or tracheal stenosis of various etiologies. RESULTS: Data on extension and localization of airway stenosis obtained by virtual endoscopy corresponded well with our endoscopic or intraoperative findings in all patients. The grade of airway stenosis as assessed by inner-surface-reconstruction could be confirmed by endoscopy in 8 of 11 cases investigated. Detection of 2 additional airway stenoses distal to an endoscopically non-passable tracheal stenosis was possible by virtual endoscopy in one case. CONCLUSION: As a supportive diagnostic procedure, virtual endoscopy offers a valuable overview on extension and localization of laryngeal or tracheal stenoses with regard to a possible therapeutic management.     

The microtrapdoor technique for the management of laryngeal stenosis

The use of the carbon dioxide laser as an endoscopic microsurgical instrument has stimulated interest in treating laryngeal and tracheal stenosis. Dedo and Sooy (1984) reported a significant improvement in the management of posterior commissure, subglottic, and high tracheal stenoses with the use of a microtrapdoor flap. A recent laboratory investigation of this method supported the clinical efficacy of this technique. Six patients are evaluated in this report, three with posterior commissure stenosis and three with subglottic or high tracheal lesions. All six patients had a tracheotomy present or performed at the time of their surgical procedures, and each patient had only one procedure. All six had improved airways after the microtrapdoor flaps. Five have been decannulated and one patient with associated hypopharyngeal stenosis still has a tracheostomy because of mild aspiration, although the airway lumen is moderately unproved. It is felt that, as a group, the subglottic and high tracheal lesions respond to this technique better than the posterior commissure stenoses. Details of the technique are presented. This brief clinical report supports the efficacy of the microtrapdoor flap procedure in selected laryngeal and tracheal stenoses.     

Aseptic necrosis of the cricoid: a complication of tracheal intubation

Necrosis of the cricoid is a rare complication of tracheal intubation. Almost all cases previously reported were purulent. A 25-year-old man presented with a respiratory distress, two months after 2 days of tracheal intubation. The posterior larynx was the site of a major inflammation with fixity of the arytenoids. After an emergency control of the airway in the acute phase, a posterior cricoid split with widening by a laryngeal stent was performed for treatment of sequelae. Evolutional data from clinical examinations, direct fiberoptic laryngoscopy, CT scan, respiratory function tests, and pathology are described. Successive phases of latency, then acute manifestations, then sequelae can be distinguished. The work-up did not reveal any sign in favor of a septic necrosis. A physiopathologic hypothesis is suggested, explaining the necrosis as an ischemic one, due to destruction of the internal perichondrium vascular network of the cricoid.     

不同术式治疗喉气管狭窄的疗效观察

目的：总结不同手术方法治疗喉气管狭窄的临床疗效。方法回顾性分析2006年7月～2014年7月手术治疗的56例喉气管狭窄患者（儿童7例,成人49例）的临床资料,其中9例行支撑喉镜下CO2激光瘢痕切除术,5例行支撑喉镜下低温等离子射频瘢痕组织消融术,5例行支撑喉镜下硅胶片或导尿管喉模置入术,6例行喉裂开聚丙烯支架置入术,23例行喉气管成型加T管植入术,8例行气管环形切除端端吻合术,术后随访6个月～2年,观察不同手术方式患者的治疗效果。结果单次手术治愈39例,多次手术治愈10例,复查无再次气道狭窄;无效5例,长期带管生存;失访2例,无死亡及并发症发生的病例。其中,5例喉模置入患者和8例气管端端吻合患者均一次手术治愈;9例行CO2激光瘢痕切除术的患者中1次手术治愈5例,2次手术治愈3例,1例喉癌复发;5例低温等离子射频消融术治疗患者中3例一次手术治愈,1例2次手术治愈,1例无效;6例聚丙烯支架置入的患者中1次手术治愈4例,2次手术治愈1例,1例无效;23例T管置入术的患者中1次手术治愈14例,2次及2次以上手术治愈5例,2例无效,2例失访。结论治疗喉气管狭窄需要依据狭窄程度、狭窄部位及喉气管支架缺损情况等采取个体化治疗方法,才能取得满意效果。     

Mitomycin: effects on laryngeal and tracheal stenosis, benefits, and complications

The management of laryngeal and tracheal stenosis continues to challenge us, despite advances in surgical techniques and better understanding of the wound healing process. Injury to the airway mucosa is the inciting event, regardless of the cause of the stenosis. Mitomycin-C is an antineoplastic antibiotic that acts as an alkylating agent by inhibiting DNA and protein synthesis. It can inhibit cell division, protein synthesis, and fibroblast proliferation. Topical application of mitomycin-C (0.4 mg/mL) was used as an adjuvant treatment in the endoscopic laser management of laryngeal and tracheal stenosis in 15 patients. Fourteen patients (93%) have shown improvement of their airway and resolution of their preoperative symptoms. After a mean follow-up of 18 months, no complication was noted with regard to the application of mitomycin-C. This study gives promising findings on the efficacy and safety of mitomycin-C as an adjuvant treatment in the management of selected cases of laryngeal and tracheal stenosis.     

咽食管严重狭窄和闭锁的手术治疗

目的　探讨咽、食管严重狭窄和闭锁的手术治疗方法及效果。方法　分析了 1983年 1月～ 2 0 0 1年 6月山东医科大学附属医院 (现山东大学齐鲁医院 )耳鼻咽喉科手术治疗的 2 7例咽、食管严重狭窄和闭锁病例 ,其中伴有喉狭窄 12例 ,颈段气管狭窄 1例。根据病因划分包括化学腐蚀伤 2 5例 ,外伤 2例。修复方式 :结肠上徙 2 0例 ,游离空肠移植 6例 ,单纯胸大肌肌皮瓣整复 1例。 12例喉狭窄中利用胸骨舌骨肌筋膜整复 8例 ,胸骨舌骨肌筋膜和会厌下移联合整复 4例。 1例颈段气管狭窄利用胸锁乳突肌肌骨膜瓣修复。结果　 2 7例中 2 5例恢复吞咽功能 ,2例失败。 12例喉狭窄 10例恢复喉的呼吸、发音及吞咽保护全功能 ,2例仅恢复喉的发音及吞咽保护功能 ;1例气管狭窄经手术整复 ,最终拔除气管套管。结论　结肠上徙适于治疗范围大、位置低的严重狭窄或食管闭锁 ;游离空肠移植需要在颈部有正常的食管腔及吻合血管 ;胸大肌肌皮瓣适于修复范围较局限的咽、颈段食管狭窄     

Growth Patterns of Piriform Sinus Carcinomas

Advances in functional surgery have encouraged many head and neck surgeons to treat piriform sinus carcinomas, whenever possible, by conservation surgery. An increased understanding of the histopathologic growth pattern and spread of piriform sinus carcinomas is needed to define criteria for appropriate application of voice preservation surgery. The purpose of this study was to analyze the patterns of tumor spread to laryngeal and parapharyngeal structures. A total of 42 specimens obtained by laryngopharyngectomy were subjected to a whole-organ section study. Carcinomas confined to the lateral wall tended to extend laterally beyond the thyroid ala and rarely infiltrated the intrinsic laryngeal muscles. As a result, conservation surgery could be possible in many cases, including T4 carcinomas. Carcinomas confined to the medial wall or occupying the whole piriform sinus tended to infiltrate laryngeal structures early, and hemilaryngeal fixation was observed in 61% of these cases; tumor spread to the contralateral side in 64%. The hemilarynx fixation was due to an invasion of intrinsic laryngeal muscles. An isolated perineural or cricoarytenoid joint involvement was never observed. For these tumors, conservation surgery would be inadequate in most cases, as they often present extensive laryngeal involvement with spread to the contralateral side.     

环上喉次全切除喉功能重建41例的疗效分析

目的 :探讨环上喉次全切除喉重建术的治疗效果和喉功能重建效果。方法 :回顾性分析 4 1例接受该术式的T2 ～T4期喉鳞癌患者的临床资料。其中声门型 35例 ,声门上型 6例 ;行环舌会厌固定术 2 3例 ,环舌固定术 18例。结果 :3年生存率 83.3% (30 / 36 ) ,5年生存率 71.4 % (2 0 / 2 8) ;拔管率为 92 .7% (38/ 4 1)。 1例因误吸严重而行咽气管分离术 ,余 4 0例均恢复了正常吞咽。全部患者均能利用新喉发音。结论 :环上喉次全切除喉功能重建术既能彻底切除肿瘤 ,又能恢复喉的三大基本功能 ,是中、晚期喉癌治疗的有效方法之一。