Interventional pulmonary procedures and their outcomes in patients with STAT3 hyper IgE syndrome.

BACKGROUND: STAT3 hyperimmunoglobulin E syndrome (STAT3-HIES) also referred to as autosomal dominant HIES (AD-HIES) is an inborn error of immunity characterized by the classic triad of eczema, frequent opportunistic infections, and elevated serum IgE levels. As a consequence of lung sequels due to repeated infections and impaired tissue healing, patients may require interventional pulmonary procedures. METHOD: Four patients with dominant-negative STAT3 mutations who had received interventional pulmonary procedures were enrolled. The demographic, clinical, and molecular characteristics were gathered through a medical record search. All reported STAT3-HIES patients in the literature requiring pulmonary procedures as part of their treatment were reviewed. RESULT: Recurrent episodes of pneumonia and lung abscess were the most prevalent symptoms. The most common non-immunological features were scoliosis, failure to thrive, and dental problems such as primary teeth retention and disseminated decays. Bronchiectasis, lung abscess, pneumatocele, and cavitary lesion were the most prevalent finding on high-resolution computed tomography at the earliest recording. All patients underwent pulmonary surgery and two of them experienced complications. CONCLUSION: Patients with STAT3-HIES have marked pulmonary infection susceptibility which may necessitate thoracic surgeries. Since surgical procedures involve a high risk of complication, surgical options are recommended to be utilized only in cases of drug resistance or emergencies.

Evaluation of clinical status, diagnosis, treatment and radiological findings of pulmonary hydatid cyst: 5-years' experience at tertiary lung center.

BACKGROUND: Hydatidosis is one of the most critical worldwide parasitic zoonotic diseases. The lung is the second most common site of hydatidosis. This study aimed to evaluate the clinical status, diagnosis, treatment, and radiological findings of pulmonary hydatid cyst in patients referred to tertiary lung center. METHODS: From April 2014 to July 2019, patients referred to Masih Daneshvari University Medical Center with the impression of alveolar hydatidosis included. Demographic data of 304 patients were collected including clinical symptoms, laboratory studies, radiological findings, location of the lung involvement, and cyst characteristics. Also, surgical procedures, medical treatments, and post-operative complications were recorded. RESULTS: Pulmonary hydatidosis was confirmed for 234 patients. 55% of patients were males with the mean age of 45.1±16.6 years. The most common symptoms were cough (59.8%), dyspnea (31.1%), and hemoptysis (26%). Left lung, right lung, and bilateral involvement were reported in 40.1%, 55.1%, and 4.8% of cases, respectively. Cyst perforation (39.8%) was the most common intra-operative finding. Surgical interventions included thoracotomy, rigid bronchoscopy, cyst aspiration, and enucleation. The liver was the most concomitant organ involved due to pulmonary hydatidosis (16.6%). The most common postoperative complication was atelectasis, with the rate of 35.7%. 52.2% of patients were discharged within 10 days after surgery. No mortality was reported. CONCLUSION: Sometimes atypical findings in different imaging modalities make the hydatid cyst diagnosis challenging. Although lobe involvement more than 50% has the indication for lobectomy, we conserved lobes with about 70% involvement in our institution, and patients had no postoperative complications.

Expression of miRNA1, miRNA133, miRNA191, and miRNA24, as Good Biomarkers, in Non-Small Cell Lung Cancer Using Real-Time PCR Method.

BACKGROUND: Lung cancer has recently shown the highest incidence among all cancers. microRNAs (miRNAs) are the molecules playing a role in regulating gene expression and contributing to many pathogenic mechanisms. Therefore, these molecules could be used as biomarkers for the detection, anticipation, and treatment of cancer. With this in mind, we decided to investigate and compare the expression of miR-1, miR-133, miR-191, and miR-24 and also the expression differences in these four RNA molecules between lung cancer patients and the controls. METHODS: A total of 50 patients with lung cancer participated in this study. In addition, 50 healthy blood samples were selected as the control group. Real-time PCR determined the expression levels of miRNA. The RNAs extracted from the patients' white blood cells were initially synthesized, and then cDNA was extracted. Finally, the synthesized cDNA was amplified using real-time PCR, and its expression was compared with the control group. RESULTS: The result indicated a low expression level of miR-1 and miR-133, and a high expression level of miR-191 and miR-24 in the blood of patients with lung cancer compared to the healthy subjects. CONCLUSION: Our findings revealed that miR-1, miR-133, miR-191, and miR-24 are oncogenes, and their expression could result in cancer. It appears that a therapy to overexpress miR-1 and miR-133 and downexpress miR-191 and miR-24 could contribute to the treatment of lung cancer.

Postintubation Multisegmental Tracheal Stenosis: A 24-Year Experience.

BACKGROUND: Management of multisegmental tracheal stenosis is challenging. In this 24-year longitudinal single-center study, we present an algorithmic treatment approach. METHODS: A retrospective analysis of 2167 patients with postintubation tracheal stenosis indicated 83 (3.83%) patients with multisegmental tracheal stenosis. Patients were assigned to 4 management groups according to the length, location, and severity of stenoses; tracheal infection/mucositis; laryngeal function; symptoms; general condition; and comorbid diseases. Type 1 (n = 13): 1-stage resection of both strictures, Type 2 (n = 6): 2-stage resection of both strictures, Type 3 (n = 40): resection of 1 stricture and nonresectional management of the other, Type 4 (n = 24): nonresectional management of both strictures. Outcomes were categorized as Good, Acceptable, or Poor. Univariate analyses for factors predictive of recurrence and outcome were performed. RESULTS: Follow-ups were completed in 70 (84.34%) patients (median, 22.5 months). Outcome was assessed as Good in 56 (82.35%), Acceptable in 10 (14.71%), Poor in 2 (2.94%), and mortality in 2 (2.94%) patients. The median length of airway resection was 46, 67.5, and 40 mm in Types 1-3, respectively. Only 11 (13.25%) patients had no history of tracheostomy or tracheal surgery. By univariate analysis, a shorter intubation period was associated with Good outcome (P = .017). No factors predictive of recurrence or outcome were ascertained. CONCLUSIONS: Multisegmental tracheal stenosis, generally caused by performing an inappropriate tracheostomy, is an iatrogenic disease that can be prevented. Although resection of both strictures may be feasible and is associated with Good results, in the majority of cases, a combination of surgical resection and non-resectional methods are sufficient to achieve Good results.

Fatal Outcome of Ruptured Pulmonary Hydatid Cyst.

Most authors believe that the optimal treatment for pulmonary hydatid cyst is surgery. Albendazole has been used as a prophylactic measure for reducing recurrence rate but there are some controversies about this strategy. Some researchers have described the increased risk of spontaneous rupture of cysts following albendazole treatment. In this case report, we present a case of spontaneous rupture of pulmonary hydatid cyst with fatal outcome that may be the adverse cause of albendazole.

The Role of Systemic Steroids in Postintubation Tracheal Stenosis: A Randomized Clinical Trial.

BACKGROUND: Most patients with postintubation tracheal stenosis are not ideal candidates for airway resection at presentation and their airways must be temporarily kept open by repeated bronchoscopic dilation (RBD). Meanwhile, some sufficiently recover by RBD without further airway resection requirement. We hypothesized whether systemic corticosteroids could lengthen RBD intervals, decrease the number of patients who eventually need airway resection, and shorten the required length of airway resection. METHODS: Between February 2009 and November 2012, a randomized double-blind clinical trial with a 1:1 ratio (corticosteroids group [group C], prednisolone 15 mg/day; placebo group [group P]) was conducted on 120 patients without tracheostomy or T tube and in no ideal situation for airway resection at presentation, whose precipitating injury had occurred recently. All underwent RBD until they became asymptomatic or prepared for airway resection. Asymptomatic patients received the capsules (prednisolone or placebo) for 6 months; others discontinued them before surgery. Those requiring RBD at short intervals underwent tracheostomy or T tube placement and were then excluded. Follow-up terminated 6 months after airway resection or capsule discontinuation. RESULTS: There were 105 patients (72 male; 50 in group C), aged 15 to 64 years, who completed their follow-up. There was no significant difference between the two groups in age, sex, history of tracheostomy, intubation cause and duration, time interval between intubation and initial bronchoscopy, length of stenosis, and subglottic involvement. Our study showed a trend for RBD with longer intervals (22 days), and fewer operations, 17% (28 of 50 versus 40 of 55) in group C, although statistically insignificant. Furthermore, the required airway resection length became significantly shorter (5.3 mm) in group C. CONCLUSIONS: Early low-dose systemic corticosteroids can be beneficial in postintubation tracheal stenosis management.

A Safe Method of Tracheal Polyflex Stent Placement: A Review of 20 Patients.

BACKGROUND: Surgery is an appropriate therapeutic approach for tracheal stenosis due to various benign and malignant conditions. When surgery is postponed for certain reasons, other options are chosen for airway patency. One alternative is using airway stents. OBJECTIVES: We aimed to introduce a safe method of tracheal polyflex stent placement in patients with tracheal stenoses. PATIENTS AND METHODS: During a 7-year period (2002 - 2008), polyflex stents were used 29 times among 20 patients for various indications. After encountering many difficulties in earlier cases, we gradually developed our new method and used it in most of our patients. In this method, without using large rigid bronchoscopes, the introducer tube could be used as a bronchoscope with the aid of a zero-degree lens and ventilating apparatus. In this method, the rate of possible trauma to the airway can be minimized by avoiding the use of thick rigid bronchoscopies and the stent can be placed faster and more accurately. RESULTS: Polyflex stents were used in 11 men (55%) and 9 women with a mean age of 38.5 years. Stents were removed and changed in 12 cases and replaced with another type of stent in 3 patients. Indications were recurrence of tracheal stenosis (7), multisegmental tracheal stenosis (3), anesthesia limitations (3), tracheal tumors (2), dehiscence of tracheal anastomosis (1), severe inflammation of the tracheal mucosa (1), esophagobronchial fistula (1), and external pressure on the left main bronchus (1). In one patient, a stent was used to open a kinked Dumon stent as a temporary life-saving procedure. We found 6 cases of stent migration, 3 cases of granulation tissue formation, 1 case of infection, and 1 case of surgical site dehiscence. CONCLUSIONS: Stents would be regarded as a temporary means of reaching the ideal condition for resection and reconstruction in most patients with tracheal stenoses. Although an optimal stent has not been introduced yet, we used polyflex stents in most of our patients with tracheal stenosis due to its availability and ease of use. We suggest that this method is safe and less time consuming than its traditional method of placement.

Ten years' experience in surgical treatment of right middle lobe syndrome.

PURPOSE: In this study we present the clinical, radiological, pathological, bronchoscopic and surgical results of 40 patients with diagnosis of middle lobe syndrome who were referred to our thoracic surgery unit for surgical intervention in a 10 years period. METHODS: Forty patients with obstructive and non-obstructive causes of middle lobe syndrome referred to our thoracic surgery unit. Clinical data were collected from the patients' records in a ten years period. This study evaluates diagnostic approaches and surgical treatments in right middle lobe syndrome. RESULTS: We studied 23 females (57.5%) and 17 males (42.5%) with a mean age of 31.7. Clinical findings were cough 95%, sputum 80% and intermittent hemoptysis in 50% of patients. Middle lobe collapse was seen in CT scan of all patients. Bronchiectasis was the most common pathologic finding (55%). Tuberculosis was not rare and was final pathology in 20% of patients. In three patients ruptured hydatid cyst was final finding. Surgery was done without mortality and with only minor complications. CONCLUSION: Lobectomy of right middle lobe is a good therapeutic option in these patients. Due to high prevalence of tuberculosis and hydatid cyst in Middle Eastern countries these two must be considered as causes of middle lobe syndrome.

Gorham's Disease With Chest Wall Involvement: A Case Report and a Review of the Literature.

INTRODUCTION: Gorham's disease is a rare disorder characterized by osteolysis and abnormal vascular growth within bones. Diagnosis of Gorham's disease is often delayed and for accurate and early diagnosis high clinical suspicion is crucial. No specific treatment is available. Management options include surgery, radiation therapy and medical therapy. We aimed to present the first case of Gorham's disease with chest wall involvement in Iran. By review of the literature we discussed important issues of this rare disease including clinical findings, diagnosis and treatment options. CASE PRESENTATION: We present a 48-year-old man with a history of dyspnea following a blunt chest trauma who was admitted to our clinic several times due to reaccumulation of pleural fluid and chylothorax. Gorham's disease was finally established according to clinical manifestations and radiological findings including massive osteolysis in his left ribs and also histological examination. DISCUSSION: According to review of the literature and considering all treatment modalities the patients was successfully treated with a combination of radiotherapy, pamidronate and thalidomide. We suggest that this disease should be considered among differential diagnoses of patients with chest pain, pleural effusion and/or chylothorax with an unknown reason and more importantly history of chest trauma. In suspected cases, it is essential to examine biopsy specimens of the bone adjacent to the inflammated tissues in order to confirm diagnosis.

Surgically treatable pulmonary arteriovenous fistula.

Arteriovenous fistuli are congenital malformations. Usually symptoms depend on size of the lesion. Lesions smaller than 2 cm are often asymptomatic. The most common symptoms are dyspnea, palpitation and fatigue. Cyanosis is indicative of right to left shunt. Helical computed tomography (CT) scan is a helpful diagnostic tool in this case. Surgery is the treatment of choice in patients with isolated lesions. Embolization is a selective method in patients with multiple or bilateral lesions. The patient was a 13-year-old boy complaining of cyanosis of lips and nails as well as dyspnea for 5 years. Definite diagnosis of pulmonary arteriovenous malformation (PAVM) in the right middle lobe was based on CT angiography. The patient underwent a thoracotomy and lobectomy of the right middle lobe. After surgery cyanosis and dyspnea were completely resolved.

A proposed grading system for post-intubation tracheal stenosis.

BACKGROUND: Tracheal stenosis is still a serious consequence of endotracheal intubation. Previous classification systems are commonly descriptive and are not intended to deal with management approach. The aim of this study was to present a classification system for post intubation tracheal stenosis and evaluate its efficacy in distinguishing critically ill patients who need surgical intervention. MATERIALS AND METHODS: This classification system was developed based on size and type of stenosis and associated clinical signs and symptoms. Stenosis was graded based on the results of clinical examination and rigid bronchoscopy. All patients received surgical or conservative treatment based on the judgment of a surgeon experienced in management of post-intubation tracheal stenosis without considering their score. ROC curve analysis was done and cut-off point was established based on the greatest Youden index. RESULTS: Sixty patients were studied. Resection and anastomosis were done for 49 patients. The mean score for all samples was 9.18 (range 8.77-9.45). Chosen cutoff point was 8.5 and calculated sensitivity and specificity were 89% and 42%, respectively. Positive and negative predictive values were 83.7% and 54.5%, respectively. A reasonable agreement between the estimated score and surgeon's clinical judgment (kappa = 0.78) was observed. A statistically significant relationship was observed between scores greater than 8.5 and need for surgical intervention (P= 0.007). CONCLUSION: We presented a scoring system for post-intubation and tracheostomy tracheal stenosis using main factors influencing diagnosis and treatment and its efficacy was evaluated prospectively. It seems that this system would be capable of assimilating the treatment interventions and comparing them.

Thyroid cancers with laryngotracheal invasion.

OBJECTIVES: Management of thyroid cancers with laryngotracheal invasion is controversial. METHODS: A retrospective analysis of our database found 69 patients (38 females, mean age 59.6 ± 11.6) between March 1995 and July 2010; of them 42 (61%) were managed by non-resectional methods due to the extensive airway or regional involvement, severe co-morbidities, diffuse metastases or patient's preference. Segmental airway resection was performed in 27 (39%) patients; concurrent with thyroidectomy in 17 (Immediate group (IG)), and as a delayed procedure in 10 referred patients (Delayed group (DG)), who had previously undergone thyroidectomy with conservative airway management, like shaving procedures. Follow-up was completed in 81% of patients with a mean duration of 30 months. RESULTS: Tracheal or laryngotracheal resection and reconstruction was performed in 18, laryngectomy in eight and pharyngolaryngectomy in one patient. There were two anastomotic dehiscence (11.1%), one resulted in mortality (3.7%). One or a combination of bronchoscopic core-out, laser, tracheostomy and stent placement was performed in 42 non-resected patients with two mortalities (4.7%). Overall 1-, 2-, 3- and 5-year survival was 85, 85, 68 and 49% in resected group, as well as 56, 46, 40 and 31% in non-resected group (P = 0.049), respectively. Among resected group, the overall 1-, 2-, 3- and 5-year survival was 92, 92, 76 and 61% in the IG as well as 75, 75, 56 and 28% in the DG (P = 0.43). CONCLUSIONS: Complete segmental airway resection during or even after thyroidectomy could be safely performed, might be curative and may be associated with improved survival.

Primary major airway tumors; management and results.

OBJECTIVE: Primary major airway tumors are rare. A retrospective analysis of referral centers experience could be helpful for their management. METHODS: Fifty-one patients, including 44 (86%) malignant and seven (14%) benign with primary tumors of subglottis, trachea, carina, and main stem bronchi, were managed in a 14-year period. Based on computed tomography (CT) scan and rigid bronchoscopy findings, those who evaluated as resectable underwent airway resection and reconstruction. The others were managed by one or a combination of these methods: core out, laser, chemotherapy, radiotherapy, and tracheostomy. Follow-up was completed in 88.2%, mean (35.2 ± 33.2 months). RESULTS: Extraluminal extension of the tumor found in CT scan was significantly associated with unresectability (p = 0.006). Thirty-two patients underwent resection with three complications (9%) and one mortality (3%). Nineteen were managed by non-resectional methods; of these, 15 were found unresectable, because of tumor length, extensive local invasion or diffuse distant metastases, and four due to risk-benefit ratio or patient preference. Among 18 patients with adenoid cystic carcinoma 13 (72%) were resected (seven with negative margins). Overall 1-, 2-, 5-, and 8-year survival was 90.9%, 90.9%, 77.9%, and 19.5%, respectively. In unresectable tumors with adenoid cystic carcinoma, overall 1- and 2-year survival was 60% and 40%, respectively. Data analysis found significant association of long-term survival with resection (p = 0.005) but not with negative margins in adenoid cystic carcinoma. Among 15 patients with carcinoid tumors, all were alive at the end of follow-up, except one who died after surgery. CONCLUSIONS: Airway resection, if feasible, may extend survival and may even be curative, with low morbidity and mortality, in most patients with major airway tumors.

Major airways trauma, management and long term results.

PURPOSE: The number of patients with traumatic and iatrogenic tracheobronchial injuries is increasing. Early diagnosis, prompt establishment of a secure airway, and appropriate management could prevent sequelae and lead to a good outcome. METHODS: Between "1994-2007", 35 patients with major airways trauma were managed. This descriptive and retrospective study evaluates clinical findings, diagnostic approaches, initial managements, definitive surgical or nonsurgical treatments and follow-up results. SPSS was used for descriptive outcomes. RESULTS: There were 27 males (77%) and 8 females, with a mean age of 28.2. There were 16 blunt, 11 penetrating and 8 iatrogenic traumas, at the level of the larynx in 1, larynx and hypopharynx in 3, laryngotracheal in 12, tracheal in 13, tracheobronchial in 1, and main bronchi in 5 patients. Fourteen patients (40%) were initially managed, and 21 patients were referred to us after their initial managements at outside hospitals. There were 7 complications (20%); one resulted in mortality (2.9%). The overall final results were good in 57.1%, acceptable in 31.4% and poor in 5.7% of patients, (mean follow-up time, 58.2 months). The respiratory status and the phonation looked better in the initially managed than the delayed managed group. CONCLUSION: We recommend that, patients only become respiratory stable with minimum intervention and then be referred to centers with sufficient experience in airway surgery.

The etiological factors of recurrence after tracheal resection and reconstruction in post-intubation stenosis.

We assessed several factors which might be responsible for the recurrence of post-intubation airway stenosis in a large group of patients who underwent resection and reconstruction surgery by one surgical team. Four hundred and ninety-four patients underwent reconstruction of post-intubation airway stenosis during 1995-2006. The case group comprised patients who had developed recurrence, while controls had no recurrence. The diagnosis of the recurrence was made based on the presence of clinical signs or symptoms and bronchoscopic evaluation. The following variables were compared in both groups: age, sex, duration of intubation, reason for intubation, period of time between intubation and surgery, history of previous tracheotomy, previous therapeutic interventions, subglottic involvement, length of resection, presence of unusual tension at the site of anastomosis and anastomotic infection. Fifty-two patients (10.5%) developed recurrence. Lengthy resection, presence of tension at the site of anastomosis, anastomotic infection and subglottic involvement were significantly higher in the case group. Logistic regression model showed that the three main predictors are anastomotic infection (OR=3.44), subglottic involvement (OR=2.43), and presence of tension (OR=1.97), respectively. It is concluded that the surgeon can play an important role in avoiding recurrence by decreasing tension, preventing infection, and preserving subglottic structure.

Postintubation multisegmental tracheal stenosis: treatment and results.

BACKGROUND: A number of postintubation tracheal stenoses involve different and separate segments. Treatment of these types of strictures is complicated with obscure results, infrequently reported in literature. METHODS: A total of 648 patients underwent treatment for tracheal or subglottic stenosis from September 1993 through October 2005; of those, 26 cases had two separate stenotic segments. Four types of therapeutic approaches were considered for these 26 patients: one-stage resection of the stenotic sites; two-stage resection of the stenotic sites; resection of one stricture and treatment of the second one by nonresectional methods such as dilatation, laser, stenting, T-tube, or tracheostomy; or treatment of both lesions by nonresectional methods. The therapeutic approach for each patient was determined by the surgeon and was based on the nature and location of stenoses, length of stenoses and the distance between the two stenotic sites. RESULTS: There were 20 male patients (76.9%) and 6 female patients (23.1%), with a mean age of 23.9 years (range, 4 to 64). Fourteen patients had tracheal stenosis and 12 had both tracheal and subglottic involvement. Five patients underwent type 1 therapeutic approach whereas 4, 9, and 8 patients underwent types 2, 3, and 4, respectively. Mean length of resection was 58.9 mm in those who underwent complete resection of the stenotic sites (range, 30 to 90 mm). There were 2 complications, 1 stomal fistula and 1 wound infection. Follow-up was accomplished in all patients with a mean period of 21.5 months (range, 1 to 108). Sixteen patients achieved satisfactory results (good voice and airway), 7 are still under treatment (requiring stent, tracheostomy, or repeated dilatation), and 3 died (2 type 3 and 1 type 4). Two deaths were due to T-tube obstruction, and 1 was due to acute obstruction of the stenotic part. CONCLUSIONS: Resection of both strictures and reconstruction of airway are feasible in some patients with multisegmental tracheal stenosis with good results. When resection of both strictures is not feasible, a combination of resectional and nonresectional managements could be helpful for the vast majority of patients.