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.

Post-intubation tracheal stenosis in pediatric age group: single-center experiences of 24 years.

BACKGROUND AND AIM: Post-intubation tracheal stenosis (PITS) is an iatrogenic injury that involves some patients. Given the importance of this issue and the referral of a significant number of children with tracheal stenosis to Masih Daneshvari Hospital in Tehran, Iran, the present study investigated tracheal stenosis following prolonged intubation in the pediatric age group. METHODS: In this observational retrospective study, from 1994 to 2018, the medical records of all children under 14 years of age with a history of PITS were reviewed. Demographic and clinical characteristics including signs and symptoms, the underlying condition that leads to intubation, duration of intubation, type of stenosis, and the therapeutic approach, type of surgery, and follow-up were collected and analyzed using SPSS. RESULTS: Among 161 patients with a mean age of 9.8 ± 4.2 years, 69% were male. The site of stenosis was limited to the trachea in 47% and others both trachea and subglottic area were involved. The most common cause of intubation was trauma. The most common symptoms were dyspnea and wheezing. Success rates of reconstruction were 93.75% in type I, 82.15% in type II, and 35.70% in type III. Among the 16 patients who underwent Type III surgery, decannulation was not performed in 11 patients. Traction in the anastomosis and complications were stated in 26 and 10% of the patients respectively, a mortality rate of 8.7% was also reported. CONCLUSION: In the case of endotracheal intubation, PITS should be considered in the differential diagnosis of dyspnea in children as well as adults.

Uncommon Complications of Lung Transplantation in a Referral Center.

Background: Many efforts were made to determine the uncommon clinical complications after lung transplantation and treatment options to tackle them; however, many of these rare complications have not been mentioned in recent publications. Evaluating and recording adverse effects after organ transplantation can significantly prevent post-transplant mortality. This study aimed to examine rejection factors by examining individuals undergoing lung transplantation surgery. Materials and Methods: In a prospective longitudinal study, we followed up on complications of 60 lung recipients post lung-transplantation surgery for six years from 2010 to 2018. All complications were recorded in follow-up visits or hospital admissions during these years. Finally, the patients' information was categorized and evaluated by designing a questionnaire. Results: From a total of 60 transplant recipients, from 2010 to 2018, 58 patients were initially enrolled in our study, but two were lost to follow-up. Uncommon complications witnessed in the post-transplantation period included endogenous endophthalmitis, herpetic keratitis, duodenal strongyloidiasis, intestinal cryptosporidiosis, myocardial infarction, diaphragm dysfunction, Chylothorax, thyroid nodule, and necrotizing pancreatitis. Conclusion: Meticulous postoperative surveillance is crucial for managing lung transplant patients for early detection and treatment of common and uncommon complications. Therefore, it is necessary to establish procedures for assessing the patients' constancy until complete recovery.

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.

Necrotizing Sarcoid Granulomatosis with Pneumothorax.

Necrotizing sarcoid granulomatosis (NSG) is a rare granulomatous pneumonitis which is composed of a background of sarcoidosis-like granulomas, granulomatous vasculitis, and variable amount of necrosis. We reported a case of a 38-year-old nonsmoking woman presented with left-sided chest pain and dyspnea for three days. Chest CT scan exhibited collapse consolidation of the left lower lobe with the presence of two separated small-sized cystic lesions within the collapsed segment. Lung lesion resection was performed, and histological examination confirmed the diagnosis by excluding other causes of granulomatous diseases. The prognosis of NSG is favorable, and medical treatment is usually not necessary, as well as in our case. NSG is a rare disease with nonspecific symptoms and good prognosis which is frequently confused with Wegener's granulomatosis, sarcoidosis, and Churg-Strauss syndrome. This entity should also be considered as differential diagnosis of necrotizing granulomatous diseases.

Technical Details of Surgical Treatment of a Severely Displaced Sternal Fracture.

Sternal fracture is an uncommon injury, which is managed conservatively in most patients. In case of failure of non-surgical management or severely displaced fractures, open reduction and internal fixation should be considered. In this case report, we present the technical details of open reduction and internal fixation for a severely displaced sternal fracture in a bicyclist. The sternal fracture was successfully treated, and the patient benefited from the rapid control of symptoms, early mobilization, and good cosmetic outcome. CONCLUSION: Open surgical treatment of a sternal fracture, when indicated, can be performed safely, with rapid control of symptoms, low risk of non-union, and good cosmetic outcome.

Toothpick Aspiration Induces Massive Hemoptysis: a Case Report.

BACKGROUND: Massive hemoptysis refers to bleeding from the sputum exceeding 100 ml/day. This condition is known to have a poor prognosis. Although foreign body aspiration is not as common as other risk factors, it may result in massive hemoptysis. In the current study, we presented a case of massive hemoptysis due to the aspiration of a toothpick. CASE PRESENTATION: The patient was a 49-year-old woman who was primarily suspected of having tuberculosis. After observing blood in the sputum, interventions, including chest computed tomography (CT) scan and conservative management, were performed. The CT scan showed no malignancy, and paraclinical investigations were negative. However, hemoptysis was progressing into an acute phase; therefore, a surgical intervention was performed for the patient. After the surgery, the cause of the lesion was found to be a toothpick. The patient was under intensive care after surgery and was discharged from the hospital in a good general condition. The morphological evaluation of the lesion showed a bronchial wall with ulceration, besides granulation tissue formation, hematoma, and fibrinoid necrosis due to foreign body aspiration into the lung, resulting in inflammatory reactions. CONCLUSION: In this case report, foreign body aspiration resulted in massive hemoptysis. Our primary attempts to diagnose the cause of lesion were unsuccessful, and surgery was performed due to the life-threatening condition of the patient. Overall, unexplained hemoptysis may occur following a serious accident due to foreign body aspiration.

Colopharyngoplasty in Patients with Severe Pharyngoesophageal Corrosive Injury: A Complicated but Worthwhile Procedure to Restore GI Tract Continuity, A Case Series.

BACKGROUND: Pharyngoesophageal strictures (PES) after corrosive injury impose a problematic condition for both physicians and patients in terms of their management and patients' quality of life. Colopharyngoplasty is a complex procedure, which is used to restore swallowing in these severely disabled patients. We describe our experience in treating nine patients with severe PES after corrosive injuries in a referral center. MATERIALS AND METHODS: A retrospective analysis of our database from 2009 to 2014 showed nine patients (seven men; age range: 18 to 47 years) with severe PES who underwent colopharyngoplasty ∼6 months (range: 4-10) after caustic material ingestion. All patients had a feeding jejunostomy tube before reconstruction. Esophagectomy with or without gastrectomy was performed in all patients, except for one; thereafter, an isoperistaltic segment of the left colon was pulled up, and a pharyngocolic anastomosis was performed. Eight patients had a tracheostomy created either before reconstruction due to respiratory symptoms or at the time of definitive surgery to prevent aspiration in the early post-operative period. RESULTS: Almost all survivors had a satisfactory swallowing at the end of the follow-up (range: 4-60 months). The jejunostomy tube could be removed in all of the patients after a median of 5 months. One patient died of sepsis due to graft necrosis in the immediate post-operative period. Another patient died 5 months after the first surgery following a revision surgery for intractable dysphagia. At the end of the follow-up, only one patient tolerated tracheostomy tube decannulation. Two patients required laryngotracheal dissociation because of massive aspiration and recurrent episodes of pneumonia. Five patients still had a tracheostomy because of an severely destroyed larynx (two patients) and aspiration (three patients). CONCLUSION: Colopharyngoplasty is considered a complicated but trustworthy procedure to restore gastrointestinal tract continuity after severe corrosive injury. Undeniably, laryngeal involvement adversely affects the functional outcome. The post-operative course is frequently protracted, accompanied with several problems. Aspiration is nearly the most problematic event in the early post-operative period, which mandates a multidisciplinary approach to manage it.

Treatment of Esophageal Perforation with Primary Closure and Reinforcement Using TachoSil.

Two patients with iatrogenic esophageal perforation following rigid esophagoscopy for foreign body removal were successfully treated with primary repair and reinforcement using a collagen patch coated with human fibrinogen and thrombin (TachoSil, Nycomed, Austria, Vienna). The clinical implication of this report is that TachoSil can be used to bolster the repair site of esophageal perforation.

An unusual salvage technique for posterior tracheal membranous laceration associated with transhiatal esophagectomy: A transcervical-transsternal approach.

Various surgical approaches may be employed for esophageal resection. Major airway injuries due to transhiatal esophagectomy include vertical tears in the membranous trachea. Tracheal injury is an uncommon but potentially fatal complication. This article describes the technique to repair the posterior membranous tracheal tear, extended just over the carina through a transcervical-transsternal approach, thereby avoiding a second thoracotomy. Six patients with posterior membranous tracheal injury underwent this procedure. The laceration ranged from 3 cm to 5 cm in length. Four patients had received neoadjuvant chemoradiation. The management of tracheal laceration added approximately 60 minutes to the total operation time. There was no mortality related to tracheal injury. Patients were followed up for 6 months after surgery, and both posterior tracheal wall and transverse tracheotomy remained intact without stenosis. The transcervical-transsternal approach decreases the need of thoracotomy and its complications in patients with tracheal laceration in any stage, even in cases of an extended tear down to the carina.

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.

Different and Unpredictable Clinical Outcome of Ruptured Pulmonary Hydatid Cysts.

Most authors believe that the best treatment for pulmonary hydatid disease is surgical evacuation. Although albendazole has been used prophylactically before surgery, there are many reports about increased incidence of the rupture of cyst after albendazole therapy, which can cause some complications. In this case report we present a patient with bilateral pulmonary hydatid cyst that was ruptured after using albendazole and different strategies were used for management of each cyst.

Massive hemoptysis, a presentation of invasion of aneurysm of descending aorta to bronchopulmonary tree.

Aortobronchial (AB) fistula is a rare disease, which is presented with massive hemoptysis; lethal if not treated. It should be suspected in any patient who presents with massive hemoptysis and had previous thoracic aortic surgery, but even it may be seen in patients without any history of operation on the thoracic aorta. Although, today in many centers endovascular therapy is done for these patients, but it is not the standard approach. Surgery in urgent situations has an essential role in saving the patients. Operative management consists of double lumen intubation and one lung ventilation, followed by femoral artery and vein cannulation, posterolateral thoracotomy and achieving proximal and distal control on the aorta, applying cardiopulmonary bypass (CPB), separation the lesion, and bypass the segment of the diseased aorta by a synthetic graft.

The role of T-tubes in the management of airway stenosis.

OBJECTIVES: When the T-tube is inserted as a temporary stent, it is unclear whether keeping it longer in place has any benefit on the outcome. METHODS: Among 1738 patients with airway stenosis (1996-2011), 134 underwent T-tube placement (mean duration = 14.3 months); temporarily while waiting for an appropriate time for surgery in 53 (Group 1), as an adjunct after a complex laryngotracheal resection in 27 (Group 2), after surgical failure in 43 (Group 3) and permanently in 11 unresectable strictures (Group 4). A logistic regression model was used for statistical analysis. RESULTS: Seventy percent of patients were males (age = 33.6 ± 17 years). The main cause was postintubation/post-tracheostomy stenosis in 87% of patients. The stenosis (29.6 ± 14 mm, 5-80 mm) was located in the subglottis in 33%, trachea in 47% and both in 20% of cases. To assess the effect of T-tubes on stabilizing the airway after decannulation, 50 patients who still had a T-tube at the end of follow-up or for <1.5 months were excluded. Of the remaining 84, 31.5, 91.5 and 32.5% of patients in Groups 1, 2 and 3 were stable at least 3 months after decannulation. Moreover, 70% of those who were decannulated at or before 6 months and 53.7% of those who were decannulated after 6 months underwent another intervention (P = 0.17). The age, sex, cause, site of stenosis and even duration of T-tube insertion (P = 0.07) showed no significant effect on the decannulation outcome. CONCLUSIONS: Although it seems that keeping the T-tube in place for >6 months may increase the chance of successful decannulation, it was not confirmed in our study.

A successful third resection-anastomosis in a tracheal restenosis.

Reoperation due to recurrence after tracheal resection and reconstruction still seems challenging. Although recurrence may lead to serious morbidity, an appropriate surgical technique plays a significant role in the cure of these patients. We report our experience of a patient who successfully underwent a third resection and anastomosis of the trachea. We believe that the number of previous operations is not a contraindication by itself against reoperating on a patient with restenosis. Also the success rate might be acceptably high if a sufficiently healthy tracheal length remains.

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.