In keeping with international trends, lung cancer incidence and mortality are increasing among the Irish population with many patients presenting with advanced disease that excludes the potential for curative management. Consequently palliative treatment options for this patient group are being increasingly explored with various degrees of success. Endobronchial stenosis represents a particularly challenging area of management among these patients and a number of techniques have been described without the identification of a single gold standard. We report our experience of the first time use of endobronchial cryotherapy in Ireland with reference to a case series, including an example of its use in the management of benign disease, in order to support patients with borderline lung function and enable definitive palliative treatment.
Bronchial stenosis; cryotherapy; lung cancer; lung transplant; palliative management
Lymphangioleiomyomatosis (LAM) is a rare disease that leads to airways and lymphatic channels obstruction due to abnormal smooth muscle proliferation. It presents with dyspnea, pneumothorax or chylothorax. Lung transplantation (LT) has emerged as a valuable therapeutic option with limited reports. We report a case of LAM that underwent double LT and complicated by refractory bilateral chylothorax which was managed successfully by povidone-iodine pleurodesis and the addition of sirolimus to the post-transplantation immunosuppressive therapy. The patient has no recurrence with 24 months follow-up.
Chylothorax; lung transplantation; lymphangioleiomyomatosis; pleurodesis; povidone iodine
Alpha 1-antitrypsin (AAT) belongs to the family of serpins (serine protease inhibitors). Loop sheet polymerization is the pathology behind serpinopathies which encompasses AAT, anti-thrombin III and neuroserpin deficiency. To the best of our knowledge, we report the first case of alpha 1-antitrypsin deficiency associated with hypogammaglobulinemia and recurrent pulmonary thrombosis without any concomitant use of drugs.
Alpha 1-antitrypsin deficiency; hypogammaglobulinemia; recurrent pulmonary thrombosis; serpinopathies
Food-dependent exercise-induced anaphylaxis (FDEIA) is one of the severe allergic reactions in which symptoms develop only if exercise takes place within a few hours of eating a specific food. It is important to consider FDEIA in cases of unexplained anaphylaxis as reactions can occur several hours after ingesting the culprit food(s). We herein report the first two cases of FDEIA in the Middle East. The first one is induced by wheat, while the other by peanut. The pathophysiology, predisposing factors, diagnosis, and treatment of FDEIA are also summarized here.
Anaphylaxis; food-dependent exercise-induced anaphylaxis; wheat
We present an interesting case of a complete vanishing of the left main bronchus in a lung transplant recipient who had a successful outcome due to acute respiratory support with venovenous extracorporeal membrane oxygenation in order to perform airway dilation.
Acute; extracorporeal membrane oxygenation; left main bronchus; lung transplantation; vanishing; venovenous
Bronchial atresia (BA) is characterized by a mucus-filled bronchocele in a blind-ending segmental or lobar bronchus with hyperinflation of the obstructed segment of the lung. We describe a neonate who presented on his 9th day of life with respiratory distress. Chest computed tomography showed a soft tissue density involving the right middle lobe (RML). RML lobectomy confirmed the diagnosis of BA. Cytomegalovirus was detected by polymerase chain reaction in blood, urine, and tracheal aspirates which may provide further insight into the pathogenesis of BA.
Bronchial atresia; congenital; cytomegalovirus; infection; neonate
We describe two cases of spontaneous pneumothorax in young healthy adults with no underlying structural lung disease. The onset of pneumothorax was following physical activity including playing musical instruments and blowing of balloons. There is sparse data evaluating the pathophysiology of primary spontaneous pneumothorax in relation to increased mouth pressures. These cases highlight the possible physical effect of valsalva manoeuvre on transpulmonary pressures, and the potential risk of developing pneumothorax in otherwise healthy individuals. This aspect of pneumothorax development is worthy of further exploration, to better elucidate the mechanism and enhance our understanding of this common respiratory presentation.
Musical/wind instruments; spontaneous pneumothorax; valsalva
Gastrobronchial fistula is a rare but serious complication of laparoscopic sleeve gastrectomy with significant morbidity and mortality. We present the case of a 30-year-old man who underwent laparoscopic sleeve gastrectomy for morbid obesity and presented later with a history of chronic productive cough. Upper gastrointestinal series showed the presence of a communicating fistula between the stomach and the left lung bronchial tree.
Gastric leak; gastrobronchial fistula; laparoscopic sleeve gastrectomy; subphrenic collection
To highlight a potentially fatal complication of broncho-vascular fistula arising from the self expanding metallic stent (SEMS) placement. We retrospectively analyzed five patients with benign and malignant airway diseases, who developed tracheo/broncho-vascular fistulas following SEMS placement in our tertiary care setting. All patients received either Wallstent or Ultraflex® stent (Boston Scientific, Natick, MA) between 1999 and 2007. All patients had received adjunct therapy such as balloon bronchoplasty, laser therapy or electrocautery. Most patients presented with massive hemoptysis. A total of 483 SEMS were placed during this period. SEMS placement can be complicated by Broncho-vascular fistula formation. True incidence and precise time interval between the insertion of stent and onset of this complication is unknown. Additional therapeutic modalities to maintain stent patency may enhance the risk of fistula formation. SEMS should only be used in a select sub-group of patients, after exhaustive evaluation of other treatment options. These cases provide evidence that broncho-vascular fistulas can develop at any time following SEMS placement, suggesting the need for a more cautious approach, especially while using them for a long term management. In benign airway disease, the stent should be removed as soon as healing has taken place.
Benign airways disease; bronchial stents; complications; endobronchial growth; ultraflex; wall stent
Ewing's sarcomas and peripheral primitive neuroectodermal tumors (ES/PNETs) are high grade malignant neoplasms. These malignancies are characterized by a chromosome 22 rearrangement, arise from bone or soft tissue, predominantly affect children and young adults, and are grouped in the Ewing family of tumors. Multimodality treatment programs are the treatment of choice. Primary localization of ES/PNET in the mediastinum is extremely rare. We describe a case of ES/PNET presenting as a mediastinal mass with tracheal compression and initial signs of superior vena cava in a 66-year-old woman.
Ewing's sarcoma; extraosseous; extraskeletal; peripheral primitive neuroectodermal tumor
A 22-year-old obese asthmatic woman with Influenza A (H1N1)-associated acute respiratory distress syndrome died from cerebral artery gas emboli with massive cerebral infarction while being treated with High-Frequency Oscillatory Ventilation in the absence of a right to left intracardiac shunt. We review and briefly discuss other causes of systemic gas emboli (SGE). We review proposed mechanisms of SGE, their relation to our case, and how improved understanding of the risk factors may help prevent SGE in positive pressure ventilated patients.
ARDS; cerebral gas embolism; high frequency oscillatory ventilation; influenza a H1N1; positive pressure ventilation; systemic gas embolism
The extraction of aspirated foreign bodies can prove challenging at times, requiring even rigid bronchoscopy. Cryotherapy probes have been reported to help with extraction of foreign bodies. We present a case where successful “cryoextraction” was performed on an aspirated chewing gum. The case highlights the fact that this technique is useful to extract all materials that have water content. This technique can be performed through flexible bronchoscopy and can save patients from more aggressive approaches.
Bronchoscopy; cryotherapy; cryoextraction; foreign body; gum
Sternal metastasis in thyroid cancer is an uncommon occurrence with only a handful of cases of chest wall resections being done. Sternal reconstruction for both primary and secondary tumors has been performed using various techniques and materials such as the mesh, methyl acrylate resin, and steel plates; however, this is a case of papillary thyroid cancer involving the sternum in a 50-year-old woman who had resection of the sternum with reconstruction using titanium bars and clips (STRATOS system) fixed to the clavicles with an underlying Proceed mesh. STRATOS system showed good recovery postoperatively. The functional results were excellent with the patient being able to perform all daily activities unassisted after 1 month and almost complete range of motion with acceptable limitations in power of the shoulder muscles after 2 months. We have reviewed all the English language publications of the subject by doing Medline search for the last 25 years and we present here the surgical management of this pathology with our novel approach by using the titanium steel bars to stabilize both medial aspects of the resected clavicles as a promising therapy for manubrial reconstruction and clavicular fixation.
Reconstruction; sternum; stratos; thyroid; titanium
We report a rare case of bilateral congenital lobar emphysema in a 2-month-old male infant who presented with severe respiratory distress leading to respiratory failure. Plain chest X-ray and later high-resolution CT scan of the chest revealed that both the right middle and the left upper lobes were emphysematous. Surgical excision of the affected lobes has been done successfully in two sequential operations of right middle lobectomy followed by left upper lobectomy which resulted in significant improvement of respiratory status. In this report, the presentation, diagnosis, and surgical management of this rare condition are discussed.
Congenital lobar emphysema; Infant; lobectomy; respiratory distress
A 6-month old baby referred to our department because of recurrent attacks of respiratory distress with chest infection. Chest radiology revealed reduction of the right hemithorax with mediastinal shift to the right. Multidetector computed tomography showed hypoplasia of the right lung and right pulmonary artery, systemic arterial supply to the lung from the abdominal aorta, and and absence of right venous drainage to the left atrium. This picture is consistent with congenital pulmonary venolobar syndrome. The patient underwent right pneumonectomy; the postoperative course was uneventful.
Congenital anomaly; lung; pulmonary sequestration; scimitar syndrome
Pulmonary tumor thrombotic microangiopathy (PTTM) is a fatal cancer-related pulmonary complication with rapidly progressing dyspnea, and occasionally induces sudden death. Here, we describe a postmortem-diagnosed PTTM case caused by gastric cancer, with the complaint of progressing dyspnea for 5 days.He did not have any abdominal symptoms or cancer history. PTTM should be considered in patients with rapidly worsening respiratory conditions, even if there is no cancer history.
Dyspnea; gastric cancer; PTTM
This was a case of a 35-year-old man with mediastinal mass requiring computed tomography (CT)-guided biopsy for tissue diagnosis. A posterior approach with an 18-gauge biopsy needle was used to obtain tissue sample. Post biopsy, patient condition deteriorated and multiphase CT study detected active bleeding in arterial phase at the biopsy site with massive hemothorax. Subsequent angiography showed arterial bleeder arising from the apical branch of the right pulmonary artery. Selective endovascular embolization with NBCA (n-Butyl cyanoacrylate) was successful. Patient survived the complication. The case highlighted a rare complication in a common radiology procedure and the value of the interventional radiology unit in avoiding a fatal outcome.
Biopsy; complications; computed tomography; guidance; mediastinum; therapeutic embolization
Poland sequence is a rare congenital anomaly involving the chest wall and arm, displaying differing degrees of severity, functional and aesthetic impairments. Here we report a series of two cases that presented to us with this anomaly. These cases illustrate, for physicians, the importance of physical diagnosis and reinforce the practice of looking for additional anomalies when one is discovered.
Aesthetic impairments; congenital anomalies; Poland sequence; Poland syndrome
“PHACES” is a neurocutaneous syndrome that refers to the following associations: Posterior fossa malformations, Hemangiomas, Arterial malformations, Coarctation of the aorta/Cardiac defects, Eye abnormalities, and Sternal defects. Herein, we report the association of PHACES syndrome with airway hemangioma, a serious association that should not be overlooked. The findings of such an association presented here are the first to be reported from Saudi Arabia.
Airway; hemangioma; PHACES; subglottic
Congenital H-type tracheoesophageal fistula (TEF) in adults is a rare presentation and can test the diagnostic acumen of a surgeon, endoscopist, and the radiologist. These undetected fistulas may present as chronic lung disease of unknown origin because repeated aspirations can lead to recurrent lung infections and bronchiectasis. Congenital TEFs should be considered in the diagnosis of infants and young adults with recurrent respiratory distress and/or infections. Here, we present the successful management of this rare case in an adult patient.
Adult; congenital tracheoesophageal fistula; late presentation
Bacille Calmette-Guerin (BCG) has been used extensively as a vaccine against human tuberculosis. Herein, we describe gluteal tuberculosis abscess due to inadvertently injected BCG a patient with bladder cancer.
Bacille Calmette-Guerin; bladder cancer; gluteal abscess; tuberculosis
Pulmonary alveolar microlithiasis (PAM) is an uncommon lung disease characterized by accumulation of intraalveolar calcifications. The disease can be diagnosed based on the radiological findings. We present a 27-year-old women with five-year history of shortness of breath. She was diagnosed with PAM due to the presence of the characteristic chest X-ray and thorax computed tomography (CT) findings. We performed 18F-fluorodeoxyglucose (FDG)-PET/CT imaging in order to detect any evidence of inflamation in the lung before deciding an anti-inflammatory treatment. The lung regions with dense calcifications revealed low FDG uptakes (SUVmax: 2.7) and the lung regions without calcifications showed lower FDG uptakes. No further treatment modality was planned besides inhaler salbutamol. Herein, we discuss this rare entity with literature search.
FDG; inflammation; PET/CT; pulmonary alveolar microlithiasis
Radiation therapy is used to treat breast malignancies. Old external radiotherapy protocols included high-dose schedules and were associated with late complications. We describe the case of a 72-year-old woman who was admitted with recurrent bleeding from chest wall. Arteriovenous fistulae were diagnosed. Embolization therapy was initially successful, even though the bleeding episodes became recurrent. Arteriovenous fistulae should be included among differential diagnosis of thoracic bleeding in patients with history of local radiation therapy. The prognosis is poor and endovascular treatment may be considered a first-line treatment.
Chest wall irradiation; complication; fistula; radiation therapy
The administration of intrapleural streptokinase (IPSK) is widely practiced in the management of loculated empyema thoracis. To our knowledge, there have been only 4 cases of hemorrhagic complications attributed to the administration of IPSK reported in the literature. In this article, we report a case of a 17-year-old girl who received IPSK and developed shock, anemia, coagulopathy and massive hemothorax. Our discussion focuses on the hemorrhagic complication of chest tube insertion and the role of IPSK in blood clot lysis and inhibition of local hemostasis.
Coagulopathy; complications; hemothorax; streptokinase; thoracic empyema
We report a case of pulmonary strongyloidiasis in a patient with large granular lymphocytosis. He was on short-term high dose immunosuppressant therapy. A 77-year-old white male presented to the emergency room with fever and shortness of breath for 10 days. The patient had been diagnosed about 3 months prior to this presentation with “large granular lymphocytosis” (LGL) after a workup for pancytopenia. Methotrexate and prednisone had been started 1 month ago for the treatment of LGL. Five days prior to the current presentation, he had been started on moxifloxacin as an outpatient but got progressively worse and came to an emergency room. Bronchial washings (bronchoalveolar lavage) demonstrated numerous filariform larvae of Strongyloides stercoralis. The patient was treated with ivermectin and improved. Pulmonary strongyloidiasis should be considered in the differential if X-ray findings show a interstitial or alveolar pattern and if the patient has visited the endemic areas, even in the remote past.
Bronchoalveolar lavage; large granular lymphocytosis; strongyloidiasis