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Immune reconstitution inflammatory syndrome in HIV-infected patients with mycobacterial infections starting highly active anti-retroviral therapy.

AIM: To describe the radiological appearances of immune reconstitution inflammatory syndrome (IRIS) in human immunodeficiency virus (HIV)-infected patients with mycobacterial infections starting highly active anti-retroviral therapy (HAART). MATERIALS AND METHODS: Five consecutive HIV infected patients with IRIS due to mycobacterial infection were studied. Intercurrent infection and poor drug compliance were excluded as causes of presentation. The chest radiological appearances at the time of starting HAART and at the time of diagnosis of IRIS were compared. RESULTS: In these five patients there was clinical and radiological deterioration, occurring between 10 days and 7 months after starting HAART, leading to unmasking of previously undiagnosed mycobacterial infection or to worsening of mycobacterial disease. All five patients had HAART-induced increases in CD4+ T lymphocyte counts and reductions in peripheral blood HIV "viral load". Chest radiographic abnormalities due to IRIS included marked mediastinal lymphadenopathy in three patients-severe enough to produce tracheal compression in two patients (one of whom had stridor)-and was associated with new pulmonary infiltrates in two patients. The other two patients had new infiltrates, which in one patient was associated with a pleural effusion. CONCLUSION: These cases illustrate the diverse chest radiographic appearances of IRIS occurring after HAART in patients with mycobacterial and HIV co-infection. Marked mediastinal lymphadenopathy occurred in three of these five patients (with associated tracheal narrowing in two patients); four patients developed pulmonary infiltrates and one had an effusion. The cases further highlight that the onset of IRIS may be delayed for several months after HAART is started.

AIDS-Related Opportunistic Infections↗

Acromegaly due to a somatroph adenoma in a dog.

A 10-year-old uncastrated male Dalmatian dog was referred for gait abnormalities consisting of chronic progressive stiffness and rigidity. Other symptoms were polyphagia associated with weight gain, polyuria and polydipsia, excessive panting, and an inspiratory stridor. The owner had noticed progressive thickening of the skin and enlargement of the tongue over the last 3 years. Physical examination revealed thickening of the skin, redundant skin folds, and enlargement of the tongue. The only remarkable abnormalities found on routine laboratory examination were mild anaemia and an increased serum fructosamine concentration. Circulating concentrations of total thyroxine, free thyroxine, and cTSH, and the results of an ACTH stimulation test were all within reference ranges. The basal serum growth hormone (GH) concentration was markedly elevated (23microg/l) and did not decrease during a glucose tolerance test or after somatostatin administration. The serum insulin-like growth factor-1 concentration was also markedly elevated (1254microg/l). Basal serum insulin concentration was high (95mU/l) and insulin concentrations increased considerably after glucose loading, consistent with insulin resistance. Abdominal ultrasonography showed no abnormalities. Survey radiographs of the vertebral column showed severe spondylosis deformans extending from the cervical to the lumbosacral spine. CT scanning of the skull showed an enlarged pituitary gland with normal enhancement pattern. On post-mortem examination, the entire vertebral column appeared as a single and inflexible structure due to the presence of multiple fused osteophytes. The pituitary gland contained an acidophilic adenoma that immunostained positively for GH (and negatively for ACTH and alpha-MSH). In conclusion, this Dalmatian dog with acromegaly and insulin resistance represents the first case of GH hypersecretion proven to be due to a somatotroph adenoma.

Acromegaly↗

A forgotten old disease: mediastinal tuberculous lymphadenitis in children.

OBJECTIVE: The purpose of the study was two-fold: (1) to highlight the varied presentation of mediastinal tuberculous lymphadenitis (MTL) in children and (2) to identify parameters, that may help in the early diagnosis of this condition. METHODS: Between January 1995 and December 2002, 13 children with histological diagnosis of MTL were retrospectively assessed for age at presentation, history of exposure to TB, presenting symptoms, investigations, initial diagnosis, surgical treatment and outcome. Stepwise multiple linear regression analysis was used to determine potential risk factors for early diagnosis of MTL. RESULTS: Thirteen children presented with: (a) fever, night sweats and weight loss (4); (b) acute respiratory distress (2); (c) cough and shortness of breath (SOB) (5); (d) stridor (1); and (e) chest pain (1). TB was suspected only in 6 children (46%) at presentation. In the other 7 cases (54%) the presumed diagnoses were: neuroblastoma (n=1), metastatic malignancy (n=1), bronchial polyp (n=1), bronchogenic cyst (n=2), and presumed foreign body (n=2). Bronchoscopy was diagnostic in identifying cheesy material within the bronchus and organisms on lavage in 4 (30%) and in identifying external compression in 2 (15%). Thoracotomy and excision of the lymph node mass was necessary to treat the mediastinal compression and to ascertain the diagnosis of TB in 3 children (23%). All 13 children had complete resolution of tuberculous lymphadenitis following anti-tuberculous treatment. The diagnostic clues in this cohort of patients were cough and SOB with history of exposure to tuberculosis (P=0.0001) and bronchoscopy and lavage with positive staining for acid-fast bacilli (P=0.0001). CONCLUSIONS: Tuberculosis was not suspected in 54% of children with MTL, and they posed diagnostic dilemma on admission. Bronchoscopy must be used as a diagnostic tool in children where tuberculosis cannot be excluded by radiology or specific skin tests. Thoracotomy and excision may be necessary to treat the obstructive symptoms.

Bronchoscopy↗

Management of congenital tracheal stenosis in infancy.

OBJECTIVE: Congenital tracheal stenosis (CTS) is a very infrequent malformation. Till recently, the outlook for these patients was dismal because medical management was the only way of treatment. Surgical and endoscopical techniques developed in the last years have improved the prognosis. We review the short- and long-term outcomes of a single institution experience in the management of children with CTS, comparing different treatment modalities. METHODS: Between 1991 and 2004, 19 cases of CTS have been managed in our Unit. Respiratory symptoms varied from mild stridor on exertion to severe distress. Bronchoscopy was performed for diagnostic purposes in all cases; other imaging techniques (computed tomography (CT), magnetic resonance imaging (MRI), bronchography, angiography, doppler-ultrasound) were performed on an individual basis. According to clinical and endoscopical features, patients were classified into three groups. The following data have been studied in each case: sex, age at diagnosis and treatment, anatomical type, associated anomalies, treatment modality, complications, outcome and time of follow-up. RESULTS: Ten boys and nine girls have been included in this study. Age at diagnosis ranged from 3 days to 7 years (median, 4 months) and 84% of cases showed associated anomalies. Five patients presented mild or no symptoms and have been managed expectantly. The other 14 cases were operated on because of persistent or severe clinical symptoms. The following procedures were performed: slide tracheoplasty (n = 7), costal cartilage tracheoplasty (n = 5), tracheal resection and reconstruction (n = 3), endoscopical dilatation (n = 3), stent placement (n = 1), and laser resection (n = 1). Three patients required two or more procedures and surgical survival rate is 78%. Overall mortality in the series is 21% and all survivors (15 patients) are asymptomatic or show mild symptoms with respiratory infections only. Follow-up is complete, ranging from 8 months to 12.3 years (mean, 5 years). CONCLUSIONS: Bronchoscopy is our preferred diagnostic tool. Selection of the type of treatment depends on the patient's clinical status and the anatomical pattern of the stenosis. In symptomatic cases with short-segment stenosis (<30% of total tracheal length), we prefer tracheal resection with end-to-end anastomosis; for long-segment stenosis (>30%), slide tracheoplasty is our procedure of choice.

Abnormalities, Multiple↗

Sixteen-row multislice computed tomography in infants with double aortic arch.

BACKGROUND: The introduction of multislice computed tomography (MSCT) in 1998 has led to a considerable boost of CT angiography. Four-row MSCT has been employed in the diagnostic assessment of vascular rings. Sixteen-row MSCT is expected to further increase the diagnostic power of MSCT. We report three cases of double aortic arch diagnosed by a 16-row MSCT, and discuss the possible advantages of this diagnostic tool. PATIENTS AND METHODS: From April 1, 2003, to September 1, 2003, three patients underwent 16-row MSCT at our institution to evaluate the possible presence of a vascular ring. All patients presented with stridor and feeding difficulties. MSCT was performed under bland sedation (chloral hydrate 60 mg/kg). ECG gating and breath hold were not employed. Three-dimensional reconstructions were employed to assess the presence of airway compromise as well as of vascular anomalies. RESULTS: Scanning time averaged 4 s (3.8-4.5 s). Motion artefacts were not relevant. The CT scan showed the existence of a vascular anomaly as well as of a significant tracheal compression in all cases, and was considered conclusively diagnostic by the cardiac surgeons. All three patients underwent uncomplicated corrective surgery without further investigations. CONCLUSION: Sixteen-row MSCT with 3D reconstruction allows a precise evaluation of the airway compromise and a detailed assessment of the anatomy of vascular anomalies. The very short scanning time allows to avoid deep sedation and anaesthesia, and to obtain higher image quality and spatial resolution, with a concomitant reduction of the radiation dose.

Aorta, Thoracic↗

Simple method of vocal cord lateralization in bilateral abductor cord paralysis in paediatric patients.

OBJECTIVE: To simplify the procedure of suture lateralization of a vocal cord in children with bilateral abductor cord palsy and to evaluate its results. METHODS: Ten tracheostomised infants and children with bilateral abductor cord palsy where there was either minimal or no recovery of vocal cord movement even after 2 years' observation were taken up for suture lateralization of one vocal cord. In this simplified procedure needles were passed twice through the larynx from one side of neck to the other. The aim was to reduce the microlaryngoscopic or telescopic manipulation inside the small larynx. Two techniques (1 and 2) were used to carry out this procedure. In technique 1 the needle used was one with a slit in the eye situated at its tail end whereas in technique 2 it was a lumbar puncture needle. Patient follow-up ranged from 6 months to 5 years. RESULTS: Intraoperative manipulation inside the larynx was considerably reduced. The technique 2 was even simpler and faster than technique 1. With both techniques the placement of suture was accurate without any knot inside the larynx. All the patients could be decanulated with only minimal stridor on exertion in two and an acceptable voice in all. CONCLUSIONS: Suture lateralization of one vocal cord using technique 2 can be tried as the first line of management in cases of bilateral abductor cord palsy requiring lateralization.

Adolescent↗

Inspiratory muscle strength training with behavioral therapy in a case of a rower with presumed exercise-induced paradoxical vocal-fold dysfunction.

Paradoxical vocal fold dysfunction (PVFD) with high effort exercise can result in disruptions to ventilation, dyspnea, inspiratory stridor, elevated heart rate, and syncope. This single subject study experimentally tested an inspiratory muscle strength training (IMST) program with behavioral therapy on a 15-year-old male crew member. Outcome variables were maximum inspiratory pressure (MIP), and dyspnea ratings. Following 5 weeks of IMST, MIP increased by 93% from baseline function while dyspnea ratings substantially decreased. Outcome included successful competition with his high-school crew team, a task he was previously unable to complete. Discussion focuses on IMST combined with traditional approaches of voice therapy for treating PVFD.

Adolescent↗

Failure to thrive caused by the coexistence of vallecular cyst, laryngomalacia and gastroesophageal reflux in an infant.

Vallecular cyst is a rare but dangerous cause of stridor in neonates and young infants. Without recognition and proper therapy, the resulting airway obstruction can lead to serious morbidity and mortality. We herein report a young infant with a vallecular cyst synchronous with laryngomalacia and gastroesophageal reflux. The infant initially presented with signs and symptoms mimicking more benign laryngomalacia. Diagnosis was delayed with accompanying failure to thrive. The cyst was revealed by a barium esophagogram and confirmed by flexible bronchoscopy. Laryngomicrosurgery with CO(2) laser not only removed the cyst, but resolved the co-existing laryngomalacia and gastroesophageal reflux as well. After surgery, the infant was symptom free and exhibited a steady weight gain. There was no recurrence during 1 year of follow-up. We have reviewed the literature for this rare and potentially devastating lesion.

Carbon Dioxide↗

Balloon dilatation following tracheal reconstruction for congenital microtrachea.

A male infant presented with failed extubation was diagnosed with congenital microtrachea. After primary combined surgery with excision of a left pulmonary artery sling and microtracheal resection with anastomosis incorporating the excised microtrachea as an autologous anterior tracheal graft he was treated with balloon dilatation of the re-stenosed segment on three occasions during the following year. During the 4 years follow-up, stridor has been minimal and intermittent. His feeding and growth have been normal. We have found balloon dilatation to be a useful adjunct to tracheal reconstructive surgery in this difficult condition.

Catheterization↗

Rhabdomyosarcoma presenting as an anterior neck mass and possible thyroid malignancy in a seven-month-old.

A seven-month-old male presented with a one month history of an enlarging left neck mass and worsening inspiratory stridor. Upon excision of the mass, pathologic examination was consistent with embryonal rhabdomyosarcoma (RMS). Preoperative imaging and intraoperative exploration were consistent with tumor replacing the left lobe of the thyroid. No cases of either anterior neck rhabdomyosarcoma or thyroid rhabdomyosarcoma have been explicitly described in the literature. The distinction between the two malignancies becomes important when considering prognosis and treatment protocols.

Antineoplastic Combined Chemotherapy Protocols↗

Ectopic thymus presenting as a subglottic mass: diagnostic and management dilemmas.

The commonest subglottic mass in infants is a congenital haemangioma, which is usually managed conservatively without a histological diagnosis. Ectopic cervical thymus is rare and usually presents as a cervical mass, with only one case of subglottic ectopic thymus reported to date. Due to its rarity, the diagnosis in most cases relies on surgical excision and histological examination. However, histological diagnosis may not always be easily reached, as is demonstrated in this case report. In this article, an infant with congenital stridor secondary to a subglottic mass is described and the clinical and diagnostic difficulties in its management are discussed.

Choristoma↗

Successful laser ablation of diffuse laryngeal swelling in posttransplant lymphoproliferative disorder: the Japanese first case.

Posttransplant lymphoproliferative disorder (PTLD) is a well-known complication of pediatric organ transplantation. The first symptoms are often in the ear, nose, or throat (ENT) area. We report the first Japanese case of an airway obstruction in PTLD. A 7-year-old boy who had a liver transplant at the age of 2 years presented with progressive stridor. Fiberoptic examination revealed enlarged epiglottis and swelling arytenoids. Highly suspicious of early-PTLD, he was treated with reduction of immunosuppression, but no improvement on his larynx was observed. Laser ablation of swelling arytenoids worked. Surgical debulking is effective to ease an airway obstruction in cases of PTLD.

Airway Obstruction↗

The role of the anterior cricoid split in facilitating extubation in infants.

OBJECTIVE: The use of the anterior cricoid split (ACS) procedure has facilitated the extubation of children with early subglottic stenosis who may otherwise have required a tracheostomy. Criteria have been established for the evaluation of patients suitable for ACS and adherence to these guidelines has increased the extubation rate and reduced mortality. The objective of the study was to assess the use of the procedure at our tertiary referral centre. METHOD: In this retrospective clinical study, 33 patients were identified over an 11-year period between 1993 and 2004 with subglottic narrowing at the level of the cricoid ring, and who subsequently underwent an ACS. Demographic data, duration of intubation, indication and number of reintubations, success rate and complications were noted. Patients included in the study were infants who repeatedly failed trials of extubation and those who presented with persistent stridor. RESULTS: Twenty-nine out of 33 (88%) children were successfully extubated as a result of the cricoid split procedure. Four children failed extubation after the cricoid split. Three required a tracheostomy and one child remained intubated for a prolonged period. CONCLUSION: The anterior cricoid split procedure has revolutionised the management of early subglottic stenosis in selected neonates with failed extubation. It is a safe operation, and if successful it avoids the formation of a tracheostomy.

Cricoid Cartilage↗

Airway obstruction secondary to tracheobronchial involvement of asymptomatic undiagnosed Crohn's disease in a pediatric patient.

Tracheal involvement in Crohn's disease is extremely rare and can present with airway obstruction in any age group, including the pediatric patient. We describe the case of a 9-year-old female who presented with a 2-week history of intermittent stridor, resolving temporarily with the administration of systemic steroids. The patient denied any abdominal complaints and was otherwise healthy. Bronchoscopy revealed progressive tracheal inflammation necessitating an urgent tracheotomy. Subsequent laboratory investigation confirmed the diagnosis of Crohn's disease. This is the first reported case of previously undiagnosed Crohn's disease presenting with airway manifestations.

Airway Obstruction↗

Obstructive sleep apnea syndrome secondary to pharyngolaryngomalacia in a neonate with Down syndrome.

We described a case of obstructive sleep apnea secondary (OSAS) to pharyngomalacia and laryngomalacia in a neonate with Down syndrome. She presented with oxygen requirement and paradoxical breathing without stridor. Sleep polysomnography revealed obstructive apnea (obstructive apneic index of 14 h(-1)). Flexible bronchoscopy revealed collapsed velopharynx and omega-shaped larynx leading to the diagnosis of pharyngolaryngomalacia. She was treated with nasal prong continuous positive airway pressure. On this treatment, repeat polysomnography revealed abolition of obstructive apnea. Our case leads us to speculate that pharyngomalacia is an under-recognized condition in Down syndrome because this is expected to occur as part of generalized floppiness of airway of Down syndrome. In fact pharyngomalacia is a newly described clinical entity, which if unnoticed is associated with failure of supraglottoplasty in relieving symptoms of airway obstruction of laryngomalacia.

Down Syndrome↗

Pediatric postcricoid vascular malformation: a diagnostic and treatment challenge.

Hemangioma of the upper aerodigestive tract is a rarely reported occurrence in the pediatric literature. To date, there have been three published case reports of postcricoid hemangiomas contributing to unexplained dysphagia and respiratory distress. We present three children with a history of swallowing difficulty and stridor who were found to have an occult postcricoid mass. Valsalva maneuvers confirmed the suspicion of a vascular malformation in both patients. Transoral laser therapy (KTP and CO2) was used to ablate the lesions. The patients are symptom-free at 5 months, 5 years, and 2 weeks, respectively. The diagnostic challenge in evaluating these children and the therapeutic choices are described.

Bronchoscopy↗

Rigid bronchoscopy for the suspicion of foreign body in the airway.

OBJECTIVE: Airway foreign bodies present a diagnostic dilemma and has been recognized for many years. Since aspiration of foreign bodies can be a serious and sometimes fatal problem, early intervention and proper management is vital. METHOD: In this retrospective study, the results of 1887 bronchoscopies, which were performed between the years 1973 and 2004 for the suspicion of foreign body aspiration in children, were presented. Various instruments and techniques were used over 31-year period and rigid bronchoscopy was the preferred method of foreign body extraction. RESULTS: There were 1106 boys and 781 girls with the median age of 2.3 years. Seventy-four percent of patients were less than 3 years old. The most common type of foreign body (89.9%) was organic; watermelon seeds (39.7%) were the most frequent organic foreign bodies. Eight hundred and twenty-three patients (43.6%) were referred to our clinic within the first 24h of the event while 4.5% of the patients were admitted to the hospital with the suspicion of foreign body in the airway later than one month. At bronchoscopy, a foreign body was identified in 79.1% of patients and no foreign body was seen in 20.9% of patients. Foreign bodies were encountered in 96.3% of the patients with positive history whereas 28.1% of the patients with negative history had foreign body. Of the patients with foreign bodies, 93.2% had positive history. Overall, the positive history was obtained from 85.2% of patients. The incidence of postbronchoscopic tracheotomy, thoracotomy, and overall mortality rate were 0.47, 0.15, and 0.21%, respectively. CONCLUSION: Otolaryngologists should consider foreign body aspiration in the airway in the differential diagnosis of any patient with the complaints of stridor, dyspnea, sudden onset of cough and intractable and recurrent lower respiratory tract infections. A careful history and physical examination were strong indicators of the diagnosis and raised the index of suspicion of an aspirated foreign body. Timely intervention with the experienced surgical team would decrease the complication rate and mortality rate. However, prevention of aspiration with the education of parents and caregivers is very important.

Adolescent↗

Subglottic cysts in children: a 10-year review.

Subglottic cysts are associated with prematurity and periods of early neonatal intubation and are a rare cause of infant stridor. In this study we analyse our experience of this rare but important problem. We discuss the aetiology, diagnosis and management of subglottic cysts. We carried out a retrospective review of all cases of subglottic cysts presenting to the otorhinolaryngology department at the Leeds teaching hospitals trust during the period between 1995 and 2005. In total seven patients were identified. A retrospective review of the case notes of all patients proven to have subglottic cysts at direct laryngoscopy and bronchoscopy was undertaken. Seven patients were identified, four males and three females. Five were born premature (24-31 weeks). All were intubated with average intubation period of 20.6 days. Six of our patients' underwent endoscopic marsupialisation using cup forceps and one with a contact diode laser. Two patients had recurrence of the cyst and had a repeat of endoscopic marsupialisation. This study highlights the associated risk of neonatal intubation with the risk of formation of subglottic cysts, and the need for early diagnosis and appropriate treatment to avoid unnecessary morbidity.

Bronchoscopy↗