Congenital neonatal pyriform aperture stenosis (CNPAS) is a rare but potentially lethal condition that causes respiratory distress. The characteristic narrowing of the pyriform aperture along with other associated craniofacial dysmorphism is diagnosed using cross-sectional imaging such as computed tomography (CT) and magnetic resonance imaging. CT scan is the imaging of choice for confirming and characterizing CNPAS. Infants are obligate nasal breathers in the first 5 months of life. Hence, a high degree of clinical suspicion, prompt imaging diagnosis and adequate respiratory support is critical to help reduce the morbidity of this condition.
The upper airway is a crucial structure. It becomes a grave problem should it be narrowed. Several methods
of treatment were rendered for patients with laryngotracheal stenosis. We share our experience with the
combination total intravenous anaesthesia and apneic pause technique with or without steroid injection.
Four cases of laryngotracheal stenosis were observed in Hospital Ampang: two adult and two paediatric
cases. Age, gender, causative factor, stenosis segment length, grade or severity were observed before and
after dilatation, number of dilatation were observed and compared. The outcome measures are
decannulation and avoidance of tracheostomy. All cases had improvement of symptoms. Half or 50% of the
patient required repeated balloon dilatations. The paediatric cases successfully avoided tracheostomy while
the adult cases successfully decannulated with no complication from the procedure. Balloon dilatation by
total intravenous anaesthesia coupled with apneic method is a safe and effective method of treatment for
the narrowed airway.
The present work was carried out to investigate the blood flow behavior and the severity of blockage caused in the
arterial passage due to the different geometries such as elliptical, trapezium and triangular shapes of stenosis. The study
was conducted with respect to various sizes of stenosis in terms of 70%, 80% and 90% area blockage of the arterial
blood flow. The study was carried out numerically with the help of advance computational fluid dynamic software. It
was found that the shape of the stenosis plays an important role in overall pressure drop across the blockage region
of artery. The highest level of pressure drop was observed for trapezoidal shape of stenosis followed by elliptical and
then by triangular shaped stenosis. The wall shear stress across the stenosis is great for trapezoidal shape followed by
triangular and elliptical stenosis for same blockage area in the artery.
Chronic total occlusions remain one of the biggest challenges for interventional cardiologists and the high risk of restenosis and stent thrombosis is still a major problem. Drug-coated balloons showed favorable results for the treatment of in-stent restenosis and other lesion types. The aim of this study was to evaluate the feasibility and outcome of a drug-coated balloon only approach for chronic total occlusion.
Centromeres are prerequisite for accurate segregation and are landmarks of primary constrictions of metaphase chromosomes in eukaryotes. In melon, high-copy-number satellite DNAs (SatDNAs) were found at various chromosomal locations such as centromeric, pericentromeric, and subtelomeric regions. In the present study, utilizing the published draft genome sequence of melon, two new SatDNAs (CmSat162 and CmSat189) of melon were identified and their chromosomal distributions were confirmed using fluorescence in situ hybridization. DNA probes prepared from these SatDNAs were successfully hybridized to melon somatic and meiotic chromosomes. CmSat162 was located on 12 pairs of melon chromosomes and co-localized with the centromeric repeat, Cmcent, at the centromeric regions. In contrast, CmSat189 was found to be located not only on centromeric regions but also on specific regions of the chromosomes, allowing the characterization of individual chromosomes of melon. It was also shown that these SatDNAs were transcribed in melon. These results suggest that CmSat162 and CmSat189 might have some functions at the centromeric regions.
Wellen's syndrome is a pre-infarction stage of coronary artery disease characterised by predefined clinical and electrocardiographic (ECG) criteria of a subgroup of patients with myocardial ischaemia. Early recognition and appropriate intervention of this syndrome carry significant diagnostic and prognostic value. We report this unusual syndrome in an elderly man who presented with recurrent angina and characteristic ECG changes as T-waves inversion in the precordial leads, especially in V2-V6 during pain-free periods and ECG obtained during episodes of pain demonstrating upright T-waves with possible elevated ST segments from V1-V4. Cardiac enzymes were positive and coronary angiography revealed critical stenosis in the proximal left anterior descending artery. It is important to timely identify this condition and intervene appropriately as these patients may develop extensive myocardial infarction that carries a significant morbidity and mortality.
Hirschsprung's disease (HD) is one of the most well-known gastrointestinal motility disorders. Diagnosis and management of other lesser-known motility disorders are often challenging and tedious. We describe a teenager who was severely constipated from birth and needed intensive care admissions for life-threatening enterocolitis. She also had concomitant anal stenosis. Several rectal biopsies were unable to yield a conclusive diagnosis. Surgical level of resection had to be identified based on the motility of the bowel as determined by transit studies using oral ingestion of a milk feed labelled with Technetium-99m colloid. After completion of all operative stages, histopathological examination of the excised specimens concluded that she had short-segment HD associated with reduced interstitial cells of Cajal in the large bowel. She is currently continent, evacuating voluntarily approximately four times a day and is relieved of all her symptoms.
Central vein stenosis is not uncommon in hemodialysis-dependent patients as a result of mechanical damage to the vessel walls from prior cannulation. It can cause ipsilateral upper limb swelling and pain, resulting in suboptimal hemodialysis. It is unfortunate for bilateral central vein stenosis to develop concomitantly, and rare in the setting of an in-situ pacemaker. This case illustrates the successful ligation of a nondependent left arteriovenous fistula and stenting of the right subclavian vein with functioning ipsilateral arteriovenous fistula, to overcome the problem of symptomatic bilateral upper limb swelling.
The aim of the study was to examine and analyze the epidemiology and outcome of treatment for paediatric acquired subglottic stenosis treated with endoscopic bougie dilatation and topical mitomycin C. There were 15 patients identified from 2008 until 2013. All of them had acquired subglottic stenosis due to history of intubation. Majority of the patients had grade III stenosis, with the total of seven. Three patients had grade IV; three were grade II and two were grade I. All of the patients with severe stenosis (grade III and IV) needed tracheostomy while only one in mild stenosis group (grade I and II) required it for prolonged ventilation rather than obstruction due to subglottic stenosis. All of them underwent direct laryngoscopy under general anesthesia followed by endoscopic dilatation with bougie and topical mitomycin C 0.4 mg/ml for 5 min. Aim of success in our study was decannulation of tracheostomy or absence of symptoms at exertion. We achieved 6 (60 %) successful decannulation out of 10 patients with tracheostomy (excluded the patient with tracheostomy in grade I stenosis due to prolonged ventilation). As for those without tracheostomy, 3 (75 %) out of 4 patients were asymptomatic even at exertion. Average number of dilatation was 3.1 times, with mean duration of 28 min. No complications were reported in our series. One patient with grade I stenosis passed away due to severe pneumonia unrelated to the stenosis or dilatation, and she did not have any dilatation before she passed away. Multiple related risk factors were identified such as intubation, prematurity, movement of endotracheal tube, respiratory infection, traumatic intubation and gastroesophageal reflux disease. Experience of open surgical method was very limited in our centre in Sabah in East Malaysia. Endoscopic technique plays an important role in treatment of subglottic stenosis with adjunct like mitomycin C possibly booster the successful rate.
Flow of an electrically conducting fluid characterizing blood through the arteries having irregular shaped multi-stenoses in the environment of a uniform transverse magnetic-field is analysed. The flow is considered to be axisymmetric with an outline of the irregular stenoses obtained from a three-dimensional casting of a mild stenosed artery, so that the physical problem becomes more realistic from the physiological point of view. The marker and cell (MAC) and successive-over-relaxation (SOR) methods are respectively used to solve the governing unsteady magnetohydrodynamic (MHD) equations and pressure-Poisson equation quantitatively and to observe the flow separation. The results obtained show that the flow separates mostly towards the downstream of the multi-stenoses. However, the flow separation region keeps on shrinking with the increasing intensity of the magnetic-field which completely disappears with sufficiently large value of the Hartmann number. The present observations certainly have some clinical implications relating to magnetotherapy which help reducing the complex flow separation zones causing flow disorder leading to the formation and progression of the arterial diseases.
Tuberculous (TB) biliary stricture with calcified liver nodules rendering obstructive jaundice is a rare clinical phenomenon. Recently, we encountered a young patient with obstructive jaundice who was investigated in a general hospital. He was sent to our hospital for subsequent management after undergoing a series of investigations and biliary stenting. The radiological imagings performed revealed multiple calcified lesions in the liver with proximal bile duct strictures. Blood investigations, tumor markers and tuberculous work up were not remarkable. Subsequently, he underwent laparotomy and biliary reconstruction. Postoperatively, he was discharged well. Histopathological examination of the hepatoduodenal nodes showed chronic infections and granulomatous lymphadenitis, suspicious of a mycobacterium infection.
Background and objectives: Radiation treatment in nasopharyngeal carcinoma (NPC) is known to be associated with increased prevalence of carotid stenosis. The objectives of the study was to determine the prevalence of radiation-induced extracranial carotid stenosis, plaque, carotid intima thickness (CIMT) in NPC patients; to explore whether the stenosis is due to direct effect of radiation rather than general tendency to atherosclerosis. Methods: This was a cross-sectional study conducted in the University Malaya Medical Centre from July 2011 to February 2012. The study subjects consisted of 47 NPC patients who were treated with radiation, and 47 healthy control. The patients and control had carotid duplex ultrasound and transcranial Doppler (TCD). Results: The mean age of the patients was 55.1 years, the time lapse from radiation was 76.7 ± 95.3 months. Internal carotid artery (ICA) and common carotid artery (CCA) stenosis of ≥50% was seen in 17.0% of patients vs 2.1 % of controls (p = 0.031), with 61.7% of patients and 19.1% of controls having plaque in ICA and CCA (p=0.004). CIMT was increased in 70.2% of patients and 44.7% of controls (p =0.022). Both the patient group and control had similar rate of intracranial stenosis of 12.8% in TCD.
Conclusion: Extracranial internal carotid artery is the most common site of stenosis following radiotherapy in NPC. This suggests that local trauma from irradiation is the most important factor in predisposition to atherosclerosis following radiation therapy.
This case report describes a rigid bronchoscopy-assisted placement of a silicone airway Y-stent in a patient who developed tracheobronchomalacia following repeated dilatations for post-tuberculous airway stenosis. This is the first report of an airway Y-stent insertion in Malaysia.
Stenting of patent ductus arteriosus (PDA) is an attractive alternative to the surgical aortopulmonary shunt in the palliation of cyanotic congenital heart disease. However, the diverse morphology of PDA in this setting limits its role, as stenting an overly tortuous duct may not be feasible, and in a significant number of patients, ductus-related pulmonary artery stenosis contraindicates this procedure. The major acute complications are stent migration, thrombosis, and cardiac failure. Early failure of palliation caused by in-stent stenosis is another limitation of this procedure.
Study site: Institut Jantung Negara (IJN), Kuala Lumpur, Malaysia
Objective: To investigate the degree of stenosis of the internal carotid artery required for continuous blood flow in an interposition vein bypass to the middle cerebral artery. Methods: Computational fluid dynamics techniques were used to investigate a case of common carotid to middle cerebral artery brain bypass with varying degrees of internal carotid artery stenosis. Blood flow patterns across the patient-specific brain bypass were evaluated. Results: Simulation found that for cross section stenosis of less than 60%, no flow occurred in the bypass graft. Further narrowing of the internal carotid artery increased flow linearly within the bypass graft. There was significant energy loss and pressure gradient difference between the proximal and distal anastomosis sites of the bypass.
Conclusion: Computational fluid dynamics helps us to quantify the flow distribution, wall shear stress and pressure gradient in brain bypass surgery. The angle of the distal anastomosis had no effect on hemodynamic indices, allowing this consideration to be ignored in modeling. This modeling technique is useful to estimate the required degree of stenosis in the artery that is to be occluded to ensure sustained flow in the bypass. This will be of importance where there is staged surgery with a time interval between the bypass and the definitive internal carotid artery occlusion.
A 45-year-old woman with poorly controlled hypertension and diabetes mellitus presented with left iliac fossa pain, constipation alternating with diarrhea, and weight loss. She had been diagnosed with idiopathic cardiomyopathy five years previously. Echocardiogram had shown a left ventricular ejection fraction (LVEF) of 35%; coronary angiogram was normal. Colonoscopy revealed sigmoid colitis with stenosis. Abdominal computed tomography revealed a 5 cm right adrenal tumor. Twenty-four hour urinary free catecholamines and fractionated metanephrine excretion values were elevated, confirming pheochromocytoma. Her colitis resolved after one month of adrenergic blockade. Repeat echocardiogram showed improvement of LVEF to 65%. After laparoscopic right adrenalectomy, the patient's hypertension resolved, and diabetic control improved. Timely management avoided further morbidity and potential mortality in our patient.
The beneficial effects of stereotactic third ventriculostomy versus ventriculoperitoneal shunt were evaluated in 62 paediatric patients and analysed in relation to age, sex, clinical history, presence of meningomyelocele, magnetic resonance imaging measurements of hydrocephalus and third ventricle floor size. The third ventriculostomy were done on 50 patients using the Richard-Wolf Caemaert Endoscope and the Leksell Stereotactic Frame Model G. These patients were operated using the 4-French Fogarty catheter to open the base of the third ventricle. During the same period of study 12 paediatric patients with aqueduct stenosis who were managed by ventriculoperitoneal shunt were included. Both surgical procedures were compared. Statistically univariate analysis revealed that those patient with an age group of more than six months undergoing ventriculostomy had good outcome. Multivariate analysis revealed that past history of haemorrhage and/or meningitis were predictors of poor outcome. Sex, size of lumbar meningocele at birth, abnormal ventricular anatomy or narrow third ventricular floor size were non predictors of bad outcome in these patients. There was no difference in outcome in both the shunt or ventriculostomy group.
Thromboangitis Obliterans (TAO) or Buerger's disease is a non-atherosclerotic, occlusive, progressive and highly inflammatory disorder of distal arteries seen predominantly affecting the lower limb in smokers. TAO presenting itself in upper limb or bilaterally involving the upper limb is a very rare entity. We report on a rare case of TAO in a 46-year-old gentleman who presented with bilateral upper extremity digital gangrene with 18 pack-years of smoking. Brachial and radial pulses were palpable bilaterally and were of good volume but right ulnar pulse was faintly felt, while on the left side it was not appreciated due to malunion. Further Computed Tomography (CT)- angiography showed occlusion of distal right ulnar artery, stenosis of distal left ulnar artery with bilateral poor opacification of palmar and digital arteries. This case reports on the unusual and rare presentation of TAO in upper extremity involving both limbs. The case is reported for the first time from Malaysia.