Congenital obstructive uropathy is a rare cause of ascites in infants. Majority of reported cases of genitourinary causes of ascites were due to posterior urethral valve. Here, we report a ...6-month-old boy who presented with progressive tense ascites and peritonitis attributed by unilateral left distal ureteric obstruction and acute pyonephrosis. He underwent left nephrostomy placement, after which there was a remarkable improvement of ascites. He then underwent left ureteral diversion procedure a month later with a tentative plan for ureteral reanastomosis in 6 months. To date, there are no reports describing ascites secondary to distal ureteric obstruction beyond the neonatal period. The objective of this case report is to highlight unilateral urinary tract obstruction as a potential cause of transudative ascites. Additionally, the superimposed infection in the obstructed collecting system can lead to acute peritonitis likely due to translocation of bacteria into the peritoneal cavity.
Pfeiffer syndrome is a rare inherited craniofacial disorder. Upper airway obstruction is common among patients with Pfeiffer syndrome due to craniosynostosis. They may also present with lower ...respiratory tract obstruction due to a rare congenital airway malformation called tracheal cartilaginous sleeve (TCS). We report the case of a patient with Pfeiffer syndrome who presented with recurrent bronchopneumonia, discovered incidentally to have TCS via direct visualisation during tracheostomy. Relevant literature for this rare clinical condition are reviewed and discussed. Clinicians should be aware of TCS when encountering patients with craniosynostosis who present with recurrent lower respiratory tract infections. Careful and meticulous investigations should be performed to look for TCS, especially in patients with craniosynostosis.
Down syndrome (DS) is the most common human chromosomal genetic disorder; caused by extra copy of chromosome 21-one out of 792 babies globally and one in 950 in Malaysia.
To obtain spectrum of upper ...airway pathology in DS children-prevalence, causes and management practices.
A cross-sectional study of children with DS aged less than 18 years old with signs of UAO was conducted. Diagnosis of UAO, management and final outcome was observed and documented.
Ninety-one patients were recruited. 20 cases (22%) had significant noisy breathing which indicates the prevalence of upper airway pathology (p=.025). Laryngomalacia (34.5%) was the most common pathology, followed by paediatric SDB (24.1%), tracheal bronchus (17.2%), tracheal stenosis (6.9%), and tracheomalacia (6.9%). Seven cases (7.7%) presented with respiratory distress. 12 cases were managed conservatively, while eight cases required further investigations under general anaesthesia (GA) with/without surgical intervention. Six cases showed symptoms resolution within a week post-surgical intervention, and another case resolved after 30 days and one case resolved after 8 months of post-surgical intervention.
Early detection and high index of suspicion is required to diagnose and treat these anomalies timely.
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DOBA, IJS, IZUM, KILJ, NUK, OILJ, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK, VSZLJ
Abstract Introduction Paediatric sleep disordered breathing (SDB) is known to cause significant complications affecting the somatic growth, metabolic and cardiovascular system. It is increasingly ...reported and believed to contribute for poor quality of life that will affect emotion and cognitive function and both children and family members. Objectives To study the impact of adenotonsillectomy on quality of life in children with SDB. Design and Setting: Prospective study conducted on children with SDB at Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia. Method Severity of SDB was assessed using preoperative ApneaLink test and scoring from preoperative OSA-18 questionnaire done by caretakers. All participants underwent standard cold instruments adenotonsillectomy. Impact of adenotonsillectomy was assessed by postoperative ApneaLink test and score of postoperative OSA-18 questionnaire. Results 32 patients were recruited in the study. The median score for preoperative OSA-18 score was 57.5. Postoperatively, the score significantly improved to 28.5. Domains of physical distress, caretaker preoccupation and sleep disorders showed major improvement. Postoperative Apnealink reading also showed significant reduction in ODI level. Conclusion Adenotonsillectomy for children with SDB significantly improved the quality of life.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
Giant cell tumor (GCT) is one of the most common tumors of bone and is the most common precursor of aneurysmal bone cysts (ABC). The clinical behavior of concurrent GCT and ABC can be very aggressive ...in children. GCT of the ribs, with or without ABC, is rarely seen in children. We report a case of an 8-year-old girl with GCT and associated ABC of the ribs who presented with sudden onset of chest pain and breathlessness due to a hemothorax. The patient was successfully treated by surgical resections and arterial embolization. She has remained well for 4 years after the initial surgery.
Background: In pediatric patients, video-assisted thoracoscopic surgery (VATS) is usually carried out using three to five working ports. The port-less technique (PLT) means only one or two ports are ...used; in most cases only the telescope would require a port. At our center, the VATS services were started in 2008, initially using the standard three-ports technique but shortly after this was replaced with PLT for all neonatal and pediatric VATS. The rationale of doing PLT was so that working instruments could move easier in the pediatric thoracic cavity. Furthermore, budget constraints did not allow us to purchase trocars of different sizes. Patients and methods: A review of all PLT cases was carried out at our institution from January 2008 to September 2018. We documented the diagnosis and type of surgery performed, age at surgery, number of ports used, conversion rate, morbidity and mortality as well as gross chest wall growth. Results: A total of 46 PLT cases were carried out; 16 were in neonates (34.7%). Conversion to thoracotomy occurred in five patients (10.8%). Diagnosis ranged from congenital anomalies like esophageal atresia to infective cause like empyema thoracis. Immediate morbidity occurred in four patients (8.7%) and there was one perioperative mortality (2.2%). The majority of PLTs (54%) were using two ports, and another 46% of PLTs were successfully carried out using one port. All neonatal and infant PLT cases were using one port (46%). Maximum follow-up was for 5 years and gross growth of chest wall was good. Conclusion: PLT is a feasible and safe technique for a variety of cases for neonatal and pediatric surgical intrathoracic pathology. We recommend PLT for all neonatal and pediatric VATS. Keywords: pediatric, thoracoscopy, VATS, port-less
A retrospective review of clinical manifestations and demographic pattern of patients diagnosed as chronic granulomatous disease (CGD) from 7 hospitals in Malaysia. An analysis of the available ...database would establish clinical characteristics, diagnoses and outcome including microbiologic pattern. Studying the demography allows us to document the occurrence of CGD amongst multiethnic groups and its geographical distribution for Malaysia.
Data from the Malaysia Primary Immunodeficiency Network (MyPIN) with cases of CGD diagnosed from 1991 until 2016 were collated and analysed.
Twenty patients were diagnosed as CGD. Males (N = 13, 65%) outnumber females (N = 7, 35%). CGD is commonest amongst the Malays (65%) followed by the Chinese (15.0%), Indians (10.0%) and natives of Borneo (10.0%), reflecting the ethnic composition of the country. The mean age of diagnosis was 3.7 years. There was a positive family history in 40% of the cases. Abscess was the main presenting feature in 16 patients (80%) with one involving the brain. Pneumonia occurred in 10 (50%) and one with complicated bronchiectasis. Catalase-positive bacteria were the most commonly isolated pathogen with Chromobacterium violaceum predominating (N = 5, 25%) with consequent high mortality (N = 4, 80%). All CGD patients with C. violaceum infection displayed CD4 + (T helper cells) lymphopenia.
This study has shown CGD occurs in the major ethnic groups of Malaysia. To the best of our knowledge, this is the first and the largest series of chronic granulomatous disease in South East Asia which may be reflective of similar clinical pattern in the region. C. violaceum infection is associated with a higher mortality in CGD patients in Malaysia. All the CGD patients with C. violaceum infection in this patient series displayed CD4 + (T helper) lymphopenia. We recorded rare clinical manifestation of CGD viz. brain abscess and bronchiectasis.
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IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
Parapneumonic effusions (PPE) is a complication of bacterial pneumonia. Factors that contribute to complicated PPE remain uncertain. This study was aimed to describe the characteristics, clinical ...predictors and outcome of children with parapneumonic effusion (PPE) in Universiti Kebangsaan Malaysia Medical Centre (UKMMC). A retrospective study on children with PPE who were admitted to UKMMC between January 2010 to December 2017 was conducted. Patients were categorised into 2 groups: simple and complicated PPE. Of 45 patients recruited, 20 (44.4%) patients had simple PPE and 25 (55.6%) had complicated PPE. Their median age was 32 months (IQR 16-63). The most common isolated organism was Streptococcus pneumoniae (61.9%), followed by Mycoplasma pneumoniae (19.0%) and Staphylococcus aureus (4.8%). Only 11.1% of patients received pneumococcal vaccination. There was no statistical significant difference in clinical features between simple and complicated PPE. Only 62.2% patients had ultrasound thorax done. Majority (95.0%) of patients with simple PPE were successfully treated with intravenous antibiotics alone. Sixty percent of patients with complicated PPE needed surgical intervention. Mean length of hospital stay for simple PPE was 10 (4.0) days and complicated PPE was 28 (16.5) days. In conclusion, clinical features could not predict complicated PPE. Use of ultrasound thorax as the main investigation tool for diagnosis and staging should be emphasised. Antibiotics therapy alone is effective therapy for simple PPE, while in complicated PPE, larger prospective studies are required to investigate which children benefit significantly from more intensive intervention.
Uncontrolled asthma may cause an increase in healthcare utilisation, hospital admission and productivity loss. With the increasing burden of asthma in Malaysia, strategies aimed at reducing cost of ...care should be explored.
This study aims to determine if a clinical pathway (CPW) for inpatient paediatric asthma would reduce average length of stay (ALOS), improve asthma management and decrease cost.
A quasi-experimental, pre-post study was used to evaluate the CPW effectiveness. Paediatric inpatients aged 5-18 years old, admitted for acute asthma exacerbation from September 2015 to April 2016 were prospectively recruited. Data from patients admitted from January-July 2015 were used as control. CPW training was carried out in August 2015 using standardised modules. Direct admission cost from the provider's prospective was calculated. Outcomes compared were differences in ALOS, discharge medication, readmission within 28 days of discharge and cost.
ALOS is 26 hours lower in the CPW group for severe exacerbations and underlying uncontrolled asthma (19.2 hours) which is clinically significant as patients have shorter hospital stay. More newly-diagnosed intermittent asthmatics were discharged with relievers in the CPW group (p-value 0.006). None of the patients in the CPW group had readmissions (p-value 0.16). Mean treatment cost for patients in the intervention group is higher at RM843.39 (SD ±48.99, versus RM779.21 SD±44.33).
This study found that management using a CPW may benefit asthmatic patients with uncontrolled asthma admitted with severe exacerbation. Further studies will be needed to explore CPW's impact on asthma management starting from the emergency department.
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