Sonographic evaluation of normal diaphragmatic thickness and excursion in Malaysia paediatric population: A single-institution cross-sectional study.

INTRODUCTION: Diaphragmatic dysfunction is often underdiagnosed as clinical presentation is non-specific and reference values for normal diaphragmatic excursion are inadequate. The rationale of this study is to provide a normal reference value of diaphragmatic excursion and thickness in Malaysia's paediatric population using M-mode sonography, as no previous local data are available to our knowledge. MATERIALS AND METHODS: A total of 119 healthy infants and children fulfilling our inclusion and exclusion criteria were recruited. They were divided into three groups according to age - 0-2 years old in group 1; 2-6 years old in group 2; 6- 12 years old in group 3. Sonography B-mode was used to assess bilateral diaphragmatic thickness and M-mode to assess diaphragmatic excursion during quiet spontaneous respiration. RESULTS: In our paediatric population, the normal right and left diaphragmatic thickness were 2.0 mm ± 0.5 and 2.0 mm ± 0.5 for group 1; 2.5 mm ± 0.8 and 2.4 mm ± 0.6 for group 2; 2.7 mm ± 0.7 and 2.5 mm ± 0.5 for group 3, respectively. The normal right and left diaphragmatic excursion were 7.7 mm ± 2.5 and 7.3 mm ± 2.6 for group 1; 11.5 mm ± 3.8 and 10.6 mm ± 3.8 for group 2; 13.8 mm ± 3.9 and 12.9 mm ± 3.3 for group 3, respectively (data presented in mean ± standard deviation). There were no significant differences between two genders for each group. Significant positive correlation between age, weight, height, and body surface area with bilateral diaphragmatic thickness and excursion were detected in all studied population. The percentage difference between excursions of both hemidiaphragm was below 40%. CONCLUSIONS: M-mode sonography is the modality of choice for diaphragmatic kinetics especially in paediatric population. This study provides normal sonographic reference value of diaphragmatic excursion and thickness in the Malaysian paediatric population as well as percentile curves for right diaphragmatic excursion plotted against body weight. The availability of this data will aid in the diagnosis of diaphragmatic dysfunction and hence immediate intervention for better recovery.

Clinical and laboratory observation on immunoglobulin replacement therapy switching from an intravenous to a subcutaneous route in a Malaysian X-linked agammaglobulinemia patient.

We report a clinical and laboratory observation in a boy with X-linked agammaglobulinemia (XLA) who underwent an immunoglobulin replacement therapy (IRT) via the subcutaneous route (IGSC) seven years after his IRT via intravenous route (IGIV). He was free of invasive infections when on IGIV but not the troublesome coughs a week before the next infusion. A switch to a subcutaneous route resulted in significant improvement of symptoms with good weight gain. When on 2-weekly IGSC cycle, adjusting dose for weight resulted in an IgG trough level of > 600 mg/dl.

Sonographic nomogram of paediatric renal size in Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM).

INTRODUCTION: A person's childhood is an important period of growth, and also one's most vulnerable, as one can be exposed to various pathologies, for example those that could affect the growth of one's kidney. Asians are physiologically different from Caucasians, and the nomogram renal size obtained from a Western population (mostly of Caucasians) is not be suitable for representing Asian children. As such a nomogram on paediatric renal size derived from Malaysia is needed. METHODS: A total of 109 (64 males and 45 females) aged 0-12 in Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM) took part in this study. They underwent ultrasonography of both kidneys, and their demographic and anthropometric data were collected. The mean and standard deviations of the renal length and renal volume according to their age groups was calculated, and the final data was compared to the ones reported by Rosenbaum et al. (1984). RESULT: Body weight and Body Surface Area (BSA) of the children reported the strongest correlation with renal size. Significant differences were found between local and the data from Rosenbaum et al (1984). A nomogram on paediatric renal size based on children in PPUKM was then created. DISCUSSION: Ultrasonography is regarded as the standard method for determining renal size. Body weight and BSA were both strongly correlated with renal size. It was shown that the widely used nomograms derived from data obtained from Caucasian was not suitable to represent the population of Malaysian children.

Diagnostic challenge of Burkitt's lymphoma at early age.

A four-year-old Ibanese boy presented with subacute abdominal distension for two months duration. Ultrasound and computed tomography (CT) scan showed solid liver masses as well as bowel and intraperitoneal lesions. Initial diagnosis of intraperitoneal inflammatory process as in tuberculosis with non-liquefied liver abscess with differential diagnosis of neoplastic process was made. Liver biopsy and peritoneal fluid analysis revealed Burkitt's lymphoma (BL). We aim to highlight the diagnostic challenge of BL in this young age group emphasizing on the ultrasound and CT features of intraabdominal BL. We would also want to stress the importance of early diagnosis of BL as it is known to be the most aggressive tumour within 24 hours yet to have good survival if early diagnosis was made.

Association of Cobb angle progression and neuraxial abnormality on MRI in asymptomatic Adolescent Idiopathic Scoliosis.

BACKGROUND: Detection of neuraxial abnormality in neurologically asymptomatic adolescent idiopathic scoliosis (AIS) is crucial prior to surgery. It can only be detected on magnetic resonance imaging (MRI), which was not routinely done in this group of patient. On the other hand, whole spine radiographs for measurement of Cobb angle have been routinely included during clinic follow-up. This study aimed to determine the correlation between Cobb angle progression and neuraxial abnormality finding on MRI in asymptomatic AIS. METHODS: A retrospective study was conducted in the Orthopaedic department of a tertiary hospital. Patients with asymptomatic AIS aged 10-20 years who attended scoliosis clinic from year 2007 to 2010 was reviewed. Patients who had whole spine MRI and two vertebral radiographs at least one year apart were further selected. Statistical analysis was done to see the association between Cobb angle progression and neuraxial abnormality on MRI. RESULTS: The mean age at first presentation was 14.4 years old. Female (n=249) to male (n=50) ratio was 5:1. Only 19 patients fulfilled the selection criteria. There were 5 patients (26.3%) who had neuraxial abnormalities. The mean curve progression was 7.05° (range from -5° to 28°). Patients with and without neuroaxial abnormality showed mean curve progression of 0.6° and 9.36° respectively. There was no significant association between Cobb angle progression and neuroaxial abnormality (p=1.000). CONCLUSION: Cobb angle progression is not a reliable indicator for predicting neuroaxial abnormality in patients with asymptomatic AIS. However, this study stressed the need to perform MRI prior to operation to document any associated neuraxial abnormality in clinically asymptomatic AIS patients.

Contrast enhanced Voiding Urosonography (ce-VUS) as a radiation-free technique in the diagnosis of vesicoureteric reflux: Our early experience.

OBJECTIVE: Contrast-enhanced ultrasound has become increasingly utilised as an alternative imaging modality for the diagnosis of vesicoureteric reflux (VUR) in paediatric patients. The study objective is to evaluate the efficacy of contrast enhanced Voiding Urosonography (ce-VUS) compared with fluoroscopic micturating cystourethrography (MCU) in the detection of VUR. METHODS: This prospective study was carried out between July 2011 and January 2013 on paediatric patients who underwent MCU. All consented patients would undergo ce- VUS prior to MCU. We documented the epidemiology details, the number of Kidney-Ureter (K-U) unit studied, baseline renal and bladder sonogram, as well as presence of VUR on ce-VUR. The technique for ce-VUS was standardized using normal saline to fill the bladder prior to administration of SonoVue® (2.5 ml) to assess the kidney-ureter (K-U) unit. Dedicated contrast detection software was used to discern the presence of microbubbles in the pelvicaliceal system (PCS). The findings were then compared with MCU. RESULTS: 27 paediatric patients were involved in the study [17 males (63%) and 10 females (37%)] involving 55 K-U units (one patient had a complete duplex system). MCU detected VUR in 10 K-U units while ce-VUS detected VUR in 8 out of the 10 K-U units. There were 2 false negative cases (both Grade 1) with ce-VUS. The sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of ce-VUS were 80%, 98%, 95%, 89% and 96%, respectively. CONCLUSION: ce-VUS is a sensitive and specific radiation-free alternative for the detection of VUR in the paediatric population.

Renal doppler assessment in differentiating obstructive from non-obstructive hydronephrosis in children.

BACKGROUND: to determine the usefulness of Doppler ultrasound measurement of resistive index (RI) in differentiating obstructive from non-obstructive hydronephrosis in children. METHODS: From August 2011 to November 2012, renal Doppler assessments of the intra-renal renal arteries were performed on 16 children (19 kidneys) with congenital hydronephrosis. the independent t-test was used to assess for significant difference in RI values between those with obstructive hydronephrosis (6 kidneys) and those with non-obstructive hydronephrosis (13 kidneys) as determined by dynamic renal scintigraphy. the assessor was blinded to the clinical findings and scintigraphy results. RESULTS: RI was significantly different between obstructive and non-obstructive hydronephrosis. Obstructive hydronephrosis returned higher RI values, with mean RI of 0.78. Mean RI in non-obstructive hydronephrosis was 0.70, and the difference was significant (p <0.05). the sensitivity and specificity of Doppler ultrasound were 100% and 53% respectively. CONCLUSION: Doppler ultrasound measurement of resistive index is useful in differentiating obstructive from nonobstructive hydronephrosis and provides an alternative non-ionizing investigation.

Multimodality imaging in the assessment of thoraco-omphalopagus conjoined twin: lessons to learn.

Conjoined twins are rare and present a unique challenge to pediatric surgeons and radiologists. An imaging strategy to accurately define anatomic fusion, vascular anomalies, and other associated abnormalities is important for surgical planning and prognostic information. A conjoined female twin with a combined weight of 2.8 kg was born by emergency caeserean. Hence, a computed tomography scan of the thorax and entire abdomen at 1.25 mm slice thickness was performed to delineate the internal structures of the twins. CT-angiography defined specific vascular supply which determined the distribution of shared structures between the twins. An echocardiogram showed four heart chambers with atrioventricular septal defect. To further evaluate the heart chambers, the twin was planned for gated cardiac magnetic resonance imaging. Unfortunately, they succumbed 6 hours apart due to complication of septicemia. Magnetic resonance imaging and CT scan provide excellent anatomic detail, demonstrating organ position, shared viscera, and limited vascular anatomy, whilst angiography defined specific vascular supply, useful in determining the distribution of shared structures between the twins in planning for surgery.