Development of image quality related reference doses called acceptable quality doses (AQD) in paediatric CT exams in Qatar.

OBJECTIVES: To describe first experience of integrating assessment of image quality in paediatric X-ray computed tomography (CT) with analysis of the radiation dose indices to develop reference doses called acceptable quality dose (AQD). METHODS: Image quality was scored by the radiologists at a tertiary care hospital in Qatar on a scale of 0 to 4 using the recently published scoring criteria. The patients undergoing head, chest and abdomen CT were divided in different weight groups as follows: < 5 kg, 5-< 15 kg, 15-< 30 kg, 30-< 50 kg, 50-< 80 kg and > 80 kg. The images that were clinically acceptable (score of 3) were included for assessment of median values of CTDIvol and DLP to obtain AQDs in different weight groups. RESULTS: After initial training in image quality scoring of CT images of 49 patients by three radiologists, the study on 715 patients indicated 665 studies (93%) were clinically acceptable as per scoring criteria. The median CTDIvol values for the above weight groups were 16, 20, 22, 22, 27 and 27 mGy and the median DLP values for these weight groups were 271, 377, 463, 486, 568 and 570 mGy cm, respectively, for head CT. Similar values are presented for chest and abdomen CTs. CONCLUSIONS: The first ever experience of starting with image quality assessment and integrating it with analysis of dose indices to obtain AQD values shall provide a workable model for others and values for comparison within the facility and in other facilities leading to optimisation. KEY POINTS: • The first study to integrate image quality assessment with analysis of patient dose indices shows feasibility for routine practice in other centres. • The values of acceptable quality dose (AQD) were provided for head, chest and abdomen CT of children divided into weight groups rather than age. They shall act as reference values for future studies. • Verification of our findings on proportional increase in exposure parameters (CTDIvol and DLP) with weight by other investigators shall be helpful.

Distribution of sickle cell disease and assessment of risk factors based on transcranial Doppler values in the Gulf region.

Background/Objective: Stroke is a potentially fatal complication of sickle cell disease (SCD). Transcranial Doppler (TCD) is useful at identifying increased risk of stroke in children with SCD and vasospasm after subarachnoid hemorrhage. The main aim of this study was to determine the proportion of patients with SCD in the Gulf region who are at a high risk of stroke, as determined by TCD.Methods: This multicenter (Oman, Qatar, and UAE), descriptive, cross-sectional study in patients (aged 2-16 years) with SCD included a baseline visit, 1 follow-up visit for patients with conditional TCD, and 3-year retrospective data analysis for all patients.Results: Of the 410 eligible patients (Oman, 86.5%; Qatar, 8.2%; UAE, 5.1%), most had a TCD finding (left side, 91.7%; right side, 92.0%) of normal velocity (<155 cm/s) at baseline. For 6 of 7 patients with conditional velocity (155-179 cm/s) and 1 patient with high velocity (≥180 cm/s), baseline TCD results were not confirmed at follow-up. As per bivariate linear regression, age, race, transfusion type, and transfusion frequency were significant predictors of the TCD velocities. Multivariate logistic regressions revealed that TCD velocities were significantly correlated with sex, race, and type of transfusion. No patients reported any adverse events at follow-up. No deaths occurred during the study.Discussion/Conclusions: The study results show that far fewer patients with SCD in the Gulf have abnormal TCD findings than the internationally reported. Larger studies are needed to identify the factors underlying this observation.

Suspected testicular torsion in children: diagnostic dilemma and recommendation for a lower threshold for initiation of surgical exploration.

PURPOSE: Testicular torsion (TT) represents a clinical challenge that needs emergency surgical assessment. It is common to have negative scrotal exploration due to confounding symptoms and signs which makes it sometimes difficult to differentiate from similar surgical emergencies that do not warrant surgery. At the same time, several occasions of misdiagnoses or late interventions occur with devastating effects. We aim at delineating the significance of the different clinical, laboratory, and radiological variables in the detection of TT. METHODS: We retrospectively reviewed the charts of 52 patients who were surgically explored with a preoperative working diagnosis of suspected TT in our center over the period from 2011 to 2015. All the patients were examined by pediatric surgeons in the emergency room and had undergone ultrasound imaging of the testes. The ultrasound images were retrospectively reviewed by a pediatric radiologist who was blinded to the intraoperative findings. Univariate and multivariate and logistic regression analyses were performed. RESULTS: Of the studied group of patients, the majority (84.6%) had TT upon surgical exploration. The most frequently presented symptom was pain (80.8%), and only a minority (11.5%) presented with vomiting. Radiological findings with the highest sensitivity were heterogeneous echogenicity in favor of TT and enlarged epididymis indicating that TT is unlikely. However, the predictability of TT by any of the assessed clinical and imaging factors was statistically insignificant. CONCLUSION: It is important to gather all relevant data from clinical, laboratory, and imaging sources when assessing pediatric patients with suspected TT given the inaccuracy of each single one of them if used alone. Keeping this in mind, Doppler ultrasound has a significant role to aid in the accuracy of the diagnosis and hence the appropriate decision-making thereafter. However, we found no single clinical or imaging sign that is sensitive enough to prove or rule out TT. Therefore, surgical exploration should take place in a timely manner. Moreover, further research is necessary to construct scoring systems where different predictors collectively have higher reliability.

Clinical and genetic association, radiological findings and response to biological therapy in seven children from Qatar with non-bacterial osteomyelitis.

AIM: We aim to report the clinical manifestations, genetic testing results, magnetic resonance imaging (MRI) findings and biologics used in the management of non-bacterial osteomyelitis in our center. METHODS: We conducted a retrospective review of medical records. A previously proposed classification was implemented as follows: chronic recurrent multifocal osteomyelitis (CRMO), chronic non-bacterial osteomyelitis (CNBO) and acute non-bacterial osteomyelitis. RESULTS: Four females and three males with a median age at presentation of 6 years (6 months-14 years) presented with arthralgia (7/7), back pain (4/7), arthritis (4/7) and bone pain (2/7). Six patients had CRMO and one patient had CNBO. Genetic testing revealed an apparent homozygote p.S734L LPIN2 mutation in two siblings, a heterozygote p.M694V MEFV mutation in one patient with familial Mediterranean fever and heterozygote p.Q219H PSTPIPI variant of unknown significance in one patient. The most common lesions on MRI involved the tibia (6/7), talar bones (5/7), fibula (4/7) and sacroiliac joints (4/7). Three patients received infliximab. Two are in remission after 2 and 5 years, and the third was advanced after 5 years to canakinumab. Two other patients received canakinumab first. One patient with Majeed syndrome and dyserythropoietic anemia exhibited evidence of improvement, and one had partial improvement and was then treated with infliximab. CONCLUSION: Non-bacterial osteomyelitis may coexist with other autoinflammatory diseases. MRI remains a favorable diagnostic tool and genetic testing may have a limited role in selected cases. Infliximab and canakinumab are associated with variable outcomes, and 6-week or less dosing intervals for both medications may be more effective.

Rarity in conspicuity-Ultrasound diagnosis of sliding left inguinal hernia through canal of Nuck with uterus, fallopian tubes and ovaries.

We present a case of a one month old female infant who presented with left inguinal swelling. She was prematurely born at 32 weeks of gestational age. Preoperative ultrasound showed features of sliding indirect inguinal hernia with both ovaries, fallopian tubes and uterus; which were also evident per operatively. Patient underwent successful hernia repair and uneventful post-operative period. Patient is on follow up currently. In conclusion, we attempt to highlight the exiguous prevalence of inguinal hernia with uterus, fallopian tubes and ovaries; which has been sparsely reported in the literature and also the importance of preoperative of sonography.

Unexplained cyanosis revealing hepatopulmonary syndrome in a child with asymptomatic congenital hepatic fibrosis: a case report.

INTRODUCTION: Hepatopulmonary syndrome is a rare disease that affects patients of any age with acute or chronic liver disease. Liver transplantation is the only therapeutic option of proved benefit, and can result in substantial improvement or total improvement in postoperative gas exchange abnormalities. CASE PRESENTATION: We report the case of a cyanotic 13-year-old Pakistani boy whose chest computed tomography scan showed normal lung fields and mediastinum with incidental findings of a prominent liver surface with a collateral vein connecting a portal cavernoma to the dilated terminal inferior vena cava. Sonography of his abdomen along with a portal venous Doppler study showed multiple collateral veins replacing the portal vein. A liver biopsy revealed congenital hepatic fibrosis. Contrast-enhanced echocardiography with agitated saline and a 99m Technetium-macroaggregated albumin perfusion lung scan confirmed intrapulmonary shunting. The patient underwent a successful liver transplantation that resulted in improved gas exchange. CONCLUSIONS: Hepatopulmonary syndrome should be included in the differential diagnosis of unexplained hypoxemia with an evaluation of possible portal hypertension or liver disease even in the absence of other clinical symptoms.