The performance of ultrasound and upper gastrointestinal study in diagnosing malrotation in children, with or without volvulus.

BACKGROUND: Rapid diagnosis is crucial for pediatric patients with midgut volvulus and malrotation to prevent serious complications. While the upper gastrointestinal study (UGIS) is the traditional method, the use of ultrasound (US) is gaining prominence. OBJECTIVES: To assess the diagnostic sensitivity and specificity of US compared to UGIS for malrotation and midgut volvulus. METHODS: A cross-sectional study was performed on 68 pediatric patients who underwent US and/or UGIS before surgery for suspected midgut volvulus or malrotation in Kuala Lumpur (PPUKM and HTA), referencing surgical outcomes as the gold standard. RESULTS: US demonstrated a higher specificity (100%) than UGIS (83%) for diagnosing malrotation, with a slightly lower sensitivity (97% vs. 100%). For midgut volvulus, US surpassed UGIS in sensitivity (92.9% vs. 66.7%) while maintaining comparable specificity. The SMA/SMV criteria showed better sensitivity (91.1%) than the D3 assessment (78.9%) on US, though both had high specificity. CONCLUSION: US is equivalent to UGIS for identifying malrotation and is more sensitive for detecting midgut volvulus, supporting its use as a primary diagnostic tool. The study advocates for combined US and UGIS when either yields inconclusive results, optimizing diagnostic precision for these conditions.

A Survey of Awareness of Parents and Caretakers on Diagnostic Radiological Examination Related Radiation Exposure in a Tertiary Hospital in Malaysia.

BACKGROUND: With the advancement in medical imaging, radiological application in the paediatric population has also increased. Children, generally more radiosensitive, have a higher risk of developing certain malignancies. Therefore, this may result in uneasiness among parents and caretakers when their children need to undergo medical imaging examination. Hence, this study aims to assess the awareness of parents' and caretakers' awareness of medical imaging-related radiation exposure in our institution and their opinion of a medical radiation exposure-tracking programme for the paediatric population. METHODOLOGY: A cross-sectional survey was conducted for 6 months duration among parents and caretakers, who brought their children (under 12 years old) for imaging. The questionnaire booklet had eleven knowledge-based questions to assess respondents on ionising radiation-associated medical imaging, the radiation-related risk and radiation safety precaution. RESULTS: Two hundred and fifteen respondents participated in this survey. More than 40% of the respondents failed to identify various dose-saving and ionising radiation-related imaging methods. Only 87 participants (40.5%) could correctly answer at least six out of eleven knowledge-based questions. Moreover, 88.4% of the respondents support a medical radiation exposure-tracking programme for their children. CONCLUSION: Parents and caretakers who visited our institution had inadequate awareness of medical radiation exposure. Appropriate measures need to be taken to address this promptly. Implementation of a medical radiation exposure-tracking programme for the paediatric population is considered timely as most respondents agree with this programme.

A Rare Tumor in the Neck of a Child: Plexiform Neurofibroma.

Plexiform neurofibroma represents an uncommon variant of neurofibromatosis type 1, constituting only 5%-30% of all cases. Plexiform neurofibroma is usually diagnosed during childhood and arises from multiple nerves, manifesting as bulging and deforming masses that can also involve connective tissue and skin folds. We report a case of a two-year-old girl who presented with worsening stridor since birth and later exhibited progressively increasing left neck swelling at the age of 10 months old. Ultrasound and magnetic resonance imaging (MRI) showed a lobulated solid mass in the left deep neck space extending to the midline and having a mass effect on the airway with involvement of the supraglottic region. Tracheostomy was done, and a biopsy of the supraglottic lesion revealed a plexiform neurofibroma. The patient was conservatively managed after a discussion with her parents concerning the associated potential of operative morbidity. The patient's parents had learned about tracheostomy care, and the patient was scheduled for yearly MRI surveillance. MRI was performed again three months after the initial diagnosis and showed stable lesion. Plexiform neurofibroma is a slow-growing tumor. A treatment decision must consider the benefits of surgery and the morbidity of the progressing disease. Hence, airway management is crucial prior to the final decision of such cases.

Autoinflammatory diseases in childhood, part 1: monogenic syndromes.

Autoinflammatory diseases constitute a family of disorders defined by aberrant stimulation of inflammatory pathways without involving antigen-directed autoimmunity. They may be divided into monogenic and polygenic types. Monogenic autoinflammatory syndromes are those with identified genetic mutations, such as familial Mediterranean fever, tumor necrosis factor receptor-associated periodic fever syndrome (TRAPS), mevalonate kinase deficiency or hyperimmunoglobulin D syndrome, cryopyrin-associated periodic fever syndromes (CAPS), pyogenic arthritis pyoderma gangrenosum and acne (PAPA) syndrome, interleukin-10 and interleukin-10 receptor deficiencies, adenosine deaminase 2 deficiency and pediatric sarcoidosis. Those without an identified genetic mutation are known as polygenic and include systemic-onset juvenile idiopathic arthritis, idiopathic recurrent acute pericarditis, Behçet syndrome, chronic recurrent multifocal osteomyelitis and inflammatory bowel disease among others. Autoinflammatory disorders are defined by repeating episodes or persistent fever, rash, serositis, lymphadenopathy, arthritis and increased acute phase reactants, and thus may mimic infections clinically. Most monogenic autoinflammatory syndromes present in childhood. However, because of their infrequency, diverse and nonspecific presentation, and the relatively new genetic recognition, diagnosis is usually delayed. In this article, which is Part 1 of a two-part series, the authors update monogenic autoinflammatory diseases in children with special emphasis on imaging features that may help establish the correct diagnosis.

Computed Tomography Dose in Paediatric Care: Simple Dose Estimation Using Dose Length Product Conversion Coefficients.

BACKGROUND: The use of multislice computed tomography (MSCT) is increasing worldwide; at the same time, there is a growing awareness of the future risk of cancer associated with greater exposure to radiation. Therefore, there is a need for an accessible method of effective dose estimation. This study aims to estimate the effective doses (EDs) of a variety of paediatric computed tomography (CT) examinations in five age groups using recently published age- and region-specific dose length products (DLPs) as effective dose conversion coefficients. METHODS: A retrospective review was performed over a 12-month period. Patients were assigned to one of five age groups: neonatal, 1-, 5-, 10- and 15-years-old. Age- and region-specific conversion coefficients were applied to the DLP data displayed on the CT console in order to estimate the ED. RESULTS: Over the 12-month period, there were a total of 283 CT scans, 211 of which were selected for study. The ED estimates for plain CT brain scans in neonatal, 1-, 5-, 10- and 15-yearolds were 2.5, 1.5, 1.4, 1.3 and 0.8 mSv, respectively. For the corresponding CT abdominal scans, the results were 18.8, 12.9, 7.8, 8.6 and 7.5 mSv; these were the highest values recorded. High-resolution CT (HRCT) temporal scans showed EDs of 2.9, 1.8, 1.5 and 1.1 mSv in 1-, 5-, 10- and 15-years-old, respectively. CT scans of the helical thorax had an estimated ED of 4.8, 4.2 and 7.0 mSv in 5-, 10- and 15-years-old, respectively. CONCLUSION: An inverse relationship between age and effective dose was demonstrated in CT scans of the brain and abdomen/pelvis. In general, our study showed lower overall EDs compared to other centres.

Neonatal intestinal obstruction secondary to a floppy Meckel's diverticulum successfully treated by minimal access surgery.

Meckel's diverticulum has several known complications including diverticulitis and perforation. The presence of mesodiverticular band or a band from the diverticulum to the anterior abdominal wall is also described and can cause obstruction or rotation of the small bowel leading to volvulus. Meckel's diverticulum is also well known as the lead point for intussusception. It may be lined by ectopic gastric mucosa and can cause life-threatening gastrointestinal bleeding. We report a neonate who presented with acute intestinal obstruction secondary to a large, mobile Meckel's diverticulum which due to a direct compression effect on the adjacent small bowel caused mechanical intestinal obstruction. Diagnosis was confirmed at laparoscopy, and treated by curative surgical resection. This is the first report of a large mobile Meckel's diverticulum causing small bowel obstruction due to direct compression that was managed by minimally invasive surgical resection.

NOMID: the radiographic and MRI features and review of literature.

Neonatal onset multisystem inflammatory disease (NOMID) is a rare autoinflammatory disorder, which manifests early in infancy. We describe a case of a 10-year-old boy who has been unwell since infancy. He presented with urticarial rash, intermittent fever and hepatosplenomegaly followed by progressive arthropathy. His joint symptoms started at two years of age, which progressively involved multiple joints, resulting in bone and joint deformities. A series of joint radiographs demonstrated bizarre enlarging physeal mass with heterogenous calcification. Magnetic resonance imaging (MRI) of the involved right ankle and knee showed characteristic thickened and calcified physeal lesions, which enhanced post-gadolinium. This debilitating disease is also known to involve the central nervous system and eyes. This case report aims to highlight the conventional radiographic and magnetic resonance imaging (MRI) findings of this physeal abnormality in NOMID syndrome.

The value of pre-operative embolisation in primary inferior vena cava paraganglioma.

We report a case of a 13-year-old boy who complained of progressive abdominal distension and symptoms of anaemia. Radiological investigations revealed that the child had a hypervascular tumour of the inferior vena cava (IVC). Unfortunately, the child presented with acute lower gastrointestinal bleed soon after the investigation. He underwent an urgent pre-operative embolisation, aimed to reduce the tumour vascularity. A total resection of the tumour, right nephrectomy, and partial duodenal resection were done within 24 hours post-embolisation. The child was stable postoperatively. The histopathological examination revealed chromogranin-positive paraganglioma originating from the IVC. We highlight the radiological findings of rare primary IVC paraganglioma and the role of embolisation prior to surgical removal of the tumour.

Total pleurectomy as the surgical treatment for recurrent secondary spontaneous pneumothorax in a child with severe pulmonary Langerhans cells histiocytosis.

Pulmonary Langerhans cell histiocytosis (LCH) in children is more extensive and is a rare cause of spontaneous secondary pneumothorax (SSP) which tends to be recurrent and refractory to conventional treatment. Its occurrence in paediatric patients posed great challenge to the choice of surgical management. Surgery in the form of pleurodesis is only considered if SSP does not improve after chemotherapy and after considering all relevant risk and benefits of surgery to patients. Chemical pleurodesis will not give the expected effect to eradicate SSP in this patient. Therefore mechanical pleurodesis is the treatment of choice. There are various techniques to perform mechanical pleurodesis; from pleural abrasion to pleurectomy. In the authors' experience, bilateral total pleurectomy provided the best outcome for this 9-year-old patient with persistent respiratory distress from SSP due to extensive pulmonary LCH.

Bleeding small bowel cavernous haemangioma following blunt trauma to the abdomen presenting as subacute intestinal obstruction in a child.

The authors report a case of a 6-year-old girl who developed subacute intestinal obstruction after a trivial blunt trauma to her abdomen. Her normal vital signs masked the presence of intestinal bleeding. An incidental finding at surgery of a haematomatous polypoid vascular growth of the ileum was subsequently confirmed to be cavernous haemangioma of the small bowel. Surgical resection was curative in this patient.