Case report: rare skeletal manifestations in a child with primary hyperparathyroidism.

BACKGROUND: Primary hyperparathyroidism (PHPT) is uncommon among children with an incidence of 1:300,000. This diagnosis is often missed in children in contrast to adults where it is detected at a pre symptomatic stage due to routine blood investigations. Etiology of PHPT can be due to adenoma, hyperplasia or rarely carcinoma. CASE PRESENTATION: A 12 year old Sri Lankan girl presented with progressive difficulty in walking since 1 year. On examination she had bilateral genu valgum. Skeletal survey revealed valgus deformity of knee joints, bilateral subluxation of upper femoral epiphysis(SUFE), epiphyseal displacement of bilateral humeri, rugger jersey spine and subperiosteal bone resorptions in lateral aspects of 2nd and 3rd middle phalanges. There were no radiological manifestations of rickets. Metabolic profile revealed hypercalcemia with hypophosphatemia. Intact parathyroid hormone levels were elevated at 790 pg/ml. Vitamin D levels were deficient. She had low bone mineral density with Z score of -3.4. Vitamin D supplementation resulted in worsening of hypercalcemia without reduction in PTH levels. Tc 99 Sestamibi uptake scan showed abnormal tracer retention in left inferior pole of thyroid. A large parathyroid gland was removed with histology favoring parathyroid adenoma. Post operatively she developed hypocalcemia. Bilateral osteotomy was done for SUFE and further surgeries for correction of limb deformities planned. CONCLUSION: PHPT in children is usually diagnosed late when irreversible organ damage has occurred. Children can present with non specific symptoms involving gastrointestinal, musculoskeletal, renal and neurological systems. PHPT can cause disarray in bone and epiphysis in children during pubertal growth spurt. Genu valgum and SUFE are rare skeletal manifestations in PHPT and only 10 cases of genu valgum and 9 cases of SUFE have been reported up to now. So far no cases have been reported on epiphyseal displacement of humeri. Awareness regarding the occurrence of these rare skeletal manifestations especially during puberty is important for early diagnosis to prevent irreversible outcomes.

IAEA survey of paediatric computed tomography practice in 40 countries in Asia, Europe, Latin America and Africa: procedures and protocols.

OBJECTIVE: To survey procedures and protocols in paediatric computed tomography (CT) in 40 less resourced countries. METHODS: Under a project of the International Atomic Energy Agency, 146 CT facilities in 40 countries of Africa, Asia, Europe and Latin America responded to an electronic survey of CT technology, exposure parameters, CT protocols and doses. RESULTS: Modern MDCT systems are available in 77 % of the facilities surveyed with dedicated paediatric CT protocols available in 94 %. However, protocols for some age groups were unavailable in around 50 % of the facilities surveyed. Indication-based protocols were used in 57 % of facilities. Estimates of radiation dose using CTDI or DLP from standard CT protocols demonstrated wide variation up to a factor of 100. CTDI(vol) values for the head and chest were between two and five times those for an adult at some sites. Sedation and use of shielding were frequently reported; immobilisation was not. Records of exposure factors were kept at 49 % of sites. CONCLUSION: There is significant potential for improvement in CT practice and protocol use for children in less resourced countries. Dose estimates for young children varied widely. This survey provides critical baseline data for ongoing quality improvement efforts by the IAEA.

Spontaneous Subcutaneous Emphysema in a Teenage Male Extending As Pneumomediastinum, Pneumothorax, Pneumopericardium, and Epidural Pneumatosis: A Rare Combination of Anatomical Locations.

Subcutaneous emphysema (SE) and pneumomediastinum can be spontaneous or traumatic in origin. Spontaneous SE involving cervical, parapharyngeal, mediastinal, pericardial, and pleural space together is rare, while epidural pneumatosis is an even rarer entity. We report a previously healthy teenage male with sudden onset chest pain whose plain radiographs and high-resolution computed tomography (HRCT) showed extensive spread of air in the mediastinum, pericardial space, pleural space, and epidural space. He was hemodynamically stable and had a spontaneous recovery after one week. Follow-up radiological imaging showed complete radiological resolution of gas lucencies. It is quite important for clinicians to be aware of this condition, common and rare routes of extension, and possible complications. Clinical suspicion is vital to plan appropriate investigations especially radiological modalities such as chest X-ray and HRCT. This will help in evaluating the severity of the condition, exclude possible etiologies, and look for potential complications so that proper management and follow-up can be planned.

IAEA survey of pediatric CT practice in 40 countries in Asia, Europe, Latin America, and Africa: Part 1, frequency and appropriateness.

OBJECTIVE: The purpose of this study was to assess the frequency of pediatric CT in 40 less-resourced countries and to determine the level of appropriateness in CT use. MATERIALS AND METHODS: Data on the increase in the number of CT examinations during 2007 and 2009 and appropriate use of CT examinations were collected, using standard forms, from 146 CT facilities at 126 hospitals. RESULTS: The lowest frequency of pediatric CT examinations in 2009 was in European facilities (4.3%), and frequencies in Asia (12.2%) and Africa (7.8%) were twice as high. Head CT is the most common CT examination in children, amounting to nearly 75% of all pediatric CT examinations. Although regulations in many countries assign radiologists with the main responsibility of deciding whether a radiologic examination should be performed, in fact, radiologists alone were responsible for only 6.3% of situations. Written referral guidelines for imaging were not available in almost one half of the CT facilities. Appropriateness criteria for CT examinations in children did not always follow guidelines set by agencies, in particular, for patients with accidental head trauma, infants with congenital torticollis, children with possible ventriculoperitoneal shunt malfunction, and young children (< 5 years old) with acute sinusitis. In about one third of situations, nonavailability of previous images and records on previously received patient doses have the potential to lead to unnecessary examinations and radiation doses. CONCLUSION: With increasing use of CT in children and a lack of use of appropriateness criteria, there is a strong need to implement guidelines to avoid unnecessary radiation doses to children.

Fibrolipomatous Hamartoma Arising from the Median Nerve-A Case Report.

Fibrolipomatous hamartoma is a rare nonhereditary, congenital condition characterized by benign hyperplasia of fibroadipose tissue around nerve bundles. The median nerve is commonly affected. Underlying pathological process is mature adipose and fibrous tissue infiltrating the epineural and perineural compartments giving typical "pseudo-onion bulb" appearance on histology and "coaxial cable" appearance on magnetic resonance imaging (MRI). This case of fibrolipomatous hamartoma well illustrates its characteristic clinical, radiological, and histopathological features. Patient is a teenage female presented with painless gradually enlarging mobile lump in the central left palm. Ultrasound scan revealed a hyperechoic subcutaneous lesion, with intervening fine hypoechoic cable like structures following the course of median nerve which was thickened. Computed tomography (CT) confirmed thickened left median nerve with radiating isodense fascicles surrounded by fatty areas. There were no calcifications. MRI showed classic "coaxial cable" like T1, T2 isointense fascicles continuous with the median nerve surrounded by a fusiform lesion demonstrating fat signals on spin-echo sequences which dropped on fat-suppressed gradient echo sequences. Diagnosis of fibrolipomatous hamartoma arising from left median nerve was made, based on typical imaging findings. Biopsy confirmed fibrolipomatous hamartoma. Diagnosis can be made confidently on imaging alone especially with MRI, without the need of biopsy. Both CT and MRI demonstrate fatty mass encasing the thickened nerve fibers. On MRI, characteristic appearance is seen as T1 and T2 low intense tubular thickened neural bundles surrounded by high signal fatty tissue.

Radiation protection of patients in diagnostic and interventional radiology in Asian countries: impact of an IAEA project.

Recognizing the lack of information on image quality and patient doses in most countries in Asia, the International Atomic Energy Agency (IAEA) initiated a project to assess the status of imaging technology, practice in conventional radiography, mammography, computed tomography (CT) and interventional procedures, and to implement optimisation actions. A total of 20 countries participated. Obsolete practices of use of fluoroscopy for positioning, photofluorography, chest fluoroscopy and conventional tomography were reported by 4 out of 7 countries that provided this information. Low-kV technique for chest radiography is in use in participating countries for 20-85% of cases, and manual processing is in 5-85% of facilities in 5 countries. Instances of the use of adult CT protocol for children in three participating countries were observed in 10-40% of hospitals surveyed. After implementation of a Quality Control programme, the image quality in conventional radiography improved by zero to 13 percentage points in certain countries and dose reduction was from 10% to 85%. In mammography, poor quality, ranging from 10 to 29% of images in different countries was observed. The project increased attention to dose quantities and dose levels in computed tomography, although doses in most cases were not higher than reference levels. In this study 16-19% of patients in interventional cardiology received doses that have potential for either stochastic risk or tissue reaction. This multi-national study is the first of its kind in the Asia, and it provided insight into the situation and opportunities for improvement.