Rick R. van Rijn

Is the Greulich and Pyle atlas still valid for Dutch Caucasian children today

Abstract.Background: In our Paediatric Radiology Department, the Greulich and Pyle technique is used to assess skeletal age. Several authors have raised questions with regard to the applicability of this technique in a contemporary paediatric and adolescent population. Objective: To compare skeletal age and calendar age in a healthy Dutch Caucasian population in order to test the applicability in this specific population. Materials and methods: For this study we enrolled 278 Dutch Caucasian boys (age range 5.0–19.5 years, mean 12.6 years) and 294 Dutch Caucasian girls (age range 5.2–19.9 years, mean 12.2 years). Radiographs of the left hand were scored according to the Greulich and Pyle atlas by two investigators. Results: Intra-observer coefficient of variation of duplicate assessment of skeletal age for investigator 1 (resident) was 2.4 % and for investigator 2 (radiologist) was 1.5 %. We found no significant systematic differences between the two observers regarding variability and levels of measurement, and the agreement was good. There was a strongly significant correlation between skeletal and calendar age rgirls = 0.974 and rboys = 0.979 (P < 0.001). On average, calendar age preceded skeletal age by a small amount (1.7 months in girls and 3.3 months in boys, both P < 0.001). Conclusions: The reliability of the Greulich and Pyle atlas in our study corresponds well with previously reported studies. Based on our data, we conclude that the Greulich and Pyle atlas is still applicable in Dutch Caucasian children and adolescents.

Skeletal imaging of child abuse (non-accidental injury)

In recent years there has been a worldwide increased awareness that children are physically abused by their carers. Radiologists play a vital role in the detection of inflicted injuries. This article reviews the skeletal imaging findings seen in child abuse.

Bone densitometry in children: a critical appraisal.

Abstract. Due to the introduction of new therapeutic regimen aimed at increasing and maintaining bone mass, bone densitometry in children has gained interest. As in all new fields of medicine we expect the interest in bone densitometry in children to increase in the coming years. Children pose a unique problem for those involved in the field of bone densitometry, because as time progresses the measured subject changes in shape and volume. It is therefore of importance that radiologists and clinicians gain insight in the available techniques. It is also important to keep in mind that all bone densitometry techniques have been exclusively designed, developed and validated for use in an adult population. In this article we give an overview of the available techniques and discuss the specific problems that can be expected when these techniques are used in children. In the discussion section general problems regarding bone densitometry in children are presented.

Bone mineral density assessed by phalangeal radiographic absorptiometry before and during long-term growth hormone treatment in girls with Turner's syndrome participating in a randomized dose-response study

To assess bone mineral density (BMD) in girls with Turners syndrome before and during long-term treatment with GH, longitudinal measurements using phalangeal radiographic absorptiometry were performed in 68 girls with Turners syndrome. These previously untreated girls, age 2–11 y, participating in a randomized, dose-response trial, were randomly assigned to one of three GH dosage groups: group A, 4 IU/m2/d (≈0.045 mg/kg/d); group B, first year 4 IU/m2/d, thereafter 6 IU/m2/d (≈0.0675 mg/kg/d); or group C, first year 4 IU/m2/d, second year 6 IU/m2/d, thereafter 8 IU/m2/d (≈0.090 mg/kg/d). In the first 4 y of GH treatment, no estrogens for pubertal induction were prescribed to the girls. Thereafter, girls started with 17β-estradiol (5 μg/kg body weight/d, orally) when they had reached the age of 12 y. BMD results were adjusted for bone age and sex, and expressed as SD scores using reference values of healthy Dutch girls. At baseline, almost every individual BMD value of bone consisting predominantly of cortical bone, as well as that of bone consisting predominantly of trabecular bone, was within the normal range of healthy girls and the SD scores were not significantly different from zero [mean (SE) 0.38 (0.22) and −0.04 (0.13)]. During 7 y of GH treatment, BMD SD scores showed a significant increase to values significantly higher than zero [mean (SE) 0.87 (0.15) and 0.95 (0.14)]. The increment in BMD SD score of bone consisting predominantly of cortical bone was significantly higher in group C compared with that of the other two GH dosage groups. The pretreatment bone age was significantly negatively related to the increment in BMD SD score. We found no significant influence of spontaneous puberty or the use of low-dose estrogens in the last 3 y of the study period on the increment in BMD SD score during 7 y of GH treatment. In conclusion, most untreated young girls with Turners syndrome have a normal volumetric BMD. During 7 y of GH treatment with 4, 6, or 8 IU/m2/d, the BMD SD score increased significantly.

Imaging findings in noncraniofacial childhood rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood. This paper is focuses on imaging for diagnosis, staging, and follow-up of noncraniofacial RMS.

Current techniques in postmortem imaging with specific attention to paediatric applications

In this review we discuss the decline of and current controversies regarding conventional autopsies and the use of postmortem radiology as an adjunct to and a possible alternative for the conventional autopsy. We will address the radiological techniques and applications for postmortem imaging in children.

Birth-related mid-posterior rib fractures in neonates: a report of three cases (and a possible fourth case) and a review of the literature

BackgroundPosterior rib fractures in young children have a high positive predictive value for non-accidental injury (NAI). Combined data of five studies on birth trauma (115,756 live births) showed no cases of rib fractures resulting from birth trauma. There have, however, been sporadic cases reported in the literature.ObjectiveWe present three neonates with both posterior rib fractures and ipsilateral clavicular fractures resulting from birth trauma. A review of the literature is also presented. The common denominator and a possible mechanical aetiology are discussed.Materials and methodsIn total, 13 cases of definitive birth-related posterior rib fractures were identified.ResultsNearly all (9/10) posterior rib fractures were (as far as reported in the original publications) in the midline. In 12 of the 13 children, birth weight was high and in 7 children birth was complicated by shoulder dystocia. An interesting finding was that in cases where a clavicular fracture was present, this was on the ipsilateral side.ConclusionRadiologists, when presented with a neonate with posterior rib fractures, should be aware of this rare differential diagnosis.

Management of tumors of the chest wall in childhood: a review.

Chest wall tumors in childhood are major challenges with respect to diagnostic workup and treatment. Incidence rate is less than 1 per 1,000,000 and various benign and malignant diseases are noted. From the malignant diseases, Ewing tumors and rhabdomyosarcoma tumors are most often seen. Depending on diagnosis, staging, and age, therapy has to be tailored for each patient, which should be discussed in a multidisciplinary team setting. Radical resection is in most cases the major component of treatment. Use of chemotherapy depends on the diagnosis. In soft-tissue tumors, previously considered to be chemotherapy insensitive, favorable results are currently reported. The role of radiotherapy is debated owing to its pronounced late sequelae in children, but should be added when adequate margins cannot be achieved by surgery.

The Use of Bone Age in Clinical Practice – Part 2

If height-limiting treatment is being considered for a child with tall stature, skeletal maturity is invaluable in the selection of appropriate patients for treatment, determining appropriate age of treatment commencement, monitoring progress of treatment, and determining the expected treatment effect on adult height. In precocious puberty, bone maturation can be usefully assessed at initial diagnosis and start of treatment and at regular intervals thereafter during treatment monitoring. Together with height, bone maturation is an essential parameter for long-term treatment monitoring in congenital adrenal hyperplasia. Bone age (BA) determination in children with skeletal dysplasia is only feasible in a few disorders and estimations should be treated with caution. Radiographs of the left hand and wrist are, however, essential in the diagnosis of many skeletal disorders. Bone mineralization and measures of bone lengths, width, thickness and cortical thickness should always be evaluated in relation to a child’s height and BA, especially around puberty. The use of skeletal maturity, assessed on a radiograph alone to estimate chronological age for immigration authorities or criminal courts is not recommended.

CRTAP mutations in lethal and severe osteogenesis imperfecta: the importance of combining biochemical and molecular genetic analysis

Autosomal recessive lethal and severe osteogenesis imperfecta (OI) is caused by the deficiency of cartilage-associated protein (CRTAP) and prolyl-3-hydroxylase 1 (P3H1) because of CRTAP and LEPRE1 mutations. We analyzed five families in which 10 individuals had a clinical diagnosis of lethal and severe OI with an overmodification of collagen type I on biochemical testing and without a mutation in the collagen type I genes. CRTAP mutations not described earlier were identified in the affected individuals. Although it seems that one important feature of autosomal recessive OI due to CRTAP mutations is the higher consistency of radiological features with OI type II-B/III, differentiation between autosomal dominant and autosomal recessive OI on the basis of clinical, radiological and biochemical investigations proves difficult in the affected individuals reported here. These observations confirm that once a clinical diagnosis of OI has been made in an affected individual, biochemical testing for overmodification of collagen type I should always be combined with molecular genetic analysis of the collagen type I genes. If no mutations in the collagen type I genes are found, additional molecular genetic analysis of the CRTAP and LEPRE1 genes should follow. This approach will allow proper identification of the genetic cause of lethal or severe OI, which is important in providing prenatal diagnosis, preimplantation genetic diagnosis and estimating recurrence risk.