Alan Sprigg

Unexplained fractures in infancy: looking for fragile bones

A fracture occurs when the force exerted on a bone exceeds the ability of the bone to absorb the force by deforming. Fractures in children are common—approximately one third of children will have a fracture by 16 years of age, with more boys experiencing fracture than girls.1 This differentiation in fracture risk is apparent from 2 years of age. Before the age of 2 years, fracture incidence is equal and occurs at a rate of approximately 80/10 000 person years. For the UK, therefore, approximately 4800 infants will have a clinically evident fracture before their first birthday each year.nnSome long-bone fractures may occur at birth2 in association with events such as shoulder dystocia3; skull fractures may occur during forceps4 and ventouse delivery.5 Some may (uncommonly) occur as a result of clearly defined trauma such as road accidents.6 Most, however, fall into the “unexplained” category. This article reviews our current approach to identifying bone disease in the infant presenting with more than one unexplained fractures, and discusses the recognised disease processes that result in increased bone fragility.nnThe history should include inquiry into specific areas as listed in the box. The two most frequently recognised underlying disease processes causing bone fragility in infancy are metabolic bone disease of prematurity7 and osteogenesis imperfecta, and directed questioning is appropriate for these conditions. For premature infants, the features commonly associated with fracture are delivery at 30 days) establishment of full enteral feeds, conjugated hyperbilirubinaemia, chronic lung disease, and use of furosemide.8,9 For a proportion of infants with osteogenesis imperfecta, there will be a family history either of osteogenesis imperfecta itself or of features that suggest osteogenesis imperfecta. The other elements of the history relating to the …

Endoscopy-guided balloon dilatation of esophageal strictures and anastomotic strictures after esophageal replacement in children☆

This study evaluates the safety, efficacy, and technical problems of the new technique of endoscopy-guided balloon dilation (EGBD) in the treatment of strictures of the esophagus and its replacement. Between 1986 and 1990, the authors treated 33 children (aged 3 weeks to 20 years) with EGBD; 18 had esophageal strictures (primary esophageal atresia repair, 13; reflux esophagitis, 5), 13 had anastomotic strictures after esophageal replacement (colon, 12; stomach, 1), and 2 had caustic strictures. The majority (23 of 33) had previously failed to respond to conventional bouginage (mean, 11.2 sessions; range, 1 to 32 sessions). EGBD was performed using flexible endoscopy and flouroscopic screening under general anesthesia. Endoscopy identified and resolved the errors or uncertainties of preoperative contrast studies in 7 patients, 5 of whom had colon interposition. EGBD was achieved in all 31 patients with esophageal or replacement strictures; the mean number of EGDB procedures per patient was 2.1 (range 1 to 7). Symptomatic relief was excellent in 24 and moderate in 7 patients. Both patients with caustic strictures had esophageal perforation from EGBD (excessive inflation, 1; false passage of guide wire, 1). Patients who had experienced both conventional bouginage and EGBD noticed less pain with EGBD and resumed eating sooner. The authors conclude that EGBD is safe and effective for treating esophageal and replacement strictures but not caustic strictures.

A randomized, controlled dose‐ranging study of risedronate in children with moderate and severe osteogenesis imperfecta

Moderate to severe osteogenesis imperfecta is associated with multiple fractures in childhood. There are no published data regarding the effects of third‐generation bisphosphonates in these children. This randomized study investigated which of three different doses of risedronate was most effective in reducing fracture incidence. We randomly assigned 53 children with moderate to severe osteogenesis imperfecta to receive 0.2, 1, or 2 mg/kg per week of risedronate. We assessed safety, fracture incidence, and bone measurement outcomes at 3, 6, 12, 18, and 24 months. At 24 months, 69% of children assigned 0.2 mg/kg per week had had new fractures compared with 44% receiving 1 mg/kg per week and 75% receiving 2 mg/kg per week. Poisson regression with age and prior fracture as covariates showed that there was no difference in incident nonvertebral fracture between groups. Fracture rate diminished in each group during the trial compared with previous the 2 years (pu2009=u2009.005). Lumbar spine bone mineral density increased significantly (pu2009=u2009.009) only in the 2 mg/kg per week group. Long bone bowing deformities reduced more in children receiving 1 or 2 mg/kg per week of risedronate [odds ratio (OR) 0.67, 95% confidence interval (CI) 0.48–0.93 per unit increase in risedronate dose, pu2009=u2009.015]. There were no serious adverse events. Bone mass increased and bowing deformities reduced with increasing risedronate dose. Children suffered fewer fractures irrespective of risedronate dose. The most appropriate dose of risedronate for children with moderate to severe osteogenesis imperfecta in this study was 2 mg/kg per week.

Inflammatory myofibroblastic tumor of the pancreas: a case report of 2 pediatric cases—steroids or surgery?

Inflammatory pseudotumors also termed inflammatory myofibroblastic tumors (IMTs) are rare, benign, solid lesions of unclear etiology more usually found in the lung and very rarely in the pancreas. We report 2 cases and outline our management for each. The first case was treated surgically, whereas the second was treated with high-dose steroids. This represents the first reported case whereby steroid treatment has been successful in pancreatic IMT.

Reduction of intussusception: defining a better index of successful non-operative treatment

The reported non-operative reduction rate for intussusception is usually the proportion of attempted non-operative (radiological) reductions that succeed, which we term the “selective reduction rate.” This value shows wide variation that may result from selection bias that is difficult to quantify because data regarding primary operative treatment are frequently lacking. The proportion of patients with late clinical presentation or pathological lead points can also distort the apparent efficacy of non-operative treatment. We found no definitions of outcome measures in the literature or practice guidelines to inform analysis. Based on analysis of our own audit data we derived a “composite reduction rate” from first principles that can account for variations in radiological and surgical treatment thresholds that might bias other measures of successful non-operative treatment. This index is the proportion of intussusceptions not requiring resection that are successfully reduced non-operatively. We propose that the composite reduction rate be used as a key component of standardised multidisciplinary outcome reporting for intussusception rather than the selective reduction rate. The reduced bias and confounding would allow fairer comparisons and lead to better outcome standards.

Temporary brittle bone disease versus suspected non-accidental skeletal injury

Temporary brittle bone disease has been proposed again as an alternative explanation for suspected non-accidental injury. This is still not considered a real entity by mainstream opinion. The recent publications remind us to look carefully for alternative explanations and to investigate for predisposing bone disorders thoroughly.nnWhen a child presents with unexplained skeletal injury, the age, history of presentation and level of mobility of the child is important. The absence of a clear history to explain an injury raises the issue of non-accidental skeletal injury (NASI). It is also important to consider any underlying bone disorder that might predispose a bone fracturing with normal handling force.nnNASI has medicolegal implications for children and parents. In the UK, experts must provide balanced and impartial evidence to assist the court. Lawyers are instructed by the various parties but the expert is independent. Against this background the judge balances contrary medical expert evidence and opinion. Many alternative explanations have to be considered. Courts are faced with issues of scientific hypothesis, leading edge research and epidemiological data but court is not the best arena for scientific discussion.nnPaterson first proposed the concept of temporary brittle bone disease (TBBD) in 1993.1 He described a personal series of 39 children who had unexplained fractures in the first year of life. He proposed TBBD as a transient predisposition to fracture with normal handling force, without any bruising, proven medical diagnosis or biochemical abnormality. They suggested this was due to temporary immaturity or fragility of collagen related to trace element deficiency (eg, copper) or transient osteogenesis imperfecta (OI). They provided no specific test to confirm TBBD. There was no complete data table on their 39 cases and there was a lack of comprehensive bone biochemistry results. TBBD presented a theoretical and attractively benign explanation for suspected NASI …

Fluoroscopy-guided insertion of nasojejunal tubes in children – setting local diagnostic reference levels

BackgroundLittle is known about the radiation burden from fluoroscopy-guided insertions of nasojejunal tubes (NJTs) in children. There are no recommended or published standards of diagnostic reference levels (DRLs) available.ObjectiveTo establish reference dose area product (DAP) levels for the fluoroscopy-guided insertion of nasojejunal tubes as a basis for setting DRLs for children. In addition, we wanted to assess our local practice and determine the success and complication rates associated with this procedure.Materials and methodsChildren who had NJT insertion procedures were identified retrospectively from the fluoroscopy database. The age of the child at the time of the procedure, DAP, screening time, outcome of the procedure, and any complications were recorded for each procedure. As the radiation dose depends on the size of the child, the children were assigned to three different age groups. The sample size, mean, median and third-quartile DAPs were calculated for each group. The third-quartile values were used to establish the DRLs.ResultsOf 186 procedures performed, 172 were successful on the first attempt. These were performed in a total of 43 children with 60% having multiple insertions over time. The third-quartile DAPs were as follows for each age group: 0–12xa0months, 2.6xa0cGyxa0cm2; 1–7xa0years, 2.45xa0cGyxa0cm2; >8xa0years, 14.6xa0cGyxa0cm2. High DAP readings were obtained in the 0–12xa0months (nu2009=u20094) and >8xa0years (nu2009=u20092) age groups. No immediate complications were recorded.ConclusionFluoroscopy-guided insertion of NJTs is a highly successful procedure in a selected population of children and is associated with a low complication rate. The radiation dose per procedure is relatively low.

The Hip Joint

Interpreting images of the developing hip can be challenging due to a number of factors. The cartilaginous nature of the proximal femoral anlage means the ossified skeleton is continually changing; however, the ossification proceeds in a predictable manner and knowledge of this is important when interpreting radiographs. In addition, femoral head perfusion is precarious and can be interrupted by injuries around the hip joint. Recognising these potential pitfalls will influence the management and imaging of the hip joint following trauma

Neonatal duodenal stenosis and reflux into the biliary tree.

istent neuroblastoma and Hirschprungs disease: another manifestation of the neurocristopathy? Pediatr Radiol 8: 161 3. Gilbert F, Feder M, Barban G, et al (1984) Human neuroblastomas and abnormalities of chromosome 1 and 17. Cancer Res 44: 5444 4. Caron H, Peter M, van Sluis P, et al (1995) Evidence for two tumor suppressor loci on chromosomal bands 1p35-36 involved in neuroblastoma: one probably imprinted, another associated with Nmyc amplification. Hum Mol Genet 4: 535 M. Malogolowkin × B.Lopez Miranda × M. I.Boechat Pediatric Radiology, UCLA Medical Center, Los Angeles, CA 90095-1725, USA 433

Lethal micromelic short-rib skeletal dysplasia with triangular-shaped humerus.

Abstract We report two brothers with a new type of lethal, micromelic, short-rib, skeletal dysplasia characterised by short limbs with distinctive triangular-shaped humeri. This condition is most likely caused by either an autosomal recessive or X-linked recessive gene.