A Sonography-based Decision Model for Use in Treatment of Developmental Dysplasia of the Hip.

The objective of this study was to delineate a model for management of developmental dysplasia of the hip (DDH) treatment that incorporates hip ultrasound with objective/predicative parameters at key decision-making times. Hip sonograms of 74 infants (59 females, 15 males; 141 hips) with DDH were retrospectively reviewed. Hip sonographic score (HSS; ranges 0-10) was developed to reflect hip status based on sonographic position, stability, and morphology. Analysis on different management groups (i.e., no treatment, successful treatment, and failed treatment) showed that the trend of HSS is helpful in predicting course of the disease and determining effectiveness of treatment. A model for the management of DDH that utilizes an HSS and frequency schedule for hip sonography that is aligned with times of critical treatment decisions is proposed. This model illustrates how hip sonography can bring added value when timed to guide critical treatment decisions. (Journal of Surgical Orthopaedic Advances 32(1):047-054, 2023).

Current utilization of pediatric postmortem imaging in North America: a questionnaire-based survey.

BACKGROUND: Postmortem imaging is used more widely as the number of conventional autopsies has decreased over the last several decades. It is widely accepted in Europe, Asia and Oceania, but there has been a delay in acceptance in North America. Education, scanning protocols, resourcing and clinical incentives are needed to support this emerging field. OBJECTIVE: To determine the use of postmortem imaging and define perceived barriers to its implementation with the goal of expanding postmortem imaging in the United States and Canada. MATERIALS AND METHODS: We sent an online survey to active members of the Society for Pediatric Radiology (SPR) addressing the use of postmortem imaging, indications, readers, practical aspects, anticipated barriers and potential solutions to more widespread use. RESULTS: More than 50% of the 50 institutions that returned surveys used postmortem computed tomography; 24% used postmortem magnetic resonance imaging. Most postmortem imaging cases were read by radiologists. Fewer than 50% had formal correlation with autopsy results or an established relationship with the local medical examiner. Seven institutions reported reimbursement for postmortem imaging. Major barriers to postmortem imaging included lack of funding and lack of interest among clinicians. Funding and education were seen as important issues requiring attention. CONCLUSION: While most responding institutions provide pediatric postmortem imaging, the modalities, protocols, reporting procedures and clinical correlation vary widely. A lack of funding and few opportunities for education are limiting factors. Attention to these issues along with active support from the SPR are seen as potential solutions to recognize the value and promote widespread acceptance of postmortem imaging.

A Comparison of Lumbar Spine and Lateral Distal Femur Bone Density in Girls With Rett Syndrome.

INTRODUCTION/BACKGROUND: Patients with Rett syndrome (RS) are at risk for low bone mineral density (BMD) and femoral fractures. In patients with RS, assessment with lateral distal femur (LDF) dual-energy X-ray absorptiometry (DXA) is recommended and clinically relevant. This study is the first to assess LDF BMD in girls with RS, and to compare LDF BMD results with lumbar spine BMD results in RS. Method Eleven girls (mean age 8.4 yr) with molecularly diagnosed RS and clinical DXA scan(s) were identified; medical charts were retrospectively reviewed. Baseline and serial lumbar spine and LDF BMD Z-scores were evaluated based on patients' ambulation status, presence of epilepsy, and mutation type. Results At the time of first scan, 8 of 11 patients had normal lumbar spine BMD and low LDF BMD Z-scores. Two patients had fracture history. Fully ambulatory (3) patients had higher lumbar spine and LDF BMD than partially (5) and nonambulatory (3) patients. Patients with epilepsy had lower average BMD at all sites. No difference was seen in lumbar spine or LDF BMD in patients with high-risk BMD mutations. Seven patients had serial DXA scans with an average observation of 5.1 yr (range 3.1 yr to 6.2 yr). Lumbar spine BMD over time was variable, while LDF bone mass accrual occurred at a lower rate than typically developing girls. Conclusion Females with RS exhibited lower BMD Z-scores at the LDF than at the lumbar spine. LDF and lumbar spine results were discordant. Ambulatory status and the presence of epilepsy were related to BMD. LDF BMD accrual deviated from normal as patients aged.

Infantile Tibia Vara (Blount Disease) with Iatrogenic Changes Causing Residual Tibial Deformities.

Treatment of infantile tibia vara or Blount disease (ITV/BD) in patients < 3 years old and Langenskiold stages I-III consists of orthosis and, in relapsing cases, proximal tibial osteotomy and/or proximal tibial guided growth laterally with a tension band plate. Our aim was to evaluate the results of treatments in a consecutive group. After Institutional Review Board approval, data from 2002 to 2018 were collected. Thirty-nine knees (average age 22.4 months) with ITV/BD were treated with orthoses, and 10 knees failed. Six knees showed hyperintense T2-weighted signal in the medial proximal tibial epiphyseal cartilage on magnetic resonance imaging. Three of six knees with tibial osteotomy failed and underwent guided growth. Tibial plateau slopes were abnormal medially from the ITV/BD and laterally from the guided growth (triangular physis and depressed plateau deformities) because of factors such as orthotic treatment, tibial osteotomy, magnetic resonance imaging "physis severity score," and guided growth. (Journal of Surgical Orthopaedic Advances 29(3):141-148, 2020).

Pearls and Pitfalls of Hip Ultrasound.

Failure to appreciate key anatomic features and suboptimal sonographic technique lead to incorrect assessment of the key elements of developmental dysplasia of the hip: position, stability, and morphology. In this article, we address common errors, identify sonographic features critical for accurate image interpretation, and address measurement variability.

Tibial Intraosseous Insertion in Pediatric Emergency Care: A Review Based upon Postmortem Computed Tomography.

Objective: The proximal tibia is a recommended and commonly used site for pediatric emergency intraosseous vascular access (IO). During forensic whole body postmortem computed tomography (PMCT), we evaluated accuracy of emergency placement of tibial IO access.Methods: We conducted a retrospective review of 92 state medical examiner cases to assess presence and placement of tibial IO needles. Insertions were classified as successful (needle tip in the medullary portion of the bone) or unsuccessful (all other non-medullary placements) based upon position of the needle tip. Medical records were reviewed for patient age, equipment, and where an insertion was attempted, as well as if IO placement occurred in a prehospital or hospital environment.Results: Thirty-one cases with 42 tibial devices (aged 3 weeks to 16 years, median 4 months) were identified. In 25 insertions (60%), the needle tip was in satisfactory position. In 17 placements (40%), needle tip was unsatisfactory and included tibia perforation (6), tip embedded in the cortex (6), and needle missed the bone (5). In patients older than 6 months, all six placements of a 15-mm needle were successful. In infants age 6 months or younger, 14 placements (56%) were successful and 11 (44%) unsuccessful. The 25-mm IO needle was successfully placed in five of six children older than 6 months. In infants age 6 months or younger, the 25-mm needle was unsuccessfully placed in five of five attempts.Conclusion: In infants 6 months of age or younger, tibial IO needle insertion had a 53% failure rate (non-medullary placement). Failures occur during both prehospital and emergency department care. In infants age 6 months or younger, use of a 25-mm needle should be avoided. Procedures for IO insertion in infants age 6 months or younger should be reviewed and modification considered.

G6PD deficiency: An update.

Although glucose-6-phosphate dehydrogenase (G6PD) deficiency is less known in Western countries than in the Middle East and Africa, global migration and immigration are bringing ethnic groups with the highest incidence of this inherited genetic disorder into the US healthcare system. The G6PD enzyme is critical to protecting erythrocytes against oxidative stress, and deficiency may lead to hemolysis in the presence of certain environmental factors such as infection and some medications and foods. Neonatal jaundice, favism, and hemolysis are associated with exposure to increased oxidative stressors in patients with G6PD deficiency. By recognizing the potential for G6PD deficiency, clinicians can screen for the disorder and teach affected patients how to avoid triggers that result in harmful clinical manifestations.

Adult Tourniquet for Use in School-Age Emergencies.

BACKGROUND: Gunshot injuries are a leading cause of morbidity and mortality in the pediatric population. The Pediatric Trauma Society supports the use of tourniquets for exsanguinating hemorrhage in severe extremity trauma. The Combat Application Tourniquet (CAT) used with success in adults has not been prospectively tested in children. Our objective with this study was to determine if the CAT is successful in arresting extremity arterial blood flow in school-aged children. METHODS: Sixty school-aged volunteers (ages 6-16 years) recruited by age cohort had the CAT applied to an upper arm and thigh while peripheral pulse was monitored by Doppler. The number of windlass turns (maximum allowed: 3 [1080°]) required to arrest arterial pulse was recorded. Success was analyzed by BMI percentile for age and extremity circumference. RESULTS: The CAT was successful in occluding arterial blood flow as detected by Doppler pulse in all 60 (100%) of the upper extremities tested. In the lower extremity, 56 (93%) had successful occlusion. The 3-turn maximum allowed by the protocol was not adequate in some obese, older subjects (BMI >30). In both the upper and lower extremity, the number of turns required to occlude blood flow gradually increased with an increase in arm and thigh circumference. CONCLUSIONS: Prospective testing of a cohort of school-aged children 6 to 16 years revealed the CAT tourniquet to be suitable for use in both the upper and lower extremity.

Patchy increased echogenicity: a sonographic sign of femoral head necrosis following reduction and casting for developmental dysplasia of the hip.

A developmental dysplasia of the hip (DDH) case treated by closed reduction and casting and subsequently confirmed to have avascular necrosis (AVN) was retrospectively noted to have an abnormal pattern of echogenicity in the femoral head on sonograms obtained within 1.5 months of surgery. Patchy increased echogenicity in parts of the unossified cartilage replaced the normal pattern of central coalescence of vessels described with development of the ossification center. An additional case with similar findings confirms this should be considered a sign of evolving AVN following closed reduction.

Pulmonary embolus as cause of death in an adolescent: demonstration on postmortem CT.

Computed tomography (CT) is widely accepted in adult forensic death investigations (determination of cause and manner of death) but is only beginning to play a larger role in the cause of death determination in infants and children. We present a case of an adolescent with nephrotic syndrome who sustained cardiac arrest and died in the emergency department. A postmortem CT was requested by the state Office of the Medical Examiner as part of the medicolegal death investigation. Postmortem CT showed a saddle pulmonary embolus that was confirmed on conventional autopsy, demonstrating a natural manner of death.

The Virtual Hydrostatic Test.

The hydrostatic test is used to help determine if there has been a live birth. Computed tomography (CT), with its ability to detect and localize air/gas in the body, offers a rapid, noninvasive tool for assessment.Four baby deaths (20 to 25 weeks' gestation) in which the hydrostatic test, radiographs, and CT were performed before autopsy are presented. In 2 cases, considered stillbirths, the lungs and liver sank, and there was no air seen in the lungs or gas in the liver on CT. Histology of the lungs showed collapsed alveoli. In 1 case, concluded to be a live birth, the lungs floated, the liver sank, and air was seen in the trachea, bronchi, and both lungs on CT. Histology of the lungs showed multiple areas of expanded alveoli. In 1 case, where both the lungs and liver floated, the CT showed gas widely distributed in the soft tissues. This reflected decomposition, and no conclusion could be made regarding birth status.Assessment of live birth is a critical and difficult decision. Postmortem CT offers another technique to consider in this determination, and it has significant advantages over radiography. Continued study and correlation with existing methods seem warranted.

Bone mineral density in mucopolysaccharidosis IVB.

To date, the only published reports of bone mineral density (BMD) in MPS IV involve patients with MPS IVA; no reports exist describing BMD for MPS IVB. In this prospective study of BMD in three patients with MPS IVB, BMD was acquired by dual-energy X-ray absorptiometry (DXA) at whole body (WB), lumbar spine (LS), and lateral distal femur (LDF). Functional abilities, ambulatory status, medical history, and height z-score were evaluated. Three patients with MPS IVB (two females), aged 17.7, 31.4 and 31.7 years, were evaluated. Every patient was ambulatory and one sustained two fractures caused by trauma. Whole body and hip DXA scans were technically invalid in every patient due to the presence of prosthetic hip hardware. Lumbar spine was valid in only 1 patient due skeletal abnormalities, and was normal (Z-score of - 0.8). The LDF was valid in every patient and was low at all three regions of interest: average LDF z-scores were - 3.1 (range, - 2.9 to - 3.6), - 2.3 (range, - 2.0 to - 2.5), and - 2.1 (range, - 2.0 to - 2.3) for region 1-region 3, respectively. Patients with MPS IVB have low BMD of the lower extremities even with full-time ambulation. Routine body sites to measure by DXA were problematic; hip and WB were invalid due to artifact, and LS had limited utility. The LDF was the only body site consistently available on all patients. Patients did not experience low-energy fractures despite low BMD.

Computed Tomographic Findings in Unsuspected Aortic Dissection and Adult Polycystic Kidney Disease.

The coexistence of adult polycystic kidney disease and aortic dissection should be recognized by forensic pathologists and radiologists. Two cases with postmortem computed tomography prior to autopsy illustrate the appearance of polycystic kidneys and the hemorrhage patterns that provide clues to the presence and approximate location of the aortic dissections. Optimal imaging technique is discussed.

Adaptation of the lateral distal femur DXA scan technique to adults with disabilities.

The technique that best addresses the challenges of assessing bone mineral density in children with neuromuscular impairments is a dual-energy X-ray absorptiometry (DXA) scan of the lateral distal femur. The purpose of this study was to adapt this technique to adults with neuromuscular impairments and to assess the reproducibility of these measurements. Thirty-one adults with cerebral palsy had both distal femurs scanned twice, with the subject removed and then repositioned between each scan (62 distal femurs, 124 scans). Each scan was independently analyzed twice by 3 different technologists of varying experience with DXA (744 analyses). Precision of duplicate analyses of the same scan was good (range: 0.4%-2.3%) and depended on both the specific region of interest and the experience of the technologist. Precision was reduced when comparing duplicate scans, ranging from 7% in the metaphyseal (cancellous) region to 2.5% in the diaphyseal (cortical) region. The least significant change was determined as recommended by the International Society for Clinical Densitometry for each technologist and each region of interest. Obtaining reliable, reproducible, and clinically relevant assessments of bone mineral density in adults with neuromuscular impairments can be challenging. The technique of obtaining DXA scans of the lateral distal femur can be successfully applied to this population but requires a commitment to developing the necessary expertise.

Natural history of hip instability in infants (without subluxation or dislocation): a three year follow-up.

BACKGROUND: The natural history of hip instability (without subluxation or dislocation) and treatment in infants remain controversial. We performed a retrospective cohort case-only study with blinded, prospectively collected data to assess normalization of the acetabular index in consecutive untreated infant hips with sonography instability. METHODS: Consecutive hips meeting inclusion criteria were followed by sonography/radiography and data analyzed using tabular and regression models. RESULTS: In 48 hips, acetabular index measured by radiography normalized within 3 years of age without treatment. Normalization by age occurred: 7 months in 35%, 12 months in 67%, 18 months in 75%, 24 months in 81%, and 36 months in 100%. Two patterns of normalization of the acetabular index were observed: group I showed ossification in a physiological range of normal by 7 months of age, and group II had delayed ossification with later normalization of the acetabular index measurement. Breech presentation (p =0.013) and cesarean delivery (p =0.004) statistically directly correlated with a later normalization. CONCLUSIONS: The natural history of infant hip instability (without subluxation or dislocation), which is reduced at rest and unstable with stress as diagnosed by the Harcke method of sonography, has spontaneous normalization of the acetabular index within 3 years of age. We suggest three patterns of acetabular ossification in unstable infants' hips: (I) normal ossification, (II) delayed ossification with normalization of the acetabular index by age 3 years, and (III) defective secondary centers of ossification with an upward tilt of the lateral acetabular rim in adolescence.

Assessment of bone health in children with disabilities.

Evaluating the bone health of children with disabilities is challenging and requires consideration of many factors in clinical decision-making. Feeding problems and growth deficits, immobility/inability to bear weight, effect of medications, and the nature of his or her disease can all directly affect a child's overall picture of bone health. Familiarity with the tools available to assess bone health is important for practitioners. The most commonly used method to assess bone density, dual energy x-ray absorptiometry, can be performed effectively when one appreciates the techniques that make scanning patients with disabilities possible. There are specific techniques that are especially useful for measuring bone density in children with disabilities; standard body sites are not always obtainable. Consideration of clinical condition and treatment must be considered when interpreting dual energy x-ray absorptiometry scans. Serial measurements have been shown to be effective in monitoring change in bone content and in providing information on which to base decisions regarding medical treatment.

Observational case series: an algorithm incorporating multidetector computed tomography in the medicolegal investigation of human remains after a natural disaster.

An algorithm incorporating multidetector computed tomography (MDCT), digital radiographs, and external examination was used to triage cases for noninvasive or complete autopsy after a natural disaster. The algorithm was applied to 27 individuals who died during or soon after the earthquake that struck the Republic of Haiti on January 12, 2010. Of the 27 cases reviewed, 7 (26%) required a complete autopsy to determine cause and manner of death. In the remaining 20 (74%), cause and manner of death were determined with a reasonable degree of medical certainty after review of circumstances, an external examination, and postmortem imaging by MDCT and digital radiography (noninvasive autopsy). MDCT was particularly useful in detecting skeletal fractures caused by blunt force injury which were not evident on digital radiographs. The algorithm incorporating postmortem MDCT can be useful in the triage of human remains for autopsy after a natural disaster.