case presentation hip

Adhesive Capsulitis of the Hip: A Case Presentation and Review

Affiliations.

• 1 Orthopaedic Surgery - Sports Medicine, Massachusetts General Hospital - Mass General Brigham.
• 2 Physical Medicine and Rehabilitation - Sports Medicine, Massachusetts General Hospital - Mass General Brigham.
• PMID: 36263193
• PMCID: PMC9560891
• DOI: 10.52965/001c.37679

There remains a paucity of literature addressing adhesive capsulitis of the hip (ACH), making the diagnosis and treatment a continued challenge for healthcare providers. ACH encompasses restricted hip range-of-motion and pain that progresses through analogous Stages (1-4) to adhesive capsulitis of the shoulder. We report a case presentation of a middle-aged man that illustrates the significance of certain patient factors and provide a review of current literature to aid in the diagnostic evaluation and treatment for addressing ACH. Initial conservative treatment of ACH includes the appropriate management of associated comorbidities, oral and/or injectable pharmacologics, and physical therapy. While frequently resolving with time, refractory cases of ACH may require more aggressive approaches including pressure dilation, manipulation under anesthesia, synovectomy, capsular release and, for select patients, total hip arthroplasty. Given the limited available literature addressing ACH, healthcare providers may be forced to rely on a small number of published case reports and outdated review articles to guide their diagnostic evaluation and treatment approaches. Thus, this case presentation and review provides an updated approach to better diagnose and manage patients with ACH.

Keywords: Adhesive Capsulitis; Adhesive Capsulitis of the Hip; Hip.

Grants and funding

(Stanford users can avoid this Captcha by logging in.)

• Send to text email RefWorks EndNote printer

A Case-based approach to hip pain [digital] : a pocket guide to pathology, diagnosis and management

Available online.

• link.springer.com

At the library

Lane Medical Library

More options.

• Find it at other libraries via WorldCat
• Contributors

Description

Creators/contributors, contents/summary.

• Acknowledgments
• 1: Hip Anatomy
• Introduction
• 2: Bursitis and Tendonitis
• Greater Trochanteric Pain Syndrome (Trochanteric Bursitis)
• Clinical Presentation
• Physical Exam
• Diagnostic Exam
• Iliopsoas Bursitis
• Ischial Bursitis
• 3: Labral Tears
• Diagnostic Studies
• Non-operative Management
• Pharmacologic
• Physical Therapy
• Interventional and Regenerative Medicine
• Operative Management
• Return to Activities
• 4: Osteoarthritis
• Hip Osteoarthritis
• Pathological Process
• Diagnostic Workup
• Radiographic Study
• Ultrasound Study
• Laboratory Test
• Holistic Approach
• Pharmacological Treatment
• Interventional Procedures
• Regenerative Medicine
• Supplements
• Treatment(s)
• Septic Arthritis
• What Is the Pathology?
• Osteomyelitis
• Juvenile Idiopathic Arthritis
• Chondrolysis
• Vascular Conditions of the Hip
• Avascular Necrosis
• Legg-Calve-Perthes Disease
• Musculoskeletal Conditions of the Hip
• Slipped Capital Femoral Epiphysis
• Avulsion Fracture

Bibliographic information

• Stanford Home
• Maps & Directions
• Search Stanford
• Emergency Info
• Terms of Use
• Non-Discrimination
• Accessibility

© Stanford University , Stanford , California 94305 .

Disclaimer » Advertising

• HealthyChildren.org

• Previous Article
• Next Article

Introduction

Definitions, incidence, risk factors, and natural history, risk factors, natural history, screening and diagnosis, physical examination, radiography, ultrasonography, referral, adjunctive imaging, and treatment, adjunctive imaging, risks of treatment, medicolegal risk to the pediatrician, best practices and state of the art, acknowledgments, lead authors, section on orthopaedics executive committee, 2014–2015, evaluation and referral for developmental dysplasia of the hip in infants.

POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.

FINANCIAL DISCLOSURE: The authors have indicated they do not have a financial relationship relevant to this article to disclose.

FUNDED: No external funding.

• Split-Screen
• Article contents
• Figures & tables
• Supplementary Data
• Peer Review
• CME Quiz Close Quiz
• Open the PDF for in another window
• Get Permissions
• Cite Icon Cite
• Search Site

Brian A. Shaw , Lee S. Segal , SECTION ON ORTHOPAEDICS , Norman Y. Otsuka , Richard M. Schwend , Theodore John Ganley , Martin Joseph Herman , Joshua E. Hyman , Brian A. Shaw , Brian G. Smith; Evaluation and Referral for Developmental Dysplasia of the Hip in Infants. Pediatrics December 2016; 138 (6): e20163107. 10.1542/peds.2016-3107

Download citation file:

• Ris (Zotero)
• Reference Manager

Developmental dysplasia of the hip (DDH) encompasses a wide spectrum of clinical severity, from mild developmental abnormalities to frank dislocation. Clinical hip instability occurs in 1% to 2% of full-term infants, and up to 15% have hip instability or hip immaturity detectable by imaging studies. Hip dysplasia is the most common cause of hip arthritis in women younger than 40 years and accounts for 5% to 10% of all total hip replacements in the United States. Newborn and periodic screening have been practiced for decades, because DDH is clinically silent during the first year of life, can be treated more effectively if detected early, and can have severe consequences if left untreated. However, screening programs and techniques are not uniform, and there is little evidence-based literature to support current practice, leading to controversy. Recent literature shows that many mild forms of DDH resolve without treatment, and there is a lack of agreement on ultrasonographic diagnostic criteria for DDH as a disease versus developmental variations. The American Academy of Pediatrics has not published any policy statements on DDH since its 2000 clinical practice guideline and accompanying technical report. Developments since then include a controversial US Preventive Services Task Force “inconclusive” determination regarding usefulness of DDH screening, several prospective studies supporting observation over treatment of minor ultrasonographic hip variations, and a recent evidence-based clinical practice guideline from the American Academy of Orthopaedic Surgeons on the detection and management of DDH in infants 0 to 6 months of age. The purpose of this clinical report was to provide literature-based updated direction for the clinician in screening and referral for DDH, with the primary goal of preventing and/or detecting a dislocated hip by 6 to 12 months of age in an otherwise healthy child, understanding that no screening program has eliminated late development or presentation of a dislocated hip and that the diagnosis and treatment of milder forms of hip dysplasia remain controversial.

Early diagnosis and treatment of developmental dysplasia of the hip (DDH) is important to provide the best possible clinical outcome. DDH encompasses a spectrum of physical and imaging findings, from mild instability and developmental variations to frank dislocation. DDH is asymptomatic during infancy and early childhood, and, therefore, screening of otherwise healthy infants is performed to detect this uncommon condition. Traditional methods of screening have included the newborn and periodic physical examination and selected use of radiographic imaging. The American Academy of Pediatrics (AAP) promotes screening as a primary care function. However, screening techniques and definitions of clinically important clinical findings are controversial, and despite abundant literature on the topic, quality evidence-based literature is lacking.

The AAP last published a clinical practice guideline on DDH in 2000 titled “Early Detection of Developmental Dysplasia of the Hip.” 1 The purpose of this clinical report is to provide the pediatrician with updated information for DDH screening, surveillance, and referral based on recent literature, expert opinion, policies, and position statements of the AAP and the Pediatric Orthopaedic Society of North America (POSNA), and the 2014 clinical practice guideline of the American Academy of Orthopaedic Surgeons (AAOS). 1 , – 3  

A contributing factor to the DDH screening debate is lack of a uniform definition of DDH. DDH encompasses a spectrum of pathologic hip disorders in which hips are unstable, subluxated, or dislocated and/or have malformed acetabula. 1 However, imaging advancements, primarily ultrasonography, have created uncertainty regarding whether minor degrees of anatomic and physiologic variability are clinically significant or even abnormal, particularly in the first few months of life.

Normal development of the femoral head and acetabulum is codependent; the head must be stable in the hip socket for both to form spherically and concentrically. If the head is loose in the acetabulum, or if either component is deficient, the entire hip joint is at risk for developing incongruence and lack of sphericity. Most authorities refer to looseness as instability or subluxation and the actual physical deformity of the femoral head and/or acetabulum as dysplasia, but some consider hip instability itself to be dysplasia. Further, subluxation can be static (in which the femoral head is relatively uncovered without stress) or dynamic (the hip partly comes out of the socket with stress). The Ortolani maneuver, in which a subluxated or dislocated femoral head is reduced into the acetabulum with gentle hip abduction by the examiner, is the most important clinical test for detecting newborn dysplasia. In contrast, the Barlow maneuver, in which a reduced femoral head is gently adducted until it becomes subluxated or dislocated, is a test of laxity or instability and has less clinical significance than the Ortolani maneuver. In a practical sense, both maneuvers are performed seamlessly in the clinical assessment of an infant’s hip. Mild instability and morphologic differences at birth are considered by some to be pathologic and by others to be normal developmental variants.

In summary, there is lack of universal agreement on what measurable parameters at what age constitute developmental variation versus actual disease. Despite these differences in definition, there is universal expert agreement that a hip will fare poorly if it is unstable and morphologically abnormal by 2 to 3 years of age. It is the opinion of the AAP that DDH fulfills most screening criteria outlined by Wilson and Jungner 4 and that screening efforts are worthwhile to prevent a subluxated or dislocated hip by 6 to 12 months of age.

The Ortolani maneuver, in which a subluxated or dislocated femoral head is reduced into the acetabulum with gentle hip abduction by the examiner, is the most important clinical test for detecting newborn hip dysplasia.

The incidence of developmental dislocation of the hip is approximately 1 in 1000 live births. The incidence of the entire spectrum of DDH is undoubtedly higher but not truly known because of the lack of a universal definition. Rosendahl et al 5 noted a prevalence of dysplastic but stable hips of 1.3% in the general population. A study from the United Kingdom reported a 2% prevalence of DDH in girls born in the breech position. 6  

Important risk factors for DDH include breech position, female sex, incorrect lower-extremity swaddling, and positive family history. These risk factors are thought to be additive. Other suggested findings, such as being the first born or having torticollis, foot abnormalities, or oligohydramnios, have not been proven to increase the risk of “nonsyndromic” DDH. 3 , 7  

Breech presentation may be the most important single risk factor, with DDH reported in 2% to 27% of boys and girls presenting in the breech position. 6 , 8 , 9 Frank breech presentation in a girl (sacral presentation with hips flexed and knees extended) appears to have the highest risk. 1 Most evidence supports the breech position toward the end of pregnancy rather than breech delivery that contributes to DDH. There is no clear demarcation of timing of this risk; in other words, the point during pregnancy when the DDH risk is normalized by spontaneous or external version from breech to vertex position. Mode of delivery (cesarean) may decrease the risk of DDH with breech positioning. 10 , – 12 A recent study suggested that breech-associated DDH is a milder form than DDH that is not associated with breech presentation, with more rapid spontaneous normalization. 13  

Genetics may contribute more to the risk of DDH than previously considered “packaging effects.” If a monozygotic twin has DDH, the risk to the other twin is approximately 40%, and the risk to a dizygotic twin is 3%. 14 , 15 Recent research has confirmed that the familial relative risk of DDH is high, with first-degree relatives having 12 times the risk of DDH over controls. 16 , – 18 The left hip is more likely to be dysplastic than the right, which may be because of the more common in utero left occiput anterior position in nonbreech infants. 1 The AAOS clinical practice guideline considers breech presentation and family history to be the 2 most important risk factors in DDH screening. 3  

A lesser-known but important risk factor is the practice of swaddling, which has been gaining popularity in recent years for its noted benefits of enhancing better sleep patterns and duration and minimizing hypothermia. However, these benefits are countered by the apparent increased rates of DDH observed in several ethnic groups, such as Navajo Indian and Japanese populations, that have practiced traditional swaddling techniques. Traditional swaddling maintains the hips in an extended and adducted position, which increases the risk of DDH. However, the concept of “safe swaddling,” which allows for hip flexion and abduction and knee flexion, has been shown to lessen the risk of DDH ( http://hipdysplasia.org/developmental-dysplasia-of-the-hip/hip-healthy-swaddling/ ). Parents can be taught the principles of safe infant sleep, including supine position in the infant’s own crib and not the parent’s bed, with no pillows, bumpers, or loose blankets. 19 , – 24 The POSNA, International Hip Dysplasia Institute, AAOS, United States Bone and Joint Initiative, and Shriners Hospitals for Children have published a joint statement regarding the importance of safe swaddling in preventing DDH. 25  

In general, risk factors are poor predictors of DDH. Female sex, alone without other known risk factors, accounts for 75% of DDH. This emphasizes the importance of a careful physical examination of all infants in detecting DDH. 6 A recent survey showed poor consensus on risk factors for DDH from a group of experts. 26  

In general, risk factors are poor predictors of DDH. Female sex, alone without other known risk factors, accounts for 75% of DDH.

Clinical and imaging studies show that the natural history of mild dysplasia and instability noted in the first few weeks of life is typically benign. Barlow-positive (subluxatable and dislocatable) hips resolve spontaneously, and Barlow himself noted that the mild dysplasia in all 250 newborn infants with positive test results in his original study resolved spontaneously. 27 , – 32  

Conversely, the natural history of a child with hip dysplasia at the more severe end of the disease spectrum (subluxation or dislocation) by walking age is less satisfactory than children treated successfully at a younger age. Without treatment, these children will likely develop a limp, limb length discrepancy, and limited hip abduction. This may result in premature degenerative arthritis in the hip, knee, and low back. The burden of disability is high, because most affected people become symptomatic in their teens and early adult years, and most require complex hip salvage procedures and/or replacement at an early age.

The 2000 AAP clinical practice guideline recommended that all newborn infants be screened for DDH by physical examination, with follow-up at scheduled well-infant periodic examinations. The POSNA, the Canadian Task Force on DDH, and the AAOS have also advocated newborn and periodic screening. A 2006 report by the US Preventive Services Task Force (USPSTF) resulted in controversy regarding DDH screening. By using a data-driven model and a strong emphasis on the concept on predictors of poor health, the USPSTF report gave an “I” recommendation, meaning that the evidence was insufficient to recommend routine screening for DDH in infants as a means to prevent adverse outcomes. 1 , – 3 , 33 , – 35 However, on the basis of the body of evidence when evaluated from the perspective of a clinical practice model, the AAP advocates for DDH screening.

In its report, the USPSTF noted that avascular necrosis (AVN) is the most common (up to 60%) and severe potential harm of both surgical and nonsurgical interventions. 33 Williams et al 36 reported the risk of AVN to be less than 1% with screening, early detection, and the use of the Pavlik harness. In a long-term follow-up study of a randomized controlled trial from Norway, the authors reported no cases of AVN and no increased risk of harm with increased treatment. 37 The USPSTF also raised concerns about the psychological consequences or stresses with early diagnosis and intervention. Gardner et al 38 found that the use of hip ultrasonography allowed for reduction of treatment rates without adverse clinical or psychological outcomes. Thus, the concerns of AVN and psychological distress or potential predictors of poor health have not been supported in literature not referenced in the USPSTF report.

In 2 well-designed, randomized controlled trial studies from Norway, the prevalence of late DDH presentation was reduced from 2.6 to 3.0 per 1000 to 0.7 to 1.3 per 1000 by using either selective or universal hip ultrasonographic screening. Neither study reached statistical significance because of the inadequate sample size on the basis of prestudy rates of late-presentation DDH. Despite this, both centers have introduced selective hip ultrasonography as part of their routine newborn screening. 39 , 40 Clarke et al 32 also demonstrated a decrease in late DDH presentation from 1.28 per 1000 to 0.74 per 1000 by using selective hip ultrasonography in a prospective cohort of patients over a 20-year period.

The term “surveillance” may be useful nomenclature to consider in place of screening, because, by definition, it means the close monitoring of someone or something to prevent an adverse outcome. The term surveillance reinforces the concept of periodic physical examinations as part of well-child care visits until 6 to 9 months of age and the use of selective hip ultrasonography as an adjunct imaging tool or an anteroposterior radiograph of the pelvis after 4 months of age for infants with identified risk factors. 3 , 5 , 32 , 41  

Wilson and Jungner 4 outlined 10 principles or criteria to consider when determining the utility of screening for a disease. The AAP believes DDH fulfills most of these screening criteria ( Table 1 ), except for an understanding of the natural history of hip dysplasia and an agreed-on policy of whom to treat. The 2006 USPSTF report and the AAOS clinical practice guideline provide a platform to drive future research in these 2 areas. Screening for DDH is important, because the condition is initially occult, easier to treat when identified early, and more likely to cause long-term disability if detected late. A reasonable goal for screening is to prevent the late presentation of DDH after 6 months of age.

World Health Organization Criteria for Screening for Health Problems

The physical examination is by far the most important component of a DDH screening program, with imaging by radiography and/or ultrasonography playing a secondary role. It remains the “cornerstone” of screening and/or surveillance for DDH, and the available evidence supports that primary care physicians serially examine infants previously screened with normal hip examinations on subsequent visits up to 6 to 9 months of age. 3 , 41 , – 44 Once a child is walking, a dislocated hip may manifest as an abnormal gait.

The 2000 AAP clinical practice guideline gave a detailed description of the examination, including observing for limb length discrepancy, asymmetric thigh or gluteal folds, and limited or asymmetric abduction, as well as performing Barlow and Ortolani tests. 1 It is essential to perform these manual tests gently. By ∼3 months of age, a dislocated hip becomes fixed, limiting the usefulness and sensitivity of the Barlow and Ortolani tests. By this age, restricted, asymmetric hip abduction of the involved hip becomes the most important finding (see video available at http://www.aap.org/sections/ortho ). Diagnosing bilateral DDH in the older infant can be difficult because of symmetry of limited abduction.

Although ingrained in the literature, the significance and safety of the Barlow test is questioned. Barlow stated in his original description that the test is for laxity of the hip joint rather than for an existing dislocation. The Barlow test has no proven predictive value for future hip dislocation. If performed frequently or forcefully, it is possible that the maneuver itself could create instability. 45 , 46 The AAP recommends, if the Barlow test is performed, that it be done by gently adducting the hip while palpating for the head falling out the back of the acetabulum and that no posterior-directed force be applied. One can think of the Barlow and Ortolani tests as a continuous smooth gentle maneuver starting with the hip flexed and adducted, with gentle anterior pressure on the trochanter while the hip is abducted to feel whether the hip is locating into the socket, followed by gently adducting the hip and relieving the anterior pressure on the trochanter while sensing whether the hip slips out the back. The examiner should not attempt to forcefully dislocate the femoral head (see video available at http://www.aap.org/sections/ortho ).

“Hip clicks” without the sensation of instability are clinically insignificant. 47 Whereas the Ortolani sign represents the palpable sensation of the femoral head moving into the acetabulum over the hypertrophied rim of the acetabular cartilage (termed neolimbus), isolated high-pitched clicks represent the movement of myofascial tissues over the trochanter, knee, or other bony prominences and are not a sign of hip dysplasia or instability.

Plain radiography becomes most useful by 4 to 6 months of age, when the femoral head secondary center of ossification forms. 48 Limited evidence supports obtaining a properly positioned anteroposterior radiograph of the pelvis. 3 If the pelvis is rotated or if a gonadal shield obscures the hip joint, then the radiograph should be repeated. Hip asymmetry, subluxation, and dislocation can be detected on radiographs when dysplasia is present. There is debate about whether early minor radiographic variability (such as increased acetabular index) constitutes actual disease. 31 Radiography is traditionally indicated for diagnosis of the infant with risk factors or an abnormal examination after 4 months of age. 1 , 2 , 8 , 49  

Ultrasonography can provide detailed static and dynamic imaging of the hip before femoral head ossification. The American Institute of Ultrasound in Medicine and the American College of Radiology published a joint guideline for the standardized performance of the infantile hip ultrasonographic examination. 50 Static ultrasonography shows coverage of the femoral head by the cartilaginous acetabulum (α angle) at rest, and dynamic ultrasonography demonstrates a real-time image of the Barlow and Ortolani tests.

Ultrasonographic imaging can be universal for all infants or selective for those at risk for having DDH. Universal newborn ultrasonographic screening is not recommended in North America because of the expense, inconvenience, inconsistency, subjectivity, and high false-positive rates, given an overall population disease prevalence of 1% to 2%. 3 Rather, selective ultrasonographic screening is recommended either to clarify suspicious findings on physical examination after 3 to 4 weeks of age or to detect clinically silent DDH in the high-risk infant from 6 weeks to 4 to 6 months of age. 1 , 2 , 35 , 50 Two prospective randomized clinical trials from Norway support selective ultrasonographic imaging when used in conjunction with high-quality clinical screening. 39 , 40  

Roposch and colleagues 51 , 52 contend that experts cannot reach a consensus on what is normal, abnormal, developmental variation, or simply uncertain regarding much ultrasonographic imaging, thereby confounding referral and treatment recommendations. Several studies have demonstrated that mild ultrasonographic abnormalities usually resolve spontaneously, fueling the controversy over what imaging findings constitute actual disease requiring treatment. 5 , 30 , 51 , 53 , – 56  

The concept of surveillance for DDH emphasizes the importance of repeated physical examinations and the adjunct use of selective hip ultrasonography after 6 weeks of age or an anteroposterior radiograph of the pelvis after 4 months of age for infants with questionable or abnormal findings on physical examination or with identified risk factors. Ultrasonography is not necessary for a frankly dislocated hip (Ortolani positive) but may be desired by the treating physician. Physiologic joint capsular laxity and immature acetabular development before 6 weeks of age may limit the accuracy of hip ultrasonography interpretations. 39 , 40 There is no consensus on exact timing of and indications for ultrasonography among expert groups. 26 , 57 However, ultrasonographic imaging does have a management role in infants younger than 6 weeks undergoing abduction brace treatment of unstable hips identified on physical examination. 3  

Early detection and referral of infants with DDH allows appropriate intervention with bracing or casting, which may prevent the need for reconstructive surgery. Primary indications for referral include an unstable (positive Ortolani test result) or dislocated hip on clinical examination. Because most infants with a positive Barlow test result at either the newborn or 2-week examination stabilize on their own, these infants should have sequential follow-up examinations as part of the concept of surveillance. This recommendation differs from the 2000 AAP clinical practice guideline. 1 Any child with limited hip abduction or asymmetric hip abduction after the neonatal period (4 weeks) should be referred. Relative indications for referral include infants with risk factors for DDH, a questionable examination, and pediatrician or parental concern. 1  

Recommendations for the evaluation and management of infants with risk factors for DDH but with normal findings on physical examination continue to evolve. The 2000 AAP clinical practice guideline recommended hip ultrasonography at 6 weeks of age or radiography of the pelvis and hips at 4 months of age in girls with a positive family history of DDH or breech presentation. The AAP clinical practice guideline also stated that hip ultrasonographic examinations remain an option for all infants born breech. 1 The recent AAOS report found that moderate evidence supports an imaging study before 6 months of age in infants with breech presentation, family history, and/or history of clinical instability. 3 , 58 , – 60  

Consider imaging before 6 months of age for male or female infants with normal findings on physical examination and the following risk factors:

Breech presentation in third trimester (regardless of cesarean or vaginal delivery)

Positive family history

History of previous clinical instability

Parental concern

History of improper swaddling

Suspicious or inconclusive physical examination

Refinement in the term “breech presentation” as a risk factor for DDH is needed to determine whether selective hip ultrasonography at 6 weeks or radiography before 6 months of age is needed for an infant with a normal clinical hip examination. More specific variables, such as mode of delivery, type of breech position, or breech position at any time during the pregnancy or in the third trimester, have received little attention to date. The AAOS clinical practice guideline reported 6 studies addressing breech presentation, but all were considered low-strength evidence. 3 Thus, the literature is not adequate enough to allow specific guidance. The risk is thought to be greater for frank breech (hips flexed, knees extended) in the last trimester. 1  

Lacking expert consensus of risk factors for DDH, 26 the questions of whether to obtain additional imaging studies with a normal clinical hip examination is ultimately best left to one’s professional judgment. One must consider, however, that the overall probability of a clinically stable hip to later dislocate is very low.

Because of the variability in performance and interpretation of the hip ultrasonographic examination and varying thresholds for treatment, the requesting physician might consider developing a regional protocol in conjunction with a consulting pediatric orthopedist and pediatric radiologist. Specific criteria for imaging and referral based on local resources can promote consistency in evaluation and treatment of suspected DDH. Realistically, many families may not have ready access to quality infant hip ultrasonography, and this may determine the choice of obtaining a pelvic radiograph instead of an ultrasound. 61  

Recommendations for treatment are based on the clinical hip examination and the presence or absence of imaging abnormalities. Infants with a stable clinical hip examination but with abnormalities noted on ultrasonography can be observed without a brace. 3 , 56  

The initiation of abduction brace treatment, either immediate or delayed, for clinically unstable hips is supported by several studies. 3 , 62 , – 64 In a randomized clinical trial, Gardiner and Dunn 62 found no difference in hip ultrasonography findings or clinical outcome for infants with dislocatable hips treated with either immediate or delayed abduction bracing at 6- and 12-month follow-up. The infants in the delayed group (2 weeks) were treated with abduction bracing if hip instability persisted or the hip ultrasonographic abnormalities did not improve. 62  

Treatment of clinically unstable hips usually consists of bracing when discovered in early infancy and closed reduction with adductor tenotomy and spica cast immobilization when noted later. After 18 months of age, open surgery is generally recommended.

As previously noted, the 2006 USPSTF report noted a high rate of AVN, up to 60% with both surgical and nonsurgical intervention. 33 Other studies have reported much lower rates of AVN. 36 , 37 One prospective study reported a zero prevalence of AVN by 6 years of age in mildly dysplastic hips treated with bracing. 30  

However, abduction brace treatment is not innocuous. The potential risks include AVN, temporary femoral nerve palsy, and obturator (inferior) hip dislocation. 65 , – 67 One study demonstrated a 7% to 14% risk of complications after treatment in a Pavlik harness. The risk was greater in hips that did not reduce in the brace. 33 Precautions such as avoiding forced abduction in the harness, stopping treatment after 3 weeks if the hip does not reduce, and proper strap placement with weekly monitoring is important to minimize the risks associated with brace treatment. 68 , 69 Double diapering is a probably harmless but ineffective treatment of true DDH.

What remains controversial is whether the selective use of ultrasonography reduces or increases treatment. A randomized controlled study from the United Kingdom showed that approximately half of all positive physical examination findings were falsely positive (ie, normal ultrasonography results) and that the use of ultrasonography in clinically suspect hips actually reduced DDH treatment. 60 However, in the United States and Canada, 21 the reverse appears to be true. In the current medicolegal climate that encourages a defensive approach, liberal use of ultrasonography in the United States and Canada has clearly fostered overdiagnosis and overtreatment of DDH, despite best-available literature supporting observation of mild dysplasia. 33 , – 35 , 70  

Undetected or late-developing DDH is a liability concern for the pediatrician, generating anxiety and a desire for guidance in best screening methodology. 71 Unfortunately, this fear may also provoke overdiagnosis and overtreatment. “Late-presenting” DDH is a more accurate term than “missed” to use when DDH is first diagnosed in a walking-aged child who had appropriate clinical examinations during infancy. 72 , 73  

Although there is no universally recognized DDH screening standard, the AAP endorses the concept of surveillance or periodic physical examinations until walking age, with selective use of either hip ultrasonography or radiography, depending on age. The AAP cautions against overreliance on ultrasonography as a diagnostic test and encourages its use as an adjunctive secondary screen and an aid to treatment of established DDH. Notably, no screening program has been shown to completely eliminate the risk of a late-presenting dislocated hip. 69  

The electronic health record can be used to provide a template, reminder, and documentation tool for the periodic examination. It also can be useful in the transition and comanagement of children with suspected DDH by providing effective information transfer between consultants and primary care physicians and ensuring follow-up. Accurate documented communication between providers is important to provide continuity of care for this condition, and it is also important to explain to the parent(s) and document those instances when observation is used as a planned strategy so it is less likely to be misinterpreted as negligence.

The AAP, POSNA, AAOS, and Canadian DDH Task Force recommend newborn and periodic surveillance physical examinations for DDH to include detection of limb length discrepancy, examination for asymmetric thigh or buttock (gluteal) creases, performing the Ortolani test for stability (performed gently and which is usually negative after 3 months of age), and observing for limited abduction (generally positive after 3 months of age). Use of electronic health records can be considered to prompt and record the results of periodic hip examinations. The AAP recommends against universal ultrasonographic screening.

Selective hip ultrasonography can be considered between the ages of 6 weeks and 6 months for “high-risk” infants without positive physical findings. High risk is a relative and controversial term, but considerations include male or female breech presentation, a positive family history, parental concern, suspicious but inconclusive periodic examination, history of a previous positive instability physical examination, and history of tight lower-extremity swaddling. Because most DDH occurs in children without risk factors, physical examination remains the primary screening tool.

It is important that infantile hip ultrasonography be performed and interpreted per American Institute of Ultrasound in Medicine and the American College of Radiology guidelines by experienced, trained examiners. Developing local criteria for screening imaging and referral based on best resources may promote more uniform and cost-effective treatment. Regional variability of ultrasonographic imaging quality can lead to under- or overtreatment.

Most minor hip anomalies observed on ultrasonography at 6 weeks to 4 months of age will resolve spontaneously. These include minor variations in α and β angles and subluxation (“uncoverage”) with stress maneuvers. Current levels of evidence do not support recommendations for treatment versus observation in any specific case of minor ultrasonographic variation. Care is, therefore, individualized through a process of shared decision-making in this setting of inadequate information.

Radiography (anteroposterior and frog pelvis views) can be considered after 4 months of age for the high-risk infant without physical findings or any child with positive clinical findings. Age 4 to 6 months is a watershed during which either imaging modality may be used; radiography is more readily available, has a lower rate of false-positive results, and is less expensive than ultrasonography but involves a very low dose of radiation.

A referral to an orthopedist for DDH does not require ultrasonography or radiography. The primary indication for referral includes an unstable (positive Ortolani test result) or dislocated hip on clinical examination. Any child with limited hip abduction or asymmetric hip abduction after the neonatal period (4 weeks of age) should be referred for evaluation. Relative indications for referral include infants with risk factors for DDH, a questionable examination, and pediatrician or parental concern.

Evidence strongly supports screening for and treatment of hip dislocation (positive Ortolani test result) and initially observing milder early forms of dysplasia and instability (positive Barlow test result). Depending on local custom, either the pediatrician or the orthopedist can observe mild forms by periodic examination and possible follow-up imaging, but actual treatment should be performed by an orthopedist.

A reasonable goal for the primary care physician should be to diagnose hip subluxation or dislocation by 6 months of age by using the periodic physical examination. Selective ultrasonography or radiography may be used in consultation with a pediatric radiologist and/or orthopedist. No screening program has been shown to completely eliminate the risk of a late presentation of DDH. There is no high-level evidence that milder forms of dysplasia can be prevented by screening and early treatment.

Tight swaddling of the lower extremities with the hips adducted and extended should be avoided. The concept of “safe” swaddling, which does not restrict hip motion, minimizes the risk of DDH.

Treatment of neonatal DDH is not an emergency, and in-hospital initiation of bracing is not required. Orthopaedic consultation can be safely obtained within several weeks of discharge for an infant with a positive Ortolani test result. Infants with a positive Barlow test results should be reexamined and referred to an orthopedist if they continue to show clinical instability.

American Academy of Orthopaedic Surgeons

American Academy of Pediatrics

avascular necrosis

developmental dysplasia of the hip

Pediatric Orthopaedic Society of North America

US Preventive Services Task Force

This document is copyrighted and is property of the American Academy of Pediatrics and its Board of Directors. All authors have filed conflict of interest statements with the American Academy of Pediatrics. Any conflicts have been resolved through a process approved by the Board of Directors. The American Academy of Pediatrics has neither solicited nor accepted any commercial involvement in the development of the content of this publication.

Clinical reports from the American Academy of Pediatrics benefit from expertise and resources of liaisons and internal (AAP) and external reviewers. However, clinical reports from the American Academy of Pediatrics may not reflect the views of the liaisons or the organizations or government agencies that they represent.

The guidance in this report does not indicate an exclusive course of treatment or serve as a standard of medical care. Variations, taking into account individual circumstances, may be appropriate.

All clinical reports from the American Academy of Pediatrics automatically expire 5 years after publication unless reaffirmed, revised, or retired at or before that time.

The authors thank Charles Price, MD, FAAP, Ellen Raney, MD, FAAP, Joshua Abzug, MD, FAAP, and William Hennrikus, MD, FAAP, for their valuable contributions to this report.

Brian A. Shaw, MD, FAAOS, FAAP

Lee S. Segal, MD, FAAP

Norman Y. Otsuka, MD, FAAP, Chairperson

Richard M. Schwend, MD, FAAP, Immediate Past Chairperson

Theodore John Ganley, MD, FAAP

Martin Joseph Herman, MD, FAAP

Joshua E. Hyman, MD, FAAP

Brian G. Smith, MD, FAAP

Niccole Alexander, MPP

Competing Interests

Re: breech presentation in preterm infants.

Thanks for the excellent review highlighting the controversies around screening, imaging and management of DDH. I have a question to the authors re: preterm infants. If an infant is born premature in third trimester with breech presentation and a normal hip examination at birth, would the authors recommend considering a hip ultrasound after 6 weeks post-menstrual age or 6 weeks chronologic age?

RE: Late diagnosis of developmental dysplasia of the hip can be eradicated

The survey published by Shaw and al in the December issue of Pediatrics concluded that no screening program has eliminated late development or presentation of a dislocated hip (1).

In the literature, there is controversy over widespread ultrasound screening since its ability to prevent late DDH diagnosis has not been proven (2,3). Techniques mainly relied on acetabular morphology classifications with no clear cut-off for early DDH diagnosis. Results are not enough reproducible for a large screening program involving non-expert radiologists (4). Moreover the effect of hip instability on acetabular shape may not be seen at one month old. These three reasons explain the failure of almost all screening programs based on these techniques.

Our experience is based on dynamic assessment of the femoral head position based on pubo-femoral distance (PFD) measurements. The normal PFD is lower than or equal to 6mm, with no more than 1.5mm between the hips (5). This simple, reliable, and reproducible method was easily taught to general radiologists involved in the screening program. With the support of perinatal network pediatricians, ultrasound screening was offered to all girls and to boys presenting with risk factors or abnormalities on clinical examination at one month old. All reports indicate a prevalence of 90% for girls, 70% with no risk factors. All infants with positive screenings were immediately referred to multidisciplinary teams involving an expert radiologist and orthopedic pediatrician. At one month old, reducible hip instability was always successfully treated by abduction splint. In 2013, we published that late diagnosis of DDH was eradicated from our region (annual births: 14,000) over a 3-year period from 2009 to 2011 (5). This period has now reached 8 years in a region of more than 1 million inhabitants in which our institution is the only referral center. Brittany (France), country of Dr Le Damany who described this disease, has a high prevalence of DDH (6/1000).

These long-term results are unique and confirm that ultrasound measurement of PFD provides a clear cut-off for DDH detection. Based on this simple technique, widespread screening, at least in girls, could eradicate late DDH diagnosis.

References:

1. Shaw BA, Segal LS. Evaluation and Referral for Developmental Dysplasia of the Hip in Infants. Pediatrics. 2016;138(6):e20163107 2. von Kries R, Ihme N, Altenhofen L, Niethard FU, Krauspe R, Rückinger S. General ultrasound screening reduces the rate of first operative procedures for developmental dysplasia of the hip: a case-control study. J Pediatr. 2012;160(2):271–5. 3. Laborie LB, Markestad TJ, Davidsen H, Brurås KR, Aukland SM, Bjørlykke JA, et al. Selective ultrasound screening for developmental hip dysplasia: effect on management and late detected cases. A prospective survey during 1991-2006. Pediatr Radiol. 2014;44(4):410–24.

4. Roposch A, Moreau NM, Uleryk E, Doria AS (2006) Developmental dysplasia of the hip: quality of reporting of diagnostic accuracy for US. Radiology, 241(3):854-860.. 5. Tréguier C, Chapuis M, Branger B, Bruneau B, Grellier A, Chouklati K, et al. Pubo-femoral distance: an easy sonographic screening test to avoid late diagnosis of developmental dysplasia of the hip. Eur Radiol. 2013 Mar;23(3):836–44.

Advertising Disclaimer »

Citing articles via

Email alerts.

Affiliations

• Editorial Board
• Editorial Policies
• Journal Blogs
• Pediatrics On Call
• Online ISSN 1098-4275
• Print ISSN 0031-4005
• Pediatrics Open Science
• Hospital Pediatrics
• Pediatrics in Review
• AAP Grand Rounds
• Latest News
• Pediatric Care Online
• Red Book Online
• Pediatric Patient Education
• AAP Toolkits
• AAP Pediatric Coding Newsletter

First 1,000 Days Knowledge Center

Institutions/librarians, group practices, licensing/permissions, integrations, advertising.

• Privacy Statement | Accessibility Statement | Terms of Use | Support Center | Contact Us
• © Copyright American Academy of Pediatrics

This Feature Is Available To Subscribers Only

Sign In or Create an Account

Hip Pain Case Studies

• First Online: 01 January 2023

Cite this chapter

• Nathaniel Milburn 6 &
• David Tietze 7  

429 Accesses

In this chapter, we will present real-life case scenarios that may be encountered in clinical practice. The purpose of these cases is to outline a scope of work to guide diagnosis and treatment of various etiologies of hip pain.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

• Available as PDF
• Read on any device
• Instant download
• Own it forever
• Available as EPUB and PDF
• Compact, lightweight edition
• Dispatched in 3 to 5 business days
• Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Chronic Hip Pain in a 7-Year-Old

Clement DB, Ammann W, Taunton JE, Lloyd-Smith R, Jesperson D, McKay H, Goldring J, Matheson GO. Exercise-induced stress injuries to the femur. Int J Sports Med. 1993;14(6):347–52. https://doi.org/10.1055/s-2007-1021191 .

Article   CAS   Google Scholar  

Landin LA, Danielsson LG, Wattsgård C. Transient synovitis of the hip. Its incidence, epidemiology and relation to Perthes’ disease. J Bone Joint Surg Br. 1987;69(2):238–42. https://doi.org/10.1302/0301-620X.69B2.3818754 .

Rogalsky RJ, Black GB, Reed MH. Orthopaedic manifestations of leukemia in children. J Bone Joint Surg Am. 1986;68(4):494–501.

Salter RB. Etiology, pathogenesis and possible prevention of congenital dislocation of the hip. Can Med Assoc J. 1968;98:933–45.

CAS   Google Scholar  

Jiménez C, Delgado-Rodríguez M, López-Moratalla M, Sillero M, Gálvez-Vargas R. Validity and diagnostic bias in the clinical screening for congenital dysplasia of the hip. Acta Orthop Belg. 1994;60(3):315–21.

Google Scholar  

Kermond S, Fink M, Graham K, Carlin JB, Barnett P. A randomized clinical trial: should the child with transient synovitis of the hip be treated with nonsteroidal anti-inflammatory drugs? Ann Emerg Med. 2002;40(3):294–9. https://doi.org/10.1067/mem.2002.126171 .

Article   Google Scholar  

Rabin KR, Gramatges MM, Margolin JF, et al. Acute lymphoblastic leukemia. In: Pizzo PA, Poplack DG, editors. Principles and practice of pediatric oncology. 7th ed. Philadelphia: Wolters Kluwer; 2015.

Traumatic Hip Pain

Kanlic EM, Pinski SE, Verwiebe EG, et al. Acute morbidity and complications of thigh compartment syndrome: a report of 26 cases. Patient Saf Surg. 2010;4:13. https://doi.org/10.1186/1754-9493-4-13 .

Egol KA. Handbook of fractures. 4th ed. Philadelphia: Wolters Kluwer Health; 2010. Chapter 27. ISBN: 978-1605477602.

Waddell BS, Mohamed S, Glomset JT, Meyer MS. A detailed review of hip reduction maneuvers: a focus on physician safety and introduction of the Waddell technique. Orthop Rev (Pavia). 2016;8(1):6253.

Dwyer AJ, John B, Singh SA, Mam MK. Complications after posterior dislocation of the hip. Int Orthop. 2006;30(4):224–7.

Anterior Hip Pain in an Adult Male

https://orthoinfo.aaos.org/en/diseases%2D%2Dconditions/femoroacetabular-impingement/ .

Schafer KA, Clohisy JC, Nepple JJ. Rapidly progressive arthritis in femoroacetabular impingement: patient characteristics and risk factors for total hip arthroplasty by the age of forty. Iowa Orthop J. 2020;40(1):129–34.

Wernecke C, Braun HJ, Dragoo JL. The effect of intra-articular corticosteroids on articular cartilage: a systematic review. Orthop J Sports Med. 2015;3(5):2325967115581163. https://doi.org/10.1177/2325967115581163 .

Anterior Hip Pain in a Young Female

May T, Marappa-Ganeshan R. Stress fractures [updated 2021 Aug 11]. In: StatPearls. Treasure Island, FL: StatPearls Publishing; 2022.

https://compedgept.com/blog/hip-impingement-causes-and-fixes .

Miyamoto RG, Kaplan KM, Levine BR, Egol KA, Zuckerman JD. Surgical management of hip fractures: an evidence-based review of the literature. I: femoral neck fractures. J Am Acad Orthop Surg. 2008;16(10):596–607.

Miller TL, Kaeding CC, Rodeo SA. Emerging options for biologic enhancement of stress fracture healing in athletes. J Am Acad Orthop Surg. 2020;28(1):1–9. https://doi.org/10.5435/JAAOS-D-19-00112 .

Download references

Author information

Authors and affiliations.

Carrell Clinic, Dallas, TX, USA

Nathaniel Milburn

UT Southwestern Medical School, Dallas, TX, USA

David Tietze

You can also search for this author in PubMed   Google Scholar

Editor information

Editors and affiliations.

Physical Medicine and Rehabilitation, Sports Medicine, Peachtree Spine and Sports Physicians, Atlanta, GA, USA

Jasmine H. Harris

Princeton Spine and Joint Center, Princeton, NJ, USA

Grant Cooper

Sports MedicineDepartment of Rehabilitation and Human Performance, Mount Sinai Health System, New York, NY, USA

Joseph E. Herrera

Ana Bracilovic

Rights and permissions

Reprints and permissions

Copyright information

© 2022 The Author(s), under exclusive license to Springer Nature Switzerland AG

About this chapter

Milburn, N., Tietze, D. (2022). Hip Pain Case Studies. In: Harris, J.H., Cooper, G., Herrera, J.E., Bracilovic, A., Patel, A. (eds) A Case-Based Approach to Hip Pain. Springer, Cham. https://doi.org/10.1007/978-3-031-17154-3_10

Download citation

DOI : https://doi.org/10.1007/978-3-031-17154-3_10

Published : 01 January 2023

Publisher Name : Springer, Cham

Print ISBN : 978-3-031-17153-6

Online ISBN : 978-3-031-17154-3

eBook Packages : Medicine Medicine (R0)

Share this chapter

Anyone you share the following link with will be able to read this content:

Sorry, a shareable link is not currently available for this article.

Provided by the Springer Nature SharedIt content-sharing initiative

• Publish with us

Policies and ethics

• Find a journal
• Track your research

• About Us and Our Mission
• IHDI Physicians
• Benefactors
• Product Acknowledgement
• What is Hip Dysplasia?
• What Causes Hip Dysplasia?
• Infant & Child
• Adolescents
• Terms of Use: Publication Images
• Orthopedic Surgeons
• Pediatric & Primary Care Providers
• Biomedical Engineers
• Research & Clinical Trials
• Teaching Boxes
• Teaching Portal
• Brochures – Multilingual
• Q’s For Your Doctor
• FAQ Infant & Child
• FAQ Adolescents
• Infant & Child – Other Resources for Parents
• View All Patient Stories
• Patient Stories – Infants & Children
• Patient Stories – Adolescents
• Patient Stories – Adults
• Submit a Story
• Latest News
• Scientific Reviews
• Hip-Healthy Products
• Raise Awareness & Volunteer
• Community Fundraising
• Share Your Story

Professional Resources

Orthopedic surgeons - case studies, adolescent peri-acetabular osteotomy (pao).

17 year old boy with painful left hip. Note large dysplastic acetabulum and femoral head subluxation on the patient’s left side. The head of the femur is moving in a false socket high on the side of the pelvis. The arthrogram below shows that the hip can be put back into the true socket.

During the arthrogram shown below, radiographic dye is injected into the hip joint. The dark area is fluid that is filling the joint space. In the first image the hip is abducted and internally rotated to demonstrate that the femoral head slides lower into the socket leaving fluid above the hip joint. The second image shows where the femoral head sits when the thigh is in neutral position as it is during standing. This is the position of the hip during the first x-ray above. It was determined that PAO might be possible to place the intact joint surface in a better position to support the femoral head during weight-bearing. The PAO will rotate the socket so that it is more on top of the femoral head.

The x-ray below shows the hip after PAO. Note that slope of the socket is more horizontal and the intact portion of the acetabulum has been rotated so that it is directly over the femoral head. This allows the intact cartilage surfaces to support weight-bearing. The patient was relieved of pain and should be able to avoid total joint replacement for many years.

• IHDI Standards
• Webinar & Courses
• Online Textbooks & Publications
• Video Library
• Additional Resources
• Case Studies
• Influential References

Founding Benefactors

• Privacy Policy
• Terms of Use
• Hip Dysplasia and Everyday Life
• Pediatric and Primary Care Providers
• Get Involved
• Infant Diagnosis
• Planning Treatment for Children
• Child Treatment Methods
• Tips for Parents
• Other Resources for Parents
• Preventing Hip Dysplasia
• FAQ Child Hip Dysplasia
• Related Hip Disorders
• Treatment Overview
• Hip Preservation Surgery
• Questions for Your Doctor
• Other Resources
• Hip Healthy Products
• FAQ Adolescent Hip Dysplasia
• Total Hip Replacement
• FAQ Adult Hip Dysplasia

Small ruminant case presentation: Overeating disease

By Drs. Chrissy Eckstrand and Christine Haake, Pathologists, WADDL

Gross Necropsy Description: A 9 kg, 3-week-old, intact male lamb was presented in fair postmortem condition and good body condition with moderate subcutaneous and visceral adipose tissue stores and moderate, bilaterally symmetric skeletal muscle mass.

Thorax : Pulmonary interlobular septa were mildly distended by clear fluid, and abundant white froth and mucus were present within the lumen of the trachea. Sections of lung floated in 10% neutral buffered formalin. There were approximately 20-25 mL of thin, watery fluid within the pericardial sac.

Urinary bladder : The bladder was moderately distended with urine. Approximately 500-1000 mg/DL glucose was detected in urine using a dipstick test.

Tissues also examined and considered free of significant gross lesions included the tongue, trachea, esophagus, thyroid, liver, spleen, kidneys, gastrointestinal tract, adrenal glands, skeletal muscle, bone marrow, eyes, testes, lymph nodes, and brain.

At this stage in the postmortem evaluation the primary gross diagnoses included 1) moderate, acute hydropericardium, and 2) mild, diffuse pulmonary edema along with moderate to marked glucosuria.  Although the proximate cause of death was not evident on gross exam, the sudden death, age, and absence of significant gross abnormalities beyond pulmonary edema and hydropericardium suggested that enterotoxemia caused by Clostridium perfringens be considered as a possible cause of death. Furthermore, glucose detected within the urine via dipstick can be supportive of enterotoxemia. A small intestinal contents smear revealed numerous gram-positive rod bacteria (supportive of Clostridium).

Histopathology was conducted leading to the following histologic descriptions:

Brain: Virchow-Robins spaces surrounding blood vessels were expanded by increased clear space (perivascular edema). Multifocal, small, acute microhemorrhages were present throughout the grey and white matter of the midbrain, cerebrum, and cerebellum.

Lungs: Connective tissue surrounding pulmonary blood vessels was expanded by increased clear space (perivascular edema). Alveolar spaces were multifocally flooded with eosinophilic proteinaceous fluid (edema).

Small intestines: Moderately to markedly increased numbers of eosinophils were present multifocally within the lamina propria. In a separate section, the small intestinal lumen contained numerous degenerate neutrophils, mixed with ingesta and very high numbers of rod-shaped bacteria.

Tissues also examined and considered free of significant histologic lesions included the trachea, esophagus, heart, spleen, rumen, reticulum, adrenal glands, skeletal muscle, and bone marrow.

Based on this histologic findings the primary histologic diagnoses included 1) mild, acute, multifocal, perivascular edema in the brain and lungs, 2) mild, acute, multifocal pulmonary edema, 3) mild, peracute, multifocal hemorrhage in the brain, kidneys and thymus, and 4) moderate to marked, acute, multifocal, neutrophilic to eosinophilic enteritis within the small intestines.

The perivascular edema within the brain and lungs, multifocal small hemorrhages within the brain, kidneys, and thymus, and suppurative enteritis were supportive of enterotoxemia caused by Clostridium perfringens , which was grown in very large numbers in bacterial culture of intestinal contents. The causative agent of enterotoxemia, C. perfringens type D, was determined via toxin genotyping PCR as both alpha and epsilon toxin genes were detected. C. perfringen s type D causes enterotoxemia in sheep (overeating disease). Predisposing factors include ingestion of excessive amounts of feed or milk in the very young and of grain in feedlot lambs, which allows overgrowth of epsilon toxin producing C. perfringens , causing systemic vascular damage. Acute death without clinical signs is common in lambs, though neurologic and respiratory signs may also be observed due to the presence of systemic edema. Given these findings it was suggested to the producer to review management, including vaccination of ewes prior to lambing, which is recommended for optimal prevention of this disease.

Necropsy investigation of acute death of a 3-week-old lamb

A. Necropsy revealed expansion of interlobular septa of the lung by clear fluid (pulmonary edema, black arrow) and increased pericardial fluid (blue arrow).

B. A urine dipstick revealed glucosuria.

C. Gram stain of an intestinal content smear revealed many gram-positive rod bacteria (supportive of Clostridium)

D. Histopathology of the brain demonstrates increased clear space around blood vessels (blue arrow, suggestive of cerebral edema)

About This Study

The report says all conclusions must be assessed in the context of the established harms of COVID-19 infection and the well-documented benefits of COVID-19 vaccines in preventing infection. The committee was not tasked with estimating the magnitude or strength of associations between vaccinations and outcomes, and did not offer conclusions regarding specific patient cases or whether VICP or CICP should award compensation in individual cases or in general.

“Given that the studies we reviewed were performed shortly after vaccines were available, the information in this report is a snapshot in time, and new vaccines will be developed and more research conducted,” said George Isham, senior fellow, HealthPartners Institute, and chair of the committee that wrote the report. “For example, the evidence does not address the real-world use of the COVID-19 vaccines in which many individuals received a ‘mix and match’ sequence of them. Many people vaccinated for COVID-19 also received other vaccines, such as influenza, simultaneously.”

The study — undertaken by the  Committee to Review Relevant Literature Regarding Adverse Effects Associated with Vaccines — was sponsored by the Health Resources and Services Administration of the U.S. Department of Health and Human Services.

The National Academies of Sciences, Engineering, and Medicine are private, nonprofit institutions that provide independent, objective analysis and advice to the nation to solve complex problems and inform public policy decisions related to science, engineering, and medicine. They operate under an 1863 congressional charter to the National Academy of Sciences, signed by President Lincoln.

Dana Korsen, Director of Media Relations Megan Lowry, Media Relations Manager Office of News and Public Information 202-334-2138; e-mail  [email protected]

Featured Publication

Evidence Review of the Adverse Effects of COVID-19 Vaccination and Intramuscular Vaccine Administration

Vaccines are a public health success story, as they have prevented or lessened the effects of many infectious diseases. To address concerns around potential vaccine injuries, the Health Resources and Services Administration (HRSA) administers the Vaccine Injury Compensation Program (VICP) and the Countermeasures Injury Compensation Program (CICP), which provide compensation to those who assert that they were injured by routine vaccines or medical countermeasures, respectively. The National Academies of Sciences, Engineering, and Medicine have contributed to the scientific basis for VICP compensation decisions for decades.

HRSA asked the National Academies to convene an expert committee to review the epidemiological, clinical, and biological evidence about the relationship between COVID-19 vaccines and specific adverse events, as well as intramuscular administration of vaccines and shoulder injuries. This report outlines the committee findings and conclusions.

Read Full Description

• Digital Resource: Evidence Review of the Adverse Effects of COVID-19 Vaccination
• Digital Resource: Evidence Review of Shoulder Injuries from Intramuscular Administration of Vaccines
• Press Release

Recent News

NAS Launches Science and Innovation Fund for Ukraine

Science Academies Issue Statements to Inform G7 Talks

Supporting Family Caregivers in STEMM

A Vision for High-Quality Preschool for All

• Load More...

CASE REPORT article

Case report: systemic presentation of alk-positive histiocytosis.

• 1 Shengli Clinical Medical College of Fujian Medical University, Fuzhou, China
• 2 Department of Urology, Fujian Provincial Hospital, Fuzhou, Fujian, China
• 3 Department of Pathology, Fujian Provincial Hospital, Fuzhou, China
• 4 Department of Pediatric Surgery, Fujian Provincial Hospital, Fuzhou, China

ALK-positive Histiocytosis (ALK-HSs) is a recently identified rare clinical entity characterized by tissue histiocytic alterations associated with ALK gene rearrangement. Clinical presentations can be solitary, multifocal, or systemic (involving multiple sites and organs). Due to limited reported cases, there is inadequate understanding of this disease. This report presents a case of ALK-HSs in a 71-year-old male patient who presented with hematuria for one week. Imaging studies conducted at an external hospital showed multiple lesions in the penis, bilateral testes, back skin, and the third lumbar vertebra. Histopathological findings included spindle and histiocytic cell proliferation with mild or indistinct cellular atypia, interstitial infiltration of lymphocytes, plasma cells, foamy histiocytes, and fibrous tissue proliferation. Immunohistochemistry of the lesion cells revealed positivity for CD68, CD163, ALK1, ALK (D5F3), and Vimentin. FISH testing indicated ALK gene separation in the lesion cells. NGS testing identified the fusion genes KIF5B(NM_004521) and ALK(NM_004304) in the lesion cells. We combined the characteristics of this case with a review of the literature to enhance our understanding of this rare clinical entity.

Introduction

ALK-positive Histiocytosis (ALK-HSs) is a rare proliferative disease of histiocytes. In the 2022 fifth edition of the “WHO Classification of Tumours of Hematopoietic and Lymphoid Tissues,” this disease type has been added as a provisional subtype ( 1 ). In recent years, scholars have gradually recognized and reported ALK-HSs. The prognosis for this condition is primarily favorable, but in a few cases, it can be poor. Searching in the international medical literature, fewer than 50 cases have been reported to date, making systemic ALK-HSs extremely rare ( 2 , 3 ). This article presents one case of systemic ALK-HSs, detailing the patient’s treatment, follow-up, and prognosis. It summarizes the clinical and pathological characteristics of systemic ALK-HSs and reviews relevant literature to provide a basis for clinical treatment.

Case report

The patient is a 71-year-old male presenting with hematuria for one week. Physical examination revealed a bladder mass and imaging from an external hospital showed multiple masses in the penis, bilateral testes, and the third lumbar vertebra, ranging from 0.5 to 4 cm in diameter. Upon specialized examination, multiple nodules were found in the bilateral testes, penis, and back skin. These nodules were firm, poorly mobile, solid to touch, non-tender, and had clear boundaries. Imaging studies revealed slightly enlarged bilateral testes with multiple hypoechoic nodules (4 on the left, 3 on the right), the largest measuring 11.3mm x 10.3mm on the right and 10.4mm x 7mm on the left, roughly circular without distinct capsules, and displaying linear blood flow signals internally. Ultrasound contrast indicated mild nodular enhancement, showing quick uptake and slow washout. A hypoechoic area was observed in the right testicular sheath, measuring approximately 51 x 24mm. Additionally, both epididymis appeared normal in size and morphology, with head nodules measuring about 4mm x 4mm, exhibiting clear boundaries and good echogenicity. Color Doppler flow imaging showed normal blood flow in both testes and epididymis without significant abnormalities in the blood flow spectrum.

MRI examination indicated a cauliflower-like soft tissue mass, about 2.0cm x 2.1cm x 1.9cm in size, with clear margins, located at the bladder’s posterior lower wall (bladder neck) ( Figure 1A ). The mass displayed iso-intensity on T1-weighted imaging, slightly hyperintensity on T2-weighted imaging, high signal on DWI, low signal on ADC, and uneven mild to moderate enhancement post-contrast administration. Multiple small lymph nodes were found in the bilateral inguinal regions. Additionally, numerous small nodular lesions within both testes showed fatty suppression on T2WI, measuring about 0.8cm in diameter, and hydrocele in the bilateral scrotum ( Figure 1B ). The scan also revealed multiple slightly extended and longer T1 and T2 signal lesions in the bilateral femoral upper segments, pelvic bones, and some lumbar-sacral vertebrae, with some lesions showing significant enhancement post-contrast. This suggested a highly vascularized mass at the posterior lower wall of the bladder (bladder neck), indicating bladder cancer, with other tumorous lesions to be further investigated. Multiple lesions in the bilateral femoral upper segments, pelvic bones, and some lumbar-sacral vertebrae were indicative of metastatic tumors awaiting confirmation.

Figure 1 Imaging of a patient with systemic manifestation of ALK-positive Histiocytosis. (A) Magnetic resonance imaging (T1-weighted imaging) displays a cauliflower-like soft tissue mass measuring approximately 2.0cm x 2.1cm x 1.9cm, exhibiting clear margins situated at the posterior lower wall of the bladder. (B) Magnetic resonance imaging (T2-weighted imaging) illustrates numerous small nodular lesions within both testes, revealing fatty suppression on T2WI and measuring approximately 0.8cm in diameter. (C) Multiple nodular distributions lacking capsules are observed under low magnification, scattered within the testicular parenchyma, displaying relatively clear boundaries of the nodules. (D) Lesions resemble xanthoma-like structures with the proliferation of foam-like tissue cells characterized by large cell volumes, irregularly folded or lobulated nuclei, and fine nuclear chromatin. (E) Immunohistochemical examination of the testicular mass displays positivity for ALK1 (cytoplasmic positivity). (F) Fluorescence In Situ Hybridization testing was conducted on the testicular and bladder masses, revealing ALK1 gene rearrangement.

CT examination revealed a nodular lesion protruding into the bladder lumen in the posterior wall (bladder trigone area) measuring approximately 2.1cm x 1.8cm with a CT value of about 35 Hounsfield Units (HU). There was adjacent thickening of the bladder wall, and post-enhancement scanning showed homogenous enhancement of the lesion with a CT value of approximately 75-98 HU.

Pathological examinations-macroscopic examination

Left testis.

Testicular tissue measuring 4.7cm × 4.1cm × 2.6cm with intact surface membrane, gray-white. Multiple nodules were palpated on incision, ranging from 0.4cm to 2.3cm in maximum diameter. Nodules appeared solid, gray-white, and gray-yellow on sectioning, with partial clear boundaries and fine texture without accumulated membrane. Epididymal tissue measuring 1.5cm × 1.1cm × 1.1cm with a gray-red and gray-yellow cut surface and a moderate texture. The diameter of the cut end of the spermatic cord was 0.8cm.

Right testis

Testicular tissue measuring 4.7cm × 4.3cm × 2.8cm with intact surface membrane, gray-white. Multiple nodules were felt on the incision, with the largest measuring 0.6cm. Nodules appeared similar to the left testis in color, texture, and structure. Epididymal tissue measuring 1.5cm × 1.3cm × 1.2cm with a gray-red and gray-yellow cut surface, moderate texture, and a cut end of the spermatic cord with a diameter of 0.8cm.

Several small tissue blocks, collectively measuring 1.3cm × 1.2cm × 0.5cm, are gray-white with a moderate texture.

One nodule measuring 2.2cm × 1.2cm × 1cm with a gray-white and gray-yellow cut surface and a moderate texture.

Pathological Examinations-microscopic Examination

Low-power microscopy revealed multifocal nodular distribution without capsules within the testicular parenchyma. High-power microscopy indicated spindle cell changes within an inflammatory background resembling early juvenile xanthogranuloma or chronic inflammation. Spindle or plump spindle-like cells showed tissue cell-like appearances arranged in bundles, crisscross patterns, and whirls. Small nucleoli were visible with fine chromatin, without significant cellular atypia, rare mitotic figures, and interstitial infiltration of lymphocytes, plasma cells, and histiocytes ( Figures 1C, D ).

Morphologically similar changes are observed in chronic inflammation, xanthomatous, and granulomatous lesions. Spindle cells and foam-like tissue cell proliferation with enlarged cellular volume, irregularly folded or lobulated nuclei, fine chromatin, small nucleoli (some containing 1-4 nucleoli), rich eosinophilic cytoplasm, occasionally engulfing lymphocytes, standard cells, red blood cells, or hemosiderin. No coagulative necrosis or multinucleated giant cells were observed.

Similar morphology to the testis and bladder, occasionally showing mixed multinucleated giant cells but lacking Touton giant cells.

Immunohistochemistry

Positive antibodies: CD68, CD163, ALK1 ( Figure 1E ) ALK (D5F3), Vimentin.

Negative antibodies: CK (AE1/AE3), P40, EMA, S-100, SOX-10, SMA, Myogenin, Desmin, CD1a, CD4, CD30, CD3, CD20, CD63, FXIIIa, Ki67 proliferation index (5%-10%).

Negative staining for CD34, STAT6, INI-1, CD99, CD10, SSTR2. Special stains (PAS, PAM) did not reveal any pathogens.

TB gene detection was negative.

Genetic testing using fluorescence in situ hybridization (FISH) showed ALK gene separation signals in lesions of the testis, bladder, and penis ( Figure 1F ).

Second-generation gene sequencing (RNA sequencing) revealed fusion genes: KIF5B(NM_004521); ALK(NM_004304) in lesions of the testis, bladder, and penis

The final pathological diagnosis was confirmed as ALK-HSs. Follow-up for 30 months showed no metastasis or recurrence.

ALK-HSs refers to tissue cell lesions associated with ALK gene rearrangement, first reported by Chan JK et al. in 2008 ( 4 ). It presents clinically as solitary, multiple, or systemic (involving multiple sites or organs), more commonly affecting individuals of Chinese and Caucasian descent ( 5 ). The age of onset varies, ranging from 2 months to 50 years for individuals with solitary lesions, and those with systemic manifestations, it ranges from neonates to 40 years old. About 70% of patients develop symptoms before the age of 2. In reviewing the literature, there’s no significant gender difference in ALK-HSs occurrence. The male-to-female ratio in solitary cases is 3:2, with 2 cases of unspecified gender. In systemic cases, the gender ratio is 5:5. Solitary ALK-HSs lesions can occur in the breast, skin, liver, spleen, appendix, and sinuses. Systemic manifestations involve the skin, lungs, liver, spleen, kidneys, intestines, bones, and central nervous system within the skull ( 2 , 3 , 5 – 7 ). The clinical manifestations include pallor, hepatosplenomegaly, anemia, and decreased platelets without fever or decreased white blood cells ( 2 , 3 , 5 – 7 ). The lesions progressively enlarge with well-defined borders and are generally non-painful. Imaging studies help detect masses in multiple locations.

Its diagnosis requires a combination of clinical imaging, pathology, and ancillary tests (including immunohistochemistry and necessary genetic testing). Pathologically, ALK-HSs in the liver, spleen, lymph nodes, and skin tissues present similarly, with infiltrated cells in the hepatic sinusoids. These cells have irregularly folded or lobulated nuclei, fine chromatin, and small nucleoli, occasionally containing 2 to 4 nucleoli. The cytoplasm is rich and eosinophilic, with vacuoles visible; occasionally, they engulf lymphocytes, normal cells, red blood cells, or hemosiderin. The skin resembles early childhood xanthogranuloma or chronic inflammation, mainly composed of non-lipid cells. The lesion cells resemble those found in the liver but occasionally mix with multinucleated giant cells with wreath-like nuclei, lacking Touton giant cell features. Some morphological features mimic xanthoma, chronic inflammation, and granulomatous presentations, showing an inflammatory background, histiocytes, or foam-like tissue cell proliferation. Careful observation of tissue cell features and necessary immunohistochemistry and special staining are needed for diagnosis. Bladder pathology, in this case, shows inflammatory features with numerous foam-like tissue cells, making it prone to misdiagnosis or oversight. Electron microscopy reveals short cell projections, rich cytoplasm containing mitochondria, rough endoplasmic reticulum, ribosomes, lysosomes, and phagolysosomes without Birbeck granules. There is no ultrastructural evidence of metabolic disorders. Immunohistochemistry shows positivity for CD68, CD163, lysozyme, ALK1 (membrane/cytoplasmic), focal expression of CD45RB, low Ki67 (<2%); negativity for CD20, CD3, S100, CD30, CD1a, and Langerin. Genetic testing differs between solitary and systemic presentations. Solitary cases mainly exhibit the ALK1-KIF5B gene fusion mutation, reported in 6 cases out of 6 tested; systemic cases show ALK gene mutations: ALK-TPM3 (1/8), ALK-KIF5B (5/8), and ALK-COLIA2 (1/8) ( 2 , 3 , 5 – 7 ).

ALK-HSs need to be differentiated from the following tumors. The first one is epithelioid fibrous histiocytoma, where the lesion is within the dermis, with a well-defined tumor border, often presenting as semi-circular or polypoid; tumor cells are often arranged in a nested pattern, with vacuolated nuclei, small eosinophilic nucleoli, rich eosinophilic cytoplasm, and eosinophilia; collagenization can occur in the stroma; a few cases exhibit whirl-like growth patterns; some may display binucleation or multinucleation, nuclear grooves, pseudo-inclusions, or clear cytoplasm, resembling epithelioid endothelial cell tumor-like or melanocytic lesions. Immunohistochemistry reveals tumor cell positivity for CD30, CD68, CD163, ALK1, ALK (D5F3), and EMA; Genetic testing shows ALK gene rearrangement in tumor cells but with different ALK fusion partners, mainly SQSTM1 (52%) and VCL (30%). In contrast, other rare fusion types include DCTN1, ETV6, PPFIBP1, and SPECC1L ( 8 ). The second one is juvenile xanthogranuloma, which is common in children and adolescents, and often occurs in the skin. It has characteristic xanthoma histiocytes, foam histiocytes and Touton giant cells, and can also be mixed with other types of cells, including epithelioid cells, oval cells, spindle cells and eosinophilic histiocytes. The cells have no polymorphism and nuclear division. There are different numbers of lymphocytes, eosinophils, plasma cells and neutrophils in the background. Immunohistochemistry: histiocytes are positive for CD68, CD163, CD4, CD14, factor XIIIa and fasin, negative for CD1a and Langerin, and mostly negative for ALK. The third is Langerhans cell histiocytosis, characterized by infiltration of spindle and histiocyte-like cells, nodular or patchy distribution, eosinophilic or pale-stained cytoplasm, rich cytoplasm, visible nuclear grooves, and one to multiple small nucleoli. The stroma often presents with eosinophil infiltration, possibly accompanied by lymphocyte and plasma cell infiltration. Immunohistochemistry shows positivity for S100, CD1a, and Langerin in the tumor cells; genetic testing reveals BRAF gene mutations in more than 50% cases. The fourth one is Rosai-Dorfman disease. Pathologically, the lesions exhibit alternating areas of cellular sparse and dense regions. In the cellular sparse areas, scattered, larger, lightly stained histiocytes with abundant cytoplasm containing phagocytized material and lymphocytes showing ‘emperipolesis.’ The cellular dense areas display infiltrations of inflammatory cells such as lymphocytes and plasma cells. Immunohistochemistry reveals CD68, CD163, lysozyme, and CyclinD1 expression in the tumor cells. The fifth one is Erdheim-Chester disease, which mainly occurs in bones and occasionally outside bones. Pathologically, the predominant feature is infiltration of histiocytes, exhibiting large cell volumes with foamy cytoplasm containing ‘lipid-like’ substances. The cell nuclei show condensation and many lack engulfed lymphocytes. Multinucleated giant cells may be present, with a lymphocyte and plasma cell infiltration background. Immunohistochemistry reveals expression of CD68, CD163, and BRAF gene mutations detected in 54% to 100% of cases ( 9 ). The sixth one is an inflammatory myofibroblastic tumor in the thoracic and abdominal cavities and soft tissues, formerly known as an inflammatory pseudotumor. Pathologically, it shows spindle cell tumor-like proliferation in an inflammatory background, primarily composed of myofibroblasts and fibroblasts. Immunohistochemistry reveals expression of myogenic markers in the spindle cells, such as Actin, SMA, Caldesmon, and Desmin, and approximately half of the cases show detectable ALK protein expression and gene rearrangement. Other differential diagnoses include chronic inflammation, vascular-type fibrous histiocytoma, ALK-positive large B-cell lymphoma, and ALK-positive anaplastic large-cell lymphoma.

Regarding its treatment and prognosis, limited reported cases and research data are available for ALK-HSs. Published articles have mentioned various treatment methods, including watchful waiting, surgical excision, chemotherapy (using dexamethasone and etoposide), and ALK-targeted therapy. Multiple or systemic presentations often require chemotherapy. Monitoring blood counts and liver-spleen condition during treatment is essential, and normalization of liver-spleen size suggests positive treatment outcomes. Prognosis is generally better for multiple lesions, while systemic presentations vary depending on the affected sites. Reviewing literature cases with follow-ups ranging from 2 to 14 years, one case resulted in death, one survived the disease, and the rest showed no abnormalities or disease recurrence ( 2 , 3 , 5 – 7 ). Recently, some scholars have successively reported isolated cases, raising whether they are related to systemic ALK-HSs, which requires further investigation ( 10 ).

Conclusions

ALK-HSs belong to rare proliferative disorders of tissue cells, with systemic ALK-HSs presenting involvement in multiple systems. Solitary ALK-HSs generally have a favorable prognosis, and some may regress spontaneously. However, systemic ALK-HSs progress rapidly, with some cases having a poorer prognosis. Clinical management necessitates appropriate treatment strategies such as surgery combined with chemotherapy and ALK inhibitors for targeted therapy. Basically, multiple ALK-HSs and systemic ALK-HSs are two clinically and prognostically different conditions.

Data availability statement

The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding author.

Ethics statement

The studies involving humans were approved by Ethics Committee of Fujian Provincial Hospital. The studies were conducted in accordance with the local legislation and institutional requirements. The participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.

Author contributions

YW: Conceptualization, Supervision, Writing – original draft, Writing – review & editing. DL: Data curation, Investigation, Writing – review & editing. RZ: Data curation, Formal analysis, Investigation, Writing – review & editing. XC: Formal analysis, Validation, Writing – review & editing. LL: Data curation, Formal analysis, Writing – review & editing. HH: Conceptualization, Investigation, Writing – review & editing.

The author(s) declare financial support was received for the research, authorship, and/or publication of this article. This research was sponsored by Natural Science Foundation of Fujian Province (grant number: 2022J05211, RCZ), Fund for Fujian Provincial Natural Science Foundation (2022J011010, LZL) and Fund for Fujian Provincial Department of Finance (20238340, LZL).

Acknowledgments

We appreciate the patient’s participation in this study.

Conflict of interest

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Publisher’s note

All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.

1. WHO. Classification of Tumours . (2022). Available at: https://tumourclassification.iarc.who.int/chapters/63 [Accessed May 31, 2023].

Google Scholar

2. He Q, Zhang W, Li Q. Failure of crizotinib based systemic treatment in ALK positive histiocytosis involving the central nervous system: a case report and literature review. BMC Pediatr . (2022) 22:308. doi: 10.1186/s12887-022-03368-1

PubMed Abstract | CrossRef Full Text | Google Scholar

3. Liu W, Liu HJ, Wang WY, Tang Y, Zhao S, Zhang WY, et al. Multisystem ALK-positive histiocytosis: a multi-case study and literature review. Orphanet J Rare Dis . (2023) 18:53. doi: 10.1186/s13023-023-02649-x

4. Kemps PG, Picarsic J, Durham BH, Hélias-Rodzewicz Z, Hiemcke-Jiwa L, van den Bos C, et al. ALK-positive histiocytosis: a new clinicopathologic spectrum highlighting neurologic involvement and responses to ALK inhibition. Blood . (2022) 139:256–80. doi: 10.1182/blood.2021013338

5. Chan JK, Lamant L, Algar E, Delsol G, Tsang WY, Lee KC, et al. ALK+ histiocytosis: a novel type of systemic histiocytic proliferative disorder of early infancy. Blood . (2008) 112:2965–8. doi: 10.1182/blood-2008-03-147017

6. Huang H, Gheorghe G, North PE, Suchi M. Expanding the phenotype of ALK-positive histiocytosis: A report of 2 cases. Pediatr Dev Pathol . (2018) 21:449–55. doi: 10.1177/1093526617740784

7. Chang KTE, Tay AZE, Kuick CH, Chen H, Algar E, Taubenheim N, et al. ALK-positive histiocytosis: an expanded clinicopathologic spectrum and frequent presence of KIF5B-ALK fusion. Mod Pathol . (2019) 32:598–608. doi: 10.1038/s41379-018-0168-6

8. Creytens D, Ferdinande L, Van Dorpe J. ALK rearrangement and overexpression in an unusual cutaneous epithelioid tumor with a peculiar whorled “Perineurioma-like” Growth pattern: epithelioid fibrous histiocytoma. Appl Immunohistochem Mol Morphol . (2017) 25:e46–8. doi: 10.1097/PAI.0000000000000418

9. Huang HJ, Zhong DR. Clinicopathological and molecular features of Erdheim-Chester disease accompanied with Langerhans cell histiocytosis. Zhonghua Bing li xue za zhi= Chin J Pathol . (2019) 48:220–4:3. doi: 10.3760/cma.j.issn.0529-5807.2019.03.010

CrossRef Full Text | Google Scholar

10. Kurita A, Yoshida M, Murata T, Yoshida A, Uchiyama N, Takayama S. A case of ALK-positive histiocytosis with multiple lesions in the unilateral breast: A case report. Int J Surg Case Rep . (2022) 97:107435. doi: 10.1016/j.ijscr.2022.107435

Keywords: ALK-positive histiocytosis, ALK, male genitourinary system, KIF5B, rare disease

Citation: Wei Y, Zhang R, Lin D, Chen X, Li L and Huang H (2024) Case report: Systemic presentation of ALK-positive Histiocytosis. Front. Oncol. 14:1366766. doi: 10.3389/fonc.2024.1366766

Received: 07 January 2024; Accepted: 05 April 2024; Published: 19 April 2024.

Reviewed by:

Copyright © 2024 Wei, Zhang, Lin, Chen, Li and Huang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY) . The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

*Correspondence: Haijian Huang, [email protected] ; Lizhi Li, [email protected]

† These authors have contributed equally to this work

Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.

‘Planet Hip Hop’: Senegalese rappers push for social and political change

This summer, we are taking you on a global journey to celebrate the 50th anniversary of hip-hop. In Senegal, hip-hop has evolved from something of a fad, to an influential force for social change.

• By Tibisay Zea

Senegalese rap artist “Paco Pat Ghetto.”

Pape Aly Guéye — also known as “Paco Pat Ghetto” — remembers when he first heard a hip-hop song on the radio, back in the 1980s.

“Yeah, absolutely. It was ‘Sama Yaye,’ one of the first raps ever recorded in Senegal, by Mbacke Dioum [Backé Chium],” said the 47-year-old rapper.

At that time, hip-hop was starting to boom in poor and working class areas, including Guediawaye, the suburban city where Paco Pat Ghetto grew up. 

“This place was just a neglected area. I witnessed a lot of violence, you know, here when I was a teen, I saw people doing a lot of violence, gangbanging, I saw people who got killed, and then I was like: ‘I want to say something.’”

He said hip-hop captivated him, because he could tell serious stories in a fun way, and everyone around him seemed to like it. 

Paco Pat Ghetto and some of his friends started a hip-hop group. They called it Pat Ghetto , and they wrote about the violence and insecurity all around them. 

The group later became a pioneer of a hip-hop movement in the capital, Dakar, that demanded social and political change.

“Hip-hop over here is always talking about politics, economy and social change. This is what hip-hop is all about mostly, in Senegal,” Paco said. 

But this wasn’t always the case with Senegalese hip-hop.

Unlike in the US or Europe, where hip-hop emerged from poor areas, in Senegal, it was the rich who first adopted the musical genre and lifestyle.

Bamba Ndiaye, a Senegalese hip-hop researcher at Emory University, explained that hip-hop arrived in Dakar through middle- and upper-class citizens who were able to travel abroad to places like New York in the US and France.

“And they used to come back with videocassettes and audio cassettes, and had access to cable television and music channels like MTV,” he said. 

“At first, in the late ’80s, early ’90s, you had, for instance, hip-hop music that tends to focus more on love, you know, ego-tripping,” he added.

One example is the group Positive Black Soul (PBS), the first Senegalese hip-hop group to get international attention. 

In the early days, Senegalese artists rapped mostly in French. But when they started switching to Wolof — the most common local language in Senegal — more people started listening, and using it to tell their own tales, Ndiaye said.

“The politically engaged hip-hop started when youth from working class neighborhoods made hip-hop also a way of expressing discontent, but also criticizing the regime that was in place, the socialist regime. 

That was the ruling party in Senegal since they got their independence from France in the 1960s, until the year 2000. During this period, several hip-hop artists got censored, arrested and even beaten up for speaking out against authorities. 

But that didn’t prevent Senegalese rappers from engaging in political critique. 

Back in 2011, there was an anti-government movement called the Y’en a Marre , or “I’m Fed Up,” that was led by rappers and journalists to encourage young people to vote. 

It was during that time when rappers Xuman and Keyti came up with the idea of rapping the news.

For several years, every week, they would take hard news, put it to a beat and mix it with a little bit of humor.

Xuman would rap the news in Wolof, and Keyti would do it in French, to imitate the way news is delivered in TV newscasts in Senegal. 

“We wanted to bridge the gap between young people and the news, because, many times, they miss what’s really happening in the country,” Xuman said. “And sometimes the government is signing new laws to change people’s lives. And people don’t even know that, you know, their life is about to change.”

His journal Rappé was so successful that it was copied in other countries abroad.

“For me, that’s an achievement, because back in the day, we used to copy American hip-hop, the American concept and, you know, it happened that now people are copying our concept,” he said. 

Xuman is not rapping the weekly news anymore, but he’s still engaged in politics. In early June, when protests erupted in Dakar, he launched a new single about police brutality and democracy.

Many rappers in Senegal are still asking questions about justice, equity and Africa’s place in the world. 

At G-Hip Hop, a youth development center in Dakar founded by Paco Pat Ghetto, students learn about songwriting, rapping, break dancing, sound engineering and music business. 

But they also take courses on civic engagement. “We teach about democracy, accountability, freedom of expression and good governance,” Paco Pat Ghetto said.  

The walls of G-Hop Hop are emblazoned with paintings of African activists, such as Thomas Sankara, Nelson Mandela and Winnie Mandela.

“These people were fighting for freedom and liberties, and we think we can do it too, on a local scale,” Paco Pat Ghetto said.

At his center, he hopes to inspire another generation of young Senegalese, like 21-year-old Rash Ga Guru, who was graduating from the program. 

“Hip-hop saved me from the drug dealers,” he said. “I grew up around my big brothers who were dealing drugs, but hip-hop saved me from all that. It gave me the courage to be who I wanted to be.”

Editor’s note:  Bamba Ndiaye contributed to this story.

Sign up for The Top of the World, delivered to your inbox every weekday morning.

Accusing a pop superstar of sex trafficking: What R. Kelly case tells us about Sean ‘Diddy’ Combs

• Show more sharing options
• Copy Link URL Copied!

Disgraced R&B singer R. Kelly was once worth hundreds of millions of dollars but is now serving what amounts to a life sentence in federal prison.

After decades of sex abuse allegations and an acquittal on child pornography charges , a documentary series titled “Surviving R. Kelly” finally gave his accusers a voice and helped bring down the singer . Within six months of its airing, Kelly was facing federal prosecution in New York.

He was convicted not only of sex trafficking but also of racketeering — charges that specify a person’s “enterprise” was used to carry out criminal conduct.

Sean “Diddy” Combs now faces a similar federal investigation, though the accusations against him are significantly different and it remains unclear whether they will result in criminal charges.

Behind the calamitous fall of hip-hop mogul Sean ‘Diddy’ Combs

In the wake of multiple lawsuits filed against him, former members of Combs’ inner circle told The Times that his alleged misconduct against women goes back decades.

Dec. 13, 2023

Authorities have said little about the probe. But law enforcement sources have confirmed to The Times that Combs is under investigation for sex-trafficking tied at least in part to civil lawsuits filed by several women who have accused him of misconduct.

Combs has denied any wrongdoing, and his attorneys have slammed the investigation as unwarranted.

After federal agents searched the artist’s homes in Florida and Los Angeles several weeks ago, his attorney decried a “premature rush to judgment of Mr. Combs” and said the investigation “is nothing more than a witch hunt based on meritless accusations made in civil lawsuits.”

Still, previous high-profile sex-trafficking cases could offer a window into how the feds typically build a case and can provide clues into what officials would need to bring charges.

“The playbook for these types of cases is R. Kelly, Jeffrey Epstein , Larry Ray and NXIVM’s founder Keith Raniere ,” said Elizabeth Geddes, who delivered a six-hour closing argument in Kelly’s conviction.

Entertainment & Arts

What to know about the Sean ‘Diddy’ Combs lawsuits, raids

Music mogul and rapper Sean ‘Diddy’ Combs, who’s facing multiple lawsuits, is the subject of a sweeping sex trafficking probe that resulted in a federal raid of his L.A. and Miami homes.

March 28, 2024

In November, Combs’ former girlfriend Casandra Ventura, the singer known as Cassie, accused him in a lawsuit of rape . Within a day , he settled.

Since then, three other women have sued Combs, accusing him of rape, sex trafficking, assault and other abuses. One of the allegations involved a minor. A male producer also has sued him over unwanted sexual contact .

Geddes, who is not involved in the Combs case, said she believes Ventura might have been the trigger for the federal investigation.

She said the docuseries about Kelly spurred the Eastern District of New York to act — and that type of high-level investigation often requires an outside catalyst. In Kelly’s case, he had been acquitted in 2008 and as a result, many of his accusers lost confidence in law enforcement. But the documentary re-engaged authorities.

“Nothing puts pressure on law enforcement like a front-page story on the major newspaper in the city,” Geddes said.

Combs’ investigation, led by Homeland Security , is several months old, according to sources, and many connected to the case — including accusers and alleged witnesses — have already been interviewed.

Geddes said Homeland Security Investigations also worked the Kelly case, and its agents tend to have years of experience working with sex-trafficking victims.

She said sex trafficking requires either “force, fraud or coercion to cause a person to engage in a commercial sex act” or the trafficking of minors under 18.

“There is no statute of limitations,” Geddes said, and the key law enacted in the 2000s applies to acts from 2001 forward.

A timeline of allegations against Sean ‘Diddy’ Combs

Hip-hop mogul and entrepreneur Diddy has been accused of physical and sexual violence dating back to 1990. Here’s a timeline of the allegations.

March 26, 2024

Geddes said that in addition to the sex charges against Kelly, she and her colleagues secured a racketeering indictment against the singer. The charge has famously been applied to mob bosses like John Gotti and James “Whitey” Bulger .

In racketeering cases, Geddes said, the “enterprise” carries out illegal conduct and prosecutors seek to show a broader pattern of conduct that stretches over years and involves many participants. A racketeering case also allows multiple victims’ narratives in one trial.

Racketeering became a federal crime in 1970 under the Racketeer Influenced and Corrupt Organizations Act, or RICO.

Over the years, its usage has expanded. It often is used against gangs, ranging from the Mexican Mafia to South L.A.’s Crips . Racketeering cases also have been brought against rappers associated with street gangs, including Young Thug , Kay Flock, Casanova, and Fetty Wap.

Federal prosecutors have succeeded in racketeering convictions not only against Kelly, but also against other sex traffickers, including NXIVM founder Raniere and Larry Ray, whose crimes were outlined in the docuseries “Stolen Youth: Inside the Cult at Sarah Lawrence.”

But it is unclear what evidence the feds have against Combs and whether there is enough to bring charges.

Few details are available, other than sources saying investigators left his two homes with electronics, data devices and other records.

Legal experts have told The Times that evidence in sex-trafficking cases must be extensive as such charges can be hard to prove.

“Sex trafficking for adults usually involves some sort of coercion or other restraints,” L.A. defense attorney Dmitry Gorin said. Prosecutors would need to show you “encouraged somebody to engage in sexual activity for money or some other inducement.”

Aaron Dyer, one of Combs’ lawyers, stressed in a statement released after the raids that “there has been no finding of criminal or civil liability with any of these allegations.”

The mother of Combs’ son Justin Dior Combs also slammed the investigation and the raids.

“The overzealous and overtly militarized force used against my sons Justin and Christian is deplorable,” designer Misa Hylton said after releasing video showing federal agents dressed in military gear pointing a gun at Combs’ sons. “If these were the sons of a non-Black celebrity, they would not have been handled with the same aggression. The attempt to humiliate and terrorize these innocent young Black men is despicable!”

Feds want Sean ‘Diddy’ Combs’ communications, flight records in sex-trafficking probe

Federal investigators are seeking records from Combs’ private charter jet firm, cellphone providers and computer companies.

March 29, 2024

Federal sex-trafficking and sexual assault laws also allow prosecutors to present evidence that shows a modus operandi.

“In the R. Kelly trial, several women testified about what Kelly did to them as part of a pattern of behavior. It is very much the same thing people saw in Harvey Weinstein’s prosecution ,” Geddes said.

If prosecutors do file charges against Combs, they also could allege the use of forced labor under threat, Geddes said. Ventura, Combs’ former girlfriend, alleged she was forced into sex acts with other men and suffered physical harm for complaining. If true, this could be considered forced labor, Geddes said.

Singer R. Kelly sentenced to 30 years in prison in sex trafficking, racketeering case

Disgraced R&B star R.

June 29, 2022

Kelly was convicted of eight counts of the Mann Act, which was passed in 1910 and sought to criminalize what’s now known as human trafficking. The law initially banned transporting a woman or girl across state lines “for the purpose of prostitution or debauchery, or for any other immoral purpose.”

The Mann Act now covers transportation across state lines “with [the] intent that such individual engages in prostitution, or in any sexual activity for which any person can be charged with a criminal offense.”

In the allegations against Combs, one woman said she was brought from Detroit as a 17-year-old to Combs’ studios so he could rape her along with his cohorts, Geddes said.

Inside the Sean ‘Diddy’ Combs’ raids: Emptied safes, dismantled electronics, gun-toting feds

Legal experts said investigators would likely seek authorization to ‘search for videos or photographs on any devices connected to the target.’

Before the highly publicized searches of Combs’ properties were executed, Geddes said, prosecutors and HSI agents had to “have made some headway into the investigations.”

“What we can say at this stage is there was enough probable cause to convince a magistrate to issue a search warrant,” she said. “Before getting such a warrant, agents have typically interviewed multiple witnesses.”

Geddes said those types of searches typically seek corroboration of evidence because high-profile individuals tend to work with others to commit such crimes. In Kelly’s case, Geddes said, his storage facility proved to be a goldmine. He kept message slips, handwritten notations and emails to pick up women and girls. And there were “videos, lots of videos,” she said.

“We had so much evidence presented in Kelly, it was hard to fit it all into the closing,” Geddes said. “He used his money and public persona to hide his crimes in plain sight,” she told jurors at the time.

More to Read

Sean Combs’ son Christian accused of sexual assault on yacht that Diddy chartered

April 5, 2024

Sean ‘Diddy’ Combs’ ex slams feds, posts dramatic video of L.A. raid where his sons were detained

April 2, 2024

Diddy returns to Instagram, amid federal probe, to celebrate Easter with youngest daughter

April 1, 2024

Start your day right

Sign up for Essential California for news, features and recommendations from the L.A. Times and beyond in your inbox six days a week.

You may occasionally receive promotional content from the Los Angeles Times.

Richard Winton is an investigative crime writer for the Los Angeles Times and part of the team that won the Pulitzer Prize for public service in 2011. Known as @lacrimes on Twitter, during almost 30 years at The Times he also has been part of the breaking news staff that won Pulitzers in 1998, 2004 and 2016.

More From the Los Angeles Times

Body of unidentified man washes ashore on ventura county beach.

Topanga Canyon could remain closed into the fall after massive landslide

A celebrated L.A. astrology influencer’s stunning fall from ‘healer’ to solar eclipse killer

Bad tattoo? Compete for fresh ink with a pic of your pooch

April 19, 2024

An official website of the United States government

The .gov means it’s official. Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

The site is secure. The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

• Publications
• Account settings

Preview improvements coming to the PMC website in October 2024. Learn More or Try it out now .

• Advanced Search
• Journal List
• Trauma Case Rep
• v.32; 2021 Apr

Recurrent traumatic hip dislocation in an 8-year-old boy

Pediatric traumatic hip dislocations (PTHD) are rare and represent around 5% of all pediatric dislocations. Associated bony or soft tissue injury can occur as often as 17%–25% of the time. We report a case of an 8-year-old boy presenting a posterior hip dislocation after a low-energy trauma, which was initially managed with closed reduction and bed rest for 1 week. Two days after hospital discharge, he suffered a recurrent posterior hip dislocation. He was now managed with 4 weeks of bed rest and lower limb skin traction followed by 1 week of no weight-bearing on crutches. With 6 months of follow-up, he is asymptomatic, walking autonomously, with complete and painless range of motion of the affected hip and no major radiographic changes. Pediatric traumatic hip dislocation is a rare and challenging injury that should be managed promptly. Currently, there is no protocol concerning treatment in the literature and its largely dependent of patient and parents' cooperation.

Introduction

Pediatric traumatic hip dislocations (PTHD) are rare and represent around 5% of all pediatric dislocations [ [1] , [2] , [3] ]. PTHD are usually posterior and are generally consequence of high-energy injuries [ [1] , [2] , [3] ]. It can also be associated with minor trauma, especially in children younger than 10 years-old, as a result of more flexible joint structures of the immature hip [ [1] , [2] , [3] , [4] ]. Associated ipsilateral fractures (as acetabular and femoral head fractures) can occur as often as 17% and bony or soft tissue entrapment may occur up to 25% of the time [ 1 , 2 ].

Case presentation

We present an 8-year-old boy who suffered a ground-level fall on his knees. As previous medical history he had an elevated body mass index (27 Kg/m 2 ). He presented to our emergency department with pain on his left hip and inability to bear weight. It was evident a limb length discrepancy, with his left lower limb in flexion, abduction and external rotation ( Fig. 1 ). No other injury, including neurovascular, was detected. Anteroposterior radiograph ( Fig. 2 ) showed a posterior hip dislocation, further confirmed with fluoroscopy later on ( Fig. 3 ).

Clinical appearance at presentation.

Anteroposterior radiograph at presentation.

Fluoroscopy before reduction of the dislocation.

Four hours since initial trauma, he was submitted to closed reduction under sedation and fluoroscopy, followed by testing of hip stability. Post-reduction radiographs showed a concentric reduction ( Fig. 4 A and B). After 48 h of bed rest, he tolerated walking with a hip orthosis and crutches without bearing weight. One week later he was discharged with indication for no weight-bearing on crutches while maintaining the hip orthosis.

Anteroposterior (A) and lateral (B) radiographs post-reduction.

He was brought to the hospital 2 days after discharge, for left hip pain while playing football. Once again, he was diagnosed with a posterior hip dislocation ( Fig. 5 ) and was emergently submitted to closed reduction under sedation. Post reduction radiographs showed concentric reduction ( Fig. 6 ). As it was an early recurrent dislocation, a magnetic resonance was done with evidence of an osteochondral avulsion of the posterior labrum and partial rupture of the posterior capsule, with no other evident acetabular or femoral head fractures ( Fig. 7 A, B, C and D). He was discharged after 4 weeks of bed rest and lower limb skin traction followed by 1 week of no weight-bearing on crutches.

Anteroposterior radiograph at the time of the second dislocation.

Anteroposterior radiograph post-reduction of the second dislocation.

Axial (A, B) and coronal (C, D) views of T2-STIR magnetic resonance imaging.

At 6 months of follow-up, he is asymptomatic, walking autonomously, with complete and painless range of motion of the left hip ( Fig. 8 A, B and C). Six months anteroposterior radiograph shows no apparent lesion ( Fig. 9 ). A control magnetic resonance was done at 6 months after initial injury, showing mild articular effusion and an apparently healed posterior labrum and acetabular wall ( Fig. 10 A and B).

Active range of motion (A, internal rotation; B, external rotation; C, abduction) at 6 months post-second dislocation.

Anteroposterior radiograph at 6 months post-second dislocation.

Coronal (A) and axial (B) views of T2-SPAIR magnetic resonance imaging.

PTHD are considered orthopedic emergencies, due to the possible disruption of the vascular supply to the proximal epiphyseal plate of the femur [ 2 ]. Performing reduction of PTHD in the first 6 h is extremely important [ 2 ]. Delayed diagnosis and reduction can lead to a significant increase of avascular necrosis and degenerative arthritis [ 2 , 5 ]. Concentric reduction is usually achieved by closed means, in approximately 85% of the cases [ 5 ]. Open reduction may be necessary if the dislocation is irreducible by closed means, if reduction is nonconcentric or the hip is unstable, and if there is an associated lesion that needs surgical repair [ 2 , 5 ].

Following successful closed reduction, it is suggested the use of spica cast with hip abduction and bed rest [ 2 , 3 ]. In older children, above 10 years-old, it can be used a hip bracing and protected weight-bearing on crutches [ 2 , 3 ]. PTHD complications are infrequent in children, except for recurrent dislocation [ 2 , 3 ]. It is usually consequence of a labrum or capsule defect and it is more commonly seen in children treated with short period immobilization and allowed for early ambulation [ 2 , 3 ]. However, literature it is not unanimous regarding the period of non-weight bearing that should be recommended after reduction [ 3 ].

If it is not possible to obtain a stable and concentric closed reduction, magnetic resonance is the study of choice [ [6] , [7] , [8] ]. This imaging technique is more appropriated for evaluating characteristic structural injuries, specially lesions of the posterior labrum and fractures of the posterior unossified acetabular wall [ 8 ]. Some authors advocate that if this labral pathology is left untreated it could lead to further pain and degeneration of the hip [ 1 , 2 ]. However, there are reports of spontaneous healing of large posterior labral detachments in children with a good outcome [ 7 ]. A recent study also described successful outcomes with arthroscopically assisted reduction without repair of the avulsed osteochondral labrum defect in incongruent hip joints after PTHD in children [ 9 ]. Therefore, the need for surgical repair of this type of lesion alone or while performing an open reduction is debatable.

In conclusion, PTHD is a rare and challenging injury that should be managed promptly. Currently, there is no protocol concerning treatment in the literature and its largely dependent of patient and parents' cooperation.