What Types of Femur Fractures Are Concerning for Possible Child Abuse?

Patient Presentation
A 6-month-old female was transferred to the emergency room of a children’s hospital for treatment of a femur fracture.
Her mother was carrying her down some stairs and fell landing on the infant.
The mother could not give more details of how she was carrying the infant before the fall, how many stairs she fell down,
nor the final position of the infant or herself.
The past medical history showed her to be a full-term infant who had received regular health supervision visits.
The family history was negative for genetic or skeletal disease.
The social history showed two other siblings living with their biological mother and father who are not married.
The mother denies any history of alcohol, drugs or violence.
The review of systems was negative.
The pertinent physical exam showed an alert infant in a leg immobilizer who smiles.
Vital signs were normal. Head circumference was 25%, weight and length could not be done accurately due to the immobilizer.
She appeared developmentally appropriate. Her examination was normal including head, eyes, abdomen, genitalia and skin.
The radiologic evaluation on the radiographs from the outside hospital showed a transverse femur fracture of the diaphysis that was angulated but not shortened.
The diagnosis of a transverse femur fracture was made.
The patient’s clinical course was that she was taken to the operating room where a spica cast was placed.
The following day, an evaluation for possible child abuse was conducted and showed a normal head computed tomography examination, normal skeletal survey, normal ophthalmological evaluation and initial screening laboratories were negative.
The Department of Human Resources was contacted and discharged the infant to a temporary foster care placement along with her siblings.
The Department of Human Resources and the courts determined after further investigation that she had been abused.
She was later placed in permanent foster care and was awaiting adoption. Her femur fracture healed with no residual problems.

Figure 59 – An AP radiograph of the left femur demonstrates a complete fracture of the femoral diaphysis with minimal angulation and overriding of the fracture fragments.

Discussion
In the western world as early as 1860, Tardius studied child abuse in France but the findings had no impact on the medical community. In 1888, West described periosteal swelling in long bones. Caffey began studies of subdural hemorrhage and associated fractures from 1920-25, but it was his landmark article in the 1946 where he described multiple fractures in multiple bones in multiple stages of healing that is regarded as the one of the first studies to receive widespread attention by the medical community for child abuse.
In Caffey’s series, 5 of the 6 cases had femur fractures.

Stair falls are common occurrences in children but usually do not cause serious injury. Studies of stair falls show that they do not cause significant injury to more than 1 body region and typically do not cause proximal extremity and truncal injuries. Stair falls also cause few bruises even after falling down several stairs.

Stair falls are a common false history for child abuse.
In a prospective study (N=29) of femur fractures with a reported history of a stair fall as the mechanism of injury, Pierce et. al. used an injury plausibility model to evaluate stair falls as a plausible history versus stair falls as a suspicious history for child abuse.
In their model they used:

  • Caregiver history of the fall – could the caregiver describe the elements of the initial position, fall dynamics, and final position. A caregiver who could not give these or fewer elements was more suspicious
  • Fracture and the possible biomechanical mechanism – did the possible biomechanics match the type of fracture
  • Time to seeking care – immediate versus delayed with or without signs of injury. Delayed care with more obvious signs of injury was more suspicious
  • Additional findings or injuries on initial exam – more additional injuries increases suspicion

They pointed out in their study at that initially the caregiver histories did not seem unusual, but additional questioning often found more vague answers that were without detail, and that the fewer the specific elements that the caregiver could describe, the more suspicious the case was.
Spiral fractures in this study only occurred in walking patients and where the leg was reported to be folded or twisted under the patient’s body. If the leg was reported to be pinned under a caregiver’s body, the fractures were commonly transverse or oblique.
Immediately seeking care, or a delay with a subtle or well aligned fracture was not suspicous for child abuse. Any additional injuries to the child were suspicous for child abuse.

Fracture types from stair falls in this study were most commonly buckle or impact fractures (58.6%), transverse or short oblique (24.1%), spiral or long oblique (13.8%), and classic metaphyseal fracture and/or high impact fracture (3.4%)

Learning Point
Child abuse or non-accidental trauma is a clinical diagnosis. It is the constellation of history, physical examination, radiographic evaluation, laboratory testing and investigation that determines if child abuse has occurred.
Fractures can be a presenting symptom of abuse but they are also common problems not associated with abuse.
Femoral shaft fractures are estimated to account for only 1.6% of all fractures in children, therefore they are potentially more suspicous for child abuse.

Scherl et al report a retrospective study of 207 patients with all histories for injury who were 6 years of age or less and had a femur fracture.
Thirteen cases (6.3%) eventually were found to be caused by child abuse and the initially presenting histories were : 4 caused by a fall, 5 with no history of trauma, 1 caused by being hit by a falling object, 1 caused by being hit accidentally by another person and 1 record was incomplete.

Sclerl et. al. report of femur fracture types and causes. All numbers given are percentages.

		Overall	Known	Fall	Pedestrian	No Trauma	Motor
			Child		Struck by	History		Vehicle
			Abuse		Auto				Accident
Overall
Sample		100	6.3	43.5	30.4		9.3		4.7

Fracture Type
Transverse	38	36	33	48		40		NA
Spiral		27	36	37	11		35		NA
Oblique		17	7	14	18		10		NA
Not
characterized	14	21	13	17		15		NA
Other		3	0	3	6		0		NA
 

There is no particular pattern of femur fractures that is pathognomonic for child abuse, therefore all femur fractures could be suspicious. Several early studies found spiral fractures to be the most common fracture type, therefore many clinicians may still believe that spiral fracture are indicative of abuse. Spiral fractures may be caused by other mechanisms.
More recent reports such as the one above, show transverse fractures to be most common overall and more common in child abuse. Therefore clinicians should consider abuse potentially in any child with a femur fracture and in particular with transverse fractures which may be overlooked.

Questions for Further Discussion
1. What other body locations could be concerning for child abuse?
2. What laboratory evaluation should be done during an investigation for possible child abuse?
3. At what ages should a head computed tomography be considered as part of the investigation for possible child abuse?
4. Which fractures are considered radiographically pathognomic for child abuse?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Information prescriptions for patients can be found at MedlinePlus for these topics: Fractures and Child Abuse and at Pediatric Common Questions, Quick Answers for this topic: Fractures/Dislocations and Child Abuse

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Caffey J. Multiple Fractures in the Long Bones of Infants Suffering From Chronic Subdural Hematoma. AJR. 1946;36(2);163-173.

Scherl SA, Miller L, Lively N, Russinoff S, Sullivan CM, Tornetta P 3rd.
Accidental and nonaccidental femur fractures in children. Clin Orthop Relat Res. 2000 Jul;(376):96-105.

Pierce MC, Bertocci GE, Janosky JE, Aguel F, Deemer E, Moreland M, Boal DK, Garcia S, Herr S, Zuckerbraun N, Vogeley E.
Femur fractures resulting from stair falls among children: an injury plausibility model. Pediatrics. 2005 Jun;115(6):1712-22.

ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Interpersonal and Communication Skills
    19. The health professional works effectively with others as a member or leader of a health care team or other professional group.

  • Professionalism
    20. Respect, compassion, and integrity; a responsiveness to the needs of patients and society that supercedes self-interest; accountability to patients, society, and the profession; and a commitment to excellence and on-going professional development are demonstrated.

  • Systems Based Practice

    26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

    Date
    April 28, 2008

  • What is the Differential Diagnosis of Neonatal Unconjugated Hyperbilirubinemia?

    Patient Presentation
    A 3-day-old female came to clinic with jaundice.
    She was a 39 week infant born by spontaneous vaginal delivery without complications who was discharged on day 2 of life with a transcutaneous bilirubin of 10.0 mg/dL.
    She had been breastfeeding well, with several stools and many voids.
    The past medical history shows she was born to a G2 P2 caucasian mother with blood type B+ with normal screening maternal laboratories and no problems during pregnancy or delivery.
    Birth weight was 3080 grams.
    The family history was negative for hematological, gastrointestinal or genetic problems.
    The review of systems was negative.
    The pertinent physical exam showed her vital signs to be normal with a current weight of 2990 grams (10-25%).
    She was alert and active with no distress.
    She was jaundiced from her head to her upper thighs. She had scleral icterus. Her abdominal examination was normal with no hepatosplenomegaly.
    The laboratory evaluation showed a total bilirubin of 17.3 mg/dL and a direct bilirubin of 0.3 mg/dL.
    The diagnosis of of neonatal unconjugated hyperbilirubinemia was made and she was admitted for phototherapy.
    The patient’s clinical course showed her to improve after 24 hours of phototherapy and continuation of breastfeeding. She had many voids and stools during that time also.
    Her bilirubin decreased to 14.6 mg/dL with the phototherapy. Six hours after discontinuing the phototherapy, it had decreased to 13.4 mg/dL. She was discharged to home with routine followup.

    Discussion
    Bilirubin is a metabolite of heme degradation. Heme is oxidized to biliverdin which is then reduced to bilirubin. Bilirubin is then taken up into the liver and conjugated; conjugated bilirubin is then excreted through the gastrointestinal tract. Bilirubin can also be converted by a blue light at wavelength 450 nm into a water-soluble compound called lumirubin which is then excreted through the kidneys.
    Hyperbilirubinemia occurs because of an increase in hemoglobin metabolites, decreased hepatic uptake, decreased hepatic conjugation and/or decreased excretion.

    Unconjugated hyperbilirubinemia in the newborn is a normal occurance.
    After birth the infant must rely on its own relatively immature liver to detoxify metabolites, the infant’s gastrointestinal tract also is not yet working as well for excretion, the infant is usually slightly fluid deficient before breastfeeding and/or bottle feeding are well established, and there is increased breakdown of red blood cells as the fetus has a higher hemoglobin than an infant and thus an infant is relatively hemoconcentrated.
    If the infant is also further stressed because of intrapartum or post-partum complications such as hypoxia, infection, shock or other problems, the hyperbilirubinemia can be exacerbated.

    The total bilirubin rises from 1.5 mg/dL to 6.5 mg/dL (+ or – 2.5 mg/dL) over the first 3-4 days of life.
    Premature infants because of the relative immaturity of the liver usually have a higher maximum bilirubin that is ~30-50% more than full-term infants.
    Visible jaundice (the yellowish staining of the skin and mucous membranes) on day 1 of life or a rate of total bilirubin rising > 5.0 mg/dL/24 hours is not normal and requires evaluation.
    Evaluation for a possible pathological process usually includes: total bilirubin and indirect bilirubin (looking for total amounts and elevated unconjugated hyperbilirubinemia), complete blood count with blood smear (to look for evidence of hemolysis), reticulocyte count (to look for evidence of physiological response), direct Coombs test (to look for antibodies), and blood and Rh type (to compare for blood group incompatabilities).
    Other tests depending on the clinical situation may include an APT test, sepsis evaluation, Glucose-6-phosphate deficiency screening, hemoglobin electrophoresis, prothrombin time, partial thromboplastin time, and thyroid function testing.

    Treatment for hyperbilirubinemia may be necessary to prevent kernicterus or the staining of the basal ganglia, hippocampus and subthalamic nucleus of the brain. This in turn causes damage to the central nervous systems with signs of hyper- and hypo-tonia, seizures, abnormal deep tendon reflexes and other reflexes, developmental delay, cranial nerve deficits and movement abnormalities.
    The level of bilirubin to begin treatment depends on gestation age, chronological age in hours/days, total bilirubin level and other risk factors such as acidosis, asphyxia, hypoalbuminemia (<3.0 g/dL), Glucose-6-phosphate deficiency, isoimmune hemolytic disease, lethargy, sepsis, and temperature instability.
    Infants with lower weights are also at increased risk of hyperbilirubinemia requiring treatment.
    Phototherapy is used most frequently for treatment. After phototherapy is begun, total bilirubin is checked within 4-6 hours looking for a 1-2 mg/dL decline (with total amount always remaining below the threshold for exchange transfusion). Sometimes exchange transfusions are necessary if phototherapy is failing or even before a trial of phototherapy if the bilirubin is very high.

    Nomograms that assist in determining hyperbilirubinemia risk and possible treatment (phototherapy or exchange transfusion) are available from the American Academy of Pediatrics (see To Learn More below).

    Conjugated hyperbilirubinemia is defined as the direct bilirubin being > 2.0 mg/dL or > 10% of the total serum bilirubin.

    Learning Point
    Causes of unconjugated hyperbilirubinemia include:

    • Transient jaundice
      • Normal physiologic jaundice
      • Breast-feeding jaundice (usually occurs in first week of life)
      • Breast-milk jaundice (usually occurs in weeks 2-3 of life)
      • Reabsorption of extravascular blood – severe caput or cephalohematoma
      • Polycythemia – twin-to-twin transfusion
    • Gastrointestinal problems
      • Increased enterohepatic circulation secondary to intestinal obstruction
        • Cystic fibrosis
        • Hirschsprung disease
        • Ileal atresia
        • Pyloric stenosis
        • Other causes of intestinal obstruction
      • Bilirubin metabolism problems
        • Crigler-Najjar syndrome
        • Gilbert syndrome
        • Hypothyroidism
        • Hypoxia
        • Lucey-Driscoll syndrome
    • Hematological problems
      • ABO incompability
      • Rh incompatability
      • Clotting disorders
      • Autoimmune disease
      • Hemoglobinopathies
      • Microangiopathies
      • Red cell enzyme defects – Glucose-6-phosphate deficiency, pyruvate kinase deficiency
      • Red cell membrane defects – spherocytosis, elliptocytosis
    • Miscellaneous
      • Acidosis
      • Dehydration
      • Hypoalbuminemia
      • Infant of a diabetic mother
      • Medication
      • Sepsis
      • Swallowed maternal blood

    Questions for Further Discussion
    1. What are the advantages and disadvantages of using a transcutaneous bilimeter to measure bilirubin levels?
    2. What is the differential diagnosis of conjugated hyperbilirubinemia?
    3. When is supplemental breast milk, formula or intravenous fluids indicated as part of treatment for hyperbilirubinemia?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Jaundice and at Pediatric Common Questions, Quick Answers for this topic: Jaundice.

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    Bakerman P, Strausbauch P. Bakerman’s ABC’s of Interpretive Laboratory Data. 4th edit. Interpretive Laboratory Data, Scottsdale, AZ. 2002. pp.. 92-94.

    Rudolph CD, et.al. Rudolph’s Pediatrics. 21st edit. McGraw-Hill, New York, NY. 2003:164-169.

    American Academy of Pediatrics Clinical Practice Guideline. Management of Hyperbilirubinemia in the Newborn Infant 35 or More Weeks of Gestation. Pediatrics 2004:114;297-316. Available from the Internet at: http://aappolicy.aappublications.org/cgi/content/full/pediatrics;114/1/297 (rev. July 2004, cited 2/25/08).

    Robertson J, Shilkofski N. The Harriet Lane Handbook. 17th. Edit. Mosby Publications: St. Louis. 2005:314-317, 467-470.

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

    nsitivity and responsiveness to patients’ culture, age, gender, and disabilities are demonstrated.

  • Systems Based Practice
    24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

    Date
    April 21, 2008

  • What Are the Indications for a DXA Scan in Children?

    Patient Presentation
    A pediatrician provided inpatient hospital care to a school-age girl with steroid-dependent rheumatoid arthritis that had caused osteopenia and extremity fractures.
    She had had a dual-energy x-ray absorpiometry (DXA scan) which confirmed baseline osteopenia and was starting bisphosphonate treatment.
    Several months later, the same pediatrician was providing care to 3 month old male infant with fractures suspicious for non-accidental trauma.
    His mother wanted a ‘bone strength’ test done and was very unhappy when the pediatrician told her that it was not an indicated test because there was no normative data with which to interpret the test even if the test was done.
    The pediatrician also thought about a patient from the distant past who was treated for female athlete triad.
    The pediatrician could not remember if this athlete had a DXA scan but wondered what the indications for the test were.
    The pediatrian performed a computerized PUBMED search and found several primary research articles, position statements from professional organizations, and a couple of review articles on the general topic of bone mass in children.
    From these information sources she learned that although DXA scan and other similar tests there are often used in screening and treatment of adults with osteopenia or osteoporosis, because of lack of research data and many other factors that make children different from adults, there are fewer indications for screening and treatment with bone mineral density measurements in children and adolescents.
    In general, pediatric patients should have a DXA scan performed if they are at risk for osteopenia and then have a fracture.

    Discussion
    Peak bone mass, size and strength reaches a maximum by early adulthood. Factors important to overall bone health include weight bearing physical activity, hormonal balance, body mass, and nutrition.
    Causes of osteopenia (i.e. low bone mass) and/or pathological fractures in children include:

    • Genetic (60-80% are caused by a heritable problem)
      • Ehlers-Danlos
      • Fibrous dysplasia
      • Homocystinuria
      • Hypophosphatasia
      • Idiopathic hypercalciuria
      • Marfan’s syndrome
      • Menke’s kinky hair syndrome
      • Osteogenesis imperfecta
    • Endocrine
      • Glucocorticoid excess including steroid medication
      • Growth hormone deficiency
      • Hyperparathyroidism
      • Hyperthyroidism
      • Sex steroid deficiency or resistance
    • Chronic disease
      • Anorexia nervosa
      • Female athlete triad with amenorrhea
      • Celiac disease
      • Cystic fibrosis
      • Diabetes, type 1
      • Inflammatory bowel disease
      • Malignancy
      • Post-transplantation
      • Renal failure
      • Rheumatological diseases
      • Sickle cell anemia
      • Systemic lupus erythematosus
      • Thalassemia
    • Musculoskeletal/Neurological associated with immobilization
      • Cerebral palsy
      • Muscular dystrophy
      • Paraplegia
      • Spina bifida
    • Other
      • Idiopathic juvenile osteoporosis
      • Idiopathic scoliosis

    Several methods may be available for performing bone densitometry, but dual-energy x-ray absorpiometry (DXA scan) is the preferred method for children and adolescents because it is widely available, is quick to perform, precise, has better safety, and has the most normative data available.
    DXA alone does not make the diagnosis of osteoporosis – osteoporosis requires osteopenia and clinically significant fractures.
    Low bone mineral density should be diagnosed only when the Z-scores on the testing are less than or equal to 2 standard deviations below the mean when adjusted for age, gender and body size when appropriate.

    Learning Point
    According to the International Society for Clinical Densitometry, DXA scan in children and adolescents (ages 5-19 years) should be considered in:

    • Patients being considered for or before beginning therapeutic treatment for osteopenia or osteoporosis
    • Patients on therapeutic treatment being monitored (minimum 6 month intervals between measurements)
    • For fracture prediction, mainly in children with previous clinically significant fractures. Clinically significant fractures are defined as fracture of long bones in the lower extremities, vertebral compression fractures, or two or more long-bone fractures of the upper extremities.
      • Patients with primary bone diseases should be measured at fracture presentation
      • Patients with potential secondary diseases should be measured at fracture presentation
      • Patients with chronic immobilization should be measured at fracture presentation
      • Patient with thalassemia major should be measured at the earlier of either fracture presentation or at 10 years of age.

    Additional evaluation for children and adolescents at risk for osteopenia or osteoporosis should include a comprehensive nutritional and physical activity history, review of underlying disease and treatments, 25-hydroxy Vitamin D level (the storage form of Vitamin D), and laboratory testing of hormonal status.

    Questions for Further Discussion
    1. What local facilities provide bone densitometry for children and adolescents?
    2. Who are my local experts that could help me take care of a child or adolescent with an abnormal DXA scan?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Osteoporosis

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    Bachrach LK. Osteoporosis and measurement of bone mass in children and adolescents.
    Endocrinol Metab Clin North Am. 2005 Sep;34(3):521-35, vii.

    Bachrach LK. Assessing bone health in children: who to test and what does it mean?
    Pediatr Endocrinol Rev. 2005 Feb;2 Suppl 3:332-6.

    International Society for Clinical Densitometry. Official Positions.
    Available from the Internet at http://www.iscd.org/Visitors/positions/OfficialPositionsText.cfm (rev. 2007, cited 2/15/2008).

    ACGME Competencies Highlighted by Case

  • Patient Care

    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    6. Information technology to support patient care decisions and patient education is used.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    12. Evidence from scientific studies related to the patients’ health problems is located, appraised and assimilated.
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
    14. Knowledge of study designs and statistical methods to appraisal clinical studies and other information on diagnostic and therapeutic effectiveness is applied.
    15. Information technology to manage information, access on-line medical information and support the healthcare professional’s own education is used.

  • Professionalism
    20. Respect, compassion, and integrity; a responsiveness to the needs of patients and society that supercedes self-interest; accountability to patients, society, and the profession; and a commitment to excellence and on-going professional development are demonstrated.

  • Systems Based Practice
    23. Differing types of medical practice and delivery systems including methods of controlling health care costs and allocating resources are known.
    24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

    Date
    April 14, 2008

  • What Causes Hilar Adenopathy?

    Patient Presentation
    A 19-year-old female came to the inpatient ward of the regional children’s hospital for an evaluation of hilar adenopathy.
    Nine days prior to admission she developed a productive cough, rhinorrhea, shortness of breath and chest pain with deep inspiration.
    She said she felt warm but had no fever. She also denied fever, chills, nausea, vomiting, recent skin changes or weight loss.
    She was seen in the local emergency room on day 4 where screening labs showed elevated liver transaminases and an ultrasound of the liver and gallbladder were normal.
    A chest radiograph showed widened mediastinum with hilar adenopathy. She was given azithromycin for bronchitis and sent to followup with her local physician which she did 5 days later.
    At that time, she had an erythrocyte sedimentation rate of 100 mm/hour, and a C-reactive protein of 7.0 mg/dl. A tuberculin skin test with candidal control was placed at that time.
    The past medical history was positive for erythema nodosum diagnosed and fully evaluated 3 months previously by a rheumatologist including a tuberculin skin test and chest radiograph that were negative. No obvious cause for the erythema nodosum was found.
    She had no previous trauma, surgeries or significant illnesses and she had current immunizations.
    The family history was negative for rheumatologic or immunological diseases. There was coronary artery disease and diabetes.
    The social history showed she had a pet parrot for several years with no other animal contacts. She had lived in the Mississippi River area for the past 8 years.
    There was no travel history, nor history of contact with persons living or working in a prison, homeless or in the military. The rest of the family and friends were healthy.
    The review of systems was positive for some fatigue. She had no weight changes, night sweats, joint or skin problems.
    The pertinent physical exam showed a teenager in no acute distress. She was afebrile, with normal respirations and growth parameters were at 90%; unchanged from outside records.
    HEENT showed no obvious eye or other mucosal changes. Skin was normal. There were a few shotty anterior cervical lymph nodes that were < 0.5 centimeters.
    She had no supraclavicular, axillary, epitrochlear, popliteal or groin nodes palpable. Heart, lungs, abdomen and musculoskeletal examinations were normal.
    The working differential at that time was infectious (including coccidiomycosis, histoplasmosis, mycoplasma, pertussis, human immunodeficiency virus or other viral or bacterial etiology), immunological (including sarcoidosis or lupus), oncological (lymphoma) and other (Castleman’s syndrome).
    The radiologic evaluation included a chest computed tomography examination which showed pretracheal and paratracheal, right hilar, and subcarinal mediastinal lymphadenopathy.
    The work-up included consultations with pediatric pulmonary, rheumatology and oncology. An ophthalmological examination to look for evidence of lupus or other immunological diseases was negative.
    The laboratory evaluation during her 2 day admission, her complete blood count, electrolytes, thyroid stimulating hormone, liver function tests, urinalysis, complement 4 level, ANCA (anti-neutrophil cytoplasmic antibodies), ANA (anti-nuclear antibody), and tuberculin skin test were negative.
    A complement 3 level was slightly elevated and the control candidal intradermal skin test was positive. At the time of discharge the patient had almost complete resolution of her pulmonary symptoms and testing for coccidiomycosis, histoplasmosis, and blastomycosis were pending along with an ACE (angiotensin converting enzyme) and ENA (extractable nuclear antigens).
    The working diagnosis at discharge was probably histoplasmosis, but possible sarcoidosis, that would be evaluated more when the pending testing was completed.
    One week after discharge, the histoplasmosis antibody testing by immunodiffusion and complement fixation were positive but her urine antigen testing was negative. All other testing was negative.
    The diagnosis of histoplasmosis was made and the results were communicated to her primary care physician who would followup with the patient
    It was felt that her primary exposure occurred around the time she presented with erythema nodosum and the hilar adenopathy was now found incidentally on the chest radiograph.


    Figure 58 – Three axial images from a chest CT performed with intravenous contrast demonstrate pretracheal and paratracheal (top), right hilar (middle) and subcarinal (bottom) mediastinal lymphadenopathy. There was also right middle lobe and lower lobe airspace disease and a small right pleural effusion (not shown). The findings were felt to be compatible with Mycobacterium tubercululi or Histoplasma capsulatum infection or sarcoidosis.

    Discussion

    Histoplasmosis is caused by Histoplasma capsulatum and is endemic in the Mississippi, Ohio and Missouri River valleys and in the Southeast of the United States.
    Infections usually occur sporadically but epidemics can occur when exposed to contaminated areas. Incubation is 1-3 weeks.
    Primary infection usually is asymptomatic and diagnosed retrospectively because of pulmonary calcification and/or calcified hilar adenopathy noted later on an incidental chest radiograph.
    If the patient receives a heavy load of the fungus they can have anorexia, chest pain, cough, fever, malaise and tachypnea.
    Before calificiation, the hilar lymph nodes may only be enlarged prompting an evaluation for malignancy, tuberculosis and other causes.
    Complications may include hemoptysis, mediastinal fibrosis, or pneumonia secondary to obstruction of the airways by the enlarged lymph nodes. Treatment is usually not necessary unless a complication arises.

    In November 2008, an outbreak of histoplasmosis occurred among employees of the American Lung Association who had attended a meeting at the Governor’s mansion in Des Moines, Iowa.

    Erythema nodosum is usually more often seen in adults than children. It usually presents as red warm plaques without epidermal changes on the lower extremities.
    It is self limited, but because of pain may require nonsteroidal anti-inflammatory medications, steroids and/or iodine for treatment. Causes of it include:

    • Infectious
      • Streptococcus – one of the most common causes
      • Bartonella
      • Campylobacteriae
      • Leishmaniasis
      • Lymphogranuloma venereum
      • Mycobacterium tuberculi
      • Mycoplasma
      • Salmonella
      • Yersinia enterocolitica
      • Coccidiomycosis
      • Histoplasmosis
    • Crohn’s disease
    • Ulcerative colitis
    • Behçet disease
    • Castleman’s syndrome – benign giant lymph node hyperplasia
    • Malignancy – Hodgkin and Non-Hodgkin lymphoma
    • Medications – sulfonamides, halide, gold, oral contraceptives
    • Pregnancy
    • Sarcoidosis

    Learning Point
    Hilar adenopathy is not a common problem in the setting of a healthy child or teen, and therefore a prompt evaluation is necessary.
    History is particularly important along with a careful physical examination as it helps to direct the workup.
    Laboratory testing assists in confirming and denying possible diagnoses.

    The differential diagnosis of hilar adenopathy includes:

    • Infectious
      • Fungal
        • Blastomycosis
        • Candidiasis
        • Coccidiomycosis
        • Histoplasmosis
      • Virus
      • Bacterial
        • Mycobacterium tuberculi
        • Mycoplasma
        • Tularemia
    • Immunological
      • Sarcoidosis
      • Systemic lupus erythematosus
    • Oncological
      • Lymphoma
      • Leukemia
      • Metastasis
    • Miscellaneous
      • Bronchiectasis
      • Castleman’s disease
      • Chronic sinusitis

    Questions for Further Discussion
    1. What fungal diseases are common locally?
    2. How does Bacille Calmette-Guérin (BCG) affect an intradermal tuberculin skin test?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Information prescriptions for patients can be found at MedlinePlus for these topics: Fungal Infections and Lung Diseases.

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    Rudolph CD, et.al. Rudolph’s Pediatrics. 21st edit. McGraw-Hill, New York, NY. 2003:1237, 1983.

    American Academy of Pediatrics. Histoplasmosis, In Pickering LD, Baker CJ, Long SS, McMillan JA, eds. Red Book: 2006 Report of the Committee on Infectious Diseases. 27th edit. Elk Grove Village, IL: American Academy of Pediatrics; 2006;371-377.

    Shaikh U, Blumberg DA. Lymphadenitis. eMedicine.
    Available from the Internet at http://www.emedicine.com/ped/TOPIC32.HTM (rev. 5/18/2006, cited 2/7/08).

    Hebel JL, Habid T. Erythema Nodosum. eMedicine.
    Available from the Internet at http://www.emedicine.com/derm/TOPIC138.HTM (rev. 10/19/2006, cited 2/7/08).

    Ley T. Health investigators link lung illness to Terrace Hill. Des Moines Register. January 29, 2008.
    Available from the Internet at http://www.desmoinesregister.com/apps/pbcs.dll/article?AID=/20080129/NEWS/801290384/-1/archive (cited 2/7/08)

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.

    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement

    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Interpersonal and Communication Skills
    19. The health professional works effectively with others as a member or leader of a health care team or other professional group.

  • Systems Based Practice
    23. Differing types of medical practice and delivery systems including methods of controlling health care costs and allocating resources are known.
    24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.
    25. Quality patient care and assisting patients in dealing with system complexities is advocated.
    26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

    Date
    April 7, 2008