Why Are My Baby's Eyes Dancing?

Patient Presentation
A 2 month-old male came to his well-child care appointment. His mother did not have any concerns but had noted that his eyes were “dancing” for the past two days.
She says that his eyes would “roll-around” like a clock, and also would move up and down. He had been otherwise well so she decided to just wait for this appointment.
With further questioning, she had noticed that his eyes were cloudy since around birth and that he did not look at faces or objects.
The past medical history was negative except for a mild runny nose at 6 weeks of age. He was the first child and was born full term without complications.
He went home on time. By 10 days of age he had regained his birth weight and was doing well. Newborn and hearing screens were normal. He wasn’t taking any medications.
The family history revealed maternal asthma, a child cousin with cancer, and a maternal grandfather with cataracts as an older man. Some family members wear glasses as they age.
There are no other family members with cataracts, retinoblastoma, birth defects, mental retardation or learning problems.
The review of systems was normal including no tearing, or eye redness.
The pertinent physical exam showed a healthy appearing male with all growth parameters at 90% for age
Head was atraumatic, normocephalic with open anterior fontanelle and closed posterior fontanelle.
Eyes had cloudy-appearing lenses. The anterior chamber appeared normal. No red reflexes were seen. Sclera and palpebral conjunctiva were normal.
Skin and neurological examinations waere negative.
The differential diagnosis of bilateral cataracts and glaucoma were considered as was retinoblastoma.
He was referred to ophthalmology, who confirmed the diagnosis of bilateral congenital cataracts. He underwent bilateral lensectomies and vitrectomies. A genetics evaluation was negative also.
The patient’s clinical course showed that at 6 months of age, he needed bilateral strabismus surgery for esotropia. At 2 years of age, he needed contact lenses and glasses for severe visual correction but the ophthalmologist was happy with his progress to date.
His developmental milestones were appropriate for his age at that time, and he was receiving special education services to assist and monitor his progress.

Discussion
Children should be screened for visual problems from birth onwards. Asking the parent whether they have any vision concerns may elicit the first clue to uncovering an abnormality.
From birth until 2 years infants and toddler should have the following eye evaluation:

  • Examination of the eyelids, conjunctiva, sclera, cornea and iris, pupils, and eyelids.
  • Corneal light reflexes can evaluate muscle balance, ocular motility, and acuity. The symmetry of the corneal light reflexes on the pupil when the eye is fixed and when the eyes are in motion are noted. The light reflex should be symmetrical at all times; if it is not, then a referral to ophthalmology should be made.
  • Unilateral cover test – must be performed with the eyes fixated on an object. The first eye is then covered while the object continues to move; the movement of the uncovered eye should be symmetric and follow the object.
    The test is then done on the second eye. If the child will not follow the object or tries to move the head to be able to see with the other eye, this may be a sign of a visual problem and a referral should be made.

  • Red reflex testing is performed to look for opacities in the visual axis and in the back of the eye. The red reflexes should be viewer from 12-18 inches away and should be symmetrical. Dark or white spots, or any unilateral differences should be referred to ophthalmology.

As the children become older, examination of the retina by ophthalmoscope becomes easier as does vision testing using picture cards, tumbling E or HOTV letter screening. Vision testing is recommended to begin at age 3 years.

Learning Point
Cataracts are the most common cause of blindness in the developing world. In the developed world, bilateral congenital cataracts are the most common cause of treatable childhood blindness; they also account for 5-20% of childhood blindness worldwide. It usually has an idiopathic cause (~50%) in the developed world, but may be associated with various systemic diseases.
The differential diagnosis of congenital cataracts includes:

  • Idiopathic
  • Metabolic disorders
    • Cholestanolosis
    • Diabetes mellitus
    • Fabrys disease
    • Galactosemia
    • Galactokinase deficiency
    • Hypocalcemia
    • Hypoparathyroidism
    • Lysosomal disorders
    • Mannosidosis
    • Mitochondrial respiratory chain disorders
    • Organic acidurias
    • Peroxisomal disorders
    • Pseudohypoparathyroidism
    • Refsum disease
    • Zellweger syndrome
  • Congenital
    • Chondrodysplasia puncta
    • Craniofacial dysostosis – Apert syndrome, Crouzon syndrome
    • Trisomy 13, 18, 21
    • Turners’ syndrome
    • Specific chromosomal abnormalities – chromosome 3, chromosome 17, translocations, deletions, and rings
  • Systemic syndromes – e.g. Alport syndrome, Conradi syndrome, Lowe syndrome, Marfan syndrome
  • Drugs
    • Busulfan
    • Corticosteroids
    • Naphthalene
    • Paradicholorenzene
    • Phenothiazine
    • Sulfonamides
    • Triparanol
    • Vitamin D, excess
  • Fetal
    • Retinopathy of prematurity
    • Trauma – amniocentesis with penetration of globe
  • Intrauterine infections
    • Cytomegalovirus
    • Herpes
    • Human Immunodeficiency Virus
    • Rubella
    • Syphilis
    • Toxoplasmosis
    • Varicella
  • Ocular abnormalities
    • Aniridia
    • Ectopic lentis
    • Coloboma of the iris
    • Coloboma of the lens
    • Lenticonus
    • Megalocornea
    • Mittendorf dot
    • Microspherophakia
    • Persistent hyperplastic primary vitreous
    • Persistent pupillary membrane
    • Retinal dysplasia
  • Ocular diseases
    • Congenital glaucoma
    • Juvenile retinoschisis
    • Medulloepithelioma
    • Norrie disease
    • Retinal detachment
    • Retinoblastoma

Unilateral congenital cataracts usually have an idiopathic cause and are rarely inherited.

Treatment depends on age of patient, cataract type, size and location. Treatment seeks to decrease visual loss and amblyopia.
Treatment may include lensectomy, vitrectomy, implantation of an artificial lens, and visual rehabilitation (including occlusion of the eye(s)). Complications can occur including infection, inflammation, and opacification of the visual axis; complications may need additional treatment and affect visual outcomes.
Overall outcome depends on many factors including age of patient, time of presentation, cataract type, size and location, preexisting ocular problems, and complications of treatment. Surgery is currently recommended at 6-12 weeks of age.

Questions for Further Discussion
1. What is the differential diagnosis of leukocoria?
2. What is the role of special education for a child with visual defects?
3. In what groups of children is photoscreening helpful?

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: Cataract.

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

To view images related to this topic check Google Images.

American Academy of Pediatrics Committee on Practice and Ambulatory Medicine, Section on Ophthalmology. Eye Examination and Vision Screening in Infants, Children and Young Adults.
Pediatrics. 1996:98;153-157. Available from the Internet at http://aappolicy.aappublications.org/cgi/reprint/pediatrics;98/1/153.pdf (rev. 7/1/1996, cited 10/16/2007).

Rudolph CD, et.al. Rudolph’s Pediatrics. 21st edit. McGraw-Hill, New York, NY. 2003:2388-89.

Zetterstrom C, Lundvall A, Kugelberg M. Cataracts in children.
J Cataract Refract Surg. 2005 Apr;31(4):824-40.

Long V, Chen S, Hatt S. Surgical interventions for bilateral congenital cataract. Cochrane Database Syst Rev. 2006 Jul 19;3:CD003171.
Available from the Internet at http://www.mrw.interscience.wiley.com/cochrane/clsysrev/articles/CD003171/frame.html.

World Health Organization. Magnitude and causes of visual impairment.
Available from the Internet at http://www.who.int/mediacentre/factsheets/fs282/en/ (rev. 11/2004, cited 10/19/2007).

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.
    16. Learning of students and other health care professionals is facilitated.

  • Systems Based Practice
    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
    November 26, 2007

  • Trying to Make Health Care Systems Work

    Patient Presentation
    A pediatrician received a telephone call on a Saturday from her sister who lived in another state.
    An infant in the nieces/nephews’ daycare had been diagnosed with rapid-onset meningitis was not expected to live.
    The sister was very worried about the infant and its family, and also concerned about her own children, so she had called her sister for support.
    The daycare provider had heard about the infant through a friend of the family and had no direct contact with the family for more information.
    The pediatrician recommended for her sister to contact her own pediatrician locally so the sister’s pediatrician could manage the problem locally as needed.
    The pediatrician also told her sister to call the daycare provider to try to contact public health.
    The daycare provider left messages with the local public health department but got no answer.
    The sister’s pediatrician said that although meningococcal meningitis potentially was a cause, more information was necessary before beginning prophylactic antibiotics.

    Over the next several days, the daycare provider found out more information from the friend of the infant’s family but because of HIPAA regulations, no information could be released directly to the daycare provider.
    The daycare provider continued to try to contact the local public health department without success. The daycare provider was also not aware if the infant’s physician’s had contacted the local public health department.
    On day 5 of admission, a culture grew Kingella kingae, but unfortunately the infant died later that evening.
    When the pediatrician received this information from her sister, she checked the AAP’s RedBook® which did not recommend any antibiotic prophylaxis nor special instructions for the contacts.
    The pediatrician was still concerned that the local public health department had not contacted the daycare provider.

    Five days after the first child died, another toddler in the same daycare was admitted to a second hospital with a fever and rash.
    The pediatrician’s sister was told that the diagnosis was a virus, but later the sister was told that the infection still required antibiotics.
    One week after the second child was admitted, a third toddler was admitted to a third hospital with fever and severe leg pain.
    The pediatrician told her sister that she was very worried about this situation because although the second child may have had a virus, Kingella kingae often causes osteoarthritis.
    The pediatrician had her sister contact the sister’s pediatrician to see if this pediatrician could get the local health department to investigate.
    Concurrently, the pediatrician also called the sister’s state health department who was surprised to be called by someone from out of state.

    Within a few hours, the state health department contacted the local health department who began investigating immediately.
    Along with advice from the Centers for Disease Control, the local health department determined that all of the children in the daycare needed prophylactic antibiotics because the third child with leg pain was very suspicious for Kingella kingae as a causative organism.
    All the children in the daycare received amoxicillin and rifampin as prophylactic antibiotics.
    The second and third children were doing well one week later.

    Discussion
    Kingella kingae is a slow-growing, gram-negative organism that was previously classified as Moraxella kingae.
    Kingella kingae is usually known for causing osteoarticular infections (particularly the femur) but also other invasive disease such as occult bacteremia, diskitis, endocarditis, meningitis, and pneumonia.
    The organism colonizes the oropharynx and is known to spread within daycare settings usually without causing disease. Infections may occur with concomitant or preceding upper airway infections or stomatitis.
    The organism can be isolated from many fluids and tissues and grows better in anaerobic conditions. It can also be difficult to isolate using standard media.
    Blood cultures are frequently negative in children with osteoarthritis and Kingella kingae should be suspected with blood culture negative skeletal infections.
    Penicillin is recommended for treatment of beta-lactam negative isolates. Other beta-lactam antibiotics are also effective.

    Learning Point
    Unfortunately the initial infant with Kingella kingae succumbed to this invasive organism.
    The second child appears not to have had Kingella kingae but another illness that coincidentally occurred at that particular time.
    The third child potentially may not have had the presumed leg infection if prophylaxis had been started earlier.
    However, recommendations from the American Academy of Pediatric are not to give prophylaxis but to monitor closely after the first case which was done.
    The public health department did determine that prophylaxis was needed after the third child with suspicious disease.
    It appears that the Kingella kingae either burnt itself out or was stopped by the prophylactic antibiotics.

    From a systems-based perspective, many problems occurred to complicate making the diagnosis, investigating, and treating contacts that could be considered for improvement:

    • Kingella kingae is slow growing and therefore takes time to isolate. It usually does not require prophylaxis for contacts and therefore the pediatrician was not as concerned about prophylaxis for this organism until after the 2nd and 3rd child presented.
      Should the pediatrician have called the state health department sooner?

    • The pediatrician and sister’s pediatrian were concerned about giving unnecessary prophylactic antibiotics for the contacts of the initial infant; meningococcus should not receive prophylaxis until the organism has been identified.
      Should the pediatrician or the sister’s pediatrician have pushed for prophylactic antibiotics sooner and if so at what point?

    • The infant with meningitis initially presented over the weekend when it can be more difficult to contact the regular health care providers working within and between health systems.
      How can off-hours contacts with healthcare providers be facilitated?

    • The pediatrician and the involved children were in different states thus the pediatrician did not know the local health care systems well enough to offer specific advice but only general advice to her sister and the daycare provider.
      What else could the pediatrician have done to learn more about the local health care systems to be able to give more specific advice?

    • The pediatrician did not have a personal or professional relationship with the initial infant and other children in the daycare and therefore could not obtain specific information, nor had a specific legal responsibility to act on their behalf.
      What defines a professional relationship with a patient? What if anything could the sister’s pediatrician who did have a professional relationship with the sister’s children potentially do if this pediatrician had been aware of the circumstances as they were evolving.

    • Information was being communicated between many different people including the pediatrician, pediatrician’s sister, daycare provider, infant’s family friend, other daycare families, healthcare teams for the second and third ill children, sister’s pediatrician, local health department, state health department, and the Centers for Disease Control.
      The information may or may not have been communicated accurately between all these people. How can accurate information be conveyed between and among laypersons and health care professionals?
      What could the pediatrician do to facilitate communication between all these people?

    • HIPAA regulations are privacy regulations and properly prevented information from being given to non-family members. The daycare provider could not receive direct information and therefore may not have been giving accurate information to the local health department.
      What are the exceptions in the HIPAA laws that allow private information to be shared for the public good?

    • The daycare provider as a layperson may not know how to make various healthcare systems work and therefore may have been contacting the wrong local health department or the wrong people within the health department.
      How can health systems be user-friendly and facilitate interactions?

    • Within the public health systems there are inconsistent divisions of responsibilities between the local and state levels in different states, so the pediatrician contacted the state level when the local level needed to be investigating.
      The state level did contact the local level and the investigation then began promptly after this contact.
      How could the pediatrician learn about how to work the health care system when she was not involved in that system and the systems she was aware of worked differently?

    Questions for Further Discussion
    1. How do you treat your own family members who ask for medical advice?
    2. How would you have handled this situation differently from the pediatrician?
    3. How do you define advocacy and when do you need to take action?

    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: Meningitis and Bone Diseases
    and at Pediatric Common Questions, Quick Answers for this topic: Bacterial Meningitis

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

    To view images related to this topic check Google Images.

    Moylett EH, Rossmann SN, Epps HR, Demmler GJ. Importance of Kingella kingae as a Pediatric Pathogen In the United States. Pediatr Infect Dis J. 2000 Mar;19(3):263-5.

    Yagupsky P. Kingella kingae: From Medical Rarity to an Emergency Paediatric Pathogen.
    Lancet Infect Dis. 2004 Jun;4(6):358-67. Available from the Internet at http://www.thelancet.com/journals/laninf/article/PIIS1473309904010461/abstract (cited 10/11/2007).

    American Academy of Pediatrics. Kingella kingae infections, 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;416.

    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.
    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.

  • Interpersonal and Communication Skills
    17. A therapeutic and ethically sound relationship with patients is created and sustained.
    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.
    21. A commitment to ethical principles pertaining to provision or withholding of clinical care, confidentiality of patient information, informed consent, and business practices 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.
    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
    November 19, 2007

  • What Causes Emesis in a Neonate?

    Patient Presentation
    A 35-day-old male came to clinic with projectile vomiting.
    He began having emesis 5 days prior that increased in frequency and amount. On the third day of emesis, he was evaluated; the parents were told that it probably was a viral syndrome.
    The emesis is non-bloody, non-bilious, with just breast milk in the vomitus. It occurs about 1- 1.5 hours after feeding.
    His mother states that initially it would just dribble out of his mouth, but now comes up forcefully and will hit an object or floor located a couple feet away.
    He seems hungry between feedings and is crying more.
    He is sleepier over the past couple of days and has had a decreased number and volume of wet diapers.
    He was having normal stools up until 1 day ago.
    The past medical history revealed a healthy infant born at 39 weeks gestation with no complications. He was healthy at day 5 and 13 of life.
    His newborn screening test was reported as normal.
    The family history is positive for a cousin with mental retardation. No other genetic or metabolic disorders and no gastrointestinal or neurological problems.
    The review of systems was negative including fever, diarrhea, rashes, sweating, color changes, etc.
    The pertinent physical exam showed his weight to be 3.65 kg (down 240 grams from the previous clinic visit 2 days ago, 10-25% for age),
    afebrile, with heart rate = 128 beats/minutes, respiratory rate = 45 breaths/minute. All other growth parameters are 25-50% for age with normal trends.
    He was alert with tacky mucous membranes and a capillary refill of 2 seconds.
    Anterior fontanelle was normal.
    Abdomen was soft, non-tender with no hepatomegaly, but with a small fullness felt in the mid- to right upper quadrant when feeding.
    Genitourinary examination found no hernias or other masses.
    Neurological and extremity examination was normal.
    The diagnosis of hypertrophic pyloric stenosis was made clinically.
    The laboratory evaluation was performed to assess for a metabolic problem, which were slightly abnormal in the CO2, showed a sodium = 142 mEq/L, potassium = 5.1 mEq/L, chloride = 99 mEq/L, and CO2 = 34 mEq/L, blood urea nitrogen = 15 mg/dl and creatinine = 0.5 mg/dl on a heel-stick blood draw.
    A complete blood count was normal.
    The radiologic evaluation of an ultrasound of the abdomen confirmed the diagnosis of pyloric stenosis with a pyloric muscle length of 2.1 cm and width of 5.3 mm.
    The patient’s clinical course over the evening was that he urinated after 2 normal saline fluid boluses were given and then he was given D5, 0.45 normal saline at a maintenance fluid rate.
    He was not given anything by mouth and but did have some minor spitting up of oral secretions.
    Repeated electrolytes the next morning were normal before he was taken to the operating room where he underwent a pyloromyotomy without complications.
    He was discharged home on Day 3, and was doing well at follow-up 2 weeks later.


    Figure 53 – Longitudinal (left) and transverse (right) images from an ultrasound of the pylorus demonstrate the pyloric muscle to be abnormally thickened and elongated. The pyloric muscle measures 5 mm thick and 21 mm in length and these measurements meet the criteria for the diagnosis of pyloric stenosis by ultrasound.

    Discussion
    Hypertrophic pyloric stenosis is the hypertrophying of the pylorus muscle with subsequent stenosis of the pyloric chanel. It usually presents in the 3-12th week of life as forceful or projectile non-bilious emesis. It occurs ~ 2-5 patients/1000 live births, more often in males than females (4;1) and most often in first-born males (30%).
    The infant often appears hungry after feeding, but with increased crying (because of hunger) or later lassitude (because of the problem not being recognized and appropriately treated).
    It can cause failure to gain normal weight, weight loss, and metabolic abnormalities (classically a hypochloremic, hypokalemia metabolic alkalosis).
    Classically a small abdominal mass about the size of an olive can be palpated at the mid- to right upper quadrant just lateral to the rectus abdominus muscle.

    On ultrasound examination, a pyloric muscle thickness of > 4 mm is considered diagnostic. The length of the muscle is variable from 14-20 mm, and pyloric diameter may be between 10-14 mm.

    The cause is unknown but may be linked to sleeping position, acidity of the stomach, and the drugs nitric oxide and erythromycin have been implicated.
    Treatment is surgical with good results. Fatality from pyloric stenosis is < 1%.

    Learning Point
    The causes of emesis are often broken down by age and also by history of being bilious or non-bilious. Bilious emesis should be considere always abnormal as it indicates ileus or obstruction distal to the common bile duct insertion into the duodenum.
    Depending on the cause and its severity, emesis may be non-bilious especially early on in the illness course and later turn bilious.
    Parents and health care providers alike may have problems accurately identifying bilious emesis and therefore the history may be inaccurate, so all causes of emesis should be considered in neonates and infants.

    Common causes of emesis in children under 1 year include:

    (Those with an * often present with true bilious emesis.)

    • Normal variation, i.e. “spitting up”
    • Overfeeding

    • Gastroesophageal reflux, severe
    • Gastrointestinal obstruction
      • Achalasia
      • *Annular pancreas
      • *Gastric/intestinal atresia/stenosis/duplications
      • Incarcerated hernia
      • *Intermittent malrotation/volvulus
      • *Intestinal or viscous organ perforation with peritonitis
      • Intussception
      • Imperforate anus
      • Hirschsprung disease
      • *Meconium plug and ileus
      • Pseudoobstruction syndrome
      • Pyloric stenosis
    • Drug overdose
      • Aspirin
      • Iron
      • Lead
      • Theophylline
      • Digoxin
    • Foreign body – esophageal, lactobezoar
    • Infections
      • Gastroenteritis
      • Meningitis
      • Necrotizing enterocolitis
      • Sepsis
      • Urinary tract infection
      • Otitis media
      • Pertussis
    • Metabolic
      • Inborn errors of metabolism
        • Aminoacidemia
        • Congenital adrenal hyperplasia
        • Galactosemia
        • Hypercalcemia
        • Organic acidemia
        • Urea cycle defects
      • Diabetic ketoacidosis
    • Neurologic
      • Hydrocephalus
      • Kernicterus
      • Mass lesion
    • Renal
      • Obstruction
      • Uremia
    • Rumination

    Questions for Further Discussion
    1. Explain the physiology behind the hypochloremia, hypokalemia metabolic alkalosis seen in pyloric stenosis?
    2. What are the indications for an upper gastrointestinal radiographic series in a child who is vomiting?
    3. What are the indications for an abdominal ultrasound radiographic examination in a child who is vomiting?
    4. When in the evaluation process should a pediatric surgeon be consulted?

    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: Pyloric Stenosis.

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

    To view images related to this topic check Google Images.

    Fleisher GR, Ludwig S. Synopsis of Pediatric Emergency Medicine. Williams and Wilkins. Baltimore, MD. 1996:266-71.

    Rudolph CD, et.al. Rudolph’s Pediatrics. 21st edit. McGraw-Hill, New York, NY. 2003:1351-54.

    Walker GM, Neilson A, Young D, Raine PA. Colour of bile vomiting in intestinal obstruction in the newborn: questionnaire study.
    BMJ. 2006 Jun 10;332(7554):1363.

    MacMahon B. The continuing enigma of pyloric stenosis of infancy: a review.
    Epidemiology. 2006 Mar;17(2):195-201.

    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.
    14. Knowledge of study designs and statistical methods to appraisal clinical studies and other information on diagnostic and therapeutic effectiveness is applied.

  • 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
    November 12, 2007

  • What are the Current Recommendations for Treatment of Acute Traumatic Head Injury?

    Patient Presentation
    A 12-year-old male was transported by ambulance to the emergency room after he was riding an all-terrain vehicle and hit a tree at an unknown speed.
    He was wearing a helmet but there was significant trauma to the helmet.
    He was initially agitated and somewhat conscious with a Glascow Coma Scale (GCS) of 13 but rapidly deteriorated in the emergency room to a GCS of 7.
    He was sedated, paralyzed and intubated using rapid sequence intubation using atropine, lidocaine, and a defasciculating dose of vecuronium.
    He was mildly hyperventilated with an arterial blood gas with pH = 7.43, pCO2 = 35, pO2 = 82, base excess = 23.
    After initial stabilization, he was taken for a radiologic evaluation of his head by computed tomography which showed multiple facial fractures of the nose, sinuses, orbits and left temporal bone.
    He also had a large frontal lobe contusion with associated edema.
    In the pediatric intensive care unit, he was given mannitol and Dilantin.
    The pertinent physical exam in the intensive care setting showed an intubated male with temperature = 36.6, heart rate = 115, respiratory rate =18 (with the ventilator), blood pressure = 109/69, and estimated weight = 45 kilograms.
    He had a cervical spine collar in place along with peripheral IVs.
    HEENT showed edema of entire face, with blackened eyes, and pupils 3-4 mm bilaterally, symmetrical and reactive to light.
    Nose had epistaxis and he was orally intubated. There was a left hemotympanum.
    His mandible was stable with teeth intact.
    Neck had no obvious tenderness.
    Lungs had normal breath sounds and chest wall movement was symmetrical.
    Abdomen was normal and he had a Foley catheter in place.
    Neurologically he was moving all extremities but not purposefully.
    Extremity examination demonstrated some scrapes and bruises of the upper limbs.
    The diagnosis of multiple trauma with head injury and facial fractures was made.
    The patient’s clinical course was that on day 2 he had improving mental status but this waxed and waned. On day 3 he had consistently improving mental status and his mannitol was stopped and he was later extubated.
    On day 6 he was taken to the operating room for definitive surgery performed by ophthalmology and otolaryngology. He was discharged at day 10 to home and at 3 weeks had a normal neurological examination except for a slight left eye droop with improved over the next 6 months.
    Dilantin had been discontinued at 6 weeks post-op.


    Figure 52 – Axial image of a CT scan of the brain performed without intravenous contrast in bone (left) and soft tissue (right) windows shows a frontal skull fracture and an associated large frontal lobe brain contusion with pneumocephaleus and edema as well as an associated frontal subgaleal hematoma. The patient also had extensive maxillofacial fractures and a left temporal bone fracture (not pictured).

    Discussion
    As part of Advanced Trauma Life Support® evaluations, assessment for neurological injury is performed early and is the paramount factor in determining treatment.
    The Glasgow Coma Scale can be quickly used for multiple assessments over time and is an important tool for communicating the patient’s neurological status to other healthcare providers.
    The GCS was originally developed for adults but has been modified for children and infants under two years of age mainly in the verbal response category.
    The score ranges from 3 to 15 with a higher score being a better neurological status. GCS fall into general categories <8 = severe head injury, 9 – 12 = moderate head injury, 13 – 15 = mild head injury.

    Score	Adult GCS			Pediatric GCS
    
    Eye Opening Response
    4	Spontaneous with blinking 	Spontaneous
    	at baseline
    3	To verbal stimuli 		To verbal stimuli
    	(e.g. commands, speech)
    2	To pain				To pain
    1	None				None
    
    Verbal Response
    5	Oriented			Coos, babbles
    4	Confused conversation,		Irritated cry
    	but able to answer questions
    
    3	Uses inappropriate words	Cries to pain
    2	Incomprehensible speech		Moans to pain
    1	No response			No response
    
    Motor Response
    6	Obeys commands for movement	Normal spontaneous
    					movement
    5	Purposeful movement to pain	Withdraws to touch
    4	Withdraws to painful stimuli	Withdraws to painful stimuli
    3	Flexion posturing in response  	Flexion posturing in response
    	to pain (decorticate posturing)	to pain (decorticate posturing)
    2	Extension posturing in response Extension posturing in response
    	to pain (decerebrate posturing)	to pain (decerebrate posturing)
    1	No response			No response
    

    Children often hit their heads and luckily most injuries are insignificant. For those that are, many children will have some degree of personality change as they are initially recovering from the injury, especially disinhibition.
    Diffuse axonal injury is still very difficult to diagnose on computed tomograph scans and therefore magnetic resonance imaging is may be used to help evaluate a child who seems to have minimal injuries but is not recovering at the expected pace.

    Learning Point
    Acute traumatic brain injury guidelines were published in 2003 which focused on severe traumatic brain injury in children < 18 years old and were for primary and secondary injury from traumatic causes including shaken baby syndrome. They were not for drowning or hypoxic injury.

    • Airway management and oxygenation- the guidelines recommend avoidance of hypoxia and administration of supplemental oxygen initially with 100% oxygen. This could be done by bag-valve-mask ventilation (particularly in the pre-hospital setting) or intubation.
      Airway should be controlled if GCS is < 8 to avoid hypoxemia, hypercarbia and aspiration. Adequate oxygenation is necessary for cell survival.

    • Hyperventilation – prophylactic hyperventilation with pCO2 &lt; 35 mg Hg is not recommended especially in the first 24 hours as it may increase cerebral ischemia. Mild hyperventilation with pCO2 30-35 mm Hg can be considered for longer periods if the patient has intracranial hypertension that is non responsive to sedation, analgesia, neuromuscular blockade, CSF drainage and hyperosmolar therapy. Aggressive hyperventilation with pCO2 < 30 mm Hg can be used in refractory intracranial hypertension.
      The cerebral edema that accompanies severe traumatic brain injury used to peak at 3 days. With the use of hyperosmolar therapy, some clinicians report a shift in that curve toward later peak swelling periods with an approximate range of 3-7 days.

    • Blood pressure – the guidelines recommend to identify and correct hypotension as rapidly as possible. Blood pressure should be maintained in normal range for age with fluid resuscitation. Sedation, analgesia and neuromuscular blockage may be useful to maintaining blood pressure.
      Adequate blood pressure is needed to perfuse the brain (see cerebral perfusion pressure below). Large swings in blood pressure should to be avoided (high or low) if possible, as both extremes are associated with poor outcomes.

    • Cerebral perfusion pressure – is the mean arterial pressure minus the intracranial pressure. Cerebral perfusion pressure must be maintained to allow appropriate perfusion of the brain to prevent cellular death.
      Active treatment at specific threshold values for cerebral perfusion pressure has not shown improvement in outcome. The guidelines recommend keeping the cerebral perfusion pressure between 40-65 mm Hg.
      The loss of cerebral autoregulation is the critical physiological problem associated with traumatic brain injury and correlates with the severity of the injury. The goal is to maintain perfusion pressure to the healthy brain, but not exacerbate cerebral edema. Consequently, the literature recommends being very cautious about hyperventilation therapy, because at very low pCO2s brain perfusion may become critically low as the cerebral vessels vasoconstrict.

    • Cerebral spinal fluid drainage – ventricular drainage (+/- lumbar drainage) is an option in patients with refractory intracranial hypertension.
    • Hyperosmolar therapy – with hypertonic saline or mannitol can be used to lower intracranial pressure. The choice is left to practitioners. Mannitol has long-standing clinical acceptance and safety but has not been studied as much. Hypertonic saline has more studies but has not been used therapeutically as long.
    • Barbiturates – high dose barbiturates may be considered in hemodynamically stable patients with salvageable severe and refractory intracranial hypertension. Barbiturates work by suppression of metabolism and also decreasing cerebral blood flow.
    • Corticosteroids – are not recommended as they do not improve long term outcomes.
    • Anti-seizure prophylaxis – phenytoin, carbamazepine or phenobarbital should be considered as treatment to prevent early post-traumatic seizures in infants and children. From adult studies these medications do not appear to affect late post-traumatic seizures or outcome.

    Questions for Further Discussion
    1. How are the mechanisms of brain injury different for hypoxia cases (such as drowning) versus traumatic brain injury?
    2. What is the American Academy of Pediatrics recommendations for children using all-terrain vehicles?
    3. What are some of the potential long-term sequelae for traumatic brain injury survivors?
    4. What are the components of Cushing’s triad?

    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: Topic

    Information prescriptions for patients can be found at MedlinePlus for these topics: Head and Brain Injuries, Facial Injuries and Disorders and Child Safety
    and at Pediatric Common Questions, Quick Answers for this topic: Head Injuries

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

    To view images related to this topic check Google Images.

    Adelson PD, Bratton SL, Carney NA, Chesnut RM, du Coudray HE, Goldstein B, Kochanek PM, Miller HC, Partington MP, Selden NR, Warden CR, Wright DW; American Association for Surgery of Trauma; Child Neurology Society; International Society for Pediatric Neurosurgery; International Trauma Anesthesia and Critical Care Society; Society of Critical Care Medicine; World Federation of Pediatric Intensive and Critical Care Societies. Guidelines for the Acute Medical Management of Severe Traumatic Brain Injury in Infants, Children and Adolescents.
    Pediatr Crit Care Med 2003:4(3); S2-75.

    Centers for Disease Control. Glascow Coma Scale.
    Available from the Internet at http://www.bt.cdc.gov/masscasualties/gscale.asp (rev. 6/23/2006, cited 9/24/07).

    Emergency Medical Services Program, Marin County California. ALS Treatment Guidelines Pediatric.
    Available from the Internet at http://www.co.marin.ca.us/depts/HH/main/ems/documents/Policies/8310PEDTXr.pdf (rev. 1/3/2005, cited 9/24/2007).

    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.
    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.

  • 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

    A. Paige Davis, MD
    Associate, University of Iowa Children’s Hospital

    Date
    November 5, 2007