What Causes Priapsim?

Patient Presentation
A 11-year-old white male came to emergency room after he disclosed that he had had intermittent priapism for 3 days.
Today, he has had continued priapism for several hours continuously. He denies trauma, medication or masturbation. He has not urinated for several hours.
The past medical history was negative.
The family history was positive for early cardiac disease.
The review of systems was negative including fever, chills, night sweats, weight loss, or medication use. He says he noticed a rash on his body today, but no other bleeding.
The pertinent physical exam showed a school age boy who was in distress secondary to pain. His vital signs showed a slightly elevated heart rate and blood pressure and growth parameters in the 25-75%.
He had petechiae on his face, neck and trunk below the nipple line. He had two bruises on the shins which are healing. HEENT had slightly pale conjunctiva. His cardiac examination reveals tachycardia with a grade I-II systolic murmur best at the lower left sternal border.
Lungs were negative but his abdomen had a liver edge that is 3 cm below the costal margin. The spleen was not palpable. Genitourinary examination revealed a circumcised male with bilateral testes in the scrotum. He has no pain on testicular examination and no inguinal hernia. He has an erect penis, that is swollen and deep red in color that is painful to examine.
He had several 0.5-1.0 cm lymph nodes in the anterior cervical chain and in the inguinal area.
The laboratory evaluation included a complete blood count which showed a hemoglobin of 8.0 mg/dl, platelets of 23 x 1000/mm2 and a white blood cell count of 78 x 1000/mm2 with almost all cells being small round blue cells, which were later confirmed to be blasts.
The diagnosis of priapism secondary to new onset acute leukemia was made.
The patient’s clinical course included being admitted to the intensive care unit where he received hydration, leukopheresis, and pain control.
Aspiration of the corpus cavernosum with saline irrigation had minor relief of symptoms. Over the next 3 hours with leukophereis the patient had a decrease in the priapism and over the next 2 hours had resolution but some continued edema.
He was transferred to the floor where he completed his initial evaluation for acute lymphocytic leukemia and began his induction chemotherapy. He stated that he had some erections since the priapism.

Discussion
Priapism is as unremitting, painful erection that is a true urological emergency.The corpora cavernosa engorgement causes compression of the venous outflow tracts, resulting in blood trapping within the corpora cavernosa. The corpora spongiosum and glans are not engorged.
Priapism can occur in any age group, with peaks at age 5-10 years and 20-50 years.

Low flow (ischemic) priapism is the most common type and is caused by an abnormal detumescence mechanism (the normal mechanism that release the venous blood ending an erection). This can be caused by excessive neurotransmitter release, blockage of the draining venules (as in sickle cell disease or leukemia), and changes in the smooth muscle of the corpora cavernosa.

Prolonged low flow priapism causes an ischemia state leading to fibrosis of the corporeal smooth muscle and cavernosal artery thrombosis. Low flow priapism lasting more than 24 hours often leads to permanent impotence.
If a malignancy is suspect then a pelvic computed tomographic scan may be indicated. Surgical treatment by shunting may be necessary if medical therapy similar to that which this patient received fails.

High-flow priapism is caused most often becuase of blunt or penetrating trauma causing a fistula between the cavernosal artery and the corpus cavernosum with unchecked flow. Doppler ultrasound or pelvic angiography may be helpful for determining fistula location.

Learning Point

Causes of priapism include:

  • Sickle cell anemia – most common cause
  • Amyloidosis
  • Asplenia
  • Black widow spider venom
  • Carbon monoxide poisoning
  • Dialysis
  • Fabry disease
  • Fat emboli
  • Leukemia and other cancers
  • Medications – alcohol, cocaine, antihypertensive, psychotropics, anticoagulants, male and female hormones, metoclopramide, omeprazole, medications for erectile dysfunction
  • Malaria
  • Trauma – blunt and penetrating
  • Thalassemia
  • Spinal cord injury
  • Vasculitis

Questions for Further Discussion
1. What are other urological emergencies?
2. What are presentations of acute leukemia?
3. What are presentations of acute sickle cell anemia?

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: Penis Disorders

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

Rudolph CD, et.al. Rudolph’s Pediatrics. 21st edit. McGraw-Hill, New York, NY. 2003:1740.

Parraga-Marquez M. Wahlheim, Stantucci RA. Priapism. eMedicine. (rev. 11/14/2004, cited 6/9/06) Available from the Internet at: http://www.emedicine.com/med/topic1908.htm

ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effecively 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 competency 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.

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

    Date
    July 17, 2006

  • Can I Use This Medicine When I am Breastfeeding?

    Patient Presentation
    A 24-year-old female came to clinic with her 1 month old healthy child. She had seasonal allergic rhinitis in the past and her symptoms that are now flaring up with itchy water eyes, itchy nose with clear rhinorrhea, and dark circles under her eyes.
    She previously took loratadine with good symptomatic relief, but she is breastfeeding and is worried about using it again.
    The past medical history reveals a normal, spontaneous vaginal birth with no complications pre- or post-natally.
    The pertinent physical exam shows a normal healthy infant.
    The diagnosis of a healthy infant with an appropriately-concerned breastfeeding mother was made.
    The physician checked the LactMed database from the National Library of Medicine whose summary stated: “Because of its lack of sedation and low milk levels, maternal use of loratadine would not be expected to cause any adverse effects in breastfed infants. Loratadine might have a negative effect on lactation, especially in combination with a sympathomimetic agent such as pseudoephedrine.”The mother was re-assured with the information, but was also told to monitor the infant and her milk supply and if there were changes to re-contact the physician.

    Discussion
    Breastfeeding provides the best possible food for an infant.
    Some advantages include providing good nutrition for the infant, low in cost, no preparation is needed and breastfeeding is instantly available.
    Breast feeding also helps mothers get back into overall physical shape and helps the uterus to tighten and return to normal size more quickly
    Breastfeeding may promote positive feelings towards the child and self.

    The disadvantages include that only the mother can breastfeed which takes time and energy and other family members may feel left out.

    There are a few medical reasons for not recommending breast-feeding such as when the mother is extremely ill and is unable to recover herself and breastfeed concurrently, or she needs to take
    certain medications that may pass into the milk and could be dangerous for the infant.
    Also if the mother has certain health conditions, such as Human Immunodeficiency Virus Syndrome (HIV), which could put her infant at risk, breastfeeding may not be advised.
    Note: The risk of acquired HIV through breastmilk is higher than the risk of infant morbidity and mortality in developed countries and breastfeeding is generally not recommended for women with HIV in developed countries.
    However, in underdeveloped countries the risk of acquired HIV through breast milk is lower than the general infant morbidity and mortality risk and therefore breastfeeding is generally promoted for women wth HIV in underdeveloped countries.

    Learning Point
    Drug information is one of the most common information needs of pediatricians and pediatric health care providers.
    Drug information for lactating women and its potential side-effects on the lactation process and the infant is often not readily available.
    The National Library of Medicine however publishes TOXNET Toxicology Data Network (http://www.toxnet.nlm.nih.gov/cgi-bin/sis/search)which includes 15 different databases that can be searched including information on hazardous materials and household products.
    It also includes LactMed Drug and Lactation Database (http://www.toxnet.nlm.nih.gov/cgi-bin/sis/htmlgen?LACT) which is “A peer-reviewed and fully referenced database of drugs to which breastfeeding mothers may be exposed. Among the data included are maternal and infant levels of drugs, possible effects on breastfed infants and on lactation, and alternate drugs to consider.”Drug name synonyms can also be searched concurrently (i.e. a search for the proprietary name of Claritin® will retrieve loratadine, the generic name)

    Questions for Further Discussion
    1. What environmental exposures should pregnant and lactating women be cautious of?
    2. What are some standards that can be used to help determine high-quality Internet information?
    3. What is the risk of an infant acquiring HIV from a mother who is breastfeeding?

    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: Breast Feeding

    and at Pediatric Common Questions, Quick Answers for this topic: Breastfeeding

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

    Shelov SP, Hanneman RE. American Academy of Pediatrics Caring for Your Baby and Young Child Birth to Age 5. Bantam Books New York NY. 1993;68-69.

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effecively 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.
    5. Patients and their families are counseled and educated.
    6. Information technology to support patient care decisions and patient education is used.
    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.

  • 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.
    15. Information technology to manage information, access on-line medical information and support the healthcare professional’s own education is 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.

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

    Date
    July 11, 2006

  • How Do You Treat a Habit Cough?

    Patient Presentation
    An 11-year-old female was referred to the pulmonologist with a several month history of daily coughing, that began after a cold.
    The cough occurred at least every 3 minutes with flares of coughs 3 times/minute.
    The cough is described as a dry “goose-honk.” The patient states that she feels a tickle in her throat, feels strange, and then coughs.
    The cough occurs only during the day especially when she first rises and it does not interfere with her school work.
    She has been tried on albuterol and inhaled steroids without relief. She has had a chest radiograph and ‘other tests’ which were normal according to her mother, including seeing a local psychologist.
    The past medical history is negative.
    The family and social history is positive for an aunt who coughs when very nervous. There are no neurological, psychiatric or learning problems in the family.
    There has not been any recent family stressors and she continues to do well in her academically gifted program at school. She has been getting along well with her family and friends also.
    The review of systems is negative including tics, throat clearing or vocalizations and other nervous habits. She has had no colds or other illnesses recently.
    She has had no fevers, weight loss, sweats, or rashes.
    The pertinent physical exam shows a healthy child with normal vital signs, height and weight for age. Her HEENT and pulmonary examinations are normal, but she is noted
    to make a “hump” sound followed by a cough that occured multiples times during the examination.
    The work-up included reviewing the previous testing and normal pulmonary function tests.
    The diagnosis of a habit cough was made.
    The physician explained the diagnosis, commenting that at this time it did not appear that she has or had any tics or Tourette syndrome features nor did she seem that she had a great deal of anxiety.
    He explained the cycle of a cough irritating the pulmonary structures that then leads to a cough, etc., and the need to break the cycle.
    A cup of water was given to the patient and she was told to take a small sip whenever she felt the need to cough. She did this, and even within the few minutes that the physician was speaking with the family, everyone noted a decrease in the frequency of the cough.
    The patient and family were instructed to continue this treatment for several days and to call the clinic back if the cough was not improving.
    They were also told that speech therapy to improve breath support could also be helpful for some patients. Before leaving
    they were also cautioned that should other behaviors such as a tic or increased anxiety occur to contact their local physician.

    Discussion
    Cough is a common complaint in the outpatient arena. The literature is inconsistent with the terminology used to define “habit cough,” “psychogenic cough” or “nervous tic,” especially in children.
    The literature generally agrees that a habit or psychogenic cough implies a non-organic etiology and one of exclusion.
    A recent guidelines noted that a habit cough is often associated with a throat-clearing noise” and before the diagnosis “???can be accurately made, biological and genetic tic disorders associated with cough must be ruled out.”Psychogenic cough is often described as having a ‘honking’ nature. Some literature suggests that patients do not cough at night, but this is not consistent. Multiple other criteria for diagnosis have been suggested but their actual usefulness is not known (e.g. increases or decreases with stress or pleasurable activities, indifference to the cough, attention seeking, cough preceeded by upper respiratory tract infection, etc.)

    The guidelines recommend that the “??? diagnoses of habit cough or psychogenic cough can only be made after tic disorders and Tourette syndrome have been evaluated and the cough improves with specific therapy such as behavior modification or psychiatric therapy.”The guidelines do not suggest a specific evaluation and note that an extensive workup may actually be harmful to children as it could require general anesthesia or other invasive testing.

    It appears that habit cough or psychogenetic cough are primarily a pediatric and adolescent disease and few cases are reported in the adult literature.

    Learning Point
    Suggestion therapy is the main treatment and can be applied in slightly different ways.

    In one study, elements of one 15-minute session of suggestion therapy that was successful with long-term relief of symptoms included:

    1. Expression of confidence by the physician that the patient could stop the coughing
    2. Explaining the cough as a viscious cycle of irritant-cough-irritant-cough, etc.
    3. Encouraging cough suppression to break cycle – holding the cough back, use of water, etc.
    4. Expressions of confidence by the physician that the patient was developing the ability to supress the cough
    5. When some cough suppression is observed, noting “You are beginning to feel you can resist the urge to cough aren’t you?”
    6. Stopping the session, by asking several times “Do you feel you can now resist the urge to cough?” which is asked after the patient has gone more than 5 minutes without coughing.

    Other treatments have included self-hypnosis, wrapping a bedsheet around the chest to help decrease cough and other behavioral modifications. Cough suppressant medication are generally not used.

    Questions for Further Discussion
    1. What are the Diagnostic and Statistical Manual of Mental Disorders criteria for tics and Tourette Syndrome?
    2. What is the differential diagnosis for a chronic cough in a child?

    Related Cases

      Symptom/Presentation

    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: Cough

    and at Pediatric Common Questions, Quick Answers for this topic: Cough

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

    Lokshin B, Lindren S. Weinberg M. Koviach J. Outcome of Habit Cough In Children – Treatment with a Brief Session of Suggestion Therapy. Ann Allergy. 1991:67;579-582.

    Irwin RS, Glomb WB, Change AB. Habit Cough, Tic Cough, and Psychogenic Cough in Adult and Pediatric Populations: ACCP Evidence-Based Clinical Practice Guidelines. Chest. 2006;129;174-179.

    Fitzgerald DA, Kozlowska K. Habit Cough: Assessment and Management. Paediatric Respiratory REviews. 2006:7;21-25.

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effecively 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 competency performed.
    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.

  • Interpersonal and Communication Skills
    17. A therapeutic and ethically sound relationship with patients is created and sustained.
    18. Using effective nonverbal, explanatory, questioning, and writing skills, the healthcare professional uses effective listening skills and elicits and provides information.

  • 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
    Associate Professor of Pediatrics, Children’s Hospital of Iowa

    Date
    June 26, 2006

  • How Common is Infantile Botulism?

    Patient Presentation
    A 7-day-old term female infant presented to the emergency room with several hours of decreased energy and poor feeding.
    She is noted to be grey in color with poor perfusion, decreased tone, weak cry, and no respiratory distress but poor effort.
    Resuscitation was begun. She had intravenous catheters placed with fluid boluses given and ampicillin, gentimicin and acyclovir begun.
    The pertinent physical exam showed a heart rate of 100, blood pressure of 52/28, respiratory rate of 40, and a pulse oximetery was not registering.
    Her pupils were 7-8 mm and not reactive to light. Her fontanelle was normal. There was no rash, or bruises. Capillary refill was 3 seconds.
    Cardiac examination revealed normal S1, S2 and no murmur. Abdomen was soft with no obvious organomegaly.
    Neurological examination revealed decreased tone, withdrawal to pain, and her peripheral reflexes were difficult to elicit.
    The laboratory evaluation showed a first blood gas with a pH = 7.14, CO2 = 56, O2 = 80. Blood cultures were drawn. She had a normal complete blood count, andelectrolytes including calcium, magnesium and phosphorus.
    The patient was then intubated and transferred to a regional children’s hospital.
    Additional work-up included a normal cerebral spinal fluid, and a normal computerized tomography of the head and chest radiograph.
    An electrocardiogram and echocardiogram were also normal.
    She had a normal electroencephalogram but an abnormal electromyelogram which was consistent with a peripheral versus a polymyopathic neuropathy. She did not respond to a tensilon test.
    The additional past medical history revealed normal labor and delivery at home for a G1P1 mother with some prenatal care.
    The patient was seen by a nurse midwife at delivery, day 1 and day 2 of life and was doing well. Her neonatal screen was normal. She did not receive Hepatitis B, Vitamin K or gonococcal conjunctivitis prophylaxis.
    She was breastfeeding well until several hours before coming to the emergency room.
    The parents denied honey ingestion and the family history revealed no neuropathy or neurological diseases.
    The diagnosis of presumed infantile botulism was made and the infant was given botulinum antitoxin.
    At day 14, Clostridium botulinum type A was recovered from her stool and she remained on life support. She was discharged home after a few weeks.

    Discussion
    A newborn infant presenting with acute deterioration causes health care providers to quickly go through a long differential diagnosis covering all organ systems as they attempt to resuscitate and stabilize the infant.
    Causes are numerous and the physicians in this case entertained some of the following in their differential:

    • Sepsis/Infection – Group B streptococcal infection, Herpes simplex, necrotizing enterocolitis, etc.
    • Congenital heart disease
    • Hematologic – bleeding secondary to sepsis, Vitamin K deficiency, etc.
    • Metabolic abnormalities – endocrinopathies, nephropathies, inborn error of metabolism, etc.
    • Neurologic – seizures, congenital or acquired neuropathies, etc.
    • Trauma – inflicted or incidental
    • Toxins/Drugs

    Like most patients, the cause for this patient’s acute deterioration not immediately recognized.
    This patient was resuscitated, stabilized, transported, and then was sustained with ventilatory support and other life sustaining measures for the most common problems such as sepsis and congenital abnormalities.
    Concurrently, her evaluation was begun and based on those results the differential was narrowed down over time and specific treatment initiated.

    Learning Point
    Infantile botulism is not a common problem in the United States generally because of improved obstetrical care, general personal hygiene and education about cultural practices which could be harmful to the infant such as giving honey or placing soil or dung on the umbilical cord.

    Botulism is classified into four categories – foodborne, infantile, wound and undetermined. Symptoms (except for infants) generally occur within hours and evolve over days. It is a decending, symmetric, flaccid paralysis, with cranial nerves palsies being the most common complication.
    Infantile botulism occurs most often in children < 6 months and can be preceeded by constipation, poor feeding, weak cry, ocular nerve palsies, general weakness and hypotonia.
    Human botulism is associated with neurotoxins A, B, E and F. Infantile botulism is almost always associated with types A and B.
    Incubation in infantile botulism is 3-30 days from exposure.
    About 100 cases occur annually of infantile botulism, after ingested spores of Clostridium botulinum are ingested, germinate, replicate and produce toxin in the gut. The toxin produces the paralysis.
    In infantile botulism the source of the spores sometimes cannot be identified and possibly is airborne dust or soil. Honey often is not certified as free of spores and should be avoided especially in the first year of life.
    Corn syrups are manufactured under sterile conditions, but are not packaged under aseptic conditions and do not receive sterilization at the end of packaging and therefore the manufacturers cannot ensure that the product is free of spotes.

    Antibiotics are avoided if possible because Clostridium lysis may increase the amount of toxin. Botulinum antitoxin (human-derived immunoglobulin) is available and in studies decreases the hospitalization length. Overall mortality is ~15%.

    Questions for Further Discussion
    1. Clostridium botulinum is a Class A bioterrorism agent. What are the other Class A agents?
    2. How is Botox® (Botulinum toxin Type A) made safe for human use?
    3. How are the presentations of foodborne and wound botulism different from the infantile form?

    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: Botulism

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

    American Academy of Pediatrics. Botulism and Infant Botulism, In Pickering LD, ed. Red Book: 2003 Report of the Committee on Infectious Diseases. 26th edit. Elk Grove Village, IL: American Academy of Pediatrics; 2003;243-245.

    FrattarrelliDAC, Abdel- Haq NM. Botulism. eMedicine.
    Available from the Internet at http://www.emedicine.com/ped/topic273.htm (rev. 7/9/2004, cited 6/1/06).

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effecively 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 competency performed.
    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.

  • Professionalism
    22. Sensitivity and responsiveness to patients’ culture, age, gender, and disabilities 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
    Associate Professor of Pediatrics, Children’s Hospital of Iowa

    Date
    June 19, 2006