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Persistent painful purposeless erection of the penis (or clitoris) more than 6 hours
EPIDIMIOLOGY
INCIDENCE
Cases per 100,000 person-years.
AGE
All age groups (including newborns)
Peak incidence 20 to 50yrs.
Younger age group associated with sickle cell disease
Peak incidence in sickle cell patients between 19 & 21 years.
SEX
Priapism is a disease of males.
Clitoral priapism has been described rarely
PATHOLOGY
CLASSIFICATION
Low flow or Ischaemic (veno-occlusive)
Most common
Painful sec to tissue ischaemia and smooth muscle hypoxia (compartment syndrome)
Penis fully erect (sludging of blood within)
Blood gases from corpora - acidosis
NO & prostacyclin
Platelet aggregation and adhesion - thrombus formation and tissue damage
Priapism Variants
ETIOLOGY
Causes of low-flow priapism
IDIOPATHIC
Most common cause.
Priapism occurring without any discernible cause is considered to be idiopathic.
Some investigators have estimated that this disorder accounts for as many as half of all
documented cases
SECONDARY
SYSTEMIC
Blood diseases
Hyperviscosity syndromes (sickle-cell disease)
Most common cause in children
42% incidence in adults with sickle-cell disease
64% incidence in boys with sickle-cell disease
Other haemoglobinopathies
Leukemic disorders
Thrombophilia
Multiple myeloma
Neurological causes
Rare
Lumbar disc lesions, spinal stenosis, seizure disorders, cerebrovascular disease
Drugs
Psychotropic drugs
Phenothiazines
Butyrophenones
Hydralazine
Prazosin, labetolol, phentolamine and other -blockers
Testosterone
Metoclopramide
Calcium-channel blockers
Anti-coagulants
Tamoxifen
Omeprazole
Hydroxyzine
Cocaine, marijuana, and ethanol
Miscellaneous
TPN, amyloid , rabies, appendicitis
LOCAL
Intracavernosal pharmacotherapy
21%
E.g. Papaverine and PGE-1 (alprostadil) intracavernosal injection.
extremely low incidence with oral agents
Post Trauma
Perineum, groin or penis
Usually cause high flow priapism but can cause low flow sec to haematoma
Solid Tumors
Malignant infiltration of corpora
High-flow priapism
Trauma
sickle-cell disease: Very rare
Fabry`s disease
PATHO-PHYSIOLOGY
- Due to one of the above etiologic factors disturbances in detumescence mechanisms
(inflow >> outflow) will happened Persistent erection of corpora cavernosa
- Corpora spongiosum of the glans and peri-urethral region rarely affected
- Recurrent episodes of priapism can result in enlarged penis, fibrotic corpora and ED
COMPLICATION
Untreated priapism leads to corporal fibrosis and impotence
early complications:
• CVS: Acute hypertension, headache, palpitations, arythmias
• Bleeding, haematoma
• Infection
• Retention
• Urethral injury
late complications:
Fibrosis and impotence
Related to type, duration of priapism and aggressiveness of treatment
Incidence: about 50%
Longer duration implies greater risk of impotence
Low-flow high incidence of ED if not treated within 12 hours
High flow good prognosis (20% rate of ED)
Fibrosis makes prosthesis placement difficult
Gangrene: due to ischemia and infection
DIAGNOSIS
HISTORY
History of disease
Prolonged erection more than 6h without purpose or sexual need which is painful
Pain is more prominent with low flow.
History of etiology
Low flow priapism may give history of ICI, Sickle cell disease, Leukemia….etc
High flow priapism may give history of perineal or pelvic trauma
About 50% of cases has no definite etiology.
History of complication
Which is more common with low flow (veno-occlusive erection).
Veno-occlusive priapism
painful erection, present for hours to days
ask about
trauma
drugs - therapeutic and illicit
self-injection
sexual stimulation
history of similar or
predisposing factors
drug history
Veno-occlusive low-flow priapism Arterial high-flow priapism
Past history
Pain painful less painful
Tumescent
Prognosis
PAST H
FAMILY H
EXAMINATION
GENERAL EXAM
Erect or semi-erect penis with a flaccid glans
Search for signs of trauma
Search for other possible
Signs of sludging - florid
Skin, petechiae,
Conjunctival injection
LOCAL EXAM
INVESTIGATION
LABORATORY
ROUTINE
urinalysis
a complete blood count may help identify a previously undiagnosed leukemia
SPECIFIC
haemoglobin S to outrule leukaemia
? Local blood gas measurments
IMAGING
radionucleotide scanning - no longer performed
colour doppler ultrasonography
OTHERS
D.D
TREATMENT
Goal is to
Abort the erection, thereby preventing permanent damage to the corpora (ED).
Relieve pain.
TREATMENT MODALITIES
Treat causal factor where identified
principle is to restore arterial inflow and venous outflow
Sickle-cell –
prompt and conservative as it recurs
Hydration, oxygenation, metabolic alkalinization
Aspiration and injection (as above)
Stuttering priapism
Self injection of -adrenergic agent if sexually active (prophylactic digoxin) or oral -
adrenergic agent (Etilefrine)
Antiandrogen if not to suppress nocturnal tumescence
Low-flow due to sickle cell disease
hydration
alkalinization
analgesia
possible exchange transfusion to get Hgb > 10 gm% and HbS <30%
intracorporeal -adrenergic may be necessary
El-Ghourab procedure
excision of a piece of tunica albuginea
30% of above techniques fail
corpora-saphenous shunt
High-flow priapism
Ice pack arterial spasm
?? spontaneous thrombosis
Most cases require arteriography and embolisation of the internal pudendal artery or a branch
Arterial priapism - 4 options
Mechanical: iced compression
Pharmacologic: -agonists (watch out for systemic blood pressure rise)
Surgical: fistula ligation (usually leads to impotence)
Selective embolization: new procedure with varying degrees of success
TREATMENT STRATEGIES
FOLLOW UP
PROGNOSIS