Partial Priapisim: A rare presentation of sickle cell anemia

Abstract

Introduction

Partial segmental thrombosis of the corpus cavernosum (PSTCC); known as partial priapism; is an uncommon urological condition which predominantly affects young men in which the proximal part of one corpus cavernosum is thrombosed. Many risk factors are described in the literature, the exact etiology of penile thrombosis and its pathogenesis remains unclear. Several treatment options are available ranging from conservative medical treatment with NSAIDs, antibiotics, analgesics, low molecular weight heparin, acetylsalicylic acid and antibiotics, surgical or to a follow-up observation without treatment. In this study we presented a sickle cell patient who presented with pain and perineal swelling and diagnosed with PSTCC using MRI and was treated conservatively.

Case description

A 23-year-old male, known case of sickle cell anemia, presented to casualty with a 1-day history of perineal pain of a sudden onset, increasing in severity, no aggravating or relieving factors. It was associated with perineal swelling, decrease in urine output and vomiting, not associated with urethral discharge, erectile dysfunction, trauma, sexual contact, fever, abdominal pain, lower urinary tract symptoms, change in bowel habits, or bleeding per rectum. He had a past history of left pyeloplasty in childhood. He was a smoker, non-alcohol consumer with a family history of liver malignancy. Examination revealed a stable vitals, abdomen was soft and non-tender, genitourinary exam findings confirmed the absence of priapism. There was a normal circumcised penis, normal bilateral testis and epididymis, separated perineal mass slightly hard in consistency, fixed and tender at the proximal part of the penis. Digital rectal examination was unremarkable. The complete blood count showed mild leukocytosis, electrolytes, coagulation profile, urine analysis and urine culture were unremarkable. MRI perineal and penis showed the right intratunical corpus cavernosum with altered signal intensity involving the root, proximal and mid third sparing the distal third of corpus cavernosum, maximum width of 26mm (predominantly hyposignal intensity with few areas of hypersignal intensity). Visualized Buck’s fascia and tunica albuginea were intact. Features mostly suggestive of right corpus cavernosum hematoma. Conservative treatment was initiated with 6 hourly IV paracetamol and the response was observed with gradually disappearing pain, reduction in swelling size and leukocytic count. On follow up patient was pain free, reduction in swelling size with a recovery of painless erection. A follow up ultrasound of the scrotum and a hematologist referral were arranged.

Results and conclusions

PSTCC is not an urological crisis and has an excellent prognosis. Conservative treatment appears to be a reliable therapeutic option. Surgery is reserved for patients in whom conservative management fails.