Case presentation:
A 28-year-old male known to have beta thalassemia major blood
transfusion-dependent, maintained on iron chelators for secondary
hemochromatosis presented with bilateral lower limb edema, scrotal, and
penile edema of 1 week duration and respiratory distress. He was found
to have acute kidney injury and metabolic acidosis secondary to
hemolysis and uric acid nephropathy with Uric acid level of 22.3 mg/dL
and LDH of 454 IU/L creatinine of 0.6 mg/dL (baseline 0.25 mg/dL). We
present his case for the unusual finding of edema of the urethral
meatus, as shown in figure 1, with penoscrotal edema in the setting of
volume overload.
The patient was admitted to the hospital for monitoring in the intensive
care unit for his acidotic state and volume status. Patient tolerated
diuresis using Lasix, electrolyte derangements were corrected, he was
administered allopurinol for his hyperuricemia, and oxygen was
administered via nasal canula and BiPAP. He was discharged after
monitoring, proper diuresis, and return of creatinine to baseline
levels.
Patients with Beta thalassemia major are dependent on regular blood
transfusions, especially early in life. These patients may develop
cardiomyopathy or pulmonary hypertension due to volume overload
secondary to these transfusions commonly manifested by bilateral lower
limb edema. (1) As the condition worsens, edema may manifest in other
regions such as ascites in the abdomen and peno-scrotal edema.
It is essential in patients with kidney injury to rule out post-renal
causes such as acute urinary retention as it may contribute to the
volume status of the patient as reported in the literature. (2) Yet
there are no reports on the unusual involvement of the urethral meatus
in penile edema such as that seen in our patient in the setting of
volume overload in Beta thalassemia major.
Management of such cases vary in terms of the severity of the kidney
injury, its chronicity, and severity of volume overload. Edema of the
urethral meatus may indicate a more severe form of volume overload
compared to only lower extremity edema.