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Urol Case Rep
Urol Case Rep
Urology Case Reports
2214-4420
Elsevier

S2214-4420(24)00129-3
10.1016/j.eucr.2024.102775
102775
Inflammation and Infection
Conservative management of segmental testicular infarction in a patient with sickle cell anemia: A case report
Wu Sarah Y. a
Gibbs Evan Mackenize b
Klose Charles b
Qosja Neda b
Zganjar Andrew b
Baird Bryce A. Baird.Bryce@mayo.edu
b⁎
a Mayo Clinic Alix School of Medicine, Scottsdale, AZ, USA
b Mayo Clinic Florida, Department of Urology, Jacksonville, FL, USA
⁎ Corresponding author. 4500 San Pablo Rd, Jacksonville, FL, 32224, USA. Baird.Bryce@mayo.edu
21 6 2024
9 2024
21 6 2024
56 10277512 6 2024
20 6 2024
© 2024 The Authors
2024
https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Vaso-occlusive crisis is a sequela of sickle cell disease that can lead to severe pain and infarction at the location of occlusion. In men, genitourinary complications include priapism, hematuria, and very rarely, testicular infarction. Few cases have been previously reported in the literature, but in all of those cases, partial or complete orchiectomy was performed. We report the first known case of segmental testicular infarct secondary to sickle cell disease treated with medical management without need for surgical intervention.

Keywords

Testicular infarction
Sickle cell crisis
Exchange transfusion
Segmental infarction
Testicle
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pmc1 Introduction

Sickle cell trait (SCT) is one of the most common hematological disorders worldwide. SCT refers to the varying clinical manifestations of mutant hemoglobin genotypes, of which sickle cell disease (SCD) is the most clinically severe form.1

One well-characterized complication associated with SCD is vaso-occlusive crisis, caused by HbS polymerizing in the de-oxygenated state, resulting in sickle-shaped red blood cells (RBCs) that can occlude small capillaries, causing severe pain and ischemia.2 In men with SCD, the most common genitourinary complication is priapism, reportedly occurring in 35–42 % of patients with SCD.2,3

A less commonly reported urologic manifestation of vaso-occlusive crisis is testicular infarction, with only 7 reported cases to date. Prior reported cases of testicular infarction have been managed with orchiectomy or testicular exploration leading to resection. In this article, we present the first known case of testicular infarction managed conservatively without surgical intervention.

2 Case presentation

A 33-year-old male with SCD presented to the emergency department with left lower quadrant abdominal pain, joint pain, nausea and vomiting. The patient reported that this prodrome was typical of previous sickle cell crises. He has a significant history of SCD complications, including acute chest syndrome, avascular necrosis of multiple joints, and liver infarcts. He was previously taking hydroxyurea; however, he discontinued this due to adverse side effects and lack of insurance coverage. His home medications included folic acid supplements and laxatives. He had a past surgical history significant for cholecystectomy with no prior urologic surgeries.

Upon admission to the hospital, he was normotensive and afebrile. Vaso-occlusive crisis was suspected. Laboratory studies revealed anemia (Hb = 8.2 g/dL), thrombocytosis, leukocytosis, and hyperbilirubinemia (total = 3.3, direct = 0.7). CT of abdomen and pelvis showed no acute inflammatory abdominal processes. Chest X-ray showed no evidence of acute chest syndrome. He was initially managed with aggressive IV fluids and multimodal pain control, with brief improvement of pain, leukocytosis and hyperbilirubinemia.

Two days later, he reported worsening pain with new-onset radiation to his left testicle. On physical examination, his left testicle was tender to palpation with no other significant findings. At this time, a scrotal ultrasound with Doppler was obtained and showed an approximately 1.6 x 1.2 × 1.1 cm wedge-shaped area of decreased echogenicity and decreased vascularity likely representing a subacute infarct (Fig. 1, Fig. 2). The surrounding testicular parenchyma and contralateral testicle had normal echotexture and vascularity.Fig. 1 Ultrasound showing hypoechogenic area of infarct.

Fig. 1

Fig. 2 Doppler ultrasonography of testicle demonstrating area of infarct.

Fig. 2

As there were no indications for urgent surgical intervention with low concern for testicular torsion or underlying malignancy, conservative therapy to treat sickle cell crisis and pain management were planned. The patient received an exchange transfusion and 1 unit of HbS-negative blood with improvement of HbS to 25.8 % from 77.4 %. He continued to improve post-exchange transfusion with no further urologic complications. By hospital day 6, his pain improved with reduction in need for pain regimen, and he was discharged with plans to follow up in clinic 4–6 weeks later for repeat imaging. Unfortunately, the patient was lost to urology follow-up due to lack of insurance. However, he saw primary care providers approximately 6 months after his initial presentation and had improved physical examination.

3 Discussion

Segmental testicular infarction is a rarely reported manifestation of vaso-occlusive crisis in patients with SCT or SCD. Only 7 cases have been previously reported in the literature, 1 of which is reported in German and thus excluded from discussion. Patients often present with acute onset testicular pain, progressively worsening with associated swelling and tenderness on physical exam.4, 5, 6, 7, 8, 9 In 2 cases, antibiotics were prescribed for suspicion of underlying infection with no effect.4,5 None of the previously reported patients nor our patient were taking hydroxyurea at the time of presentation. While most of these cases occurred in the setting of diagnosed SCT or SCD, testicular infarction has also been reported as the first indication of underlying SCT or SCD.5,7,8

Common laboratory findings included anemia, leukocytosis, thrombocytosis, and hemoglobin electrophoresis showing elevated HbS level. Scrotal ultrasound with Doppler showed a well-circumscribed, hypoechogenic area with decreased or absent arterial flow, while surrounding parenchyma exhibits normal echogenicity and blood flow. A major reported diagnostic issue leading to surgical intervention was inability to distinguish infarct from malignancy on ultrasound. Serum tumor markers (bHCG and AFP) were drawn in 2 reported cases and resulted negative.6,8 Conservative therapy including IV fluids, pain management, and high-dose hydroxyurea were trialed in 2 cases without resolution of symptoms.4,9 Radical or partial orchiectomy were eventually performed in all reported cases, and pathology showed hemorrhagic necrosis, inflammation and sickling. Our case is unique in that surgical intervention was unnecessary.

Medical therapies for testicular infarction in patients with SCT or SCD logically follow those of other vaso-occlusive crises and includes pain management, preventative pharmacotherapy, and exchange transfusions.10,11 Of the preventative pharmacotherapy options, hydroxyurea is one of the most commonly used in adults with pain crises. Hydroxyurea, which primarily works by increasing the levels of fetal hemoglobin (HbF), has been shown to cause a 44 % reduction in vaso-occlusive crises, hospitalization and need for transfusions.10, 11, 12 Finally, exchange transfusions are used to decrease the level of pathological HbS.

In our patient, hydroxyurea therapy was not considered due to a history of adverse effects; however, supportive treatment including IV fluids, adequate pain management and exchange transfusions were used to excellent effect. While surgical intervention was unnecessary following initial treatment, surgery may be considered to shorten the disease course or when the diagnosis of infarct is unclear.

4 Conclusion

Testicular infarction as a manifestation of SCT or SCD is a rare but potentially significant urologic complication. This diagnosis can be suspected in patients with acute, progressive testicular pain and swelling. Scrotal ultrasound with Doppler shows a well-circumscribed hypoechoic area and significantly decreased intratesticular flow. Orchiectomy is often pursued due to an inability to rule out malignancy from imaging alone. As discussed in this case, medical management including supportive therapy, preventative pharmacotherapy, and exchange transfusions can be effective, presenting an option for conservative management of this rare condition.

Conflict of interest

The authors have no conflicts of interest to declare.

Funding

There was no funding obtained for this research.

CRediT authorship contribution statement

Sarah Y. Wu: Conceptualization, Writing – original draft, Writing – review & editing. Evan Mackenize Gibbs: Writing – review & editing. Charles Klose: Validation, Writing – review & editing. Neda Qosja: Writing – original draft, Writing – review & editing. Andrew Zganjar: Supervision. Bryce A. Baird: Conceptualization, Writing – review & editing.
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References

1 Ware R.E. de Montalembert M. Tshilolo L. Abboud M.R. Sickle cell disease Lancet 390 10091 2017 311 323 10.1016/S0140-6736(17)30193-9 28159390
2 Bruno D. Wigfall D.R. Zimmerman S.A. Rosoff P.M. Wiener J.S. Genitourinary complications of sickle cell disease J Urol 166 3 2001 803 811 11490223
3 Huang A.W. Muneyyirci-Delale O. Reproductive endocrine issues in men with sickle cell anemia Andrology 5 4 2017 679 690 10.1111/andr.12370 28662541
4 Alsulmi H.A. Testicular infarction in a patient with sickle cell anemia: a case report Int J Health Sci 12 5 2018 100 102
5 Holmes N.M. Kane C.J. Testicular infarction associated with sickle cell disease J Urol 160 1 1998 130 9628625
6 Li M. Fogarty J. Whitney K.D. Stone P. Repeated testicular infarction in a patient with sickle cell disease: a possible mechanism for testicular failure Urology 62 3 2003 551
7 Urwin G.H. Kehoe N. Dundas S. Fox M. Testicular infarction in a patient with sickle cell trait Br J Urol 58 3 1986 340 341 10.1111/j.1464-410x.1986.tb09075.x 3719262
8 deBeck C. Ghasemian R. Acute segmental testicular infarction Int J Urol 13 5 2006 651 652 10.1111/j.1442-2042.2006.01367.x 16771749
9 Gofrit O.N. Rund D. Shapiro A. Pappo O. Landau E.H. Pode D. Segmental testicular infarction due to sickle cell disease J Urol 160 3 Pt 1 1998 835 836 10.1016/S0022-5347(01)62803-9 9720564
10 Charache S. Terrin M.L. Moore R.D. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia N Engl J Med 332 20 1995 1317 1322 10.1056/NEJM199505183322001 7715639
11 Darbari D.S. Sheehan V.A. Ballas S.K. The vaso-occlusive pain crisis in sickle cell disease: definition, pathophysiology, and management Eur J Haematol 105 3 2020 237 246 10.1111/ejh.13430 32301178
12 Bunn H.F. Pathogenesis and treatment of sickle cell disease N Engl J Med 337 11 1997 762 769 10.1056/NEJM199709113371107 9287233
