Recurrent haematospermia: an unusual presentation of autosomal dominant polycystic kidney disease

Abstract

Haematospermia, even though alarming, is usually benign and self-limiting, especially in a sexually active male. Nevertheless recurrent, refractory or painful haematospermia is troublesome and warrants thorough evaluation. In this context, we describe a rare case of recurrent haematospermia whereby evaluation revealed haemorrhage in seminal vesicle cysts and consequently established the aetiology of autosomal dominant polycystic kidney disease.

Background

Haematospermia is described as the macroscopic presence of blood in the semen. It usually just requires reassurance and monitoring after excluding pseudo-haematospermia which can either be due to gross haematuria, or the source being the patient’s sexual partner (menstruation, vaginal microtears). Haematospermia, if recurrent, refractory or painful, mandates evaluation to rule out underlying infection, ejaculatory obstruction, malignancy and vascular malformation. Seminal vesicle cysts in autosomal dominant polycystic kidney disease (ADPKD) often remain asymptomatic, however, may present as recurrent urinary tract infection, epididymitis, dysorgasmia, haematuria, pelvic pain and rarely as haematospermia, particularly if associated with cyst haemorrhage.

Case presentation

A man in his mid-30s presented with recurrent episodes of haematospermia for 6 months. The episodes were unrelated to sexual intercourse and resolved spontaneously within a week to recur again (three to four episodes/month). Initially, considering the possibility of genitourinary tract infection, the patient was empirically prescribed oral antibiotics by a general practitioner. However, in view of minimal symptomatic relief and recurrence of haematospermia, the patient was referred to our hospital for a detailed evaluation. The patient denied history of fever, dysuria, haematuria, flank pain, weight loss, dyspareunia or pelvic trauma. Clinical evaluation was remarkable for bilaterally enlarged, ballotable, non-tender kidneys. The prostate was non-tender on digital rectal examination (DRE). Macroscopic examination of the ejaculate suggested haematospermia.

Investigations

Laboratory investigations including complete blood count, liver and coagulation profile were normal. Serum creatinine was 0.8 mg/dL (0.5–1.2 mg/dL) and urine microscopy did not reveal any active sediment. Serum procalcitonin level was 0.1 ng/mL (0.1–0.25 ng/mL). Microscopic examination of semen confirmed erythrocytes along with spermatozoa (figure 1) in the absence of leucocytes. Sentinel cultures including urine, blood and semen were sterile. Serum prostate-specific antigen level was 1 ng/mL (0–2.5 ng/mL). Transabdominal ultrasound (TAUS) revealed bilateral enlarged cystic kidneys (right kidney 16.7 × 9.8 cm and left kidney 17.4 × 9.5 cm) with multiple cysts in the liver. MRI abdomen was consistent with polycystic kidneys with extrarenal cysts in the liver as well as bilateral seminal vesicles with shading on T2-weighted image sequence and susceptibility artefacts on gradient echo sequence suggestive of haemorrhage within seminal vesicle cysts (figure 2), presumptive aetiology of haematospermia in our patient.

Figure 1.

Light microscopy of semen showing erythrocytes along with spermatozoa in the absence of leucocytes.

Figure 2.

T2-weighted MRI demonstrating bilateral seminal vesicle cysts with T2 shading in the right-sided cyst—consistent with haemorrhage within.

Differential diagnosis

In this man with recurrent episodes of haematospermia, the possibility of either infective (urinary tract infection, prostatitis, epididymitis) or obstructive aetiology (postinfection fibrosis or compression of the ejaculatory ducts) was considered initially. However, non-tender prostate on DRE with bland urine sediment and sterile sentinel cultures including urine, blood and semen made these possibilities unlikely. Bilaterally enlarged, ballotable and non-tender kidneys suggested ADPKD, which was substantiated by radiology and later confirmed by family screening. Further, MRI revealed cyst haemorrhage in bilateral seminal vesicle cysts, thereby circumventing the need for transrectal ultrasonography (TRUS).

Treatment

The patient was counselled about the natural history of haemorrhage in renal and extrarenal cysts in ADPKD and was managed conservatively.

Outcome and follow-up

The patient’s symptoms resolved spontaneously. At 1 year of follow-up, the patient has remained asymptomatic with no recurrence of symptoms thereafter. Screening of family members revealed ultrasonographic evidence of polycystic kidneys with hepatic cysts in his first-degree relative, who is otherwise asymptomatic.

Discussion

Extrarenal cysts in ADPKD are most frequent in the liver (over 50%), followed by pancreas (10%), arachnoid cysts (5%) and spleen (3%). Cysts in the lower male genitourinary tract^{1 2} (epididymis, seminal vesicle,³ testis and prostate⁴) are relatively infrequent.

Seminal vesicle cysts in ADPKD are congenital with reported prevalence varying from 6% by TAUS to 30% by TRUS.⁵ Usually asymptomatic, the patient may occasionally present with recurrent urinary tract infection, epididymitis, dysorgasmia, pelvic pain, infertility and haematuria.

Haematuria is a known complication of ADPKD and may result from varied aetiology including cyst haemorrhage, nephrolithiasis, infection and, rarely, renal cell or urothelial carcinoma. Gross haematuria in ADPKD may infrequently result in pseudo-haematospermia.

Approximately 6% of simple renal cysts in ADPKD are complicated by haemorrhage, usually as a result of trauma, enlargement or bleeding diathesis. Nevertheless, episodes of cyst haemorrhage in ADPKD are usually self-limiting and resolve within a week.

Haematospermia is most commonly caused by urogenital infections, especially in men younger than 40 years.⁶ Haemorrhage in seminal vesicle cysts manifesting as recurrent haematospermia is rare⁷ however, ADPKD presenting as recurrent haematospermia consequent to seminal vesicle cyst haemorrhage is infrequently reported.⁸

A diagnostic workup⁹ of haematospermia includes careful history, physical examination, laboratory testing and imaging. It is recommended to question the patient about the duration and amount of bleeding, as well as sexual and voiding habits. DRE for assessment of prostate guides to aetiology; tenderness indicates infection, whereas a hard or nodular feel is indicative of neoplasm, especially in an elderly patient. The urethral meatus should be re-examined for presence of bloody discharge after DRE. Presence of leucocytes in semen warrants a urethral swab for exclusion of infectious aetiology including bacteria, Chlamydia trachomatis and Ureaplasma urealyticum. If clinically indicated, serology for Cytomegalovirus, herpes simplex virus and Schistosoma haematobium is recommended.⁶

MRI¹⁰ remains the modality of choice for evaluation of recurrent haematospermia. Hyperintense signal at precontrast T1-weighted MRI with no enhancement after contrast administration is indicative of haemorrhage in seminal vesicles as well as in the prostate. T2- weighted MRI with fat saturation is the key sequence for assessment of inflammation. In addition, MR angiography may provide further information for localisation of bleeding in refractory haematospermia.

The cornerstone of management of haematospermia depends on the underlying pathological conditions, if detected.¹¹ Haematospermia can be very stressful for both patients and their sexual partners. While younger patients associate it with infertility or sexual dysfunction, elderly subjects may worry about underlying malignancy. In most instances, bleeding is slight and self-limiting, and relieving patients’ anxieties is fundamental for management. It is, therefore, considered important to accurately recognise the stress levels of individual subjects. When a patient’s anxiety cannot be appropriately relieved through good communication and thorough investigations, psychological consultation may be warranted.

Conclusion

Recurrent haematospermia consequent to haemorrhage in seminal vesicle cysts is an infrequent presentation of ADPKD and hence has been reported for its novelty.

Patient’s perspective.

I was very apprehensive and bothered from frank blood in semen for last six months. Initially, my doctor attributed it to sexual intercourse with my wife, but later I noticed similar episodes despite abstaining from sexual intercourse. Further, I was informed about possibility of genito-urinary tract infection and was prescribed antibiotics, with minimal relief of symptoms. Consequentially, I got indulged in taking over the counter drugs. The episodes used to resolve within a week to recur again (3–4 episodes/ month) which was troubling me and motivated me for a detailed evaluation. Finally, I was diagnosed to have autosomal dominant polycystic kidney disease with hemorrhage in seminal vesicle cysts. I have been counselled and reassured about my condition with need of regular follow-up.

Learning points.

• Haematospermia is an anxiety-provoking but self-limiting benign condition, particularly in a sexually active male.

• Recurrent, refractory or painful haematospermia mandates evaluation for underlying infection, ejaculatory obstruction and malignancy in the elderly.

• Seminal vesicle cysts in autosomal dominant polycystic kidney disease (ADPKD) are usually asymptomatic and recurrent haematospermia consequent to haemorrhage in seminal vesicle cysts is an infrequent presentation of ADPKD.

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