Oral Propranolol in the Treatment of Ulcerated Bleeding Scrotal Arteriovenous Malformation.

Arteriovenous malformations (AVMs) of the scrotum are very rare, with only 35 adult cases in the literature. An 8-year-old boy presented with an ulcerated bleeding AVM of the scrotum. The patient was resuscitated and managed conservatively initially. After the control of bleeding, oral propranolol was started. There was a decrease in the size of scrotal and penile swelling, healing of ulcer with total healing by 1 month, and no recurrence of bleeding episode. To the best of our knowledge, this was the first case of pediatric scrotal AVM treated by oral propranolol. Copyright:

INTRODUCTION

Arteriovenous malformations (AVMs) of the scrotum, involving the testes or other scrotal components, are extremely rare.[1234] To the best of our knowledge, only 35 cases have been reported in medical literature to date. Most of the cases reported were adults with only a few pediatric cases. Scrotal AVMs are usually managed with angioembolization and/or surgery similar to AVMs at any other sites.[45] Few reports have described the use of oral propranolol in the management of AVMs [678] but none in scrotal AVMs. Herein, we report a case of large scrotal AVM extending to the penile shaft, presented with ulceration and acute bleeding, and after local control of bleeding was treated with oral propranolol.

CASE REPORT

An 8-year-old boy presented with progressive and asymmetric enlargement of both scrotum and swelling of the penile shaft since birth and acute bleeding from right-sided scrotal skin for 1 day. Bleeding was spontaneous with no history of any recent or remote obvious trauma or any other diseases. The patient was resuscitated immediately with intravenous fluids and blood transfusion. Local control of bleeding was achieved with direct pressure. After control of bleeding, local examination revealed large irregular swelling involving both scrotum (right > left) and the penile shaft more on dorsal surface. An ulcer of 3 cm × 2 cm size was present on anterolateral side of right hemiscrotum, and there was bleeding from the floor and edges of ulcer. Surrounding scrotal skin was edematous and indurated [Figure 1]. Bilateral testes were normally descended and palpable and were free from overlying swelling and ulcer. Hemogram and coagulation profile were within normal limits. Clinically, the provisional diagnosis of a vascular anomaly was made. Color Doppler Ultrasonography (USG) of the penile and scrotal region showed diffuse vascular lesion, involving subcutaneous tissue of right scrotum, part of left hemiscrotum, and penile shaft showing both arterial and venous waveforms. Both testes were free from the lesion; however, the right testis was smaller in size. A provisional diagnosis of scrotal AVM was made, and it was confirmed with computerized tomography (CT) angiography of scrotum and penis. CT angiography revealed a tangle of vessels in the subcutaneous tissue of right hemiscrotum, part of left hemiscrotum, and penile shaft showing high flow both during the arterial and venous phase. The left testis was normal, but the right testis was smaller [Figure 2].

Figure 1

Enlargement of right hemiscrotum extending to dorsum of penile shaft and adjacent part of left hemiscrotum. An ulcer with indurated margin over anterolateral surface of right hemiscrotum is also seen

Figure 2

Coronal (a) and axial (b) sections of computerized tomography angiography showing tangles of dilated vessels in the skin and dartos tissue of right hemiscrotum penile shaft and part of left hemiscrotum

After the control of bleeding, the child's parents refused any sort of invasive intervention either angioembolization or surgery for definitive therapy. Therefore, after explaining the potential complications or side effects of using propranolol to the child's parents, oral propranolol therapy was started at a dose of 2 mg/kg. The patient was in regular follow-up for 11 months, and then he was lost to follow-up. During the follow-up, there was a gradual decrease in size in scrotal and penile swelling, healing of ulcer with total healing by 1 month, and no recurrence of bleeding episode.

DISCUSSION

Scrotal vascular lesions are unusual and include varicocele, hemangioma, venous malformations, lymphangiomas, other vascular tumors of the soft tissue, arteriovenous fistula, and AVMs.[1234] Among these scrotal vascular lesions, varicoceles are the most common lesions, and the AVMs are the least common.[1234]

Scrotal AVMs are both congenital and posttraumatic. These lesions have a progressive growth with no evidence of involution and present with scrotal swelling, pain, ulceration, acute bleeding or, rarely with impotence or infertility. Sometimes, it is difficult to differentiate scrotal AVMs from other vascular lesions such as varicocele and hemangioma clinically.[9] Although the presence of bruit or thrill has been described as typical of AVMs,[1] it is not always present. Therefore, imaging studies such as color Doppler USG and CT angiography or magnetic resonance imaging with angiography are used to differentiate between various scrotal vascular lesions and extension of vascular lesion to the penis, perineum, and structures of the lesser pelvis.[124] CT/MR angiography can show the arterial feeders and the draining vein and help in planning of management; however, angiography is the gold standard for the exact delineation of anatomy.[245] Since our patient had scrotal swelling, ulceration, and bleeding at the time of presentation but no bruit or thrill, initial clinical diagnosis of bleeding and the ulcerated vascular anomaly was made. The final diagnosis of AVM was reached with the help of color Doppler USG and CT angiography.

Treatment of acute bleeding scrotal AVMs involves local control of bleeding with direct pressure or use of hemostatic agents before definitive treatment in the form of percutaneous sclerotherapy, angioembolization, and/or surgery.[345] Angioembolization may be required for control of acute bleeding if bleeding continues despite direct pressure or the use of hemostatic agents.[4] Most patients had recurrence after attempted resection or embolization.[10] In some patients, the occurrence of necrosis of scrotal skin and gluteal muscles, bladder infarction, and even impotence after embolization or sclerotherapy have been reported.[4] The role of oral propranolol has been well established in the treatment of proliferative infantile hemangiomas and ulcerated and bleeding hemangiomas.[11] Based on it, it's role in the treatment of AVMs has been studied in a few case reports,[678] while a few reports found positive response of oral propranolol in the treatment of AVMs;[67] others have not found any clear efficiency.[8] The proposed mechanisms of action of propranolol for remission of proliferative infantile hemangiomas include nonselective vasoconstriction, decreased expression of growth factors such as vascular endothelial growth factor (VEGF) or basic fibroblast growth factor contributing to inhibition of angiogenesis and apoptosis of capillary endothelial cells through β-ADR signaling and the caspase pathway, inhibition of CD34þ/VEGF-2þ endothelial progenitor cells through the renin–angiotensin pathway, and inhibition of angiogenesis by control of pro-angiogenic cytokines such as interleukin-6.[12]

We have used oral propranolol in our patient and found quick healing of ulcer and slow but convincing reduction in the size of scrotal swelling during 11-month follow-up although then patient lost to follow-up.

CONCLUSION

Scrotal AVMs should be considered in the differential diagnosis of scrotal vascular lesion presenting with scrotal swelling, pain, or ulceration or acute bleeding even in the absence of bruit. Usual definitive therapy includes complete surgical excision and/or angioembolization. Oral propranolol may have a role in the remission of scrotal AVMs. Further studies will be required to establish the role of propranolol in the treatment of scrotal AVMs so that it can be used as an alternative treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.