SUBMITTED 8 DEC 21 1 REVISIONS REQ. 21 FEB 22; REVISIONS RECD. 5 APR 22 2 ACCEPTED 19 APR 22 3 ONLINE-FIRST: APRIL 2022 4 DOI: https://doi.org/10.18295/squmj.4.2022.034 5 6 Recurrent scrotal Arteriovenous Malformation as a Slowly 7 Increasing Left Testicular Swelling 8 A case report 9 Faisal M. Alashaikh,1,2 *Moustafa S. Alhamadh,1,2 Sulaiman I. 10 Alsugair,1,2 Abduallah Alkhayal,1,2,3 Saad Abumelha,1,2,3 Yousof Al 11 Zahrani,2,4 AbdulRahman BinSwilim5 12 13 1College of Medicine, King Saud ben Abdulaziz University for Health Sciences 14 (KSAU-HS), Ministry of the National Guard-Health Affairs, Riyadh, Saudi Arabia; 15 2King Abdullah International Medical Research Center, Ministry of the National 16 Guard-Health Affairs, Riyadh, Kingdom of Saudi Arabia; 3Department of Surgery, 17 Division of Urology and 4Department of Medical Imaging, Division of Interventional 18 Radiology, King Abdulaziz Medical City, Ministry of the National Guard-Health 19 Affairs, Riyadh, Kingdom of Saudi Arabia; 5Department of Medical Imaging, King 20 Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia. 21 *Corresponding Author’s e-mail: Alhamadhmo@gmail.com 22 23 Abstract 24 Arteriovenous malformations (AVMs) are benign vascular lesions. Although, the 25 majority of AVMs occur in the central nervous system, there are published reports of 26 AVMs involving all systems including the scrotum, kidney, and uterus. Herein we 27 report a case of 37 years old male presented with recurrent gradual scrotal swelling 28 for 4 years attributed to scrotal AVM. Embolization was done but one year later his 29 symptoms reoccurred. As a result, left partial scrotal wall excision was carried out 30 without complications. 31 Keywords: arteriovenous malformation, AVM, scrotal swelling, scrotal malformation. 32 33 Introduction 34 Arteriovenous malformations (AVMs) are benign vascular lesions. They are described 35 as abnormal vessels fed by arteries and drained by veins without intervening 36 capillaries.1,2 Although the majority of AVMs occur in the central nervous system, 37 there are published reports of AVMs involving the scrotum, kidney, and uterus.2,3,4 38 Few cases of scrotal AVMs have been described in the literature. Based on the 39 published reports, the clinical presentation of scrotal AVMs is highly variable, 40 ranging from an incidental finding on imaging for infertility to a bleeding mass.1,5 41 Since scrotal AVMs have variable presentations and is rarely described in the 42 literature, we are reporting a case of a 37-year-old male presented with a slowly 43 increasing left testicular swelling attributed to scrotal AVM. 44 45 Case Report 46 37 years old male smoker presented to the urology clinic with a gradual scrotal 47 swelling that started four years ago. He complained of on and off scrotal pain, 48 occasional feeling of scrotal warmth, and scrotal discomfort. The patient was 49 diagnosed in another hospital with a testicular artery aneurysm and left testicular 50 varicocele. The patient denied any history of trauma, urinary tract infection, voiding 51 symptoms, previous surgeries, and his past medical history was unremarkable. Upon 52 physical examination, the testes were intra-scrotal. There were no signs of 53 inflammation, and the cremasteric reflex was intact bilaterally. Both epididymides 54 were palpable and non-tender. However, pampiniform plexus at the neck of the 55 scrotum was very pulsatile (figure A). Moreover, multiple skin varices over the left 56 scrotum were seen. Urinalysis was normal and urine culture was negative. Routine 57 laboratory tests were unremarkable. Abdominal and pelvis computed tomography 58 (CT) showed left scrotal arteriovenous malformation with enlarged small and 59 medium-sized serpiginous structures with a feeder artery arising from the proximal 60 superficial artery. Two months later, the patient was referred to interventional 61 radiology for embolization, which was successfully done utilizing Onyx 18% (figure 62 B). After one year, on follow-up, the pampiniform plexus were pulsatile again which 63 necessitated a CT angiogram. CT angiogram confirmed the recurrence of 64 arteriovenous malformation. The patient was counselled about the available treatment 65 options and given time to decide. Due to the risk of recurrence as well as the 66 possibility of technical failure with embolization, he decided to go with the surgical 67 treatment. The patient was booked for surgery, and partial scrotal wall excision was 68 done through an elliptical incision (figure C). Three arteries that feed into the 69 arteriovenous malformation were identified and controlled with vicryl ties. The 70 malformation and the skin that covering it were removed and sent to the pathology 71 lab. Dartos muscle was closed in a multi-fashion layer. The skin was closed by vicryl 72 rapide 4-0 in a vertical mattress. The histopathological study confirmed the diagnosis 73 by detecting vascular structures extending from fibrofatty tissues measuring 8x0.5 cm 74 grossly, and prominent subcutaneous large congested vascular spaces 75 microscopically. The patient was discharged one day after the surgery with no 76 complications. Two months postoperatively, the patient was doing fine with no active 77 complaint, and the wound healed properly. 78 79 The consent was obtained orally as the images were taken from the patient in the 80 clinic. We explained to him the importance of reporting and publishing his case for 81 educational purposes, and he agreed. 82 83 Discussion 84 AVMs are malformations in the circulatory system characterized by arteries and veins 85 that are not connected by capillaries leading to various degrees of ischemia and 86 pain.1,2 Even though Central nervous system cases represent the majority of AVMs, 87 there are published reports of AVMs involving the kidney, uterus, and scrotum.2,3,4 88 AVMs are rarely present in the urinary tract.6 We reviewed four previously published 89 scrotal AVM cases (Table 1). All revealed ages ranging from 19 to 31 years while our 90 patient was 37 years old. Scrotal AVM embraces wide-ranging presentations 91 including infertility, acute recurrent pain in the hemiscrotum, pain and swelling on the 92 testicle, and progressive diffused swelling in the scrotum with flashing skin and local 93 warmth. Our case presented with gradually increasing left testicular swelling with on 94 and off scrotal pain, and occasional feeling of scrotal warmth and discomfort. Of the 95 four cases we have reviewed, three denied any history of trauma, and one had a 96 positive trauma history which was a severe pelvic fracture, and the patient indicated 97 that there is difficulty in maintaining erection since. Our patient denied any trauma 98 history. Varicocele was found in two of the cases and was seen by sonography 99 whereas our patient had multiple skin varices above the left scrotum that was seen 100 during physical examination. Each one of the four cases we reviewed diagnosed 101 scrotal AVM with a different modality. Some were challenging and required 102 orchiectomy for a diagnosis while others were simple and detected by pelvic 103 angiography. Our patient was diagnosed by abdominal and pelvic CT. Two studies 104 were able to find and embolize the feeding arteries. Our patient underwent 105 embolization but had recurrence one-year later. Similar to our case, surgical 106 intervention was eventually done in all four cases, and it varied from left scrotal AVM 107 excision, orchiectomy, ileo-femoral bypass, and resection of the whole left side of the 108 scrotum. In our case, partial scrotal wall excision was done. After the surgical 109 intervention, all patients were symptom-free.5,6,7,8 We believe that the difference in 110 the presentation could be attributed to the location of the AVM, onset, duration, and if 111 there is a history of trauma. A possible explanation for the differences in imaging 112 modalities used to diagnose scrotal AVMs is the availability of imaging techniques in 113 the hospitals that encountered those cases. The decision of surgical intervention is 114 mainly based on the symptoms and how symptoms negatively affect the patient's 115 quality of life. 116 117 Conclusion 118 Our case calls attention to a rare and challenging diagnosis that is scrotal AVM. 119 Recurrent scrotal pain, swelling, and warmth together with varicocele should raise 120 suspicion for scrotal AVM. Treatment varies depending on the symptoms present. We 121 believe that embolization of the feeding arteries is a possible option to start with, and 122 surgery should be preserved for recurrent cases. 123 124 Authors’ Contribution 125 FMA, MSA, and SIA were responsible for conceiving the idea, literature search, data 126 acquisition, and manuscript writing and revision. AA, SA, YA, and AB were 127 primarily involved in the management of the case and critically reviewed the final 128 version of the manuscript. All the authors have read and approved the final version of 129 this manuscript. 130 131 References 132 1. Capitano M, Schieda N, Robertson SJ, et al. Images: Ruptured intratesticular 133 arteriovenous malformation. Can Urol Assoc J. 2018;12(11):E489-E491. 134 doi:10.5489/cuaj.5049 135 2. Lawton MT, Rutledge WC, Kim H, et al. Brain arteriovenous malformations. Nat 136 Rev Dis Primers. 2015;1:15008. Published 2015 May 28. doi:10.1038/nrdp.2015.8 137 3. Wong C, Leveillee RJ, Yrizarry JM, Kirby K. Arteriovenous malformation 138 mimicking a renal-cell carcinoma. J Endourol. 2002;16(9):685-686. 139 doi:10.1089/089277902761403050 140 4. Szpera-Goździewicz A, Gruca-Stryjak K, Bręborowicz GH, Ropacka-Lesiak M. 141 Uterine arteriovenous malformation - diagnosis and management. Ginekol Pol. 142 2018;89(5):276-279. doi:10.5603/GP.a2018.0047 143 5. Monoski MA, Gonzalez RR, Thomas AJ, Goldstein M. Arteriovenous 144 malformation of scrotum causing virtual azoospermia. Urology. 2006;68(1):. 145 doi:10.1016/j.urology.2006.01.019 146 6. Agrawal V, Dangle P, Minhas S, Ralph D, Christopher N. Recurrent arteriovenous 147 malformation of the scrotum secondary to pelvic trauma. Urol Int. 2006;77(4):365-148 7. doi: 10.1159/000096343. PMID: 17135789. 149 7. Sountoulides P, Bantis A, Asouhidou I, Aggelonidou H. Arteriovenous 150 malformation of the spermatic cord as the cause of acute scrotal pain: a case report. 151 J Med Case Rep. 2007 Oct 16;1:110. doi: 10.1186/1752-1947-1-110. PMID: 152 17939869; PMCID: PMC2194703. 153 8. Mohammad A, Sahyouni W, Almeree T, Alsaid B. Angioembolization of Scrotal 154 Arteriovenous Malformations: A Case Report and Literature Review. Case Rep 155 Vasc Med. 2020 Feb 8;2020:8373816. doi: 10.1155/2020/8373816. PMID: 156 32089946; PMCID: PMC7031727. 157 158 Figure 1: a picture of the left scrotal swelling with clear multiple skin varices. 159 160 161 Figure 2: the left image (A) showing a feeder artery supplying scrotal AVM. the right 162 image (B) angiogram following Onyx embolization through the AVM is almost 163 occluded. 164 165 166 Figure 3: partial left scrotal wall with AVM excision.167 A B Author Age Trauma History Presentation Semen analysis Varicocele Diagnosis method Embolization Treatment Follow-up Monoski et al.5 31 No history of trauma Infertility Severe oligospermi a A left varicocele Pelvic angiography Performed Surgical left scrotal AVM excision 3 years post- surgery, successful spontaneous pregnancy Agrawal et al.6 25 Positive -Severe pelvic fracture 4 years ago Pain associated with a soft swelling on his right testicle Not Performed No evidence of varicocele Histopathological examination Not performed ileo-femoral bypass surgery Not mentioned Sountoulides et al.7 22 No history of trauma Acute recurrent pain in the right hemiscrotum Not Performed No evidence of varicocele Post-orchiectomy specimen Not performed Orchiectomy 2 years post- surgery, there was no complain Mohammed et al.8 19 No history of trauma Progressive diffused swelling in the scrotum with flashing skin and local warmth Not Performed Varicocele with 1 cm dilated veins CT arteriography Performed The whole left side of the scrotum was removed, and the left testicle was fixed to the right side 12 months post- surgery, there was no complain Current case 37 No history of trauma Gradually increasing scrotal mass with on and off scrotal pain and discomfort Not performed Multiple skin varices over the left scrotum were seen Abdominal and pelvis CT Performed Partial scrotal wall excision Two months post- surgery, there was no complain 168