SUBMITTED 22 SEP 21 1 REVISION REQ. 1 DEC 21; REVISION RECD. 21 DEC 21 2 ACCEPTED 6 JAN 22 3 ONLINE-FIRST: JAN 2022 4 DOI: https://doi.org/10.18295/squmj.1.2022.009 5 6 Urethral Caruncle with Associated Renoureteric Anomalies 7 Sohail Ahmad, *Akash B. Pati, Santosh K. Mahalik, Kanishka Das 8 9 Department of Pediatric Surgery, All India Institute of Medical Sciences, Bhubaneswar, India 10 *Corresponding Author’s e-mail: patiakash.ap@gmail.com 11 12 Abstract 13 A urethral caruncle is a rare differential diagnosis for a prolapsed mass at the urethral meatus in a 14 prepubertal girl. The aetiopathogenesis of this entity is unclear. We describe the presentation and 15 treatment of a patient with a urethral caruncle and associated renal anomalies that have not been 16 described earlier. The aetiology and treatment of the entity has also been discussed. 17 Keywords: Urethral Caruncle, Premature menarche, Paediatric Bleeding per vagina, Crossed 18 fused ectopia 19 20 Introduction 21 Prepubertal bleeding at the introitus is infrequent and raises concern among parents and health 22 providers alike. A wide variety of differential diagnoses are considered from an innocuous 23 abrasion to malignancy. Urethral etiologies include mucosal prolapse or polyps. A caruncle is a 24 rare cause that is commoner in middle aged or postmenopausal women. Of 14 cases of urethral 25 caruncle in pediatric age group described in English literature, only 4 have been documented 1 in 26 detail, and none had an associated upper urinary tract anomaly. We describe a patient with a 27 urethral caruncle, bleeding per urethra and associated upper urinary tract anomaly. 28 29 Case Report 30 A 3–year female with normal perinatal and past medical history presented with intermittent 31 mailto:patiakash.ap@gmail.com bleeding from the introitus for a month. There was no history of sexual abuse, genital trauma or 32 recurrent urinary tract infections. In the interim, she was asymptomatic. General physical 33 examination and abdominal examination was unremarkable. The external genitalia were 34 phenotypically female; however, the urethral meatus was circumferentially large and exuberant. 35 The mucosa was relatively more everted along the inferior meatal margin. (Figure 1- A, B). 36 37 The results of routine hematological and biochemical examinations were normal. The urinalysis 38 showed plenty of red blood cells. An ultrasonogram revealed a small left kidney (45X20mm), a 39 larger right kidney (69X28mm) and crossed fused renal ectopia. Renal cortical scintigraphy 40 (dimercapto succinic acid) showed a left to right crossed fused renal ectopia (Figure -2). The left 41 renal unit was hydronephrotic and had mildly impaired cortical function and a mid-polar cortical 42 irregularity. The differential function was 43% in the left renal unit and 57% in the right renal 43 unit which was normal. A voiding cystourethrogram was normal. 44 45 Examination under anesthesia and cystourethroscopy was performed. The urethral meatus was 46 wide (diameter 1.8cm) with its edges uniformly protuberant and firm. The mucosa was relatively 47 more everted at the inferior circumference. Cystoscopy revealed anormal urethra with a mildly 48 trabeculated bladder. The right ureteric orifice was normal, whereas the left was 49 grossly superolateral and patulous. Vaginoscopy was unremarkable. 50 51 The patient was discharged on Sitz bath (advised to sit immersed in a warm water bath up to hip, 52 aids in reducing congestion) twice daily and advised to apply 0.1% betamethasone cream locally 53 once daily. By 6 weeks of therapy, there was a significant reduction in size of the everted mucosa 54 which appeared normal. At 2 years follow up, there has been no further gross hematuria and the 55 lesion has disappeared completely. The initial hydronephrosis is non progressive and renal 56 function is stable on scintigraphy. Informed consent was obtained from the parents for publication 57 of this case report. 58 59 Discussion 60 The urethral caruncle is a common benign tumor of the female urethra usually seen in 61 postmenopausal women. An occurrence in the premenarche period, as described here, is rare. The 62 caruncle appears like a raspberry protruding from a quadrant commonly posterior wall of the 63 urethral circumference. It is rarely observed at other locations.2 A circumferential caruncle 64 mimicking a urethral prolapse has been reported only twice since 1964. 1 65 66 The exact etiology of urethral caruncle is unknown; however, chronic inflammation and estrogen 67 deficiency have been implicated.1 According to Jeffcott, a true caruncle is a vascular papilloma 68 that arises as a polyp from the posterior lip of the urethra, whereas a pseudo caruncle is a granuloma 69 arising as a diffuse, sessile red lesion. 3 Urethral caruncles have been reported at birth; hence, a 70 congenital origin is possible. 4 71 72 Symptoms include pain during micturition (51%), bleeding (49%), a mass at the meatus (41%) and 73 an increase in urinary frequency and urgency (36%)3 A ‘premature menarche’ without other 74 secondary sexual characters may point towards a bleeding urethral caruncle.5 The patient described 75 herein had gross intermittent hematuria and a prominent peri meatal mass. The clinical 76 differential diagnosis for a periurethral mass includes urethral prolapse, prolapsing ureterocele, 77 and a botryoid bladder or vaginal rhabdomyosarcoma. A urethral prolapse 78 79 protrudes circumferentially around the meatus like a soft rosette with a central dimple.6 Also, 80 both urethral prolapse and polyp are mucosa covered. In contrast, a caruncle is covered with 81 granulation tissue and is liable to bleed. Microscopically, the urethral caruncle is a bed of 82 granulation tissue that may feature squamous or transitional epithelium at places. Besides, marked 83 inflammatory infiltrate and vascular engorgement of the stroma is common. 84 85 Most authors consider them as acquired anomalies. 1,7 The present case features an ectopic left 86 kidney, ectopic termination of left ureter, and a urethral mega meatus. To the best of our 87 knowledge, such anomalies have not been previously reported. We suggest that patients with 88 urethral caruncle need to be evaluated for associated anomalies in the upper urinary tract. No 89 reports available regarding malignancy in urethral caruncle in children. However, in adults, 2.4% 90 of all patients with a preoperative diagnosis of urethral caruncle were found to have carcinoma.8 91 92 Various treatment modalities are advocated ranging from conservative management with Sitz bath, 93 estrogen creams, topical corticosteroids to surgery. 1,5,7 In unresponsive cases or those exhibiting 94 a progressive, irregular or suspicious growth, electrocoagulation of the base or excision are 95 surgical options. 1 Surgical excision may cause urethral stenosis in a circumferential lesion. The 96 present case responded favorably to the initial conservative management. 97 98 Conclusion 99 The patient had a granulated mass at the posterior margin of the circumference of the urethral 100 meatus that was diagnosed as a urethral caruncle. She presented with hematuria instead of the 101 typical blood spotting. It was associated with a crossed fused renal ectopia and a routine 102 ultrasonographic screening is suggested to detect associated reno ureteric anomaly. It was 103 managed conservatively with steroid creams and Sitz baths. 104 105 Authors’ Contributions 106 All authors were involved in the conceptualization and preparing of the manuscript. All authors 107 approved the final version. 108 109 References 110 1. Chiba M, Toki A, Sugiyanma A, Suganuma R, Osawa S, Ishii R et al . Urethral caruncle 111 in a 9-year-old girl: A case report and review of the literature. J med case reports, 2015: 112 28:9 https://doi.org/10.1186/s13256-015-0518-7 113 2. Turkeri L, Simsek F, Akdas A. Urethral caruncle in an unusual location occurring in 114 prepubertal girl.Eur. Urol. 1989;16:153-4 https://doi.org/10.1159/0004715553 115 3. Becker LE. Urethral caruncle: a herald lesion for distal urethral stenosis? J Natl Med 116 Assoc. 1975; 67: 228-30 117 4. Jarvi OH, Marin S, De Boer WG. Further studies in intestinal heterotopia in urethral 118 caruncle. Acta Pathol Microbiol Immunol Scand.1984;92(6):469 119 https://doi.org/10.111/j4.tb04429.x 120 5. Manori G, Beneragama D. Urethral caruncle presented as premature menarche in a 4- 121 year-old girl. Case report in pediatrics. 2018, https://doi.org/10.1155/2018/34860326 122 6. Murphy JP, Gatti JM. Abnormalities of the urethra, penis and scrotum. In : Coran AG 123 editor Pediatric Surgery 7th ed Philadelphia Elseiver Saunders; 2o12. P 1558 124 https://doi.org/10.1186/s13256-015-0518-7 https://doi.org/10.1159/0004715553 7. Kim KK, Sin DY, Park HW. Urethral caruncle occurring in a young girl: A case 125 report. J Korean Med Sci. 1993;8:160-1 https://doi.org/10.3346/jkms.1993.8.2.160 126 8. Marshall FC, Uson AC, Melicow MM. Neoplasms and caruncles of the female urethra. 127 Surg Gynecol Obstet. 1960 ;110:723-33. PMID: 14421527. 128 129 130 Figure 1: A: Hyperaemic exuberant mucosa at the urethral meatus. B: The exuberant mucosa 131 was more prononced inferiorly 132 133 Figure 2: Renal Scintigraphy showing left to right crossed fused renal ectopia. 134 https://doi.org/10.3346/jkms.1993.8.2.160