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Gross Hematuria in a 3-Year-old Girl Caused by a Large Isolated Bladder Hemangioma
Pediatric Case Report Gross Hematuria in a 3-Year-old Girl Caused by a Large Isolated Bladder Hemangioma Richard A. Ashley and T. Ernesto Figueroa Hemangiomas of the urinary tract can arise anywhere along the system. Although rare, these lesions can result in significant hematuria, with potential for patient morbidity. We report a case of an isolated bladder hemangioma identified in a 3-year-old girl presenting with recurrent high-volume gross hematuria. The lesion was not amenable to endoscopic resection because of its size. We present our operative management strategy as a potential method for other urologists who may be presented with similar scenarios. UROLOGY 76: 952–954, 2010. © 2010 Elsevier Inc.
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ladder hemangioma are rare causes for gross hematuria. These lesions are infrequent in both adult and pediatric settings, and may occur sporadically or part of a syndrome, such as in the case of Klippel–Trenaunay (KT).1 This benign tumor is said to account for 0.6% of all bladder tumors.2 Three histologic subtypes have been characterized, with the cavernous form being the most common, followed by capillary and arteriovenous subtypes. These vascular tumors range in size from less than 1 cm to more than 10 cm, with median size for most hemangiomas at 0.7 cm.3,4 Most patients with bladder hemangiomas present with gross hematuria and cystoscopy often reveals the lesion located on the posterior and lateral bladder walls. In many cases endoscopic treatments are the procedures of choice, with surgical resection reserved for more extensive clinical scenarios1,4-6 Herein we report our unique case of a 3-year-old girl presenting with multiple episodes of gross hematuria who was found to have an extensive bladder hemangioma. This lesion warranted open surgical resection; our treatment strategy is reviewed.
CASE REPORT A 3-year-old girl presented to our emergency room with a history of 8 days of intermittent gross hematuria progressing to clots. She did not have any signs of urinary tract infection but did have pharyngitis. Physical examination was normal with the exception of erythema in the posterior pharynx. No cutaneous rashes or lesions were identified. Renal– bladder ultrasound was performed and was found to be normal. Urinalysis revealed gross
From the Department of Urology, Nemours A. I. DuPont Hospital for Children, Wilmington, Delaware Reprint requests: Richard A. Ashley. Department of Urology, Nemours A.I. DuPont Hospital for Children, 1600 Rockland Road, Wilmington, DE 19803. E-mail: [email protected] Submitted: March 9, 2010, accepted (with revisions): March 23, 2010
952
© 2010 Elsevier Inc. All Rights Reserved
hematuria (50 red blood cells), whereas urine culture showed no growth. Calcium:creatinine ratio was calculated at 0.3. Rapid Strep test was positive, and the child was dismissed with the working diagnosis of Streptococcal pharyngitis. However, within 4 days, the hematuria progressed, and the child returned to the emergency room. Upon physical examination, blood clots were noted in the child’s underwear without any signs of trauma or vaginal laceration. Repeat urinalysis revealed persistent gross hematuria and urine gram stain was not suggestive of infection. Blood counts, electrolytes, and coagulation studies were all normal. It was determined that the girl warranted cystoscopic evaluation to identify the cause for hematuria and to evacuate clots. At cystoscopy, blood clots were evacuated, and a large 4 ⫻ 2 cm bladder hemangioma was visualized on the posterior and left lateral bladder wall (Figure 1a). There was no persistent bleeding. No fulgeration or biopsies were performed, as the lesion had not been fully characterized by imaging studies. The patient was stable, voided spontaneously without hematuria, and was followed closely for 4 weeks. There were small-volume intermittent bleeding episodes during this time period. She returned for magnetic resonance imaging (MRI) to determine the extent of the hemangioma. This revealed a large vascular lesion with an area of 2 ⫻ 2 cm appearing to extend in transmural fashion (Figure 1b). Based on the patient’s clinical history and evaluation, it appeared safest to proceed with open surgical resection. At surgery, the bladder hemangioma visualized endoscopically without bleeding. We approached the lesion via Pfannenstiel incision followed by a completely extraperitoneal dissection. The palpable portion of the hemangioma was identified on the exterior of the bladder. The bladder was hydrodistended and elevated in stay sutures for full surgical control. With dimmed operating room lights, and full cystoscopic illumination (Figure 2), the lesion was circumscribed from outside the bladder 0090-4295/10/$34.00 doi:10.1016/j.urology.2010.03.062
Figure 2. After careful extravesical exposure and bladder elevation, cystoscopic illumination was used to achieve a safe resection margin and avoid disturbing the normal surrounding bladder tissue.
Figure 1. (A) Cystoscopic assessment of 3-year-old girl’s bladder hemangioma with a raw surface not actively bleeding. (B) Depiction of how magnetic resonance imaging was used to fully characterize the hemangioma (4 ⫻ 2 cm), bladder wall, and surrounding structures.
while maintaining a safe margin. Pathologic examination confirmed this was a cavernous hemangioma-lymphangioma measuring 3.8 ⫻ 2 ⫻ 2.5 cm without extension beyond the muscularis. The bladder was closed in 2 layers of running 4-0 polydioxanone. Estimated bladder capacity was 125 mL. A urethral catheter was maintained for 4 days and, once removed, the patient was able to void spontaneously without hematuria before hospital dismissal.
COMMENT Gross hematuria in children is a rare event and is often associated with benign conditions.7,8 In this report, we detailed a clinical course for 1 young patient who had UROLOGY 76 (4), 2010
persistent gross hematuria with clots. Cystoscopy was warranted as the history and clinical scenario were not suggestive of the common reasons for gross hematuria. Unlike most prior reports, the size and MRI characteristics of her lesion precluded endoscopic resection. Open surgery was elected as the safest management strategy. Our case is interesting in that this was an isolated hemangioma. The lesion was located well away from the trigone, similar to those in other reports, but her tumor was larger than most, especially given her young age. Early reports suggested that biopsy should be avoided because only a small portion of the hemangioma may appear at cystoscopy.3 We agree with avoiding biopsy or endoscopic resection of large hemangiomas until the lesion has been fully characterized. This could result in significant bleeding that would be very difficult to control through a small pediatric resectoscope. Unfortunately, standard ultrasound was insufficient to fully characterize the lesion, as other authors have noted.3 Therefore, we found MRI valuable in fully defining the tumor. This information led us to elect an open surgical approach. Although prior reports and case series detail that many lesions can be treated endoscopically,5,6,9 in this scenario, the risks of the endoscopic approach were outweighed by the benefits of open surgery. Although spontaneous regression of some bladder hemagioma via fibrosclerosis have been described,3 this child’s lesion was large, caused significant morbidity, and did not show signs of improvement over a 4-week observation period. We recognize this is 1 case report of a rare event, and that the diagnostic process that we followed, along with our surgical approach, may not apply in all situations of bladder hemangioma. However, the implications of our report are significant and will assist others. When gross hematuria is persistent and is not explained by other common etiologies, we believe that cystoscopy is warranted to rule out uncommon causes of lower urinary tract bleeding.7 In addition, we support the use of MRI to fully define the soft tissues surrounding a bladder hemangioma to provide the most accurate preoperative plan. 953
We would recommend this study in cases of large bladder hemangioma before attempting resection with a pediatric resectoscope in which visualization can be compromised once intravesicular bleeding ensues. For these scenarios, open surgical resection with transvesical illumination provides for tissue control and precise removal of these vascular lesions. References 1. Husmann DA, Rathburn SR, Driscoll DJ. Klippel-Trenaunay syndrome: incidence and treatment of genitourinary sequelae. J Urol. 2007;177:1244-1249. 2. Grignon DJ. Neoplasms of the urinary bladder. In: Bostwick DG and Eble JN, eds. Urologic Surgical Pathology. St. Louis: Mosby-Year Book; 1997.
954
3. Jahn H, Nissen HM. Haemangioma of the urinary tract: review of the literature. Br J Urol. 1991;68:113-117. 4. Cheng L, Nascimento AG, Neumann RM, et al. Hemangioma of the urinary bladder. Cancer. 1999;86:498-504. 5. Smith JA Jr. Laser treatment of bladder hemangioma. J Urol. 1990; 143:282-284. 6. Tavora F, Montgomery E, Epstein JI. A series of vascular tumors and tumorlike lesions of the bladder. Am J Surg Pathol. 2008;32:12131219. 7. Greenfield SP, Williot P, Kaplan D. Gross hematuria in children: a ten-year review. Urology. 2007;69:166-169. 8. Quigley R. Evaluation of hematuria and proteinuria: how should a pediatrician proceed? Curr Opin Pediatr. 2008;20:140-144. 9. Kato M, Chiba Y, Sakai K, Orikasa S. Endoscopic neodymium: yttrium aluminium garnet (Nd: YAG) laser irradiation of a bladder hemangioma associated with Klippel-Weber syndrome. Int J Urol. 2000;7:145-148.
UROLOGY 76 (4), 2010
B
ladder hemangioma are rare causes for gross hematuria. These lesions are infrequent in both adult and pediatric settings, and may occur sporadically or part of a syndrome, such as in the case of Klippel–Trenaunay (KT).1 This benign tumor is said to account for 0.6% of all bladder tumors.2 Three histologic subtypes have been characterized, with the cavernous form being the most common, followed by capillary and arteriovenous subtypes. These vascular tumors range in size from less than 1 cm to more than 10 cm, with median size for most hemangiomas at 0.7 cm.3,4 Most patients with bladder hemangiomas present with gross hematuria and cystoscopy often reveals the lesion located on the posterior and lateral bladder walls. In many cases endoscopic treatments are the procedures of choice, with surgical resection reserved for more extensive clinical scenarios1,4-6 Herein we report our unique case of a 3-year-old girl presenting with multiple episodes of gross hematuria who was found to have an extensive bladder hemangioma. This lesion warranted open surgical resection; our treatment strategy is reviewed.
CASE REPORT A 3-year-old girl presented to our emergency room with a history of 8 days of intermittent gross hematuria progressing to clots. She did not have any signs of urinary tract infection but did have pharyngitis. Physical examination was normal with the exception of erythema in the posterior pharynx. No cutaneous rashes or lesions were identified. Renal– bladder ultrasound was performed and was found to be normal. Urinalysis revealed gross
From the Department of Urology, Nemours A. I. DuPont Hospital for Children, Wilmington, Delaware Reprint requests: Richard A. Ashley. Department of Urology, Nemours A.I. DuPont Hospital for Children, 1600 Rockland Road, Wilmington, DE 19803. E-mail: [email protected] Submitted: March 9, 2010, accepted (with revisions): March 23, 2010
952
© 2010 Elsevier Inc. All Rights Reserved
hematuria (50 red blood cells), whereas urine culture showed no growth. Calcium:creatinine ratio was calculated at 0.3. Rapid Strep test was positive, and the child was dismissed with the working diagnosis of Streptococcal pharyngitis. However, within 4 days, the hematuria progressed, and the child returned to the emergency room. Upon physical examination, blood clots were noted in the child’s underwear without any signs of trauma or vaginal laceration. Repeat urinalysis revealed persistent gross hematuria and urine gram stain was not suggestive of infection. Blood counts, electrolytes, and coagulation studies were all normal. It was determined that the girl warranted cystoscopic evaluation to identify the cause for hematuria and to evacuate clots. At cystoscopy, blood clots were evacuated, and a large 4 ⫻ 2 cm bladder hemangioma was visualized on the posterior and left lateral bladder wall (Figure 1a). There was no persistent bleeding. No fulgeration or biopsies were performed, as the lesion had not been fully characterized by imaging studies. The patient was stable, voided spontaneously without hematuria, and was followed closely for 4 weeks. There were small-volume intermittent bleeding episodes during this time period. She returned for magnetic resonance imaging (MRI) to determine the extent of the hemangioma. This revealed a large vascular lesion with an area of 2 ⫻ 2 cm appearing to extend in transmural fashion (Figure 1b). Based on the patient’s clinical history and evaluation, it appeared safest to proceed with open surgical resection. At surgery, the bladder hemangioma visualized endoscopically without bleeding. We approached the lesion via Pfannenstiel incision followed by a completely extraperitoneal dissection. The palpable portion of the hemangioma was identified on the exterior of the bladder. The bladder was hydrodistended and elevated in stay sutures for full surgical control. With dimmed operating room lights, and full cystoscopic illumination (Figure 2), the lesion was circumscribed from outside the bladder 0090-4295/10/$34.00 doi:10.1016/j.urology.2010.03.062
Figure 2. After careful extravesical exposure and bladder elevation, cystoscopic illumination was used to achieve a safe resection margin and avoid disturbing the normal surrounding bladder tissue.
Figure 1. (A) Cystoscopic assessment of 3-year-old girl’s bladder hemangioma with a raw surface not actively bleeding. (B) Depiction of how magnetic resonance imaging was used to fully characterize the hemangioma (4 ⫻ 2 cm), bladder wall, and surrounding structures.
while maintaining a safe margin. Pathologic examination confirmed this was a cavernous hemangioma-lymphangioma measuring 3.8 ⫻ 2 ⫻ 2.5 cm without extension beyond the muscularis. The bladder was closed in 2 layers of running 4-0 polydioxanone. Estimated bladder capacity was 125 mL. A urethral catheter was maintained for 4 days and, once removed, the patient was able to void spontaneously without hematuria before hospital dismissal.
COMMENT Gross hematuria in children is a rare event and is often associated with benign conditions.7,8 In this report, we detailed a clinical course for 1 young patient who had UROLOGY 76 (4), 2010
persistent gross hematuria with clots. Cystoscopy was warranted as the history and clinical scenario were not suggestive of the common reasons for gross hematuria. Unlike most prior reports, the size and MRI characteristics of her lesion precluded endoscopic resection. Open surgery was elected as the safest management strategy. Our case is interesting in that this was an isolated hemangioma. The lesion was located well away from the trigone, similar to those in other reports, but her tumor was larger than most, especially given her young age. Early reports suggested that biopsy should be avoided because only a small portion of the hemangioma may appear at cystoscopy.3 We agree with avoiding biopsy or endoscopic resection of large hemangiomas until the lesion has been fully characterized. This could result in significant bleeding that would be very difficult to control through a small pediatric resectoscope. Unfortunately, standard ultrasound was insufficient to fully characterize the lesion, as other authors have noted.3 Therefore, we found MRI valuable in fully defining the tumor. This information led us to elect an open surgical approach. Although prior reports and case series detail that many lesions can be treated endoscopically,5,6,9 in this scenario, the risks of the endoscopic approach were outweighed by the benefits of open surgery. Although spontaneous regression of some bladder hemagioma via fibrosclerosis have been described,3 this child’s lesion was large, caused significant morbidity, and did not show signs of improvement over a 4-week observation period. We recognize this is 1 case report of a rare event, and that the diagnostic process that we followed, along with our surgical approach, may not apply in all situations of bladder hemangioma. However, the implications of our report are significant and will assist others. When gross hematuria is persistent and is not explained by other common etiologies, we believe that cystoscopy is warranted to rule out uncommon causes of lower urinary tract bleeding.7 In addition, we support the use of MRI to fully define the soft tissues surrounding a bladder hemangioma to provide the most accurate preoperative plan. 953
We would recommend this study in cases of large bladder hemangioma before attempting resection with a pediatric resectoscope in which visualization can be compromised once intravesicular bleeding ensues. For these scenarios, open surgical resection with transvesical illumination provides for tissue control and precise removal of these vascular lesions. References 1. Husmann DA, Rathburn SR, Driscoll DJ. Klippel-Trenaunay syndrome: incidence and treatment of genitourinary sequelae. J Urol. 2007;177:1244-1249. 2. Grignon DJ. Neoplasms of the urinary bladder. In: Bostwick DG and Eble JN, eds. Urologic Surgical Pathology. St. Louis: Mosby-Year Book; 1997.
954
3. Jahn H, Nissen HM. Haemangioma of the urinary tract: review of the literature. Br J Urol. 1991;68:113-117. 4. Cheng L, Nascimento AG, Neumann RM, et al. Hemangioma of the urinary bladder. Cancer. 1999;86:498-504. 5. Smith JA Jr. Laser treatment of bladder hemangioma. J Urol. 1990; 143:282-284. 6. Tavora F, Montgomery E, Epstein JI. A series of vascular tumors and tumorlike lesions of the bladder. Am J Surg Pathol. 2008;32:12131219. 7. Greenfield SP, Williot P, Kaplan D. Gross hematuria in children: a ten-year review. Urology. 2007;69:166-169. 8. Quigley R. Evaluation of hematuria and proteinuria: how should a pediatrician proceed? Curr Opin Pediatr. 2008;20:140-144. 9. Kato M, Chiba Y, Sakai K, Orikasa S. Endoscopic neodymium: yttrium aluminium garnet (Nd: YAG) laser irradiation of a bladder hemangioma associated with Klippel-Weber syndrome. Int J Urol. 2000;7:145-148.
UROLOGY 76 (4), 2010