Pediatric Case Report Multidisciplinary Management of Complex Vascular Malformation Invading Bladder Wall Amber K. Norris, Gresham T. Richter, and Stephen Canon A 5-year-old African American male patient, with a history of complex vascular anomalies, underwent treatment for an infiltrating urinary bladder malformation initially misdiagnosed as a rhabdomyosarcoma. Neodymium:yttriumaluminum-garnet laser was used to treat the bladder wall before surgical resection. Vascular malformations of the bladder are rare. This report demonstrates a multimodal technique for management of large infiltrating bladder venous and lymphatic malformation. At the 3-month follow-up, his symptoms have resolved and his cystoscopy demonstrated no evidence of residual disease. UROLOGY -: -e-, 2015. 2015 Elsevier Inc.
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ascular malformations (VMs) are lesions of abnormal vascular development that represent a defect in vascular morphogenesis. Although they can occur anywhere in the body, head and neck malformations comprise the majority seen clinically. As such, venous and lymphatic malformations involving the bladder are extremely rare and have not been reported in the literature. Because these lesions are progressive, intervention is necessary for complete resolution of disease. This case presents the treatment of a combined venous and lymphatic malformation where a multimodal technique was used to provide comprehensive management of disease.
CASE REPORT The patient presented to Urology with persistent gross hematuria. He had a history of vascular anomalies beginning from birth when he was originally diagnosed with a cyst in his groin and a nodule in his left foot, which later revealed to be a vascular malformation. The patient had no family history of VMs, and after meeting with a geneticist, no genetic defect or syndrome was identified. The patient later underwent ultrasound and fluoroscopic-guided sclerotherapy of the left thigh and left calf region when he was 3 years old. That same year, the patient underwent an additional treatment of sclerotherapy targeting malformations of the left lateral foot, thigh, and calf. Financial Disclosure: The authors declare that they have no relevant financial interests. From the Division of Pediatric Urology, Department of Urology, University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, Little Rock, AR; and the Division of Pediatric Otolaryngology, Department of Otolaryngology-Head and Neck Surgery, University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, Little Rock, AR Address correspondence to: Amber K. Norris, M.D., Division of Pediatric Urology, Department of Urology, University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, 1 Children’s Way, Little Rock, AR 72202. E-mail: [email protected] Submitted: September 21, 2014, accepted (with revisions): January 21, 2015
ª 2015 Elsevier Inc. All Rights Reserved
He later presented to Urology with hematuria and penile pain leading to evaluation with renal and bladder ultrasound and magnetic resonance imaging. A mass of the bladder suspicious for malignancy was observed (Fig. 1). Cystoscopy with bladder biopsy and frozen section was performed with findings consistent with malignancy rather than vascular anomaly. This did not fit the clinical picture, and on completion of the final pathology with immunohistochemistry staining, the mass was determined to be a mixed venous and lymphatic malformation rather than a malignancy. The decision was made to control the anomaly with less-invasive tools. Therefore, in conjunction with the Vascular Anomalies team, Urology returned for repeat cystoscopy with neodymium:yttrium-aluminum-garnet (Nd:YAG) photothermolysis of the venous component of the urinary bladder wall malformation under direct visual guidance with cystoscopy. A glass fiber Nd:YAG laser with a wavelength of 1064 nm was passed via the cystoscope and used to treat the venous malformation at 10-15 W with a pulse duration of 1.0 ms. On introduction of the cystoscope into the bladder via the urethra, 2 small venous anomalies were incidentally found in the proximal bulbar urethra and were treated superficially with the laser therapy. After the urethra, 1-2 cm2 of the bladder wall was treated with the laser at several locations with venous involvement. During the procedure, the Vascular Anomalies team observed the additional presence of a significant lymphatic component to the known venous anomaly making this a mixed vascular anomaly. Because of the extent of the lymphatic involvement and size of the mass, the consensus decision was to perform excision of the bladder vascular anomaly. The patient later returned to the operating room for excision of the mass (Fig. 2). Repeat cystoscopy revealed the patient’s bladder capacity to be 210 mL (estimated http://dx.doi.org/10.1016/j.urology.2015.01.025 0090-4295/15
1
Figure 1. Ultrasound of suspected bladder malignancy.
Figure 2. Venous and lymphatic mass measuring 3 cm2. (Color version available online.)
bladder capacity for a 5-year old ¼ 210 mL). A Pfannenstiel incision with pelvic exploration and en bloc resection of the vascular tumor of the bladder dome was performed without complication. Foley catheter drainage was maintained for 5 days with the catheter removed as an outpatient. Since excision of the mass, the patient’s hematuria and penile pain have resolved. Postoperative cystoscopy 3 months after excision of the lesion demonstrated resolution of his urethral venous anomalies and no evidence of recurrence of the bladder dome vascular anomaly.
COMMENT VMs are congenital vascular anomalies composed of irregular vascular networks. Through disruption of normal vein development resulting in an incongruous connection between venous channels, these lesions are created and expand as blood accumulates within the network of ill-draining veins. These lesions ultimately infiltrate normal tissue, and without treatment, these 2
malformations expand, become painful, and interfere with daily activities. VMs are very prevalent in the head and neck region, some articles citing up to 40% of all cases. However, they can occur anywhere in the body with the extremities and trunk comprising 40% and 20%, respectively.1 As such, the urinary system is rarely affected, and an anomaly in the bladder is extremely rare.2 Because of the rarity of such lesions in the pelvic area, multiple techniques were used during this case to maximize the target area and prevent further VM development. Multimodal therapeutic approaches are recommended to treat VMs.3 The location and size of the anomaly is often the defining factor in determining the treatment of choice. Reasonable disease control can be achieved using laser therapy on mucosally associated malformations. The Nd:YAG laser is selective to venous malformation causing direct photocoagulation. Sclerotherapy and surgical excision are used to manage deep and focal slow flow vascular anomalies, namely lymphatic and venous malformations. Laser therapy involves the process by which intravascular proteins absorb light energy; the absorption enables the proteins to heat, clot, and ultimately damage the vessel involved. Sclerotherapy involves the injection of a sclerosing agent directly into the malformation, leading to fibrosis and ultimately regression.4 Depending on the tortuous blood supply, as well as cellular composition of the VM, surgical excision may be the ideal management.5 The team began by using laser therapy on the venous component of the malformation to bolster the strength of the bladder wall and eliminate any mucosal disease. This provided primary control of urologic symptoms. In the case of a venous malformation, lasers with longer wavelengths are necessary for better treatment because this type of anomaly has larger blood vessels.3 Thus, the solidstate Nd:YAG laser (1064 nm) was the best choice to maximize results. Because the lesion also was composed of lymphatic malformation not amenable to laser therapy, the Vascular Anomalies and Urology teams agreed on excision rather than the other modalities to provide complete control of this well-circumscribed mass. Sclerotherapy ran the risk of inflammation and irritation of the bladder and other important intra-abdominal structures. A previous report cites 80% complete or near-complete resolution with 1 episode of excision treatment for lymphatic malformations.5 However, total removal of the mass is preferential for lymphatic malformations, and excision most reliably provides this outcome.6 VMs involving the bladder are extremely rare, but when present, can lead to hematuria and pain. These lesions are progressive and thereby ultimately require intervention using multimodal techniques. Little precedence exists for management of bladder vascular anomalies due to the rarity of this problem. To approach the VM in a comprehensive manner, a variety of medical technology and a multimodal team unit were UROLOGY
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(-), 2015
implemented for this case leading to an excellent shortterm outcome. References 1. Legiehn GM, Heran MK. Venous malformations: classification, development, diagnosis, and interventional radiologic management. Radiol Clin North Am. 2008;46:545-597. 2. Niu ZB, Hou Y, Sun RG, et al. Cystic lymphatic malformation of bladder presenting as a pelvic mass. J Pediatr Surg. 2011;46:12841287.
UROLOGY
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3. Richter GT, Braswell L. Management of venous malformations. Facial Plast Surg. 2012;28:1-8. 4. de Lorimier AA. Sclerotherapy for venous malformations. J Pediatr Surg. 1995;30:188-194. 5. Bajaj Y, Hewitt R, Ifeacho S, Hartley BEJ. Surgical excision as primary treatment modality for extensive cervicofacial lymphatic malformations in children. Int J Pediatr Otorhinolaryngol. 2011;75: 673-677. 6. Richter GT, Friedman AB. Hemangiomas and vascular malformations: current theory and management. Int J Pediatr. 2012;10: 645-678.
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V
ascular malformations (VMs) are lesions of abnormal vascular development that represent a defect in vascular morphogenesis. Although they can occur anywhere in the body, head and neck malformations comprise the majority seen clinically. As such, venous and lymphatic malformations involving the bladder are extremely rare and have not been reported in the literature. Because these lesions are progressive, intervention is necessary for complete resolution of disease. This case presents the treatment of a combined venous and lymphatic malformation where a multimodal technique was used to provide comprehensive management of disease.
CASE REPORT The patient presented to Urology with persistent gross hematuria. He had a history of vascular anomalies beginning from birth when he was originally diagnosed with a cyst in his groin and a nodule in his left foot, which later revealed to be a vascular malformation. The patient had no family history of VMs, and after meeting with a geneticist, no genetic defect or syndrome was identified. The patient later underwent ultrasound and fluoroscopic-guided sclerotherapy of the left thigh and left calf region when he was 3 years old. That same year, the patient underwent an additional treatment of sclerotherapy targeting malformations of the left lateral foot, thigh, and calf. Financial Disclosure: The authors declare that they have no relevant financial interests. From the Division of Pediatric Urology, Department of Urology, University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, Little Rock, AR; and the Division of Pediatric Otolaryngology, Department of Otolaryngology-Head and Neck Surgery, University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, Little Rock, AR Address correspondence to: Amber K. Norris, M.D., Division of Pediatric Urology, Department of Urology, University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, 1 Children’s Way, Little Rock, AR 72202. E-mail: [email protected] Submitted: September 21, 2014, accepted (with revisions): January 21, 2015
ª 2015 Elsevier Inc. All Rights Reserved
He later presented to Urology with hematuria and penile pain leading to evaluation with renal and bladder ultrasound and magnetic resonance imaging. A mass of the bladder suspicious for malignancy was observed (Fig. 1). Cystoscopy with bladder biopsy and frozen section was performed with findings consistent with malignancy rather than vascular anomaly. This did not fit the clinical picture, and on completion of the final pathology with immunohistochemistry staining, the mass was determined to be a mixed venous and lymphatic malformation rather than a malignancy. The decision was made to control the anomaly with less-invasive tools. Therefore, in conjunction with the Vascular Anomalies team, Urology returned for repeat cystoscopy with neodymium:yttrium-aluminum-garnet (Nd:YAG) photothermolysis of the venous component of the urinary bladder wall malformation under direct visual guidance with cystoscopy. A glass fiber Nd:YAG laser with a wavelength of 1064 nm was passed via the cystoscope and used to treat the venous malformation at 10-15 W with a pulse duration of 1.0 ms. On introduction of the cystoscope into the bladder via the urethra, 2 small venous anomalies were incidentally found in the proximal bulbar urethra and were treated superficially with the laser therapy. After the urethra, 1-2 cm2 of the bladder wall was treated with the laser at several locations with venous involvement. During the procedure, the Vascular Anomalies team observed the additional presence of a significant lymphatic component to the known venous anomaly making this a mixed vascular anomaly. Because of the extent of the lymphatic involvement and size of the mass, the consensus decision was to perform excision of the bladder vascular anomaly. The patient later returned to the operating room for excision of the mass (Fig. 2). Repeat cystoscopy revealed the patient’s bladder capacity to be 210 mL (estimated http://dx.doi.org/10.1016/j.urology.2015.01.025 0090-4295/15
1
Figure 1. Ultrasound of suspected bladder malignancy.
Figure 2. Venous and lymphatic mass measuring 3 cm2. (Color version available online.)
bladder capacity for a 5-year old ¼ 210 mL). A Pfannenstiel incision with pelvic exploration and en bloc resection of the vascular tumor of the bladder dome was performed without complication. Foley catheter drainage was maintained for 5 days with the catheter removed as an outpatient. Since excision of the mass, the patient’s hematuria and penile pain have resolved. Postoperative cystoscopy 3 months after excision of the lesion demonstrated resolution of his urethral venous anomalies and no evidence of recurrence of the bladder dome vascular anomaly.
COMMENT VMs are congenital vascular anomalies composed of irregular vascular networks. Through disruption of normal vein development resulting in an incongruous connection between venous channels, these lesions are created and expand as blood accumulates within the network of ill-draining veins. These lesions ultimately infiltrate normal tissue, and without treatment, these 2
malformations expand, become painful, and interfere with daily activities. VMs are very prevalent in the head and neck region, some articles citing up to 40% of all cases. However, they can occur anywhere in the body with the extremities and trunk comprising 40% and 20%, respectively.1 As such, the urinary system is rarely affected, and an anomaly in the bladder is extremely rare.2 Because of the rarity of such lesions in the pelvic area, multiple techniques were used during this case to maximize the target area and prevent further VM development. Multimodal therapeutic approaches are recommended to treat VMs.3 The location and size of the anomaly is often the defining factor in determining the treatment of choice. Reasonable disease control can be achieved using laser therapy on mucosally associated malformations. The Nd:YAG laser is selective to venous malformation causing direct photocoagulation. Sclerotherapy and surgical excision are used to manage deep and focal slow flow vascular anomalies, namely lymphatic and venous malformations. Laser therapy involves the process by which intravascular proteins absorb light energy; the absorption enables the proteins to heat, clot, and ultimately damage the vessel involved. Sclerotherapy involves the injection of a sclerosing agent directly into the malformation, leading to fibrosis and ultimately regression.4 Depending on the tortuous blood supply, as well as cellular composition of the VM, surgical excision may be the ideal management.5 The team began by using laser therapy on the venous component of the malformation to bolster the strength of the bladder wall and eliminate any mucosal disease. This provided primary control of urologic symptoms. In the case of a venous malformation, lasers with longer wavelengths are necessary for better treatment because this type of anomaly has larger blood vessels.3 Thus, the solidstate Nd:YAG laser (1064 nm) was the best choice to maximize results. Because the lesion also was composed of lymphatic malformation not amenable to laser therapy, the Vascular Anomalies and Urology teams agreed on excision rather than the other modalities to provide complete control of this well-circumscribed mass. Sclerotherapy ran the risk of inflammation and irritation of the bladder and other important intra-abdominal structures. A previous report cites 80% complete or near-complete resolution with 1 episode of excision treatment for lymphatic malformations.5 However, total removal of the mass is preferential for lymphatic malformations, and excision most reliably provides this outcome.6 VMs involving the bladder are extremely rare, but when present, can lead to hematuria and pain. These lesions are progressive and thereby ultimately require intervention using multimodal techniques. Little precedence exists for management of bladder vascular anomalies due to the rarity of this problem. To approach the VM in a comprehensive manner, a variety of medical technology and a multimodal team unit were UROLOGY
-
(-), 2015
implemented for this case leading to an excellent shortterm outcome. References 1. Legiehn GM, Heran MK. Venous malformations: classification, development, diagnosis, and interventional radiologic management. Radiol Clin North Am. 2008;46:545-597. 2. Niu ZB, Hou Y, Sun RG, et al. Cystic lymphatic malformation of bladder presenting as a pelvic mass. J Pediatr Surg. 2011;46:12841287.
UROLOGY
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(-), 2015
3. Richter GT, Braswell L. Management of venous malformations. Facial Plast Surg. 2012;28:1-8. 4. de Lorimier AA. Sclerotherapy for venous malformations. J Pediatr Surg. 1995;30:188-194. 5. Bajaj Y, Hewitt R, Ifeacho S, Hartley BEJ. Surgical excision as primary treatment modality for extensive cervicofacial lymphatic malformations in children. Int J Pediatr Otorhinolaryngol. 2011;75: 673-677. 6. Richter GT, Friedman AB. Hemangiomas and vascular malformations: current theory and management. Int J Pediatr. 2012;10: 645-678.
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