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INTRODUCTION: Klippel-Trenaunay Syndrome (KTS) is a rare genetic disorder which is characterised by vascular nevi, venous varicosity, and hyperplasia of soft tissue or bone. Renovascular involvement is considered uncommon in KTS. PRESENTATION OF CASE: A 79-year-old man presented with a left sided varicocele, lymphedema, hydrocele, and microscopic haematuria. After a series of investigations his imaging and clinical features were suggestive KTS. His images, which showed a 2.7 cm renal artery aneurysm, were discussed in the multi-disciplinary team (MDT) meeting and the decision was made to proceed with a laparoscopic nephrectomy. DISCUSSION: Given the size of the aneurysm, treatment was offered and accepted by the patient. This is first recorded case in the literature in which a successful laparoscopic nephrectomy has been carried out for preventing severe haemorrhage in KTS. The patient presented in his 7th decade with a varicocele which would be considered unusual for KTS. Like in many cases, the renal artery aneurysm was asymptomatic. Pathological outcomes of the sample confirmed features suggestive of KTS, thus, validating the radiological findings. CONCLUSION: Here, we report a favourable outcome of a patient referred for consideration of varicocele management, diagnosed with renal artery aneurysms on a background of KTS. KTS, with significant renovascular abnormalities, can be treated with laparoscopic nephrectomy. Careful discussion in MDT with regards to management options should be performed and a shared decision reached with the patient with regards to management. Though rare, patients presenting with varicoceles and lymphedema may have underlying capillary-lymphatic-venous malformations.
RESUMO
OBJECTIVE: Visceral venous aneurysms are very rare, especially in the kidney. The diagnosis of renal venous aneurysms is difficult. If complications such as thrombosis, embolism or rupture, there can be corresponding clinical symptoms. In severe cases, it can lead to the death of the patient. Endoscopic resection of renal venous aneurysms has not been reported in the literature. This paper preliminarily discusses the experience of laparoscopic resection of renal venous aneurysms. METHODS: Recently, a patient with left retroperitoneal space occupying lesion was admitted to our hospital. More than a year ago, the patient was found to have left retroperitoneal space occupying lesion by CT plain scan, accompanied by occasional upper abdominal and precordial discomfort at night. After admission, enhanced CT showed that the size of the space occupying lesion was about 3.0×2.0×2.0 cm, adjacent to the left abdominal aorta, left renal artery and left renal vein. The space occupying density was similar to that of renal parenchyma in the unenhanced phase, whereas the enhancement was less pronounced in the arterial phase, more pronounced in the venous phase, and the attenuation was less pronounced in the delayed phase. After further refining the preoperative preparation, the surgical approach was "transabdominal 3D laparoscopic left retroperitoneal space occupying resection". Intraoperatively, a space occupying was found at the angle between the abdominal aorta and renal pedicle vessels, which were dark red, soft in quality and had a heavy adhesion to the renal artery. An atraumatic vascular clip was used to block the left renal artery, the gap between the free renal artery and the space occupying, and then the renal artery noninvasive vascular clip was loosened. Continuing free space occupying, we found that the space occupying originated from the left renal vein, gradually enlarged, terminated at the psoas muscle, and connected with the renal vein approximately 1 cm in width. Closely apposed renal veins were blocked with a vascular clip, clipped, and finally a complete resection space was taken. RESULTS: The procedure was uneventful, without trauma to the surrounding tissue organs. After complete resection of retroperitoneal mass, the patient recovered well. No complications were found, and the discomfort symptoms disappeared. The pathological result was renal venous aneurysm, which was considered due to lumbar venous variation. CONCLUSION: No treatment modality for the endoscopic resection of renal venous aneurysms has been documented, and the previous treatment modalities were usually nephrectomy or intervention. This surgical procedure may be the first in the world and open a new way for the diagnosis and treatment of renal venous aneurysms.