The patient in his childhood, had undergone multiple surgeries for arteriovenous malformations (AVMs) in his right lower limb. He was diagnosed with Klippel Ternaunay syndrome involving lower limb, distal rectum, anal canal perineum and spleen. The patient had been passing a large amount of blood in his stools with each bowel movement for the past three-four years and required three-four units of blood transfusions every month.
On evaluation, a contrast CT scan showed varicosities and AVMs in the right lower limb and gluteal region and multiple dense varicosities and AVMs around the rectum. As the patient’s lesions were not easily manageable by intervention radiology approach, he was advised surgery. The resection was successfully performed with the help of vessel sealing devices and done without a stoma. The patient has made an uneventful recovery after the surgery.
Rectal bleed in Klippel-Trenaunay syndrome is unusual and as per the medical literature only nine such cases have been successfully managed through surgery, according to the medical team. The laparoscopic approach was reported only in a couple of cases. The medical team further said that the excellent result in successfully managing the case s is also an example of multidisciplinary team approach as well as technical expertise in laparoscopic surgery supported by excellent equipment and infrastructure in managing such cases at Aster Medcity.
