klippel  trenaunay syndrome ( kts ) is a rare congenital malformation first described in 1900 by maurice klippel and paul trenaunay .
kts is characterized by the triad of capillary malformations , atypical venous malformations and varicosities , and bony and/or soft tissue hypertrophy .
kts is estimated to affect 1 in 100 000 people worldwide with no predilection to race or sex .
an 18-year - old man presented for consideration of exenteration of the rectum and giant venous malformations extending out the sciatic notch .
he had experienced multiple significant episodes of haematochezia with associated anaemia ( haemoglobin of 4.5 g / dl ) and lethargy .
this is on a background of kts complicated by a right leg amputation , bleeding skin lesions and chronic haematochezia .
a non - obstructive pedunculated malformation was present near the ileocaecal valve occupying 50% of the lumen with the majority of malformations present in the rectum as a diffuse - dilated venous plexus ( fig .  1 ) .
n - of-1 random allocation was performed to inject the rectum with 5 ml of phenol in almond oil to the left and 20 ml of 75% ethanol to the right - sided vessels just beyond the anorectal junction .
the patient returned at 6 months for a flexible sigmoidoscopy to assess the rectal venous malformations . at the time of presentation
the flexible sigmoidoscopy again identified the previous venous malformations with an obvious but subjective reduction in the right - sided lesions ( ethanol - injected lesions ) more so than the left ( fig .  2 ) .
a further 20 ml of 75% ethanol was injected into the left - sided lesions as well as 10 ml of 75% ethanol into the right - sided lesions .
the rate of venous malformations in patients with kts is 70% . however , case studies suggest that the rate of problematic haematochezia associated with kts is quite low [ 1 , 3 ] .
surgical management of rectal haemangiomas is dependent on the extent of the anatomy of the haemangioma .
proctocolectomy with coloanal anastomosis is an option that allows for preserved anal function with control of bleeding .
however , extension of the haemangioma to the perianal and perineal region may require abdominoperineal resection with a permanent colostomy .
parashette and cuffari describe the case of a child with kts complicated by haematochezia where endoscopic sclerotherapy with 75% ethanol was successful .
ethanol has been used in sclerotherapy for many years and is tolerated in most cases . however , systemic toxic side - effects of ethanol have been reported in the literature .
minor side - effects of percutaneous ethanol include skin blistering , ulceration and scar formation , while major side - effects include pulmonary embolism , cardiovascular collapse and death .
wong et al .   have described cardiovascular collapse in the case of an 11-year - old child with kts that underwent ethanol sclerotherapy .
yakes and baker have described desaturation , bradycardia and cardiopulmonary collapse in three adults who underwent ethanol sclerotherapy .
retrospective study has suggested that the only factor significantly associated with a systemic adverse event is the ethanol dose / weight ratio per intervention .
suggest a maximal ethanol dose / weight ratio of 0.2 ml / kg in the treatment of venous malformations with ethanol as a sclerosant .
this case offers further support for 75% ethanol as a sclerosant in the management of rectal venous malformations and their use in an adult population .
it is important to note that the options for treatment of rectal venous malformations include surgery and/or endoscopic sclerotherapy , and that each of these options is associated with its own complications .
evidence does not exist in support of one mode of therapy over the other and the treatment option should be based on an individual assessment .
 klippel  trenaunay syndrome ( kts ) is a rare congenital disorder characterized by the triad of capillary malformations , atypical venous malformations and varicosities and bony and/or soft tissue hypertrophy . 
 we present the case of an 18-year - old man with kts affected by haematochezia secondary to rectal venous malformations that was managed with endoscopic sclerotherapy . in this case 
 , we compared the use of ethanol to phenol as a sclerosant . 
INTRODUCTION
CASE REPORT
DISCUSSION