Long term follow up of 2 interesting cases
case1:
24 year old female
nChronic
bleeding/rectum, anal pain, anal incontinence
nLimb
deformity + haemangiomas
nHgb:
3.9gm%
Abdominal U/S
nNormal
liver
nPortal
vein cavernoma
nSplenomegaly
nUrinary
bladder
mass
Upper Endoscopy
nGrade
iv oesophageal varices with signs of impending rupture.
nCongestive
gastropathy
CT ABDOMEN & PELVIS
nCalcified
haemangioma of the rectum and sigmoid.
nTRIAD:
- Cutaneous Haemangiomas
-Varicose veins
(Limb +- pelvis)
-Hypertrophy of bone +- soft tissue of
a limb
KLIPPEL-TRENAUNAY-WEBER
syndrome
Associations
nRectal
varices
nHaemangiomas
of the colon, urinary
bladder and vagina
nVisceral
vascular malformations involving liver and kidney. (Portal
vein cavernoma)
nLymphatic
vascular malformations
Problems in our patient:
nBleeding:
-Portal hypertension
-Haemangioma of the rectosigmoid
-Pelvic vascular malformations (A-V fistulas
in rectal wall)
nAnal
incontinence + Anal Pain
Pressured pudendal nerve
Initial management
nBlood
transfusion
nOctreotide
drip (sandostatin)
nTranexamic
acid (Dycinone)
àBleeding
almost stopped and pain was reduced
§Propranolol
(Inderal)
à
Suicide attempt using inderal tablets!
Maintenance
treatment
nDiosmin
(Daflon)
nTranexamic
acid (Dycinone)
nHaematinics
(Haematon caps)
nAnalgesicsà
Nerve ablation (alcohol)
2 years later
àPersistent
bleeding/rectum
àHgb=5.5gm,
repeated blood transfusion
àPersistent
pain + incontinence
àSevere
depression
Options
nEndoscopic
- ligation - APC
nPortosystemic
shunt surgery
nEmbolotherapy
|
