DR. ALEXANDER EBENEZAR

DR. SHIVANAND BUBANALE, DR. SMITHA K.S., DR. SANKALP KAKDE

Abstract

A 14 year old girl presented with bilateral nasal subluxation of lens with DOV since 7 years,her antenatal and postnatal history is uneventful.she is a term baby with birth weight of 2.2 kg and no significant family history. O/E : Asthenic thin normal intellectual activity Steinberg sign and thumb sign is positive,disproportionately long arms and legs,fingers are normal no knuckle hyperpigmentation and joint laxaity is normal.Ocular findings: BCVA: OD 6/24 OS 6/24 Near vision:OD N36 OS N36 IOP: OD 15 OS 13.5 mmhg,.blood homocysteine 35.65 vit b12 253.10,2D Echo: normal,Bscan : normal ,planned for phacoaspiration+bagpreservation+ring placement +PCIOL under GA .

Conclusion:we believe that homocystinuria should not be consider for general anesthesia due to high risk of thrombosis .In pyridoxine responsive patient general anesthesia is not hazardous if the platelet function can be controlled by the vitamine