Dislocated Posterior Chamber Intraocular Lens (PCIOL) in Patients with Retinitis Pigmentosa (RP)

Subluxation or dislocation of PCIOL is one of the complications of cataract operation in RP patients. This paper reports the presentation of PCIOL dislocation and subluxation and the management and outcome in 3 eyes of 2 RP patients. Two medical records of patients with RP who developed dislocated or subluxated PCIOL and subsequently underwent explantation of the dropped IOL were evaluated. Two patients had bilateral eye cataract operation done and had PCIOL implanted. Patient 1 developed left eye subluxated PCIOL inferiorly after 2 years of the cataract operation and right eye dislocated PCIOL anteriorly 4 years after cataract operation. Patient 2 develop right eye subluxated PCIOL inferiorly after 12 years of the cataract operation. Patient 1 with right eye dislocated PCIOL underwent intraocular lens (IOL) explantation and was left aphakic as her visual prognosis was poor due to advanced RP. The left IOL remained within the visual axis despite subluxation and no intervention has been done. Patient 2 with right eye subluxated PCIOL underwent IOL explantation and anterior chamber intraocular lens (ACIOL) implantation. ACIOL remained stable and visual acuity improved post-operation. Both the operations were uneventful. Post-operatively, there was no elevated intraocular pressure and no prolonged ocular inflammation, which required prolonged anti-inflammatory and no retinal detachment was seen. Both patient and surgeon should be aware of potential PCIOL subluxation or dislocation in RP. The presentation may be as late as more than a decade after the cataract operation.

Very long-chain acyl-CoA dehydrogenase deficiency presenting as acute hypercapnic respiratory failure.

Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) is a key enzyme catalysing the dehydrogenation of long-chain fatty acids in mitochondrial beta-oxidation. VLCAD deficiency is a genetic disorder that commonly presents in infancy or childhood with episodes of hypoketotic hypoglycaemia, cardiomyopathy and liver dysfunction. The present study reports an 18-yr-old Chinese female who presented with acute hypercapnic respiratory failure and rhabdomyolysis after a period of prolonged fasting and exertion. VLCAD deficiency was confirmed with decreased VLCAD activity in cultured fibroblasts. The patient completely recovered with supportive care. Pulmonary function tests after the acute episode showed evidence of chronic subclinical respiratory muscle weakness. In conclusion, this rare metabolic disorder should be considered in patients presenting with unexplained acute respiratory paralysis and failure.

Vertebral artery dissection: a treatable cause of ischaemic stroke.

Vertebral artery dissection used to be an uncommon diagnosis but it is now being diagnosed more frequently owing to the use of magnetic resonance imaging. We report on three patients with vertebral artery dissection to illustrate the importance of establishing this diagnosis by using magnetic resonance imaging in patients who present with cerebrovascular accident that involves the posterior territory. Treatment with heparin can help prevent recurrent embolic events and should be given in the absence of subarachnoid haemorrhage or other contra-indications.