Ectopia lentis (EL) is the malposition of the natural lens caused by a defect in the ciliary zonules [1]. The most common cause for EL is Marfan syndrome (MFS) [2]. EL occurs in ~70% of patients with MFS [3, 4]. Up to 100% of patients with MFS have an abnormal cardiovascular system predisposing to increased mortality and morbidity [5]. Echocardiography is the mainstay of cardiological assessment for patients with MFS [6].
Ophthalmologists are well aware of the ocular risks of EL and routinely survey for these. However, ensuring cardiovascular assessments may be less-regularly documented. Herein we audit documentation of whether patients with EL are known to cardiologists, geneticists or physicians for their systemic cardiovascular risks.
Eligible patients were identified from the database of a tertiary ophthalmic centre: Moorfields Eye Hospital, London. A database search between 1 January 1959 and 31 December 2011 was performed. The inclusion criteria were all referrals for EL or for syndromes associated with EL. Patients were excluded if the EL diagnosis was not confirmed, or if patients were subsequently seen elsewhere.
The standard of the audit was that all patients should have documentation that the patient is under cardiovascular review.
In all, 191 patients fulfilled the inclusion criteria. Ninety-seven patients (51%) had documentation that they were under cardiovascular review. Of these, 38 (20%) were documented to be known as having cardiovascular issues, while 59 patients (31%) did not. A total of 94 patients (49%) had no documentation regarding cardiovascular problems.
Our findings describe the lack of documentation ensuring cardiovascular review for patients with EL. Ophthalmologists are well aware that the most common cause of EL is MFS and associated cardiovascular risks. It is important for patient safety that these patients are known to cardiology or medical services. Ophthalmologists first see ~40% of patients with MFS [7]. It is critical therefore to ensure that patients with EL are under the care of appropriate physicians. There are currently no guidelines on referral pathways for patients who present to ophthalmologists with EL, suggesting the need to review investigation and management of this cohort.
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest.
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