Peripapillary Retinal Haemorrhages in Wernicke’s Encephalopathy Following Bariatric Surgery in a Young Patient

ABSTRACT

Wernicke’s encephalopathy (WE) is an acute neuropsychiatric syndrome resulting from thiamine deficiency (vitamin B₁). It is characterised classically by a triad of ophthalmoplegia, confusion, and ataxia. WE is classically associated with alcoholism but increasingly has been observed due to other causes, particularly in undernourished post-bariatric surgery patients. Herein, we describe a case of WE following laparoscopic sleeve gastrectomy in a young male patient who presented with binocular horizontal diplopia and was found to have preretinal peripapillary haemorrhages. This case raises the awareness that posterior segment findings can occur in WE but have been under-reported previously.

Introduction

Vitamin B₁, or thiamine, is a water-soluble vitamin that functions as a cofactor for energy production. There is a limited intracellular reserve so constant cellular resupply is needed.¹ Thiamine deficiency can manifest with two clinical phenotypes: Wernicke-Korsakoff’s syndrome and beriberi. Wernicke’s encephalopathy is characterised classically by a triad of ophthalmoplegia, confusion, and ataxia. Korsakoff’s syndrome (KS) occurs as a late complication of WE and is defined by memory impairment associated with confabulation. KS has a mortality rate of 20% if left untreated. Beriberi may present with congestive cardiac failure (wet beriberi) or polyneuropathy (dry beriberi).¹ WE can occur in the setting of poor nutrition or absorption, for example, after bariatric surgery. Whenever there is clinical suspicion, thiamine replacement should be started immediately because of the cognitive consequences of any delay. Herein, we describe a case of WE following laparoscopic sleeve gastrectomy in a young male patient who presented with binocular horizontal diplopia and was found to have preretinal peripapillary haemorrhages.

Case report

A 17-year-old male presented to our tertiary eye hospital emergency room complaining of seeing double for 10 days associated with variable headache, lower limb weakness, a feeling of imbalance, and an inability to walk (requiring a wheelchair for ambulation). He had undergone laparoscopic sleeve gastrectomy (LSG) 8 weeks prior to presentation. He was given oral multivitamin tablets and was scheduled weekly vitamin B₁₂ injections. However, he was not compliant with the supplements or injection appointments, and his overall nutrition and appetite were poor since the surgery. He denied any history of substance abuse. On physical examination his blood pressure was 120/73 mmHg and body temperature was 36.6°C. His corrected visual acuity was 20/25 in the right eye (OD) and 20/30 in the left eye (OS). He could see 15/15 of the Ishihara plates with each eye and the confrontation visual fields were normal. The intraocular pressure was 16 mmHg OD and 18 mmHg OS. His pupil examination was within normal limits. The ocular motility examination showed right and left gaze palsies with intact convergence associated with gaze-evoked nystagmus (Figure 1a). The anterior segment examination was within normal limits. Fundus examination revealed hyperaemic optic discs in both eyes associated with preretinal peripapillary haemorrhages in the right eye (Figure 2) and peripapillary telangiectasia in the left eye. The macula and retinal vessels otherwise appeared to be normal. The rest of the cranial nerve examination was unremarkable. The upper limb exam was normal. The lower limb exam showed weakness of dorsiflexion with impaired sensation in the toes. The cerebellar function was normal. A computed tomography scan of the brain and orbit showed no detectable abnormalities. Magnetic resonance imaging showed bilateral medial thalamus linear diffusion restriction, suggestive of WE (Figure 3). Nerve conduction studies were normal and electromyography showed subacute axonal degeneration. He was admitted for laboratory work-up and treatment. His initial vitamin B₁ level was on the lower side of the normal range at 75.42 nmol/L (normal range: 74–222 nmol/L), which helped to confirm the clinical diagnosis of WE. He was started on intravenous thiamine 500 mg three times per day (TID) for 3 days followed by 250 mg TID for 5 days and was advised to continue on 100 mg oral thiamine per day for life. He responded quickly to the parenteral therapy with resolution of the diplopia and the gaze palsies within a few days (Figure 1b).

Figure 1.

(a) Composite gaze photographs at presentation showing bilateral horizontal gaze restriction. (b) After thiamine therapy the eye movements have returned to normal.

Figure 2.

Fundus photograph of the right eye showing a hyperaemic optic disc with two areas of preretinal haemorrhage.

Figure 3.

Magnetic resonance imaging finding. Axial (a) T2 (b) fluid attenuated inversion recovery and (c) diffusion weighted imaging at the level of the basal ganglia showing high signal lesions in both medial thalami.

Discussion

The characteristic presentation of thiamine deficiency following bariatric surgery may develop 4–12 weeks after the procedure. WE may occur following all types of bariatric surgical procedures. However, the development of WE after restrictive procedures, such as LSG, is very uncommon. A systematic review of the literature has identified only 16 cases of WE after restrictive weight loss procedure in all age groups, and only 36% of those patients reported a complete recovery. Poor outcome was attributed to delayed diagnosis and treatment.² This highlights WE being a clinical diagnosis and the importance of starting parenteral therapy as soon as possible without the need to wait for the laboratory results. Whenever the diagnosis is suspected, thiamine supplementation should be promptly started to prevent irreversible damage. A review by Armstrong-Javors et al. revealed only nine definitive cases of WE in adolescents undergoing bariatric surgery.³ Sechi et al. reported that only 16% of the WE cases in alcoholics exhibited all three features of the triad at presentation.¹ This is in contrast to Oudman et al. who reported that the full triad was present in 54.2% of WE cases post-bariatric surgery. The most common sign was ataxia (84.7%), followed by confusion (76.3%) then eye movement disorders (73.7%).⁴

Patients may also present with a wide range of neuro-ophthalmological symptoms and signs outside of the triad, like diplopia, as noted in our patient, and fundus findings, such as optic disc swelling, hyperaemia, peripapillary telangiectasia, and retinal haemorrhages, as noted in our patient. All of these signs have only rarely been previously documented in WE.^4,5 It has been postulated that deficiency of thiamine leads to the accumulation of free radicals that cause mitochondrial dysfunction, and this will cause the optic disc swelling, telangiectasia, and nerve fibre layer haemorrhages.⁶

Conclusion

WE is a serious condition that can be reversed easily with vitamin replacement. WE should be recognised as soon as possible to increase the chances of recovery and prevent permanent damage. In the modern era of bariatric surgery WE needs to be considered in every patient with neuro-ophthalmological findings, especially if there has been poor compliance with vitamin supplements. WE is a clinical diagnosis and even a low normal level of Vitamin B₁ can cause significant neurological deficits. This case report highlights the fundus findings in WE, which are scant in the literature.

Funding Statement

The authors reported there is no funding associated with the work featured in this article.

Disclosure statement

No potential conflict of interest was reported by the authors.